{"slug":"rare","tag":"rare","variants":["rare"],"description":"Cancers that are rare, where trials are small and expertise is concentrated.","count":106,"kinds":{"cancer":105,"trial":1},"related":[{"slug":"subtype-page","tag":"subtype-page","shared":70},{"slug":"wave4","tag":"wave4","shared":70},{"slug":"haematologic","tag":"haematologic","shared":22},{"slug":"kidney","tag":"kidney","shared":8},{"slug":"sarcoma","tag":"sarcoma","shared":7},{"slug":"testicular","tag":"testicular","shared":7},{"slug":"endocrine","tag":"endocrine","shared":5},{"slug":"paediatric","tag":"paediatric","shared":4},{"slug":"gastrointestinal","tag":"gastrointestinal","shared":3},{"slug":"gynaecologic","tag":"gynaecologic","shared":3},{"slug":"head-and-neck","tag":"head-and-neck","shared":3},{"slug":"skin","tag":"skin","shared":3}],"records":[{"id":"sarcoma","kind":"cancer","name":"Sarcomas (soft tissue, bone, GIST)","route":"/cancers/sarcoma/","tldr":"Sarcomas are dozens of rare cancers of bone and connective tissue. GIST was the first solid tumour cured-in-practice by a targeted pill; synovial sarcoma got the first TCR-T therapy."},{"id":"extragonadal-germ-cell-tumour","kind":"cancer","name":"Extragonadal germ cell tumour","route":"/cancers/extragonadal-germ-cell-tumour/","tldr":"Extragonadal germ cell tumours are the same cancers as testicular germ cell tumours but arising in the midline of the body, most often the chest or the back of the abdomen. Seminomas are highly curable with chemotherapy; non-seminomas of the chest are the hardest germ cell tumours to cure and are treated with intensive chemotherapy followed by surgery."},{"id":"gallbladder","kind":"cancer","name":"Gallbladder cancer","route":"/cancers/gallbladder/","tldr":"Gallbladder cancer starts in the small bile-storing sac under the liver and is one of the biliary tract cancers. Most cases are found late, or by chance when a gallbladder is removed for gallstones. It is rare in the UK, with about 1,300 cases a year, and much commoner in Chile, Bolivia and northern India. Found early, an operation can cure it."},{"id":"mcc-rational-treatment","kind":"trial","name":"Rational treatment selection for Merkel cell carcinoma","route":"/trials/mcc-rational-treatment/","status":"completed","tldr":"The only randomised trial ever attempted in Merkel cell carcinoma, run from Birmingham, closed to recruitment in 2018, and with no published result on its registry record: the reason the first treatment decision in this cancer is still made on opinion."},{"id":"bpdcn","kind":"cancer","name":"Blastic plasmacytoid dendritic cell neoplasm (BPDCN)","route":"/cancers/bpdcn/","tldr":"Blastic plasmacytoid dendritic cell neoplasm is a rare aggressive leukaemia-like blood cancer of dendritic-cell precursors, a few hundred US cases a year, that often first appears as bruise-like skin lesions. Two CD123-directed drugs, tagraxofusp and pivekimab sunirine, are the first targeted therapies; allogeneic transplant in first remission is still the only route to long-term control."},{"id":"merkel-cell-carcinoma","kind":"cancer","name":"Merkel cell carcinoma","route":"/cancers/merkel-cell-carcinoma/","tldr":"Merkel cell carcinoma is a rare, fast-growing skin cancer, usually caused by a common virus (Merkel cell polyomavirus) or by sun damage. Once it had spread there was no treatment that worked; PD-1/PD-L1 immunotherapy now gives lasting responses in about half of patients."},{"id":"uveal-melanoma","kind":"cancer","name":"Uveal melanoma","route":"/cancers/uveal-melanoma/","tldr":"A melanoma inside the eye that is biologically unrelated to skin melanoma: different mutations, no response to standard immunotherapy, and a tendency to spread to the liver years later. Tebentafusp is the first drug ever to extend survival in the metastatic disease."},{"id":"appendiceal","kind":"cancer","name":"Appendiceal cancer and pseudomyxoma peritonei","route":"/cancers/appendiceal/","tldr":"Rare tumours of the appendix that range from slow mucin-producing growths that fill the abdomen (pseudomyxoma peritonei) to aggressive adenocarcinomas. The slow forms are treated by extensive surgery with heated chemotherapy in the abdomen; the fast ones like colon cancer."},{"id":"gestational-trophoblastic","kind":"cancer","name":"Gestational trophoblastic neoplasia","route":"/cancers/gestational-trophoblastic/","tldr":"Cancers that grow from placental tissue after a pregnancy. They make a hormone (hCG) that acts as a perfect blood test, and they were the first solid cancer ever cured by chemotherapy. Immunotherapy now rescues the few that resist drugs."},{"id":"thymic-epithelial","kind":"cancer","name":"Thymoma and thymic carcinoma","route":"/cancers/thymic-epithelial/","tldr":"Thymoma and thymic carcinoma are rare tumours of the thymus gland in the chest. Thymomas grow slowly, often cause autoimmune diseases such as myasthenia gravis, and are usually cured by surgery; thymic carcinomas behave like other aggressive cancers and have few effective drugs."},{"id":"adrenocortical","kind":"cancer","name":"Adrenocortical carcinoma","route":"/cancers/adrenocortical/","tldr":"Adrenocortical carcinoma is a rare, aggressive cancer of the adrenal gland that often over-produces hormones. Surgery is the only cure, mitotane is the one drug specific to it (with real toxicity), and chemotherapy or immunotherapy help only a minority."},{"id":"salivary-gland","kind":"cancer","name":"Salivary gland cancers","route":"/cancers/salivary-gland/","tldr":"Salivary gland cancers are a family of over 20 rare cancers, each with its own behaviour and often its own gene fusion. Surgery and radiation treat most; drug therapy is now chosen by the specific subtype, from anti-HER2 or anti-androgen drugs to NTRK inhibitors."},{"id":"chordoma","kind":"cancer","name":"Chordoma","route":"/cancers/chordoma/","tldr":"Chordoma is a slow-growing bone cancer (a sarcoma) of the skull base and spine that arises from leftover embryonic notochord cells. Complete surgery followed by high-dose proton or carbon-ion radiotherapy controls most tumours, and the whole disease depends on a single transcription factor, brachyury, which vaccines and degraders are now trying to hit."},{"id":"desmoid-tumour","kind":"cancer","name":"Desmoid tumour","route":"/cancers/desmoid-tumour/","tldr":"Desmoid tumours are locally aggressive growths of fibroblast-like cells, classed with soft-tissue sarcomas, driven by WNT mutations, that never spread to distant organs but can invade nerves, bowel and muscle. Many stop growing or shrink on their own, so watching first is standard; if they progress, the gamma-secretase inhibitor nirogacestat, approved in 2023, shrinks tumours and relieves pain."},{"id":"tenosynovial-giant-cell-tumour","kind":"cancer","name":"Tenosynovial giant cell tumour (TGCT)","route":"/cancers/tenosynovial-giant-cell-tumour/","tldr":"TGCT is a benign but destructive tumour of the joint lining, classed with soft-tissue sarcomas, in which a few cells carrying a CSF1 gene fusion recruit a crowd of normal immune cells that eat away at the joint. Surgery cures most localised cases, and for diffuse or recurrent disease two pills that block the CSF1 receptor, pexidartinib and vimseltinib, shrink tumours and restore joint function."},{"id":"epithelioid-sarcoma","kind":"cancer","name":"Epithelioid sarcoma","route":"/cancers/epithelioid-sarcoma/","tldr":"Epithelioid sarcoma is a rare soft tissue cancer that has lost a gene brake called SMARCB1, leaving it dependent on the enzyme EZH2. Surgery cures localised tumours. The EZH2 inhibitor tazemetostat was approved in 2020 and withdrawn worldwide in March 2026 after secondary blood cancers in a lymphoma trial; the dependency it proved is still a target in development, and chemotherapy remains in use."},{"id":"inflammatory-myofibroblastic-tumour","kind":"cancer","name":"Inflammatory myofibroblastic tumour (IMT)","route":"/cancers/inflammatory-myofibroblastic-tumour/","tldr":"IMT is a rare tumour, grouped with the sarcomas, of spindle cells mixed with inflammatory cells, most often in the lung or abdomen of children and young adults. Surgery cures most, and about half carry an ALK gene fusion, so the ALK-blocking pill crizotinib is approved for those that cannot be removed, one of the first targeted approvals for a childhood solid tumour."},{"id":"vascular-tumours","kind":"cancer","name":"Vascular tumours (angiosarcoma, epithelioid haemangioendothelioma, kaposiform haemangioendothelioma)","route":"/cancers/vascular-tumours/","tldr":"Vascular tumours range from angiosarcoma, an aggressive cancer of blood vessel lining cells, to the slow-growing EHE and the infant tumour KHE. Angiosarcoma responds to paclitaxel and, in the sun-damaged scalp form, to immunotherapy; EHE and KHE depend on growth signals that the mTOR blocker sirolimus quiets, and EHE without symptoms is watched."},{"id":"uterine-sarcoma","kind":"cancer","name":"Uterine sarcoma","route":"/cancers/uterine-sarcoma/","tldr":"Uterine sarcomas are rare cancers of the muscle and supporting tissue of the womb, distinct from the far commoner endometrial cancer. Removing the uterus intact is the main treatment and is followed by observation for stage I disease; low-grade stromal sarcomas respond to hormone-blocking pills, while advanced leiomyosarcoma is treated with doxorubicin and trabectedin."},{"id":"nut-carcinoma","kind":"cancer","name":"NUT carcinoma (midline carcinoma with NUTM1 rearrangement)","route":"/cancers/nut-carcinoma/","tldr":"NUT carcinoma is a fast-growing cancer of the midline of the body driven by a single fused gene, BRD4-NUTM1, that locks cells in an immature state. Chemotherapy and surgery rarely control it for long, but drugs that block the BET proteins the fusion depends on have produced responses and are the focus of trials."},{"id":"sinonasal","kind":"cancer","name":"Nasal cavity and paranasal sinus cancers (including esthesioneuroblastoma)","route":"/cancers/sinonasal/","tldr":"Cancers of the nose and sinuses are a mixed group, from squamous carcinoma to the nerve-derived esthesioneuroblastoma and the aggressive undifferentiated carcinoma SNUC. Surgery through the nose with an endoscope followed by precise radiotherapy has replaced disfiguring open operations, and giving chemotherapy first to see who responds now guides how SNUC is treated."},{"id":"pheochromocytoma-paraganglioma","kind":"cancer","name":"Pheochromocytoma and paraganglioma (PPGL)","route":"/cancers/pheochromocytoma-paraganglioma/","tldr":"Pheochromocytomas and paragangliomas are tumours of adrenaline-producing tissue that cause dangerous blood pressure surges. Surgery after careful blood-pressure blockade cures most, genetic testing finds an inherited cause in nearly half, and for the minority that spread there are now radioactive drugs that home to the tumour and, since 2025, the first oral targeted pill, belzutifan."},{"id":"parathyroid-carcinoma","kind":"cancer","name":"Parathyroid carcinoma","route":"/cancers/parathyroid-carcinoma/","tldr":"Parathyroid carcinoma is a cancer of the glands that control blood calcium, seen in a few people per ten million a year; most of its harm comes from dangerously high calcium rather than spread. Removing the tumour intact at the first operation gives the best chance of cure, and cinacalcet and bone-protecting drugs control calcium when it cannot be removed."},{"id":"pituitary-tumours","kind":"cancer","name":"Pituitary tumours (pituitary neuroendocrine tumours) and pituitary carcinoma","route":"/cancers/pituitary-tumours/","tldr":"Pituitary tumours are usually benign growths of the hormone gland at the base of the brain that cause trouble by overproducing hormones or pressing on the optic nerves. Prolactin-producing tumours melt away with a tablet, most others are cured by surgery through the nose, and the rare aggressive ones respond to the chemotherapy drug temozolomide."},{"id":"multiple-endocrine-neoplasia","kind":"cancer","name":"Multiple endocrine neoplasia syndromes (MEN1, MEN2, MEN4)","route":"/cancers/multiple-endocrine-neoplasia/","tldr":"The MEN syndromes are inherited faults in a single gene that cause tumours in several hormone glands over a lifetime. Because the gene can be found in childhood, at-risk relatives can be tested, watched and in MEN2 have the thyroid removed before cancer develops; and for MEN2 thyroid cancer that does spread there is now a precise pill, selpercatinib, that blocks the faulty RET protein."},{"id":"ampullary","kind":"cancer","name":"Ampullary cancer (ampulla of Vater)","route":"/cancers/ampullary/","tldr":"Ampullary cancer, a biliary tract cancer, starts where the bile and pancreatic ducts empty into the small bowel. Because it blocks bile flow early it is often caught while still removable, and the Whipple operation cures a good share of patients. Tumours come in two flavours, intestinal-like and pancreas-like, and chemotherapy is increasingly chosen by which one the pathologist sees."},{"id":"small-bowel","kind":"cancer","name":"Small intestine cancer (small bowel adenocarcinoma)","route":"/cancers/small-bowel/","tldr":"Cancers of the small intestine are rare and often found late because the small bowel is hard to see and symptoms are vague. Surgery cures early disease, chemotherapy borrowed from bowel cancer helps after surgery and in advanced disease, and a large minority of tumours have a repair defect that makes them respond well to immunotherapy."},{"id":"penile","kind":"cancer","name":"Penile cancer","route":"/cancers/penile/","tldr":"Penile cancer is a squamous skin-type cancer, about half of it caused by HPV. Caught early it is usually cured with organ-sparing surgery that has replaced amputation, and HPV vaccination and circumcision prevent it; the hard cases are those with lymph-node spread, where cisplatin-based chemotherapy plus surgery and now immunotherapy are being tested in the InPACT trial."},{"id":"urethral","kind":"cancer","name":"Urethral cancer","route":"/cancers/urethral/","tldr":"Urethral cancer grows in the tube that carries urine out of the body, with fewer than one case per million people a year. With no randomised trials, it is treated by borrowing from bladder, anal or vulvar cancer depending on cell type and location; chemotherapy with radiotherapy before or instead of surgery lets more patients keep their organs."},{"id":"vaginal","kind":"cancer","name":"Vaginal cancer","route":"/cancers/vaginal/","tldr":"Primary vaginal cancer is rare and mostly caused by HPV, the virus behind cervical cancer. It is treated like cervical cancer, with weekly cisplatin alongside external and internal radiotherapy, which controls most tumours while preserving the organ; HPV vaccination and cervical screening, which also detects vaginal precursors, are steadily reducing it."},{"id":"male-breast-cancer","kind":"cancer","name":"Male breast cancer","route":"/cancers/male-breast-cancer/","tldr":"Men get breast cancer too, usually a hormone-sensitive kind found as a lump near the nipple. It is treated much as in women, with surgery, radiotherapy and tamoxifen, and inherited BRCA2 mutations are found often enough that every man diagnosed is offered genetic testing. The main fix under way is including men in trials so their care stops being borrowed from women."},{"id":"hiv-associated-lymphoma","kind":"cancer","name":"HIV-associated (AIDS-related) lymphomas","route":"/cancers/hiv-associated-lymphoma/","tldr":"People living with HIV have a raised risk of aggressive lymphomas, driven by immune suppression and viruses such as Epstein-Barr virus. The transformation of the last two decades is that, with antiretroviral therapy continued through treatment, these lymphomas are treated with the same full-dose chemotherapy and antibody regimens as in anyone else, with similar chances of cure."},{"id":"cmml","kind":"cancer","name":"Chronic myelomonocytic leukaemia and MDS/MPN overlap neoplasms","route":"/cancers/cmml/","tldr":"Chronic myelomonocytic leukaemia and its relatives are bone-marrow cancers that behave partly like myelodysplasia (poorly made blood cells) and partly like a proliferative disease (an excess of monocytes or platelets). Hypomethylating agents produce responses in a minority and stabilise counts in more, transplant can cure the fit, and RAS-pathway and JAK inhibitors are in trials."},{"id":"systemic-mastocytosis","kind":"cancer","name":"Systemic mastocytosis","route":"/cancers/systemic-mastocytosis/","tldr":"Systemic mastocytosis is a clonal disease of mast cells, the immune cells that release histamine; almost every case is driven by a single mutation in the KIT gene. Precise KIT-blocking pills now shrink the mast cell burden, ease symptoms and, in the aggressive forms, prolong life. Most patients have the indolent form, where the goal is controlling symptoms and preventing anaphylaxis."},{"id":"histiocytoses","kind":"cancer","name":"Erdheim-Chester disease, Rosai-Dorfman disease and other histiocytic neoplasms","route":"/cancers/histiocytoses/","tldr":"Histiocytoses are diseases in which immune scavenger cells build up in bone, heart, brain, kidneys and skin. They used to be treated as inflammatory conditions with steroids and interferon. The discovery that most carry mutations in the same growth pathway as melanoma turned them into targetable cancers: BRAF and MEK inhibitor pills now produce responses in nearly every treated patient."},{"id":"post-transplant-lymphoproliferative-disorder","kind":"cancer","name":"Post-transplant lymphoproliferative disorder (PTLD)","route":"/cancers/post-transplant-lymphoproliferative-disorder/","tldr":"After an organ or stem cell transplant, the drugs that stop rejection also stop the immune system from policing Epstein-Barr virus, and infected B cells can grow into a lymphoma. The first move is to ease the immunosuppression; then the antibody rituximab, chemotherapy if needed, and, newest of all, off-the-shelf virus-specific T cells that restore the missing immune control."},{"id":"mediastinal-germ-cell-tumour","kind":"cancer","name":"Mediastinal germ cell tumour","route":"/cancers/mediastinal-germ-cell-tumour/","tldr":"A mediastinal germ cell tumour is a germ cell tumour that starts in the chest, between the lungs, rather than in the testis. Seminomas here are cured almost as often as testicular seminoma, but non-seminomas of the chest are the hardest germ cell tumours to cure, so they get four cycles of chemotherapy and surgery for what is left."},{"id":"retroperitoneal-germ-cell-tumour","kind":"cancer","name":"Retroperitoneal germ cell tumour","route":"/cancers/retroperitoneal-germ-cell-tumour/","tldr":"A retroperitoneal germ cell tumour is a germ cell tumour found in the back of the abdomen with no obvious tumour in the testis. Many turn out to be spread from a tiny testicular tumour that has scarred over, so the testes are examined closely; treatment and cure rates are close to those of testicular disease."},{"id":"men1-syndrome","kind":"cancer","name":"Multiple endocrine neoplasia type 1 (MEN1)","route":"/cancers/men1-syndrome/","tldr":"MEN1 is an inherited condition, one of the multiple endocrine neoplasia syndromes, in which a faulty MEN1 gene lets tumours grow in the parathyroid glands, the pancreas and the pituitary. Each tumour is treated much as it would be in anyone else, but because there are many of them and they come back, families are followed for life in specialist clinics."},{"id":"men2-syndrome","kind":"cancer","name":"Multiple endocrine neoplasia type 2 (MEN2A and MEN2B)","route":"/cancers/men2-syndrome/","tldr":"MEN2 is an inherited condition, one of the multiple endocrine neoplasia syndromes, in which a faulty RET gene causes medullary thyroid cancer in almost every carrier, often with adrenal tumours and overactive parathyroids. Because the thyroid cancer is so predictable, children who inherit the gene have the thyroid removed at an age set by which RET mutation they carry."},{"id":"hyperparathyroidism-jaw-tumour-syndrome","kind":"cancer","name":"Hyperparathyroidism-jaw tumour syndrome (CDC73-related parathyroid carcinoma)","route":"/cancers/hyperparathyroidism-jaw-tumour-syndrome/","tldr":"Hyperparathyroidism-jaw tumour syndrome is an inherited condition in which a faulty CDC73 gene causes parathyroid tumours, and in about one in five people a parathyroid carcinoma, together with bony tumours of the jaw and kidney and womb growths. It matters because it is the commonest inherited route to parathyroid carcinoma and a reason to test the gene in anyone with that cancer."},{"id":"urethral-urothelial-carcinoma","kind":"cancer","name":"Urothelial carcinoma of the urethra","route":"/cancers/urethral-urothelial-carcinoma/","tldr":"Urothelial carcinoma of the urethra is the type of urethral cancer that grows from the same lining as bladder cancer, usually in the part of the urethra nearest the bladder or running through the prostate. It is treated by borrowing from bladder cancer: surgery, and chemotherapy with cisplatin before surgery when the disease is advanced."},{"id":"urethral-squamous-cell-carcinoma","kind":"cancer","name":"Squamous cell carcinoma of the urethra","route":"/cancers/urethral-squamous-cell-carcinoma/","tldr":"Squamous cell carcinoma of the urethra is the type of urethral cancer that grows in the outer part of the urethra, where the lining is skin-like; in some cases HPV is involved. It is treated like anal or vulval cancer of the same cell type, with chemotherapy and radiotherapy together to avoid removing the urethra."},{"id":"urethral-adenocarcinoma","kind":"cancer","name":"Adenocarcinoma of the urethra (including clear cell adenocarcinoma)","route":"/cancers/urethral-adenocarcinoma/","tldr":"Adenocarcinoma of the urethra is a gland-forming type of urethral cancer, more often seen in women, that can start in the small glands beside the urethra or in a pouch (diverticulum) in its wall. The clear cell form is its own entity in the WHO classification. No trial has been run in it, so it is treated by surgery at a specialist urological centre, advanced disease as on the parent page."},{"id":"urethral-melanoma","kind":"cancer","name":"Melanoma of the urethra","route":"/cancers/urethral-melanoma/","tldr":"Melanoma of the urethra is a very rare urethral cancer that starts in pigment cells of the urethral lining, usually near its outer opening, in older adults. It is a form of mucosal melanoma, so it is removed surgically and, when it spreads, treated with the immunotherapy drugs used for melanoma elsewhere."},{"id":"collecting-duct-carcinoma","kind":"cancer","name":"Collecting duct carcinoma of the kidney","route":"/cancers/collecting-duct-carcinoma/","tldr":"Collecting duct carcinoma is a rare, aggressive kidney cancer that starts in the tubes deep in the kidney that collect urine. It is treated with surgery where possible and the platinum chemotherapy used for bladder cancer, not the usual kidney cancer drugs, which do not work in it. It is usually advanced when found: 74.4 percent of 286 US SEER patients were stage III or IV."},{"id":"renal-medullary-carcinoma","kind":"cancer","name":"Renal medullary carcinoma (SMARCB1-deficient)","route":"/cancers/renal-medullary-carcinoma/","tldr":"Renal medullary carcinoma is a rare kidney cancer of young people with the sickle cell trait, driven by loss of SMARCB1. Platinum chemotherapy and removing the kidney are the main treatments. Most have spread when found; in the 52-patient series median survival was 13.0 months, 16.4 with nephrectomy against 7.0 without. So few centres see it that a trial and a second opinion are worth asking for."},{"id":"tfe3-rearranged-renal-cell-carcinoma","kind":"cancer","name":"TFE3-rearranged (translocation) renal cell carcinoma","route":"/cancers/tfe3-rearranged-renal-cell-carcinoma/","tldr":"Translocation renal cell carcinoma is a rare kidney cancer, more often seen in children and young adults, driven by a rearrangement of the TFE3 gene (or its relative TFEB). It can look like clear cell or papillary kidney cancer under the microscope, so it is found by testing for the gene. There is no standard treatment for advanced disease, which is treated with the usual kidney cancer drugs."},{"id":"fh-deficient-renal-cell-carcinoma","kind":"cancer","name":"Fumarate hydratase-deficient renal cell carcinoma (HLRCC-associated)","route":"/cancers/fh-deficient-renal-cell-carcinoma/","tldr":"Fumarate hydratase-deficient renal cell carcinoma is a rare, aggressive kidney cancer in which the FH gene is lost, most often because the person was born with a faulty copy as part of the HLRCC syndrome, which also causes skin and womb fibroids. It strikes younger adults, is found by a stain for the missing enzyme, and needs family testing; advanced disease gets the usual kidney cancer drugs."},{"id":"sdh-deficient-renal-cell-carcinoma","kind":"cancer","name":"Succinate dehydrogenase-deficient renal cell carcinoma","route":"/cancers/sdh-deficient-renal-cell-carcinoma/","tldr":"Succinate dehydrogenase-deficient renal cell carcinoma is a very rare kidney cancer of younger adults who carry a faulty SDH gene, the same fault that causes hereditary paraganglioma and some stomach stromal tumours. Most are low grade and cured by surgery, but a minority spread years later, so patients and relatives need gene testing and follow-up."},{"id":"mucinous-tubular-spindle-cell-carcinoma","kind":"cancer","name":"Mucinous tubular and spindle cell carcinoma of the kidney","route":"/cancers/mucinous-tubular-spindle-cell-carcinoma/","tldr":"Mucinous tubular and spindle cell carcinoma is a rare, usually slow-growing kidney cancer, commoner in women, whose cells form small tubes and spindles in a mucus-rich background. It is driven by loss of the Hippo growth-control pathway rather than the faults of common kidney cancer, and surgery cures most cases."},{"id":"eosinophilic-solid-cystic-renal-cell-carcinoma","kind":"cancer","name":"Eosinophilic solid and cystic renal cell carcinoma","route":"/cancers/eosinophilic-solid-cystic-renal-cell-carcinoma/","tldr":"Eosinophilic solid and cystic renal cell carcinoma is a recently named kidney cancer, almost always in women, made of pink cells in solid areas and cysts. It was first seen in people with tuberous sclerosis and then found on its own, it is nearly always confined to the kidney, and surgery cures it in almost every reported case."},{"id":"clear-cell-papillary-renal-cell-tumour","kind":"cancer","name":"Clear cell papillary renal cell tumour","route":"/cancers/clear-cell-papillary-renal-cell-tumour/","tldr":"Clear cell papillary renal cell tumour is a small, low-grade kidney tumour that looks like a mix of clear cell and papillary kidney cancer but behaves harmlessly: no case has been reported to spread. The WHO renamed it from carcinoma to tumour in 2022 for that reason. It is common in people with kidney failure and is cured by removing it."},{"id":"leydig-cell-tumour","kind":"cancer","name":"Leydig cell tumour of the testis","route":"/cancers/leydig-cell-tumour/","tldr":"Leydig cell tumour is the commonest testicular tumour that is not a germ cell tumour; it grows from the hormone-making cells between the tubules. It often makes testosterone or oestrogen, causing early puberty in boys or breast growth in men, and nine in ten are benign and cured by surgery. No chemotherapy works in the malignant tenth, managed case by case at a specialist centre."},{"id":"sertoli-cell-tumour","kind":"cancer","name":"Sertoli cell tumour of the testis","route":"/cancers/sertoli-cell-tumour/","tldr":"Sertoli cell tumour is a rare testicular tumour arising from the cells that support sperm production. Most are benign and cured by removing the testis. About one in ten spread, and no systemic treatment has been shown to work for those, so care is planned case by case at a specialist centre. A calcifying form occurs in boys with Peutz-Jeghers or Carney syndromes, usually with breast enlargement."},{"id":"spermatocytic-tumour","kind":"cancer","name":"Spermatocytic tumour of the testis","route":"/cancers/spermatocytic-tumour/","tldr":"Spermatocytic tumour, once called spermatocytic seminoma, is a rare testicular germ cell tumour of older men that arises from maturing sperm precursors rather than from the fetal germ cells behind ordinary testicular cancer. It almost never spreads, so removing the testis is the whole treatment and no chemotherapy or radiotherapy is needed."},{"id":"germ-cell-neoplasia-in-situ","kind":"cancer","name":"Germ cell neoplasia in situ (GCNIS)","route":"/cancers/germ-cell-neoplasia-in-situ/","tldr":"Germ cell neoplasia in situ is the pre-cancer of testicular germ cell tumours: abnormal fetal-type germ cells sitting inside the seminiferous tubules, which will become seminoma or non-seminoma if left. It is found beside almost every testicular cancer and sometimes on its own in the other testis, where low-dose radiotherapy or surgery prevents a second cancer."},{"id":"embryonal-carcinoma-testis","kind":"cancer","name":"Embryonal carcinoma of the testis","route":"/cancers/embryonal-carcinoma-testis/","tldr":"Embryonal carcinoma is the most aggressive and most common building block of non-seminoma testicular cancer, made of primitive cells that resemble an early embryo and can turn into the other tumour types. On its own or as the main component it spreads early to lymph nodes and lungs, but it is highly sensitive to cisplatin chemotherapy and most men are cured."},{"id":"yolk-sac-tumour-postpubertal","kind":"cancer","name":"Yolk sac tumour of the testis, postpubertal type","route":"/cancers/yolk-sac-tumour-postpubertal/","tldr":"Yolk sac tumour is a germ cell tumour whose cells copy the yolk sac of an early embryo and make the protein alpha-fetoprotein, which is measured in the blood to track it. In adults it almost always occurs mixed with other non-seminoma components and is cured with cisplatin chemotherapy; the pure infant form is a different, gentler disease covered on the childhood page."},{"id":"testicular-choriocarcinoma","kind":"cancer","name":"Choriocarcinoma of the testis","route":"/cancers/testicular-choriocarcinoma/","tldr":"Choriocarcinoma is the rarest and most dangerous form of non-seminoma testicular cancer, made of placenta-like cells that pour out the pregnancy hormone hCG and spread early through the blood to the lungs, liver and brain, where they can bleed. Fewer than eight in ten men survive five years, against more than 95 for testicular cancer overall, so it is treated urgently with intensive chemotherapy."},{"id":"malt-lymphoma","kind":"cancer","name":"Extranodal marginal zone lymphoma of mucosa-associated lymphoid tissue (MALT lymphoma)","route":"/cancers/malt-lymphoma/","tldr":"MALT lymphoma is a slow-growing lymphoma that starts in lymphoid tissue lining an organ, most often the stomach, where it is usually caused by long-standing Helicobacter pylori infection and can be cured with antibiotics alone. Other sites include the eye socket, salivary glands, thyroid, lung and skin; localised disease is treated with low-dose radiotherapy and widespread disease with rituximab."},{"id":"splenic-marginal-zone-lymphoma","kind":"cancer","name":"Splenic marginal zone lymphoma","route":"/cancers/splenic-marginal-zone-lymphoma/","tldr":"Splenic marginal zone lymphoma is a slow-growing lymphoma that grows in the spleen and bone marrow, causing a very large spleen and a raised lymphocyte count but rarely swollen lymph nodes. Many people need no treatment for years; when they do, rituximab has largely replaced removal of the spleen, and hepatitis C should be treated first where it is present."},{"id":"nodal-marginal-zone-lymphoma","kind":"cancer","name":"Nodal marginal zone lymphoma","route":"/cancers/nodal-marginal-zone-lymphoma/","tldr":"Nodal marginal zone lymphoma is a slow-growing lymphoma of the lymph nodes that looks like the MALT and splenic types under the microscope but has no organ or spleen involvement to explain it. It lacks a diagnostic marker, so it is diagnosed by excluding the other small B-cell lymphomas, and it is treated like follicular lymphoma with rituximab-based therapy."},{"id":"primary-cutaneous-marginal-zone-lymphoma","kind":"cancer","name":"Primary cutaneous marginal zone lymphoma","route":"/cancers/primary-cutaneous-marginal-zone-lymphoma/","tldr":"Primary cutaneous marginal zone lymphoma is a very slow-growing lymphoma that appears as pink or purple lumps in the skin, usually on the arms or trunk, and almost never spreads inside the body. WHO-HAEM5 now calls it a lymphoproliferative disorder because it behaves so well; surgery or radiotherapy clears most lesions, and relapses in the skin are common but harmless."},{"id":"primary-cutaneous-follicle-centre-lymphoma","kind":"cancer","name":"Primary cutaneous follicle centre lymphoma","route":"/cancers/primary-cutaneous-follicle-centre-lymphoma/","tldr":"Primary cutaneous follicle centre lymphoma is a slow-growing lymphoma of germinal-centre B cells that stays in the skin, usually as lumps on the head or trunk. Its outlook is excellent, with about 95 in 100 people alive at five years, and radiotherapy or excision is usually all that is needed; the important thing is not to mistake it for the aggressive leg-type large B-cell lymphoma."},{"id":"sezary-syndrome","kind":"cancer","name":"Sezary syndrome","route":"/cancers/sezary-syndrome/","tldr":"Sezary syndrome is the leukaemic form of skin lymphoma: the whole skin turns red and scaly, the lymph nodes swell, and malignant T cells circulate in the blood. It is treated to control rather than cure, with photopheresis, the antibody mogamulizumab, and drugs such as bexarotene and interferon, and a stem cell transplant is the only treatment that can cure it in fit patients."},{"id":"angioimmunoblastic-t-cell-lymphoma","kind":"cancer","name":"Nodal T-follicular helper cell lymphoma, angioimmunoblastic type (angioimmunoblastic T-cell lymphoma)","route":"/cancers/angioimmunoblastic-t-cell-lymphoma/","tldr":"Angioimmunoblastic T-cell lymphoma, now called nodal T-follicular helper cell lymphoma of angioimmunoblastic type, is one of the commonest T-cell lymphomas and mostly affects people over 60. It presents with widespread swollen nodes, fever, rash and immune upsets such as anaemia; about four in ten people are alive five years after chemotherapy, more after a transplant in first remission."},{"id":"hepatosplenic-t-cell-lymphoma","kind":"cancer","name":"Hepatosplenic T-cell lymphoma","route":"/cancers/hepatosplenic-t-cell-lymphoma/","tldr":"Hepatosplenic T-cell lymphoma is a rare, very aggressive lymphoma of young men in which gamma-delta T cells fill the liver, spleen and bone marrow without forming lumps in the nodes. It is linked to long-term immune suppression, above all thiopurines with or without anti-TNF drugs for inflammatory bowel disease, and is treated with intensive chemotherapy then a stem cell transplant where possible."},{"id":"intravascular-large-b-cell-lymphoma","kind":"cancer","name":"Intravascular large B-cell lymphoma","route":"/cancers/intravascular-large-b-cell-lymphoma/","tldr":"Intravascular large B-cell lymphoma is a rare form of large B-cell lymphoma in which the cancer cells grow inside small blood vessels rather than forming lumps, so it causes fevers, confusion, skin patches or breathlessness and is often found late or only after death. Rituximab-based chemotherapy with drugs that reach the brain has turned a nearly always fatal disease into one often controlled."},{"id":"lymphomatoid-granulomatosis","kind":"cancer","name":"Lymphomatoid granulomatosis","route":"/cancers/lymphomatoid-granulomatosis/","tldr":"Lymphomatoid granulomatosis is a rare Epstein-Barr virus-driven disease of B cells that invades and destroys blood vessels, almost always in the lungs and often the brain and skin, in people whose immune control of the virus is weak. Low-grade disease can be treated with interferon and high-grade disease as a large B-cell lymphoma with rituximab-based chemotherapy."},{"id":"t-cell-prolymphocytic-leukaemia","kind":"cancer","name":"T-cell prolymphocytic leukaemia","route":"/cancers/t-cell-prolymphocytic-leukaemia/","tldr":"T-cell prolymphocytic leukaemia is a rare, aggressive leukaemia of mature T cells in older adults, with a very high white cell count, a big spleen and liver, swollen nodes and sometimes skin changes. The antibody alemtuzumab given into a vein clears it in most people, but it returns within a year or two unless a stem cell transplant is done in remission."},{"id":"splenic-b-cell-lymphoma-leukaemia-prominent-nucleoli","kind":"cancer","name":"Splenic B-cell lymphoma/leukaemia with prominent nucleoli (formerly B-cell prolymphocytic leukaemia and hairy cell leukaemia variant)","route":"/cancers/splenic-b-cell-lymphoma-leukaemia-prominent-nucleoli/","tldr":"Splenic B-cell lymphoma/leukaemia with prominent nucleoli is the new WHO name for a rare group of B-cell leukaemias of older adults with a big spleen, large cells with obvious nucleoli and a poor response to standard treatment; it absorbs the old diagnoses B-cell prolymphocytic leukaemia and hairy cell leukaemia variant. It is treated with rituximab-based chemotherapy or newer targeted drugs."},{"id":"t-large-granular-lymphocytic-leukaemia","kind":"cancer","name":"T-cell large granular lymphocytic leukaemia","route":"/cancers/t-large-granular-lymphocytic-leukaemia/","tldr":"T-cell large granular lymphocytic leukaemia is a slow, usually non-fatal leukaemia in which a clone of cytotoxic T cells builds up in the blood and marrow and turns the immune system against the body, causing low neutrophil counts, anaemia and often rheumatoid arthritis. It is treated only when it causes problems, with low-dose immune-suppressing drugs rather than chemotherapy."},{"id":"mixed-phenotype-acute-leukaemia","kind":"cancer","name":"Mixed-phenotype acute leukaemia","route":"/cancers/mixed-phenotype-acute-leukaemia/","tldr":"Mixed-phenotype acute leukaemia is a rare acute leukaemia whose cells carry markers of both lymphoid and myeloid lines, so it fits neither acute lymphoblastic nor acute myeloid leukaemia. Pooled evidence favours starting with the drugs used for acute lymphoblastic leukaemia, adding a targeted drug when the Philadelphia chromosome is present, and a stem cell transplant in first remission."},{"id":"myeloid-leukaemia-of-down-syndrome","kind":"cancer","name":"Myeloid leukaemia of Down syndrome","route":"/cancers/myeloid-leukaemia-of-down-syndrome/","tldr":"Myeloid leukaemia of Down syndrome is a form of acute myeloid leukaemia in young children with Down syndrome, driven by a GATA1 mutation on top of the extra chromosome 21 and often preceded by a transient leukaemia-like illness in the newborn. Its cells are unusually sensitive to chemotherapy, so children are cured about nine times in ten with gentler treatment than other childhood leukaemia."},{"id":"burkitt-leukaemia","kind":"cancer","name":"Burkitt leukaemia","route":"/cancers/burkitt-leukaemia/","tldr":"Burkitt leukaemia is Burkitt lymphoma presenting mainly in the bone marrow and blood, so that it looks like acute lymphoblastic leukaemia but is a mature B-cell cancer driven by the MYC gene. It is treated as Burkitt lymphoma, with short, very intensive chemotherapy plus rituximab and protection of the brain, and most children and many adults are cured."},{"id":"adamantinoma","kind":"cancer","name":"Adamantinoma of bone","route":"/cancers/adamantinoma/","tldr":"Adamantinoma is a very rare, slow-growing bone cancer of young adults that almost always affects the shin bone, made of epithelial cells inside fibrous bone, and listed with the bone sarcomas in the WHO classification. It is cured by cutting it out with a margin; chemotherapy and radiotherapy do not work, and a minority spread to the lungs many years later."},{"id":"dedifferentiated-chordoma","kind":"cancer","name":"Dedifferentiated chordoma","route":"/cancers/dedifferentiated-chordoma/","tldr":"Dedifferentiated chordoma is a rare form of chordoma in which part of the tumour has turned into a high-grade sarcoma, usually after recurrence or radiotherapy but sometimes from the start. The sarcoma part decides the outcome and made up 3 to 95 percent of the tumour in the defining series, smaller areas doing better, so it is treated with surgery and sarcoma chemotherapy."},{"id":"poorly-differentiated-chordoma","kind":"cancer","name":"Poorly differentiated chordoma (SMARCB1-deficient)","route":"/cancers/poorly-differentiated-chordoma/","tldr":"Poorly differentiated chordoma is a rare, aggressive form of chordoma of children and young adults, mostly at the base of the skull or in the neck, defined by loss of the SMARCB1 (INI1) protein. It grows faster and spreads more than ordinary chordoma, and the SMARCB1 loss makes it a candidate for drugs that block EZH2, though surgery and radiotherapy remain the treatment."},{"id":"pineal-parenchymal-tumours","kind":"cancer","name":"Pineocytoma and pineal parenchymal tumour of intermediate differentiation","route":"/cancers/pineal-parenchymal-tumours/","tldr":"Pineocytoma and pineal parenchymal tumour of intermediate differentiation are rare brain tumours of the pineal gland in adults, sitting between the benign end and the aggressive pineoblastoma. Pineocytoma is cured by surgery; the intermediate tumour recurs and spreads through the spinal fluid more often, so radiotherapy is usually added after surgery."},{"id":"pineoblastoma","kind":"cancer","name":"Pineoblastoma","route":"/cancers/pineoblastoma/","tldr":"Pineoblastoma is a rare, aggressive brain tumour of the pineal gland, mostly in children, made of primitive cells like those of medulloblastoma. It is treated with surgery, radiotherapy to the whole brain and spine in children old enough, and chemotherapy; survival is lowest in infants, in whom radiotherapy is limited by age, and the subgroups found in 2020 now guide treatment."},{"id":"papillary-tumour-pineal-region","kind":"cancer","name":"Papillary tumour of the pineal region","route":"/cancers/papillary-tumour-pineal-region/","tldr":"Papillary tumour of the pineal region is a rare brain tumour of young adults arising near the pineal gland from cells of the embryonic subcommissural organ. It usually presents with raised pressure from blocked spinal fluid, is removed surgically at a centre that does pineal surgery and often given radiotherapy, and it recurs locally more often than it seeds the spine."},{"id":"choroid-plexus-carcinoma","kind":"cancer","name":"Choroid plexus carcinoma","route":"/cancers/choroid-plexus-carcinoma/","tldr":"Choroid plexus carcinoma is a rare, aggressive brain tumour of infants and young children that grows from the tissue that makes spinal fluid inside the brain's ventricles, causing fluid build-up and pressure. It is strongly linked to an inherited TP53 fault (Li-Fraumeni syndrome), so families are tested; treatment is surgery, then chemotherapy and, in older children, radiotherapy."},{"id":"optic-pathway-glioma","kind":"cancer","name":"Optic pathway glioma","route":"/cancers/optic-pathway-glioma/","tldr":"Optic pathway glioma is a slow-growing childhood brain tumour of the nerves that carry sight, often in children with neurofibromatosis type 1. It rarely kills but can take away vision, so treatment aims to preserve sight: watching if stable, chemotherapy such as carboplatin and vincristine if vision is threatened, or the MEK-blocking tablet selumetinib, with radiotherapy avoided in young children."},{"id":"pleuropulmonary-blastoma-dicer1","kind":"cancer","name":"Pleuropulmonary blastoma (types I, Ir, II and III)","route":"/cancers/pleuropulmonary-blastoma-dicer1/","tldr":"Pleuropulmonary blastoma is a rare lung cancer of young children, and the signature tumour of the inherited DICER1 syndrome. It starts as a lung cyst (type I) that can turn into a solid, aggressive tumour (types II and III). Cysts are removed surgically with an excellent outlook; solid tumours need chemotherapy as well, and every family is offered DICER1 testing."},{"id":"glucagonoma","kind":"cancer","name":"Glucagonoma","route":"/cancers/glucagonoma/","tldr":"Glucagonoma is a very rare pancreatic neuroendocrine tumour that pours out the hormone glucagon, causing weight loss, diabetes and a distinctive migrating red rash. Because the rash is so characteristic, spotting it early can lead to diagnosis before the tumour has spread to the liver; surgery is the definitive treatment and somatostatin analogues control the symptoms."},{"id":"vipoma","kind":"cancer","name":"VIPoma","route":"/cancers/vipoma/","tldr":"VIPoma is a very rare pancreatic neuroendocrine tumour that secretes vasoactive intestinal peptide, causing litres of watery diarrhoea a day with dangerous loss of potassium. Most have spread to the liver by diagnosis. Fluid replacement and somatostatin analogues control the diarrhoea, surgery cures the few caught early, and the usual neuroendocrine tumour treatments are used for spread."},{"id":"somatostatinoma","kind":"cancer","name":"Somatostatinoma","route":"/cancers/somatostatinoma/","tldr":"Somatostatinoma is one of the rarest neuroendocrine tumours, arising in the pancreas or the duodenum and secreting somatostatin, a hormone that switches off digestion, which can cause mild diabetes, gallstones and fatty diarrhoea. Duodenal cases are often linked to neurofibromatosis type 1 and rarely cause symptoms. Surgery is the main treatment and the outlook depends on site and grade."},{"id":"conjunctival-melanoma","kind":"cancer","name":"Conjunctival melanoma","route":"/cancers/conjunctival-melanoma/","tldr":"Conjunctival melanoma is a rare melanoma of the clear membrane over the white of the eye, usually growing out of a flat brown patch called primary acquired melanosis. It is removed with a margin and the edges frozen or treated with chemotherapy drops; about one in five spread within five years, and advanced disease is treated like skin melanoma with targeted or immune drugs."},{"id":"desmoplastic-small-round-cell-tumour","kind":"cancer","name":"Desmoplastic small round cell tumour","route":"/cancers/desmoplastic-small-round-cell-tumour/","tldr":"Desmoplastic small round cell tumour is a very rare sarcoma of adolescents and young men that grows across the lining of the abdomen as many nodules, driven by an EWSR1-WT1 fusion. Treatment is intensive chemotherapy, surgery to remove every nodule, sometimes heated abdominal chemotherapy, and radiotherapy. Most still relapse within three years, so it is treated at a sarcoma centre running trials."},{"id":"lactotroph-pitnet","kind":"cancer","name":"Lactotroph pituitary neuroendocrine tumour (prolactinoma)","route":"/cancers/lactotroph-pitnet/","tldr":"A prolactinoma is a pituitary tumour of the cells that make prolactin, the milk hormone; it is the commonest hormone-producing pituitary tumour and causes missed periods, infertility, milk production or, in men, low testosterone. Almost uniquely among tumours it is treated first with a tablet, cabergoline, which shrinks it in most people; surgery is kept for those the drug fails."},{"id":"somatotroph-pitnet","kind":"cancer","name":"Somatotroph pituitary neuroendocrine tumour (acromegaly)","route":"/cancers/somatotroph-pitnet/","tldr":"A somatotroph tumour is a pituitary tumour that makes too much growth hormone, causing acromegaly in adults (enlarging hands, feet and face, diabetes, heart and joint disease) or gigantism in children. Surgery through the nose comes first; if hormone levels stay high, somatostatin analogue injections, the blocker pegvisomant or radiotherapy bring them down, restoring a normal life expectancy."},{"id":"corticotroph-pitnet","kind":"cancer","name":"Corticotroph pituitary neuroendocrine tumour (Cushing disease and silent corticotroph tumour)","route":"/cancers/corticotroph-pitnet/","tldr":"A corticotroph tumour is a pituitary tumour of the cells that make ACTH. When it secretes it floods the body with cortisol, causing Cushing disease (weight gain, diabetes, high blood pressure, thin skin); the silent form is found as a large non-functioning mass. Surgery through the nose is the main treatment; cortisol-lowering drugs, repeat surgery or radiotherapy follow for those not cured."},{"id":"gonadotroph-pitnet","kind":"cancer","name":"Gonadotroph pituitary neuroendocrine tumour (non-functioning adenoma)","route":"/cancers/gonadotroph-pitnet/","tldr":"A gonadotroph tumour is a pituitary tumour of the cells that normally make the fertility hormones, but it almost never secretes enough to cause symptoms, so it is found as a large non-functioning mass pressing on the optic nerves or by chance. Surgery through the nose is the treatment when it threatens vision or grows; there is no drug for it, and radiotherapy is used for regrowth."},{"id":"thyrotroph-pitnet","kind":"cancer","name":"Thyrotroph pituitary neuroendocrine tumour (TSH-secreting)","route":"/cancers/thyrotroph-pitnet/","tldr":"A thyrotroph tumour is a very rare pituitary tumour that secretes TSH, driving the thyroid to overactivity with a TSH level that is not suppressed. It is often mistaken for common hyperthyroidism and wrongly treated by destroying the thyroid, which makes the pituitary tumour grow. Surgery is the treatment of choice; somatostatin analogues control most of the rest."},{"id":"thymoma-type-a-ab","kind":"cancer","name":"Type A and type AB thymoma","route":"/cancers/thymoma-type-a-ab/","tldr":"Type A and type AB thymoma are the indolent end of thymoma, tumours of the thymus gland made of spindle-shaped epithelial cells (type A) or mixed with lymphocyte-rich areas (type AB), mostly in older adults and driven by a GTF2I mutation. Nine in ten are found at an early stage and almost none come back after complete surgery, so surgery alone is usually the whole treatment."},{"id":"thymoma-type-b1-b2","kind":"cancer","name":"Type B1 and type B2 thymoma","route":"/cancers/thymoma-type-b1-b2/","tldr":"Type B1 and type B2 thymoma are thymus gland tumours in which the epithelial tumour cells are mixed with many immature lymphocytes, resembling the normal thymic cortex; B2 is the commonest thymoma of all. They are strongly linked to myasthenia gravis, are usually cured by surgery, and get radiotherapy when they have grown beyond the gland or could not be fully removed."},{"id":"thymoma-type-b3","kind":"cancer","name":"Type B3 thymoma","route":"/cancers/thymoma-type-b3/","tldr":"Type B3 thymoma is the most aggressive thymoma, a thymus gland tumour made mostly of epithelial cells with few lymphocytes, sitting at the border with thymic carcinoma. More than a third have already grown into the chest structures when found, so it is treated with surgery plus radiotherapy, and chemotherapy before surgery when it is too large to remove at once."},{"id":"micronodular-thymoma","kind":"cancer","name":"Micronodular thymoma with lymphoid stroma","route":"/cancers/micronodular-thymoma/","tldr":"Micronodular thymoma with lymphoid stroma is a rare, benign-behaving thymoma made of small nests of spindle-shaped epithelial cells separated by abundant B lymphocytes, unlike other thymomas whose lymphocytes are T cells. It is cured by surgery; its curiosity is that a third harbour a clonal B-cell population, and a few develop a low-grade lymphoma within the tumour."},{"id":"acinic-cell-carcinoma-salivary","kind":"cancer","name":"Acinic cell carcinoma of the salivary glands","route":"/cancers/acinic-cell-carcinoma-salivary/","tldr":"Acinic cell carcinoma is a salivary gland cancer, almost always of the parotid gland, whose cells resemble the gland's normal enzyme-making cells. It is usually low grade and slow, presenting as a painless lump that is easily mistaken for a benign tumour, and surgery cures most; a high-grade minority behaves aggressively and needs radiotherapy too."},{"id":"carcinoma-ex-pleomorphic-adenoma","kind":"cancer","name":"Carcinoma ex pleomorphic adenoma","route":"/cancers/carcinoma-ex-pleomorphic-adenoma/","tldr":"Carcinoma ex pleomorphic adenoma is a salivary gland cancer that grows out of a long-standing benign pleomorphic adenoma, the commonest salivary tumour, usually in the parotid. Its outlook depends on how far the cancer has grown beyond the old adenoma's capsule: cancers still inside it are cured by surgery, while widely invasive ones need radiotherapy and do poorly."},{"id":"cervical-adenosquamous-carcinoma","kind":"cancer","name":"Adenosquamous carcinoma of the cervix","route":"/cancers/cervical-adenosquamous-carcinoma/","tldr":"Adenosquamous carcinoma of the cervix is an uncommon cervical cancer containing both gland-forming and squamous cancer cells. Like cervical adenocarcinoma it is caught less well by smear screening and does somewhat worse than squamous cancer stage for stage, but it is treated the same way, with surgery when early and chemoradiation when advanced."},{"id":"cervical-small-cell-neuroendocrine-carcinoma","kind":"cancer","name":"Small cell neuroendocrine carcinoma of the cervix","route":"/cancers/cervical-small-cell-neuroendocrine-carcinoma/","tldr":"Small cell neuroendocrine carcinoma of the cervix is a rare, aggressive cervical cancer that behaves like small cell lung cancer, spreading early to nodes and distant organs even when the tumour is small. Fewer than four in ten women with early disease are alive at five years, and treatment combines the cervical cancer approach with the chemotherapy used for small cell lung cancer."},{"id":"bartholin-gland-carcinoma","kind":"cancer","name":"Bartholin gland carcinoma","route":"/cancers/bartholin-gland-carcinoma/","tldr":"Bartholin gland carcinoma is a very rare vulvar cancer arising in the lubricating glands at the entrance to the vagina, often mistaken at first for a cyst or abscess in a postmenopausal woman. It can be a squamous cancer, an adenocarcinoma or an adenoid cystic carcinoma, each behaving differently; it is treated with surgery and radiotherapy borrowed from vulvar cancer, having no trials of its own."},{"id":"vulvar-melanoma","kind":"cancer","name":"Vulvar melanoma","route":"/cancers/vulvar-melanoma/","tldr":"Vulvar melanoma is the second most common vulvar cancer, a melanoma of the mucosal skin of the vulva in older women that is usually found late and has shorter survival than skin melanoma. It is removed with a margin, staged like skin melanoma by thickness, and treated when advanced with the immunotherapy and, for the quarter with a BRAF or KIT mutation, the targeted drugs used for other melanomas."},{"id":"vaginal-melanoma","kind":"cancer","name":"Vaginal melanoma","route":"/cancers/vaginal-melanoma/","tldr":"Vaginal melanoma is a very rare, aggressive melanoma of the vaginal lining in older women, usually found late because it is hidden, with the worst outlook of any melanoma site. It is treated with surgery where the tumour can be removed and radiotherapy where it cannot; advanced disease gets the drugs used for mucosal melanoma. Unlike skin melanoma it is as common in black as in white women."}]}