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Some childhood cancers are so rare that no single hospital sees enough to learn from. The NCI groups them together: heart tumours, airway papillomas, cancers of the thyroid, adrenal, nose and throat, melanoma and carcinomas more typical of adults. The answer has been international registries and expert networks that pool every case, so treatment guidance exists even without trials.
The NCI PDQ summary on rare cancers of childhood collects tumours that fall outside the common paediatric disease groups: head and neck carcinomas (nasopharyngeal carcinoma, salivary, laryngeal, thyroid, oesthesioneuroblastoma, NUT carcinoma), thoracic tumours (pleuropulmonary blastoma, tracheobronchial tumours, thymoma, cardiac tumours, mesothelioma), abdominal tumours (adrenocortical carcinoma, gastrointestinal carcinomas, carcinoid, pancreatic tumours, pancreatoblastoma), genital and urinary tumours (bladder, testicular non-germ cell, ovarian epithelial), skin cancers (melanoma, basal and squamous cell carcinoma), and multiple endocrine neoplasia syndromes. Where OnCo has a dedicated record (nasopharyngeal, thyroid, adrenocortical, melanoma, pleuropulmonary blastoma, NUT carcinoma, sinonasal, pheochromocytoma-paraganglioma, multiple endocrine neoplasia) this page points to it; this record covers the residue and the organisational response.
Two entities on the NCI list have no other home. Childhood cardiac tumours are mostly benign rhabdomyomas associated with tuberous sclerosis complex, which regress spontaneously and, when obstructive, respond to mTOR inhibitors (everolimus or sirolimus) rather than surgery; fibromas, myxomas and rare sarcomas are treated surgically. Recurrent respiratory (laryngeal) papillomatosis is a benign HPV 6/11 disease acquired at birth that causes hoarseness and airway obstruction, requires repeated debulking, rarely transforms to squamous carcinoma, and is being prevented by HPV vaccination of mothers and reduced by systemic bevacizumab in refractory cases; the nonavalent vaccine as adjuvant therapy is also studied.
The defining feature of these tumours is that randomised trials are impossible, so the field built other tools: the COG Rare Tumors committee and its registry, the European Cooperative Study Group for Paediatric Rare Tumours (EXPeRT) and its consensus recommendations, the Pediatric MATCH molecular-assignment platform that accepts any refractory solid tumour, and the practice of borrowing adult protocols with paediatric dose adjustments. Adult-type carcinomas in children are often linked to germline predisposition (TP53, DICER1, mismatch repair, APC) and trigger genetic testing.
Individually vanishingly rare, but together the tumours grouped by the NCI as rare cancers of childhood account for roughly one in ten childhood cancers, and their share rises in adolescence (NCI PDQ).
Nothing recorded yet.
Nothing recorded yet.
Also on OnCo: Symptoms and red flags · Early detection roadmap.
Observation for spontaneous regression; mTOR inhibitor (everolimus or sirolimus) for haemodynamically significant tumours; surgery reserved for obstruction unresponsive to medical therapy.
Repeated microdebrider or laser debulking to maintain the airway; systemic bevacizumab for severe refractory disease; HPV vaccination (prevention through maternal vaccination; adjuvant use under study).
Treat per the adult site-specific standard with paediatric dose adjustment, enrol in the COG or EXPeRT rare tumour registry, and test for germline predisposition.
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Query for this cancer: (TITLE:"Rare cancers of childhood" OR ABSTRACT:"Rare cancers of childhood" OR TITLE:"NCI PDQ umbrella" OR ABSTRACT:"NCI PDQ umbrella" OR TITLE:"Childhood cardiac tumours" OR ABSTRACT:"Childhood cardiac tumours" OR TITLE:"Laryngeal papillomatosis" OR ABSTRACT:"Laryngeal papillomatosis" OR TITLE:"Recurrent respiratory papillomatosis" OR ABSTRACT:"Recurrent respiratory papillomatosis" OR TITLE:"Childhood melanoma" OR ABSTRACT:"Childhood melanoma") AND (treatment OR therapy OR trial OR survival OR diagnosis). Results are unfiltered search hits about Rare cancers of childhood (NCI PDQ umbrella), not a curated reading list.
Creates a registry and protocols for infrequent tumours.
European Cooperative Study Group for Paediatric Rare Tumours pools national registries (Italy, Germany, France, Poland, UK).
Case series lead to medical management replacing surgery.
Molecular assignment platform for any refractory childhood solid tumour.
The targets of this cancer's medicines and the ones linked to it directly.
Cases by country, the UK and NHS pathway and other country lenses, the expert centres with trials on record, and the centres named on this cancer's subtypes.
One section per setting: the options named, what each is for, the trials behind them, the recorded trade-offs and the questions to ask.
Sudden severe abdominal pain, a hard or very tender abdomen, or abdominal pain with vomiting and fever. Boxed warning for gastrointestinal perforation on bevacizumab.
Avoid grapefruit. Live vaccines are contraindicated.
7.5 mg (mild), 5 mg (moderate), 2.5 mg (severe).
The shared side effects of drugs that block blood vessel growth (bevacizumab, ramucirumab and VEGFR kinase inhibitors): high blood pressure, protein leaking into the urine, nosebleeds and more serious bleeding, slow wound healing, and rarely holes in the bowel.
High blood sugar as a side effect of drugs that block the PI3K/AKT pathway, because the same pathway carries insulin's signal in muscle and liver. It is the main reason these otherwise effective breast cancer drugs are hard to give.
Death of brain tissue months to years after radiosurgery or high-dose brain radiotherapy, which can look exactly like tumour growing back on a scan.
See all on the product pages:BevacizumabEverolimus·Printable cards in the navigator
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Everything in development, the medicines held by this cancer's subtypes, the open problems and what is being done about them, the roadmaps, and what changed on this record.
Every connected record, the notes, the JSON, Markdown and RDF twins, and where the record came from and when it was checked.