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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.
Sertoli cell tumours are sex cord-stromal tumours of the testis; the WHO classification separates the not otherwise specified type from the large cell calcifying and the intratubular large cell hyalinising types (Moch 2016). In the series of 60 tumours not otherwise specified, presentation was usually a slowly enlarging painless mass, all were unilateral, size ranged from 0.3 to 15 cm (mean 3.6), nuclear atypia was absent or mild in 54, and four patients had metastases at presentation (Young 1998). Exon 3 mutations of CTNNB1 with nuclear beta-catenin were found in 10 of 14 tumours, linking the tumour to WNT pathway activation (Am J Surg Pathol 2014). In boys, intratubular Sertoli cell proliferations and large cell calcifying tumours occur with Peutz-Jeghers syndrome and gynaecomastia (Am J Surg Pathol 2001).
How it differs from its parent: like Leydig cell tumours it is not a germ cell tumour, does not respond to cisplatin chemotherapy, does not raise the germ cell markers and is usually benign; malignancy is judged from size over 5 cm, necrosis, vascular invasion, atypia and mitoses.
| Setting | Approach | Guideline |
|---|---|---|
| All cases | Orchidectomy, testis-sparing in syndromic boys; node dissection for malignant features; no systemic therapy has proven benefit in metastatic disease, so trials and referral to a specialist testicular centre are the route. | not mapped |