Leydig cell tumour | |
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Other names | Testicular interstitial cell tumour |
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Histopathology of a Leydig cell tumor, high magnification,H&E stain, showing typical features.[1] | |
Specialty | Oncology,endocrinology ![]() |
Leydig cell tumour, alsoLeydig cell tumor (US spelling),(testicular) interstitial cell tumour and(testicular) interstitial cell tumor (US spelling), is a member of thesex cord-stromal tumour group[2] ofovarian andtesticular cancers. It arises fromLeydig cells. While the tumour can occur at any age, it occurs most often in young adults.
ASertoli–Leydig cell tumour is a combination of a Leydig cell tumour and aSertoli cell tumour fromSertoli cells.
The majority of Leydig cell tumors are found in males, usually at 5–10 years of age or in middle adulthood (30–60 years). Children typically present withprecocious puberty.[citation needed]
Due to excesstestosterone secreted by the tumour, one-third of female patients present with a recent history of progressivemasculinization. Masculinization is preceded byanovulation,oligomenorrhea,amenorrhea anddefeminization. Additional signs includeacne andhirsutism, voice deepening,clitoromegaly, temporal hair recession, and an increase in musculature. Serum testosterone level is high.[citation needed]
In men, testicular swelling is the most common presenting feature. Other symptoms depend on age and the type of tumour. If it is secretingandrogens the tumour is usually asymptomatic, but can causeprecocious puberty in pre-pubertal boys. If the tumour secretesoestrogens it can cause feminisation in young boys. In adults, this causes a number of problems includinggynaecomastia, erectile dysfunction, infertility, feminine hair distribution, gonadogenital atrophy, and a loss of libido.[3]
Animal studies a suggest possible link with C8 (C8HF15O2,perfluorooctanoic acid).[4]
Presence of an ovarian tumour plus hormonal disturbances suggests a Leydig cell tumour,granulosa cell tumour orthecoma. However, hormonal disturbances, in Leydig tumours, is present in only 2/3 of cases. Testicular Leydig cell tumours can be detected sonographically, ultrasound examinations may be ordered in the event of a palpable scrotal lump, however incidental identification of these lesions is also possible.[5]
A conclusive diagnosis is made viahistology, as part of a pathology report made during or after surgery.Reinke crystals are classically found in these tumours and help confirm the diagnosis, although they are seen in less than half of all Leydig cell tumours. Immunohistochemical markers of Leydig cell tumours includeinhibin-alpha,calretinin, andmelan-A.[6]
The usual chemotherapy regimen has limited efficacy in tumours of this type, althoughimatinib has shown some promise.[7] There is no current role for radiotherapy.[8]
The usual treatment is surgery. The surgery for females usually is a fertility-sparing unilateral salpingo-oophorectomy. For malignant tumours, the surgery may be radical and usually is followed by adjuvant chemotherapy, sometimes by radiation therapy. In all cases, initial treatment is followed by surveillance. Because in many cases Leydig cell tumour does not produce elevatedtumour markers,[9] the focus of surveillance is on repeated physical examination and imaging.
In males, a radical inguinal orchiectomy is typically performed. However, testes-sparing surgery can be used to maintain fertility in children and young adults. This approach involves an inguinal or scrotal incision and ultrasound guidance if the tumour is non-palpable. This can be done because the tumour is typically unifocal, not associated with precancerous lesions, and is unlikely to recur.[10]
Theprognosis is generally good as the tumour tends to grow slowly and usually isbenign: 10% aremalignant.[3][11] For malignant tumours with undifferentiated histology, prognosis is poor.[9]
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