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Leydig cell tumour

From Wikipedia, the free encyclopedia
Medical condition
Leydig cell tumour
Other namesTesticular interstitial cell tumour
Histopathology of a Leydig cell tumor, high magnification,H&E stain, showing typical features.[1]
SpecialtyOncology,endocrinology Edit this on Wikidata

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.

Presentation

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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]

Cause

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Animal studies a suggest possible link with C8 (C8HF15O2,perfluorooctanoic acid).[4]

Diagnosis

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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]

Low magnificationmicrograph of aLeydig cell tumour.H&E stain.

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]

Treatment

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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]

Additional images

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  • Intermediate magnification micrograph of a Leydig cell tumour. H&E stain.
    Intermediate magnificationmicrograph of a Leydig cell tumour.H&E stain.
  • High magnification micrograph of a Leydig cell tumour. H&E stain.
    High magnificationmicrograph of a Leydig cell tumour.H&E stain.
  • Typical gross pathology of a Leydig cell tumor (in this case of the ovary): A well circumscribed, solid homogeneous mass with golden brown to brownish green cut surface.[12]
    Typical gross pathology of a Leydig cell tumor (in this case of the ovary): A well circumscribed, solid homogeneous mass with golden brown to brownish green cut surface.[12]

See also

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References

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  1. ^Zhengshan Chen, M.D., Ph.D., Manju Aron, M.D."Testis & epididymis - Sex cord-stromal tumors - Leydig cell tumor".PathologyOutlines.{{cite web}}: CS1 maint: multiple names: authors list (link) Topic Completed: 4 March 2021. Minor changes: 12 April 2021.
  2. ^Sachdeva P, Arora R, Dubey C, Sukhija A, Daga M, Singh DK (April 2008). "Sertoli-Leydig cell tumor: a rare ovarian neoplasm. Case report and review of literature".Gynecol. Endocrinol.24 (4):230–4.doi:10.1080/09513590801953465.PMID 18382911.S2CID 42384623.
  3. ^ab"Leydig Cell Tumors: Practice Essentials, Background, Pathophysiology". 2016-10-27.{{cite journal}}:Cite journal requires|journal= (help)
  4. ^Biegel, L. B.; Liu, R. C.; Hurtt, M. E.; Cook, J. C. (1995). "Effects of ammonium perfluorooctanoate on Leydig cell function: in vitro, in vivo, and ex vivo studies".Toxicology and Applied Pharmacology.134 (1):18–25.doi:10.1006/taap.1995.1164.PMID 7676454.
  5. ^Reddan, Tristan; Powell, Jennifer; Long, Gillian (2017)."Ultrasound of a prepubertal Leydig cell tumour of the testis"(PDF).Sonography.4 (3):125–128.doi:10.1002/sono.12111.S2CID 79812660.
  6. ^Ulbright TM, Srigley JR, Hatzianastassiou DK, Young RH (November 2002). "Leydig cell tumors of the testis with unusual features: adipose differentiation, calcification with ossification, and spindle-shaped tumor cells".Am. J. Surg. Pathol.26 (11):1424–33.doi:10.1097/00000478-200211000-00004.PMID 12409718.S2CID 25993642.
  7. ^Basciani, Sabrina; Brama, Marina; Mariani, Stefania; De Luca, Gabriele; Arizzi, Mario; Vesci, Loredana; Pisano, Claudio; Dolci, Susanna; Spera, Giovanni; Gnessi, Lucio (2005-03-01)."Imatinib Mesylate Inhibits Leydig Cell Tumor Growth: Evidence forIn vitro andIn vivo Activity".Cancer Research.65 (5):1897–1903.doi:10.1158/0008-5472.can-04-2181.PMID 15753388.
  8. ^"Leydig Cell Tumors Treatment & Management: Medical Care, Surgical Care". 2016-10-27.{{cite journal}}:Cite journal requires|journal= (help)
  9. ^abLenhard M, Kuemper C, Ditsch N, Diebold J, Stieber P, Friese K, Burges A (2007)."Use of novel serum markers in clinical follow-up of Sertoli-Leydig cell tumours".Clin. Chem. Lab. Med.45 (5):657–61.doi:10.1515/CCLM.2007.120.PMID 17484630.S2CID 12883618.
  10. ^Ferretti L, Sargos P, Gross-Goupil M, Izard V, Wallerand H, Huyghe E, Rigot JM, Durand X, Benoit G, Ferriere JM, Droupy S (2014)."Testicular-sparing surgery for bilateral or monorchide testicular tumours: a multicenter study of long-term oncological and functional results".BJU Int.114 (6):860–4.doi:10.1111/bju.12549.PMID 24180380.S2CID 24924124.
  11. ^Al-Agha OM, Axiotis CA (February 2007)."An in-depth look at Leydig cell tumor of the testis".Arch. Pathol. Lab. Med.131 (2):311–7.doi:10.5858/2007-131-311-AILALC.PMID 17284120.
  12. ^Zhengshan Chen, M.D., Ph.D., Manju Aron, M.D."Testis & epididymis - Sex cord-stromal tumors - Leydig cell tumor".PathologyOutlines.{{cite web}}: CS1 maint: multiple names: authors list (link) Topic Completed: 4 March 2021. Minor changes: 12 April 2021.

External links

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Classification
External resources
Adnexa
Ovaries
Glandular and epithelial/
surface epithelial-
stromal tumor
CMS:
Sex cord–gonadal stromal
Germ cell
Fibroma
Fallopian tube
Uterus
Myometrium
Endometrium
Cervix
Placenta
General
Vagina
Vulva
Testicles
Sex cord–
gonadal stromal
Germ cell
G
NG
Prostate
Penis
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