Sertoli cell tumour

Sertoli cell tumour
Classification and external resources

Micrograph of a Sertoli cell tumour. H&E stain.
ICD-9 183.0, 256.1
ICD-O: 8631
MeSH D012707

A Sertoli cell tumour, also Sertoli cell tumor (US spelling), is a Sex cord-gonadal stromal tumor of a specific type that produces Sertoli cells. Although Sertoli cells normally occur only in the testis, this type of tumor can occur not only in the testis[1] but also in the ovary.[2]

A tumor that produces both Sertoli cells and Leydig cells is known as a Sertoli-Leydig cell tumor.

Contents

Presentation

Due to excess testosterone secreted by the tumour, one-third of female patients present with a recent history of progressive masculinization. Masculinization is preceded by anovulation, oligomenorrhea, amenorrhea and defeminization. Additional signs include acne and hirsutism, voice deepening, clitoromegaly, temporal hair recession, and an increase in musculature. Serum testosterone level is high.

Diagnosis

Presence of an ovarian tumour plus hormonal disturbances suggests a Leydig cell tumour, granulosa cell tumour or thecoma. However, hormonal disturbances, in Leydig tumours, is present in only 2/3 of cases. A conclusive diagnosis is made via histology, as part of a pathology report made during or after surgery. See also Sex cord-stromal tumour.

Treatment

The usual treatment is surgery. The surgery 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 elevated tumour markers,[3] the focus of surveillance is on repeated physical examination and imaging.

The prognosis is generally good as the tumour tends to grow slowly and usually is benign: 25% are malignant. For malignant tumours with undifferentiated histology, prognosis is poor.[3]

In non-humans

Sertoli cell tumors are known to occur in other species, including domestic ducks,[4] dogs,[5][6] and horses.

Additional images

See also

Notes

  1. ^ Young RH, Koelliker DD, Scully RE (June 1998). "Sertoli cell tumors of the testis, not otherwise specified: a clinicopathologic analysis of 60 cases". Am. J. Surg. Pathol. 22 (6): 709–21. doi:10.1097/00000478-199806000-00008. PMID 9630178. http://meta.wkhealth.com/pt/pt-core/template-journal/lwwgateway/media/landingpage.htm?issn=0147-5185&volume=22&issue=6&spage=709. 
  2. ^ Oliva E, Alvarez T, Young RH (February 2005). "Sertoli cell tumors of the ovary: a clinicopathologic and immunohistochemical study of 54 cases". Am. J. Surg. Pathol. 29 (2): 143–56. PMID 15644771. http://meta.wkhealth.com/pt/pt-core/template-journal/lwwgateway/media/landingpage.htm?issn=0147-5185&volume=29&issue=2&spage=143. 
  3. ^ a b Lenhard 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. http://www.reference-global.com/doi/abs/10.1515/CCLM.2007.120. 
  4. ^ Leach S, Heatley JJ, Pool RR, Spaulding K (December 2008). "Bilateral testicular germ cell-sex cord-stromal tumor in a pekin duck (Anas platyrhynchos domesticus)". J. Avian Med. Surg. 22 (4): 315–9. doi:10.1647/2007-017.1. PMID 19216259. 
  5. ^ Gopinath D, Draffan D, Philbey AW, Bell R (December 2008). "Use of intralesional oestradiol concentration to identify a functional pulmonary metastasis of canine sertoli cell tumour". J Small Anim Pract 50 (4): 198–200. doi:10.1111/j.1748-5827.2008.00671.x. PMID 19037884. 
  6. ^ Vegter AR, Kooistra HS, van Sluijs FJ, van Bruggen LW, Ijzer J, Zijlstra C, Okkens AC (October 2008). "Persistent Mullerian Duct Syndrome in a Miniature Schnauzer Dog with Signs of Feminization and a Sertoli Cell Tumour". Reprod. Domest. Anim. 45 (3): 447–52. doi:10.1111/j.1439-0531.2008.01223.x. PMID 18954385. 

External links