Sertoli-Leydig cell tumor

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1] Associate Editor(s)-in-Chief: Maria Fernanda Villarreal, M.D. [2]

Synonyms and Keywords: Arrhenoblastoma; Androblastoma; Sertoli-stromal cell tumor; Ovarian androblastoma

Overview

Sertoli-Leydig cell tumors is a group of uncommon sex cord stromal tumors, composed of variable proportions of Sertoli cells, Leydig cells, intermediate and poorly differentiated neoplasms, and primitive gonadal stroma. The majority of Sertoli-Leydig cell tumors are benign, unilateral, and confined to the ovaries. However, approximately 10-20% of Sertoli-Leydig cell tumors can be malignant, and also bilateral. The pathogenesis of Sertoli-Leydig cell tumor is characterized by an excess of testosterone secreted by the tumor, therefore early symptoms of Sertoli-Leydig cell tumor are related to the androgenic activity. Common symptoms, include: temporal hair recession, voice deepening, amenorrhea, and oligomenorrhea. The mainstay of therapy for Sertoli-Leydig cell tumor is platinum-based adjuvant chemotherapy and surgery.[1] Fertility-sparing unilateral salpingo-oophorectomy is the most common approach to the treatment of Sertoli-Leydig cell tumor in young women.

Historical Perspective

  • Sertoli-Leydig cells were first discovered by Enrico Sertoli, an Italian physician, in 1865.[2]

Classification

  • Sertoli-Leydig cell tumor may be mainly classified according to histopathological features into 4 subtypes:[1]
  • Well differentiated
  • Intermediately differentiated
  • Poorly differentiated
  • Retiform
  • Variants of Sertoli-Leydig cell tumor, include: pure Sertoli cell tumour of ovary.

Pathophysiology

  • The pathogenesis of Sertoli-Leydig cell tumor is characterized by an excess of testosterone secreted by the tumor.[1]
  • Germline mutation in the DICER1 gene has been associated with the development of Sertoli-Leydig cell tumor.[2]
  • On gross pathology, findings of sertoli-leydig cell tumor, include:[2]
  • Golden-yellow blood filled
  • Unilocular cyst
  • Large size mass
  • On microscopic histopathological analysis, findings of Sertoli-Leydig cell tumor, include:[2]
  • Leydig cells:
  • Polygonal pink cells
  • Abundant solid or somewhat granular eosinophilic cytoplasm.
  • Round nuclei with fine chromatin and a small or indistinct nucleolus.
  • Often in small clusters ~ 5-25 cells/cluster.
  • Sertoli cells:
  • Pale/clear vacuolated cytoplasm.
  • Irregular nuclei with irregular/vacuolated-appearing chromatin.
  • Architecture: tubules, cords or sheets.
  • Classic Sertoli tubule shows an antipodal arrangement of nuclei
  • On immunohistochemistry, findings of Sertoli-Leydig cell tumor, include:[1][2]
  • Inhibin
  • Calretinin
  • WT-1
  • Melan A (MART-1) (hallmark)
  • Vimentin

Causes

  • There are no known causes of Sertoli-Leydig cell tumor.

Differentiating Sertoli-Leydig Cell Tumor from Other Diseases

  • Sertoli-Leydig cell tumor must be differentiated from other diseases that cause acne, hirsutism, and voice deepening, such as:[2]

Epidemiology and Demographics

  • Sertoli-Leydig cell tumors constitute less than 0.5 percent of ovarian cancers.[3]

Age

  • The median age at diagnosis of Sertoli-Leydig cell tumor is 14 years.[2]
  • Sertoli-Leydig cell tumor is commonly observed among patients aged 15 to 35 years old.[2]
  • Sertoli-Leydig cell tumor is more commonly observed among younger females.

Race

  • There is no racial predilection for Sertoli-Leydig cell tumor.

Risk Factors

  • There are no known risk factors in the development of Sertoli-Leydig cell tumor.[1]

Natural History, Complications and Prognosis

  • The majority of patients with Sertoli-Leydig cell tumor are asymptomatic.
  • Early clinical features Sertoli-Leydig cell tumor, include acne, hirsutism, and voice deepening.
  • If left untreated, patients with Sertoli-Leydig cell tumor may progress to develop lymph node invasion and metastasis.
  • Common complications of Sertoli-Leydig cell tumor, include ovarian torsion or peritoneal metastasis.
  • Prognosis is generally good, and the 5 ­year mortality of patients with benign Sertoli-Leydig cell tumors is approximately 85-90%. For malignant tumors with undifferentiated histology, prognosis is poor.[2]

Diagnosis

Diagnostic Criteria

  • The diagnosis of Sertoli-Leydig cell tumor is made with the following diagnostic criteria:[1]
  • Elevated serum testosterone level
  • Clinical features:
  • Compatible imaging findings

Symptoms

  • Sertoli-Leydig cell tumor is usually asymptomatic.
  • Symptoms of Sertoli-Leydig cell tumor may include the following:[1]

Physical Examination

  • Patients with Sertoli-Leydig cell tumor usually are well-appearing.
  • Physical examination may be remarkable for the following features:[2]
  • Progressive masculinization

Laboratory Findings

  • Laboratory findings consistent with the diagnosis of Sertoli-Leydig cell tumor, include:[4]

Imaging Findings

  • On ultrasound, findings of Sertoli-Leydig cell tumor, include:[1]
  • Hypoechoic lesion
  • Usually solitary, unless part of a syndrome
  • Well-defined, enhancing solid mass
  • On MRI, findings of Sertoli-Leydig cell tumor, include:[1]
  • Limited characterization
  • Nonspecific appearance
  • The signal intensity on T2-weighted MR images depends on their fibrous content.
  • Most tumors demonstrate predominantly low T2 signal intensity with scattered areas of high signal intensity.

Treatment

Medical Therapy

  • The mainstay of therapy for Sertoli-Leydig cell tumor is platinum-based adjuvant chemotherapy.[1]
  • Common chemotherapy regimens for Sertoli-Leydig cell tumor, include:

Surgery

  • Surgery is the mainstay of therapy for sertoli-leydig cell tumor.[1]
  • Fertility-sparing unilateral salpingo-oophorectomy is the most common approach to the treatment of Sertoli-Leydig cell tumor in females before childbearing.
  • Total abdominal hysterectomy with bilateral salpingo-oophorectomy is the most common approach to the treatment of Sertoli-Leydig cell tumor in females after childbearing.

Prevention

  • There are no primary preventive measures available for Sertoli-Leydig cell tumor.

References

  1. 1.00 1.01 1.02 1.03 1.04 1.05 1.06 1.07 1.08 1.09 1.10 Sertoli-Leydig Tumor. Wikipedia. https://en.wikipedia.org/wiki/Sertoli%E2%80%93Leydig_cell_tumour Accessed on April 18, 2016
  2. 2.0 2.1 2.2 2.3 2.4 2.5 2.6 2.7 2.8 2.9 Tandon R, Goel P, Saha PK, Takkar N, Punia RP (2007). "A rare ovarian tumor - Sertoli-Leydig cell tumor with heterologous element". MedGenMed. 9 (4): 44. PMC 2234306Freely accessible. PMID 18311394. 
  3. DiSaia PJ, Creasman WT. Germ cell, stromal and other ovarian tumors. In: Clinical Gynecologic Oncology, Mosby-Yearbook, 1997. p.351.
  4. Liggins CA, Ma LT, Schlumbrecht MP (2016). "Sertoli-Leydig cell tumor of the ovary: A diagnostic dilemma". Gynecol Oncol Rep. 15: 16–9. PMC 4750013Freely accessible. PMID 26937481. doi:10.1016/j.gore.2015.12.003. 

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