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Table of Contents
Definition / general | Terminology | Epidemiology | Sites | Etiology | Clinical features | Diagnosis | Laboratory | Radiology description | Prognostic factors | Case reports | Treatment | Gross description | Microscopic (histologic) description | Microscopic (histologic) images | Positive stains | Negative stains | Differential diagnosisCite this page: Sertoli - Leydig cell tumor. PathologyOutlines.com website. http://www.pathologyoutlines.com/topic/testissertolileydig.html. Accessed July 15th, 2017.
Definition / general
- Group of tumors composed of variable proportions of Sertoli cells, Leydig cells and in the case of intermediate and poorly differentiated neoplasms, primitive gonadal stroma and sometimes heterologous elements
- More common in ovary than testis
Terminology
- Previously known as androblastoma or arrhenoblastoma
Epidemiology
- Extremely rare (<1% of all testicular tumors)
- Occurs at any age but most often in young adults
- Occasionally associated with complete androgen insensitivity
Sites
- Normally descended testis
Etiology
- Exact etiology is not known
- No recurrent genetic abnormality has been documented in testicular Sertoli - Leydig cell tumors
- However, >50% of ovarian Sertoli Leydig cell tumors harbor DICER1 mutations (Br J Cancer 2013;109:2744, N Engl J Med 2012;366:234, JAMA 2011;305:68)
Clinical features
- Gynecomastia occurs in 1/3, most common presentation
- May have enlargement of testis
Diagnosis
- Definitive diagnosis based on histology
Laboratory
- Elevated levels of testosterone may be present
- AFP, β-HCG and LDH levels are usually normal
Radiology description
- Hypoechoic well circumscribed lesion is commonly seen on ultrasound imaging
- However, these findings are not specific and do not help to differentiate from other tumors or between benign and malignant tumors
Prognostic factors
- Most tumors are benign
- Malignancy is associated with poorly differentiated histology and older age
- Prognostic factors are similar as those for sex cord stromal tumors in general
Case reports
- 15 year old girl with coexisting complete androgen insensitivity syndrome (J Pediatr Adolesc Gynecol 2014;27:113)
- A malignant mixed gonadal stromal tumor of the testis with heterologous components and i(12p) in one of its metastases (Cancer Genet Cytogenet 1989;41:105)
Treatment
- High inguinal orchidectomy is treatment of choice as it is difficult to rule out other more aggressive tumors without histological examination
- Some evidence suggests wide local excision instead of radical orchidectomy may be adequate in tumors without any demonstrable clinical / radiological / biochemical evidence of malignancy
Gross description
- Yellow to yellowish white
- Entirely or predominantly solid, often lobulated and encapsulated
- Usually restricted to the testis
Microscopic (histologic) description
- Histologically similar to ovarian counterpart
- Diagnostic features are unequivocal tubular component of Sertoli cell type or sex cord patterns typical of ovarian Sertoli - Leydig cell tumor, as well as a neoplastic stromal component that contains at least focal Leydig cells
- Most reported cases were of intermediate differentiation but well differentiated (J Urol 1992;148:883) and poorly differentiated tumors have also been described
- As in ovarian cases, retiform patterns (Ulbright: Tumors of the Testis, Adnexa, Spermatic Cord, and Scrotum (AFIP Third Series, 1999) and heterologous elements have been described (Cancer Genet Cytogenet 1989;41:105)
- Well differentiated (Meyer type I):
- Nodular / tubular (hollow > solid), easily identifiable Leydig cells and Sertoli cells in a tubular pattern
- Crystals of Reinke in 20%
- Often associated with androgen insensitivity syndrome
- Intermediate differentiated (Meyer type II):
- Outlines of immature tubules with immature Sertoli cells and large Leydig cells
- May have mucus filled epithelial cells, cartilage and skeletal muscle
- Poorly differentiated (Meyer type III):
- Resembles indifferent gonad
- May have sarcomatous appearance, Leydig cells in 2/3
- May have mucus filled epithelial cells, cartilage, skeletal muscle
Microscopic (histologic) images
Images hosted on other servers:
Various images
Positive stains
Differential diagnosis
- Pure sex cord stromal tumors:
- Show only one cell type and not more than an occasional microscopic focus of other elements
- Presence of more than occasional occurrence of two or more sex cord stromal cell types warrants a diagnosis of mixed sex cord stromal tumor
- Sex cord stromal tumor with entrapped germ cells (Am J Surg Pathol 2000;24:535) and mixed germ cell-sex-cord stromal tumors
- Show clusters of germ cells in the center of the tumor
- Only one sex cord stromal cell type present
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