Ovary tumor
Sex cord stromal tumors
Sertoli-Leydig tumor

Reviewer: Nat Pernick, M.D. (see Authors page)

Revised: 11 April 2016, last major update December 2008

Copyright: (c) 2002-2016, PathologyOutlines.com, Inc.

PubMed Search: Sertoli-Leydig tumor ovary
General
  • Also called Sertoli-stromal cell tumor; formerly called androblastoma, arrhenoblastoma
  • Rare, < 0.1% of ovarian neoplasms
  • Morphologically resembles cells of testis at various stages of development, but ultrastructurally resembles ovarian granulosa cell tumors and contains female sex chromatin
  • Usually young women (mean age 25 years, 75% < age 30), but 10% > age 50; some with edema are diagnosed during pregnancy
  • Virilization in 30-50% with oligo / amenorrhea, loss of secondary sex characteristics, breast atrophy, masculinization [clitoral hypertrophy, hirsutism, deepening of voice]; also increased RBC count, increased serum testosterone and androstenedione, variable increases in serum alpha-fetoprotein and usually normal serum 17-ketosteroids
  • Feminine characteristics return after surgery, but manifestations of masculinization disappear more slowly
  • 50% have no endocrine abnormalities
  • 2% bilateral, 97% stage I, 2% spread beyond ovary
  • 5% recur / metastasize
  • Well differentiated: older age, usually not associated with masculinization; usually clinically benign
  • Intermediate differentiated: associated with virilization; 11% clinically malignant
  • Poorly differentiated: 59% clinically malignant; case report of AFP production (Arch Pathol Lab Med 1986;110:65)
  • Tumors with heterologous elements: 19% clinically malignant
  • Retiform pattern: often young girls; tumors are stage I
  • Some cells show differentiation into both Sertoli cells and cells of rete testis, but lack presence of sex-determining region Y (Hum Pathol 2001;32:796, Hum Pathol 1997;28:1206)
  • References: Am J Surg Pathol 1985;9:543, Am J Surg Pathol 1984;8:405, Am J Surg Pathol 1983;7:755 (retiform pattern)
  • Case Reports
  • 4 year old girl with retiform pattern (Arch Pathol Lab Med 2002;126:377)
  • 16 year old girl with ovarian mass and hirsutism (Case of the Week #384)
  • 13 year old girl with elevated serum AFP, heterologous elements (hepatocytes) and retiform pattern (Am J Surg Pathol 1984;8:709)
  • 17 year old girl with elevated AFP and clusters of hepatocytes (Arch Pathol Lab Med 1995;119:1075)
  • 22 year old woman with retiform pattern and mucinous component (Arch Pathol Lab Med 2004;128:e93)
  • Treatment
  • Conservative surgery if tumor confined to ovary
  • Gross Description
  • Similar to granulosa cell tumors - golden-yellow but fewer blood filled cysts, almost never have unilocular cyst
  • Gross Images

    Images hosted on PathOut server:

    Case of the Week #384

    Solid yellow lobulated mass

    Cystic tumor

    Heterologous elements

    Retiform pattern



    Images hosted on other servers:

    Solid yellow-poorly differentiated tumor

    Partially cystic with polypoid or grapelike nodules protruding into cystic spaces;
    the more solid areas were soft and tan to brown; areas of hemorrhage

    Micro Description
  • Hemorrhage / necrosis suggests poorly differentiated tumors or heterologous elements; edematous papillae suggests retiform subtype
  • Degenerative changes (bizarre and multiple nuclei) have no prognostic significance
  • Multiple subtypes may exist in same tumor (so % add up to > 100%)
  • Well differentiated (Meyer type I): 11%, may be nodular; tubular (hollow > solid), easily identifiable Leydig cells and Sertoli cells in a tubular pattern; may resemble endometrioid carcinoma or atrophic seminiferous tubules; crystals of Reinke in 20%
  • Intermediate differentiated (Meyer type II): 54%, 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): 13%, resemble indifferent gonad; may have sarcomatous appearance, Leydig cells in 2/3; may have mucus filled epithelial cells, cartilage, skeletal muscle
  • Pure Sertoli cell tumors (tubular androblastoma): similar to well differentiated Sertoli-Leydig cells but no Leydig cells and no primitive stromal elements; cytoplasm may have abundant lipid; tubular or follicle-like patterns; variable amyloid
  • With heterologous elements (teratoid androblastoma): 22%, associated with mucinous epithelium of GI type, liver, skeletal muscle and cartilage
  • Retiform ("netlike"): 15%, irregular branching, elongated and narrow tubules and cysts, which often have papillary structures; also ribbons of immature Sertoli cells; degenerative cells (large, bizarre cells without mitotic activity); usually associated with intermediate or poorly differentiated tumors; often have homologous or heterologous tissues including hepatocyte differentiation (positive for AE1-AE3, CAM5.2, AFP, ferritin, Hum Pathol 1999;30:611); resemble yolk sac tumors (Schiller-Duval bodies, AFP+), serous borderline tumors (older patients, no ribbons of immature cells)
  • Micro Images

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    Images hosted on PathOut server:

    Case of the Week #384


    Well differentiated tumor


    Intermediate differentiated tumor


    Poorly differentiated tumor


    Retiform pattern



    Heterologous elements



    Images hosted on other servers:

    Inhibin alpha
    (fig 2)

    Fig 2B: papillary area; fig 3A: moderate to well differentiated
    intestinal type mucinous epithelium with goblet cells;
    fig 3B: focal stratification and nuclear atypia; fig 4A/B: inhibin+


    Positive Stains
  • Testosterone, estradiol, cytokeratin, AFP (in hepatoid areas), CD99 (Mod Pathol 1998;11:769), inhibin alpha, low molecular weight cytokeratin, anti-Mullerian hormone focally (Hum Pathol 2000;31:1202), progesterone receptor, androgen receptor and vimentin (Hum Pathol 1997;28:1206)
  • Negative Stains
  • EMA, PLAP, CEA, CA19-9, CA125 and S100 (use to distinguish from carcinosarcoma)
  • Electron Microscopy Description
  • Elongated nuclei with deep indentations and annulate lamellae (resemble Sertoli cells), apical microvilli and frequent desmosomes (resemble rete testis)
  • Molecular / Cytogenetics Description
  • Trisomy 8 in case report (Hum Pathol 2001;32:559)