6 April 2016 - Case of the Week #384

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Case of the Week #384

Clinical history:
A 16 year old girl presented with hirsutism and an ovarian mass, which was excised.


Gross images:


Micro images:



What is your diagnosis?































Diagnosis:
Sertoli-Leydig cell tumor, intermediate grade, with heterologous liver


Discussion:
Sertoli-Leydig cell tumors (SLCT) of the ovary are uncommon sex-cord stromal tumors. They comprise less than 0.1% of ovarian neoplasms and are usually found in young patients (average 25 years). Most tumors are unilateral and confined to the ovaries (MedGenMed 2007;9:44). These tumors are characterized by testicular structures that produce androgens, and nearly 50% are accompanied by androgen excess (amenorrhea, breast atrophy, loss of subcutaneous fat, deepening of the voice and hirsutism, Rosai and Ackerman's Surgical Pathology, 2011, MedGenMed 2007;9:44).

Grossly, they are predominantly solid, although cystic areas may be present. Microscopically, they are composed of a variable mixture of cells resembling male Sertoli and Leydig cells. Growth patterns, which may coexist in the same tumor, include well differentiated, intermediate, poorly differentiated, pure Sertoli cell, retiform, and containing heterologous elements (Rosai and Ackerman's Surgical Pathology, 2011). Well differentiated tumors are composed of tubules lined by Sertoli-like cells separated by variable numbers of Leydig-like cells. Intermediate grade tumors are characterized by sheets and cords of Sertoli-like cells, separated by spindle stromal cells and recognizable Leydig cells. Poorly differentiated tumors show spindle cells in a sarcomatoid pattern. Pure Sertoli cell tumors are composed entirely of Sertoli cells. Tumors with heterologous elements account for 20% of Sertoli-Leydig cell tumors and contain mesenchymal cells including bone, cartilage or skeletal muscle, or endodermal elements such as cysts or glands (MedGenMed 2007;9:44). Tumors with heterologous mesenchymal elements are usually poorly differentiated, but those with endodermal elements typically are of intermediate differentiation (Arch Gynecol Obstet 2001;264:206). In the retiform variant, tumor cells coexist with formations resembling the rete of the ovary or testis. Bizarre nuclei may be seen in all variants, with no apparent prognostic significance.

The gonadal component strongly expresses inhibin, the areas of Sertoli differentiation stain for keratin and SOX9, and WT1 is usually positive in both components (Rosai and Ackerman's Surgical Pathology, 2011).

The differential diagnosis of Sertoli-Leydig cell tumor with heterologous elements includes carcinosarcoma, and IHC is useful in making this determination (Rosai and Ackerman's Surgical Pathology, 2011).

Prognosis correlates with tumor stage and differentiation (MedGenMed 2007;9:44). The overall incidence of clinical malignancy in one large series was 18%; all well differentiated tumors had benign behavior, but 19% with heterologous elements and 59% of poorly differentiated tumors had malignant behavior (Am J Surg Pathol 1985;9:543). In intermediate or poorly differentiated SLCT, a survival rate of 92% was noted at both 5 and 10 years (Am J Surg Pathol 1984;8:405).


Discussion by Dr. Jennifer R. Kaley, University of Arkansas for Medical Sciences (USA).