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A 17-year-old woman presented with a 5 month history of progressive abdominal fullness and pain and was found to have a 20.0 cm left ovarian mass. Serum a-fetoprotein and ß-HCG were within normal limits. A left salpingo-oophorectomy specimen with a 20.0 cm smooth-surfaced mass replacing the ovary was received; the fallopian tube was unremarkable. On cut section, the ovarian mass was solid and cystic with the cysts containing seromucinous and bloody fluid and the solid areas being soft and yellow-tan.
Archive Case and Diagnosis:
This case first appeared as Performance Improvement Program in Surgical Pathology (PIP) 2008, Case 15, and is juvenile granulosa cell tumor.
Criteria for Diagnosis and Comments:
This cellular neoplasm with a nodular growth pattern interspersed by variably sized cysts containing basophilic secretions and composed of small, round to cuboidal cells with pale eosinophilic cytoplasm represents a juvenile granulosa cell tumor.
Granulosa cell tumors, which comprise approximately 1-2% of all ovarian tumors, are separated into two distinct clinicopathologic and histologic types: adult granulosa cell tumor and juvenile granulosa cell tumor. The adult type accounts for 95% of granulosa cell tumors; the juvenile form, so-called because it typically occurs in the first three decades of life, comprises the remainder. On gross examination, granulosa cell tumors are typically large, unilateral tumors with a mean diameter of 12.0 cm. Tumors may be solid with a yellow/white sectioned surface that can show areas of hemorrhage and necrosis or they may have both a solid and cystic component in which the cysts (which are often filled with blood-tinged fluid or clotted blood) are separated by solid yellow or white nodules of varying size. Rarely, the tumors may predominantly be multicystic with a smooth internal lining.
Juvenile granulosa cell tumor (JGCT) most commonly occurs in the first three decades of life and when it occurs in prepubertal patients, it is frequently associated with isosexual pseudoprecocity. Histologically, these tumors are characterized by solid sheets of neoplastic cells with interspersed follicles, which usually vary in size. Often a nodular pattern of growth is present in which the nodules are separated by fibrothecomatous stroma. The granulosa cells typically have abundant pale eosinophilic, often vacuolated, cytoplasm with round, hyperchromatic nuclei that lack nuclear grooves. Mitotic activity is variable but may be brisk. Tumor cells are typically positive for inhibin, and are negative for epithelial membrane antigen. Stage is the most important prognostic factor, with Stage I JGCT having an excellent prognosis. In contrast to AGCT, recurrences in patients with JGCT typically occur within the first three years.
Adult granulosa cell tumor (AGCT) typically occurs in menopausal or postmenopausal women and is the most common ovarian tumor associated with estrogenic manifestations. It is associated with endometrial carcinoma (usually well differentiated) in approximately 5% of cases. Androgenic manifestations may also occasionally occur and are typically associated with tumors that have an extensive cystic component. Histologic examination may reveal a variety of often admixed patterns including 1) diffuse (sarcomatoid), 2) trabecular, 3) insular, 4) watered silk (characterized by parallel wavy rows of tumor cells), 5) gyriform (characterized by cords of cells in a ‘zigzag’ arrangement), and 6) follicular, of which the microfollicular pattern is the most common. This latter pattern is characterized by the presence of islands of tumor cells that contain numerous small round follicle-like spaces that simulate Call-Exner bodies of a normally developing graafian follicle. In all patterns, tumor cells typically have scant amounts of cytoplasm and round to oval uniform nuclei. The presence of a nuclear groove, which imparts a ‘coffee bean’ appearance to the nucleus, is a characteristic, but not necessarily prominent or common finding in all cases of AGCT. Rarely, tumors may focally contain cells with enlarged, pleomorphic and hyperchromatic nuclei. The stromal component of AGCTs, which is typically minor, may have a fibromatous appearance or contain theca-like cells. Tumor cells are typically positive for inhibin, and are negative for epithelial membrane antigen. All AGCTs are considered to have malignant potential, and late recurrences, sometimes decades after initial diagnosis, are not uncommon. Tumors typically spread to the pelvis and abdominal cavity, although distant metastasis may also occur. Serum inhibin levels can be monitored to detect residual or recurrent disease.
JGCT is distinguished from AGCT by 1) more commonly occurring in young women (< 30 years of age), 2) having follicles that are more irregular in shape and size, 3) lacking Call-Exner bodies, 4) having rounder nuclei that lack nuclear grooves, 5) typically having more abundant eosinophilic to vacuolated cytoplasm and 6) having follicles containing basophilic secretions.
Small cell carcinoma, hypercalcemic type may be mistaken for a JGCT since both tumors occur in young women and are characterized by the presence of follicle-like spaces. Small cell carcinoma is distinguished from JGCT by 1) being associated with paraendocrine hypercalcemia in approximately two thirds of patients, 2) its greater propensity for spread beyond the ovary at the time of presentation, 3) its lack of estrogenic manifestations, 4) containing cells with scanty cytoplasm, 5) having more frequent mitoses, 6) lack of inhibin immunoreactivity and 7) epithelial membrane antigen positivity, if present (can be variable).
Yolk sac tumor may be mistaken for JGCT since both occur at a young age and may have a myxoid appearing stroma. However, yolk sac tumor can be distinguished from JGCT by 1) lacking the characteristic nodular growth pattern with fibrothecomatous stroma of JGCT, 2) lacking follicle-like spaces, 3) the typical presence of other characteristic features of yolk sac tumor (e.g. Schiller-Duval bodies, hyalin bodies, reticular pattern of growth), 4) lack of inhibin staining, and 5) positive staining for α-fetoprotein.
Clear cell carcinoma, particularly the tubulocystic pattern, may be mistaken for a JGCT that has prominent follicle formation; however, clear cell carcinoma is distinguished from JGCT by 1) typically occurring in older patients, 2) the presence of other patterns characteristic of clear cell carcinoma (e.g. papillary growth with hyalin cores, hobnail cells) and 3) its typical occurrence in the background of an adenofibroma or endometriosis.