Background

A 36-year-old female presented with menorrhagia and abdominal pain. CECT revealed a right sided large enhancing mass in the pelvis measuring 12.8cm in size. Serum CA-125: 30.3U/ml. Patient underwent debulking surgery. Sections from ovary for examination.

Microscopy

Fig.9a; H&E; 0.38x

Fig.9b; H&E; 5x

Fig.9c; H&E; 10x

Fig.9d; H&E; 20x

Fig.9e; H&E; 10x

Images show a tumor arranged in diffuse sheets, nests and cords [Fig.9a-b]. At foci, microfollicular pattern of arrangement of tumor cells (Call-Exner bodies) is also observed [Fig.9c]. The tumor cells are round to oval having angulated vesicular nuclei showing prominent nuclear grooves [Fig.9d]. The stroma is edematous with foci of theca cell proliferation [Fig.9e].

Fig.9f; Inhibin

Fig.9g; SF-1

On Immunohistochemistry, tumor cells are positive for Inhibin [Fig.9f] & SF-1 [Fig.9g].

Final Impression: Adult granulosa cell tumor.

POINTS TO REMEMBER

Adult Granulosa cell tumor:

• It is a low grade malignant sex cord stromal tumor composed of granulosa cells with a variable number of fibroblasts and theca cells.
• Age of presentation: Wide age range with an average age of 45 to 55 years
• Clinical presentation:
  • Post-menopausal bleeding in older women
  • Menorrhagia, metrorrhagia or amenorrhea in young women
• Tumor is confined to the ovary (FIGO Stage I) in 80-90% cases
• Serum Inhibin levels: markedly elevated in nearly all patients
• Gross: Typically solid and cystic gross appearance
• Microscopically:
  • Mixture of several histological growth patterns can be seen.
  • Microfollicular pattern (Call-Exner bodies) is the most characteristic and consists of nest and sheets of granulosa cells punctated by small spaces filled with eosinophilic secretions.
  • Nuclear grooves are characteristic.
• Immunohistochemistry:
  • Positive stains: Inhibin, Calretinin, FOXL2, SF-1, CD56 and WT-1
  • Negative stains: CK7 and EMA
• Unfavourable prognostic factors:
  • Advanced stage
  • Large size (>15cm)
  • Bilaterality
  • Tumor rupture
• Molecular: Mis-sense somatic point mutation in FOXL2 gene (402C-G)- seen in >90% of adult granulosa cell tumors

Juvenile Granulosa cell tumor:

• Age of presentation: Children and young adults, usually in first three decades
• Clinical presentation: Isosexual pseudo precocity in young girls
• Microscopically:
  • Nodular or diffuse growth pattern punctuated by follicles of varying sizes containing basophilic or eosinophilic secretions
  • Nucleus lacks presence of longitudinal grooves
• Molecular:
  • Most common genetic abnormality: Trisomy 12
  • Absence of FOXL2 gene mutations
• Prognosis: Typically limited to ovary at diagnosis with good prognosis
• Associated syndromes :
  • Olliers disease (enchondromatosis)
  • Mafucci syndrome (enchondromatosis and multiple subcutaneous hemangiomas)

Contributed by: Dr. Anila Sharma

Compiled by: Dr. Ankur Kumar & Dr. Saloni Pahwa

In case of queries, email us at: kumar.ankur@rgcirc.org

Female genital tract Granulosa cell tumor Ovary

Last modified: 04/06/2021