ovarian granulosa cell tumor
Digital slides
NCK1-03 : ovarian juvenile granulosa cell tumor (Flash or ICS-Viewer)
NCK1-02 : ovarian juvenile granulosa cell tumor (Flash or ICS-Viewer)
Case 44 : Ovarian juvenile granulosa cell tumo
Definition: Ovarian sex cord stromal tumor showing differentiation towards follicular granulosa cells that can occur in adults (adult-type granulosa cell tumor) and in younger patients (juvenile granulosa cell tumor).
Clinical Features
adult-type granulosa cell tumor
- Usually childbearing age
- 75% have hyperestrinism, which may result in:
- isosexual precocious puberty in children
- metrorrhagia in adults, including postmenopausal
- Some cases clinically hormonally inactive
- A few androgenic
- Elevated serum inhibin and follicle regulatory proteins
- 75% have hyperestrinism, which may result in:
juvenile granulosa cell tumor
- more aggressive than adult
- more likely to produce distant metastases
- ≈80% during first two decades of life
- Usually presents with isosexual precocity
- Occasionally associated with:
- enchondromatosis (Ollier disease)
- Maffucci syndrome (Maffucci disease)
Pathogenesis
Differentiation toward follicular granulosa cells
Debatable whether arises from:
- granulosa cells in preexisting follicles
- specialized ovarian stroma
Types
juvenile granulosa cell tumor
adult-type granulosa cell tumor
Subtypes
ovarian granulosa cell tumors of the ovary, pseudopapillary pattern (#18301054#)
Macroscopy
Granulosa cell tumor with solid cut surface
Granulosa cell tumor showing admixture of solid and cystic areas
Predominantly cystic granulosa cell tumor
Granulosa cell tumor with an entirely cystic gross appearance
Diagnosis
Suspect granulosa cell origin of a metastatic tumor if a combination of microcystic and trabecular formations, especially if also Call–Exner bodies and grooved nuclei.
Open references
The new molecular biology of granulosa cell tumors of the ovary. Kalfa N, Veitia RA, Benayoun BA, Boizet-Bonhoure B, Sultan C. Genome Med. 2009 Aug 25;1(8):81. PMID: #19725933# [Free]
References
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