Juvenile granulosa cell tumour

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Juvenile granulosa cell tumour
Diagnosis in short

Juvenile granulosa cell tumour. H&E stain.

LM microcystic spaces, cuboidal-to-polygonal cells in sheets or stands or cords, with moderate-to-marked nuclear atypia, and basophilic cytoplasm
LM DDx Brenner tumour, small cell carcinoma of hypercalcemic type
IHC inhibin +ve, calretinin +ve
Molecular trisomy 12
Gross mass lesion, solid
Site ovary - ovarian tumours

Associated Dx endometrial hyperplasia, endometrioid endometrial carcinoma
Symptoms +/-menorrhagia
Prevalence uncommon
Prognosis good to moderate (dependent on stage)
Treatment surgery

Juvenile granulosa cell tumour is an uncommon ovarian tumour in the sex cord stromal tumours group.

General

  • Uncommon.[1]
  • Reported in males.[2]

May secrete estrogen - can present with:

Prognosis - granulosa cell tumours (adult and juvenile):[1]

  • Low-stage: 97% five year survival.
  • High-stage: 67% five year survival.

Gross

  • Classically solid.

Microscopic

Features:

  • Microcystic spaces.
  • Moderate-to-marked nuclear atypia.
  • Cuboidal-to-polygonal cell in sheets or stands or cords.
  • Basophilic cytoplasm.

Notes:

  • Juvenile variant of GCT has more nuclear pleomorphism.

DDx:

Images

Juvenile granulosa cell tumor Juvenile granulosa cell tumor Juvenile granulosa cell tumor Juvenile granulosa cell tumor Juvenile granulosa cell tumor of ovary of child. A. A pink tumor shows cyst-like areas with occasional nodules at low power. B. Tumor cells haphazardly spread without longitudinal grooves, with small nucleoli, in an edematous background. C. The cysts are in fact follicles, some producing, as here, the typical basophilic fluid. D. In some regions, abundant pink cytoplasm is obvious.

IHC

  • Inhibin positive.[5]
  • Calretinin +ve.
  • Ki-67 <5% (12/12 cases[6]).
  • CD34 -ve (0 +ve/12 cases[6]).
  • Vimentin +ve (11 +ve/12 cases[6]).
  • CD99 +ve.[7]

Molecular

Currently not used for the diagnosis.

See also

References

  1. 1.0 1.1 Oztekin, D.; Kurt, S.; Camuzcuoglu, H.; Balsak, D.; Dicle, N.; Tinar, S.. "Granulosa cell tumors of the ovary: review of 43 cases.". J BUON 17 (3): 461-4. PMID 23033282.
  2. URL: http://path.upmc.edu/cases/case631.html. Accessed on: 26 January 2012.
  3. Hashemipour, M.; Moaddab, MH.; Nazem, M.; Mahzouni, P.; Salek, M. (Jul 2010). "Granulosa cell tumor in a six-year-old girl presented as precocious puberty.". J Res Med Sci 15 (4): 240-2. PMID 21526089.
  4. Bárcena, C.; Oliva, E. (Nov 2011). "WT1 expression in the female genital tract.". Adv Anat Pathol 18 (6): 454-65. doi:10.1097/PAP.0b013e318234aaed. PMID 21993272.
  5. Cotran, Ramzi S.; Kumar, Vinay; Fausto, Nelson; Nelso Fausto; Robbins, Stanley L.; Abbas, Abul K. (2005). Robbins and Cotran pathologic basis of disease (7th ed.). St. Louis, Mo: Elsevier Saunders. pp. 1102. ISBN 0-7216-0187-1.
  6. 6.0 6.1 6.2 Kondi-Pafiti, A.; Grapsa, D.; Kairi-Vassilatou, E.; Carvounis, E.; Hasiakos, D.; Kontogianni, K.; Fotiou, S. (2010). "Granulosa cell tumors of the ovary: a clinicopathologic and immunohistochemical study of 21 cases.". Eur J Gynaecol Oncol 31 (1): 94-8. PMID 20349790.
  7. Schmidt, D.; Kommoss, F. (May 2007). "[Diagnosis and differential diagnosis of granulosa cell tumor].". Pathologe 28 (3): 195-202. doi:10.1007/s00292-007-0908-8. PMID 17387475.
  8. Schofield, DE.; Fletcher, JA. (Dec 1992). "Trisomy 12 in pediatric granulosa-stromal cell tumors. Demonstration by a modified method of fluorescence in situ hybridization on paraffin-embedded material.". Am J Pathol 141 (6): 1265-9. PMID 1466394.
  9. Mayr, D.; Kaltz-Wittmer, C.; Arbogast, S.; Amann, G.; Aust, DE.; Diebold, J. (Sep 2002). "Characteristic pattern of genetic aberrations in ovarian granulosa cell tumors.". Mod Pathol 15 (9): 951-7. doi:10.1097/01.MP.0000024290.55261.14. PMID 12218213.