Juvenile granulosa cell tumour

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

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

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 tumour - intermed. mag. (WC)

  • Juvenile granulosa cell tumour - high mag. (WC)

  • Juvenile granulosa cell tumour - very high mag. (WC)

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.
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