Difference between revisions of "Juvenile granulosa cell tumour"

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Juvenile granulosa cell tumour (view source)

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#redirect [[Ovarian_tumours#Juvenile_granulosa_cell_tumour]]
{{ Infobox diagnosis
| Name      = {{PAGENAME}}
| Image      = Juvenile_granulosa_cell_tumour_-_very_high_mag.jpg
| Width      =
| Caption    = Juvenile granulosa cell tumour. [[H&E stain]].
| Synonyms  =
| Micro      = microcystic spaces, cuboidal-to-polygonal cells in sheets or stands or cords, with moderate-to-marked nuclear atypia, and basophilic cytoplasm
| Subtypes  =
| LMDDx      = [[Brenner tumour]], [[small cell carcinoma of hypercalcemic type]]
| Stains    =
| IHC        = inhibin +ve, calretinin +ve
| EM        =
| Molecular  = trisomy 12
| IF        =
| Gross      = mass lesion, solid
| Grossing  =
| Site      = [[ovary]] - ''[[ovarian tumours]]''
| Assdx      =  [[endometrial hyperplasia]], [[endometrioid endometrial carcinoma]]
| Syndromes  =
| Clinicalhx =
| Signs      =
| Symptoms  = +/-menorrhagia
| Prevalence = uncommon
| Bloodwork  =
| Rads      =
| Endoscopy  =
| Prognosis  = good to moderate (dependent on stage)
| Other      =
| ClinDDx    =
| Tx        = surgery
}}
'''Juvenile granulosa cell tumour''' is an uncommon [[ovarian tumour]] in the ''sex cord stromal tumours'' group.
 
==General==
*Uncommon.<ref name=pmid23033282>{{Cite journal  | last1 = Oztekin | first1 = D. | last2 = Kurt | first2 = S. | last3 = Camuzcuoglu | first3 = H. | last4 = Balsak | first4 = D. | last5 = Dicle | first5 = N. | last6 = Tinar | first6 = S. | title = Granulosa cell tumors of the ovary: review of 43 cases. | journal = J BUON | volume = 17 | issue = 3 | pages = 461-4 | month =  | year =  | doi =  | PMID = 23033282 }}</ref>
*Reported in males.<ref>URL: [http://path.upmc.edu/cases/case631.html http://path.upmc.edu/cases/case631.html]. Accessed on: 26 January 2012.</ref>
 
May secrete estrogen - can present with:
*Endometrial pathology, e.g. [[endometrial hyperplasia]] ''or'' endometrioid [[endometrial carcinoma]].
*Precocious puberty.<ref name=pmid21526089>{{Cite journal  | last1 = Hashemipour | first1 = M. | last2 = Moaddab | first2 = MH. | last3 = Nazem | first3 = M. | last4 = Mahzouni | first4 = P. | last5 = Salek | first5 = M. | title = Granulosa cell tumor in a six-year-old girl presented as precocious puberty. | journal = J Res Med Sci | volume = 15 | issue = 4 | pages = 240-2 | month = Jul | year = 2010 | doi =  | PMID = 21526089 }}</ref>
 
Prognosis - granulosa cell tumours (adult and juvenile):<ref name=pmid23033282/>
*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:
*[[Brenner tumour]].
*[[Adult granulosa cell tumour]].
*[[Small cell carcinoma of hypercalcemic type]].<ref name=pmid21993272>{{Cite journal  | last1 = Bárcena | first1 = C. | last2 = Oliva | first2 = E. | title = WT1 expression in the female genital tract. | journal = Adv Anat Pathol | volume = 18 | issue = 6 | pages = 454-65 | month = Nov | year = 2011 | doi = 10.1097/PAP.0b013e318234aaed | PMID = 21993272 }}</ref>
 
===Images===
<gallery>
Image:Juvenile_granulosa_cell_tumour_-_intermed_mag.jpg | Juvenile granulosa cell tumour - intermed. mag. (WC)
Image:Juvenile_granulosa_cell_tumour_-_high_mag.jpg | Juvenile granulosa cell tumour - high mag. (WC)
Image:Juvenile_granulosa_cell_tumour_-_very_high_mag.jpg | Juvenile granulosa cell tumour - very high mag. (WC)
</gallery>
[[File:3 13627319654987 sl 1.png| Juvenile granulosa cell tumor]]
[[File:3 13627319654987 sl 2.png| Juvenile granulosa cell tumor]]
[[File:3 13627319654987 sl 3.png| Juvenile granulosa cell tumor]]
[[File:3 13627319654987 sl 4.png| 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.<ref name=Ref_PBoD1102>{{Ref PBoD|1102}}</ref>
** Inhibin negative in ''[[Brenner tumour]]''.
*Calretinin +ve.
*Ki-67 <5% (12/12 cases<ref name=pmid20349790>{{Cite journal  | last1 = Kondi-Pafiti | first1 = A. | last2 = Grapsa | first2 = D. | last3 = Kairi-Vassilatou | first3 = E. | last4 = Carvounis | first4 = E. | last5 = Hasiakos | first5 = D. | last6 = Kontogianni | first6 = K. | last7 = Fotiou | first7 = S. | title = Granulosa cell tumors of the ovary: a clinicopathologic and immunohistochemical study of 21 cases. | journal = Eur J Gynaecol Oncol | volume = 31 | issue = 1 | pages = 94-8 | month =  | year = 2010 | doi =  | PMID = 20349790 }}</ref>).
*CD34 -ve (0 +ve/12 cases<ref name=pmid20349790/>).
*Vimentin +ve (11 +ve/12 cases<ref name=pmid20349790/>).
*CD99 +ve.<ref name=pmid17387475>{{Cite journal  | last1 = Schmidt | first1 = D. | last2 = Kommoss | first2 = F. | title = [Diagnosis and differential diagnosis of granulosa cell tumor]. | journal = Pathologe | volume = 28 | issue = 3 | pages = 195-202 | month = May | year = 2007 | doi = 10.1007/s00292-007-0908-8 | PMID = 17387475 }}</ref>
 
==Molecular==
:''Currently '''not''' used for the diagnosis.''
*Trisomy 12.<ref name=pmid1466394>{{Cite journal  | last1 = Schofield | first1 = DE. | last2 = Fletcher | first2 = JA. | title = Trisomy 12 in pediatric granulosa-stromal cell tumors. Demonstration by a modified method of fluorescence in situ hybridization on paraffin-embedded material. | journal = Am J Pathol | volume = 141 | issue = 6 | pages = 1265-9 | month = Dec | year = 1992 | doi =  | PMID = 1466394 }}</ref><ref name=pmid12218213>{{Cite journal  | last1 = Mayr | first1 = D. | last2 = Kaltz-Wittmer | first2 = C. | last3 = Arbogast | first3 = S. | last4 = Amann | first4 = G. | last5 = Aust | first5 = DE. | last6 = Diebold | first6 = J. | title = Characteristic pattern of genetic aberrations in ovarian granulosa cell tumors. | journal = Mod Pathol | volume = 15 | issue = 9 | pages = 951-7 | month = Sep | year = 2002 | doi = 10.1097/01.MP.0000024290.55261.14 | PMID = 12218213 }}
</ref>
 
==See also==
*[[Ovarian tumours]].
 
==References==
{{Reflist|2}}
 
[[Category:Ovarian tumours]]
[[Category:Diagnosis]]
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