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Juvenile Granulosa Cell Tumor

MedGen UID:
83141
Concept ID:
C0334403
Neoplastic Process
Synonyms: Juvenile granulosa cell tumor; Juvenile Type Granulosa Cell Neoplasm; Juvenile Type Granulosa Cell Tumor
SNOMED CT: Juvenile granulosa cell tumor (77029009)

Definition

A sex cord-stromal tumor occurring in the ovary and testis. In females it occurs predominantly in the first three decades of life and presents unilaterally as stage I disease in the vast majority of cases. It is characterized by the presence of granulosa cells forming macrofollicular structures. The majority of cases have a good prognosis. In males it represents the most frequent congenital testicular neoplasm, and the vast majority of cases occur in the perinatal period. It presents as a scrotal or abdominal mass, and it more often affects the left testis. Approximately 20% of the patients have ambiguous external genitalia. It is characterized by the presence of cystic spaces lined by granulosa cells and cells resembling theca cells. Metastases have not been reported. [from NCI]

Professional guidelines

PubMed

Han LM, Weiel JJ, Longacre TA, Folkins AK
Adv Anat Pathol 2022 Sep 1;29(5):297-308. Epub 2022 Jul 1 doi: 10.1097/PAP.0000000000000351. PMID: 35778792
Hayes-Jordan A, Lopez C, Green HL, Xiao LC, Huh W, Herzog CE
Pediatr Surg Int 2016 Jan;32(1):71-3. doi: 10.1007/s00383-015-3814-9. PMID: 26500075Free PMC Article

Recent clinical studies

Etiology

Hamzaoğlu Canbolat K, Biçer E, İlvan Ş, Beşe T, Çepni İ, Demirkıran F
Ginekol Pol 2022;93(10):787-792. doi: 10.5603/GP.a2022.0107. PMID: 36748172
Han LM, Weiel JJ, Longacre TA, Folkins AK
Adv Anat Pathol 2022 Sep 1;29(5):297-308. Epub 2022 Jul 1 doi: 10.1097/PAP.0000000000000351. PMID: 35778792
Parikshaa G, Ariba Z, Pranab D, Nalini G, Manish R, Vanita S, Bhavana R, Devi D, Ram S, Prema M, Arvind R, Radhika S
Ann Diagn Pathol 2021 Jun;52:151721. Epub 2021 Feb 10 doi: 10.1016/j.anndiagpath.2021.151721. PMID: 33725665
Wu H, Pangas SA, Eldin KW, Patel KR, Hicks J, Dietrich JE, Venkatramani R
J Pediatr Adolesc Gynecol 2017 Feb;30(1):138-143. Epub 2016 Oct 1 doi: 10.1016/j.jpag.2016.09.008. PMID: 27702598
Daubenton JD, Sinclair-Smith C
Med Pediatr Oncol 2000 Apr;34(4):301-3. doi: 10.1002/(sici)1096-911x(200004)34:4<301::aid-mpo22>3.0.co;2-d. PMID: 10742079

Diagnosis

Ordulu Z
Clin Lab Med 2023 Jun;43(2):245-274. doi: 10.1016/j.cll.2023.03.001. PMID: 37169445
Seabra J, Silva A, Santos I, Almeida J, Correia L, Correia M
Int J Gynaecol Obstet 2023 Mar;160(3):1052-1053. Epub 2022 Oct 22 doi: 10.1002/ijgo.14496. PMID: 36208031
Ordulu Z
Surg Pathol Clin 2022 Jun;15(2):235-258. doi: 10.1016/j.path.2022.02.004. PMID: 35715160
Pommert L, Bradley W
Curr Oncol Rep 2017 Jul;19(7):44. doi: 10.1007/s11912-017-0604-7. PMID: 28501984
Scully RE
Pediatr Pathol 1988;8(4):423-7. doi: 10.3109/15513818809041579. PMID: 3211812

Therapy

Ordulu Z, Young RH
Am J Surg Pathol 2021 Jan;45(1):59-67. doi: 10.1097/PAS.0000000000001544. PMID: 32815828
Zhao D, Song Y, Zhang Y, Li B
Int J Gynecol Cancer 2019 May;29(4):787-791. Epub 2019 Feb 6 doi: 10.1136/ijgc-2018-000083. PMID: 30728165
Powell JL, Kotwall CA, Shiro BC
J Pediatr Adolesc Gynecol 2014 Aug;27(4):e89-92. Epub 2014 Mar 19 doi: 10.1016/j.jpag.2013.10.001. PMID: 24656706
Tao X, Sood AK, Deavers MT, Schmeler KM, Nick AM, Coleman RL, Milojevic L, Gershenson DM, Brown J
Gynecol Oncol 2009 Sep;114(3):431-6. Epub 2009 Jun 13 doi: 10.1016/j.ygyno.2009.04.021. PMID: 19524286Free PMC Article
Erdreich-Epstein A, Monforte HL, Lavey RS, Joshi S, Phillips JD, Villablanca JG
J Pediatr Hematol Oncol 2002 Mar-Apr;24(3):229-33. doi: 10.1097/00043426-200203000-00015. PMID: 11990313

Prognosis

Ordulu Z
Clin Lab Med 2023 Jun;43(2):245-274. doi: 10.1016/j.cll.2023.03.001. PMID: 37169445
Ordulu Z
Surg Pathol Clin 2022 Jun;15(2):235-258. doi: 10.1016/j.path.2022.02.004. PMID: 35715160
Parikshaa G, Ariba Z, Pranab D, Nalini G, Manish R, Vanita S, Bhavana R, Devi D, Ram S, Prema M, Arvind R, Radhika S
Ann Diagn Pathol 2021 Jun;52:151721. Epub 2021 Feb 10 doi: 10.1016/j.anndiagpath.2021.151721. PMID: 33725665
San TH, Ota Y, Fushimi S, Fujisawa M, Yanai H, Toda H, Kunitomo T, Kodama K, Matsukawa A
Acta Med Okayama 2020 Apr;74(2):159-163. doi: 10.18926/AMO/58275. PMID: 32341591
El Abiad JM, Robbins SM, Cohen B, Levin AS, Valle DL, Morris CD, de Macena Sobreira NL
Am J Med Genet A 2020 May;182(5):1093-1103. Epub 2020 Mar 7 doi: 10.1002/ajmg.a.61530. PMID: 32144835Free PMC Article

Clinical prediction guides

Marino G, Marchetta L, Negri S, Testa F, Lugotti D, Cavallo G, Grassi T, Jaconi M, De Ponti E, Bonazzi MC, Landoni F, Fruscio R
Gynecol Oncol 2025 Jan;192:89-93. Epub 2024 Nov 18 doi: 10.1016/j.ygyno.2024.11.007. PMID: 39561630
Hamzaoğlu Canbolat K, Biçer E, İlvan Ş, Beşe T, Çepni İ, Demirkıran F
Ginekol Pol 2022;93(10):787-792. doi: 10.5603/GP.a2022.0107. PMID: 36748172
Ye Y, Lv C, Xu S, Chen Y, Qian R, Wang P, Wang S
Am J Clin Pathol 2020 Oct 13;154(5):635-644. doi: 10.1093/ajcp/aqaa081. PMID: 32561911
Young RH
Arch Pathol Lab Med 2018 Dec;142(12):1459-1484. doi: 10.5858/arpa.2018-0291-RA. PMID: 30500284
Chan YF, Restall P, Kimble R
J Pediatr Surg 1997 May;32(5):752-3. doi: 10.1016/s0022-3468(97)90025-7. PMID: 9165470

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