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Juvenile type ovarian granulosa cell tumor

MedGen UID:
340891
•Concept ID:
C1855515
•
Neoplastic Process
Synonym: Juvenile ovarian granulosa cell tumor
 
HPO: HP:0031919

Definition

Juvenile granulosa cell ovarian tumor (JGCOT) is a rare sex cord stromal tumor, occuring most frequently in premenarchal girls or young women. In contrast to adult granulosa cell tumor, JGCOT has a high mitotic index and more aggressive tumor growth. Microscopically it is seen as diffuse and regularly distributed neoplastic cells with a wide cytoplasm and pleomorphic hyperchromatic nucleus. Follicle formation, in various sizes and shapes, is important in JGCOT. Call-Exner bodies are infrequently seen in JGCOT in contrast to the adult type. [from HPO]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVJuvenile type ovarian granulosa cell tumor

Conditions with this feature

Tumor predisposition syndrome 2
MedGen UID:
1823959
•Concept ID:
C5774186
•
Disease or Syndrome
Tumor predisposition syndrome-2 (TPDS2) is an autosomal recessive cancer predisposition syndrome characterized by the onset of various types of tumors or malignancies in young adulthood. The most common clinical manifestations include acute myeloid leukemia (AML), myelodysplastic syndrome, colorectal adenomatous polyposis and carcinoma, and uveal melanoma, although other tumors and malignancies have been reported (summary by Palles et al., 2022). For a discussion of genetic heterogeneity of TPDS, see TPDS1 (614327).
See: Condition Record
Tumor predisposition syndrome 2

Professional guidelines

PubMed

Ovarian sex-cord stromal tumours and small cell tumours: Pathological, genetic and management aspects.
Boussios S, Moschetta M, Zarkavelis G, Papadaki A, Kefas A, Tatsi K
Crit Rev Oncol Hematol 2017 Dec;120:43-51. Epub 2017 Oct 16 doi: 10.1016/j.critrevonc.2017.10.007. PMID: 29198337
DICER1-related Sertoli-Leydig cell tumor and gynandroblastoma: Clinical and genetic findings from the International Ovarian and Testicular Stromal Tumor Registry.
Schultz KAP, Harris AK, Finch M, Dehner LP, Brown JB, Gershenson DM, Young RH, Field A, Yu W, Turner J, Cost NG, Schneider DT, Stewart DR, Frazier AL, Messinger Y, Hill DA
Gynecol Oncol 2017 Dec;147(3):521-527. Epub 2017 Oct 14 doi: 10.1016/j.ygyno.2017.09.034. PMID: 29037807Free PMC Article
Cytoreductive surgery (CRS) and hyperthermic intraperitoneal chemotherapy (HIPEC) in pediatric ovarian tumors: a novel treatment approach.
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

Stage I juvenile granulosa cell tumors of the ovary: A multicentre analysis from the MITO-9 study.
Bergamini A, Ferrandina G, Candotti G, Taccagni G, Scarfone G, Bocciolone L, Cassani C, Marinaccio M, Pignata S, Candiani M, Mangili G
Eur J Surg Oncol 2021 Jul;47(7):1705-1709. Epub 2021 Feb 9 doi: 10.1016/j.ejso.2021.02.003. PMID: 33583630
Ovarian tumors: a survey of selected advances of note during the life of this journal.
Young RH
Hum Pathol 2020 Jan;95:169-206. Epub 2019 Oct 22 doi: 10.1016/j.humpath.2019.09.002. PMID: 31654691
Ovarian sex cord-stromal tumours and their mimics.
Young RH
Pathology 2018 Jan;50(1):5-15. Epub 2017 Nov 11 doi: 10.1016/j.pathol.2017.09.007. PMID: 29132723
Ovarian sex-cord stromal tumours and small cell tumours: Pathological, genetic and management aspects.
Boussios S, Moschetta M, Zarkavelis G, Papadaki A, Kefas A, Tatsi K
Crit Rev Oncol Hematol 2017 Dec;120:43-51. Epub 2017 Oct 16 doi: 10.1016/j.critrevonc.2017.10.007. PMID: 29198337
Adult-type granulosa cell tumor of the testis: report of a case and review of literature.
Elbachiri M, Taleb A, Derrabi N, Bouchbika Z, Benchakroun N, Jouhadi H, Tawfiq N, Sahraoui S, Benider A
Pan Afr Med J 2017;26:198. Epub 2017 Apr 4 doi: 10.11604/pamj.2017.26.198.11523. PMID: 28674591Free PMC Article

Diagnosis

Stage I juvenile granulosa cell tumors of the ovary: A multicentre analysis from the MITO-9 study.
Bergamini A, Ferrandina G, Candotti G, Taccagni G, Scarfone G, Bocciolone L, Cassani C, Marinaccio M, Pignata S, Candiani M, Mangili G
Eur J Surg Oncol 2021 Jul;47(7):1705-1709. Epub 2021 Feb 9 doi: 10.1016/j.ejso.2021.02.003. PMID: 33583630
Gynecological neoplasms associated with paraneoplastic hypercalcemia.
Lim D, Oliva E
Semin Diagn Pathol 2019 Jul;36(4):246-259. Epub 2019 Feb 1 doi: 10.1053/j.semdp.2019.01.003. PMID: 30772079
Paediatric ovarian tumours and their associated cancer susceptibility syndromes.
Goudie C, Witkowski L, Vairy S, McCluggage WG, Foulkes WD
J Med Genet 2018 Jan;55(1):1-10. Epub 2017 Nov 24 doi: 10.1136/jmedgenet-2017-104926. PMID: 29175835
Ovarian sex cord-stromal tumours and their mimics.
Young RH
Pathology 2018 Jan;50(1):5-15. Epub 2017 Nov 11 doi: 10.1016/j.pathol.2017.09.007. PMID: 29132723
Ovarian sex-cord stromal tumours and small cell tumours: Pathological, genetic and management aspects.
Boussios S, Moschetta M, Zarkavelis G, Papadaki A, Kefas A, Tatsi K
Crit Rev Oncol Hematol 2017 Dec;120:43-51. Epub 2017 Oct 16 doi: 10.1016/j.critrevonc.2017.10.007. PMID: 29198337

Therapy

The clinical efficacy and safety of single-agent pembrolizumab in patients with recurrent granulosa cell tumors of the ovary: a case series from a phase II basket trial.
How JA, Jazaeri A, Westin SN, Sood AK, Ramondetta LM, Xu M, Abonofal A, Karp DD, Subbiah V, Stephen B, Rodon JA, Yang F, Naing A
Invest New Drugs 2021 Jun;39(3):829-835. Epub 2021 Jan 7 doi: 10.1007/s10637-020-01043-9. PMID: 33415580Free PMC Article
Adult-type granulosa cell tumor of the testis: report of a case and review of literature.
Elbachiri M, Taleb A, Derrabi N, Bouchbika Z, Benchakroun N, Jouhadi H, Tawfiq N, Sahraoui S, Benider A
Pan Afr Med J 2017;26:198. Epub 2017 Apr 4 doi: 10.11604/pamj.2017.26.198.11523. PMID: 28674591Free PMC Article
Cytoreductive surgery (CRS) and hyperthermic intraperitoneal chemotherapy (HIPEC) in pediatric ovarian tumors: a novel treatment approach.
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

Prognosis

ERβ in Granulosa Cell Tumors and Its Clinical Potential.
Birgersson M, Indukuri R, Antonson P, Nalvarte I, Archer A, Williams C
Endocrinology 2023 Apr 17;164(6) doi: 10.1210/endocr/bqad063. PMID: 37075218Free PMC Article
Stage I juvenile granulosa cell tumors of the ovary: A multicentre analysis from the MITO-9 study.
Bergamini A, Ferrandina G, Candotti G, Taccagni G, Scarfone G, Bocciolone L, Cassani C, Marinaccio M, Pignata S, Candiani M, Mangili G
Eur J Surg Oncol 2021 Jul;47(7):1705-1709. Epub 2021 Feb 9 doi: 10.1016/j.ejso.2021.02.003. PMID: 33583630
Ovarian Granulosa Cell Tumor: A Clinicoradiologic Series with Literature Review.
Kilinc YB, Sari L, Toprak H, Gultekin MA, Karabulut UE, Sahin N
Curr Med Imaging 2021;17(6):790-797. doi: 10.2174/1573405616666201228153755. PMID: 33371855
Ovarian sex-cord stromal tumours and small cell tumours: Pathological, genetic and management aspects.
Boussios S, Moschetta M, Zarkavelis G, Papadaki A, Kefas A, Tatsi K
Crit Rev Oncol Hematol 2017 Dec;120:43-51. Epub 2017 Oct 16 doi: 10.1016/j.critrevonc.2017.10.007. PMID: 29198337
Adult-type granulosa cell tumor of the testis: report of a case and review of literature.
Elbachiri M, Taleb A, Derrabi N, Bouchbika Z, Benchakroun N, Jouhadi H, Tawfiq N, Sahraoui S, Benider A
Pan Afr Med J 2017;26:198. Epub 2017 Apr 4 doi: 10.11604/pamj.2017.26.198.11523. PMID: 28674591Free PMC Article

Clinical prediction guides

Ovarian Granulosa Cell Tumor: A Clinicoradiologic Series with Literature Review.
Kilinc YB, Sari L, Toprak H, Gultekin MA, Karabulut UE, Sahin N
Curr Med Imaging 2021;17(6):790-797. doi: 10.2174/1573405616666201228153755. PMID: 33371855
Ovarian tumors: a survey of selected advances of note during the life of this journal.
Young RH
Hum Pathol 2020 Jan;95:169-206. Epub 2019 Oct 22 doi: 10.1016/j.humpath.2019.09.002. PMID: 31654691
Ovarian Sex Cord-Stromal Tumors: Reflections on a 40-Year Experience With a Fascinating Group of Tumors, Including Comments on the Seminal Observations of Robert E. Scully, MD.
Young RH
Arch Pathol Lab Med 2018 Dec;142(12):1459-1484. doi: 10.5858/arpa.2018-0291-RA. PMID: 30500284
Granulosa cell tumors of the ovary: a clinicopathologic and immunohistochemical study of 21 cases.
Kondi-Pafiti A, Grapsa D, Kairi-Vassilatou E, Carvounis E, Hasiakos D, Kontogianni K, Fotiou S
Eur J Gynaecol Oncol 2010;31(1):94-8. PMID: 20349790
Ovarian granulosa cell tumors frequently express EGFR (Her-1), Her-3, and Her-4: An immunohistochemical study.
Leibl S, Bodo K, Gogg-Kammerer M, Hrzenjak A, Petru E, Winter R, Denk H, Moinfar F
Gynecol Oncol 2006 Apr;101(1):18-23. Epub 2005 Dec 5 doi: 10.1016/j.ygyno.2005.10.009. PMID: 16330088

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