Gynandroblastoma With Juvenile Granulosa Cell Tumor and Concurrent Renal Cell Carcinoma

Gynandroblastoma is an extremely rare primary tumor of the ovary showing morphological evidence of both female (granulosa cell tumor) and male (Sertoli or Sertoli–Leydig tumor) differentiation. We report an unusual case of a 32-year-old female who presented with hyperandrogenism and was found on imaging to have concurrent ovarian and renal masses. Following surgical excision, the ovarian mass was diagnosed as gynandroblastoma, which consisted of 45% juvenile granulosa cell tumor and 55% intermediately differentiated Sertoli–Leydig tumor. The renal mass was diagnosed as a conventional renal clear cell carcinoma. Gynandroblastoma, especially with juvenile granulosa cell tumor, is an extremely rare ovarian tumor. Concurrent gynandroblastoma with another malignant neoplasm has not been reported in the literature.

[1]  R. Veitia,et al.  FOXL2, GATA4, and SMAD3 Co-Operatively Modulate Gene Expression, Cell Viability and Apoptosis in Ovarian Granulosa Cell Tumor Cells , 2014, PloS one.

[2]  Bo Eun Lee,et al.  Association of polymorphisms in microRNA machinery genes (DROSHA, DICER1, RAN, and XPO5) with risk of idiopathic primary ovarian insufficiency in Korean women , 2013, Menopause.

[3]  G. Kenter,et al.  The Incidence of Endometrial Hyperplasia and Cancer in 1031 Patients With a Granulosa Cell Tumor of the Ovary: Long-Term Follow-Up in a Population-Based Cohort Study , 2013, International Journal of Gynecologic Cancer.

[4]  Yingmei Wang,et al.  Androgen Insensitivity Syndrome with Gynandroblastoma and Vulvar Leiomyoma: Case Report and Literature Review , 2013, Journal of lower genital tract disease.

[5]  W. Foulkes,et al.  Biallelic DICER1 mutations occur in Wilms tumours , 2013, The Journal of pathology.

[6]  B. Benayoun,et al.  Adult ovarian granulosa cell tumor transcriptomics: prevalence of FOXL2 target genes misregulation gives insights into the pathogenic mechanism of the p.Cys134Trp somatic mutation , 2013, Oncogene.

[7]  Bin Yang,et al.  14-3-3 Sigma is a Useful Immunohistochemical Marker for Diagnosing Ovarian Granulosa Cell Tumors and Steroid Cell Tumors , 2013, International journal of gynecological pathology : official journal of the International Society of Gynecological Pathologists.

[8]  A. Stenhouse,et al.  The C134W (402 C>G) FOXL2 mutation is absent in ovarian gynandroblastoma: insights into the genesis of an unusual tumour , 2012, Histopathology.

[9]  Steven J. M. Jones,et al.  Recurrent somatic DICER1 mutations in nonepithelial ovarian cancers. , 2012, The New England journal of medicine.

[10]  W. Foulkes,et al.  Extending the phenotypes associated with DICER1 mutations , 2011, Human mutation.

[11]  Gretchen M. Williams,et al.  Ovarian sex cord-stromal tumors, pleuropulmonary blastoma and DICER1 mutations: a report from the International Pleuropulmonary Blastoma Registry. , 2011, Gynecologic oncology.

[12]  Kangseok Lee,et al.  Differential apoptotic activities of wild-type FOXL2 and the adult-type granulosa cell tumor-associated mutant FOXL2 (C134W) , 2011, Oncogene.

[13]  A. Nicholson,et al.  DICER1 syndrome: clarifying the diagnosis, clinical features and management implications of a pleiotropic tumour predisposition syndrome , 2011, Journal of Medical Genetics.

[14]  W. Foulkes,et al.  DICER1 mutations in familial multinodular goiter with and without ovarian Sertoli-Leydig cell tumors. , 2011, JAMA.

[15]  M. Xue,et al.  Gynandroblastoma with the symptoms of infertility and secondary amenorrhea: a case report. , 2011, Clinical and experimental obstetrics & gynecology.

[16]  B. Benayoun,et al.  Functional Exploration of the Adult Ovarian Granulosa Cell Tumor-Associated Somatic FOXL2 Mutation p.Cys134Trp (c.402C>G) , 2010, PloS one.

[17]  J. Kelley,et al.  Recurrent gynandroblastoma of ovary-A case report: a molecular and immunohistochemical analysis. , 2007, International journal of gynecological pathology : official journal of the International Society of Gynecological Pathologists.

[18]  P. Russell,et al.  Ovarian gynandroblastoma with juvenile granulosa cell component and raised alpha fetoprotein , 2005, Pathology.

[19]  A. Talerman Gynandroblastoma with elements of juvenile granulosa cell tumor. , 1998, International journal of gynecological pathology : official journal of the International Society of Gynecological Pathologists.

[20]  L. Roth,et al.  Gynandroblastoma with elements resembling juvenile granulosa cell tumor. , 1997, International journal of gynecological pathology : official journal of the International Society of Gynecological Pathologists.

[21]  J. Sloan,et al.  Gynandroblastoma of ovary with juvenile granulosa cell component and heterologous intestinal type glands , 1996, Histopathology.