Sexcord/ stromal ovarian tumors pathophysiology

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief:  ; Maneesha Nandimandalam, M.B.B.S.[2]

Overview

The exact pathogenesis of sexcord/ stromal ovarian tumors is not fully understood. Mutations mainly involving FOXL2, DICER1, STK11 are involved. They are associated with ollier disease and maffucci syndrome.The microscopic pathology varies with the individual subtype of sexcord stromal ovarian tumors.

Pathophysiology

Pathogenesis

Genetics

FOXL2:[2][3][4][5][6][7][8]

Schematic representation of the cell signaling pathways in GCT development. PI3K, phosphatidylinositol-3-kinase; AKT, serine/threonine kinase; FOXO 1/3, forkhead box O1/3; AMH, Source:Li J, Bao R, Peng S, Zhang C. The molecular mechanism of ovarian granulosa cell tumors. J Ovarian Res. 2018;11(1):13. Published 2018 Feb 6. doi:10.1186/s13048-018-0384-1

DICER1:[3][9][10][11][2][12]

STK11:

Associated Conditions

Gross and Microscopic Pathology

The gross and microscopic features of the most common tumors are described below:[6][13][14][15][16][17][18][7][19][20][21]

Types Gross pathology Microscopic pathology Images
Adult granulosa cell tumours
Juvenile granulosa cell tumours
Microcystic spaces, cuboidal-to-polygonal cells in sheets or stands or cords, with moderate-to-marked nuclear atypia, and basophilic cytoplasm https://librepathology.org/wiki/File:Juvenile_granulosa_cell_tumour_-_very_high_mag.jpg
Sex cord tumour with annular tubules
  • They are usually solid and yellow
well-circumscribed nests of cells with nuclei at the periphery, annular tubules (ring-shaped tubules) with dense hyaline material,https://librepathology.org/wiki/Sex_cord_tumour_with_annular_tubules
Sertolileydig cell tumors
  • Retiform tumors have a spongy sectioned surface
Sclerosing stromal tumor
  • A pseudolobular pattern, admixed spindled and rounded weakly luteinised cells,
Pseudolobular pattern showing hypo and hypercellular areas seperated by oedematous fibrous strands://openi.nlm.nih.gov/detailedresult?img=PMC4175603_ogs-57-405-g001&query=sclerosing%20stromal%20tumour%20of%20ovary&it=xg&req=4&npos=5
Luteinised thecomas with sclerosing peritonitis
  • They are bilateral with abnormal in appearance due to a hypercerebriform contour
Microcystic stromal tumor
Fibroma
Thecoma
  • They appear yellow on solid sectioned surface
Bland oval or spindled nuclei, abundant cytoplasm that is pale and vaculolated,https://librepathology.org/wiki/Thecoma
Fibrosarcoma

Immunohistochemistry

Adult granulosa cell tumors stain positive for:[22][23][24][25][26][27][28]

They are negative for:

Sertoli–stromal cell tumors are positive for:

Microcystic stromal tumor:[19][20][29]

References

  1. Karnezis AN, Cho KR, Gilks CB, Pearce CL, Huntsman DG (January 2017). "The disparate origins of ovarian cancers: pathogenesis and prevention strategies". Nat. Rev. Cancer. 17 (1): 65–74. doi:10.1038/nrc.2016.113. PMID 27885265.
  2. 2.0 2.1 Lim, Diana; Oliva, Esther (2018). "Ovarian sex cord-stromal tumours: an update in recent molecular advances". Pathology. 50 (2): 178–189. doi:10.1016/j.pathol.2017.10.008. ISSN 0031-3025.
  3. 3.0 3.1 Fuller PJ, Leung D, Chu S (February 2017). "Genetics and genomics of ovarian sex cord-stromal tumors". Clin. Genet. 91 (2): 285–291. doi:10.1111/cge.12917. PMID 27813081.
  4. Li, Jiaheng; Bao, Riqiang; Peng, Shiwei; Zhang, Chunping (2018). "The molecular mechanism of ovarian granulosa cell tumors". Journal of Ovarian Research. 11 (1). doi:10.1186/s13048-018-0384-1. ISSN 1757-2215.
  5. Li J, Bao R, Peng S, Zhang C (February 2018). "The molecular mechanism of ovarian granulosa cell tumors". J Ovarian Res. 11 (1): 13. doi:10.1186/s13048-018-0384-1. PMC 5802052. PMID 29409506.
  6. 6.0 6.1 6.2 Schultz KA, Harris AK, Schneider DT, Young RH, Brown J, Gershenson DM, Dehner LP, Hill DA, Messinger YH, Frazier AL (October 2016). "Ovarian Sex Cord-Stromal Tumors". J Oncol Pract. 12 (10): 940–946. doi:10.1200/JOP.2016.016261. PMC 5063189. PMID 27858560.
  7. 7.0 7.1 Boussios, Stergios; Moschetta, Michele; Zarkavelis, George; Papadaki, Alexandra; Kefas, Aristides; Tatsi, Konstantina (2017). "Ovarian sex-cord stromal tumours and small cell tumours: Pathological, genetic and management aspects". Critical Reviews in Oncology/Hematology. 120: 43–51. doi:10.1016/j.critrevonc.2017.10.007. ISSN 1040-8428.
  8. Leung, Dilys T.H.; Fuller, Peter J.; Chu, Simon (2016). "Impact of FOXL2 mutations on signaling in ovarian granulosa cell tumors". The International Journal of Biochemistry & Cell Biology. 72: 51–54. doi:10.1016/j.biocel.2016.01.003. ISSN 1357-2725.
  9. Goulvent T, Ray-Coquard I, Borel S, Haddad V, Devouassoux-Shisheboran M, Vacher-Lavenu MC, Pujade-Laurraine E, Savina A, Maillet D, Gillet G, Treilleux I, Rimokh R (January 2016). "DICER1 and FOXL2 mutations in ovarian sex cord-stromal tumours: a GINECO Group study". Histopathology. 68 (2): 279–85. doi:10.1111/his.12747. PMID 26033501.
  10. 10.0 10.1 Stewart CJ, Charles A, Foulkes WD (June 2016). "Gynecologic Manifestations of the DICER1 Syndrome". Surg Pathol Clin. 9 (2): 227–41. doi:10.1016/j.path.2016.01.002. PMID 27241106.
  11. Wang Y, Karnezis AN, Magrill J, Tessier-Cloutier B, Lum A, Senz J, Gilks CB, McCluggage WG, Huntsman DG, Kommoss F (August 2018). "DICER1 hot-spot mutations in ovarian gynandroblastoma". Histopathology. 73 (2): 306–313. doi:10.1111/his.13630. PMID 29660837.
  12. Xu Q, Zou Y, Zhang XF (October 2018). "Sertoli-Leydig cell tumors of ovary: A case series". Medicine (Baltimore). 97 (42): e12865. doi:10.1097/MD.0000000000012865. PMC 6211859. PMID 30334998.
  13. Bremmer F, Schweyer S (February 2016). "[Leydig cell, Sertoli cell and adult granulosa cell tumors]". Pathologe (in German). 37 (1): 71–7. doi:10.1007/s00292-015-0131-y. PMID 26782032.
  14. Bremmer F, Behnes CL, Radzun HJ, Bettstetter M, Schweyer S (May 2014). "[Sex cord gonadal stromal tumors]". Pathologe (in German). 35 (3): 245–51. doi:10.1007/s00292-014-1901-7. PMID 24819979.
  15. Roth LM, Czernobilsky B (March 2011). "Perspectives on pure ovarian stromal neoplasms and tumor-like proliferations of the ovarian stroma". Am. J. Surg. Pathol. 35 (3): e15–33. doi:10.1097/PAS.0b013e31820acb89. PMID 21317704.
  16. Young RH (January 2018). "Ovarian sex cord-stromal tumours and their mimics". Pathology. 50 (1): 5–15. doi:10.1016/j.pathol.2017.09.007. PMID 29132723.
  17. Zhang HY, Zhu JE, Huang W, Zhu J (2014). "Clinicopathologic features of ovarian Sertoli-Leydig cell tumors". Int J Clin Exp Pathol. 7 (10): 6956–64. PMC 4230071. PMID 25400781.
  18. Chen, Vivien W.; Ruiz, Bernardo; Killeen, Jeffrey L.; Cot�, Timothy R.; Wu, Xiao Cheng; Correa, Catherine N.; Howe, Holly L. (2003). "Pathology and classification of ovarian tumors". Cancer. 97 (S10): 2631–2642. doi:10.1002/cncr.11345. ISSN 0008-543X. replacement character in |last4= at position 4 (help)
  19. 19.0 19.1 Irving JA, Lee CH, Yip S, Oliva E, McCluggage WG, Young RH (October 2015). "Microcystic Stromal Tumor: A Distinctive Ovarian Sex Cord-Stromal Neoplasm Characterized by FOXL2, SF-1, WT-1, Cyclin D1, and β-catenin Nuclear Expression and CTNNB1 Mutations". Am. J. Surg. Pathol. 39 (10): 1420–6. doi:10.1097/PAS.0000000000000482. PMID 26200099.
  20. 20.0 20.1 Irving JA, Young RH (March 2009). "Microcystic stromal tumor of the ovary: report of 16 cases of a hitherto uncharacterized distinctive ovarian neoplasm". Am. J. Surg. Pathol. 33 (3): 367–75. doi:10.1097/PAS.0b013e31818479c3. PMID 18971779.
  21. Mathur A, Seth A, Pant L (2018). "Ovarian fibroma with luteinized thecal cells and minor sex cord element: A rare case report". Indian J Pathol Microbiol. 61 (2): 264–267. doi:10.4103/IJPM.IJPM_446_17. PMID 29676374.
  22. Kaspar, Hanna G.; Crum, Christopher P. (2015). "The Utility of Immunohistochemistry in the Differential Diagnosis of Gynecologic Disorders". Archives of Pathology & Laboratory Medicine. 139 (1): 39–54. doi:10.5858/arpa.2014-0057-RA. ISSN 0003-9985.
  23. Zhao C, Vinh TN, McManus K, Dabbs D, Barner R, Vang R (March 2009). "Identification of the most sensitive and robust immunohistochemical markers in different categories of ovarian sex cord-stromal tumors". Am. J. Surg. Pathol. 33 (3): 354–66. doi:10.1097/PAS.0b013e318188373d. PMID 19033865.
  24. McCluggage WG, McKenna M, McBride HA (July 2007). "CD56 is a sensitive and diagnostically useful immunohistochemical marker of ovarian sex cord-stromal tumors". Int. J. Gynecol. Pathol. 26 (3): 322–7. doi:10.1097/01.pgp.0000236947.59463.87. PMID 17581419.
  25. Zhao C, Bratthauer GL, Barner R, Vang R (September 2007). "Diagnostic utility of WT1 immunostaining in ovarian sertoli cell tumor". Am. J. Surg. Pathol. 31 (9): 1378–86. doi:10.1097/PAS.0b013e3180339961. PMID 17721194.
  26. Zhao C, Barner R, Vinh TN, McManus K, Dabbs D, Vang R (October 2008). "SF-1 is a diagnostically useful immunohistochemical marker and comparable to other sex cord-stromal tumor markers for the differential diagnosis of ovarian sertoli cell tumor". Int. J. Gynecol. Pathol. 27 (4): 507–14. doi:10.1097/PGP.0b013e31817c1b0a. PMID 18753972.
  27. Deavers MT, Malpica A, Liu J, Broaddus R, Silva EG (June 2003). "Ovarian sex cord-stromal tumors: an immunohistochemical study including a comparison of calretinin and inhibin". Mod. Pathol. 16 (6): 584–90. doi:10.1097/01.MP.0000073133.79591.A1. PMID 12808064.
  28. Oliva E, Garcia-Miralles N, Vu Q, Young RH (October 2007). "CD10 expression in pure stromal and sex cord-stromal tumors of the ovary: an immunohistochemical analysis of 101 cases". Int. J. Gynecol. Pathol. 26 (4): 359–67. doi:10.1097/PGP.0b013e318064511c. PMID 17885484.
  29. Bi R, Bai QM, Yang F, Wu LJ, Cheng YF, Shen XX, Cai X, Zhou XY, Yang WT (December 2015). "Microcystic stromal tumour of the ovary: frequent mutations of β-catenin (CTNNB1) in six cases". Histopathology. 67 (6): 872–9. doi:10.1111/his.12722. PMID 25913412.

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