Ovarian sex cord tumor with annular tubules: case report and review of the literature
DOI:
https://doi.org/10.18597/rcog.3888Keywords:
Gonadal sex cord and stromal tumors, ovarian cancer, Peutz-Jeghers syndromeAbstract
Objectives: To report a case of ovarian sex cord tumor with annular tubules (SCTAT) and conduct a literature review on diagnosis, treatment and prognosis of this condition.
Material and methods: Case report of a woman with a final diagnosis of advanced SCTAT seen at the National Cancer Institute in Bogota (Colombia) who received surgical treatment and chemotherapy with a satisfactory course after 6 months. A literature search was conducted in the Medline via PubMed, LILACS and Scielo databases, including case reports and series of women diagnosed with SCTAT published since 1990, not using age ranges. Information about diagnosis, treatment and reported prognosis was retrieved. A narrative summary of the findings was prepared.
Results: Fourteen publications with 26 patients were included. Mean age at diagnosis was 22.5 years. The main symptoms were menstruation abnormalities and pelvic pain. Computed tomography (CT) was the imaging technology most frequently used. Surgical treatment was used in all cases, together with chemotherapy in 29 %; 2 patients received radiotherapy. Recurrence occurred in 20 % of cases. Mortality was 12.5 %, with all deaths occurring within the first year.
Conclusions: There is a paucity of information about the diagnostic utility of imaging, tumor markers and histochemical studies, as well as prognosis of this disease condition. Surgery is the treatment of choice, taking into consideration the patient’s wishes regarding fertility, as well as the stage of the tumor. Further studies are needed to provide more detailed information about this condition.
Author Biographies
Franco Rafael Ruiz-Echeverría, Instituto Nacional de Cancerología, Bogotá (Colombia).
Fellow de Ginecología Oncológica. Universidad Militar Nueva Granada, Bogotá (Colombia). Instituto Nacional de Cancerología, Bogotá (Colombia).
María Islena Beltrán-Salazar, Instituto Nacional de Cancerología, Bogotá (Colombia).
Instituto Nacional de Cancerología, Bogotá (Colombia).
Pedro Hernando Calderón-Quiroz, Instituto Nacional de Cancerología, Bogotá (Colombia).
Instituto Nacional de Cancerología, Bogotá (Colombia).
Juan David Lalinde-Triviño, Instituto Nacional de Cancerología, Bogotá (Colombia).
Fellow de Ginecología Oncológica. Universidad Militar Nueva Granada, Bogotá (Colombia). Instituto Nacional de Cancerología, Bogotá (Colombia).
Maribel Palencia-Palacios, Universidad del Cauca, Popayán (Colombia).
Residente de Anatomía Patológica. Universidad del Cauca, Popayán (Colombia).
Oscar Suescún-Garay, Universidad Militar Nueva Granada, Bogotá (Colombia).
Fellow de Ginecología Oncológica. Universidad Militar Nueva Granada, Bogotá (Colombia).
References
Young RH, Kiyokawa T, Stewart C. Sex cord tumour with annular tubules. WHO Classification of Tumours Editorial Board. Female Genital Tumours. 2020. p. 111–2.
Scully RE. Sex cord tumor with annular tubules a distinctive ovarian tumor of the Peutz-Jeghers syndrome. Cancer. 1970;25(5):1107–21. https://doi.org/c4tsmh
Young RH, Welch WR, Dickersin GR, Scully RE. Ovarian sex cord tumor with annular tubules. Review of 74 cases including 27 with Peutz-Jeghers syndrome and four with adenoma malignum of the cervix. Cancer. 1982;50(7):1384-402. https://doi.org/cgxtzx
Tacheci I, Kopacova M, Bures J. Peutz-Jeghers syndrome. Curr Opin Gas-troenterol.2021;37(3):245–54. https://doi.org/10.1097/MOG.0000000000000718
Hemminki A, Markie D, Tomlinson I, Avizienyte E, Roth S, Loukola A, et al. A serine/threonine kinase gene defective in Peutz-Jeghers syndrome. Nature. 1998;391(6663):184–7. https://doi.org/10.1038/34432
Scully RE. The prolonged gestation, birth, and early life of the sex cord tumor with annular tubules and how it joined a syndrome. Int J Surg Pathol. 2000;8(3):233–8. https://doi.org/10.1177/106689690000800312
Oliva E, García-Miralles N, Vu Q, Young RH. CD10 expression in pure stromal and sex cordstromal tumors of the ovary: an immunohistochemical analsis of 101 cases: An immunohistochemical analysis of 101 cases. Int J Gynecol Pathol. 2007;26(4):359–67. https://doi.org/10.1097/PGP.0b013e318064511c
Dolan J, AlTimimi AH, Richards SM, Jeffs JB, Mason GC, Smith DB, et al. Does ovarian sex cord tumour with annular tubules produce progesterone? J Clin Pathol. 1986;39(1):29–35. https://doi.org/10.1136/jcp.39.1.29
Sugarbaker PH. Peritoneal carcinomatosis: principles of management. Boston, MA: Kluwer Academic Publishers; 1996. https://doi.org/10.1007/978-1-4613-1247-5
Moon WK, Kim SH, Kim WS, Kim IO, Yeon KM, Han MC. Case report: ovarian sex cord tumour with annular tubules: imaging findings. Clin Radiol. 1995;50(8):581–2. https://doi.org/10.1016/S0009-9260(05)83200-3
Qian Q, You Y, Yang J, Cao D, Zhu Z, Wu M, et al. Management and prognosis of patients with ovarian sex cord tumor with annular tubules: a retrospective study. BMC Cancer. 2015;15(1):270. https://doi.org/10.1186/s12885-015-1277-y
Mayerson B, Cuello D, Brañes M, Leiva J, Castiblanco V. Tumor de los cordones sexuales con túbulos anulares del ovario no asociado a síndrome de Peutz-Jeghers. Rev chil Obstet ginecol. 2006; 71(1): 57-62. https://doi.org/10.4067/s0717-75262006000100010
Singh M, Mandal S, Majumdar K. Sex cord tumor with annular tubules: An incidental finding in an endometriotic cyst - The first known cooccurrence. Biomed Res Int. 2014; 2014:970243. https://doi.org/10.1155/2014/970243
Senn D, Videira H, Haagsma B, El-Bahrawy M, Madhuri TK. Sex cord tumour with annular tubulesan unusual case of abdominal pain. J Obstet Gynaecol Can. 2021; 43(3):361–4. https://doi.org/10.1016/j.jogc.2020.06.025
Yahaya JJ, Mshana D, Mremi A. Ovarian sex cord tumour with annular tubules in a 13-year old female: a case report. Oxf Med Case Reports. 2020; 2020(4):omaa024. https://doi.org/10.1093/omcr/omaa024
Chatziioannidou K, Botsikas D, Tille JC, Dubuisson J. Preservation of fertility in non Peutz- Jegher syndromeassociated ovarian sex cord tumour. BMJ Case Reports. 2015. https://doi.org/10.1136/bcr-2014-207841
Sánchez J, Ruiz J, Sánchez S. Causa muy rara de pubertad precoz periférica en una niña: tumor de cordones sexuales con túbulos anulares. Arch Argent Pediatr 2017; 115(3):e179-e182. https://doi.org/10.5546/aap.2017.e179
Hales SA, Cree IA, Pinion S. A poorly differentiated Sertoli-Leydig cell tumour associated with an ovarian sex cord tumour with annular tubules in a woman with Peutz-Jeghers syndrome. Histopathology. 1994; 25(4):391–3. https://doi.org/10.1111/j.1365-2559.1994.tb01361.x
López Corella E, Garza Elizondo R, Silva Martínez MT, González del Ángel A, Fuentes-Gutiérrez D. Tumor de cordón sexual con túbulos anulares y cistadenoma mucinoso de ovario, en una adolescente con Síndrome de Peutz Jeghers. Acta pediátr Méx. 2020; 41(4):178. https://doi.org/10.18233/apm41no4pp178-1862098
Ravishankar S, Mangray S, Kurkchubasche A, Yakirevich E, Young RH. Unusual Sertoli cell tumor associated with sex cord tumor with annular tubules in Peutz-Jeghers syndrome: Report of a case and review of the literature on ovarian tumors in Peutz-Jeghers syndrome: Report of a case and review of the literature on ovarian tumors in peutz-jeghers syndrome. Int J Surg Pathol. 2016; 24(3):269–73. https://doi.org/10.1177/1066896915620663
Gustafson ML, Lee MM, Scully RE, et al. Müllerian inhibiting substance as a marker for ovarian sexcord tumor. N Engl J Med. 1992; 326(7):466–71. https://doi.org/10.1056/nejm199202133260707
Puls LE, Hamous J, Morrow MS, Schneyer A, MacLaughlin DT, Castracane VD. Recurrent ovarian sex cord tumor with annular tubules: tumor marker and chemotherapy experience. Gynecol Oncol. 1994; 54(3):396–401. https://doi.org/10.1006/gyno.1994.1232
Nosov V, Park S, Rao J, Memarzadeh S. Non-Peutz-Jeghers syndrome associated ovarian sex cord tumor with annular tubules: a case report. Fertil Steril 2009;92(4):1497.e5-1497.e8. https://doi.org/10.1016/j.fertnstert.2009.07.1002
How to Cite
Downloads
Downloads
Published
Issue
Section
License
This work is licensed under a Creative Commons Attribution-NonCommercial-NoDerivatives 4.0 International License.
Article metrics | |
---|---|
Abstract views | |
Galley vies | |
PDF Views | |
HTML views | |
Other views |