Journal of

Case Reports and Images in Oncology

 
     
Case Report
 
Recurrence of sex cord tumor with annular tubules in young patient with Peutz-Jeghers syndrome
Meher Slimane1, Selma Gadria1, Manel Hadidane1, Houyem Mansouri1, Maha Driss2, Khaled Rahal1
1Department of Surgical Oncology, Salah Azaiz Institut, Tunis, Tunisia.
2Pathology Departement, Salah Azaiz Institut, Tunis, Tunisia.

Article ID: 100027Z10MS2016
doi:10.5348/Z10-2016-27-CR-18

Address correspondence to:
Selma Gadria
Surgical Oncology departement
Salah Azaiz Institute
Postal address: C3 residence Cordoba, Rue Tachkand, 2037, Ennasr1, Ariana
Tunisia

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How to cite this article:
Slimane M, Gadria S, Hadidane M, Mansouri H, Driss M, Rahal K. Recurrence of sex cord tumor with annular tubules in young patient with Peutz-Jeghers syndrome. J Case Rep Images Oncology 2016;2:74–78.


Abstract
Introduction: Peutz-Jeghers syndrome (PJS) is a hereditary autosomal dominant disease characterized by polyposis in the gastro-intestinal tract and melanin pigmented macules on the skin mucosa. In gynecology, it can be complicated with sex cord tumor with annular tubules (SCTAT). Multifocal, bilateral, small and benign lesions that develop into mucinous and serous ovarian tumors commonly characterize it. The SCTATs associated with PJS are usually benign and do not have malignant potentials, thus, unilateral oophorectomy is curative. Recurrence is a common event. Subsequently, radical surgery is recommended in these cases.
Case Report: We report the case of a 32 years PJS patient, who underwent conservative surgery for SCTAT five years ago. She shows up with a recurrent contralateral 75 mm adnexal cyst. A conservative surgery was planned and the patient underwent a laparoscopic cystectomy because of her will of childbearing. One month after surgery, hormonal rates were measured after ovarian stimulation and did not show hormonal activity.
Conclusion: SCTAT associated to PJS is a rare and unusual variant of ovarian tumors. Conservative surgery is indicated but recurrence is still a common event. Further studies are needed to confirm the effect of radical surgery and adjuvant therapies because of its rarity and high recurrence rates.

Keywords: Hereditary disease, Ovarian tumors, Peutz-Jeghers syndrome, Sex cord tumors


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Author Contributions
Meher Slimane – Substantial contributions to conception and design, Acquisition of data, Analysis and interpretation of data, Drafting the article, Revising it critically for important intellectual content, Final approval of the version to be published
Selma Gadria – Analysis and interpretation of data, Revising it critically for important intellectual content, Final approval of the version to be published
Manel Hadidane – Analysis and interpretation of data, Revising it critically for important intellectual content, Final approval of the version to be published
Houyem Mansouri – Analysis and interpretation of data, Revising it critically for important intellectual content, Final approval of the version to be published
Maha Driss – Analysis and interpretation of data, Revising it critically for important intellectual content, Final approval of the version to be published
Khaled Rahal – Analysis and interpretation of data, Revising it critically for important intellectual content, Final approval of the version to be published
Guarantor of submission
The corresponding author is the guarantor of submission.
Source of support
None
Conflict of interest
Authors declare no conflict of interest.
Copyright
© 2016 Meher Slimane et al. This article is distributed under the terms of Creative Commons Attribution License which permits unrestricted use, distribution and reproduction in any medium provided the original author(s) and original publisher are properly credited. Please see the copyright policy on the journal website for more information.



About The Authors

Meher Slimane is Assistant in Department of Surgical Oncology at Salah Azaiz Institut.



Selma Gadria is Resident in Department of Surgical Oncology at Salah Azaiz Institut.



Manel Hadidane is Resident in Department of Surgical Oncology at Salah Azaiz Institut.



Houyem Mansouri is Resident in Department of Surgical Oncology at Salah Azaiz Institut.



Maha Driss is Assistant in the Pathology Department at Salah Azaiz Institut.



Khled Rahal is the Head of Department of Surgical Oncology at Salah Azaiz Institut.




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