Malignant Brenner tumors (MBT) of the ovary are rare diseases, representing 1% of all ovarian cancers and 3-5% of Brenner tumors. They carry a poor prognosis. They generally affect women during the perimenopausal and postmenopausal periods. The standard treatment is surgery; however, the indication of adjuvant chemotherapy remains controversial. The present study aims to report our experience in the treatment of MBT of the ovary, to better characterize this disease. Methods: In this study, a retrospective case series involving four patients diagnosed with MBT of the ovary and treated between 2006 and 2014. Results: Four cases of MBT of the ovary were diagnosed over a seven-year period. The mean age of our patients was 59.3±11.1 years. Three patients were in the menopause period. The tumor was staged as IC in one case, IIC in one case, and IIIC in two cases of the International Federation of Gynecology and Obstetrics classification. All patients underwent surgery, followed by adjuvant chemotherapy. Three patients underwent a loco-regional recurrence that occurred respectively, after nine months in one patient and 11 months in two patients. The treatment was based on chemotherapy combined with surgery in one case. Two patients presented distant metastasis. The treatment consisted of chemotherapy and surgery. The median follows up period was 49.0 (14.0-64.0) months. Conclusion: The treatment approach of MBT of the ovary is not well established since its scarcity and poor prognosis. Thus, more case series and meta-analysis should be conducted.
Dermatofibrosarcoma protuberans (DFSP) is a rare sarcoma of soft tissue representing about 1% of all tumors. In addition, DFSP occurs commonly on the trunk and extremities, and only a few cases of DFSP have been observed on the breast. In men, only 11 cases, including this case, have been reported. In this article, we present a case of left breast DFSP that occurred in a 44-year-old man. The physical examination revealed a left breast tender mass, which invaded the skin. The tumor was staged as T4b N0 M0. Mammography and sonography showed a suspect mass of the left breast. The biopsy and immunochemistry permitted the diagnosis of DFSP of the left breast. The patient had a left mammectomy, with free margins. He presents no evident sign of recurrence 7 months later.
Introduction
Desmoplastic small round cell tumor is a rare malignancy with poor prognosis, affecting young male patients. It frequently presents as a large abdominal mass with widespread peritoneal involvement at diagnosis. In late stages, metastases may be present.
Aim
We retrospectively reviewed patient characteristics, presenting symptoms, tumor pathology, treatment, and outcome of four patients with desmoplastic small round cell tumor at our institution.
Cases presentation
The first three cases reported are 32-, 17-, and 30-year-old North African males with intraabdominal desmoplastic small round cell tumor treated by surgery, chemotherapy, and radiation therapy with different follow-ups. The final case is a 16-year-old North African male with ganglionic desmoplastic small round cell tumor but no evidence of a tissue mass. He underwent two lines of chemotherapy with no response. The patient was lost after 2 years of follow-up. In all cases, desmoplastic small round cell tumor was confirmed by presence of t(11,22) (p13,q12) translocation.
Conclusion
Treatment of desmoplastic small round cell tumor is based on multidisciplinary therapy. Despite high-dose chemotherapy, extensive surgical resection, and radiotherapy, desmoplastic small round cell tumor remains lethal.
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