Ahmed Atef1, Khaled Gaballa1, Mohammad Zuhdy2, Khalid Atallah1, Wagdi Elkashef3, Shadi Awny1, Basma Gadelhak4, Basel Refky1. 1. Surgical Oncology Unit, Oncology Center, Mansoura University, 70 Gomhoriya Street, Mansoura, 35516, Egypt. 2. Surgical Oncology Unit, Oncology Center, Mansoura University, 70 Gomhoriya Street, Mansoura, 35516, Egypt. mzuhdy@mans.edu.eg. 3. Pathology department, Faculty of medicine Mansoura University, Mansoura, Egypt. 4. Radiology department, Mansoura University hospitals, Mansoura, Egypt.
Abstract
BACKGROUND: Desmoplastic small-round-cell tumor (DSRCT) is an extremely rare and highly aggressive malignancy. It is of yet unclear origin, but it is assumed to be of a mesothelial origin based on its tendency for widespread metastasis in serosal linings. CASE PRESENTATION: In this report, we describe a young female who presented with bilateral ovarian masses that mimicked the classic clinical picture of ovarian cancer. The patient had a cytoreductive surgery done in the form of total abdominal hysterectomy, bilateral salpingo-oophorectomy, omentectomy, pelvic peritonectomy, low para-aortic and bilateral iliac lymphadenectomy. Postoperative course was smooth with no adverse events. The final pathology report revealed desmoplastic small-round-cell tumor. Afterwards, the patient was referred to medical oncologist to receive her adjuvant therapy. CONCLUSIONS: DSRCT is still an unknown disease to us given the limited number of cases and poor survival. Given the lack of clear guidelines, treatment is offered based on the best available evidence and the collaborative effort of a multi-disciplinary team.
BACKGROUND:Desmoplastic small-round-cell tumor (DSRCT) is an extremely rare and highly aggressive malignancy. It is of yet unclear origin, but it is assumed to be of a mesothelial origin based on its tendency for widespread metastasis in serosal linings. CASE PRESENTATION: In this report, we describe a young female who presented with bilateral ovarian masses that mimicked the classic clinical picture of ovarian cancer. The patient had a cytoreductive surgery done in the form of total abdominal hysterectomy, bilateral salpingo-oophorectomy, omentectomy, pelvic peritonectomy, low para-aortic and bilateral iliac lymphadenectomy. Postoperative course was smooth with no adverse events. The final pathology report revealed desmoplastic small-round-cell tumor. Afterwards, the patient was referred to medical oncologist to receive her adjuvant therapy. CONCLUSIONS: DSRCT is still an unknown disease to us given the limited number of cases and poor survival. Given the lack of clear guidelines, treatment is offered based on the best available evidence and the collaborative effort of a multi-disciplinary team.
Entities:
Keywords:
Case report; Desmoplastic small-round-cell tumor; Metastasis; Neoplasm; Ovarian cancer