Primary desmoplastic small-round-cell tumor of the ovary.
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.
['Atef|Ahmed|A|', 'Gaballa|Khaled|K|', 'Zuhdy|Mohammad|M|https://orcid.org/0000-0001-6841-1218', 'Atallah|Khalid|K|', 'Elkashef|Wagdi|W|', 'Awny|Shadi|S|', 'Gadelhak|Basma|B|', 'Refky|Basel|B|']
D003131:Combined Modality Therapy D065426:Cytoreduction Surgical Procedures D058405:Desmoplastic Small Round Cell Tumor D005260:Female D006801:Humans D010051:Ovarian Neoplasms D016896:Treatment Outcome D055815:Young Adult
Desmoplastic small-round-cell tumor Metastasis Ovarian cancer Case report Neoplasm