A 23-Year-Old Jordanian Woman with a Desmoplastic Small Round Cell Tumor Involving the Ovary

Abstract

Patient: Female, 23Final Diagnosis: Desmoplastic small round cell tumorSymptoms: Nausea • vomiting • severe abdominal pain of two weeks • weight lossMedication: —Clinical Procedure: —Specialty: Obstetrics and GynecologyObjective:Rare diseaseBackground:Desmoplastic small round cell tumor (DSRCT) is a rare soft tissue sarcoma that usually arises in the abdomen or pelvis in young boys and adolescents. Presenting symptoms include abdominal pain and ascites. However, DSRCT is often disseminated throughout the peritoneal cavity at diagnosis, and the prognosis is poor. This report is of a case of DSRCT in a 23-year-old Jordanian woman who presented with abdominal pain.Case Report:An unmarried 23-year-old woman presented with abdominal pain. On examination, she was found to have ascites. A computed tomography (CT) scan of the abdomen and pelvis showed a complex cystic mass in the left ovary, multiple peritoneal deposits, a large amount of ascitic fluid, two hypodense lesions in the liver, and multiple enlarged lymph nodes. Diagnostic laparoscopy was performed, and multiple tumor biopsies were obtained. Histopathology showed a cellular tumor composed nests of small round cells embedded in desmoplastic stroma. Immunohistochemistry showed positive staining of the tumor cells for pan-cytokeratin, desmin, Wilms tumor 1 (WT1) antigen, epithelial membrane antigen (EMA), and CD56, which supported the diagnosis of DSRCT. After the second cycle of the P6 Protocol, which included seven courses of chemotherapy, the patient developed a severe and fatal infection.Conclusions:It is important to consider the diagnosis of DSRCT that may present atypically, particularly in patients who present with abdominal and pelvic masses. DSRCT has a rapid and aggressive course that requires early and definitive diagnosis with prompt treatment that includes systemic chemotherapy.

Intra-Abdominal Desmoplastic Small Round Cell Tumor: Current Treatment Options and Perspectives

“…The chemotherapy regimen of IDSRCT mainly follows the same schedule as that used in ES ( 25 ). The P6 chemotherapy regimen, comprising vincristine/doxorubicin/cyclophosphamide alternating with etoposide/ifosfamide (VDC/IE), is the most common neoadjuvant chemotherapy regimen for IDSRCT patients ( 43 , 69 ). Vincristine, doxorubicin, and ifosfamide are a reasonable alternative regimen for older people who may not tolerate the intense regimen ( 73 ).…”

Primary desmoplastic small round cell tumor of the submandibular gland: a case report and literature review

“…DSRCT has no specific clinical symptoms. Most patients present with initial symptoms of abdominal pain, constipation, ascites, and vomiting [ 10 – 14 , 27 , 28 ]. These can be accompanied by the manifestations of cachexia, such as fatigue and emaciation.…”

“…There is no standardized approach for the treatment of this malignant disease. Effective cytoreduction combined with comprehensive therapies, as the best treatment strategy presented in most studies, may improve patient survival rate [ 14 , 15 , 28 , 33 , 34 , 57 ]. With the in-depth analysis of molecular genetics of DSRCT, targeted therapy, immunotherapy, and other methods have been gradually applied to the treatment of DSRCT in recent years [ 58 , 59 ].…”

Desmoplastic small round cell tumor of the kidney: a case report

“…DSRCT shows a specific reciprocal chromosomal translocation, t(11;22)(p13;q12) (EWSR1-WT1 fusion) which generates a chimerical protein with transcriptional regulatory activity. Extraserosal DSRCTs are extremely rare with only a few cases reported in lungs, ovary, soft tissues, bones, intracranial and sinonasal locations [2,3].…”