Desmoplastic Small Round Cell Tumor of Stomach

Abu‐Zaid, Ahmed; Azzam, Ayman; Alnajjar, Asma; Al–Hussaini, Hussa; Amin, Tarek

doi:10.1155/2013/907136

Cited by 4 publications

(3 citation statements)

References 27 publications

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“…Also, Idris reported a case of gastric DSRCT in a 28-year-old Syrian man [4]. In 2013, Abu-Zaid et al described a case of DSRCT in a 26-year-old woman from Saudi Arabia [5]. Recently, Nacef et al reported a case in a 46-year-old Tunisian woman [6].…”

Section: Discussionmentioning

confidence: 99%

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

et al. 2019

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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.

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Section: Discussionmentioning

confidence: 99%

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

et al. 2019

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“…4 Very rarely, DSRCT can arise from the head and neck, base of skull, paratesticular region, ovary, brain, or thoracic viscera. 10 Extraperitoneal organ involvement is inconstant and secondary; including lung, 11 liver, 12 kidney, 13 stomach, 14 and pancreas. 15 Liver and lungs are the 2 most common sites of distant metastasis.…”

Section: Discussionmentioning

confidence: 99%

Desmoplastic Small Round Cell Tumor in Transverse Colon: Report of a Rare Case

Huang

Sha

Zhang

et al. 2015

International Surgery

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Desmoplastic small round cell tumor (DSRCT) is an extremely rare, highly aggressive, malignant tumor of undetermined histogenesis. Adolescent males are primarily affected with a typically abdominal or pelvic mass. Diagnosis is based on histologic analysis of biopsy and cytogenetic studies. Owing to the rarity of the tumor and the unusually aggressive presentation, treatment is challenging and has not been standardized. DSRCT has a very poor prognosis, with a median survival range of 17 to 25 months. In this work, we report a case of DSRCT in the transverse colon, which has never before been reported in the literature. Our case study is of a 30-year-old Chinese female who presented with a history of 6 months abdominal discomfort and fatigue. A palpable, hardly mobile mass was detected in the right lower quadrant abdomen by physical examination. Abdominal and pelvic magnetic resonance imaging revealed an 8.0 3 10.5 3 11.1-cm mass with no pulmonary and hepatic metastasis. The patient underwent exploratory laparotomy and transverse colectomy, which revealed a mass in the transverse colon and no metastasis in the peritoneum, greater omentum, or mesentery. Immunohistochemistry findings revealed positive staining for epithelial, mesenchymal, and neural markers, which confirmed the presentation of DSRCT. A 6-month postoperative follow-up failed to find any recurrence. DSRCT is a highly aggressive, malignant, mesenchymal tumor with a very poor prognosis. No consensus has been reached for the treatment protocol of DSRCT. However, debulking surgery with postoperative chemotherapy or radiotherapy might promise more optimistic results on survival.

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“…They were first described in 1989 by Gerald and Rosai as small round blue cell tumours with predilection for serosal surfaces and occurring predominantly in young Caucasian males with predominantly intra-abdominal locations with focal rhabdoid pattern with an intense desmoplastic reaction [ 1 , 2 ]. Up till 2013, less than 200 cases had been reported in the world literature [ 3 ].…”

Section: Discussionmentioning

confidence: 99%

A Rare Case of Metastatic Desmoplastic Small Round Cell Tumour: Diagnosis and Management

Nabi

Saste

Gulati

2015

Case Reports in Oncological Medicine

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A 26-year-old male without any significant past medical history presented to the hospital with shortness of breath, cough, pleuritic chest pain, and weight loss for the past 3 months. On chest CT, he was found to have extensive mediastinal and hilar lymphadenopathy and multiple pulmonary nodules. On physical examination, a right groin mass was noted which had been slowly growing for the past 2 years. Ultrasound of the groin showed complex solid mass with internal vascular channels. CT guided biopsy of the mass showed desmoplastic small round cell tumour. His hospital course was complicated by hypoxic respiratory failure requiring emergent intubation and ICU admission where he completed one cycle of vincristine, cyclophosphamide, and doxorubicin with subsequent improvement, followed by extubation. His condition continued to improve after second cycle of chemotherapy and he was ultimately discharged in a stable condition to continue outpatient chemotherapy after a 2-month inpatient stay.

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Desmoplastic Small Round Cell Tumor of Stomach

Cited by 4 publications

References 27 publications

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

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

Desmoplastic Small Round Cell Tumor in Transverse Colon: Report of a Rare Case

A Rare Case of Metastatic Desmoplastic Small Round Cell Tumour: Diagnosis and Management

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