Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0000737 (abdominal pain)
 31,184 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

BACKGROUND 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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PMID:A 23-Year-Old Joradanian Woman with a Desmoplastic Small Round Cell Tumor Involving the Ovary. 3172 17

Desmoplastic small round cell tumor (DSRCT) is a type of soft-tissue sarcoma with poor prognosis. Current treatments include multidisciplinary treatment options such as surgery, chemotherapy, and radiotherapy. Apatinib is an oral, small-molecule, anti-tumor, angiogenesis-targeted drug, which acts mainly on the intracellular binding site of vascular endothelial growth factor receptor-2. In this study, we administered apatinib in combination with chemotherapy to achieve good disease control. This is a 31-year-old male who presented with upper abdominal pain, nausea, and anorexia for over a month. Imaging revealed multiple solid masses and ascites in the liver and abdominal cavity. He was diagnosed as having cholangiocarcinoma with metastasis to the liver, both lungs, bone, and multiple lymph nodes in the neck, abdominal and pelvic cavity, retroperitoneum, and palpitate angle, based on a percutaneous biopsy of the liver and an abdominal mass, and other examinations. Computed tomography revealed disease progression after two cycles of gemcitabine combined with nedaplatin chemotherapy. Next-generation sequencing detection based on the Illumina high-throughput sequencing platform suggested EWSR1 exon7- Wilms tumor 1 exon8 fusion. The pathology was verified and diagnosed as DSRCT. The chemotherapy regimen was changed to cyclophosphamide, epirubicin, vincristine, and oral apatinib for two cycles. The lesions were mostly reduced, and partial response was evaluated. This case is the first report of the efficacy of apatinib combined with systemic chemotherapy in the treatment of DSRCT, which can become an alternative treatment for this disease.
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PMID:Chemotherapy combined with apatinib for the treatment of desmoplastic small round cell tumors: A case report. 3300 67