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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.
Assuntos
Neoplasias Abdominais/diagnóstico , Neoplasias Abdominais/tratamento farmacológico , Tumor Desmoplásico de Pequenas Células Redondas/diagnóstico , Tumor Desmoplásico de Pequenas Células Redondas/tratamento farmacológico , Neoplasias Ovarianas/diagnóstico , Neoplasias Ovarianas/tratamento farmacológico , Dor Abdominal/etiologia , Protocolos de Quimioterapia Combinada Antineoplásica/administração & dosagem , Ascite/etiologia , Biomarcadores Tumorais , Ciclofosfamida/administração & dosagem , Doxorrubicina/administração & dosagem , Etoposídeo/administração & dosagem , Evolução Fatal , Feminino , Humanos , Ifosfamida/administração & dosagem , Jordânia , Vincristina/administração & dosagem , Adulto JovemRESUMO
INTRODUCTION: Primary renal Ewing's sarcoma is a rare tumor with dismal prognosis. The main treatment modality of treatment is surgical excision with adjuvant or neoadjuvant chemotherapy. CASE PRESENTATION: We present a case of primary renal EWS in 27-year-old male. He presented to our center complaining of three days history of severe left flank pain associated with nausea and vomiting. Computerized tomography (CT) revealed a 10â¯×â¯7â¯×â¯6.5â¯cm left lower pole renal mass. Left radical nephrectomy was performed and revealed a tumor with uniform round to oval nuclei cells. The cells stained strongly positive for CD 99. The diagnosis was established as primary renal Ewing's sarcoma. The patient refused the chemotherapy. Fortunately, he was free of the disease on regular clinical and radiological follow-up, the last follow up was 30 months post-nephrectomy. CONCLUSION: This case highlights the importance of proper surgical treatment and its role in the managing this type of malignancy especially in localized disease at presentation.
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INTRODUCTION: Impacted foreign bodies in the esophagus have the potential to cause serious complications. Ingested sharp objects carry the risk of acute complications as: perforation, acute mediastinitis, and acute bleeding. Rarely, such foreign bodies might migrate through the esophageal wall and present as chronic esophageal foreign body. CASE PRESENTATION: We present a case of a 36-month-old girl presented with solid food dysphagia and regurgitation proved to be secondary to esophageal stricture after 26 months of accidental ingestion of aluminum can tab which has migrated through the wall of the upper esophagus into the mediastinum. After two trials of endoscopic treatment; she underwent thoracotomy and partial esophagectomy. Multiple trials of dilation and Mitomycin C injection were followed because of re-stricture. CONCLUSION: Foreign body impaction or secondary stricture needs to be considered in the differential diagnosis of children presenting with new onset dysphagia and regurgitation. Metallic Foreign body might be even radiolucent. Practitioners should keep a high index of suspicion for a retained esophageal FB in the child with gastrointestinal or respiratory symptoms that do not respond to standard therapy.
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Biliary tract cancers (BTC) represent an aggressive disease with a dismal prognosis. Gemcitabine in combination with cisplatin is the standard first-line palliative treatment for advanced BTC. There is no established treatment following progression on gemcitabine-cisplatin. In this article, we present two cases for individuals with advanced BTC who were treated with pembrolizumab and the tumors have completely resolved.
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BACKGROUND: The unicornuate uterus is caused by abnormal or failed development of one Müllerian duct. Unicornuate uteri with functioning non-communicating rudimentary horns are susceptible to many gynaecologic and obstetric complications such as hematometra, endometriosis and ectopic pregnancy and thus surgical resection is usually recommended.. CASE PRESENTATION: We report a rare case of a unicornuate right uterus with rudimentary non-communicating (functional) cavitary left horn (class U4a) in a 17-year-old girl who was diagnosed with VACTERL association. She was presented to our centre with 3 years history of secondary sever dysmenorrhea. Pelvic magnetic resonance imaging revealed a normal uterus on the right side, a 7 × 8 cm left endometrioma, a tortuous dilated fluid-filled structure in the left hemipelvis, mostly represented left-sided hematosalpinx, and a well-defined lesion with thick enhancing wall in the left hemipelvis measuring 6.7 × 5.7 × 5.6 cm with a similar enhancement to the uterus in the right. She underwent laparotomy that showed a right unicornuate uterus with a normal cervix and a rudimentary non-communicating distended left horn. In addition, there was a left endometrioma and left hematosalpinx. Resection of the left communicating horn, left salpingectomy and left ovarian cystectomy were performed. The right tube and both ovaries were preserved. At 9-months follow up, the patient had a regular period and the pain subsided completely. CONCLUSION: We report yet the second case of VACTERL association and unicornuate uterus with non-communicating functional rudimentary horn, in hope of expanding the knowledge of a rare occurrence. This case also highlights the importance of considering the diagnosis of Müllerian duct anomalies in patients with a history of other anomalies, and/or history of early-age secondary dysmenorrhea.