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Key Clinical Message: Duodenal GISTs are rare and challenging tumors. Acute life-threatening upper GI bleeding is a possible presentation of duodenal GISTs. Surgery is the standard treatment for localized duodenal GISTs. Imatinib is an effective adjuvant therapy for duodenal GISTs. Abstract: GIST is the most common mesenchymal neoplasm of the gastrointestinal tract, accounting for 1%-2% of gastrointestinal tumors. They originate from the interstitial cells of Cajal and are rare in patients younger than 30 years. The stomach is the most common site, followed by the small intestine and colon. GISTs are caused by a gain-of-function mutation in the proto-oncogene receptor tyrosine kinase, with activating mutations in KIT being the most common. Most GISTs are asymptomatic. Even if gastrointestinal bleeding is the most common complication life-threatening hemorrhage is extremely uncommon. We present a case of a 31-year-old male patient presented with massive active hematemesis and melena with hemorrhagic shock. The patient presented with massive hematemesis and melena of 1 h duration. Endoscopy showed pulsating active bleeding from the third part of the duodenum which was difficult to manage via endoscopy. Histopathologic evaluation showed spindle cell type GIST. Intraoperatively, there was a nodular mass with active bleeding on the third part of the duodenum. Duodenectomy with end-to-end anastomosis was done. Discharged with no postoperative complication and was put on imatinib. There are considerable challenges that arise in the diagnosis and treatment of duodenal gastrointestinal stromal tumors (GISTs) when they present with life-threatening upper gastrointestinal hemorrhage. In order to achieve the best possible outcomes for patients, it is crucial to have a comprehensive understanding of the clinical presentation, diagnostic methods, and treatment approaches.
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INTRODUCTION AND IMPORTANCE: Extraskeletal Ewing's sarcoma is an uncommon tumor with a devastating prognosis and a very high mortality rate, particularly in metastatic forms, it primarily affects young people mainly in the 2nd to 4th decades of life. It can affect different parts of the body, without a particular clinical presentation which delays diagnosis. CASE PRESENTATION: 12-year-old male patient presented with swelling over the left proximal thigh of 7 months duration associated with pain and limping. On physical examination, he had about 20 × 15 cm firm, tender, and erythematous proximal left thigh swelling that is fixed to the structures with an overlying scar. CLINICAL DISCUSSION: Extra skeletal Ewing's sarcoma (EES) is a member of the Ewing Sarcoma Family of tumors. EES is a rare tumor with an incidence ranging from 0.1 to 0.4 per a million people. It presented with rapidly increasing swelling mainly over the soft tissue of the proximal thigh, pelvis, paravertebral region, chest wall, upper arm and shoulder. Age at presentation ranges 10 to 30 years with no gender preference. Imaging is crucial in the diagnosis, preoperative assessment, in staging and evaluation of treatment outcomes. Histopathology study is mandatory for the definitive diagnosis of EES among competitive differential diagnoses. The management of EES includes Surgery, chemotherapy and/or radiation therapy based on the stage of the disease. CONCLUSION: EES is a rare tumor but it should be in the differential diagnosis of adolescent patients presented with soft tissue mass/swelling.
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Struma ovarii is an infrequent type of teratoma arising from the ovary accounting for only 2% of all ovarian teratomas. These tumors have a benign biology with rare malignant transformation in about 3% of cases. The most common malignant transformation that arises from struma ovarii is papillary thyroid carcinoma. These neoplasms act in the same way as those arising from the thyroid gland, but due to the rarity of their occurrence there is still a debate over therapeutic options. We present a case of a 41-year-old Ethiopian Para IX woman presented with abdominal swelling for four years, accompanied by dull pain, satiety, and weight loss. Her vital signs were normal, and her abdominal examination revealed a large abdominopelvic mass. Her CA-125 was elevated, and her blood count, organ function tests, and serum electrolyte levels were normal. Abdominal ultrasound revealed a complex abdominopelvic mass with cystic and solid components, possibly ovarian teratoma. The patient underwent surgery, revealing a 14 by 10 cm right ovarian mass and a 3×3 cm appendiceal mass. Subsequently, total abdominal hysterectomy, bilateral salpingo-oophorectomy, total omentectomy, and appendectomy was done. Histopathologic evaluation revealed follicular proliferation of enlarged cells, with no papillary growth pattern. The case was diagnosed as malignant struma ovarii, a follicular variant of papillary thyroid carcinoma (FVPTC) with secondary deposits on the appendix. A complete thyroidectomy was done after the histopathology diagnosis. Malignant struma ovarii is rare making it challenging to treat since there are no established prognosticating histopathologic or clinical characteristics. The tumor size and metastasis determine the surgical treatment scope. Large-scale investigations are essential for prognostication and treatment options considering pathologic traits.
