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INTRODUCTION: Myofibroblastoma is usually occurred in the breast and extra-mammary disease is rare. PRESENTATION OF CASE: A 38-year-old man was admitted to our hospital for further examination of the left inguinal tumour, present and enlarging for 16 months. The tumor was 50 mm in diameter, well-circumscribed, firm, and painless. Ultrasonogaphy, computed tomography(CT) and magnetic resonance imaging (MRI) could not provide the definitive diagnosis. Surgical exploration confirmed a 50 mm tumour with a clear surface with a thin capsule. Complete excision was achieved. Histopathology confirmed the tumor had oval and spindle shaped fibroblastic cells with rich collagen deposition. It stained positive for ER, CD34, desmin and CD10 but negative for -smooth muscle actin and S-100. A mammary-type myofibroblastoma was diagnosed based on these results. DISCUSSION: An extra-mammary myofibroblastoma is very rare and first reported in 2001. Since then, over 160 cases have been reported. On immunohistochemistry, these lesions are characteristically positive for CD34 and desmin, with variable staining for α-smooth muscle actin. Once the diagnosis is made, regardless of size or location, this tumour behaves in a benign fashion after surgical excision reported so far. CONCLUSION: This case is rare, but the correct diagnosis and treatment is important for good prognosis.
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There is no consensus guidelines for treating duodenal variceal bleeding, which is a rare and life-threatening complication of portal hypertension. Here we report an exceedingly unusual case in a 9-year-old boy who had developed left-sided portal hypertension after surgical treatment for pancreatoblastoma followed by a duodenal variceal bleeding with massive melena, severe anemia (hemoglobin 4.5 g/dL) and hypovolemic shock. Emergency partial splenic arterial embolization (PSE) provided a reduction of variceal bleeding and improved blood pressure. Endoscopic injection sclerotherapy (EIS) was subsequently performed and stopped the duodenal variceal bleeding without the complication of portal vein thrombosis caused by injected sclerosant under hepatopetal flow. Our case demonstrates that emergency combined therapy with PSE and EIS can be considered as the therapeutic option for the management of left-sided portal hypertension-induced ectopic variceal bleedings in order to avoid the complication of portal embolization by EIS and provide effective hematostasis.