Melanotic Xp11-associated tumor of the sigmoid colon:A case report

BACKGROUND Melanotic Xp11-associated tumors are rare mesenchymal-derived tumors. So far,most primary melanotic Xp11-associated tumors have been reported in the kidney, and reports of this tumor in the gastrointestinal tract are rare.CASE SUMMARY Here we describe the case of a 25-year-old woman who presented with a melanotic Xp11-associated tumor in the sigmoid colon. Colonoscopy revealed a large mucosal bulge in the sigmoid colon, approximately 32 cm inside the anus.The surface was rough with local erosion. The tumor was brittle on biopsy and bled easily. Computed tomography revealed thickening of the rectal wall with edema. Postoperative pathology indicated the likelihood of a perivascular epithelioid cell tumor. Histologically, the tumor comprised plump epithelioid cells with abundant clear to lightly eosinophilic cytoplasm and round nuclei arranged in an alveolar or trabecular pattern. The tumor cells were strongly positive for HMB-45, Melan-A, Cathepsin K, and TFE3 but negative for vimentin,smooth muscle actin, S100 protein, CD10, CK20, and desmin. The tumor cells had a low Ki-67 labeling index(approximately 2%). Fluorescence in situ hybridization revealed TFE3 fracture. Based on these histologic and immunohistochemical features, a diagnosis of melanotic Xp11-associated tumor of the sigmoid colon was made.CONCLUSION In summary, we report the clinicopathological features of a primary tumor that is extremely rare in the sigmoid colon and review the clinicopathological characteristics of melanotic Xp11-associated tumors, compatible with the very rare tumor termed "melanotic Xp11 translocation renal cancer" in all aspects.

Systemic treatment for severe concentrated sulfuric acid burns in an adult male at high altitude:A case report

BACKGROUND The aim of this study was to report a case of a patient who suffered from severe concentrated sulfuric acid burns while working at high altitude.This patient recovered after systemic treatment.We also provide a literature review for a better understanding of the disease.CASE SUMMARY A 30-year-old male,who was working in a local chemical plant in Xining at an altitude of 2261 m,suffered from 85%burns(Ⅲ°70%,deep Ⅱ°15%)after a tank containing 80%concentration of sulfuric acid exploded.The patient immediately received a series of first aid treatments,as well as rigorous wound managements after admission,which included protection for the whole body and organs,prevention and treatment of eye burns,and the appropriate oxygen therapy.After 65 d of treatment,the burn wounds had completely healed,and the patient was transferred to another specialized hospital for further eye treatment.The first aid before admission and the emergency treatment of wounds following admission were appropriate.No severe complications of sepsis,severe renal insufficiency,septic costal chondritis,corneal perforation or other burns occurred during the treatment.CONCLUSION The main causes of concentrated sulfuric acid burns consisted of accidental burns at work,accidents in the outside,factitious injuries and improper laboratory operations.The clinical manifestations were mostly deep Ⅱ°and Ⅲ°burns,with a formation of brown-black,leather-like eschar on the wound surface and locally embolized dendrite-like vessels.The clear cause of the injury and typical clinical manifestations in this case made it easy to diagnosis.However,adult cases with severe concentrated sulfuric acid burns in high altitude areas are rare,so the successful treatment of this case is of great significance.