Résumé
Aims: The authors presents a case of pure Renal epithelioid angiomyolipoma (EAML) and discuss the clinicopathological characteristics and the diagnosis problems of this entity. Case Presentation: A 51 year-old man presented with a persistant pain in the left lumbar region. Imaging revealed a left kidney tumor that was hemorrhagic and necrotic on gross examination. At histology, proliferation of 99% of atypical epithelioid cells, staining for HMB-45 and Melan A was observed. Our patient had lung and right adrenal metastasis six months after surgery and succumbed one year later. Conclusion: In summary, the pure form of EAML is extremely rare. When diagnosed, the risk of tumor progression is high, especially if other histological predictive parameters of progression are observed. In that case, active treatment should be undergone and patients should be closely monitored.
Résumé
Background: Gastrointestinal stromal tumors - commonly called GISTs - are rare; yet, they represent an important type of mensenchymal tumors arising within the gastrointestinal tract. The acronym GIST was introduced in 1998 to define a well established pathological entity which bears a highly specific marker called KIT [CD117]. Scientific interest for these tumors in recent years is mainly due to the progress in identification by immunohistochemistry and the advent of targeted molecular therapy
Aim: To reclassify mesenchymal tumors of the digestive tract using advanced immunophenotyping
Methods: We examined the digestive tissue tumors operated at the Principal Military Hospital of Instruction of Tunis over a 19-year period from 1992 to 2011
Results: We collected 22 cases of benign mesenchymal tumors of the digestive tract. Tumors were initially diagnosed as leiomyomas [4 cases], schwannomas [2 cases], gastrointestinal stromal tumors [14 cases], a leiomyoblastoma and a mesenchymal tumor with fusiform cells. The immunohistochemical study has allowed to correct some diagnoses and, in one case, the block has been exhausted. In total, we selected 18 cases of gastrointestinal stromal tumor, a schwannoma and two leiomyosarcomas among 21 mesenchymal tumors. The final diagnosis of GIST was confirmed by the positivity of C-kit or DOG1
Conclusions: The combined use of C-kit and DOG1 ensures accurate diagnosis of GIST. The DOG1 has allowed us to detect the 3 stromal tumors negative for C-kit. This antibody has achieved a diagnostic gain of 15%. The rate of GIST tumors labeled increased from 71% to 86% among mesenchymal tumors