Intraocular ME is frequently a properly circumscribed and benign tumor, although CNS ME is an aggressive neoplasm linked using a good prognosis only if absolutely re moved. Taking into consideration the rarity of peripheral medul loephitelioma, the optimal treatment has yet to become established. We report a case of PME with an exciting target pro tein expression suggesting a attainable option thera peutic strategy for this rare tumor. Case presentation A three year old female presented with a 1 month his tory of abdominal pain and anorexia. The abdominal ultrasonography showed a retroperitoneal mass confirmed by CT scan having a ideal hydronephrosis without proof of metastatic spread. An open biopsy of your lesion was performed.
The pathology revealed a malignant neoplasm composed of tubules, pap illary structures, ribbons of primitive stratified columnar cells, vesicular nuclei, and higher nuclear cytoplasmic ratio. This histopathology the full report is similar to the structure in the primitive epithelium of the medullary plate and neural tube. The absence of cilia and blepharoplasts ruled out the hypothesis of a papillary ependymoma. The neoplastic cells showed a diffuse positivity for CD56 and WT1 as well as a variable positivity for NSE, Synaptophisin, S100 protein and Cytokeratin MNF116, although they had been damaging for CD99, alpha fetoprotein, CD30, OCT3 four, B HCG. The diagnosis was neuroectodermal embryonal tumor with patterns of ME. The youngster started chemotherapy in accordance with our neighborhood protocol for Ewing Sarcoma Family Tumor. Immediately after 2 ICE courses and two CAV, she achieved partial response, the mass measuring 5 three.
three three. 8 cm. Grade 4 bone marrow toxicity that required red blood cells and platelets transfusion and hospitalization for neutopenic fever, was recorded after all courses. A complete resection of your lesion was performed. selleck Nilotinib The pathology showed comprehensive involutive post chemotherapy elements. The residual viable tumor showed histologic as pects overlapping with these on the first biopsy, partly characterized by additional solid areas, with the same immuno phenotypic pattern. The kid, in complete remission, completed the treat ment with two CE courses plus a last CAV course. As a consolidation treatment, she received a high dose chemotherapy according to Busulfan and Melphalan with autologous peripheral blood stem cells rescue and, fi nally, radiotherapy to the primary tumor bed. Throughout the whole chemotherapy treatment, only grade IV bone marrow toxicity was recorded. Throughout radiotherapy the patient presented only grade I diarrhea. Six months just after remedy discontinuation, she pre sented with an abdominal relapse. Surgery was performed, achieving a second full remission. The pathology confirmed a ME together with the same characteris tics with the primary tumor.