EPITHELIOID INFLAMMATORY MYOFIBRO-BLASTIC SARCOMA: A CASE REPORT

Case report: A 58 - year old man presented with weight loss, asthenia and increased abdominal volume. Abdominal CT scan showed extensive abdominal lesion, solid heterogeneous, with central necrosis areas and calcification focuses, 2,0×16,5 cm, located in the right flank/iliac fossa, involving mesenteric vessels and dislocating small bowel loops without intestinal obstruction. 3 months ago went to surgery to elucidate diagnosis, beyond realizing biopsy, the tumor was considered inoperable. The immunohistochemical study (IHC) indicated a lesion with fibrosis, vascular proliferation and presence of innumerable epithelioid cells, broad cytoplasm, eosinophilic with big nucleus and prominent nucleolus (figure 1). Between the cells there was inflammatory infiltration. IHC also revealed intense and diffuse CD30 expression (figure 2) and negative expression for ALK, suggesting Epithelioid Inflammatory Myofibroblastic Sarcoma. Patient is being treated with palliative chemotherapy protocol with Carbo + Taxol every 21 days for 6 cycles. Discussion The inflammatory myofibroblastic tumor (IMT) is rare and occurs especially in the abdomen, pelvis and thorax. Approximately 50% of IMTs present abnormal anaplastic lymphoma kinase (ALK) due to rearrangements of the ALK gene. An intra-abdominal subclass of this tumor was described, in 2011, termed by Epithelioid Inflammatory Myofibroblastic Sarcoma, an aggressive variant of the tumor, constituted principally of epithelioid cells and proeminent neutrophilic infiltration. The EIMS has predilection for intra abdominal regions; appearing generally in male patients. The related cases denote wide morphological, immunohistochemical (CD30 expression) and clinical (anatomic location) related to EIMS. The solely absence of ALK expression would not correspond to EIMS. Nonetheless, it is important to mention that a part of the cases do not express ALK. Final Comments A rare Epithelioid Inflammatory Myofibroblastic Sarcoma case was described, ALK negative and unresectable, still in investigation. Though the absence of ALK expression, the IHQ results suggest EIMS. Considering the case rarity, a NGS Foundation HEME was requested to confirm negativity of ALK expression. If it is confirmed, the current treatment will continue. In case that positivity is confirmed, the patient will start immunotherapy.

 

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No conflict of interest has been declared by the author(s).

Publication History

Article published online:
23 October 2019