Gadeyne Laura, Creytens David, Dekeyser Sam, Van der Meulen Joni, Haspeslagh Marc
Dermpat, Ghent, Belgium.
Department of Pathology, Ghent University Hospital, Ghent, Belgium.
Am J Dermatopathol. 2022 Apr 1;44(4):302-305. doi: 10.1097/DAD.0000000000002096.
Inflammatory myofibroblastic tumors are rare soft tissue neoplasms with an uncertain biological behavior, derived from fibroblastic and myofibroblastic cells. In rare cases, a peculiar epithelioid phenotypic variant of this tumor is encountered, named epithelioid inflammatory myofibroblastic sarcoma (EIMS). EIMS has overlapping features with inflammatory myofibroblastic tumor but has been correlated with a more aggressive clinical course, a characteristic nuclear membrane or perinuclear anaplastic lymphoma kinase (ALK) immunostaining pattern and a very specific RANBP2-ALK fusion. To date, EIMS has been reported almost exclusively in the abdominal and pelvic cavity, with the exception of some intrathoracic cases. Herein, we present the first case of primary cutaneous EIMS, confirmed by molecular analysis showing the diagnostic RANBP2-ALK fusion.
炎性肌纤维母细胞瘤是一种罕见的软组织肿瘤，其生物学行为尚不确定，由成纤维细胞和肌成纤维细胞衍生而来。在罕见情况下，会遇到这种肿瘤的一种特殊上皮样表型变体，称为上皮样炎性肌纤维母细胞肉瘤（EIMS）。EIMS与炎性肌纤维母细胞瘤有重叠特征，但与更具侵袭性的临床病程、特征性的核膜或核周间变性淋巴瘤激酶（ALK）免疫染色模式以及非常特异的RANBP2-ALK融合相关。迄今为止，除了一些胸腔内病例外，EIMS几乎仅在腹腔和盆腔被报道。在此，我们报告首例经分子分析证实存在诊断性RANBP2-ALK融合的原发性皮肤EIMS病例。
