Paredes B E, Mentzel T, Denfeld R
Dermatopathologie Friedrichshafen, Siemensstr. 6/1, 88048, Friedrichshafen, Deutschland.
Hautarzt. 2012 Sep;63(9):719-23. doi: 10.1007/s00105-012-2383-2.
Malignant skin tumors are seldom polypoid lesions. Thus lesions presenting as large skin tags, as in this case, may initially not be recognized and treated as a malignancy. Often such protuberant lesions are removed by shave biopsy. Then, as in this case, the clinician is very surprised to receive the diagnosis of myxofibrosarcoma (MFS). This case shows how important it is to further investigate a common tumor, like a large skin tag, when its clinical appearance is unusual. Myxofibrosarcoma or myxoid malignant fibrous histiocytoma is one of the common, perhaps most common, sarcoma in adults. Clinical presentation as a large skin tag is extremely uncommon. The rapid growth of the polypoid sarcoma indicates the need for completely operative removal. The behavior of the MFS depends on the degree of malignancy, depending on the tumor size, depth of penetration and local control. While smaller dermal or subcutaneous localized MFS hold potentially a risk of recurrence, do larger examples with a high tumor grade and seated in the deep soft tissue posses a variable metastatic potential. Despite large variability of cell number, cellular atypia and the numbers of mitotic figures all myxofibrosarcoma have in common the multinodular structure with incomplete septa, the mucinous stroma and curvilinear fine vessels.
恶性皮肤肿瘤很少表现为息肉样病变。因此,像本例这样表现为巨大皮赘的病变,最初可能不会被识别并当作恶性肿瘤进行治疗。这类突出的病变通常通过削除活检来切除。然后,就像本例一样,临床医生在收到黏液纤维肉瘤(MFS)的诊断时会非常惊讶。这个病例表明,当一个常见肿瘤(如巨大皮赘)的临床表现不寻常时,进一步检查是多么重要。黏液纤维肉瘤或黏液样恶性纤维组织细胞瘤是成人中常见的,也许是最常见的肉瘤之一。表现为巨大皮赘极为罕见。息肉样肉瘤的快速生长表明需要进行完整的手术切除。MFS的行为取决于恶性程度,这又取决于肿瘤大小、浸润深度和局部控制情况。较小的位于真皮或皮下的局限性MFS有潜在的复发风险,而较大的、肿瘤分级高且位于深部软组织的病例则有不同程度的转移潜能。尽管细胞数量、细胞异型性和有丝分裂象数量差异很大,但所有黏液纤维肉瘤都具有共同特征,即有不完全间隔的多结节结构、黏液样间质和曲线形细血管。