Reid Robin, de Silva M V Chandu, Paterson Lindsay, Ryan Eleanor, Fisher Cyril
University Department of Pathology/Scottish Bone Tumour Registry Western Infirmary, Glasgow, United Kingdom.
Am J Surg Pathol. 2003 Sep;27(9):1229-36. doi: 10.1097/00000478-200309000-00006.
Low-grade fibromyxoid sarcoma (LGFMS) is a rare metastasizing soft tissue tumor with deceptively bland histologic features. The hyalinizing spindle cell tumor with giant rosettes (HSCT) is thought to be a closely related tumor differing only by the presence of collagen rosettes. We report the occurrence of a common t(7;16)(q34;p11) translocation in 2 cases of HSCT and 2 cases of LGFMS, thereby providing the first cytogenetic proof that LGFMS and HSCT are variants of the same entity. The tumors occurred in the thighs of 2 females and in the buttock and supraclavicular fossa of 2 males. One HSCT had a spectrum of unusual histologic features, including the presence of plump epithelioid cells with abundant cytoplasm and strands and nests of clear epithelioid cells separated by eosinophilic hyalinized stroma. Two cases showed a hitherto unreported, focal staining with epithelial membrane antigen, thus adding to the immunohistochemical profile of these tumors. LGFMS and HSCT probably have a wider spectrum of morphologic features than previously thought, the awareness of which will help pathologists to avoid diagnostic pitfalls. Demonstration of the t(7;16)(q34;p11) translocation will help to diagnose difficult cases with unusual histologic features.
低度恶性纤维黏液样肉瘤（LGFMS）是一种罕见的转移性软组织肿瘤，其组织学特征看似平淡无奇。伴有巨大玫瑰花结的透明变性梭形细胞瘤（HSCT）被认为是一种密切相关的肿瘤，仅因存在胶原玫瑰花结而有所不同。我们报告了在2例HSCT和2例LGFMS中出现常见的t(7;16)(q34;p11)易位，从而提供了首个细胞遗传学证据，证明LGFMS和HSCT是同一实体的不同变体。这些肿瘤发生在2名女性的大腿以及2名男性的臀部和锁骨上窝。1例HSCT具有一系列不寻常的组织学特征，包括存在胞质丰富的丰满上皮样细胞以及由嗜酸性透明变性间质分隔的透明上皮样细胞条索和巢团。2例病例显示出一种迄今未报道的上皮膜抗原局灶性染色，从而丰富了这些肿瘤的免疫组化特征。LGFMS和HSCT可能具有比先前认为的更广泛的形态学特征，认识到这一点将有助于病理学家避免诊断陷阱。t(7;16)(q34;p11)易位的证实将有助于诊断具有不寻常组织学特征的疑难病例。
