St Teresa's Hospital, Kowloon, Hong Kong SAR, China.
Queen Elizabeth Hospital, Kowloon, Hong Kong SAR, China.
Int J Surg Pathol. 2021 Feb;29(1):64-68. doi: 10.1177/1066896920930799. Epub 2020 Jun 3.
Hemosiderotic fibrolipomatous tumor is a rare soft tissue tumor that preferentially affects the dorsum of foot, shows recurrent t(1;10) translocation targeting and () genes, and has a high recurrence potential. Hemosiderin deposits, mature adipocytes, and interspersed spindle cells are the 3 cardinal morphologic features of this tumor. We describe a "pauci-hemosiderotic" example involving the left wrist of a 45-year-old female, posing a diagnostic pitfall. The tumor comprised mature adipose tissue traversed by variably thick fibrous septa containing short fascicles of spindle cells. Prominent small- to medium-sized blood vessels were present, often with perivascular fibrosis or aggregates of foamy histiocytes, sometimes associated with red cell extravasation. Hemosiderin was not conspicuous, but fine deposits could be found focally on careful search and with the aid of Perls stain. The diagnosis was further confirmed by diffuse expression of CD34 and presence of translocation by fluorescence in situ hybridization. Pathologists should be aware that hemosiderin deposition can be scanty and focal in hemosiderotic fibrolipomatous, but the rich vasculature with a "damaged" appearance is a useful diagnostic clue.
含铁血黄素性纤维脂肪瘤样肿瘤是一种罕见的软组织肿瘤,好发于足背部,表现出反复的 t(1;10)易位,涉及 和 ()基因,具有较高的复发潜能。含铁血黄素沉积、成熟脂肪细胞和散在的梭形细胞是该肿瘤的 3 个主要形态学特征。我们描述了一例发生于 45 岁女性左侧腕部的“少血铁质”样病变,这是一个诊断陷阱。肿瘤由成熟脂肪组织构成,其间穿插着纤维性间隔,其中包含短束状梭形细胞。存在明显的小至中等大小的血管,常伴有血管周围纤维化或泡沫状组织细胞聚集,有时伴有红细胞渗出。含铁血黄素不明显,但仔细检查和 Perls 染色可发现局灶性细微沉积物。CD34 弥漫表达和荧光原位杂交检测到 易位进一步证实了诊断。病理医生应该意识到,在含铁血黄素性纤维脂肪瘤样肿瘤中,含铁血黄素沉积可能稀少且局灶性,但丰富的具有“损伤”外观的血管是一个有用的诊断线索。