Department of Dermatology, Kansai Medical University Medical Center, Osaka, Japan.
Department of Plastic Surgery, Kansai Medical University Medical Center, Osaka, Japan.
J Dermatol. 2019 Mar;46(3):267-270. doi: 10.1111/1346-8138.14755. Epub 2019 Jan 4.
An 82-year-old Japanese man presented with a 90 mm × 80 mm subcutaneous nodule on the left chest, not connected to the pleura, described as hypointense on T -weighted magnetic resonance imaging. Histologically, a well-demarcated nodule demonstrated a patternless pattern composed of hemangiopericytoma-like and short storiform patterns from the subcutis into deeper tissue. Antler-like branching blood vessels and keloidal-type collagen bundles were seen. Myxoid change was seen in hypocellular areas. Bizarre cells and multinucleated giant cells could be easily identified. Approximately 5 mitoses per 10 high-power fields could be detected. The majority of proliferating spindle cells were positive for CD34, CD99 and BCL2 expression. Signal transducer and activator of transcription (STAT)6 was strongly expressed in the nuclei. The Ki-67 proliferation index was 60%. We performed wide local excision with a 30-mm margin, along with resection of intercostal muscles, fasciae and periostea. Neither local recurrence nor distant metastases have occurred in 16 months of follow up. The present case is consistent with malignant solitary fibrous tumor in the subcutis histologically mimicking myxofibrosarcoma or undifferentiated pleomorphic sarcoma, definitively diagnosed by immunoexpression of STAT6. We performed a review of the published work and identified six previously reported patients with malignant superficial solitary fibrous tumor to validate the characteristics of this rare type. Four men and two women were included. The sarcomas involved the head area, trunk and thigh. Only one tumor-associated death occurred despite excision and radiotherapy. Complete excision is a possible treatment choice for this tumor type.
一位 82 岁的日本男性,左侧胸部出现一个 90mm×80mm 的皮下结节,与胸膜不相连,在 T1 加权磁共振成像上呈低信号。组织学上,一个界限清楚的结节显示出一种无模式的模式,由皮下组织向深部组织的血管外皮细胞瘤样和短束状模式组成。可见鹿角状分支血管和瘢痕样胶原束。在细胞稀少区可见黏液样变性。容易识别奇异细胞和多核巨细胞。每 10 个高倍视野可检测到约 5 个有丝分裂。大多数增殖的梭形细胞阳性表达 CD34、CD99 和 BCL2。信号转导和转录激活因子(STAT)6 在核内强烈表达。Ki-67 增殖指数为 60%。我们进行了广泛的局部切除,切除了 30mm 的边缘,同时切除了肋间肌、筋膜和骨膜。在 16 个月的随访中,没有发生局部复发或远处转移。本病例符合组织学上模仿黏液纤维肉瘤或未分化多形性肉瘤的皮下恶性孤立性纤维瘤,通过 STAT6 的免疫表达明确诊断。我们对已发表的文献进行了回顾,确定了 6 例以前报道的恶性浅表性孤立性纤维瘤患者,以验证这种罕见类型的特征。包括 4 名男性和 2 名女性。肉瘤累及头部、躯干和大腿。尽管进行了切除和放疗,但仅发生 1 例与肿瘤相关的死亡。完全切除可能是这种肿瘤类型的治疗选择。