A case of pleomorphic fibroma of the skin presenting as intradermal nodule
A case of pleomorphic fibroma of the skin presenting as intradermal nodule
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表现为皮内结节的皮肤多形性纤维瘤一例
DOI：
10.1097/dad.0b013e318288cd71
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发表时间：
2015
期刊：
影响因子：
--
通讯作者：
Masahiko Muto
中科院分区：
文献类型：
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作者：
Yoshitaka Nakamura;Akiko Nakamura;Masahiko Muto
致编辑：皮肤多形性纤维瘤（PFS）是一种罕见的皮肤纤维瘤，首先由Kamino等人于1989年描述。1 PFS通常表现为中老年人躯干或四肢上的肉色圆顶状丘疹。很少，PFS可能出现在其他部位，如面部和指甲下区域。组织学上，病变细胞稀疏，主要由厚的、杂乱排列的胶原蛋白组成。典型特征是散在的梭形或星状细胞，包括多核巨细胞，核大而多形，深染，核仁小。病变直径几乎总是0.5-2.0 cm，临床上常被误认为痣、神经纤维瘤或血管瘤。在这里，我们报告了一例PFS病例，患者为一名健康的50岁日本男性，表现为背部无痛、核桃大小的皮内结节，临床上与表皮囊肿相似。一名50岁的日本男性被转诊到我科，他的背部有一个无症状的皮下肿块的长期病史，在大约8年的时间里逐渐扩大，以前没有任何受伤的历史。他在其他方面健康，病史不明显。体格检查时，肿块直径3 cm，轻微隆起，可移动的，弹性硬，并伴有覆盖皮肤的轻微红斑（图1A）。超声检查发现一个等回声到低回声的肿块，大小为25.2× 27.3× 11.9 mm，后方声学增强，侧方阴影（图1B）。常规实验室检查结果在正常范围内。初步诊断为表皮囊肿，并在局部麻醉下手术切除病变。组织学检查显示真皮网状层有一个细胞减少的病变，并延伸到皮下组织的边缘（图2A）。上覆表皮既无棘层，也无色素沉着。病变由散在排列的梭形细胞组成，细胞核呈梭形，胞浆嗜酸性，胶原基质中有星状单核细胞伴轻度细胞学异常（图2B）。未观察到有丝分裂像或坏死。免疫组织化学显示病变细胞表达波形蛋白、平滑肌肌动蛋白、因子XIIIa、CD 99和CD 34（图2C），但S100蛋白和结蛋白阴性。根据这些结果，我们将病变诊断为PFS。1年随访时未发现复发。PFS是一种罕见的纤维性肿瘤，其特征是核内瘤，但具有良性临床过程。医学文献中的报告不到20例。2免疫组化，肿瘤细胞弥漫性波形蛋白染色，不同数量的细胞平滑肌肌动蛋白和CD 34阳性，提示肌纤维母细胞或真皮树突状细胞起源。S100蛋白、结蛋白和细胞角蛋白的免疫反应为阴性。一些PFS也具有骨纤维瘤的特征。事实上，一些研究者假设PFS实际上是硬皮病纤维瘤的一种变体。[3]或者，其他研究者将这些肿瘤称为多形性巩膜纤维瘤。4在我们的病例中，没有增厚和均质化的嗜酸性胶原纤维束以层状方式排列（螺旋图案），中间有明显的裂缝;因此，我们诊断病变为PFS，而不是硬皮病或多形性硬皮病纤维瘤。PFS临床上可能类似于痣，神经纤维瘤，血管瘤，纤维角化瘤或纤维上皮息肉。我们的初步临床诊断是表皮...
To the Editor: Pleomorphic fibroma of the skin (PFS) is a rare cutaneous fibrous tumor first described by Kamino et al in 1989. 1 PFS typically presents as a flesh-colored, dome-shaped papule on the trunk or extremities of middle-aged to older adults. Rarely, PFS may appear at other sites such as the face and subungual area. Histologically, the lesion is sparsely cellular and composed predominantly of thick, haphazardly arranged collagen. Characteristic features are the presence of scattered, spindle-shaped, or stellate cells, including multinucleated giant cells with large pleomorphic, hyperchromatic nuclei and a small nucleolus. The lesion is almost always 0.5-2.0 cm in diameter and is often mistaken clinically for a nevus, neurofibroma, or hemangioma. Here, we report a case of PFS in an otherwise healthy 50-year-old Japanese man manifesting as a painless, walnut-sized, intradermal nodule on the back that was clinically similar to an epidermal cyst. A 50-year-old Japanese man was referred to our department with a longstanding history of an asymptomatic subcutaneous lump on his back, which had gradually enlarged over a period of approximately 8 years without any history of preceding injury to the area. He was otherwise healthy with an unremarkable medical history. On physical examination, the mass was 3 cm in diameter, slightly raised, mobile, elastic hard, and associated with slight erythema of the overlying skin (Fig. 1A). Ultrasonography revealed an isoechoic to low echoic mass, 25.2× 27.3× 11.9 mm in size, with posterior acoustic enhancement and lateral shadowing (Fig. 1B). The results of routine laboratory investigations were within normal limits. An initial diagnosis of an epidermal cyst was made, and the lesion was surgically removed under local anesthesia. Histopathological examination of the excised specimen showed a hypocellular lesion involving the reticular dermis and extending to the borders abut the subcutis (Fig. 2A). There was neither acanthosis nor hyperpigmentation of the overlying epidermis. The lesion was composed of haphazardly arranged, spindle-shaped cells with fusiform nuclei and eosinophilic cytoplasm, and stellate mononucleated cells with mild cytologic atypia in a collagenous stroma (Fig. 2B). Neither mitotic figures nor necrosis were noted. Immunohistochemistry revealed that the lesional cells expressed vimentin, smooth muscle actin, factor XIIIa, CD99, and CD34 (Fig. 2C) but was negative for S100 protein and desmin. From these findings, we diagnosed the lesion as PFS. No recurrence has been noted at 1-year follow-up. PFS is a rare fibrous tumor characterized by nuclear atypia but with a benign clinical course. Less than 20 reports exist in the medical literature. 2 Immunohistochemically, the tumor cells stain diffusely for vimentin, and a variable number of cells are positive for smooth muscle actin and CD34, suggesting either a myofibroblastic or dermal dendritic cell origin. Immunoreactivities for S100 protein, desmin, and cytokeratins are negative. Some PFSs also have features of sclerotic fibromas. Indeed, some investigators postulate that PFS is actually a variant of sclerotic fibroma. 3 Alternatively, other researchers have labeled these tumors as pleomorphic sclerotic fibromas. 4 In our case, thickened and homogenized eosinophilic collagen bundles arranged in a laminated fashion (whorled pattern) with intervening prominent clefts were absent; therefore, we diagnosed the lesion as PFS rather than sclerotic or pleomorphic sclerotic fibroma.PFS may clinically resemble a nevus, neurofibroma, hemangioma, fibrokeratoma, or fibroepithelial polyp. The initial clinical diagnosis in our case was an epidermal …