Pennacchia Ilaria, Kutzner Heinz, Kazakov Dmitry V, Mentzel Thomas
Institute of Pathology, Catholic University of the Sacred Heart, Rome, Italy.
Dermopathologie Bodensee, Friedrichshafen, Germany.
J Cutan Pathol. 2017 Oct;44(10):827-834. doi: 10.1111/cup.12993. Epub 2017 Jul 19.
We present herein a series of 14 lesions showing overlapping features with the newly defined benign cutaneous mesenchymal neoplasm labeled as fibroblastic connective tissue nevus (FCTN).
Total of 8 patients were male and 5 were female, ranging in age from 1 to 56 years. Lesions appeared as isolated nodules or plaques on the trunk (7 cases), the limbs (4 cases) and the neck (2 cases). Histologically, all cases were composed of bundles of bland spindle cells of fibroblastic/myofibroblastic lineage irregularly branching within the reticular dermis and along fibrous septa in the subcutis. Adnexal structures and dermal adipocytes were entrapped by the fascicles, the epidermis was often papillomatous and elastic fibers were decreased and fragmented. Expression of CD34 and ASMA was found in 8 and 7 cases, respectively. Follow-up was available for 7 patients (mean follow-up, 5 years; range, 1-10 years). None of the cases metastasized or recurred, even when incompletely excised.
The differential diagnosis of FCTN is broad and includes hypertrophic scar, dermatofibroma, dermatomyofibroma, pilar leiomyoma, plaque-stage DFSP, CD34-positive plaque-like dermal fibroma, fibroblastic-predominant plexiform fibrohistiocytic tumor, lipofibromatosis, superficial desmoid fibromatosis and fibrous hamartoma of infancy, of which it represents probably the monophasic variant.
我们在此展示了一系列14个病变，这些病变具有与新定义的良性皮肤间叶性肿瘤重叠的特征，该肿瘤被标记为成纤维细胞性结缔组织痣（FCTN）。
8例为男性，5例为女性，年龄范围为1至56岁。病变表现为孤立的结节或斑块，位于躯干（7例）、四肢（4例）和颈部（2例）。组织学上，所有病例均由成纤维细胞/肌成纤维细胞谱系的温和梭形细胞束组成，这些细胞束在网状真皮内和皮下纤维间隔中不规则分支。附属结构和真皮脂肪细胞被束状结构包绕，表皮常呈乳头瘤样，弹性纤维减少且断裂。分别在8例和7例中发现CD34和α-平滑肌肌动蛋白（ASMA）表达。7例患者获得随访（平均随访5年；范围1至10年）。即使切除不完全，所有病例均未发生转移或复发。
FCTN的鉴别诊断范围广泛，包括肥厚性瘢痕、皮肤纤维瘤、皮肤肌纤维瘤、毛发平滑肌瘤、斑块期隆突性皮肤纤维肉瘤、CD34阳性斑块样真皮纤维瘤、以成纤维细胞为主的丛状纤维组织细胞瘤、脂肪纤维瘤病、浅表性硬纤维瘤病和婴儿纤维性错构瘤，其中FCTN可能代表单相变体。
