Sandrini Jérémy, Michalak Sophie, Croué Anne, Hubault Pascale, Rousselet Marie-Chistine
Département de pathologie, CHU d'Angers, 4, rue Larrey 49033 Angers cedex 01, France.
Ann Pathol. 2011 Jun;31(3):222-5. doi: 10.1016/j.annpat.2011.02.011. Epub 2011 May 19.
We report the unusual case of a three-year-old girl which presented since birth a pigmented tumor of the left side 0.5cm in diameter. Surgical removal was decided given the hypothesis of a congenital naevo-cellular naevus. The histological study ended with the diagnosis of plexiform fibrohistiocytic tumor (PFHT). Two other congenital PFHT have been reported until now. PFHT is a rare mesenchymal neoplasm of intermediate malignancy, first reported by Enzinger and Zhang in 1988 which may be difficult to diagnose, because of its low frequency, particulary in congenital cases. It is important to distinguish it from others childhood cutaneous tumors (particulary plexiform neurofibroma, cellular neurothekeoma, infantile myofibromatosis, and fibrous hamartoma). The tumor has a high local recurrence rate and complete surgical resection of the tumor, with wider margins, is required.
我们报告了一例罕见病例，一名三岁女童自出生以来左侧有一个直径0.5厘米的色素性肿瘤。鉴于先天性痣细胞痣的假设，决定进行手术切除。组织学研究最终诊断为丛状纤维组织细胞瘤（PFHT）。到目前为止，另外两例先天性PFHT也有报道。PFHT是一种罕见的中间恶性间叶性肿瘤，1988年由恩津格和张首次报道，由于其发病率低，尤其是先天性病例，可能难以诊断。将其与其他儿童皮肤肿瘤（尤其是丛状神经纤维瘤、细胞性神经鞘瘤、婴儿肌纤维瘤病和纤维性错构瘤）区分开来很重要。该肿瘤局部复发率高，需要对肿瘤进行完整的手术切除，并切除更宽的边缘组织。
