Ulcerated congenital plexiform fibrohistiocytic tumor: Case report and literature review

Abstract

A newborn boy presented with a progressively infiltrating and painful congenital ulcerated plaque on the back of his left foot. A partial excision was performed and histopathologic examination confirmed a diagnosis of a plexiform fibrohistiocytic tumor. This rare tumor usually appears in children and adolescents, with congenital presentations even more uncommon. This case details the exceptional presentation of a congenital ulcerated plexiform fibrohistiocytic tumor with a review of the current literature.

Solitary nodule on the shoulder of an 11-year-old child

“…A congenital origin has been described in only very few patients [3][4][5]. Despite a female prevalence being initially suggested, recent studies demonstrate a similar distribution between genders [2][3][4].…”

“…Although its age of presentation ranges from two months to 77 years [2,3], PFHT shows a distinct predilection for children and young adults, with 70% of cases occurring in individuals under 20 years of age [3]. A congenital origin has been described in only very few patients [3][4][5]. Despite a female prevalence being initially suggested, recent studies demonstrate a similar distribution between genders [2][3][4].…”

“…It clinically presents as a painless, solitary, slowgrowing dermal or subcutaneous nodule [1][2][3][4][5][6] or, less frequently, as a firm, indurated plaque [4][5][6]. In rare instances, ulceration can develop [4]. Up to 50% of cases occur on the upper extremities, followed by the lower extremities, trunk, and head and neck regions [1,5,6].…”

“…The differential diagnosis is vast and must include other lesions with a plexiform growth pattern, namely fibrous hamartoma of infancy, plexiform neurofibroma, plexiform spindle cell nevus, fibromatosis (extra-abdominal desmoid), soft tissue giant cell tumor, or cellular neurothekeoma [1,[4][5][6].…”

“…The local recurrence rate is high and ranges from 12.5 to 50% in the published literature [4][5][6], reflecting the infiltrative nature of the tumor and the consequent difficulty in achieving a complete excision [6]. Regional lymph node metastases have been reported in 2.4% of cases.…”