An Algorithmic Approach to the Management of Infantile Digital Fibromatosis

Review of Literature and a Case Report

Elizabeth H. Eypper, MD; Johnson C. Lee, MD; Ashley J. Tarasen, MD; Maxene H. Weinberg, BS; Oluwaseun A. Adetayo, MD

Disclosures

ePlasty. 2018;18(e19) 

In This Article

Abstract and Introduction

Abstract

Objective: Infantile digital fibromatosis is a rare benign childhood tumor, infrequently cited in the literature. Hallmarks include nodular growths exclusive to fingers and toes and the presence of eosinophilic cytoplasmic inclusions on histology. This article aims to exemplify diagnoses of infantile digital fibromatosis and possible treatment options.

Methods: A computerized English literature search was performed in the PubMed/MEDLINE database using MeSH headings "infantile," "juvenile," "digital," and "fibromatosis." Twenty electronic publications were selected and their clinical and histological data recorded and used to compile a treatment algorithm.

Results: A 9-month-old male child was referred for a persistent, symptomatic nodule on the third left toe. A direct excision with Brunner-type incisions was performed under general anesthesia. The procedure was successful without complications. The patient has no recurrence at 2 years postsurgery and continues to be followed. Histological examination revealed a proliferation of bland, uniformly plump spindle cells with elongated nuclei and small central nucleoli without paranuclear inclusions consistent with fibromatosis.

Conclusions: Asymptomatic nodules should be observed for spontaneous regression or treated with nonsurgical techniques such as chemotherapeutic or steroid injection. Surgical removal should be reserved for cases with structural or functional compromise.

Introduction

Infantile digital fibromatosis (IDF) is a rare, benign tumor of myofibroblasts occurring on the fingers and toes in early childhood. As a rare entity, available literature describing diagnostic criteria and treatment options for IDF is proportionally scarce. Reye[1] first classified these tumors in 1965, noting that they can be distinguished from other fibrous growths on the basis of their common anatomic location, presenting age group, and pathology. Infantile digital fibromatosis typically appears as singular or multiple nodules on the digits within the first year of life but may also be present at birth in up to one third of patients.[1–3] The nodules are typically firm, less than 2 cm in diameter, and often painless, although some cases present with decrease in or loss of joint function or discomfort.[2–9] Larger tumors may additionally begin to compromise adjacent digits with long-term functional consequences.[7,10]

Histopathological diagnosis is usually based on a few characteristic findings: cellular proliferation restricted to the dermis; interlacing fascicles extending to the epidermis; and tumor cells surrounding the adnexal structures.[3] The most pathognomonic feature is the presence of eosinophilic paranuclear inclusions consisting of an actin aggregate, although they are not necessary for a diagnosis of IDF if the clinical picture and other histological findings are standard.[3]

Treatment of IDF has also been varied, owing to a paucity of data on remission rates and different treatment modalities. Numerous case reports have demonstrated success with either surgical excision of nodules or a more conservative observational approach. Surgical intervention has been widely effective, although recurrence remains a problem in up to 61% to 74% of cases.[2,3] Additional studies reporting on more aggressive excision techniques have nevertheless shown a lower recurrence rate, closer to 13% to 36%.[11–14] Other approaches to treatment have included topical steroids, intralesional steroids, and intralesional 5-fluorouracil.[6,15,16]

Here, we describe a case of IDF in a 9-month-old male child presenting with a painful nodule on the dorsum of his left third toe, summarize case management, and review the available literature to provide a management algorithm for IDF (Table 1).

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