Plaque‐Like Myofibroblastic Tumor: Report of Three Cases

Abstract

Plaque-like myofibroblastic tumor of infancy (PMTI) was first reported in 2007. The first two cases described large, plaque-like tumors presenting in infancy with microscopic features consistent with dermatofibroma but with immunohistochemical features of myofibrocytic lineage. We present three additional cases of PMTI, the first cases reported since the initial two cases, and describe additional clinical features of this condition, including presentation in early childhood as opposed to infancy, development of ulceration, and aggressive growth. We propose shortening the name of this condition to plaque-like myofibroblastic tumor because presentation can occur in infancy or in early childhood.

Plaque‐like myofibroblastic tumor, a rare entity of childhood: Possible pitfalls in differential diagnosis

“…It appears as a slowly growing plaque reaching sometimes several (2‐9) centimeters in diameter, made up of multiple nodules. PLMT is generally asymptomatic, sometimes itchy, or mildly painful …”

“…A peculiar variant of DF, more similar to PLMT, is represented by multiple clustered DFs (MCD) . MCD appears as multiple 0.5 to 1 cm reddish brown papules clustered in one anatomical area larger than 5 cm, that usually merge into one plaque or are arranged in a linear pattern.…”

“…MCD appears as multiple 0.5 to 1 cm reddish brown papules clustered in one anatomical area larger than 5 cm, that usually merge into one plaque or are arranged in a linear pattern. MCD is mainly located on the hip or upper legs; it is a rare condition appearing during adolescence, but congenital cases were rarely reported. The grouped papules grow over many years and then stabilize.…”

“…DFSP manifests as slow‐growing firm protuberant brown/blue solitary or multiple, scattered, confluent nodules of the trunk or the proximal extremities; DFSP typically occurs in the third to fifth decades of life, but some infantile or early childhood cases were reported. DFSP is rarely congenital and occasionally presents as an atrophic plaque . Histological examination is characterized by densely packed spindle cells arranged in a storiform pattern and infiltrating the subcutis …”

Beyond infections: tumors and malformations of the diaper area

“…Another entity that may be part of the clinical spectrum of PMTI is known as multiple clustered dermatofibromas, which predominately affects children and young adults. The preferred treatment for the condition is to remove it completely 16 ( Fig. 2).…”