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[Unusual presentation of infantile myofibromatosis with an ulcered plaque]
Insights
Infantile myofibromatosis, a rare tumor in infants, can present atypically. This case highlights a unique presentation as a single, ulcerated plaque, challenging diagnosis.
Area of Science:
- Pediatric Oncology
- Dermatopathology
- Rare Pediatric Tumors
Background:
- Infantile myofibromatosis is a rare fibrovascular tumor appearing before age two.
- It typically presents as isolated or multicentric tumors, potentially affecting bone or organs.
Observation:
- A case of infantile myofibromatosis presented atypically in an infant.
- Initial symptoms included asymptomatic papules on the back, diagnosed histologically as multiple histiocytofibromatous clusters.
- Over one year, these nodules coalesced into a large, ulcerated plaque.
Findings:
- Histological examination revealed a histiocytofibromatous appearance.
- The final diagnosis of myofibromatosis was confirmed after surgical excision of the plaque.
- This presentation of a single, ulcerated plaque is novel in medical literature.
Implications:
- Diagnosing infantile myofibromatosis requires careful clinical and histological correlation.
- The varied clinical presentations underscore the diagnostic challenges.
- This case expands the known spectrum of infantile myofibromatosis.
Introduction:
Infantile myofibromatosis is a rare fibrovascular-like, isolated or multicentric tumor, occasionally of the bone or an organ and appearing before the age of 2. We report a case of infantile myofibromatosis in a child in an atypical form with a single, ulcerated plaque and having developed after the onset of clusters of papular nodules.
Observation:
An infant was seen in consultation because of asymptomatic papules that had developed on the back. The histological examination of a partial biopsy revealed a histiocytofibromatus aspect and led to the diagnosis of clusters of multiple histiocytofibromatous. One year later, the papular nodules had converged, forming a large plaque with ulcerated center. The progressive extension and the absence of healing prompted surgical exeresis and the final diagnosis of myofibromatosis.
Discussion:
Diagnosis of infantile myofibromatosis is difficult histologically and clinically and relies on a clear anatomoclinical confrontation. The clinical aspects are varied. To our knowledge, myofibromatosis with a single ulcerated plaque has never been reported in the literature before.
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