Orbital and periorbital myofibromas in childhood: Two case reports

Andrew C. Westfall, Atiya Mansoor, Scot A. Sullivan, David J. Wilson, Roger A. Dailey

Research output: Contribution to journalArticle

19 Scopus citations

Abstract

Purpose: Infantile myofibromatosis is an uncommon tumor that occurs rarely in the periorbit and orbit. This article reports two cases of infantile myofibromatosis of the orbital adnexa and describes the associated clinical, histopathologic, and immunohistochemical findings. Design: Two retrospective, interventional case reports with clinicopathologic correlation. Intervention: Treatment consisted of excision of the tumors. Main Outcome Measures: Histologic and immunohistochemical evaluation and clinical evaluation for tumor recurrence. Results: The first patient was a newborn male with a large tumor extending from his eyelid that was excised at day 2 of life. Histologic and immunohistochemistry analyses were used to make a diagnosis of infantile myofibromatosis. He remains disease free at age 7 years. The second case was a 6-year-old boy with a 1 -month history of proptosis resulting from an orbital mass. Incisional biopsy revealed a tumor consistent with infantile myofibromatosis. He remains tumor free 12 months after complete gross surgical resection. Conclusions: Infantile myofibromatosis is an uncommon tumor that is rare in the orbit. Differential diagnosis can be difficult based solely on histologic analysis. Immunohistochemistry evaluation demonstrating cytoplasmic actin filaments within neoplastic spindle cells confirms the diagnosis. As soon as the diagnosis is made, chest and abdominal imaging is of value to define the prognosis and to direct further treatment. After the diagnosis of nonvisceral infantile myofibromatosis, complete gross resection, if possible, is the treatment of choice.

Original languageEnglish (US)
Pages (from-to)2000-2005
Number of pages6
JournalOphthalmology
Volume110
Issue number10
DOIs
StatePublished - Oct 1 2003

ASJC Scopus subject areas

  • Ophthalmology

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