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Case Reports
. 2020 Oct 9:20:100955.
doi: 10.1016/j.ajoc.2020.100955. eCollection 2020 Dec.

Solitary adult orbital myofibroma: Report of a case and review of the literature

Affiliations
Case Reports

Solitary adult orbital myofibroma: Report of a case and review of the literature

Nicole C Morrow et al. Am J Ophthalmol Case Rep. .

Abstract

Purpose: Myofibromas are benign soft tissue tumors commonly encountered in infancy and childhood. Developing usually within the first two years of life, they can be multicentric and involve deep visceral organs.

Observations: We present the rare occurrence of a solitary orbital myofibroma in an adult patient. The clinical, histopathologic and immunohistochemical findings of the tumor are documented.

Conclusions: A comprehensive review of pediatric and adult orbital and periocular involvement by myofibroma is presented. Its characteristic pathologic and molecular findings are reviewed.

Importance: Myofibromas are uncommon but important tumors that can occur in the head and neck region, including the orbit. Seen more often in children, they can rarely be encountered in adult patients. Diagnosis is possible with a panel of immunostains and molecular analysis can be further confirmatory.

Keywords: Myofibroma; Myofibromatosis; Myopericytoma; PDGFRB mutation.

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Conflict of interest statement

Anand Rajan KD previously served as a member of a Scientific Advisory Board to Roche Diagnostic Corporation. The following authors have no financial disclosures: NCM, MRT, NAS.

Figures

Fig. 1
Fig. 1
Sagittal T1-weighted post-contrast magnetic resonance image (MR) showing an orbital mass involving the left orbit, denoted by white arrows.
Fig. 2
Fig. 2
A – H&E stain, 100x original magnification, low-power photomicrograph showing architectural features of myofibroma with haphazard bundles of spindled cells arranged around numerous vascular channels. B–H&E stain, 200x original magnification, higher-power showing bland oval-to-spindle tumor cells with minimal pleomorphism. C – Immunohistochemistry for smooth muscle actin (SMA) highlights smooth muscle in vascular walls and tumor cells. D – Desmin shows strong cytoplasmic positivity in tumor. E − CD34 highlights endothelial cells in vascular channels and is negative in tumor. F – STAT6 shows weak cytoplasmic expression and is negative in tumor nuclei.

References

    1. Wiswell T.E., Sakas E.L., Stephenson S.R., Lesica J.J., Reddoch S.R. Infantile myofibromatosis. Pediatrics. 1985;76:981–984. - PubMed
    1. Martignetti J.A., Tian L., Li D. Mutations in PDGFRB cause autosomal-dominant infantile myofibromatosis. Am J Hum Genet. 2013;92:1001–1007. - PMC - PubMed
    1. Servat J., Williamson J., Piepmeier J., Sinard J., Bernardino R.C. Giant myofibroma of the orbit in an adult male. Orbit. 2011;31:21–23. - PubMed
    1. Hemalatha A.L., Sindhuram V.A., Asha U. Myfibroma which was associated with A rudimentary eyeball, which masqueraded as an ocular palpebral cyst. J Clin Diagn Res. 2013;7:557–559. - PMC - PubMed
    1. Duffy M.T., Harris M., Hornblass A. Infantile myofibromatosis of orbital bone A case report with computed tomography, magnetic resonance imaging, and histologic findings. Ophthalmology. 1997;104:1471–1474. - PubMed

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