Esthesioneuroblastoma in Maffucci's syndrome

Skeletal Radiology
Sobha KurianChristopher Rassekh

Abstract

Maffucci's syndrome consists of multiple cutaneous hemangiomas, dyschondroplasia, and enchondromas with potential for malignant change. We report a case of a 33-year-old man with Maffucci's syndrome who presented with a several month history of nasal congestion, facial pain, and diminished vision in his left eye. Radiological studies showed a large soft tissue mass centered in the sinonasal area, extending bilaterally into maxillary sinuses and orbits with compression of left optic nerve. Biopsy of the mass showed esthesioneuroblastoma (olfactory neuroblastoma). Chemotherapy resulted in initial improvement, but the tumor recurred and did not respond to further treatment, resulting in his death. Sarcomatous tumors are reported in Maffucci's syndrome, but this is a rare case of a neuroendocrine tumor in a patient with Maffucci's syndrome.

Citations

Sep 1, 1996·International Journal of Pediatric Otorhinolaryngology·Q A NguyenD M Crockett
Nov 4, 2008·Journal of Neurosurgery·Joana Ruivo, João Lobo Antunes
Jan 27, 2005·The Australasian Journal of Dermatology·Victoria ShepherdTerence Casey
Jun 25, 2019·World Neurosurgery·Soliman OushyJamie J Van Gompel

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