title: Esthesioneuroblastoma in Maffucci's syndrome.
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.
authors.affiliation: Section of Hematology/Oncology, Department of Medicine, Health Science Center, West Virginia University, Morgantown, WV 26506, USA. skurian@hsc.wvu.edu
authors.forename: Sobha
authors.initials: S
authors.lastname: Kurian
authors.forename: Esmer
authors.initials: E
authors.lastname: Ertan
authors.forename: Barbara
authors.initials: B
authors.lastname: Ducatman
authors.forename: Edward B
authors.initials: EB
authors.lastname: Crowell
authors.forename: Christopher
authors.initials: C
authors.lastname: Rassekh
mesh_terms: D000328:Adult; D004687:Enchondromatosis / Q000150:complications*; D018304:Esthesioneuroblastoma, Olfactory / Q000175:diagnosis*; D017809:Fatal Outcome; D006801:Humans; D008297:Male; D008444:Maxillary Sinus Neoplasms / Q000175:diagnosis; D009296:Nasal Cavity / Q000473:pathology*; D009364:Neoplasm Recurrence, Local / Q000473:pathology; D009669:Nose Neoplasms / Q000175:diagnosis*; D009918:Orbital Neoplasms / Q000175:diagnosis