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Esthesioneuroblastoma in Maffucci’s syndrome

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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.

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Reference

  1. Lewis RJ, Ketcham AS. Maffucci’s syndrome—functional and neoplastic significance, case report and review of literature. J Bone Joint Surg Am 1993; 15:1465

    Google Scholar 

  2. Lowell SH, Mathog RH. Head and neck manifestations of Maffucci’s syndrome. Arch Otolaryngol 1979; 105:427–430

    CAS  PubMed  Google Scholar 

  3. Albrechts E, Rapini RP. Malignancy in Maffucci’s syndrome. Dermatol Clin 1995; 13:73–78

    CAS  PubMed  Google Scholar 

  4. Burger L, Luc H, Richard R. L’esthesioneuroepitheliome olfactif Bull assoc. Fr Pour Etud Cancer 1924; 13:410–420

    Google Scholar 

  5. Kadish S, Goodman M, Wang CC. Olfactory neuroblastoma: a clinical analysis of 17 cases. Cancer 1976; 37:1571–1576

    CAS  PubMed  Google Scholar 

  6. Schuster JJ, Phillips CD, Levine PA. MR of esthesioneuroblastoma and appearance after craniofacial resection. AJNR Am J Neuroradiol 1994; 15:1169–1177

    CAS  PubMed  Google Scholar 

  7. Goldweig HG, Sundaresan N. Chemotherapy of recurrent esthesioneuroblastoma. Am. J Clin Oncol 1990; 13:139–145

    Google Scholar 

  8. Broich G, Pagliari A, Ottaviani F. Esthesioneuroblastoma; a general review of the cases published since the discovery of the tumor. Anticancer Res 1997; 17:2683–2428

    CAS  PubMed  Google Scholar 

  9. Levine PA, Gallagher R, Cantrell RW (1999) Esthesioneuroblastoma—reflection of A 21-year experience. Laryngoscope 1999; 109:1539–1543

    Article  CAS  PubMed  Google Scholar 

  10. O’ Connor TA, McLean P, Jullard GJ et al. Olfactory neuroblastoma. Cancer 1989; 63:2426–2428

    PubMed  Google Scholar 

  11. Bhattacharyya N, Thornton AF, Joseph MP et al. Successful treatment of esthesioneuroblastoma and neuroendocrine carcinoma with combined chemotherapy and proton radiation. Arch Otolarynol Head Neck Surg 1997; 123:34–40

    CAS  Google Scholar 

  12. McElroy EA Jr, Buckner JC, Lewis JE. Chemotherapy for advanced esthesioneuroblastoma—Mayo Clinic experience. Neurosurgery 1998; 42:1023–1028

    PubMed  Google Scholar 

  13. Polin RS, Sheehan JP et al. The role of pre-operative adjuvant treatment in management of esthesioneuroblastoma: the University of Virginia experience. Neurosurgery 1998; 42:1029–1037

    CAS  PubMed  Google Scholar 

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Correspondence to Sobha Kurian.

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Kurian, S., Ertan, E., Ducatman, B. et al. Esthesioneuroblastoma in Maffucci’s syndrome. Skeletal Radiol 33, 609–612 (2004). https://doi.org/10.1007/s00256-004-0802-x

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  • DOI: https://doi.org/10.1007/s00256-004-0802-x

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