Maffucci Syndrome with Nasal Enchondroma: A Rare Entity - Case Report
Author Shruti Bansal, Arpit Sharma, JP Dabholkar
ISSN 0974-4630
JournalTitle Clinical Rhinology An International Journal
DOI 10.5005/jp-journals-10013-1278
Volume 9
Issue 2
Publishing Year 2016
Pages 94-97
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ABSTRACT

Introduction: Maffucci syndrome is a congenital nonhereditary disorder in which patients develop multiple enchondromas and cutaneous, visceral, or soft tissue hemangiomas. Less than 200 cases of Maffucci syndrome have been published in the English literature. These lesions most commonly occur in the limb bones, especially in the hands and feet. Rarely, patients of Maffucci syndrome may present with mesodermal dysplasia involving head and neck region with nasal septum enchondromas reported only in two cases till now.

Aim: To report a rare case of nasal enchondroma in a patient of Maffucci syndrome.

Case description: A 15-year-old girl diagnosed with Maffucci syndrome 2 years back was referred to our ENT department with symptoms of left-sided nasal obstruction and epiphora. On thorough clinical examination and proper investigation, she was diagnosed to have nasal enchondroma. She successfully underwent endonasal endoscopic resection of nasal enchondroma.

Conclusion: Maffucci syndrome is a rare entity characterized by multiple enchondromas and hemangiomas, especially in the extremities. A manifestation in the head and neck region is rare and cartilaginous tumors of nasal septum are even rarer; however, differential of nasal cartilaginous tumors should be borne in mind in patients with underlying mesodermal dysplastic disorders like Maffucci syndrome.

Clinical significance: Patients of Maffucci syndrome with enchondromas are at high risk of undergoing malignant transformation, especially chondrosarcomas. These patients need vigilant and close follow-up. Despite their rarity, chondromas should be taken into consideration in the differential diagnosis of nasal tumors, especially those arising from the nasal septum.

Keywords: Maffucci syndrome, Mesodermal dysplasia, Nasal enchondromas.

How to cite this article: Bansal S, Sharma A, Dabholkar JP. Maffucci Syndrome with Nasal Enchondroma: A Rare Entity. Clin Rhinol An Int J 2016;9(2):94-97.

Source of support: Nil

Conflict of interest: None

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