Chondrosarcoma of the nasal cavity in a patient with Maffucci syndrome: case report and review of the literature

Abstract

IntroductionMaffucci syndrome is a rare, congenital, non-hereditary mesodermal dysplasia, manifested by multiple enchondromas and hemangiomas. Malignant transformation of these lesions is seen in up to 40% of the cases.Case reportWe present a case of a patient with Maffucci syndrome and an associated chondrosarcoma of the nose. Treatment consisted of surgical resection. Because of the low grade of the tumor, additional treatment, such as radiotherapy, was not necessary.ConclusionMaffucci syndrome is an exceedingly rare mesodermal dysplasia. Its manifestation in the head and neck region is even less common. Malignant transformation of the associated enchondromas is common, and should be considered whenever a change of the clinical course occurs. Random, periodically performed X-ray examinations give little additional information on malignant transformation and are considered useless.

Rare case of nasal cavity chondrosarcoma presenting only with unilateral decreased vision

“…The most common presenting symptoms are local swelling and pain, 2,17 but due to the proximity of the orbit, ocular symptoms and signs may appear first in cases of malignancy in paranasal sinuses. Most of the cases of nasal cavity chondrosarcoma report nasal obstruction and congestion as the leading symptom 4,5,8‐10 . There are only few reports of nasal cavity and paranasal sinuses chondrosarcoma presenting with ocular pathology 18,19 .…”

“…Most of the cases of nasal cavity chondrosarcoma report nasal obstruction and congestion as the leading symptom. 4 , 5 , 8 , 9 , 10 There are only few reports of nasal cavity and paranasal sinuses chondrosarcoma presenting with ocular pathology. 18 , 19 Due to their relatively poor response to conventional radiotherapy and chemotherapy, surgical resection is the main modality of treatment, 7 but desired complete resection is not often feasible due to the proximity to vital structures, which was the case in our patient.…”

“…[4][5][6] Sinonasal chondrosarcoma is usually diagnosed in the third and fourth decade of life, 7,8 with nasal obstruction and congestion as the most frequently documented initial symptoms. 5,[8][9][10] We report a case of a 57-year-old patient with a massive chondrosarcoma of the nasal cavity, extending to paranasal sinuses and orbit, primarily presenting with unilateral vision deterioration.…”

Tracheal Chondrosarcoma Excised With Extracorporeal Circulation

“…The age of presentation is around the 6th decade of life, but there are 2 cases described at a much younger age; appearing in patients with Maffucci Syndrome [6,8]. This syndrome is characterized by diffuse enchondromatosis and cutaneous haemangiomas distributed throughout the body, even though head and neck involvement is not so common [21]. These tumors are not often presented with metastasis.…”

Maffucci’s syndrome in association with giant tubular adenoma of the breast: Case report and literature review

“…This case has been reported in accordance with the surgical case report guidelines (SCARE) criteria [1]. Maffucci’s syndrome is a congenital, non-hereditary mesodermal dysplasia characterized by multiple enchondromas and vascular lesions [2]. Affected patients suffer from physical deformity resulting in aesthetic and functional sequelae and as well as pathological fractures.…”