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Chondrosarcoma of the nasal septum.

Abstract

Chondrosarcoma of the nasal septum is a rarely encountered malignancy. When it does occur, early diagnosis is difficult because patients generally present with common, nonspecific sinonasal complaints. We describe a case of chondrosarcoma of the nasal septum in a 38-year-old Hispanic man. The lesion was discovered on computed tomography during a workup for symptoms of chronic sinus disease. The tumor was removed in its entirety and identified as a low-grade lesion that required no further treatment. The patient remained disease-free 4 years postoperatively. We review the diagnosis of this neoplasm and its treatment and follow-up.

Introduction

Head and neck chondrosarcoma accounts for only 10% of all cases of chondrosarcoma. (1) In the head and neck, most of these neoplasms occur in the larynx, maxilla, and skull base. Chondrosarcoma of the nasal septum is very rare; our review of the English-language literature revealed that only about 50 cases have been reported to date. Nasoseptal chondrosarcomas can occur at any time from the second through the eighth decades of life, and they occur equally in males and females. (1,2) We report a new case of chondrosarcoma of the nasal septum in a middle-aged man.

Case report

A 38-year-old Hispanic man presented with complaints of nasal obstruction, postnasal drip, and persistent occipital headaches of several months' duration. Fiberoptic nasopharyngoscopy detected diffuse polypoid disease in the right middle turbinate and bilateral septal spurs. Coronal computed tomography (CT) of the paranasal sinuses demonstrated a benign-appearing bony expansion of the superior aspect of the nasal septum (figure). The expansion measured 1.5 cm in its transverse diameter and 2 cm in length from anterior to posterior.

The patient was taken to the operating room, and the tumor was exposed through a hemitransfixion incision with dissection and preservation of bilateral mucoperichondrial and mucoperiosteal flaps. The mass was removed completely. Final histopathologywas consistent with a grade I (low-grade) chondrosarcoma. No other treatment was deemed necessary.

The patient was followed with nasal endoscopy and CT--twice yearly at first and later once yearly. At 4 years postoperatively, he showed no evidence of recurrence.

Discussion

Chondrosarcoma was first distinguished as a separate entity from osteosarcoma by Ewing in 1939. (3) Four years later, Lichtenstein and Jaffe established the histologic criteria that led to the proper diagnosis of this neoplasm. (4)

Chondrosarcomas arise from either chondrocytes, embryonal rests, or mesenchymal cells that undergo multidirectional differentiation. (1) Histologically, they are categorized into three grades on the basis of a particular tumor's degree of cellularity, nuclear size and atypia, and mitotic activity; grade I tumors are considered low-grade tumors, and grade II and grade III neoplasms are considered high-grade tumors. (1) The histologic distinction between a low-grade chondrosarcoma and a chondroma is very difficult to make. The reported rate of histologic uncertainty between low-grade chondrosarcomas and benign chondromas is 10%. (5) Some authors advocate that all neoplasms that contain hyaline cartilage should be considered a chondrosarcoma until proven otherwise. (5,7) Indicators of the benign nature of chondrosarcomas are the pathologic features of the entire lesion and an absence of recurrence or metastasis after follow-up of several years' duration. (7)

The clinical presentation of a chondrosarcoma of the nasal septum is nonspecific. In cases where the tumor is confined to the sinonasal cavities, the symptoms are similar to those of chronic sinus disease. While the tumor's behavior is variable, its growth for the most part is slow, as the reported duration from the onset of symptoms to diagnosis ranges between 3 months and 1 year. (2,5)

Radiographic imaging is imperative prior to surgical intervention. Chondrosarcomas demonstrate characteristic findings on both CT and magnetic resonance imaging (MRI), and the two studies complement one another. (l) On CT, a chondrosarcoma usually appears as a hypodense lesion with scattered, ring-forming calcifications that suggest bone erosion. MRI is particularly helpful in assessing intracranial or intraorbital extension, in visualizing the tumor in a multiplanar fashion, in determining the extent of any soft-tissue involvement, and in differentiating between tumor and inspissated secretions. In general, a chondrosarcoma appears as a low-intensity signal on T1-weighted MRI and as a high-intensity signal on T2-weighted imaging; heterogeneous enhancement is seen with gadolinium. (6)

Wide surgical excision is the treatment of choice for head and neck chondrosarcoma. When a lesion arises primarily from the nasal septum, the tumor can be approached in a multitude of ways, including a Weber-Fergusson incision, a lateral rhinotomy, a sublabial transnasal approach, a LeFort I downfracture, an anterior craniofacial approach, an endoscopic resection, and a transseptal approach. (7) The goal of surgery is to achieve histologically clear margins, as recurrence rates have been reported to be as high as 65% when margins are positive (all grades included). (1) Postoperative radiation therapy is recommended for patients with positive margins, high-grade lesions, or recurrent disease and for palliation. (7) Chemotherapy as a primary or adjuvant therapy has not been proven to be beneficial. (6) Overall survival at 5 years for all grades combined ranges from 54 to 81%. (6) A high-grade lesion, positive surgical margins, and a nasopharyngeal or posterior nasal cavity location all indicate a poorer prognosis. (7)

Recurrences after long disease-free intervals have been reported; one recurrence developed 20 years after initial treatment. (6) Therefore, lifelong follow-up with imaging studies and endoscopic examinations is recommended.

The specimen obtained in the present case was entirely examined by a pathologist, who confirmed the low-grade nature of the lesion. Margins were close but clear anteriorly, posteriorly, superiorly, and inferiorly. The left and right margins where the lesion encountered the mucous mucoperichondrial flaps were not assessable. The patient was not returned to the operating room for a resection of the mucoperichondrium once the diagnosis of a low-grade tumor was confirmed. He remained disease-free 4 years postoperatively under regular follow-up.

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References

(1.) Coppit GL, Eusterman VD, Bartels J, Downey TJ. Endoscopic resection of chondrosarcomas of the nasal septum: A report of 2 cases. Otolaryngol Head Neck Surg 2002;127(6):569-71.

(2.) McCoy JM, McConnel FM. Chondrosarcoma of the nasal septum. Arch Otolaryngol 1981;107(2):125-7.

(3.) Ewing JA. Review of the classification of bone tumors. Surg Gynecol Obstet 1939;40:971-6.

(4.) Lichtenstein L, Jaffe HL. Chondrosarcoma of bone. Am J Pathol 1943;19:553-89.

(5.) Walshe P, Timon C. Chondrosarcoma of the nasal septum. Ir Med J 2002;95(3):90-1.

(6.) Kaufman JK, Pritz MB, Righi PD, Bizal JC. Craniofacial resection of a nasoseptal chondrosarcoma: Case report and review of the literature. Surg Neurol 1999;52(3):265-8; discussion 268-9.

(7.) Downey TJ, Clark SK, Moore DW. Chondrosarcoma of the nasal septum. Otolaryngol Head Neck Surg 2001; 125(1):98-100.

Akram Rahal MD, FRCSC; Joseph R. Durio, PA-C; Michael L. Hinni, MD

From the Department of Otolaryngology-Head and Neck Surgery, Mayo Clinic Arizona, Scottsdale.

Corresponding author: Michael L. Hinni, MD, Mayo Clinic Arizona, 5777 E. Mayo Blvd., Phoenix, AZ 85054. Phone: (480) 342-2928; fax: (480) 342-2626; e-mail: hinni.michael@mayo.edu
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Title Annotation:ORIGINAL ARTICLE
Author:Rahal, Akram; Durio, Joseph R.; Hinni, Michael L.
Publication:Ear, Nose and Throat Journal
Article Type:Case study
Date:Jan 1, 2009
Words:1155
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