Chondrosarcoma

Abstract

Advances in endoscopic sinus surgery technique and technology have provided the otolaryngologist with a wide array of surgical options for the management of nasal and paranasal sinus diseases that were once limited to procedures requiring invasive surgery. We describe the case of a patient with a chondrosarcoma of the posterior nasal septum that was resected endoscopically. There have been fewer than 30 reports in the English-language literature of chondrosarcomas arising at this location, and the reported approaches to surgical excision in these cases involved craniofacial procedures, facial degloving, facial splitting, and maxillectomy techniques. Until now, no case of endoscopic removal has been reported.

Introduction

Chondrosarcomas of the head and neck are uncommon. A 10-year review of 355,019 cases of head and neck cancers compiled in the National Cancer Database revealed that only 400 of these tumors (0.1%) were chondrosarcomas. (1) A review by Rassekh et al found that there have been fewer than 30 reports in the English-language literature of chondrosarcomas arising from the nasal septum. (2)

Surgical resection is generally regarded as the primary treatment for head and neck chondrosarcomas. The only surgical approaches that have been reported thus far are craniofacial procedures, facial degloving, facial splitting, and various types of maxillectomy. (2-7) Until now, there had been no report of endoscopic resection of a nasal septal chondrosarcoma. In this article, we report our use of this technique in a patient with a nasal septal chondrosarcoma.

Case report

A 73-year-old woman came to us with a 5-month history of a progressive right-sided nasal obstruction, postnasal drip, and a thick mucoid nasal discharge. On examination, the patient was noted to have an obstructing mass in the posterior portion of the right nasal cavity that appeared to be arising from the posterior septum (figure 1).

Computed tomography (CT) revealed the presence of a 2.8 x 2.4 x 3.3-cm soft-tissue mass that was centered on the posterior septum. The mass bowed the medial wall of the right maxillary sinus and extended into the anterior nasopharynx (figure 2). It eroded the floor of the right sphenoid sinus, and there was a small amount of extension into the sphenoid sinus. However, no skull base erosion was identified.

An intranasal endoscopic biopsy of the septal mass was performed. Frozen-section evaluation revealed an atypical cartilaginous neoplasm with tapering chondrocytes and a grade 2 cellularity with nuclear atypia (figure 3). In addition, the presence of scattered mitotic figures and a large focus of necrosis led to the pathologic diagnosis of a chondrosarcoma.

The chondrosarcoma's relatively small size, intranasal location, and lack of skull base invasion led us to attempt an intranasal endoscopic resection as opposed to a traditional surgical approach. The patient was positioned and draped in a fashion similar to that used during endoscopic sinus surgery. Decongestion was administered with oxymetazoline and 1% lidocaine with 1:100,000 epinephrime. The nasal cavity was visualized through a 0[degrees] Storz Hopkins endoscope attached to a video unit. Tm-Cut forceps were used to resect the middle turbinate and expose the chondrosarcoma. A Freer elevator was then used to obtain a clear plane of dissection from the adjacent nasal mucosa. The chondrosarcoma was left tethered to the posterior nasal septum. A 1-cm margin along the septum was obtained by removing the posterior septum from the face of the sphenoid sinus to the nasal floor with the Tm-Cut forceps. The anterior wall of the sphenoid sinuses was then clearly visible. The faces of both sphenoid sinuses were remo ved with Kerrison's forceps and a Xomed microdebrider. No further tumor was visualized, and multiple frozen sections revealed a clear surgical margin.

The patient was discharged home on postoperative day 1. One week later, she was hospitalized for 2 days after undergoing ligation of the left sphenopalatine artery to control epistaxis. Since then, surveillance of the posterior nasal cavity has been easily accomplished by office-based rigid endoscopy (figure 4). The patient has done well 27 months after surgery, and she shows no clinical evidence of recurrent disease.

Discussion

Since Wigand's use of the optical endoscope for intranasal surgery, otolaryngologists have adopted new advances in technology that provide better illumination and visualization for the management of nasal and paranasal sinus diseases. Modern endoscopic sinus surgery equipment allows the otolaryngologist to take minimally invasive intranasal approaches to disease processes that were once limited to treatment by external approaches. Our report of a nasal septal chondrosarcoma resected via an intranasal approach with endoscopic techniques and equipment is testimony to the technologic improvements in fiberoptic endoscopes, monitoring systems, and endoscopic surgical technique. All previously published reports of surgical excisions of nasal septal chondrosarcomas involved craniofacial procedures, facial degloving, facial splitting, and various types of maxillectomy. (2-7)

Chondrosarcomas are malignant tumors of cartilaginous origin. They occur infrequently in the head and neck region, and they are extremely rare in the nasal septum. In fact, the largest series of nasal septal chondrosarcomas reported to date numbered only six patients. (2) Symptoms are typically caused by mass effect or erosion of adjacent structures. Sinonasal chondrosarcomas have been noted to cause nasal obstruction, headache, and cranial neuropathies.

CT usually demonstrates a hypodense mass with areas of spotty calcification, and it can determine the extent of bony destruction. Magnetic resonance imaging has been shown to accurately define tumor extent and intracranial involvement. Histopathologic specimens are needed to make a definitive diagnosis and to grade a tumor on the basis of its histologic characteristics (i.e., grade 1, 2, or 3, depending on nuclear size, atypia, mitotic activity, and cellularity). A correlation has been made between low-grade chondrosarcomas and longer survival. (1, 2) The primary treatment of head and neck chondrosarcomas is radical surgical resection with clear margins. The use of chemo- and radiotherapy is limited.

Our review of the literature on nasal septal chondrosarcomas revealed that they are typically diagnosed at a late stage because of the nonspecific nature of their symptoms. Reports of the size of chondrosarcomas on initial evaluation have varied. We were fortunate to have discovered our patient's nasal septal chondrosarcoma at a relatively early stage.

Four factors led us to offer our patient this minimally invasive endoscopic resection approach: (1) the small size of the tumor, (2) the location of the tumor, which afforded excellent visualization with endoscopic equipment, (3) the lack of skull base involvement, and (4) the ability to easily conduct surveillance for recurrent disease in the office with rigid endoscopy. Although we were able to endoscopically accomplish a complete tumor resection with clear margins, we did obtain preoperative consent to switch to a traditional maxillectomy if the need had arisen.

We certainly do not advocate that endoscopic removal of nasal septal chondrosarcomas be performed routinely. Nevertheless, in very carefully selected patients (e.g., those with small tumors and a lack of skull base invasion), endoscopic resection offers a less-invasive means of surgical excision.

References

(1.) Koch BB, Karnell LH, Hoffman HT, et al. National cancer database report on chondrosarcoma of thc head and neck. Head Neck 2000;22:408-25.

(2.) Rassekh CH, Nuss DW, Kapadia SB, et al. Chondrosarcoma of the nasal septum: Skull base imaging and clinicopathologic correlation. Otolaryngol Head Neck Surg 1996;115:29-37.

(3.) Gray R, Leonard G. Chondrosarcoma of the nasal septum. J Laryngol Otol 1977;91:427-31.

(4.) Peppard SB, Matz GJ. Chondrosarcoma of the nasal septum extending into the maxillary sinus. Otolaryngol Head Neck Surg 1979;87:635-9.

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

(6.) Bailey CM. Chondrosarcoma of the nasal septum. J Laryngol Otol 1982;96:459-67.

(7.) Nishizawa S, Fukaya T, Inouye K. Chondrosarcoma of the nasal septum: A report of an uncommon lesion. Laryngoscope 1984;94:550-3.

From the Department of Otolaryngology, Baptist Medical Center, Wake Forest University, Winston-Salem, N.C.

Reprint requests: Brian Matthews, MD, Department of Otolaryngology, Medical Center Blvd., Winston-Salem, NC 27157-1034. Phone:(336)716-3902; fax:(336)716-9440; e-mail: bmatthew@wfubmc.edu

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