Journal of Otology & RhinologyISSN: 2324-8785

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Case Report, J Otol Rhinol Vol: 5 Issue: 5

Clinical Diagnosis and Management of Septal Chondrosarcoma

Osama A Marglani1, Omar A Abu Suliman2*, Rafat I Abu Shakra3, Maryam M Fairag4 and Fatimah Y Sabbagh4
1Faculty of Medicine, Umm Al Qura University, Makkah, Saudi Arabia, Consultant Otolaryngology Head and Neck Surgery Head of Department at King Abdullah Medical City, Makkah, Saudi Arabia
2Otolaryngology, Head and Neck surgery, King Abdullah Medical City, Makkah, Saudi Arabia
3Department of Pathology, International Medical Center, Jeddah, Saudi Arabia
4Umm Al Qura University, Makkah, Saudi Arabia

Corresponding author : Omar A Abu Suliman, MBBS
Senior Registrar,Otolaryngology, Head and Neck Surgery, King Abdullah Medical City,Makkah, Saudi Arabia
Tel: +966555505152; 
E-mail: [email protected]

Received: September 12, 2016Accepted: September 20, 2016 Published: September 27, 2016
Citation: Marglani OA, Suliman OAA, Shakra RIA, Fairag MM, Sabbagh FY (2016) Clinical Diagnosis and Management of Septal Chondrosarcoma. J Otol Rhinol 5:5. doi:10.4172/2324-8785.1000289

Abstract

Chondrosarcoma of the nasal septum is an extremely rare malignancy. Chondrosarcoma are known to be slow-growing, invasive and usually high-grade malignancies. They most commonly occur in middle-aged men. Histology remains the mainstay of diagnosis. We report a case of a 36-year-old man who presented with nasal obstruction for 2 months. Endoscopic examination showed a nasal septal mass occupying the nasal cavity. Radiological findings showed a large soft tissue mass arising from the septum and Filling the nasal cavity with partial erosion of the posterior bony nasal septum. The patient underwent endoscopic nasal resection. The microscopic and immunophenotypic findings were consistent with moderately differentiated chondrosarcoma. Clinical, radiological and therapeutic challenges are discussed.

Keywords: Chondrosarcoma; Nasal Septum; Endoscopic; Resection

Keywords

Chondrosarcoma; Nasal Septum; Endoscopic; Resection

Introduction

Chondrosarcomas are primary malignancies that arise in the long bones, uncommonly in the head and neck regions and extremely rarely in the nasal septum [1-3]. Sinonasal chondrosarcomas are known to be slow-growing, invasive and usually high-grade malignancies. They are thought to arise from cartilaginous remnant tissues and also known to arise from non-harbor cartilage tissues [4,5]. They most commonly occur in middle-aged men. Histological diagnosis is the mainstay of diagnosing the disease, and the definitive treatment is surgical resection. Radiation can be used as an adjuvant therapy in cases of local tumor recurrence, while chemotherapy is generally ineffective for treating chondrosarcomas [1,2].

Case Report

A 36-year–old man presented with approximately 2 months of nasal obstruction that was unresponsive to medical management. Nasal endoscopy showed a large, smooth, posterior nasal mass that mainly occupied the left nasal cavity. A computed tomography (CT) scan showed a large soft tissue mass within the nasal cavity with partial erosion of the posterior bony nasal septum and vomer. This erosion led to remodeling and refraction of the lateral nasal wall effacing the osteo–meatal complex on both sides, with extension into the left posterior ethmoidal aircells (Figure 1A and B).
Figure 1: Coronal CT scans Showing Mass Invading Ethmoid Sinuses and Left Maxillary Wall. B: Axial CT scans Cut Showing Mass Eroding Vomer.
Magnetic resonance imaging (MRI) PNS revealed that the intranasal mass mainly occupied the left nasal cavity, with no Orbital or Intracranial invasion (Figure 2).
Figure 2: MRI T1 with Contrast Coronal View showing Mass Occupying the nasal cavity with no orbital or intracranial involvement.
As the mass was resectable from the surgical and radiological perspective with the aim of obtaining symptomatic cure and histopathological tissue diagnosis, the patient underwent endoscopic resection of nasal septal mass under general anesthesia. The septal flaps were elevated bilaterally and the mass was excised and sent for histopathology. After complete resection the flaps were reapproximated completely and sutured together with no septal perforations left. On gross examination, the specimen consisted of multiple fragments of firm grayish tissue measuring 8.0×8.0 cm. Microscopic examination revealed a lobulated tumor formed of cells producing a chondroid matrix . The cells showed large hyperchromatic nuclei, prominent nucleoli, mitotic activity and binucleation (Figure 3A)
Figure 3A: The tumor shows mitotic activity, notice the atypical mitosis (arrow) (H&E stain, 40x).
The excision margin could not be evaluated because of the fragmented nature of the specimen. Immunostaining revealed positive staining for s100 and p53 (Figure 3B). Staining for ki67 was positive in a high percentage of tumor cells. The microscopic and immunophenotypic findings are consistent with moderately differentiated chondrosarcoma, the patient has followed closely in the outpatient setting for the last year.
Figure 3B: Slide showing S100 immunostaining (20x).

Discussion

Chondrosarcomas are uncommon, slow growing, malignant tumors that affect cartilage and are commonly found in the pelvis, ribs and long bones of the extremities [1]. Head and neck chondrosarcomas account for only 10% of all chondrosarcomas and occur most commonly in the larynx, maxilla and skull base, it is considered extremely rare for chondrosarcomas to arise from the Sinonasal region [4].
Patients with nasal septum chondrosarcoma may present with a variety of symptoms depending on tumor involvement in the surrounding structures, which may include the paranasal sinuses, skull base and palate.
Nasal obstruction is the most common symptom. Other symptoms include: headache, proptosis, epistaxis, chronic nasal discharge, cheek pain, loose teeth, toothache and sometimes a painless mass [1-3]. Our patient presented with nasal obstruction for two months and was otherwise healthy. Imaging has an important role in diagnosing and surgical planning.
In CT Scan Chondrosarcoma demonstrate hypodense Matri with scattered small calcification with sometimes large or ring-like calcifications, erosion of surrounding structures are usually common.
MRI show images of low intensity on T1 weighting, high intensity on T2 weighting and inhomogenous type of enhancement on T1 weighting on gadolinium. MRI have a great advantage of showing soft tissue extension [1,3,4].
Histologically, chondrosarcomas are divided into 3 grades based on the degree of cellularity, nuclear size, atypia and mitotic activity.
Grade 1: tumors have an abundant chondroid matrix with clusters of chondrocytes and normal or near-normal nuclei; rare nucleoli and rare binucleate cells may be present.
Grade 2: tumors exhibit more cellularity, less matrix, the presence of mitotic figures, enlarged nuclei, and higher numbers of binucleate cells.
Grade 3: tumors are characterized by higher cellularity, prominent nuclear pleomorphism, prominent mitotic figures, and stellate or irregularly shaped chondrocytes.
It can be difficult to histologically differentiate chondrosarcoma from chondroma, but doing so is important because chondrosarcoma is known to have a better prognosis.
Treatment options are limited. However, the literature supports a combined treatment modality. The surgical component requires wide surgical excision of the tumor with margin resection of normal tissue. Radiation therapy and chemotherapy are usually recommended as adjuvant treatments for local recurrent disease.
Endoscopic resection is becoming a Standard procedure for most of Sinonasal diseases with extended approaches which in comparison to open techniques reduces hospital stay and therefore decrease the overall cost.
Although these tumors are slow growing, at least 60% of patients have a local recurrence within 5 years. Prognosis depends on the location and involvement of the adjacent structures. Higher grade and positive surgical margins indicate poor prognosis. Distant metastases occur in 20% of patients and occur mostly in the lungs. Lifelong follow-up is recommended.

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