Nasal osteogenic chondrosarcoma

A 28-year-old male presented with mass on right lateral wall of nose. He had symptoms of right side nasal obstruction, pain and headache. He also had a history of trauma to the nose. Physical examination revealed, significant deviation of the nasal septum and mass right nasal cavity. Probe could be passed with difficulty. Computed tomography showed an expansile lesion arising from right nasal bone filling the right nasal cavity [Figure 1a]. Fine needle aspiration cytology (FNAC) revealed moderately cellular smears with oval and polygonal cells. The cells were arranged as loosely cohesive clusters and as single cells in lacunae. Cytoplasm was abundant with distinct cell boundaries. Some of the tumor cells had cytoplasmic vacuolization. The nuclei were uniform, round to oval, with variably fine to coarse, even chromatin, with prominent nucleoli. Nuclear atypia, binucleation and multinucleation, were noted. The tumor cells were seen scattered in a background of metachromatic, myxoid, cartilaginous matrix which stained a characteristic purple in Giemsa smears [Figure 1b]. Diagnosis of chondroid tumor; chondrosarcoma was suggested. Endoscopic excision of the mass was performed. The mass was seen to involve the lateral wall and floor, the nasopharynx and nasal septum were free of tumor. Subsequent curetting of the tumor on histopathological examination revealed features consistent with chondrosarcoma grade 2 [Figure 1c].

Figure 1.

(a) CT scan reveals expansile lesion arising from right nasal bone and filling the right nasal cavity. (b) FNAC smear shows binucleate and mononuclear chondrocytes with moderate to abundant bluish cytoplasm. Background shows myxoid, cartilaginous matrix (Giemsa, ×400). Inset: Bizarre cell with large hyperchromatic nucleus and abundant blue cytoplasm (Giemsa, ×400). (c) Section shows tumor with chondroid differentiation, two or more nuclei per lacunae. Centre shows focus of endochondral ossification (H and E, ×400). Inset: Large cells with hyperchromatic nuclei (H and E, ×400)

Differential diagnosis of nasal chondrosarcomas includes chordoma, chondroma, metastatic renal cell carcinoma and mucin-producing adenocarcinoma. Chordomas are known to occur in the spheno-occipital region and would form an important differential of tumors in this region. They yield large tumor cells (physaliphorous) with abundant bubbly cytoplasm and myxoid material in the background. Low grade chondrosarcomas are very difficult or impossible to differentiate from chondromas on aspiration cytology. In our case the chondrosarcoma was grade two and thus did not pose a diagnostic challenge. The cells in renal cell carcinoma tend to have clear cytoplasm which is more appreciable in alcohol fixed smears and these lesions usually lack the characteristic myxoid matrix.

Chondrosarcoma make up only 10-20% of malignant primary bone tumors, with 5-10% located in the head and neck and nasal septal osteogenic chondrosarcoma (arising from bony part of nasal septum) is extremely rare with very few documented cases.[1]

Chondrosarcoma are a heterogeneous group of malignant tumors derived from a cartilaginous origin. Although most chondrosarcoma tumors arise from cartilaginous or bone structures, they may also develop in soft tissues, in which cartilage is normally not found.

They are usually diagnosed in older individuals in bones of the axial skeleton; three-fourths arise in the pelvis (26%), proximal femur, proximal humerus, shoulder girdle, and ribs. In our case the patient was young male and site of presentation was nose which accounts for only 2% of cases.[2] Like osteosarcomas, they are more common in men. The male-female ratio is approximately 1.2:1.[3] Although sometimes patients are asymptomatic, there may be pain, tenderness or local swelling. Most histopathological grading schemes of cartilage tumors and chondrosarcomas are modifications of the one proposed by Lichtenstein and Jaffe in 1943.[4] Grade I chondrosarcomas show increased cellularity, variation in cellular size and shape, lobular growth pattern and hyperchromatism of cells. In grade II lesions, cellularity is increased, more variation is seen, infiltrative or permeative pattern and binucleate cells are noted. In grade III, there is a marked increase in cellularity, and cells form sheets. A chondroid differentiation becomes less obvious, with areas of spindle cells appearing. In our case the tumor had maintained a lobular architecture, however cellularity was increased, variation in size and binucleate forms were also observed [Figure 1b]. Some areas exhibited increase in cellularity and few areas of endochondral ossification were still present.

The radiological appearance of the case presented herein is highly suggestive of chondrosarcoma. Plain radiograph revealed an osteolytic lesion. On computed tomography (CT) scan, chondrosarcoma appears as a lobulated mass containing an irregular chondroid matrix with bone invasion and destruction. The signal density of the chondroid matrix is lower than that of the bone matrix, although regions of bone density may be observed because of localized ossification.[5]

The natural history of chondrosarcomas is slow growth and late metastasis. Distant metastasis is rare in association with chondrosarcoma and most often involves the lung.[3] Survival has been linked to grade and drops precipitously with large, axial and high-grade tumors. The patient presented did not have any metastases at presentation. The main treatment for chondrosarcoma is surgical resection with a wide margin. Unfortunately, most chondrosarcomas are resistant to radiation and chemotherapy.