Skull base chordoma presenting as nasopharyngeal mass with lymph node metastasis
A 30-year-old female presented with mild headache, sore throat, gradual onset of difficulty in swallowing and speech for the last 2 years. On examination, a growth was seen behind the uvula obstructing the view of oropharynx with normal overlying mucosa. A level two right cervical lymph node (in the parotid region) was also present.\nRoutine hematological investigations and chest radiograph was within normal limits.\nComputed tomography scan of the neck revealed a well-defined oval-shaped mass 67 × 22 mm in superior nasopharynx slightly to the right side causing pressure erosion of body and lesser wing of sphenoid bone. The mass showed marginal contrast enhancement but no breakdown within. The radiological differentials were nasopharyngeal carcinoma, lymphoma, chordoma, and chondrosarcoma.\nMagnetic resonance imaging scan of the upper cervical region revealed a well-defined lobulated space occupying lesion at the skull base near foramen magnum having extension anteriorly along the prevertebral soft tissue having hypointensity in T1 and hyperintensity in T2 and Short T1 Inversion Recovery (STIR) sequence. The lesion also extended intracranially along the anterior aspect of the brain stem and to extra-axial space at C1 and C2 levels involving right lateral recess.\nFine-needle aspiration cytology of the cervical lymph node was done. Aspirated material was clear jelly like. Smears were stained with hematoxylin-eosin and May-Grünwald-Giemsa. Microscopic examination of the smears showed a cellular tumor composed of aggregates and dispersed single cells embedded in abundant chondromyxoid ground substance. The cells were round to oval with bland nuclear chromatin, smooth nuclear membrane, and inconspicuous nucleoli. Mild anisocytosis was apparent. Cytoplasm was clear to eosinophilic. Sparse number of multivacuolated cells was also seen []. Based on these findings a diagnosis of a chondromyxoid tumor was suggested. The possible differential includes salivary gland tumor or metastatic chordoma/chondrosarcoma.\nA biopsy from the nasopharyngeal mass showed a lobular growth separated by connective tissue septa. The cells were round to oval with vacuolated to eosinophilic cytoplasm and embedded in abundant myxoid matrix. Typical multivacuolated physaliferous cells were appreciable []. Hence, a provisional diagnosis of chordoma was suggested. Mucinous carcinoma, myxoid liposarcoma, and chondrosarcoma were considered among the differentials. Immunohistochemistry was performed that showed positivity with cytokeratin (CK) [ inset], vimentin and S-100, thus confirming the diagnosis of chordoma. As surgery was not possible owing to the location of the tumor, the patient underwent radiation therapy.