Introduction
Chordomas are rare, locally aggressive tumors that originate from the embryonic remnant of the notochord. They account for 1-4% of all primary malignant bone tumors, with an incidence rate of 0.8 per 100,000 per year.1 They occur twice as frequently in men compared to women, with an age range around 40-60 years.2 The sacrococcygeal area (65%) is the most common site, followed by the spheno-occipital area (25%), and rarely the cervico-thoraco-lumbar spine (15%) is involved.3 Skull base tumors may occur at a younger age and have been reported in children and adolescents.2 Primary chordoma of the nasopharynx commonly presents as a nasal mass, which causes headaches, nasal obstruction with discharge and pressure symptoms, and dysphagia in those with pharyngeal spread.2
Radiographically, chordomas are destructive midline lesions that are readily evident on CT and MRI, but are difficult to identify on plain radiographs. Lesions arising in the skull base arise in the midline, destroy surrounding bone, and encroach on the brain stem or optic nerve. In the sacrum and coccyx, they form lobular masses that extend anteriorly toward the rectum and bladder.4
Grossly, chordomas are lobulated, gelatinous, soft, and contain areas of haemorrhage. Surgical excision is the treatment of choice because chordomas have poor sensitivity to radiotherapy and chemotherapy.
Case Report
A 28-year-old female presented with right nasal bleeding for the last month. She also complained of bilateral nasal blockage, bilateral supraorbital headache, purulent discharge, and nasal twang for the same duration.
A CT scan of the paranasal sinuses and neck revealed evidence of a well-defined soft tissue density lesion involving the sphenoid sinus, posterior ethmoidal air cells, and nasopharyngeal region. The lesion caused expansion and erosion of the floor of the sella, roof of the sphenoid, and floor of the anterior cranial fossa. Furthermore, there was destruction of the septum encroaching on the right retro-orbital region, with indentation over the medial rectus muscle and destruction of the medial wall of the right orbit.
Figure 2
High power view shows physaliphorous cells showing abundant eosinophilic vacuolated cytoplasm and moderate pleomorphic nuclei. (H&E 400X).
A biopsy was taken from the right nasal mass and sent to the surgical pathology section. The biopsy was processed, and a hematoxylin and eosin stain was performed on it, revealing small foci of a tumor composed of sheets of physaliphorous cells with moderate pleomorphic nuclei. Additionally, a few mitotic activities were observed. The biopsy also showed extensive extracellular chondromyxoid material with haemorrhage and a dense mixed inflammatory infiltrate. Immunohistochemistry performed on the biopsy showed positive reactivity to Pan-CK, S100, and diffuse positive epithelial membrane antigen (EMA). Based on the hematoxylin and eosin slides and the results of immunohistochemistry, the diagnosis of chondroid chordoma was confirmed.
Discussion
Chordoma is a rare nonepithelial malignant bone tumor primarily involving both ends of the axial skeleton, and it arises from remnants of the notochord. Primary nasopharyngeal chordoma arises in extraosseous nasopharyngeal soft tissue. It commonly occurs in the young adult and adolescent age groups. It affects males more commonly than females, with a male-to-female ratio of 2:1.5 The sacrococcygeal area is the most common site, followed by the spheno-occipital area, and rarely the cervico-thoraco-lumbar spine is involved.3 The most common clinical presentation is nasal obstruction. It also presents with other symptoms such as hearing impairment, headache, difficulty in swallowing, dryness of the mouth, nasal bleeding, diplopia, nasal speech, and difficulty in breathing. On gross examination, chordomas are lobulated, gelatinous, soft, and contain areas of haemorrhage.
Chordomas are classified into three different subtypes: conventional or classic chordomas, chondroid chordomas, and dedifferentiated types. Classic chordomas show numerous physaliphorous cells arranged in cords and lobules with a myxoid stroma separated by fibrous tissue. Chondroid variants show chondroid differentiation, and dedifferentiated variants show a biphasic tumor with two components: the first component being a conventional chordoma and the second component being a high-grade sarcomatous component. A few cases show a poorly differentiated type.6, 7, 8, 9, 10, 11, 12
The patient in the present case report had a chondroid type chordoma. Microscopically, it showed foci of tumor cells arranged in sheets composed of physaliphorous cells characterized by large intracytoplasmic vacuoles and moderate pleomorphic nuclei embedded in chondromyxoid stroma (Figure 1, Figure 2). The tumor cells stained strongly positive for cytokeratin, S100, and EMA (Figure 3, Figure 4).
Chondrosarcoma would be an important differential diagnosis of chondroid chordoma. Cytokeratin will be positive for chondroid chordoma and negative for chondrosarcoma.3
Local recurrence with an incidence of 50-68% is common due to the aggressive nature of this tumor, and systemic metastasis to the lung, bone, liver, and lymph nodes are found in 17.8-43% of patients with chordoma.1
Adverse prognostic factors include subtotal resection, dedifferentiated histological subtype, age >40 years, history of prior treatment, tumor size ≥4cm, gross tumor volume >25cm³, pharyngeal involvement, optic nerve and brain stem compression, none or low-dose radiation therapy, and female sex.1
Surgery is the primary modality to achieve the best long-term control.
Conclusion
In conclusion, chondroid chordoma of the nasopharynx is a rare condition that poses challenges in clinical diagnosis, often resulting in misdiagnosis. Due to its rarity and nonspecific symptoms, it is important to rely on histopathological examination to establish an accurate diagnosis. This examination helps differentiate chondroid chordoma from other similar conditions and enables appropriate management planning. Surgical intervention remains the primary treatment modality for achieving optimal long-term control. Awareness of this rare entity and its characteristic features is crucial for timely diagnosis and appropriate management of chondroid chordoma of the nasopharynx.
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