Rare Malignant Parapharyngeal Tumors: Is There a Dilemma?

Abstract

Objective: To study the clinical, radiological, and histopathological characteristics along with the treatment outcome of 23 patients presenting with malignant parapharyngeal space tumors.

Method: Retrospective chart review between 2001 to 2010, at Department of Otolaryngology–Head and Neck Surgery, Chandigarh, India. Retrospective analysis of malignant parapharyngeal tumors from 2001 to 2010. Subjects: Twenty-three patients. Interventions: Clinical, radiological, and histopathological characteristics. Outcome measures: Disease-free survival at 1 year.

Results: Out of 23 cases, 17 were rare malignancies of the parapharyngeal space. Of these, 6 tumors arose de novo (sarcoma, squamous cell carcinoma, synovial sarcoma, fibrosarcoma, rhabdomyosarcoma, and leiomyosarcoma) and 3 were direct extension of deep lobe parotid tumor (undifferentiated carcinoma with neck metastasis). Other rare tumors were extra osseous chordoma of the parapharyngeal space (n = 1), malignant fibrous histiocytoma (n = 1), malignant ameloblastoma (n = 1), malignant melanoma extending to the infratemporal fossa (n = 1), malignant schwannoma (n = 1), malignant peripheral nerve sheath tumor with rhabdomyoblastic differentiation (triton tumor; n = 1), neurofibroma with atypia (n = 1), and extraosseus Ewing’s sarcoma (n = 1). Fourteen out of 23 (60.8%) patients are alive with no evidence of disease on variable follow up with mean follow up of 15 months.

Conclusion: Tumors of the parapharyngeal space are uncommon, comprising less than 1% of all head and neck neoplasms. Accurate diagnosis and management is challenging and requires a team approach. When the diagnosis is ambiguous, a complete surgical excision and histopathological examination should be the preferred approach for these rare tumors.