Author(s)
Peter Nguy, MD
Jonathan P. Giurintano, MD
Timothy Deklotz, MD
Affiliation(s)
MedStar Georgetown University Hospital;
Abstract:
Introduction: Our objective is to describe a rare case of nasal cavity squamous cell carcinoma, which was masked in a patient with granulomatosis with polyangiitis (GPA).
Methods: Case presentation and review of the literature
Results: The patient is a 55-year-old female diagnosed with systemic vasculitis at age 14. After development of pulmonary and renal manifestations, including renal failure requiring multiple renal transplants, biopsy confirmed GPA. Surgical history was notable for resection of a left septal inverted papilloma 15 years ago. Recurrent episodes of sinusitis were attributed to GPA flares by multiple otolaryngologists prior to presentation to our service with severe right-sided forehead and orbital pain. Exam was notable for saddle nose deformity and anterior septal perforation. Nasal endoscopy revealed a large friable, granular mass originating from the posterior nasal septum, with extension to skull base superiorly and right nasopharynx and sphenoid floor posteriorly; there was additional anterior involvement of the septal perforation with superficial spread along the lateral nasal wall and ventral surface of the nasal bones. Pathology was consistent with squamous cell carcinoma, arising in a background of inverted papilloma. Imaging demonstrated a soft tissue density within the right olfactory cleft, with continued progression seen on serial imaging dating to 3 years prior to presentation. Tumor board recommendations advocated for induction chemotherapy, followed by definitive radiation and possible surgical salvage. Functional imaging confirmed that patient remains disease-free 6 months after radiation.
Conclusions: GPA is a systemic vasculitis characterized by renal, pulmonary, and sinonasal inflammation, with sinusitis being a common complaint. Treatment is primarily with immunosuppression. Otolaryngologists must maintain a high index of suspicion for malignancy in a patient with GPA and prior history of inverted papilloma.