When Snoring isn’t just Snoring: Primary Nasopharyngeal Mantle Cell Lymphoma - A Rare Pathology in the Nasopharynx.

Abstract

Snoring is a common complaint in the primary care and otolaryngology clinic with a wide differential diagnosis. Primary nasopharyngeal mantle cell lymphoma is a rare cause of a nasopharyngeal mass, which can commonly manifest as snoring. The patient in this case presented with extensive history of recent worsening snoring as well as nasal congestion over the past several months. Additionally, the patient had previously undergone endoscopic sinus surgery several years prior but was lost to follow up. During nasal endoscopy, a nasopharyngeal mass was visualized with near-complete obstruction of the nasal airway. Intraoperative biopsies indicated MCL which is an uncommon pathology presenting in a rare location. Flow cytometry of the biopsy specimen was CD19+, CD20+, CD5+, and positive for lambda light chains with immunohistochemistry showed strong diffuse cyclin D1 nuclear staining on lymphoid cells. PET/CT and bone marrow biopsy were essential in staging disease, predicting success of treatment, and determining optimal treatment planning. Once the diagnosis was established, R-CHOP therapy alternating with R-DHAP for a total of six cycles. This case report highlights the importance of recognizing new or changing symptoms, appropriate diagnostic workup for lymphoma, as well as one of few case reports describing primary nasopharyngeal mantle cell lymphoma.

Fungating nasal mass as a rare presenting manifestation of relapsed mantle cell lymphoma

“…3 While cases reporting relapse of MCL involving the nasopharynx have been reported, the occurence of a fungating mass (5 cm × 3 cm) protruding out from external nare is an extremely rare presentation and highlights the extent of aggressive nature the cancer can develop. [4][5][6] Our patient had an early relapse within 2 months, which is associated with a poor prognosis. In a study by Bond et al, an inferior median overall survival of 1.3 years was seen in patients with early progression of disease within 6 months after both intensive and less-intensive frontline treatment.…”

“…While cases reporting relapse of MCL involving the nasopharynx have been reported, the occurence of a fungating mass (5 cm × 3 cm) protruding out from external nare is an extremely rare presentation and highlights the extent of aggressive nature the cancer can develop 4–6…”