Case of the Week
Section Editors: Matylda Machnowska1 and Anvita Pauranik2
1University of Toronto, Toronto, Ontario, Canada
2BC Children's Hospital, University of British Columbia, Vancouver, British Columbia, Canada
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December 8, 2016
Primary Nasopharyngeal Non-Hodgkin Lymphoma
- Background:
- More than 90% of head and neck cancers are squamous cell carcinomas. Lymphomas are the second most common head and neck malignancy after squamous cell carcinoma.
- The most common site for extranodal involvement by NHL is the Waldeyer ring. Oral and paraoral regions are the second most affected sites by extranodal lymphomas, after those of the GI tract.
- Clinical Presentation:
- Presentation varies depending on the site of origin and extension.
- In this case, it was progressive jaw swelling, unilateral nasal block, recurrent ear infection, and headache.
- Key Diagnostic Features:
- Homogeneously enhancing, infiltrating, multicompartmental mass with no necrosis
- Bone erosion with a permeative pattern
- No arterial narrowing or venous luminal invasion
- Differential Diagnoses:
- Rhabdomyosarcoma: Most common nasopharyngeal lesion in pediatric population; imaging characteristics overlap significantly with lymphoma; usually seen in younger patients, with a mean age of 5 years, which is the key differentiating feature
- Nasopharyngeal carcinoma: Most common primary malignant extracranial neoplasm to invade the CS; difficult to differentiate from nasopharyngeal NHL on imaging basis alone; age and ethnic background (Asian population) are key features
- Juvenile angiofibroma: Benign, locally invasive, hypervascular lesion with flow voids on MR imaging; almost exclusively seen in male teenagers; expands the pterygopalatine fossa and usually does not have bone destruction
- Treatment:
- Stages I and II: Combination of chemotherapy and radiotherapy
- Stages III and IV: Combination of chemotherapy
