Non-Hodgkin's lymphoma (NHL) is a malignant neoplasm of lymphocytes that generally occurs within lymph nodes but that may also occur at extra-nodal sites such as the oral cavity. Here, they tend to occur either in the Waldeyer's ring (palatine tonsils, lingual tonsils, and pharyngeal adenoids) or within the jaw bones. Most NHL are of B-cell derivation, although many lymphomas involving the nasal cavity and subsequently the hard and/or soft palate are of T-cell derivation. Burkitt's lymphoma is an unusual high grade lymphoma that occurs in young children (especially in sub-Saharan Africa) and that tends to occur within the jawbones and is associated with EBV.
Histiocytic lymphomas are rare in the oral cavity. Oral NHL (non-Burkitt's type) is a disease of adults. Patients notice a non-tender swelling or mass in the palate or gingiva, or enlargement of the jawbones (usually the mandible) if the lymphoma is intra-osseous. There may be vague pain and discomfort and/or a sensation of pressure. Mandibular lesions may cause mental nerve paresthesia.
The oral presentation may be part of systemic nodal lymphoma in which case lymph nodes (such as in the neck) may be enlarged, firm, and matted. T-cell lymphomas by virtue of their location in the nasal cavity cause nasal stuffiness and epistaxis.
In Stage III and IV disease, fever, weight loss, and night sweats (also called B symptoms) may be present.
Patients who are immunocompromised (eg, patients with combined variable immunodeficiency or AIDS) and those who have immune dysregulation (eg, those with Sjögren syndrome) are prone to developing lymphoma. Patients who have received organ transplants (eg, hematopoietic stem cell or heart transplants) are prone to developing EBV-associated lymphoma.
Codes
ICD10CM: C85.90 – Non-Hodgkin lymphoma, unspecified, unspecified site
SNOMEDCT: 118601006 – Non-Hodgkin's lymphoma
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Diagnostic Pearls
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Differential Diagnosis & Pitfalls
Oral NHL, B-cell type
Dental abscesses are painful, fluctuant, and associated with carious, infected teeth. They are of acute onset.
Other soft tissue tumors, malignant or otherwise, should be considered (eg, neurofibromas often present as a diffuse swelling of the palate).
T-cell lymphoma
Granulomatosis with polyangiitis presents as a destructive ulcer of the palate or with strawberry-like gingival nodules. Patients are often pANCA and cANCA positive, and there may be involvement of the kidneys.
Deep fungal infection, especially in immunocompromised patients and diabetics, may appear similar. A biopsy confirms the diagnosis.
Chronic cocaine use with perforation of the palate appears similar, but the history confirms the diagnosis.
Necrotizing sialometaplasia tends to present with a punched-out, clean ulcer on the palate that evolves and resolves over a few weeks.
Odontogenic cysts such as odontogenic keratocysts can cause jaw expansion and facial swelling in children. When associated with the Gorlin-Goltz syndrome, lesions may also be bilateral.
Odontogenic tumors in the mandible tend to occur in a slightly older age group.
Non-odontogenic tumors such as central giant cell granuloma tend to occur in young patients and cause facial swelling. Biopsy is diagnostic.
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Therapy
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Drug Reaction Data
Below is a list of drugs with literature evidence indicating an adverse association with this diagnosis. The list is continually updated through ongoing research and new medication approvals. Click on Citations to sort by number of citations or click on Medication to sort the medications alphabetically.
