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Neuroradiology Case of the Week

Case 306

Scott Rudzinski, Taik-Kun Kim, MD and P-L Westesson, MD, PhD, DDS

Clinical Presentation: The patient is a 55-year-old male presenting with right-sided neck mass.

Imaging Findings: There is an isodense homogeneous right mandibular destructive mass measuring 6 x 1.9 cm with irregular borders accompanying soft tissue. No necrosis identified. Air was incidental due to biopsy. There is no lymph node involvement. On PET scan there is a hypermetabolic right mandibular soft tissue mass consistent with lymphoma.

Diagnosis: Diffuse large B-cell lymphoma of mandible

Discussion: Lymphomas can be simply defined as malignant neoplasms of lymphocytes and their precursor cells. Lymphoma has traditionally been separated into two subtypes: Hodgkin’s disease (HD) and non-Hodgkin’s lymphoma (NHL). HD often presents as nodal disease, with a predilection for neck and mediastinal nodes. NHL presents up to 40% of the time at an extranodal site. Moreover, 2% to 3% of these extranodal cases may arise primarily in the oral cavity and jaws. Oral lymphomas are the second most common malignant disease in the oral region after squamous cell carcinoma. Most such lymphomas have been shown to be predominantly of B-cell lineage, and may involve both osseous and soft tissues. Lymphoma shows a male predominance, with a male-to-female ratio of 3:2. The average age of presentation is 50-55, with incidence rate increasing with age.
     It is generally accepted that the most commonly affected site of NHL of the oral cavity and maxillofacial region is Waldeyer’s ring (tonsil, nasopharynx, base of tongue, palatine tonsil), with tonsils being the most frequent site. Jaw involvement by NHL is rare, with exception to Burkitt’s Lymphoma. Among jaw lesions the maxilla is more frequently involved than the mandible.
     NHLs arising in the jaws frequently present with non-specific signs and symptoms, such swelling or pain. Numbness, parasthesia, loose teeth, and cervical adenopathy have also been reported. Early lesions may be attributed to inflammatory odontogenic conditions resulting in unnecessary local treatment (i.e. root canal), delaying effective treatment of the underlying condition. Diagnosis is made by biopsy and immunochemistry.
     Radiologic findings of oral NHL are not specific, commonly exhibiting diffuse bone destruction, appearing as a solitary defect or as a disappearing of lamina dura or lowering of the alveolar bone margin to a condition resembling periodontitis or periodontal abscesses. Mandibular NHL has the presence of focal or diffuse osteolytic areas, usually without edge sclerosis. Often small areas of calcification can be seen in these masses. One half of the lesions are more likely to show reactive bone proliferation or some degree of radiopacity. On PET NHL is highly FDG-avid.
     Most patients with NHL of the jaw have high or intermediate grade disease with low grade lesions relatively uncommon. The intermediate and the high grade malignancy of the majority of NHL of the oral cavity and maxillofacial region indicates poor prognosis. In addition, malignant lymphoma of the jaws has a much greater incidence of local recurrence compared with other sites of involvement. Treatment of NHL is local radiation therapy in patients who are in lower stages and combination chemotherapy in patients with advanced disease.

References:

1. Cox DP, Treseler P, Dong R, Jordan RC. Rare oral cavity presentation of a B-cell lymphoblastic lymphoma. A case report and review of the literature. Oral Surg Oral Med Oral Pathol Oral Radiol Endod. 2007 Jun;103(6):814-9. [Medline]
2. Kemp S, Gallagher G, Kabani S, Noonan V, O'Hara C. Oral non-Hodgkin's lymphoma: review of the literature and World Health Organization classification with reference to 40 cases. Oral Surg Oral Med Oral Pathol Oral Radiol Endod. 2008 Feb;105(2):194-201. [Medline]
3. Kirita T, Ohgi K, Shimooka H, Okamoto M, Yamanaka Y, Sugimura M. Primary non-Hodgkin's lymphoma of the mandible treated with radiotherapy, chemotherapy, and autologous peripheral blood stem cell transplantation. Oral Surg Oral Med Oral Pathol Oral Radiol Endod. 2000 Oct;90(4):450-5. [Medline]
4. Kolokotronis A, Konstantinou N, Christakis I, Papadimitriou P, Matiakis A, Zaraboukas T, Antoniades D. Localized B-cell non-Hodgkin's lymphoma of oral cavity and maxillofacial region: a clinical study. Oral Surg Oral Med Oral Pathol Oral Radiol Endod. 2005 Mar;99(3):303-10. [Medline]
5. Someya M, Sakata K, Nagakura H, Itou K, Nakata K, Oouchi A, Satoh M, Hareyama M. Three cases of diffuse large B-cell lymphoma of the mandible treated with radiotherapy and chemotherapy. Radiat Med. 2005 Jun;23(4):296-302. [Medline]