Relapsed Waldenström macroglobulinemia presenting as bilateral parotid gland enlargement with multiple cystic lesions: A case report.
Hsin-Yao Hung, Yu-Cheng Chang, Tsung-Yu Yen
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Case in brief
A 79-year-old man with Waldenström macroglobulinemia (WM), previously stable for several years after multiple courses of chemotherapy, developed bilateral parotid enlargement. Imaging showed multiple cystic lesions. Relapsed WM was considered most likely in the context of markedly elevated serum IgM and immunoglobulin heavy-chain gene rearrangement detected by polymerase chain reaction in fine-needle aspirates. Because of his poor clinical condition, he received palliative care and died of sepsis 4 months after the parotid enlargement appeared.
What made this case unusual
The suspected extramedullary relapse presented as bilateral parotid enlargement with multiple cystic lesions, an exceptionally uncommon manifestation of WM.
Diagnostic / clinical pearl
In a patient with prior WM, bilateral parotid enlargement warrants consideration of relapse; correlate imaging with serum IgM and molecular findings from fine-needle aspirates.