This case report describes a 79-year-old man with previously stable Waldenström macroglobulinemia (WM) who presented with bilateral parotid gland enlargement and multiple cystic lesions, considered relapsed WM, and it highlights the importance of considering relapsed WM in the differential diagnosis of parotid gland enlargement, particularly in patients with a prior history of lymphoplasmacytic malignancy.
Key Findings
Background
Relapsed WM can present as bilateral parotid gland enlargement, an exceptionally uncommon extramedullary manifestation.
The patient was a 79-year-old man with a history of WM.
He had achieved stable disease for several years after multiple courses of chemotherapy.
He then developed bilateral parotid gland enlargement that was suspicious for relapsed WM.
Results
Imaging of the enlarged parotid glands showed multiple cystic lesions.
Imaging studies revealed multiple cystic lesions in the context of bilateral parotid enlargement.
The abstract does not specify the imaging modalities used.
Results
Relapsed WM was considered the most likely diagnosis based on elevated serum IgM and clonal IgH gene rearrangement in fine-needle aspirates.
Serum immunoglobulin M (IgM) levels were markedly elevated.
Polymerase chain reaction analysis of fine-needle aspirates demonstrated immunoglobulin heavy chain (IgH) gene rearrangement.
The diagnosis was described as 'most likely' rather than definitively confirmed by the abstract's wording.
Results
Because of poor clinical condition, the patient received palliative care and died of sepsis 4 months after parotid enlargement appeared.
No further disease-directed therapy was given due to poor clinical condition.
Death was attributed to sepsis.
Time from presentation of parotid enlargement to death was 4 months.
Conclusions
Relapsed WM should be considered in the differential diagnosis of parotid gland enlargement, especially with a prior lymphoplasmacytic malignancy.
This is the authors' stated conclusion from a single case.
The emphasis is on patients with a history of lymphoplasmacytic malignancy.
What This Means
This case report describes an elderly man with Waldenström macroglobulinemia, a rare blood cancer, that had been stable for several years after chemotherapy. He later developed swelling of both parotid glands (the major salivary glands near the ears). Scans showed multiple cystic lesions in the glands.
Blood tests showed very high levels of IgM, the antibody typically overproduced in this disease, and genetic testing of needle samples from the glands showed a clonal antibody gene rearrangement. Together these findings pointed to relapsed Waldenström macroglobulinemia as the most likely cause. Because of his poor health, he received comfort-focused care and died of sepsis four months later.
This research suggests that doctors evaluating swollen salivary glands may need to consider relapse of a prior lymphoplasmacytic cancer, even though this presentation is very rare. As a single case report, it cannot show how often this happens or how best to treat it.
Hung H, Chang Y, Yen T. (2026). Relapsed Waldenström macroglobulinemia presenting as bilateral parotid gland enlargement with multiple cystic lesions: A case report.. Medicine. https://doi.org/10.1097/MD.0000000000051176