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- Title
A Rare Case of Subcutaneous Amyloidoma Associated with Localized Lymphoplasmacytic Lymphoma: Diagnostic Challenges and Treatment Considerations.
- Authors
Vivian, Lisa Francesca; Marcelis, Lukas; Leoni, Eleonora; De Bruecker, Yves; Maes, Helena; Pierré, Erwin; Ballaux, Florence M.; Tousseyn, Thomas
- Abstract
Objective: Rare disease Background: AL amyloidomas are solitary, localized, tumor-like deposits of immunoglobulin light-chain-derived amyloid fi- brils in the absence of systemic amyloidosis. A rare entity, they have been described in various anatomical sites, typically in spatial association with a sparse lymphoplasmacytic infiltrate, ultimately corresponding to a clonal, malignant, lymphomatous disorder accounting for the amyloidogenic activity. Most frequently, the amyloidoma-associated hematological disorder corresponds to either a solitary plasmacytoma or an extranodal marginal zone lymphoma of MALT. Much rarer is the association with lymphoplasmacytic lymphoma, which by itself is usually a bone marrow-bound disorder with systemic burden. The almost anecdotic combination of an amyloidoma and a localized lymphoplasmacytic lymphoma deserves attention, as it entails a thorough diagnostic workup to exclude systemic involvement and a proportionate therapeutic approach to avoid overtreatment. A review of the literature provides an insight on pathogenesis and prognosis, and can assist both pathologists and clinicians in establishing optimal patient management strategies. Case Report: We herein report the incidental finding of a subcutaneous amyloidoma caused by a spatially related, similarly localized lymphoplasmacytic lymphoma diagnosed in a 54-year-old female patient with no other disease localizations and a complete remission following 2 subsequent surgical excisions. Conclusions: Whatever the specific combination of an amyloidoma and the related hematological neoplasm, a multidisciplinary collaboration and a comprehensive clinical-pathological staging are warranted to exclude systemic involvement and identify patients with localized diseases who would benefit from local active treatment and close follow-up.
- Subjects
WALDENSTROM'S macroglobulinemia; MUCOSA-associated lymphoid tissue lymphoma; CARDIAC amyloidosis; BLOOD diseases; LITERATURE reviews; CEREBRAL amyloid angiopathy; THERAPEUTICS
- Publication
American Journal of Case Reports, 2023, Vol 24, p1
- ISSN
1941-5923
- Publication type
Case Study
- DOI
10.12659/AJCR.940789