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Extramedullary plasmacytomas in the head and neck region
Jos Straetmans1, Robert Stokroos
1Department of Otorhinolaryngology and Head and Neck Surgery, University Hospital Maastricht, P. Debyelaan 25, P.O Box 5800, 6202 AZ, Maastricht, The Netherlands. jstra@skno.azm.nl
Summary
Extramedullary plasmacytoma (EMP), a rare cancer outside bone marrow, often affects the head and neck. Early diagnosis and treatment, typically surgical, are crucial for managing EMP and its potential link to multiple myeloma (MM).
Area of Science:
- Oncology
- Head and Neck Surgery
- Pathology
Background:
- Extramedullary plasmacytoma (EMP) is a plasma cell neoplasm originating outside the bone marrow.
- It frequently occurs in the head and neck region, including the nasopharynx, nasal cavity, sinuses, and tonsils.
- EMP can occur independently or be associated with multiple myeloma (MM).
Observation:
- This report details three cases of head and neck EMP: subglottic EMP post-multiple myeloma treatment, solitary middle ear EMP presenting as jugular foramen syndrome, and epiglottic EMP recurring in the nasal cavity.
- Presenting symptoms are diverse, including epistaxis, rhinorrhea, sore throat, dysphonia, and hemoptysis.
- Diagnostic confirmation relies on histopathology with immunophenotyping (immunohistochemistry or flow cytometry).
Findings:
- Surgical intervention was the primary treatment for all three presented cases of EMP.
- Imaging modalities such as CT and MRI are essential for accurate staging of EMP.
- Prognosis is influenced by tumor size and nodal status, with a 10-year survival rate of 50-80%.
Implications:
- Prompt evaluation for multiple myeloma is critical in patients diagnosed with EMP.
- Multidisciplinary management involving surgery and/or radiotherapy is recommended for optimal outcomes.
- Understanding the varied clinical presentations and diagnostic pathways is key for effective patient care.
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