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Primary extramedullary plasmacytoma in the middle ear: differential diagnosis and management
D C Kandiloros1, T P Nikolopoulos, E A Ferekidis
1Department of Otolaryngology, Athens University School of Medicine, Greece.
Abstract:
Primary extramedullary plasmacytoma (PEP) is an uncommon neoplasm of plasma cell origin which afflicts the head and neck mainly. In this study we report a rare case of a 34-year-old man who presented with left ear tinnitus, hearing loss, blocked feeling and headache. Exploratory tympanotomy revealed a mass extending into the attic and the mastoid antrum. Following canal wall-up mastoidectomy, the tumour was carefully removed. Histological examination (including immunoperoxidase staining) and thorough clinical, laboratory and radiological evaluation revealed an exclusively cytoplasmic monoclonal IgG immunoglobulin PEP. The combination of surgery (including a second-look procedure) and radiotherapy used in this case may be an over-treatment. However, the patient is still disease-free seven years after his first admission to hospital.
Insights
This study details a rare case of primary extramedullary plasmacytoma (PEP) in a 34-year-old man
Area of Science:
- Oncology
- Pathology
- Otolaryngology
Background:
- Primary extramedullary plasmacytoma (PEP) is a rare plasma cell neoplasm, predominantly affecting the head and neck.
- Extramedullary plasmacytomas can present with diverse symptoms depending on the affected site.
Observation:
- A 34-year-old male presented with left ear symptoms including tinnitus, hearing loss, aural fullness, and headache.
- Exploratory tympanotomy identified a mass in the middle ear, extending into the attic and mastoid antrum.
- Surgical resection via canal wall-up mastoidectomy was performed.
Findings:
- Histopathological examination, including immunoperoxidase staining, confirmed an exclusively cytoplasmic monoclonal IgG immunoglobulin PEP.
- Comprehensive clinical, laboratory, and radiological evaluations ruled out systemic involvement (multiple myeloma).
Implications:
- This case highlights the importance of considering rare neoplasms like PEP in the differential diagnosis of unexplained ear symptoms.
- The treatment strategy involving both surgery and radiotherapy may warrant further investigation for optimal management of localized PEP.
- Long-term disease-free survival is achievable with appropriate management, as demonstrated by the patient's seven-year follow-up.