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Fig. 1.81 Extramedullar plasmacytoma. A Poorly-differentiated plasmacytoma. Note mononuclear and multinucleated neoplastic plasma cells with prominent nucleoli. B Immunoperoxidase staining of plasmacytoma shows monoclonal kappa staining in cytoplasm of neoplastic plasma cells. C Immunoperoxidase staining shows absence of staining for lamda light chains in neoplastic plasma cells.

Clinical features

EMP tends to be solitary, with multiple tumours present in only 10% of cases at diagnosis. The presenting features of head and neck EMP are: soft tissue mass (80%), airway obstruction (35%), epistaxis (35%), local pain (20%), proptosis (15%), nasal discharge (10%), regional lymphadenopathy (10%), and cranial nerve palsy (5%). The mean duration of symptoms is about 4.5 months. The tumour ranges in size from 2-5 cm. The appearance varies from grey to red, soft to firm, and sessile or pedunculated. EMP bleeds easily and is usually smooth without mucosal ulceration. Cervical lymph nodes are enlarged in only 10% of patients {2500}. Occasional primary EMP may be associated with serum parapro-teinaemia. An underlying multiple myeloma should always be excluded.

Macroscopy

EMP is lobulated, smooth or nodular, and has a fleshy or rubbery consistency.

Table 1.5 Sites of occurrence for head and neck extramedullary plasmacytomas

Site

Frequency

Nasal cavity

28%

Paranasal sinuses

22%

Nasopharynx

22%

Tonsil

7%

Larynx

5%

Pharynx

5%

Soft palate

3%

Salivary gland

2%

Thyroid

1%

Tongue

1%

Gingiva

1%

Cervical lymph node

1%

Miscellaneous sites, e.g.

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