Solitary plasmacytoma of the paranasal sinuses are uncommon neoplasms of B lymphocyte origin. They comprise one per cent of all head and neck tumours of the upper respiratory tract. They can be solitary plasmacytomas of the bone (SPB), an extramedullary plasmacytoma or a local manifestation of multiple myeloma. Conversion to multiple myeloma happens more frequently in SPB. Radiotherapy is the common modality of treatment with, or without, adjuvant chemotherapy. Extramedullary plasmacytoma carries a better prognosis than a solitary plasmacytoma of the bone. We report four cases of solitary plasmacytoma of the bone and an extramedullary plasmacytoma of the paranasal sinuses and soft palate.

Extramedullary plasmacytoma in the submandibular region is rare and on initial evaluation must be distinguished from multiple myeloma. The diagnostic evaluation includes appropriate radiological and pathological studies including immunohistochemistry. We report a case of extramedullary plasmacytoma in the submandibular gland. A review of the literature suggests that it has a good prognosis, if multiple myeloma is excluded. This patient was treated by surgical excision followed by radiotherapy.

A rare case of upper airway obstruction due to an extramedullary plasmacytoma of the larynx in a patient with long-standing IgD myeloma is presented. Reports of patients with extramedullary plasmacytomas eventually developing multiple myeloma are common, however, the converse appears to be an extremely rare event. Attention is drawn to the problem of acquiring adequate tissue for diagnostic purposes as well as the use of immunohistochemical staining techniques. The patient required an urgent tracheostomy and was treated with radiotherapy.

We report a rare case of isolated extramedullary plasmacytoma (EMP) of the right true vocal fold in a 38-year-old male with a one-year history of hoarseness. Immunohistochemical staining of plasma cells in the tumour, showed over 90 per cent of them to be positive for kappa light chains. After two attempts at local surgical excision and recurrence within 10 months, the tumour was irradiated.

Only seven reported cases of isolated EMP of the true vocal fold are reported in the literature. The therapeutic options are discussed.

Plasma cell tumours of the mastoid are rare malignancies of haematological origin. Two patients are described with a plasma cell tumour in the mastoid bone. In one patient it concerned a solitary plasmacytoma of the bone and in the other a focus in the mastoid of a recently diagnosed multiple myeloma. Symptoms in these patients were non-specific. Computed tomography (CT) and magnetic resonance image (MRI) scanning showed a nonspecific space-occupying lesion in the mastoid. The diagnosis was made on immunohistological examination, that showed diffuse sheets of monoclonal plasma cells in a clear matrix. When a plasmacytoma lesion is found multiple myeloma should always be excluded. Treatment in case of solitary plasmacytoma of the mastoid bone consists of radiotherapy. In case of localization in the mastoid of multiple myeloma the treatment consists of palliative chemotherapy.

A rare case of extramedullary plasmacytoma arising from the nasal septum with localized amyloid deposition is reported. A 75-year-old woman presented with a history of post-nasal discharge and nasal obstruction for several months. Endonasal endoscopic observation revealed the presence of a mass arising from her nasal septum with an extension to the nasopharynx. No cervical lymph nodes were palpable. The biopsy specimen was diagnosed as a plasmacytoma (IgG, lambda-light chain type). Both serum myeloma-protein and urine Bence-Jones protein were negative. Bone marrow biopsy, a chest radiograph, total body skeletal survey and 67Ga- and 99mTc-scintigrams showed no other systemic lesions. These findings confirmed the diagnosis of extramedullary plasmacytoma in the nasal septum. The patient received irradiation of 40 Gy without clinically detectable reduction of tumour size. The patient eventually underwent complete resection of the tumour by KTP/532 laser under endonasal endoscopic control. Pathologically, the tumour mass was composed mainly of amyloid deposition with a marked reduction of tumour cells. This indicated the radiosensitiveness of tumour cells, which was clinically masked by the increased amyloid deposition. The clinical presentation, pathological features and surgical procedures are described with a review of the literature.

Solitary plasmacytoma of the skull base is a rare entity with only a few reported cases in the literature. We review the literature and present our experience with this lesion that produced ipsilateral sensorineural hearing loss, vertigo and ipsilateral sixth nerve palsy.

A 59-year-old white male presented with a two-month history of dry cough and shortness of breath. At bronchoscopy, a 1 cm mass on the laryngeal surface of the epiglottis was found. Immunohistochemical stain of the biopsy specimen revealed a monoclonal proliferation of plasma cells. Serological tests revealed normal serum immunoglobulin levels, and a bone marrow aspirate and biopsy was also normal; no abnormalities were found on serum or urine electrophoresis. He received radiotherapy (50 Gy), and was doing well three years following therapy without evidence of disease.

Solitary extramedullary plasmacytomas are uncommon malignant neoplasms with a great predilection for the upper respiratory tract. Extramedullary plasmacytoma involving the parotid gland is an extremely rare condition. This report describes the case of a primary extramedullary plasmacytoma arising in the right parotid gland of a 63-year-old man. A review of the literature reveals that this disease has generally a good prognosis, provided that multiple myeloma has been excluded. The treatment of choice should be surgical excision followed by radiotherapy, although from this study there is insufficient evidence to suggest that this is more effective than surgery alone. All patients must have regular long-term follow-up to monitor for recurrence or dissemination, in which event chemotherapy may be considered in addition to the other treatment modalities.

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.

A case of plasmacytoma arising from the hyoid bone is described, this is a distinctly unusual site, as the marrow-containing flat bones are more commonly affected. On a review of the literature this appears to be the first report of plasmacytoma occurring in the hyoid bone. The patient underwent a CT scan and plain radiographs. Histopathology was diagnostic.

Primary extramedullary piasmacytomas (PEMP) are uncommon plasma cell neoplasms that generally occur in the submucosal tissue of the upper airway. Salivary gland PEMP is an extremely rare condition. This report describes a unique case of PEMPin the parotid gland asociated with pulmonary amyloidosis. A review of all salivary gland PEMP's suggests that they behave similarly to PEMP's in other locations. The treatment of choice forlocalized disease is radiotherapy which should include regional lymphatics. Local controlcan be achieved in the majority of cases. The minority of patients develop systemic metastases and die from their disease. Chemotherapy can control disseminated disease and may induce remission.