Plasmacytomas of the Head and Neck

Abstract

Plasmacytomas are rare tumors that often appear in the head and neck region and are characterized by a monoclonal proliferation of plasma cells. On both clinical presentation and pathologic examination these tumors may be confused with more common tumors of the head and neck. The purpose of this article is to review our experience with these rare neoplasms, with emphasis on clinical, pathologic, and therapeutic features. On retrospective chart review, we identified 20 patients with the diagnosis of plasmacytoma of the head and neck region at the Cleveland Clinic Foundation between 1976 and 1993. Records were reviewed with regard to initial symptoms, location of the neoplasm, diagnostic evaluation, treatment modalities, and survival. Of the 20 cases we identified, the tumor arose in the sinonasal/nasopharyngeal region in 11 (55%). Two cases (10%) represented medullary plasmacytomas, arising in the clavicle and presenting as supraclavicular masses. The mean follow-up was 60.2 months (range 6 to 131 months). In 15 of the 20 cases, immunohistochemistry staining for immunoglobulin light chain production was conducted. One of the two cases (50%) classified as medullary plasmacytoma demonstrated conversion to multiple myeloma, whereas only 2 of 18 cases of extramedullary plasmacytoma (11%) converted to multiple myeloma. The primary modality of treatment was radiation therapy with typical doses of 4500 to 6000 cGy. Kaplan-Meier survival estimates demonstrated 95% survival at 1 year, 82% survival at 5 years, and 10-year estimated survival of 72%. Plasmacytomas of the head and neck region are rare and on initial evaluation must be distinguished from multiple myeloma. The diagnostic evaluation includes appropriate radiologic and pathologic studies including immunohistochemistry. Despite the typical presentation as a locally destructive tumor, plasmacytomas are highly radiosensitive, and 70% to 80% survival may be obtained with the use of radiotherapy. Patients with plasmacytomas require long-term follow-up to detect conversion to multiple myeloma.

Extramedullary Plasmacytoma of the Nasopharynx Treated with Surgery and Adjuvant Radiation: Case Report and Review of the Literature

“…However, given the retrospective nature of the literature, there is no evidence based dosing regimen, with most larger series reporting the use of 35-60 Gy. 3,4,8,9,10 The most recent recommendations of the UK Multiple Myeloma Forum range from 40-50 Gy depending on the size of the tumor.…”

“…2 This position is supported by other authors. 1,8 In terms of the nasopharynx there are no reports directly addressing surgical resection, although some authors have utilized endoscopic or open methods to resect such lesions. 11,12 Our case is unique in that surgery was used both for diagnosis and to debulk the lesion to improve quality of life.…”

An Extramedullary Plasmacytoma of the Nasopharynx : A rare entity

“…It as a rare monoclonal neoplastic proliferation of plasma cells characterized by an extramedullary tumor of clonal plasma cells with no or small amounts of monoclonal protein in the serum and/or urine, normal bone marrow, normal skeletal survey, and no end-organ damage from the plasma cell proliferative process. 3 EMPs make up 4% of all plasma cell tumors and occur mainly in the upper aerodigestive tract (UAD) 1 . EMPs account for only 1% of all tumors of head and neck region and only 4% of all nonepithelial tumor of the nasal tract 1 .…”

“…3 EMPs make up 4% of all plasma cell tumors and occur mainly in the upper aerodigestive tract (UAD) 1 . EMPs account for only 1% of all tumors of head and neck region and only 4% of all nonepithelial tumor of the nasal tract 1 . Typical location of EMPs in head and neck region are pharynx (21.5%), nasal cavity (19.3%), oral cavity (14.7%) and paranasal sinus (13.0%).…”

Plasma Cell Tumors of the Skull Base: Four Case Reports and Literature Review

“…An evaluation for multiple myeloma is needed to establish the diagnosis of an isolated EMP or SPB. 1,2,3 A plasmacytoma involving the skull base is unusual, and a limited number have been reported. We present the clinical history and radiographic findings of four patients with a plasma cell tumor of the skull base and review the rates of progression of skull base primary sites to multiple myeloma.…”