Extramedullary plasmacytoma in the nasal cavity. (Imaging Clinic).

Extramedullary plasmacytomas are rare neoplastic lesions that can appear in the head and neck. They are characterized by monoclonal proliferation of plasma cells. These lesions account for 4% of all nonepithelial tumors of the nasal cavity, paranasal sinuses, and nasopharynx and 0.4% of all head and neck malignancies. (1) Approximately 80% of extramedullary plasmacytomas are localized in the submucosa of the upper respiratory tract. They usually occur in patients between 50 and 60 years of age, and they are more common in men than in women. (2) The nasal cavity and nasal septum are probably the most common locations. (3) Other sites in the head and neck that have been affected include the nasopharynx, maxillary sinus, thyroid gland, soft tissues of the anterior cervical region, parotid gland, tonsil, oropharynx, larynx, orbit, choroid, and eyelid. There have also been reported cases of solitary bone plasmacytomas affecting the sphenoid bone, mastoid, calvaria, skull vault, and hyoid bone, as well as cases aff ecting the temporomandibular joint, maxilla, and mandible. (4)

Extramedullary plasmacytomas of the upper respiratory and digestive tracts can be solitary or multiple. They have been associated with multiple myeloma at its initial appearance, and they have been reported to precede the eventual development of multiple myeloma by months or years.

We evaluated a 62-year-old man who had a small palpable mass in his left nostril. The patient had no history of nasal obstruction or bleeding. Endoscopic examination detected a somewhat darkish-red submucosal mass in the left piriform aperture of the nasal cavity. No other mass was identified in the neck. Computed tomography (CT) of the paranasal sinuses and neck detected a 1.5-cm softtissue mass in the anterior portion of the nasal cavity on the left side (figure). There was a suggestion of periosteal reaction. Following a biopsy, the lesion was histologically diagnosed as a plasmacytoma. Findings on bone marrow biopsy and radiographic and nuclear-medicine bone surveys were unremarkable. Bence Jones proteins in urine and serum were also negative for myeloma. The lesion was treated with radiotherapy, but after several months there was only minimal resolution of the mass. The tumor was later excised, and at the 9-month followup examination there was no evidence of recurrence.

The treatment of localized extramedullary plasmacytoma is somewhat controversial. Some authors favor radiotherapy, while others prefer only surgical management; still others advocate a combination of both. A follow-up examination with CT is suggested to determine recurrence and to rule out simultaneous lesions in different sites of the head and neck, which are known to occur in 10 to 20% of patients. (5)

References

(1.) Miller FR, Lavertu P, Wanamaker JR, et al. Plasmacytomas of the head and neck. Otolaryngol Head Neck Surg 1998;119:614-8.

(2.) Paris J, Dessi P, Moulin G, et al. [Extramedullary plasmacytoma of the nasal cavity: A case report]. Rev Laryngol Otol Rhinol (Bord) 1999;120:343-5.

(3.) Gonzalez Aguilaro O, Dragosky M, Pardo HA, et al. [Single extramedullary plasmacytoma of the nose]. Acta Otorrinolaringol Esp 2000;51:348-52.

(4.) Courtmans I, Pigeolet Y, Hedayat AH, Vilain J. Upper airways locations of plasmacytoma. Acta Otorhinolaryngol Belg 2000;54:487-90.

(5.) Windfuhr JP, Ott G. Extramedullary plasmacytoma manifesting as a palpable mass in the nasal cavity. Ear Nose Throat J 2002; 81:110-4.