Extramedullary nasal plasmacytoma: Literature review and a rare case report

Abstract

Summary Introduction: Extramedullary plasmacytoma is a plasma cell tumor that grows within any of the soft tissues of the organism. Similar to all plasmacytic dyscrasia, the disease is extremely rare. Aim: This study reports a case of an extramedullary plasmacytoma of the nasal cavity and provides a literature review on the topic. Case report: A 51-year-old woman presented at our tertiary university hospital with a 6-month history of progressive nasal obstruction, predominantly to the right side, and self-limiting epistaxis. Examination revealed a large pale-reddish tumor within the right nasal cavity. Anatomopathological analysis showed features consistent with a plasmacytoma diagnosis, which was subsequently confirmed by immunohistochemical techniques. Further assessment revealed the solitary nature of the condition, consistent with extramedullary plasmacytoma. Radiotherapy was initiated, which led to partial regression of the symptoms. The tumor was surgically removed by using a mid-facial degloving approach. The patient evolved with a naso-oral fistula and underwent 3 corrective surgeries. No evidence of associated systemic disease was found after 5 years of follow-up. Conclusions: This case report, which describes a rare tumor of the nasal cavity, is expected to improve the recognition and referral of this condition by ear, nose, and throat (ENT) specialists for multidisciplinary management and long-term follow-up.

Strategy for the treatment and follow-up of sinonasal solitary extramedullary plasmacytoma: a case series

“…2013 [ 7 ] 3 43/M Nasal cavity Nasal obstruction, epistaxis RT+surgery Surgery+RT (44 Gy) RT No No No No No No 1 year 1 year 3 years Corvo et al . 2013 [ 13 ] 1 51/F Nasal cavity/maxillary sinus Nasal obstruction, epistaxis RT (48 Gy)+surgery No No 6 years Verim et al . 2014 [ 10 ] 1 69/F Frontal sinus (4×3 cm) Chronic headache Surgery+RT (40 Gy) No No 18 months D’Aguillo et al .…”

“… Local disease with regional lymph node “metastasis” without evidence of distant spread. Local disease, recurrent or followed by dissemination and development of another neoplasm of plasma cells and/or MM [ 13 ]. …”

“…Local disease, recurrent or followed by dissemination and development of another neoplasm of plasma cells and/or MM [ 13 ].…”

Extramedullary plasmacytoma with intranasal localization

“…In the upper respiratory tract, the plasmacytoma has exceptional intraosseous localization, the most common being the extramedullary form. Extramedullary plasmacytoma occurs in approximately 4% of non-epithelial tumors of the upper respiratory tract, with occasional localization in the gastrointestinal tract, skin or lungs, but 80-90% are found in the head and neck region 3,4 .…”

“…Extramedullary plasmacytoma (EMP) represents less than 1% of all head and neck tumors and less than 0.5% of all the upper airway tumors [4][5][6] . In terms of location, depending on the frequency, EMP can be found in the submucosa of the nasal cavity, paranasal sinuses, oropharynx or larynx 4 . Regarding the rhinosinusal localization, the maxillary sinus is the most commonly involved.…”