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Isolated maxillary sinus plasmacytoma: A rare entity

1 Department of ENT, Command Hospital (Eastern Command), Kolkata, West Bengal, India
2 Department of ENT, INHS Asvini, Mumbai, Maharashtra, India

Date of Submission30-Mar-2022
Date of Decision29-May-2022
Date of Acceptance14-Jul-2022
Date of Web Publication17-Oct-2022

Correspondence Address:
Tanuj Madan,
Senior Resident, Department of ENT, Command Hospital (Eastern Command), Kolkata, West Bengal
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/jmms.jmms_47_22


Plasmacytoma is a monoclonal plasma B cell malignancy originating in either bone named as solitary plasmacytoma of the bone or in soft tissue labeled as extramedullary plasmacytoma (EMP). Solitary EMP is rare accounting for <1% of all head-and-neck malignancies, occurring primarily in the upper aerodigestive tract and nasal cavity, with nasal septum being the most common site. It accounts for 4% of all nonepithelial sinonasal tumors. EMPs are four times more common in men and affect geriatric age group routinely in the 6th–8th decade of life. It is pertinent to exclude systemic involvement by utilizing a systematic and well-coordinated approach of clinical, laboratory, and radiologic investigations. EMP of the nasal cavity is rare, but it should be considered in the differential diagnosis of nasal cavity masses in all age groups, especially elderly. The present case report describes a patient affected by EMP, in which tumor arose from left maxillary sinus. The patient was managed with endoscopic resection of the mass followed by external beam radiotherapy. The patient was followed up for 2 years with routine endoscopies and 18 fluorodeoxyglucose positron emission tomography with complete resolution of the lesion.

Keywords: CD138, extramedullary plasmacytoma, isolated maxillary sinus plasmacytoma, paranasal sinuses, sinus tumor

How to cite this URL:
Madan T, Naga R, Akhtar MZ, Dutta A. Isolated maxillary sinus plasmacytoma: A rare entity. J Mar Med Soc [Epub ahead of print] [cited 2023 Mar 26]. Available from: https://www.marinemedicalsociety.in/preprintarticle.asp?id=358785

  Introduction Top

Plasmacytoma is characterized by neoplastic proliferation of a single clone of plasma B cells, which synthesize a monoclonal immunoglobulin. It was first described by Schridde in 1905.[1] It can present as a single lesion (solitary plasmacytoma) or as multiple lesions (multiple myeloma [MM]). Solitary plasmacytoma can present as solitary plasmacytoma of the bone or extramedullary plasmacytoma (EMP), which develops outside the skeletal system as a soft tissue neoplasm. Diagnosis of solitary EMP can be confirmed only after elimination of a systemic disease using battery of clinical, biological, and radiological investigations.[2]

It accounts for 1%–3% of human cancers and approximately 4% of nonepithelial tumors occurring in the nasal cavity.[3] EMPs are rare tumors with a low global incidence between 0.04 and 3 cases/100,000 and also account for <4% of all plasma cell tumors.[3] EMPs classically affect the head-and-neck region, particularly the nasal cavity, paranasal sinuses (PNSs), tonsillar fossa, and oral cavity, but may also arise in the gastrointestinal tract, urinary bladder, lymph nodes, and skin.[4]

Its etiopathogenesis remains undetermined. However, chronic irritation caused by inhaled irritants such as smog and viruses have been implicated.[5] Its symptomatology is highly staggered owing to the anatomical sites of origin, which creates drastic differences in neural, vascular, and bone involvement. Unilateral nasal obstruction is the most common presentation of a sinonasal EMP reported in 29.8% of cases. Other symptoms include epistaxis, facial swelling, facial pain, reduction or loss of visual acuity, and rhinorrhea. Cervical lymphadenopathy at the time of diagnosis is present in 5%–20% of cases.[6]

Various therapeutic modalities for EMP have been suggested; however, unfortunately, its treatment has not been standardized yet owing to its rarity. Here, we report an interesting case of EMP of the maxillary sinus presenting as a sinonasal mass.

  Case Report Top

A 65-year-old male patient with primary hypertension presented with left-sided nasal blockage and hyposmia of 5 years' duration, which was insidious and gradually progressive. The patient also complained of recurrent sneezing and persistent rhinorrhea. The patient was being managed as allergic rhinitis and was referred to our center because of refractory symptoms and history of spontaneous epistaxis. Clinical examination with nasal endoscopy revealed a solitary, reddish, polypoidal mass in the left nasal cavity, extending between inferior turbinate and septum arising from lateral wall of the nose with nonvisualization of middle turbinate and extending posteriorly till choana. Right nasal cavity showed allergic changes with polypoidal mucosa. Rest of otolaryngological examination was normal.

Contrast-enhanced computed tomography of PNS revealed hypodense nonenhancing mass lesion measuring approximately 34 mm × 30 mm × 21 mm filling entire left maxillary sinus and extending medially into left nasal cavity and posteriorly to choana. There was no bony erosion of the sinus walls with bilateral soft tissue densities in ethmoidal and frontal sinuses [Figure 1]a and [Figure 1]b. The patient underwent endoscopic excision of the nasal mass under general anesthesia. Intraoperatively, an atypical, multilobulated, friable, loculated, polypoidal mass was seen arising from the left lateral nasal wall extending into left maxillary antrum. Left middle turbinate was absent. Left-sided atypical mass was delineated and excised, and the representative specimen was taken as biopsy and sent for tissue diagnosis. Uncinectomy and antrostomy were performed on the left side. Postoperative period was uneventful.
Figure 1: (a) Coronal cut showing hypodense nonenhancing mass lesion involving left maxillary extending into left nasal cavity and posteriorly to choana. There was no bony erosion of the sinus walls with bilateral soft tissue densities in the ethmoidal and frontal sinuses, (b) axial cut

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Histopathological report was suggestive of plasmacytoma as shown in [Figure 2]. Immunohistochemistry of the tissue blocks was positive for CD138, with few cells showing kappa light chain restriction.
Figure 2: HPR showing tumor cells in atypical polypoidal mass arranged in sheets and nests showing marked pleomorphism with coarse chromatin and conspicuous nucleoli with scanty eosinophilic cytoplasm and atypical mitosis (3–4/HPF), HPR: Histo-pathological Report, HPF: High power field.

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With clinicopathological diagnosis of plasmacytoma, the patient was referred to a medical oncologist and a radiation oncologist for further management. Peripheral blood smear and bone marrow aspiration cytology revealed normal trilineage differentiation with erythroid hyperplasia. Result of serum electrophoresis performed is as shown in [Figure 3].
Figure 3: Serum electrophoresis was positive with IgG band and kappa band; however, comprehensive myeloma protein panel including Bence-Jones proteins and beta-2-microglobulin was within normal limits

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Since there was a strong suspicion of sinonasal malignancy, the patient also underwent a whole-body fluorodeoxyglucose positron emission tomography (FDG PET) scan, which was negative for distant metastasis. The case after discussion in a multispecialty tumor board was recommended to be managed with definitive external beam radiotherapy. The patient received 28 sessions of radiation with advanced conformal technique and three-dimensional planning. The patient has been on regular outpatient department follow-up. Recent endoscopic evaluation revealed a well-epithelialized nasal cavity. The patient underwent whole-body FDG PET scan after 7 months of surgery, which showed complete resolution of previously ill-defined soft tissue mass lesion involving left maxillary sinus [Figure 4]a and [Figure 4]b.
Figure 4: (a) Preoperative FDG-PET scan showing an ill-defined soft tissue mass lesion involving left maxillary sinus, extending across medial wall of maxillary sinus into left nasal cavity, (b) postoperative FDG-PET scan showing only a thin rim of mucosal thickening seen peripherally along inner walls of the left maxillary sinus with only minimal metabolic activity representing posttreatment sequelae, FDG-PET: Fluorodeoxyglucose positron emission tomography

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  Discussion Top

Eighty percent of EMPs originate from rich lymphatic soft tissues of the head-and-neck region, with nasal cavities, PNSs, nasal septum, nasopharynx, and oropharynx being the most common sites.[7] EMPs are more common in men, with a male-to-female ratio of 2.3:1 with a range of 5–79 years (mean age of diagnosis being 55 years). In our reported case, the patient was a 65-year-old male.

They present insidiously as a space-occupying lesion with imprecise obstructive symptoms delaying its timely diagnosis. Nasal obstruction, usually unilateral, is the most common presentation of sinonasal EMP reported in up to 29.8% cases. Other symptoms are epistaxis, facial swelling, facial pain, painless mass, rhinorrhea, and change or loss of vision.[6] In the present case, the patient had a delay in diagnosis because he was being dubiously managed as allergic rhinitis and was diagnosed only after an episode of epistaxis with associated refractory response to treatment.

The endoscopic appearance of EMP can mimic a benign mass, especially in early stages, and can, therefore, get misdiagnosed as a polyp and be treated with corticosteroids. Diagnosis is clinched by tumor biopsy with morphologic findings of localized monoclonal plasma cells in the absence of plasma cell proliferation at other sites, especially in the bone marrow and immunophenotypic markers such as CD138, CD 38, CD19, CD56, CD27, CD117, and cyclin D1. Furthermore, laboratory tests such as serum and urine protein electrophoresis, quantitative serum immunoglobulin, and beta-2-microglobulin assay; substantial imaging studies such as computed tomography and magnetic resonance imaging (MRI); and functional scans such as 18 FDG-PET or sestamibi scintigraphy complete the battery of tests.[8] EMPs must be distinguished from reactive plasma cell lesions such as plasma cell granuloma, chronic granulomatous inflammation, rhinoscleroma, and lymphoma.

Wiltshaw delineated clinical stages of soft tissue plasmacytoma as Stage I – limited to an extramedullary site, Stage II – regional lymphadenopathy, and Stage III– multiple metastasis. Kilciksiz et al. reported that the overall 10-year survival rate is 69%.[9]

Diverse therapeutic modalities have been suggested and tried, but no course of treatment could be standardized due to singularity of the tumor, high frequency among elderly with various comorbidities, and preponderance to treat it as an aggressive malignancy. Treatment should therefore be customized for every patient to preserve the intricate anatomy of the site of origin. EMPs are radiosensitive, and therefore, radiotherapy is often used as the first-line treatment to avoid disfiguring results and eliminate need for further invasive surgical procedures. Solitary EMPs that are smaller than 5 cm show appreciable local control rates ranging between 80% and 100%, with radiation doses of 30–40 Gy in 20 fractions, whereas larger tumors are subjected to 40–50 Gy. Cervical nodes are included if involved. Only small localized cases are amenable to surgical excision with local control rates similar to that achieved with radiotherapy alone.[10] Sometimes, radiotherapy followed by surgical excision is also planned to reduce the tumor volume, thereby reducing its invasiveness and also as salvage therapy for recurrence. The role of chemotherapy is controversial as it neither improves local control nor improves overall survival but can simultaneously cause significant morbidity. Nevertheless, case reports are available on the use of vincristine, adriamycin, and dexamethasone scheme with complete disease remission. Adjuvant chemotherapy is considered only in high-grade tumors larger than 5 cm in refractory/relapsed cases, disseminated disease, and progression to MM.[9] Since head-and-neck EMPs can be very hostile with high propensity of local recurrence, it is extremely prudent to adequately irradiate all cancer cells with optimum doses. EMPs can progress and get converted to MM but at a rate lower than other plasma cell neoplasms. The highest risk of conversion is in the first 2 years postdiagnosis, and after conversion to MM is complete, the 10-year survival rate is <10% highlighting the need for regular follow-up. Cantone et al. based on their case series proposed a follow-up protocol of nasal endoscopy and serum examinations every 3 months and MRI follow-up 3 months after radiotherapy and then biannually for the next 5 years.[11]

  Conclusion Top

EMPs are sporadic tumors with staggered sites of affection and proximity to critical structures in head-and-neck regions and present with innocuous symptoms of nose and PNS, leading to delay in diagnosis. This case report highlights the fact that this rare entity should be kept in mind as differential diagnosis of a unilateral nasal mass, especially in the elderly. On confirmation, an elaborated diagnostic multidisciplinary workup is the recommended approach toward its management involving an otorhinolaryngologist, oncohematologist, oncopathologist, and radiation oncologist. With whatever experience available till date in the management of this rare tumor, radiotherapy by far has been the treatment of choice with a high 10-year survival rate. The treatment approach is still controversial as some clinicians favor radiotherapy alone, whilre prefer surgical excision, whereas few also advocate combined approach. We favor an individualized therapy based on tumor site, size, and extent, risk of transformation to MM, and its histopathology. Considering a 10% incidence of progression of EMP into disseminated MM, a lifelong follow-up of these patients is urged upon. The authors propose that follow up of a patient with Plasmacytoma should be lifelong with endoscopy and imaging. For the first 2 years the nasal endoscopy should be performed every 3 months and every 6 months for the next 3 years. CT nose and paranasal sinus should performed annually for the first 2 years and if any suspicion of recurrence on endoscopy or symptomatic patient. After 5 years, patient should be followed up on annual basis.

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form, the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.

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Conflicts of interest

There are no conflicts of interest.

  References Top

Soutar R, Lucraft H, Jackson G, Reece A, Bird J, Low E, et al. Guidelines on the diagnosis and management of solitary plasmacytoma of bone and solitary extramedullary plasmacytoma. Br J Haematol 2004;124:717-26.  Back to cited text no. 1
Agarwal A. Neuroimaging of plasmacytoma. A pictorial review. Neuroradiol J 2014;27:431-7.  Back to cited text no. 2
Nasr Ben Ammar C, Ghorbel I, Kochbati L, Gargouri W, Touati S, Maalej M. Solitary and extramedullary plasmocytoma in the head and neck region: Five cases report. Cancer Radiother 2010;14:755-8.  Back to cited text no. 3
Bachar G, Goldstein D, Brown D, Tsang R, Lockwood G, Perez-Ordonez B, et al. Solitary extramedullary plasmacytoma of the head and neck-Long-term outcome analysis of 68 cases. Head Neck 2008;30:1012-9.  Back to cited text no. 4
Kitamura A, Yamashita Y, Hasegawa Y, Kojima H, Nagasawa T, Mori N. Primary lymphoma arising in the nasal cavity among Japanese. Histopathology 2005;47:523-32.  Back to cited text no. 5
D'Aguillo C, Soni RS, Gordhan C, Liu JK, Baredes S, Eloy JA. Sinonasal extramedullary plasmacytoma: A systematic review of 175 patients. Int Forum Allergy Rhinol 2014;4:156-63.  Back to cited text no. 6
Araújo Rde P, Gomes EF, Menezes DB, Ferreira LM, Rios AS. Rare nasosinusal tumors: Case series and literature review. Braz J Otorhinolaryngol 2008;74:307-14.  Back to cited text no. 7
Hughes M, Soutar R, Lucraft H, Owen R, Bird J. Guidelines on the diagnosis and management of solitary plasmacytoma of bone, extramedullary plasmacytoma and multiple solitary plasmacytomas: 2009 update. London, UK: British Committee for Standards in Haematology. 2009;14.  Back to cited text no. 8
Kilciksiz S, Karakoyun-Celik O, Agaoglu FY, Haydaroglu A. A review for solitary plasmacytoma of bone and extramedullary plasmacytoma. ScientificWorldJournal 2012;2012:895765.  Back to cited text no. 9
Cantone E, Di Lullo AM, Marano L, Guadagno E, Mansueto G, Capriglione P, et al. Strategy for the treatment and follow-up of sinonasal solitary extramedullary plasmacytoma: A case series. J Med Case Rep 2017;11:219.  Back to cited text no. 10
Manickam H, Tang CL. A Rare Incidence of Solitary Extramedullary Plasmacytoma of Nasal Cavity. International journal of scientific study. 2019;7:111-4.  Back to cited text no. 11


  [Figure 1], [Figure 2], [Figure 3], [Figure 4]


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