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Table of Contents
CASE REPORT
Year : 2022  |  Volume : 24  |  Issue : 4  |  Page : 172-175

Extramedullary plasmacytoma of paranasal sinuses: Diagnostic and therapeutic aspects of six cases with literature review


1 Department of Otolaryngology, Head and Neck Surgery, Indira Gandhi Medical College, Shimla, India
2 Department of Otolaryngology, Head and Neck Surgery, Dr. Radhakrishnan Government Medical College, Hamirpur, Himachal Pradesh, India

Date of Submission28-Jul-2022
Date of Decision22-Aug-2022
Date of Acceptance23-Aug-2022
Date of Web Publication24-Sep-2022

Correspondence Address:
Dr. Trilok Chand Guleria
Department of Otolaryngology, Head and Neck Surgery, Dr. Radhakrishnan Government Medical College, Hamirpur, Himachal Pradesh
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/sjoh.sjoh_36_22

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  Abstract 


Extramedullary plasmacytoma (EMP) of the head and neck is a rare malignant tumor that accounts for just 3% of all plasma cell tumors. The mucosa-associated lymphoid tissue of the upper airways is involved in around 80%–90% of EMP. Because there is no conventional treatment regimen for plasmacytoma of the paranasal sinuses (PNS), therapy might be difficult. In this article, we will discuss six cases of EMP PNS, as well as the problems we encountered throughout their treatment.

Keywords: Extramedullary, mucosa-associated lymphoid tissue, paranasal, plasma, plasmacytoma


How to cite this article:
Thakur N, Thakur J, Guleria TC, Sharma DK, Azad RK. Extramedullary plasmacytoma of paranasal sinuses: Diagnostic and therapeutic aspects of six cases with literature review. Saudi J Otorhinolaryngol Head Neck Surg 2022;24:172-5

How to cite this URL:
Thakur N, Thakur J, Guleria TC, Sharma DK, Azad RK. Extramedullary plasmacytoma of paranasal sinuses: Diagnostic and therapeutic aspects of six cases with literature review. Saudi J Otorhinolaryngol Head Neck Surg [serial online] 2022 [cited 2023 Feb 5];24:172-5. Available from: https://www.sjohns.org/text.asp?2022/24/4/172/356933




  Introduction Top


Extramedullary plasmacytoma (EMP) is a rare malignant tumor of the head and neck that accounts for around 3% of all plasma cell tumors and <1% of all head-and-neck malignancies. The mucosa-associated lymphoid tissue of the upper respiratory tract is affected by 80%–90% of EMP. Multiple myeloma, solitary plasmacytoma of the bone, and EMP, as seen in the cases, are three clinical variants of plasma cell-derived neoplasms.[1],[2] EMPs are B-cell lymphocyte-derived proliferations of malignantly altered monoclonal plasma cells. EMPs are vascularized soft-tissue masses with no marrow extension that originate in the submucosa. The signs and symptoms of the EMP depend on the site of involvement.[3] The management of choice in EMP is debatable. In small localized lesions, surgery can provide targeted disease management with a high success rate. Although complete removal of the tumor is not always achievable due to the location and size.[4]

In this article, we present six cases of EMP affecting the paranasal sinuses (PNS) that were successfully treated with radiotherapy and chemotherapy, resulting in complete clinical and radiographic tumor regression. In all instances, we found a better outcome, and there was no progression to multiple myeloma during the follow-up period. Written informed consent was taken from all patients.


  Case Report Top


Case 1

A 52-year-old female presented with unilateral nasal obstruction from the right side along with intermittent nasal bleed for the last 5 months. Clinical examination showed a pinkish mass in the right side of the nasal cavity and a smooth surface bulge on the hard palate right side [Figure 1]a. No palpable cervical nodes were present. Computed tomography (CT) scan revealed a soft-tissue mass involving the entire right maxillary sinus and nasal cavity [Figure 1]b. The histological findings, following nasal biopsy, showed granulomatous lesion suggestive of tuberculosis for which antitubercular treatment was given for 6 months. The patient then presented again after 6 months without any improvement. Therefore, biopsy was repeated and subsequent histopathological examination (HPE) was suggestive of plasmacytoma. Immunohistochemistry further showed positivity for CD79a with a light Lambda chain. A systemic workup was performed with normal renal and liver function, absent Bence-Jones protein in urine, normal bone marrow needle biopsy, and skeletal survey, to exclude multiple myeloma. Hence, a diagnosis of EMP was made. The patient thereafter received external-beam radiation therapy (EBRT) with total radiation dose of 40 Gy, no recurrence was there in the follow-up period of 2 years.
Figure 1: Clinical and radiological image of Case 1. (a) Smooth surface bulge on the hard palate right side (b) Computed tomography scan showing soft-tissue mass involving the entire right maxillary sinus and nasal cavity

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Case 2

A 70-year-old female presented with unilateral nasal obstruction on the left side with facial swelling for the last 1 month. No history of nasal bleed, loosening of teeth, epiphora, or diplopia present. On clinical examination, diffuse swelling with mild tenderness was present on the left side of the face on the medial aspect with obliteration of the medial canthus [Figure 2]a. Eye movements and vision were normal. On anterior rhinoscopy, the lateral wall was touching the nasal septum of the left nasal cavity. Nasal endoscopy with a 0-degree rigid endoscope revealed projection of mass from the lateral wall of the nose at the level of the middle meatus. CT scan of nose and PNS showed a mass in the left nasal cavity, maxillary sinus, ethmoid sinus, frontal sinus with extension to the subcutaneous tissue [Figure 2]b. The X-ray of the chest, blood, and urine tests were within the normal range. Biopsy was taken under local anesthesia and sent for HPE which revealed poorly differentiated malignant plasma cells. Immunohistochemical stains were positive for light κ-chains and plasma cell markers. Bone marrow biopsy and plain X-rays of the bony skeleton were normal. Hence, the diagnosis of EMP was made. She was subjected to EBRT with a total dose of 60 Gy. However, unfortunately after gap of 10 months, she developed local recurrence treated with chemotherapy, with complete regression of the disease. Further no recurrence was there in the follow-up period of 2 years.
Figure 2: Clinical and radiological image of Case 2. (a) Diffuse swelling left side of face on medial aspect with obliteration of medial canthus (b) Computed Tomography showing a mass in left nasal cavity, maxillary, frontal, and ethmoid sinus with extension to the subcutaneous tissue

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Case 3

A 54-year-old male presented with unilateral nasal obstruction on the right side with swelling over the right side of the face and difficulty in opening the mouth. There was no history of nasal bleed, epiphora, diplopia, and loosening of teeth. On clinical examination, a pinkish mass was present in the nasal cavity right side. CT scan nose and PNS showed a soft-tissue mass in the right maxillary sinus, nasal cavity, ethmoidal sinus, nasopharynx, infratemporal fossa along with associated partial destruction of the medial wall of the maxillary sinus and the pterygoid plates [Figure 3]. There was no associated cervical adenopathy. Biopsy revealed poorly differentiated malignant plasma cells. Immunohistochemical stains were positive for light κ-chains and plasma cell markers. Results of bone marrow aspirate and biopsy were normal, as were results of serum and urine protein electrophoresis. Therefore, keeping EMP as the diagnosis the patient received EBRT with total radiation dose of 60 Gy, following which there was complete clinical and radiologic regression of the tumor. However, after 7 months due to recurrence of the disease, he received the cycles of chemotherapy, with stable disease in the follow-up period of 2 years after that.
Figure 3: Radiological image of Case 3. CT scan showing a soft-tissue mass in the right maxillary sinus, nasal cavity, ethmoidal sinus, nasopharynx, infratemporal fossa along with associated partial destruction of the medial wall of the maxillary sinus and the pterygoid plates. CT: Computed tomography

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Case 4

A 48-year-old male presented with a history of bleeding from the nose on and off for the last 1 month. Nasal endoscopy revealed a soft, friable mass occupying the right nasal cavity. CT scan revealed a soft-tissue mass in the right maxillary sinus, nasal cavity, and ethmoidal sinus with the destruction of medial wall of the orbit. The HPE, following nasal biopsy and the immunohistochemical study confirmed the diagnosis of plasmacytoma. Urine tests and serum electrophoresis were negative for Bence-Jones protein or myeloma component. Skeletal survey and bone marrow needle biopsy were within the normal limits. The patient was then treated with EBRT 40 Gy in 20 fractions, with no evidence of recurrence on follow-up for 2 years.

Case 5

A 63-year-old male reported with nasal obstruction and facial pain left side for 2 months and left side facial swelling for 2 weeks along with a history of intermittent nasal bleeding. He was a smoker. Clinical examination of the nose revealed a fleshy red mass, which bleeds on touch in the left nasal cavity, and a diffuse swelling left cheek. On oral examination, a bulge was present on the hard palate on the left side [Figure 4]a. There was no palpable cervical lymphadenopathy. Ophthalmic examination showed visual acuity of 6/9 in both eyes with no papilledema. Blood investigations showed no abnormalities. On CT scan, there was a heterogeneous mass in the left maxillary sinus with extension into the nasal cavity. It was also eroding the anterior wall of the left maxillary sinus and involving the subcutaneous tissue plane [Figure 4]b. Punch biopsy from the mass was taken and sent for HPE, which made the diagnosis of EMP. Immunohistochemistry was positive for CD38. Urine analysis for Bence-Jones protein was negative and no gammopathy was seen in serum electrophoresis. X-rays for skeletal survey did not demonstrate any skeletal involvement. Bone marrow and skeletal examinations were within the normal limits. Then patient was subjected to EBRT 40 Gy in 20 fractions with regression of the disease completely and no recurrence was there in the follow-up period of 2 years.
Figure 4: Clinical and radiological image of Case 5. (a) Bulge on hard palate left side (b) CT scan with a soft-tissue mass occupying the left maxillary sinus and nasal cavity. CT: Computed tomography

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Case 6

A 54-year-old male presented with unilateral nasal obstruction and facial swelling left side for the last 1 month. There was no history of bleeding from the nose, teeth loosening, watering from the eye, double or decreased vision. On clinical examination, a soft, friable mass was present in the left side nasal cavity and a diffuse swelling was present on the left side of the face. Eye movements and vision were normal. CT scan of the nose and PNS showed a mass in the left nasal cavity and maxillary sinus with extension to the subcutaneous tissue. Blood and urine tests were within the normal range. Biopsy from the mass taken under local anesthesia and sent for HPE which confirmed the diagnosis of plasmacytoma. On immunohistochemical analysis, CD-38 was positive. Bone marrow biopsy and X-rays of the bony skeleton were normal. The patient, later on underwent EBRT with total radiation dose of 40 Gy, with complete clinical and radiologic regression of the tumor and no recurrence was there in the follow-up period of 2 years.


  Discussion Top


EMP belongs to the type of non-Hodgkin's lymphoma. It is characterized as a soft-tissue plasma cell tumor that develops without systemic symptoms such as plasma cell invasion of the bone marrow, osteolytic lesions, urine or serum myeloma protein (M-component).[5] Early detection is critical for effective management and treatment of EMPs. Their diagnosis is complex because they are infrequent and have atypical presenting symptoms later on. In the diagnosis of any paranasal sinus tumor, a CT scan and tissue biopsy are essential tools. When EMP is suspected, histological assessment generally necessitates an immunohistochemical analysis. They must be distinguished from multiple myeloma at all times. In the presence of CD38 positive on immunohistochemistry and monoclonal cytoplasmic light chain expression in malignant plasma cells, EMP is distinguished from other forms of plasma cell malignancies or lymphomas. As a result, bone marrow biopsy, skeletal survey, urine, and serum tests should always be included in the evaluation.[6]

The location and size of the tumor, as well as its extension into the surrounding tissues, all influence treatment. EMP that is localized is extremely radiosensitive and radiotherapy is the gold standard treatment. Tumors larger than 5 cm may require higher radiation dose of 40–50 Gy, solitary EMPs smaller than 5 cm actually exhibit excellent local control with radiation doses of 30–40 Gy in 20 parts. If cervical nodes are involved, they should be included in the radiation field. Complete surgical excision is justifiable only in small localized cases.[7],[8] As in this case series, patients with tumor size of more than 5 cm received 60 Gy radiation dose and 40 Gy in tumor of size smaller than 5 cm. Progression to multiple myeloma is possible and some authors report one to three of their patients developing Multiple Myelomas after a median of approximately 2 years. In patients with localized plasmacytomas, adequate local irradiation can result in the long-term survival with a minimal risk of developing multiple myeloma. When compared to multiple myeloma, the prognosis for EMP is better.[9],[10]

Chemotherapy's effectiveness in preventing the development of multiple myeloma has yet to be established. Although some authors claim that chemotherapy has little benefit, other studies have shown that adjuvant chemotherapy can at least slow the course of multiple myeloma. Chemotherapy is recommended for widespread EMP. Many studies have shown that combining chemoradiotherapy improves remission and survival time.[3],[10]

In the presented cases, all the patients received radiotherapy with complete clinical and radiological regression of the tumor was obtained with treatment. Due to a recurrence of the disease, only one patient had chemotherapy. We found the better outcome in all cases and there was no progression to multiple myeloma in any case during the follow-up period.


  Conclusion Top


In conclusion, although the prognosis of the paranasal sinus plasmacytoma is good, a close follow-up is necessary to detect multiple myeloma development or recurrence. Due to rarity, a standard diagnostic, treatment, and follow-up protocol is necessary for a better outcome.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.



 
  References Top

1.
Dores GM, Landgren O, McGlynn KA, Curtis RE, Linet MS, Devesa SS. Plasmacytoma of bone, extramedullary plasmacytoma, and multiple myeloma: Incidence and survival in the United States, 1992-2004. Br J Haematol 2009;144:86-94.  Back to cited text no. 1
    
2.
Tsakiropoulou E, Konstantinidis L, Efstratiou I, Printza A, Constantinidis J. Extramedullary plasmacytoma of the larynx – Case presentation. Otorhinolaryngol Head Neck Surg 2011;44:41-3.  Back to cited text no. 2
    
3.
Park SH, Kim YZ, Lee EH, Kim KH. Endoscopic endonasal transsphenoidal resection of solitary extramedullary plasmacytoma in the sphenoid sinus with destruction of skull base. J Korean Neurosurg Soc 2009;46:156-60.  Back to cited text no. 3
    
4.
Michalaki VJ, Hall J, Henk JM, Nutting CM, Harrington KJ. Definitive radiotherapy for extramedullary plasmacytomas of the head and neck. Br J Radiol 2003;76:738-41.  Back to cited text no. 4
    
5.
Galieni P, Cavo M, Pulsoni A, Avvisati G, Bigazzi C, Neri S, et al. Clinical outcome of extramedullary plasmacytoma. Haematologica 2000;85:47-51.  Back to cited text no. 5
    
6.
Wein RO, Popat SR, Doerr TD, Dutcher PO. Plasma cell tumors of the skull base: Four case reports and literature review. Skull Base 2002;12:77-86.  Back to cited text no. 6
    
7.
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. 7
    
8.
Attanasio G, Viccaro M, Barbaro M, De Seta E, Filipo R. Extramedullary plasmacytoma of paranasal sinuses. A combined therapeutic strategy. Acta Otorhinolaryngol Ital 2006;26:118-20.  Back to cited text no. 8
    
9.
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. 9
    
10.
Knobel D, Zouhair A, Tsang RW, Poortmans P, Belkacémi Y, Bolla M, et al. Prognostic factors in solitary plasmacytoma of the bone: A multicenter rare cancer network study. BMC Cancer 2006;6:118.  Back to cited text no. 10
    


    Figures

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



 

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