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  Table of Contents    
Year : 2019  |  Volume : 62  |  Issue : 1  |  Page : 167-168
Extramedullary plasmacytoma of tonsil: an unusual site

1 Department of Pathology, Lab Medicine and Transfusion Medicine, Medanta - The Medicity Hospital, Gurgaon, Haryana, India
2 Department of Radiation Oncology, Medanta - The Medicity Hospital, Gurgaon, Haryana, India

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Date of Web Publication31-Jan-2019

How to cite this article:
Sachdev R, Tayal M, Goel S, Sharma R, Gautam D. Extramedullary plasmacytoma of tonsil: an unusual site. Indian J Pathol Microbiol 2019;62:167-8

How to cite this URL:
Sachdev R, Tayal M, Goel S, Sharma R, Gautam D. Extramedullary plasmacytoma of tonsil: an unusual site. Indian J Pathol Microbiol [serial online] 2019 [cited 2019 Jul 24];62:167-8. Available from: http://www.ijpmonline.org/text.asp?2019/62/1/167/251278

Extramedullary plasmacytomas (EMPs) are rare soft-tissue plasma cell neoplasm and found usually in the head and neck region rarely involving the tonsil.[1],[2] They may present as a primary lesion with no other foci elsewhere or they may be a part of more generalized form of plasma cell dyscrasias such as plasma cell myeloma (PCM). EMPs are seen in the elderly with male preponderance, presenting as swelling, pain, and obstruction of the upper respiratory tract.[2] The diagnosis is by histomorphology along with immunohistochemistry (IHC) as it mimics various benign and malignant conditions. The exclusion of skeletal involvement and other myeloma-related manifestations is important due to the difference in management and prognosis of EMP and PCM. According to the literature, it is controversial whether EMP is a separate entity or an early stage of PCM,[1] and hence, long-term follow-up to detect early recurrence or progression to PCM has been recommended.[3] Here, we report a case of EMP in a young female with only tonsillar swelling and weight loss.

A 28-year-old female presented with left tonsillar swelling and weight loss for 5 months. On examination, a well-defined left tonsillar mass with no palpable lymphadenopathy was noted. An incisional biopsy was done and revealed a lesion with completely ulcerated epithelium, subepithelium composed of sheets of plasma cells [Figure 1]b; inset], and occasional binucleate forms positive for CD138 with lambda restriction; CD3 and CD20 labeled scattered T- and B-cells, respectively. Diagnosis of tonsillar EMP was given. Further investigations including skeletal survey, related organ or tissue impairment, bone marrow examination, and immunofixation (IFE) studies were advised. Peripheral blood and bone marrow examination were normal. IFE showed the presence of monoclonal immunoglobulin G with lambda light chain immunoglobulins. Serum calcium, creatinine, and B2-microglobulin were normal. Positron emission tomography–computed tomography (PET-CT) showed fludeoxyglucose avid bilaterally enlarged tonsils (left > right) [Figure 1]a.
Figure 1: (a) Positron emission tomography–computed tomography showing bilaterally enlarged tonsils (left > right) showing fludeoxyglucose avidity. (b) Histopathology of the excisional biopsy showing ulcerated surface epithelium (H and E, ×100) with sheets of plasma cells with eccentrically placed cartwheel nucleus and eosinophilic cytoplasm with perinuclear Hoff in the subepithelium (H and E, ×400; inset). Occasional binucleate cells were also seen. CD138 (×100, ×400) showing sheets of plasma cells with lambda light chain restriction (×400). CD3 and CD20 (×400) label the scattered T- and B-lymphoid cells, respectively. (c) Positron emission tomography–computed tomography on follow-up showing resolution of the disease

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The patient was planned for external beam radiotherapy (EBRT) with the dosage of 4500 cGy in 25 fractions over a period of 5 weeks. The patient tolerated the treatment well with Grade 2 dysphagia and recovered on follow-up. The follow-up PET-CT showed posttreatment changes with resolution of metabolic activity in the tonsillar region [Figure 1]c. Protein electrophoresis was done twice (2 and 4 months) after completion of EBRT and showed suspicious band in the gamma region (17.6 g/dl and 16.0 g/dl, respectively). The patient is on regular follow-up till date with no comorbidities.

EMP is a rare disease with 80% of the cases seen in the upper aerodigestive tract with tonsillar involvement in 10.5% cases.[2] Unilateral tonsillar presentation is usually rare with the reported literature documenting six cases with most cases showing involvement of the right tonsil.[1],[2],[3],[4],[5] Huoh et al. reported left tonsillar involvement, similar to the present case.[6] All the reported cases were males in 50–80 years' age group.[1],[2],[3],[4],[5],[6] EMP of the tonsil in a female at young age has not been reported in the documented literature till date. The usual presentation is obstructed breathing, sore throat with odynophagia, dysphagia, and dysphasia,[1],[2],[3],[4],[5],[6] while our case had no obstructive symptoms. The differential diagnosis of unilateral tonsillar swelling considered is certain inflammatory lesions such as reactive plasma cell hyperplasia, plasma cell granuloma, and malignant neoplasms such as metastatic or undifferentiated carcinoma, olfactory neuroblastoma, and granulocytic sarcoma. In the present case, histomorphology was suggestive of the presence of mature and immature plasma cell, thus ruling out the other malignant neoplasms. IHC played a crucial role in differentiating it from other benign conditions involving plasma cells. Among the six reported cases, kappa restriction was seen with one study reporting lambda restriction.[6] Since the prognosis and treatment of EMP are different from PCM, it is important to evaluate the extent of the disease for proper management of the patient. In majority of cases of EMP, the serum and urine monoclonal immunoglobulin are not demonstrable because of low tumor load; however, the presence of low levels does not prevent the diagnosis.[1] Low levels of serum monoclonal immunoglobulin with lambda light chain were identified in the present case, similar to study by Bazaadut et al., having kappa light chains.[3] It should be kept in mind that high levels of monoclonal immunoglobulin most commonly indicate disseminated disease such as PCM.[1]

In the literature, in five studies, only tonsillectomy was done. Martines et al.[2] preferred surgical removal over RT with reevaluation quarterly for 1 year, twice for subsequent year, and annually thereafter. Kalan et al.[1] suggested excision biopsy followed by local RT as EMPs are radiosensitive. This was done in the present case.

The diagnosis of tonsillar EMP is important not only because it is rare neoplasm but also because it mimics many reactive and malignant neoplasms at this site. Differentiating EMP from myeloma is important based on imaging, hematological, and biochemical workup since the prognosis and management of both the entities differ. Tonsillar EMP has better prognosis than PCM, and long-term follow-up should be done to recognize advancement to PCM.

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   References Top

Kalan A, Asare-Owusu L, Tariq M. Solitary extramedullary plasmacytoma of tonsil – A rare location. Indian J Otolaryngol Head Neck Surg 2000;52:285-9.  Back to cited text no. 1
Martines F, Salvago P, Costanzo R, Marzo MD, Ferrara S, Iovane A, et al. Extramedullary plasmacytoma of the tonsil: A new management. Acta Med Mediterr 2015;31:603-6.  Back to cited text no. 2
Bazaadut S, Soodin D, Singh P, Khalafallah A, Withers S, Taylor S, et al. Extramedullary plasmacytoma of the tonsil with nodal involvement. Int J Otolaryngol 2010;2010. pii: 302656.  Back to cited text no. 3
Ozturk K, Sahin M, Midilli R, Gursan G, Ozsan N, Savas R. Extramedullary plasmacytoma of head and neck region: Report of six cases with different localizations. Otorhinolaryngol Clin Int J 2014;3:10-6.  Back to cited text no. 4
Bhat RV, Prathima K, Harendra Kumar M, Narayana G. Plasmacytoma of tonsil diagnosed by fine-needle aspiration cytology. J Cytol 2010;27:102-3.  Back to cited text no. 5
[PUBMED]  [Full text]  
Huoh KC, Van Zante A, Eisele DW. Extramedullary plasmacytoma of the tonsil. Case Rep Otolaryngol 2011;2011:430809.  Back to cited text no. 6

Correspondence Address:
Ritesh Sachdev
Department of Pathology and Laboratory Medicine, Medanta - The Medicity Hospital, Gurgaon - 122 001, Haryana
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/IJPM.IJPM_808_17

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