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Extramedullary plasmacytoma of the nasal cavity and ethmoidal sinus in human immunodeficiency virus-positive patient


1 Department of Otorhinolaryngology, Head and Neck Surgery, Jos University Teaching Hospital, Jos, Plateau State, Nigeria
2 Department of Pathology, Jos University Teaching Hospital, Jos, Plateau State, Nigeria

Correspondence Address:
Adeyi A Adoga,
Department of Otorhinolaryngology, Head and Neck Surgery, Jos University Teaching Hospital, PMB 2076, Jos, Plateau State
Nigeria
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/jcrt.JCRT_102_18

 > Abstract 


Extramedullary plasmacytoma (EMP) occurring in the nose and paranasal sinus regions are rare with a male preponderance in the fifth and seventh decades of life. We report a case of EMP of the nasal cavity and ethmoid sinus in a 28-year-old female with human immunodeficiency virus infection.

Keywords: Ethmoid sinus, human immunodeficiency virus, immunohistochemistry, nasal cavity, plasmacytoma



How to cite this URL:
Adoga AA, Silas OA, Yaro JP. Extramedullary plasmacytoma of the nasal cavity and ethmoidal sinus in human immunodeficiency virus-positive patient. J Can Res Ther [Epub ahead of print] [cited 2019 Dec 7]. Available from: http://www.cancerjournal.net/preprintarticle.asp?id=264211




 > Introduction Top


Extramedullary plasmacytoma (EMP) is an uncommon plasma cell neoplasm occurring within soft tissues outside the bone marrow and can be associated with multiple myeloma.[1],[2] Accounting for approximately 1%–2% of all plasma cell tumors, 75%–80% of them involve the head and neck region especially the subepithelial tissues of the upper aerodigestive tract, commonly the nasal, paranasal regions, and nasopharynx[1],[3],[4] usually running an indolent course but have destructive tendencies and ability for local recurrence.[5]

The clinical features include nasal obstruction, epistaxis, and rhinorrhea with examination findings of a fleshy soft-tissue mass that bleeds on contact, cranial nerve palsy, and cervical lymphadenopathy.[6]

The diagnostic criteria for EMP are as follows: monoclonal plasma cell histology on tissue biopsy, bone marrow plasma cell infiltration not exceeding 5% of all nucleated cells, absence of osteolytic bone destructive lesions on computerized tomographic (CT) scan or other tissue involvement, absence of hypercalcemia or renal failure and low-serum monoclonal proteins (M-proteins) concentration. Other imaging techniques such as positron emission tomography are beneficial in detecting and staging extramedullary tumors in individuals diagnosed with solitary bone plasmacytoma (SBP), and magnetic resonance imaging of the abdomen is used to determine disease extent and potential for resectability.[2]

Protein electrophoresis shows M-proteins in one-third of patients with normal uninvolved immunoglobulins just as Bence-Jones protein in the urine.[7]

Radiotherapy is the treatment modality of choice with the role of surgery being to obtain tissue for diagnosis and the excision of residual disease.[1]

Reports of EMP in the head and neck region in the Nigerian literature are rare, and we present a case involving the nose and ethmoid sinus in a young woman with the human immunodeficiency virus (HIV), emphasizing the need for a high index of suspicion in making a diagnosis. The roles of surgical excision to obtain accurate histological diagnosis and immunohistochemistry are emphasized.


 > Case Report Top


A 28-year-old female of the Tiv ethnic group in Nigeria was referred from a peripheral hospital to our Accident and Emergency department with a 6-h history of recurrent unprovoked left nasal bleeding which she had been experiencing for 4-month before the presentation. She was clinically pale at presentation with a packed cell volume of 24% for which she had 2-unit of whole blood transfusion after achieving hemostasis.

Anterior rhinoscopy revealed a fleshy finger-like mass filling the left nasal cavity, which extended from the roof of the nose and bled easily on contact. The right nostril was patent; however, there were no otologic, throat, and eye symptoms. There were also no comorbid medical conditions. A provisional diagnosis of inverted papilloma was made with nasal squamous cell carcinoma as a differential diagnosis.

CT imaging of the paranasal sinuses and base of the skull showed a contrast enhancing isodense mass filling up the left nasal cavity with lateral deviation and erosion of the medial wall of the left maxillary sinus and extension into the ipsilateral ethmoid sinus. Mucosal thickening was also seen involving both maxillary sinuses [Figure 1]. There was no breach of the base of skull.
Figure 1: (a) Computerized tomographic scan - coronal view showing mass in the left maxillary antrum (b) Computerized tomographic scan - axial view showing mass extending into the left ethmoidal cells

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She was prepared for and had endoscopic left nasal clearance and bilateral middle meatal antrostomy under general anesthesia through oroendotracheal intubation. At surgery, the findings were a hemorrhagic friable mass filling the left nasal cavity and polypoid maxillary antral mucosae [Figure 2].
Figure 2: Exenterated hemorrhagic friable mass

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Histologic sections showed a tumor composed of sheets of plasmacytoid cells, which were discrete, moderately pleomorphic with eccentric nuclei and prominent nucleoli. Perinuclear halos with abundant eosinophilic cytoplasm were noted with the tumor cells invading the fibrocollagenous stroma [Figure 3]. These features were consistent with a diagnosis of plasmacytoma.
Figure 3: Photomicrograph showing sheets of plasmacytoid cells, with eccentric nuclei and prominent nucleoli (H and E, ×40)

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Complete blood profile, renal, and liver function tests were within normal limits. She was seropositive to the HIV.

We do not have facilities for serum M-protein and urinary Bence-Jones protein estimations.

Bone marrow biopsy and skeletal survey were done following the histological diagnosis, and the results turned in normal.

Immunohistochemistry done on paraffin wax sections using prognostic biomarkers CD10 and CD38 confirmed the diagnosis of EMP.

Postoperatively, she was placed on ciprofloxacin, paracetamol, ethamsylate, and Vitamin C.

She was discharged on the 8th postoperative day in good condition to be seen by the infectious disease unit who eventually commenced her on antiretroviral therapy and commenced radiation therapy 7 weeks' postoperatively. She remained asymptomatic for 14 months of follow-up and has defaulted since then.


 > Discussion Top


Plasmacytomas can occur outside the bone marrow as SBP or as solitary EMP and can be associated with multiple myeloma.[6],[7] However, the progression of EMP to multiple myeloma is markedly lower than in SBP.[8]

Various mean ages of presentation are noted in literature, but commonly, it affects predominantly the male gender in their fifth and seventh decades of life with about 75%–80% involving the nose, paranasal sinus, and nasopharynx.[1],[3],[4] This is however in contrast with our index case of a female in the third decade of life.

The etiology is unknown, but it has been suggested that chronic stimulation of the mucosa of the upper respiratory tract by inhaled irritants or viral infection have been implicated previously.[3]

The clinical features depend on the site of tumor and for the head and neck region include nasal obstruction, epistaxis, and rhinorrhea with examination findings of a fleshy soft-tissue mass that bleeds on contact, cranial nerve palsy, and cervical lymphadenopathy.[6] Majority of these features were seen in our patient. It is important to note that cervical lymphadenopathy is seen in about 10%–20% of patients, a feature not seen in this patient.[8]

To determine the extent of the tumor, a CT scan of the paranasal sinus and base of skull was done which revealed a contrast-enhancing isodense mass filling up the left nasal cavity with erosion of the medial wall of the left maxillary sinus and extension into the ipsilateral ethmoid sinus. Mucosal thickening was also seen involving both maxillary sinuses, and middle meatal antrostomies were done. There was no evidence of intracranial extension.

To rule out multiple myeloma, a systemic workup was done which was of assistance even in the absence of facilities for serum M-protein and Bence-Jones proteinuria estimation.

The treatment of choice is radiotherapy with radiation dose directed at the primary site and surgery being used for obtaining tissue for diagnosis and the excision of residual disease.[1] A study in the past proposed a pathological grading system according to the grading criteria of multiple myeloma that was found to correlate well with treatment outcomes.[8] This proposal recommended grading into low, intermediate, and high grades with the use of adjuvant chemotherapy for patients with higher-grade tumors. Chemotherapy has been considered for patients with high-risk factors for local treatment failure such as tumor size >5 cm diameter and in situations of refractory disease.[9] The tumor size, extent, and location in our patient established our decision for surgery to excise the tumor followed by radiotherapy.

Recurrence is a feature in 10%–30% of cases with an overall 10-year survival of about 70%, therefore a long-term follow-up is of importance in monitoring recurrence of the disease.[9]

Reports of EMP involving the nose and paranasal sinus regions in HIV-positive patients are rare with a male preponderance in the few reports in literature.[10] Although the relationship between solitary EMP and HIV infection is poorly understood, it is said to occur in younger individuals tending to be more aggressive with an eventual poor prognosis as a result of poor immunity. HIV has been suggested to play a role in plasmacytomagenesis by reducing the immunosurveillance to a level as to impair the immune response against tumor cell growth, which facilitates the transforming malignant role of possible Epstein–Barr virus infection occurring in plasma cell tumors.[10] This probably explains the findings in our patient.

Immunohistochemistry is used to confirm the plasmatic nature of cells using biomarkers and differentiating the inflammatory processes in these cells indicating the need for evaluating for a differential diagnosis from other malignancies.[9] A modality that was helpful in confirming diagnosis in our patient.

Disease recurrence was not seen in our patient, and the default of follow-up did not offer the opportunity to consider complete disease remission. However, the treatment of our patient proved effective despite some limitations.


 > Conclusion Top


EMPs of the nasal cavity and ethmoid sinuses in HIV-positive patients are rare plasma cell tumors requiring a high index of suspicion to make a diagnosis.

Immunohistochemistry if available is a valuable tool in confirming the diagnosis.

Consent

Written informed consent was obtained from the patient for publication of this case report and any accompanying images. A copy of the written consent is available for review by the Editor-in-chief of this journal.

Declaration of patient consent

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

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.



 
 > References Top

1.
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. 1
    
2.
Straetmans J, Stokroos R. Extramedullary plasmacytomas in the head and neck region. Eur Arch Otorhinolaryngol 2008;265:1417-23.  Back to cited text no. 2
    
3.
Chang YL, Chen PY, Hung SH. Extramedullary plasmacytoma of the nasopharynx: A case report and review of the literature. Oncol Lett 2014;7:458-60.  Back to cited text no. 3
    
4.
Ghazizadeh M, Alavi Amlashi H, Mehrparvar G. Radioresistant extramedullary plasmacytoma of the maxillary sinus: A case report and review article. Iran J Otorhinolaryngol 2015;27:313-8.  Back to cited text no. 4
    
5.
Wu W, Pasch W, Zhao X, Rezk SA. Extraosseous plasmacytoma with an aggressive course occurring solely in the CNS. Neuropathology 2013;33:320-3.  Back to cited text no. 5
    
6.
Hazarika P, Balakrishnan R, Singh R, Pujary K, Aziz B. Solitary extramedullary plasmacytoma of the sinonasal region. Indian J Otolaryngol Head Neck Surg 2011;63:33-5.  Back to cited text no. 6
    
7.
Erdogan BA, Sekercan O, Dursun N, Tatar G, Bora F. Extramedullary plasmacytoma of maxillary sinus. J Craniofac Surg 2013;24:e85-7.  Back to cited text no. 7
    
8.
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. 8
    
9.
Ben Salah H, Hdiji S, Makni S, Ghorbel AM, Boudawara T, Elloumi M, et al. Extramedullary plasmocytomas. Cancer Radiother 2012;16:282-7.  Back to cited text no. 9
    
10.
Joseph AA, Pulimood S, Manipadam MT, Viswabandya A, Sigamani E. Extramedullary plasmacytoma: An unusual neoplasm in a HIV-positive patient. Int J STD AIDS 2016;27:909-11.  Back to cited text no. 10
    


    Figures

  [Figure 1], [Figure 2], [Figure 3]



 

 
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