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 Table of Contents  
CASE REPORT
Year : 2019  |  Volume : 3  |  Issue : 1  |  Page : 45-48

Otologic presentation of multiple myeloma with isolated temporal bone involvement: Diagnostic and therapeutic implications


1 Department of ENT-HNS, Apollo Hospital, Bengaluru, Karnataka, India
2 Department of Medical Oncology, Apollo Hospital, Bengaluru, Karnataka, India
3 Department of Pathology, Apollo Hospital, Bengaluru, Karnataka, India
4 Department of Radiology, Apollo Hospital, Bengaluru, Karnataka, India

Date of Web Publication22-Aug-2019

Correspondence Address:
Dr. J G Aishwarya
Department of ENT-HNS, Apollo Hospital, 154/11, Opp. IMM, Bannerghatta Road, Bengaluru - 560 076, Karnataka
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/aiao.aiao_25_18

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  Abstract 


Plasma cell disorder spectrum ranges from the benign plasma cell granuloma to malignant multiple myeloma characterized by the monoclonal proliferation of plasma cells. Multiple myeloma with temporal bone involvement is a rare entity and may present with nonspecific otological symptoms similar to chronic ear disease leading to a diagnostic challenge. Suspicion of multiple myeloma involving temporal bone primarily is difficult, and most of the cases are diagnosed after initial surgical excision. The differential diagnosis includes temporal bone carcinoma and glomus tumor due to similar radiological features. The final diagnosis is, however, made by the histopathological and immunohistochemical analysis. The mainstay of treatment of multiple myeloma is chemotherapy; however, surgical excision of the lesion is warranted in cases of pressure symptoms and intracranial involvement. We present a case who presented with ear canal mass, facial palsy, and cerebellar symptoms and was diagnosed to be a case of multiple myeloma with temporal bone involvement and was treated successfully. He is on regular follow-up for 1 year and remains in complete remission.

Keywords: Facial palsy, multiple myeloma, plasma cell disorder, plasmacytoma, temporal bone lesion


How to cite this article:
Aishwarya J G, Nair S, Anoop P, Shivakumar S, Patil AR, Babu S. Otologic presentation of multiple myeloma with isolated temporal bone involvement: Diagnostic and therapeutic implications. Ann Indian Acad Otorhinolaryngol Head Neck Surg 2019;3:45-8

How to cite this URL:
Aishwarya J G, Nair S, Anoop P, Shivakumar S, Patil AR, Babu S. Otologic presentation of multiple myeloma with isolated temporal bone involvement: Diagnostic and therapeutic implications. Ann Indian Acad Otorhinolaryngol Head Neck Surg [serial online] 2019 [cited 2019 Nov 19];3:45-8. Available from: http://www.aiaohns.in/text.asp?2019/3/1/45/265140




  Introduction Top


Multiple myeloma is characterized by the monoclonal proliferation of plasma cells and represents the malignant spectrum of plasma cell disorders.[1],[2] It is a systemic disorder characterized by anemia, hypercalcemia, renal impairment, multiple osteolytic lesions of the skull, and >30% of plasma cells in the bone marrow.[1] Multiple myeloma with the solitary bone lesion is a rare entity if present it is more common in spine followed by the skull and facial skeleton. Multiple myeloma with temporal bone involvement may present with nonspecific otological symptoms similar to chronic ear disease leading to a diagnostic challenge.[1],[2] Suspicion of multiple myeloma involving temporal bone primarily is difficult, and most of the cases are diagnosed after initial surgical excision. We present a case of multiple myeloma who presented with otological symptoms and posed a diagnostic challenge with a review of the relevant literature.


  Case Report Top


A 72-year-old male patient presented with the complaints of the right ear discharge, earache, decreased hearing, and tinnitus for 3 months and right-sided facial weakness and giddiness of 1-month duration. On examination, pinkish, polypoidal, and nonpulsatile mass [Figure 1] was seen filling the right external auditory canal (EAC) and Grade V/VI (House-Brackmann grading) facial palsy was present on the right side with cerebellar signs of ataxia and past-pointing.
Figure 1: Patient with right external auditory canal mass

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Pure-tone audiometry showed air-bone gap of 45 dB. Contrast-enhanced magnetic resonance imaging (MRI) study [Figure 2] showed a homogeneously enhancing mass measuring 4.1 cm × 3.4 cm × 3.4 cm centered in the right temporal bone mastoid segment just posterior to the jugular foramen. The mass was isointense on both T1- and T2-weighted images. Extension to EAC, petrous apex, and jugular foramen was noted. Intracranial extradural involvement of the cerebellum was noted. Positron-emission tomography–computed tomography (PET-CT) scan [Figure 3] was done to rule out other lesions that revealed similar findings as that of MRI, in addition documenting the bone destruction and fluorodeoxyglucose avidity (standardized uptake value - 14.8) and no lesions noted elsewhere. A radiological differential of atypical glomus jugulare versus temporal bone carcinoma was suggested. The biopsy from EAC was done, which was suggestive of highly vascular granulation tissue.
Figure 2: T1- and T2-weighted images in coronal plane and T1 postcontrast in axial plane shows an isointense mass with central heterogeneity (arrow) involving the mastoid part of temporal bone posterior to the jugular foramen extending into the external auditory canal (arrow). Posteromedially, the mass is extending into the cerebellum (arrow)

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Figure 3: Fused positron-emission tomography–computed tomography image showing destructive lesion in the right mastoid region with intracranial involvement with avid fluorodeoxyglucose uptake (standardized uptake value - 14.8)

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Due to the extensive pressure effects on the facial nerve and cerebellum, the patient was planned for the excision of the mass through infratemporal fossa approach (FISCH type B). Intraoperative findings showed tumor involving the entire middle ear cleft with the 7th cranial nerve engulfed in the lesion [Figure 4] and [Figure 5]. The lesion was extending posteriorly to erode the outer cortex of the mastoid and occipital bone with an extradural extension to the cerebellum and petrous apex. The lower extent of the lesion was the C2 vertebra involving the trapezius and paravertebral muscles. Level II cervical lymph nodes were found to be enlarged and were removed for evaluation. Intraoperative frozen section of the lesion was reported as glomus tumor.
Figure 4: Intraoperative image showing proliferative mass eroding the outer cortex of the mastoid

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Figure 5: Intraoperative image showing the mass engulfing the facial nerve

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Postoperative period was uneventful, and the patient was discharged on the 5th postoperative day. Histopathological examination [Figure 6] revealed tumor tissue with cells arranged in diffuse sheets and in organoid pattern separated by delicate vascular channels. The tumor was composed of small round cells with plasmacytoid cells having an eccentric nucleus with moderate amount of basophilic to acidophilic cytoplasm. The majority were mature plasma cells with few showing binucleate forms with occasional cells having bizarre hyperchromatic nuclei. Occasional plasmablasts were seen with few mitosis and necrosis. The level II lymph node also revealed similar histological findings. The final diagnosis was plasmacytoma. The patient was further evaluated with bone marrow biopsy and serum protein electrophoresis to evaluate for underlying multiple myeloma. Trephine biopsy showed hypercellular marrow with 30% infiltration by plasma cells. Electrophoresis revealed the presence of M band, IgG-3300 mg/dl, and free lambda-147 mg/L, which was suggestive of multiple myeloma. The patient's hemoglobin, serum calcium level, and renal function tests were within the normal limits. The case was discussed within the institutional multidisciplinary committee, and the patient was advised for chemotherapy with cyclophosphamide, thalidomide, and dexamethasone regimen (CTD) for 6 months. Posttherapy PET-CT was done at 3 months, which revealed no disease, and he is on regular follow-up for the past 1 year.
Figure 6: Histopathological examination showing plasma cells arranged in diffuse pattern

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  Discussion Top


Temporal bone houses variety of lesions both benign and malignant in nature. The most common destructive lesion of the temporal bone is carcinoma either primary or secondary. Temporal bone involvement secondary to multiple myeloma has been reported, and it usually represents the advanced disease stage.[1],[2],[3],[4] Temporal bone lesion is usually accompanied by osteolytic lesions of other bones on further evaluation. Multiple myeloma presenting only as an otologic disease is very rare with only few reported cases in the literature.[1],[2],[3] When the temporal bone is involved in the disease process, it presents as otalgia, ear canal mass, facial palsy, hearing loss, and giddiness depending on the extent of the tumor.[1],[2],[3]

The diagnosis of multiple myeloma is characterized by the typical radiological and hematopathological features. The characteristic radiological features are multiple osteolytic lesions, most commonly involving vertebrae (66%), ribs (45%), skull (40%), shoulder (40%), pelvis (30%), and long bones (25%).[5] Plasmacytoma is a bony lesion consisting of plasma cells might also be a sole presenting complaint in some cases of multiple myeloma. Due to nonspecific radiological features, the usual differential diagnosis for such lesion in the temporal bone region is usually carcinoma or paraganglioma. However, in our case, the radiological findings revealed well enhancing destructive lesion in the region of the temporal bone, and thus, the differential of atypical glomus jugulare versus temporal bone carcinoma was suggested.

The histopathological examination is the confirmatory test for the diagnosis of plasmacytoma (bony lesion encompassing plasma cells).[6],[7] In our case, the frozen section was reported as glomus tumor as the sections showed the organized arrangement of tumor cells with intervening fibrovascular stroma with rich vascularity which is rare in plasmacytoma (plasma cells are arranged in a diffuse pattern and with poor vascularity). The paraffin fixed follow-up histopathological evaluation of the tissue and the lymph nodes revealed small rounded plasma cells based on which the final diagnosis of plasmacytoma was made.

Multiple myeloma sometimes presents as a bony lesion leading to the diagnosis of solitary plasmacytoma and 40% of solitary plasmacytoma cases in advanced conditions progress to multiple myeloma. Hence, it is mandatory to rule out the systemic involvement like multiple myeloma before rendering the diagnosis of plasmacytoma. On the evaluation of hematological and biochemical parameters, serum and urine electrophoresis would show increased monoclonal immunoglobulins and bone marrow biopsy would reveal the plasma cell proliferation of >30% which is diagnostic.[6],[7] In our patient, bone marrow biopsy revealed plasma cells >30% and lambda chain immunoglobulins on serum electrophoresis, which clinched the diagnosis of multiple myeloma.

There are various staging system and guidelines for the treatment of multiple myeloma in vogue.[4],[6],[7],[8] Among the plasma cell dyscrasias, there is a diagnostic dilemma between multiple myeloma and solitary plasmacytoma. As per the International Myeloma Working Group updated criteria for the diagnosis of multiple myeloma when the solitary lesion has >30% of plasma cells, it should be termed as multiple myeloma and if <10% it should be termed as solitary plasmacytoma.[7]

The mainstay of treatment for multiple myeloma is chemotherapy with CTD or similar combination regimens. Surgical excision of the bony lesion is warranted only in cases of pressure symptoms or intracranial involvement. Our patient had facial palsy and cerebellar symptoms due to the pressure effect of the tumor.


  Conclusion Top


Plasma cell disorder spectrum ranges from the benign plasma cell granuloma to malignant multiple myeloma. The otological findings are not discrete in such lesions. There exists a diagnostic dilemma among multiple myeloma, glomus tumor, and temporal bone carcinoma due to similar radiological and hematological features. The final diagnosis is, however, made by the histopathological and immunohistochemical analysis. The mainstay of treatment of multiple myeloma is chemotherapy. However, surgical excision of the lesion is warranted in cases of pressure symptoms and intracranial involvement. The main aim is to highlight the diagnostic dilemmas in such cases with emphasis on the choice of investigations available for appropriate diagnosis that will aid in appropriate management.

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.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.



 
  References Top

1.
Naraev BG, Linthicum FH Jr. Temporal bone involvement in multiple myeloma. Otol Neurotol 2006;27:580-1.  Back to cited text no. 1
    
2.
Quinodoz D, Dulguerov P, Kurt AM, Ruefenacht D, Abele R, Allal AS, et al. Multiple myeloma presenting with external ear canal mass. J Laryngol Otol 1998;112:469-71.  Back to cited text no. 2
    
3.
Kim SW, Oh CH, Kim JH, Jung MK. A case of multiple myeloma involving temporal bone. Korean J Otolaryngol 2002;45:1014-7.  Back to cited text no. 3
    
4.
International Myeloma Working Group. Criteria for the classification of monoclonal gammopathies, multiple myeloma and related disorders: A report of the international myeloma working group. Br J Haematol 2003;121:749-57.  Back to cited text no. 4
    
5.
D'Sa S, Abildgaard N, Tighe J, Shaw P, Hall-Craggs M. Guidelines for the use of imaging in the management of myeloma. Br J Haematol 2007;137:49-63.  Back to cited text no. 5
    
6.
Pratt G, Jenner M, Owen R, Snowden JA, Ashcroft J, Yong K, et al. Updates to the guidelines for the diagnosis and management of multiple myeloma. Br J Haematol 2014;167:131-3.  Back to cited text no. 6
    
7.
Rajkumar SV, Dimopoulos MA, Palumbo A, Blade J, Merlini G, Mateos MV, et al. International myeloma working group updated criteria for the diagnosis of multiple myeloma. Lancet Oncol 2014;15:e538-48.  Back to cited text no. 7
    
8.
Bird J, Behrens J, Westin J, Turesson I, Drayson M, Beetham R, et al. UK myeloma forum (UKMF) and nordic myeloma study group (NMSG): Guidelines for the investigation of newly detected M-proteins and the management of monoclonal gammopathy of undetermined significance (MGUS). Br J Haematol 2009;147:22-42.  Back to cited text no. 8
    


    Figures

  [Figure 1], [Figure 2], [Figure 3], [Figure 4], [Figure 5], [Figure 6]



 

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