Journal of Indian Academy of Oral Medicine and Radiology

CASE REPORT
Year
: 2014  |  Volume : 26  |  Issue : 4  |  Page : 454--457

Multiple myeloma of the jaw: A case report


Shubhasini A Raghavan, Praveen Birur Nagaraj, Bhanushree Ramaswamy, Darshana S Nayak 
 Department of Oral Medicine and Radiology, KLE Society's Institute of Dental Sciences, Bangalore, Karnataka, India

Correspondence Address:
Shubhasini A Raghavan
Department of Oral Medicine and Radiology, KLE Society«SQ»s Institute of Dental Sciences, No. 20, Yeshwanthpur Suburb, Tumkur Road, Bangalore - 560 022, Karnataka
India

Abstract

Multiple myeloma is a systemic B-cell lymphoproliferative disease that causes osteolytic lesions in the vertebra, ribs, pelvic bone, skull and jaw. Rarely jaw lesions are seen as the first sign in multiple myeloma. This is a case report with follow up of a 57-year-old female patient, previously treated for osteoporosis, who presented with a swelling of the jaw. On radiographic examination, she was found to have osteolytic lesions in the mandible and skull bones. These conventional aids led to the diagnosis of multiple myeloma thereby proving that the osteoporotic lesions were a part of the spectrum of multiple myeloma. The patient underwent chemotherapy and is currently on follow-up. This case report emphasizes the importance of early diagnosis of multiple myeloma in the jaw using readily available technologies and illustrates the contribution that oral assessment can provide.



How to cite this article:
Raghavan SA, Nagaraj PB, Ramaswamy B, Nayak DS. Multiple myeloma of the jaw: A case report.J Indian Acad Oral Med Radiol 2014;26:454-457


How to cite this URL:
Raghavan SA, Nagaraj PB, Ramaswamy B, Nayak DS. Multiple myeloma of the jaw: A case report. J Indian Acad Oral Med Radiol [serial online] 2014 [cited 2019 Aug 19 ];26:454-457
Available from: http://www.jiaomr.in/text.asp?2014/26/4/454/155664


Full Text

 Introduction



Multiple myeloma is a hematologic malignancy which is the multifocal disseminated form of plasma cell myeloma; its other forms being solitary plasmacytoma and extramedullary plasmacytoma. [1] Multiple myeloma is characterized by monoclonal proliferation of abnormal plasma cells. [2] Patients present with painful, lytic bone lesions and fracture. [3] More than 30% of the patients with multiple myeloma develop osteolytic lesions in the jaws. [4] Prognosis of multiple myeloma is poor with the median survival of approximately 4 years. [2] Early diagnosis and management can increase overall survival. The oral cavity can provide early clues to diagnosis, as reported in this paper.

 Case Report



A 57-year-old female patient reported with a chief complaint of swelling on the right side of the jaw since 1 month, which was spontaneous in onset. This was followed by mobility of the lower right back tooth which was extracted 1 week later, after which the swelling rapidly increased in size. It was painless initially and the patient developed mild pain 3 weeks after its onset. No systemic symptoms or paresthesia was present.

Medical history revealed that the patient was apparently alright until 6 months prior to her presentation, when she developed generalized body ache, knee and back pain, and had consulted a general physician for the same. Subsequently, based on the Dual Energy X-Ray Absorptiometry (DEXA) Bone Densitometry, she was diagnosed with osteoporosis and was prescribed Risedronate Sodium 35 mg for 12 weeks, which she took for 7 weeks only. The patient was also prescribed calcium tablets.

Extraorally, there was a solitary diffuse swelling over the right lower third of the face [Figure 1]. It was firm with no local rise in temperature or tenderness. Teeth numbers 46 and 47 were missing and the sockets were healed. A non-tender buccal vestibular obliteration with cortical expansion was observed. A provisional diagnosis of residual cyst was made, with a differential diagnosis of ameloblastoma and keratocystic odonogenic tumor.{Figure 1}

Panoramic radiograph [Figure 2] revealed a large irregular osteolytic lesion in the right body of the mandible extending from 45 to 48 region; superoinferiorly from the alveolar crest to inferior cortex. The lesion had a well-defined margin without sclerosis and a totally radiolucent internal structure. There was loss of lamina dura at the apex of 44 and 45, as well as discontinuity of the lower border of mandible, suggestive of a pathologic fracture.{Figure 2}

To evaluate the inferior cortex and buccal cortical expansion, a postero-anterior (PA) view [Figure 3] and lateral skull view were carried out. The images showed multiple punched-out radiolucencies involving the skull bones, facial bones and mandible. Considering the radiographic pattern and age of the patient, a radiographic diagnosis of multiple myeloma was reached upon, with a differential diagnosis of Langerhans cell histiocytosis, metastatic carcinoma and hyperparathyroidism.{Figure 3}

Hematological investigations revealed normocytic hypochromic anemia with decreased Hb (7.5 gms/dl), raised ESR (75 mm/hr), and raised serum calcium level (12.05 mg/dl). Serum protein electrophoresis showed monoclonal increase in the M protein in the gamma-region and a positive Bence Jones protein test. Serum urea (71 mg/dl), creatinine (6.6 mg/dl) and uric acid (7.24 mg/dl) were raised. Ultrasonography revealed bilateral renal parenchymal disease. Other advanced radiographic investigations were not warranted as the conventional images provided pathognomonic information.

Incisional biopsy of the right lower alveolus was carried out which revealed sheets of malignant plasma cells [Figure 4]. Serum and urine protein electrophoresis revealed a monoclonal spike in the gamma region. Bone marrow biopsy and aspiration revealed sheets of more than 70% malignant plasma cells and plasmablasts, thereby, confirming the diagnosis of multiple myeloma.{Figure 4}

The patient was placed on chemotherapy with slow intravenous injection of Bortezomib 2 mg and injection dexamethasone 40 mg once a week for 12 weeks. The patient has completed this treatment and is doing well and remains under follow-up [Figure 5]. Surgical and prosthetic management of the jaw lesions have been planned.{Figure 5}

 Discussion



Multiple myeloma is a plasma cell neoplasm seen in patients above 40 years of age. Males are more affected than females. [5] It makes up 1% of all malignancies and 10-15% of hematologic malignancies. [6] The International Myeloma Working Group provided the criteria for diagnosis of multiple myeloma. [7] Durie-Salmon staged multiple myeloma into three stages [8] and the International Staging System staged multiple myeloma based on serum β2 -microglobulin level. [9] Karyotypic instability is seen at the earliest stage and increases with disease progression. Cytogenetic abormalities can be used as a prognostic indicator. [10] Based on Durie-Salmon staging, our patient was in Stage III of the disease.

Incidence of primary manifestation of multiple myeloma in the jaw varies from 8 to 15%. [1] It mainly affects the mandibular molar region, ramus and the angle of mandible, because these areas exhibit intense hematopoietic activity. Maxillary lesions are more frequent in the posterior regions. [4] There is increased risk of pathologic fracture, renal insufficiency, anemia, infection and bleeding following the accumulation of malignant plasma cells in the bone marrow. [2] These cells produce monoclonal immunoglobulin fragment, which presents as a homogeneous spike-like peak in the gamma-globulin zone on serum protein electrophoresis; [11] osteoclast-activating factor and osteoblast inhibitory factors leading to lytic bone lesions, osteoporosis and pain. [12] About 15% of myelomas secrete only light chains, detected in urine as Bence Jones protein. [2] Our patient had involvement of the posterior mandible and multiple punched-out lesions in the skull. She also presented with anemia, raised ESR, raised serum calcium and a positive Bence Jones protein test.

Administration of Dexamethasone or Prednisone, either alone or in combination with Thalidomide, remains a corner stone of multiple myeloma therapy. This is combined with autologous stem cell transplantation as part of the standard initial treatment. Single infusion of Melphalan at a dose of 200 mg/m 2 of body surface area has also emerged as a common regimen for multiple myeloma treatment. [2] Bortezomib is believed to cause apoptosis through decreased NF- K B signaling or accumulation of cellular debris. It also inhibits proteasome, an intracellular complex that degrades ubiquitinated proteins. [2]

 Conclusion



Multiple myeloma can present with varied clinical features, which in isolation may be difficult to diagnose, as in our patient, who was mistakenly diagnosed with osteoporosis. Jaw lesions occur in about 30% of these cases. Involvement of jaw bones, recognition of oral lesions and evaluation of the systemic status, which can be performed by a dentist, aids in early diagnosis and prompt management. The present case was diagnosed with multiple myeloma based on the oral findings.

References

1Baykul T, Aydin U, O'Carroll MK. Unusual combination of presenting features in multiple myeloma. Dentomaxillofac Radiol 2004;33:413-9.
2Stoopler ET, Vogl DT, Stadtmauer EA. Medical management update: Multiple myeloma. Oral Surg Oral Med Oral Pathol Oral Radiol Endod 2007;103:599-609.
3Amirchaghmaghi M, Pakfetrat A, Mozafari PM, Saghafi S. Mandibular swelling as the first manifestation of multiple myeloma. Iran J Med Sci 2010;35:331-4.
4da Silva Santos PS, Klingbeil MF, Abrahão AC, Gallottini M, de Sousa SC. Multiple myeloma with primary manifestation in mandibular area. Oral Surg 2012;5:26-9.
5Greipp PR, San Miguel J, Durie BG, Crowley JJ, Barlogie B, Bladé J, et al. International staging system for multiple myeloma. J Clin Oncol 2005;23:3412-20.
6Neville BW, Damm DD, Allen CM, Bouquot JE. Hematologic disorders. In: Neville BW, Damm DD, Allen CM, Bouquot JE, editors. Oral and Maxillofacial Pathology. 2 nd ed. New Delhi, India: Elsevier; 2002. p. 526-7.
7International 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 Hematol 2003;121:749-57.
8Durie BG, Salmon SE. A clinical staging system for multiple myeloma. Correlation of measured myeloma cell mass with presenting clinical features, response to treatment, and survival. Cancer 1975;36:842-54.
9Sachidanandan MK, Manoharan S, Rajaram K, Palanisamy MK, Soundarapandi P, Elumalai C. Multiple myeloma: Report of a very rare case and review. J Indian Acad Oral Med Radiol 2012;24:91-4.
10Pratt G. Molecular aspects of multiple myeloma. Mol Pathol 2002;55:273-83.
11O'Connell TX, Horita TJ, Kasravi B. Understanding and interpreting serum protein electrophoresis. Am Fam Physician 2005;71:105-12.
12Munshi NC, Longo DL, Anderson KC. Plasma Cell Disorders. In: Longo DL, Fauci AS, Kasper DL, Hauser SL, Jameson JL, Loscalzo J, editors. Harrison's Principles of Internal Medicine. 18 th ed. Vol. 1. USA: Mc Graw-Hill Co.; 2012. p. 936-44.