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Case Report

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

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.
Key words: Anemia, Bence Jones protein, mandible, multiple myeloma

Introduction Case Report

M
ultiple 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.
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DOI:
10.4103/0972-1363.155664
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

Address for correspondence: Dr. Shubhasini A. Raghavan, Department of Oral Medicine and Radiology, KLE Society’s Institute
of Dental Sciences, No. 20, Yeshwanthpur Suburb, Tumkur Road, Bangalore - 560 022, Karnataka, India.
E-mail: subhashiniar@gmail.com
Received: 23-08-2014  Accepted: 26-03-2015  Published: 22-04-2015

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Raghavan SA et al.: Multiple myeloma of jaw
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.
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.
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
Figure 1: Patient with extraoral swelling
Figure 3: PA skull view revealing multiple punched-out radiolucencies
Journal of Indian Academy of Oral Medicine & Radiology | Oct-Dec 2014 | Vol 26 | Issue 4
a differential diagnosis of Langerhans cell histiocytosis,
metastatic carcinoma and hyperparathyroidism.
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.
The patient was placed on chemotherapy with slow
intravenous injection of Bortezomib 2 mg and injection
Figure 2: OPG revealing osteolytic lesion
Figure 4: Histopathology showing sheets of plasma cells with several
atypical forms
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Raghavan SA et al.: Multiple myeloma of jaw
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.
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
Figure 5: Post-treatment view of patient
456 Journal of Indian Academy of Oral Medicine & Radiology | Oct-Dec 2014 | Vol 26 | Issue 4
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/m2 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-KB 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.
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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;26:454-7.
Source of Support: Nil, Conflict of Interest: None declared.

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