Case Report

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J Res Adv Dent 2021;11:3:88-95.
JRAD Journal of Research and
Advancement in Dentistry
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Solitary Plasmacytoma Of Mandible: A Rare Case Report

B.V. Harish1* Manoj Kumar Kanta2 T. Surya kiran3 Sowmya Mantha4 K. Anant Nag5 Soundarya
Mopidevi6 Nvvsk Chaitanya7

1Senior Lecturer, Department of Oral and Maxillofacial Surgery, St Joseph Dental College, Duggirala, Eluru, India.
2Reader, Department of Oral and Maxillofacial Surgery, GSL Dental College and Hospital, Rajahmundry, India.
3Senior Lecturer, Department of Oral and Maxillofacial Surgery, GSL Dental College and Hospital, Rajahmundry, India.
4BDS, i dentist, Eluru, India.
5BDS, i dentist, Eluru, India.
6BDS, i dentist, Eluru, India.
6Krishna Chaitanya Dental Care, Kakinada, Andhra Pradesh, India.

ABSTRACT

Background: Plasmacytoma found as a mixed radiopaque radiolucent lesion around an impacted molar is
uncommon, however it is essential to rule out for its counterpart i.e., multiple myeloma. Solitary bone
plasmacytoma, typified by clonal proliferation of plasma cells, belongs to a group of lymphoproliferative clinical
disorders. It most typically involves a single bone or extramedullary site. It is a dyscrasia of plasma cells that
involves the jaw bones very rarely, and which is commonly seen in the retromolar area, angle, and ramus of the
mandible. They must be differentiated from multiple myeloma as prognosis between both vary considerably. It
accounts for 3% of all plasma cell neoplasms, with approximately 50% of the cases transforming into MM over 3-
4 years. This report describes a case of a mixed radiopaque-radiolucent lesion in the mandible, later diagnosed
as plasmacytoma in an old-aged male patient, treated surgically, and systemic workup was done to rule out
multiple myeloma. Despite the rarity of plasmacytoma in the jaws, there is an incidence of 0.15% of cases of
multiple myeloma, the first manifestation of the disease appears in the jawbones and oral cavity.

Keywords: Solitary plasmacytoma, plasma cells, multiple myeloma.

INTRODUCTION

Uncontrolled proliferation of plasma cells is the (3) SBP is observed as centrally localized in bones.
characteristic of plasma cell neoplasms. Solitary The prognosis of SBP is poorer than EMP, and
bone plasmacytomas (SBPs) and extramedullary approximately 50% of them will transform to MM
plasmacytomas (EMPs) are the localized forms of (4) been observed to occur within three years in as
the disease. In contrast, multiple myeloma (MM) is a much as two-thirds of cases. Patients may usually
systemic clonal proliferation of plasma cells based present with pain, tooth mobility, severe bleeding,
in the bone marrow. (1) SBP usually manifests as a or pathological bone fractures. With its non-specific
spinal disease most frequently affects the thoracic clinical and radiological features, biopsy becomes
vertebrae. (2) Other common bones that may be essential in order to establish the diagnosis. (5)
involved are the sternum, clavicle, rib, and humerus,
but that of the mandible is a rarity. (3) Although the histopathological features of solitary
plasmacytoma are characteristic, few clinical
In the maxillofacial area, plasmacytoma may be conditions of the oral cavity as poorly differentiated
classified into different clinical forms: a carcinoma and lymphoproliferative disorders may
manifestation of the disseminated form (MM), a also exhibit similar microscopic features to those of
solitary lesion in the soft tissues, or a solitary bone in plasmacytoma. Hence, the diagnosis of solitary
lesion with or without invasion of local structures. bone plasmacytoma (SBP) requires a solitary bone
_______________________________________________________________________________________
Received: Jan. 3, 2021: Accepted: Mar. 2, 2021
*Correspondence Dr. B.V. Harish.
iDentist Multispecialty Dental Clinic and Implant Centre, RR Pet, Eluru, Andhra Pradesh, India.
Email: Not Disclosed

Copyright ©2021 pISSN 2278-0076

www.jrad.co.in eISSN 2321-9270
JRAD Journal of Research and
Advancement in Dentistry Harish BV et al.
lesion, with both confirmatory histopathological Panoramic radiograph revealed mixed radiopaque-
and Immuno-histo Chemistry (IHC) analysis, with radiolucent lesion surrounding an impacted molar
the definite support of the hematological tooth extending from lower right central incisor up
investigation. (6) Investigations must be to the right sigmoid notch. (figure 2) The extent of
undertaken to determine the absence or presence of the lesion was confirmed through cone- beam
multiple myeloma. (5) computed tomography (CBCT).

Regardless of its course, it has a significant effect on

the patients’ quality of life, and mortality rates are
still significant. (7) In this article, we report clinical,
radiological, histological, as well as
immunohistochemical features in a 60-year- old
male patient with solitary plasmacytoma of the
mandible.

CASE REPORT

A 60-year-old patient reported to our outpatient Fig 2: Panoramic radiograph

department in December 2019 with a chief
complaint of pain and red-colored fluid discharge The preoperative complete blood picture was
from the lower right back tooth region of jaw since normal. An incisional biopsy of the bony lesion was
five days. Pain was gradual, moderate, lancinating performed by general dentist under local anesthesia
and continuous, radiating towards right ear with no and was sent to general pathologist, Histopathology
history of paresthesia. Patient gives medical history features were suggestive of “benign odontogenic
of diabetes since five years and is under medication. tumor” most possibly calcifying epithelial
Non-hypertensive. odontogenic tumor (pindborg tumor).

On clinical examination, the maximal mouth TREATMENT:

opening was 4 cm, without deviation or clicking on
the temporomandibular joint. There was no Resection was planned under general anesthesia.
evidence of submandibular, submental, or cervical The procedure along with complications, were
lymphadenopathy. There was a small painful explained to the patient, and informed surgical
mucosal opening in the right lower back tooth consent was taken. Under general anesthesia, lip
region. (figure 1) Aspiration of the lesion was split incision with chevrons modification was given
negative, excluding the possibility of vascular and and then continued up to submandibular and post
cystic lesions. ramal regions combined with intraoral vestibular
incision up to the coronoid notch, layer by layer
dissection was carefully done, the facial artery was
ligated in submandibular region, the marginal
mandibular nerve was identified and preserved.
The lesion was exposed. (fig: 3)

Fig 1: Mucosal opening in the right lower back tooth

region.

Fig 3: Surgical exposure of lesion

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The bony cut was made with a safety margin of 1cm
away from the lesion up to the region of left
mandibular central incisor (31), and bony cut was
extended from sigmoid notch to posterior border of
ramus sparing the unaffected condylar stump.
(figure 4,5)

Fig 6: Reconstruction with recon plate

On the second postoperative day, there was a

sudden fall of Hb% {7%} that was taken care with
blood transfusion. On the fourth postoperative day,
the patient complained of urinary retention,
shortness of breath with swelling of legs there were
Fig 4: Resection increased levels of fasting, and postprandial blood
sugars along with serum creatinine and urea
indicating acute renal failure, Immediate referral to
nephrologist was carried out to address acute renal
failure and dialysis was carried regularly for one
week, renal function tests along with sugar levels
and Hb% were normal. The nephrologist
commented that the altered levels of various
parameters are due to surgical stress and post-
operative medication. The Patient’s overall health
improved and he was discharged with the advice of
monthly reviews for six months.

To our surprise, the histopathological report

Fig 5: Resected specimen revealed the postoperative specimen as a
plasmacytoma. The sections revealed fibro
Initial reconstruction was done with a 20-hole 2.5 edematous connective tissue with inflammatory
mm long reconstruction plate. The reconstruction infiltrate, interspersed among the connective tissue
plate was adapted to the remaining mandible of left are sheets of plasma cells exhibiting nuclear and
side and to the remaining condylar stump on right cellular pleomorphism and increased abnormal
side and fixed with screws and also additional mitotic figures. Aggregations of immunoglobulins
reinforcement by suturing surrounding were seen in the stroma. (figure 7) The specimen
musculature to holes of reconstruction plate. (figure was further sent to immune histochemistry studies
6) A drain was placed in position followed by layer- for confirmation
by-layer closure, and the specimen was sent for
histopathological analysis.

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Fig 7: Histopathology picture

Immunohistochemical staining for kappa and

lambda antibodies showed positivity for kappa Fig 9: Radiograph of long bones.
protein in the tumor cell membrane, and tumor
secretions were positive for kappa protein and
faintly positive for lambda protein. These features
suggest plasmacytoma.

To rule out multiple myeloma, we performed

systemic workup, a skeletal survey with plain
radiographs of chest (figure8), long
bones(figure9,10), and skull (figure 11) was done
and showed no other osteolytic lesions, urine
analysis was negative for Bence Jones proteins .
Bone marrow aspiration revealed-features
suggestive of plasmacytosis.

Fig 10: Radiograph of long bones.

Fig 8: Plain radiographs of chest.

Fig 11: Radiograph of skull.

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As there were post-operative anemia, acute renal is more prevalent in males than females with the
failure, bone marrow plasmacytosis and ratio being. The present case shows SBP in a male of
anticipating the conversion of solitary 60yrs which is in harmony with the literature.
plasmacytoma to multiple myeloma as evident from
the literature, the patient was referred to an The most common clinical symptoms of SPB are
oncologist for necessary treatment. In 2nd and 9th pain in the jaws and teeth with
month post-operative review the wound healing paraesthesia/anesthesia, mobility and migration of
was satisfactory and no signs of recurrence were the teeth, hemorrhage, swelling in hard and soft
observed. (figure 12) tissues, and pathological fractures. But
plasmacytoma does not show any characteristic
sign related to the tumor (10). In our case, the
patient presented with a chief complaint of red
colored fluid discharge from the lower right back
tooth region of the jaw since five days, with no
significant medical history. On examination, there
was a small painful mucosal opening in the right
lower back tooth region.

Based on the multilocular radiolucent lesion of the

mandible on orthopantomograph, the differential
diagnosis for this lesion included ameloblastoma,
odontogenic myxoma, keratocystic odontogenic
tumor; central giant cell granuloma; aneurysmal
bone cyst and metastatic carcinoma. Оn plain
Fig 12: 9th month post-op picture. radiographs, SBP appears like odontogenic tumor
with multilocular or unilocular appearance with
DISCUSSION
well-defined borders. (11)
There are different forms of plasma cell
In our case, there was well defined “punched out”
(PC)neoplasms. The general location of multiple
lytic lesion extending from parasymphysis to ramus
myeloma is in the bone marrow and shows a wide
region sparing condyle on the right side of the
range of laboratory, clinical, and radiographic
mandible.
findings (8). On the other hand, solitary
plasmacytoma (SP) is characterized by an absence For a diagnosis of SP, the International Myeloma
or minimum plasmacytosis of bone marrow and an Working Group (IMWG) requires all four of the
isolated mass of clonal plasma cells, with symptoms following criteria be met:
of principal lesion. It is present either as a soft
tissue variety called extraosseous or extramedullary • biopsy-proven lesion,
type or a single lesion, i.e., solitary bone
plasmacytoma. The solitary variety is a rare type
• normal bone marrow,
with an incidence of 0.15% (8). • normal skeletal survey and

Normally Plasma cells generate a spectrum of

• absence of end-organ damage.

antibodies with different heavy chains (IgM, IgG, Table 1: Diagnostic criteria have been enumerated
IgA, IgD, and IgE) and light chain (kappa and by Bataille and coworkers(12)
lambda) characteristics. Except for a very few non-
secreting cases, a common early finding in early Diagnostic criteria for SP of bone (12):
Plasma Cell Disease is the presence of a monoclonal
heavy, and light chain restricted antibody, referred Typically, no M protein in serum/urine (a small M
component is present in 50%)
to as a paraprotein or M protein. (9)
solitary area of bone destruction due to clonal
plasma cells Bone marrow not consistent with MM
SPB occurs in a slightly younger age group than MM,
Normal skeletal survey
with an average age of diagnosis being 55 years and

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No end-organ damage other than solitary bone presence of <10% clonal bone marrow. But, SPB of
lesion, presence of a solitary bone tumor, a biopsy this subset has a markedly different course with
showing plasma cell histology, absence of myeloma 60% of cases advancing to PCM in 3 years. In
cells in bone marrow examinations, absence of contrast, the EMP of this subset will progress to
anemia, hypercalcemia or renal involvement,
PCM in 3 years, in 20% of cases only (13)
absence of or a low monoclonal component on
serum electrophoresis, and normal levels of
Table 2: Definition and course of plasmacytoma
immunoglobulin after treatment.
adopted from (13)

Solitary plasmacytoma, Not otherwise specified

Even though there is a clear difference between
solitary plasmacytoma of bone and extramedullary 1. Solitary disorder of bone or soft tissue, composed of
plasmacytoma, it is important to mark the updated monoclonal plasma cells (by biopsy).
criteria given by International Myeloma Working
2. Bone marrow biopsy, remote from the
Group (IMWG) for the diagnosis of multiple
plasmacytoma, is normal.
myeloma (13)
3. MRI or CT show normal skeleton, except for
As per the update given by WHO, there are two plasmacytoma.
subtypes of SPB : one which does not have clonal
4. No evidence of anemia, hypercalcemia, renal
marrow plasma cells distant from plasmacytoma
insufficiency or bone lesions, due to plasma cell
and thus, shows a normal bone marrow. In 10% of neoplasm (CRAB).
cases this variety may progress to multiple Solitary plasmacytoma with < 10% of bone marrow
myeloma in 3, conversely there is 60% progression 5. Progression to multiple myeloma in 10% of cases
commitment.
rate in a SPB subtype which shows less than 10% 1. As above mentioned with additional monoclonal
clonal marrow cells (13). plasmacytes < 10% in a sporadic bone marrow
biopsy
The International Myeloma Working Group (IMWG)
did not adopt to the clear distinction made by the 2. Of the above lesions, 60% of solitary plasmacytoma
of bone will develop into multiple myeloma
WHO update between SPB and EMP. Instead it has
blended the two varieties into a single lesion i.e., 3. 20% of Extramedullary plasmacytoma will transform
solitary plasmacytoma (13), which could be into Multiple Myeloma
described as "not otherwise specified" (NOS) type.
This represents a single type lesion, showing
variable differentiation of clonal plasma cells and Recently IMWG has given a new updated definition
this lesion is of either soft tissue or bone marrow. of SP with the advent of more sensitive techniques
The bone marrow of iliac crest does not show any to assess BM plasmacytosis (8), as per which SPB
clonal plasma cells in these patients. When isolated can defined as a presence of solitary lytic lesion due
primary tumor is excluded pelvis and spine show to infiltration of monoclonal plasma cells with or
normal bones under CT and magnetic resonance without soft-tissue extension, and EMP consists of a
imaging (MRI). Moreover, there is no evidence of soft-tissue mass that is not in contact with bone (8)
end organ damage relating directly to disorder of
plasma cells, such as the characteristic symptoms of In the present case initially, an incisional biopsy was
Multiple myeloma viz., hypercalcemia, renal performed which revealed it to be a “benign
insufficiency, anemia, bone injury (CRAB). There are odontogenic tumor most possibly “pindborg tumor”.
chances of multiple myeloma within 3years in 10% Based on the radiographic and histopathological
of these patients (13). report, aggressive resection of the lesion with
healthy bony margins was planned under general
From the above group of lesions IMWG study has anaesthesia. The post-operative resected specimen
further segregated, solitary plasmacytomas with was sent for histopathology, which revealed fibro
less than 10% clonal bone marrow plasma cells edematous connective tissue with inflammatory
(BMPC)as a separate entity, which is the only infiltrate. interspread among the connective were
difference between this sub type and the NOS sub sheets of plasma cells exhibiting nuclear and
type of solitary plasmacytoma (SP-NOS) i.e., cellular pleomorphism and increased abnormal

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mitotic figures, aggregations of immunoglobulins disease-free period, for infrequently, multiple
were also seen in the stroma. Immunohistochemical myeloma can still develop.
staining for kappa and lambda antibodies showed
positive for kappa protein in tumor cell membrane CONFLICTS OF INTEREST
and tumor secretions were positive for kappa
The authors declare they have no potential conflict
proteins with faintly positive for lambda proteins.
of interests regarding this article.
These features suggested it to be a plasmacytoma.
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