S. Nawaiseh, S. Al-Assaf, A. Al-Qudah, A. Abu Al-Samen

SOLITARY EXTRAMEDULLARY PLASMACYTOMA OF
THE TONSIL
Sufian T. Nawaiseh MD*, Salman M. Al-Assaf, MD*, Ahmad M. Al-Qudah, MD*,
Ahmad A. Abu Al-Samen, MD**
ABSTRACT
We report a rare case of extramedullary plasmacytoma of the left tonsil in a 45 year old male patient. He
presented with a two months history of left sided throat discomfort, clinical examination revealed a left sided
diffuse tonsillar enlargement. Histopathologic examination of the tonsillectomy specimen was consistent with
plasmacytoma. Serum electrophoresis failed to detect any myeloma component. Assays for Bence Jones
protein were negative. All other screening tests to rule out multiple myeloma were negative. These findings
confirmed the diagnosis of extramedullary plasmacytoma. The patient received full course of radiotherapy and
he is currently without evidence of disease twelve months postoperatively.
Key words :Extramedullary plasmacytoma , multiple myeloma , tonsil
JRMS August 2009; 16(2): 51-53
Introduction
Plasmacytoma is traditionally divided into
medullary and extramedullary type, which in turn
could be either solitary or multiple in distribution.
Solitary primary extramedullary plasmacytoma
(EMP) is a neoplasm of the plasma cells arising in
regions other than bone marrow in patients with no
clinical or biochemical evidence of multiple
myeloma. (1) EMP represents up to 4% of
nonepithelial tumours of the upper respiratory tract.
They generally occur in the submucosal tissue of the
upper airways (80% of cases), with a predilection
for nasopharynx, nasal cavity, paranasal sinuses and
tonsils. (2,3)
Case Report
We report a case of solitary EMP of the left tonsil
in a 45-year-old male who presented to ENT clinic
at Queen Alia Military Hospital with left sided
throat discomfort of two months duration. Clinical
evaluation revealed a left sided diffuse tonsillar
enlargement, with smooth surface and hard
consistency. Constitutional symptoms were absent
in this patient and no lymphadenopathy or
organomegaly was noted. Tonsillectomy was done
and both tonsils were removed. The histopathologic
examination of the left tonsil showed tonsillar tissue
with effaced architecture replaced by a sheath of
tumor cells with abundant cytoplasm and eccentric
nuclei. Adjacent to the tumor the tonsil showed
intense lymphoid infiltrate with germinal center
formation (Fig.1).
Immunostains were done and the plasmacytoid
cells revealed positive stain for lambada light chain
and IgG and negative stain for kappa light chain,
CD20, CD79a, BCL2, LCA ABD CD45 Ro. The
appearances were consistent with Extraosseous
(tonsillar ) plasmacytoma (Fig. 2). The right tonsil
section showed reactive lymphoid tissue with no
evidence of malignancy.
From the Departments of:
*ENT, Queen Alia Military Hospital, (QAMH), Amman-Jordan
**Pathology, (QAMH)
Correspondences should be addressed to Dr. S. Nawaiseh, P. O. Box 396 Amman 11732 Jordan, E-mail: sufiannawaiseh@hotmail.com
Manuscript received January 23, 2006. Accepted November 13, 2006
JOURNAL OF THE ROYAL MEDICAL SERVICES
Vol. 16 No. 2 August 2009
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upper airway. manuscript.
The first case of EMP was reported in 1905.
Approximately 80% of cases occur in the upper
respiratory tract, and 10 to 20% manifest as multiple
lesions. (4) The most frequently affected areas are the
nasal cavity and paranasal sinuses, followed by the
nasopharynx, tonsils, larynx and the pharynx. (1-7) It
usually occurs in patients between 50 and 60 years
of age and three fourths of soft tissue plasmacytoma
cases involve males. (3,5,8)
EMP of upper respiratory tract may be single or
multiple, and form polypoid or pedunculated masses
or diffuse swellings. Tumor ulceration is infrequent
and occurs later in the disease. EMP may spread
Fig. 1. Tonsillar tissue with effaced architecture
from the primary site locally to the regional lymph
replaced by a sheath of tumor cells with abundant
nodes or adjacent bone or results in systemic
cytoplasm and eccentric nuclei.
dissemination. Stage I is defined as tumor confined
to the primary site, stage II as invasion of the
draining lymph nodes and stage III as metastatic
spread. (9)
Histopathological analysis alone is not sufficient in
order to make a diagnosis of primary EMP; multiple
myeloma must be excluded by a thorough skeletal
and marrow workup, complete blood count, kidney
and liver function test, immuno-electrophoresis and
quantitative immunoglobulins with absent urinary
Bence Jones proteins. (1,4-6)
Treatment of solitary EMP consists primarily of
eradication of the local lesion and in this context
surgery seems to be the primary line of treatment.
Fig. 2. Plasmacytoid cells revealed positive stain for
lambada light chain
Radiation therapy either alone in cases deemed
unsuitable for surgery or as an adjunct with surgical
removal has also been used. Local recurrences are
The patient had full work-up done including
complete blood count and blood chemistry, serum
also usually treated by radiation therapy. (1,4,5,9)
Intensive radiation therapy with at least 4500cGy
protein electrophoresis, which were all covering all apparent disease and generous margins
unremarkable. Findings on bone marrow biopsy and
on radiographic and nuclear medicine bone surveys
were also unremarkable. Assays for Bence Jones
protein in urine and serum were negative. These
results confirmed the diagnosis of EMP of the left
tonsil. The patient received a radiotherapy course of
5000 cGy in the form of 200 cGy fractions delivered
over six weeks and he is currently (twelve months
postoperatively) without evidence of disease.
of normal tissue, is enough to eradicate the tumor in
most patients. (5,9)
Although a favorable prognosis of EMP when
treated locally by irradiation and/or surgery were
reported by many studies. (2,4,5,8) It should be kept in
mind that each individual clinical course is
unpredictable and that the development of multiple
myelomas have been observed in 10 to 32% of
patients 28 to 36 years later, therefore lifetime
follow-up is mandatory. (4)
Discussion
EMP is a rare neoplastic lesion that may appear in
the head and neck. It is characterized by monoclonal
Acknowledgment
The authors would like to thank Prof. Hesham
proliferation of plasma cells (3) and occur Negm, From the Department of
predominantly in the head and neck area with a
tendency to involve the submucosal tissues of the
Otorhinolaryngology of Cairo University for his
support and help in the preparation of this
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