Title of the article: Ethmoidal Sinus Plasmacytoma with Intracranial extension:
A case report with Clinical and Radiological features
Abstract:Plasmacytoma is a neoplastic proliferation of plasma cells that may manifest as multiple
myeloma, primary amyloidosis, or monoclonal gammapathy of unknown significance.
Plasmacytoma may be primary or secondary to disseminated multiple myeloma and may arise
from osseous (medullary) or nonosseous (extramedullary) sites. Primary extramedullary
plasmacytoma can be solitary or multiple (1). The International Myeloma Working Group in
2003 recognized a separate classification of plasmacytomas that occur as multiple sites of
disease in soft tissue, bone, or both soft tissue and bone as multiple solitary plasmacytoma
(17). Primary extramedullary plasmacytoma is rare, accounting for only 4% of all plasma cell
tumors. Here we report a rare case of extra medullary ethmoidal sinus plasmacytoma with
intracranial extension. The patient had history of seizure with nasal bleeding with swelling on
left side of face. This is a reported case with its clinical, radiological and histological features.
Key words:- Extramedullary Plasmacytoma, Ethmoidal, Intracranial extension.
Introduction:Plasmacytoma is a mass of neoplastic monoclonal plasma cells. It is classified into
medullary (arising in bone) or extramedullary (arising in soft tissue) depending on its origin.
Extramedullary plasmacytoma (EMP) represents 3% of all plasma cell tumors (2), 1% of all head
and neck malignancies and 0.4% of upper respiratory tract malignancies (3). Eighty percent of
them affect the head and neck (4). The main sites of involvement are the nasal cavity, paranasal
sinuses, nasopharynx and the oral cavity. Men are affected 3 times more than women (5) with
average age presentation of 60-70 years (6). Local lymphnode affection occurs in 10 to 20 % of
extramedullary plasmacytoma is converted to multiple myeloma (7). Metastasis occurs in 35 to
50% of cases (8) its diagnosis depends on histology and by immunocytochemistry (9) EMP
microscopically consists of sheets of plasma cells which may be monomorphous or pleomorphic
(10). By immunohistochemical demonstration of one light chain monoclonal staining and one
heavy chain class, most EMP can be differentiated form reactive plasma cell infiltrates with
polyclonal staining (10). It is divided into 3 grades according to its cellular a typia into low,
intermediate and high grade (11). Multiple myeloma should be excluded by serum and urine
protein electrophoresis and immunoelectrophoresis , skeletal survey, bone scan and marrow
biopsy (5). It should be differentiated from other destructive diseases in the maxillary sinus
such as olfactory neuroblastoma, lymphoma, anaplastic carcinoma and metastatic tumors (12).
Extramedullary plamacytomas are highly radiosensitive, so radiotherapy is the best choice of
treatment (2). Fifty percent of EMP patients survive more than 10 years (6). However, Maxillary
sinus EMP is associated with poorer prognosis than other sites of head neck especially if it is
associated with bone destruction (3). Long- term follow up is mandatory, as local recurrence
and dissemination can occur many years after the original lesion has been treated (13).
Chemotherapy may be added to treatment if there is recurrence of metastasis. This is a report
of solitary EMP in the etmoidal sinus in a 50-year old man.
Case Report:A 60 years old patient presented to medicine OPD of Mahatma Gandhi Medical College
& Hospital, Sitapura Jaipur, Rajasthan with history of seizure and nasal bleeding for last 15 days
and past history of left sided facial swelling and upper eye lid partial closure for three months.
On examination his general condition was good, B.P. 120/80, Pulse 80/min., Chest and
Heart were clinically normal.
CT scan of Head and PNS showed 6 X 5cm soft tissue large ethmoidal mass with mild
hetrogenenous enhancement occupying left ethmoid sinus extending into the frontal sinus,
extrachoanal compartment of left orbit and anterior cranial fossa destructing the cribriform
plate, bilateral lamina papyracea. A non enhancing white matter hypodensity in left frontal lobe
is also seen suspicious of brain parenchymal involvement in the frontal region.
MRI of Brain was performed which revealed 6X5X3cm well defined mass lesion
isointense on T1 and hyperintense on T2 in anterior sino-nasal region extending into bilateral
fronto-ethmoid sinuses and extra coronal space of both orbits causing bone destruction
involving the cribriform plate, bilateral lamina papyracea and extending to the frontal region
intracranially.
MR Spectroscopy revealed raised Choline peak at the site of lesion in ethmoid and intracranially
indicating malignant neoplasm.
Biopsy was taken from the mass and was sent for histopathological analysis and the
report showed: Microscopically cells were positive for CD138 and EMA but negative for CD19.
Morphology favors plasma cell neoplasm with very low KI67.
Discussion:Schridde in 1905 was the first describe extramedullary plasmacytoma. 80% of the
extramedullary plasmacytoma of the head and neck arise from the submucosa of upper
aerodigestive tract (7). Solitary extramedullary plasmacytoma of the paranasal sinuses are
uncommon and of B lymphocyte origin (4). To diagnose solitary EMP, one year of follow up
from the original diagnosis should pass without evidence of skeletal radiological deposits as
EMP may long precede occult but nevertheless disseminated myelomatosis (14).
This is a rare case of left ethmoid sinus extramedullary plasmacytoma. The patient
presented with history of seizure and nasal bleeding for last 15 days and left sided facial
swelling and upper eye lid partial closure for three months. CT scan of Head and PNS showed
6X5cm soft tissue large ethmoidal mass with mild heterogeneous enhancement occupying left
ethmoid sinus extending into the frontal sinus, extrachoanal compartment of left orbit and
anterior cranial fossa destructing the cribriform plate, bilateral lamina papyracea. A non
enhancing white matter hypo density in left frontal lobe is also seen suspicious of brain
parenchymal involvement in the frontal region. MRI of Brain revealed 6 X 5 X 3cm well defined
mass lesion isointense on T1 and hyperintense on T2 in anterior sino-nasal region extending
into bilateral fronto-ethmoid sinuses and extra coronal space of both orbits causing bone
destruction involving the cribriform plate, bilateral lamina papyracea and extending to the
frontal region intracranially. MR Spectroscopy revealed raised Choline peak at the site of lesion
in ethmoid and intracranially indicating malignant neoplasm.
Kamal-Eldin A. et. al reported a case of left maxillary sinus extramedullary
plasmacytoma in a 50 year old male patient in 2008 (18). The patient had recurrent epistaxis,
nasal obstruction, exopthalmos, left facial swelling and bulging of hard palate on same side
following trauma. There was soft tissue swelling filling the maxillary sinus with bony erosion
and extension into ethmoid sinuses, orbits, subcutaneous with associated sinusitis in sphenoid
sinus and opposite maxillary sinus. There was no intracranial extension.
Majumdar etal reported a case of right maxillary extramedullary plasmacytoma in a 53
year old woman in 2002 (7). In this case, maxillary destruction was much less than in our case
although the patient also presented with proptosis and recurrent epistaxis. There were no MRI
findings of the mass.
Gromer and Duvall reported 7 cases of EMP, 2 of them in the left maxillary sinus, one in
the right maxillary sinus, 2 in the nasal cavity (13). One of them had local cervical node
metastasis, another one converted to multiple myeloma and one with distant metastasis. Singh
et al. reported 3 cases of EMP, one of them in the left maxillary sinus which recurred one year
after total maxillectomy and one nasal case (14). There was report of a case of right maxillary
and nasal cavity EMP destructing the bony structures in all directions with a history of trauma
and surgery for the nose 6 months earlier to its presentation (15). It is same like our presenting
case. Out of 22 cases of EMP reported in Birmingham in the period 1963-1973, 2 cases involved
the left maxillary sinus in which recurrence occurred 7 years after maxillectomy in one of them
(16). Another report of 3 nasal cases of EMP was published one of them involved the ethmoid
sinus and another one converted into multiple myeloma after one year (10).
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