Dural plasmacytoma mimicking meningioma in a young patient with multiple myeloma
NN Rahmah*,1,2, MD,
HK Brotoarianto2, MD,
E Andar2, MD,
G Kusnarto2, MD,
Z Muttaqin2, MD,
K Hongo1, MD
1 Department of Neurosurgery, Shinshu University School of Medicine, Matsumoto, Japan
2 Department of Neurosurgery, Diponegoro University, Dr. Kariadi Hospital, Semarang, Indonesia
Part of the paper was presented at AACNS meeting in Nagoya, November 2007
Intracranial involvement in multiple myeloma (MM) is
rarely found, especially with dural involvement. There are only a few cases
found concerning MM with intracranial involvement. MM usually involves an older
group of patients. Cases involving young patients are very rare. The
differential diagnosis of a dural plasmacytoma includes meningioma, metastasis,
lymphoma and sarcoma of the dura mater. We present a young patient, 33 years
old, with MM presenting an intracerebral mass mimicking meningioma on MRI. MM
was diagnosed the previous year. The patient presented with headache, balance
disturbance and back pain. MRI revealed an occipital extra-axial mass with a
dural tail. Histopathological examination after excision showed MM. Published
literatures on intracranial involvement of MM are also discussed. Plasmacytoma
should be considered in the differential diagnosis of a solitary dural mass,
particularly in a patient with MM. � 2009 Biomedical Imaging and
Intervention Journal. All rights reserved.
Multiple myeloma (MM) is a malignant tumor that may
involve the vertebrae and cranium. In the skull, they are typically multiple
lytic lesions, exhibiting a �moth-eaten� appearance on radiograph . The term
plasmacytoma refers to a single lytic lesion of malignant plasma cells
infiltration. From a classical point of view; intracranial plasmacytomas and MM
may involve the cranial vault and/or the skull base only, the brain parenchyma,
arising in the cranial vault and/or the skull base and the orbit . However,
intracranial involvement in plasmacytomas and MM is rare . In this study, an
intracranial mass of dural origin in a patient with one year history of MM is
reported. Review of the literatures is also presented.
A 33-year-old male was referred to the neurosurgical
department by an internist with chronic headache. MRI study (0.5 T) performed
revealed a posterior fossa mass. The mass was located behind the cerebellum,
31.3 x 31.2 x 23.0 mm in size and enhances post contrast (Figure 1). It has
similar appearance to meningioma, but with a past history of MM, intracranial
MM was considered as a differential diagnosis.
The earlier diagnosis of MM was established by chance. The
patient complained of back pain a year ago and underwent several examinations
including plain radiographs. Several lytic lesions of the bones were noticed
(Figure 2). Laboratory examinations performed, were positive for Bence Jones
protein. At the time of referral, he had already started chemotherapy of
He underwent surgery for the posterior fossa lesion.
Preoperatively, the patient was fully alert and showed no neurological deficit.
Suboccipital approach craniotomy was performed. Skin was incised vertically, 5
cm in length, in the midline between the external occipital protuberance and C2
process. Bone was drilled just below the external occipital protuberance and was
removed so that foramen magnum was widened.
The mass was visualized under the occipital bone, and it
was found to have infiltrated the bone. The mass was grayish, soft on palpation
and easily bled. After being cauterized from its surrounding blood supply, the
extradural mass was removed and a small round defect was seen on the dura. The
dura was then incised T-shaped and an intradural mass was visualized. Its
appearance was similar to the extradural mass, and it had to be carefully
cauterized before its removal from the surrounding structures. Arachnoid layer
was perfectly attached and total mass removal was done. Dura was closed by
dural plasty using fascia and the bone defect was left open considering that
muscles in that area were thick enough to replace its function.
Postoperatively, the patient was alert and showed no
additional neurological deficit. Histopathological study with H&E staining
demonstrated a monomorphous appearance of plasma cells with characteristic of
round-oval cells, eccentric nuclei, and abundant cytoplasm. There was also an
increased nuclear to cytoplasmic ratio and some cells had prominent nucleoli
(Figure 3). It confirmed a diagnosis of plasma cells tumor. Unfortunately,
patient died within one year after the diagnosis of intracranial involvement of
MM is a neoplasm of a single clone of plasma cells. It is
characterized by proliferation of plasma cells in the bone marrow, infiltration
of adjacent tissues with mature and immature plasma cells, and the production
of immunoglobulin, usually monoclonal IgG or IgA (referred to collectively as M
protein) . Plasmacytoma is referred to a single lesion without any evidence
of MM in any other part of the body [1,4]. Primary extramedullary plasmacytomas
are uncommon, accounting for 4% of all plasma cell tumors, mainly arising in
the head and neck, particularly the upper aerodigestive tract. Plasmacytomas
generally present as bone or soft-tissue tumors with a variable mass effect,
pain, and infiltrative behavior . The typical clinical features of MM are
bone pain, weakness, fatigue, fever and infection.
Chiang et al in his 11 plasmacytoma-case report stated
that solitary plasmacytoma arises from medullary tissue, and is believed to be
an early manifestation of MM . Within 3 to 5 years, approximately 50% of
these tumors eventually disseminate throughout the skeletal system and become
indistinguishable from MM. Patients are most commonly diagnosed during the
fifth to sixth decade of life, and 70% are men . Schwartz et al reported
intramedullary plasmacytoma progressed to MM more frequently (approximately
50%) than extramedullary plasmacytomas (approximately 30%). Intracranial,
calvarial lesions and most cranial base lesions, being intramedullary, also
have a greater chance of progressing to MM than do dural-based lesions . Nevertheless,
one review of the literature demonstrated that, of 18 calvarial and 13
dural-based lesions, only 2 calvarial lesions progressed to MM . In
contrast, Bindal et al., who presented two cranial base plasmacytomas and
reviewed the literature on intracranial plasmacytomas, observed that all
cranial base-infiltrating plasmacytomas were associated with MM . Extracranially,
MM may take several years to develop from a plasmacytoma .
Interestingly from previous literature, several reports
were made on MM. Some mentioned the progress of solitary plasmacytoma into MM;
others mentioned case reports of patients with MM and metastasized into
intracranial lesions, whereas several reports did not put clear borders between
the two. This patient presented to the authors because of a history of MM;
therefore, the authors would like to find published reports in the literatures
with similar appearance. By excluding those who were admitted with intracranial
plasmacytoma as their first reason for admission, and excluding those who had
no MRI and/or autopsy report confirming the intracranial involvement of MM, 24
cases were found [2,6,9-23]. Details were shown in table 1. Mean age was 56.2
years old, with female predominance. Mean interval before diagnosis of
intracranial involvement was established was 20.57 months. 90% of cases died
within a month after diagnosis was established. 34% of those cases were IgG
kappa MM, and 30.4% were IgG alpha. Petersen et al reported on 54 cases of MM with
myelomatous meningeal involvement and a male/female ratio of 2:4. This type of
involvement occurred more frequently in IgA- and IgD-MM and in Durie-Salmon Stage
III disease. They suggested that advanced stage of MM observed in cases with
circulating plasma cells in the peripheral blood showed a hematogenous spread
of tumor cells to the meninges .
52.2% of cases involved the cranial vault, and 47.8% were
in skull base. Of those, only 1 case involved the posterior fossa dura. Intracranial
plasmacytoma involving the posterior fossa in a young patient such as this
patient is a rare case. The authors found only 24 cases of MM with intracranial
involvement in the literatures, and most of them were beyond 40 years of age.
Ishida et al reported a total of 32 cases of MM in patients aged 30 years and
below in the literatures . The authors' patient died within 1 year after
being diagnosed, and most of the cases found in the literatures also showed
short survival rates. Higurashi et al reported prediction indicators of
progression to MM that were of intramedullary nature, the existence of residual
tumor, and location in the skull base . 60-70% of intramedullary lesions
arising from the bone progress to MM, compared with 10% to 20% of
extramedullary lesions arising from the soft tissues, such as the mucosal
linings of the middle ear, mastoid air cells, and paranasal sinuses. Many of
the skull base lesions that were initially considered to be solitary eventually
progressed to MM . They suggested that most plasma cell myelomas of the
skull base are of intramedullary nature and are actually in the early stage of
MM. On the contrary, plasma cell myelomas of the other cranial locations are
mostly of extramedullary nature and appear to be solitary plasmacytoma .
MRI study in this patient revealed a single oval lesion in
the posterior fossa, which was enhanced homogenously by contrast injection, had
defined border with cerebellum, and was located under the occipital bone. It
appeared similar to meningioma; in addition, the lesion showed an enhanced line
dividing the mass in half which was thought to be a blood supply to the
meningioma. Plasmacytoma can be mistaken with meningioma since it has typical
radiological features as an extra-axial mass with broad attachment to the dura,
hyperdense on CT, isointense with grey matter on T1-weighted MRI, more variable
signal intensity on T2-weighted MRI, and strongly and homogeneously enhancing
with contrast [2,4,11,13,26]. During operation, it was revealed that the mass
was located extra- and intradural, and that enhanced line in the middle of the
lesion was actually the dura itself. Sahin et al found that intracranial
plasmacytomas are usually highly vascular and pathological vessels and a tumor blush
may be seen on angiography. However, the majority of dural or intraparenchymal
plasmacytomas are less vascular and angiography may be normal. Therefore,
angiography is not reliable for differential diagnosis, as 30-50% of
meningiomas may also be associated with normal angiography . Radiological
ﬁndings of intracranial MM or plasmacytoma are not speciﬁc. They
may mimic lymphoma, metastasis, sarcoma of the dura mater, osteochondroma,
infectious meningitis or meningioma [27,28]. The authors also examined the mass
histopathologically. The mass revealed monomorphous plasma cells, which were in
accordance with his history of MM. Dural origin mass and bone-attached mass
were both examined. Both pointed out the same type of cells.
In conclusion, despite its systemic manifestation, MM may
appear as intracranial mass. Although dural inﬁltration is very rare, it
should be considered in the differential diagnosis, particularly if a solitary
intracranial mass with typical features of meningioma is detected on MRI. Its
origin and MRI appearance should be carefully observed before performing
Figure 1 MRI study demonstrated a homogenously enhanced intracranial mass of the posterior fossa. (left) hypointense mass on T1-weighted image, (centre) well-absorbed contrast agent on T1 with contrast agent, (right) note the hyperintense line in the middle of the mass of the sagittal view.
Figure 3 Histopathological study of the intracranial mass, (left) monomorphous spread of plasma cell (40x), (right) round-oval cell with eccentric nuclei and abundant cytoplasm, and increased nuclei cytoplasm ratio, and mitotic phase of plasma cell.
Table 1 Characteristics of Multiple Myeloma Patients with Intracranial Involvement.
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Received 12 November 2008; received in revised form 21 January 2009, accepted 21 January 2009
Correspondence: Department of Neurosurgery, Shinshu University School of Medicine, Matsumoto, Japan. Tel.: +818032027926; Fax: +81263370480; E-mail: firstname.lastname@example.org (Nunung Nur Rahmah).
Please cite as: Rahmah NN, Brotoarianto HK, Andar E, Kusnarto G, Muttaqin Z, Hongo K,
Dural plasmacytoma mimicking meningioma in a young patient with multiple myeloma, Biomed Imaging Interv J 2009; 5(2):e5
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