A 31 year old Saudi football Coach presented to King Faisal Specialist
Hospital and Research Centre (KFSH&RC) with three month history of occipital
headache on exercising, and oscillopsia when focusing on a particular
object. No abnormality was detected on complete physical examination.
A CT scan of the brain revealed a markedly enhancing, well?demarcated
lesion with bone destruction (Fig. 1).
The patient was referred to the Neurosurgery Department at KFSH&RC for
further management of the proposed diagnosis of intracranial meningioma.
MRI of the brain showed the extraaxial lesion had Tl hyperintensity
and T2 isointensity, with marked homogenous enhancement (Fig. 2). There
was no mention of the results of complete blood count and renal studies
in the report from the referring hospital. Complete blood count and
blood analysis was completed on admission to KFSH&RC which gave the
following concentrations (range in parenthsis): haemoglobin 62 g/L (132?172),
platelets 80 x 109/L (150?430), leukocytes 6.4 x 109/L (3.4?9.3), urea
9.9 mmol (4.2?7.2), creatinine 249 umol/L (60?115), calcium 2.67 mmol/L
(2.1.2.5), phosphorous 1.99 mmol/L (0.7?1.45). Urine analysis showed
microhaematuria, protein 4.17 g/24 hr (0.05?0.1), and the creatinine
clearance was 0.8 ml/sec (1.3?2.1). Bone marrow biopsy showed normal
marrow replaced with small plasma cells which had deeply basophilic
cytoplasm and round eccentric nucleus with peripheral condensation of
chromatin. Electrophoresis showed a monoclonal band of IgG lambda in
the beta region. The lambda light chains were present on urine immunofixation
electrophoresis. B2 microprotein level was 5.6 mg/L (1.12.4). The patient
was started on hydration therapy and allopurinol 300 mg daily for 10
days, followed by Melphalan 16 mg and Prednisone 50 mg daily for five
days, then Prednisone 25 mg daily for four weeks. He had five sessions
of plasmaphersis followed by two courses at an interval of three weeks,
of Melphalan 16 mg daily for five days. Follow up CT scan of the brain
showed complete regression of the lesion (Fig. 3), confirming its myelomatous
nature. The patient was lost to follow up for one year. When he was
next seen, he was in renal failure and put on dialysis. Skeletal X?ray
survey revealed multiple punched?out lesions. His condition gradually
deteriorated and he expired 26 months after his first presentation.
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Figure
1a — CT scan showing uniformly
enhancing mass. |
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Figure
1b — CT scan showing bony windows
with bone destruction because of the intracranial lesion. |
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Multiple myeloma, a monoclonal gammopathy, is characterised by single
B?cell clonal proliferation with a remarkable potential for focal destruction
of bone, especially in the vertebrae, ribs and skull. The incidence
in males is twice that in females; peak incidence is between 50?70 years
of age. The lesions are characteristically multiple, punched?out and
osteolytic. Localised growth of multiple myleloma occurs in 5% of cases.(5)
Although approximately 80% of myeloma patients will have punched?out
osteolytic lesions in different sites, the skull is the most frequently
affected. (16) Intracranial growth has rarely occurred; the incidence
given in the literature is from 0.03% to 0.7%.(2,5) In the cranium,
the growth of the neoplastic tissue is usually directed towards the
outside, occasionally towards the epidural space. The dura may be involved
and infiltrated by tumour cells, but this is not usual. (7,21,25) Primary
nervous system involvement, in the form of intracranial spaceoccupying
lesion, is rare and secondary involvement is occasional. (7,8,18) The
clinical presentation includes asymptomatic meningeal involvement, (1)
encephalopathy, (17,26) cranial nerve palsies, (4,6,9,13,19,30) neuropathy,
(28,29) convulsions or hemiparesis, (9) obstruction of the superior
sagital sinus, (22) or features of increased intracranial pressure and
possibly uncal herniation. (13,22) Spontaneous haemorrhage into the
tumour has been reported: (14,29) this may be related to increased vascularity
of the lesion, or the bleeding diathesis associated with the disease.
Radiologically, the intracranial growth is usually mistaken for meningioma.
(2,10,14,19) The most characteristic features include osteolytic lesions
on plain skull X?ray, although intracranial plasmacytoma may occur without
evidence of bone erosion. (15) On CT scan the lesion is usually slightly
hyperdense with homogenous enhancement following injection of contrast
material. (2,14,19,23) Angiographic studies usually show a highly vascular
lesion. (2,19) In spite of similarities between meningioma and intracranial
plasmacytoma on radiological studies, the most important features which
can differ between plasmacytoma and meningioma is the short duration
between the onset of symptoms, the development of signs of increased
intracranial pressure (3) and prominence of the extracranial arteries
supplying the lytic area. Histologic changes in the nervous tissue include
acute swelling of nerve cells, cytoplasmic fatty infiltration, shrinkage,
excessive deposit of lipofuscin, demyelination and decrease in the number
of nerve fibres, and dystrophic changes in the glia. (15,27) Treatment
of multiple myeloma has included chemotherapy, (9,6,14) radiotherapy,
(4,6) the administration of alpha interferon, (1) and surgical removal.
(2,8,12,19,20)
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Figure
2 — T1 weighted MRI showing hyperintense
mass occupying the supra and infratentorial regions. |
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Figure
3 — Post treatment CT scan showing
completely resolved mass. |
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In the case reported here, there was complete disappearance of the intracranial
lesion following medical therapy. Central nervous system (CNS) screening
of patients with multiple myeloma is recommended to identify those at
risk of developing CNS involvement.(9) Progression of the localised tumour
into classical multiple myeloma was noted. (5,14,19) Bindal et al, reported
on eight cases of intracranial plasmacytoma. They concluded that cases
which did not show evidence of multiple myeloma in the immediate presentation/post
operative period did not have progression into multiple myeloma during
long term follow up period. (8) It should be remembered that simple routine
investigations will provide important clues for diagnosis. Complete blood
count reveals anaemia in 80% of patients with multiple myeloma. This is
usually of the normocytic normochromic type. The anaemia is either secondary
to invasion of the bone marrow or to hematopoiesis inhibition by circulating
tumour factors. (11) Erythrocyte sedimentation rate is elevated. (11)
Serum calcium, uric acid, urea, and creatintine levels may be ele vated.
The urine will show Bence?Jones protein in 75% of the myeloma patients.(24)
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