Primary spinal leptomeningeal gliomatosis in a 3-year

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Rev Esp Med Nucl Imagen Mol. 2014;33(2):127–128
Interesting image
Primary spinal leptomeningeal gliomatosis in a 3-year-old boy revealed with MRI
and FDG PET/CT mimicking tuberculosis meningitis
Gliomatosis leptomeníngea medular primaria en un niño de 3 años de edad, identificada
por RM y FDG PET/TC imitando meningitis tuberculosa
E.B. Erdogan a,∗ , S. Asa b , S. Yilmaz Aksoy b , M. Ozhan b , A. Aliyev b , M. Halac b
a
b
Department of Nuclear Medicine, Bezmialem Vakif University, Faculty of Medicine, Istanbul, Turkey
Department of Nuclear Medicine, Istanbul University, Cerrahpasa Medical Faculty, Istanbul, Turkey
a r t i c l e
i n f o
Article history:
Received 6 June 2013
Accepted 17 July 2013
Available online 26 September 2013
A 3-year-old boy was referred to neurologist for inward deviation of his left eye, weakness, lack of appetite, headache and
behavioral change. On physical examination, the findings of
bilateral abducens nerve paralysis were noted. Cranial magnetic
resonance imaging (MRI) revealed diffuse leptomeningeal contrast
enhancement at the supra-infratentorial regions predominantly
involving the basal cisterns and also hydrocephalus and initially
suggesting basilar meningitis. Cerebrospinal fluid (CSF) studies
showed normal glucose and elevated protein levels but no tumor
cells. No growth was detected in any of the bacterial cultures
including acid-resistant bacteria. The patient was started on oral
anti-tuberculosis medication and corticosteroid treatment with
the presumed diagnosis of tuberculosis meningitis and a ventriculoperitoneal (VP) shunt was inserted due to hydrocephalus. After
4 months of treatment, the patient was re-admitted to hospital with vomiting, convulsion and confusion. Cranial MRI showed
the similar findings to previous one, and spinal MRI depicted
diffuse leptomeningeal contrast enhancement as well as microcystic changes along the spinal cord (Fig. 1). Histopathology of the
dural biopsy material revealed widespread glioma infiltration of
the subarachnoid space suggestive of leptomeningeal gliomatosis.
This patient with unknown primary was referred for FDG PET/CT.
Under child sedation, brain and whole body non-contrast enhanced
PET/CT images were obtained 90 min after the intravenous injection of 148MBq (4mCi) 18F FDG using a Biograph 6 PET/CT. The
scan depicted slightly increased FDG uptake (SUVmax 2.1)along the
spinal cord without any other FDG-avid primary tumor. The patient
was started on chemotherapy including vincristine and carboplatin
(Figs. 2 and 3). His symptoms were relieved after completion of
chemo.
Primary diffuse leptomeningeal gliomatosis (PDLG) is a rare
disease thought to arise from isolated astrocytic cell nests also
known as heterotropic neuroglial tissue within the leptomeninges
(HLNT) in the subarachnoid space. The HLNT can transform into two
∗ Corresponding author.
E-mail address: erdogan [email protected] (E.B. Erdogan).
Fig. 1. A and B. T1-weighted spin-echo MR images showed diffuse pathologic contrast enhancement at all spinal levels (A). Turbo inversion recovery magnitude
(TIRM) with T2-weighted MR images revealed hyperintensity at these surfaces and
multiple microcystic lesions (B).
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E.B. Erdogan et al. / Rev Esp Med Nucl Imagen Mol. 2014;33(2):127–128
Fig. 2. Sagittal (upper row) and axial (lower row) sections of PET and PET/CT fusion images revealed slightly increased FDG uptake along the spinal cord including cervical
and thoracic regions.
along CSF pathways. However, the diagnosis of PDLG should only be
established in the absence of intraparenchymal tumor. When contrast enhanced MRI of the brain and spinal cord fails to demonstrate
a primary tumor, a clinical antemortem diagnosis can be made.
FDG PET has been used in leptomeningeal gliomatosis to search
for an underlying systemic malignancy.2 Tripathi et al. showed
diffuse leptomeningeal metastases in a case of medulloblastoma
by using FDG PET/CT.3 The usefulness of FDG-PET in differentiating benign from malignant disease and searching for the primary
disease are well established but certain infections including tuberculosis show FDG uptake equivalent to that of tumors. However,
as in our case, the presence of non-improving symptoms despite
anti-tuberculosis and corticosteroid medication let us consider the
diagnosis of malignant processes rather than inflammatory diseases.
Fig. 3. Anterior (A) and lateral (B) view of maximum intensity projection (MIP)
images, and axial PET (C) and axial fusion (D) images did not demonstrate any
primary tumor.
patterns, and the diffuse type has poorer prognosis than solitary
type.1 PDLG initially was misdiagnosed as tuberculosis meningitis
because of characteristic clinical findings and CSF profile. Clinical symptoms and signs of PDLG include headache, cranial nerve
involvement, meningismus, and decreased mental status. PDLG
has to be differentiated from secondary diffuse gliomatosis which
represent leptomeningeal infiltration by a primary parenchymal
glioma, particularly medulloblastoma and glioblastoma, spreading
Conflicts of interest
The authors have no conflicts of interest to declare.
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