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Neuropathology 2015; 35, 165–169
doi:10.1111/neup.12156
C a se Repor t
Lipoastrocytoma: A rare case report and review of literature Divya Sharma and Nita Khurana Department of Pathology, Maulana Azad Medical College and Associated LNJP Hospital, New Delhi, India
Lipoastrocytoma is an extremely rare tumor, with only a few cases described. We report a case of a low-grade astrocytoma occupying the right cortical lobe in the parafalcine location. The patient was admitted with headache, vomiting and altered sensorium for duration of 1 year. MRI revealed a large heterogeneous enhancing mass in the right fronto-parieto-temporal lobe with intratumoral fat along with cystic changes and calcification (correlated with CT) showing mass effect in the third ventricle. A gross total excision of the tumor was performed. Histologically, the tumor showed glial cells that contained lipid droplets coalescing into a single large droplet, similar in appeareance to adipocytes. Immunohistocemically, tumor cells strongly expressed GFAP and S-100 protein. Ki-67 labelling index was low. The patient remained in good neurological condition at 3 months follow-up. This case represents a rare variant of low-grade astrocytoma that is designated as lipoastrocytoma. Key words: astrocytoma.
astrocytoma,
lipomatous,
low
grade
INTRODUCTION Lipomatous transformation is a rare but well characterized finding in a variety of neuroectodermal tumors of the CNS.1 Lipid may accumulate within the cytoplasm of tumor cells as multiple droplets giving a xanthomatous appearance as demonstrated in pleomorphic xanthoastrocytoma (PEXA),2,3 glioblastoma4 and hemangioblastoma.5 This must be distinguished from lipomatous tumor cells which have fat droplets coalescing into a single clear vacuole pushing the nucleus to the periphery, resembling a mature adipocyte. The latter phenomenon is observed
in ependymomas,6 central liponeurocytomas,7 cerebellar neurolipocytomas8 and low-grade astrocytomas.9,10 We report the case of a low-grade astrocytoma with a diffuse lipomatous component.
CLINICAL SUMMARY A 12-year-old girl was admitted with complaints of headache, vomiting and altered sensorium for 1 year. Noncontrast computed tomography (NCCT) head scan revealed a large heterogeneous mass lesion with internal areas of fat attenuation and wall calcification in the right frontoparietotemporal region with mass effect (Fig. 1). In view of the above features, a diagnosis of teratoma was suggested and MRI was advised for correlation. On examination at admission, the child was drowsy with opisthotonic posture. MRI showed a lobulated mass measuring 10 × 9 × 8 cm in the right parafalcine location with extension into the right lateral ventricle and exhibiting mass effect in the form of midline shift toward the left with compression of the third ventricle and posterior displacement of the right thalamus. The lesion was heterogeneously hyperintense, suggestive of fat with post-contrast enhancement (Fig. 2). Cystic areas along with multiple foci of calcification, perilesional edema and noncommunicating hydrocephalus was also noted. Bilateral cerebellar hemispheres appeared normal in morphology. A complete resection of the tumor was performed. Intraoperatively, the tumor was soft to firm, lobulated, vascular and removed piecemeal. The patient did well after surgery without any neurological deficits.
PATHOLOGICAL FINDINGS Correspondence: Divya Sharma, MD, 1F, Shivalika Apartment, Plot 16, Sector-9, Dwarka-110075 Delhi, India. Email: sharmadivya20@ rediffmail.com Received 22 June 2014; revised and accepted 18 August 2014; published online 28 September 2014.
© 2014 Japanese Society of Neuropathology
Histologically the tumor was composed of welldemarcated cellular glioma composed of cells with eosinophilic cytoplasm with irregular nuclear contours and small nucleoli. Some cells showed a spindle cell morphology with
166
D Sharma and N Khurana
elongated cells and eosiniphilic fibrillary processes. There were extensive areas of tumor cells showing cytoplasmic vacuolization due to lipidization, thus resembling adipocytes (Fig. 3). Eosinophilic granular bodies were abundant without any Rosenthal fibers. Occasional foci of calcification were observed and no necrosis and vascular
proliferation was encountered. The tumor cells were not associated with reticulin fibers. Mitosis activity was low (
Neuropathology 2015; 35, 165–169
doi:10.1111/neup.12156
C a se Repor t
Lipoastrocytoma: A rare case report and review of literature Divya Sharma and Nita Khurana Department of Pathology, Maulana Azad Medical College and Associated LNJP Hospital, New Delhi, India
Lipoastrocytoma is an extremely rare tumor, with only a few cases described. We report a case of a low-grade astrocytoma occupying the right cortical lobe in the parafalcine location. The patient was admitted with headache, vomiting and altered sensorium for duration of 1 year. MRI revealed a large heterogeneous enhancing mass in the right fronto-parieto-temporal lobe with intratumoral fat along with cystic changes and calcification (correlated with CT) showing mass effect in the third ventricle. A gross total excision of the tumor was performed. Histologically, the tumor showed glial cells that contained lipid droplets coalescing into a single large droplet, similar in appeareance to adipocytes. Immunohistocemically, tumor cells strongly expressed GFAP and S-100 protein. Ki-67 labelling index was low. The patient remained in good neurological condition at 3 months follow-up. This case represents a rare variant of low-grade astrocytoma that is designated as lipoastrocytoma. Key words: astrocytoma.
astrocytoma,
lipomatous,
low
grade
INTRODUCTION Lipomatous transformation is a rare but well characterized finding in a variety of neuroectodermal tumors of the CNS.1 Lipid may accumulate within the cytoplasm of tumor cells as multiple droplets giving a xanthomatous appearance as demonstrated in pleomorphic xanthoastrocytoma (PEXA),2,3 glioblastoma4 and hemangioblastoma.5 This must be distinguished from lipomatous tumor cells which have fat droplets coalescing into a single clear vacuole pushing the nucleus to the periphery, resembling a mature adipocyte. The latter phenomenon is observed
in ependymomas,6 central liponeurocytomas,7 cerebellar neurolipocytomas8 and low-grade astrocytomas.9,10 We report the case of a low-grade astrocytoma with a diffuse lipomatous component.
CLINICAL SUMMARY A 12-year-old girl was admitted with complaints of headache, vomiting and altered sensorium for 1 year. Noncontrast computed tomography (NCCT) head scan revealed a large heterogeneous mass lesion with internal areas of fat attenuation and wall calcification in the right frontoparietotemporal region with mass effect (Fig. 1). In view of the above features, a diagnosis of teratoma was suggested and MRI was advised for correlation. On examination at admission, the child was drowsy with opisthotonic posture. MRI showed a lobulated mass measuring 10 × 9 × 8 cm in the right parafalcine location with extension into the right lateral ventricle and exhibiting mass effect in the form of midline shift toward the left with compression of the third ventricle and posterior displacement of the right thalamus. The lesion was heterogeneously hyperintense, suggestive of fat with post-contrast enhancement (Fig. 2). Cystic areas along with multiple foci of calcification, perilesional edema and noncommunicating hydrocephalus was also noted. Bilateral cerebellar hemispheres appeared normal in morphology. A complete resection of the tumor was performed. Intraoperatively, the tumor was soft to firm, lobulated, vascular and removed piecemeal. The patient did well after surgery without any neurological deficits.
PATHOLOGICAL FINDINGS Correspondence: Divya Sharma, MD, 1F, Shivalika Apartment, Plot 16, Sector-9, Dwarka-110075 Delhi, India. Email: sharmadivya20@ rediffmail.com Received 22 June 2014; revised and accepted 18 August 2014; published online 28 September 2014.
© 2014 Japanese Society of Neuropathology
Histologically the tumor was composed of welldemarcated cellular glioma composed of cells with eosinophilic cytoplasm with irregular nuclear contours and small nucleoli. Some cells showed a spindle cell morphology with
166
D Sharma and N Khurana
elongated cells and eosiniphilic fibrillary processes. There were extensive areas of tumor cells showing cytoplasmic vacuolization due to lipidization, thus resembling adipocytes (Fig. 3). Eosinophilic granular bodies were abundant without any Rosenthal fibers. Occasional foci of calcification were observed and no necrosis and vascular
proliferation was encountered. The tumor cells were not associated with reticulin fibers. Mitosis activity was low (
