Cerebellar liponeurocytoma Sreedhar Babu et al Case Report: Cerebellar liponeurocytoma: a case-report

K.V. Sreedhar Babu,1 A.K. Chowhan,2 N. Rukmangadha,2 B.C.M. Prasad,3 M. Kumaraswamy Reddy2 Departments of 1Immuno Haematology & Blood Transfusion, 2Pathology and 3Neurosurgery, Sri Venkateswara Institute of Medical Sciences, Tirupati ABSTRACT

Cerebellar liponeurocytoma is a rare cerebellar neoplasm of adults with advanced neuronal / neurocytic and focal lipomatous differentiation, a low proliferative potential and a favorable clinical prognosis corresponding to World Health Organization grade I or II. Only a few cases have been described in the literature (approximately 20 cases) by different names. A 48-years old female, presented with history of headache and dizziness associated with neck pain; restricted neck movements, drop attacks and occasional regurgitation of food since one year. Magnetic resonance imaging disclosed a right cerebellar mass lesion. Gross total resec- tion of the tumour was accomplished through a suboccipital craniotomy. The excised tissue was diagnosed as cerebellar liponeurocytoma, a rare entity, based on histopathological examination and immunohistochemistry. The morphological appearance of this neoplasm can be confused with that of , , , , solid hemangioblastoma and metastatic carcinomas etc., with unpredictable prognosis, which require postoperative radiotherapy, hence the importance of accurately diagnosing this rare neoplasm. This tumour should be added to the differential diagnosis of mass lesions of the posterior fossa. Key words: Liponeurocytoma, Cerebellum, Posterior cranial fossa

Sreedhar Babu KV, Chowhan AK, Rukmangadha N, Prasad BCM, Kumaraswamy Reddy M. Cerebellar liponeurocytoma: a case- report. J Clin Sci Res 2012;1:39-42.

INTRODUCTION liponeurocytoma with clinical, histological, and radiological studies. Cerebellar liponeurocytoma (ICD-O Code - 9506/1) is a rare tumour of the posterior CASE REPORT fossa that has been reported fewer than 20 times in the literature. This uncommon tu- A 48-year old female patient presented 1 with head ache and dizziness of one year mour was first described by Bechtel et al. in 1978 in a 44-year old man. The terms duration associated with neck pain, re- 2 stricted neck movements, drop attacks and neurocytoma /lipoma (neurolipocytoma), occasional regurgitation of food. On physi- lipomatous medulloblastoma,3 lipidized cal examination there was mild motor defi- medulloblastoma,4 medullocytoma,5 lipo- 6 cit in the right upper limb, loss of pain and matous glioneurocytoma and lipidized ma- touch sensations by 20% of normal, right ture neurectodermal tumour of the cerebel- 7 upper motor paralysis of VII lum have been proposed, so as to empha- nerve and positive cerebellar signs on size its similarity to central neurocytoma ipsilateral side. and the prognostic difference from the ordi- Investigations revealed neutrophilic leuko- nary medulloblastoma. It has been sug- cytosis with elevated erythrocyte sedimen- gested in the available literature that these st 3,5 tation rate (ESR) (38 mm at the end of 1 tumours have a favorable prognosis. hour) and normal biochemical parameters. Cerebellar liponeurocytomas were recog- Magnetic resonance imaging (MRI) nized in the revised WHO classification of showed heterogeneously enhancing mass tumours of the lesion in the right cerebellar hemisphere 2000, as a distinct clinicopathological entity.8 Here, we report a case of cerebellar

Received: 7 January, 2012. Corresponding author: Dr Kinnera Vijay Sreedhar Babu, Associate Professor, Department of Immuno Hae- matology & Blood Transfusion, Sri Venkateswara Institute of Medical Sciences Tirupati 517 507, India. e-mail: [email protected] Cerebellar liponeurocytoma Sreedhar Babu et al

Involving vermis with compressed and effaced Histopathological examination fourth ventricle and transtentorial herniation. revealed cerebellar tissue with the With the clinical and radiological suspicion tumour component exhibiting of either high grade or me- biphasic pattern comprising dulloblastoma, the patient underwent of isomorphic small neuronal cells suboccipital craniotomy and excision of the with round to oval nuclei and clear tumour. The excised specimen was grey- cytoplasm. These cells are seen as reddish colored fragmented tissue in toto sheets and at areas densely populated measuring 5.5×5.0×1.2 cm. The entire tissue with indiscernible cytoplasm, highly was subjected for histopathological pleomorphic nuclei with atypical examination followed by mitotic figures. The tumour is signifi- immunohistochemistry (IHC) for further cantly vascular (plexiform capillary confirmation. The postoperative course was arcade). Fine fibrillar intervening uneventful and the patient was discharged on matrix, psammoma body like the tenth postoperative spherules of calcifications, foci of day.

Figure 2: Photomicrograph showing Figure 1: Photomicrograph showing cerebellar pleomorphic tumour cells, mildly acidophilic tissue with tumour component (Haematoxylin and to clear cytoplasm with intervening nucleus eosin × 400) - free areas of neuropil (Haematoxylin and eosin × 400)

Figure 4: Photomicrograph showing tumour Figure 3: Densely populated highly pleomorphic com- ponent exhibiting lipomatous tumour cells with indiscernible cytoplasm and differentiation and foci of microscystic atypical mitotic figures (Haematoxylin and eosin degeneration (Haematoxylin and eosin × 400) × 400)

Cerebellar liponeurocytoma Sreedhar Babu et al

Figure 5: Photomicrograph showing tumour component with intervening lipidized cells Figure 6: Photomicrograph showing tumour and psammoma body like calcific spherule component with immunoreactivity for GFAP (Horse (Haematoxylin and eosin × 400) radish peroxidase × 400)

Figure 7: Photomicrograph showing tumour Figure 8: Photomicrograph showing tumour component with immunoreactivity for NSE. component with immunoreactivity for synapto- (Horse radish peroxidase×400) physin (Horse radish peroxidase×400).

lipoid cell change and microcystic This suggests that the fat containing degeneration present (Figures 1, 2, 3, 4 cells result from lipomatous differentia- and 5). Immunohistochemically these tion of tumour cells, rather than admix- neoplastic cells were reactive for glial ture of non-neoplastic adipocytes9. Since fibrillary acidic protein (GFAP), neuron 2000, the WHO Central Nervous specific enolase (NSE) and System tumours classification includes synaptophysin (Figures 6, 7 and 8). a subset called cerebellar liponeurocy- Basing on these findings a diagnosis of toma.10 These tumours tend to occur in cerebellar liponeurocytoma with focal older patients ranging from 36 to 77 aggressive areas [World Health years of age, with a mean age of Organization (WHO) Grade II] was approximately 53 years.2-5 Cerebellar made. liponeurocytoma has a relatively DISCUSSION benign clinical course and a recurrence Cerebellar liponeurocytomas are may appear after a long period of neurectodermal tumours consisting of both time.2,4-6,8 Reviews published in the neuronal and glial elements. Immuno- literature show a 5 year survival rate of histochemistry for GFAP, synaptophysin 81%11 but Jenkinson12 reported a patient and NSE are usually positive indicating who developed a recurrence 12 months the mixed glial and neuronal elements. after a subtotal resection. His case Histogenetically immunoreactivity to demonstrated an atypical clinical course neuronal antigens and GFAP includes cell of a highly aggressive and radiation bodies embracing fat globules.

Cerebellar liponeurocytoma Sreedhar Babu et al

-resistant tumour, despite the consistent vorable prognosis. Human Pathol 1994;25:730-1. absence of aggressive histological features. 4. Davis DG, Wilson D, Schmitz M, Markesbery Radiological diagnosis is difficult due to WR. Lipidized medulloblastoma in adults. Human the rarity of the tumour and a variable Pathol 1993;24:990-4. imaging appearance.13 The most 5. Giangaspero F, Cenacchi G, Roncarolli F, challenging differential diagnosis of these Rigobello L, Manvetto V, Gambacorta M, tumours is to distinguish them from Alle- granza A. Medullocytoma (lipidized , , medulloblastoma). A cerebellar neoplasm of , solid hemangioblastomas, adults with favourable prognosis. Am J Surg and metastases. MRI Pathol 1996;20:656-64. appearance is variable and may be 6. Alleyne CH, Hunter S, Olson JJ, Barrow DL. related to the distribution Lipomatous glioneurocytoma of the posterior 4 and proportion of lipidized tissue. fossa with divergent differentiation: Case Surgery to establish the diagnosis and report. Neurosurgery 1998;42:639-43. remove the lesion should be the initial 7. Gonzalez-Campora R, Weller RO. Lipidized therapeutic manoeuver. The aim of the mature neuroectodermal tumor of the cerebellum surgery is gross total resection of the with myoid differentiation. Neuropathol Appl tumour. In most of cases there is a reason- Neurobiol 1998;24:397-402. able border between the tumour and 8. Kleihues P, Louis DN, Scheitauer BW, 11,13 surrounding tissue and gross total Rorke LB, Reifenberger G, Burger PC, Cavenee removal of the tumour is possible. The WK. The WHO classification of tumors of the recent 2000 WHO classification of nervous system. J Neuropathol Exp Neurol tumours of the nervous system describes a 2002;61:215-25. 10 new entity, cerebellar liponeurocytoma. 9. Budka H. Intracranial lipomatous This rare neoplasm manifests with hamartomas (intracranial “lipomas”). A study of cerebellar symptoms in adults and is 13 cases including combinations with characterized histopathologically by the medulloblastoma, colloid and epidermoid cysts, presence of the tumour with neuronal, angiomatosis and other malformations. Acta astrocytic and focal lipomatous Neuropathol (Berl) 1974;28:205-22. differentiation having low proliferative 10. Kleihues P, Chimelli L, Giangaspero F. Cere- potential in an otherwise typical primitive bellar liponeurocytoma. In: Kleihues P, neuroectodermal neoplasm. The present Cavenee WK, editors, Pathology and Genetics of case looks aggressive which warrants the Nervous System. Lyon: WHO classification close follow up. Most of the information of tumors 2000:110-11. available from previous case reports 11. Jackson TR, Regine WF, Wilson D, Davis indicate that this tumour has a favorable DG. Cerebellar liponeurocytoma. Case report prognosis, but this is yet to be confirmed and review of the literature. J Neurosurg in a larger series of cases. 2001;95:700-3. REFERENCES 12. Jenkinson MD, Bosma JJD, Du Plesis D, Oh- 1. Bechtel JT, Patton JM, Takei Y. Mixed mesen- gaki H, Kleihues P, Warnke P, et al. Cerebellar chymal and neuroectodermal tumor of the liponeurocytoma with unusually aggressive cerebellum. Acta Neuropathol 1978;41:261-3. clinical course: Case report. Neurosurgery 2. Ellison DW, Zygmunt SC, Weller RO. 2003;53:1425-8. Neurocytoma/lipoma (neurolipocytoma) of the 13. Alkadhi H, Keller M, Brander S, Yonekawa cerebellum. Neuropathol Appl Neurobiol Y, Kollias S. Neuroimaging of cerebellar 1993;19:95-8. liponeurocytoma. J Neurosurg 2001;95:324-31 3. Budka H ,Chimelli L. Lipomatous medulloblas- toma in adults: a new tumor type with possible fa-