Arvind Rishi

Myxopapillary ependymoma of lumbosacral region with metastasis to both cerebellopontine angles: report of a rare case

IntroductionMyxopapillary ependymomas are low grade tumours that are known to recur locally even after complete excision, but metastasis to distant sites is extremely uncommon.Case reportWe report an unusual case of lumbo-sacral myxopapillary ependymoma in a 13-year-old boy with metastasis to both cerebellopontine angles. To the best of our knowledge, this is the youngest patient of metastatic myxopapillary ependymoma.

Chordoid glioma: report of two rare examples with unusual features

SummaryBackground. Chordoid glioma, a rare tumour of the third ventricle, represents a distinct clinico-pathologic entity. Thirty nine examples have been described in the literature, mostly in females and in the third ventricle. The clinical presentation is variable but they tend to occur mostly in adults. There is only one report of a chordoid glioma in a 12 year old child.Finding. This paper describes two examples of chordoid glioma in a seven year old female child and a 70 year old male respectively. Radiologically, the paediatric chordoid glioma was located in the juxtaventricular region in the occipital horn of the lateral ventricle and was of mixed density whereas the adult patient had a typical third ventricle location with homogenous contrast enhancement. Gross total surgical removal was achieved in both but the adult patient died post-operatively due to intra ventricular bleeding and bacterial meningitis. The younger patient is doing well at the last follow up two years post-operatively. Microscopically, both showed the classic morphology of chordoid glioma. Ultrastructural examination was suggestive of ependymal differentiation.Conclusion. The younger age and unusual location are some of the rare features which need documentation and have not been described earlier. We propose that chordoid glioma is a variant of an ependymoma (WHO grade II) which arises from tanycytes and should be included in the WHO classification of brain tumors.

Primary intracranial squamous cell carcinoma arising in an epidermoid cyst—A case report and review of literature

Primary intracranial squamous cell carcinoma is extremely rare, with most of the cases arising from malignant transformation of an epidermoid or a dermoid cyst. We report here a case of a 45-year-old male patient who presented with 1-month history of intermittent headache and recent onset of altered sensorium. Imaging revealed a midline posterior fossa mass lesion compressing the fourth ventricle and causing hydrocephalus. A provisional diagnosis of dermoid cyst was considered. Histopathological examination revealed a squamous cell carcinoma possibly arising from an underlying epidermoid cyst. This entity is being reported for its rarity.

A clinicopathological and immunohistochemical study of clinically non-functioning pituitary adenomas: A single institutional experience

BACKGROUND Non-functioning pituitary adenomas (NFPA) are characterized by the lack of clinical syndrome as compared to functioning adenomas (FA) but not all functioning adenomas have clinical effects. Their exact incidence varies in different series. MATERIALS AND METHODS This study was undertaken to analyze the hormonal profile of NFPA at the immunohistochemical level in the Indian population and to see if any differences exist from the earlier studies. Their biological aggressiveness was also studied by MIB-1 labeling index (MIB-! LI) and Epidermal Growth Factor Receptor (EGFR) expression. These parameters along with their clinical behavior were correlated with radiological features of invasiveness and size. RESULTS Of the 151 pituitary adenomas diagnosed during a period of one and half years, 77 (51%) were NFPA with a male predominance. There was increase in the incidence of NFPA with increase in age. Immunopositivity for various hormones was observed in 64 (83%) cases, either singly or in various combinations. On the basis of immunohistochemistry, NFPA were classified into three subtypes; gonadotroph adenomas, silent adenomas, and null cell adenomas. Gonadotroph adenomas were the commonest subtype. In general, NFPA showed low MIB-1LI but invasive NFPA had LI on the higher side, however, this difference was not significant. We observed EGFR positivity in two cases only; therefore the tumorigenesis mechanism may be different in NFPA. CONCLUSION Although non-functional at the clinical level immunohistochemistry showed reactivity for various hormones. If a battery of immunostains including seven hormones is studied, a significant number of cases are shifted to the functional group.

Glioneuronal tumor with neuropil-like islands : A new entity

Glioneuronal tumor with neuropil‐like islands (GTNI) is a distinctive and rare tumor characterized by both glial and neuronal differentiation. However, unlike other mixed glioneuronal tumors, which are characterized by a favorable prognosis, this neoplasm has been found to be potentially aggressive. We report here a case arising in a 60‐year old male patient who presented with seizures, forgetfulness and right‐sided hemiparesis, due to a left frontal lobe tumor. Unlike most cases described in the literature, the present tumor was unique in its radiological appearance, which was cystic. On microscopic evaluation, the glial component was chiefly gemistocytic punctuated by neuropil‐like islands. Strong nuclear immunolabeling of p53 and absence of 1p/19q deletion by fluorescence in situ hybridization assay were consistent with those in previous reports.

Spinal teratoma with renal differentiation--a rare phenomenon: report of two patients.

The occurrence of intradural spinal teratomas in association with spinal dysraphism is uncommon and even rarer is renal differentiation in a teratoma. We report two rare cases of patients with spinal dysraphism, split cord malformation and intraspinal teratomas with renal differentiation in a boy of 1 year and a girl of 3 years of age. Surgical excision of the mass, detethering of the cord and repair of the lipomyelomeningocele was done. Pathologic examination in both the cases revealed features of a mature teratoma with nephrogenic differentiation in the form of elements of mature renal tissue. To the best of our knowledge, spinal teratoma with renal component has not been described previously.

Intracranial inflammatory pseudotumor: Report of a rare case

Inflammatory pseudotumors (IPs) arising in the CNS (IP‐CNS) are quite rare. We report here a case of IP in a 5‐year‐old boy. Intraoperatively there was a well‐defined extra‐axial lesion in the right frontobasal region with CSF all around. There was no dural attachment. The tumor was removed piece‐meal. On pathological examination, the lesion was well circumscribed and comprised of spindled cells in a collagenous background with sprinkling of inflammatory cells. The case was worked up considering the possibilities of low grade glioma, lymphoplasmacyte‐rich meningioma, fungal/tubercular infection or inflammatory pseudotumors. Appropriate immunohistochemical stains were performed to rule out the rare possibility of lymphoma or plasmacytoma. The spindled cells were diffusely immunopositive for vimentin and only occasional cells were positive for smooth muscle actin (SMA). The cells did not show anaplastic lymphoma kinase‐1 immunopositivity. Based on the above morphological and immunohistochemical analysis, a diagnosis of inflammatory pseudotumor was rendered.

Symptomatic bilateral cerebellar mass lesions: An unusual presentation of intracranial xanthogranuloma

Xanthogranulomas are a group of benign lesions with an incidence of 1.6-7.0% in various autopsy series. Although the lateral ventricle is the most frequent intracranial site involved, most symptomatic cases occur in the choroid plexus, involving the third ventricle. Multicentric bilateral symptomatic cerebellar xanthogranuloma has not been reported previously. We describe a 35-year-old man who presented with a complaint of vertigo, with nystagmus and left-side cerebellar signs found on clinical examination. Radiological evaluation revealed bilateral cerebellar lesions abutting the cerebellopontine angle cistern. The left-side lesion was excised and diagnosed as xanthogranuloma up on histopathological examination. The right-side lesion was subsequently excised 4 years later when it became symptomatic. The role of histopathology cannot be overemphasized in the diagnosis of this rare lesion and long-term follow-up is advised in cases managed conservatively owing to the propensity of this tumor to grow over time.

Fatal subacute necrotising brainstem encephalitis in a young man due to a rare parasitic (Balamuthia) infection

We describe a case of brainstem inflammation in a young man which at first defied diagnosis. However, after his death, and notwithstanding our inability to find a cause at autopsy, we did not give up. After sending paraffin blocks to the Centers for Disease Control in Atlanta, Georgia, USA, they suggested the diagnosis of Balamuthia (amoebic) infection.

SPINAL TERATOMA WITH PULMONARY DIFFERENTIATION: A REPORT OF RARE CASE AND REVIEW OF LITERATURE

The occurrence of intradural spinal teratomas in association with spinal dysraphism is uncommon and even rarer is pulmonary differentiation in a teratoma. We report a case of spinal dysraphism, duplication of lumbosacral spine, split cord malformation (SCM I), and meningomyelocele in an 8-month-old child. Excision of meningomyelocele and detethering of cord was done. Pathologic examination revealed a mature teratoma with pulmonary differentiation. To the best of our knowledge this is the second case of spinal teratoma with pulmonary differentiation.