Congenital occipital encephalocele with Dabska tumor: report of an unusual case.

Encephaloceles arise from developmental defects in neural tube formation. These lesions contain brain and meninges which herniate through a defect in the skull. These may present as isolated malformations or rarely be associated with brain tumors. We hereby discuss a case of an unusual association of an occipital encephalocele with papillary intralymphatic angioendothelioma or Dabska tumor arising from the sac itself. The patient underwent resection of the herniated brain tissue with repair and closure of the dural defect. Histopathological examination revealed evidence of Dabska tumor from the sac. This is the first case report of the association of an occipital encephalocele with a rare vascular tumor, i.e. papillary intralymphatic angioendothelioma.

Pediatric epidural tumors of the spine--experience of a decade from the Kashmir Valley.

We present our experience after analyzing retrospectively the clinicopathological characteristics, surgical approaches, immediate and long-term postoperative complications and survival outcome of pediatric spinal epidural tumors treated over a period of 10 years from 2000 to 2009 in a tertiary-care hospital. Our study included benign and malignant tumors. The majority of our patients were boys (14:8). The duration of symptoms was longer in benign lesions. Non-Hodgkin's lymphoma was the commonest malignant tumor and aneurysmal bone cyst was the commonest benign tumor. The mean duration of follow-up was 19 months (range 3-48 months). Most of the patients improved in their neurological grade after surgery; however, the degree of excision had no bearing on the length of survival for malignant lesions.

Frontal intraparenchymal schwannoma.

Intraparenchymal schwannomas are rare. The usual presentation is of a classical intracranial mass lesion. We report a frontal intraparenchymal schwannoma in a pediatric patient with a history of seizure. An MRI scan revealed a uniformly enhancing tumor with perilesional edema. This type of tumor is rarely found in this location. It is important to recognize and distinguish a schwannoma from other more common frontal brain tumors as the outcome is good and recurrence is rare.

Lymphocytic hypophysitis with normal pituitary function mimicking a pituitary adenoma: a case report and review of literature.

Lymphocytic hypophysitis (LYH) is a rare autoimmune inflammatory disorder of the pituitary gland usually affecting young women, often in pregnancy or post partum period. It is rare in non-pregnant females and in men. Patients present with symptoms of an expanding pituitary mass and/or varying degrees of pituitary dysfunction. We, hereby report a case of lymphocytic hypophysitis in a 28-year-old non-pregnant female who presented with clinical and radiological features of a pituitary tumor with normal pituitary hormones which on histopathological examination revealed features of lymphocytic hypophysitis. The case is presented here for its rarity and its unique presentation as an expanding pituitary mass with normal pituitary function. Peculiar clinical, radiological and histopathological features of this uncommon entity are discussed.

Primary spinal paragangliomas: a clinicopathological and immunohistochemical study of six cases.

Spinal paragangliomas are uncommon neoplasms and subject of much debate regarding the factors governing their biological behaviour. We describe the clinicopathological and immunohistochemical (IHC) features of six cases of spinal paraganglioma. The mean age of patients was 40 years (range 20-60 years) with a male to female ratio of (2:1). Majority presented with low backache, sphincter disturbances and sensory symptoms. All tumors were intradural in the cauda region one of them extending to the filum. Only one showed focal extradural extension on microscopy. Gross total resection of tumors was possible in all cases. Histologically four showed classical 'zell-ballen' pattern and two revealed an ependymal morphology. On immunohistochemistry, in all the six cases the chief cells were intensely labeled by antibody to chromogranin but not for GFAP while staining for synaptophysin was less intense and variable in five. Sustentacular cells in all cases showed strong expression for S-100 protein and chief cells were stained light in three cases. Low MIB-1 labeling index of 0.01-2% was noted in five cases and in the sixth it was 5%. None of the tumours recurred. Immunohistochemistry assisted in differentiating these relatively benign neural crest tumours from the more aggressive spinal ependymomas.

Cestode parasitic infestation: intracranial and spinal hydatid disease--a clinicopathological study of 29 cases from South India.

In developing countries hydatidosis is both a medical and economic problem related to environmental hygiene and healthy veterinary practice. This cestode parasitic infestation, uncommonly involving the nervous system, presents with varied clinical manifestation, at times causing diagnostic dilemmas. Multiple intracranial and spinal hydatidosis is rare. A series of 29 histologically confirmed cases of hydatidosis of neuraxis (21 intracranial and 8 spinal) from South India are presented. Among the 21 cases of intracranial hydatidosis, 12 cases were in pediatric age, while only 1 spinal lesion was noted in a 5-year-old child. The clinical presentation of intracranial lesions was predominantly that of raised intracranial pressure and visual symptoms, while spinal hydatidosis manifested with severe back pain, weakness and sphincter disturbances. The cranial cysts were usually single and uniloculated (12 cases), multiple in 7 and single but multiloculated in 2. In spinal hydatidosis, the cysts are usually multiple and extradural, rare ones being intramedullary and intradural. Based on clinical features and imaging, the differential diagnosis for intracranial lesions were cystic tumors and arachnoid cyst while metastasis and tuberculosis were considered in cases of spinal hydatidosis because of vertebral bony involvement. The majority of the cysts could be surgically resected totally and some were aspirated under control suction and resected. None of the cases had anaphylactic reaction, with no significant post-operative morbidity and no mortality. One intracranial and 2 spinal lesions caused by fertile cysts recurred to undergo repeated surgery.