Sushila Jaiswal

Primary melanocytic tumors of the central nervous system: A neuroradiological and clinicopathological study of five cases and brief review of literature

Primary melanocytic tumors of the central nervous system (CNS) are uncommon lesions. These lesions arise from the melanocytes located within leptomeninges and include diffuse melanocytosis and meningeal melanomatosis (seen in neurocutaneous melanosis), melanocytoma, and malignant melanoma. To study, the clinical course, neuroradiological features, morphology and immunohistochemistry of primary melanocytic tumor of CNS. Demographic, clinical and surgico-pathologic findings of five patients with melanocytic tumors seen between 1996 and 2003 were studied. In this study, five cases of primary melanocytic tumors have been reported: four cases of malignant melanoma and one case of melanocytoma. Three of the 5 cases were intracranial and 2 were spinal. The mean age in the present study was 26 years. Presenting features varied according to the location. Primary melanocytic tumor of CNS are rare. Whenever possible, complete surgical excision is the best treatment.

Intramedullary tuberculoma of the conus.

We present a conus medullaris tuberculoma in a 12-year-old girl. She presented with low backache, weakness of both lower limbs and urinary disturbance. Magnetic resonance imaging revealed a D10-L1 intramedullary mass. The tumour was excised and the biopsy was suggestive of tuberculoma. The patient received antituberculous therapy postoperatively and improved. The relevant literature is discussed briefly.

A clinicopathological, immunohistochemical and neuroradiological study of eight patients with central neurocytoma

Central neurocytomas are low-grade tumors of neuronal origin located in the lateral ventricle that present predominantly with raised intracranial pressure. In this retrospective study, we investigated the clinical, radiological, histopathological and immunohistochemical features of eight patients (seven males and one female; age range 16-61 years; mean=35.1 years) with neurocytoma. Raised intracranial pressure was the most common presenting feature. In addition, one patient presented with marked visual deterioration and one presented with a visual field defect. All lesions were located in the lateral ventricle (right lateral ventricle: four patients, left lateral ventricle: three patients, both ventricles: one patients). Radiology showed marked intratumoral calcification in two patients. Total microsurgical excision was achieved in seven patients. Histopathology showed sheets of monotonously small-to-medium-sized neoplastic cells with uniform round-to-oval nuclei and inconspicuous nucleoli. Immunohistochemistry was positive for synaptophysin and neuron-specific enolase (NSE) in all tumors, and glial fibrillary acidic protein was focally positive in two patients. One patient had lipomatous differentiation within the tumor. No recurrence was noted in any of our patients until the last follow-up; however, there were two deaths in our series.

Choroid plexus tumors: A clinico-pathological and neuro-radiological study of 23 cases

Background: Choroid plexus tumors are intraventricular tumors derived from choroid plexus epithelium. Aim: To study the choroid plexus tumors with reference to their clinical, radiological, and pathological features. Materials and Methods: The study was performed by the retrospectively reviewing the clinical, radiological, and pathological records of patients of choroid plexus tumors. Results: A total of 23 cases (11 males, 13 females) of choroid plexus tumor were diagnosed from 1997 to 2009. Fourteen patients were below 15 years of age. Raised intracranial pressure was the main presenting feature in all the cases. Tumor was located in lateral ventricle (n = 14; in 3 cases tumor was going into third ventricle), fourth ventricle (n = 7), and cerebellopontine angle (n = 2). Total tumor excision was achieved in 21 cases. The histopathology was suggestive of choroid plexus papilloma (n = 19), atypical choroid plexus papilloma (n = 1), and choroid plexus carcinoma (n = 2). Clears cell areas were noted in three cases. Other histopathological features observed were foci of calcification (n = 5), Psammoma bodies (n = 2), hemorrhage (n = 5), hyalinization (n = 2), and oncocytic changes (n = 1). Conclusions: Choroid plexus tumors are intraventricular tumors arising from choroid plexus epithelium. The predominant clinical presentation is raised intracranial pressure. Surgery is the mainstay of treatment; histopathologically, they include choroid plexus papilloma, atypical choroid plexus papilloma, and choroid plexus carcinoma.

Intraoperative squash cytology of central nervous system lesions: A single center study of 326 cases

Cytology has been shown to be of great value in intraoperative consultations of central nervous system (CNS) pathology. Intraoperative smear cytology provides a rapid and reliable intraoperative diagnosis and guidance to the neurosurgeon during surgical resection and lesion targeting. It also helps the surgeon to monitor and modify the approach at surgery.

Tumors of the posterior third ventricular region in pediatric patients: The Indian perspective and a review of literature.

Background: Diverse tumors in the posterior third ventricular region (TPTVR) frequently occur in children. A decades experience with pediatric TPTVR is presented, focusing on the Indian perspective. Materials and Methods: 25 children (age range: 3-18 years; mean age: 13.32 years; presentation range: 7 days-2.5 years) had clinico-radiological assessment with contrast computed tomography (CT) and magnetic resonance imaging (MRI). The ventricular/lumbar cerebrospinal fluid (CSF) alpha feto protein (AFP)/beta human chorionic gonadotrophin (HCG) estimation was done when radiological suspicion of a germ cell tumor was present. Extent of resection was deemed partial when some tumor mass remained at the end of surgery, near total when <10% was retained over vital neurovascular structures, and total when complete resection was attained. Results: Operations included infratentorial supracerebellar approach (n = 12), occipito-transtentorial approach (n = 2), endoscopic biopsy and third ventriculostomy (n = 1), frontal parasagittal craniotomy, interhemispheric transcallosal subchoroidal approach (n = 2), middle temporal gyrus transcortical transventricular approach (n = 1), fronto-temporo-zygomatic combined transylvian and subtemporal approach (n = 1) and right ventriculoperitoneal shunt and stereotactic biopsy (n = 1). Only CSF diversion was performed for five patients with a small TPTVR. CSF diversion was required in 12 (48%) patients. Tumor pathology included pinealoblastoma (n = 4; one with pineocytic differentiation), nongerminomatous germ cell tumor (NGGCT; n = 3), germinoma (n = 3), pilocytic astrocytoma (n = 2), epidermoid (n = 3) and primitive neuroectodermal tumor (PNET), fibrillary astrocytoma, glioblastoma, teratoma, and meningioma (n = 1, respectively). A patient with neurocysticercosis was diagnosed solely on MRI (four did not undergo biopsy). Fractionated radiotherapy was administered in 13 patients with primary pineal tumors, PNET, NGGCT, fibrillary astrocytoma and glioblastoma. Extent of excision was total in 10 (40%), near total in 5 (20%), partial in 3 (12%) and a biopsy in 2 (8%) patients. Conclusions: Histopathologic characterization of TPTVR is essential prior to their further management. Benign lesions often have a good prognosis following gross total surgical resection. Pure germinomas are highly susceptible to radiotherapy. NGGCTs often have malignant components that require adjuvant therapy following surgery. The advancements in microsurgical techniques have led to gratifying perioperative results in these deep-seated lesions.

Choroid plexus papilloma in children: Diagnostic and surgical considerations

Background: Choroid plexus papilloma (CPP) is a benign neoplasm that arises from the ventricular choroid plexus. The clinical features, radiological characteristics, and treatment have been discussed in this study for a pediatric population. Methods: Over an eight-year period, seven pediatric (≤12 years) CPP patients were treated. Tumors were located in the lateral ventricle (n = 4), IVth ventricle (n = 2), and in both the lateral and IIIrd ventricles (n = 1). The patients presented predominantly with features of raised intracranial pressure. Total microsurgical excision was carried out in all cases. Results: There was complete relief of symptoms at follow-up in six patients. A 2.5 year-old child with a large trigonal CPP with hydrocephalus leading to complete visual impairment, died due to postoperative hypokalemia that caused ventricular fibrillation. One of our patients required a postoperative, permanent CSF diversion procedure while another required a subduroperitoneal shunt for persisting postoperative subdural CSF collection. Conclusions: Coagulation of the tumor under constant irrigation to shrink and excise it in toto, avoids excessive bleeding during surgery. The vascular pedicle supplying the tumor should be adequately dealt with during the last part of tumor removal as retraction of a bleeding pedicle may result in ventricular hemorrhage and brain edema. Following surgery, an external ventricular drain for three days helps in preventing the development of acute hydrocephalus in lateral ventricular lesions, and the color of the drained CSF gives an estimate of the ventricular hemostasis achieved. Total excision is usually possible in these cases with excellent postoperative outcomes.

Alveolar soft part sarcoma of the frontal calvarium and adjacent frontal lobe

Alveolar soft part sarcoma is a rare tumor affecting mainly adolescent and young children. It presents as a slowly growing tumor and is usually overlooked due to lack of symptoms. Early metastasis is a characteristic feature of this tumor and, in a good number of cases, metastasis to the lung or brain is the first manifestation of the disease. In this report, we present a case of alveolar soft part sarcoma predominantly located in the right frontal bone with dural breach and contiguous right frontal lobe involvement in a 17-year-old girl without any evident primary or other secondaries. A brief review of literature is also presented.

Meningioma with hemorrhagic onset: Two case reports

Haemorrhage is a rare complication of meningiomas that can occur spontaneously, after embolization, stereotactic radiation and perioperatively. Our first case was a 16 year old male, admitted with spastic quadriparesis, and retention of urine. Magnetic Resonance Imaging (MRI) revealed anteriorly placed cervical intradural extramedullary mass. Patient underwent emergency surgery following sudden worsening of neurological symptoms and intratumoral bleed was noted peroperatively. Tumor was labeled as angiomatous meningioma with hemorrhage. The second case was of a 45 year female who presented with history of sudden onset weakness in right upper and lower limb followed by unconsciousness. MRI revealed heterogeneous lesion in left parasagittal area with intratumoral bleed. Left frontal craniotomy with tumour decompression was performed. Tumour was labelled as meningothelial meningioma with haemorrhage. Meningiomas with hemorrhagic onset remain rare, and pathophysiology is still incompletely understood. Prevention and outcome of intratumoral haemorrhage highly depends on early diagnosis and adequate treatment.

Ewing's sarcoma of the orbit with intracranial extension: A rare cause of unilateral proptosis.

Ewings sarcoma causing unilateral proptosis along with bifrontal extradural infiltration in a child is an unusual presentation. A female patient presented with features of painless proptosis of the left eye with visual deterioration. Her radiology revealed an infiltrating intraorbital, extraconal tumor with intracranial bifrontal extradural extension causing mass effect. Total excision of the intraorbital and intracranial part of the tumor along with postoperative chemo- and radiotherapy brought about a substantial relief. The clinicoradiological presentation and management of this rare entity are discussed.