Kuntal Kanti Das

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.

Cerebral gliosarcoma: Analysis of 16 patients and review of literature.

Background: Gliosarcoma (GS), a subtype of glioblastoma (GBM), is a rare primary neoplasm of the central nervous system. Certain features like temporal lobe affinity, tendency for extraneural metastasis and poorer outcome compared to GBM indicate that GS may indeed be a separate clinicopathologic entity. This led us to revisit this entity in our settings. Materials and Methods: Between 2009 and 2014, 16 cases of histologically proven GSs (14 primary, two secondary) were treated. Patient data were retrieved retrospectively. Statistical analysis was performed with? Statistical Package for Social Sciences, version 17.0. (Chicago, Illinois, USA). Survival was analyzed by Kaplan–Meier method. Results: GS predominantly affected males in their fifth decade of life. Raised intracranial pressure was the most common mode of clinical presentation. Temporal lobe was the most commonly affected part of the brain and majority of primary and all of secondary GBM were located peripherally. In 7 (43.8%) patients, tumor was radiologically well-demarcated and enhanced strongly and homogenously on contrast as compared to 9 (56.2%) patients where the tumor was ill-defined and showed heterogenous patchy or ring enhancement. Extent of excision was total in seven patients (43.8%), near total in 4 (25%) and subtotal in five patients (31.2%). Median survival was 6 months. Patients with well-demarcated, enhancing mass on imaging intraoperatively had firm tumors with a good plane of cleavage and had a better survival (8 months) compared to those in whom the tumor radiologically and intraoperatively mimicked GBM (2 months). Conclusion: GS is associated with poor survival (median survival 6 months). Radiological and intraoperative findings help categorize these tumors into GBM like GS and meningioma like GS. While the former histologically mimics GBM and has very poor survival (2 months), GS with meningioma like feature tends to have better survival (8 months).

Angio negative spontaneous subarachnoid hemorrhage: Is repeat angiogram required in all cases?

Background: In some cases of spontaneous subarachnoid hemorrhage (SAH), the cause of bleed remains obscure on initial evaluation. These patients may harbor structural lesions. We aim to determine the utility of repeat angiogram in these subsets of patients. Methods: In this prospective study, patients with SAH with a negative computed tomographic angiogram (CTA) and digital subtraction angiogram (DSA) were included. A repeat angiogram was done after 6 weeks of initial angiogram. Patients were divided into perimesencephalic SAH (PM-SAH) and diffuse classic SAH (Classic-SAH) groups. Outcome was determined by modified Rankin score (mRS). Results: A total of 22% (39/178) of all SAH were angio-negative. A total of 90% (n = 35) of these were in Hunt and Hess grade 1-3. A total of 22 patients had PM-SAH and 17 had a Classic-SAH. Repeat angiogram did not reveal any pathology in the PM-SAH group, whereas two patients with Classic-SAH were found to have aneurysms. At 6 months follow-up, 95% patients of PM-SAH and 83.3% of Classic-SAH had mRS of 0. Conclusion: Repeat angiogram is probably not necessary in patients of PM-SAH and they tend to have better outcome. Classic-SAH pattern of bleed is associated with fair chances of an underlying pathology and a repeat angiogram is recommended and these cases and they have poorer outcome.

Extradural spinal meningioma: Revisiting a rare entity

Spinal meningiomas are mostly intradural in location although at times these are associated with some extradural extensions. Purely extradural spinal meningiomas (EDSMs) are however, extremely rare and when present, may cause diagnostic dilemma preoperatively. Only seven cases of pure EDSM have been reported till date. In this paper, we describe two cases of EDSM affecting the cervical spine and present their clinical profiles, radiological findings, operative management, and follow-up data, along with a review of the literature.

Pediatric intramedullary spinal cord lesions: Pathological spectrum and outcome of surgery

Background: Pediatric intramedullary spinal cord lesions are not only rare but also different from adults in a number of aspects. We aimed to study the incidence and the frequencies of various pediatric intramedullary mass lesions, their outcome to treatment and the factors determining their outcome of treatment. Materials and Methods: Thirty-one consecutive children (aged 1-18 years, mean 11.1 years, male: female = 1.8:1) with pathologically proven intramedullary spinal cord lesions treated at our center were studied. Clinico-radiological, histopathological, operative, and outcome data were reviewed retrospectively. The functional status was assessed using the modified McCormick grading system. Results: Gross total tumor excision was performed in 19 patients (61.3%), subtotal in 9 patients (29%), partial excision was performed in 2 (6.5%) patient, and only biopsy was performed in 1 patient (6.5%). There was one peroperative death, 2 patients died at follow-up. Complications included wound related complications (n = 4), transient deterioration in the motor power, and respiratory complication requiring a tracheostomy. Six patients showed recurrence at a mean follow-up of 16.4 months. Developmental tumors, high-grade ependymomas, and incompletely excised grade 2 ependymomas showed a tendency to recur. Conclusions: Children constituted nearly 1/5 th (17.4%) of intramedullary spinal cord tumors. Astrocytomas and ependymomas taken together constituted the most common intramedullary spinal lesions in children; however, developmental tumors predominated in the first decade. Children usually presented in good functional grades preoperatively and maintained good grades after surgery. Functional outcome was dependent on the preoperative neurological status and histopathology of the lesions.

Extramedullary foramen magnum tumors and their surgical management: An experience with 29 cases.

Introduction: Surgical management of foramen magnum (FM) tumors is challenging by virtue of their location and vital neurovascular relationships. The ideal approach to anterior/anterolateral tumors continue to evoke controversy even in the modern era. In this article, we present and discuss our experience in the surgical management of these tumors. Materials and Methods: This retrospective study includes 29 consecutive patients (mean age 36.6 years, M: F = 2.63:1) of extramedullary tumors at the surgical foramen magnum, operated at our center, between 2007 and 2012. Results: Their mean duration of symptoms was 14. 6 months. A majority of the patients presented with motor symptoms (quadri/paraparesis, n = 21, 72.4%), neck pain with/without suboccipital radiation (n = 16, 55.2%) and sensory symptoms like tingling/numbness (n = 16, 55.2%). There were nine extradural (31%) and 20 intradural tumors (69%). Most of the tumors were located posterolateral to the neuraxis (n = 13, 44.8%). Nerve sheath tumors (n = 11, 38%) and meningiomas (n = 5, 17.2%) were the most commonly encountered histologies in our series. The standard posterior approach was the most frequently employed surgical approach (n = 20, 69%). Operative mortality and morbidity were 3.4 and 18.9%, respectively. At a mean follow-up of 27.3 months, 13 out of the 18 available patients improved. Conclusion: A majority of the foramen magnum tumors are amenable to excision via the standard posterior approach. Small anterior dural-based meningiomas/recurrent tumors may require a lateral approach like the far lateral approach.

Aggressive osteoblastoma involving the craniovertebral junction: A case report and review of literature

Osteoblastoma (OB) is a rare bony neoplasm constituting around 1% of all primary bone tumors. Although the vertebrae and long bones are the most common sites affected by OB, skull remains a relatively uncommon site of occurrence. Aggressive variant of OB is histologically intermediate between an indolent conventional OB and a malignant osteosarcoma. To the best of our knowledge, aggressive osteoblastoma (AO) affecting the craniovertebral junction has not been previously described in the literature. In this report, we present a 34-year-old gentleman who presented with a mass involving the left side of the neck and oral cavity along with ipsilateral lower cranial nerve paresis. Computed tomography and magnetic resonance imaging scans of the craniovertebral junction revealed a heterogeneously enhancing expansile lesion with areas of destruction involving the clivus, left sided jugular foramen and left side of first two cervical vertebras. Angiography showed distortion of the V3 segment of the left vertebral artery and shift of the ipsilateral internal carotid artery. The tumor was maximally excised through far lateral approach. Histopathologic examination revealed a diagnosis of AO. The patient was referred for radiotherapy for the residual tumor and was doing well at 5 months follow-up.

Clear cell meningioma in a child: A case report and review of literature

An 11-year-old boy presented with holocranial headache associated with vomiting and heaviness in right paranasal region. On examination, he had left sided upper motor neuron type facial palsy along with a subtle ipsilateral hemiparesis (grade 4/5 Medical Research Council). Magnetic resonance imaging brain revealed a contrast enhancing large well-defined extra-axial mass lesion in right middle cranial fossa with extension into the infratemporal fossa. The mass was excised in two stages. Histopathology was suggestive of clear cell meningioma (CCM). Supratentorial pediatric CCM is a rare entity. The case is discussed along with review of literature on this rare clinico-pathological entity.

Concurrent cerebellar and cervical intramedullary tuberculoma: Paradoxical response on antitubercular chemotherapy and need for surgery.

Spinal intramedullary tuberculoma (SIT) is a rare manifestation of neurotuberculosis. Concurrent SIT and intracranial tuberculoma are further unusual. Most of these tuberculomas respond completely to medical therapy, and surgical excision is seldom required. In this report, we describe a 17-year-old boy who developed cervical intramedullary tuberculoma at C3-C6 level with a concurrent lesion involving the right cerebellar hemisphere while on treatment for tubercular meningitis. This patient had paradoxical increase in size of the cervical lesion even though the cerebellar lesion showed regression in size. In this article, we discuss the paradoxical response to anti-tubercular therapy in central nervous system tuberculosis, possible causes of nonresolution of tuberculoma on medical therapy and evaluate the role of surgery in these cases.

Atlanto-axial dislocation associated with anomalous single vertebral artery and agenesis of unilateral internal carotid artery

We report a case of an anomalous single midline vertebral artery (VA) in a case of atlanto-axial dislocation (AAD). The left VA coursed in the midline at craniovertebral junction as there was no right VA. The left internal carotid artery was also not formed. This was a case report of a 34-year-old male patient who presented with features of high cervical myelopathy. On evaluation, he had fixed AAD. There was inadvertent intra-operative VA injury, which was sealed to control the brisk bleeding to require stenting ultimately. Patient did not survive and expired after 5 days due to brain edema secondary to compromised anomalous intracranial circulation.