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Background Mucormycosis of the central nervous system is an uncommon infection caused by saprophytic or parasitic fungi of the subphylum Mucormycotina and order Mucorales viz. Rhizopus, Mucor, and Rhizomucor. Isolated, chronic involvement of the central nervous system is a rare occurrence. To the best of our knowledge, isolated chronic ventricular involvement in an infant has not been reported previously. Isolated intracerebral mucormycosis is a disease of the immunocompromised patient, and to date only 6 cases have been reported in immunocompetent patients, including 2 pediatric cases. Case Description We present the case of an immunocompetent infant presenting with features of increased intracranial tension. He underwent cerebrospinal fluid diversion and was found to harbor mucormycosis on histopathologic examination of intraventricular debris. We also present a brief review of the relevant literature. Conclusions Although mucormycosis is an acute fulminant infection, chronic isolated cerebral cases are known in the immunocompetent patient. Patients also may present with isolated hydrocephalus, and hence fungal infection must be ruled out in all, especially if a shunt is warranted.

Background A majority of patients with glioneuronal tumors present with seizures. Although a number of studies have shown that greater extent of resection improves overall patient survival, few studies have focused on post-operative seizure outcome after resection of these tumors. We aim to characterize seizure control rates in patients undergoing glioneuronal tumor resection and evaluate the association between poor seizure outcome and tumor recurrence or progression. Methods The study population included patients who had undergone resection of glioneuronal tumors between 2014 and 2019 at our institution. Seizure outcome was assessed using Engel grading. Preoperative seizure characteristics, tumor characteristics, surgical factors, and postoperative seizure outcomes were reviewed. Results 26 patients (n=16, temporal lobe; n=6, frontal lobe; n=4, parietal lobe) with mean seizures duration of 56.9-months, were assessed. Histopathologically, n=15 dysembryoplastic neurepithelial tumour, n=7 ganglioglioma and n=4 Diffuse lepto-meningeal neuroepithelial tumour. There were 2 cases of complex DNET and one case of DLMNT had associated cortical dysplasia. At mean follow-up of 49.7 months, n=20 Engel 1, n=4 Engel 2 and n=2 had Engel 3 outcome. n=20 underwent gross total excision (n=18 Engel 1 and n=2 Engel 2) and n=6 sub-total excision. Among the 4 patients who needed resurgery, two were in Engel 2 and another two were in Engel 3. Conclusions Good seizure-outcome is likely associated with extent of resection. Younger age of patient, less than one-year of seizure duration and absence of generalization of seizure are good prognostic indicators. The best seizure-control can be achieved by early surgical intervention.

Background: Mesial temporal lobe epilepsy attributed to low-grade glioma is known for intractable seizures and choice of surgery range from lesionectomy (Lo) to lesionectomy with anteromesial temporal resection (L0 + AMTR) is still debatable. We intend to analyze the seizure outcome after lesionectomy alone or with AMTR. Subjects and Methods: Retrospective analyses of patients operated for medial low-grade temporal lobe tumors with seizures were included in the study. Preoperative records include video-electroencephalographic, magnetic resonance imaging (epilepsy protocol), and neuropsychological evaluation for language, memory, and dominance were assessed. Two groups (Lo [Group I] and Lo + AMTR [Group II]) were assessed after surgery by the international league against epilepsy (ILAE) seizure outcome scale. Results: A total of 39 patients underwent Lo (n = 20) and Lo + AMTR (n = 19) with a mean age of 26.92 ± 12.96 months, and mean duration of seizures was 36.87 46.76 months. A total of 23 patients had long-term intractable seizures for >1 year despite >2 drugs(Group I [n = 10], Group II [n = 13]); remaining 16 had frequent seizures of

Background Tumors presenting with drug-resistant seizures are termed as long-term epilepsy-associated tumors (LEATs). LEATs are more common in the temporal lobe, occur predominantly in pediatric age, and focal neurological deficits are rare. In this article, we aim to highlight our surgical experience in terms of seizure outcome among LEATs and discuss the factors affecting outcome. Methodology We have retrospectively analyzed all the operated cases of intra-axial brain tumors with seizures (2015–2019). The clinical and radiographic data were collected from the hospital record system. For comparison, 2 groups were made (group 1 with good seizure control, i.e., Engel 1; and group 2 poor seizure outcome, i.e., Engel 2 and 3). Results A total of 51 cases were included; the temporal lobe was the most common location (n = 27); 23 patients had seizure frequency of “more than 1 seizure per week.” Focal unaware seizures/complex partial seizures were the most common type of seizures encountered (n = 28). At a mean follow-up of 39.60 months, 38 patients had Engel 1 (78.5%) outcome (35 cases [71.05%] had the seizure duration of ≤2 years). The median duration of symptoms (group 1, 25 months vs. group 2, 65 months) was significantly different (P = 0.002). On comparing patients with seizure duration, we found a statistically significant difference (P Conclusion A shorter duration of symptoms, younger age of the patient, partial/focal seizures, and gross total excision were predictors of a good seizure outcome. Histopathology of the tumor does not affect the outcome when one compares glioneuronal tumors with non–glioneuronal tumors.

Intracranial peripheral primitive neuroectodermal tumors (pPNETs) are rare lesions, accounting for only 1% of all pPNETs, and usually arise from bone and soft tissue. These tumors primarily affect young patients (30 years old) and rarely affect older patients. The common intracranial locations of these lesions are frontal dura and tentorium. These lesions rarely may involve the cerebellopontine angle, cavernous sinus, and jugular foramen. Involvement of clivus has not been reported to date. Intracranial metastasis from primary intracranial pPNET is a further rare occurrence.A 74-year-old woman presented with gradually progressive painless diminution of vision in both eyes alongwith headache for 6 months duration. Radiology demonstrated a clival mass as well as a suspected intracranial metastasis. Histopathology was suggestive of pPNET. We also present a brief review of the literature on pPNETs.pPNETs of the clival region are exceptionally rare. In elderly patients with clival mass lesions, the rare possibility of pPNET should be kept in mind.

Pleomorphic xanthoastrocytomas are rare tumors of glial origin comprising1% of all astrocytic tumors of brain. These tumors are rare in the infratentorial compartment and were not known to arise from the middle cerebellar peduncle.We discuss the case of a 16-year-boy who presented with altered sensorium and features of increased intracranial pressure and was found to have a 4 × 4 × 3 cm mass arising from the middle cerebellar peduncle.We also discuss the histopathologic features of these tumors suggesting their resemblance to other maldevelopmental tumors, the rarity of pure World Health Organization grade II pleomorphic xanthoastrocytoma in the infratentorial compartment, as well as the appropriate management.

Background Cerebellar liponeurocytoma (cLNC) is a rare benign glioneuronal tumor with only ~60 cases reported since its first description in 1978. They have occurred largely as sporadic cases; however, familial cases with possible autosomal dominant inheritance have also been reported. Surgical excision has been considered the main modality of treatment, even for recurrent lesions. Uncertainties exist regarding the natural history, long-term outcomes, and optimal postoperative management and follow-up protocols for cLNC in general and multifocal cLNC in particular. Multifocality is exceedingly rare, with only 4 cases reported previously. Case Description We present a case of multifocal cLNC in a young woman who had presented with progressive cerebellar symptoms of 5 months’ duration. Her diagnostic neuroimaging evaluations revealed 2 asymmetric mass lesions (1 on each cerebellar hemisphere). We excised the larger lesion to relieve the mass effect and decided to monitor the smaller lesion radiologically. However, she developed a new lesion during the follow-up period and required adjuvant radiotherapy. Conclusion Surgical excision forms the cornerstone of management of cLNCs. However, it is necessary to vigilantly monitor these patients owing to the high recurrence rates of cLNC despite the innocuous histologic features.

Posterior fossa hemangioblastomas are WHO grade I benign lesions with a surprisingly high recurrence rate. This study determines the factors responsible for recurrence and the clinico-radiological and histopathological differences between primary (group A; n = 60) and recurrent/symptomatic residual (group B; n = 24) tumors. Radiologically, tumors were differentiated into cystic, cystic with a mural nodule, solid-cystic/microcystic and solid. Surgery was undertaken via a midline or lateral suboccipital approach. Histopathology differentiated them into reticular, cellular or mixed subtypes. Truncal-appendicular ataxia in group A and von Hippel-Lindau (VHL) disease and raised intracranial pressure in group B were the predominant presentations. VHL patients in group B had a longer duration of symptoms (median 72 months) than those with non-VHL recurrences (median: 36 months). Multicentric mural nodules (n = 9/24, 37.50%, P = 0.0001) and bilateral cerebellar hemispheric involvement (n = 6/24, 25%, P = 0.0003) were exclusively seen in the preoperative radiology of group B tumors. Brainstem involvement was seen in the tumors of ten (16.67%) patients in group A and six (25.00%) patients in group B. One subset of patients required several resurgeries for repeated recurrences. Abnormal vascular proliferation and papillary projections into adjacent brain, and highly cellular stroma were unique histological features at recurrence. Total resection was achieved in 45 group A and 19 group B patients. The outcome based on the Karnofsky performance scale (KPS) was assessed at follow-up at 6 weeks (A: n = 60, B: n = 24): KPS0: A = 4, B = 1; KPS10–40 (dependent): A: 4, B = 5; KPS50–70 (independent for daily needs): A = 36, B = 16; KPS80–100 (fully independent): A = 16; B = 2. In recurrent/residual tumors, the radiological as well as histopathological features showed a distinctive change toward a more aggressive nature. Higher incidences of multiple mural nodules, bilateral spread, remote recurrence and VHL disease at preoperative radiology; as well as proliferative angioarchitecture, an irregular brain tumor interface and highly cellular stroma at histology were found in these patients compared to their primary counterparts.

Background The simultaneous presence of Lhermitte-Duclos disease (LDD) with focal areas of nodular dysembryoplastic neuroepithelial tumor (DNET) differentiation in the cerebellar hemisphere is reported in a patient who showed the characteristic magnetic resonance imaging (MRI) appearance of “tiger striping.” Case Report A 25-year-old man presented with a 7-month history of holocranial headache, progressive vision diminution, and right-sided cerebellar signs. Computed tomography (CT) scan revealed a hypodense, nonenhancing right cerebellar lesion effacing the fourth ventricle. There were thin hyperdense, linear striations across the tumor with hypodense streaks between them. The third and lateral ventricles were dilated with periventricular lucency. MRI showed the hypointense-to-isointense right cerebellar lesion with linear striations on T1-weighted images. The lesion was hyperintense on T2-weighted images with areas of linear hypointensity streaks running throughout the lesion characteristic of the “tiger striping” effect of LDD. Sagittal MRI revealed tonsillar herniation. Gross total removal of the tumor was accomplished with right paramedian suboccipital craniectomy. The postoperative course was unremarkable, and the patient remained symptom-free at 3-month follow-up examination. Histology revealed the simultaneous presence of LDD with DNET. Conclusions To the best of the authors' knowledge, the simultaneous presence of cerebellar LDD with DNET has never been reported before. This case report may point to their common genesis with cortical dysplasia and neuronal migrational abnormalities playing an important role. LDD and DNET may represent different points in the spectrum of the lesion—from being purely hamartomatous to having a greater proliferative potential. The simultaneous presence of LDD and DNET was perhaps responsible for the unusually rapid neurologic deterioration in this patient before surgical intervention.

There are significant differences in the prevalence and behavior of differentiated thyroid cancers (DTC) in the iodine-deficient areas (IDA) and iodine-sufficient areas (ISA) of the world. The sodium iodide symporter (NIS), mediates active transport of iodide across the basolateral aspect of the thyroid follicular cell. However, no study had specifically addressed the issue of expression of sodium iodide symporter (NIS) in thyroid cancer specimens from IDA. The aim of the present study was to find an expression pattern of NIS in DTC in an iodine-deficient population, and to correlate it with histological subtypes, i.e., papillary carcinoma (PTC), follicular carcinoma (FTC), poorly differentiated carcinoma (PDTC), as well as with clinicopathological risk factors and iodine ((131)I) uptake by distant metastases.Immunohistochemistry was carried out in 39 cases of thyroid cancer (41 samples) including PTC (15), FTC (10), PDTC (9), anaplastic cancer (5), and resected metastases (2). Expression was correlated with the patient's age, sex, tumor size, presence or absence of extrathyroidal invasion, distant and lymph node metastases, and whole body radioiodine scan.Overall, 61.8% of DTC patients showed NIS expression. There was no significant difference in expression rate between PTC (73.3%) and FTC (70.0%). However, expression was significantly less in PDTC (33.3%). There was no correlation between NIS expression and any clinicopathological risk factor (p.05). The results of NIS expression were not concordant with (131)I uptake by metastases in 4 of 10 cases. (131)I uptake was absent in one case despite the finding that a metastatic site itself showed NIS expression in that case, whereas in the remaining 9 cases (131)I uptake was present although three cases did not show NIS expression.In our experience, overall expression of NIS was comparable to other studies from ISA. We conclude that expression may not accurately predict radioactive iodine (RAI) uptake by metastases.

Primary spinal primitive neuroectodermal tumor (PSPNET) is extremely rare and only 25 cases have been reported in the world literature so far. Three patients of 8, 9 and 18 years of age, who presented with variable grades of neurological deficit were diagnosed as having a dorsal intramedullary lesion, a holocord lesion and cervical extradural tumor with extraspinal extension, respectively, and were operated at our institute. The histopathology of all 3 children revealed PNET. The clinical course, image characteristics and outcome of the 3 children are described, and the relevant literature is reviewed. The following conclusions were drawn from the present study and review of the literature. PNET may manifest itself as a primary lesion of the spine unlike the more common drop metastases from an intracranial lesion. PSPNET may be intramedullary, intradural and extradural with variable extraspinal extension. PSPNET may present as holocord intramedullary lesion, an entity which has not been described earlier. These lesions have a short history, significant neurological deficits and rapid course of illness. PSPNET, though an established entity, did not find a place in the WHO 2000 classification of CNS tumors. Hence its status has to be defined.

Endometriosis involving the neural axis is extremely rare. A twenty-five-year-old woman presented with acute onset paraparesis with bladder involvement of five-day duration. We missed her history of cyclical back-ache related to menses at the emergency room. Magnetic resonance imaging (MRI) of spinal cord showed an intramedullary tumour with bleeding at conus–epiconus level. She was operated in the emergency department with laminectomy–durotomy and tumour decompression. Histopathology of the tumour was suggested endometriosis.

A 25-year-old male presented with off-and-on vertigo of 10-year duration. He had left-sided cerebellar signs, left vocal cord paresis, and minimal left-sided hearing impairment. Computed tomography of the head revealed significant hyperostosis of squamous occipital, mastoid, and petrous temporal bone with no adjacent soft tissue mass. On excision, it turned out to be paraganglioma. Paraganglioma as a pure bony mass is not reported in the literature. The site of origin of such a tumor could not be ascertained, even on generous screening. The tumor remained nonsecretory on clinical and biochemical investigations.

In vestibular schwannomas (VS), the tumour size, as well as the size of the cystic component, have a considerable bearing on the outcome. This study addresses the differences between the cystic and solid variants of giant vestibular schwannomas. The study included 62 patients with giant VSs, of which 40 were solid and 22 were cystic (those in which cystic component greater or equal to 30% of the total tumour volume). The cystic tumour group was further divided into type A (31-60% volume of the cyst within tumour), type B (61-90% intra-tumoural cyst volume) and type C (more than 90% volume of the cyst). The clinicoradiological features, operative findings, histopathological characteristics and outcome of surgery of the two groups were compared. The mean duration of symptoms for the solid and cystic tumours were 21.1 and 26.2 months, respectively. However, six patients with cystic tumours showed recent and rapid neurological deterioration after a protracted existence. Papilloedema, lower cranial nerve involvement, facial paraesthesias and preoperative hydrocephalus were significantly more in cystic tumours. Total excision was achieved in 38 of the solid and 18 of the cystic tumours. VIIth nerve preservation was higher in the cystic lesions [solid 33/40 (82.5%), cystic 21/22 (95.4%)]. Myxoid degeneration, lobular growth patterns and cellular atypia were more prominent in the cystic variants. The giant vestibular schwannomas were associated with a higher incidence of cystic degeneration than has been reported for smaller tumours in literature. In cystic lesions, VIIth nerve preservation was higher due to early decompression of the lesion that facilitated in early identification of the VIIth nerve, except in patients with type C cystic tumour.

This paper highlights the etiology, diagnosis, management and outcome in nine unusual cases of extrahepatic biliary obstruction in children. Extrahepatic biliary atresia and choledochal cyst constituted 127 out of 136 (93%) cases of all pediatric surgical biliary disorders managed between March 2000 and February 2007 at the reporting centre. However, nine children (aged 1.5-15 years) presented with uncommon causes like (1) idiopathic benign non-traumatic inflammatory stricture (n = 3), (2) idiopathic fibrosing chronic pancreatitis (n = 2), (3) post-cholecystectomy type 4 benign biliary stricture (n = 1), (4) post-acute pancreatitis pseudo-cyst of pancreas (n = 1), (5) non-Hodgkin's lymphoma (NHL) with extramural common bile duct compression and gall bladder perforation (n = 1), and (6) Langerhan cell histiocytosis (LCH, n = 1). The clinical features and the diagnostic work up of each group are discussed. A preoperative endoscopic/percutaneous biliary drainage was required in four children because of cholangitis at presentation. A biliary-enteric anastomosis was performed for all seven children in groups (1)-(4). The patients with NHL and LCH were referred for chemotherapy after establishing tissue diagnosis at laparotomy. With a follow-up period of 3 months to 7 years, seven children (with the exception of patients with NHL and LCH) are currently anicteric. This paper draws attention to some infrequently discussed causes of extrahepatic biliary obstruction in children. The management entails a carefully planned combination of endoscopic interventions, interventional radiology and surgery. The outcome in benign cases is usually satisfactory.

Neuropathy in rheumatoid arthritis (RA) may result secondary to entrapment, vasculitis, and drug toxicity. We aimed to study clinical and electrophysiological neuropathy and pathological changes in sural nerve in patients with RA. One hundred eight patients of RA, fulfilling American College of Rheumatology 1987 criteria (mean age, 45.83 years; M/F 1:3, 80.3% seropositive) were examined clinically and electrophysiologically for evidence of peripheral neuropathy. Sural nerve biopsies were performed in the involved cases. In all RA patient medications, disease activity, results of blood tests, and X-rays of affected joints were recorded. Twenty-three patients complained of paresthesias in the extremities. Vibration sensations were decreased in 9, and tendon reflexes were decreased or absent in 28 patients. Sixty-two (57.4%) patients had electrophysiologic evidence of neuropathy. Of these 53 (85.5%) patients had pure sensory or sensory motor axonal neuropathy (mononeuritis multiplex, n = 7), while 9 (14.5%) had demyelinating neuropathy (chronic inflammatory demyelinating polyneuropathy, n = 1). Carpal tunnel syndrome was seen in 11 (10.1%) patients (associated with neuropathy in 6). Of 23 sural nerve biopsies available, perineurial thickening (n = 5, amyloid deposits n = 4), perivascular lymphomononuclear cell infiltrate (n = 4), loss of myelin fibers (n = 2), and necrotizing vasculitis (n = 1) were found. Clinically, however, seven patients had evidence of cutaneous vasculitis. Comparing the clinical characteristics of the patients with or without electrophysiological neuropathy, absence of deep tendon jerks (p < 0.005) and presence of extra articular manifestations (p < 0.01) were conspicuous in the neuropathic group. There was no relation of neuropathy with the duration of RA, seropositivity, joint erosions, joint deformities, prior disease-modifying anti-rheumatic drugs or glucocorticoid intake, and 28-joint disease activity score. Neuropathy in RA was mostly subclinical and predominantly axonal. Pathologically, neuropathy secondary to amyloid infiltration was second only to vasculitic neuropathy. Absence of deep tendon jerks and presence of vasculitis were more commonly observed in patients with neuropathy.

Gliomatosis cerebri(GC) is a highly aggressive, rare form of neuroepithelial tumor. Due to the diffuse nature of the tumor, its pathological grade is not conclusively established unless a biopsy is carried out from multiple sites. The tumor typically infiltrates the cortex in the subpial plane and also insinuates in the perineuronal space and around the Virchow-Robin spaces with relative preservation of the underlying brain parenchyma. Most of the previously reported cases of GC are astrocytic in nature. In this study, we report 2 pediatric patients of GC with oligodendroglial morphology on histopathological examination. In view of the rarity of the oligodendroglial phenotype of gliomatosis cerebri as well as the rare occurrence in children, we report these 2 cases.

BACKGROUND In Langerhans-cell histiocytosis, there occurs an uncontrolled clonal proliferation of dendritic cells that have Langerhans cell like characteristics. In this report, a unique case of a solitary, skull base Langerhans-cell histiocytosis (LCH) is described. CASE DESCRIPTION A 15-year-old boy presented with raised intracranial pressure, decreased visual acuity, bilateral abducent nerve palsy, and 25% hypoesthesia in all three divisions of the right trigeminal nerve. He had normal strength but with a left-sided upper and lower limb hypertonia and hyper-reflexia and an upgoing plantar reflex. The magnetic resonance (MR) imaging revealed a heterogeneously iso-to hyperintense lesion of the clivus and the sphenoid sinus, extending to the right cavernous sinus to encase the right cavernous internal carotid artery segment, and also involving the right petrous apex and the extradural space in the prepontine region. The lesion was brilliantly enhancing on contrast. An extended frontobasal approach was used to excise the lesion. Following surgery, the residual parasellar and the right petrous apex tumor was treated with a low dose radiation therapy. At follow-up after 1.5 years, there was significant clinical improvement and the computed tomographic scan showed no residual lesion. CONCLUSION A review of the literature reveals that this is only the second reported case of a spheno-clival LCH. An additional feature includes extensions into the parasellar as well as the petrous apex regions. Despite the extensive spread, the surgical excision with low dose radiation therapy was successful in providing complete resolution of the tumor.

Background. Hydatid cysts, the larvae of the parasite Echinococcus granulosus , may lodge in any organ of intermediate hosts, namely, man and sheep. Complete cyst removal is the treatment of choice; however, spillage of fertile cysts during surgery leads to disease recurrence that may be prevented by preoperative detection of the fertility status of the cyst. With this perspective, ex vivo proton ( 1 H) MR spectroscopy of hydatid fluid of human and sheep origin was performed to differentiate fertile from sterile cysts on the basis of their metabolite pattern. Patients and materials. Cysts of sheep and human origin were used as source of hydatid fluid. A fraction of this fluid was tested for cyst fertility and the rest was used for ex vivo 1 H spectroscopy. Histopathology of the cyst wall was done as a gold standard for this study. Results. Of 10 sheep samples, 7 were fertile and 3 were sterile, while among 6 human samples, 5 were fertile and 1 was sterile. Spectroscopic and histopathological results corroborated each other. The fluid from microbiologically proven fertile cysts contained malate and fumarate along with other resonances and the histopathology of the fertile cyst wall demonstrated germinal lining and protoscoleces. Conclusions. The ex vivo spectroscopic differentiation of fertile and sterile cysts may be a stepping-stone for their in vivo separation in future and thus help in framing strategies for percutaneous/surgical management.