Pediatric meningiomas an aggressive subset: a clinicopathological and immunohistochemical study.

BACKGROUND: Meningiomas are uncommon neoplasms in the pediatric age group and differ in various aspects from their adult counterparts. They account for 0.4-4.6% of all primary brain tumors. AIMS: To retrospectively analyze the clinicopathological and immunohistochemical features of pediatric meningiomas. MATERIALS AND METHODS: Meningiomas in patients under 18 years of age diagnosed between January 2001 to December 2011 were analyzed retrospectively. The hematoxylin and eosin stained sections and Ki 67 labelling index (LI) were reviewed for all the cases Results: The pediatric meningiomas accounted for 1.52% of total meningiomas (15/983). The mean age at presentation was 12 years with male to female ratio of 1.5:1. The presenting symptoms were headache, seizures, and motor deficits. The histology included 9 cases (60%) of atypical meningioma (WHO grade II) followed by 4 cases (26.67%) of WHO grade-I and 2 cases (13.33%) of anaplastic meningioma (WHO grade III). Five cases had a recurrence. Ki67 LI ranged from 0.5% to 1.5% in grade I, 0.5% to 15% in grade II and 13% to 24% in grade III meningiomas. CONCLUSION: Meningiomas are rare in children and show a male preponderance. There was a higher incidence of atypical and anaplastic meningiomas in pediatric population.

Assessment of expression of epidermal growth factor receptor and p53 in meningiomas.

BACKGROUND: Meningiomas represent about 30% of primary adult central nervous system tumors. Though slow growing, they recur, causing significant morbidity and mortality. OBJECTIVE: The objective of the following study is to grade meningiomas according to World Health Organization (WHO) 2007 criteria and to correlate the grade with degree of expression of epidermal growth factor receptor (EGFR) and p53. MATERIALS AND METHODS: Meningiomas diagnosed in the year 2010 in the Department of Pathology of our institute, were included in the study. Clinical and radiological findings were noted from medical records. The histopathology slides were reviewed and the tumors were graded according to WHO 2007 criteria. Tissue microarrays (TMA) were prepared and immunohistochemical analysis with epithelial membrane antigen, Vimentin, Ki67, EGFR and p53 was performed on the TMA slides. RESULTS: A total of 79 meningiomas diagnosed during the study period included 30 male and 49 female patients with an age range of 10-75 years. There was a female preponderance with M:F ratio of 1:1.63. EGFR was found to be higher in grade I (82.93%) compared with grade II (35.71%) and grade III tumors (20%) with an overall positivity of 60.81%. Mean p53 indices were higher in grade III (50%) compared with grade II (39.29%) and grade I tumors (38.46%) with an overall positivity of 39.44%. Ki67 labeling index (LI) was significantly high in grade III (16.4%) compared with grade II (6.46%) and grade I tumors (3.13%). CONCLUSION: EGFR expression and Ki67 LI correlated with grade of meningioma P < 0.0001 and P < 0.0001 respectively which were statistically significant whereas p53 expression did not correlate (P - 0.90).

Meningiomas: correlation of Ki67 with histological grade.

BACKGROUND: Meningiomas are slow-growing tumors. Grading of meningiomas based on histological features has certain limitations in predicting the exact biological behavior, necessitating ancillary techniques. OBJECTIVE: To study the Ki67 labeling index (Ki67 LI) in various histological subtypes and grades of meningioma and correlate it with various parameters for recurrence. MATERIAL AND METHODS: All intracranial and intraspinal meningiomas diagnosed between 2005 and 2008 were graded according to WHO 2007 criteria. Immunohistochemistry was performed using Ki67 (Dako, USA 1:50) in 300 cases. Statistical analysis was performed. Results : There was female predominance. The age ranged from 2-75 years including 11 children below the age of 18 years. There were 211 Grade I, 78 Grade II and 11 Grade III meningiomas. The mean Ki67 LI increased from Grade I to II and from Grade II to III and these were statistically significant. The Ki67 LI was high for the subtypes of clear cell, chordoid, papillary and rhabdoid but there was no statistical significance between the subtypes. The difference in Ki67 LI between recurrent versus non-recurrent and brain-invasive versus non-invasive meningiomas was statistically significant. CONCLUSION: High Ki67 LI indicates higher grade of meningioma. The difference in KI67 LI between recurrent and non-recurrent meningiomas was statistically significant.

Optic chiasmatic-hypothalamic gliomas: is tissue diagnosis essential?

BACKGROUND: Optic chiasmatic-hypothalamic gliomas are sellar-suprasellar lesions with variable radiological features. The advocated treatment is mainly primary radiotherapy without a histological diagnosis. However, in developing countries, like India infective granulomas (tuberculomas) in the suprasellar region radiologically can mimic optic chiasmatic-hypothalamic gliomas. Hence primary radiotherapy without histological confirmation may have deleterious consequences. AIM: The aim of the paper was to analyze the sensitivity and specificity of magnetic resonance imaging (MRI) in these lesions and to analyze the feasibility of primary radiotherapy. PATIENTS AND METHODS: The magnetic resonance imaging (MRI) characteristics of 24 patients with either histologically proven optic chiasmatic "pilocytic astrocytoma" or radiologically suspected optic chiasmatic-hypothalamic gliomas were analyzed. They were grouped into three groups on the basis of radiological features and treated with a suspected diagnosis. The final diagnosis was correlated with preoperative diagnosis, and the feasibility of managing these lesions without a histopathological confirmation is discussed. RESULTS: The three radiological groups were: Group-1 solid tumors with or without microcysts in 9 patients (histology: 8 pilocystic astrocytomas and 1 tuberculoma); Group-2 mixed tumors with solid and cystic components in 9 patients (histology: 7 pilocytic astrocytomas and 2 craniopharyngiomas); Group-3 ring enhancing lesions in 6 patients (all the 6 patients initially received antituberculous treatment, in 3 patients the lesion resolved and in the remaining 3 patients the lesion was subjected to biopsy as it did not resolve, the biopsy was suggestive of pilocytic astrocytoma). Thus, MRI was shown to have a sensitivity of 83.33% and a specificity of 50% for diagnosing optic chiasmatic-hypothalamic gliomas. CONCLUSIONS: Various lesions like craniopharyngiomas, tuberculomas can mimic optic chiasmatic-hypothalamic gliomas radiologically, and it is not possible to diagnose them with certainty on the basis of radiological findings alone. Biopsy and tissue diagnosis should always be sought before instituting radiotherapy or chemotherapy for optic chiasmatic-hypothalamic gliomas.

Teratomas in central nervous system: a clinico-morphological study with review of literature.

BACKGROUND: Cranio-spinal axis teratomas are rare. This subset is interesting because symptoms can be varied, depending on the location. Histopathology is diagnostic; most of the lesions are benign. Rarely, malignancy develops in any of the somatic components. AIMS: To study the demographic, clinico-morphological and follow-up data of central nervous system (CNS) teratomas. MATERIALS AND METHODS: Cases diagnosed as mature or immature teratomas in the CNS over a 20-year period were included in the study. Clinico-radiological, demographic and follow-up data of these cases were analyzed. RESULTS: A total of 14 tumors were diagnosed as teratomas. Of these, 11 were mature cystic teratomas; and 1 case each, of teratoma with malignant transformation, terato-carcinoma and mixed germ cell tumor (immature teratoma with germinoma). Six of the 14 cases were intracranial and 8 were spinal. Presenting features varied according to the location. Radiologically, contrast enhancement with predominantly solid component was suggestive of malignancy or an aggressive tumor. Morphologically, a variety of tissue derivatives were seen in the cases. Excision was curative or provided symptomatic relief in most cases; terato-carcinoma and mixed germ cell tumor patients needed adjuvant radiotherapy. CONCLUSION: CNS teratomas are rare. Morphology and location decide outcome.

Factors affecting outcome of acute cervical spine injury: A prospective study.

BACKGROUND: Injury to the spine and spinal cord is one of the common cause of disability and death. Several factors affect the outcome; but which are these factors (alone and in combination), are determining the outcomes are still unknown. The aim of the study was to evaluate the factors influencing the outcome following acute cervical spine injury. MATERIALS AND METHODS: A prospective observational study at single-center with all patients with cervical spinal cord injury (SCI), attending our hospital within a week of injury during a period of October 2011 to July 2013 was included for analysis. Demographic factors such as age, gender, etiology of injury, preoperative American Spinal Injury Association (ASIA) grade, upper (C2-C4) versus lower (C5-C7) cervical level of injury, imageological factors on magnetic resonance imaging (MRI), and timing of intervention were studied. Change in neurological status by one or more ASIA grade from the date of admission to 6 months follow-up was taken as an improvement. Functional grading was assessed using the functional independence measure (FIM) scale at 6 months follow-up. RESULTS: A total of 39 patients with an acute cervical spine injury, managed surgically were included in this study. Follow-up was available for 38 patients at 6 months. No improvement was noted in patients with ASIA Grade A. Maximum improvement was noted in ASIA Grade D group (83.3%). The improvement was more significant in lower cervical region injuries. Patient with cord contusion showed no improvement as opposed to those with just edema wherein; the improvement was seen in 62.5% patients. Percentage of improvement in cord edema ≤3 segments (75%) was significantly higher than edema with >3 segments (42.9%). Maximum improvement in FIM score was noted in ASIA Grade C and patients who had edema (especially ≤3 segments) in MRI cervical spine. CONCLUSIONS: Complete cervical SCI, upper-level cervical cord injury, patients showing MRI contusion, edema >3 segments group have worst improvement in neurological status at 6 months follow-up.

Junctional Chiasmatic Syndrome Due to Large Anterior Communicating Artery Aneurysm.

Even though aneurysm involving the anterior communicating artery (A Com A) was common in clinical practice, producing compressive symptoms such as visual loss was rare. We report a case, in which patient had gradually progressive visual loss with features of the junctional chiasmatic syndrome, imaging revealed partially thrombosed large A Com A aneurysm. Intraoperatively, aneurysm was found compressing the optic chiasm and right optic nerve, following clipping and decompression of the optic nerve and chiasm there was gradually improvement in the vision over 2 weeks postoperatively.

C2 Primary leiomyoma in an immunocompetent woman: A case report and review of literature.

Clinical case report and review of the literature. This is the first case of primary leiomyoma in an immunocompetent woman without previous history of uterine leiomyoma being reported in the literature to the best of our knowledge. Leiomyoma, a type of smooth muscle cell tumor, involving the vertebra is extremely rare. There were very few primary leiomyoma in patients with AIDS or in the immune-suppressed patients. This 48-year-old female came with H/o neck pain, weakness and bladder retention. On examination, tone increased in all four limbs, power on the right side of the limbs 4/5, power on the left upper limb 0/5, lower limb 3/5, left plantar was up going, decreased sensation over the left second cervical vertebra (C2) dermatome and all modalities decreased below C2. X-ray and magnetic resonance imaging (MRI) of the cervical spine showed kyphosis of the cervical spine with destruction of the C2 vertebral body along with pathological fracture. The patient underwent decompression of the C2 lesion through the C2 right pedicle with occipito-C1-C3 lateral mass screws fixation. Lesion anterior to the cord was reached by a transpedicular approach and decompression was performed. The lesion was pinkish grey, firm and moderately vascular and was destroying the C2 vertebral body. The patient improved symptomatically in power in the left upper limb and lower limb over the next 1 week duration from 0/5 to 4+/5. Histopathology revealed primary leiomyoma. The patient was evaluated with ultrasound abdomen and contrast tomogram of the chest, abdomen and pelvis to rule out other possible lesions in the lung, intestines and uterus. We suggest that leiomyoma should be included in the differential diagnosis of destructive lytic lesions involving the C2 vertebra. Histopathological examination with immunohistochemistry is necessary for the definitive diagnosis. Treatment of choice is surgery with complete removal.

Pleomorphic xanthoastrocytoma with anaplastic features: A rare case report and review of literature with reference to current management.

Pleomorphic xanthoastrocytoma (PXA) is an uncommon tumor constitutes less than 1% of all astrocytic glial neoplasms was first reported in 1979. PXA commonly occurs in young patients and manifests itself first as seizures followed by focal neurological deficits. The role of radiotherapy or chemotherapy has not yet been established because of the relative infrequency of this disease. PXA is classified as grade II tumor in the WHO classification of tumors of the CNS. In literature 9 to 20 % PXA may undergo malignant change at recurrence or may display at the time of initial presentation. Malignant transformation is mainly associated with high mitotic activity and necrosis. The criteria for PXA with anaplastic features was five or more mitotic activity per 10 high power fields, necrosis, microvascular proliferation, marked cellular anaplasia, and high Ki-67 labeling indices. PXA with anaplastic features management is highly controversial as very sparse literature is available. We are reporting a case of PXA with anaplastic features with atypical radiology and tried to review the up to date literature regarding this rare tumor.

Vascular malformations of central nervous system: A series from tertiary care hospital in South India.

AIMS AND OBJECTIVES: To describe clinicopathological features of surgically resected vascular malformations (VMs) of central nervous system (CNS). MATERIALS AND METHODS: Histologically diagnosed cases of VMs of CNS during April 2010-April 2014 were included. Demographic data, clinical and radiological features were obtained. Hematoxylin and eosin slides were reviewed along with Verhoeff-Van Gieson (VVG), Masson's trichrome, periodic acid-Schiff, and Perls' stains. Morphologically, cavernomas and arteriovenous malformations (AVMs) were distinguished on the basis of vessel wall features on VVG and intervening glial parenchyma. RESULTS: Fifty cases were diagnosed as VMs of CNS with an age range of 14-62 years. These included 36 cavernomas, 12 AVMs, 2 mixed capillary-cavernous angiomas. Most of the cavernoma patients (15/36) presented with seizures, whereas AVM patients (8/12) had a headache as the dominant symptom. Twenty-nine patients were reliably diagnosed on radiological features. Microscopic evidence of hemorrhage was seen in 24/36 cavernomas and 6/12 AVMs, as opposed to radiologic evidence of 10 and 4, respectively. Reactive gliosis was seen in 16 cavernomas. CONCLUSIONS: Histological features are important for classifying the VMs of CNS as there are no specific clinical and radiological features. Type of VM has a bearing on management, prognosis, and risk of hemorrhage.

Isolated Rosai Dorfman disease of the central nervous system presenting as dural-based and intraparenchymal lesions.

OBJECTIVE: To report three patients with isolated Rosai Dorfman disease of the central nervous system. CASE REPORTS: We report two patients with dural-based lesions diagnosed imageologically as meningiomas, and one patient with multiple intraparenchymal lesions diagnosed imageologically as lymphoma. Two patients were males and one was female. All were above 35 years of age with no nodal or other system involvement. The diagnosis was established on surgically excised masses during histopathology. One patient died due to unrelated causes 10 years later and the other two are symptom-free at 21.5- and 11.5-year-follow-up. CONCLUSIONS: Awareness of the entity and consideration in the differential diagnosis of dural-based/intraparenchymal lesions is necessary for diagnosis and prognosis.

Gliomatosis cerebri: Case series of six cases with review of literature.

BACKGROUND: Gliomatosis cerebri is characterized by diffuse infiltration of glial cells with preservation of neuronal architecture. It is an uncommon glial neoplasm of astrocytic origin that occurs in adults and is exceedingly rare in children. MATERIALS AND METHODS: The authors retrospectively analyzed the data of 6 patients of gliomatosis cerebri operated between 2007 and 2012. RESULT: All patients underwent surgical decompression, followed by chemoradiotherapy. The survival ranged between 3 and 45 months. The mean survival was 18.5 years. CONCLUSION: Performance scores at presentation and the nonglioblastomatous histology seems to favorably affect the prognosis. Larger studies are required to comment on the role of combination of surgery, chemoradiotherapy as a treatment modality.

The isolation of peste des petits ruminants virus from northern India.

The aetiological agent responsible for an epizootic of a rinderpest-like disease afflicting sheep and goats in three states of northern India was confirmed as peste des petits ruminants virus. To differentiate the virus from rinderpest a number of diagnostic tests were used, including immunocapture ELISA, specific oligonucleotide primers in a reverse transcriptase polymerase chain reaction, immunofluorescence with virus specific monoclonal antibodies and virus isolation. The virulence profile of one isolate in cattle sheep and goats was established. Infected animals developed specific antibody responses and excreted specific antigen in their lachrymal secretions.

Cranial and intracranial actinomycosis.

Five patients with central nervous system actinomycosis are presented. There were risk factors in 2 patients like penetrating head injury and tetralogy of Fallot. All the cases were diagnosed by histopathology. Four patients recovered after surgery and antibiotic therapy, and 1 patient died.

Exclusive CNS involvement by lymphomatoid granulomatosis in a 12-year-old boy: a case report.

BACKGROUND: Lymphomatoid granulomatosis [LYG] is an angiocentric, angiodestructive disease at the higher grade end of the spectrum of angiocentric immunoproliferative lesions. It primarily involves the lungs, but it may also involve several extrapulmonary sites including the central nervous system (CNS), skin, and kidneys. CASE DESCRIPTION: Clinical presentation, radiology and treatment of LYG in a 12-year-old male child with multiple intracranial extraaxial lesions is described. A 12-year-old boy presented with sudden onset of left focal motor seizures with associated history of headache and vomiting. Computerized tomographic scan of the brain suggested high-density, bilateral, parietal extraaxial lesions. On magnetic resonance imaging, the lesions were iso- to hyperintense on T1-weighted images and hyperintense on T2-weighted images. The lesions were excised in two stages and histopathological examination confirmed the diagnosis. CONCLUSION: LYG seldom involves the CNS exclusively. The present case demonstrates exclusive CNS involvement by LYG in a young boy.

Claudicatory penile erection in lumbar canal stenosis with prolapsed disc : a case report of 2 cases.

Two adult male patients having lumbar canal stenosis with prolapsed disc, developed claudicatory priapism along with classical symptoms of claudication, are reported in this article. The first patient complained of severe claudicatory pain in anal canal along with other symptoms. The second patient had a protruded bony rim at the lower margin of L4 vertebra in addition to the prolapsed disc. All the symptoms totally subsided after relieving the compression.

CSF orbitorrhoea with tension pneumocephalus.

A seventy eight year old man sustained penetrating injury to right orbit about 15 years ago. Later he developed right orbital infection leading to phthisis bulbi. Two months before admission he developed CSF leak from the right orbit, tension pneumocephalous and meningitis. A rare case of CSF orbitorrhoea is reported here along with the discussion on mechanisms and management.

Distal calcarine fusiform aneurysm: a case report and review of literature.

A 50 year old female who was operated for atrial septal defect 8 years back, presented with clinical features suggestive of subarachnoid haemorrhage (grade I, Hunt and Hess). CT scan of brain revealed haemorrhage in all the supratentorial basal cisterns, sylvian cistern and small haematoma in the left occipital lobe. Conventional CT and MR angiography revealed aneurysm in relation to distal part of the calcarine branch of the left posterior cerebral artery (PCA). Left occipital craniotomy in prone position followed by deep dissection in the occipital lobe showed fusiform aneurysm of the distal part of the calcarine branch. PCA aneurysms constitute only 0.2 to 1% of all intracranial aneurysms and among them distal PCA aneurysms are most rare, constituting only 1.3%. They too are mostly seen at the bifurcation of the PCA. The present case however, is unique in the sense that it has developed as a fusiform aneurysm in the distal part of the calcarine branch. To the best of our knowledge this is rare among the rarest.

Benign meningioma metastasizing through CSF pathways: a case report and review of literature.

Metastasis of intraventricular meningiomas through CSF pathways is a rarity and only 4 cases have been reported in world literature describing meningiomas which were intraventricular and malignant. Here we report a case of benign intraventricular meningioma which had spread through CSF pathways, the recurrences as well as the primary tumor being benign in nature.