Primary Dural Non-Hodgkin's Lymphoma as a Diagnostic Pitfall: Report of a Rare Presentation with Review of Literature.

Primary central nervous system lymphomas (PCNSLs) account for 2%-3% of all primary brain tumors. Lymphomas arising from the dura, without brain and other systemic involvement, constitute a tiny fraction of this rare group. Most dural lymphomas present as dural-based masses and an acute presentation as a subdural hematoma is exceptional, with only five such cases reported previously in the literature. We present the case of a 65-year-old female who was admitted with complaints of slurring of speech, visual disturbances, and reduced appetite for 2 days. Computerized tomography scan showed a crescentic hyperdense area in the left fronto-temporo-parietal region with no bony or extracranial involvement suggestive of acute subdural hematoma. There was a history of a similar episode 3 months back, wherein she was operated upon and followed up conservatively. Craniotomy for drainage was performed, and histopathology revealed a non-Hodgkin's Lymphoma. Primary lymphoma of the dura is a rare subtype of PCNSL. This case is being reported for its unusual presentation as a common entity-an acute subdural hematoma.

Multiple Primary Bilateral Cerebral Echinococcosis in an Adult: A Neurological Rarity.

Although cases of intracranial hydatid cysts are commonly encountered, majority of them are secondary and are seen in children. Primary bilateral multiple intracranial hydatid cysts in adults are a neurological rarity. Intracranial involvement usually occurs following infestation of the other organs in the body by the pathogen. Multiple lesions in brain can manifest either following rupture of a single parent cyst in brain or by systemic dissemination from other organs. Here, we are presenting an adult patient who underwent successful surgical removal of multiple primary hydatid cysts of brain. A 47-year-old male patient presented with features of raised intracranial pressure and other focal deficits. Magnetic resonance imaging showed multiple cystic lesions on both sides of the cerebrum. Similar primary lesions elsewhere in the body were ruled out by other investigations. He underwent successful surgical removal of all the lesions. Intraoperative picture and histology were suggestive of hydatid cyst. In published literature, there are very few cases similar to ours. Echinococcosis should be considered as a differential diagnosis in multiple cystic lesions of the brain in endemic areas. Early diagnosis, proper surgical technique, and standard antihelminthic drug regimen are the key to achieve good outcome in these patients.

Extra-axial adult cerebellopontine angle medulloblastoma: Revisiting a rare entity.

A purely extra-axial position of medulloblastoma in adults at cerebellopontine (CP) angle is extremely rare. To the best of our knowledge, only ten cases have been reported till date. The authors report a case of extra-axial medulloblastoma in a 30-year-old female located at right CP angle. It was surgically treated with a provisional diagnosis of meningioma. Histopathological diagnosis of desmoplastic/nodular medulloblastoma was made with the routine hematoxylin eosin (HE) stain and immunohistochemical markers. This case report highlights the fact that, although extremely rare, the possibility of an extra-axial CP angle mass being a medulloblastoma still needs to be considered in the differential diagnoses, even in adults.

Efficacy and Outcome of Embolization of Intracranial Meningiomas With Minimal Resources.

BACKGROUND: Preoperative embolization of intracranial meningiomas is an accepted treatment modality and offers many advantages for subsequent surgery. It has also been used as a primary therapy without subsequent surgery in selected cases. In this study, the authors share their experience of this procedure with minimal resources carried out in cases where definitive surgery was not advisable. METHODS: This is a prospective study of 6 cases of meningiomas treated over a period of 11 months (November 2020 to September 2021). None of the cases were fit for prolonged surgery under general anesthesia and also had certain comorbid conditions. Embolization was done in a setup with limited resources without a fully equipped catheterization lab. Post-procedure magnetic resonance imaging (MRI) was done within 48 hours of the procedure. Follow-up was done in the outpatient department. RESULTS: There was complete disappearance of tumor blush in all the 6 cases. Immediate post-procedure MRI revealed significant necrosis of the tumor cavity. Three cases succumbed to their primary comorbid conditions and in the other cases follow-up was uneventful. Following embolization, clinical improvement of presenting symptoms was seen in all 6 cases. CONCLUSIONS: In resource-restricted settings, by introducing such innovative methods it is possible to perform selected endovascular procedures. Embolization can be used as a primary treatment modality avoiding subsequent surgery in selected cases with a good outcome.

Gangliocytic paraganglioma of the spine.

Paragangliomas are rare, encapsulated, benign neuroendocrine tumors that can arise from the adrenal medulla or extra-adrenal paraganglia. Extra-adrenal paragangliomas may develop a gangliocytic component with ganglion cells (Gangliocytic paragangliomas). Nearly 25%of cauda equina paragangliomas are gangliocytic paragangliomas. Here, we describe the case of a 35-year-old male who presented with weakness of both lower limbs over the last two months. Radiological findings were suggestive of myxopapillary ependymoma. However, the histopathological examination revealed a tumor with cells arranged in sheets, papillae, lobules, and around vessels forming pseudo rosettes. Ganglion cells were seen in small groups and, also singly. Tumor cells were immunopositive for chromogranin, synaptophysin, and S-100. Ganglion cells were immunopositive for synaptophysin, NSE, and NFP. A final histological diagnosis of Gangliocytic paraganglioma (WHO grade I) was made. To date, only nine gangliocytic paraganglioma cases have been previously reported, and to the best of our knowledge, this is the largest gangliocytic paraganglioma.

Historadiological Correlation of Cerebellopontine Angle Tumors: Series of 122 Cases.

Cerebellopontine angle (CPA) tumours, are diverse pathologically with regard to the site of tumour origin and displacement of the neurovascular structures. In general CPA tumours are divided into acoustic and non-acoustic tumours. The aim of the present study was to see the spectrum of lesions at cerebellopontine angle and their historadiological correlation. A retrospective analysis of 122 cases of CPA tumors diagnosed in the Department of Pathology, IHBAS was done from January 2004 to August 2019. There were 56 males and 66 females in the age group of 8-68 years. Clinical history ranged from 10 days to 5 years. Clinical symptoms included hearing deficit, visual loss, facial nerve dysfunction, nausea, vomiting, headache, seizures, unsteadiness and disequilibrium. Historadiological correlation was found in 112 cases (91%).In our series of CPA tumors, nonacoustic tumors were more frequent than in previous studies. We found that presence of intrameatal fraction is not exclusive only for schwannomas. There can be a rare occurrence of medullobastoma/small round cell tumor in CP angle. The final pathological verification can at times give unexpected results. Immunohistochemistry did not have a significant role in diagnosis of cerebellopontine angle tumors.

Congenital Myopathies: A Clinicopathological Study of 10 Cases in a Tertiary Care Hospital of North India.

Objective: Congenital myopathies (CMs) are rare neuromuscular disorders. Through this article, authors want to present a clinicopathological study of 10 cases of CM. Materials and Methods: The study included patients with histopathologically confirmed CM attending the neurology services at the Institute of Human Behavior and Allied Sciences for 2 years. After collecting the demographic data, all patients were subjected to comprehensive workup including a detailed neurological examination and investigations, including muscle biopsy from representative involved muscle. Results: Ten patients diagnosed with CM. The most common CM type was congenital fiber-type disproportion (CFTD) seen in four cases followed by centronuclear myopathy in two cases and one each in desmin-related myopathy, central core disease, nemaline myopathy, CM with type II fiber hypoplasia. Clinically, they have variable features. Conclusion: This study from India highlights the importance of specific clinical features to look for when suspecting a CM coupled with specific features in histopathology. However, studies with longer duration are needed to find out the true prevalence and various spectra of CMs.

Gangliocytic paraganglioma of the spine

Paragangliomas are rare, encapsulated, benign neuroendocrine tumors that can arise from the adrenal medulla or extra-adrenal paraganglia. Extra-adrenal paragangliomas may develop a gangliocytic component with ganglion cells (Gangliocytic paragangliomas). Nearly 25%of cauda equina paragangliomas are gangliocytic paragangliomas. Here, we describe the case of a 35-year-old male who presented with weakness of both lower limbs over the last two months. Radiological findings were suggestive of myxopapillary ependymoma. However, the histopathological examination revealed a tumor with cells arranged in sheets, papillae, lobules, and around vessels forming pseudo rosettes. Ganglion cells were seen in small groups and, also singly. Tumor cells were immunopositive for chromogranin, synaptophysin, and S-100. Ganglion cells were immunopositive for synaptophysin, NSE, and NFP. A final histological diagnosis of Gangliocytic paraganglioma (WHO grade I) was made. To date, only nine gangliocytic paraganglioma cases have been previously reported, and to the best of our knowledge, this is the largest gangliocytic paraganglioma.

A rare case of diffuse intracranial aspergillosis masquerading as skull base meningioma in an immunocompetent patient.

Intracranial aspergillosis is rare in immunocompetent patients. Its presentation is subtle, often without any diagnostic characteristics, and is frequently mistaken for tuberculous meningitis, pyogenic abscess, or a space-occupying lesion. The authors report a case of diffuse intracranial aspergillosis, in an immunocompetent 34-year-old male, that mimicked a meningioma on preoperative imaging. The origin, clinical course, radiological features, histopathological findings, and surgical treatment are discussed based on review of literature.
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Intramedullary schwannoma of conus medullaris: rare site for a common tumor with review of literature.

INTRODUCTION: Schwannomas at an intramedullary location constitute only 0.3% of primary spinal tumors. Those in the conus are extremely rare, especially in the absence of neurofibromatosis, with only 12 cases reported in the literature so far, excluding the present case. CASE PRESENTATION: We report the case of a 27-year-old female who presented with complaints of backache since 1 year, weakness in both lower limbs with tingling and numbness since 6 months, and urinary incontinence since 1 month. There was no history of trauma or any skin lesions. Magnetic resonance imaging (MRI) of the spine provided a provisional diagnosis of ependymoma. The patient was operated upon, histopathology revealed it to be a schwannoma. DISCUSSION: Intramedullary schwannomas without neurofibromatosis are extremely rare at the conus and can be completely resected unless there is infiltration. Gliomas and ependymomas are commoner entities at this site, which are usually subtotally resected. This case highlights that although extremely rare, the possibility of a schwannoma may be considered in the differentials of an intramedullary spinal lesion.

Endoscopic Treatment of Hydrocephalus with Minimal Resources: Resource Utilization and Indigenous Innovation in Developing Countries like India.

CONTEXT: Neuroendoscopic surgeries need specialized equipments, unavailable in neurosurgical departments of majority of public healthcare institutions of India. Aims: Neuroendoscopic treatment of hydrocephalus in the setting of minimal resources using utilization of available resources, inter-departmental co-ordination and indigenous innovations. METHODS: Study was carried out at a public sector institute of India with scarce resources. Senior author (DKJ) used indigenously designed stainless steel working sheath along with equipments of 'awake endoscopic intubation system' of department of neuroanesthesia and 18 cm, 4 mm, 0° rigid telescope for neuroendoscopic surgeries for various intraventricular pathologies. RESULTS: Thirty-four neuroendoscopic surgeries in 32 patients were done over last 3 years. There were 18 males and 14 females with average age of 23 years. It included hydrocephalus due to tubercular meningitis (n = 19), neurocysticercosis (NCC) (n = 4), intra-ventricular (n = 2) and para-ventricular (n = 2) space occupying lesions, aqueduct stenosis with (n = 2) or without (n = 1) shunt malfunction and one case each of pyogenic meningitis and right cerebellar infarction. Endoscopic third ventriculostomy (ETV) (n = 28), septostomy (n = 6), removals of cystic lesions (n = 3) and biopsies of intraventricular lesions (n = 2) were done in a total of 34 neuroendoscopic surgeries. Overall there were four failures of ETVs, which were managed by ventriculo-peritoneal shunts. Two mortalities in the study group were unrelated to the surgical procedures. CONCLUSION: Indigenous innovations and interdisciplinary co-ordination are the way ahead to tackle resource scarcity in public sector healthcare institutions of India in the scenario of plenty of neuroendoscopic trainings opportunities for young neurosurgeons and paucity of equipments required.

Peripheral neuropathy and the role of nerve biopsy: A revisit.

BACKGROUND: Nerve biopsy has been widely used to investigate patients with peripheral neuropathy and in many centers, it is still a part of the diagnostic armamentarium. In this study, the histopathological spectrum of the nerve biopsies received is being revisited to analyze the various clinical and pathologic features and also to assess their relevance. MATERIALS AND METHODS: Retrospective analysis of the data retrieved was done for 74 cases of nerve biopsies. RESULTS: On the basis of the data and histopathological features, broad diagnoses were obtained in 52 cases and further categorized into biopsies being supportive for patient management (including acute and chronic axonopathies and demyelinating neuropathies) and biopsies considered essential for patient management (including vasculitic neuropathies, leprous neuropathies, hereditary neuropathies, and chronic inflammatory demyelinating neuropathies). Nine nerve biopsies did not show any abnormal histopathological features, while 13 nerve biopsies were found to be inadequate for diagnosis, both these groups were categorized as noncontributory. CONCLUSION: With advanced nerve conduction studies available, nerve biopsy is losing its relevance. However, in our experience, nerve biopsy did complement the clinical findings and nerve conduction studies, with which a close correlation is required to make the histopathology of nerve biopsy more relevant in terms of guiding further specific workup and management.

Giant Cell Glioblastoma in a Child with Clinical and Family History of Neurofibromatosis.

We report a case of giant cell glioblastoma (GCG) in a 13-year-old child with clinical features and family history of neurofibromatosis type 1 (NF1). To the best of our knowledge, only two cases of GCG have been reported in a scenario of NF1, and only one of that was in a pediatric age group. A report on our case is presented here along with a review of literature.

Spinal tumour: primary cervical extradural meningioma at an unusual location.

Extradural spinal meningiomas are extremely rare, more so in the cervical region. A purely extradural location as reported in this paper is quite exceptional. The authors report a case of extradural meningioma in a 50-year-old male located in the cervical spine that was surgically treated with the provisional diagnosis of a neurofibroma. Histopathological diagnosis of meningothelial meningioma was made with the routine hematoxylin and eosin (HE) stain. The origin, clinical course, radiological features, pathological findings with the differential diagnosis and surgical treatment are discussed based on a review of the literature. An extradural spinal meningioma, an extremely rare entity, is still a diagnostic dilemma on radiology as the radiologic findings overlap with many other common extradural spinal masses.

Spinal cysticercosis: A report of two cases with review of literature.

Neurocysticercosis is the most common parasitic infection of the central nervous system worldwide. However, Cysticercosis affecting the spine is considered extremely rare. We report two cases of spinal cysticercosis with review of literature.

Extra-axial medulloblastoma in the cerebellopontine angle: Report of a rare entity with review of literature.

With only 33 cases reported so far, a purely extra-axial position of medulloblastoma at cerebellopontine (CP) angle is quite exceptional. We report a case of extra-axial medulloblastoma in a 15-year-old male child located in the CP angle that was surgically treated with a provisional diagnosis of schwannoma. Histopathological diagnosis of medulloblastoma was made with the routine hematoxylin and eosin stain and immunohistochemical markers. This case report highlights the fact that although extremely rare, the possibility of an extra-axial mass being a medulloblastoma does exist.

Muscle histopathology in today's era of molecular genetics: Role and limitations.

INTRODUCTION: Past few decades have seen an increasing application of techniques like electron microscopy, western blotting, and molecular genetics in the evaluation of muscle diseases. However, due to their limited availability, histopathological interpretation of muscle biopsies still remains an important component of diagnostic approach to muscle diseases. A systematic methodology is required in the evaluation and interpretation of muscle biopsies. This study was undertaken to analyze the histopathological spectrum of 164 muscle biopsies and to assess the diagnostic yield of basic histopathological procedures in the work up of muscle biopsy. MATERIALS AND METHODS: Retrospective analysis was done for 164 cases of muscle biopsies. Step-wise approach was adopted to assess the efficacy of routine stains, enzyme histochemistry, and immunohistochemistry. Based on hematoxylin and rosin (H and E) appearance, biopsies were broadly categorized into destructive, nondestructive but myopathic, and inflammatory morphology. Role of special stains, enzyme, and immunohistochemical stains in each category was then evaluated. RESULTS: On the basis of histopathological features, 164 muscle biopsies were broadly categorized into biopsies with abnormal histopathological features (115) and biopsies with normal histopathology (49). Abnormal muscle biopsies were further categorized into destructive morphology (56.5%), nondestructive but myopathic morphology (30.5%), and inflammatory pathology (13%). A near definitive diagnosis could be made in 115 cases out of 164 muscle biopsies on the basis of routine histopathology, enzyme histochemistry, and immunohistochemistry. CONCLUSION: Though advanced techniques like electron microscopy, western blotting, and molecular genetics are essential for confirmatory diagnosis, a substantive diagnostic yield can be offered with the basic armamentarium of routine (frozen) stains, enzyme histochemistry, and immunohistochemistry.

Central nervous system tumors: Radiologic pathologic correlation and diagnostic approach.

OBJECTIVE: This study was conducted to formulate location-wise radiologic diagnostic algorithms and assess their concordance with the final histopathological diagnosis so as to evaluate their utility in a rural setting where only basic facilities are available. MATERIALS AND METHODS: A retrospective analysis to assess the concordance of radiology (primarily MRI) with final histopathology report was done. Based on the most common incidence of tumor location and basic radiology findings, diagnostic algorithms were prepared. RESULTS: For supratentorial intraaxial parenchymal location concordance was seen in all high-grade astrocytomas, low- and high-grade oligodendrogliomas, metastatic tumors, primitive neuroectodermal tumors, high-grade ependymomas, neuronal and mixed neuro-glial tumors and tumors of hematopoietic system. Lowest concordance was seen in low-grade astrocytomas. In the supratentorial intraaxial ventricular location, agreement was observed in choroid plexus tumors, ependymomas, low-grade astrocytomas and meningiomas; in the supratentorial extraaxial location, except for the lack of concordance in the only case of metastatic tumor, concordance was observed in meningeal tumors, tumors of the sellar region, tumors of cranial and paraspinal nerves; the infratentorial intraaxial parenchymal location showed agreement in low- as well as high-grade astrocytomas, metastatic tumors, high-grade ependymoma, embryonal tumors and hematopoietic tumors; in the infratentorial intraaxial ventricular location, except for the lack of concordance in one case of low-grade astrocytoma and two cases of medulloblastomas, agreement was observed in low- and high-grade ependymoma; infratentorial extraaxial tumors showed complete agreement in all tumors of cranial and paraspinal nerves, meningiomas, and hematopoietic tumors. CONCLUSION: A location-based approach to central nervous system (CNS) tumors is helpful in establishing an appropriate differential diagnosis.

Intravascular lymphoma: an unusual cause of rapid cognitive decline and the role of brain biopsy.

Intravascular lymphoma (IVL) is a rare extra nodal variant of non Hodgkin's lymphoma characterised by neoplastic lymphoid cells growing inside the lumina of medium and small vessels. IVL limited to the central nervous system (CNS) is an extremely rare condition as IVL is usually found with systemic lesions. Most cases of IVL are not diagnosed until post mortem because of variable clinical presentation and non-specific laboratory findings. Even if diagnosed early the disease is clinically aggressive and usually fatal, even with early detection and treatment. We present a case of a 37-year-old woman with a short history of behavioural abnormality, rapidly progressive cognitive decline and seizures. There were no cutaneous manifestations. Diagnosis was established only after the brain biopsy. The case is presented for the rarity of its presentation and role of brain biopsy in diagnosis.