Clinical spectrum, phenotypic and molecular characterization, and antifungal susceptibility of an emerging human pathogen, Acrophialophora, from India.

Acrophialophora is implicated in superficial and invasive infections, especially in immunosuppressed individuals. The present study was undertaken to provide clinical, microbiological, phylogenetic, and antifungal susceptibility testing (AFST) profile of Acrophialophora isolated from India. All the isolates identified as Acrophialophora species at the National Culture Collection for Pathogenic Fungi, Chandigarh, India were revived. Phenotypic and molecular characterization was performed, followed by temperature studies, scanning electron microscopy (SEM), and AFST. We also performed systematic review of all the cases of Acrophialophora species reported till date. A total of nine isolates identified as Acrophialophora species were identified by molecular method as A. fusispora (n = 8) and A. levis (n = 1), from brain abscess (n = 4), respiratory tract (n = 3), and corneal scraping (n = 2). All patients but two had predisposing factors/co-morbidities. Acrophialophora was identified as mere colonizer in one. Temperature studies and SEM divulged variation between both species. Sequencing of the internal transcribed spacer ribosomal DNA and beta-tubulin loci could distinguish species, while the LSU ribosomal DNA locus could not. AFST showed the lowest minimum inhibitory concentrations (MICs) for triazoles and the highest for echinocandins. Systematic literature review revealed 16 cases (11 studies), with ocular infections, pulmonary and central nervous system infections, and A. fusispora was common species. All the patients except three responded well. High MICs were noted for fluconazole, micafungin, and caspofungin. This is the first study delineating clinical, phenotypic, and genotypic characteristics of Acrophialophora species from India. The study highlights microscopic differences between both species and emphasizes the role of molecular methods in precise identification. Triazoles appear to be the most effective antifungals for managing patients.

We describe clinical, phenotypic, and genotypic characteristics of Acrophialophora species. This species causes mild infection to fatal infection in immunosuppressed individuals. Triazoles are effective in treating such infections.

Isolated embryonal rhabdomyosarcoma of the anterior petrous bone in a young child: Focusing on the intra-operative diagnosis and differentials.

OBJECTIVE: Rhabdomyosarcoma is a common soft tissue tumor, but isolated involvement of anterior portion of petrous bone is exceedingly rare. Here, we present a case of embryonal rhabdomyosarcoma involving the anterior petrous without involvement of the mastoid and middle ear. PATIENT: A 6-year-old boy presented with a progressive right side lower motor neuron facial paresis for 1-month duration along with headache and recurrent vomiting episodes. Radiology showed a contrast-enhancing lesion involving the right petrous apex. He underwent craniotomy and excision of the lesion. Based on the frozen section, a diagnosis of rhabdomyosarcoma was rendered, and gross total resection could be achieved. Postoperative course was uneventful. CONCLUSION: Isolated petrous bone involvement of embryonal rhabdomyosarcoma is a rare presentation. Intra-operative frozen section plays a key role in decision making regarding the extent of excision. Hence, a prompt and accurate diagnosis is essential in managing these cases.

Suprasellar masquerader: Chordoid glioma.

BACKGROUND: Chordoid glioma is a rare well-circumscribed glial neoplasm arising in adults and predominantly affects females. Tanycytes of the third ventricle have been proposed as the cell of origin owing to its location. It is characterized by chordoid features with myxoid and inflammatory stroma and recurrent PRKCA p.D463H missense mutation. CASE REPORT: We present two cases (30-year-old female and 45-year-old male) with similar complaints of behavioral change and headache. Midline suprasellar homogeneously enhancing mass was seen on contrast-enhanced magnetic resonance imaging. Histopathology and immunohistochemistry was characteristic of chordoid glioma with cords and clusters of epithelioid cells arranged in a solid pattern. There were variable amounts of myxoid stroma and lymphoplasmacytic infiltrate. No mitosis, necrosis, or brain invasion was noted. The cells expressed strong diffuse positivity for glial fibrillary acid protein (GFAP) and weak nuclear thyroid transcription factor (TTF-1). Epithelial membrane antigen (EMA)and brachyury were negative. Subsequently, the lady underwent gross total excision and died soon after the operation. The male patient received radiotherapy and is currently doing well after 6 months of follow-up. CONCLUSION: The rare occurrence as well as the radiological and morphological overlaps in chordoid gliomas make them a true masquerader. Combination of GFAP and TTF-1 in the immunohistochemical panel can be useful in differential diagnosis. Mainstay of treatment is complete surgical excision, with adjuvant radiotherapy becoming increasingly important.

Interdural extra-arachnoidal resection of fifth nerve schwannomas with tailored petrous drilling: Eliminating the blind spot.

BACKGROUND AND OBJECTIVES: Around 20-40% of trigeminal schwannomas (TS) are dumbbell shaped, spanning the middle and posterior cranial fossa The petrous apex is often truncated in these patients, aiding surgical resection of both compartments through the middle fossa approach. However, a less eroded petrous creates a blind spot, making total resection difficult. This study describes the feasibility of an approach combining expanded Meckel cave access with tailored petrous bone drilling to optimize tumor visualization and resection. METHODS: Eleven patients with dumbbell TS and minimal petrous apex erosion underwent the described surgery. Surgical steps included temporo-orbito-zygomatic craniotomy, middle fossa floor drilling, navigation aided (tailored) extradural petrous bone drilling and extra-arachnoidal tumor excision. Extent of resection and postoperative outcomes were recorded. RESULTS: Patients presented with trigeminal nerve dysfunction (n = 9; sensory -9 and motor - 5), headache(8/11), ataxia (7/11) and pseudobulbar palsy (3/11). Complete tumor resection was achieved in all patients. Postoperatively, 8 out of 9 patients had transient increase in facial hypoesthesia with conjunctival injection in 3. It improved within 3-6 months, except in four cases wherein mild hypoesthesia persisted. Motor symptoms improved in 2 of 5 patients. Two developed transient 6th nerve paresis, that resolved in 2 months. Cerebellar and brainstem pressure symptoms resolved in all. No patients developed new onset permanent neurological deficit. Two patients reported mild post-craniotomy masticatory difficulty. CONCLUSIONS: The additional tailored petrous bony drilling enhances the surgeon's view, allowing a higher chance of total resection with no major operative morbidity.

Controlled arachnoid opening to aid endoscopic trans-sphenoidal extrarachnoidal dissection of pituitary macroadenoma.

OBJECTIVES: The arachnoid often bulges prematurely during surgical excision of large pituitary tumors obscuring the deeper regions and crevices preventing total excision. Pushing the arachnoid may not be helpful and may tear it inadvertently and extensively leading to complications. We have described controlled arachnoid opening in large pituitary macroadenomas during the final stages of excision to gain access to the hidden portions and compared our results to the conventional technique. PATIENTS AND METHODS: Patients with pituitary macroadenoma of Hardy's III and KNOSP II in whom arachnoid had bulged prematurely were considered for study. They were grouped temporally. In first group (n = 12), the arachnoid was pushed to retrieve the tumor and in the second group (n = 10) it was punctured to aid resection. The extent of resection was assessed on postoperative scans, and complications in both groups, were noted. RESULTS: In the first group where arachnoid was pushed to retrieve tumor, Gross Total Resection (GTR) could be achieved in 5 patients. Inadvertent large arachnoid tear occurred in 3 patients of which, 2 developed CSF Rhinorrhoea. No patient had neurovascular injury.In the second group, GTR could be achieved in all without any added complications. CONCLUSION: Deliberate needle puncture and controlled drainage of CSF from arachnoid that bulges prematurely while endoscopic Transsphenoidal surgery for large pituitary tumors is a safe and effective method to gain access to the hidden portions of tumor to achieve GTR.

Delayed yet effective response of an endolymphatic sac tumor to radiosurgery: case report focusing on its radio-biological behavior.

Endolymphatic sac tumors (ELST), though benign are locally invasive lesions. Owing to its vascularity, complete surgical resection is often not possible and adjuvant gamma knife radiosurgery (GKRS) is advocated to control tumor growth. These lesions do not uniformly respond to radiation therapy in the initial phase and their early radiobiological course after GKRS is less understood. We discuss a case of residual ELST where a mild increase was noted at 36 months following GKRS and then regressed completely after a decade. This report possibly has the longest follow-up revealing the true efficacy of GKRS in these tumors. ELST shows a variable response in the early years after GKRS. They may remain static, regress or increase in size. One should be aware of these patterns of early radiological responses and a long term follow up is warranted as some lesions may show radiosurgical effectiveness after a long latent period.

Feasibility of awake endoscopic third ventriculostomy in selected patients of obstructive hydrocephalus.

BACKGROUND: Endoscopic third ventriculostomy (ETV) is usually performed under general anesthesia (GA) with proper head immobilization. However a few patients with hydrocephalus (HCP) may not be suitable for GA. Once the surgeon is familiar with endoscopic ventricular anatomy and gains adequate surgical experience with the procedure, ETV can be attempted under local anesthesia (LA) in selected patients. Here we discuss our experience of treating 32 patients of HCP with ETV under LA. METHODS: 32 symptomatic HCP patients with in the age range of 13 and 65 years, conscious, alert, cooperative and at high risk for GA owing to deranged liver or renal function, associated co-morbidities, pregnancy were considered for ETV under scalp block. All patients were evaluated for any discomfort during the surgical intervention. RESULT: All procedures were completed under LA. Four patients needed additional sedation prior to the scalp block to alleviate their apprehension. Four patients complained of bilateral orbital pain. In three it coincided with irrigation of fluid lower than body temperature. One patient had pain while touching the dorsum sella and needed analgesic supplement. All of them improved and none required additional CSF diversion within the average follow up of 9.5 months. CONCLUSION: ETV can be performed under local anesthesia in conscious, alert and cooperative patients in experienced hands. Unnecessary stimulation of the painful structures should be avoided and fluid for irrigation should be at body temperature. This ensures patient comfort and safety of the procedure.

Multiple Fusarium brain abscesses in a young child.

Isolated intracranial fungal infection is infrequent and mostly seen in high-risk, immunocompromised patients. Fusarium, a primary plant fungus, rarely contributes to such disease. Amongst the very few cases of Fusarium brain abscess that have been reported, the infection has occurred mostly in adults. We present a case of a 6-year-old boy with tuberculous meningitis diagnosed with multiple Fusarium brain abscess caused by Fusarium falciforme during his clinical course. An immunocompromised state secondary to tuberculous meningitis presumably led to this infection. After tapping the abscesses, the child was treated with a combination of amphotericin B, voriconazole and terbinafine. Despite an aggressive therapy, he remained in poor neurological state. This is the second report of an isolated Fusarium abscess in pediatric age and the first one in a young child and provides pertinent review of this unusual central nervous system fungal infection. Such unusual infectious spectrum should be borne in mind in patients with co-existent immunosuppression.

Extra-axial, dural-based atypical teratoid/rhabdoid tumor.

Atypical teratoid/rhabdoid tumors (ATRTs) are malignant central nervous system tumors that affect early childhood (< 3 years), and mostly located in the infratentorial space. Owing to an infrequent occurrence, their radiological features have not been completely defined. Nevertheless, these are characteristically intra-axial except for few instances in the cerebellopontine angle region. We describe a case of a 10-year-old boy who harbored an extra-axial, dural-based ATRT in the right parietal region. The lesion was totally excised followed by adjuvant chemo-radiotherapy. At 10-month follow-up, he was well with no recurrence. The report intends to highlight an atypical imaging presentation of ATRT in an older child, and adds to the radiological spectrum. This uncommon pathology should be borne in mind, even in a supratentorial dural-based location.

ALK-1-negative anaplastic large-cell lymphoma presenting as a dura-based mass in an infant.

INTRODUCTION: Anaplastic large-cell lymphoma (ALCL) rarely occurs in the central nervous system in the pediatric population. CASE PRESENTATION: We describe a diagnostically challenging case of an 11-month-old infant presenting with cranial nerve palsies and peripheral eosinophilia. Imaging demonstrated meningeal thickening with enhancement and dura-based deposits, the biopsy of which revealed features of ALK-1 negative ALCL on histologic and immunophenotypic analysis. A thorough investigation excluded the possibility of any extra-cranial origin. Hence, a diagnosis of "primary" ALCL was rendered. CONCLUSION: ALCL arising in the dura in an infant has not been described earlier, to the best of our knowledge.

Co-existent epidermoid and dermoid in a child with spinal dysraphism.

Both spinal epidermoids and dermoids, given their common embryological origin, are referred as a single entity under the category of spinal inclusion tumors. Many theories, although speculative, have been proposed in relevance to their development. We present a unique case of dual pathology consisting of both epidermoid and dermoid components in a child with spinal dysraphism and succinctly touch upon the related embryological aspects and plausible pathogenesis. To the best of our knowledge, such co-existent entity has not been observed in the pediatric spine. The report adds to the gamut of the diverse observations of spinal dysraphic anomalies.

Displaced odontoid synchondrosis fracture with C1-2 dysjunction in an 18-month-old child: challenges and solutions.

BACKGROUND: Odontoid synchondral fractures in very young children with displacement/angulation are highly unstable and require surgical intervention. Soft and small bones with poor pull-out strengths make instrumentation and manipulation difficult. CASE REPORT: We report an 18-month-old child with such a fracture where minimal traction made C1-2 dysjunction apparent with neurological worsening. The C1-2 facets were fixed with a short plate and facetal screws. The child had a good outcome. CONCLUSION: Traction should be applied cautiously to avoid distraction injuries. Careful intraoperative manipulation should be planned to avoid any pull outs/fractures while realigning the spine and fixing it.

Intraoperative consultation in the diagnosis of posterior fossa brain tumors following the 2016 WHO update.

OBJECTIVE: Intraoperative crush/squash smear or frozen section consultation is routinely performed at several centers and offers rapid onsite assessment of tumor type and provides invaluable information to the neurosurgeons. The WHO classification of central nervous system neoplasms underwent a paradigm shift in 2016 with the incorporation of molecular data with the morphological features, such that several new entities came to be distinctly defined. With this paper, we present our experience at intraoperative consultation of brain tumors arising in posterior fossa and aim to apprise the pathologists with the spectrum of cytomorphologic appearances that can occur during such consultation and highlight the diagnostic dilemmas and pitfalls encountered in this setting. METHODS: This is a retrospective observational study illustrating the salient morphological features of commonly encountered brain tumors arising in the posterior fossa (prototype example of each type) reported at our institute over a period of seven years. Both squash smears and rapid snap frozen section were prepared and stained with Toluidine blue and rapid hematoxylin and eosin (H&E) stains. RESULTS AND CONCLUSIONS: While the majority of tumors at this location comprise of pilocytic astrocytoma, ependymoma, and medulloblastoma, some rare examples may also arise; common differentials must be considered and prudently excluded to arrive at the diagnosis which is crucial in guiding the neurosurgeon. Both squash smears and rapid frozen section should be prepared and complement each other for rapid on-site evaluation.

The collateral fallout of COVID19 lockdown on patients with head injury from north-west India.

BACKGROUND: The COVID19 lockdown has altered the dynamics of living. Its collateral fallout on head injury care has not been studied in detail, especially from low- and middle-income countries, possibly overwhelmed more than developed nations. Here, we analyze the effects of COVID19 restrictions on head injury patients in a high-volume Indian referral trauma center. METHODS: From the prospective trauma registry, clinico-epidemiological and radiological parameters of patients managed during 190 days before and 190 days during COVID19 phases were studied. As an indicator of care, the inpatient mortality of patients with severe HI was also compared with appropriate statistical analyses. RESULTS: Of the total 3372 patients, there were 83 head injury admissions per week before COVID19 restrictions, which decreased to 33 every week (60% drop) during the lock phases and stabilized at 46 per week during the unlock phases. COVID19 restrictions caused a significant increase in the proportion of patients arriving directly without resuscitation at peripheral centers and later than 6 h of injury. Though the most common mechanism was vehicular, a relative increase in the proportion of assaults was noted during COVID19. There was no change in the distribution of mild, moderate, and severe injuries. Despite a decrease in the percentage of patients with systemic illnesses, severe head injury mortality was significantly more during the lock phases than before COVID19 (59% vs. 47%, p = 0.02). CONCLUSIONS: COVID19 restrictions have amplified the already delayed admission among patients of head injury from north-west India. The severe head injury mortality was significantly greater during lock phases than before COVID19, highlighting the collateral fallout of lockdown. Pandemic control measures in the future should not ignore the concerns of trauma emergency care.

Head injury care in a low- and middle-income country tertiary trauma center: epidemiology, systemic lacunae, and possible leads.

BACKGROUND: Although head injury (HI) from low- and middle-income countries (LMIC) heavily contributes to the global disease burden, studies are disproportionately less from this part of the world. Knowing the different epidemiological characteristics from high-income nations can target appropriate prevention strategies. This study aims to provide a comprehensive overview of the clinico-epidemiological data of HI patients, focusing on the existing challenges with possible solutions from a developing nation's perspective. METHODS: This is a prospective, registry-based, observational study of HI in an Indian tertiary trauma-care center over 4 years. Various clinico-epidemiological parameters, risk factors, and imaging spectrum were analyzed in a multivariate model to identify the challenges faced by LMIC and discuss pragmatic solutions. RESULTS: The study included a large-volume cohort of 14,888 patients. Notably, half of these patients belonged to mild HI, despite most were referred (90.3%) cases. Only one-third (30.8%) had severe HI. Less than a third reached us within 6 h of injury. Road traffic accidents (RTA) accounted for most injuries (61.1%), especially in the young (70.9%). Higher age, males, RTA, helmet non-usage, drunken driving, systemic injuries, and specific imaging features had an independent association with injury severity. CONCLUSIONS: The study represents the much-needed, large-volume, epidemiological profile of HI from an LMIC, highlighting the suboptimal utilization of peripheral healthcare systems. Strengthening and integrating these facilities with the tertiary centers in a hub and enhanced spoke model, task sharing design, and efficient back-referrals promise effective neurotrauma care while avoiding overburden in the tertiary centers. Better implementation of road safety laws also has the potential to reduce the burden of HI.

Pineal Region Meningioma in a Very Young Child.

INTRODUCTION: Meningiomas in childhood are infrequently seen. Furthermore, they are extremely rare at a pineal location with few reported cases above 5-years of age. We present a very young child with a large pineal region meningioma which mimicked the usually occurring pathologies at this site. CASE PRESENTATION: A 2.5-year-old child presented with symptoms of raised intracranial pressure. MRI demonstrated a homogenously enhancing pineal lesion with small cystic areas. After an initial cerebrospinal fluid evaluation for germ cell tumors, the child underwent excision of the lesion by the Krause approach. The tumor showed no definitive dural attachment, had well-defined arachnoid interface, and was completely excised. The final histopathology was meningioma. CONCLUSION: Although unusual, we highlight the importance of considering meningiomas among the childhood pineal region lesions, given their good outcome with total resection. Also, pertinent brief literature of the pediatric pineal region meningiomas has been provided. An assessment of preoperative and intraoperative features (clear arachnoid plane) along with adjuncts such as frozen studies can help discern various entities of this region, and decide the extent of excision.

Clinicopathological characteristics and outcomes in embryonal tumor with multilayered rosettes: A decade long experience from a tertiary care centre in North India.

BACKGROUND: Embryonal tumor with multilayered rosettes (ETMR) are a heterogenous group clinically, pathologically and topographically. Due to limited cases, data regarding its molecular genetics, pathology and prognostic factors is evolving. We retrospectively analysed our cohort of ETMR over last decade in order to study their clinicopathological characteristics and outcome. METHODS: Our cohort consisted of patients diagnosed with Embryonal tumor with abundant neuropil and true rosettes (ETANTR)/Ependymoblastoma (EBL)/ Medulloepithelioma (MEPL) over the past decade. Clinical details, including outcome and imaging data was retrieved. Histological analysis including immunohistochemical work-up was performed. RESULTS: Cohort included 15 patients with age range between 1 and 28 years and M:F ratio of 1.5:1. Supratentorial location predominated in comparison to tumors arising in posterior fossa. ETANTR and EBL patterns were equally distributed (40% each), followed by one case each of mixed pattern (EBL + ETANTR), MEPL and embryonal tumor, unclassified. All tumors readily expressed LIN 28A and INI-1 was retained. Recurrence with evidence of glial and rhabdoid differentiation was noted in a single patient 9 months following resection. Follow-up period ranged from 1 to 31 months, with overall median survival of 6.4 months. Eight patients were planned for adjuvant treatment following surgery, of which only four could complete it. All patients, except for one, succumbed to the disease. CONCLUSIONS: ETMR have a heterogenous morphology and gathers ETANTR, EBL, MEPL within its spectrum. Following treatment, the recurrent tumor may feature glial/rhabdoid differentiation. LIN28A is expressed in all cases, however should be interpreted in context of histology. Prognosis of ETMR remains dismal despite multimodal therapy.

Customising the surgical management for intraventricular meningiomas - 'one size doesn't fit all'.

BACKGROUND: Intraventricular meningiomas are uncommon and gross total resection is the recommended treatment. However, total resection may not always be possible, especially in locations in which the lesions are adherent to veins and neural structures. We share our experience with intraventricular meningiomas, focusing on the management strategies and outcomes. PATIENTS AND METHODS: We describe the data of 7 patients with intraventricular meningiomas operated at our institute over the last 9 years. Three patients had a third ventricular tumor of which two had lesions straddling across the foramen of Monro. The remaining 4 patients had trigonal mass. The clinico-radiological features, management strategies and outcomes have been elaborated with a mean follow-up of 57 months. RESULTS: The common clinical presentations were raised intracranial pressure symptoms, visual field defects and memory deficits. One patient had multiple meningiomas. Total excision was achieved in all except in 2 patients in whom the lesion straddled across the foramen of Monro with dense adhesions to veins and neural structures. Staged resection was required in one patient with a large trigonal mass. All patients had a low-grade lesion. The tumor recurred in one patient (post-pregnancy) after partial resection. All the patients improved neurologically, and none had added deficits. CONCLUSIONS: Gross total resection of intraventricular meningiomas although desirable may not be possible in certain cases in which the risks outweigh the benefits. These tumors often are of low histological grade and the treatment strategies should be individualized. Regular follow-up is warranted as these tumors may recur despite a low histological grade.

COVID-19: changing patterns among neurosurgical patients from North India, efficacy of repeat testing, and inpatient prevalence.

OBJECTIVE: COVID-19 has affected surgical practice globally. Treating neurosurgical patients with the restrictions imposed by the pandemic is challenging in institutions with shared patient areas. The present study was performed to assess the changing patterns of neurosurgical cases, the efficacy of repeated testing before surgery, and the prevalence of COVID-19 in asymptomatic neurosurgical inpatients. METHODS: Cases of non-trauma-related neurosurgical patients treated at the Postgraduate Institute of Medical Education and Research (PGIMER) before and during the COVID-19 pandemic were reviewed. During the pandemic, all patients underwent a nasopharyngeal swab reverse transcription-polymerase chain reaction test to detect COVID-19 at admission. Patients who needed immediate intervention were surgically treated following a single COVID-19 test, while stable patients who initially tested negative for COVID-19 were subjected to repeated testing at least 5 days after the first test and within 48 hours prior to the planned surgery. The COVID-19 positivity rate was compared with the local period prevalence. The number of patients who tested positive at the second test, following a negative first test, was used to determine the probable number of people who could have become infected during the surgical procedure without second testing. RESULTS: Of the total 1769 non-trauma-related neurosurgical patients included in this study, a mean of 337.2 patients underwent surgery per month before COVID-19, while a mean of 184.2 patients (54.6% of pre-COVID-19 capacity) underwent surgery per month during the pandemic period, when COVID-19 cases were on the rise in India. There was a significant increase in the proportion of patients undergoing surgery for a ruptured aneurysm, stroke, hydrocephalus, and cerebellar tumors, while the number of patients seeking surgery for chronic benign diseases declined. At the first COVID-19 test, 4 patients (0.48%) tested were found to have the disease, a proportion 3.7 times greater than that found in the local community. An additional 5 patients tested positive at the time of the second COVID-19 test, resulting in an overall inpatient period prevalence of 1%, in contrast to a 0.2% national cumulative caseload. It is possible that COVID-19 was prevented in approximately 67.4 people every month by using double testing. CONCLUSIONS: COVID-19 has changed the pattern of neurosurgical procedures, with acute cases dominating the practice. Despite the fact that the pandemic has not yet reached its peak in India, COVID-19 has been detected 3.7 times more often in asymptomatic neurosurgical inpatients than in the local community, even with single testing. Double testing displays an incremental value by disclosing COVID-19 overall in 1 in 100 inpatients and thus averting its spread through neurosurgical services.

Pineal Atypical Teratoid Rhabdoid Tumor in a 5-Month-Old Child.

INTRODUCTION: Atypical teratoid rhabdoid tumors (ATRT), an uncommon malignant intracranial tumor with aggressive behavior are mostly seen in posterior fossa in young pediatric age-group. CASE PRESENTATION: We present an infrequent location of this tumor in the pineal region in a 5-month-old infant. Also, the lesion was non-enhancing which was highly atypical of an ATRT. It was near-totally excised with the child placed in sitting position. However, within a short interval, a tumor recurrence was noted. CONCLUSION: The case possibly represents an extended spectrum of congenital childhood brain tumors. Importantly, it highlights an atypical imaging of ATRT in very young children.