Background:Olfactory neuroblastoma (ONB) is a rare malignant neuroectodermal tumor of the nasal cavity. Mixed olfactory neuroblastoma which contains areas of divergent differentiation is even rare. Till date, only 4 cases of mixed olfactory neuroblastomas have been reported.Case Description:We report the case of a 46-year-old male who presented with the chief complaints of nasal bleeding and nasal obstruction since 4 months. Radiological imaging was suggestive of a large heterogeneous mass in the left superior nasal cavity with extensions into bilateral maxillary, ethmoidal, and sphenoidal sinuses, as well as into the anterior cranial fossa. Bifrontal osteoplastic craniotomy and excision of the intracranial part of the tumor from above and transnasal endoscopic removal of the mass in the nasal cavities and paranasal sinuses from below was done. Postoperative radiological imaging was suggestive of gross complete excision of the mass. Histopathological diagnosis was “mixed olfactory neuroblastoma-carcinoma (squamous and glandular differentiation) Hyams grade IV.” On immunohistochemistry, the tumor cells were positive for neuron specific enolase (NSE), synaptophysin, chromogranin, and CD56 and peripherally for S100. Because of personal reasons, the patient did not take adjuvant radiotherapy. He presented again after 2 months with a full blown recurrence of esthesioneuroblastoma with similar extensions as before. The patient is now planned for salvage surgery followed by adjuvant chemoradiation.Conclusion:We report the 5th case in the world of mixed olfactory neuroblastoma-carcinoma with squamous and glandular differentiation. From an analysis of the findings in the 5 reported cases of mixed olfactory neuroblastomas, one might infer that a separate subcategory of ONB, i.e., mixed ONB, should be considered because mixed ONBs have an aggressive behavior, high rates of recurrence, and these tumors should be treated aggressively by multimodality treatment.

Phaeohyphomycosis is a collective term used for fungal infections caused by moulds and yeasts that have brown pigmented cell walls due to the presence of melanin. These are also known as dematiaceous fungi. We report this patient who presented with headache, right hemiparesis, slurred speech, and altered sensorium. Patient was a known case of rheumatic valvular heart disease (RVHD) and had undergone balloon valvotomy for mitral stenosis 1 year back. Radiological features were suggestive of high grade glioma. Left fronto-parietal decompressive craniectomy with complete excision of mass lesion was performed. Histopathological examination of the surgical specimen revealed multiple granulomas with giant cells. These giant cells contained branched septate pigmented fungal hyphae in their cytoplasm. After the histopathology report, patient was started on intravenous amphotericin and was discharged on oral itraconazole 200 mg twice daily. Unfortunately, the patient was non-compliant and stopped taking oral itraconazole after 1 month. He landed up in fulminant fungal meningo-encephalitis and died 10 weeks after the initial diagnosis. We report a rare case of cerebral phaeohyphomycosis in a patient of RVHD which, to our knowledge, is nowhere mentioned in the literature.

Background:Spinal schwannomas are slow growing, benign nerve sheath tumors. These may be asymptomatic or may present as backache with radicular pain, slowly progressive neurological deficits, but rarely with acute spastic quadriparesis attributed to intratumoral hemorrhage.Case Description:A 38-year-old male presented with the chief complaint of neck pain radiating to the left upper extremity for the last 8 months. On admission, he exhibited diffuse hyper-reflexia but had no motor or sensory deficit. Magnetic resonance imaging showed a solid-cystic intradural extramedullary (IDEM) C2 to C4 mass severely compressing the spinal cord. The same day the patient acutely developed a spastic quadriparesis. Immediately, a partial C2, C3, and C4 laminectomy was performed for tumor excision; within 5 postoperative days, he fully regained neurological function. The final histopathology was consistent with a “schwannoma showing areas of congestion and hemorrhage.”Conclusion:Spinal schwannomas rarely present with intratumoral hemorrhage and acute spastic quadriparesis. Immediate operative decompression may lead to excellent postoperative neurological recovery.

A BSTRACT Aims and Objectives: This study aimed to analyze the spectrum and surgical outcome of cases of craniosynostosis operated at a tertiary referral institute in India Design: This was a cross-sectional study. Materials and Methods: We retrospectively examined 60 cases of craniosynostosis operated at our institute from 2008 to 2014 (with a minimum follow-up of 2 years). Data was collected including name, age, gender, involved sutures, other medical conditions, whether syndromic craniosynostosis or not, whether symptoms and signs of intracranial hypertension were present or not, associated findings on magnetic resonance imaging of brain and cervico-medullary junction, type of surgery performed, age at which surgery was performed, perioperative complications (if any), and findings on follow-up. To be able to analyze the surgical results, we used the seven category classification system used by Sloan et al. Results: Craniosynostosis affected more men than women. The incidence of syndromic craniosynostosis was 11.67%. Mean age at first surgery was 3.85 years. Chiari malformation was present in 80% of the Crouzon’s syndrome cases, 62.5% of the oxycephaly cases, and 4.44% of the non-syndromic, non-oxycephaly cases. Intracranial hypertension was present in 80% of the Crouzon’s syndrome cases, 75% of the oxycephaly cases, and 6.67% of the non-syndromic, non-oxycephaly cases. Perioperative complications were present in 42.86% of the syndromic craniosynostosis cases, 50% of the oxycephaly cases, and 15.56% of the non-syndromic, non-oxycephaly cases. Compromised overall correction was present in 4 of 7 cases of syndromic craniosynostosis, 3 of 8 cases of oxycephaly, and 2 of 45 cases of non-syndromic, non-oxycephaly group. Conclusion: The study highlights the importance of educating the masses so that cases of craniosynostosis present early. The incidence of Chiari malformation, intracranial hypertension, and perioperative complications was significantly higher in the syndromic craniosynostosis and oxycephaly groups than in single-suture craniosynostosis. The best surgical outcome and the least perioperative complications were seen in the trigonocephaly group. Compromised overall correction and reoperations were more common in the syndromic and complex craniosynostosis groups than in single-suture craniosynostosis.

Spinal clear cell meningiomas (CCMs) are rare and dural-based lesion usually affecting the younger population. We report the rare case of giant nondural-based spinal CCM mimicking schwannoma and review the literature. A literature search was performed at PubMed and Embase until January 1, 2020. A total of 19 cases of nondural-based spinal CCM was reported. The following relevant data were extracted: authors, publication year, patient and tumor characteristics, treatment, and outcome. The mean age of the presentation was 20.58 years. Twelve (63.16%) were female and seven patients (36.84%) were male. The most common location was lumbosacral region 15 (79%). Fifteen (79%) tumors had cranio-caudal dimension ≤2 vertebral level, and only four (21%) tumors had dimension ≥2 vertebral level. Gross total resection (GTR) was performed in 18 (95%) patients and subtotal resection (STR) in 1 patient. Recurrences were reported in five (26.14%) patients. Four of them showed recurrences within 6 months; earliest at 2.3 months in the patient had undergone STR. Our patient is 19-year-old male diagnosed with a lumbosacral intradural lesion. Craniocaudal dimension is ≥2 vertebral level shows the foraminal extension and vertebral scalloping. GTR is performed. Intraoperatively, the tumor has foraminal extension and shows attachment with right S1S2 nerve root. No dural attachment is found. Six-month follow-up magnetic resonance image shows no evidence of disease. Nondural-based spinal CCMs are extremely rare and should be kept as a differential diagnosis in young patients with giant intradural tumor, and whose radiological features suggesting of schwannoma. It affects young patients and usually involves more than one vertebral level. The chances of recurrences and metastasis are always high even after GTR; hence, close follow-up of the entire neuraxis is warranted.