B. D. Radotra

Papillary glioneuronal tumor: a new entity awaiting inclusion in WHO classification

Papillary glioneuronal tumor (PGNT) is a recently described lesion of the brain, which is still not included as a separate entity in WHO classification. To date 32 cases of PGNT have been reported in the world literature. We report the 33rd case, a 41-year-old male who presented with pain in the nape of the neck. MRI showed a large, predominantly solid mass involving the cerebral parenchyma of the left temporal and parieto-occipital lobes with extension across the midline. Histologically, it was a mixture of glial and neuronal components. Architecturally, the tumor was notable for its pseudopapillary pattern with hyalinized vessels. PGNT is considered as a low grade neoplasm and surgical excision has been curative in most of the cases. More cases of PGNT need to be reported as they may add further knowledge about its biologic behavior and allow its recognition and classification.

Spontaneous hemorrhages in pediatric supratentorial pilocytic astrocytomas. Malignant presentation of a benign entity.

BackgroundHemorrhage as a presenting feature in pilocytic astrocytoma is an extremely rare phenomenon. When seen in children, most of such tumors exist in the cerebellum. Rarely, a supratentorial pilocytic astrocytoma can present with bleeding.ResultsWe present similar two cases and discuss the pathophysiology of such hemorrhage and histopathological changes in thinned hyalinised vessels of this tumor.ConclusionThe presence of calcifications in the peri-hemorrhagic areas and the presence of mass effect disproportionate to the size of the bleeding are harbingers of the presence of a benign neoplasm that may have bled.

A typical radiological presentation in a case of choroid plexus carcinoma.

Dear Sir, Choroid plexus carcinomas are uncommon intracranial neoplasms accounting for 15-20% of choroid plexus tumors. About 80% of these are found in childhood.[3] The radiology usually shows a brilliantly enhancing heterogenous intraventricular mass invading the ependyma with edema.[9] However, the imaging may be atypical at times.[6] We describe here a case of choroid plexus carcinoma in an infant with atypical radiological features. A 1-year-old male child presented with ataxia and intracranial hypertension since 2 months. The noncontrast computerized tomography (CT) scan showed a heterogenously hyperdense lesion in right posterior fossa region with gross hydrocephalus. The magnetic resonance imaging (MRI) revealed a posterior fossa lesion with the epicenter being located in the 4th ventricle, the presence of a solid and cystic component with extension into the right cerebellar hemisphere. The cystic component was uniformly hyperintense on MRIT1 and T2 sequences, and the fluid attenuation inversion recovery (FLAIR) sequence. The solid component appeared isointense on T1, T2 sequences and had a heterogeneous contrast enhancement [Figure 1]. Although diffusion weighted imaging (DWI) would provide us with added information, this was not available as the patient was referred to us with imaging. With the above image findings, we considered the cyst contents being high in protein or fat contents, and one of the differential diagnoses we kept in mind was an atypical teratoid tumor among other, which include ependymoma with cyst or bleed, ectopic cranipharyngioma, and infected dermoid. At surgery, the cyst had machine oil like fluid, usually characteristic of craniopharyngioma cysts.[1] The solid component of the tumor was soft, friable, and vascular with a clear margin separating the tumor from the brain stem with an absent interface and vermian infiltration at places. We achieved a gross total resection (GTR) and histopathology revealed a choroid plexus carcinoma [Figure 2]. Figure 1 Noncontrast CT scan (First Row) showing heterogenously hypeerdense mass lesion in the posterior fossa compressing the fourth ventricle causing hydrocephalus. MRI showing posterior fossa lesion with cystic component hyperintense on T1, T2, and FLAIR (Second ... Figure 2 Micro-photograph shows solid as well as papillary architecture with nuclear atypia and many atypical mitosis. (H and E, ×20) The radiological imaging in our patient was unique in that the T1 and T2 sequences revealed a hyperintense signal suggestive of methemoglobin, high protein, or fat contents. However, in the absence of a fluid level we ruled out a prior hemorrhagic cyst. Furthermore, the CT scan images showed a slight hyperdensity within the lesion enabling us to rule out a high lipid containing lesion. The overall picture suggested a cystic solid lesion with high protein content. The differential in such cases would be craniopharyngiomas or atypical teratoid tumor and ependymoma with cyst.[1] Rare cases of ectopic fourth ventricular have been reported.[5] Craniopharyngiomas often appear isointense on T1, hyperintense on T2 with ring enhancement on contrast MRIs and, DWI shows no restriction.[1] Atypical teratoid tumor or teratomaand, ependymomas with a cystic component may share features with choroid plexus carcinoma. Atypical teratoid tumors are heterogeneously hypo-to isointense on T1, iso-to hyperintense on T2 with heterogenous contrast enhancement on MRI and shows mild to moderate diffusion restriction on DWI.[4] Ependymoma are heterogeneously hypointense on T1, hyperintense on T2 and FLAIR with homogenous contrast enhancement on MRI. DWI shows no restriction.[4] Our case turned out to be a choroid plexus carcinoma. Tumors arising from the choroid plexus account for 0.4-0.6% of all intracranial tumors. Among these, 20-40% are choroid plexus carcinoma and 70% of them occur in younger than 2 years of age.[3] These lesions on MRI usually appear as contrast enhancing heterogenous large intraventricular mass that invades the ependyma or surrounding parenchyma with vasogenic edema. Usually they are isointense on T1 with areas of increased signal intensity due to hemorrhage, heterogenous hyperintense on T2 and homogenously enhancing on contrast MRI. On FLAIR sequence, they appear heterogenous with surrounding edema. DWI show no restriction.[4] Heterogeneity is likely to be due to cysts, hemorrhage, necrosis, and calcification.[6,9] Horska et al. studied MR Spectroscopy of childhood intraventricular lesions.[2] Both Choroid plexus carcinoma and papilloma was characterized by high levels of choline-containing compounds and a complete absence of creatine and the neuronal/axonal marker N-acetyl aspartate. However, the Choroid plexus carcinoma showed higher levels of choline compared with the choroid plexus papilloma, and it also had elevated lactate.[2] MRS was not performed in our patient. MRS may provide additional diagnostic information but is neither sensitive nor specific in these cases. Treatment strategies include surgical resection followed by radiotherapy and/or chemotherapy depending on the extent of resection achieved. GTR of choroid plexus carcinoma increases the overall survival as well as progression free survival and is recommended if it can be safely performed. Five-year survivals are 58.1 ± 6.1% and 20.9 ± 5.1% for GTR and STR, respectively.[7] The role of adjuvant chemotherapy and/or radiotherapy in choroid plexus carcinoma is controversial, but increasing evidence suggests that combined radiation and chemotherapy should be given after subtotal resection.[8] Radiation is recommended following GTR in adults but not in children. Neoadjuvant chemotherapy (ifosfamide, carboplatin, and etoposide or ICE) regimen in choroid plexus carcinoma has shown to reduce vascularity, thereby increasing the chances of complete resection.[3] In case of subtotal resection, a reexploration to achieve a GTR may be attempted after completion of chemotherapy. This combined approach increases the survival rate.

Survival and failure patterns in atypical and anaplastic meningiomas: A single-center experience of surgery and postoperative radiotherapy

INTRODUCTION Intracranial meningiomas are the second most common tumor of the CNS. The high-grade tumors are atypical and malignant meningioma comprising 5-7% and 1-3% of all meningiomas. The high-grade meningioma have an aggressive histopathological and clinical behavior. MATERIALS AND METHODS We retrospectively reviewed 37 patients of high-grade meningioma treated in our institute from 2002 to 2011. Clinical characteristics and treatment modality in form of surgery and radiotherapy (RT) were noted. Statistical analysis was done with regards to progression-free survival (PFS) and overall survival (OS) using Kaplan-Meier survival analysis. RESULTS The median age of the patients was 45.0 years. The median duration of symptoms was 6 months. Headache was the most common presenting symptom. Fourteen patients underwent complete excision, while 23 had subtotal excision. Twenty-two patients had World Health Organization (WHO) grade II histology tumors and 15 patients had grade III histology. Median RT dose delivered was 50 Gy for grade II tumors and 54 Gy for grade III tumors. Five-year PFS for grade II and III tumors was 58 and 20%, respectively. Five-year OS for grade II and III tumors was 83 and 23%, respectively. CONCLUSIONS High-grade meningiomas represent a rare and aggressive neoplasm. The mainstay of therapy is gross total resection (GTR) at the initial surgery. Postoperative adjuvant RT should be offered to all patients, regardless of the degree of resection achieved. Long-term follow-up is important as local recurrences and progression can develop years after the initial treatment.

Malignant cerebellar peduncle lesions - rapid progression and poor outcome.

Background: Tumors arising from cerebellar peduncle are extremely rare and behave aggressively. The inclusion of these into either cerebellar or brainstem gliomas is contentious. Case Description: We performed clinicopathological review of three patients treated at our institute and surveyed the literature for previous such reported cases. Mean duration of symptoms in our patients was 2 weeks. Subtotal tumor resection was performed in two patients while the third underwent stereotactic biopsy followed by chemoradiotherapy. Histopathology revealed glioblastoma in initial two patients and medulloblastoma Grade IV in the third. The two patients who underwent surgical excision succumbed to the illness within 2 days and a month, respectively. Conclusion: Malignant cerebellar peduncular lesions have poor overall survival despite surgical debulking. It is not confirmed whether these tumors should be considered as cerebellar lesions or brainstem gliomas due to aggressive clinical behavior, and so the ideal line of management is not yet known.

Long term outcome analysis of role of radiotherapy in Grade I meningiomas: A single centre experience from North India.

Background: Intracranial meningiomas are the second most common tumor of the central nervous system. Grade I tumors are the most common variety of meningioma and have a benign course. Surgery is the mainstay of treatment. Radiotherapy (RT) decreases the local recurrence rates and progression in patients with subtotal excision (STE). The authors present our institutes experience in combined modality management of 18 successive patients of Grade I meningioma. Materials and Methods: We retrospectively reviewed 18 patients of Grade I meningioma treated in our institute from 2003 to 2011. Clinical characteristics and treatment modality in form of surgery and RT were noted. Statistical analysis was done with regards to recurrence free survival and overall survival using Kaplan–Meier survival analysis. Results: The median age of the patients was 52.5 years. Seven patients were males and 11 patients were females. The median duration of symptoms was 8 months. Headache was the most common presenting symptom followed by vomiting, seizures, motor weakness and visual deficits. Five patients underwent complete excision while 13 had STE. 11 patients received early RT while 5 patients received RT at recurrence. Median RT dose delivered was 50 Gy. RT had significant effect on local control especially in subtotal resections, with overall 93.75% local control rates. Conclusions: Grade I meningiomas represent a benign neoplasm. The mainstay of therapy is gross total resection at the initial surgery. Postoperative adjuvant RT should be offered to patients with subtotal resection. Long-term follow-up is important as local recurrences and progression can develop years after the initial treatment.

Ruptured venous aneurysm of cervicomedullary junction

Background: Ruptured venous aneurysm is often seen with arterio-venous malformation (AVM) or developmental venous anomaly (DVA). However, isolated venous aneurysm is unusual. Case Description: We present a case of ruptured venous aneurysm that presented with subarachnoid hemorrhage (SAH) and intraventricular hemorrhage (IVH). Digital substraction angiography (DSA) revealed a saccular contrast filling pouch in the left lateral aspect of cervicomedullary junction (CMJ). Endovascular intervention was not a viable option. During surgery, a saccular pliable structure approx. 1.5 × 1 cm was found in the subarachnoid space that was clipped and excised. There were no arterial feeders, no evidence of surrounding AVM, and no dilated perimedullary vein. Conclusion: This is perhaps the first reported case of ruptured venous aneurysm (without associated AVM) of CMJ, which was successfully managed surgically. The possible etiologies remain an unnoticed head trauma or a congenital vessel wall abnormality. Surgically clipping and excision remains the treatment of choice for such lesion.

Solitary plasmacytoma of temporal bone: A rare case report

Solitary plasmacytoma of base of skull is a rare disease. It presents with headache, swelling, and other vague symptoms. Systemic work-up to rule out multiple myeloma is necessary. Radiotherapy is the treatment of choice. We, here, present a case of solitary plasmacytoma of base of skull in right temporal bone in a 50-year-old female treated with surgery and radiotherapy.

Revisiting the metastatic potential of childhood pineal parenchymal tumor of intermediate differentiation: a case report.

Dear Editor: Pineal parenchymal tumor of intermediate differentiation (PPTID) is a rare entity, the biological behavior of which is largely unknown due to few reported cases overall and diagnostic pitfalls in the small sample [1, 2]. The extent of surgery as well as the role of adjuvant chemoradiotherapy in PPTID is still contentious. Dissemination into spinal axis, a known phenomenon for childhood pineoblastoma (PB), has been reported rarely in PPTID [2–4] and embarks upon redefining the treatment paradigms for these tumors. Simultaneous intracranial as well as spinal dissemination at presentation in childhood PPTID is a rarity indeed and provides new insights to the varied biology of this potentially malignant tumor. A 10-year-old male presented with history of headache and gait disturbance for 2 months. Examination revealed papilloedema, upgaze palsy, gait ataxia and spasticity in both lower limbs. Magnetic resonance imaging revealed a well defined lesion at pineal region that seemed hypointense on T1W, hyperintense on T2W and showed dense enhancement on gadolinium. Associated non communicating hydrocephalus was remarkable. Another non-contiguous, small, well-defined enhancing lesion was seen juxta third ventricle (Fig. 1a–d), while spinal imaging was suggestive of an intradural focal lesion at the D6 level causing cord compression (Fig. 1e). The child underwent endoscopic biopsy and third ventriculostomy. Cerebrospinal fluid analysis for alpha fetoprotein and beta HCG was negative. Histopathology showed a moderately cellular tumor with the tumor cells arranged in lobules and sheets with high N:C ratio and vesicular chromatin. Mitotic activity was 3–4/10 high-power fields. There was no necrosis or rosette formation. The tumor cells showed strong and diffuse synaptophysin positivity and focal patchy positivity for neurofilament protein (NFP) while being negative for cytokeratin and CD117. Ki-67 labeling index (LI) was 18 % (Fig. 2a–e). Overall features were suggestive of WHO grade III PPTID. Two weeks later, the child underwent D6 laminectomy and excision of the intradural lesion. Histopathology suggested metastasis with tumor cells showing similar morphology. PPTID exists as a spectrum with varied proliferative potential of the tumor cells rather than a single entity. The classification of these tumors into grade II and grade III is based on the number of mitoses and positivity for NFP (less than 6 mitoses/10 high power fields with positivity for NFP as grade II and absent or minimal positivity for NFP as grade III) [1]. While surgery remains the treatment of choice for pineocytoma (PC), the management protocol in PPTID is still contentious [1, 2] with no established benefit of adjuvant chemoradiotherapy. Dissemination along the spinal axis, a known phenomenon in pineal germ cell tumors and PB, has been rarely reported in PPTID [2–4] while we observed * Ankur Kapoor dr.ankurkapoor@gmail.com

A case of cerebral pheohyphomycosis in an immunocompetent patient: Emphasis on intraoperative findings

A 42-year-old male, cook by profession and apparently immunocompetent presented with headache and altered sensorium of 2 weeks duration. Examination was unremarkable except for bilateral mild papilledema. Magnetic resonance imaging (MRI) showed a T1-weighted hypointense lesion in right parietal region. The lesion was intensively contrast enhancing with multiple small lesions surrounding the main lesion [Figure 1]. It was hyperintense on T2-weighted sequence and there was restriction on diffusion-weighted imaging (DWI) [Figure 1]. In view of altered sensorium he was taken up for surgery with a preoperative diagnosis of high grade malignancy. During surgery, aspiration of the lesion showed pus. The abscess wall was removed in total after defining a clear plane of cleavage between brain and abscess. The wall of abscess had multiple blackish spots. The frozen section of abscess wall revealed granulomas and multinucleated giant cells containing pigmented fungi. Loading dose of amphotericin B was given intraoperatively. Biopsy report confirmed the diagnosis of fungal infection, pheohyphomycosis [Figure 2]. Patient has received 1,500 mg. of amphotericin B and is on oral voriconazole, doing well at follow-up.