Encouraging Experience with Image-Guided Pencil Beam Scanning Proton Therapy in Craniopharyngioma-First Case Series From India.

OBJECTIVE: We report our early clinical experience with image-guided, pencil beam scanning proton beam therapy (PBS-PBT) for residual and recurrent craniopharyngioma. METHODS: Between September 2019 and January 2023, 19 consecutive patients with residual or recurrent craniopharyngioma, suitable for radiotherapy and treated with image-guided PBS-PBT were analyzed. We documented detailed dosimetric data, acute toxicities, early outcomes, and imaging response on follow-up magnetic resonance imaging scans. RESULTS: A total of 19 patients (11 males and 8 females) with residual or recurrent craniopharyngioma were treated during the study period. The median age of the cohort was 14 years (range, 3-33 years). The histology of most lesions was the adamantinomatous subtype (95%). The most common clinical presentation (before PBT) and most common endocrine deficit was visual disturbance (79%) and hypocortisolism (74%), respectively. Of the 19 patients, 13 had recurrent craniopharyngioma, and 5 had undergone radiotherapy previously. Five patients (26%) had undergone surgery ≥3 times before proton therapy. The median dose delivered was 54 GyE. The most common acute toxicity was grade 1 alopecia (63%). No patient experienced grade ≥3 acute toxicity. With a median follow-up of 18 months (range, 3-40 months), 12 patients showed shrinkage of the residual tumor and/or cyst, and 4 showed a dramatic cyst reduction at 3-9 months of follow-up. Two patients experienced a reduction in both solid and cystic components, with the remaining experiencing a reduction in the cystic component only. The remaining 8 patients had stable disease on magnetic resonance imaging, with 100% disease control and overall survival. Visual function remained stable after treatment. CONCLUSIONS: Our preliminary experience with modern PBS-PBT and image guidance for craniopharyngioma is encouraging. Proton therapy in our cohort was well tolerated, resulting in limited toxicity and promising early outcomes.

Rare Case of Extracranial Metastases in a Patient with IDH-Mutant Glioblastoma.

Rishan Thimma SudarsanGlioblastoma are known for its aggressive intracranial course of disease, where the overall survival is less than 18 months. Of late, the World Health Organization has reclassified and renamed secondary glioblastomas as isocitrate dehydrogenase (IDH)-mutant grade 4 astrocytomas, which is relatively better than its IDH wild-type counterpart; however, overall survival remains poor. In such tumors, metastases outside the craniospinal neuraxis is very rare, and does sometimes present with symptoms which create a diagnostic dilemma and arriving at such diagnosis is still challenging even for the best of the clinicians worldwide. Here we present such a rare case scenario, where a grade 4 astrocytoma that has transformed from a low-grade glioma, presenting with bone metastases, its workup, treatment, and various possible mechanisms underlying such a rare event, and the need of such clinical scenario especially long-term survivors to be wary of distant metastases.

Successful Implementation of Image-Guided Pencil-Beam Scanning Proton Therapy in Medulloblastomas.

Medulloblastoma is the most common malignant brain tumour in children, while much rarer in adults. Although the prognosis and outcomes have greatly improved in the era of modern multidisciplinary management, long-term treatment-induced toxicities are common. Craniospinal irradiation followed by a boost to the primary and metastatic tumour sites forms the backbone of treatment. Proton therapy has been endorsed over conventional photon-based radiotherapy due to its superior dosimetric advantages and subsequently lower incidence and severity of toxicities. We report here our experience from South-East Asia's first proton therapy centre of treating 40 patients with medulloblastoma (38 children and adolescents, 2 adults) who received image-guided, intensity-modulated proton therapy with pencil-beam scanning between 2019 and 2023, with a focus on dosimetry, acute toxicities, and early survival outcomes. All patients could complete the planned course of proton therapy, with mostly mild acute toxicities that were manageable on an outpatient basis. Haematological toxicity was not dose-limiting and did not prolong the overall treatment time. Preliminary data on early outcomes including overall survival and disease-free survival are encouraging, although a longer follow-up and data on long-term toxicities are needed.

Amide proton transfer imaging-arterial spin labeling mismatch: a new imaging biomarker for pilocytic astrocytoma.

We describe the potential utility of Amide Proton Transfer weighted (APTw) Magnetic Resonance Imaging and arterial spin labeling (ASL) in characterizing pilocytic astrocytoma (PA), a type of brain tumor that can be challenging to accurately diagnose and treat. The study included 50 patients with solid or predominantly solid intra-cranial and intra-axial tumors, with 25 patients diagnosed with PA and 25 patients diagnosed with other types of tumors. The study found that the APTw imaging-arterial spin labeling (ASL) mismatch is a new imaging biomarker that could be used to differentiate PA from other types of tumors with a high degree of sensitivity and specificity. The results suggest that APTw imaging and ASL may be useful in characterizing PA, potentially improving diagnosis and treatment planning for this type of brain tumor.

Metabolically Active Subacute Infarct Masquerading as Metastasis: A Finding not to be overlooked in Asymptomatic Oncology Patients.

A 50-year-old man with carcinoma of the right buccal mucosa underwent staging whole-body 18F-fluorodeoxyglucose positron emission tomography-computed tomography, which revealed a hypermetabolic heterogeneously enhancing lobulated primary lesion in the right buccal region and an incidental finding of subacute stroke. The case highlights the importance of discriminating brain neoplasms mimicking stroke from true ischemic stroke, which is crucial for appropriate management of patients in an oncology setting.

Cardiac Tuberculosis on 18 F-FDG PET Imaging-A Great Masquerader of Cardiac Sarcoidosis.

A young gentleman with suspected cardiac sarcoidosis and LV dysfunction whose CMR revealed multifocal subepicardial to mid myocardial linear enhancement in the left ventricular myocardium underwent cardiac 18F-FDG PET imaging. The images revealed patchy regions of increased FDG uptake involving the apical to mid anterolateral, mid to basal anteroseptal/ right ventricular and mildly increased FDG uptake in apical inferior segments of the LV myocardium concordant with CMR findings. Whole body PET CT imaging showed multiple hypermetabolic supra and infra diaphragmatic lymphadenopathy, with no pulmonary lesion identified. Biopsy from the left para aortic lymph node revealed necrotizing granulomatous inflammation consistent with tuberculosis. Based on the histopathological findings of the lymph nodes, diagnosis of cardiac tuberculosis was made, given the similar imaging appearances in both sarcoidosis and TB. This case highlights that cardiac TB although rare, should be included in the differential diagnosis in patients with suspected infiltrative cardiomyopathy, particularly in TB endemic regions.

Volumetric T2-weighted MRI improves the diagnostic accuracy of spinal vascular malformations: comparative analysis with a conventional MR study.

BACKGROUND: To date, very little study of the importance of a volumetric T2-weighted MR sequence in the evaluation of spinal vascular malformations (SVMs) has been carried out. OBJECTIVE: To determine the utility and accuracy of a volumetric T2 MR sequence compared with conventional T2 in the diagnosis of SVMs. METHODS: Retrospective analysis of all patients who underwent spinal DSA for suspected SVMs was conducted. Conventional T2 and volumetric T2 MR images were analysed for the presence of flow voids and parenchymal changes, and SVMs were characterized. The sensitivity, specificity, and overall diagnostic accuracy of these MRI diagnoses were calculated. RESULTS: Of 89 subjects included in the final analysis, 70 patients had angiographically proved SVMs (38 patients with spinal cord arteriovenous malformations [SCAVM-intramedullary or perimedullary] and 32 cases of spinal dural arteriovenous fistula (SDAVF)) and the remaining 19 subjects were normal. The sensitivity and specificity for identification of SVMs were 98.1% and 90% for volumetric T2 sequences, compared with 82.8% and 89.4% for conventional T2 MRI, respectively. For characterization of spinal vascular lesions, volumetric MRI showed high sensitivity, specificity, and accuracy for SDAVF (100%, 90%, 97%, respectively) compared with conventional T2 MRI (71.8%, 89%, 79%, respectively). The positive likelihood ratio was high and negative likelihood ratio was zero for volumetric MRI evaluation of SDAVF, while these ratios were comparable between the two sequences for SCAVM. CONCLUSION: Volumetric T2 MRI is highly sensitive for the detection of SVMs, especially for SDAVF. Volumetric T2 MRI could be introduced into routine clinical practice in the screening of suspected SVMs.

A Case of Pleuroparenchymal Metastasis: Rare Aetiology.

A phyllodes tumour is a malignancy of both mesenchymal and epithelial origin affecting the breast. The malignant course of this breast tumour causing lung metastasis is rare. Here we report a treated case of borderline phyllodes tumour that presented with pleuroparenchymal metastasis. Our case highlights the possibility of recurrence of borderline phyllodes tumour as pleuroparenchymal metastasis even after a long disease free interval.

Ruptured concomitant dermoid cysts of conus medullaris and cervico medullary junction.

Cranio spinal dermoid tumors are rare, benign, slow growing congenital, cystic uni or multi locular tumors, which arise from the inclusion of epithelial tissue within the neural grove during embryonic development.[1] In this case report, we present an uncommon presentation, of a case of concomitant dermoid cysts at conus medullaris and cervico medullary junction that ruptured into the central canal.