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1.
J Neurooncol ; 167(1): 189-198, 2024 Mar.
Article in English | MEDLINE | ID: mdl-38265748

ABSTRACT

INTRODUCTION: CDKN2A/B homozygous deletion is one of the defining features of grade 4 in IDH-mutant astrocytic tumours. AIM: To evaluate CDKN2A/B-deletion in IDH-mutant astrocytic tumours and its clinicopathological impact. MATERIALS AND METHODS: CDKN2A/B-deletion was evaluated by Fluorescence in-situ hybridisation (FISH) and interpreted by two recently accepted methods. RESULTS: Eighty-three out of 94 cases (histologically-grade 2: 3, grade 3: 46, grade 4: 34) were interpretable on FISH. Concordant CDKN2A/B-deletion was observed in 71% (27/38) of lower-grade tumours (n = 49) and 90% (27/30) of histological grade 4 tumours (n = 34). Both the interpretation methods showed good agreement (Kappa = 0.75). CDKN2A/B-deletion showed an inverse correlation for < 10% MIB-1 labeling index (p = 0.01) while that by method-2 showed a significant correlation for grade 4 (p = 0.02). No significant correlation was observed for any other clinicopathological parameters. Twenty-four patients showed progression/recurrence (including deaths), and no significant difference in frequency of CDKN2A/B deletion was observed among cases with disease progression across different histological grades. CONCLUSIONS: CDKN2A/B-deletion was observed across all the histological grades of IDH-mutant astrocytic tumours, expectedly more in the higher grade. FISH, as a method, can be used for the detection of CDKN2A/B homozygous deletion, when there is concordant interpretation.


Subject(s)
Astrocytoma , Brain Neoplasms , Humans , Astrocytoma/genetics , Astrocytoma/pathology , Brain Neoplasms/genetics , Brain Neoplasms/pathology , Cyclin-Dependent Kinase Inhibitor p16/genetics , Fluorescence , Homozygote , Isocitrate Dehydrogenase/genetics , Mutation , Sequence Deletion , Cyclin-Dependent Kinase Inhibitor p15/genetics
2.
Strahlenther Onkol ; 198(3): 291-303, 2022 Mar.
Article in English | MEDLINE | ID: mdl-35059761

ABSTRACT

PURPOSE: Imaging features are known to reflect inherent disease biology in various cancers including brain tumors. We report on the prognostic impact of magnetic resonance imaging (MRI) features on survival in patients with medulloblastoma treated between 2007 and 2018 at our institute. METHODS: Sixteen semantic imaging features (with predefined categories) were extracted from pre- and postcontrast T1-weighted and T2-weighted MRI by consensus. Univariate analysis and multivariate Cox regression analysis were performed to assess the correlation of semantic features with relapse-free survival (RFS) and overall survival (OS). RESULTS: The study cohort comprised 171 medulloblastoma patients (median age 9 years) treated with maximal safe resection followed by risk-stratified adjuvant radio(chemo)therapy. A total of 55 patients experienced recurrent/progressive disease (commonly neuraxial metastases) resulting in 44 deaths, including one treatment-related death. At a median follow-up of 45 months (interquartile range 19-65 months), 5­year Kaplan-Meier estimates of RFS and OS were 64% and 71%, respectively. Semantic MRI features such as non-central tumor location on vertical axis, absence of brainstem involvement, ≤ 80% solid tumor area with contrast uptake, heterogenous pattern of contrast enhancement, necrosis, calcification, and T2-weighted heterogeneity were associated with significantly worse RFS and/or OS in univariate analysis. Cox regression analysis identified tumor location on the vertical axis, brainstem involvement, and calcification as independent prognostic factors impacting outcomes. Distinctive MRI features correlated with survival even within individual molecular subgroups of medulloblastoma. CONCLUSION: Distinctive semantic MRI features correlate significantly with survival outcomes in medulloblastoma, also within individual molecular subgroups, reflecting their prognostic impact.


Subject(s)
Cerebellar Neoplasms , Medulloblastoma , Cerebellar Neoplasms/diagnostic imaging , Cerebellar Neoplasms/therapy , Child , Humans , Magnetic Resonance Imaging/methods , Medulloblastoma/diagnostic imaging , Medulloblastoma/therapy , Neoplasm Recurrence, Local , Prognosis , Retrospective Studies , Semantics
3.
J Pediatr Hematol Oncol ; 44(5): 264-267, 2022 07 01.
Article in English | MEDLINE | ID: mdl-35129147

ABSTRACT

Secondary neoplasms (SNs) are being increasingly identified in long-term survivors of childhood cancer. Phyllodes tumor (PT) form a distinctly uncommon SN. We report a series of 6 female childhood cancer survivors who developed PT as SN. The median age at primary diagnosis was 13 years. Their primary tumors were bone sarcoma (4) and acute leukemia (2), and all were treated with chemotherapy, predominantly with alkylating agents and/or anthracyclines. None had received direct radiotherapy to the chest wall. Subsequently, PT were detected after a median interval of 7.5 years, with 2 patients developing bilateral and malignant PT. The series highlights a rare SN in childhood cancer survivors, underscoring the importance of regular long-term follow-up.


Subject(s)
Bone Neoplasms , Breast Neoplasms , Cancer Survivors , Leukemia, Myeloid, Acute , Neoplasms, Second Primary , Phyllodes Tumor , Bone Neoplasms/therapy , Breast Neoplasms/therapy , Child , Female , Humans , Neoplasms, Second Primary/etiology , Phyllodes Tumor/etiology , Retrospective Studies , Tertiary Healthcare
4.
Eur Arch Otorhinolaryngol ; 279(5): 2591-2598, 2022 May.
Article in English | MEDLINE | ID: mdl-34410472

ABSTRACT

BACKGROUND: Endolymphatic sac tumour (ELST) is a rare low-grade locally aggressive neoplasm arising from the endolymphatic duct or sac. It presents mostly with vestibulo-cochlear symptoms either sporadically or as part of von Hippel-Lindau (VHL) syndrome. Micro-neurosurgical excision remains the cornerstone of therapy with the role of radiotherapy (RT) being controversial. This is a clinico-pathological analysis of consecutive ELST patients presenting to a single-institution in India. METHODS: Neuropathology database of a tertiary-care comprehensive cancer centre was searched electronically to identify consecutive patients with histopathological diagnosis of ELST registered at the institute over last one decade. Data regarding demographic profile, clinical presentation, histopathological features, treatment details and outcomes were retrieved from electronic medical records for this retrospective analysis. RESULTS: Electronic search identified seven unique patients with biopsy-proven ELST registered at the institute between 2009 and 2020. Median age of the study cohort was 39 years (range 24-65 years) with strong male predilection (5:2 ratio) and left-sided preponderance (71%). Most common presenting symptoms were hearing loss (86%) and earache (71%) on affected side followed by headache (43%). All patients underwent maximal safe resection at initial diagnosis and were followed-up closely with periodic surveillance imaging. Two patients underwent salvage RT using high-precision conformal techniques at recurrence/progression. CONCLUSION: ELST is a rare low-grade locally aggressive neoplasm that arises generally as part of VHL syndrome or sometimes sporadically. Gross total resection provides the best chance of cure with RT being reserved for unresectable disease, large residue, medical inoperability, or as salvage therapy for recurrent/progressive tumor.


Subject(s)
Adenoma , Bone Neoplasms , Ear Neoplasms , Endolymphatic Sac , Labyrinth Diseases , von Hippel-Lindau Disease , Adenoma/pathology , Adult , Aged , Bone Neoplasms/pathology , Ear Neoplasms/diagnosis , Ear Neoplasms/pathology , Ear Neoplasms/surgery , Endolymphatic Sac/pathology , Endolymphatic Sac/surgery , Humans , Male , Middle Aged , Retrospective Studies , Young Adult , von Hippel-Lindau Disease/complications , von Hippel-Lindau Disease/diagnosis , von Hippel-Lindau Disease/pathology
5.
Pediatr Blood Cancer ; 68(5): e28925, 2021 05.
Article in English | MEDLINE | ID: mdl-33533557

ABSTRACT

PURPOSE: To assess the safety and efficacy of concurrent carboplatin during craniospinal irradiation (CSI) in high-risk/metastatic medulloblastoma defined as either residual tumor >1.5 cm2 or leptomeningeal metastases. METHODS: This single-arm combined prospective (2005-2011) and retrospective (2011-2019) study was undertaken at a tertiary care cancer center in India. Following surgery, patients with newly diagnosed high-risk/metastatic medulloblastoma received concurrent carboplatin (35 mg/m2 ) for 15 days (day 1 to day 15) during CSI plus posterior fossa/tumor bed boost, followed by six cycles of standard adjuvant chemotherapy. RESULTS: All 97 patients completed their planned course of radiotherapy without interruptions, except for two (2.1%) patients who had brief gaps due to treatment-related toxicity. Grade 3-4 anemia, neutropenia, thrombocytopenia, and febrile neutropenia were seen in four (4.1%), 41 (42.2%) 21 (21.6%), and 18 (18.6%) patients, necessitating packed cell transfusion, granulocyte colony-stimulating factor, and platelet support in five (5.1%), 41 (42.2%), and five (5.1%) patients, respectively, during the concurrent phase. Following myelorecovery, 92 (94.9%) patients completed the planned six cycles of standard adjuvant systemic chemotherapy. There were no treatment-related deaths during the concurrent chemo-radiotherapy phase, while three (3.1%) toxic deaths were ascribed to adjuvant chemotherapy-related complications. At a median follow-up of 82 months, the 5-year Kaplan-Meier estimates of progression-free survival and overall survival were 60.2% and 62.1%, respectively. On univariate analysis, leptomeningeal metastases (M0/M1 vs. M2/M3) and histological subtype (large cell/anaplastic vs. others) emerged as significant prognostic factors for survival. CONCLUSION: Addition of concurrent carboplatin to RT as radiosensitizing chemotherapy is a simple and effective way of treatment intensification in high-risk/metastatic medulloblastoma.


Subject(s)
Antineoplastic Agents/administration & dosage , Carboplatin/administration & dosage , Cerebellar Neoplasms/therapy , Chemoradiotherapy/methods , Medulloblastoma/therapy , Adolescent , Chemotherapy, Adjuvant/methods , Child , Craniospinal Irradiation/methods , Female , Humans , Male
6.
Ann Diagn Pathol ; 53: 151763, 2021 Aug.
Article in English | MEDLINE | ID: mdl-34111707

ABSTRACT

BACKGROUND: Primary mediastinal germ tumours (PMGCT) constitute, a mere 3-4% of all germ cell tumours (GCT). Although they account for approximately 16% of mediastinal tumours in adults and 19-25% in children as per western literature, there is hardly any large series on PMGCT reported from the Indian subcontinent. DESIGN: We have retrospectively analysed clinicopathological features of 98 cases of PMGCT diagnosed over 10 years (2010-2019) from a tertiary-care oncology centre. RESULTS: The study group (n = 98) comprised predominantly of males (n = 92) (M:F ratio-15:1), with an age range between 3 months to 57 years (median: 25 years). The tumours were predominantly located in the anterior mediastinum (n = 96). Broadly, Non-seminomatous germ cell tumours (NSGCT) were more common (n = 73, 74%) compared to pure seminoma (n = 25, 26%). Mixed NSGCT was the most common histological subtype (n = 30) followed by pure mature teratoma (n = 18), pure Yolk sac tumour (n = 13), mixed seminoma and NSGCT (n = 5), pure immature teratoma (n = 3) and GCT; NOS (n = 4). Interestingly, all female patients had exclusive teratomas. Nine cases revealed secondary somatic malignancy (5 carcinomas and 4 sarcomas). The majority of patients received neoadjuvant chemotherapy (n = 71). Surgical excision was performed in 60 patients. Follow up was available in 68 patients. NSGCT showed a poor prognosis as compared to seminoma (p value = 0.03) and tumours with somatic malignancies had a more aggressive clinical course. CONCLUSION: PMGCT was seen predominantly in young adult males and somatic malignancies were noted in as high as 9% of cases. Patient with somatic malignancy have aggressive clinical course, hence, extensive sampling and careful histopathological evaluation are recommended for the identification and definitive characterization.


Subject(s)
Mediastinal Neoplasms/pathology , Neoplasms, Germ Cell and Embryonal/diagnosis , Seminoma/diagnosis , Testicular Neoplasms/diagnosis , Adolescent , Adult , Child , Child, Preschool , Endodermal Sinus Tumor/diagnosis , Endodermal Sinus Tumor/epidemiology , Endodermal Sinus Tumor/pathology , Humans , India/epidemiology , Infant , Male , Middle Aged , Neoadjuvant Therapy/methods , Neoplasms/epidemiology , Neoplasms, Germ Cell and Embryonal/epidemiology , Neoplasms, Germ Cell and Embryonal/pathology , Neoplasms, Germ Cell and Embryonal/therapy , Oncology Service, Hospital/organization & administration , Prevalence , Prognosis , Retrospective Studies , Seminoma/epidemiology , Seminoma/pathology , Teratoma/diagnosis , Teratoma/epidemiology , Teratoma/pathology , Tertiary Healthcare , Testicular Neoplasms/epidemiology , Testicular Neoplasms/pathology , Young Adult
7.
J Neurooncol ; 147(2): 405-415, 2020 Apr.
Article in English | MEDLINE | ID: mdl-32072441

ABSTRACT

PURPOSE: To report clinical outcomes of salvage re-irradiation (re-RT) in recurrent/progressive ependymoma. METHODS: Medical records of patients treated with curative-intent re-RT as multi-modality management for recurrent/progressive ependymoma were analyzed retrospectively. The linear-quadratic model was used to provide estimates of biologically effective dose (BED) of irradiation using an α/ß value of 2 for late CNS toxicity for each course of irradiation and summated to derive cumulative BED without correcting for the assumed recovery. RESULTS: A total of 55 patients (median age 10 years at index diagnosis) treated with curative-intent re-RT between 2010 and 2018 were included. Median time to first recurrence was 29 months with an inter-quartile range (IQR) of 16-64 months. Majority (n = 46, 84%) of patients underwent surgical re-excision of recurrent disease. Median interval from first course of irradiation (RT1) to second course (RT2) was 35 months (IQR = 26-66 months) with a median re-RT dose of 54 Gy in 30 fractions (range 40-60 Gy), resulting in median cumulative equivalent dose in 2 Gy fraction (EQD2) of 106.2 Gy (range 92.4-117.6 Gy). Volume of re-RT was based on location and pattern of relapse, comprising uni-focal (n = 49, 89%), multi-focal (n = 3, 5.5%), or craniospinal irradiation (CSI) in 3 (5.5%) patients respectively. Thirty-six (66%) patients received platinum-based salvage chemotherapy either before or after RT2. At a median follow up of 37 months (range 6-80 months), the Kaplan-Meier estimates of 3-year progression-free survival (PFS) and overall survival (OS) for the entire study cohort were 40% and 51% respectively. Gross total resection at recurrence; early salvage re-RT (prior to chemotherapy, if any); and longer (> 2 years) disease-free interval (DFI) were associated with better survival outcomes. Salvage re-RT was generally well tolerated with only 3 (5.5%) patients developing symptomatic radiation necrosis necessitating corticosteroids. CONCLUSION: Extent of re-excision, sequence/timing of re-RT, and DFI impact upon outcomes in curative-intent, multi-modality salvage therapy for recurrent ependymoma.


Subject(s)
Brain Neoplasms/mortality , Craniospinal Irradiation/mortality , Ependymoma/mortality , Neoplasm Recurrence, Local/mortality , Neurosurgical Procedures/mortality , Re-Irradiation/mortality , Salvage Therapy , Adolescent , Adult , Brain Neoplasms/pathology , Brain Neoplasms/radiotherapy , Brain Neoplasms/surgery , Child , Child, Preschool , Combined Modality Therapy , Ependymoma/pathology , Ependymoma/radiotherapy , Ependymoma/surgery , Female , Follow-Up Studies , Humans , Infant , Male , Middle Aged , Neoplasm Recurrence, Local/pathology , Neoplasm Recurrence, Local/radiotherapy , Neoplasm Recurrence, Local/surgery , Prognosis , Retrospective Studies , Survival Rate , Young Adult
8.
J Neurooncol ; 144(2): 283-291, 2019 Sep.
Article in English | MEDLINE | ID: mdl-31236820

ABSTRACT

PURPOSE: To report outcomes of salvage re-irradiation (re-RT) in recurrent/progressive medulloblastoma (MB). METHODS: Medical records of patients treated with curative-intent re-RT as multi-modality management for recurrent/progressive MB between 2008 and 2018 were analyzed retrospectively. RESULTS: A total of 28 patients (median age 18 years at index diagnosis) were included. Molecular subgrouping was done using real-time reverse transcriptase polymerase chain reaction (RT-PCR) based on the differential expression of select set of 12 protein coding genes and 9 microRNAs. Fifteen of 17 (88%) patients with sonic hedgehog (SHH)-MB developed isolated local recurrence within the index tumor-bed, while 5 of 7 (72%) patients with Group 4 MB developed localized relapse outside the posterior fossa. Diffuse neuraxial dissemination was seen in 2 patients with SHH-MB, and one each of Group 4 and wingless (WNT)-MB. Molecular subgrouping was not known in 3 patients. The dose and volume of re-RT was based on site and patterns of relapse, comprising unifocal in 18 (64%), multi-focal in 3 (11%), and repeat craniospinal irradiation (re-CSI) in 7 (25%) patients. Median interval from primary irradiation to re-RT was 49.5 months (range 24-98 months) with median cumulative biologically effective dose of 117 Gy (range 78-132 Gy). All patients received platinum-based salvage chemotherapy either before or after re-RT. One patient developed symptomatic radiation necrosis following re-CSI. At a median follow-up of 24 months (range 6-84 months), 2-year post-re-RT progression-free survival (PFS) and overall survival (OS) was 46% and 51% respectively. Younger age (< 18 years) at index diagnosis, primary risk stratification (standard-risk) and molecular subgrouping (Group 4) were associated with significantly better post-re-RT outcomes. CONCLUSION: Salvage re-RT provides good local control and encouraging survival outcomes with acceptable toxicity in selected patients with recurrent/progressive MB.


Subject(s)
Cerebellar Neoplasms/mortality , Medulloblastoma/mortality , Neoplasm Recurrence, Local/mortality , Re-Irradiation/mortality , Risk Assessment/methods , Salvage Therapy , Adolescent , Adult , Age Factors , Cerebellar Neoplasms/classification , Cerebellar Neoplasms/pathology , Cerebellar Neoplasms/radiotherapy , Child , Child, Preschool , Female , Follow-Up Studies , Humans , Male , Medulloblastoma/classification , Medulloblastoma/pathology , Medulloblastoma/radiotherapy , Neoplasm Recurrence, Local/classification , Neoplasm Recurrence, Local/pathology , Neoplasm Recurrence, Local/radiotherapy , Prognosis , Retrospective Studies , Survival Rate , Young Adult
10.
Neuropathology ; 39(5): 378-381, 2019 Oct.
Article in English | MEDLINE | ID: mdl-31373070

ABSTRACT

This is a report of an uncommon primary pigmented papillary epithelial tumor of the sella in a 38-year-old man, who presented with clinicoradiological features of pituitary adenoma. Histologically, the tumor showed features reminiscent of choroid plexus papilloma, that is, conspicuous papillary epithelial morphology with presence of intacytoplasmic melanin and no mitotic activity. Immunohistochemically, the tumor was positive for pancytokeratin (AE1/AE3), S-100 protein and CD56, while it was negative for glial fibrillary acid protein, thyroid transcription factor-1, epithelial membrane antigen, other cytokeratins and pituitary hormones. These findings were not typical of any WHO-defined entity and is thus best regarded as a pigmented papillary epithelial tumor of sella of uncertain histogenesis. The present case is a valuable addition to the spectrum of primary pigmented papillary epithelial tumors originating at an unusual location.


Subject(s)
Neoplasms, Glandular and Epithelial/pathology , Pituitary Neoplasms/pathology , Adult , Humans , Male , Melanins
11.
Pol J Pathol ; 68(2): 182-184, 2017.
Article in English | MEDLINE | ID: mdl-29025254

ABSTRACT

CASE REPORT: We herein report a case of chromoblastomycosis presenting as a verrucous lesion over the leg. A 56-year-old male patient was a known case of carcinoma larynx and was treated for the same. At presentation to our hospital, the patient, in addition to the recurrent local disease, was suspected to have second primary in the form of verrucous carcinoma of the leg. Histopathological examination of the skin biopsy revealed the presence of characteristic pigmented sclerotic bodies with pseudoepitheliomatous hyperplasia of the overlying epithelium. The case was reported as chromoblastomycosis and the patient responded well to anti-fungal chemotherapy in the form of itraconazole.


Subject(s)
Chromoblastomycosis/pathology , Carcinoma, Squamous Cell/complications , Chromoblastomycosis/complications , Head and Neck Neoplasms/complications , Humans , Laryngeal Neoplasms/complications , Leg/microbiology , Male , Middle Aged , Squamous Cell Carcinoma of Head and Neck
13.
Arch Pathol Lab Med ; 148(4): 453-460, 2024 Apr 01.
Article in English | MEDLINE | ID: mdl-37490416

ABSTRACT

CONTEXT.­: Human epidermal growth factor (HER2/neu) gene amplification, a poor prognostic factor in invasive breast cancer, has shown substantial utility as a predictive marker, with significantly improved survival following anti-HER2 therapies like trastuzumab. Dual-color dual in situ hybridization (D-DISH), a recently introduced fully automated assay for HER2/neu evaluation on light microscopy, has several advantages over fluorescence in situ hybridization (FISH). OBJECTIVE.­: To standardize and validate the D-DISH assay using FISH as the gold standard and assess interobserver reproducibility in interpreting the D-DISH assay. DESIGN.­: D-DISH was performed using the latest HER2 Dual ISH DNA Probe Cocktail assay (Ventana Medical Systems Inc, Tucson, Arizona) in 148 cases of invasive breast cancer. The same block was used for performing immunohistochemistry by Ventana PATHWAY anti-HER2/neu (4B5) antibody and FISH assay by ZytoLight SPEC ERBB2/CEN17 Dual Color Probe. D-DISH was separately interpreted by 4 pathologists blinded to FISH results. RESULTS.­: Concordance of 98.65% and a Cohen κ value of 0.97 were observed between FISH and D-DISH. Intraclass correlation coefficient (0.93-0.97) and κ values (0.98-1.0) for interobserver reproducibility showed almost perfect agreement by D-DISH. Interobserver reproducibility was also evaluated for genomic heterogeneity, HER2 group categorization, and polysomy (κ values 0.42-0.74, 0.89-0.93, and 0.98-1.0, respectively). CONCLUSIONS.­: We successfully validated the latest version of D-DISH assay as a substitute for FISH in predicting HER2 gene status with significant interobserver reproducibility, concluding that this D-DISH assay may be introduced in routine diagnostic services as a reflex test to ascertain HER2 gene status.


Subject(s)
Breast Neoplasms , Genes, erbB-2 , Humans , Female , Breast Neoplasms/diagnosis , In Situ Hybridization, Fluorescence/methods , Reproducibility of Results , Receptor, ErbB-2/genetics , Receptor, ErbB-2/metabolism , Immunohistochemistry
14.
Explor Target Antitumor Ther ; 5(3): 766-779, 2024.
Article in English | MEDLINE | ID: mdl-38966175

ABSTRACT

Aim: Supratentorial ependymoma (STE) is a rare tumor with distinct genetic alterations, whose imaging features have been scarcely studied. This study aims to review the computed tomography (CT) and magnetic resonance imaging (MRI) features of a cohort of histopathologically proven STE to identify the distinguishing features of STE, and look for specific signs of zinc finger translocation associated (ZFTA) fused STEs. Methods: Ethical clearance was obtained from the institutional ethics committee. The magnetic resonance (MR) images, CT images when available, clinical details, and pathological reports of 25 patients from a single institute with histopathologically proven STE were retrospectively reviewed. Imaging features, demographic details, pathological and molecular features, and type of surgical resection were described and tabulated. Relevant associations with imaging features were computed and tabulated. Results: The study showed that STEs are common in the pediatric population with no sex predilection. The periventricular location was the most common. A significant association between periventricular location and the presence of a cystic component (P value = 0.023) and the presence of the periwinkle sign/stellate sign (P value = 0.045) was found. Common features of ZFTA fused STEs included periventricular or intraventricular location, cystic component, necrosis, and the periwinkle sign. A significant association was found between ZFTA fusion and cystic component (P value = 0.048). Conclusions: This study attempts to identify the imaging features of STEs and their associations with molecular pathology and surgical outcome, and the distinguishing features of ZFTA fused STEs.

15.
Diagnostics (Basel) ; 14(4)2024 Feb 07.
Article in English | MEDLINE | ID: mdl-38396397

ABSTRACT

Medulloblastoma (MB) comprises four broad molecular subgroups, namely wingless (WNT), sonic hedgehog (SHH), Group 3, and Group 4, respectively, with subgroup-specific developmental origins, unique genetic profiles, distinct clinico-demographic characteristics, and diverse clinical outcomes. This is a retrospective audit of clinical outcomes in molecularly confirmed WNT-MB patients treated with maximal safe resection followed by postoperative standard-of-care risk-stratified adjuvant radio(chemo)therapy at a tertiary-care comprehensive cancer centre. Of the 74 WNT-MB patients registered in a neuro-oncology unit between 2004 to 2020, 7 patients accrued on a prospective clinical trial of treatment deintensification were excluded, leaving 67 patients that constitute the present study cohort. The median age at presentation was 12 years, with a male preponderance (2:1). The survival analysis was restricted to 61 patients and excluded 6 patients (1 postoperative mortality plus 5 without adequate details of treatment or outcomes). At a median follow-up of 72 months, Kaplan-Meier estimates of 5-year progression-free survival and overall survival were 87.7% and 91.2%, respectively. Traditional high-risk features, large residual tumour (≥1.5 cm2), and leptomeningeal metastases (M+) did not significantly impact upon survival in this molecularly characterized WNT-MB cohort treated with risk-stratified contemporary multimodality therapy. The lack of a prognostic impact of conventional high-risk features suggests the need for refined risk stratification and potential deintensification of therapy.

16.
World Neurosurg ; 181: e809-e819, 2024 Jan.
Article in English | MEDLINE | ID: mdl-37923012

ABSTRACT

OBJECTIVE: Craniopharyngioma (CP) is a benign neuroepithelial tumor generally treated with maximal safe resection and radiation therapy (RT) in incompletely resected CP or in recurrent tumors to achieve long-term control. We analyzed the clinical outcomes of patients with CPs treated with a multimodality approach. PATIENTS AND METHODS: A retrospective clinical audit of histologically proven CPs registered between 2008 and 2019 at a specialized neuro-oncology center in India was performed. Time-to-event outcomes (overall survival [OS] and progression-free survival [PFS]) were analyzed. RESULTS: One hundred and twenty-two patients with CP were analyzed. The median age of the population was 14 years (interquartile range [IQR], 8-26) with a significant male preponderance. Gross total resection was achieved in only 25% of patients. At a median follow-up of 57.1 months (IQR, 27.8-87.8), 5-year estimates of PFS and OS were 52% (95% confidence interval, 46%-63.4%) and 85.8% (95% confidence interval, 78.6%-93%), respectively. Recurrence or progression was observed in 48 of 122 patients (39.3%) at a median time of 84.4 months (IQR, 24.7-174.8). On multivariate analysis, the absence of residual disease (P = 0.004), near-total resection (P = 0.035), and use of up-front adjuvant RT (P < 0.001) significantly improved the 5-year PFS, whereas the absence of extracavernous extension (P = 0.058) and any use of postoperative RT (P = 0.026) significantly improved the 5-year OS. CONCLUSIONS: This study represents one of the largest single-institutional series of CPs, showing improved PFS with up-front adjuvant RT in most cases of CP. Deferring adjuvant RT should be considered only in patients with no evidence of residual disease (as shown on dedicated sellar imaging) after primary surgery.


Subject(s)
Craniopharyngioma , Pituitary Neoplasms , Humans , Male , Adolescent , Treatment Outcome , Retrospective Studies , Craniopharyngioma/radiotherapy , Craniopharyngioma/surgery , Pituitary Neoplasms/radiotherapy , Pituitary Neoplasms/surgery , Neoplasm Recurrence, Local
17.
Neurol India ; 72(2): 278-284, 2024 Mar 01.
Article in English | MEDLINE | ID: mdl-38691470

ABSTRACT

PURPOSE: Refractory and/or recurrent meningiomas have poor outcomes, and the treatment options are limited. Peptide receptor radionuclide therapy (PRRT) has been used in this setting with promising results. We have documented our experience of using intravenous (IV) and intra-arterial (IA) approaches of Lu-177 DOTATATE PRRT. METHODS: Eight patients with relapsed/refractory high-grade meningioma received PRRT with Lu-177 DOTATATE by IV and an IA route. At least 2 cycles were administered. Time to progression was calculated from the first PRRT session to progression. The response was assessed on MRI using RANO criteria, and visual analysis of uptake was done on Ga-68 DOTANOC PET/CT. Post-therapy dosimetry calculations for estimating the absorbed dose were performed. RESULTS: Median time to progression was 8.9 months. One patient showed disease progression, whereas seven patients showed stable disease at 4 weeks following 2 cycles of PRRT. Dosimetric analysis showed higher dose and retention time by IA approach. No significant peri-procedural or PRRT associated toxicity was seen. CONCLUSION: PRRT is a safe and effective therapeutic option for relapsed/refractory meningioma. The IA approach yields better dose delivery and should be routinely practised.


Subject(s)
Meningeal Neoplasms , Meningioma , Octreotide , Octreotide/analogs & derivatives , Humans , Meningioma/radiotherapy , Meningioma/diagnostic imaging , Meningeal Neoplasms/radiotherapy , Meningeal Neoplasms/diagnostic imaging , Female , Male , Octreotide/therapeutic use , Octreotide/administration & dosage , Middle Aged , Adult , Organometallic Compounds/therapeutic use , Aged , Treatment Outcome , Radiopharmaceuticals/therapeutic use , Receptors, Peptide , Tertiary Care Centers , Disease Progression
18.
Indian J Cancer ; 61(Suppl 1): S52-S79, 2024 Jan 01.
Article in English | MEDLINE | ID: mdl-38424682

ABSTRACT

ABSTRACT: The incidence of breast cancer is increasing rapidly in urban India due to the changing lifestyle and exposure to risk factors. Diagnosis at an advanced stage and in younger women are the most concerning issues of breast cancer in India. Lack of awareness and social taboos related to cancer diagnosis make women feel hesitant to seek timely medical advice. As almost half of women develop breast cancer at an age younger than 50 years, breast cancer diagnosis poses a huge financial burden on the household and impacts the entire family. Moreover, inaccessibility, unaffordability, and high out-of-pocket expenditure make this situation grimmer. Women find it difficult to get quality cancer care closer to their homes and end up traveling long distances for seeking treatment. Significant differences in the cancer epidemiology compared to the west make the adoption of western breast cancer management guidelines challenging for Indian women. In this article, we intend to provide a comprehensive review of the management of breast cancer from diagnosis to treatment for both early and advanced stages from the perspective of low-middle-income countries. Starting with a brief introduction to epidemiology and guidelines for diagnostic modalities (imaging and pathology), treatment has been discussed for early breast cancer (EBC), locally advanced, and MBC. In-depth information on loco-regional and systemic therapy has been provided focusing on standard treatment protocols as well as scenarios where treatment can be de-escalated or escalated.


Subject(s)
Breast Neoplasms , Female , Humans , Middle Aged , Breast Neoplasms/diagnosis , Breast Neoplasms/epidemiology , Breast Neoplasms/therapy , Emotions , Family Characteristics , India/epidemiology
19.
Indian J Nucl Med ; 38(2): 154-156, 2023.
Article in English | MEDLINE | ID: mdl-37456183

ABSTRACT

Neuroendocrine tumor (NET) of the prostate is an extremely rare entity which represents <1% of the prostatic cancers, but with increasing incidence. Its spectrum encompasses several histological variants ranging from well-differentiated tumor which are often indolent in nature; to aggressive neuroendocrine carcinoma which portends aggressive management. Hence, such rare entities are to be characterized and treated accordingly. We report an unusual case of well-differentiated NET of prostate which was flagged on fluorodeoxyglucose positron emission tomography computed tomography (PET/CT) performed for other indication and confirmed on Gallium-68 DOTANOC PET/CT. Histopathology and immunohistochemistry confirmed the findings subsequently.

20.
Clin Nucl Med ; 48(1): e28-e30, 2023 Jan 01.
Article in English | MEDLINE | ID: mdl-36240749

ABSTRACT

ABSTRACT: Sporadic cerebellar hemangioblastomas are rare with majority of these tumors presenting as a part of von Hippel-Lindau syndrome. We demonstrate an unusual case of a symptomatic sporadic cerebellar hemangioblastoma mimicking a meningioma on MRI and 68 Ga-DOTANOC PET imaging.


Subject(s)
Cerebellar Neoplasms , Hemangioblastoma , von Hippel-Lindau Disease , Humans , Hemangioblastoma/metabolism , Positron Emission Tomography Computed Tomography , von Hippel-Lindau Disease/pathology , Cerebellar Neoplasms/diagnostic imaging
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