Mixed neuroendocrine-non-neuroendocrine neoplasms (MiNENs) of gastrointestinal (GI) tract: A single institution experience.

2020 ◽  
Vol 38 (4_suppl) ◽  
pp. 633-633
Author(s):  
Manik A. Amin ◽  
Nikolaos Trikalinos
Keyword(s):  
Surgical Resection ◽  
Poor Prognosis ◽  
Braf V600e ◽  
Low Grade ◽  
Sequencing Analysis ◽  
High Grade ◽  
Gi Tract ◽  
Intermediate Grade ◽  
Disease Free

633 Background: MiNENs are heterogeneous group of rare tumors and represent less than 1% of all GI malignancies. These are pathologically classifiable according to neuroendocrine component into low grade, intermediate grade and high grade MiNENs but and managed based upon non-neuroendocrine component rather than true NENs. They have poor prognosis and less is known about treatment options. Methods: We identified 34 cases of MiNENs from 1/1/2000 to 1/1/2018 from Siteman Cancer Registry Database at Siteman Cancer Center, Washington University, St. Louis. Treatment and follow up information was obtained from medical charts of patients. Results: Of 34 identified cases of MiNENs, our database showed equal distribution of MiNENs in male and female patients (N = 17 each). Site of origin included appendix (N = 14), colon (N = 10), esophageal (N = 2), stomach (N = 3), rectum (N = 3), small intestine (N = 1) and ampulla of Vater (N = 1) locations. Histology was high grade (N = 22), intermediate grade (N = 8), low grade (N = 3) and unknown (1). Twenty nine (85%) underwent surgical resection with curative intent and 13 (40%) patients were disease free at five years of follow up. Disease was localized in 15 patients (44%) and distant metastasis were reported in 13 patients (40%). Ten out of 15 patient with localized disease were disease free with combined modality therapy of surgery and chemotherapy. Most common chemotherapy regimen used in the metastatic setting was FOLFOX (11), 5FU/capecitabine (3), and carboplatin plus etoposide (5). Next generation sequencing analysis was available on limited patients which showed MMR proficient (3), MMR deficiency (1), BRAF V600E mutation (1) and KRAS wild type (1). Conclusions: MiNENs of GI tract are rare and aggressive tumors and represent a distinct entity. Surgical resection whenever possible offers curative option. Our database of GI MiNENs showed efficacy with commonly used GI chemotherapy regimens. Patients with low grade tumor histology had better survival as compared to moderately and poorly differentiated tumors. Genomic testing through NGS would be recommended for personalized treatment decisions given poor prognosis in these patients.

scholarly journals Validation of a clinicopathological score for the prediction of post-surgical evolution of pituitary adenoma: retrospective analysis on 566 patients from a tertiary care centre

2019 ◽  
Vol 180 (2) ◽  
pp. 127-134 ◽  
Author(s):  
S Asioli ◽  
A Righi ◽  
M Iommi ◽  
C Baldovini ◽  
F Ambrosi ◽  
...  
Keyword(s):  
Pituitary Adenomas ◽  
Proliferative Activity ◽  
Disease Free Survival ◽  
Low Grade ◽  
High Grade ◽  
Tumour Type ◽  
Disease Evolution ◽  
Free Survival ◽  
Disease Free

Objective and design A clinicopathological score has been proposed by Trouillas et al. to predict the evolution of pituitary adenomas. Aim of our study was to perform an independent external validation of this score and identify other potential predictor of post-surgical outcome. Methods The study sample included 566 patients with pituitary adenomas, specifically 253 FSH/LH-secreting, 147 GH-secreting, 85 PRL-secreting, 72 ACTH-secreting and 9 TSH-secreting tumours with at least 3-year post-surgical follow-up. Results In 437 cases, pituitary adenomas were non-invasive, with low (grade 1a: 378 cases) or high (grade 1b: 59 cases) proliferative activity. In 129 cases, tumours were invasive, with low (grade 2a: 87 cases) or high (grade 2b: 42 cases) proliferative activity. During the follow-up (mean: 5.8 years), 60 patients developed disease recurrence or progression, with a total of 130 patients with pituitary disease at last follow-up. Univariate analysis demonstrated a significantly higher risk of disease persistence and recurrence/progression in patients with PRL-, ACTH- and FSH/LH-secreting tumours as compared to those with somatotroph tumours, and in those with high proliferative activity (grade 1b and 2b) or >1 cm diameter. Multivariate analysis confirmed tumour type and grade to be independent predictors of disease-free-survival. Tumour invasion, Ki-67 and tumour type were the only independent prognostic factors of disease-free survival. Conclusions Our data confirmed the validity of Trouillas’ score, being tumour type and grade independent predictors of disease evolution. Therefore, we recommend to always consider both features, together with tumour histological subtype, in the clinical setting to early identify patients at higher risk of recurrence.

Outcome of leiomyosarcoma of bone: A single center experience.

2013 ◽  
Vol 31 (15_suppl) ◽  
pp. e21502-e21502
Author(s):  
Rajeev Rajendra ◽  
Seth Pollack ◽  
Eve T. Rodler ◽  
Ernest U. Conrad ◽  
Darin J Davidson ◽  
...  
Keyword(s):  
Local Recurrence ◽  
Neoadjuvant Chemotherapy ◽  
Surgical Resection ◽  
Treatment Strategies ◽  
Recurrence Time ◽  
Low Grade ◽  
High Grade ◽  
Response To Chemotherapy

e21502 Background: Leiomyosarcoma (LMS) of bone is a very rare sarcoma subtype. These tumors are managed akin to osteosarcoma, with neoadjuvant chemotherapy followed by surgery. The precise role of chemotherapy remains to be defined. Methods: Patients treated with primary bone LMS at the University of Washington between 2002 and 2012 were included. Patients with high grade tumors were treated with neoadjuvant chemotherapy and surgery; whereas those with low grade tumors were treated with surgical resection alone. Chemotherapy consisted of doxorubicin and ifosfamide x 2 cycles. Treatment details included: initial treatment (surgery versus chemo), surgical and pathological margins, and timing of chemotherapy. Follow-up data included: time to local recurrence, time to metastasis, time to last follow up if alive, or time to death. Results: Ten patients were identified, 4 male and 6 female. Median age at diagnosis: 52 years (range 29 - 91). The primary site was the distal femur in 5 patients, and the hemipelvis, acetabulum, proximal femur, distal clavicle and mid-shaft of femur in 1 patient each. Median tumor size at diagnosis was 8 cm. Five were high-grade tumors; 3 were intermediate and 2 were low grade. Four of 10 patients received neoadjuvant chemotherapy, with the following histological response; 70%, 30%, 15% and <5%. None of these patients had a dimensional radiological response to chemotherapy. Of the patients treated with surgery alone, one developed a local recurrence and another developed metastatic disease. Of the patients treated with chemotherapy and surgery, 1 died from an unrelated cause. Median follow-up was 9 months (range 0 - 83). Median DFS was 9 months (range 0 - 83). OS has not yet been reached. Conclusions: Surgical resection remains the mainstay of management of LMS of bone. The role of neoadjuvant chemotherapy requires further evaluation. Larger collaborative studies and biomarker analyses are essential to evaluate optimal treatment strategies for this rare disease.

Patterns in the care of oligodendrogliomas and oligoastrocytomas: A single center experience

2009 ◽  
Vol 27 (15_suppl) ◽  
pp. e13034-e13034
Author(s):  
S. H. Kizilbash ◽  
L. Nadeau
Keyword(s):  
Radiation Therapy ◽  
Surgical Resection ◽  
Progression Free Survival ◽  
Low Grade ◽  
High Grade ◽  
Single Center Experience ◽  
Data Collection And Analysis ◽  
Unique Identity

e13034 Background: Although rare, oligodendrogliomas (ODs) and oligoastrocytomas (OAs) have established a unique identity due to their sensitivity to chemotherapy, especially if they exhibit combined loss of heterozygosity (LOH) of 1p and 19q. However, optimal management is still controversial and data is lacking on actual practice patterns. This study describes the epidemiology of ODs/OAs at a community based hospital in Michigan and evaluates the management of these tumors, especially with respect to 1p/19q LOH. Methods: A retrospective review was conducted of all cases of OD and OA that were managed at our institution from 1975 to 2007. 135 patients were isolated. Of these, 48 patients had already been evaluated for 1p/19q LOH. This latter subset underwent data collection and analysis. Results: 31 patients had low-grade tumors (either ODs or OAs) while 17 had high grade tumors (anaplastic ODs or anaplastic OAs). Of the low grade tumors, 35% had combined 1p/19q LOH. 71% underwent surgical resection, 35% received chemotherapy, and 26% received radiation therapy. As for the high grade tumors, 47% had combined 1p/19q LOH. 88% underwent surgical resection, 76% received chemotherapy, and 59% received radiation therapy. At a mean of 29 months of follow up, eight patients had tumor progression while 12 died. Median progression free survival (MPFS) for patients receiving chemotherapy was increased in patients with combined 1p/19q LOH, for both low-grade tumors (34 vs. 20 months) and high-grade tumors (14 vs. 8 months). Also, significantly more patients with low-grade tumors and combined 1p/19q LOH were offered chemotherapy when compared to those without 1p/19q LOH (64% vs 20%, p = 0.04, Fisher exact). However, in patients with high-grade tumors, knowledge of 1p/19q LOH did not significantly impact the choice to administer chemotherapy (88% vs. 67%, p = 0.67). Conclusions: Patients with ODs/OAs and combined 1p/19q LOH who have been treated with chemotherapy demonstrate increased MPFS when compared with those without this genotype. Despite this, determination of 1p/19q LOH significantly affects physician choice to offer chemotherapy only in patients with low-grade tumors, but not in those with high-grade tumors. A future study is in development to determine 1p/19q LOH status for the remaining 87 patients. No significant financial relationships to disclose.

Abstract TMP118: Clinical Outcomes of Surgical Resection After Stereotactic Radiosurgery Among Patients With Cerebral Arteriovenous Malformations

Stroke ◽  
2020 ◽  
Vol 51 (Suppl_1) ◽  
Author(s):  
Mark G Bigder ◽  
Omar Choudhri ◽  
Mihir Gupta ◽  
Ephraim Church ◽  
Steven Chang ◽  
...  
Keyword(s):  
Stereotactic Radiosurgery ◽  
Surgical Resection ◽  
Arteriovenous Malformations ◽  
Perioperative Period ◽  
Multivariate Regression Analysis ◽  
Low Grade ◽  
High Grade ◽  
Eligibility Criteria ◽  
Cerebral Avms

Introduction: Surgical treatment of arteriovenous malformations (AVMs), particularly higher grade lesions, can be aided by staged treatment consisting of stereotactic radiosurgery (SRS) followed by surgical resection in a delayed fashion. This strategy can be used to downgrade the AVM S-M grade, reduce blood flow through the AVM and often results in histopathological changes making AVMs more amenable to microsurgical resection. We present our 28-year clinical experience in managing AVMs with pre-operative SRS as a surgical adjunct. Methods: We retrospectively reviewed and analyzed records of all patients treated for cerebral AVMs between February 1991 and July 2019 at our institution. All patients that underwent SRS, with and without embolization, followed by microsurgery were included in the study. Of the 1245 cerebral AVM patients treated at our institution, 62 patients met eligibility criteria. Univariate and multivariate regression analysis was performed where appropriate to examine relationships between key variables and outcomes. Results: The majority of lesions (50%) were high grade (SM 4-5), 28.6% were intermediate (SM 3), while 21.4% were low grade (SM 1-2). Hemorrhage was the presenting sign among 22.6% of patients. Complete resection was achieved among 64.5%, 79% and 82% of patients after first, second and third surgical stages respectively; 16.1% of patients had partial resection requiring further treatment. Radiographic cure was achieved among 53 patients (85.5%), while 8 (12.9%) patients had residual AVM at last follow up. Six of 8 patients without radiographic cure received post-operative SRS. Thirty-seven patients (63.8%) had improved (26, 44.8%) or stable mRS scores (11, 19%), while 21 (36.2%) had a decline in mRS at final follow up compared to mRS at presentation; this includes 4 (6.9%) deaths due to hemorrhage, outside of the perioperative period, but occurring during follow up prior to AVM obliteration. Conclusion: SRS is a useful adjunct in the surgical management of cerebral AVMs. Multimodal therapy allowed for high obliteration rates with acceptable morbidity in this series of patients with predominantly high grade AVMs.

scholarly journals EPCT-13. SINGLE INSTITUTION RETROSPECTIVE ANALYSIS OF TUMOR MUTATIONAL BURDEN AND SURVIVAL IN PEDIATRIC BRAIN TUMORS

2021 ◽  
Vol 23 (Supplement_1) ◽  
pp. i49-i49
Author(s):  
Rose Parisi ◽  
Roshal Patel ◽  
Lauren Weintraub
Keyword(s):  
Retrospective Analysis ◽  
Medical Center ◽  
Braf V600e ◽  
Cns Tumors ◽  
Driver Mutations ◽  
Low Grade ◽  
High Grade ◽  
Mutational Burden ◽  
Tumor Mutational Burden

Abstract Tumor mutational burden (TMB) has been studied across numerous cancer types as a means of risk stratification. To examine the prognostic relevance of TMB to pediatric central nervous system (CNS) tumors, we conducted a retrospective analysis of patients at Albany Medical Center diagnosed from 2012 to present. Patients were &lt;21 at diagnosis, had a primary CNS tumor and available genomic data. Forty-seven patients were included – 22 low-grade gliomas, 10 high-grade gliomas, 5 medulloblastomas, 3 ependymomas, 2 choroid plexus carcinomas, and 5 other CNS tumors, with a median follow up of 36 months, median age at diagnosis 10 (1–19), and 47% female. Median TMB was 1 mutation per megabase (mut/mb); range 0–6. Nine patients did not have available TMB data. Twenty-seven patients had driver mutations and other alterations implicated in cancer development including, including BRAF-KIAA1549 fusion (n=6), NF1 loss (n=5), FGFR1 amplification (n=4), TP53 inactivation (n=4), BRAF V600E mutation (n=3), and H3F3A K28M mutation (n=3). Patients with low TMB (&lt;3 muts/mb; n=24) versus high TMB (≥3 muts/mb; n=14) had a survival of 87% versus 71%, respectively, at last follow-up. Of note, all but one patient in the low TMB cohort had localized disease at diagnosis versus three in the high TMB cohort. High TMB was more prevalent in high- (45%, 9/20) versus low-grade histologies (22%, 4/18). Patients with BRAF alterations had LGGs and low TMB (0–1 muts/mb) with all patients surviving at last follow up. Of the eight deaths observed (median 18 months from diagnosis) TMB was high in 4, low in 3, and unknown in 1; all had high-grade histology. Although limited, our data suggests higher TMB may be associated with worse outcome. This analysis will be expanded via a multi-institutional review of TMB and genomic alterations in pediatric CNS patients to better identify high-risk patients requiring alternative treatment strategies.

Phase I Study of Fludarabine Plus Cyclophosphamide in Patients With Previously Untreated Low-Grade Lymphoma: Results and and Long-Term Follow-Up—A Report From the Eastern Cooperative Oncology Group

2000 ◽  
Vol 18 (5) ◽  
pp. 987-987 ◽  
Author(s):  
Howard S. Hochster ◽  
Martin M. Oken ◽  
Jane N. Winter ◽  
Leo I. Gordon ◽  
Bruce G. Raphael ◽  
...  
Keyword(s):  
Phase Ii ◽  
Bone Marrow Involvement ◽  
Survival Rates ◽  
Eastern Cooperative Oncology Group ◽  
Disease Free Survival ◽  
Low Grade ◽  
Survival Times ◽  
Free Survival ◽  
Disease Free

PURPOSE: To determine the toxicity and recommended phase II doses of the combination of fludarabine plus cyclophosphamide in chemotherapy-naive patients with low-grade lymphoma. PATIENTS AND METHODS: Previously untreated patients with low-grade lymphoma were entered onto dosing cohorts of four patients each. The cyclophosphamide dose, given on day 1, was increased from 600 to 1,000 mg/m2. Fludarabine 20 mg/m2 was administered on days 1 through 5. The first eight patients were treated every 21 days; later patients were treated every 28 days. Prophylactic antibiotics were required. RESULTS: Prolonged cytopenia and pulmonary toxicity each occurred in three of eight patients treated every 3 weeks. The 19 patients treated every 28 days, who were given granulocyte colony-stimulating factor as indicated, did not have undue nonhematologic toxicity. Dose-limiting toxicity was hematologic. At the recommended phase II/III dose (cyclophosphamide 1,000 mg/m2), grade 4 neutropenia was observed in 17% of all cycles and 31% of first cycles. Grade 3 or 4 thrombocytopenia was seen in only 1% of all cycles. The median number of cycles per patient was six (range, two to 11) for all patients enrolled. The response rate was 100% of 27 patients entered; 89% achieved a complete and 11% a partial response. Nineteen of 22 patients with bone marrow involvement had clearing of the marrow. Median duration of follow-up was more than 5 years; median overall and disease-free survival times have not been reached. Kaplan-Meier estimated 5-year overall survival and disease-free survival rates were 66% and 53%, respectively. CONCLUSION: The recommended dosing for this combination in patients with previously untreated low-grade lymphoma is cyclophosphamide 1,000 mg/m2 day 1 and fludarabine 20 mg/m2 days 1 through 5. The regimen has a high level of activity, with prolonged complete remissions providing 5-year overall and disease-free survival rates as high as those reported for other therapeutic approaches in untreated patients.

scholarly journals Oral Abstract

2016 ◽  
Author(s):  
Dharma Ram
Keyword(s):  
Survival Data ◽  
Eligible Patient ◽  
Stage Iv ◽  
Endometrial Stromal Sarcoma ◽  
Uterine Sarcoma ◽  
Low Grade ◽  
Uterine Leiomyosarcoma ◽  
High Grade ◽  
Lost To Follow Up

Introduction: Uterine sarcoma accounts for nearly 3% of all uterine malignancies. They have 4 major pathology includes endometrial stromal sarcoma high grade, ESS low grade, uterine leiomyosarcoma (uLMS) and undifferentiated uterine sarcoma (UUS). Recent WHO classification 2014, recognizes low grade ESS and high grade ESS as distinct entity. They differ from endometrial carcinoma in their aggressive nature and poor prognosis. We review our database and found total 44 eligible patient treated at our institute. Materials and Methods: Its retrospective analysis of computer based database of our institute from January 2009 to December 2015. We analyzed demographic, pathological, treatment and survival data. Results: Total 44 patient treated for uterine sarcoma at our institute. Among these 16 were operated at our institute during study period. Here we reporting results of operated patients at our institute. The histological diagnosis LMS (5/16), ESS-L (4/16), MMMT (3/16), UUS (3/16) and ESS-H (1/16). Stage distribution was stage I, (6/16) stage II, (5/16) stage III, (3/16) stage IV, (0/16) and unknown stage (2/16). Two patients underwent completion surgery for outside myomectomy. The adjuvant treatment was CT in 3/16, CT with RT in 7/16, HT in 4/16 and one lost to follow up with one was put on observation. Median follow up is 30 month with 14 patients alive and one lost to follow up. At last follow up 4 patients alive with metastatic disease and 10 patients alive with no evidence of disease. Conclusion: Uterine sarcoma are uncommon disease with

Abstract CT025: Dabrafenib plus trametinib in BRAF V600E-mutant high-grade (HGG) and low-grade glioma (LGG)

2021 ◽  
Author(s):  
Vivek Subbiah ◽  
Alexander Stein ◽  
Martin van den Bent ◽  
Antje Wick ◽  
Filip Y. de Vos ◽  
...  
Keyword(s):  
Low Grade Glioma ◽  
Braf V600e ◽  
Low Grade ◽  
High Grade

EPCO-17. METHYLATION ANALYSIS OF MATCHED PRIMARY AND RECURRENT IDHmt ASTROCYTOMA; AN UPDATE FROM THE GLIOMA LONGITUDINAL ANALYSIS NL (GLASS-NL) CONSORTIUM

2021 ◽  
Vol 23 (Supplement_6) ◽  
pp. vi5-vi5
Author(s):  
Wies Vallentgoed ◽  
Anneke Niers ◽  
Karin van Garderen ◽  
Martin van den Bent ◽  
Kaspar Draaisma ◽  
...  
Keyword(s):  
Dna Methylation ◽  
Surgical Resection ◽  
Molecular Mechanisms ◽  
Relative Proportion ◽  
Low Grade ◽  
High Grade ◽  
Primary Tumors ◽  
Genome Wide ◽  
Dna Methylation Profiling ◽  
Methylation Profiling

Abstract The GLASS-NL consortium, was initiated to gain insight into the molecular mechanisms underlying glioma evolution and to identify markers of progression in IDH-mutant astrocytomas. Here, we present the first results of genome-wide DNA-methylation profiling of GLASS-NL samples. 110 adult patients were identified with an IDH-mutant astrocytoma at first diagnosis. All patients underwent a surgical resection of the tumor at least twice, separated by at least 6 months (median 40.9 months (IQR: 24.0, 64.7). In 37% and 18% of the cases, patients were treated with radiotherapy or chemotherapy respectively, before surgical resection of the recurrent tumor. DNA-methylation profiling was done on 235 samples from 103 patients (102 1st, 101 2nd, 29 3rd, and 3 4th resection). Copy number variations were also extracted from these data. Methylation classes were determined according to Capper et al. Overall survival (OS) was measured from date of first surgery. Of all primary tumors, the methylation-classifier assigned 85 (87%) to the low grade subclass and 10 (10%) to the high grade subclass. The relative proportion of high grade tumors increased ~three-fold at tumor recurrence (32/101, 32%) and even further in the second recurrence (15/29, 52%). Methylation classes were prognostic, both in primary and recurrent tumors. The overall DNA-methylation levels of recurrent samples was lower than that of primary samples. This difference is explained by the increased number of high grade samples at recurrence, since near identical DNA-methylation levels were observed in samples that remained low grade. In an unsupervised analysis, DNA-methylation data derived from primary and first recurrence samples of individual patients mostly (79%) cluster together. Recurrent samples that do not cluster with their primary tumor, form a separate group with relatively low genome-wide DNA-methylation. Our data demonstrate that methylation profiling identifies a shift towards a higher grade at tumor progression coinciding with reduced genome-wide DNA-methylation levels.

Endoscopic Surveillance Practice for Barrett's Oesophagus in Scotland and Early Experience in Implementing Local Guidelines

2003 ◽  
Vol 48 (2) ◽  
pp. 43-45 ◽  
Author(s):  
E F Shen ◽  
S Gladstone ◽  
G Milne ◽  
S Paterson-Brown ◽  
I D Penman
Keyword(s):  
Early Experience ◽  
High Grade Dysplasia ◽  
Low Grade ◽  
High Grade ◽  
Wide Variability ◽  
Low Grade Dysplasia ◽  
Surveillance Practice ◽  
Four Quadrant ◽  
The Impact

Management of columnar lined oesophagus (CLO; Barrett s oesophagus) is controversial. We prospectively audited surveillance practices in Scotland and prospectively assessed the impact of introducing local guidelines for Barrett s surveillance in Edinburgh. Most respondents were gastroenterologists. The majority take random, not four quadrant, biopsies from the CLO. In Edinburgh during 2000, 80 patients underwent surveillance. The guideline protocol was not followed in 30 (37.5%) patients. Follow up of patients without dysplasia generally conformed to the guidelines. Follow up of patients with low grade dysplasia was highly variable while management of those with high grade dysplasia followed the guidelines. Overall we found a wide variability in the management and surveillance of CLO. Early experience suggests that implementation of guidelines is helpful but there is still variation in practice.

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