scholarly journals HGG-29. A CASE OF CIRCUMSCRIBED HIGH-GRADE ASTROCYTOMA WITH ATRX AND CDKN2A/B ALTERNATIONS WHO WAS INITIALLY DIAGNOSED AS GLIOBLASTOMA AND HAS 20 YEARS SURVIVAL

2020 ◽  
Vol 22 (Supplement_3) ◽  
pp. iii349-iii349
Author(s):  
Yusuke Kobayashi ◽  
Yosuke Sato ◽  
Takashi Kon ◽  
Daisuke Tanioka ◽  
Katsuyoshi Shimizu ◽  
...  

Abstract Pediatric high-grade gliomas are rare and often hard to classify, which grow locally and show longer survival than diffuse high-grade gliomas in adults. We report a case of circumscribed high-grade astrocytoma who was initially diagnosed as glioblastoma and has 20 years survival. A 7-year-old girl suffered from epileptic seizure due to a left occipital lobe tumor. The tumor was resected in another hospital and diagnosed as glioblastoma. The tumor disappeared after extended local irradiation and chemotherapy using nimustine hydrochloride (ACNU) and cisplatin (CDDP). Eighteen years after initial onset, first recurrence was confirmed as the intra-tumoral hemorrhage. The tumor was resected and diagnosed as anaplastic oligoastrocytoma. After 6 courses of temozolomide (TMZ), the tumor disappeared. Twenty years after initial onset, the second local recurrence was confirmed. Although gamma knife and TMZ was performed, the tumor did not disappear. The tumor was surgically resected. Histopathology showed localized growth with some infiltration and mitosis but lacked pseudopallisading and microvascular proliferation. The tumor was diagnosed as circumscribed high-grade astrocytoma. Immunostaining revealed ATRX nuclear loss and CDKN2A / B homozygous deletion. After 10 courses of TMZ, the third local recurrence was confirmed. The tumor was completely removed and has not occurred recurrence more than 3 months after the last operation. Circumscribed high-grade glioma is expected to survive longer than invasive glioma. Pediatric gliomas should differ from adult gliomas in the genes of tumorigenesis. Care should be taken for its diagnosis and treatments. We also need a new classification based on histology and gene profile. HGG-30, ANALYSIS OF PEDIATRIC GLIOMAS IN OUR INSTITUTE Kaoru Tamura, Mai Fujioka, Masae Kuroha, Motoki Inaji, Yoji Tanaka, Tadashi Nariai, and Taketoshi Maehara; Tokyo Medical and Dental University, Tokyo, Japan. PURPOSE: Recent advances in genetic interrogation of pediatric glioma increase the importance of molecular diagnosis using surgical specimen. However, surgical resection may be avoided to preserve quality of life, especially in brain stem glioma cases. We retrospectively examined diagnosis and treatment of pediatric gliomas in our hospital. METHODS: This study includes 14 consecutive glioma patients under the age of 18 who underwent initial treatment at our hospital from 2000 to 2019. Histopathological diagnosis, clinical course and molecular status such as IDH, H3F3A and BRAF were analyzed. RESULTS: 5 patients (1 pilocytic astrocytoma (PA), 3 diffuse astrocytomas, 1 oligodendroglioma were treated only by surgical resection (group A). 7 patients (1 PA, 1 anaplastic oligodendroglioma, 2 diffuse midline gliomas and 3 glioblastomas (GBM)) received radiation and/or chemotherapy after surgical resection (group B). 2 diffuse intrinsic pontine gliomas (DIPG) received radiation and chemotherapy without surgical resection (Group C). No IDH mutation was observed in all pathological specimen obtained cases. BRAF alteration was observed in all PA cases. 1 case of GBM had BRAF V600Emutation and the other had H3K27M mutation. During a median of 7.7 years of follow-up, group A patients have no recurrence. Group B includes various diagnosis and prognosis. 2 group C patients diagnosed DIPG by MRI showed different clinical courses. CONCLUSION: Pediatric gliomas include diverse biological subgroups and show broad range of clinical behavior. Since pediatric glioma has a low incidence and a wide variety of genetic mutations, multicenter study is important to improve the treatment of pediatric glioma.

2003 ◽  
Vol 127 (7) ◽  
pp. 840-844 ◽  
Author(s):  
Athanase Billis ◽  
Luis A. Magna

Abstract Context.—Recently, prostatic atrophy associated with chronic inflammation has been linked to carcinoma either directly or indirectly by first developing into high-grade prostatic intraepithelial neoplasia. Objective.—The purpose of our study was to test this hypothesis in autopsies. Design.—A step section method was used to cut the posterior lobe in coronal planes at intervals of 0.3 to 0.5 cm in 100 consecutive autopsies of men older than 40 years. Prostatic atrophy was classified as simple, hyperplastic (or postatrophic hyperplasia), and sclerotic and was analyzed for the presence of chronic inflammation. Prostatic atrophy without (group A) and with inflammation (group B) was correlated with the following variables: age, race, histologic (incidental) carcinoma, high-grade prostatic intraepithelial neoplasia, and extent of both these lesions. Results.—Of the 100 prostates examined, 12%, 22% and 66%, respectively, had no atrophy, atrophy without inflammation (group A), and atrophy with inflammation (group B). There was no statistically significant difference between groups A and B for age (P = .55), race (P = .89), presence of histologic (incidental) carcinoma (P = .89), extensive carcinoma (P = .43), presence of high-grade prostatic intraepithelial neoplasia (P = .65), extensive high-grade intraepithelial neoplasia (P = .30), or subtypes of prostatic atrophy. Neither a topographical relation nor a morphologic transition was seen between prostatic atrophy and histologic carcinoma or high-grade intraepithelial neoplasia. Sclerotic atrophy either alone or combined with other subtypes was more frequent in the group with inflammation. A striking morphologic finding was a topographical relation of focal inflammation with sclerotic atrophy in areas with erosion of the epithelium. Conclusions.—Inflammatory prostatic atrophy does not appear to be associated with histologic (incidental) carcinoma or high-grade intraepithelial neoplasia. One possible cause of inflammatory infiltrate associated with prostatic atrophy may be the extravasated prostatic secretions, which were noted in areas of eroded epithelium, a common finding in the sclerotic type of prostatic atrophy.


Blood ◽  
2015 ◽  
Vol 126 (23) ◽  
pp. 5110-5110
Author(s):  
Haiwen Huang ◽  
Tianwen Fu ◽  
Qiangli Wang ◽  
Ting Xu ◽  
Xiaochen Chen ◽  
...  

Abstract Objectives Clinically, primary gastric diffuse large B cell lymphoma (PG-DLBCL) is not encountered commonly. The optimal treatment of PG-DLBCL remains controversial. Whether patients should receive surgical resection, Rituximab or not was most concerned about. Here we analized 83 patients with PG-DLBCL retrospectivly and evaluated the effect of surgical option and Rituximab in the treatment of PG-DLBCL. Methods From January 2009 to December 2014, 83 cases of PG-DLBCL patients in the First Affiliated Hospital of Soochow University were retrospectively studied. Forty cases received surgical resection plus chemotherapy (group A) and 43 patients underwent chemotherapy alone (group B). The operation mode is decided by the surgeon according to the patients¡¯ current condition and the chemotherapy regimens of two groups were CHOP or R-CHOP. Patients¡¯ characteristics were listed in Table 1. The main outcomes of overall survival (OS) and the progression free survival (PFS) were analized by using the Kaplan-Meier (K-M) method. Results The K-M analysis showed that the 3-year PFS and OS in group A were 66.7% and 68.4%, respectively. On the other hand, the 3-year PFS and OS of group B were 82.6%and 85.7%, respectively. There is no significant difference between the two groups. For patients received CHOP or R-CHOP, the 5-year OS were 77.7% and 78.2% (p=0.178). And the 3-year PFS were 74.9% and 75.5% (p=0.347). The difference between the two groups was not statistically significant. In group A, the 5-year PFS of R-CHOP group and CHOP group is 62.5% and 71.2% £¨p=0.747£©, the 5-year OS of R-CHOP group and CHOP group is 64.2% and 73.6% (p=0.853). In group B, the 5-year PFS of R-CHOP group and CHOP group is 83.4% and 81.8% £¨p=0.706£©, the 5-year OS of R-CHOP group and CHOP group is 85.7% and 83.5% (p=0.753). The univariate analyses indicated that age and lactate dehydrogenase (LDH) level were related to prognosis. Multivariate analysis of prognostic factors with a Cox model showed that IPI was the only independent prognostic factor. Conclusions This study shows that PG-DLBCL patients have a similar long-term survival rate when adopted surgery plus chemotherapy. Therefore, resection of the primary tumor before systemic chemotherapy does not improve the survival of the patients with PG-DLBCL. At the same time, the addition of Rituximab to chemotherapy doesn¡¯t make difference for the survival of PG-DLBCL. More prospective clinical trials about the effect of surgical operation and rituximab are needed to confirm the results of our study. Table 1. Patients¡¯ baseline characteristics Patients £¨%£© P value With surgical resection(Group A, n£½40£© Without chemotherapy (Group B, n £½ 43 £© Gender Male 19£¨47.5%£© 24£¨55.8%£© 0.449 Female 21£¨52.5%£© 19£¨44.2%£© Age ¡Ü60 15£¨37.5%£© 22£¨51.2%£© 0.211 £¾60 25£¨62.5%£© 21£¨48.8%£© Ann Arbor Stage I/II 13£¨32.5%£© 7£¨16.3%£© 0.084 Stage III/IV 27£¨67.5%£© 36£¨83.7%£© ECOG £¼2 19£¨47.5%£© 22£¨51.2%£© 0.739 ¡Ý2 21£¨52.5%£© 21£¨48.8%£© Treatment plan R-CHOP 23£¨57.5%£© 24£¨55.8%£© 0.887 CHOP 17£¨42.5%£© 19£¨44.2%£© LDH ¡Ü245 24£¨60.0%£© 27£¨62.8%£© 0.794 £¾245 16£¨40.0%£© 16£¨37.2%£© IPI ¡Ü2 13£¨32.5%£© 15£¨34.9%£© 0.818 £¾2 27£¨67.5%£© 28£¨65.1%£© ECOG: Eastern Cooperative Oncology Group; CHOP: cyclophosphamide, doxorubicin, vincristine, prednisone; R-CHOP: rituximab, cyclophosphamide, doxorubicin, vincristine, prednisone; LDH: lactate dehydrogenase Disclosures No relevant conflicts of interest to declare.


2014 ◽  
Vol 16 (suppl 2) ◽  
pp. ii64-ii64
Author(s):  
M. Riva ◽  
A. Comi ◽  
A. Casarotti ◽  
E. Fava ◽  
T. Alfiero ◽  
...  

2015 ◽  
Vol 12 (1) ◽  
pp. 19-30 ◽  
Author(s):  
Giuseppe MV Barbagallo ◽  
Stefano Palmucci ◽  
Massimiliano Visocchi ◽  
Sabrina Paratore ◽  
Giancarlo Attinà ◽  
...  

Abstract BACKGROUND Intraoperative magnetic resonance imaging is the gold standard among image-guided techniques for glioma surgery. Scant data are available on the role of intraoperative computed tomography (i-CT) in high-grade glioma (HGG) surgery. OBJECTIVE To verify the technical feasibility and usefulness of portable i-CT in image-guided surgical resection of HGGs. METHODS This is a retrospective series control analysis of prospectively collected data. Twenty-five patients (Group A) with HGGs underwent surgery using i-CT and 5-aminolevulinic acid (5-ALA) fluorescence. A second cohort of 25 patients (Group B) underwent 5-ALA fluorescence–guided surgery but without i-CT. We used a portable 8-slice CT scanner and, in both groups, neuronavigation. Extent of tumor resection (ETOR) and pre- and postoperative Karnofsky performance status (KPS) scores were measured; the impact of i-CT on overall survival (OS) and progression-free survival (PFS) was also analyzed. RESULTS In 8 patients (32%) in Group A, i-CT revealed residual tumor, and in 4 of them it helped to also resect pathological tissue detached from the main tumor. EOTR in these 8 patients was 97.3% (96%-98.6%). In Group B, residual tumor was found in 6 patients, whose tumor's mean resection was 98% (93.5-99.7). The Student t test did not show statistically significant differences in EOTR in the 2 groups. The KPS score decreased from 67 to 69 after surgery in Group A and from 74 to 77 in Group B (P = .07 according to the Student t test). Groups A and B did not show statistically significant differences in OS and PFS (P = .61 and .46, respectively, by the log-rank test). CONCLUSION No statistically significant differences in EOTR, KPS, PFS, and OS were observed in the 2 groups. However, i-CT helped to verify EOTR and to update the neuronavigator with real-time images, as well as to identify and resect pathological tissue in multifocal tumors. i-CT is a feasible and effective alternative to intraoperative magnetic resonance imaging. Portable i-CT can provide useful real-time information during brain surgery and can be easily introduced in neurosurgical theaters in daily practice.


2013 ◽  
Vol 31 (15_suppl) ◽  
pp. e21502-e21502
Author(s):  
Rajeev Rajendra ◽  
Seth Pollack ◽  
Eve T. Rodler ◽  
Ernest U. Conrad ◽  
Darin J Davidson ◽  
...  

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.


2019 ◽  
Vol 37 (4_suppl) ◽  
pp. 675-675
Author(s):  
Kate Jessica Wilkinson ◽  
Sharlyn Kang ◽  
Stephanie Hui-Su Lim ◽  
Cheok Soon Lee ◽  
Ray Asghari ◽  
...  

675 Background: Consensus international guidelines recommend the use of neoadjuvant chemo-radiotherapy in patients with stage II-III rectal cancer. Despite this, due to factors including inaccurate/under-staging, patient co-morbidities and acute presentations, a proportion will undergo up-front surgical resection. The survival benefit of adjuvant therapy is unclear in this real world, non-trial population. Methods: A retrospective analysis of patients presenting with stage II-III rectal adenocarcinoma in South Western Sydney and Illawarra Shoalhaven Health Districts, Australia, between 2006 to 2015 was performed. Data was extracted from electronic health records, with institutional ethics approval. Treatment modalities, clinicopathological, recurrence and survival data were analyzed. The primary endpoint was overall survival (OS) by treatment modality. Results: 549 patients were identified, of which 295 (54%) underwent up-front surgical resection without neoadjuvant therapy. Of this cohort, 137 (46%) had no adjuvant therapy (Group A), 103 (35%) had adjuvant chemotherapy alone (Group B), and 55 (19%) had adjuvant radiotherapy +/- chemotherapy (Group C). Receipt of any adjuvant treatment was significantly associated with improved OS (5 year OS 56 vs. 79%, HR 0.44, 95% CI 0.3 – 0.6, p < 0.0001) and recurrence free survival (5 yr RFS 25% vs. 47%, HR 0.66, 95% CI 0.5 – 0.9, p=0.01), but not cancer specific survival (5yr CSS 75 vs. 80%, HR 0.78, 95% CI 0.5 – 1.3, p = 0.30). Group B had improved OS compared to Group A (5 yr OS 56% vs. 80%, HR 0.35, 95% CI 0.22 – 0.55, p < 0.0001). There was a trend to improved OS in Group C vs. Group A (5yr OS 56.0% vs. 69.2%, HR 0.79 95% CI 0.6 – 1.01, p = 0.052). The improved OS in Group B versus Group A remained significant in multivariate analysis (HR 0.41, 95% CI 0.22 – 0.77, p = 0.005). Conclusions: Adjuvant chemotherapy improved OS in this real world cohort, and there was a trend to a benefit with adjuvant chemo-radiotherapy. However, the lack of difference in cancer specific survival suggests that this benefit may be partly driven by patient selection bias. Further exploratory analyses to identify sub-groups deriving a cancer specific survival benefit are required.


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