Acute lymphoblastic leukemia subtypes in israel: The sheba medical center experience

During the period from 1978 to 1981, 52 patients with ALL were diagnosed and treated at the Chaim Sheba Medical Center. Using standard cell markers to subtype the blasts, 49 of the patients could be classified: 16 were found to be T-cell ALL, 10 common ALL, five null ALL, four pre-B and 14 were partially characterized as non-B, non-T. Analysis of the series revealed two distinctive features: high prevalance (30%) of T-cell ALL among both Jews and Arabs and a high proportion, two-thirds, of high risk patients due to high initial WBC counts, unfavourable age or T-cell characteristics.The minimal incidence of ALL among the Gaza Strip Arab children during the study period is 4: 100,000, which is close to the incidence in the Western world. During previous years the leukemia incidence in the Gaza Strip was very low while the most common lymphatic malignancies were Burkitt tumor and other non-Hodgkin lymphomas.     

The microbiome in pediatric oncology

The human microbiome comprises a diverse set of microorganisms, which play a mostly cooperative role in processes such as metabolism and host defense. Next-generation genomic sequencing of bacterial nucleic acids now can contribute a much broader understanding of the diverse organisms composing the microbiome. Emerging evidence has suggested several roles of the microbiome in pediatric hematology/oncology, including susceptibility to infectious diseases, immune response to neoplasia, and contributions to the tumor microenvironment as well as changes to the microbiome from chemotherapy and antibiotics with unclear consequences. In this review, the authors have examined the evidence of the role of the microbiome in pediatric hematology/oncology, discussed how the microbiome may be modulated, and suggested key questions in need of further exploration.     

Orbital rhabdomyosarcoma: Multidisciplinary treatment experience

Orbital rhabdomyosarcoma (RMS) accounts for 10% of childhood RMS and has a relatively good prognosis of up to 85% 5‐year survival. Improved survival has led to increased interest in late effects of treatment. The objective of this study was to review the results of treating orbital RMS with multidisciplinary treatment at Women's and Children's Hospital and Royal Adelaide Hospital with emphasis on late effects of treatment. A retrospective review was carried out of all patients with orbital RMS treated with multidisciplinary treatment including radiation therapy and chemotherapy in the two institutions between 1982 and 2002. A total of five patients (age range 5.5‐12 years) satisfied the eligibility requirements. Late effects were significant and included facial bone hypoplasia, cataract formation and growth hormone deficiency. Overall survival was 80% (4/5) with mean follow up of 8 years (range 3‐13 years). Given the high cure rates achieved, future treatments must aim to maintain the good results but to reduce the high incidence of late effects of treatment. Standardised rating of late toxicity, long‐term follow‐up clinics, and implementation of modern radiation techniques (3‐D conformal radiotherapy, intensity modulated radiotherapy, proton therapy) for patients with orbital RMS are important to improving outcome.     

胃肠道间质肿瘤的诊治体会

目的:探讨胃肠道间质肿瘤的诊断与治疗。方法:回顾2003年~2004年我院收治23例经病理证实的胃肠道间质肿瘤的临床资料。结果:23例均行手术治疗。病理:CD34阳性20例(87.0%),CD117阳性20例(87.0%)。.结论:胃肠道间质肿瘤好发中老年人,常以腹痛和腹部不适、腹部肿块,呕血黑便为主要表现。肿瘤大小是判断良恶性重要指标之一。完整的局部切除或扩大切除是有效的手段。对无法切除或肿瘤残留者可应用甲磺酸伊马替尼(格列卫)进行治疗。     

Pretreatment and routine echocardiogram monitoring during chemotherapy for anthracycline-induced cardiotoxicity rarely identifies significant cardiac dysfunction or alters treatment decisions: a 5-year review at a single pediatric oncology center

BACKGROUND:

The widespread use of anthracycline chemotherapy has contributed to improved outcomes in children with cancer. The most feared complication of the anthracyclines is cardiotoxicity. Routine echocardiographic monitoring typically is used before, during, and after treatment to minimize cardiotoxicity. The ideal use of screening before and during chemotherapy remains uncertain.

METHODS:

This was a retrospective review of children who were treated at a single cancer treatment center over 5 years. The results of all echocardiograms and related clinical decisions were reviewed.

RESULTS:

In 356 patients who were identified for review (age range, 3 months to 22 years; mean age, 10 years; median age, 11 years), 991 echocardiograms were reviewed (average, 2.78 echocardiograms per patient; median, 2 echocardiograms per patient; mode, 1; maximum, 11 echocardiograms per patient). Nine abnormal echocardiograms were identified (2.5% of patients and 0.9% of echocardiograms performed). Four echocardiograms were performed during episodes of septic shock, 2 echocardiograms represented false‐positive studies after repeat evaluation, and 1 echocardiogram demonstrated mild abnormality of function on the day of surgical resection of a large Wilms tumor. None of the 356 pretreatment echocardiograms altered treatment decisions. In 635 follow‐up echocardiograms during treatment, cardiac defects were detected in 2 patients (0.5%).

CONCLUSIONS:

The routine use of echocardiograms to screen for anthracycline‐induced cardiac damage before and during chemotherapy rarely identified significant cardiac damage to impact treatment decisions. Improved screening techniques with better discrimination and predictability are needed. Pediatric Oncology cooperative groups should consider a revision of standard monitoring protocols before and during treatment. Cancer 2012;. © 2011 American Cancer Society.     

Primary gastrointestinal lymphoma: 30-year experience at the cancer institute,madras, india

A retrospective study was conducted of 49 patients with primary gastrointestinal lymphoma to analyze clinicopathological features, prognostic factors, and results of treatment. Intestinal lymphomas (63%) were more common than gastric lymphomas (29%). Endoscopic biopsy was diagnostic in 64% of gastric lymphomas, while 97% of intestinal lymphomas required laparotomy for diagnosis. Lymphoblastic (35%) and diffuse large cell (33%) lymphomas were the most frequent histologic types. Complete remission (CR) was achieved in 61% of patients, with a 5-year survival of 72% in those who achieved CR. The overall 5-year survival and disease-free survival were 47% and 40%, respectively. All disease-related mortality occurred within 2 years of diagnosis, with 79% occurring in the first year. There was no overall significant difference in survival between the different treatment groups. Stage, extent of surgical resection, response to treatment, serosal involvement, multimodality treatment, and performance status were significant prognostic factors. Achievement of complete remission and complete surgical resection were the most favorable independent prognostic factors on multivariate analyses. © 1995 Wiley-Liss, Inc.     

Retinoblastoma: Review of 30 years’ experience with external beam radiotherapy

A review of the experience at the Peter MacCallum Cancer Centre (Peter Mac), Melbourne, Australia in treating retinoblastoma with external beam radiotherapy was conducted. Outcomes of particular interest were tumour control, vision preservation and treatment late effects. The review was restricted to patients that had intact eyes treated at Peter Mac from 1965 until 1997 with at least 2 years of follow up. Histories were reviewed regarding patient and tumour characteristics and treatment details. Thirty‐five patients were identified in whom 47 eyes were treated. Of the tumours, 47% were Reese?Ellsworth stage IV or V and the majority of others were at high risk for vision loss because of tumour location. The radiation treatment technique became increasingly sophisticated during the study period. Radiation dose and fraction size have similarly evolved but most patients received 30?50 Gy. Since 1989, a highly accurate contact lens immobilization technique has been used to deliver 40 Gy in 20 fractions. Thirteen eyes required additional local therapy. Of the treated eyes, 34 (72%) remain intact and 74% of these have useful vision. One patient died from retinoblastoma and three from second malignant neoplasms. With modern radiotherapy, late toxicities other than growth arrest and non‐progressive cataract did not occur during the study period. Tumour control was high and a very acceptable rate of organ and vision preservation was achieved in a relatively high‐risk population. Modern radiotherapy continues to develop in an attempt to improve treatment accuracy and minimize late radiation toxicity.     

Chemotherapy for neuroendocrine tumours: The beatson oncology centre experience

The role of chemotherapy in malignant neuroendocrine tumours is difficult to assess because of their rarity and variation in biological behaviour. We present a retrospective review of chemotherapy given to 18 patients with metastatic and one with locally advanced neuroendocrine tumours. There were eight poorly differentiated neuroendocrine tumours, six thyroid medullary carcinomas, two phaeochromocytomas, two pancreatic islet cell tumours and one undifferentiated neuroblastoma. Four patients were given 3-weekly dacarbazine, vincristine and cyclophosphamide (DOC) chemotherapy. In eight patients, this regimen was modified by substituting the dacarbazine and cisplatin and etoposide (OPEC). A further six patients were treated with dacarbazine reintroduced into the 3-weekly regimen (DOPEC). The remaining patient received cisplatin and etoposide.There were two complete responses (both with OPEC) and eight partial responses (two with DOC, three with OPEC and three with DOPEC). Five patients had stable disease and four progressed. Four received further chemotherapy on relapse, producing one complete and one partial response. The median response duration to initial chemotherapy was 10 months (range 3–34). The median survival was 12 months (range 1–42). The main toxicity was haematological, with grade 3–4 neutropenia in 12 patients; eight suffered episodes of sepsis. One death was treatment related. Other toxicity was mild although three patients discontinued vincristine with grade 2 neurotoxicity.The response rate and side effects of these three regimens appear comparable. We conclude that, although these patient numbers are small, combination chemotherapy produces an encouraging response rate (53%; 95% CI 30–75) in malignant neuroendocrine tumours, with acceptable toxicity.     

Molecular predictive testing in precision oncology: The Italian experience

In this review, we describe molecular pathology testing to predict response to targeted treatment of solid tumors, focusing on Italian routine clinical practice. The combination of the universal health care system organized at national, regional, and local levels has led a decentralized model, with a large number of local laboratories performing in-house molecular testing following guidelines issued and external quality assessment organized by the Italian Society of Pathology and Cytopathology–Italian Division of the International Academy of Pathology. In this framework, in the early days of predictive testing, sponsored informatics platforms support to set up national programs that aimed to integrate the activity of oncologists and pathologists to test cancer patients for druggable alterations. More recently, reimbursement for molecular testing is being covered completely by the Italian National Health Service. In the near future, considering the development of complex technologies, we expect that outsourcing samples to next-generation sequencing referral laboratories will take place.     

手术切除肝脏巨大肿瘤63例经验总结

目的:探讨手术治疗肝脏巨大肿瘤的可行性及疗效。方法:自2000年1月～2005年6月间行手术治疗63例巨大肝脏肿瘤(其中原发性肝癌53例),采用常温下间歇性肝门阻断,阻断时间6～48分钟。结果:本组肝切除术取得较好疗效,原发性肝癌患者的1、3、5年生存率分别为63.4%、36.5%和15.4%,8例出现明显的并发症,仅1例(1.59%)术后20天死于肝功能衰竭。结论:如能掌握好手术适应证和手术技巧,手术治疗肝脏巨大肿瘤是安全和有效的。     

Successful ambulatory treatment of Hodgkin's disease in Iranian children based on German-Austrian DAL-HD 85-90: single institutional results.

BACKGROUND: Hodgkin's disease (HD) accounts for 7.5% of childhood malignancies in Iran. In order to minimize chemotherapy toxicity and avoid eventual hospitalization and psychological and financial burdens we have applied since 1988, for the first time in Iran, a treatment regimen based on subsequently revised DAL-HD 85-90 and later GPOH-HD 95 protocols. PATIENTS AND METHODS: During the period 1988-2004, 40 children with HD received DAL/GPOH-HD-adapted treatment; 25 males (62.5%) and 15 females (37.5%) (male/female ratio 1.7; age 4-14 years, mean 8.8). Clinical evaluation and staging was performed in all patients. Constitutional symptoms: 24 patients were asymptomatic (A; 60%) and 16 had constitutional complaints (B; 40%). Staging was as follows: stage I; seven (17.5%); II, 11 (27.5%); III, 11 (27.5%); and IV, 11 (27.5%). Histopathology: 22 patients had mixed cellularity (MC; 55%), 13 nodular sclerosis (32.5%), four lymphocyte predominance (LP; 10%) and one patient lymphocyte depletion (2.5%). Stage IA and IIA patients (n = 15) received either OPA x2 (vincristine, prednisolone, doxorubicin) or OPPA x2 or OPEA x2 (vincristine, prednisolone, procarbazine and doxorubicin), the latter receiving etoposide instead of procarbazine, and applied to males. Stages IIB, IIIA/B and IV received OPPA x2, followed by CO(P)P x4 (cyclophosphamide, vincristine, prednisolone in alternate courses and procarbazine). Twenty nine patients (72.5%) received radiotherapy (20-25 Gy); four to the involved field (stage I), 25 to the upper mantel (stage II and also III with either residual or mediastinal mass) and three additionally to spleen and para-aortic lymph nodes. Eleven patients received only chemotherapy. RESULTS: All patients achieved complete remission (CR). Relapse occurred in eight patients (20%); seven stage IV (MC) and one stage IA (LP) with progression to IIIB. Salvage chemotherapy consisted of MOPP/ABVD hybrid; six patients achieved a second sustained remission and three patients died: two due to relapse and progressive disease and the third one in CR, owing to thrombocytopenic hemorrhage and foudroyant pneumonia. The achieved overall and event-free survival was 88.1% and 75.4%, respectively. Aside from minor acute toxicities, three patients demonstrated azoospermia at the age of 18 years and one of these patients suffered non-Hodgkin lymphoma as a second malignancy. HD occurred as a second malignancy in two patients with acute lymphoblastic leukemia. Both received appropriate treatment and are over 10 years in CR. CONCLUSIONS: The DAL/GPOH-HD-based treatment approach proved to achieve long-term sustained cure even in children with advanced HD disease. The essentially outpatient diagnosis and treatment modus did not compromise the disease outcome, and was well tolerated and accepted by the patients and their parents. The employed drugs are easily available and affordable. This treatment approach is suitable for ambulatory use in developing countries. However, male infertility remains the major obstacle to procarbazine and cyclophosphamide use.     

Treatment results for gastric cancer surgery: 12 years’ experience at a single institute in Korea

Aims

To evaluate the changing trends of clinicopathologic features, surgical procedures and treatment outcomes of gastric cancer in a large-volume center.

Methods

We divided the time period into two parts: the first is 1989–1996 (period I) and the second is 1997–2001 (period II). Then we analyzed prospectively collected data on 1816 patients treated at Kangnam St. Mary's Hospital, The Catholic University of Korea, from 1989 to 2001.

Results

Upper one-third cancer was seen more prevalently in period II than period I (9.4% versus 6.6%) (p = 0.000) and total gastrectomy was performed more frequently in period II than period I (25% versus 18%) (p = 0.000). A diagnosis of early gastric cancer was made more prevalently in period II than period I (40% versus 27%) (p = 0.000). D2 lymphadenectomy was done in 74% of the period I patients and 83% of their period II counterparts (p = 0.000). Between the two periods, there was a significant difference in the incidence of operation-related major complications (9.9% in period I versus 3.9% in period II) (p = 0.000) and the mortality (1.8% versus 0.6%) (p = 0.023). The overall 5-year and 10-year survival rates were significantly higher in period II than period I (63% and 57% in period I versus 69% and 64% in period II) (p = 0.009).

Conclusions

The overall survival of gastric cancer significantly increased because of the early detection and aggressive surgical approaches by experienced surgeons in a large-volume center. More effective multidisciplinary approaches are warranted to improve the prognosis of advanced gastric cancer.     

Precision oncology based on omics data: The NCT Heidelberg experience

Precision oncology implies the ability to predict which patients will likely respond to specific cancer therapies based on increasingly accurate, high‐resolution molecular diagnostics as well as the functional and mechanistic understanding of individual tumors. While molecular stratification of patients can be achieved through different means, a promising approach is next‐generation sequencing of tumor DNA and RNA, which can reveal genomic alterations that have immediate clinical implications. Furthermore, certain genetic alterations are shared across multiple histologic entities, raising the fundamental question of whether tumors should be treated by molecular profile and not tissue of origin. We here describe MASTER (Molecularly Aided Stratification for Tumor Eradication Research), a clinically applicable platform for prospective, biology‐driven stratification of younger adults with advanced‐stage cancer across all histologies and patients with rare tumors. We illustrate how a standardized workflow for selection and consenting of patients, sample processing, whole‐exome/genome and RNA sequencing, bioinformatic analysis, rigorous validation of potentially actionable findings, and data evaluation by a dedicated molecular tumor board enables categorization of patients into different intervention baskets and formulation of evidence‐based recommendations for clinical management. Critical next steps will be to increase the number of patients that can be offered comprehensive molecular analysis through collaborations and partnering, to explore ways in which additional technologies can aid in patient stratification and individualization of treatment, to stimulate clinically guided exploratory research projects, and to gradually move away from assessing the therapeutic activity of targeted interventions on a case‐by‐case basis toward controlled clinical trials of genomics‐guided treatments.     

骨巨细胞瘤的地舒单抗药物治疗

骨巨细胞瘤 ( giant cell tumor of bone,GCTB ) 是一种交界性的原发骨肿瘤,在临床上,具有局部侵袭性,可出现局部复发和远处转移 [1-2].GCTB 的发病率在不同国家和地区并不相同,在欧美,GCTB 的发病率为每年 1.03～1.17 /百万人 [3],我国的发病率明显高于欧美人群,约为...