PS02.138: SALVAGE ESOPHAGECTOMY: SINGLE ONCOLOGICAL CENTER EXPERIENCE

2018 ◽  
Vol 31 (Supplement_1) ◽  
pp. 159-160
Author(s):  
Flávio Sabino ◽  
Marco Guimaraes ◽  
Carlos Eduardo Pinto ◽  
Daniel Fernandes ◽  
Luis Felipe Pinto ◽  
...  
Keyword(s):  
Overall Survival ◽  
Esophageal Cancer ◽  
Conflicts Of Interest ◽  
High Volume ◽  
Published Data ◽  
Surgical Morbidity ◽  
Salvage Esophagectomy ◽  
Thoracic Approach ◽  
Clinical Conditions ◽  
Local Advanced

Abstract Background Surgical resection is considering the gold standard in esophageal cancer treatment, with 15–40% cure global rates. Radical exclusive chemoradiotherapy (CRT) is used in patients with local advanced esophageal cancer or without clinical conditions for esophagectomy, with a 5-year overall survival up to 30%. However, locoregional control is poor with a 40–60% recurrence rate and salvage esophagectomy maybe an option for these patients. Methods Our objective is to report the experience of a single high volume oncological institution with salvage esophagectomy. Retrospective analysis of 28 patients medical records, with esophageal cancer, submitted to Salvage Esophagectomy in Brazilian NCI after radical exclusive CRT or RT between January 1990 and December 2015. Results Median age was 56 years and most are male (78,5%). Esophageal middle third was the tumor principal location (50%) and histological type was squamous cell carcinoma (82%). Thoracic approach for esophagectomy was the principal surgical technique, and gastric tube the most used conduit for reconstruction (78,5%). Surgery was R0 in 83% of the cases, with a surgical morbidity of 64%. Median hospital time was 15 days (8–58) and surgical mortality 14% (4 patients), with 7% in the first 30 days. Median overall survival was 22,3 months. Conclusion Our results are in line with published data in the literature. Besides surgical morbidity and mortality, Salvage Esophagectomy remains de only chance of cure for patients with locoregional recurrence after radical exclusive CRT. Disclosure All authors have declared no conflicts of interest.

PS01.184: ESOPHAGEAL CANCER SURGICAL TREATMENT IN BRAZILIAN NATIONAL CANCER INSTITUTE: 25 YEARS EXPERIENCE

2018 ◽  
Vol 31 (Supplement_1) ◽  
pp. 102-102
Author(s):  
Marco Antonio Guimaraes Filho ◽  
Flávio Sabino ◽  
Daniel Fernandes ◽  
Carlos Eduardo Pinto ◽  
Luis Felipe Pinto ◽  
...  
Keyword(s):  
Overall Survival ◽  
Esophageal Cancer ◽  
Surgical Treatment ◽  
Surgical Resection ◽  
National Cancer Institute ◽  
Conflicts Of Interest ◽  
Morbidity Rate ◽  
Surgical Mortality ◽  
Surgical Morbidity ◽  
Hospitalization Time

Abstract Background Esophageal cancer is the 8th most common cancer in the world. It is an lethal disease, responsible for almost 400.000 deaths by year. Surgical resection is considered the gold standard in esophageal cancer treatment, with a global 15–40% cure rate. In this study, the results of esophageal cancer surgical treatment at Brazilian National Cancer Institute, Abdominal-pelvic Surgical Section, is analyzed. Methods The medical records of 215 patients with esophageal cancer, treated with surgical resection (esophagectomy), between January 1999 and December 2015, were retrospectively studied. The endpoints analyzed in the study were: hospitalization time, operative complications and mortality, and overall survival. Results Esophageal cancer was predominant in male patients; median age was 58 years (27–78). Primary tumor location varied between 7,5 - 41 cm (median 32cm) and tumor extension 1 - 16cm (median 5cm). Median surgical time was 330 minutes (120–720); transhiatal esophagectomy with gastric tube reconstruction was the most used surgical approach. Tumors histopathological types were equaly distributed. ICU (Intensive Care Unit) stay median time was 5 days (1–87) and median hospitalization time was 15 days (5–166). Most common surgical complications were anastomotic leakage (25,5%) and pneumonia (20%), with a surgical morbidity rate of 61,8%. Surgical mortality rate was 12%, with 61% of these cases occuring in the 30 days after surgery. Median 2-year overall survival was 44,3 months. Conclusion Besides the high surgical morbidity, esophagectomy for esophageal cancer remains the standard treatment for patients with ressectable tumors and without clinical contraindications for surgery. Reduction of surgical mortality depends on rigorous patients selection, surgical team expertise and adequate perioperative and postoperative care. Disclosure All authors have declared no conflicts of interest.

RA02.08: SALVAGE LYMPHADENECTOMY FOR 14 PATIENTS OF ESOPHAGEAL CANCER AFTER DEFINITIVE CHEMORADIOTHRAPY

2018 ◽  
Vol 31 (Supplement_1) ◽  
pp. 22-22
Author(s):  
Kazuki Odagiri ◽  
Makoto Yamasaki ◽  
Koji Tanaka ◽  
Yasuhiro Miyazaki ◽  
Tomoki Makino ◽  
...  
Keyword(s):  
Overall Survival ◽  
Esophageal Cancer ◽  
Lymph Nodes ◽  
Conflicts Of Interest ◽  
Treatment Guideline ◽  
Hospital Death ◽  
Curative Surgery ◽  
Term Outcome ◽  
Long Term Outcome ◽  
Salvage Lymphadenectomy

Abstract Background Salvage Lymphadenectomy is regarded as the only curative surgery to residual or recurrence lymph nodes of esophageal cancer after definitive chemoradiotherapy (dCRT). However, salvage lymphadenectomy is not described in the Japanese esophageal cancer treatment guideline because of little evidences for the safety and efficacy. Methods From January 2011 to December 2015, we performed 14 salvage lymphadenectomies to residual or recurrence LN of esophageal squamous cell carcinoma(ESCC) in Osaka University. We assessed postoperative complications and long-term outcome. Results Average age was 64 year-olds (SD: 5.2). Male: Female = 11: 3. cStage I: II-IV = 7: 7. Surgery to cervical LN were 11 patients and abdominal LN were 3 patients. Surgery to residual LN (res-LN) were 9 patients and recurrence LN (rec-LN) were 5 patients. rec-LN patient's median time to recurrence after dCRT was 14.3 months (10.2–29.3). 4 patients were performed lymphadenectomy resecting with adjacent organs, 3 patients were bronchus (trachea? ) and 1 patient was right subclavian artery. 4 patients had postoperative complication, two were pneumonia, one was pulmonary thrombosis and one was lymphorrhea, but there was no serious case (Clavien-Dindo Grade II or less). We didn’t have hospital death. Six of 14 patients had recurrence and died after salvage lymphadenectomy. Recurrence sites were 2 mediastinal lymph nodes and liver, lung, loco-regional and peritoneal. But no patients had recurrence of main tumor. 5-year overall survival rate was 51.1%. Median survival time in 9 patients, surgery to res-LN, was 18.9 months (10.4–132 months) and 5 patients, surgery to rec-LN, was 4.9 months (1.4–26.6 months). Surgery to res-LN patients were longer than rec-LN patients in overall survival after salvage lymphadenectomy (P = 0.395). There was no difference due to the difference in recurrence site of the cancer in overall survival after salvage lymphadenectomy. Conclusion Our data show salvage lymphadenectomy safety and effectiveness after dCRT. Salvage lymphadenectomy may extend the prognosis of patients with esophageal cancer after dCRT. Thus, salvage lymphadenectomy may be one of the treatment options for the patients with residual or recurrent, especially the former, lymph node after definitive CRT, although it is necessary to evaluate in many cases. Disclosure All authors have declared no conflicts of interest.

PS01.236: MEDIASTINOSCOPIC SALVAGE ESOPHAGECTOMY FOR RECURRENT ESOPHAGEAL CANCER AFTER DEFINITIVE CHEMORADIOTHERAPY IN A PREVIOUSLY PNEUMONECTOMIZED PATIENT

2018 ◽  
Vol 31 (Supplement_1) ◽  
pp. 116-117
Author(s):  
Tomoyuki Okumura ◽  
Yasuyuki Seto ◽  
Susumu Aikou ◽  
Makoto Moriyama ◽  
Shinich Sekine ◽  
...  
Keyword(s):  
Esophageal Cancer ◽  
Tumor Recurrence ◽  
Conflicts Of Interest ◽  
Primary Lung Cancer ◽  
Esophageal Wall ◽  
Thoracic Esophagus ◽  
Definitive Chemoradiotherapy ◽  
Transhiatal Approach ◽  
Salvage Esophagectomy ◽  
Sealing Device

Abstract Background Mediastinoscopic esophagectomy is a minimally invasive surgery for thoracic esophageal cancer avoiding one-lung ventilation or transthoracic procedure. Methods We performed for the first time in the literature, salvage esophagectomy with combination of mediastinoscopic cervical approach and laparoscopic/mediastinoscopic transhiatal approach for recurrent thoracic esophageal squamous cell carcinoma (ESCC) after definitive chemoradiotherapy (dCRT) in a patient who had previously undergone a left pneumonectomy for primary lung cancer. Results A 66-year-old man was diagnosed as local recurrence of lower ESCC (cT3N0M0 cStage II) at 9 years after dCRT. His medical history included left-sided pneumonectomy for lung adenocarcinoma 9 years previously. Then the patient was diagnosed as lower thoracic ESCC (cT3N1M0 cStage III) at 2 months after pneumonectomy. He received dCRT consisting of CDDP/5-FU infusion and irradiation (60 Gy) and achieved complete response. No evidence of tumor recurrence was observed at endoscopic surveillance up until 6 years after dCRT. For this present surgery, a cervical wound was made and the intramediastinal procedure was performed under pneumomediastinum. After mobilization of upper/middle thoracic esophagus, the esophageal wall was safely separated from the remaining part and the stump of the left main bronchus. Dense adhesions between the esophagus and fibrotic tissue at the site of previous left mediastinal pleural resection was divided using a sealing device. In the abdomen, 5 ports were inserted to perform abdominal and transhiatal procedures under CO2 insufflation. After mobilization of the stomach, fibrotic scar tissue around the lower esophagus was divided using a sealing device and the peri-esophageal space dissected from cervical and transhiatal approach were connected to completely mobilize the thoracic esophagus. The esophagectomy was uneventfully carried out followed by reconstruction with gastric conduit via retrosternal rout. Pathological findings demonstrated a moderately differentiated ESCC (pT3-AD pN1 M0 pStage III), indicating that R0 resection was successfully performed. The patient has been closely observed as an outpatient and was alive and healthy at 3 months after the operation without tumor recurrence. Conclusion Mediastinoscopic esophagectomy is a safe and curative treatment strategy for esophageal cancer patients who had a previous pneumonectomy, even in salvage surgery for recurrent cancer after dCRT. Disclosure All authors have declared no conflicts of interest.

PS02.080: CLINICAL SIGNIFICANCE OF THE NUMBER OF PERITUMORAL LYMPHATIC VESSELS AFTER CHEMOTHERAPY IN ESOPHAGEAL CANCER PATIENTS

2018 ◽  
Vol 31 (Supplement_1) ◽  
pp. 143-143
Author(s):  
Takeo Hara ◽  
Tomoki Makino ◽  
Makoto Yamasaki ◽  
Koji Tanaka ◽  
Yasuyuki Miyazaki ◽  
...  
Keyword(s):  
Overall Survival ◽  
Esophageal Cancer ◽  
Primary Tumor ◽  
Conflicts Of Interest ◽  
Locally Advanced ◽  
Lymphatic Vessels ◽  
Tumor Location ◽  
Resected Specimen ◽  
Long Term Outcome ◽  
Cutoff Value

Abstract Background Neoadjuvant chemotherapy (NAC), a standard treatment for locally-advanced esophageal cancer, often achieves significant antitumor effect as clinically or microscopically confirmed. However, how chemotherapy histologically impacts upon normal tissues, in particular lymphatic vessels, adjacent to a tumor remains unclear. Methods A total of 137 patients who underwent curative esophagectomy with (NAC group n = 62)/without (nonNAC group n = 75) NAC for thoracic esophageal cancer in our department from 2004 to 2012 were analyzed. The number of lymphatic vessels (NLV) adjacent to primary tumor (within 1000μm from the edge of tumor) in lamina propria mucosae layer was assessed by immunostaining of D2–40 and its association with clinico-pathological parameters was analyzed. Results The NLV was significantly lower in the NAC group as compared with the nonNAC group (NAC vs nonNAC; 19.1 ± 9.0 vs 22.8 ± 8.6, P = 0.014). In the nonNAC group, when classified into two (high vs low NLV) groups by using the cutoff value of the median NLV in nonNAC group, NLV did not correlated with any clinico-pathological factors including age, gender, tumor location, pT, pN, pM, ly, v, and overall survival. On the other hand, in the NAC group, high NLV (classified by the same cutoff value as noted above) was significantly associated with good histological response (grade1b-2) (high vs low NLV; 52 vs 26%, P = 0.026) and less development of lymph node recurrence (16 vs 40%, P = 0.029) but not with other parameters including age, gender, tumor location, pT, pN, pM, ly, and v. Notably, the high NLV group showed the more favorable 5-year overall survival compared to the low NLV group (61 vs 49%, P = 0.0041). Multivariate analysis of overall survival further identified low NLV (HR = 3.68, 95%CI 1.54–10.83, P = 0.0005) to be one of independent prognostic factors along with pT(HR = 2.87, 95%CI 1.37–6.35, P = 0.0050) and pN(HR = 4.04, 95%CI 1.53–13.89, P = 0.0034) in the NAC group. Conclusion NAC might decrease the number of lymphatic vessels adjacent to primary tumor in resected specimen, and this number was associated with tumor response to NAC and long-term outcome in patients who underwent NAC plus surgery. Disclosure All authors have declared no conflicts of interest.

Lymph Node Harvest as a Measure of Quality and Effect on Overall Survival in Esophageal Cancer: A National Cancer Database Assessment

2019 ◽  
Vol 85 (2) ◽  
pp. 201-205 ◽  
Author(s):  
Mark J. Dudash ◽  
Sasha Slipak ◽  
James Dove ◽  
Marie Hunsinger ◽  
Jeffrey Wild ◽  
...  
Keyword(s):  
Overall Survival ◽  
Esophageal Cancer ◽  
Lymph Node ◽  
Private Insurance ◽  
High Volume ◽  
Academic Programs ◽  
Preoperative Radiation ◽  
National Cancer Database ◽  
Cure Rate ◽  
Extensive Lymphadenectomy

Surgical therapy for esophageal cancer is the cornerstone of treatment, and the highest quality operation should lead to the highest cure rate. Evaluated lymph node (ELN) count is one quality measure that has been championed. The objective of this study was to explore ELN in esophagectomy, examine predictors of harvesting ≥12 nodes, and determine whether higher ELN improves overall survival (OS). ELN was examined in patients with resected esophageal cancer using the National Cancer Database from 2004 to 2013. In this study, 41,746 patients met the inclusion criteria. Fifty-two per cent of patients had 12 or more nodes harvested. Academic programs were most likely to harvest ≥12 nodes (58% of cases) compared with other programs (43–56% of cases). Seventy per cent of cases with ≥12 nodes harvested were performed at high-volume centers. Preoperative radiation or preoperative chemoradiation led to lower ELN (46% and 48%) versus preoperative chemotherapy alone (66%). Multivariate analysis showed that patients who had ≥12 nodes removed had better OS (Hazard Ratio 0.843 [95 confidence interval 0.820–0.867]). In addition, care at a high-volume facility, care at an academic facility, private insurance, and income ≥$63,000 were all associated with improved OS. Higher ELN count is associated with OS in patients with esophageal cancer. Patients who receive care at high-volume centers and academic centers are more likely to undergo more extensive lymphadenectomy. All centers should strive to examine at least 12 nodes to provide a quality esophagectomy.

PS02.139: THE TREATMENT STRATEGY AND CLINICAL OUTCOMES FOR SALVAGE ESOPHAGECTOMY AFTER DEFINITIVE CHEMORADIOTHERAPY

2018 ◽  
Vol 31 (Supplement_1) ◽  
pp. 160-160
Author(s):  
Kyohei Ogawa ◽  
Kosuke Narumiya ◽  
Kenji Kudo ◽  
Yosuke Yagawa ◽  
Shinsuke Maeda ◽  
...  
Keyword(s):  
Esophageal Cancer ◽  
Clinical Outcomes ◽  
Treatment Strategy ◽  
Conflicts Of Interest ◽  
High Rate ◽  
Hospital Death ◽  
Definitive Chemoradiotherapy ◽  
Unresectable Tumor ◽  
Back Ground ◽  
Salvage Esophagectomy

Abstract Background Definitive chemoradiotherapy for esophageal cancer which was unresectable tumor has become common therapy. In recently, we have perform chemoradiotherapy for resectable tumor because esophagectomy for esophageal cancer is an invasive surgical procedure. But some cases were recurrent. We examined the treatment strategy and clinical outcomes of salvage esophagectomy after definitive chemoradiotherapy for esophageal cancer. Methods We reviewed 46 cases of subjects with esophageal cancer who underwent salvage esophagectomy after definitive chemoradiotherapy with more than 50Gy of radiation from 2000 to 2017. We exam (1) Back ground (2) Term after Chemoradiotherapy (3)Surgical approach (4) Route of reconstruction (5)Rang of lymphanodectomy (6)Complication (7)Prognosis. Results Age 63.0(43–79), Male: Female = 44:2, Location: Upper/Middle/Lower = 15/25/8, T1/T2/T3/T4 = 11:2:25:8 R0: R1: R2 = 35:9:2 (2) Within1 year/More than 1 year = 31/15 (3) Neck digection/Right thoracotomy: Left thoracotomy: laparotomy = 8:31:5:2 (4) Mediastinal rute/Ante/Retro = 34:9:3 (5) 1 Field:2 Field:3 Field = 11:27:8 (6) Anastleakage/Pneumonia/Abcess/Meningitis/Fluid in the thoracic cavity = 11: 9: 4: 1: 8 (7) 5-years survival rate was 36%. Hospital death was4.3% Conclusion There were high rate complications for salvage esophagectomy after definitive chemoradiotherapy for esophageal cancer. Patients should be carefully selected for salvage esophagectomy.Surgeons should consider the indications and techniques for esophageal surgery to increase cure rates and decrease morbidity. Disclosure All authors have declared no conflicts of interest.

Disparities in esophageal cancer care based on race: a National Cancer Database analysis

2021 ◽  
Author(s):  
Ikenna C Okereke ◽  
Jordan Westra ◽  
Douglas Tyler ◽  
Suzanne Klimberg ◽  
Daniel Jupiter ◽  
...  
Keyword(s):  
Overall Survival ◽  
Esophageal Cancer ◽  
African Americans ◽  
Racial Disparities ◽  
Cancer Patients ◽  
Racial Differences ◽  
Tumor Biology ◽  
High Volume ◽  
National Cancer Database ◽  
Survival Difference

Summary Esophageal cancer is one of the most common cancer killers in our country. The effects of racial disparities on care for esophageal cancer patients are incompletely understood. Using the National Cancer Database, we investigated racial disparities in treatment and outcome of esophageal cancer patients. The National Cancer Database was queried from 2004 to 2017. Logistic regression and survival analysis were used to determine racial differences in access, treatment and outcome. A total of 127,098 patients were included. All minority groups were more likely to be diagnosed at advanced stages versus Caucasians after adjusting for covariates (African American OR—1.64 [95% confidence interval 1.53—1.76], Hispanic OR—1.19 [1.08—1.32], Asian OR—1.78 [1.55—2.06]). After adjustment, all minorities were less likely at every stage to receive surgery. Despite these disparities, Hispanics and Asians had improved survival compared with Caucasians. African Americans had worse survival. Racial disparities for receiving surgery were present in both academic and community institutions, and at high-volume and low-volume institutions. Surgery partially mediated the survival difference between African Americans and Caucasians (HR—1.13 [1.10–1.16] and HR—1.04 [1.02–1.07], without and with adjustment of surgery).There are racial disparities in the treatment of esophageal cancer. Despite these disparities, Hispanics and Asians have improved overall survival versus Caucasians. African Americans have the worst overall survival. Racial disparities likely affect outcome in esophageal cancer. But other factors, such as epigenetics and tumor biology, may correlate more strongly with outcome for patients with esophageal cancer.

PS02.184: PROFILING RETURN TO INTENDED ONCOLOGIC THERAPY (RIOT) IN LOCALLY ADVANCED ESOPHAGEAL CANCER PATIENTS RECEIVING ESOPHAGECTOMIES FOLLOWED BY POSTOPERATIVE CHEMOTHERAPY

2018 ◽  
Vol 31 (Supplement_1) ◽  
pp. 174-174
Author(s):  
Wei Dai ◽  
Qiuling Shi ◽  
Yongtao Han ◽  
Lin Peng
Keyword(s):  
Overall Survival ◽  
Esophageal Cancer ◽  
Cox Regression ◽  
Conflicts Of Interest ◽  
Locally Advanced ◽  
Minimal Invasive ◽  
Postoperative Chemotherapy ◽  
Risk Of Death ◽  
Oncologic Therapy

Abstract Background As a novel metric to evaluate the quality of oncosurgical therapy, such as minimal invasive surgery, Return to Intended Oncologic Therapy (RIOT) has not been applied in patients with esophageal cancer (EC). This study aims to profile RIOT in locally advanced EC patients and to quantify its relationship with overall survival. Methods We conducted a retrospective study on consecutive locally advanced EC (T3–4 and/or N1–3) patients who received esophagectomies followed by postoperative chemotherapy (PC) from April 2015 to August 2017. RIOT included whether the patient did or did not undergo intended PC and the time between surgery and the start of PC. Overall survival at each RIOT group was compared via log-rank test. Cox regression models were used to estimate the prognostic value of RIOT. Results Among 658 locally advanced EC patients (547 males and 111 females) with complete PC data, 433 received minimal invasive esophagectomies (MIE) and 225 received open esophagectomies (OE). The RIOT rates were 58.0% for MIE and 54.2% for OE (P = 0.358). The 1-year overall survival rate of patients receiving PC was higher than that of patients not receiving PC (88.2% vs 76.4%; P = 0.005). After adjustment of age, gender, surgery type and postoperative length of stay, patients with PC showed significantly better OS than those without PC (HR 0.60, 95% CI 0.41–0.87; P = 0.007). Total 253 patients (MIE 168, OE 85) presented verified dates of starting PC. Median RIOT time was 42 days (min-max, 13–162) for MIE and 43 days (16–169) for OE (P = 0.855). Among those 253 patients, 179 (70.8%) started RIOT within 8 weeks. After 8 weeks, every one week delay of RIOT related to a 17% increase on the risk of death (P = 0.014). Conclusion Using a real world data, our study provided baseline profiles of RIOT in locally advanced EC patients who received esophagectomies and PC. Compared to OE, MIE did not show a significant advantage in RIOT rates and RIOT time. In spite of the short follow-up, successful RIOT is related to better OS. Prospective studies with longer follow-up are required for further application of RIOT in the evaluation of oncosurgical therapy. Disclosure All authors have declared no conflicts of interest.

Acute Leukaemias with TEL/ABL Fusion: a Subgroup with Poor Prognosis and Prenatal Origin.

Blood ◽  
2009 ◽  
Vol 114 (22) ◽  
pp. 2596-2596
Author(s):  
Jan Zuna ◽  
Marketa Zaliova ◽  
Katerina Muzikova ◽  
Claus Meyer ◽  
Libuse Lizcova ◽  
...  
Keyword(s):  
Overall Survival ◽  
Myeloid Leukaemia ◽  
Poor Prognosis ◽  
Conflicts Of Interest ◽  
Fusion Gene ◽  
Chromosome 1 ◽  
Published Data ◽  
Prognostic Impact ◽  
Guthrie Card ◽  
Childhood All

Abstract Abstract 2596 Poster Board II-572 The TEL/ABL (ETV6/ABL1) fusion gene is a rare aberration in human oncology. Only 19 cases of TEL/ABL-positive haematological malignancy were published so far, diagnosed as chronic myeloid leukaemia (n=9) or other type of chronic myeloproliferative disease (n=3), acute myeloid leukaemia (n=4) or acute lymphoblastic leukaemia (ALL) (n=3). Median age at diagnosis in the published cases was 48 years, with only 2 children (aged 22 months and 4 years) diagnosed thus far, both with ALL. Altogether, the TEL/ABL-positive leukaemias tend to be diagnosed at higher age suggesting post-natal origin and aetiology of this aberration - at least in a vast majority of cases. In the present study we report 3 new cases (aged 8 months, 5 years and 33 years) of ALL with TEL/ABL fusion gene revealed by screening of 392 newly diagnosed ALL patients (335 children and 57 adults). In two patients the mechanism of the TEL/ABL fusion was probably identical - part of the 9q34 including C-terminus of the ABL gene was inserted (in inverse orientation) into the TEL gene at the 12p13. The mechanism of changes was probably more complicated in the infant patient. Our data suggest that in this case part of the TEL gene was inserted into ABL and, moreover, the chromosome 1 was included in the complex cytogenetic changes. In all 3 patients array-CGH analysis was performed showing short deletion in the CDKN2A/CDKN2B locus at 9p21 in 2/3 cases. Analysis of all published data including our cases reveals poor prognosis of the TEL/ABL-positive acute leukaemias - overall survival in 2 years reaches only 15%. In particular, 4 patients (including 2 in the present study) were children (0-5 years), all of them diagnosed as ALL. Despite the prognosis of childhood ALL is generally better (overall survival reaching 80–90%) than the outcome of other haematological malignancies, only 1 out of the 4 reported paediatric cases survived more than 13 months from diagnosis (the 5 year old patient from the present study is now in complete remission for 16 months). On the other hand, survival of chronic leukaemias exceeds 50% corresponding to the outcome of TEL/ABL-negative chronic leukaemias in adults. Eosinophilia was suggested as a clinical hallmark of the TEL/ABL-positive leukaemia. However, we can not confirm the 100% correlation as 2/3 patients in this report lack this feature. In the two paediatric cases the course of the leukaemia was monitored using minimal residual disease (MRD). The data demonstrate excellent correlation of the MRD levels assessed by TEL/ABL transcript and immunoreceptor genes rearrangements quantification. Scrutiny of the neonatal blood spot (Guthrie card) revealed that in the 5 year old child the TEL/ABL rearrangement initiating the ALL was originated prenatally. The relatively long latency period suggests that secondary genetic hit(s) were required for the overt disease in this patient. The ALL diagnosed in the infant case at the age of 8 months was also very likely initiated prenatally, however, we were not able to locate the genomic breakpoint between the two genes and prove this hypothesis. The deletion at the CDKN2A/CDKN2B region in 2/3 presented cases (described also previously in TEL/ABL-positive ALL) suggests that loss of function in this region or in other tumour-suppressor loci (not necessarily detectable by cytogenetics) might contribute to leukaemogenesis of the primary aberration. In conclusion, we report three new cases of ALL characterised by TEL/ABL fusion and we integrate the new patients into already published data. For the first time we show detailed characterisation of the course of the disease including MRD data, we assess prognostic impact of the aberration using “meta-analysis” and we demonstrate prenatal origin of the TEL/ABL fusion. The work was supported by grants MSM0021620813 and IGA-MZ NS1000-4. Disclosures: No relevant conflicts of interest to declare.

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