scholarly journals Malignant Extracranial Germ Cell Tumours: A First Report by the South African Children’s Cancer Study Group

Author(s):  
Marc Hendricks ◽  
Annibale Cois ◽  
Jennifer Geel ◽  
Jan du Plessis ◽  
Mairi Bassingthwaighte ◽  
...  

OBJECTIVE  To determine the overall survival (OS) and prognostic factors influencing outcomes in children and adolescents with malignant extracranial germ cell tumours (MEGCTs) in preparation for the development of a harmonised national treatment protocol.METHODS A retrospective folder review was undertaken at nine South African paediatric oncology units to document patient profiles, tumour and treatment-related data and outcomes for all children with biopsy proven MEGCTs from birth up to and including 16 years of age. RESULTS Between 1 January 2000 and 31 December 2015, 218 patients were diagnosed with MEGCTs. Female sex (HR 0.284 p=0.037) and higher socio-economic status (SES) (HR 0.071; p=0.039) were associated with a significantly lower risk of death. Advanced clinical stage at diagnosis significantly affected 5-year OS: stage I -96%; stage II - 94.3%; stage III -75.5%; (p=0.017) and stage IV (60.1%; p<0.001). There was a significant association between earlier stage at presentation and higher SES (p=0.03). Patients with a serum AFP level of more than 33,000 ng/ml at diagnosis had significantly poorer outcomes (p=0.002). The use of chemotherapy significantly improved survival, irrespective of the regimen used (p<0.001). CONCLUSIONS The cohort demonstrated a 5-year OS of 80.3% with an EFS of 75.3%. Stage, the use of chemotherapy and an elevated serum AFP level of more than 33,000ng/ml were independently predictive of outcome. The relationship between SES and outcome is important as the implementation of the new national protocol hopes to standardise care across the socio-economic divide.

Author(s):  
Marc Hendricks ◽  
Annibale Cois ◽  
Jennifer Geel ◽  
Jan du Plessis ◽  
Mairi Bassingthwaighte ◽  
...  

OBJECTIVE To determine the overall survival (OS) and prognostic factors influencing outcomes in children and adolescents with malignant extracranial germ cell tumours (MEGCTs) in preparation for the development of a harmonised national treatment protocol. METHODS A retrospective folder review was undertaken at nine South African paediatric oncology units to document patient profiles, tumour and treatment-related data and outcomes. RESULTS Between 1 January 2000 and 31 December 2015, 218 patients were diagnosed with MEGCTs. Female sex (OR 2.26; p=0.037) and higher socio-economic status (SES) (HR 0.071; p=0.039) were associated with a significantly lower risk of death. Advanced clinical stage at diagnosis significantly affected 5-year OS: stage I -96%; stage II - 94.3%; stage III -75.5%; (p=0.017) and stage IV (60.1%; p<0.001). There was a significant association between earlier stage at presentation and higher SES (p=0.03). Patients with a serum AFP level of more than 33,000 ng/ml at diagnosis had significantly poorer outcomes (p=0.002). The use of chemotherapy significantly improved survival, irrespective of the regimen used (p<0.001). Forty-one patients (18.9%) died: 37 (16.9%) from disease progression, three (1.9%) from infection, two (0.9%) from chemotherapy toxicity and one (0.5%) from surgical complications. CONCLUSIONS The cohort demonstrated a 5-year OS of 80.3% with an EFS of 75.3%. Stage, the use of chemotherapy and an elevated serum AFP level >33,000ng/ml were independently predictive of outcome. The relationship between SES and outcome is important as the implementation of a new national protocol aims to standardise care across the socio-economic divide.


Author(s):  
Klaus-Peter Dieckmann ◽  
Cansu Dumlupinar ◽  
Arlo Radtke ◽  
Cord Matthies ◽  
Renate Pichler ◽  
...  

Abstract Purpose Lymphovascular invasion (LV1) and presence of > 50% embryonal carcinoma (> 50% EC) represent risk factors for progression in patients with clinical stage 1 (CS1) nonseminomatous (NS) testicular germ cell tumours. As serum levels of microRNA-371a-3p (M371) are capable of detecting small amounts of GCT, we evaluated if LV1 and > 50% EC are associated with M371 levels. Methods M371 serum levels were measured postoperatively in 153 NS CS1 patients and both pre- and postoperatively in 131 patients. We registered the following factors: age, tumour size, LV status, > 50% EC, teratoma in primary, preoperative elevation of classical tumour markers. M371 expression was compared among subgroups. The ability of M371 to predict LV1 was calculated by receiver operating characteristics (ROC) curves. Multiple regression analysis was used to look for associations of M371 levels with other factors. Results Postoperatively elevated M371 levels were found in 29.4% of the patients, but were neither associated with LV status nor with > 50% EC. Likewise, relative decrease of M371 was not associated. ROC analysis of postoperative M371 levels revealed an AUC of 0.5 for the ability to predict LV1 while preoperative M371 had an AUC of 0.732. Multiple regression analysis revealed significant associations of preoperative M371 levels with LV status (p = 0.003), tumour size (p = 0.001), > 50% EC (p = 0.004), and teratoma component (p = 0.045). Conclusion Postoperatively elevated M371 levels are not associated with risk factors for progression in NS CS1 patients. However, the significant association of preoperative M371 expression with LV1 deserves further evaluation.


2021 ◽  
Author(s):  
Marc Hendricks ◽  
Annibale Cois ◽  
Jennifer Geel ◽  
Johan du Plessis ◽  
Mairi Bassingthwaighte ◽  
...  

2001 ◽  
Vol 16 (1) ◽  
pp. 27-30 ◽  
Author(s):  
N. M. Forones ◽  
M. Tanaka ◽  
D. Matos

Introduction CA19-9 is one of the most important tumor markers used in patients with colorectal cancer, mainly in radical surgery follow-up. Aim: The purpose of this study was to evaluate the preoperative CA19-9 level obtained from a peripheral vein (PV) and compare it to the level obtained from the mesenteric vein (MV). Materials and methods Blood was collected from a PV of the arm and from the MV of 59 patients with colorectal cancer before primary surgery. Of these 59 patients fourteen had stage I disease, 10 stage II, 22 stage III, and 13 stage IV. CA19-9 was determined in serum by immunoenzymatic assay (Abbott Diagnostica). Results Fifteen patients (24%) had elevated serum levels of CA19-9 in the MV and 13 (22%) in the PV. None of the stage I or II patients had elevated serum levels of CA19-9. There were no differences between marker levels in blood collected from the MV or PV, independent of clinical stage. The CA19-9 values obtained from the MV differed significantly in the different stages of the disease according to the Kruskal-Wallis analysis (p=0.026); this difference was not statistically significant (p=0.08) in serum from the PV. There was no correlation between venous infiltration by the tumor and positivity of CA19-9 serum levels collected from the mesenteric vein. We observed a close correlation between the serum levels of CA19-9 collected from the PV and from the MV (r=0.9). Conclusion The current study demonstrates a close correlation between the serum levels of CA19-9 collected from a peripheral vein and from the mesenteric vein. Our results confirmed the poor sensitivity of serum CA19-9 at diagnosis, independent of the collection site.


2021 ◽  
pp. 1-12
Author(s):  
Sabine Kliesch ◽  
Stefanie Schmidt ◽  
Doris Wilborn ◽  
Clemens Aigner ◽  
Walter Albrecht ◽  
...  

<b><i>Introduction:</i></b> This is the first German evidence- and consensus-based clinical guideline on diagnosis, treatment, and follow-up on germ cell tumours (GCTs) of the testis in adult patients. We present the guideline content in two publications. Part I covers the topic’s background, methods, epidemiology, classification systems, diagnostics, prognosis, and treatment recommendations for the localized stages. <b><i>Methods:</i></b> An interdisciplinary panel of 42 experts including 1 patient representative developed the guideline content. Clinical recommendations and statements were based on scientific evidence and expert consensus. For this purpose, evidence tables for several review questions, which were based on systematic literature searches (last search was in March 2018) were provided. Thirty-one experts entitled to vote, rated the final clinical recommendations and statements. <b><i>Results:</i></b> We provide 161 clinical recommendations and statements. We present information on the quality of cancer care and epidemiology and give recommendations for staging and classification as well as for diagnostic procedures. The diagnostic recommendations encompass measures for assessing the primary tumour as well as procedures for the detection of metastases. One chapter addresses prognostic factors. In part I, we separately present the treatment recommendations for germ cell neoplasia in situ, and the organ-confined stages (clinical stage I) of both seminoma and nonseminoma. <b><i>Conclusion:</i></b> Although GCT is a rare tumour entity with excellent survival rates for the localized stages, its management requires an interdisciplinary approach, including several clinical experts. Quality of care is highly related to institutional expertise and can be reassured by established online-based second-opinion boards. There are very few studies on diagnostics with good level of evidence. Treatment of metastatic GCTs must be tailored to the risk according to the International Germ Cell Cancer Collaboration Group classification after careful diagnostic evaluation. An interdisciplinary approach as well as the referral of selected patients to centres with proven experience can help achieve favourable clinical outcomes.


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