Clinical features and outcomes of secondary somatic malignancy (SSM) associated with primary mediastinal nonseminomatous germ cell tumors (PM-NSGCT).

2019 ◽  
Vol 37 (7_suppl) ◽  
pp. 531-531
Author(s):  
Lucas W. Dean ◽  
Nathan Colin Wong ◽  
Shawn Dason ◽  
Sumit Isharwal ◽  
Mark Donoghue ◽  
...  

531 Background: The incidence of secondary somatic malignancy (SSM) arising from teratoma is increased in 2 settings; late relapse and primary mediastinal nonseminomatous germ cell tumors (PM-NSGCT). Here, we report the clinical features and outcomes of patients with SSM in the setting of PM-NSGCT. Methods: Between 1985 and 2018, 29 patients with PM-NSGCT and SSM who had sufficient clinical follow-up to evaluate outcome were identified. Clinical and pathologic parameters were reviewed. The Kaplan-Meier method was used to estimate overall survival (OS) from time of SSM diagnosis and the log rank test to compare estimates. Results: Median age was 28 years (range 18-59) and all patients were male. Most presented with local symptoms (n=24, 83%), elevated tumor markers (n=26, 90%), and disease isolated to the mediastinum (n=25, 86%). A total of 39 SSM histologies were present in the 29 cases, with 8 (28%) having 2 (n=6) or 3 (n=2) SSM histologies; 25 (86%) also had viable non-teratomatous GCT in the mediastinal mass. Sarcoma was found in all 29 cases including rhabdomyosarcoma (n=15), angiosarcoma (n=6), sarcoma NOS (n=5), spindle cell (n=4), PNET (n=3), and other (n=3). Non-sarcoma histologies (n=1 each) included AML, SCC, and neuroblastoma. Most patients received GCT-directed chemotherapy followed by an attempt at surgical resection (90%). With a median follow-up of 2 years for survivors, median OS was 1.8 years (95% CI 0-3.9 years), with 18 patients succumbing to disease. Complete surgical resection was achieved in 23 men (79%) and was associated with superior OS (3.1 vs. 0.3 years, p=0.005). At relapse or progression, 11 received SSM histology-directed and 7 GCT-directed chemotherapy with no difference in OS (1.3 vs. 1.2 years, p=0.993). 7 patients developed SSM in the form of leukemia, a finding associated with significantly inferior OS (0.3 vs. 3.0 years, p=0.009). Conclusions: Sarcoma is the predominant SSM histology associated with PM-NSGCT and portends a poor prognosis even with initially localized disease. Complete resection following chemotherapy is critical to achieving long-term survival whereas SSM in the form of leukemia portends especially poor outcome.

Author(s):  
Yue Che ◽  
Achim Lusch ◽  
Christian Winter ◽  
Robert Große Siemer ◽  
Carolin Buddensieck ◽  
...  

Abstract Purpose Late relapsing germ cell tumors (LR-GCT) are considered a rare distinct biologic entity as their clinical presentation and response to treatment is different to early recurrences. While serum tumor markers (AFP and ß-HCG) play an important role at the time of first diagnosis to correctly classify prognosis and treatment of germ cell tumors, they may not have the same significance in a late relapse situation. Patients and methods Thirty-seven patients with LR-GCT with elevated serum tumor markers were identified in our database. Twenty-six patients underwent primary surgical resection of the late relapsing tumor. Eleven patients received salvage chemotherapy and a post-chemotherapy residual tumor resection. Serum tumor markers, histological findings and oncological outcome were analyzed. Results In the histopathological specimen, viable cancer was found in 20 cases (54%) and teratoma was found in 16 cases (43%). In nine cases (24%), a somatic-type malignant transformation was present. In 19 of 37 patients (51.4%), the late relapse specimen presented a histological type of GCT, which was not present in the primary histology. Twenty-two patients (59.5%) were included in follow-up analysis. Mean and median follow-up time was 62.2 and 53 months, respectively. Seventeen patients (77.3%) suffered a relapse or had progressive disease after LR therapy. Five patients (22.7%) have been relapse-free after LR therapy (mean FU 61.6 months). Ten patients died of disease during follow-up (45.5%) and had a mean time from LR to death of 66.4 months. Eleven patients were alive at last follow-up (mean FU 62.2 months). Relapse and survival rate were similar between patients who received primary resection of LR tumor and patients who received salvage chemotherapy followed by surgery. Conclusion Patients with a late relapsing germ cell tumor and elevated markers have a poor prognosis and a high risk for another relapse independent on primary treatment. The histological type and aggressiveness of a late relapsing tumor cannot be predicted with serum tumor marker levels at the time of diagnosis of LR. In up to 54% of cases, primary histology did not coincide with LR histology. Therefore, we propose primary surgical resection of a late relapsing tumor if a complete resection is feasible in order to gain exact histology and tailor further treatment.


2020 ◽  
Vol 22 (Supplement_2) ◽  
pp. ii57-ii57
Author(s):  
Qingjun Hu ◽  
Juan Li ◽  
Mingyao Lai ◽  
Cheng Zhou ◽  
Zhaoming Zhou ◽  
...  

Abstract OBJECTIVE To evaluate the clinical factors related to the prognosis of basal ganglia germ cell tumors. METHODS A retrospective analysis of 52 cases of the basal ganglia germ cell tumors treated from January 2009 to January 2019 in the department of oncology of Guangdong Sanjiu Brain Hospital. The median age: 12 years (range: 5–32), The median course of disease: 11.7 months (range: 1–54). Thirteen cases were diagnosed by biopsy and 39 cases were diagnosed by elevated tumor markers. There were 31 patients (59.6%) diagnosed with germinomas and 21 patients (40.4%) with non-germ germ cell tumors. Univariate and multivariate survival analysis was performed. RESULTS To October 15, 2019, the median follow-up time was 30.4 months (range 2–124 months). The 5-year survival rate was 85%, and the 5-year progression-free survival rate was 84%. Multivariate analysis found whether serum AFP was greater than 100mIU / ml, (with HR: 11.441,95% CI: 2.09–47.66, P = 0.005),the degree of surgical resection(with HR 5.323 (1.19–23.812), P = 0.029), PD as the effect of radiotherapy (HR: 16.53, (1.19–23.81), P = 0.001) were independent prognostic factor affecting survival. CONCLUSION The pathological type, degree of surgical resection, and response to initial treatment can all affect survival.


2019 ◽  
Vol 104 (6) ◽  
pp. e4.3-e5
Author(s):  
V Gotta ◽  
A Atkinson ◽  
O Marsenic ◽  
M Pfister

BackgroundHemodialysis (HD) prescription significantly differs between pediatric and adult patients on maintenance HD, resulting in greater difference between prescribed and delivered HD dose.1,2 HD dose targets have formally not been evaluated for children, hence targets are mainly derived from adults (spKt/V >1.4; sp: single-pool model of urea distribution, K: urea clearance, t: duration of HD session, V: urea distribution volume). This analysis aimed to evaluate the relationship between delivered dialysis dose and survival in a large cohort of patients having started HD therapy in childhood.MethodsThis retrospective analysis included a cohort of patients < 30 years (y) on chronic HD treatment since childhood, having received thrice-weekly HD between 2004 and 2016 in outpatient DaVita dialysis centers. Survival while on HD (death from any cause) was investigated using Kaplan-Meier analysis stratified by age at start of HD (0–2, >2–6, >6–12, and >12–18 y), and three mean delivered dialysis dose levels (spKt/V < 1.4, 1.4–1.6, >1.6). Survival curves between subgroups were compared using the Log-rank test.Results1773 patients were included in the analysis, among n=34 having started HD at age of 0–2y, n=57 at >2–6y, n=244 at >6–12y, and n=1438 at >12–18y. Median follow-up on HD ranged between 1.5 (>2–6y) to 4.7 years (>6–12y) with maximal follow-up of 23 years. Death while on HD occurred in 1/34, 6/57, 26/244, and 101/1438 patients during recorded follow-up (p=0.075, n.s.). Patients with mean spKt/V < 1.4 had lower survival on HD than those with spKt/V >1.4–1.6 (p=0.019) and those with spKt/V >1.6 (p=0.035), with 10-year survival estimated to 75% (65.2–86.2%) versus 84.5% (78.5–90.9%) and 85.0% (80.8–89.5%), respectively.ConclusionsThis is the first study to report long term survival and its relationship with delivered HD dose in patients starting HD in childhood. Our results support targeting spKt/V(urea)>1.4 in children on chronic HD treatment.ReferencesGotta V, Marsenic O, Pfister M. Age- and weight-based differences in haemodialysis prescription and delivery in children, adolescents and young adults. Nephrol Dial Transplant 2018 Apr 18.Gotta V, Marsenic O, Pfister M. Understanding urea kinetic factors that enhance personalized hemodialysis prescription in children. ASAIO J 2019 Jan 14.Disclosure(s)M Pfister is a consultant at Quantitative Solutions a Certara Company. V Gotta has been supported for this project by the Research Fund for Junior Researchers, University of Basel, Switzerland. O Marsenic and A Atkinson declare no financial conflict of interest.


2019 ◽  
Vol 21 (Supplement_6) ◽  
pp. vi225-vi225
Author(s):  
Linbo Cai ◽  
Juan Li ◽  
Mingyao Lai ◽  
Cheng Zhou ◽  
Qingjun Hu ◽  
...  

Abstract OBJECTIVES The present study aimed to investigate the clinical features associating with intracranial germ cell tumors(GCTs) of poor prognosis. METHODS We retrospectively analyzed the death data cohort based on 47 patients (30 males and 17 females) who had been diagnosed with GCTs between 2005 and 2018 in our hospital. The median age at initial diagnosis was 16 years (range, 3¬60 years). Among those, 18 patients were germinoma and 29 patients were non¬seminomatous germ-cell tumors (NGGCTs). RESULTS According to Kaplan-Meier survival analysis, children aged ≤ 10 years were observed with a very short survival compared to patients aged > 10 years, indicating a 5-year survival rate of 0 and 50%, respectively (P < 0.001). To be more specific, disseminated tumors were found to be the most prominent factor for prognosis in children aged ≤ 10 years: the 1-year survival for MR positive versus MR negative of disseminated tumors were 0 and 62.5%, respectively (P < 0.05). On the other hand, the pre¬treatment serum alpha-fetoprotein (AFP) level was found to be an important surrogate for prognosis. In the subgroup of patients > 10 years, the 5-year survival for AFP > 1.5ng/ml was 25%, in comparison to 66% for AFP ≤ 1.5ng/ml (P < 0.05). Further, solid survival advantages were found in patients received stereotactic radiosurgery (SRS) compared to none. The 5¬year survival for SRS versus non-SRS patients were 62% and 29%, respectively (P < 0.05). CONCLUSION Our data highlights a number of critical factors that may facilitate the prognosis stratifications of GCTs. What’s more, neither the locations of the primary tumors nor the numbers of lesions are associated with the prognosis of GCTs. These results warrant further investigation via randomized prospective studies.


1998 ◽  
Vol 16 (2) ◽  
pp. 725-732 ◽  
Author(s):  
K Fizazi ◽  
S Culine ◽  
J P Droz ◽  
A Kramar ◽  
C Théodore ◽  
...  

PURPOSE Primary mediastinal nonseminomatous germ cell tumors (NSGCT) are uncommon neoplasms and clinically and biologically distinct from other germ cell tumors (GCT). We describe the clinical and biologic features of these patients and evaluate the results of treatment during the cisplatin era. PATIENTS AND METHODS Between 1976 and 1993, 38 patients with mediastinal NSGCT received cisplatin-based chemotherapy as part of their primary treatment. Twenty-nine of them were initially treated at the Institut Gustave-Roussy (IGR), VillejuiF, France, and nine were referred for salvage treatment. RESULTS Of the 29 patients initially treated at IGR, 11 (39%) had metastasis. A complete response (CR) to therapy was obtained in 19 of 29 patients (66%) after chemotherapy and surgery. Ten patients (34.5%) have remained free of disease with a median follow-up of 89 months. All patients who did not achieve a CR died of disease. The 2-year overall survival rate for the IGR patients is 45% and the 2-year disease-free survival is 37%. Only the presence of extrapulmonary metastasis was of prognostic significance in the univariate analysis (P = .0095). None of the 20 patients who required salvage therapy is currently disease-free. Five patients developed and subsequently died of a hematologic malignancy at an interval range of 1 to 47 months from treatment of mediastinal NSGCT. Cytogenetic analysis of leukemic cells found an isochromosome of the short arm of chromosome 12 (12p) in two cases. The incidence of leukemia was 21% in patients who attained a CR. CONCLUSION Primary mediastinal NSGCT is a clinical and biologic entity that should be distinguished from other GCT. About 40% of these patients can envisage long-term survival with modern therapy that includes cisplatin-based chemotherapy followed by surgical resection of residual masses. New strategies are required for patients who do not attain a CR. Predictive factors and improvement in therapy are required for mediastinal NSGCT-associated leukemia.


2019 ◽  
Vol 37 (15_suppl) ◽  
pp. e16064-e16064
Author(s):  
Yanal Mufeed Alnimer ◽  
Khalil Katato

e16064 Background: Extragonadal germ cell tumor is an uncommon type of malignancy. We aim to identify the clinical features and the prognosis in various histological subtypes in these patients using the Surveillance, Epidemiology, and End Results Program (SEER) database. Methods: We used the (SEER) database to identify the patients with non-gonadal germ cell tumors between 1998-2015. 1299 patients were identified and included in our analysis. Results: 1299 patients had non-gonadal germ cell tumors between 1998-2015. The mean age was 26 years-old. 568 patients (44%) were females and 731 patients (56%) were males. 276 patients (21%) died after a mean follow up time of 41.16 months (1-60 months). The mean overall survival (OS) was 49.4 months (95%CI 48.21-50.52) with the median being unreached. The Females had better OS compared to the males with a Hazard ratio (HR) = 0.50 (95%CI 0.40-0.63). Age more than 60 years-old at the time of diagnosis was associated with a substantially significant increase in mortality with an HR = 43 (95% CI 19.17-94.41). The mediastinal germ cell tumors were associated with worse OS compared to other sites with a HR = 4.69 (95%CI 3.37-6.52). The median OS for Embryonal cell carcinoma was 22 months, and it was associated with worse OS compared to seminoma and other histological subtypes with a HR = 4.10 (95% CI 1.69-9.72) and 2.77 (95% 1.16-6.58) respectively. On the other hand, Seminoma was associated with better OS compared to other histological subtypes with an HR = 0.66 (95%CI 0.52-0.84). Moreover, tumor size less than 80 mm was associated with better OS with a HR = 0.42 (95%CI 0.30-0.64). In multivariate analysis, patients above the age of 60 had worse OS with an HR = 4.04 (95%CI 1.71-9.58), patients with mediastinal germ cell tumors had poorer OS compared to other sites with an HR = 2.38 (95%CI 1.51-3.79). Furthermore, embryonal carcinoma, mixed germ cell tumors and choriocarcinoma were associated with worse OS compared to seminoma and immature teratoma with an HR = 10.39 (95% CI 4.36-24.72), HR = 2.07 (95% CI 1.23-3.46) and HR = 6.16 (95%CI 2.98-12.74) respectively. Conclusions: Non-gonadal seminoma and immature teratoma carry better prognosis compared to other histological subtypes. Also, among non-gonadal germ cell tumors, non-mediastinal seminoma has the best survival outcome.


2020 ◽  
Vol 38 (15_suppl) ◽  
pp. e17058-e17058
Author(s):  
Arnav Srivastava ◽  
Hiren V. Patel ◽  
Sinae Kim ◽  
Isaac Kim ◽  
Eric A. Singer ◽  
...  

e17058 Background: Clinical stage (CS) dictates treatment in men with testicular cancer and its inaccuracy may affect clinical outcome. We evaluate the accuracy of clinical staging in men with CS I and CS IIA/B NSGCT and explore the implications of inaccurate staging on overall survival. Methods: Using the National Cancer Database (NCDB), we abstracted all patients with clinical Stage I-IIB NSGCT who received a primary retroperitoneal lymph node dissection (RPLND) from 2004 to 2014. Primary RPLND was defined as RPLND performed for CS I-IIB patients without prior chemotherapy. CS was cross-tabulated with pathologic nodal staging data. Survival for patients who were accurately staged (CS I patients with pN0 disease, CS IIA patients with pN1 disease) and for CS I patients found to have pN+ disease was determined using the Kaplan Meier method. Results: 1,639 CS I-IIB patients underwent primary RPLND. Among CS I patients, 23% had upstaging of disease (pN1-3), of which 13.9%, 8%, and 1.1% were pN1, pN2, and pN3, respectively (Table). Pathologic N1-3 disease was higher in CS IB vs. CS IA patients (35.1% vs 14.2%, respectively). Of CS IIA patients, 23.1% had pN0 disease, while 44.8%, 13.4%, and 1.3% had pN1, pN2, and pN3 disease, respectively. At a median follow-up of 56.3 months, mortality rates for CS I patients who had pN1, pN2, and pN3 disease were 2.8%, 4%, and 9.1%, respectively, and < 1% for men with pN0 disease. 10-year overall survival for CS1 patients was significantly less favorable if upstaged to pN2 or pN3 disease after RPLND vs. pN0 or pN1. Conclusions: Nearly a quarter of patients with CS I NSGCT are under-staged and are found to have pN1-3 after RPLND. Nodal disease burden is associated with survival. Novel imaging techniques and biomarkers are needed to improve the sensitivity of detecting NSGCT. [Table: see text]


2005 ◽  
Vol 23 (36) ◽  
pp. 9290-9294 ◽  
Author(s):  
G. Varuni Kondagunta ◽  
Jennifer Bacik ◽  
Dean Bajorin ◽  
Deborah Dobrzynski ◽  
Joel Sheinfeld ◽  
...  

Purpose To assess response, overall survival, and relapse-free survival of patients with good-risk metastatic germ cell tumor (GCT) by International Germ Cell Consensus Classification Group (IGCCCG) criteria treated with four cycles of etoposide and cisplatin (EP). Patients and Methods Two hundred eighty-nine patients with IGCCCG good-risk GCT were treated with four cycles of EP. EP consisted of four cycles of etoposide 100 mg/m2 and cisplatin 20 mg/m2 on days 1 to 5 every 21 days. Results Two hundred eighty-two of 289 patients (98%) achieved a complete response; 269 (93%) responded to chemotherapy alone and 13 (5%) responded to chemotherapy plus surgical resection of viable disease (GCT other than mature teratoma). Seventeen (6%) experienced relapse, and nine (3%) died as a result of disease at a median follow-up of 7.7 years (range, 0.4 to 21.1 years). Sixty-two of 204 patients (30%) with nonseminoma had findings of teratoma or viable GCT at postchemotherapy surgery. Conclusion Four cycles of EP is a highly effective therapy for patients with good-risk GCT, with a high cure rate, low relapse rate, and little evidence of late relapse. Postchemotherapy surgery resection of residual disease remains an important aspect of treatment for these patients. Four cycles of EP is acceptable as a standard regimen for the treatment of good-risk metastatic GCT, and serves as an alternative to three cycles of bleomycin and etoposide before cisplatin.


2006 ◽  
Vol 24 (18_suppl) ◽  
pp. 14538-14538
Author(s):  
A. J. Rodney ◽  
A. Siefker-Radtke ◽  
N. M. Tannir ◽  
S. Swisher ◽  
G. Walsh ◽  
...  

14538 Background: PMGCT are uncommon germ cell malignancies. Mediastinal nonseminomatous germ cell tumors (NSGCT) have a poor prognosis, whereas pure seminoma (Sem) has a good or intermediate prognosis irrespective of mediastinal presentation. Methods: We retrospectively identified 19 male pts with PMGCT seen at MDACC between October 1998 and September 2004 from a clinical database. Pts with resectable NSGCT were offered surgery upon plateau of their chemotherapy response. Prior to referral, 1 pt had primary surgical resection without preoperative (preop) chemotherapy. Results: There were 14 pts with NSGCT and 5 with good prognosis Sem. The median age was 29.5 (20–60). Seven pts with NSGCT had mixed or undifferentiated histology, and the remainder had pure yolk sac (5 pts) or choriocarcinoma (2 pts). The estimated median survival (Kaplan-Meier) for all patients (Sem + NSGCT) was 21 months. All pts with Sem were alive and disease-free at last follow-up (median 12 months, range 7–34). All pts with Sem received 4 courses of etoposide and cisplatin (EP); one also received bleomycin (BEP); one received radiotherapy consolidation; none received surgery. Of the pts with NSGCT, 9 (64%) have died, including 1 who refused surgery. Five pts with NSGCT were alive at last follow-up and 3 (21%) were disease-free (15+, 27+ and 35+ months). Four pts with NSGCT (29%) reached beyond 2 years survival (27+, 28, 35+, and 63+ months) including 3 with lung metastases and one with elevated preop alpha-fetoprotein (28,022 ng/ml). Each of these pts received 6–10 courses of multiple-regimen preop chemotherapy, and 2 received initially 4 courses of BEP without marker normalization. Conclusions: Mediastinal Sem treated with 4 courses EP had an excellent outcome without surgery. Pts with mediastinal NSGCT had a 64% mortality rate despite aggressive treatment. Several pts with mediastinal NSGCT did achieve long-term survival following aggressive chemotherapy and surgery, even with lung metastases and failure to normalize markers. A phase III trial of BEP versus dose-dense chemotherapy for poor-prognosis NSGCT is now in progress at MDACC. No significant financial relationships to disclose.


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