Extragonadal Germ Cell Tumors of the Mediastinum and Retroperitoneum: Results From an International Analysis

2002 ◽  
Vol 20 (7) ◽  
pp. 1864-1873 ◽  
Author(s):  
Carsten Bokemeyer ◽  
Craig R. Nichols ◽  
Jean-P. Droz ◽  
Hans-J. Schmoll ◽  
Alan Horwich ◽  
...  

PURPOSE: To characterize the clinical and biologic features of extragonadal germ cell tumor (EGCT) and to determine the overall outcome with currently available treatment strategies.PATIENTS AND METHODS: Of an unselected population of 635 consecutive patients treated from 1975 through 1996 at 11 cancer centers, 341 patients (54%) had primary mediastinal EGCT, and 283 patients (45%) had retroperitoneal EGCT. Five hundred twenty-four patients (83%) had a nonseminomatous germ cell tumor (GCT), and 104 patients (16%) had a seminomatous histology.RESULTS: After platinum-based induction chemotherapy with or without secondary surgery, 141 patients (49%) with mediastinal nonseminomas (median follow-up, 19 months; range, 1 to 178 months) and 144 patients (63%) with retroperitoneal nonseminoma (median follow-up, 29 months; range, 1 to 203 months) are alive (P = .0006). In contrast, the overall survival rate for patients with a seminomatous EGCT is 88%, with no difference between patients with mediastinal or retroperitoneal tumor location (median follow-up, 49 months; range, 4 to 193 months; respective 70 months; range, 1 to 211 months). A significantly lower progression-free survival rate was found in seminoma patients treated with initial radiotherapy alone compared with chemotherapy. Nonseminomatous histology, presence of nonpulmonary visceral metastases, primary mediastinal GCT location, and elevated beta-human chorionic gonadotropin were independent prognostic factors for shorter survival. Hematologic malignancies (n = 17) occurred without exception in patients with primary mediastinal nonseminoma. Sixteen patients developed a metachronous testicular cancer despite the use of platinum-based chemotherapy.CONCLUSION: Whereas patients with pure seminomatous EGCT histology have a long-term chance of cure of almost 90% irrespective of the primary tumor site, 45% of patients with mediastinal nonseminomas are alive at 5 years. This outcome is clearly inferior compared with patients with nonseminomatous retroperitoneal primary tumors.

2020 ◽  
Vol 22 (Supplement_3) ◽  
pp. iii329-iii329
Author(s):  
Hiroki Yamada ◽  
Tomohiro Abiko ◽  
Hirokazu Fujiwara ◽  
Kazunari Yoshida ◽  
Hikaru Sasaki

Abstract INTRODUCTION Germ cell tumors in the central nervous system (CNS) typically arise either at suprasellar and/or pineal region, and occasionally at basal ganglia. We report a case of diagnostically challenging, recurrent germ cell tumor presented with diffuse intraaxial abnormality in and across the lower brainstem, which was diagnosed by the elevated placental alkaline phosphatase (PLAP) level in cerebrospinal fluid (CSF). CASE DESCRIPTION: A 28-year-old man had been treated by chemoradiotherapy at the previous hospital for bifocal suprasellar and pineal lesions with the provisional diagnosis of germinoma without histological confirmation. Three years later, he presented with progressive weakness of bilateral extremities for weeks. Magnetic resonance imaging showed a diffuse, bilaterally symmetric high intensity lesion on T2-weighted image with slight contrast enhancement across the ventral side of the medulla oblongata to the upper cervical spinal cord. Serum and CSF hCG, hCG-β, and AFP were all negative. Since the image findings were atypical for recurrent germ cell tumor, some kind of myelitis was initially suspected. Therefore, steroid pulse therapy was administered. However, the patient’s symptom was still gradually progressing. Then, the CSF PLAP turned out to be positive, indicating the recurrence of germinoma. Accordingly, platinum-based chemotherapy was administered, and the imaging findings, patient’s symptoms, and CSF PLAP began to improve. The patient is to be treated with radiotherapy following chemotherapy. CONCLUSION We report a rare case of CNS germ cell tumor that presented with diffuse intraaxial lesion in the lower brainstem in which examination of CSF PLAP was extremely useful.


2013 ◽  
Vol 2013 ◽  
pp. 1-4
Author(s):  
M. Y. V. Homs ◽  
H. W. R. Schreuder ◽  
G. N. Jonges ◽  
P. O. Witteveen

Malignant ovarian germ cell tumor is a rare disease, but with current treatment strategies including surgery and platinum based chemotherapy survival is excellent. After treatment, intensive followup is indicated to encounter tumor relapse at an early stage. This case describes a 22-year-old female with a history of common variable immune deficiency (CVID) who underwent a resection of a large ovarian germ cell tumor followed by 4 cycles of cisplatin and etoposide resulting in clinical complete remission. During followup, she developed a mass at the umbilicus and ascites. Initially, the cytology of the ascites was interpreted as tumor positive, suspicious of relapse of the disease, but tumor markers remained negative. However, during laparoscopy it turned out to be a mature teratoma, which can develop after chemotherapy, the so called growing teratoma syndrome. In retrospect, the ascites was false positive. This case shows that current diagnostic tools are not sufficient to distinguish between vital tumor and mature teratoma and can be misleading. Tumor biopsy and/or laparoscopic inspection are therefore indicated.


Author(s):  
Branislava Golub Jakovljevic ◽  
Dejan Đokanović ◽  
Snježana Miličević ◽  
Anđa Škobić ◽  
Dejan Ćazić ◽  
...  

Cancer of the testis is a relatively uncommon disease, accounting for approximately 1-1.5% of all cancers in males.  5% of the malignant germ cell tumors are made of extragonadal origin. Germ cell tumors occur in men younger, usually between 20 and 35 years old. We report a case of a patient with metastatic extragonadal germ cell tumor with multiple sites of metastases, and very high initial values of tumor marker human chorionic gonadotrophin (HCG)- 1351308. At the time of diagnosis, the patient was in a very poor general condition. After the applied chemotherapy, there was a complete response and 7 years later the patient is without any symptoms of disease.


2015 ◽  
Vol 37 (03) ◽  
pp. 247-251
Author(s):  
Ana Machado ◽  
Ricardo Taipa ◽  
Manuel Pires ◽  
Carla Silva ◽  
Mário Gomes

AbstractGerm cell tumors of the central nervous system (CNS) are usually located along the midline. Yolk sac tumor is a rare germ cell tumor very uncommonly located outside the midline, and, in such cases, it can be mistaken with other primary tumors. We report a case of a 32-year-old male patient who presented with a right temporal lobe tumor suggestive of a high grade glioma. He was submitted to a right temporal lobectomy with complete tumor removal. The histological exam revealed a germ cell tumor (later confirmed to be a yolk sac tumor). The search for a primary tumor outside of the CNS (including a positron emission tomography scan) was negative, making this a primary temporal lobe yolk sac tumor. The patient was submitted to chemotherapy and radiotherapy, but died 7 months after the surgery.


2016 ◽  
Vol 34 (2_suppl) ◽  
pp. 488-488
Author(s):  
Bivas Biswas ◽  
Deepak Dabkara ◽  
Sandip Ganguly ◽  
Amit Dutt Dwary ◽  
Indranil Ghosh ◽  
...  

488 Background: Late metabolic syndrome is well known in survivors of testicular germ cell tumor (GCT). Literature about early metabolic syndrome is scarce. Methods: Single institutional review of testicular GCT between June’2011 and December’2014. Patients with minimum post-treatment follow-up of 6 months were screened for signs of metabolic syndrome as per National Cholesterol Education Program (NCEP), if they had recorded blood pressure ≥130/85 mm of Hg and/or ≥10% weight gain from baseline. Partial metabolic syndrome was defined as - at least one abnormal criteria as per NCEP. Results: Sixty-three patients of testicular GCT completed treatment and were eligible for evaluation. Twenty-five patients fulfilled the screening criteria with median follow-up of 15 months (range: 6-40); median age of 35 years (range: 24-65). Treatment protocol was combined platinum based chemotherapy in 68% (n=17/25), single agent carboplatin in 20% (n=5/25), and surveillance only in 12% (n=3/25). Complete metabolic syndrome was found in 36% (n=9/25), partial metabolic syndrome in 44% (n=11/25). Blood pressure ≥130/85 mm of Hg was found in 64% (n=16/25), serum triglyceride ≥1.7 mmol/L in 61% (n=14/23), serum HDL cholesterol <1 mmol/L in 76% (n=16/21), and fasting plasma glucose ≥5.6 mmol/L in 30% (n=6/20). Median weight gain was 5 Kg (range: 2-14). Conclusions: Features of early metabolic syndrome (complete or partial) were detected in 80% of testicular GCT survivors in symptomatic and screened patients, and also in those who were in surveillance or who received single agent carboplatin only. So, all testicular GCT survivors should be screened for early metabolic syndrome and early intervention should be initiated as it may have significant long-term effects on mortality and morbidity.


2021 ◽  
Author(s):  
Yang Yu ◽  
Yong Jiang ◽  
Xuanwei Zhang ◽  
Feifei Na ◽  
Weigang Xiu ◽  
...  

Abstract Background: Primary mediastinal yolk sac tumors is a kind of primary mediastinal non-seminomatous germ cell tumor . The current treatment strategies in primary mediastinal non-seminomatous germ cell tumor is neoadjuvant chemotherapy followed by residual mass surgical resection . We reviewed our institutional 5 years' experience with Primary mediastinal yolk sac tumors who treated with platinum-based neoadjuvant chemotherapy and extended resection.Methods: We experienced 10 cases of Primary mediastinal yolk sac tumors from October of 2014 to October of 2019. 7 patients received preoperative platinum-based chemotherapy followed by surgical resection of residual mediastinal mass. The other 3 patients were received initial surgical resection without preoperative chemotherapy.Results:R0 resection was achieved in 8 patients (80%), and R2 resection was in the other 2 patients (20%). All the 7 patients with neoadjuvant chemotherapy were R0 resections, however all of them had viable tumor in their surgical specimen. Morbidities after surgery occurred in 2 patients, including 2 pneumonias ,1 type I respiratory failure and 1 acute left heart failure, and They were died within 2 months after surgery. At the time of writing ,3 patients are alive without evidence of disease,7 patients died , of which 5 patients have died of tumor-related causes and 2 died of postoperative complications. 8 patients were included in the follow-up. Among them 7patients experienced progressed within one year. 8 patients were included in the follow-up,mPFS and mOS in 8 patients were 3.7 months (2.6-41.3m) and 23.15 months (8.6-41.3m) , respectively. The 7 patients with neoadjuvant chemotherapy followed by surgical resection of residual diseases, 2-year survival rate was 57.1%,The 3-year survival rate was 28.6%Conclusion: An aggressive, multidisciplinary treatment including neoadjuvant chemotherapy followed by residual mass surgical resection is the optimal treatment and can be associated with prolonged survival.


2007 ◽  
Vol 25 (18_suppl) ◽  
pp. 20019-20019
Author(s):  
J. Robles-Castro ◽  
R. Rivera-Luna ◽  
C. Calderon-Elvir ◽  
C. A. Leal-Leal

20019 Background: The knowledge regarding treatment and prognosis of pediatric germ cell tumors has been taken from the development of these in the adult population. The object of this work was to compare the International Classification (IGCCCG) used in adults versus the POG classification used traditionally in a pediatric population. Methods: We reviewed clinical records from March 1994 through June 2003 with the diagnosis of malignant germ cell tumor of testis, retroperitoneal and mediastinum. All patients completed the treatment proposed initially with a follow up of more than 24 months. They were staged with the POG classification at diagnosis and with the International Classification for this study. Results: We obtained 61 patients with a 4 year follow up. They were all male, with germ cell tumor of testis or mediastinum. There were no retroperitoneal primary tumors. The age range was from 4 months to 188 months with an average of 48.7 months. Prognosis and treatment with the POG and the IGCCCG classifications had concordance of 0.0004 and 0.0371 respectively. Conclusions: We found limitations such as diverse chemotherapy protocols used in patients within the same risk group. Biological tumor Markers sampling play an important role in the the initial approach to allocate staging and risk based treatment protocols. It is necessary to carry out new studies with a homogeneous based population with similar diagnosis, treatment protocols and staging system to corroborate the utility and implementation of the International Classification in order to ameliorate morbidity and mortality of our pediatric patients. [Table: see text] No significant financial relationships to disclose.


2020 ◽  
Vol 2 (1) ◽  
pp. 62-67
Author(s):  
Fahmi Prasetya ◽  
◽  
Susanthy Djajalaksana ◽  
Diah Retnani ◽  
Artono sharanto ◽  
...  

Background: Malignant mixed germ cell tumors account for 13-25% of all non seminoma germ cell tumors, almost all cases were found in males (85%), increased in the third decade, and had an average survival rate of 40-45%. With modern management the average 5-year survival rate can be over 80%. Case Report: We reported a case report in Saiful Anwar Malang Hospital, Indonesia, a young man, 26 years old with an malignant epithelial tumor derived from mediastinal or lung. The CT scan of the chest showed an lobulated solid mass of iso-hipodens on the anterior superior mediastinum, mass encompassing the aortic arch, left pulmonalis artery, subclavian artery. The result from transthoracic FNAB with USG guidance showed an malignant epithelial tumor derived from mediastinal or lung, impression of an adeno squamous carcinoma. The AFP, LDH, and NSE serum level was elevated. Patient underwent a surgical sternotomy and tumor debulking. The biopsy showed an malignant mixed germ cell tumor (yolksac and embrional carcinoma). These were confirming the diagnosis of malignant mixed germ cell mediastinal tumor (yolksac and embrinonal carcinoma). Patient received a combination bleomycin, etoposid, cisplatin 3 series chemoteraphy and evaluated. Conclusion: Some procedures like biopsy, tumor marker, could differentiate the subtype of mediastinal germ cell tumor. The multimodality treatment by combining surgical therapy (surgical sternotomy and tumor debulking) with chemotherapy could increase the survival rate of patients.


2020 ◽  
Vol 22 (Supplement_3) ◽  
pp. iii333-iii333
Author(s):  
Lei Wen ◽  
Zhaoming Zhou ◽  
Qingjun Hu ◽  
Juan Li ◽  
Mingyao Lai ◽  
...  

Abstract PURPOSE Intracranial non-germinomatous germ cell tumors (NGGCTs) have lower overall survival than germinoma because relatively higher recurrence usually occurs after first line therapy. METHODS Between January 2003 and December 2018, 111 consecutive patients diagnosed with NGGCTs reviewed. Those who progressed after first line therapy were included in this study. Data of first line treatment, salvage treatment, clinicopathological features and survival were collected and analyzed. RESULTS Totally, thirty patients (30/111, 27.0%) relapsed in our cohort, including 19 patients with accurate relapse information detail, and 11 patients who died of disease progression during follow up but without exact time and site of relapse. The median OS from diagnosis of the disease was 49.2 months (95% CI: 14.1 to 84.3 months) and 3-year OS was 54.3%. Patients who received both CSI and chemotherapy relapsed less than those who received reduced volume of radiotherapy or only CSI or only chemotherapy (22.5% vs. 45.5%, p=0.034). Of 19 patients who had detail information of recurrence time and site, the median time from diagnosis of disease to relapse was 9.5 months (2.2 to 72.1 months). Regarding to recurrence site, most patients relapsed in primary site (10/19, 52.6%) or distant intracranial (6/19, 31.6%). The recurrence site of other 3 patients were spinal (n=1), ventricular (n=1) and peritoneal (n=1). CONCLUSION Protracted follow-up is recommended because late recurrence is not uncommon. Primary tumor site and distant intracranial are the most prevalent relapsed location. Patients who relapsed could benefited from both CSI and salvage chemotherapy.


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.


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