scholarly journals Epidemiology, natural history, and optimal management of neurohypophyseal germ cell tumors

2020 ◽  
pp. 1-9
Author(s):  
Hirokazu Takami ◽  
Christopher S. Graffeo ◽  
Avital Perry ◽  
Caterina Giannini ◽  
David J. Daniels
Keyword(s):  
Natural History ◽  
Germ Cell ◽  
Germ Cell Tumors ◽  
Local Therapy ◽  
Hormone Deficiency ◽  
Pituitary Insufficiency ◽  
Single Institution ◽  
Presenting Symptoms ◽  
Biochemical Abnormalities

OBJECTIVEIntracranial germ cell tumors (iGCTs) often arise at the neurohypophysis, their second most common origination, following the pineal region. Neurohypophyseal iGCTs present with stereotypical symptoms, including pituitary dysfunction and visual field deficit, due to their suprasellar location. The goal of this study was to present a large, longitudinal single-institution experience with neurohypophyseal iGCTs to better understand their natural history and identify opportunities for further improvement in treatment outcomes.METHODSThis is a retrospective, single-institution cohort study of neurohypophyseal iGCTs treated between 1988 and 2017, with a focus on the epidemiology, presentation, natural history, and treatment.RESULTSThirty-five neurosurgically managed patients met inclusion criteria; the median age was 18 years (3 months to 49 years), and 74% of patients were male (n = 26). Thirty-one tumors were germinomas, and 4 were nongerminomatous iGCTs. Presenting symptoms included pituitary insufficiency in 76% (n = 25), visual deficit in 45% (n = 15), and diabetes insipidus (DI) in 61% (n = 20) of patients. Index symptoms included isolated DI in 10 (36%), isolated hormone deficiency in 14 (50%), and concomitant DI and hormone deficiency symptoms in 4 (14%). Radiographic diagnostic latency was common, occurring at a median of 363 days (range 9–2626 days) after onset of the first symptoms and was significantly associated with both DI and hormone deficiency as the index symptoms (no DI vs DI: 360 vs 1083 days, p = 0.009; no hormone deficiency vs hormone deficiency: 245 vs 953 days, p = 0.004). Biochemical abnormalities were heterogeneous; each pituitary axis was dysfunctional in at least 1 patient, with most patients demonstrating at least 2 abnormalities, and pretreatment dysfunction demonstrating a nonsignificant trend toward association with long-term posttreatment hormone supplementation. Among germinomas, whole-brain or whole-ventricle radiotherapy demonstrated significantly improved progression-free and overall survival compared with local therapy (p = 0.009 and p = 0.004, respectively).CONCLUSIONSNeurohypophyseal iGCTs are insidious tumors that may pose a diagnostic dilemma, as evidenced by the prolonged latency before radiographic confirmation. Serial imaging and close endocrine follow-up are recommended in patients with a characteristic clinical syndrome and negative imaging, due to the propensity for radiographic latency. Pretreatment biochemical abnormalities may indicate higher risk of posttreatment pituitary insufficiency, and all patients should receive careful endocrine follow-up. Local radiotherapy is prone to treatment failure, while whole-ventricle treatment is associated with improved survival in germinomas.

scholarly journals GCT-27. CLINICAL FEATURES AND PROGNOSTIC FACTORS OF NONGERMINOMATOUS GERM CELL TUMORS IN 111 CONSECUTIVE PATIENTS IN A SINGLE INSTITUTION: IMPACT OF IRRADIATION AND CHEMOTHERAPY CYCLES ON SURVIVAL

2020 ◽  
Vol 22 (Supplement_3) ◽  
pp. iii333-iii333
Author(s):  
Lei Wen ◽  
Juan Li ◽  
Qingjun Hu ◽  
Mingyao Lai ◽  
Cheng Zhou ◽  
...  
Keyword(s):  
Prognostic Factors ◽  
Germ Cell ◽  
Chinese Population ◽  
Germ Cell Tumors ◽  
Tumor Diameter ◽  
Limited Data ◽  
Single Institution ◽  
Large Institution ◽  
Significant Difference

Abstract BACKGROUND Limited data is available in intracranial nongerminomatous germ cell tumors (NGGCTs) in Chinese population. Here we aimed to retrospectively assess the clinical-pathological and prognostic factors of NGGCTs in a single large institution in China. METHODS From June 2003 to December 2018, 111 consecutive NGGCTs were treated in Guangdong Sanjiu Brain Hospital, China. RESULTS The median follow-up was 36.2 months (range, 1.2 to 131.2 months). Three-year EFS and OS for 111 NGGCTs patients were 78.5%±4.5% and 82.8%±4.0%, respectively. 98 patients received CSI plus boost yielded better survival than those who received reduced-volume radiotherapy or no radiotherapy (3y OS, 86.7% vs. 51.4%, p=0.007). Patients had at least four cycles of chemotherapy were strongly associated with improved 3-year OS, compared to those received less than 4 cycles (94.1% vs. 63.6%, p＜0.001). There was no significant difference in survival of patients stratified by age, surgery, hydrocephalus, as well as tumor diameter. Multivariate analysis identified chemotherapy cycles less than 4 was the only prognostic factor that conferring a worse OS (p=0.003). Patients both received CSI and at least 4 courses of chemotherapy were correlated with lower incidence of relapse (p=0.044). CONCLUSIONS Multimodal approach including CSI and enough courses of chemotherapy was effective and should be recommended for the treatment of newly diagnosed NGGCTs in Chinese population.

Prevalence of childhood growth hormone deficiency in survivors of pediatric intracranial germ cell tumors.

2020 ◽  
Vol 38 (15_suppl) ◽  
pp. e22526-e22526
Author(s):  
Diana Lone ◽  
Karim Thomas Sadak ◽  
Bradley S Miller ◽  
Michelle Roesler ◽  
Jenny N Poynter
Keyword(s):  
Growth Hormone ◽  
Germ Cell ◽  
Growth Hormone Deficiency ◽  
Late Effects ◽  
Germ Cell Tumors ◽  
Hormone Deficiency ◽  
Endocrine Disorders ◽  
Intracranial Germ Cell Tumors

e22526 Background: Survival rates for childhood cancer continue to rise, and there are now greater than 420,000 survivors in the United States. However, high cure rates come at the cost of short and long-term treatment-related toxicities. Endocrine disorders are among the most common late effects and are associated with poor health outcomes and lower quality of life. Survivors of pediatric intracranial germ cell tumors (iGCTs) are at high risk for endocrine disorders, particularly for growth hormone deficiency (GHD), due to their exposures to cranial radiation, chemotherapy, and brain surgery. To date, no long-term follow-up studies have explored the late effects experienced by survivors of iGCTs. Methods: Study participants were enrolled in the Germ Cell Tumor Epidemiology Study, which is a case-parent triad study conducted using the resources of the Children’s Oncology Group’s Childhood Cancer Research Network. Eligibility criteria included diagnosis with a germ cell tumor in any location at age 0-19 years in the years 2008-2015. The study population included 233 cases with a diagnosis of iGCT. We are currently following the cohort to evaluate outcomes and late effects of treatment, including medical record review to extract data on treatment characteristics and hormone deficiencies. This interim analysis includes chart review for 57 iGCT cases. Results: Of the 57 cases reviewed, there was a male predominance (73.7%) with the highest prevalence in non-Hispanic whites (80.4%). Cases of iGCTs can be subdivided into two main histologic subtypes, germinomas (36 cases) and non-germinomatous GCTs (NGGCT, 21 cases). The median age at diagnosis was 14.6 years for the germinomas and 10.5 years for NGGCTs. Data on growth hormone deficiency (GHD) was available for 42 of the 57 cases with a median follow-up of 7.4 years. Twenty-eight of the 42 cases (66.7%) had GHD; 19 in the germinoma group and 9 in the NGGCT group (p = 0.47). 17 of those with GHD were males (p = 0.10). There was no significant difference in prevalence of GHD by age of tumor diagnosis (p = 0.20). Conclusions: Survivors of iGCTs are at high risk for growth hormone deficiency. Identifying specific risk factors for developing GHD amongst these survivors can enhance the current guidelines for screening and management.

Stage I nonseminomatous germ cell tumors of the testis: Clinical outcome of 45 patients on a surveillance protocol after orchiectomy alone at a single institution in Japan

2009 ◽  
Vol 27 (15_suppl) ◽  
pp. e16165-e16165
Author(s):  
K. Kakimoto ◽  
Y. Ono ◽  
N. Meguro ◽  
K. Takezawa ◽  
T. Yoshida ◽  
...  
Keyword(s):  
Prognostic Factors ◽  
Lymph Nodes ◽  
Germ Cell ◽  
Embryonal Carcinoma ◽  
Clinical Stage ◽  
Germ Cell Tumors ◽  
Stage I ◽  
Single Institution ◽  
Surveillance Protocol

e16165 Background: In Japan, risk-adapted treatment for patients with clinical stage I nonseminomatous germ cell tumor of the testis (NSGCTT) has been performed in very few institutions. This retrospective study was performed to evaluate histopathologic prognostic factors with stage I NSGCTT for whom careful follow-up with a surveillance protocol was possible at a single institution. Methods: We included 45 patients with a median age of 31 years (range 16 - 58) who were managed with a surveillance strategy after orchiectomy in our department between 1972 and 2006. Mean duration of follow-up was 8.1 years (range 1.4 –30). The patients were monitored at follow-up evaluation for tumor marker (AFP, beta-hCG) levels and by abdominal CT scan, chest x-ray, and physical examination. Primary testis tumor samples were assessed for prognostic factors including lymphatic and/or vascular (LV) invasion and pathological components such as the presence of embryonal carcinoma. Log-rank analyses were performed to identify prognostic factors. Results: All patients were alive and disease-free. Relapses occurred in 16 (35.6%) patients after a median follow-up of 5.7 months (range 3–45). In 11 patients (68.8 %), relapse was detected in the retroperitoneal lymph nodes. Two patients (12.5%) had metastases in the retroperitoneal lymph nodes and lungs, two patients (12.5%) had metastases in the lungs alone, and one patient (6.2%) had metastases in the retroperitoneal lymph nodes, lungs, and brain. LV invasion was identified in 17 patients, 53% of whom had relapsed, and relapse was found in 25% of 28 patients without LV invasion (p<0.01). Of 31 patients with an embryonal carcinoma component, 13 patients (42%) developed metastases, whereas 21% of those without an embryonal carcinoma component developed metastases (p=0.04). After chemotherapy and/or surgical treatment for relapse, the 5-year overall survival rate was 100%. Conclusions: As in previous reports, the presence of an embryonal carcinoma component and LV invasion appeared to be factors suggesting a high likelihood of relapse. The surveillance protocol described here is a reliable strategy for stage I NSGCTT patients if careful long-term follow-up is possible. No significant financial relationships to disclose.

scholarly journals CTNI-62. CLINICAL FEATURES AND PROGNOSTIC FACTORS OF NONGERMINOMATOUS GERM CELL TUMORS IN 111 CONSECUTIVE PATIENTS IN A SINGLE INSTITUTION: IMPACT OF IRRADIATION AND CHEMOTHERAPY CYCLES ON SURVIVAL

2020 ◽  
Vol 22 (Supplement_2) ◽  
pp. ii56-ii57
Author(s):  
Lei Wen ◽  
Juan Li ◽  
Qingjun Hu ◽  
Mingyao Lai ◽  
Cheng Zhou ◽  
...  
Keyword(s):  
Prognostic Factors ◽  
Germ Cell ◽  
Chinese Population ◽  
Germ Cell Tumors ◽  
Tumor Diameter ◽  
Limited Data ◽  
Single Institution ◽  
Large Institution ◽  
Significant Difference

Abstract BACKGROUND Limited data is available in intracranial nongerminomatous germ cell tumors (NGGCTs) in Chinese population. Here we aimed to retrospectively assess the clinical-pathological and prognostic factors of NGGCTs in a single large institution in China. METHODS From June 2003 to December 2018, 111 consecutive NGGCTs were treated in Guangdong Sanjiu Brain Hospital, China. RESULTS The median follow-up was 36.2 months (range, 1.2 to 131.2 months). Three-year EFS and OS for 111 NGGCTs patients were 78.5%±4.5% and 82.8%±4.0%, respectively. 98 patients received CSI plus boost yielded better survival than those who received reduced-volume radiotherapy or no radiotherapy (3y OS, 86.7% vs. 51.4%, p=0.007). Patients had at least four cycles of chemotherapy were strongly associated with improved 3-year OS, compared to those received less than 4 cycles (94.1% vs. 63.6%, p＜0.001). There was no significant difference in survival of patients stratified by age, surgery, hydrocephalus, as well as tumor diameter. Multivariate analysis identified chemotherapy cycles less than 4 was the only prognostic factor that conferring a worse OS (p=0.003). Patients both received CSI and at least 4 courses of chemotherapy were correlated with lower incidence of relapse (p=0.044). CONCLUSIONS Multimodal approach including CSI and enough courses of chemotherapy was effective and should be recommended for the treatment of newly diagnosed NGGCTs in Chinese population.

scholarly journals GCT-28. RECURRENCE PATTERN AND SURVIVAL FOR RELAPSED INTRACRANIAL NON-GERMINOMATOUS GERM CELL TUMORS: A SINGLE-INSTITUTION EXPERIENCE

2020 ◽  
Vol 22 (Supplement_3) ◽  
pp. iii333-iii333
Author(s):  
Lei Wen ◽  
Zhaoming Zhou ◽  
Qingjun Hu ◽  
Juan Li ◽  
Mingyao Lai ◽  
...  
Keyword(s):  
Germ Cell ◽  
Germ Cell Tumors ◽  
Late Recurrence ◽  
Salvage Chemotherapy ◽  
Recurrence Time ◽  
First Line ◽  
Primary Tumor Site ◽  
First Line Therapy ◽  
Line Therapy

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.

Adjuvant therapy of ovarian germ cell tumors with cisplatin, etoposide, and bleomycin: a trial of the Gynecologic Oncology Group.

1994 ◽  
Vol 12 (4) ◽  
pp. 701-706 ◽  
Author(s):  
S Williams ◽  
J A Blessing ◽  
S Y Liao ◽  
H Ball ◽  
P Hanjani
Keyword(s):  
Germ Cell ◽  
Gynecologic Oncology ◽  
Cell Cancer ◽  
Germ Cell Tumors ◽  
Germ Cell Cancer ◽  
Gynecologic Oncology Group ◽  
Long Term Follow Up ◽  
Acute Myelomonocytic Leukemia

PURPOSE This study was performed to determine the effectiveness of postoperative adjuvant chemotherapy in patients with surgically resected ovarian germ cell tumors. PATIENTS AND METHODS After tumor removal and thorough surgical staging, patients were enrolled on this study and treated with three courses of cisplatin, etoposide, and bleomycin (BEP). Reassessment laparotomy was required of consenting, appropriate patients initially, but became an optional procedure in 1989. RESULTS Of 93 patients assessable on this trial, 89 are continuously free of germ cell cancer. At second-look laparotomy, two other patients were found to have small foci of immature teratoma; both remain clinically free of recurrence. One received subsequent alternate chemotherapy and one did not. Thus, 91 of 93 patients are currently free of germ cell cancer. Follow-up duration ranges from 4.0 to 90.3 months, with 67 patients monitored for longer than 2 years. Acute toxicity was moderate. One patient developed acute myelomonocytic leukemia 22 months after diagnosis. Another patient was noted to have a malignant lymphoma 69 months after protocol treatment. CONCLUSION Three courses of BEP will nearly always prevent recurrence in well-staged patients with completely resected ovarian germ cell tumors and should be given to all such patients. The development of acute leukemia as a complication of treatment is disturbing and mandates careful long-term follow-up, but is unusual and does not alter the risk-to-benefit ratio of treatment.

Importance of a new tumor market TRA-1-60 in the follow-up of patients with clinical stage I nonseminomatous testicular germ cell tumors

1997 ◽  
Vol 4 (4) ◽  
pp. 321-327 ◽  
Author(s):  
Mariël E. Gels ◽  
Jan Marrink ◽  
Petra Visser ◽  
Dirk Th. Sleijfer ◽  
Jos H. J. Droste ◽  
...  
Keyword(s):  
Germ Cell ◽  
Clinical Stage ◽  
Germ Cell Tumors ◽  
Stage I ◽  
Testicular Germ Cell Tumors ◽  
Testicular Germ Cell

scholarly journals CTNI-63. PROGNOSTIC FACTORS IN PATIENTS WITH BASAL GANGLIA GERM-CELL TUMORS: A RETROSPECTIVE ANALYSIS OF THE SINGLE CHINESE INSTITUTE EXPERIENCE FROM 2009 TO 2019

2020 ◽  
Vol 22 (Supplement_2) ◽  
pp. ii57-ii57
Author(s):  
Qingjun Hu ◽  
Juan Li ◽  
Mingyao Lai ◽  
Cheng Zhou ◽  
Zhaoming Zhou ◽  
...  
Keyword(s):  
Survival Rate ◽  
Basal Ganglia ◽  
Germ Cell ◽  
Retrospective Analysis ◽  
Surgical Resection ◽  
Germ Cell Tumors ◽  
Progression Free Survival ◽  
Course Of Disease ◽  
Free Survival

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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