Site of metastases does not influence the clinical outcome of children with metastatic Germ Cell Tumors (GCT). A report from the Childrens Oncology Group (COG)

2006 ◽  
Vol 24 (18_suppl) ◽  
pp. 9002-9002
Author(s):  
M. H. Malogolowkin ◽  
W. B. London ◽  
B. Cushing ◽  
R. Giller ◽  
M. Davis ◽  
...  

9002 Background: To describe the clinical outcome of children with metastatic GCT (stage IV) at diagnosis according to the primary metastatic site(s). Methods: From March 1990 to February 1996, 299 children and adolescents with stage III/IV gonadal and stage I-IV extragonadal GCT were eligible for a Pediatric Intergroup high-risk (HR) GCT trial. Patients were randomized to receive 4–6 courses of cisplatin (P) standard dose [ 20 mg/m2/day (d) × 5] or high-dose (HDP) [40 mg/m2/d × 5] with etoposide (E) 100 mg/m2/d × 5 and bleomycin (B) 15 mg/m2 on d1. We retrospectively investigated the outcome of patients with stage IV and compared their outcome according to metastatic site(s). Results: There were 133 patients with stage IV disease. The median age was 2.6 years (y) [range, 3 d-19.3 y], 70 were female. Primary sites included: 43 testicular, 14 ovarian, 76 extragonadal (45 sacroccocygeal, 28 mediastinal, 3 other). Histologies included: 66 pure yolk sac tumors, 21 immature teratomas and yolk sac tumors, 26 mixed germ cell tumors, 7 pure germinoma/seminoma/dysgerminomas, 1 immature teratoma with a non-classic germ cell tumor, 2 mixed germ cell tumor admixed with a nonclassic germ cell tumor, 5 pure choriocarcinomas, and 5 patients with unknown histology. There were no statistically significant differences in the 5-year EFS or OS rates by site of metastases. Of the 19 patients with either bone or brain involvement, 17 patients had bone and 3 had brain metastases. Conclusion: The outcome for patients with metastatic GCT is excellent with contemporary cisplatin-based regimes and is independent of the site of metastatic disease. [Table: see text] No significant financial relationships to disclose.

2020 ◽  
Vol 35 (2) ◽  
pp. 119-122
Author(s):  
SM Rashed Zahangir Kabir ◽  
Md Waheed Akhtar ◽  
Farida Yasmin

Introduction: Germ cell tumors are a group of tumors with different clinical presentation and histological and biological characteristics. Malignant germ cell tumors occur at all ages with a trend of bimodal distribution in infancy and adolescence. Objective: To evaluate the demographic characteristics, distribution of different types of germ cell tumor, treatment modalities and outcome of germ cell tumor in children in a tertiary care hospital of Bangladesh. Methods: In this retrospective study, data regarding age and sex distribution, location, types of tumors, management of germ cell tumor in children were retrieved from the medical records of pediatric oncology department in NICRH, Dhaka from 2008 to 2014. Results: Out of total 87 patients female were 50 and male 37. Most of the patients were up to 5 years of age. The gonadal germ cell tumors (80%) were more than extragonadal tumor (20%) in both male and female patients. The most common germ cell tumor was dysgerminoma (32%) followed by yolk sac tumor (29.8%) and teratoma (19.5%). Yolk Sac Tumor (51.4%) was the most common in male and dysgerminoma (56%) the commonest in female. Out of 87, seventy two (82.7%) received chemotherapy following surgery. Among those 72 patients who received chemotherapy 49 (68 %) patients completed their treatment. Until the last follow up 71.4% patients remained alive and tumor free. Conclusion: Germ cell tumors are the most variable tumor of all childhood malignancies that has difference in age, sex, location and histological subtypes. Gonadal tumors have better prognosis than extragonadal tumors in both the sex. DS (Child) H J 2019; 35(2) : 119-122


2016 ◽  
Vol 34 (4) ◽  
pp. 303-306 ◽  
Author(s):  
Timothy Gilligan

The Oncology Grand Rounds series is designed to place original reports published in the Journal into clinical context. A case presentation is followed by a description of diagnostic and management challenges, a review of the relevant literature, and a summary of the authors' suggested management approaches. The goal of this series is to help readers better understand how to apply the results of key studies, including those published in Journal of Clinical Oncology, to patients seen in their own clinical practice. A 32-year-old man with a history of a mixed germ cell tumor of the testis presented with acute-onset, right-sided weakness and numbness. His previous treatment included orchiectomy, which revealed a 5-cm tumor that was 95% yolk sac tumor and 5% embryonal carcinoma, and retroperitoneal lymph node dissection for clinical stage I disease in January 2010, which revealed no nodal metastases. Starting in June 2010, he was treated with four cycles of etoposide and cisplatin for pulmonary and thoracic lymph node metastases and a rising serum alpha-fetoprotein (AFP) level. He subsequently received four cycles of paclitaxel, ifosfamide, and cisplatin for relapse in the lungs and mediastinal nodes with a rising AFP level starting in January 2011. He reported having a 2-week history of intermittent headaches in December 2011, when he presented with acute-onset, right-sided weakness and numbness. Computed tomographs of the head was obtained and demonstrated a left parietal intracranial hemorrhage without midline shift or hydrocephalus. Brain magnetic resonance imaging (MRI) showed a complex, 4.5-cm mass consistent with a hemorrhagic metastasis. His serum AFP level was elevated at 47 ng/mL. The patient became progressively obtunded and underwent emergency surgical decompression and resection of the tumor. Histopathologic evaluation of the resected tissue showed metastatic germ cell tumor predominantly consisting of a yolk sac element ( Fig 1 ). His AFP level declined rapidly after resection, and computed tomography of the chest, abdomen, and pelvis showed no evidence of metastatic disease. However, 2 weeks later, his AFP level rose again, and repeat MRI of the brain showed a 3-cm mass in the left mesial parietal lobe adjacent to the resection site. He started treatment with filgrastim to facilitate collection of circulating hematopoietic stem cells. Several days later, after apheresis, he received his first of two cycles of high-dose carboplatin 700 mg/m2 on days −5, −4, and −3 and etoposide 750 mg/m2 on days −5, −4, and −3. The patient had a complete response to high-dose chemotherapy and no major acute complications. His cancer remains in complete remission 3 years later without additional treatment. His three lines of chemotherapy left him with chronic peripheral neuropathy.


1970 ◽  
Vol 1 (2) ◽  
pp. 63-65
Author(s):  
Anil Dev Pant ◽  
Geeta Sayami ◽  
Viswanath Prasad ◽  
Anjan Shrestha

Yolk sac tumors, the most common germ cell tumor in young infants and children, however, are distinctly uncommon site in the vagina. A baby with bleeding per vaginum since one month presented at 9 months of age with raised Serum α- fetoprotein (AFP) but normal levels of ß-HCG and CA-125 is reported. Fine needle aspiration from the mass yielded material suggestive of a germ cell tumor. The histopathological findings further confirmed the diagnosis of a yolk sac tumor. Key words: yolk sac tumor, endodermal sinus tumor, pediatric, vagina, germ cell tumor, α-fetoprotein (AFP) doi:10.3126/njog.v1i2.2402 N. J. Obstet. Gynaecol Vol. 1, No. 2, p. 63-65 Nov-Dec 2006


2021 ◽  
Vol 27 (1) ◽  
pp. 47-51
Author(s):  
Alexander G. Weil ◽  
Natalie Mathews ◽  
Jean-Pierre Farmer ◽  
Christine St. Martin ◽  
Steffen Albrecht ◽  
...  

Here, the authors present 2 cases of nongerminomatous germ cell tumor (NGGCT): a neonate with a mixed malignant germ cell tumor, 5% yolk sac tumor (YST) and 95% immature teratoma components, originating from the right mesial temporal lobe; and a 2-month-old infant with a pure YST originating from the left middle cranial fossa. These tumors with yolk sac components, which are thought to have a poor prognosis, were successfully treated with complete tumor resection alone and subtotal tumor resection with chemotherapy, respectively. Event-free survival exceeds 5 years for each patient even though neither received radiotherapy. The authors highlight the role of radical surgery and the successful treatment of neonatal YST with aggressive resection (and chemotherapy in 1 case) while avoiding radiation therapy. They also report the very rare non-midline location of these neonatal NGGCTs and emphasize the importance of considering YSTs and mixed NGGCTs with YST components in the differential diagnosis of non-midline hemispheric or skull base tumors in newborns.


2013 ◽  
Vol 31 (6_suppl) ◽  
pp. 325-325
Author(s):  
Elizabeth O'Donnell ◽  
Kathryn P. Gray ◽  
Michelle S. Hirsch ◽  
Praful Ravi ◽  
Clair Beard ◽  
...  

325 Background: In 2011, there were about 8260 cases of Germ Cell Tumor (GCT) diagnosed in the US, of those, 350 or 4% will die of their disease. We sought to review our experience with a 10-year cohort of 819 patients treated for GCT at Dana-Farber and synthesize the cumulative findings of those who died from their disease specifically looking for different sub-types of incurable GCTs. Methods: Retrospective review of 819 germ-cell tumors treated in our center between 2000 and 2010 to identify patients not cured with cisplatin-based chemotherapy. Inclusion criteria were men over the age of 18 treated for malignant germ cell tumor between 2000 and 2010 at the Dana-Farber Cancer Institute that died from their disease. The outcomes of interest were smoking history, extent of disease at diagnosis, primary site of disease, histology, presence of lymphovascular invasion, outcomes to first- and second-line therapies, treatment with high dose chemotherapy (HDC), late relapse, brain metastases, and presence or absence of transformed teratoma as cause of death. Results: 38 men were identified. Median age 35. More than half had a smoking history. 3 presented with clinical stage 1 disease, 8 good-risk metastatic disease, 4 intermediate-risk and 22 poor-risk at diagnosis. The majority (28) had testicular primaries, 7 mediastinal, one pituitary, one retroperitoneal and one unknown. 21 of 48 had complete responses to first-line therapy. 4 received HDC for relapsed disease. 10 relapsed after 2 years of disease-free survival. 7 died of transformed teratoma. 63% progressed directly through cisplatin-based chemotherapy and died as a result of non-teratomatous germ cell tumor burden. 18% died from unresectable or transformed teratoma and 26% died after suffering a late relapse of disease. Conclusions: Within the cohort of patient who died from their GCTs there are three distinct biological subtypes – the majority is platinum-refractory germ cell tumor while unresectable/transformed teratoma and late relapse make up the remainder. Understanding the unique biology of these disease states compared with curable disease may provide informative insights into chemotherapy resistance for cancer in general.


2020 ◽  
Vol 4 (1) ◽  
pp. 43-51
Author(s):  
Galuh Ayu Treswari ◽  
Bambang Soeprijanto ◽  
Indrastuti Normahayu ◽  
Lenny Violetta

Yolk sac tumor called endodermal sinus tumor, is a rare and very malignant germ cell tumor. The second largest ovarian germ cell tumor after dysgerminoma, with an incidence of 1% of ovarian malignancies. Tumors usually appear as fast-growing masses in young women. The radiological imaging of this tumor is seen as a large dense cystic mass with heterogeneous enhancement, a component of dilated intratumoralblood vessels accompanied by an intralesional hemorrhagic focus. The best radiological modality is CT scan or MRI. CT imaging useful for distinguishing yolk sac tumors from other ovarian tumors. In this article was reported a case of ovarial york sac tumor in 7 years old girl. USGand CT scan examination showed solid with cystic mass in the pelvic cavity. Histologically was malignant germ cell as york sac tumor.


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.


2012 ◽  
Vol 2012 ◽  
pp. 1-5
Author(s):  
Toshihide Takahashi ◽  
Eiichi Ishikawa ◽  
Yosuke Masuda ◽  
Tetsuya Yamamoto ◽  
Taiki Sato ◽  
...  

Intracranial nongerminomatous germ cell tumors (NGGCTs) in unusual locations are extremely rare. Here, we report a case of a yolk sac tumor in the frontal lobe in a middle-aged patient. A 42-year-old man was admitted to our hospital for headache and nausea. Magnetic resonance imaging (MRI) showed an enhanced mass lesion with a marked cyst component. The serum alpha-fetoprotein (αFP) level was extremely high. Histological examination of specimens after subtotal removal revealed a primary mixed germ cell tumor with extensive yolk sac tumor elements, often referred to as an intracranial “yolk sac tumor.” The preoperative diagnosis of NGGCTs in unusual age and locations is extremely difficult. Clinicians should consider the possibility of NGGCTs, including yolk sac tumors, when intracranial tumors with unusual MRI findings are encountered.


2016 ◽  
Author(s):  
Priyanka Soni ◽  
Shalini Mishra ◽  
Sandeep Jain ◽  
Gauri Kapoor

Background: Germ-cell tumors (GCT) are the commonest ovarian neoplasm in the first two decades of life. Aim: To study the profile of ovarian GCT in children and their outcome. Methods: Retrospective study of all cases of malignant ovarian GCT in the pediatric age (up to 18 years) was done from January 2002 to December 2015. The medical records of all admitted cases during this period were reviewed and the data was analysed with respect to age at diagnosis, clinical presentation, tumor markers, surgical stage, tumor histology, therapy, clinical course, and outcome. Results: Girls with malignant ovarian GCT were seen at our institute during the study period. Out of these 25 underwent treatment. Mean age at presentation was 11.7 years (range: 3-18 years). Abdominal pain was the commonest presentation. Twelve (47.3%) had right sided disease, 11 (42%) had left sided disease and 2 had bilateral disease. Twelve cases (57.8%) were diagnosed as stage I disease, 5 (10.5%) as stage II, 7 (26.3%) as stage III and 1 (5.2%) as stage IV. Elevated AFP >1000 was found in 9 (47.3%), elevated B-HCG (>50) in 7 (42%) and elevated LDH (>1000) in 7 (36.8%) patients at presentation. Twenty (73.6%) patients underwent surgery prior to chemotherapy out of which 4 (21%) patients presented after undergoing surgery at other centre. Fourteen (57.8%) patients received 4 cycles of BEP based chemotherapy, 6 (21%) received 3 cycles, 2 (10.5%) received 2 cycles and 1 patient did not receive any chemotherapy as it was mature teratoma. The most common histology was dysgerminoma in 8 (42%) patients followed by mixed germ cell tumor in 4 (21%), teratoma in 3 (15.7%), embryonal carcinoma in 2 (10.5%) and yolk sac tumor and mature teratoma in 1 patient each. Four (21%) patients had relapse on contralateral side which was salvaged. 1 patient presented with relapse who underwent only surgery outside, 1 patient had ovarian torsion. Median follow up is 27months. The event free survival rate was 78.9%. Conclusion: This study confirms an excellent outcome for girls with ovarian germ cell tumor. Patients with advanced surgical stage relapsed frequently. The mainstay of treatment is fertility preserving surgery and cisplatin-based chemotherapy.


2020 ◽  
Vol 22 (Supplement_3) ◽  
pp. iii331-iii331
Author(s):  
Sang Hee Youn ◽  
Joo-Young Kim

Abstract PURPOSE To evaluate the clinical outcome of patients with intracranial germ cell tumor treated with proton beam therapy (PBT). MATERIALS AND METHODS Fifty-seven patients with intracranial germ cell tumor treated with PBT between 2009 and 2016 were retrospectively analyzed. RESULTS Median follow-up duration was 63.7 months (range, 5.6–204.5). Thirty-seven patients (64.9%) were pure germinoma and 20 patients (35.1%) were non-germinomatous germ cell tumor (NGGCT). All patients except 2 patients received chemotherapy before PBT. Twenty-one patients (36.8%) of localized germinoma were treated with whole ventricle irradiation (WVI), while 36 (63.2%) patients who were diagnosed as disseminated germinoma or NGGCT received cranio-spinal irradiation (CSI). Two patients with pure germinoma in basal ganglia showed disease relapse at 3.0 and 6.9 years after PBT at the primary site and pituitary gland, respectively. There was one patient with NGGCT who died of chemotherapy-related mortality at 4.7 years after PBT while her disease was complete remission. The 7-year progression-free survival and overall survival were 70.8% and 100% for focal germinoma, 100% and 100% for disseminated germinoma, 100% and 100% for focal NGGCTs, and 100% and 80.0% for disseminated NGGCTs, respectively. CONCLUSIONS PBT of pure germinoma resulted in comparable clinical outcomes to that with photon radiotherapy. Our result for NGGCT is also excellent compared to other reports. Failure patterns of germ cell tumors originating in basal ganglia needs to be assessed in large pooled data.


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