Prognostic Value of Tumor Size, Metastases, Extension into Bone, and Increased Tumor Marker in Children With Malignant Sacrococcygeal Germ Cell Tumors: A Prospective Evaluation of 71 Patients Treated in the German Cooperative Protocols Maligne Keimzelltumoren (MAKEI) 83/86 and MAKEI 89

2003 ◽  
Vol 21 (5) ◽  
pp. 781-786 ◽  
Author(s):  
G. Calaminus ◽  
D.T. Schneider ◽  
J.P.M. Bökkerink ◽  
H. Gadner ◽  
D. Harms ◽  
...  

Purpose: To evaluate the prognostic value of metastases, extension into bone, and alpha-fetoprotein (AFP) elevation in children with malignant sacrococcygeal germ cell tumors (GCTs) prospectively collected in two cooperative Maligne Keimzelltumoren (MAKEI) protocols (83/86 and 89). Patients and Methods: Between October 1983 and October 1995, 76 of 210 registered patients with sacrococcygeal primaries presented either with pure yolk sac tumor, embryonal carcinoma (EC), or yolk sac tumor and EC mixed with immature and mature teratoma elements. Stages T1 and T2 disease were diagnosed in 15 and 61 children, respectively, 41 patients had metastases, and 35 children presented with extension into bone. At diagnosis, 22 children had an AFP elevation of less than 10,000 ng/mL. Thirty-six children showed an AFP level between 10,000 and 100,000 ng/mL, and 12 patients had values of greater than 100,000 ng/mL. Five patients died of complication during treatment and were excluded from further evaluation. Seventy-one patients could be analyzed. Results: The 5-year relapse-free survival rate (RFS, Kaplan-Meier) was 0.76 ± 0.03 (54 of 71 patients; median observation time, 54 months after diagnosis). The RFS of patients with and without metastases was different, but not significantly so (0.71 v 0.82). The outcome of patients with extension into bone (n = 31) and without this extension (n = 40) was 0.71 versus 0.80 (RFS, 5 years). Above-normal AFP level had no prognostic significance (P = .52). Conclusion: In children with malignant sacrococcygeal GCTs treated with an intensive, short-interval, platinum-based regimen, the stage, extent of metastases, extension into bone, and AFP level had no prognostic significance.

1998 ◽  
Vol 5 (1) ◽  
pp. E3
Author(s):  
Keiji Sano

The author studied 153 cases of intracranial germ cell tumors (GCTs) through 1994, 62.7% of which showed monotypic histological patterns and 37.3% of which were shown to be mixed tumors. All of these cases, except for six patients who died soon after admission and underwent autopsy, underwent surgery followed by radio- and/or chemotherapy. All patients with choriocarcinoma died within 2 years. Patients with yolk sac tumor (endodermal sinus tumor) and embryonal carcinoma also had poor outcomes. Patients with mature teratoma had 5- and 10-year survival rates of 92.9% each. Patients with immature teratoma and malignant teratoma had a 5- and 10-year survival rate of 70.7% each. Patients with germinoma had a 5-year survival rate of 95.4% and a 10-year survival rate of 92.7%. These results may bring into question the validity of the germ cell theory, because germinoma, which should be the most undifferentiated according to the theory, was the most benign and choriocarcinoma and yolk sac tumor (endodermal sinus tumor), which should be the most differentiated, were the most malignant according to results obtained during follow-up study. Therefore, GCTs other than germinoma may not originate from one single type of cell (primordial germ cells). The embryonic cells of various stages of embryogenesis may perhaps be misplaced in the bilaminar embryonic disc at the time of the primitive streak formation, becoming involved in the stream of lateral mesoderm and carried to the future cranial area to become incorrectly enfolded into the brain at the time of the neural tube formation. The authors propound the following law: tumors composed of cells resembling the cells that appear in the earlier stages of embryogenesis (ontogenesis) are more malignant than those composed of cells resembling the cells that appear in the later stages of embryogenesis.


2017 ◽  
Vol 26 (3) ◽  
pp. 319-324 ◽  
Author(s):  
Akitake Mukasa ◽  
Shunsuke Yanagisawa ◽  
Kuniaki Saito ◽  
Shota Tanaka ◽  
Keisuke Takai ◽  
...  

Primary spinal germ cell tumors are rare, and spinal nongerminomatous germ cell tumors represent an even rarer subset for which no standard therapy has been established. The authors report the case of a 24-year-old woman with multifocal primary spinal germ cell tumors scattered from T-12 to L-5 that consisted of yolk sac tumor and mature teratoma. After diagnostic partial resection, the patient was treated with 30 Gy of craniospinal irradiation and 30 Gy of local spinal irradiation, followed by 8 courses of chemotherapy based on ifosfamide, cisplatin, and etoposide (ICE). Salvage surgery was also performed for residual mature teratoma components after the third course of ICE chemotherapy. Chemotherapy was continued after the operation, but ifosfamide was entirely eliminated from the ICE regimen because severe myelosuppression was observed after previous courses. The patient remains recurrence free as of more than 5 years after the completion of chemotherapy. This case suggests that this treatment strategy is an effective option for primary spinal yolk sac tumor.


2016 ◽  
Vol 2016 ◽  
pp. 1-4 ◽  
Author(s):  
Sammy G. Nakhla ◽  
Srinath Sundararajan

Mediastinal germ cell tumors are extragonadal germ cell tumors (EGGCTs) commonly seen in children and young adults. They are more common in men. Clinically they are classified as teratomas, seminomas, and nonseminomatous germ cell tumors. Primary mediastinal yolk sac neoplasm is an extremely rare tumor. We present here a very rare case of primary yolk sac tumor of the anterior mediastinum in a 73-year-old male. Mediastinal germ cell tumors have a worse prognosis than gonadal germ cell tumors. Chemotherapy followed by adjuvant surgery improves overall response in EGGCTs. However, comorbidities can render treatment with chemotherapy and surgery challenging in elderly patients.


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


2019 ◽  
Vol 29 (2) ◽  
pp. 398-403 ◽  
Author(s):  
Beijiao Qin ◽  
Wenyan Xu ◽  
Yanfang Li

ObjectiveTo evaluate the role of omentectomy and lymphadenectomy in the treatment of clinically apparent early-stage malignant ovarian germ cell tumors.MethodsWe retrospectively reviewed 245 patients with malignant ovarian germ cell tumors (yolk sac tumor, dysgerminoma, and immature teratoma) and with clinically early-stage disease, who were treated at Sun Yat-sen University Cancer Center between January 1, 1970 and December 31, 2017. The survival of patients who underwent either omentectomy or lymphadenectomy, or both (omentectomy/lymphadenectomy group) was compared with that of patients who did not undergo omentectomy or lymphadenectomy (non-omentectomy/lymphadenectomy group).ResultsSixty patients were diagnosed with yolk sac tumor, 74 with dysgerminoma, and 111 with immature teratoma. Of these 245 patients, 216 patients had stage I disease, 28 patients had stage II, and 1 patient had stage IIIA. There were 190 patients who underwent omentectomy and/or lymphadenectomy and 55 patients in the non-omentectomy/lymphadenectomy group, respectively. In the omentectomy/lymphadenectomy group, 112 patients underwent both omentectomy and lymphadenectomy, 71 underwent omentectomy only, and 7 underwent lymphadenectomy only. Two hundred and fourteen of 245 patients (87.3%) received post-operative chemotherapy. Median follow-up was 73 months (range 1–388). The 10-year overall survival rates in the omentectomy/lymphadenectomy group and non-omentectomy/lymphadenectomy groups were 96.8% and 100%, respectively (p=0.340). Multivariate analysis evaluating all potential prognostic factors showed that omentectomy and lymphadenectomy are not prognostic factors for survival.ConclusionsOmentectomy and lymphadenectomy do not appear to improve survival and may be omitted in patients with clinically apparent early-stage malignant ovarian germ cell tumors.


2001 ◽  
pp. 779-784 ◽  
Author(s):  
L Cobellis ◽  
P Cataldi ◽  
FM Reis ◽  
G De Palo ◽  
F Raspagliesi ◽  
...  

OBJECTIVE: Inhibin and activin are proteins produced by ovarian granulosa cells and testicular Sertoli cells and are members of the transforming growth factor-beta superfamily. Since increased circulating levels of immunoreactive inhibin were detected in women with malignant ovarian tumors, they were proposed as tumor markers for ovarian carcinoma. Immunohistochemical studies later confirmed the presence of inhibin and activin subunits in granulosa cell tumors and epithelial ovarian cancer, as well as in Sertoli and Leydig cell testicular cancer. However, there is discrepant information on the detection of inhibin and activin in malignant germ cell tumors (MGCT). The aim of the present study was to evaluate the immunohistochemical expression of the inhibin/activin alpha, betaA and betaB subunits in ovarian and testicular MGCT specimens using polyclonal antisera. METHODS: The ovarian tissue samples were composed of 19 MGCT, including dysgerminoma (n=18) and yolk sac tumor (n=1). The testis specimens included classic seminomas (n=20), embryonal carcinomas (n=7), choriocarcinomas (n=2), and yolk sac tumor (n=1). RESULTS: Ovarian and testicular malignant germ cell tumors expressed positive staining for inhibin/activin alpha, betaA and betaB subunits, with some variations between and within individual tumors: while ovarian dysgerminomas were diffusely positive for alpha, betaA and betaB, testicular tumors expressed alpha and betaB subunits, whereas betaA staining was weak. CONCLUSIONS: The present results show positive staining for inhibin/activin subunits in ovarian and testicular MGCT, suggesting a possible role in tumorigenesis with the resultant clinical implication.


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