scholarly journals Malignant ovarian germ cell tumors in children: A single centre experience

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.

Author(s):  
Faraz S. Vali ◽  
Amit Kyal ◽  
Parul I. Chaudhary ◽  
Sujatha Das ◽  
Aprateem Mukherjee ◽  
...  

Germ cell tumors represent only 20% to 25% of all benign and malignant ovarian neoplasms. Mixed germ cell tumors are a rare variety of non–dysgerminomatous germ cell tumors. They contain two or more elements; the most frequent combination being a dysgerminoma and an EST (Endodermal Sinus Tumor). We present a case of malignant mixed germ cell tumor comprising of yolk sac tumor, embryonal carcinoma and choriocarcinoma. A 13-year-old girl presented with a huge 25 x 18 cm mass in abdomen with raised values of CA-125, hCG, AFP (alpha-feto protein) and LDH (lactate dehydrogenase). She underwent laparotomy followed by unilateral salpingoopherectomy and infracolic omentectomy. Histopathology report revealed malignant mixed germ cell tumor comprising predominantly of EST with elements of embryonal carcinoma and non-gestational choriocarcinoma. Following surgery, she was started on adjuvant chemotherapy (Bleomycin, Etoposide and Cisplatin regimen). Mixed germ cell tumor (YST/EST, non-gestational choriocarcinoma and embryonal carcinoma) is a very rare tumor. Careful initial surgery with adequate staging biopsies followed by combination chemotherapy can greatly improve the prognosis of these patients


2019 ◽  
Vol 1 (Supplement_2) ◽  
pp. ii23-ii23
Author(s):  
Kaishi Satomi ◽  
Hirokazu Takami ◽  
Shintaro Fukushima ◽  
Yoichi Nakazato ◽  
Shota Tanaka ◽  
...  

Abstract BACKGROUND Gain of short arm of chromosome 12 (12p) is commonly observed in testicular germ cell tumors (tGCTs). 12p gain is also frequently seen in intracranial GCTs (iGCTs). However, little is known about the clinical significance of 12p gain in iGCTs. MATERIALS AND METHODS We have collected over 200 fresh frozen tissue samples of iGCTs through the Intracranial Germ Cell Tumor Genome Analysis Consortium in Japan. Firstly, we analyzed DNA methylation status in 83 iGCTs, 3 seminomas and 6 normal control samples using Infinium Human Methylation 450K BeadChip array (Illumina, CA). Idat files were processed using R (Version 3.5.3) and minfi package (1.30.0) to generate copy number variations. Compared with average genome-wide copy number level, 12p gain was determined. Then, 58 iGCTs with clinicopathological information were analyzed for progression-free survival (PFS) and overall survival (OS). Those tumors that consist of only either germinoma and/or mature teratoma components were classified as Favorable Histology (FH) and all the others that contains malignant histological components were classified as Unfavorable Histology (UFH). RESULT 12p gain was observed in 100% (3/3) of seminoma, 13.6% (3/22) of germinoma, 16.7% (1/6) of mature teratoma, 25% (1/4) of immature teratoma, 55% (11/20) of mixed germ cell tumor, 100% (4/4) of yolk sac tumor, 100% (1/1) of embryonal carcinoma, and 100% (1/1) of choriocarcinoma. In total, 44.6% (37/83) of iGCT showed 12p gain. Regarding histological classification, the 12p gain rate in UFH (72%, 18/25) was significantly higher than that in FH (12.1%, 4/33, P<0.01). Both PFS and OS were significantly worse in iGCTs with 12p gain (PFS: P=0.027, OS: P=0.0012). DISCUSSION 12p gain can be a molecular marker to predict prognosis and histological malignancy in iGCTs.


2020 ◽  
Vol 22 (1-2) ◽  
pp. 88-92
Author(s):  
Rumita Kayastha ◽  
S Pradhan ◽  
R Acharya ◽  
M Aryal ◽  
A Shrestha ◽  
...  

Primary testicular germ cell tumors (PGCT) can be classified as seminomatous and non-seminomatous germ-cell tumor (NSGCT) types. Mixed germ cell tumors (MGCT), a subtype of NSGCT, contain more than one germ cell components. Here, we present a rare case of a MGCT composed of yolk sack tumor and teratoma which had a continuous large abdominal and retroperitoneal extension. A 43 years old male presented with complaints of discomfort and swelling over the right inguinoscrotal region. Ultrasonography (USG) showed a large ill-defined heteroechoic mass in the right inguinoscrotal region with vascularity and without separate visualization of right testis. Subsequent contrast enhanced Computed Tomography (CT) showed large enhancing mass in the right scrotal sac which was continuous with large abdominopelvic and retroperitoneal mass through the right inguinal canal. Tru-Cut biopsy of the scrotal mass showed MGCT with yolk sac and teratoma component. Patient underwent 6 cycles of chemotherapy followed by Right Radical Inguinal Orchidectomy.


2005 ◽  
Vol 8 (1) ◽  
pp. 92-97 ◽  
Author(s):  
Sevgi Bakaris ◽  
Sefa Resim ◽  
Nurdan Tunali

We report the case of a 17-year-old male with a testicular tumor and high serum levels of α-fetoprotein. The patient was treated with surgery followed by combination chemotherapy with bleomycin, etoposide, and cisplatin. Histologic examination showed features of a mixed germ cell tumor composed of mature teratoma, immature teratoma, embryonal carcinoma, yolk sac tumor, and polyembryoma. He is currently well, and his serum levels of α-fetoprotein have been normal more than 5 months after treatment. His brother, aged 17 years at the time, had a similar tumor removed from the right testicle 5 years previously.


2019 ◽  
Vol 6 (2) ◽  
pp. 611
Author(s):  
Siddhartha Verma ◽  
Heeralal Jakhar

Cryptorchidism is the most common predisposing factor in the development of testicular germ cell tumors. Seminoma is the most common malignancy developing in a cryptorchid testis. A rare case of seminoma with mixed germ cell tumor in an undescended testis is reported here. A 35-year-old male patient presented with swelling in left inguinal region science 1.5year. This  was smooth, firm to hard in consistency, restricted mobility and his left scrotum was empty. Serological markers α-FP, β-HCG, LDH were raised.  Sonography and CT scan revealed a testicular tumor in undescended left inguinal testis. High inguinal orchidectomy was done. Patient had an uneventful recovery. The histopathology report of biopsy revealed a seminoma with mixed germ cell tumor. Early diagnosis and management of the undescended testicle are needed to preserve fertility and improve early detection of testicular malignancy. Therapy should begin between six months and two years of age and may consist of hormone or surgical treatment.


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.


2016 ◽  
Vol 2016 ◽  
pp. 1-4 ◽  
Author(s):  
Houda Alatassi ◽  
Brittany E. O’Bryan ◽  
Jamie C. Messer ◽  
Zhenglong Wang

Adult extrarenal nephroblastoma is a very rare tumor. Nephroblastoma arising from primary testicular germ cell tumor is exceedingly rare. To our knowledge, only three cases have been reported in the English literature. We report a case of a 19-year-old man who presented with a large right testicle. Image studies showed a large retroperitoneal mass along with liver and lung metastases. Orchiectomy demonstrated a mixed germ cell tumor composed of yolk sac tumor, embryonal carcinoma, and mature and immature teratoma with a significant portion of nephroblastoma. The patient received chemotherapy and no recurrence was noted during six months of followup. WT-1 expression was also studied due to the lack of consistency of its expression in testicular nephroblastoma in the literature. We also present a discussion and review of the literature due to its rarity, which indicate an adverse prognosis for patients with nephroblastoma components receiving standard chemotherapeutical regimes for testicular germ cell tumors.


2005 ◽  
Vol 12 (6) ◽  
pp. 588-590 ◽  
Author(s):  
TESTUYA FUJIMURA ◽  
YUTA YAMADA ◽  
MICHIYO NASU ◽  
KIMIHISA HAMASAKI ◽  
SHIGERU MINOWADA ◽  
...  

2019 ◽  
Vol 31 (3) ◽  
pp. 395-398 ◽  
Author(s):  
Vincent J. Tavella ◽  
Jessica N. Walters ◽  
Lisa M. Crofton ◽  
Tanya LeRoith

A 5-y-old backyard Araucana–Americana rooster was presented to the regional diagnostic laboratory with a history of progressive lethargy and respiratory signs. Autopsy revealed a single large mass of testicular origin in the coelomic cavity, causing compression of other organs. Histologically, the mass was 1 neoplasm with mixed components of 2 different germ cell tumors, namely a teratoma composed of elements of all 3 primordial germ cell lines (ectoderm, mesoderm, and endoderm), and a seminoma consisting of round or polygonal cells arranged in sheets supported by a scant fibrovascular stroma. Teratomas and seminomas are both considered to be uncommon neoplasms in poultry medicine. A testicular teratoma is composed of mature embryonic tissue derived from at least 2 of the 3 germinal layers. Seminomas and teratomas both arise from the germinal epithelium of seminiferous tubules and are classified as germ cell tumors. This neoplastic mass thus is a rare case of a mixed germ cell tumor.


Sign in / Sign up

Export Citation Format

Share Document