scholarly journals Mediastinal Teratoma with Neuroendocrine Features in 34-Year-Old Male with Syncope

2015 ◽  
Vol 2015 ◽  
pp. 1-5
Author(s):  
Peter A. Andrawes ◽  
Masood A. Shariff ◽  
Qing Chang ◽  
Fanyi Kong ◽  
Frank M. Rosell

Neuroendocrine tumors that arise in an extragonadal teratoma are extremely rare. Somatic-type malignancy, defined as any sarcoma, carcinoma, leukemia, or lymphoma developing in a germ cell tumor, occurs in approximately 2% of all germ cell tumors. Our case represents a mediastinal mass that was incidentally found in a patient with syncope. Surgical resection confirmed mature teratoma with neuroendocrine features.

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.


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.


2016 ◽  

Introduction: Immature teratoma represents 3% of all teratomas, 1 % of all ovarian cancers and 20% of malignant ovarian germ cell tumors. It is found either in pure form or as a component of a mixed germ cell tumor. It occurs essentially during the first two decades of life. According to WHO, immature teratoma is defined as a teratoma containing a variable amount of immature embryonal type neuroectodermal tissue Case: We present here a report of 23 years old unmarried female who presented with complaint of abdominal pain since 1 month and her CT scan done outside, showed fibroid uterus. She had history of typhoid fever 1 month back for which USG was done which suggested large uterine fibroid. On examination she was hemodynamically stable. On abdominal examination a non-tender supra-pubic mass of 24 weeks size with firm consistency, irregular margin was felt. On investigation CA 125 was 64.90 IU/L, LD- 223, beta HCG- 1.14. On MRI a large abdomino-pelvic lesion, likely left adnexal lesion with multiple cystic areas, with hemorrhage, with ascites and enlarged retroperitoneal lymph nodes with omental infiltration suggestive of a possibility of malignant germ cell tumor. In view of large ovarian tumor, possibly malignant decision for staging laparotomy was taken. Intra-operatively a large irregular vascular solid mass of 20 x 20 cms with bosselated appearance with few cystic lesions over it was seen, arising from left ovary and was sent for frozen section which reported malignant mature teratoma with components of immature teratoma. She underwent laparotomy with left salpingo-oophorectomy with right ovarian biopsy, omentectomy, appendectomy with B/L pelvic lymphadenectomy. Histopathology was suggestive of grade III immature teratoma. In view of grade III immature teratoma, she received chemotherapy (BEP regimen) post-operatively and is currently under follow up. Conclusion: This case reflects the importance of early diagnosis in cases of pelvic masses in young females. Fertility preservation should be considered in young women with germ cell tumors. Patients with grade II or III tumors or a mere advanced stage disease should be treated with adjuvant chemotherapy (BEP) in addition to surgery.


2020 ◽  
Vol 22 (Supplement_3) ◽  
pp. iii336-iii336
Author(s):  
Kaishi Satomi ◽  
Hirokazu Takami ◽  
Shintaro Fukushima ◽  
Yoichi Nakazato ◽  
Shota Tanaka ◽  
...  

Abstract Gain of short arm of chromosome 12 (12p) is commonly observed in testicular germ cell tumors (tGCTs) and also seen in intracranial GCTs (iGCTs). However, little is known about the clinical significance of 12p gain in iGCTs. 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 profile in 83 iGCTs, 3 tGCTs (seminomas) and 6 normal control samples using Infinium Human Methylation 450K BeadChip array (Illumina, CA, USA) in order to determine 12p gain status. Then, fluorescence in situ hybridization (FISH) study was carried out on 3 mixed iGCT cases using 12p/CEP12 probe (Abbott Molecular, Abbott park, IL, USA). Lastly, 58 iGCTs with clinicopathological information were analyzed for progression-free survival (PFS) and overall survival (OS). Gain of 12p was observed in 100% (3/3) of seminoma, 14% (3/22) of germinoma, 17% (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, 45% (37/83) of iGCT showed 12p gain. Different histological components in each mixed GCT shared the same 12p copy number status within each mixed GCT case. Both PFS and OS were significantly worse in iGCTs with 12p gain (PFS: P=0.027, OS: P=0.0012). Gain of 12p can be a molecular marker to predict prognosis and represents an early event in tumorigenesis prior to histological differentiation in iGCTs.


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.


1992 ◽  
Vol 61 (1) ◽  
pp. 67-73 ◽  
Author(s):  
Willem E. de Graaff ◽  
J. Wolter Oosterhuis ◽  
Bauke de Jong ◽  
Jannie van Echten-Arends ◽  
Janneke Wiersema-Buist ◽  
...  

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


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


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