scholarly journals Pediatric Ovarian Growing Teratoma Syndrome

2017 ◽  
Vol 2017 ◽  
pp. 1-8 ◽  
Author(s):  
Rebecca M. Rentea ◽  
Aaron Varghese ◽  
Atif Ahmed ◽  
Alexander Kats ◽  
Michelle Manalang ◽  
...  

Ovarian immature teratoma is a germ cell tumor that comprises less than 1% of ovarian cancers and is treated with surgical debulking and chemotherapy depending on stage. Growing teratoma syndrome (GTS) is the phenomenon of the growth of mature teratoma elements with normal tumor markers during or following chemotherapy for treatment of a malignant germ cell tumor. These tumors are associated with significant morbidity and mortality due to invasive and compressive growth as well as potential for malignant transformation. Current treatment modality is surgical resection. We discuss a 12-year-old female who presented following resection of a pure ovarian immature teratoma (grade 3, FIGO stage IIIC). Following chemotherapy and resection of a pelvic/liver recurrence demonstrating mature teratoma, she underwent molecular genetics based chemotherapeutic treatment. No standardized management protocol has been established for the treatment of GTS. The effect of chemotherapeutic agents for decreasing the volume of and prevention of expansion is unknown. We review in detail the history, diagnostic algorithm, and previous reported pediatric cases as well as treatment options for pediatric patients with GTS.

2002 ◽  
Vol 5 (5) ◽  
pp. 499-507 ◽  
Author(s):  
Pimlak Charoenkwan ◽  
Christof Senger ◽  
Sheila Weitzman ◽  
Elizabeth Sexsmith ◽  
Christopher G. Sherman ◽  
...  

Twenty-nine pediatric immature teratomas were reviewed to determine the frequency and clinical significance of p53 expression. Tumors were stained for p53 expression by immunohistochemistry and results were correlated with the presence of other germ cell tumor elements and with outcome. Sequencing of p53 for mutations was performed on positive cases. Eighteen cases showed widespread positive p53 staining of the immature teratoma elements, 9 showed staining only in very occasional cells, and 2 cases showed no staining. Of the 18 positive cases, 5 recurred. All five were pure immature teratomas at diagnosis. Four recurred as immature or mature teratoma and one as a sarcoma; all except one showed frequent cells positive for p53 in the recurrent tumor. Another 5 of the 18 diffusely positive cases contained immature teratoma as well as other malignant germ cell elements at diagnosis; none of these recurred. None of the remaining eight cases with frequent positive cells, the nine cases with occasionally positive p53 staining, or the two cases with no staining recurred or demonstrated other germ cell tumor elements. We conclude that p53 expression is not unusual in immature teratoma and diffuse p53 immunopositivity is associated with recurrence or the presence of malignant elements in ~50% of cases. In only 1 of 29 cases tested was p53 immunopositivity associated with mutations in the p53 gene; hence, overexpression in the majority of cases is presumed to reflect increased half-life of the protein from undetermined stabilizing factors. Expression of p21, a p53 target gene, was only focal, suggesting impaired transcriptional activation by p53. The finding of frequent p53-positive cells in immature teratoma should prompt a search for malignant elements within the tumor and affected patients should be followed closely for evidence of recurrence.


Neurosurgery ◽  
2005 ◽  
Vol 56 (1) ◽  
pp. E191-E194 ◽  
Author(s):  
Wenya Linda Bi ◽  
Serguei I. Bannykh ◽  
Joachim Baehring

Abstract OBJECTIVE AND IMPORTANCE: We report a rare complication after resection of a recurrent intracranial nongerminomatous germ cell tumor in an adult. The growing teratoma syndrome, as originally described with pediatric germ cell neoplasms, represents tumor recurrence, often cystic, that sometimes is observed after partial response to multimodality therapy and despite decreasing tumor serum markers. The enlarging tumor consists of elements of a mature teratoma that presumably are refractory to chemotherapy or radiation. To our knowledge, this is only the third case of the growing teratoma syndrome in an adult patient with nongerminomatous germ cell tumor. CLINICAL PRESENTATION: A 26-year-old man had signs of recurrent obstructive hydrocephalus 6 months after multimodality treatment of a diencephalic yolk sac tumor and endoscopic third ventriculostomy. Imaging studies revealed large multilocular cystic masses originating from the tumor bed and partially obstructing the ventriculostomy. INTERVENTION: Near total tumor resection and fenestration was performed. Histopathological analysis demonstrated a mature teratoma. CONCLUSION: Surgical resection, if feasible, is the treatment of choice for the growing teratoma syndrome to establish the correct diagnosis and prevent complications.


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.


Open Medicine ◽  
2021 ◽  
Vol 16 (1) ◽  
pp. 892-898
Author(s):  
Xianwen Hu ◽  
Dandan Li ◽  
Jinhua Xia ◽  
Pan Wang ◽  
Jiong Cai

Abstract Mixed germ cell tumor (MGCT) mainly occurs in young women’s ovaries and men’s testicles and rarely occurs outside the gonad. Fewer than 10 cases of mediastinal MGCT are available in PubMed, Embase, and other databases in English, while mediastinal MGCT with three pathological components, such as yolk sac tumor, immature teratoma, and embryonal carcinoma, has not been reported previously. A 12-year-old male sought medical attention for chest discomfort and underwent a computed tomography (CT) scan. A large soft tissue mass occupying most of the left thoracic cavity and mediastinum was detected. A CT-guided biopsy was performed, and an MGCT was diagnosed with pathological components, including yolk sac tumor, immature teratoma, and a small amount of embryonal carcinoma. Due to the large size of the tumor, the patient was treated with an EP regimen (etoposide + cisplatin) and paclitaxel + ifosfamide + cisplatin interstitial chemotherapy. The patient was followed up for 6 months and was alive with the disease. To the best of our knowledge, this is the 10th patient with MGCT in the mediastinum. The incidence of mediastinal MGCT is low, but it should still be considered one of the differential diagnoses of isolated pleural fibroma and neurogenic tumors.


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 ◽  
...  

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.


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