Successful treatment of non-midline primary malignant germ cell tumors with yolk sac components in neonates: report of 2 cases

2021 ◽  
Vol 27 (1) ◽  
pp. 47-51
Author(s):  
Alexander G. Weil ◽  
Natalie Mathews ◽  
Jean-Pierre Farmer ◽  
Christine St. Martin ◽  
Steffen Albrecht ◽  
...  
Keyword(s):  
Germ Cell ◽  
Germ Cell Tumor ◽  
Successful Treatment ◽  
Tumor Resection ◽  
Germ Cell Tumors ◽  
Middle Cranial Fossa ◽  
Yolk Sac ◽  
Cell Tumor ◽  
Malignant Germ Cell Tumor ◽  
The Right

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.

scholarly journals Pediatric Germ Cell Tumors: An Experience of 7 Years in a Tertiary Hospital of Bangladesh

2020 ◽  
Vol 35 (2) ◽  
pp. 119-122
Author(s):  
SM Rashed Zahangir Kabir ◽  
Md Waheed Akhtar ◽  
Farida Yasmin
Keyword(s):  
Germ Cell ◽  
Germ Cell Tumor ◽  
Tertiary Care ◽  
Germ Cell Tumors ◽  
Yolk Sac ◽  
Cell Tumor ◽  
Yolk Sac Tumor ◽  
Treatment Modalities ◽  
Care Hospital ◽  
Childhood Malignancies

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

scholarly journals Non Seminomatous Mixed Germ Cell Tumor of Testis with a Large Abdominal and Retroperitoneal Extension: A Case Report

2020 ◽  
Vol 22 (1-2) ◽  
pp. 88-92
Author(s):  
Rumita Kayastha ◽  
S Pradhan ◽  
R Acharya ◽  
M Aryal ◽  
A Shrestha ◽  
...  
Keyword(s):  
Germ Cell ◽  
Germ Cell Tumor ◽  
Germ Cell Tumors ◽  
Cell Tumor ◽  
Testicular Germ Cell ◽  
Mixed Germ Cell Tumor ◽  
Contrast Enhanced ◽  
Cell Components ◽  
The Right ◽  
Enhanced Computed Tomography

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.

scholarly journals Multi-region exome sequencing of ovarian teratomas reveals 2N near-diploid genomes, paucity of somatic mutations, and extensive allelic imbalances shared across mature, immature, and disseminated components

2019 ◽  
Author(s):  
Michael B. Heskett ◽  
John Z. Sanborn ◽  
Christopher Boniface ◽  
Benjamin Goode ◽  
Jocelyn Chapman ◽  
...  
Keyword(s):  
Germ Cell ◽  
Exome Sequencing ◽  
Loss Of Heterozygosity ◽  
Germ Cell Tumor ◽  
Ovarian Tumor ◽  
Somatic Mutations ◽  
Immature Teratoma ◽  
Cell Tumor ◽  
Malignant Germ Cell Tumor ◽  
The Right

AbstractImmature teratoma is a subtype of malignant germ cell tumor of the ovary that occurs most commonly in the first three decades of life, frequently with bilateral ovarian disease. Despite being the second most common malignant germ cell tumor of the ovary, little is known about its genetic underpinnings. Here we performed multi-region whole exome sequencing to interrogate the genetic zygosity, clonal relationship, DNA copy number, and mutational status of 52 pathologically distinct tumor components from 10 females with ovarian immature teratomas, with bilateral tumors present in 5 cases and peritoneal dissemination in 7 cases. We found that ovarian immature teratomas are genetically characterized by 2N near-diploid genomes with extensive loss of heterozygosity and an absence of genes harboring recurrent somatic mutations or known oncogenic variants. All components within a single ovarian tumor (immature teratoma, mature teratoma with different histologic patterns of differentiation, and yolk sac tumor) were found to harbor an identical pattern of loss of heterozygosity across the genome, indicating a shared clonal origin. In contrast, the 4 analyzed bilateral teratomas showed distinct patterns of zygosity changes in the right versus left sided tumors, indicating independent clonal origins. All disseminated teratoma components within the peritoneum (including gliomatosis peritonei) shared a clonal pattern of loss of heterozygosity with either the right or left primary ovarian tumor. The observed genomic loss of heterozygosity patterns indicate that diverse meiotic errors contribute to the formation of ovarian immature teratomas, with 11 out of the 15 genetically distinct clones determined to result from the failure of meiosis I or II. Overall, these findings suggest that copy-neutral loss of heterozygosity resulting from meiotic abnormalities may be sufficient to generate ovarian immature teratomas from germ cells.

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 ◽  
...  
Keyword(s):  
Clinical Outcome ◽  
Germ Cell ◽  
Germ Cell Tumor ◽  
Germ Cell Tumors ◽  
Stage Iv ◽  
Yolk Sac ◽  
Cell Tumor ◽  
High Dose ◽  
Metastatic Site ◽  
Yolk Sac Tumors

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.

NAD(P)H: Quinone oxidoreductase (NQO1) and catalase expression in nonseminomatous germ cell tumors.

2020 ◽  
Vol 38 (6_suppl) ◽  
pp. 413-413
Author(s):  
Neda Hashemi-Sadraei ◽  
Nabil Adra ◽  
Kenneth A. Kessler ◽  
Muhammad T. Idrees ◽  
Lawrence Einhorn ◽  
...  
Keyword(s):  
Germ Cell ◽  
Germ Cell Tumor ◽  
Tumor Resection ◽  
Germ Cell Tumors ◽  
Predictive Marker ◽  
Cell Tumor ◽  
Cancer Center ◽  
Quinone Oxidoreductase ◽  
Lung Cancers ◽  
Multiple Tumor

413 Background: NAD(P)H:quinone oxidoreductase 1 (NQO1) is an enzyme which detoxifies quinones and reduces oxidative stress. NQO1 is expressed in multiple tumor types at levels up to 200-fold above normal tissue, including in breast, pancreatic, and non-small cell lung cancers. NQO1 bioactivatable drugs have the potential to deliver tumor-selective DNA damage and cell death by exploiting the elevation of NQO1. Alterations in catalase expression can cause marked cytoprotection. The ratio of NQO1:catalase activities is presumed to be a predictive marker for therapeutic activity of NQO1 bioactivatable drugs. There is no data available on NQO1 and catalase expression in germ cell tumors. Methods: Patients with germ cell tumor who underwent orchiectomy/tumor resection between January 2016 and December 2018 were identified from the Indiana University Melvin and Bren Simon Cancer Center database. Patients with non-seminomatous germ cell tumor of testis or primary mediastinal non-seminomatous germ cell tumor were selected. Immunohistochemistry staining for NQO1 and catalase was performed on tumor tissue. Results: NQO1 and catalase expression were assessed in 16 patients. Fifteen of 16 tumors stained positive for NQO1, 13 of which were moderately to strongly positive. Conversely, the majority of tumors were catalase deficient or only mildly positive for catalase. The details of the Immunostaining is summarized in the table below. Conclusions: Non-seminomatous germ cell tumors appear to have overexpression of NQO1 and deficiency of catalase. NQO1 bioactivatable agents could be beneficial in treating patients with refractory non-seminomatous germ cell tumors.[Table: see text]

YOLK SAC TUMOR OVARIUM

2020 ◽  
Vol 4 (1) ◽  
pp. 43-51
Author(s):  
Galuh Ayu Treswari ◽  
Bambang Soeprijanto ◽  
Indrastuti Normahayu ◽  
Lenny Violetta
Keyword(s):  
Germ Cell ◽  
Ct Scan ◽  
Germ Cell Tumor ◽  
Cystic Mass ◽  
Yolk Sac ◽  
Cell Tumor ◽  
Yolk Sac Tumor ◽  
Pelvic Cavity ◽  
Yolk Sac Tumors ◽  
Malignant Germ Cell Tumor

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.

scholarly journals Yolk Sac Tumor of the Temporal Lobe: Case Report

2015 ◽  
Vol 37 (03) ◽  
pp. 247-251
Author(s):  
Ana Machado ◽  
Ricardo Taipa ◽  
Manuel Pires ◽  
Carla Silva ◽  
Mário Gomes
Keyword(s):  
Germ Cell ◽  
Temporal Lobe ◽  
Germ Cell Tumor ◽  
Germ Cell Tumors ◽  
High Grade Glioma ◽  
Yolk Sac ◽  
Cell Tumor ◽  
Temporal Lobectomy ◽  
Yolk Sac Tumor ◽  
Primary Tumors

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.

scholarly journals Unusual Presentations of Gynecologic Tumors: Extragonadal Yolk Sac Tumor of the Vulva

2016 ◽  
Vol 141 (2) ◽  
pp. 293-297 ◽  
Author(s):  
Elizabeth D. Euscher
Keyword(s):  
Germ Cell ◽  
Case Reports ◽  
Germ Cell Tumors ◽  
Yolk Sac ◽  
Cell Tumor ◽  
Yolk Sac Tumor ◽  
Small Series ◽  
Gynecologic Tumors ◽  
Malignant Germ Cell Tumor ◽  
Extragonadal Germ Cell Tumors

Extragonadal germ cell tumors are uncommon, and although they morphologically resemble their gonadal counterparts, unexpected gonadal presentation increases the potential for erroneous diagnoses. Yolk sac tumor is a malignant germ cell tumor characterized by an extraembryonic yolk sac line of differentiation, and relative to other germ cell tumors, is characterized by varied and diverse histologic patterns. When occurring outside of typical age parameters or in extragonadal locations, the histologic variability of yolk sac tumor and its tendency to mimic somatic tumors pose diagnostic challenges. Because extragonadal yolk sac tumor of the vulva is very rare, with only isolated case reports and small series in the literature, it is often not considered in the differential diagnosis. As both prognosis and management of yolk sac tumor differ significantly from those of somatic tumors, accurate diagnosis is essential. This review discusses histologic features of extragonadal yolk sac tumor, addresses somatic tumors arising in the vulva for which yolk sac tumor may be confused, and provides guidance with respect to the use of immunohistochemistry in the diagnosis of yolk sac tumor.

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