scholarly journals Extragonadal mediastinal germ cell tumor

2021 ◽  
pp. 144-151
Author(s):  
G. V. Tishchenko ◽  
A. I. Shalyga ◽  
I. A. Tsishchanka
Keyword(s):  
Germ Cell ◽  
Histological Examination ◽  
Germ Cell Tumor ◽  
Germ Cells ◽  
Clinical Case ◽  
Germ Cell Tumors ◽  
Cell Tumor ◽  
Malignant Neoplasms ◽  
Mediastinal Germ Cell Tumor ◽  
Extragonadal Germ Cell Tumors

Extragonadal germ cell tumors (GCTs) are neoplasms that develop from germ cells and have a similar histopathological structure with the gonadal forms of GCTs, but are located outside the gonads. GCTs are the most common malignant neoplasms in men aged 15–35. From 1 to 5 % of malignant GCTs are of the extragonadal origin.The work describes a clinical case of extragonadal mediastinal germ cell tumor, which was diagnosed at the stage of the histological examination of the surgical material.

scholarly journals Klinefelter syndrome and germ cell tumors: review of the literature

2020 ◽  
Vol 2020 (1) ◽  
Author(s):  
Kimberley Bonouvrie ◽  
Jutte van der Werff ten Bosch ◽  
Machiel van den Akker
Keyword(s):  
Germ Cell ◽  
Germ Cell Tumor ◽  
Klinefelter Syndrome ◽  
Case Reports ◽  
Age Groups ◽  
Germ Cell Tumors ◽  
Young Patients ◽  
Cell Tumor ◽  
Healthy Population ◽  
Extragonadal Germ Cell Tumors

Abstract Objective The most common presentation of Klinefelter syndrome (KS) is infertility and features of hypogonadism. Currently no consensus exists on the risk of malignancy in this syndrome. Several case reports show an incidence of extragonadal germ cells tumors (eGCT) of 1.5 per 1000 KS patients (OR 50 against healthy population). Malignant germ cell tumors are rare in children. They account for 3% of all children cancers. Young patients with a germ cell tumor are not routinely tested for Klinefelter syndrome. This can therefore result in underdiagnosing. Literature data suggest a correlation between eGCT and KS. To the best of our knowledge there is no precise description of the primary locations of germ cell tumors in KS patients. The purpose of this study is to evaluate age groups and primary locations of extragonadal germ cell tumors in Klinefelter patients. With this data we investigate whether it is necessary to perform a cytogenetic analysis for KS in every eGCT patient. Study design This study is based on case report publications in PubMed/Medline published until march 2020 that described “Klinefelter Syndrome (MeSH) AND/OR extragonadal germ cell tumors”. Publications were included when patients age, location and histology of the germ cell tumor was known. Two double blinded reviewers selected the studies.Results: 141 KS patients with eGCTs were identified. Mean age at presentation was 17.3 years (StDev + − 10.2). In contrast to the extragonadal germ cell tumors in adults, most eGCT in children were mediastinal or in the central nervous system (respectively 90/141; 64% and 23/141; 16% of all tumors). Distribution of histologic subtypes showed that the largest fraction represented a teratoma, mixed-type-non-seminomateus GCT and germinoma, respectively 34/141; 24%, 26/141; 18% and 20/141; 14% of all tumors. Conclusion These data suggest a correlation between primary extragonadal germ cell tumors and Klinefelter syndrome. There appears to be an indication for screening on KS in young patients with an eGCT in the mediastinum. A low threshold for radiologic examinations should be considered to discover eGCT. We emphasize the need for genetic analysis in all cases of a male with a mediastinal germ cell tumor for the underdiagnosed Klinefelter syndrome.

Klinefelter's syndrome associated with mediastinal germ cell neoplasms.

1987 ◽  
Vol 5 (8) ◽  
pp. 1290-1294 ◽  
Author(s):  
C R Nichols ◽  
N A Heerema ◽  
C Palmer ◽  
P J Loehrer ◽  
S D Williams ◽  
...  
Keyword(s):  
Germ Cell ◽  
Germ Cell Tumor ◽  
Case Reports ◽  
Germ Cell Tumors ◽  
Cell Tumor ◽  
Klinefelter’S Syndrome ◽  
Klinefelter's Syndrome ◽  
Cell Neoplasm ◽  
Mediastinal Germ Cell Tumor ◽  
Mediastinal Germ Cell Tumors

Several case reports have suggested an association of primary mediastinal germ cell tumor (PMGCT) and Klinefelter's syndrome (KS). In an effort to confirm this association, 22 patients with mediastinal germ cell tumors had chromosome studies performed in a prospective fashion. Five patients (22%) had karyotypic or pathologic evidence of KS. All of the patients with KS had germ cell tumors of the nonseminomatous subtype and were relatively young (median age, 15 years). The literature confirms the findings of a young median age (18 years), nonseminomatous subtype, and mediastinal location of the germ cell neoplasm. We conclude that patients with KS are predisposed to the development of mediastinal nonseminomatous germ cell cancers.

Mediastinal Germ Cell Tumor and Acute Megakaryoblastic Leukemia– a Systematic Review of Cases Reported in the Literature

Blood ◽  
2014 ◽  
Vol 124 (21) ◽  
pp. 3694-3694 ◽  
Author(s):  
Sonia John ◽  
Mohammed Abdul Muqeet Adnan ◽  
Mohammad O. Khalil ◽  
George Selby ◽  
Adam S. Asch ◽  
...  
Keyword(s):  
Systematic Review ◽  
Germ Cell ◽  
Germ Cell Tumor ◽  
Germ Cell Tumors ◽  
Cell Tumor ◽  
Cell Transplant ◽  
Acute Megakaryoblastic Leukemia ◽  
Megakaryoblastic Leukemia ◽  
Mediastinal Germ Cell Tumor ◽  
Mediastinal Germ Cell Tumors

Abstract Background and Aim: The association of mediastinal germ cell tumors and acute megakaryoblastic leukemia has been known for many years. Characterization of this association is not well reported systematically in the literature. We hereby present this systematic review to describe the salient features of this association, treatment options and overall prognosis. Material and Methods: A systematic review of PUBMED, Medline and EMBASE databases via OVID engine was conducted to search for primary articles and case reports under keywords “germ cell tumors” and “acute myeloid leukemia”. Search was extensive from 1946 to 2014; all the cases that were written in English language in pediatric and adult patients were included. The search yielded 679 results and was individually examined by two authors. All the studies that described acute lymphoblastic leukemia or other malignancies aside from AML were excluded. A total of 25 studies reported mediastinal germ cell tumors (MGCT) and acute megakaryoblastic leukemia (M7). We also included in this analysis, one additional case with mediastinal germ cell tumor with subsequent development of acute megakaryoblastic leukemia that was recently diagnosed and treated at our institution. Results: After our extensive review of the literature a total of 25 previous patients with MGCT + M7 were found from 1946-2014. With the inclusion of our case we report a total of 26 cases from 1946-2014.Twenty of the included patients were males and in 6 cases there was no mention of the gender. Median age at diagnosis of MGCT was 23 years (range - 15 to 36 y). In 92 % of cases the germ cell tumor was of non-seminomatous origin with 27% cases reporting teratoma and 40.7 % of cases reporting embryonal carcinoma. All the reported cases were stage III and had elevated tumor markers with significant elevation in serum AFP compared to beta-HCG. MGCT occurred prior to appearance of leukemia in 46% of cases and concomitantly in 30% of cases. The rest of the cases did not describe the sequence of disease occurrence. M7 leukemia was never reported prior to the appearance of GCT. All the patients were treated with platinum based chemotherapy which was primarily directed towards management of the germ cell tumors. The time from diagnosis of MGCT to development of M7 leukemia ranged from 9 weeks to 39 months with the median time being 4 months. Median time to death from the initial diagnosis of NSGCT was 6 months (range - 3 to 39 months). Only one reported case with MGCT+M7 combination was successfully treated with an allogenic stem cell transplant for the M7 leukemia with surgical resection of the mediastinal mass. Among the cases that reported cytogenetic abnormalities, trisomy 8 and complex cytogenetics were reported in 18.6 % cases each and hyperdiploidy was reported in 22 % cases. Surprisingly, Klinefelters syndrome (47, XXY) that is historically associated in 20 % of cases of mediastinal germ cell tumors was only reported in one case in this review. The i(12p) abnormality was reported only in 14.8 % of cases. Conclusion: Patients with history of mediastinal germ cell tumors are at higher risk of developing acute leukemia especially the megakaryoblastic subtype. This warrants long term follow up of such patients with regular monitoring of blood counts and a high degree of suspicion for hematological malignancies. In selected cases, allogeneic stem cell transplant may be considered in those who achieve leukemia remission after optimal surgical resection. In spite of the advances in chemotherapeutic options, the overall outcome in patients with mediastinal germ cell tumors who have acute megakaryoblastic leukemia remains poor. Disclosures No relevant conflicts of interest to declare.

Possible common origin of α-fetoprotein- and human chorionic gonadotropin—secreting cells in intracranial germ cell tumor

1998 ◽  
Vol 88 (3) ◽  
pp. 576-580 ◽  
Author(s):  
Haruhiko Kishima ◽  
Keiji Shimizu ◽  
Yasuyoshi Miyao ◽  
Eiichiro Mabuchi ◽  
Toru Hayakawa
Keyword(s):  
Germ Cell ◽  
Germ Cell Tumor ◽  
Chorionic Gonadotropin ◽  
Germ Cells ◽  
Human Chorionic Gonadotropin ◽  
Cultured Cells ◽  
Germ Cell Tumors ◽  
Cell Tumor ◽  
Intracranial Germ Cell Tumor ◽  
Surgical Specimen

✓ A primary intracranial germ cell tumor in a 16-year-old boy secreted both a-fetoprotein (AFP) and human chorionic gonadotropin (HCG). The tumor, located in the right thalamus, contained germinomatous, trophoblastic, and endodermal sinus components. To identify AFP- and HCG-secreting cells, germ cells from the surgical specimen were cultured in vitro. These cultured cells secreted AFP and HCG for 10 weeks, and immunohistochemical studies showed that some of the cells secreted both AFP and HCG. These findings suggest that multipotential germ cells migrate to the encephalic region and may become germ cell tumors containing various types of tissue.

Growing teratoma syndrome in primary mediastinal germ cell tumor: our experience

2019 ◽  
Vol 27 (2) ◽  
pp. 98-104 ◽  
Author(s):  
Ashwani Kumar Sachdeva ◽  
Prasanth Penumadu ◽  
Pavneet Kohli ◽  
Biswajit Dubashi ◽  
Hemachandren Munuswamy
Keyword(s):  
Germ Cell ◽  
Germ Cell Tumor ◽  
Germ Cell Tumors ◽  
Surgical Excision ◽  
Cell Tumor ◽  
Growing Teratoma Syndrome ◽  
Mediastinal Germ Cell Tumor ◽  
Mediastinal Involvement ◽  
Nonseminomatous Germ Cell Tumors

Background Growing teratoma syndrome is a rare phenomenon seen in nonseminomatous germ cell tumors after chemotherapy, where the tumor grows paradoxically despite normalization of tumor markers. It has been found in various locations, most commonly, the retroperitoneum in association with metastatic disease. The occurrence of growing teratoma syndrome in a mediastinal primary is very rare and there are only a few reports in the literature. Methods In a retrospective review, out of 12 patients with mediastinal involvement by a germ cell tumor, 5 had a primary from the mediastinum. We present a series of 3 cases of primary germ cell tumor of the mediastinum, which after chemotherapy, fulfilled the criteria for growing teratoma syndrome and were managed with surgical excision. Conclusion Development of growing teratoma syndrome in a primary mediastinal germ cell tumor is extremely rare. Its awareness and early detection can lead to successful surgical excision and long-term cure.

scholarly journals Mixed Mediastinal Germ Cell Tumor: A Case Report

2020 ◽  
pp. 1-4
Keyword(s):  
Germ Cell ◽  
Germ Cell Tumor ◽  
White Male ◽  
Germ Cell Tumors ◽  
Cell Tumor ◽  
Axial Tomography ◽  
X Ray ◽  
Mixed Germ Cell Tumor ◽  
Computed Axial Tomography ◽  
Mediastinal Germ Cell Tumor

Germ cell tumors constitute a heterogeneous group of neoplasms derived from primitive germ cells, arising as a consequence of an embryogenic error. A clinical case of a 16-year-old white male patient, with no previous pathological history or toxic habits, who was admitted to the internal medicine room for presenting a clinical picture of asthenia and anorexia was added, to whom light fever was added in evening schedule. Several examinations were performed, including: Immunohistochemistry, biopsy, Computed Axial Tomography, X-ray of the chest and Mixed germ cell tumor of Mediastinum was diagnosed. Chemotherapy was started. The prognosis of the disease depends on the precociousness with which the diagnosis and treatment are made.

scholarly journals Germ Cell Tumors in the Intersex Gonad: Old Paths, New Directions, Moving Frontiers

2006 ◽  
Vol 27 (5) ◽  
pp. 468-484 ◽  
Author(s):  
Martine Cools ◽  
Stenvert L. S. Drop ◽  
Katja P. Wolffenbuttel ◽  
J. Wolter Oosterhuis ◽  
Leendert H. J. Looijenga
Keyword(s):  
High Risk ◽  
Germ Cell ◽  
Germ Cell Tumor ◽  
Germ Cells ◽  
Germ Cell Tumors ◽  
Tumor Development ◽  
Disorders Of Sex Development ◽  
Cell Tumor ◽  
Maturation Delay ◽  
Made In

The risk for the development of germ cell tumors is an important factor to deal with in the management of patients with disorders of sex development (DSD). However, this risk is often hard to predict. Recently, major progress has been made in identifying gene-products related to germ cell tumor development (testis-specific protein-Y encoded and octamer binding transcription factor 3/4) and in recognizing early changes of germ cells (maturation delay, preneoplastic lesions, and in situ neoplasia). The newly recognized “undifferentiated gonadal tissue” has been identified as a gonadal differentiation pattern bearing a high risk for the development of gonadoblastoma. It is expected that the combination of these findings will allow for estimation of the risk for tumor development in the individual patient (high risk/intermediate risk/low risk). This article reviews the recent literature regarding the prevalence of germ cell tumors in patients with DSD. Some major limitations regarding this topic, including a confusing terminology referring to the different forms of intersex disorders and unclear criteria for the diagnosis of malignant germ cells at an early age (maturation delay vs. early steps in malignant transformation) are discussed. Thereafter, an overview of the recent advances that have been made in our knowledge of germ cell tumor development and the correct diagnosis of early neoplastic lesions in this patient population is provided. A new classification system for patients with DSD is proposed as a tool to refine our insight in the prevalence of germ cell tumors in specific diagnostic groups.

scholarly journals A Young Female with Mass Originating from Middle Lobe of Lung-Choriocarcinoma: Extragonadal Germ Cell Tumor

2021 ◽  
Vol 2 (9) ◽  
pp. 768-770
Author(s):  
Muhammad Umer Salim ◽  
Mohammad Rabie Alrohaibani ◽  
Mohammed Hassan Shabrawishi ◽  
Sobhy Atalla Ibrahim ◽  
Abed Sabry Elfar ◽  
...  
Keyword(s):  
Pleural Effusion ◽  
Germ Cell ◽  
Germ Cell Tumor ◽  
Germ Cell Tumors ◽  
Young Female ◽  
Middle Lobe ◽  
Cell Tumor ◽  
Reverse Migration ◽  
Rare Tumors ◽  
Extragonadal Germ Cell Tumors

Extragonadal germ cell tumors are the rare tumors originate commonly from the mediastinum and the reason is still unknown however; there are theories, which suggest that the gonadal cells in the mediastinum represent reverse migration from the gonads. A young female presented with mass originating from the middle lobe of lung and right sided pleural effusion that was drained, and biopsy was taken from the mass; diagnosed as choriocarcinoma. She received (BEP) Bleomycin, Etoposide and Cisplatin chemotherapy; responded well and her symptoms improved.

scholarly journals GCT-06. DIAGNOSIS OF A RARE CASE OF RECURRENT GERM CELL TUMOR BY CSF PLACENTAL ALKALINE PHOSPHATASE PRESENTING WITH DIFFUSE INTRAAXIAL ABNORMALITY IN THE LOWER BRAINSTEM

2020 ◽  
Vol 22 (Supplement_3) ◽  
pp. iii329-iii329
Author(s):  
Hiroki Yamada ◽  
Tomohiro Abiko ◽  
Hirokazu Fujiwara ◽  
Kazunari Yoshida ◽  
Hikaru Sasaki
Keyword(s):  
Alkaline Phosphatase ◽  
Germ Cell ◽  
Germ Cell Tumor ◽  
Rare Case ◽  
Cervical Spinal Cord ◽  
Germ Cell Tumors ◽  
Cell Tumor ◽  
Placental Alkaline Phosphatase ◽  
Steroid Pulse ◽  
Platinum Based Chemotherapy

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.

scholarly journals Metastatic mediastinal germ‐cell tumor and concurrent COVID ‐19: when chemotherapy is not deferrable

2020 ◽  
Author(s):  
Paolo Pedrazzoli ◽  
David Rondonotti ◽  
Carlo Cattrini ◽  
Simona Secondino ◽  
Paolo Ravanini ◽  
...  
Keyword(s):  
Germ Cell ◽  
Germ Cell Tumor ◽  
Cell Tumor ◽  
Mediastinal Germ Cell Tumor
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