Primary Germ Cell Tumors Research Articles

A primary third ventricle mixed germ cell tumor with leptomeningeal dissemination of immature teratoma component

A 17-year-old male patient presented to the clinic with a headache, nausea, and vomiting. Magnetic resonance imaging demonstrated a fat-containing and -enhancing heterogeneous tumor in the third ventricle, and fat droplets within the ventricles and the subarachnoid space. Obstructive hydrocephalus was also present. Emergency subtotal removal of the mass was performed via interhemispheric transcallosal approach. The histopathological diagnosis was a mixed germ cell tumor that was composed of embryonal carcinoma, yolk-sac tumor, germinoma, and immature teratoma containing a large amount of mature elements. The patient was referred for postoperative chemoradiotherapy. A mixed germ cell tumor is a rare type of nongerminomatous germ cell tumor that is made up of at least two different types of germ cell tumors. These may include germinoma, choriocarcinoma, embryonal carcinoma, yolk sac tumor, mature teratoma, immature teratoma, or teratoma with malignant degeneration. As far as we know, this is the first reported case of a primary third ventricle mixed germ cell tumor with leptomeningeal dissemination of the immature teratoma component that contains grossly visible mature elements at admission.

DIAGNOSIS OF GERM CELL TUMORS OF EXTRAGONADAL LOCALIZATION IN TERMS OF ALGORITHMS FOR DETECTING METASTASIS OF CARCINOMAS OF UNKNOWN PRIMARY LOCALIZATION

The analysis of cases of extragonadal teratomas makes it clear that tumours derived from germ cells and mainly characteristic of adult gonads can also occur in other areas, such as the anterior mediastinum (thymus) and midline brain (germinomas of the epiphysis and the area above the Turkish saddle), that requires differential diagnosis with metastases of carcinomas of other origin. Accurate diagnosis of estrogenic germ-cell tumours by only routine staining with haematoxylin-eosin requires high expertise and experience due to their non-specific clinical symptoms and variability of morphological characteristics. Tumour morphologists consider that immunohistochemical investigation plays an important role in accurate histological diagnosis of these tumours. The goal of this study is to explore the expression features of immunohistochemical markers and morphometric parameters of the area, perimeter and "roundness" of the nuclei in various types of extragonadal germ-cell tumours compared to similar primary ovarian / testicular tumours in order to improve diagnostic algorithms. A study was conducted on biopsy or postoperative samples taken from 8 patients (group 1) with extragonadal germ-cell tumours and from 16 patients with primary germ cell tumours of testicles / ovaries (group 2). The diagnoses were confirmed by immunohistochemical investigation on the basis of the pathologic department of the “Pharmacy of Medical Academy” Diagnostic Centre for 2015 to 2018. PLAP and CD117 had the highest percentage of expression in seminomas and gerninomas of both studied groups; morphological parameters were 3-fold higher in area and ̴2-fold higher around the perimeter then the values of normal lymphocytes (p<0.05). Expression of markers CD30, EMA and CK AE1/3 was diagnostically significant in samples of embryonic carcinoma, and morphological parameters ̴were ̴ 2.1 times higher in area and ̴ 1.7 along the perimeter that exceeded the values of normal lymphocytes (p<0.05). αFP-positive staining was indicative of yolk sac tumours, which morphological parameters were more than ̴ 1.7 times higher in the area and ̴ 1.5 times higher along the perimeter compared with the normal lymphocytes (p<0.05). Taking into account the variability of morphological characteristics and the possibility of extragonadal location of tumours derived from germ cells, immunohistochemical research supported by morphometry is an important tool in the differential diagnosis of carcinomas of unknown primary localization.

Primary Mediastinal Germ Cell Tumor Successfully Treated with Chemotherapy Followed by Surgery: a Case Report

Primary Mediastinal Germ Cell Tumor Successfully Treated with Chemotherapy Followed by Surgery: a Case Report

Serum Tumour Markers in Testicular Germ Cell Tumours: Frequencies of Elevated Levels and Extents of Marker Elevation Are Significantly Associated with Clinical Parameters and with Response to Treatment

Introduction Although serum tumor markers beta human chorionic gonadotropin (bHCG), alpha-fetoprotein (AFP), and lactate dehydrogenase (LDH) are well-established tools for the management of testicular germ cell tumours (GCTs), there are only few data from contemporary cohorts of primary GCT patients regarding these biomarkers. Our aim was to evaluate marker elevations in testicular GCTs and to document their associations with various clinical characteristics. Patients and Methods A total of 422 consecutive patients with GCTs were retrospectively analysed regarding serum levels of bHCG, AFP, and LDH during the course of treatment. Additionally, the following characteristics were recorded: histology, age, laterality, clinical stage (CS), pT-stage, and tumour size. Marker elevations were first tabulated in dichotomized way (elevated: yes/no) in various subgroups and second as continuous measured serum values. Descriptive statistical methods were employed to look for differences among subgroups and for associations of elevations with clinical parameters. Results In all GCT patients, the frequencies of elevated levels of bHCG, AFP, LDH, and bHCG or AFP were 37.9%, 25.6%, 32.9%, and 47.6%; in pure seminomas 28%, 2.8%, 29.1%, and 30.3%; and in nonseminoma 53.0%, 60.1%, 38.7%, and 73.8%. Significant associations were noted with pT-stages >pT1, clinical stages >CS1, tumour size, and younger age. Frequencies of marker elevations dropped significantly after treatment, but LDH levels remained elevated in 30.5%-34.1%. Relapsing patients (n=27) had elevated levels of bHCG, AFP, and LDH in 25.9%, 22.2%, and 29.6%, respectively, thirteen of whom with a changed marker pattern. Conclusions The classical GCT-biomarkers correlate with treatment success. Clinical utility is limited due to proportions of < 50% of patients with elevated levels and the low specificity of LDH. The elevation rates are significantly associated with histology, clinical and pT-stages, tumour size, and younger age. Individual marker patterns may change upon relapse. Clinically, ideal biomarkers are yet to be found.

Pure Primary Extragonadal Yolk Sac Tumor involving stomach in a Young Child: A Case Report

Germ Cell Tumors (GCTs) constitute approximately only 3% of all malignancies in children younger than 15 years of age. Primary extragonadal GCTs constitute only 1-5% of all GCTs and a retroperitoneal site is seen only in 4% of all extragonadal GCTs. Yolk sac tumor is the predominant variant in newborns and younger children. GCTs of stomach are very very rare.We report for the first time, the presentation of a primary yolk sac tumor arising from the stomach of a small child.&#x0D;

Case report: primary mediastinal germ cell tumour initially presenting as level IV cervical lymphadenopathy

AbstractThis case report describes an unusual case of a primary mediastinal germ cell tumour presenting initially as lymphadenopathy in the neck. This case highlights the rare occurrence of germ cell tumours in an area common to the routine extra oral dental examination of lymph nodes.

Mediastinal Germ Cell Tumors – A Clinicopathologic Study

Background: Primary mediastinal extragonadal germ cell tumors (GCT) are relatively rare lesions and account for only 1 to 2 percent of all germ cell tumors. They are clinically distinct from testicular germ cell tumors despite their similar histologic features. Objective of this study is to classify and evaluate the histomorphology of mediastinal germ cell tumors and correlate the clinical findings with radiological and other serologic parameters. Method: A 5 year study was done in Dept. of Pathology, of a private Medical College on mediastinal lesions. Total number of cases studied was 66, with GCTs making up 7 cases. Histopathology sections taken were stained with routine Hematoxylin and Eosin stains. Results: The age range of patients with mediastinal GCTs were 6 months to 43 years with a mean of 23 years. Only one case was seen above 40 years. Both malignant GCTs were below 20 years. There was a male predominance with male:female ratio of 2.5:1. Conclusion: Mediastinal GCTs are rare & interesting neoplasms, usually located in anterior compartment of mediastinum. A histomorphological analysis aided by radiology and serology permits an exact diagnosis in many cases.

Clinical features and outcomes of secondary somatic malignancy (SSM) associated with primary mediastinal nonseminomatous germ cell tumors (PM-NSGCT).

531 Background: The incidence of secondary somatic malignancy (SSM) arising from teratoma is increased in 2 settings; late relapse and primary mediastinal nonseminomatous germ cell tumors (PM-NSGCT). Here, we report the clinical features and outcomes of patients with SSM in the setting of PM-NSGCT. Methods: Between 1985 and 2018, 29 patients with PM-NSGCT and SSM who had sufficient clinical follow-up to evaluate outcome were identified. Clinical and pathologic parameters were reviewed. The Kaplan-Meier method was used to estimate overall survival (OS) from time of SSM diagnosis and the log rank test to compare estimates. Results: Median age was 28 years (range 18-59) and all patients were male. Most presented with local symptoms (n=24, 83%), elevated tumor markers (n=26, 90%), and disease isolated to the mediastinum (n=25, 86%). A total of 39 SSM histologies were present in the 29 cases, with 8 (28%) having 2 (n=6) or 3 (n=2) SSM histologies; 25 (86%) also had viable non-teratomatous GCT in the mediastinal mass. Sarcoma was found in all 29 cases including rhabdomyosarcoma (n=15), angiosarcoma (n=6), sarcoma NOS (n=5), spindle cell (n=4), PNET (n=3), and other (n=3). Non-sarcoma histologies (n=1 each) included AML, SCC, and neuroblastoma. Most patients received GCT-directed chemotherapy followed by an attempt at surgical resection (90%). With a median follow-up of 2 years for survivors, median OS was 1.8 years (95% CI 0-3.9 years), with 18 patients succumbing to disease. Complete surgical resection was achieved in 23 men (79%) and was associated with superior OS (3.1 vs. 0.3 years, p=0.005). At relapse or progression, 11 received SSM histology-directed and 7 GCT-directed chemotherapy with no difference in OS (1.3 vs. 1.2 years, p=0.993). 7 patients developed SSM in the form of leukemia, a finding associated with significantly inferior OS (0.3 vs. 3.0 years, p=0.009). Conclusions: Sarcoma is the predominant SSM histology associated with PM-NSGCT and portends a poor prognosis even with initially localized disease. Complete resection following chemotherapy is critical to achieving long-term survival whereas SSM in the form of leukemia portends especially poor outcome.

Autologous Hematopoietic Stem Cell Transplantation for Male Germ Cell Tumors: Improved Outcomes Over 3 Decades.

The curative potential of autologous hematopoietic cell transplantation (autoHCT) for male germ cell tumors (GCTs) is well established. The optimal timing and number (single transplant [ST] versus tandem transplants [TT] versus triple transplants) of autoHCT are controversial, with wide practice variations. We examined survival trends among 2395 recipients of autoHCT for male GCTs between 1990 and 2015 reported to the Center for International Blood and Marrow Transplant Research. Trends and outcomes were analyzed by year of transplantation for intervals 1990 to 1994 (N = 288), 1995 to 1999 (N = 351), 2000 to 2004 (N = 376), 2005 to 2009 (N = 509), and 2010 to 2015 (N = 871). Multivariate analysis was restricted to the subset from 2000 to 2015 with research-level data (n = 267). The median duration of follow-up was 51 months. The median age at autoHCT was 31 years; 633 patients (26%) had primary extragonadal GCT, and 1167 (49%) underwent TT. The 3-year progression-free (PFS) and overall survival (OS) improved from 24% (95% confidence interval [CI], 18% to 31%) and 35% (95% CI, 29% to 40%), respectively, in 1990 to 1994 to 47% (95% CI, 43% to 50%) and 54% (95% CI, 50% to 57%), respectively, in 2010 to 2015 (P <.0001). TT recipients were more likely than ST recipients to undergo autoHCT as first salvage treatment. The proportion of TTs increased from 38% of all autoHCTs in 2000 to 2004 to 77% in 2010 to 2015. Nonseminoma histology, residual disease at autoHCT, >1 line of pretransplantation chemotherapy, and ST versus TT were associated with inferior PFS and OS. Post-transplantation survival has improved significantly over time for relapsed/refractory male GCT and is associated with the increased use of TTs (compared with STs) and performance of autoHCT earlier in the disease course.

Critical review of the management of primary central nervous nongerminomatous germ cell tumors.

Multimodal strategies have significantly improved the outcomes for patients with central nervous system nongerminomatous germ cell tumors. Two large cooperative group studies have recently reported much improved outcomes compared with historical series. However, a substantial proportion of patients still attain inadequate responses to initial chemotherapy prior to irradiation, with adverse impact upon survival; optimal induction chemotherapy regimens and radiotherapy strategies are as yet unidentified. Outcomes for patients with relapsed disease remain poor. There is an obvious need to incorporate molecular studies within prospective clinical trials that will likely lead to the incorporation of targeted, more effective future treatment strategies.

Pre-orchiectomy tumor marker levels should not be used for International Germ Cell Consensus Classification (IGCCCG) risk group assignment.

To investigate whether the use of pre-orchiectomy instead of pre-chemotherapy tumor marker (TM) levels has an impact on the International Germ Cell Consensus Classification (IGCCCG) risk group assignment in patients with metastatic germ cell tumors (GCT). Demographic and clinical information of all patients treated for primary metastatic testicular non-seminomatous GCT in our tertiary care academic center were extracted from medical charts. IGCCCG risk group assignment was correctly performed with pre-chemotherapy marker levels and additionally with pre-orchiectomy marker levels. Agreement between pre-chemotherapy and pre-orchiectomy risk group assignments was assessed using Cohen's kappa. Our cohort consisted of 83 patients. The use of pre-orchiectomy TMs resulted in an IGCCCG risk group upstaging in 12 patients (16%, 8 patients from good to intermediate risk and 4 patients from intermediate to poor risk) and a downstaging in 1 patient (1.2%, from intermediate- to good-risk). The agreement between pre-orchiectomy and pre-chemotherapy IGCCCG risk groups resulted in a Cohen's kappa of 0.888 (p < 0.001). Using pre-orchiectomy TMs can result in incorrect IGCCCG risk group assignment, which in turn can impact on the clinical management and follow-up of patients with metastatic GCT. Thus, adherence to the IGCCCG standard using pre-chemotherapy TMs levels is recommended.

Clinical and radiographic characterization of primary seminomas and nonseminomatous germ cell tumors.

Primary malignant mediastinal germ cell tumors (PMMGCTs) including seminomas and nonseminomatous germ cell tumors (NSGCTs) are rare, and sometimes the diagnosis is very difficult. The purpose of this study is to compare the clinical characteristics, biomarkers, and imaging findings of seminomas and NSGCTs and to determine whether these features could help distinguish these two types of PMMGCT. A retrospective study of 24 male patients with histopathologically proven PMMGCT was performed. We collected the information of computed tomography (CT) (the scan area ranged from the apex of lung to the costophrenic angles) and magnetic resonance imaging blood test and histology characteristics of these patients. Twelve of 24 cases were confirmed to be seminomas, whereas the other 12 cases were NSGCTs. Alfa-fetoprotein (AFP) was found to be elevated in all patients with NSGCT, whereas none of the patients with seminomas had elevated AFP level. Beta-human chorionic gonadotropin (β-HCG) level was elevated in all the patients with seminomas (seven/seven), whereas in NSGCT only two of seven patients had elevated β-HCG. Lactate dehydrogenase level was increased in five of the nine patients with seminomas, as well as in the eight patients with NSGCT. CT imaging revealed that 12 masses from the seminoma group were homogeneous, soft tissue opacity and showed minimal contrast enhancement. On the contrary, all 12 NSGCT cases showed cystic and solid masses; on contrast-enhanced CT, heterogeneous enhancement was found on the capsule of the tumor, septum, and solid masses. Seminomas and NSGCT showed different profiles of tumor biomarkers and radiographic features. Evidence from serum test, histopathological analysis, and imaging should be combined to ensure the accurate diagnosis of these two types of PMMGCT.

Rare Primary Mediastinal Seminoma in a 60-Year-Old

Mediastinal germ cell tumours are a rare susbset of primary mediastinal germ cell tumours. This study presented a case of an incidentally diagnosed primary mediastinal seminoma managed with complete surgical excision and subsequent oncological management

Surgical Role in Management of Intracranial Germ Cell Tumors in Pediatric Age Group

Introduction: Primary intracranial germ cell tumors (ICGCTs) are rare, histologically diverse, and diagnostically challenging tumors that are usually localized in the pineal and suprasellar regions of the brain. Advanced neurosurgical techniques such as neuroendoscopy and frameless stereotactic biopsy have made diagnosis of newly discovered cases of ICGCTs easier and safer. Material and methods: Seventeen patients with intracranial germ cell tumors operated upon between 2008 to 2012 at the Children's Cancer Hospital Egypt, were retrospectively reviewed and analyzed regarding the surgical decision, clinical outcome and surgical complications. Results: There were 9 cases of germinoma (53%), and 8 cases of non-germinomatous germ cell tumors (47%). Nine cases were in the pineal region, six in the suprasellar, and two in the thalamic region. Ten cases were operated upon initially by open surgery and frozen section with subtotal resection and seven cases were biopsied either endoscopically (3 cases) or by frameless guided stereotaxic (4 cases). Accurate pathology was achieved in all biopsied cases without major complications. In the germinoma group, the 4-year overall survival and progression free survival rate were 75% for both at a median follow up period of 26 (range 1 -50) months. For the non-germinomatous germ cell tumors group, the 4-year OS and PFS rates were 36.5% and 31.2% at a median follow up period of 11 (range 2-54) months, respectively. Conclusion: In cases of intracranial germ cell tumors with negative tumor markers the role of surgery is important in the establishment of proper histopathological diagnosis. However, in Non Germinomatous Germ Cell Tumors, further investigations should be done regarding the extent of resection owing to the poor long-term outcome.

Primary Spinal Germ Cell Tumors: An Unusual Case Analysis

Primary Spinal Germ Cell Tumors: An Unusual Case Analysis

Hematologic Malignancies Arising in Patients with Germ Cell Tumors: Secondary Somatic Differentiation of Hematopoietic Malignancies from Germ Cell Precursors

Genomic analyses have recently illuminated our understanding of therapy-associated myeloid neoplasms in patients receiving therapy for other cancers. One of the most intriguing relationships between solid tumors and myeloid neoplasms involves a unique clinical entity of patients with germ cell tumors (GCT) and myeloid neoplasms. One in 17 patients with primary mediastinal germ cell tumor (PMGCT) develops a hematologic malignancy (most commonly AML, MDS, or histiocytosis) and the median survival in such patients is poor at only 5 months. Intriguingly, the presence of isochromosome 12p [i(12p)], a clonal marker common to GCTs but absent in de novo myeloid neoplasms, has been demonstrated as shared across GCTs and myeloid neoplasms in such individuals. While these data suggest a clonal relationship between the two, the exact nature of the shared origin is unknown. There are two competing hypotheses to explain this: (1) an embryonic progenitor with the capacity to differentiate into germ cell and hematopoietic lineages harbors the initiating genetic alterations and drives development of both malignancies or (2) the leukemia is derived directly from GCTs with the capacity to differentiate into hematopoietic lineages. To trace the clonal evolution of these seemingly unrelated cancer types and identify recurrent genomic lesions in leukemias present in GCT patients, we applied whole exome sequencing (WES), targeted genomic analyses, and/or RNA-seq to leukemias, GCTs, and germline DNA in a series of patients with myeloid neoplasms and concurrent GCTs.We collected 12 patients with GCT and synchronously or metachronously occurring myeloid neoplasms (8 AML, 5 MDS/CMML, 2 histiocytic sarcoma (some had >1 hematologic malignancy)) with an average of 4 months between the two diagnoses. Consistent with prior reports, all were young men (median age 26) with PMGCT and nonseminomatous histology and a 3-month median survival from leukemia diagnosis (Fig. A). In each case, at least one copy number alteration or coding mutation was shared across the GCT and hematopoietic neoplasm, demonstrating the shared origin of both lesions. For example, half of the patients (6/12) carried i(12p) in both the GCT and hematopoietic neoplasm. In the i(12p) negative cases, somatic genetic alterations identified in the GCT were also found in the leukemia. The most common genomic alterations in leukemias beyond i(12p) included mutations activating RAS-PI3K-AKT signaling (including PTEN, RAS and PI3K isoform mutations) or inactivating TP53 (Fig. B). The only exception was a testicular-only GCT patient who developed clonally distinct acute promyelocytic leukemia; however, further analysis identified this as a chemotherapy-induced neoplasm with the PML-RARa breakpoint mapped to an etoposide sensitive area and this patient was not counted amongst the 12 cases.We next traced the evolutionary history of clonally related GCTs and leukemias based on cancer cell fraction of all coding mutations and copy number alterations using WES of DNA from each tumor type and finger nails. In each instance, we identified clonal evolution of the hematopoietic malignancies from antecedent precursors within the GCT. To illustrate this, a 19-year-old male developed successive diagnoses of histiocytic sarcoma, CMML, and AML within 18 months of GCT diagnosis. Lineage tracing by WES of each of these four individual cancers revealed that all four were clonally related, and the histiocytic sarcoma, CMML, and AML were all derived from the GCT with a common precursor giving rise to the three hematopoietic malignancies (Fig. C-D). Moreover, the histiocytic sarcoma evolved separately from CMML/AML in this patient, where the AML represented leukemic transformation from the CMML.These data conclusively demonstrate that myeloid neoplasms developing in patients with PMGCT represent secondary somatic differentiation of a hematologic progenitor from totipotent aberrant cells that are present in the GCT. Thus, GCT-associated leukemias have a unique ontogeny from de novo and/or secondary myeloid neoplasms, which originate from progenitors within the bone marrow. As such, GCT-associated leukemias have characteristic genomic alterations hallmarked by frequent i(12p) in combination with mutations activating RAS-PI3K-AKT signaling and inactivating TP53, and these patients do poorly even when treated with aggressive contemporary chemotherapy. DisclosuresRampal:Jazz: Consultancy, Honoraria; Celgene: Honoraria; Constellation: Research Funding; Incyte: Honoraria, Research Funding; Stemline: Research Funding. Tallman:ADC Therapeutics: Research Funding; Orsenix: Other: Advisory board; AROG: Research Funding; Cellerant: Research Funding; AbbVie: Research Funding; Daiichi-Sankyo: Other: Advisory board; BioSight: Other: Advisory board.

Cytokeratin 7, inhibin, and p63 in testicular germ cell tumor: superior markers of choriocarcinoma compared to β-human chorionic gonadotropin

Choriocarcinoma can be difficult to differentiate from other subtypes of testicular germ cell tumor and can occur unexpectedly in a distant, late metastasis. The aim of this investigation was to identify a marker superior to β-human chorionic gonadotropin (β-hCG) for choriocarcinoma. Sixty-two primary and metastatic testicular germ cell tumors (27 choriocarcinomas, 19 yolk sac tumors, 29 embryonal carcinomas, 28 seminomas, 22 teratomas, 3 epithelioid trophoblastic tumors [ETTs]) were analyzed for immunohistochemical expression of cytokeratin 7 (CK7), inhibin, p63, and β-hCG. All choriocarcinomas and ETTs were strongly positive for CK7, whereas seminomas were negative and 52% of embryonal carcinomas had weak reactivity. Eighty-four percent of yolk sac tumors and 59% of teratomas were CK7 positive. Eighty-nine percent of choriocarcinomas and 100% of ETTs were positive for inhibin, with reactivity highlighting syncytiotrophoblasts, whereas seminomas, embryonal carcinomas, yolk sac tumors, and teratomas were negative. Eighty-five percent of choriocarcinomas expressed p63, with staining mostly in mononucleated trophoblasts, whereas seminomas, embryonal carcinomas, and yolk sac tumors were negative. Teratomas expressed p63 in 32% of cases. β-hCG was reactive in 96% of choriocarcinomas, 33% of ETTs, 46% of seminomas, 54% of embryonal carcinomas, 47% of yolk sac tumors, and 32% of teratomas. β-hCG staining within other subtypes was more likely if choriocarcinoma was present elsewhere in the tumor (P = .0002). CK7 is a highly sensitive marker for choriocarcinoma and differentiates choriocarcinoma from seminoma and embryonal carcinoma. Inhibin and p63 are sensitive and specific for choriocarcinoma versus seminoma, embryonal carcinoma, and yolk sac tumor. To identify choriocarcinoma, CK7, inhibin, and p63 are superior to β-hCG.

Thoracoscopic resection of a cystic seminoma of the mediastinum

Mediastinal seminoma is an uncommon tumor that accounts for 25% of primary mediastinal germ cell tumors, which in turn comprise fewer than 5% of all germ cell tumors. Although CT normally shows a solid, lobulated tumor, mediastinal cystic seminoma has rarely been described. Here, we report a 24-year-old man who presented with a mediastinal cystic tumor that was resected after an 18-month delay via video-assisted thoracoscopic surgery while in the supine position; the procedure involved lifting the chest wall with a subcutaneous Kirschner wire. Pathological examination revealed a mediastinal cystic seminoma. No evidence of recurrence has been noted during 25 months of follow-up. Mediastinal cystic seminoma should be considered in the differential diagnosis of cystic lesions of the mediastinum. Moreover, video-assisted thoracoscopic resection may be an appropriate option for the diagnosis and treatment of such lesions.

Baseline characteristics and patterns of care in testicular cancer patients: first data from the Swiss Austrian German Testicular Cancer Cohort Study (SAG TCCS)

The majority of germ cell tumour (GCT) patients can be cured by orchiectomy followed by active surveillance or subsequent systemic and/or local treatments. There are various guidelines for a structured follow-up including radiographic and clinical examinations. The Swiss Austrian German Testicular Cancer Cohort Study (SAG TCCS) prospectively evaluates follow-up, indicator of relapse and late toxicities. This is a descriptive analysis; we present baseline characteristics and treatment strategies for the first 299 patients with primary GCT or relapsed GCT after completion of treatment. Of the patients included in this study, 192 (64.2%) had seminoma and 107 (35.8%) non-seminoma. Mean age was 41 years (standard deviation [SD] 11.7) for seminoma and 31 (SD 9.3) years for non-seminoma patients. Median tumour size was 3.5 cm (interquartile range 2.5¬‒5.0 and 2.3‒4.5 in seminoma and non-seminoma, respectively) in both histological groups. Among seminoma patients, 81 (42.2%) had primary tumours >4cm; 154 (80.2%) seminoma patients had stage I, 26 (13.5%) stage II and 12 (6.3%) stage III disease. Fifty-seven (53.3%) non-seminoma tumours were stage I, 29 (27.1%) stage II and 21 (19.6%) stage III. Marker-positive disease was present in 58 (30.2%) seminoma patients and 78 (72.9%) non-seminoma patients. Of 154 stage I seminoma patients, 89 (57.8%) chose active surveillance and 65 (42.2%) adjuvant chemotherapy. Twenty-six (45.6%) stage I non-seminoma patients had high-risk disease; 23 of these were treated with adjuvant chemotherapy and 3 chose active surveillance. Among the 30 (52.6%) low risk stage I patients, all opted for active surveillance. Twelve (46.2%) stage II seminoma patients had radiotherapy, 14 (53.8%) were treated with three to four cycles of chemotherapy. All stage III seminoma patients, and all stage II and III non-seminoma patients were treated with three to four cycles of chemotherapy. Treatment decisions were made at the respective centre. Eleven patients did not receive therapy that conformed with guidelines. It is important to enrol GCT patients in prospective studies in general, but also in follow-up studies to assess baseline characteristics, oncological outcome, and long-term toxicity and to validate the performance of follow-up schedules. This is the first time that the distribution of disease, detailed baseline characteristics and the respective treatment of men with GCT is collected in a prospective manner in German speaking countries (Switzerland, Austria and Germany) and therefore patterns of care have been evaluated. SAG TCCS results will inform on future modifications of surveillance schedules and follow-up procedures. NCT02229916 (Clinicaltrials.gov).

Mediastinal surgery: modern treatment of primary germ cell tumor of the mediastinum

The mediastinum is the most common location of extragonadal germ cell tumors. Chemotherapy and/or surgery is the mainstay of treatment of these primary mediastinal germ cell tumors. Pure mediastinal seminomas are usually curable with chemotherapy alone, while mature teratomas are treated with surgery alone. The remaining nonseminomatous germ cell tumors are treated with neoadjuvant chemotherapy and post-chemotherapy resection regardless of tumor marker persistence. These typically anterior tumors can be found adjacent to and invading any of the major mediastinal vessels, nerves, lung, and hila. Surgery can be challenging due to tumor size, location, and adherence to important structures. Appropriate pre-surgical planning, positioning, approach, exposure, and intraoperative decision making are all integral to successful resection. We describe the work up and crucial preoperative considerations, various incisions and their applications to tumor size and location, and resectable structures and factors to consider in their removal.