Incidence Of Germ Cell Tumors Research Articles

A Perplexing Case of a Germ Cell Tumor: A Case Report.

Germ cell tumors (GCTs) are associated with pure gonadal dysgenesis or Swyer syndrome. Swyer syndrome usually presents with primary amenorrhea, streak ovaries, and mixed GCT. However, our patient presented with secondary amenorrhea, normal female external genitalia, and a mixed GCT. Constitutional karyotype was suggestive of 46,XY. Management comprised chemotherapy, followed by surgery. Histopathology was suggestive of dysgerminoma complicating a gonadoblastoma. The purpose of reporting this case is its rarity and the importance of diagnosing an XY karyotype, as the incidence of GCTs is higher in these patients.

No evidence for seasonal variations of the incidence of testicular germ cell tumours in Germany.

The pathogenesis of testicular germ cell tumours (GCTs) is still incompletely understood. Any progress in its understanding must derive from observational studies. Recently, it has been suggested that the incidence of GCTs may follow a seasonal pattern based on circannual changes in the Vitamin D serum levels, with maximum incidence rates in winter months. To examine this promising hypothesis, we studied monthly incidence rates of testicular GCTs in Germany by analysing 30,988 GCT cases aged 15-69 years, diagnosed during 2009-2019. Monthly incident case numbers with data regarding histology and patient age were obtained from the Robert Koch Institut, Berlin, along with annual male population counts. We used precision weighting for deriving pooled monthly incidence rates for GCTs of the period 2009-2019. We stratified pooled rates by histology (seminoma and nonseminoma) and age (15-39 and 40-69 years). By assuming a cyclical effect, we used an estimator of the intensity of seasonal occurrence and report seasonal relative risks (RR). The mean monthly incidence rate was 11.93/105 person-months. The seasonal RR for testicular cancer over-all is 1.022 (95% CI 1.000-1.054). The highest seasonal RR was found in the subgroup of nonseminoma aged 15-39 years, with a RR 1.044 (95% CI 1.000-1.112). The comparison of the pooled monthly rates of the winter months (October-March) with the summer months (April-September) revealed a maximum relative difference of 5% (95% CI 1-10%) for nonseminoma, aged 15-39 years. We conclude that there is no evidence of a seasonal variation of incidence rates of testicular cancer. Our results are at odds with an Austrian study, but the present data appear sound because the results were obtained with precision weighted monthly incidence rates in a large population of GCT cases.

Abdominal and gonadal mature cystic teratomas

The estimated incidence of germ cell tumours (GCT), is approximately 0.9/100,000 population [1,2]. One type of germ cell tumour, known as a teratoma, is typically derived from primordial germ cells, which have the potential to differentiate into somatic cells [3]. A mature teratoma consists of tissues from two or three germ layers derived from the endoderm, mesoderm, and ectoderm [4]. The tissues from the three germ layers include lipid, epithelium, bone, cartilage, hair, fat, muscle, and nerve tissue. A mature teratoma usually forms in the gonads, either in the male testis or the female ovary. Teratomas have also been reported along the central axis of the body, including the sacrococcygeal region, mesentery, and adrenal glands [5,6]. Extragonadal abdominopelvic teratomas are quite rare, however, and occur in both sexes. Because they represent only 1–5% of GCT [7], some clinicians may not consider them as a possible diagnosis. Nevertheless, the radiological appearance of extragonadal abdominopelvic teratomas have unremarkable characteristics and similar features. Consequently, a better understanding of rare abdominopelvic teratomas will lead to improved radiological diagnoses and treatment, as well as the prevention of avoidable complications due to delayed diagnosis.

An extragonadal germ cell tumor with dermatomyositis: A case report and literature review.

The risk of malignancy in inflammatory myopathy patients is well recognized. However, the incidence of germ cell tumor (GCT) with inflammatory myopathy is low, and most reported cases of GCT also exhibit testicular tumors. Therefore, a case of extragonadal GCT with dermatomyositis (DM) is reported in the current study to better understand this paraneoplastic syndrome. A 53-year-old man presented with bilateral cervical lymph node enlargement. A lymph node biopsy showed embryonal carcinoma, and computed tomography showed multiple lymph node and lung metastases. A period of one month after bleomycin, etoposide and cisplatin (BEP) chemotherapy, this patient developed an erythematous eruption over the extensor surfaces of bilateral fingers, or Gottron's sign and facial erythema. The patient was diagnosed with DM with a positive anti-TIF-1γ-antibody result. High-dose prednisolone was effective, and there has been no evidence of cancer recurrence for over one year. The literature review identified 17 cases of GCT with inflammatory myopathy that have been reported so far, and it was indicated that this is the first case of extragonadal GCT with DM following chemotherapy. This case highlights the importance of monitoring after the completion of cancer treatment, as distinctive dermal and muscular symptoms should cause us to consider the possibility of paraneoplastic inflammatory myopathy.

Prospective Study of Gross and microscopic changes in 146 cases of Cryptorchid Testes at varied locations with particular reference to the incidence of Germ cell Tumors in Biopsy specimens.

Cryptorchidism or undescended testes is a commonly observed congenital anomaly of male children seen in patients presenting in general and paediatric surgical practice of our region. Cryptorchid testes are observed unilaterally or bilaterally in one to ten per cent of male infants at birth1 and its incidence decreases to 0.8 per cent at the age of one year. Occasionally a truly cryptorchid testis descends spontaneously at puberty or in response to parental chorionic gonadotropin therapy , but this is not the rule and usually surgical exploration with orchidopexy or orchidectomy (in rare cases) is required. Cryptorchid testes are associated with varied pathological changes depending upon age at presentation, location of undescended testes and familial and genetic factors. In this study, we examined gross and microscopic changes in histological specimens of 146 patients operated in the department of General Surgery at Govt. Medical College Baramulla from Nov. 2014 to Dec. 2019 and concluded that most of the patients tend to present late for surgery especially in the rural settings of north Kashmir with consequent increased risks of malignancy and infertility.

Non-epithelial ovarian cancer in Denmark – Incidence and survival over nearly 40 years

ObjectiveTo examine trends in incidence and survival of non-epithelial ovarian cancer in Denmark over nearly 40 years, using nationwide, population-based cancer registry data. MethodsFrom 1978 to 2016, we identified the non-epithelial ovarian cancer cases among all ovarian malignancies in the Danish Cancer Registry. Age-specific incidence rates, age-standardized incidence rates, and average annual percentage change (AAPC) were estimated with 95% confidence intervals (CIs). Overall and 5-year relative survival analyses were conducted and supplemented with Cox regression to explore the effect of different characteristics on overall mortality. ResultsA total of 720 non-epithelial ovarian cancers were identified, corresponding to 3.4% of all ovarian malignancies. The majority of non-epithelial ovarian cancers were germ cell tumors (49.9%) and sex cord-stromal tumors (38.6%). The age-standardized incidence rate of germ cell tumors was stable over the study period, ranging between 0.33 and 0.39 per 100,000 woman-years. In contrast, the age-standardized incidence rate of sex cord-stromal tumors declined from 0.30 (1978–1987) to 0.09 (2008–2016) per 100,000 woman-years (AAPC = −5.15%; 95% CI: −7.29, −2.96). The 5-year relative survival of germ cell tumors and sex cord-stromal tumors was 94% and 79%, respectively, in the most recent period (2008–2011). Cox regression showed that overall mortality was associated with calendar year, age, and stage. ConclusionsThe incidence of germ cell tumors was stable over calendar time, whereas the incidence of sex cord-stromal tumors decreased significantly. Non-epithelial ovarian cancer overall mortality has decreased during the study period and this could not be explained by taking stage and age at diagnosis into account.

Descriptive epidemiology of germ cell tumors of the central nervous system diagnosed in the United States from 2006 to 2015.

Germ cell tumors (GCT) in the central nervous system (CNS) are rare tumors that occur with highest frequency in males, Asian populations, and children less than age 20years. Due to the rarity of these tumors, their patterns of incidence are not well-described. The aim of this study is to provide the most up-to-date data on incidence and survival patterns for CNS GCT by sex, race, and age at diagnosis. The Central Brain Tumor Registry of the United States (CBTRUS) is the largest aggregation of population-based incidence data on primary brain and other CNS tumors in the United States, containing incidence data from 51 central cancer registries and representing 100% of the US population. The current study used the CBTRUS analytic file to examine incidence (IR) of CNS GCT from 2006 to 2015, as well as registry data from the Surveillance, Epidemiology, and End Results (SEER) program to examine survival. Males had greater IR than females in all CNS GCT histologies examined. Asian and Pacific Islanders had a significantly greater IR of CNS GCT than the other race categories. We confirmed that CNS GCT IR was greatest for those age 10-14years and male. Overall survival rates were high for malignant CNS GCT, germinoma, mixed GCT, and malignant teratoma. There is significant variation in CNS GCT incidence by sex, race, and age at diagnosis. Ascertaining accurate incidence and survival rates of CNS GCT provides vital information usable in real time for clinicians, public health planners, patients, and their families.

Germ Cell Tumors in Dysgenetic Gonads

This review describes the germ cell neoplasms that are malignant and most commonly associated with several types of gonadal dysgenesis. The most common neoplasm is gonadoblastoma, while others including dysgerminomas, yolk-sac tumors and teratomas are rare but can occur. The purpose of this review is to evaluate the incidences of these abnormalities and the circumstances surrounding these specific tumors.According to well-established methods, a PubMed systematic review was performed, to obtain relevant studies published in English and select those with the highest-quality data.Initially, the first search was performed using gonadal dysgenesis as the search term, resulting in 12,887 PubMed papers, published, from 1945 to 2017. A second search using ovarian germ cell tumors as the search term resulted in 10,473 papers, published from 1960 to 2017. Another search was performed in Medline, using germ cell neoplasia as the search term, and this search resulted in 7,560 papers that were published between 2003 to 2016, with 245 new papers assessing gonadoblastomas.The higher incidence of germ cell tumors in gonadal dysgenesis is associated with a chromosomal anomaly that leads to the absence of germ cells in these gonads and, consequently, a higher incidence of neoplasms when these tumors are located inside the abdomen. Several hypotheses suggest that increased incidence of germ cell tumors involves all or part of the Y chromosome or different genes.

Spatial and temporal variations of childhood cancers: Literature review and contribution of the French national registry.

BackgroundSignificant increases in childhood cancer incidence since the 1970s have been consistently reported worldwide, but the persistence of the increase on recent periods is discussed. No conclusion can be drawn concerning the spatial variations of childhood cancer, either. This study is an in‐depth investigation of the spatial and temporal variations of childhood cancer in France. An extensive review of all the studies published since 2000 on those issues is provided.MethodsThe study included 25 877 cases of childhood cancer registered nationwide over 2000‐2014. The spatial heterogeneity (overdispersion, autocorrelation, overall heterogeneity) was tested, on two geographic scales, and two spatial scan methods were used to detect clusters of cases. The annual average percent change (AAPC) in incidence rate was estimated with Poisson regression models, and joinpoint analyses were considered.ResultsGlioma and non‐Hodgkin lymphoma cases exhibited some spatial heterogeneity and two large clusters were detected. Overall, the incidence rate of childhood cancer was stable over 2000‐2014 (AAPC = −0.1% [−0.3%; 0.2%]). A log‐linear positive trend was significantly evidenced for gliomas other than pilocytic astrocytomas (AAPC = 1.8% [0.9%; 2.7%]), with some suggestion of a leveling‐off at the end of the period, while Burkitt lymphoma and germ cell tumor incidence rates decreased (AAPC = −2.2% [−3.8%; −0.5%] and AAPC = −1.9% [−3.4%; −0.3%], respectively). No spatial heterogeneity or significant time variation was evidenced for other cancers.ConclusionSeveral types of childhood cancer displayed some spatial heterogeneity and two large clusters were detected, the origins of which are to be investigated and might include differences in case ascertainment. Overall, the results do not support a sustained increase in incidence rates of childhood cancer in recent years.

Intracranial Germ Cell Tumors: Spectrum of Disease in an Indian Cohort and Management Strategies.

Background:Germ cell tumors (GCTs) represent approximately 3% of primary pediatric brain tumors in the West, whereas in Asia, they constitute between 8% and 15% of pediatric brain tumors.Methods:We retrospectively studied all patients with intracranial GCT managed at our institute from January 1998 to December 2013. The clinical data and radiological data were analyzed.Results:Forty-eight patients with intracranial GCT including 36 males and 16 females formed the cohort. The proportion of GCT in our study was 0.29%. The mean age was 16.5 ± 2.5 years. Germinomas constituted 56.3% and nongerminomatous GCTs constituted 43.7% of all the tumors. The most common location was posterior third ventricle (58.3%) followed by suprasellar (22.9%). Histopathological diagnosis was obtained in almost all patients (96%). Surgical procedures included tumor decompression (71.7%), stereotactic biopsy (13%), and endoscopic third ventriculostomy and biopsy (15%). Patient's age, location of the tumor, and histology did not influence the survival. Women with GCTs had poorer survival when compared to men.Conclusions:The present study documented a lower hospital-based incidence of GCT in Indian cohort. A multidisciplinary approach including surgical strategy based on location, appropriate radiation planning, and chemotherapy is needed for effective treatment and improved outcomes.

Racial and Ethnic Disparities in the Incidence of Pediatric Extracranial Embryonal Tumors.

Few studies have comparatively assessed differences in the incidence of childhood cancer by race and ethnicity that could inform etiologic research. We aimed to identify disparities in the incidence of pediatric extracranial embryonal tumors by race and ethnicity in the United States using a population-based cancer registry. Cases of extracranial embryonal tumors among children age 0 to 19 years diagnosed between 2000 and 2010 were retrieved from the Surveillance, Epidemiology, and End Results Program 18 (n = 8188). Age-standardized incidence rates and incidence rate ratios (IRRs) were obtained by race/ethnicity. Whites were the reference group. The percentage of families living below the poverty line by county was used to stratify by socioeconomic status (SES). All minority groups had a lower incidence of neuroblastoma (Hispanics: IRR = 0.53, 95% confidence interval [CI] = 0.47 to 0.59; blacks: IRR = 0.70, 95% CI = 0.61 to 0.81; Native-Hawaiian/Asian-Pacific-Islander (API): IRR = 0.56, 95% CI = 0.46 to 0.67; and American Indian/Alaska Native (AI/AN): IRR = 0.28, 95% CI = 0.15 to 0.48) while Hispanics had a higher incidence of retinoblastoma (IRR = 1.26, 95% CI = 1.07 to 1.48). Incidence of nephroblastoma was lower among Hispanics (IRR = 0.80, 95% CI = 0.71 to 0.91) and API (IRR = 0.43, 95% CI = 0.33 to 0.56) while equivalent for blacks. Similarly, incidence of rhabdomyosarcoma was low among Hispanics (IRR = 0.85, 95% CI = 0.74 to 0.98) and API (IRR = 0.61, 95% CI = 0.47 to 0.79) while equivalent for blacks. However, incidence of hepatoblastoma was low among blacks (IRR = 0.44, 95% CI = 0.28 to 0.68) while equivalent for Hispanics and API. Incidence of germ cell tumors was higher among Hispanics (IRR = 1.30, 95% CI = 1.19 to 1.42) and lower among blacks (IRR = 0.52, 95% CI = 0.44 to 0.61) and API (IRR = 0.79, 95% CI = 0.67 to 0.93). No effect modification by SES was observed. Unique incidence patterns of childhood extracranial embryonal tumors exist by race and ethnicity in the United States. The interplay between race/ethnicity and genetics, epigenetics, and gene-environment interactions in the causation of these cancers deserves further investigation.

Future of testicular germ cell tumor incidence in the United States: Forecast through 2026.

Testicular germ cell tumors (TGCTs) are rare tumors in the general population but are the most commonly occurring malignancy among males between ages 15 and 44 years in the United States (US). Although non-Hispanic whites (NHWs) have the highest incidence in the US, rates among Hispanics have increased the most in recent years. To forecast what these incidence rates may be in the future, an analysis of TGCT incidence in the Surveillance, Epidemiology, and End Results program and the National Program of Cancer Registries was conducted. TGCT incidence data among males ages 15 to 59 years for the years 1999 to 2012 were obtained from 39 US cancer registries. Incidence rates through 2026 were forecast using age-period-cohort models stratified by race/ethnicity, histology (seminoma, nonseminoma), and age. Between 1999 and 2012, TGCT incidence rates, both overall and by histology, were highest among NHWs, followed by Hispanics, Asian/Pacific Islanders, and non-Hispanic blacks. Between 2013 and 2026, rates among Hispanics were forecast to increase annually by 3.96% (95% confidence interval, 3.88%-4.03%), resulting in the highest rate of increase of any racial/ethnic group. By 2026, the highest TGCT rates in the US will be among Hispanics because of increases in both seminomas and nonseminomas. Rates among NHWs will slightly increase, whereas rates among other groups will slightly decrease. By 2026, Hispanics will have the highest rate of TGCT of any racial/ethnic group in the US because of the rising incidence among recent birth cohorts. Reasons for the increase in younger Hispanics merit further exploration. Cancer 2017;123:2320-2328. © 2017 American Cancer Society.

Nationwide Population-Based Incidence and Survival Rates of Malignant Central Nervous System Germ Cell Tumors in Korea, 2005-2012.

PurposeMalignant central nervous system (CNS) germ cell tumors (GCTs), although rare, are thought to occur more frequently among Asians. However, a recent population-based study revealed no differences in GCT incidence between Asians and Caucasians. Therefore, this study was conducted to determine the incidence and survival rates of CNS GCTs using the national cancer incidence database, and to compare these rates to those in the United States and Japan.Materials and MethodsWe extracted CNS GCT patients diagnosed between 2005 and 2012 from the Korea Central Cancer Registry database. Age-standardized rates (ASRs), annual percentage change, and the male-female incidence rate ratios (IRRs) were calculated. To estimate the survival rate, we used data for patients diagnosed between 2005 and 2010 and followed their cases until December 31, 2013.ResultsThe ASR for CNS GCT between 2005 and 2012 was 0.179 per 100,000 (95% confidence interval, 0.166 to 0.193), with an overall male-to-female (M:F) IRR of 2.95:1. However, when stratified by site, the M:F IRR was 13.62:1 for tumors of the pineal region and 1.87:1 for those located in nonpineal regions. The most frequent histologic type was germinoma (76.0%), and the most frequent location was the suprasellar region (48.5%). The 5-year survival rate of germinoma patients was 95.3%.ConclusionThe incidence rate of CNS GCTs in Korea during 2005-2012 was 0.179 per 100,000, which was similar to that of the Asian/Pacific Islander subpopulation in the United States. Moreover, the CNS GCT survival rate in Korea was similar to rates in Japan and the United States.

Incidence of cancer in children aged 0–14 years in Taiwan, 1996–2010

Studies have found lower risk of childhood cancer among Asian children. We aim to characterize the recent incidence and incidence-trend of childhood cancer in Taiwan after the National Health Insurance program was launched in March 1995. Data were extracted from the Taiwan Cancer Registry, a population-based database established in 1979. Cases diagnosed at age 0–14 from 1996 to 2010 were analyzed and categorized according to the International Classification of Childhood Cancer, Third Edition (ICCC-3). In total, 8032 childhood cancer cases were included, with a microscopic verification rate of 93.9%. The overall age-standardized rate (ASR) of incidence adjusted to the 2000 World Standard Population is 125.0 cases/million, with a male-to-female ratio of 1.3. The top five cancer types (ICCC-3 subgroup[s]; ASR per million) are acute lymphoblastic leukemia (Ia, 30.3), acute myeloid leukemia (Ib; 9.4), non-Hodgkin lymphoma (IIb,c,e, 9.0), extracranial germ cell tumor (Xb,c; 8.3), and neuroblastoma (IVa; 7.8). The median age of diagnosis was 6 years for both genders. During the study period, the ASR of childhood cancer has been increasing at a rate of 1.2% per year (95% confidence interval, 0.6–1.7%). In contrast to Western countries, China, Japan, and Taiwan have lower incidence of childhood cancer; however, Taiwan's incidence rates of childhood germ cell tumors and hepatic tumors are higher. In conclusion, this population-based study reveals that the incidence rate of childhood cancer in Taiwan is rising consistently. The high incidence of germ cell tumors warrants further investigation.

Increase in testicular germ cell tumor incidence among Hispanic adolescents and young adults in the United States

Although rising incidence rates of testicular germ cell tumors have been well documented in white men, relatively little is known about rates in men of Hispanic origin. In the current study, the authors compared germ cell tumor trends between men of Hispanic and non-Hispanic origin as a function of age at diagnosis. Trends in testicular germ cell tumor incidence among white men were analyzed according to Hispanic ethnicity in 2 data sets of the National Cancer Institute's Surveillance, Epidemiology, and End Results Program, spanning from 1992 to 2010 and from 2000 to 2010 and sampling 15% and 28% of the United States population, respectively. Rates were age-adjusted to the year 2000 US standard population. Between 1992 and 2010, the annual incidence of testicular germ cell tumors among Hispanic whites ages 15 to 39 years increased 58%, from 7.18 cases per 100,000 in 1992 to 11.34 cases per 100,000 by 2010 (P<1 × 10(-9) ). Their incidence rates increased in metropolitan areas for both seminoma and nonseminoma subtypes and for all stages at diagnosis. During the same 19-year interval, incidence among non-Hispanic white young adults increased 7%, from 12.41 to 13.22 per 100,000. During the 2000 to 2010 interval, no significant trends were observed in incidence among non-Hispanic whites. There has been a recent substantial increase in testicular germ cell tumor incidence among Hispanic adolescents and young adults in the United States. Similar trends were not observed in non-Hispanic whites.

Suboptimal survival of male germ-cell tumors in southern Portugal—a population-based retrospective study for cases diagnosed in 1999 and 2000

BackgroundThe outcome of germ-cell tumors (GCTs) is a hallmark of good-quality cancer care. In the Eurocare-4 study, the mean 5-year survival for patients diagnosed from 1995 to 1999 was 95.5%. Patients and methodsWe carried out a population-based retrospective chart review study of male patients diagnosed with GCT in 1999 and 2000 in southern Portugal (2 119 065 males). ResultsThere were 87 GCTs—79 testicular, 2 retroperitoneal, 3 mediastinal, 2 of the central nervous system and 1 of the stomach. For the 81 patients with testicular or retroperitoneal primaries, 35 had stage I, 13 stage II and 30 stage III at presentation (3 unknown). Classification by International Germ Cell Consensus Classification Group criteria, 17 belonged to the poor prognosis group (mediastinal primary 3, liver metastases 11 and very elevated markers 3). With median follow-up of 89 months, the 5-year absolute overall survival was 80% (100% for stage I, 92% for stage II and 53% for stage III disease). ConclusionsWhile GCT incidence was similar to neighboring Spain, the 5-year overall survival was lower than that of other European countries. This may result from delays in diagnosis, suggested by high proportion of high-stage and large-burden disease, and poor adherence to recommended treatment algorithms.

Trends in incidence and survival of pediatric and adolescent patients with germ cell tumors in the United States, 1975 to 2006

Pediatric germ cell tumors (GCTs) are rare and heterogeneous tumors with uncertain etiology. In the current study, data from the National Cancer Institute's Surveillance, Epidemiology and End Results (SEER) Program were used to evaluate trends in incidence and survival of GCTs in boys and girls ages ≤19 years. To the authors' knowledge, few studies to date have evaluated trends in pediatric GCTs. Results from these analyses may provide clues to the etiology of GCTs. Frequencies, incidence rates, and 5-year relative survival rates stratified by sex were evaluated overall and by demographic subgroups based on age (birth to 9 years and 10-19 years), race (white, black, and other), and ethnicity (non-Hispanic and Hispanic) as sample size permitted. In whites, the incidence of GCTs was lower for females than males in the 10-year to 19-year age group (rate ratio [RR], 0.47; 95% confidence interval [95% CI], 0.42-0.53), whereas the rates were similar in the age group for birth to 9 years. In contrast, incidence rates were higher in black females than in black males in both age groups (RR, 2.01 [95%CI, 1.08-3.84] in those ages birth to 9 years; RR, 3.30 [95% CI, 2.13-5.28] in those ages 10-19 years). The incidence of ovarian GCT was significantly higher in Hispanic compared with non-Hispanic girls in the groups aged 10 to 19 years. Incidence rates increased during the study period in boys ages 10 to 19 years (annual percentage change [APC], 1.2; 95% CI, 0.4-2.1) and girls ages birth to 9 years (APC, 1.9; 95% CI, 0.3-2.5). The incidence of pediatric GCTs in the United States appears to be increasing only in certain subgroups, suggesting that the etiology is not completely overlapping in all age groups. Differences in incidence patterns by race and ethnicity merit further investigation.

Morphological Findings

The pathogenesis of cryptorchidism is not known and likely to be multifactorial. On one hand, the extent of testicular changes—for example, the reduced number of spermatogones per tubular cross section, the reduced diameter of the tubuli, the fibrosis of the testes—depends on the position of the gonads. Intra-abdominal testicles have the most severe lesions. On the other hand, the patients’ age at the time of the operation is important. The older the boys are at the time of their surgery the more pronounced the changes. The unphysiological position of the gonads impairs normal testicular development in adolescence and results in an increase of existing lesions. The decrease in spermatogones is crucial as it may impair fertility at a later stage. Patients with unilateral cryptorchidism may also develop impaired fertility or even infertility. In unilateral testicular retention, the scrotal testicle will be affected by the same severe changes as the retained gonad in one third of cases. The “increase” in spermatogones after GnRH therapy probably only seems like an increase, because these cells are stimulated by hormone therapy and are thus more clearly visible. In so called testicular aplasia, no genuine aplasia is present; the term may be established but is erroneous. The primary embryonic gonads are subsequently partially or completely scarred, and the cause of this is usually not found. The cause of the increased incidence of germ cell tumors in cryptorchidism remains unexplained. The presence of atypical germ cells in retained prepubertal testicles has not been confirmed as far as we are aware.

Germ Cell Tumor Concomitant with Acquired Immune Deficiency Syndrome

Patients with acquired immune deficiency syndrome (AIDS) are susceptible to secondary malignant tumors. Among those malignancies, the increased incidence of germ cell tumor (GCT) in patients with AIDS has recently been documented in Western countries, while that is still rare in Japan. Here, we report a man patient with advanced GCT (seminoma) complicated with AIDS who was continuously treated with highly active antiretroviral therapy (HAART). A partial response was obtained after resection of the primary left testis and three courses of chemotherapy. During the clinical course, he contracted unexpected gastric bleeding that made it impossible to take HAART agents and prophylactic agents for opportunistic infection. Thereafter, he suffered from a severe pulmonary infection and consequently died of severe respiratory failure. The lymphopenia related to both chemotherapy and AIDS synergistically rendered this patient immunoincompetent and thus he suffered from this fatal pulmonary infection. The recent progress in AIDS treatment has been reported to prolong the survival of tumor-bearing AIDS patients, especially GCT-bearing AIDS patients. Because of the current increase in the number of AIDS patients in Japan, it is important to report the present case which indicated that careful chemotherapy against GCT with strict management of the immunoincompetence can provide a good prognosis for GCT-bearing AIDS patients.

Chromosome X modulates incidence of testicular germ cell tumors in Ter mice.

Germ cell tumor development in humans has been proposed to be part of testicular dysgenesis syndrome (TDS), which manifests as undescended testes, sterility, hypospadias, and, in extreme cases, as germ cell tumors. Males of the Ter mouse strain show interesting parallels to TDS because they either lack germ cells and are sterile or develop testicular germ cell tumors. We found that these defects in Ter mice are due to mutational inactivation of the Dead-end (Dnd1) gene. Here we report that chromosome X modulates germ cell tumor development in Ter mice. We tested whether the X or the Y chromosome influences tumor incidence. We used chromosome substitution strains to generate two new mouse strains: 129-Ter/Ter that carry either a C57BL/6J (B6)-derived chromosome (Chr) X or Y. We found that Ter/Ter males with B6-Chr X, but not B6-Chr Y, showed a significant shift in propensity from testicular tumor development to sterile testes phenotype. Thus, our studies provide unambiguous evidence that genetic factors from Chr X modulate the incidence of germ cell tumors in mice with inactivated Dnd1.