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Malignant histiocytosis associated with mediastinal germ cell tumor:A case report

World Journal of Clinical Cases 2022年第20期10卷 7116-7123页

作者： Pei-Yi Yang Xiao-Li Ma Wen Zhao Li-Bing Fu Rui Zhang Qi Zeng Hong Qin Tong Yu Yan Su Department of Medical Oncology Pediatric Oncology CenterBeijing Children’s HospitalCapital Medical UniversityNational Center for Children’s HealthBeijing 100045China Department of Pathology Beijing Children’s HospitalCapital Medical UniversityNational Center of Children’s HealthBeijing 100045China Hematology Center Beijing Children’s HospitalCapital Medical UniversityNational Center of Children’s HealthBeijing 100045China Department of Thoracic Surgery Beijing Children's HospitalCapital Medical UniversityNational Center for Children's HealthBeijing 100045China Department of Surgical Oncology Capital Medical UniversityNational Center for Children’s HealthBeijing 100045China Department of Image Center Beijing Children’s HospitalCapital Medical UniversityNational Center of Children’s HealthBeijing 100045China

BACKGROUND germ cell tumors(GCTs)account for 2%of human malignancies but are the most common malignant tumors among males aged 15-35.Since 1983,an association between mediastinal GCT(MGCT)and hematologic malignancies has been recognized.CASE SUMMARY We report a case in which malignant histiocytosis was associated with mediastinal GCTs.The clinical data of a male patient with MGCT admitted to Beijing Children's Hospital were collected retrospectively.The patient was first diagnosed according to imaging and pathological features as having MGCT,and was treated with surgery and chemotherapy.One year after stopping chemotherapy,imaging showed metastases in the right supraclavicular,mediastinum,hilar region and retroperitoneal lymph node,right pleura,right lung,and right para-cardiac margin.Pathological diagnosis of the liver nodular and hilar lymph nodes included systemic juvenile xanthogranuloma and Rosai-Dorfman lesions with malignant transformation(i.e.,morphological characteristics and immunophenotype of histiocytic sarcoma).Following diagnosis,the patient accepted chemotherapy with vindesine,cytarabine and dexamethasone.Positron emission tomography–computed tomography showed partial remission.The patient was followed-up for 10 mo after the diagnosis of malignant histiocytosis,and no sign of progression or relapse was observed.CONCLUSION Physicians should recognize the possibility of hematologic malignancies being associated with MGCT.Suitable sites should be selected for pathological examination.

关键词： germ cell tumor Mediastinal Malignant histiocytosis Histiocytic sarcoma Hematologic malignancies Case report

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Testicular mixed germ cell tumor:A case report

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世界临床病例杂志 2023年第28期11卷 6902-6907页

作者： Qi-Fan Xiao Jie Li Bin Tang Yu-Qing Zhu Department of International General Practice China-Japan Friendship HospitalBeijing 100029China Department of General Practice Xinggu Street Community Health Service CenterBeijing 101200China

BACKGROUND Testicular mixed germ cell tumors(TMGCTs)are rare malignant tumors that are more common in men aged 20–40 years.TMGCTs comprise two or more types of germ cell tumors that primarily affect the testis.Their onset is undetectable;thus,early diagnosis is challenging.However,early recognition and diagnosis substantially improve patient prognosis.CASE SUMMARY We evaluated a rare case of TMGCT in a male patient presenting with recurrent fever and left supraclavicular lymphadenectasis instead of testicular enlargement and pain,which may easily lead to misdiagnosis.We report the clinical signs and symptoms,histopathological characteristics,and immunohistochemical results of this case of malignant TMGCT.CONCLUSION Our case,which was typical with multiple components,along with a literature review,may serve as a basis for early diagnosis.

关键词： germ cell tumor Testicular tumor Lymphadenectasis Immunotherapy Case report

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Primary mixed germ cell tumor of the liver with sarcomatous components

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World Journal of Gastroenterology 2010年第5期16卷 652-656页

作者： Xu, Ai-Min Gong, Shao-Juan Song, Wei-Hua Li, Xiao-Wei Pan, Chun-Hua Zhu, Jun-Jun Wu, Meng-Chao Second Mil Med Univ Dept Intervent Radiotherapy 3 Eastern Hepatobiliary Surg Hosp Shanghai 200438 Peoples R China

germ cell tumor(GCT)of the liver is extremely rare. Here,we describe a case of hepatic mixed GCT with significant sarcomatous components and elevated serum α-fetoprotein(AFP)in a 34-year-old man.Histopathologically,the tumor was composed of two GCTs components:yolk sac tumor and immature teratoma.The predominant components of immature teratoma consisted of several types of tissue that represented different germinal layers(endoderm,mesoderm and ectoderm) and showed varying degrees of differentiation with significant sarcomatous components.The yolk sac component showed positivity for AFP and cytokeratin(AE1/ AE3).The immature teratoma components showed positivity for varying differentiation markers.Interphase cytogenetic analysis revealed that the yolk sac tumor and immature teratoma were positive for i(12p)and 12p over-representation.In particular,the rhabdomyoblastic components also showed typical i(12p)and 12p overrepresentation.This suggested that sarcomatous components may be associated with dedifferentiation or malignant transformation of certain mesenchymal components within teratoma.

关键词： germ cell tumor Teratoma Sarcoma Liver neoplasms

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Assessment of Prognostic Factors of Racial Disparities in Testicular germ cell tumor Survival in the United States(1992–2015)

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Biomedical and Environmental Sciences 2021年第2期34卷 152-162页

作者： WU Jing JI Yi Bing TANG Bi Wei BROWN Matthew WANG Bao Hua DU Chen Lei DU Jian Shu WANG Xue Mei CAI Li Jun WU Guo Yi ZHOU Yan National Center for Chronic and Non-communicable Diseases Control and Prevention Chinese Center for Disease Control and PreventionBeijing 100050China Inner Mongolia Medical University Hohhot 010110Inner MongoliaChina National Cancer Insitiute National Institutes of HealthBethesda 20892MarylandUnited States Shanghai Topgen Biopharm Technology Co. LtdShanghai 201203China Scientific Research Center Shanghai Public Health Clinical CenterFudan UniversityShanghai 201508China State Key Laboratory of Genetic Engineering School of Life SciencesFudan UniversityShanghai 200433China Shanghai-MOST Key Laboratory of Health and Disease Genomics Chinese National Human Genome Center at ShanghaiShanghai 200000China

Objective Testicular germ cell tumors(TGCT) are the most common cancer among men aged 15 to 39 years. Previous studies have considered factors related to TGCT survival rate and race/ethnicity, but histological type of the diagnosed cancer has not yet been thoroughly assessed.Methods The data came from 42,854 eligible patients from 1992 to 2015 in the Surveillance Epidemiology and End Results 18. Frequencies and column percent by seminoma and nonseminoma subtypes were determined for each covariates. We used Cox proportional hazard regression to assess the impact of multiple factors on post-diagnostic mortality of TGCT.Results Black males were diagnosed at a later stage, more commonly with local or distant metastases.The incidence of TGCT in black non-seminoma tumors increased most significantly. The difference in survival rates between different ethnic and histological subtypes, overall survival(OS) in patients with non-seminoma was significantly worse than in patients with seminoma. The most important quantitative predictor of death was the stage at the time of diagnosis, and older diagnostic age is also important factor affecting mortality.Conclusion Histological type of testicular germ cell tumor is an important factor in determining the prognosis of testicular cancer in males of different ethnic groups.

关键词： Testicular cancer germ cell tumor Race Survival rate Survival analysis

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Panhypopituitarism caused by a suprasellar germinoma: A case report

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World Journal of Clinical Cases 2024年第10期12卷 1844-1850页

作者： Jelena Roganovic Lea Saric Silvije Segulja Ana Dordevic Mia Radosevic Department of Pediatrics Clinical Hospital Centre RijekaRijeka 51000Croatia Faculty of Medicine University of RijekaRijeka 51000Croatia Faculty of Health Sciences University of RijekaRijeka 51000Croatia Department of Business Development Jadran Galenski LaboratorijRijeka 51000Croatia

BACKGROUND Suprasellar germinomas are rare intracranial tumors frequently associated with permanent endocrine disorders.We present the clinical picture,treatment,and complications of suprasellar germinoma at pediatric age which,besides being lifethreatening,has lifelong endocrinological consequences.CASE SUMMARY A 12-year-old female patient was presented having had intensive headaches for three weeks and visual disturbances for six months.An ophthalmological examination revealed bilateral papilledema and a marked loss of vision.Emergency brain magnetic resonance imaging(MRI)showed a suprasellar tumor,involving the infundibulum and the optic chiasm,extending to the third ventricle.Laboratory tests confirmed decreased levels of thyroxine,cortisol,gonadotropins,and insulin-like growth factor 1.Maximal tumor reduction was performed,and immunohistopathology established the diagnosis of suprasellar germinoma.MRI of the spine and cerebrospinal fluid cytology confirmed the localized disease.Adjuvant chemotherapy and radiotherapy were performed according to the SIOP CNS GCT II protocol.A post-treatment MRI showed no residual tumor,but pituitary function had not recovered.Three and a half years after the end of the treatment,the patient is in a complete remission,requiring hormonal replacement therapy,continuous education,and psychological support.CONCLUSION This complex case highlights the importance of timely diagnosis,a multidisciplinary approach,and close follow-up in children with suprasellar germinomas.

关键词： germinoma germ cell tumor Suprasellar tumor Hypopituitarism Management Pediatrics Case report

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Management of Testicular Cancers in Brazzaville

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Open Journal of Urology 2024年第5期14卷 324-332页

作者： Roland Bertile Banga-Mouss Yanick Dimi Nyanga Brice Nkoua Epala Armel Melvin Atipo Ondongo Steve Aristid Ondziel-Opara Eliane Ndounga Alexis Bolenga Louis Constant Mbele Mantsouaka Judith Nsonde Malanda Aanani Séverin Wencesl Odzébé Prosper Alain Bouya Departement of Urology and Andrology University Hospital of Brazzaville Brazzaville Republic of the Congo Departement of Medicale Oncology and Radiotherapy University Hospital of Brazzaville Brazzaville Republic of the Congo

Introduction: Testicular cancer accounts for 5% of urological tumors, predominantly affecting young men. The aim of our study was to report the diagnostic and evolutionary aspects of testicular cancer cases treated in our center. Patients and Methods: A retrospective study conducted over a 15-year period involving 12 patients treated for testicular cancer at the University Hospital of Brazzaville. Results: The median age was 31 years (range 11 to 49 years), with a median consultation delay of 10.6 months (range 3 to 27 months). Scrotal mass was the most common reason for consultation. Cancer was bilateral in two patients. Two patients were admitted with metastatic disease. Histopathological examination favored germ cell tumors in 7 cases, two cases of non-Hodgkin’s malignant lymphoma, and one case of epididymo-testicular adenocarcinoma. Adjuvant chemotherapy resulted in complete remission in patients with germ cell tumors. However, neoadjuvant chemotherapy was not effective in patients admitted with advanced-stage disease. Conclusion: Testicular cancer is a rare condition that is curable in the majority of cases, but its management is often complicated in our setting due to delayed diagnosis caused by taboos surrounding genital organ pathologies.

关键词： Testicular Cancer germ cell tumor Cisplatin Testicular Lymphoma Testicular Adenocarcinoma

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Molecular and epigenetic pathogenesis of germ cell tumors

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Asian Journal of Urology 2021年第2期8卷 144-154页

作者： Melanie R.Müller Margaretha A.Skowron Peter Albers Daniel Nettersheim Department of Urology Urological Research LabTranslational UroOncologyUniversity Hospital DüsseldorfDüsseldorfGermany Department of Urology University Hospital DüsseldorfDüsseldorfGermany

The development of germ cell tumors(GCTs)is a unique pathogenesis occurring at an early developmental stage during specification,migration or colonization of primordial germ cells(PGCs)in the genital ridge.Since driver mutations could not be identified so far,the involvement of the epigenetic machinery during the pathogenesis seems to play a crucial role.Currently,it is investigated whether epigenetic modifications occurring between the omnipotent two-cell stage and the pluripotent implanting PGCs might result in disturbances eventually leading to GCTs.Although progress in understanding epigenetic mechanisms during PGC development is ongoing,little is known about the complete picture of its involvement during GCT development and eventual classification into clinical subtypes.This review will shed light into the current knowledge of the complex epigenetic and molecular contribution during pathogenesis of GCTs by emphasizing on early developmental stages until arrival of late PGCs in the gonads.We questioned how misguided migrating and/or colonizing PGCs develop to either type Ⅰ or type Ⅱ GCTs.Additionally,we asked how pluripotency can be regulated during PGC development and which epigenetic changes contribute to GCT pathogenesis.We propose that SOX2 and SOX17 determine either embryonic stem cell-like(embryonal carcinoma)or PGC-like cell fate(seminoma).Finally,we suggest that factors secreted by the microenvironment,i.e.BMPs and BMP inhibiting molecules,dictate the fate decision of germ cell neoplasia in situ(into seminoma and embryonal carcinoma)and seminomas(into embryonal carcinoma or extraembryonic lineage),indicating an important role of the microenvironment on GCT plasticity.

关键词： germ cell tumor Primordial germ cell Epigenetic reprogramming Plasticity Microenvironment BMP signaling SOX2 SOX17

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Recent advances in molecular biology and treatment strategies for intracranial germ cell tumors

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世界儿科杂志:英文版 2016年第3期12卷 275-282页

作者： Xiang Huang Rong Zhang Ying Mao Liang-Fu Zhou Chao Zhang Department of Neurosurgery Huashan HospitalFudan University12 Wulumuqi Zhong RoadShanghaiChina

Background:Intracranial germ cell tumors(IGCTs)are a group of rare pediatric brain tumors which include various subtypes.The current understanding of the etiology of the tumors and their optimal management strategies remain controversial.Data sources:The data on IGCTs were collected from articles published in the past 20 years,and the origin and etiology of IGCTs at molecular level as well as the relative roles of varied treatment strategies in different prognosis groups according to Matsutani's classifi cation were reviewed.Results:Recent cellular and molecular evidence suggests that IGCTs may arise from the transformation of endogenous brain cells;and findings in the molecular characterization of IGCTs suggest roles of CCND2,RB1,and PRDM14 in the pathogenesis of IGCTs and identify the KIT/RAS and AKT1/mTOR pathways as potential therapeutic targets in future.According to Matsutani's classifi cation of IGCTs,the good prognosis group includes both germinomas and mature teratomas.For germinomas,both radiation alone and reduced-dose radiotherapy in combination with adjuvant chemotherapy are effective,while complete surgical excision is recommended for mature teratomas.In the intermediate prognosis group,immature teratoma has been successfully treated with gamma knife surgery.However,for intermediate prognosis IGCTs other than immature teratomas,gross total resection with adjuvant chemotherapy and radiotherapy or gamma knife surgery may be necessary to achieve cure.In the poor prognosis group,survival outcomes are unsatisfactory,and complete surgical resection combined with more intensive chemotherapy and radiotherapy remains the best available treatment option at this time.Conclusions:IGCTs should be strictly classified according to their pathological categories before administering pathology-specific treatments.Although open microsurgical excision is the traditional surgical strategy for IGCTs,recent publications also support the role of endoscopic surgical options for pineal region IGCTs.

关键词： etiology germ cell tumor prognosis treatment

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Spermatocytic tumor:A rare case report

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World Journal of Clinical Cases 2021年第35期9卷 11115-11121页

作者： Mei-Ling Hao Chun-Hui Li Department of Pathology Chengde Medical College Affiliated HospitalChengde 067000Hebei ProvinceChina

BACKGROUND Spermatocytic tumor is a rare,malignant neoplasm of the testes.Since the prognosis for this tumor type is favorable,accurate diagnosis and differentiation from other malignant testicular neoplasms(classic seminoma and lymphoma)are crucial.To add to the existing literature on the diagnosis of spermatocytic tumor,herein we report the detailed clinical and histopathologic findings for a case that we encountered.CASE SUMMARY A 60-year-old Chinese man presented with a solid mass in the right scrotum.The mass was surgically removed and spermatocytic tumor was diagnosed.On microscopy,the tumor cells displayed an unusual arrangement in lobules,presenting a pseudo-glandular appearance.To summarize and compare the diagnostic features of this tumor and those of the differential diagnoses,we report our case findings and those mentioned in the literature for various testicular tumors.Although imaging methods can detect masses early in development,their diagnostic capabilities are limited.Biopsy,histopathology,and immunohistochemistry are necessary for confirmatory diagnosis.CONCLUSION It is important to identify and review the key diagnostic features of spermatocytic tumor.

关键词： Spermatocytic tumor germ cell tumor Immunohistochemistry Pseudoglandular Case report

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Refractory Ascites Revealing an Ovarian Yolk Sac tumor with Intraperitoneal Rupture

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Open Journal of Pathology 2023年第2期13卷 93-99页

作者： Vahatra Joëlle Razafimahefa Herilalao Elisabeth Razafindrafara Tojo Rémi Rafaralahivoavy Tsitohery Francine Andriamampionona Nantenaina Soa Randrianjafisamindrakotroka Department of Pathology CHU Andrainjato Fianarantsoa Madagascar Department of Pathology Soavinandriana Hospital Center Antananarivo Antananarivo Madagascar Department of Radiology CHU Andrainjato Fianarantsoa Madagascar Department of Pathology Medical School of Antananarivo Antananarivo Madagascar

Yolk sac tumors of the ovary are rare entities that account for 2% - 5% of all ovarian tumors. They represent the second most common histological variant of malignant germ cell tumors of the ovary after dysgerminomas. Yolk sac tumors are most commonly encountered in women in the second and third decades. Microscopically, they are highly polymorphic and can present in a pure form or associated with another contingent of germ cell tumor. We report the case of a 26-year-old woman, who underwent surgery for a large right ovarian tumor rupturing into the peritoneal cavity. The ovarian tumor was revealed by ascites of great abundance and abdomino-pelvic pain. On histological examination, the diagnosis of yolk sac tumor in its pure and polyvesicular vitelline pattern was made. Through this observation, we propose to discuss the anatomoclinical particularities of these tumors by emphasizing the importance of histology for the diagnosis as well as the need of an early and appropriate management.

关键词： germ cell tumor Yolk Sac tumor Ovary Abdominal Distension Schiller-Duval Ascite

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