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1.
Medicine (Baltimore) ; 99(39): e22146, 2020 Sep 25.
Artigo em Inglês | MEDLINE | ID: mdl-32991408

RESUMO

MOGCTs (malignant ovarian germ cell tumors) are rare tumors that mainly affect patients of reproductive age. The aim of this study was to evaluate the fertility and survival outcomes in young women with MOCGTs treated with fertility-sparing surgery (FSS).From 2000 to 2018, data from 28 patients of reproductive age with a diagnosis of MOGCT at the University of Bari were collected. Most received FSS, and in patients treated conservatively, the reproductive outcome and survival were investigated. Data of patient demographics, clinical presentation, oncology marker dosage, staging, type of surgery, histological examination, survival, and reproductive outcome were collected from hospital and office charts. All informed consent was obtained from all patients. The median age was 24 (range: 9-45 years). The majority of the patients had stage IIIC. Twenty-four woman received FSS consisting of unilateral ovariectomy and omentectomy, whereas only 4 women, based on their stage (IIIC), received a radical surgery (hysterectomy with bilateral adnexectomy, lymphadenectomy, and omentectomy). Our study shows that FSS in MOGCTs can produce good results both on reproductive outcomes and on survival. Indeed, in our group, there was only 1 case of exitus as result of recurrence. Furthermore, patients after FSS maintained normal ovarian function and 5 of 5 women who tried to get pregnant succeeded spontaneously. The median follow-up was 90 months (range 3-159).Conservative surgery for MOGCTs should be considered for women of reproductive age who wish to preserve fertility.


Assuntos
Preservação da Fertilidade/métodos , Neoplasias Embrionárias de Células Germinativas/cirurgia , Neoplasias Ovarianas/cirurgia , Adolescente , Adulto , Criança , Feminino , Humanos , Pessoa de Meia-Idade , Estadiamento de Neoplasias , Neoplasias Embrionárias de Células Germinativas/patologia , Neoplasias Ovarianas/patologia , Adulto Jovem
3.
Bull Cancer ; 107(9): 912-924, 2020 Sep.
Artigo em Francês | MEDLINE | ID: mdl-32653158

RESUMO

Seminomatous (SGCT) and non-seminomatous (NSGCT) germ cell tumors (GCT) are rare but their incidence are increasing. We will discuss different therapeutic strategies in relapse disease: patients with stage I germ cell tumor have an excellent prognosis with a cure rate approaching 98-99 %, whatever the histology and the chosen treatment (surveillance strategy or adjuvant treatment). Relapses are observed among 20% of patients with stage I SGCT or low risk NSGCT and 50 % of patients with high risk NSGCT. Patients are treated according to the international prognosis group (IGCCCG) for SGCT and low risk NSGCT, naïve of chemotherapy. After an adjuvant treatment, the protocol must be adapted to the number of previous cycles (1 or 2 BEP) and to the prognosis group. Five to 50% of patients relapse after a first line of metastatic chemotherapy according to initial prognosis group. Dose-dense chemotherapy according to the GETUG13 protocol reduces the risk of relapse for the patients with poor-risk group NSGCT and unfavorable tumor marker decline. The prognosis of patients with relapsed or refractory GCT after a first line is more negative since only half of them will be cured by salvage standard chemotherapy. An international therapeutic trial (TIGER) is ongoing in first line salvage treatment evaluating high-dose chemotherapy (HDCT) with hematopoietic stem cell transplantation (HSCT). Finally, developing biomarkers for predicting clinical relapse, the management in expert centers of these patients and participation in therapeutic innovation are important perspectives for a better understanding and treatment of these patients with a poorer prognosis.


Assuntos
Recidiva Local de Neoplasia/terapia , Neoplasias Embrionárias de Células Germinativas/terapia , Neoplasias Testiculares/terapia , Algoritmos , Humanos , Recidiva Local de Neoplasia/tratamento farmacológico , Recidiva Local de Neoplasia/patologia , Estadiamento de Neoplasias , Neoplasias Embrionárias de Células Germinativas/tratamento farmacológico , Neoplasias Embrionárias de Células Germinativas/patologia , Neoplasias Testiculares/tratamento farmacológico , Neoplasias Testiculares/patologia
5.
Mutat Res ; 854-855: 503200, 2020.
Artigo em Inglês | MEDLINE | ID: mdl-32660824

RESUMO

Germ cell tumour (GCT) patients who fail to respond to chemotherapy or who relapse have a poor prognosis. Timely and accurately stratifying such patients could optimise their therapy. We identified endogenous DNA damage levels as a prognostic marker for progression-free (PFS) and overall (OS) survival in chemotherapy-naïve GCT patients. In the present study, we have extended our previous results and reviewed the prognostic power of DNA damage level in GCTs. Endogenous DNA damage levels were measured with the comet assay. Receiver operator characteristic analysis was applied to determine the optimal cut-off value and to evaluate its prognostic accuracy. PFS and OS were estimated by the Kaplan-Meier method and compared using the log-rank test. Hazard ratio (HR) estimates were calculated by Cox regression analysis. A cut-off value of 6.34 provided the highest sensitivity and specificity, with area under curve values of 0.813 and 0.814 for disease progression and mortality, respectively. A % DNA in tail > 6.34 was significantly associated with shorter PFS (HR = 9.54, 95 % confidence interval [CI]: 3.43-26.55, p < 0.001) and OS (HR = 14.62, 95 % CI: 3.14-67.95, p = 0.001) by univariate analysis. The prognostic value of DNA damage measurement was confirmed by multivariate models (HR = 6.45, 95 % CI: 2.22-18.75, p = 0.001 for PFS and HR = 9.40, 95 % CI: 1.70-52.09, p = 0.010 for OS), when HR was adjusted for relevant clinical categories. The added prognostic value of DNA damage in combination with International Germ Cell Cancer Collaborative Group (IGCCCG) risk groups has been revealed. Endogenous DNA damage is an independent prognosticator for PFS and OS in GCT patients and its clinical use, particularly in combination with IGCCCG risk groups, may help in stratifying these patients.


Assuntos
Células Sanguíneas/patologia , Dano ao DNA/genética , Neoplasias Embrionárias de Células Germinativas/genética , Neoplasias Embrionárias de Células Germinativas/patologia , Adulto , Células Cultivadas , Ensaio Cometa/métodos , Progressão da Doença , Intervalo Livre de Doença , Feminino , Humanos , Estimativa de Kaplan-Meier , Leucócitos Mononucleares/patologia , Masculino , Pessoa de Meia-Idade , Prognóstico , Intervalo Livre de Progressão , Modelos de Riscos Proporcionais , Fatores de Risco
6.
Crit Rev Oncol Hematol ; 150: 102946, 2020 Jun.
Artigo em Inglês | MEDLINE | ID: mdl-32353705

RESUMO

The presence of brain metastases (BMs) from germ cell tumor (GCT) remains a rare situation. BMs predominantly occur among patients with testis primary tumor site, and are almost exclusively associated with non-seminomatous (NS) histologies. Two situations must be distinguished, which differ in terms of clinical presentation, overall prognostic and management. At diagnosis, BMs are almost systematically associated with extra-cerebral metastases and the cornerstone of treatment is chemotherapy, while the role of local treatment remains controversial. In the metachronous setting, BMs more frequently constitute an isolated site of relapse, the outcome is poorer, and the role of local treatment is more consensual. However, all these data widely come from old reports, with outdated radiation techniques. The recent advances in radiation oncology, especially the rising use of stereotactic radiotherapy, could lead to the reconsideration of ancient dogmas regarding the "radiosensitivity" of (NS)GCT and the role of radiotherapy among patients with BMs.


Assuntos
Neoplasias Encefálicas/cirurgia , Neoplasias Embrionárias de Células Germinativas/cirurgia , Radiocirurgia , Neoplasias Testiculares/cirurgia , Neoplasias Encefálicas/patologia , Humanos , Masculino , Recidiva Local de Neoplasia , Neoplasias Embrionárias de Células Germinativas/patologia , Estudos Retrospectivos , Neoplasias Testiculares/patologia , Resultado do Tratamento
7.
Pediatr Blood Cancer ; 67(7): e28407, 2020 07.
Artigo em Inglês | MEDLINE | ID: mdl-32426927

RESUMO

Little is known about pseudoprogression in brain tumours other than gliomas. A 9-year-old male child with a pineal teratoma/germinoma underwent surgical resection followed by adjuvant chemo-radiotherapy. The magnetic resonance imaging scan 4 months post-radiotherapy showed a contrast-enhancing lesion within the surgical cavity suspicious of recurrence. These radiological findings subsequently resolved without any specific intervention. The child continues in remission 2 years post-treatment. This case illustrates the occurrence of pseudoprogression post-radiotherapy in intracranial GCT and highlights an unmet need for greater implementation of functional imaging techniques in paediatric neuro-oncology to avoid undue discontinuation of effective treatments or inappropriate enrolment in clinical trials.


Assuntos
Imagem por Ressonância Magnética/métodos , Imagem Molecular/métodos , Neoplasias Embrionárias de Células Germinativas/patologia , Pinealoma/patologia , Radioterapia Adjuvante/métodos , Teratoma/patologia , Criança , Progressão da Doença , Humanos , Masculino , Neoplasias Embrionárias de Células Germinativas/diagnóstico por imagem , Neoplasias Embrionárias de Células Germinativas/radioterapia , Pinealoma/diagnóstico por imagem , Pinealoma/radioterapia , Prognóstico , Teratoma/diagnóstico por imagem , Teratoma/radioterapia
8.
World Neurosurg ; 139: 310-313, 2020 07.
Artigo em Inglês | MEDLINE | ID: mdl-32339726

RESUMO

BACKGROUND: Pituitary blastoma is a malignant neoplasm of the pituitary gland that was recognized by the World Health Organization in 2017. It is commonly diagnosed in children before 24 months of age. Here, we report the first case of a young adult patient who was diagnosed with pituitary blastoma with increased levels of growth hormone instead of adrenocorticotropic hormone and provide a review of the literature. CASE DESCRIPTION: A 19-year-old woman presented to our hospital with visual disturbance. She had a medical history of Wilms' tumor and multinodular goiter. The brain imaging showed a 3.2 × 2.5 × 1.8-cm solid sellar and suprasellar cystic mass that upwardly displaced the optic chiasm. She had an elevated level of growth hormone but a normal level of adrenocorticotropic hormone, cortisol, and prolactin. The mass was subtotally removed through the left pterional craniotomy. The pathologic examination suggested a pituitary blastoma. Thereafter, the patient was treated with chemotherapy and radiotherapy. At 4-year follow-up postsurgery, her overall well-being is good. CONCLUSIONS: Although in this case the patient was a young adult, pituitary blastoma should be taken into consideration when children have an enhanced sellar and suprasellar mass with peripherally located cysts.


Assuntos
Neoplasias Embrionárias de Células Germinativas/patologia , Neoplasias da Glândula Tireoide/patologia , Feminino , Bócio Nodular , Hormônio do Crescimento/metabolismo , Humanos , Neoplasias Renais , Neoplasias Embrionárias de Células Germinativas/metabolismo , Neoplasias Embrionárias de Células Germinativas/cirurgia , Segunda Neoplasia Primária/patologia , Neoplasias da Glândula Tireoide/metabolismo , Neoplasias da Glândula Tireoide/cirurgia , Tumor de Wilms , Adulto Jovem
9.
Arch Gynecol Obstet ; 301(5): 1227-1233, 2020 05.
Artigo em Inglês | MEDLINE | ID: mdl-32253553

RESUMO

PURPOSE: To describe a case series of patients with malignant ovarian germ cell tumors (MOGCT) treated exclusively with fertility-sparing surgery (FSS) with or without adjuvant chemotherapy. METHODS: We retrospectively reviewed the records of 27 patients with MOGCT treated in the Department of Obstetrics and Gynecology, University Hospital Center Zagreb, Croatia, between January 2009 and July 2019. RESULTS: The median age at diagnosis was 22 years, and the main symptom was abdominal distension (57.0%). The most prevalent histological subtype was immature teratoma (n = 13, 48.1%). Twenty-three patients (85.2%) had laparotomy and 4 (14.8%) had laparoscopy, without conversions. Lymphadenectomy was performed in 16 (59.3%) patients, with 184 removed lymph nodes, and omentectomy was performed in 19 (70.4%) patients. The rate of chemotherapy administration was 81.5%. The follow-up length ranged between 6.30 and 115.1 months (median: 49.60 months). No patient experienced tumor recurrence. The rate of complete gross resection was 100%. At the time of analysis, all patients were alive and disease free. Fifty percent of patients who actively tried to conceive after FSS became pregnant, with 12 deliveries. CONCLUSION: This study suggests that FSS is a safe treatment option for MOGCT, regardless of tumor stage and histological type.


Assuntos
Preservação da Fertilidade , Neoplasias Embrionárias de Células Germinativas/cirurgia , Neoplasias Ovarianas/tratamento farmacológico , Adolescente , Adulto , Quimioterapia Adjuvante/efeitos adversos , Croácia , Feminino , Fertilidade , Humanos , Excisão de Linfonodo , Pessoa de Meia-Idade , Recidiva Local de Neoplasia/patologia , Estadiamento de Neoplasias , Neoplasias Embrionárias de Células Germinativas/tratamento farmacológico , Neoplasias Embrionárias de Células Germinativas/patologia , Omento/cirurgia , Neoplasias Ovarianas/patologia , Neoplasias Ovarianas/cirurgia , Ovariectomia/métodos , Gravidez , Estudos Retrospectivos , Teratoma/patologia , Centros de Atenção Terciária , Adulto Jovem
10.
Bull Cancer ; 107(3): 385-390, 2020 Mar.
Artigo em Inglês | MEDLINE | ID: mdl-32115180

RESUMO

The group of rare malignant ovarian tumors includes the group of germ cell tumors, sex cords stromal ovarian tumors, small cell carcinoma, malignant Brenner tumors, rare epithelial tumors such as mucinous carcinoma, clear cell carcinoma, or low-grade serous carcinoma, as well as ovarian carcinosarcoma. Together they comprise about 10% of all ovarian tumors. Due to their low prevalence and their heterogeneity, data and treatment recommendations are limited. Even though all ovarian tumors are staged according to the FIGO staging of epithelial ovarian tumors, treatment differs especially in germ cell tumors and sex cords stromal ovarian tumors. Non-epithelial ovarian tumors can arise from a variety of ovarian precursor cells such as germ cells, granulosa cells, theca cells, or stromal fibroblasts. As can be expected already due to their divergent precursor lesions, these malignancies are substantially different but united by their rarity. This overview article gives a comprehensive summary on the pathology and clinical presentation, as well as therapy recommendations of a selection of those rare ovarian tumors, based on the latest national guidelines and related important publications.


Assuntos
Neoplasias Ovarianas , Doenças Raras , Adenocarcinoma de Células Claras/patologia , Adenocarcinoma de Células Claras/terapia , Adenocarcinoma Mucinoso/patologia , Adenocarcinoma Mucinoso/terapia , Tumor de Brenner/patologia , Tumor de Brenner/terapia , Carcinoma de Células Pequenas/patologia , Carcinoma de Células Pequenas/terapia , Carcinossarcoma/patologia , Carcinossarcoma/terapia , Cistadenocarcinoma Seroso/patologia , Cistadenocarcinoma Seroso/terapia , Feminino , Humanos , Neoplasias Embrionárias de Células Germinativas/patologia , Neoplasias Embrionárias de Células Germinativas/terapia , Neoplasias Ovarianas/patologia , Neoplasias Ovarianas/terapia , Doenças Raras/patologia , Doenças Raras/terapia , Tumores do Estroma Gonadal e dos Cordões Sexuais/patologia , Tumores do Estroma Gonadal e dos Cordões Sexuais/terapia
11.
Arch Gynecol Obstet ; 301(4): 901-912, 2020 04.
Artigo em Inglês | MEDLINE | ID: mdl-32185550

RESUMO

PURPOSE: Non-gestational ovarian choriocarcinoma (NGOC) is a rare malignant germ cell tumor. Through literature review and cases collection, we aim to analyze prognostic factors for NGOC and summarize its clinicopathological characteristics to guide the individualized treatment. METHODS: We searched PubMed database, Cochrane library, and Google Scholar for cases published between January 1, 1967 and July 31, 2018 using various search terms. We retrieved patients' clinicopathological characteristics, treatment, and prognosis information from included studies. These patients were divided into two groups: died (case group) or alive (control group) group. We summarized and compared their clinical (age, symptoms, R0 resection, serum HCG levels, chemotherapy regimen) and pathological (pure vs non-pure type, tumor size, tumor location, metastasis sites, stage) features by statistical analysis. RESULTS: Only 39 patients were retrieved from 36 studies in total. The median age was 30 years (range 12- to 65-years old). The peak incidence was in the adolescent age 12-25 years. Median follow-up was 20.3 months (range 1-84 months). 9 (23%) patients died; 24 (62%) patients were alive; 6 (15%) were lost to follow-up. Upon univariate analysis, we found age had a poor impact on overall survival (OS) in NGOC, HR - 0.057, 95% CI - 0.111 to - 0.004. Pure type NGOC has a better OS than mixed type, HR - 2.621, 95% CI - 4.577 to - 0.666. R0 resection is a good prognostic factor for OS, HR 2.967, 95% CI 0.709-5.224. CONCLUSION: Clinicians should try to achieve R0 resection to improve the prognosis for NGOC patients even among advanced patients.


Assuntos
Coriocarcinoma/diagnóstico , Neoplasias Embrionárias de Células Germinativas/diagnóstico , Neoplasias Ovarianas/diagnóstico , Adolescente , Adulto , Idoso , Criança , Coriocarcinoma/patologia , Feminino , Humanos , Masculino , Pessoa de Meia-Idade , Neoplasias Embrionárias de Células Germinativas/patologia , Neoplasias Ovarianas/patologia , Gravidez , Prognóstico , Adulto Jovem
12.
Virchows Arch ; 477(1): 103-110, 2020 Jul.
Artigo em Inglês | MEDLINE | ID: mdl-32144540

RESUMO

INTRODUCTION: Two types of testicular teratomas are distinguished by the current WHO classification. Prepubertal-type teratomas are benign, while postpubertal-type teratomas are considered malignant with metastatic potential, and are associated with germ cell neoplasia in situ. Prepubertal-type cases have been reported in the adult testis potentially causing confusion and overtreatment. Demonstration of the absence of 12p abnormalities with fluorescence in situ hybridization may facilitate diagnosis. Recently, IMP3 has emerged as a potential marker of malignancy in this context. AIMS: The aim of this study was to assess histological characteristics, IMP3 expression and the presence of 12p abnormalities of pure testicular teratomas. RESULTS: Thirty-seven cases were studied, 7 patients were children and 30 were adults. Six out of 7 pediatric cases showed no 12p abnormality and were IMP3 positive. Seventy-four percent and 79% of adult cases showed 12p abnormalities and IMP3 expression, respectively. Negative cases were not associated with in situ neoplasia or metastasis, they were smaller (mean, 14 vs 39 mm), showed less histological diversity (2.4 vs 4.0 types of tissues on average) compared to positive cases. CONCLUSION: Our study provides further evidence that prepubertal-type (type I) teratomas may appear in adult testes, thus teratomas in adults may be either benign (type I) or malignant (type II). IMP3 expression may aid the distinction between type I and type II teratomas of the postpubertal testis even when GCNIS and 12p status cannot be assessed.


Assuntos
Neoplasias Embrionárias de Células Germinativas/patologia , Teratoma/patologia , Neoplasias Testiculares/patologia , Adolescente , Adulto , Biomarcadores Tumorais/metabolismo , Criança , Cromossomos Humanos Par 12 , Feminino , Técnicas Histológicas/métodos , Humanos , Hibridização in Situ Fluorescente/métodos , Masculino , Pessoa de Meia-Idade , Proteínas de Ligação a RNA/metabolismo
13.
Curr Opin Oncol ; 32(3): 250-255, 2020 05.
Artigo em Inglês | MEDLINE | ID: mdl-32168037

RESUMO

PURPOSE OF REVIEW: Although testicular cancer remains a highly curable malignancy, challenges and uncertainty still remain in certain aspects of management. Residual disease after chemotherapy in patients with germ cell tumors (GCT) remains one of these challenges. We aim to highlight the recent literature on the management of residual disease after chemotherapy in GCT and the emerging innovations that may provide further guidance into this area. RECENT FINDINGS: A subset of patients with GCT will have residual disease after chemotherapy, and management of these patients involves highly skilled multidisciplinary experts including medical oncologists, surgeons, radiologists, and pathologists. Management options depend on histologic subtype, either seminoma or nonseminoma, and involve size criteria, possible further imaging modalities, and tumor markers. Even with these tools at highly specialized expert centers, uncertainty in management remains, and recent literature has explored the use of newer biomarkers to aid in these cases. SUMMARY: Postchemotherapy residual masses in GCT can prove to be complicated cases to manage. Balancing survival with quality of life outcomes is important and requires a multidisciplinary team experienced in treating GCT.


Assuntos
Neoplasias Embrionárias de Células Germinativas/tratamento farmacológico , Neoplasias Embrionárias de Células Germinativas/patologia , Neoplasias Testiculares/tratamento farmacológico , Neoplasias Testiculares/patologia , Biomarcadores Tumorais/sangue , Humanos , Masculino , Neoplasia Residual/sangue , Neoplasia Residual/patologia , Neoplasia Residual/cirurgia , Neoplasias Embrionárias de Células Germinativas/sangue , Neoplasias Embrionárias de Células Germinativas/cirurgia , Seminoma/sangue , Seminoma/tratamento farmacológico , Seminoma/patologia , Seminoma/cirurgia , Neoplasias Testiculares/sangue , Neoplasias Testiculares/cirurgia
14.
Adv Exp Med Biol ; 1226: 111-121, 2020.
Artigo em Inglês | MEDLINE | ID: mdl-32030680

RESUMO

Testicular germ cell tumors (TGCTs) represent the most common neoplasia among young men. Management of TGCTs is an excellent example of curative outcomes in clinical oncology. The unique sensitivity to cisplatin-based chemotherapy regimens has led to establishing TGCTs as a "model of cancer cure." However, mechanisms and factors underlying pervasive growth of TGCTs are still poorly understood. It is suggested that unique cancer stem cell (CSC) niche exists in the testicular tumor microenvironment. CSC niche potentially contributes to the progression of germ cell tumors. Furthermore, rich infiltration of TGCTs with immune cells indicates involvement of immune system in biology of this cancer type. This review summarizes current knowledge regarding specific cancer microenvironment in TGCTs and discusses the role of cancer stem cells as well as immune mechanisms in these tumors.


Assuntos
Neoplasias Embrionárias de Células Germinativas/imunologia , Neoplasias Embrionárias de Células Germinativas/patologia , Células-Tronco Neoplásicas/citologia , Nicho de Células-Tronco , Neoplasias Testiculares/imunologia , Neoplasias Testiculares/patologia , Microambiente Tumoral/imunologia , Humanos , Masculino
15.
Urologe A ; 59(3): 278-283, 2020 Mar.
Artigo em Alemão | MEDLINE | ID: mdl-32020239

RESUMO

In prepubertal boys, testicular tumors are rare with an incidence between 2 and 5/million. In contrast to testicular tumors in adolescents and adults, more than 2/3 of these tumors are benign. Unfortunately, in Germany in most cases, only malignant tumors (usually yolk sac tumors) are reported to the study center (MAKEI IV and now V). Therefore, the incidence in Germany is unknown. Since the introduction of polychemotherapy in the 1970s, the prognosis of malignant testicular tumors has improved enormously and has become a curable disease, even in the case of recurrence. Today the orchiectomy, which was usually carried out in the past, appears to be no longer justified in most prepubertal boys due to the high incidence of benign tumors. It has been shown in various studies that organ-sparing surgery in germ cell tumors (epidermoid cysts, teratoma); gonadal stoma tumors (Sertoli, Leydig and granulosa cell tumors) and cystic lesions (intratesticular cysts and tubular ectasia of the rete testis) is reliable and safe. In cases with preoperative significantly increased AFP (caution: norm values not valid in the first year of life) and a clear testicular tumor in the ultrasound (yolk sac tumor) or if no testicular parenchyma is sonographically detectable, orchiectomy can still be carried out. Today orchiectomies in prepubertal boys should be an exception and the reasons for an orchiectomy must be well documented.


Assuntos
Neoplasias Embrionárias de Células Germinativas/cirurgia , Orquiectomia , Preservação de Órgãos , Neoplasias Testiculares/cirurgia , Adolescente , Biomarcadores Tumorais , Alemanha , Humanos , Masculino , Recidiva Local de Neoplasia , Neoplasias Embrionárias de Células Germinativas/patologia , Neoplasias Testiculares/patologia
16.
Anticancer Res ; 40(2): 759-766, 2020 Feb.
Artigo em Inglês | MEDLINE | ID: mdl-32014918

RESUMO

BACKGROUND/AIM: Yolk sac tumour (YST) is a rare malignant ovarian germ cell tumour that often occurs in young women or adolescents and exhibits an unfavourable outcome. To evaluate the biological behavior of carcinomas in vitro, permanent tumour cell lines are required. However, previously, only a few human YST cell lines have been established. Therefore, we aimed to establish a novel YST cell line. MATERIALS AND METHODS: We established a novel YST cell line, TC587, from an adolescent patient with ovarian YST. RESULTS: The cell line expressed AFP and SALL4, the characteristics of YST. In addition, we evaluated somatic mutations using next-generation sequencing and revealed some pathogenic variants, including mutations in the NRAS, KIT, KMT2C, RSF1, and TP53 genes. CONCLUSION: The newly established TC587 cell line may represent an effective tool for developing treatments and conducting molecular analyses for YST.


Assuntos
Tumor do Seio Endodérmico/diagnóstico , Neoplasias Embrionárias de Células Germinativas/diagnóstico , Neoplasias Ovarianas/diagnóstico , Linhagem Celular Tumoral , Criança , Tumor do Seio Endodérmico/patologia , Tumor do Seio Endodérmico/terapia , Feminino , Humanos , Neoplasias Embrionárias de Células Germinativas/patologia , Neoplasias Embrionárias de Células Germinativas/terapia , Neoplasias Ovarianas/patologia , Neoplasias Ovarianas/terapia
17.
World Neurosurg ; 137: e194-e207, 2020 05.
Artigo em Inglês | MEDLINE | ID: mdl-32001412

RESUMO

OBJECTIVE: The midline supracerebellar infratentorial (SCIT) approach and its paramedian development are commonly used for dealing with pineal lesions. Comparative clinical studies are lacking, however. We aim to establish the better performance of the paramedian SCIT approach in terms of clinical safety in surgically treated pineal cysts and pineal region tumors. Procedural functionality and effectiveness have been also analyzed. METHODS: A comparative analysis of clinical, radiologic, pathologic, and surgical features, and outcome was performed between 55 midline and 57 paramedian SCIT approaches that were exclusively performed in 112 patients (57 pineal cysts and 55 tumors of the pineal region) operated in sitting position by a single surgeon. Information was retrieved from hospital records and microsurgical videos. RESULTS: The paramedian SCIT approach linked with fewer postoperative complications (odds ratio [OR]: 0.40) and fewer approach-related complications (OR: 0.28) than the midline SCIT approach. The SCIT paramedian approach was achieved in a shorter time, by a smaller bone flap, and with fewer complex procedural steps than the midline approach. The SCIT paramedian approach did not require the opening of the falx cerebelli, midline cerebellar retraction, section of the midline cerebellar draining veins, nor wide opening of the dura. Gross total resection, size of the lesion, microsurgical time for removal, histopathological diagnosis and postoperative outcome were statistically similar in both groups. CONCLUSIONS: The SCIT approach represents a safer and more functional approach for the removal of cysts and tumors of the pineal region than the classic midline approach, while maintaining the same effectiveness.


Assuntos
Neoplasias Encefálicas/cirurgia , Cistos do Sistema Nervoso Central/cirurgia , Glioma/cirurgia , Microcirurgia/métodos , Neoplasias Embrionárias de Células Germinativas/cirurgia , Procedimentos Neurocirúrgicos/métodos , Glândula Pineal/cirurgia , Pinealoma/cirurgia , Adolescente , Adulto , Neoplasias Encefálicas/patologia , Cistos do Sistema Nervoso Central/patologia , Feminino , Glioma/patologia , Humanos , Masculino , Neoplasias Meníngeas/patologia , Neoplasias Meníngeas/cirurgia , Meningioma/patologia , Meningioma/cirurgia , Pessoa de Meia-Idade , Neoplasias Embrionárias de Células Germinativas/patologia , Pinealoma/patologia , Carga Tumoral , Adulto Jovem
18.
Pediatr Blood Cancer ; 67(4): e28169, 2020 04.
Artigo em Inglês | MEDLINE | ID: mdl-32020769

RESUMO

BACKGROUND: Disorders of sex development (DSD) are rare conditions. Although they are known to predispose to germ cell tumors (GCT), there is a paucity of information regarding the circumstances of DSD discovery. DESIGN/METHODS: All patients with DSD registered in two French pediatric GCT protocols (TGM95 and 13) were analyzed. RESULTS: Sixteen patients were identified among 276 ovarian, 160 testicular, and 24 mediastinal GCT. Eleven phenotypic females (median age 15 years) exhibited gonadal GCT, including 10 with a 46,XY karyotype and gonadal dysgenesis and one with 46XX,45X0 mosaicism. None had genital anomalies, seven had spontaneous pubertal changes, and one had spontaneous menarche. The tumors were bilateral in four cases. DSD was diagnosed after the GCT diagnosis in seven cases. The reasons for karyotyping were bilateral tumors (3), gonadoblastoma/streak gonad/absence of egg follicles (3), or systematic for GCT (1). The karyotyping was performed before the GCT diagnosis in four cases: for polymalformative syndrome (2) or primary amenorrhea (2). Four males (median age 14 years) exhibited mediastinal GCT (metastatic in two cases) indicative of Klinefelter syndrome, despite typical phenotypes in all cases. The remaining patient had severe hypospadias, leading to the discovery of 46,XY/45,X0 mosaicism before the diagnosis of testicular nonseminomatous GCT at 16 years of age. CONCLUSION: DSD are often uncovered at the time of GCT diagnosis (11/16 cases). This should prompt oncologists to rule out a DSD in patients with GCT, even in case of pubertal development. Earlier recognition of Klinefelter syndrome could potentially lead to GCT detection at an earlier stage.


Assuntos
Transtornos do Desenvolvimento Sexual , Neoplasias Embrionárias de Células Germinativas , Neoplasias Ovarianas , Neoplasias Testiculares , Adolescente , Criança , Transtornos do Desenvolvimento Sexual/diagnóstico , Transtornos do Desenvolvimento Sexual/etiologia , Transtornos do Desenvolvimento Sexual/patologia , Feminino , Humanos , Masculino , Neoplasias Embrionárias de Células Germinativas/complicações , Neoplasias Embrionárias de Células Germinativas/diagnóstico , Neoplasias Embrionárias de Células Germinativas/patologia , Neoplasias Ovarianas/complicações , Neoplasias Ovarianas/diagnóstico , Neoplasias Ovarianas/patologia , Neoplasias Testiculares/complicações , Neoplasias Testiculares/diagnóstico , Neoplasias Testiculares/patologia
19.
Eur J Cancer ; 127: 139-149, 2020 03.
Artigo em Inglês | MEDLINE | ID: mdl-32007714

RESUMO

BACKGROUND: Up to 50% of men with poor prognosis, non-seminoma germ cell tumours (GCTs) die with standard BEP (bleomycin, etoposide and cisplatin) chemotherapy. An intensive regimen, CBOP/BEP (carboplatin, bleomycin, vincristine and cisplatin/BEP), met response targets in a randomised, phase II trial (74% complete response or partial response marker negative, 90% confidence interval (CI) 61%-85%). AIM: To assess long-term outcomes and late toxicity associated with CBOP/BEP. METHODS: Patients with poor prognosis extracranial GCT were randomised to 4xBEP or CBOP/BEP (2xCBOP, 2xBO, 3xBEP with 15,000iu of bleomycin). Low-dose, stabilising chemotherapy before entry was permitted. Response rates (primary outcome) were reported previously. Here, we report secondary outcomes: progression-free survival (PFS), overall survival (OS) and late toxicity. Prognostic factors and the impact of marker decline are assessed in exploratory analysis. RESULTS: Eighty-nine patients (43 CBOP/BEP) were randomised. After median 63 months follow-up, 3-year PFS is 55.7% (95% CI: 39.7%, 69.0%) for CBOP/BEP and 38.7% (95% CI: 24.7%, 52.4%) for BEP (hazard ratio [HR]: 0.59 (0.33, 1.06), p = 0.079). Three-year OS is 65.0% (48.8%, 77.2%) and 58.5% (43.0%, 71.2%), respectively (HR: 0.79 (0.41, 1.52), p = 0.49). Twelve-month toxicity was affected by subsequent treatments, with no clear differences between arms. Stabilising chemotherapy was associated with poorer PFS (HR: 2.09 (1.14, 3.81), p = 0.017), whereas unfavourable marker decline, in 60 (70%) patients, was not. CONCLUSION: Although not powered for PFS, results for CBOP/BEP are promising. Impact on OS was less clear (and will be affected by subsequent therapy). Further study in an international phase III trial is warranted. TRIAL REGISTRATION: ISRCTN 53643604.


Assuntos
Protocolos de Quimioterapia Combinada Antineoplásica/uso terapêutico , Recidiva Local de Neoplasia/tratamento farmacológico , Neoplasias Embrionárias de Células Germinativas/tratamento farmacológico , Adolescente , Adulto , Bleomicina/administração & dosagem , Carboplatina/administração & dosagem , Cisplatino/administração & dosagem , Etoposídeo/administração & dosagem , Seguimentos , Humanos , Masculino , Pessoa de Meia-Idade , Recidiva Local de Neoplasia/patologia , Neoplasias Embrionárias de Células Germinativas/patologia , Prognóstico , Taxa de Sobrevida , Vincristina/administração & dosagem , Adulto Jovem
20.
J Urol ; 204(1): 96-103, 2020 Jul.
Artigo em Inglês | MEDLINE | ID: mdl-32003612

RESUMO

PURPOSE: We analyzed the oncologic outcomes of men undergoing primary retroperitoneal lymph node dissection and characterized the use of adjuvant chemotherapy and template dissections. MATERIALS AND METHODS: Retrospective review of the Indiana University testis cancer database identified patients who underwent primary retroperitoneal lymph node dissection between January 2007 and December 2017. Patients and providers were contacted to obtain information regarding adjuvant therapy, recurrence and survival. The primary outcome was recurrence-free survival. Kaplan-Meier curves assessed survival differences stratified by pathological stage, template of dissection and use of adjuvant chemotherapy. RESULTS: A total of 274 patients were included in the study. Most men presented with clinical stage I disease (214, 78%). A modified unilateral template was performed in 257 (94%) and bilateral template in 17 (6%). Overall 148 (54%) and 126 (46%) men had pathological stage (PS) I and PS-II disease, respectively. Thirteen patients (10%) with PS-II disease were treated with adjuvant chemotherapy. With a median followup of 55 months only 33 (12%) patients had recurrence. Of the 113 patients with PS-II disease who did not receive chemotherapy 21 (19%) had disease relapse and 81% were cured with surgery alone and never had recurrence. No difference in recurrence-free survival was noted between modified and bilateral template dissections. CONCLUSIONS: The use of adjuvant chemotherapy has been minimal during the last decade. The majority (81%) of men with PS-II disease were cured with retroperitoneal lymph node dissection alone and were able to avoid chemotherapy. Modified unilateral template dissection provided excellent oncologic control while minimizing morbidity.


Assuntos
Quimioterapia Adjuvante/estatística & dados numéricos , Excisão de Linfonodo , Espaço Retroperitoneal/cirurgia , Neoplasias Testiculares/patologia , Neoplasias Testiculares/terapia , Adulto , Seguimentos , Humanos , Masculino , Recidiva Local de Neoplasia , Neoplasias Embrionárias de Células Germinativas/mortalidade , Neoplasias Embrionárias de Células Germinativas/patologia , Neoplasias Embrionárias de Células Germinativas/terapia , Espaço Retroperitoneal/patologia , Estudos Retrospectivos , Seminoma/mortalidade , Seminoma/patologia , Seminoma/terapia , Teratoma/mortalidade , Teratoma/patologia , Teratoma/terapia , Neoplasias Testiculares/mortalidade
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