Your browser doesn't support javascript.
loading
Show: 20 | 50 | 100
Results 1 - 20 de 230
Filter
1.
Arch. argent. pediatr ; 119(2): e171-e175, abril 2021. ilus
Article in Spanish | LILACS, BINACIS | ID: biblio-1152122

ABSTRACT

Los tumores del sistema nervioso central representan la segunda enfermedad oncológica más habitual en niños y adolescentes. Entre los tumores intracraneales, los de células germinales son infrecuentes. Los síntomas que desencadenan son cefalea, náuseas, vómitos, déficits hormonales, alteraciones visuales, pérdida de peso, pobre crecimiento y pubertad precoz. Menos frecuentemente, producen trastornos del movimiento o psiquiátricos. Algunos de estos tumores pueden ser asintomáticos un largo período, lo que desencadena un diagnóstico tardío.Se presenta a una paciente femenina de 14 años con pérdida de peso y falla del crecimiento, con diagnóstico erróneo de trastorno de la conducta alimentaria. Tras estudios pertinentes, se arribó al diagnóstico de germinoma del sistema nervioso central. Al ser esta patología infrecuente y de presentación variable, requiere alto sentido de alerta por parte de la familia involucrada y del equipo de salud para evitar retrasos en el diagnóstico y el tratamiento


Central nervous system tumors are the second most frequent oncological disease among children and teenagers. Among the intracranial tumors, the germ cells ones are infrequent. The symptoms they cause are headaches, nausea and vomiting, hormonal deficits, visual disturbances, weight loss, poor growth and early puberty. Less frequently, they produce movement or psychiatric disorders. Some of these tumors can be asymptomatic for a long period leading to a late diagnosis.The case of a 14-year-old female patient is presented. She showed weight loss and growth failure, with wrong diagnosis of eating disorder. After proper study methods, we arrived to central nervous system germinoma diagnosis. Because this pathology is rare and has a variable form of presentation, it requires that the family involved and the health team to be alert, to avoid delays in diagnosis and treatment.


Subject(s)
Humans , Female , Adolescent , Germinoma/diagnostic imaging , Brain Neoplasms , Weight Loss , Germinoma/therapy , Failure to Thrive , Hypopituitarism
2.
Article in Portuguese | LILACS | ID: biblio-1359762

ABSTRACT

RESUMO: O hipopituitarismo é a deficiência de dois ou mais hormônios hipofisários, que se expressa por sintomas dependentes do tipo e grau de déficit hormonal. A adequada condução destes pacientes é de fundamental importância para que não acarrete atraso no crescimento e desenvolvimento, óbito ou mudanças na qualidade de vida dos indivíduos. Objetiva-se, neste estudo, relatar caso clínico de abordagem de paciente pediátrico com pan-hipopituitarismo e descrever o manejo adotado, bem como a importância do acompanhamento pelo endocrinologista pediátrico. Trata-se de paciente do sexo masculino, 14 anos, com pan-hipopituitarismo iniciado na infância, secundário ao surgimento de germinoma e ao tratamento realizado para o mesmo. A primeira deficiência hormonal apresentada foi diabetes insipidus, seguida, após a realização de quimioterapia e radioterapia, de múltiplas deficiências. O menor segue em acompanhamento especializado, e faz uso de levotiroxina, desmopressina, somatropina, testosterona e prednisolona. A partir do presente relato, percebe-se a importância do diagnóstico oportuno e da adequada abordagem da criança com pan-hipopituitarismo e de seu seguimento a fim de se manter uma qualidade de vida satisfatória. (AU)


ABSTRACT: Hypopituitarism is the deficiency of two or more pituitary hormones. Its symptoms depend on the type and degree of hormonal deficit. Proper care of these patients is of fundamental importance to avoid delay in growth and development, death, or changes in the quality of life. The objective of this study is to report a clinical case of a pediatric patient with pan-hypopituitarism and describe the care adopted, as well as the importance of monitoring by the pediatric endocrinologist. The patient was a 14-year-old boy, with pan-hypopituitarism beginning in childhood, secondary to the appearance of germinoma and the treatment performed for it. The first hormonal deficiency presented was diabetes insipidus, followed by multiple deficiencies after chemotherapy and radiotherapy. The patient is under specialized monitoring and takes levothyroxine, desmopressin, somatropin, testosterone, and prednisolone. From the present report, timely diagnosis and adequate approach to a child with pan-hypopituitarism and its follow-up are important to maintain a satisfactory quality of life. (AU)


Subject(s)
Humans , Male , Adolescent , Pituitary Hormones , Quality of Life , Germinoma , Failure to Thrive , Medication Therapy Management , Transitional Care , Hypopituitarism
3.
Article in English | WPRIM | ID: wpr-750409

ABSTRACT

@#Introduction: Intratumoral calcification is a feature that is more often observed in pineal parenchymal tumour than germinoma. We describe a 13-year-old male with pineal region germinoma demonstrating extensive intratumoral calcification. Case report: He presented with worsening headache that was associated with fatigue, nausea and vomiting. Radiologic examination revealed a multilobular mass in the pineal region with internal calcifications. Biopsy showed a pure germinoma with unusually extensive calcification. Discussion: Although a diagnosis may be suggested with a careful evaluation of imaging, there is no pathognomonic pattern. Thus, histologic verification is necessary for most pineal region masses.


Subject(s)
Germinoma
4.
Article in English | WPRIM | ID: wpr-765258

ABSTRACT

Intracranial germ cell tumors (iGCTs) are a heterogeneous group of tumors with peculiar characteristics clearly distinguished from other brain tumors of neuroepithelial origin. Diverse histology, similarity to gonadal GCT, predilection to one sex, and geographic difference in incidence all present enigmas and fascinating challenges. The treatment of iGCT has advanced for germinoma to date; thus, clinical attention has shifted from survival to long-term quality of life. However, for non-germinomatous GCT, current protocols provide only modest improvement and more innovative therapies are needed. Recently, next-generation sequencing studies have revealed the genomic landscape of iGCT. Novel mutations in the KIT-RAS-MAPK and AKT-MTOR pathways were identified. More importantly, methylation profiling revealed a new method to assess the pathogenesis of iGCT. Molecular research will unleash new knowledge on the origin of iGCT and solve the many mysteries that have lingered on this peculiar neoplasm for a long time.


Subject(s)
Brain Neoplasms , Germ Cells , Germinoma , Gonads , Incidence , Methods , Methylation , Neoplasms, Germ Cell and Embryonal , Quality of Life , Therapies, Investigational
5.
Article in English | WPRIM | ID: wpr-717502

ABSTRACT

Germinoma is the most common type of intracranial germ cell tumors (GCTs). Pineal gland and suprasellar region are the most frequent sites of central nervous system (CNS) involvement. Intracranial masses caused by Langerhans cell histiocytosis (LCH) mimics features of CNS GCTs. LCH frequently involve spine and is the most common cause of vertebra plana in children. A 15-year-old boy presented with progressing symptoms of polydipsia, polyuria, general headache, nausea and severe back pain. Brain MRI showed brain tumor with simultaneous involvement of suprasellar region and pineal gland. An excisional biopsy of suprasellar mass was done. The pathologic assessment confirmed the diagnosis of germinoma. Patient's treatment continued accordingly. A spine MRI, done due to persistent backache, showed a vertebra plana. We reevaluated the primary diagnosis suspecting LCH. Germinoma of CNS was confirmed and a biopsy of vertebral lesion resulted in hemangioma. Thus we report a case of CNS germinoma with co-occurrence of vertebra plana. We emphasized the importance of histopathologic diagnosis of pineal/suprasellar masses and primary investigation of other CNS regions including spine for possible metastasis or comorbidities.


Subject(s)
Adolescent , Back Pain , Biopsy , Brain , Brain Neoplasms , Central Nervous System , Child , Comorbidity , Diagnosis , Germinoma , Headache , Hemangioma , Histiocytosis, Langerhans-Cell , Humans , Magnetic Resonance Imaging , Male , Nausea , Neoplasm Metastasis , Neoplasms, Germ Cell and Embryonal , Pineal Gland , Polydipsia , Polyuria , Spine
6.
Korean Journal of Medicine ; : 404-408, 2018.
Article in Korean | WPRIM | ID: wpr-716216

ABSTRACT

A 32-year old male came to our hospital with chief complaint of paraplegia. He had symptom of radiating pain to right leg 3 months ago before paraplegic symptom appeared. Magnetic resonance (MR) imaging from outside hospital showed intramedullary mass involving from T8 to T10 level of spinal cord. According to the imaging result, tumor removal with total laminectomy was performed between T8 and T10 level in our hospital. Pathologic result was compatible with germinoma. Spine radiation (39.6 Gy/22 fx) from T7 to T12 level without chemotherapy was performed 3 weeks later since tumor removal. Follow-up MR imaging showed no recurrence without any distant metastasis. And our patient's neurologic symptom had been improved. According to this case, postoperative radiotherapy is thought to be effective to primary spinal germinoma.


Subject(s)
Drug Therapy , Follow-Up Studies , Germinoma , Humans , Laminectomy , Leg , Magnetic Resonance Imaging , Male , Neoplasm Metastasis , Neurologic Manifestations , Paraplegia , Radiotherapy , Recurrence , Spinal Cord , Spine
7.
Article in English | WPRIM | ID: wpr-788688

ABSTRACT

Intracranial germ cell tumors (iGCTs) are a heterogeneous group of tumors with peculiar characteristics clearly distinguished from other brain tumors of neuroepithelial origin. Diverse histology, similarity to gonadal GCT, predilection to one sex, and geographic difference in incidence all present enigmas and fascinating challenges. The treatment of iGCT has advanced for germinoma to date; thus, clinical attention has shifted from survival to long-term quality of life. However, for non-germinomatous GCT, current protocols provide only modest improvement and more innovative therapies are needed. Recently, next-generation sequencing studies have revealed the genomic landscape of iGCT. Novel mutations in the KIT-RAS-MAPK and AKT-MTOR pathways were identified. More importantly, methylation profiling revealed a new method to assess the pathogenesis of iGCT. Molecular research will unleash new knowledge on the origin of iGCT and solve the many mysteries that have lingered on this peculiar neoplasm for a long time.


Subject(s)
Brain Neoplasms , Germ Cells , Germinoma , Gonads , Incidence , Methods , Methylation , Neoplasms, Germ Cell and Embryonal , Quality of Life , Therapies, Investigational
8.
Article in English | WPRIM | ID: wpr-63854

ABSTRACT

PURPOSE: Malignant 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 METHODS: We 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. RESULTS: The 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%. CONCLUSION: The 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.


Subject(s)
Asian Continental Ancestry Group , Central Nervous System , Germ Cells , Germinoma , Humans , Incidence , Japan , Korea , Neoplasms, Germ Cell and Embryonal , Survival Rate , United States
9.
Article in English | WPRIM | ID: wpr-132134

ABSTRACT

Langerhans cell histiocytosis (LCH) has diverse clinical manifestations, including intracranial mass lesions. We report a case of LCH that manifested as a suprasellar mass, and initially misdiagnosed as a germ cell tumor. A 29-year-old woman presented with polyuria, polydipsia and amenorrhea. Laboratory findings revealed hypopituitarism with central diabetes insipidus, and a suprasellar mass and a pineal mass were observed on magnetic resonance imaging. Under the clinical impression of a germ cell tumor, the patient was treated with germ cell tumor chemotherapy (cisplatin and etoposide) and radiation therapy without biopsy. After initial shrinkage of the lesions, further growth of the tumor was observed and a biopsy was performed. The histopathology revealed LCH. After chemotherapy according to the LCH III protocol, the tumor disappeared. She is on regular follow up for 5 years without relapse. The present findings indicate that LCH should be included in the differential diagnosis of a suprasellar mass, even in adults, especially when it manifests with diabetes insipidus. This case also underscores the importance of a histopathologic diagnosis in patients with suprasellar tumors before the initiation of a specific therapy, even if the clinical findings are highly suggestive of a specific diagnosis.


Subject(s)
Adult , Amenorrhea , Biopsy , Central Nervous System Neoplasms , Diabetes Insipidus , Diabetes Insipidus, Neurogenic , Diagnosis , Diagnosis, Differential , Drug Therapy , Female , Follow-Up Studies , Germinoma , Histiocytosis, Langerhans-Cell , Humans , Hypopituitarism , Magnetic Resonance Imaging , Neoplasms, Germ Cell and Embryonal , Polydipsia , Polyuria , Recurrence , Sella Turcica
10.
Article in English | WPRIM | ID: wpr-132131

ABSTRACT

Langerhans cell histiocytosis (LCH) has diverse clinical manifestations, including intracranial mass lesions. We report a case of LCH that manifested as a suprasellar mass, and initially misdiagnosed as a germ cell tumor. A 29-year-old woman presented with polyuria, polydipsia and amenorrhea. Laboratory findings revealed hypopituitarism with central diabetes insipidus, and a suprasellar mass and a pineal mass were observed on magnetic resonance imaging. Under the clinical impression of a germ cell tumor, the patient was treated with germ cell tumor chemotherapy (cisplatin and etoposide) and radiation therapy without biopsy. After initial shrinkage of the lesions, further growth of the tumor was observed and a biopsy was performed. The histopathology revealed LCH. After chemotherapy according to the LCH III protocol, the tumor disappeared. She is on regular follow up for 5 years without relapse. The present findings indicate that LCH should be included in the differential diagnosis of a suprasellar mass, even in adults, especially when it manifests with diabetes insipidus. This case also underscores the importance of a histopathologic diagnosis in patients with suprasellar tumors before the initiation of a specific therapy, even if the clinical findings are highly suggestive of a specific diagnosis.


Subject(s)
Adult , Amenorrhea , Biopsy , Central Nervous System Neoplasms , Diabetes Insipidus , Diabetes Insipidus, Neurogenic , Diagnosis , Diagnosis, Differential , Drug Therapy , Female , Follow-Up Studies , Germinoma , Histiocytosis, Langerhans-Cell , Humans , Hypopituitarism , Magnetic Resonance Imaging , Neoplasms, Germ Cell and Embryonal , Polydipsia , Polyuria , Recurrence , Sella Turcica
11.
Article in Korean | WPRIM | ID: wpr-788571

ABSTRACT

Central nervous system germ cell tumor is a rare but important tumor in childhood brain tumors. It requires a multidisciplinary approach to increase survival and promote quality of life, and all three treatment modalities including surgery, radiotherapy and chemotherapy has its own distinct role for germ cell tumor. For germinoma, radiotherapy alone can cure the disease but, the effort to limit the long term toxicity and the proper combination of chemotherapy and radiotherapy are under investigation. Craniospinal irradiation is reserved only for the disseminated germinoma or nongerminomatous germ cell tumor (NGGCT). For germinoma, craniospinal irradiation of 20 to 24 Gy is sufficient to control microscopic disease in the spinal axis. Chemotherapy and radiotherapy composed of 30 to 40 Gy of local field radiotherapy and 20 to 24 Gy of whole ventricular irradiation are required for localized germinoma, but the proper combination of two modalities has yet to be defined. For NGGCT, both the chemotherapy and radiotherapy should be performed, and survival rate is substantially increasing with modern treatment protocols. The omission of craniospinal irradiation is being tried for the localized NGGCT in international cooperative group trials. Surgery has its role for the resection of residual disease after the treatment, and the extent of resection in NGGCT has the prognostic implication. Bifocal germ cell tumors and basal ganglia germ cell tumor have distinctive clinical course and mandate special attention. To advance clinical and biological perspectives in central nervous germ cell tumor, the cooperation and communication of the multidisciplinary specialists are essential.


Subject(s)
Axis, Cervical Vertebra , Basal Ganglia , Brain Neoplasms , Central Nervous System , Clinical Protocols , Craniospinal Irradiation , Drug Therapy , Germ Cells , Germinoma , Neoplasms, Germ Cell and Embryonal , Quality of Life , Radiotherapy , Specialization , Survival Rate
12.
Article in Korean | WPRIM | ID: wpr-15189

ABSTRACT

Central nervous system germ cell tumor is a rare but important tumor in childhood brain tumors. It requires a multidisciplinary approach to increase survival and promote quality of life, and all three treatment modalities including surgery, radiotherapy and chemotherapy has its own distinct role for germ cell tumor. For germinoma, radiotherapy alone can cure the disease but, the effort to limit the long term toxicity and the proper combination of chemotherapy and radiotherapy are under investigation. Craniospinal irradiation is reserved only for the disseminated germinoma or nongerminomatous germ cell tumor (NGGCT). For germinoma, craniospinal irradiation of 20 to 24 Gy is sufficient to control microscopic disease in the spinal axis. Chemotherapy and radiotherapy composed of 30 to 40 Gy of local field radiotherapy and 20 to 24 Gy of whole ventricular irradiation are required for localized germinoma, but the proper combination of two modalities has yet to be defined. For NGGCT, both the chemotherapy and radiotherapy should be performed, and survival rate is substantially increasing with modern treatment protocols. The omission of craniospinal irradiation is being tried for the localized NGGCT in international cooperative group trials. Surgery has its role for the resection of residual disease after the treatment, and the extent of resection in NGGCT has the prognostic implication. Bifocal germ cell tumors and basal ganglia germ cell tumor have distinctive clinical course and mandate special attention. To advance clinical and biological perspectives in central nervous germ cell tumor, the cooperation and communication of the multidisciplinary specialists are essential.


Subject(s)
Axis, Cervical Vertebra , Basal Ganglia , Brain Neoplasms , Central Nervous System , Clinical Protocols , Craniospinal Irradiation , Drug Therapy , Germ Cells , Germinoma , Neoplasms, Germ Cell and Embryonal , Quality of Life , Radiotherapy , Specialization , Survival Rate
13.
Article in Korean | WPRIM | ID: wpr-788540

ABSTRACT

Germ cell tumors the designation given to neoplasm arising from the cells of the germline, the cells that are destined to become either the egg or the sperm. These tumors have a number of unique features that includes bimodal and wide age distribution, remarkable phenotypic diversity, and varying biologic behavior. During infancy, sacrococcygeal locations predominate with either teratomas in neonates or endodermal sinus tumors in infants above three months. After puberty, non-germinomatous germ cell tumors predominate with gonadal, mediastinal or intracranial tumor. Specific subtypes of germ cell tumors secrete proteins as tumor markers. Surgical resection of the tumor is necessary to establish the diagnosis and for staging of the extent of tumor spread. Except for teratoma, germ cell tumors are highly sensitive to chemotherapy in particular cisplatin. The most commonly used chemotherapy regimen for malignant germ cell tumors is PEB (cisplatin, etoposide and bleomycin). Prognosis is good even in metastatic diseases. Patients with relapsed or recurrent disease may be candidates for high dose chemotherapy and autologous hematopoietic stem cell transplantation.


Subject(s)
Adolescent , Age Distribution , Choriocarcinoma , Cisplatin , Diagnosis , Drug Therapy , Endodermal Sinus Tumor , Etoposide , Female , Germinoma , Gonads , Hematopoietic Stem Cell Transplantation , Humans , Infant , Infant, Newborn , Neoplasms, Germ Cell and Embryonal , Ovum , Pediatrics , Pregnancy , Prognosis , Puberty , Spermatozoa , Teratoma , Biomarkers, Tumor
14.
Article in English | WPRIM | ID: wpr-83802

ABSTRACT

Intracranial germ cell tumors (ICGCT) occur in 2-11% of children with brain tumors between 0-19 years of age. For treatment of germinoma, relatively low radiation doses with or without chemotherapy show excellent 10 year survival rate of 80-100%. Past studies showed that neoadjuvant chemotherapy combined with focal radiotherapy resulted in unacceptably high rates of periventricular tumor recurrence. The use of generous radiation volume which covers the whole ventricular space with later boost treatment to primary site is considered as standard treatment of intracranial germinomas. For non-germinomatous germ cell tumors (NGGCT), 10-year overall survival rate is still much inferior than that of intracranial germinoma despite intensive chemotherapy and high-dose radiotherapy. Craniospinal radiotherapy combined with cisplatin-based chemotherapy provides the best treatment outcome for NGGCT; 60-70% of overall survival rate. There is a debate on the surgical role whether surgery can contribute to improved treatment outcome of NGGCT when added to combined chemoradiotherapy. Because higher dose of radiotherapy is required for treatment of NGGCT than for germinoma, it is tested whether whole ventricular irradiation can replace craniospinal irradiation in intermediate risk group of NGGCT to minimize radiation-related late toxicity in the recent studies. To minimize the treatment-related neural deficit and late sequelae while maintaining long-term survival rate of ICGCT patients, optimized administration of chemotherapy and radiotherapy should be selected. Use of technically upgraded radiotherapy modalities such as intensity-modulated radiotherapy or proton beam therapy is expected to bring an improved neurocognitive outcome with longitudinal assessment of the patients.


Subject(s)
Biology , Brain Neoplasms , Chemoradiotherapy , Child , Craniospinal Irradiation , Drug Therapy , Germinoma , Humans , Neoplasms, Germ Cell and Embryonal , Proton Therapy , Radiotherapy , Radiotherapy, Intensity-Modulated , Recurrence , Survival Rate , Treatment Outcome
15.
Article in Korean | WPRIM | ID: wpr-13546

ABSTRACT

Germ cell tumors the designation given to neoplasm arising from the cells of the germline, the cells that are destined to become either the egg or the sperm. These tumors have a number of unique features that includes bimodal and wide age distribution, remarkable phenotypic diversity, and varying biologic behavior. During infancy, sacrococcygeal locations predominate with either teratomas in neonates or endodermal sinus tumors in infants above three months. After puberty, non-germinomatous germ cell tumors predominate with gonadal, mediastinal or intracranial tumor. Specific subtypes of germ cell tumors secrete proteins as tumor markers. Surgical resection of the tumor is necessary to establish the diagnosis and for staging of the extent of tumor spread. Except for teratoma, germ cell tumors are highly sensitive to chemotherapy in particular cisplatin. The most commonly used chemotherapy regimen for malignant germ cell tumors is PEB (cisplatin, etoposide and bleomycin). Prognosis is good even in metastatic diseases. Patients with relapsed or recurrent disease may be candidates for high dose chemotherapy and autologous hematopoietic stem cell transplantation.


Subject(s)
Adolescent , Age Distribution , Choriocarcinoma , Cisplatin , Diagnosis , Drug Therapy , Endodermal Sinus Tumor , Etoposide , Female , Germinoma , Gonads , Hematopoietic Stem Cell Transplantation , Humans , Infant , Infant, Newborn , Neoplasms, Germ Cell and Embryonal , Ovum , Pediatrics , Pregnancy , Prognosis , Puberty , Spermatozoa , Teratoma , Biomarkers, Tumor
16.
Arq. bras. endocrinol. metab ; 57(7): 566-570, out. 2013. ilus, tab
Article in English | LILACS | ID: lil-690597

ABSTRACT

Intracranial germinomas (GE) are malignant neoplasms most commonly found in the suprasellar region, which may cause anterior and particularly posterior pituitary hormone deficits with central diabetes insipidus (DI). Differential diagnosis of pituitary stalk thickening includes granulomatous, inflammatory, infectious, and neoplastic lesions. Although careful analysis of clinical, laboratory, and imaging findings may facilitate the diagnosis, transsphenoidal biopsy is indicated to confirm the disease, as the correct diagnosis directs the appropriate treatment.


Germinomas intracranianos (GE) são neoplasias malignas comumente na região suprasselar, podendo causar deficiência hormonal da hipófise anterior, em particular da hipófise posterior, com diabetes insípido central (DI). Entre os diagnósticos diferenciais do espessamento de haste hipofisária, incluem-se doenças granulomatosas, inflamatórias, infecciosas e neoplásicas. Embora as avaliações clínica, laboratorial e a ressonância magnética selar sugiram o diagnóstico, a biópsia transesfenoidal está indicada para confirmação, visto que o diagnóstico correto direciona o tratamento.


Subject(s)
Adult , Female , Humans , Brain Neoplasms/pathology , Germinoma/pathology , Hypopituitarism/pathology , Pituitary Gland/pathology , Biomarkers, Tumor/analysis , Biopsy , Hypopituitarism/etiology , Pituitary Hormones
17.
Chinese Journal of Pediatrics ; (12): 288-294, 2013.
Article in Chinese | WPRIM | ID: wpr-359753

ABSTRACT

<p><b>OBJECTIVE</b>To examine the recent incidences and trends of childhood malignant solid tumors in Shanghai.</p><p><b>METHOD</b>Data from the population-based Shanghai Cancer Registry and related retrospective survey were used to analyze the patterns of incidence and trends of malignant solid tumors diagnosed between 2002 and 2010 in children aged 0-14 years. The distributions of incidences were described according to gender, age and cancer types which were classified according to International Classification of Childhood Cancer (ICCC). Annual age-standardized rates (ASRs) were adjusted by the world standard population. Approximate confidence intervals for standardized rate ratios (SRR) based Poisson distribution test-based methods were used to assess changes in incidence over the period 2002 - 2006 and 2007 - 2010.</p><p><b>RESULT</b>(1)A total of 868 cases of childhood malignant solid tumors were diagnosed in Shanghai during 2002 - 2010, accounting for 65.8% of all childhood cancers. The ASR of 2002 - 2010 was 80.2 per million for all solid tumors. (2) The ASR was higher in boys (86.3 per million) than in girls (73.8 per million) with SRR 1.2 (95%CI 1.0 - 1.3). Incidence rate was the highest in the first five years of life with 93.4 per million. The age-specific incidence rates in 5 - 9 and 10 - 14 age groups were 65.2 and 79.3 per million, respectively. (3) CNS tumors, lymphomas, germ cell tumors, neuroblastoma, and soft tissue sarcomas were the top 5 most common solid tumors in children, with the incidence rate of 23.8, 11.0, 7.8, 7.7 and 6.8 per million, respectively. The patterns of subgroups varied in different age groups. Blastomas, such as neuroblastoma, retinoblastoma, were more common in the children aged 0 - 4 years, whereas epithelial carcinomas and bone tumors developed more frequently in elder children aged 10 - 14 years. (4) Compared with the ASR in 2002 - 2006, the ASR for both genders in 2007 - 2010 had no substantial changes (78.7 per million in 2002 - 2006 and 82.9 per million in 2007 - 2010). However, among boys, the incidence rate in 2007 - 2010 was significantly higher than that in 2002 - 2006 with SRR 1.2 (95%CI: 1.0 - 1.4). For specific subgroups of cancer, there were no substantial changes. Some cautions should be taken when interpreting results involving a small number of cases per year and those with wide 95% confidence intervals.</p><p><b>CONCLUSION</b>The incidence rate of pediatric malignant solid tumors among males was higher than females during 2002 - 2010, and it differed among different age groups with the highest in the first five years of life. CNS tumor was the most common type of solid tumors in children. This was a unique characteristics comparing with adult reflected in disease spectrum and age of onset. The patterns of incidence and its trends for childhood malignant solid tumors in Shanghai could provide a basis for etiologic research and preventive interventions. The findings also suggest an urgent need for longer population-based surveillance to verify the pattern and changing trends.</p>


Subject(s)
Adolescent , Age Distribution , Central Nervous System Neoplasms , Epidemiology , Pathology , Child , Child, Preschool , China , Epidemiology , Female , Germinoma , Epidemiology , Pathology , Humans , Incidence , Infant , Lymphoma , Epidemiology , Pathology , Male , Neoplasm Staging , Neoplasms , Classification , Epidemiology , Pathology , Registries , Risk Factors , Sex Distribution , Time Factors , Urban Population
18.
Article in Chinese | WPRIM | ID: wpr-814889

ABSTRACT

OBJECTIVE@#To report the prospective efficacy of 45 patients intracranial germinoma treated by radiotherapy and discuss its treatment.@*METHODS@#From February 1998 to October 2007, a total of 45 intracranial germinoma patients were performed radiotherapy, including 15 combined chemotherapy in the Department of Oncology. Of them 23 were pathologically diagnosed while 22 cases were clinical diagnosed. Life table method showed the 5-year and 10-year survival rate.@*RESULTS@#Forty patients were followed-up. Most symptoms of the patients were significantly reduced or disappeared completely. The 5-year and 10-year survival rate of all patients were 84% and 74%.@*CONCLUSION@#Radiotherapy is the main treatment for intracranial germinoma. Craniospinal irradiation, whole brain irradiation and partial brain irradiation are the main treatments. Radiotherapy combined with chemotherapy, which can reduce the radiation range and dose will be the trend.


Subject(s)
Adolescent , Adult , Antineoplastic Agents , Therapeutic Uses , Brain Neoplasms , Drug Therapy , Mortality , Radiotherapy , Child , Female , Follow-Up Studies , Germinoma , Drug Therapy , Radiotherapy , Humans , Male , Young Adult
19.
Indian J Hum Genet ; 2012 Jan; 18(1): 119-121
Article in English | IMSEAR | ID: sea-139457

ABSTRACT

Familial testicular germ cell tumors are well known in literature. Only few cases are reported where both brother and sister of the same family suffered from germ cell malignancies. We present a family where the proband is a survivor of ovarian dysgerminoma stage IA. Her elder male sibling became acutely ill and was detected to have disseminated testicular malignancy with grossly elevated markers and vegetations in the mitral valve leaflets. Despite all measures he could not be saved. Presence of germ cell malignancies in the siblings of different sex in the same family points toward a genetic susceptibility. Literature review revealed only six similar cases. A discussion regarding the rare occurrence of familial germ cell malignancies with the affected family members may be worthwhile.


Subject(s)
Adolescent , Dysgerminoma/etiology , Dysgerminoma/genetics , Female , Family , Germinoma/etiology , Germinoma/genetics , Humans , Male , Neoplasms, Germ Cell and Embryonal/etiology , Neoplasms, Germ Cell and Embryonal/genetics , Siblings , Testicular Neoplasms/etiology , Testicular Neoplasms/genetics
20.
Chinese Medical Journal ; (24): 2790-2793, 2012.
Article in English | WPRIM | ID: wpr-244350

ABSTRACT

Intracranial germinomas are malignant neoplasms of gonadal origin, which have some features in terms of age, sex, and clinical manifestations. They mainly occur in children and adolescents. Patients with intracranial germinomas mainly manifest with hypothalamic pituitary axis dysfunction and/or compression syndromes. Visual disturbance is one of the most significant clinical presentations, which is mainly caused by tumor cell infiltration into the optic pathway. In this article, we present three cases of patient with intracranial germinoma to analyze the ocular manifestations. All the three patients presented with endocrine symptoms in the early stage and with visual disturbances (including decreased visual acuity and visual field defects) later. In general, germinoma is diagnosed by its characteristic radiological appearance, supported by tumor markers and/or stereotactic biopsy. However, decisive diagnoses were established when ocular manifestations were presented. A suspicion for germinoma should be considered, when young patients manifest visual disturbances accompanied by endocrine symptoms.


Subject(s)
Adolescent , Adult , Brain Neoplasms , Diagnosis , Female , Germinoma , Diagnosis , Humans , Male , Middle Aged , Vision Disorders , Young Adult
SELECTION OF CITATIONS
SEARCH DETAIL