RESUMO
El tumor neuroectodérmico primitivo periférico (PPNET) o neuroepitelioma periférico es una neoplasia maligna de células redondas azules pequeñas derivadas de la cresta neural. Es uno de los tipos histológicos más indiferenciados entre los tumores malignos. Por ser indistinguible del sarcoma de Ewing, desde el punto de vista de imagen e histología, se considera una única patología para su estudio. Puede aparecer en la infancia o más frecuente en la adolescencia; el tratamiento incluye quimioterapia, cirugía y radioterapia. Se presenta el caso clínico de un paciente de 77 años de edad que consulta por ptosis palpebral y es catalogado como Síndrome de Horner. El diagnóstico final fue tumor neuroectodérmico periférico confirmado con histopatología e inmunohistoquímica
A primitive neuroectodermal tumor (PPNET) or peripheral neuroepithelioma is a malignant neoplasm of small round blue cells derived from the neural crest. It is one of the most undifferentiated histological types among malignant tumors, because it is indistinguishable from Ewing's sarcoma from the imaging and histology perspective. It considers a single pathology for this paper. It can appear in childhood or more frequently in adolescence; treatment includes chemotherapy, surgery, and radiation therapy. We present the clinical case of a 77 year-old patient who consulted for palpebral ptosis, and it classifies as Horner's Syndrome. The final diagnosis was a peripheral neuroectodermal tumorconfirmed with histopathology and immunohistochemistry.
Assuntos
Humanos , Masculino , Idoso , Síndrome de Horner , Tumores Neuroectodérmicos Primitivos Periféricos , Tumores Neuroectodérmicos Primitivos Periféricos/diagnóstico , Blefaroptose , Acuidade Visual , Tumores Neuroectodérmicos Primitivos Periféricos/cirurgia , Diagnóstico DiferencialAssuntos
Humanos , Masculino , Adolescente , Tórax/diagnóstico por imagem , Diagnóstico por Imagem/estatística & dados numéricos , Tumores Neuroectodérmicos Primitivos Periféricos/diagnóstico , Tumores Neuroectodérmicos Primitivos Periféricos/cirurgia , Tumores Neuroectodérmicos Primitivos Periféricos/terapia , BiópsiaRESUMO
Seminal vesicle tumor is a rare disease with unclear origin. Generally, it is presented as a pelvic mass that can be detected by sonography and digital rectal exam. The authors report a 25-year-old patient with a pelvic mass which the magnetic resonance and surgical specimen reveal a seminal vesicle tumor. Immunohistochemical findings favored a primitive neuroectodermal tumor of the seminal vesicle. Herein, the treatment, histological and histochemical findings of this entity are discussed.
Assuntos
Neoplasias dos Genitais Masculinos/patologia , Tumores Neuroectodérmicos Primitivos Periféricos/patologia , Glândulas Seminais/patologia , Adulto , Protocolos de Quimioterapia Combinada Antineoplásica/uso terapêutico , Quimioterapia Adjuvante , Seguimentos , Neoplasias dos Genitais Masculinos/tratamento farmacológico , Neoplasias dos Genitais Masculinos/cirurgia , Humanos , Imuno-Histoquímica , Imageamento por Ressonância Magnética , Masculino , Tumores Neuroectodérmicos Primitivos Periféricos/tratamento farmacológico , Tumores Neuroectodérmicos Primitivos Periféricos/cirurgia , Glândulas Seminais/cirurgia , Resultado do TratamentoRESUMO
Seminal vesicle tumor is a rare disease with unclear origin. Generally, it is presented as a pelvic mass that can be detected by sonography and digital rectal exam. The authors report a 25-year-old patient with a pelvic mass which the magnetic resonance and surgical specimen reveal a seminal vesicle tumor. Immunohistochemical findings favored a primitive neuroectodermal tumor of the seminal vesicle. Herein, the treatment, histological and histochemical findings of this entity are discussed.
Assuntos
Humanos , Masculino , Adulto , Neoplasias dos Genitais Masculinos/diagnóstico , Tumores Neuroectodérmicos Primitivos Periféricos/diagnóstico , Glândulas Seminais/patologia , Protocolos de Quimioterapia Combinada Antineoplásica , Quimioterapia Adjuvante , Seguimentos , Neoplasias dos Genitais Masculinos/tratamento farmacológico , Neoplasias dos Genitais Masculinos/cirurgia , Imuno-Histoquímica , Imageamento por Ressonância Magnética , Tumores Neuroectodérmicos Primitivos Periféricos/tratamento farmacológico , Tumores Neuroectodérmicos Primitivos Periféricos/cirurgia , Glândulas Seminais/cirurgia , Resultado do TratamentoRESUMO
Os autores relatam um caso de meduloepitelioma teratóide maligno intra-ocular acometendo uma menina de 7 anos com história de glaucoma congênito. O padrão histológico demonstrou ilhas de epitélio primitivo, com freqüentes estruturas tubulares e focos de cartilagem. Após procedimento cirúrgico, a paciente encontra-se bem, sem evidência de recorrência tumoral
Assuntos
Humanos , Feminino , Criança , Corpo Ciliar , Exoftalmia , Enucleação Ocular , Glaucoma , Esclera , Tumores Neuroectodérmicos Primitivos Periféricos/cirurgia , Tumores Neuroectodérmicos Primitivos Periféricos/diagnóstico , Tumores Neuroectodérmicos Primitivos Periféricos/patologia , Neoplasias Uveais , Acuidade Visual , TomografiaRESUMO
The dysembrioplastic neuroepithelial tumor (DNT) is a benign and slow-growing temporal lobe cortical lesion that presents with clinically unresponsive partial and complex seizures, with onset before age 20 yr. DNT shows a multinodular architecture and the presence of mixed glioneural elements. The authors report on a case of DNT diagnosed by the intraoperative smear cytological technique. We describe the cytological characteristics of smear preparations of DNT, with emphasis on the differential diagnosis. The authors present a 5-yr-old male with partial seizures for the preceding 3 yr. The patient showed no other neurological deficits. Magnetic resonance identified a lesion in the cortical area of the right temporal lobe. The smear cytology showed small, round, oligodendroglial-like cells arranged in lobules, with microcystic spaces containing ganglion cells floating in a fine fibrillary background. Histology confirmed the diagnosis of DNT. This report shows that the smear preparation can be trustworthy for the intraoperative diagnosis of DNTs, helping to determine the appropriate neurosurgical procedure and thus avoiding extensive and unnecessary resections.
Assuntos
Neoplasias Encefálicas/patologia , Tumores Neuroectodérmicos Primitivos Periféricos/patologia , Astrocitoma/patologia , Neoplasias Encefálicas/cirurgia , Pré-Escolar , Citodiagnóstico , Diagnóstico Diferencial , Ganglioglioma/patologia , Humanos , Masculino , Tumores Neuroectodérmicos Primitivos Periféricos/cirurgia , Oligodendroglioma/patologia , Convulsões/etiologia , Resultado do TratamentoRESUMO
Os autores relatam caso de tumor neuroectodérmico primitivo periférico originado de ramo do nervo ciático, em paciente de 17 anos, do sexo feminino. O tumor foi extirpado cirurgicamente e o tratamento foi complementado com rádio e quimioterapia. A lesão apresentava tanto um aspecto histológico característico, com células pequenas e arredondadas formando pseudo-rosetas, como um perfil imunoistoquímico compatível. Os tumores neuroectodérmicos primitivos de nervos periféricos são lesões raras, descritas em 36 casos da literatura. Neste relato são discutidas as principais características dessa lesão e enfatizada a importância de um tratamento cirúrgico agressivo associado à terapêutica adjuvante apropriada.
Assuntos
Humanos , Feminino , Adolescente , Tumores Neuroectodérmicos Primitivos Periféricos/cirurgia , Tumores Neuroectodérmicos Primitivos Periféricos/terapiaRESUMO
A retrospective historical analysis of patients under 18 years of age with the histopathological diagnosis of infratentorial primitive neuroectodermal tumor (PNET) is presented. The survey embraced two different groups of children. Group 1 was defined as those patients treated from 1972 to 1984 with surgical resection plus neuraxis radiotherapy alone. Group 2 was made up of children treated from 1990 to 1996 with the same approach but with the addition of adjuvant chemotherapy: cisplatin (day 1) and etoposide (days 1-3) every 3 weeks for 6 months. Group 1 embraced 42 children with an age range of 1-16 years (mean 6 years, SD 4.4 years). In group 2 there were 34 children, their ages ranging from 1 to 18 years (mean 7.2, SD 4.6 years). The prevalence of stages T2M0 and T3M0 was similar in both groups, but in group 1 there were 4 patients (9.5%) whose spinal fluid was positive for tumor cells (M1), while in group 2 there were 7 children (20.5%) with positive spinal fluid. There was an unequivocal initial response to treatment in 86% of these children in group 1 and in 79% in group 2. The event-free survival (EFS) was 30% at 252 months in group 1, while for group 2 the EFS was 67.6% at 63 months (P 0.002). Mortality from tumor activity was noted in 26 patients (70%) in group 1, while in group 2 mortality attributable to tumor progression was documented in 11 children (32%). We conclude that the use of adjuvant chemotherapy in these patients improves survival without any significant morbidity.
Assuntos
Protocolos de Quimioterapia Combinada Antineoplásica/uso terapêutico , Neoplasias Cerebelares/radioterapia , Neoplasias Cerebelares/cirurgia , Tumores Neuroectodérmicos Primitivos Periféricos/radioterapia , Tumores Neuroectodérmicos Primitivos Periféricos/cirurgia , Adolescente , Adulto , Antineoplásicos/administração & dosagem , Antineoplásicos/uso terapêutico , Antineoplásicos Fitogênicos/administração & dosagem , Antineoplásicos Fitogênicos/uso terapêutico , Neoplasias Cerebelares/patologia , Criança , Pré-Escolar , Cisplatino/administração & dosagem , Cisplatino/uso terapêutico , Terapia Combinada , Etoposídeo/efeitos adversos , Etoposídeo/uso terapêutico , Feminino , Humanos , Lactente , Masculino , Estadiamento de Neoplasias , Tumores Neuroectodérmicos Primitivos Periféricos/patologia , Estudos Retrospectivos , Análise de Sobrevida , Resultado do TratamentoRESUMO
Overtreatment by radiotherapy and/or chemotherapy for central nervous system tumors in infancy and childhood may be deleterious, so the recognition of surgically curable clinicopathological entities is mandatory. The dysembryoplastic neuroepithelial tumor is a complex multinodular lesion consisting of glial nodules, associated with a specific glioneuronal element and/or with focal cortical dysplasia, and occurring in young patients presenting with intractable, mostly complex partial, seizures without neurological deterioration. We report on 14 patients; 9 were from a series of 600 pediatric patients with intracranial central nervous system tumors studied at a single institution from 1988 to 1993, and 5 were referred from other pediatric hospitals. Six tumors were frontal, six were temporal, one was parietal, and one was occipitoparietal. Computed tomographic scans disclosed hypodense lesions with cystic appearances in 4 patients and slight focal postcontrast enhancements in only 2 patients, whereas magnetic resonance imaging, available for 7 of 14 patients, showed hypointense lesions in T1-weighted images and hyperintense lesions in T2-weighted images. Deformities of the overlying cranium were also observed in five patients. The age range at the time of surgery (excluding a 20-year-old male patient who underwent surgery at the main pediatric hospital) was 2.6 to 13 years, with a mean of 6.68 years. The male to female patient ratio was 10:4, and the duration of symptoms was 0.2 to 6 years.(ABSTRACT TRUNCATED AT 250 WORDS)