RESUMO
This case report describes a congenital ganglioneuroblastoma in a 38-day-old male Japanese Black calf. The cervical multinodular mass was present at birth and grew rapidly. The cut surface was pale gray-to-yellow and had a gelatinous appearance. Hemorrhagic cysts of various sizes were observed in the nodule. Histologically, the mass contained clusters of neuroblastic cells, ganglionic cells, and Schwann-like cells. Immunohistochemically, the ganglionic cells showed strong positivity for neuron-specific enolase, neurofilament, synaptophysin, and chromogranin A, whereas the Schwann-like cells strongly expressed S-100, glial fibrillary acidic protein, and vimentin. Ultrastructurally, neurosecretory granules resembling catecholamine were observed in the neuroblastic and Schwann-like cells. Based on the pathology, the diagnosis was congenital cervical nodular ganglioneuroblastoma.
Assuntos
Doenças dos Bovinos/congênito , Ganglioneuroblastoma/veterinária , Neoplasias de Cabeça e Pescoço/veterinária , Animais , Bovinos , Doenças dos Bovinos/patologia , Vértebras Cervicais , Ganglioneuroblastoma/congênito , Ganglioneuroblastoma/ultraestrutura , Neoplasias de Cabeça e Pescoço/congênito , Neoplasias de Cabeça e Pescoço/ultraestrutura , MasculinoRESUMO
Crinophagy is a well-described ultraphysiological phenomenon encountered in a variety of cells and tissues. This process reflects a form of autophagy in which degradation of excess or nonfunctional cellular constituents occurs, specifically of neuroendocrine granules. The diagnostic ultrastructural features are the identification of neuroendocrine granules within lysosomes, often encased in or accompanied by myelin bodies. An impressive variety of neuroendocrine/secretory cells and tumors have demonstrated crinophagy from the neuroendocrine cells of the pancreas, small bowel, prostate, and urinary tract. To our knowledge, however, crinophagy has not been previously described in neuroblastoma, despite the fact that these tumors characteristically produce neuroendocrine granules in abundance. This case further supports the idea that crinophagy represents a common ultrastructural mechanism for the disposal and degradation of excess neuroendocrine granules.
Assuntos
Autofagia , Grânulos Citoplasmáticos/ultraestrutura , Ganglioneuroblastoma/ultraestrutura , Lisossomos/ultraestrutura , Biomarcadores Tumorais/análise , Biópsia , Criança , Grânulos Citoplasmáticos/química , Feminino , Ganglioneuroblastoma/química , Humanos , Imuno-Histoquímica , Lisossomos/química , Microscopia EletrônicaAssuntos
Ganglioneuroblastoma/patologia , Ganglioneuroma/patologia , Neuroblastoma/patologia , Neoplasias do Sistema Nervoso Periférico/patologia , Fatores Etários , Criança , Pré-Escolar , Ganglioneuroblastoma/genética , Ganglioneuroblastoma/metabolismo , Ganglioneuroblastoma/ultraestrutura , Ganglioneuroma/genética , Ganglioneuroma/metabolismo , Ganglioneuroma/ultraestrutura , Amplificação de Genes , Regulação Neoplásica da Expressão Gênica , Humanos , Lactente , Proteína Proto-Oncogênica N-Myc , Estadiamento de Neoplasias , Neuroblastoma/genética , Neuroblastoma/metabolismo , Neuroblastoma/ultraestrutura , Proteínas Nucleares/genética , Proteínas Nucleares/metabolismo , Proteínas Oncogênicas/genética , Proteínas Oncogênicas/metabolismo , Neoplasias do Sistema Nervoso Periférico/classificação , Neoplasias do Sistema Nervoso Periférico/genética , Neoplasias do Sistema Nervoso Periférico/metabolismo , Neoplasias do Sistema Nervoso Periférico/ultraestrutura , Prognóstico , Proteínas Proto-Oncogênicas c-myc/metabolismo , Receptor trkA/metabolismo , Proteínas S100/metabolismoRESUMO
Nerve tissue tumors are rarely encountered in reptiles and mainly represented by some documented cases of malignant peripheral nerve sheath tumor (MPNST). The paper is the first to describe a tumor mimicking MPNST by some ultrastructural features of tumor cells; however, significantly differing in the combination of immunohistochemical characteristics. Based on the data of electronic microscopy, immunohistochemistry, cytology, and histology, the tumor was classified as ganglioneuroblastoma. Since this nosological entity, unlike MPNST, cannot be assigned to a group of sarcomatoid tumors, the described pathology should be regarded as the first registered case of neuroectodermal histogenesis of tumors in reptiles.
Assuntos
Ganglioneuroblastoma/veterinária , Lagartos , Animais , Ganglioneuroblastoma/ultraestrutura , Masculino , Neoplasias de Bainha Neural/ultraestrutura , Neoplasias de Bainha Neural/veterináriaRESUMO
Los tumores del sistema periférico en oftalmología son poco frecuentes y algunos excepcionales. Los derivados de las simpaticogonias o neuroblastos constituyen el espectro de los neuroblastomas, ganglioneuroblastomas y ganglioneuromas; los derivados de los troncos nerviosos son los neurofibromas, schwannomas y neuromas. Los paragangliomas y quimiodectomas son excepcionales y el tumor de tritón es una verdadera rareza. Algunas de estas neoplasias se encuentran formando parte de síndromes complejos con clara connotación heredofamiliar. Se realiza una revisión clinicopatológica cuidadosa de estas entidades.
Assuntos
Olho/patologia , Neoplasias , Células APUD , Sistema Nervoso Periférico/patologia , Paraganglioma , Ganglioneuroblastoma/ultraestrutura , Neurilemoma/ultraestrutura , Neuroblastoma/ultraestrutura , Neurofibroma , Neuroma/ultraestruturaRESUMO
The study presents ultrastructural findings in 89 neuroblastic tumors. All of them including 21 undifferentiated neuroblastomas (NB) were characterized by neural cell processes which contained neurofilaments, mitochondria and neurosecretory granules. The granules were generally sparse and their number and distribution was a feature independent on other signs of differentiation of the tumor cells. We found degenerative changes in the tumor cell bodies and their neuronal processes to be a prominent phenomenon in neuroblastic tumors. Although they occurred both in patients who were operated primarily and in those who received a preoperative chemotherapy they were most conspicuous in the latter group (28 of 80 patients). The degenerative changes included swelling and dilatations of the neuronal processes, accumulation of heterophagosomes, myelinated bodies and cytoskeletal filaments. Some ganglion cells had dilated endoplasmic reticulum, and contained lipofuscin. In one ganglioneuroblastoma we detected dense granulated bodies resembling neuromelanin. The diagnosis of undifferentiated NB was established on the basis of ultrastructural investigations in 13 out of 21 undifferentiated NB; in the remaining 8 cases the electron microscopy was an additional confirmatory tool together with the results of immunohistochemical reactions. The electron microscope is still a valuable instrument for diagnosis of neuroblastic tumors in addition to newer diagnostic approaches such as immunohistochemical and molecular investigations.
Assuntos
Neuroblastoma/ultraestrutura , Criança , Ganglioneuroblastoma/ultraestrutura , HumanosRESUMO
Neuroblastic tumors have the unique ability to differentiate and mature. This family of tumors is composed of the neuroblastoma, ganglioneuroblastoma, and ganglioneuroma. These tumors are derived from primordial neural crest cells that form the sympathetic nervous system. The purpose of this study was to characterize the ultrastructural features of neuroblastic tumors in a pediatric population. Forty-five neuroblastic tumors (15 neuroblastomas, 15 ganglioneuroblastomas, and 15 ganglioneuromas) were examined using standard transmission electron microscopic techniques. Undifferentiated neuroblastomas were composed of primitive cells with rare neurite-like processes containing clear secretory vesicles and no Schwann cell differentiation. Poorly differentiated and differentiating neuroblastomas showed more frequent neuritic processes with infrequent dense core granules and infrequent immature Schwann-like cells. Ganglioneuroblastomas possessed an admixture of cell types, including immature ganglion cells without associated satellite cells, intermediate cells, and differentiating neuroblasts. The neuropil contained immature Schwann cells encasing haphazardly arranged neuritic processes. Ganglioneuromas were composed of mature ganglion cells with occasional binucleation. The neuropil contained mature Schwann cells with well-organized neuritic processes and abundant collagen deposition. Differentiation or maturation of tumor cells, neuritic processes, and Schwann cells may thus be discerned ultrastructurally in primary neuroblastic tumors in pediatric patients.