Assuntos
Regulador de Condutância Transmembrana em Fibrose Cística/genética , Fibrose Cística/genética , Países em Desenvolvimento , Testes Genéticos , Mutação/genética , Afeganistão , Pré-Escolar , Consanguinidade , Comportamento Cooperativo , Fibrose Cística/diagnóstico , Análise Mutacional de DNA , Acessibilidade aos Serviços de Saúde , Humanos , Comunicação Interdisciplinar , MasculinoRESUMO
The authors report a new case of cardiac sarcoma treated by cardiac transplantation. This treatment has been proposed for these malignant tumours of poor prognosis when simple excision is impossible, with variable results. This patient is in good general condition 20 months after transplantation. Transplantation is a therapeutic procedure which should be considered in malignant tumours limited to the heart.
Assuntos
Neoplasias Cardíacas , Neoplasias Cardíacas/cirurgia , Transplante de Coração/métodos , Sarcoma/cirurgia , Adulto , Ecocardiografia Transesofagiana , Neoplasias Cardíacas/diagnóstico , Humanos , Masculino , Prognóstico , Sarcoma/diagnóstico , Resultado do TratamentoRESUMO
BACKGROUND: Basal cell carcinomas (BCCs) have been described in various locations, such as skin, anal canal, tongue, larynx, and recently, the lungs. These tumors seem to have a poor prognosis. METHODS: A series of 115 surgically resected lung tumors, previously classified as poorly or undifferentiated carcinoma, were reviewed retrospectively. From these, 37 cases were reclassified as BCCs and were compared in terms of clinical features and survival, with 40 cases reclassified as poorly differentiated squamous cell (PDSC) carcinoma of the lung. RESULTS: There was no difference between the groups regarding age, clinical presentation, pattern of relapse, and cause of death. Median and overall survival were different between the two groups, especially for Stage I and II patients: 5-year actuarial survival in the BCC group was 15% and in the PDSC group 47% (P = 0.004). CONCLUSIONS: This subset of non-small cell lung cancer (NSCLC) has a worse prognosis than other NSCLC, and this should be considered in survival studies and new treatment trials.
Assuntos
Neoplasias Brônquicas/mortalidade , Neoplasias Brônquicas/patologia , Carcinoma Basocelular/mortalidade , Carcinoma Basocelular/patologia , Carcinoma Pulmonar de Células não Pequenas/mortalidade , Carcinoma Pulmonar de Células não Pequenas/patologia , Neoplasias Pulmonares , Adulto , Idoso , Carcinoma de Células Escamosas/mortalidade , Carcinoma de Células Escamosas/patologia , Causas de Morte , Feminino , Humanos , Masculino , Pessoa de Meia-Idade , Prognóstico , Estudos Retrospectivos , Taxa de SobrevidaRESUMO
Three patients of a French family demonstrated an association of multiple endocrine neoplasia type 2A (MEN 2A) with a pruritic scapular skin lesion. The lesions are similar to those described as familial cutaneous lichen amyloidosis in unrelated MEN 2A and medullary thyroid carcinoma families, but histological, immunohistochemical, and ultrastructural analysis of skin biopsies from each patient in the French family did not show amyloid deposition. The topography of the lesion follows dermatomes C8-D3. The patients report not only pruritus but also paresthesia and hyperalgesia, and one showed touch hypoesthesia and pain hyperesthesia in the area of the lesion. Such an association of cutaneous and neurological features suggests notalgia paresthetica (NP), a neuropathy of the posterior dorsal rami nerves. We thus suggest that the cutaneous lesions associated with MEN 2A might be secondary to pathology in the neural crest-derived dorsal sensory nerves. The amyloid, when present, would be secondary to scratching. We propose that patients presenting with familial NP be suspect for MEN 2A.
Assuntos
Amiloidose/complicações , Neoplasia Endócrina Múltipla/complicações , Parestesia/complicações , Dermatopatias/complicações , Adulto , Amiloidose/genética , Amiloidose/patologia , Dor nas Costas/patologia , Feminino , Humanos , Masculino , Pessoa de Meia-Idade , Neoplasia Endócrina Múltipla/genética , Parestesia/genética , Parestesia/patologia , Linhagem , Doenças do Sistema Nervoso Periférico/complicações , Prurido/patologia , Pele/patologia , Dermatopatias/genética , Dermatopatias/patologiaRESUMO
UNLABELLED: We studied GH secretion in a patient with acromegaly and a bronchial carcinoid tumor before and again after surgical removal of this tumor. Before removal of the carcinoid tumor, plasma GH increased slightly after glucose loading (OGTT) and markedly after TRH (650%) and insulin (440%) treatment. Plasma GH did not change after GH-releasing hormone (GHRH), LHRH, or L-dopa administration. Somatostatin (SRIH) infusion lowered plasma GH. No change in plasma immunoreactive GHRH (IR-GHRH) occurred after TRH, glucose, insulin, or SRIH administration. Two weeks after removal of the carcinoid tumor, TRH induced GH secretion (250%) when the IR-GHRH level was undetectable and somatomedin-C was within normal limits. Fifteen weeks after surgery, the patient had normal GH secretion. IN CONCLUSION: no pattern of GH secretion is diagnostic of acromegaly due to ectopic GHRH secretion, but the lack of GH response to exogenous GHRH and a large response during hypoglycemia may be features of this condition. When acromegaly and abnormal GH responsiveness are induced by a GHRH-secreting tumor, the increases in plasma GH after TRH, glucose, and insulin administration are not mediated by GHRH. After removal of the GHRH-secreting tumor, persistent paradoxical GH response to TRH does not require abnormally high IR-GHRH levels and does not preclude complete recovery.
Assuntos
Acromegalia/etiologia , Neoplasias Brônquicas/metabolismo , Tumor Carcinoide/metabolismo , Hormônio Liberador de Hormônio do Crescimento/metabolismo , Síndromes Endócrinas Paraneoplásicas , Acromegalia/sangue , Acromegalia/terapia , Neoplasias Brônquicas/cirurgia , Tumor Carcinoide/cirurgia , Feminino , Hormônio do Crescimento/metabolismo , Humanos , Pessoa de Meia-IdadeRESUMO
The protein A-gold technique, a simple and reliable two-step postembedding immunocytochemical method, allows the light and electron microscopic detection of antigens in routinely fixed and embedded tissue. At the light microscopic level a permanent, nonfading stain is obtained. High resolution studies on intracellular antigens can be performed since the particulate nature of the colloidal gold permits accurate determination of the labeled cellular structures. The applicability of the protein A-gold technique for the localization of various peptide and protein antigens by light and electron microscopy is demonstrated.
Assuntos
Duodeno/citologia , Rim/citologia , Neoplasias/patologia , Pâncreas/citologia , Adenoma/patologia , Animais , Tumor Carcinoide/patologia , Ouro , Técnicas Histológicas , Humanos , Hipoglicemia/patologia , Insulinoma/patologia , Microscopia Eletrônica/métodos , Neoplasias/ultraestrutura , Pâncreas/patologia , Neoplasias Pancreáticas/patologia , Neoplasias Hipofisárias/patologia , Ratos , Proteína Estafilocócica ARESUMO
Two cases of pleomorphic xanthoastrocytoma (P X A) of young subjects (Kepes et al., 1979) are reported. Case 1 arose in 15-year-old boy admitted to the hospital with the complaint of severe headaches associated with nausea and vomiting of 1 month's duration. Computed tomographic scans showed a large well-defined low density area in the left temporo-parietal region of which an anterior portion was enhanced by contrast medium. Craniotomy revealed a large superficial and cystic tumor with a mural nodule. Histological and immunohistochemical features were those of a P X A confirmed by an electron microscopic study. No radiotherapy was given. The patient made a complete recovery, and 32 months later was asymptomatic. Case 2, a 17-year-old boy was admitted to the hospital in 1977. He presented with seizures that started 18 months prior to surgery. Carotid and humeral angiograms and air studies indicated the presence of a right, internal temporal mass with herniation. The craniotomy revealed a firm superficial tumor with an infratentorial, extraparenchymal extension. The histological diagnosis was giant cell glioblastoma or gliosarcoma. The patient received post-operative radiation of 5.500 rads and chemotherapy (CCNU and VM 26). He died on the 7th post-operative month. In this 2nd case, the diagnosis of P X A was made retrospectively based upon histological and immunohistochemical observations similar to case 1. We are aware of 24 P X A in the literature. In their clinical and histological features these neoplasms resemble closely each other. P X A are superficial, supratentorial astrocytomas occurring in youngs subjects (ages 3 to 32). Their typical microscopic structure include a marked cellular pleomorphism with bizarre giant cells, some mitotic figures and no necrosis. Many cells contain lipid and hyalin droplets in their cytoplasm. Characteristically, the tumoral stroma contain a very rich reticulin fiber network. Immunoperoxidase technique reveal glial fibrillary acidic protein in the tumor cells. Electron microscopic studies demonstrate abundant intracytoplasmic glial filaments. Individual cells or group of cells are surrounded by a prominent basal lamina. Some hemidesmosomes or primitive attachments are seen at the margins of the tumor cells. The biological behaviour of PXA with or without radiotherapy is relatively favorable. Long survival times (up to 25 years) are reported but in 5 cases, P X A follow a less favorable course with malignant transformation and death. Morphologic and immunohistochemical studies support the subpial astrocytic origin of P X A.