RESUMO
BACKGROUND: Mesectodermal leiomyoma is a benign tumor of smooth muscle of the ciliary body, which is derived from the neural crest. CLINICAL CASE: We report the case of a 35-year-old Mexican woman with visually impaired and blurred vision of the right eye of 2 months duration. The clinical and imaging presuntional diagnosis was adenoma of the non pigmented epithelium of the ciliary body and it was surgically resected. Microscopically, the tumor was composed of cells with round nuclei and scant cytoplasm without atypia or mitosis, arranged in a fibrillary background. The immunohistochemical markers for vimentin, muscle specific actin, smooth muscle actin and calponin were strongly positive in the cytoplasm of the neoplastic cells, while for glial fibrillary acidic protein and S-100 protein were negative in the same cellular population. CONCLUSIONS: Mesectodermal leiomyoma of the ciliary body is benign tumor of smooth muscle extremely rare in this location. Until now, there are just 25 previous reported cases in the literature and, the main differential diagnosis is uveal malignant melanoma, therefore some eyes were enucleated. The ultrabiomicroscopy, A and B-scan imaging studies are useful in the evaluation, however, is mandatory the microsocpic examination with routine and histochemical stains as well as the use of immunohistochemical markers such as vimentin, specific muscle actin, smooth muscle actin andcalponin to stablish the smooth muscle origin of this neoplasm, and rule out other malignant neoplams such as malignant melanoma.
Antecedentes: el leiomioma mesoectodérmico es un tumor benigno excepcional que se origina en el músculo liso del cuerpo ciliar y deriva de la cresta neural. Caso clínico: se comunica el caso de una mujer de 35 años, con disminución de la agudeza visual y visión borrosa de 2 meses de evolución en el ojo derecho. El diagnóstico presuncional clínico e imagenológico fue: adenoma del epitelio no pigmentado del cuerpo ciliar, por lo que se resecó quirúrgicamente. Microscópicamente, el tumor estaba formado por células de núcleos redondos de escaso citoplasma sin atipia ni mitosis, dispuestas en una matriz fibrilar. Los inmunomarcadores para vimentina, actina músculo específica, actina de músculo liso y calponina fueron todos positivos en el citoplasma de las células neoplásicas, excepto de los inmunomarcadores para la proteína ácida gliofibrilar y la proteína S-100 que resultaron negativos en la misma población celular. Conclusiones: el leiomioma mesoectodérmico del cuerpo ciliar es un tumor benigno de músculo liso extremadamente raro en esta localización. Hasta el momento, sólo hay 25 casos informados en la bibliografía médica y su principal diagnóstico diferencial es melanoma uveal, motivo por el que algunos ojos se enuclearon. Los estudios de ultrabiomicroscopia y ecografía modos A y B son útiles en la evaluación; sin embargo, es obligado el estudio microscópico con tinciones de rutina, y el uso de marcadores inmunohistoquímicos, como los utilizados en este caso para establecer la naturaleza del músculo liso de esta neoplasia y descartar algunas otras, como el melanoma.
Assuntos
Corpo Ciliar/patologia , Erros de Diagnóstico , Leiomioma/diagnóstico , Neoplasias Uveais/diagnóstico , Adenoma/diagnóstico , Adulto , Biomarcadores Tumorais/análise , Corpo Ciliar/química , Corpo Ciliar/diagnóstico por imagem , Diagnóstico Diferencial , Proteínas do Olho/análise , Feminino , Humanos , Leiomioma/química , Leiomioma/diagnóstico por imagem , Leiomioma/patologia , Leiomioma/cirurgia , Melanoma/diagnóstico , Microscopia Acústica , Neoplasias Uveais/química , Neoplasias Uveais/diagnóstico por imagem , Neoplasias Uveais/patologia , Neoplasias Uveais/cirurgiaRESUMO
PURPOSE: Interstitial cells of Cajal were identified in the gastrointestinal tract of several species, with close relation to the enteric nervous system. Since it was recognized that interstitial cells of Cajal express the gene product of c-kit, we performed immunohistochemistry for c-kit protein in ciliary muscle specimens of monkeys' eyes. METHODS: Eight eyes from four adult male new world monkeys (Cebus apella) were studied. After blocking endogenous peroxidase activity and nonspecific protein binding, 1:100 dilution of mouse monoclonal antibody against c-kit human oncoprotein was applied to tissues. Antigen-antibody reaction was visualized using the avidin-biotinylated horseradish peroxidase complex in each slide. RESULTS: We observed some groups of fusiform c-kit expressing cells located amongst muscle bundles of the ciliary muscle. Other pigment cells and mast cells were also observed. CONCLUSION: C-kit expressing cells observed in the ciliary muscle of Cebus apella, showed no similarity to melanocytes or mast cells and they could be associated with their gastrointestinal interstitial cells of Cajal counterpart.
Assuntos
Corpo Ciliar/química , Trato Gastrointestinal/citologia , Proteínas Proto-Oncogênicas c-kit/análise , Animais , Cebus , Corpo Ciliar/citologia , Masculino , Músculo Liso/químicaRESUMO
PURPOSE: Interstitial cells of Cajal were identified in the gastrointestinal tract of several species, with close relation to the enteric nervous system. Since it was recognized that interstitial cells of Cajal express the gene product of c-kit, we performed immunohistochemistry for c-kit protein in ciliary muscle specimens of monkeys' eyes. METHODS: Eight eyes from four adult male new world monkeys (Cebus apella) were studied. After blocking endogenous peroxidase activity and nonspecific protein binding, 1:100 dilution of mouse monoclonal antibody against c-kit human oncoprotein was applied to tissues. Antigen-antibody reaction was visualized using the avidin-biotinylated horseradish peroxidase complex in each slide. RESULTS: We observed some groups of fusiform c-kit expressing cells located amongst muscle bundles of the ciliary muscle. Other pigment cells and mast cells were also observed. CONCLUSION: C-kit expressing cells observed in the ciliary muscle of Cebus apella, showed no similarity to melanocytes or mast cells and they could be associated with their gastrointestinal interstitial cells of Cajal counterpart.
OBJETIVO: As células intersticiais de Cajal estão presentes no trato gastrintestinal de diversas espécies animais, em íntima relação com o sistema nervoso entérico. Uma vez que as células intersticiais de Cajal expressam o produto do gene c-kit, realizou-se um ensaio imuno-histoquímico a fim de se verificar a marcação da proteína c-kit no músculo ciliar de amostras de olhos de macacos. MÉTODOS: Oito olhos de quatro macacos do novo mundo (Cebus apella) foram estudados. Após bloqueio da peroxidase endógena e de ligação protéica não específica, os tecidos receberam aplicação de anticorpos de camundongos antioncoproteína c-kit humana (1:100). A reação antígeno-anticorpo foi verificada através da aplicação do complexo avidina-biotinilada-peroxidase em cada lâmina. RESULTADOS: Foram observados grupos de células que expressam c-kit, localizadas entre as fibras do músculo ciliar. Mastócitos e outras células pigmentadas também foram observadas. CONCLUSÃO: Algumas células que expressam c-kit, observadas no músculo ciliar de Cebus apella, não mostraram similaridade com mastócitos ou melanócitos e podem ser classificadas como análogas das células intersticiais de Cajal gastrintestinais.
Assuntos
Animais , Masculino , Corpo Ciliar/química , Trato Gastrointestinal/citologia , Proteínas Proto-Oncogênicas c-kit/análise , Cebus , Corpo Ciliar/citologia , Músculo Liso/químicaRESUMO
Primary uveal-tract neoplasms are extremely rare in childhood; the most common lesions found are melanocytic. We report here the case of a 7-year-old girl who underwent enucleation of the right eye with clinical suspicion of choroid melanoma as a result of a ciliary body mass that extended to the posterior chamber. Histologically, the neoplasm featured spindle cell morphology, atypia, and mitoses. The tumor expressed smooth muscle alpha actin, pan-actin HHF-35, and desmin, whereas immunohistochemistry for melanocytic markers, such as S-100, Melan-A, and HMB-45, was negative. Based on these features, the diagnosis of leiomyosarcoma of the ciliary body was firmly established. Although several leiomyomas have been reported in the literature, there are only 2 previously reported cases of primary leiomyosarcoma of the uveal tract. Immunohistochemical expression of muscle proteins allowed distinction from the most common melanocytic tumors arising in this location.