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Authors
Abstract(s)
paraganglioma do corpo carotídeo, também conhecido como tumor glómico carotídeo, quimiodectoma ou tumor de células não-cromafins, é uma neoplasia rara, geralmente benigna, pertencente ao grupo dos tumores neuroendócrinos. Apresenta-se habitualmente sob a forma de uma massa não dolorosa de localização latero-cervical; tem sido, contudo, diagnosticado cada vez mais de forma incidental. A ecografia duplex, a tomografia computorizada e a ressonância magnética nuclear são os exames complementares de diagnóstico mais úteis e acessíveis. De forma a prevenir complicações locais futuras, que ocorrem com a evolução natural da doença, e eliminar o pequeno risco de malignidade, é frequentemente proposta a ressecção cirúrgica, não livre de riscos. Existem por isso várias correntes de abordagem alternativas a esta patologia, que incluem diferentes técnicas cirúrgicas, embolização pré-cirúrgica e radioterapia, o que demonstra a falta de consenso nesta matéria. A classificação de Shamblin é utilizada para prever a complexidade de uma eventual ressecção cirúrgica. Têm havido desenvolvimentos recentes na compreensão das bases genéticas dos paragangliomas, especulando-se ainda sobre a patofisiologia molecular.
Como suporte à revisão bibliográfica expõe-se o caso de uma paciente do sexo feminino que se apresentou aos oitenta anos de idade com uma massa cervical não dolorosa com tempo de evolução prolongado, que se veio a diagnosticar como paraganglioma do corpo carotídeo; a paciente apresentava também um quadro sintomatológico mal definido desencadeado pela deglutição, que se admite ser propiciado pela massa tumoral.
Pela revisão da literatura conclui-se que a ressecção cirúrgica do tumor deve ser considerada a abordagem de primeira linha quando não há risco inaceitável de complicações; técnicas cirúrgicas ou pré-cirúrgicas adjacentes podem ser aplicadas de acordo com a experiência pessoal. Exames imagiológicos como a ecografia duplex, a tomografia computorizada e/ou a ressonância magnética nuclear são geralmente suficientes para fazer o diagnóstico; recomenda-se ainda o estudo genético a todos os doentes. O seguimento deve ser prolongado e incluir exames de imagem.
Prevêem-se novos desenvolvimentos num futuro próximo sobre a etiopatogenia genética dos paragangliomas, o que permitirá aprofundar os conhecimentos actuais sobre a biologia molecular e, presumivelmente, criar tratamentos médicos específicos para esta patologia.
Carotid body paraganglioma, also known as carotid glomus tumor, chemodectoma or non-chromaffin cells tumor, is a rare neoplasm, usually benign, which belongs to the group of the neuroendocrine tumors. Its classic presentation is a painless mass in the lateral cervical region; it has been, nevertheless, increasingly diagnosed incidentally. Duplex ultrasound, computerized tomography and nuclear magnetic resonance are the most useful and accessible complementary exams. With the intention of preventing future local complications that occur with the natural evolution of the disease, and to eliminate the small risk of malignancy, it’s frequently proposed a surgical resection, not risk free. There are therefore several alternative approaches to this disorder, which include different surgical techniques, preoperative embolization and radiotherapy, proving the lack of consensus in the subject. The Shamblin classification is used to predict the intricacy of a possible surgical resection. There have been recent progresses in the understanding of the genetic background of the paragangliomas, but its molecular pathophysiology is still unknown. We present here, as a support to this review, the case of an eighty years old female patient who presented with a longstanding cervical painless mass, diagnosed as a carotid body paraganglioma; the patient also featured an ill-defined set of symptoms triggered by swallowing, arguably caused by the tumor mass. Through the review of the literature we conclude that the surgical resection of the tumor should be considered the first line of treatment, in the absence of unacceptable risks; operative or preoperative specific techniques may be employed according to personal experience. Imaging techniques such as duplex ultrasound, computerized tomography and nuclear magnetic resonance are generally enough to assure the diagnosis; it’s additionally recommended the genetic study to all patients. The follow-up should be long-lived and include imaging techniques. Further progresses are expected in the near future about the genetic etiopathogenesis of the paragangliomas, which will allow the expansion of our current knowledge about its molecular biology and, presumably, the development of targeted treatments to this disease.
Carotid body paraganglioma, also known as carotid glomus tumor, chemodectoma or non-chromaffin cells tumor, is a rare neoplasm, usually benign, which belongs to the group of the neuroendocrine tumors. Its classic presentation is a painless mass in the lateral cervical region; it has been, nevertheless, increasingly diagnosed incidentally. Duplex ultrasound, computerized tomography and nuclear magnetic resonance are the most useful and accessible complementary exams. With the intention of preventing future local complications that occur with the natural evolution of the disease, and to eliminate the small risk of malignancy, it’s frequently proposed a surgical resection, not risk free. There are therefore several alternative approaches to this disorder, which include different surgical techniques, preoperative embolization and radiotherapy, proving the lack of consensus in the subject. The Shamblin classification is used to predict the intricacy of a possible surgical resection. There have been recent progresses in the understanding of the genetic background of the paragangliomas, but its molecular pathophysiology is still unknown. We present here, as a support to this review, the case of an eighty years old female patient who presented with a longstanding cervical painless mass, diagnosed as a carotid body paraganglioma; the patient also featured an ill-defined set of symptoms triggered by swallowing, arguably caused by the tumor mass. Through the review of the literature we conclude that the surgical resection of the tumor should be considered the first line of treatment, in the absence of unacceptable risks; operative or preoperative specific techniques may be employed according to personal experience. Imaging techniques such as duplex ultrasound, computerized tomography and nuclear magnetic resonance are generally enough to assure the diagnosis; it’s additionally recommended the genetic study to all patients. The follow-up should be long-lived and include imaging techniques. Further progresses are expected in the near future about the genetic etiopathogenesis of the paragangliomas, which will allow the expansion of our current knowledge about its molecular biology and, presumably, the development of targeted treatments to this disease.
Description
Keywords
Paraganglioma do Corpo Carotídeo Quimiodectoma Tumor Glómico Carotídeo Tumor Neuroendócrino