Doege-Potter syndrome: a rare presentation of a solitary fibrous tumor of the pleura

ABSTRACT

Solitary fibrous tumors of the pleura usually have a benign behavior, although some may present malignant characteristics - recurrences, metastases, and paraneoplastic syndromes. When a solitary fibrous tumor is responsible for the development of hypoglycemia, we have a case of Doege-Potter syndrome. We describe a case of a male patient that presented recurring and severe hypoglycemia, being also diagnosed with an extensive intrathoracic mass, which was proven to be a solitary fibrous tumor of the pleura. The treatment, through the maintenance of proper serum levels of glucose (administration of solutions with glucose and steroids), followed by the surgical resection of the tumor, promoted the complete resolution of the hypoglycemia episodes. The early recognition of this condition and the proper treatment is essential for a good clinical outcome. It is important to underscore that the multidisciplinary evaluation (oncologists, radiologists, surgeons, and pathologists) of rare tumors is recommended to achieve better therapeutic results.

RESUMO

Os tumores fibrosos solitários da pleura costumam ter um comportamento benigno, embora alguns possam apresentar características malignas - recidivas, metástases e síndromes paraneoplásicas. Quando um tumor fibroso solitário é responsável pelo desenvolvimento de hipoglicemia, temos um caso de síndrome de Doege-Potters. Descrevemos o caso de um paciente do sexo masculino que apresentou hipoglicemia recorrente e grave, sendo diagnosticado também uma extensa massa intratorácica, que se comprovou ser um tumor fibroso solitário da pleura. O tratamento, por meio da manutenção de níveis séricos adequados de glicose (administração de soluções com glicose e esteroides), seguido da ressecção cirúrgica do tumor, promoveu a resolução completa dos episódios de hipoglicemia. O reconhecimento precoce dessa condição e o tratamento adequado são essenciais para uma boa evolução clínica. É importante ressaltar que a avaliação multidisciplinar (oncologistas, radiologistas, cirurgiões e patologistas) de tumores raros é recomendada para obter melhores resultados terapêuticos.

Keywords:

Solitary fibrous tumor - Pleural - Paraneoplastic endocrine syndromes - Thoracic neoplasms.

Descritores:

Tumor fibroso solitário da pleura - Síndromes endócrinas paraneoplásicas - Neoplasias torácicas.

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