Surgically cured hypoglycemia secondary to pleural solitary fibrous tumour: case report and update review on the Doege-Potter syndrome

Abstract

The association of paraneoplastic hypoglycemia [Doege-Potter syndrome] and finger clubbing [Pierre-Marie-Bamberg syndrome] with pleural solitary fibrous tumour is rare. We present a previously unpublished but typical example of this rare occurrence together with a detailed updated literature review of previously published cases of pleural SFT discussing the histopathology of SFT; pathophysiology of the hypoglycemia and finger clubbing; treatment and outcome of pleural SFT. The patient, a 57-year-old African male was admitted at our hospital with recurrent episodes of hypoglycemia. He was found to have digital clubbing and decreased breath sounds in the right lower chest but no other significant clinical findings. His insulin level measured during an episode of hypoglycemia was undetectable. Chest radiograph and CT-scan revealed a lobulated mass in the right chest which was diagnosed to be SFT on histology. Surgical excision of the mass resulted in cure of the hypoglycemic episodes and rapid regression of the clubbing. Less than 65 cases of pleural SFT manifesting with hypoglycemia with or without finger-clubbing have been published in the English literature. The mean diameter of these tumours manifesting with hypoglycemia is 20 cm, 54% being benign while 42% were malignant. They predominantly present in the 6th-8th decade, average age of 64 years and a slight male preponderance at 58%. Complete surgical resection remains the most important predictor of clinical outcome in terms of recurrence and metastases, while providing instant cure for the hypoglycemia and rapid resolution of the finger clubbing.

Giant solitary fibrous tumor of pleura revealed by epileptic seizures: A case report

“…[3] Solitary fibrous tumor of the pleura associated with severe hypoglycemia is rare. [4] The cause of hypoglycemia is believed to be related to IGFs produced by the tumor. Insulin-like growth factor-II is among the insulin-like peptides commonly believed to be responsible for non-islet cell tumor hypoglycemia.…”

“…[3] Hypoglycemia was observed to be associated with large SFTP (>10 cm), but had no obvious relevance with histological features of malignancy. [4] In this article, we present a giant SFTP complicated with epileptic seizures caused by severe tumor-induced hypoglycemia.…”

Síndrome Doege-Potter. Reporte de caso

“…El 80% es de naturaleza benigna y no se ha establecido de manera definitiva las características específicas de malignidad (7,8). La mayoría de estos tumores se originan en la pleura visceral y 25% de los casos presentan hipoglicemia, dedos hipocráticos o efusión pleural (9).…”

“…El rango de edad de presentación es de 38 a 83 años, con una media de 64 años (4,7,9) y ligera preponderancia en varones (6). El tamaño promedio de los tumores fue de 20 cm, con un peso promedio de 2 071 g (rango de 850 a 4 000g) (7,9).…”

“…Inicialmente, se consideró que el TFSP tenía un origen mesotelial; pero, evidencia reciente ha revelado que tendría una histogénesis mesenquimal (7). Se desconocen los factores etiológicos, pero no existe asociación con el hábito de fumar o la exposición a asbesto (10).…”