Doege–Potter syndrome

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Doege–Potter syndrome
Synonyms
Pronounce
Specialty	Oncology, Endocrinology
Symptoms	Hypoglycemia, weakness, confusion, seizures
Complications
Onset
Duration
Types
Causes	Solitary fibrous tumor
Risks
Diagnosis	Blood test, imaging studies
Differential diagnosis
Prevention
Treatment	Surgical resection
Medication
Prognosis
Frequency	Rare
Deaths

Doege–Potter syndrome is a rare paraneoplastic syndrome characterized by hypoglycemia associated with solitary fibrous tumors, most commonly solitary fibrous tumors of the pleura. This condition is named after Karl Doege and Roy Potter, who first described the syndrome in the 1930s.

Pathophysiology

The hypoglycemia in Doege–Potter syndrome is caused by the production of insulin-like growth factor 2 (IGF2) by the tumor. IGF2 is a hormone similar in molecular structure to insulin, and it can lower blood glucose levels by increasing glucose uptake into cells and inhibiting glucose production by the liver. The overproduction of IGF2 by the tumor leads to persistent hypoglycemia, which can be severe and symptomatic.

Clinical Presentation

Patients with Doege–Potter syndrome typically present with symptoms of hypoglycemia, which may include dizziness, sweating, confusion, seizures, and even loss of consciousness. These symptoms are often episodic and may be exacerbated by fasting or physical exertion.

Diagnosis

The diagnosis of Doege–Potter syndrome is based on the clinical presentation of hypoglycemia in conjunction with the presence of a solitary fibrous tumor. Imaging studies such as computed tomography (CT) or magnetic resonance imaging (MRI) are used to identify the tumor. Biochemical tests may reveal low blood glucose levels and elevated levels of IGF2.

Treatment

The primary treatment for Doege–Potter syndrome is the surgical resection of the tumor. Removal of the tumor typically resolves the hypoglycemia. In cases where the tumor is not resectable, medical management of hypoglycemia may be necessary, which can include dietary modifications and medications to increase blood glucose levels.

Prognosis

The prognosis for patients with Doege–Potter syndrome is generally favorable if the tumor can be completely resected. However, the prognosis may be poorer if the tumor is malignant or if complete resection is not possible.

See Also

• Solitary fibrous tumor
• Hypoglycemia
• Insulin-like growth factor 2