A Case Report of Doege–Potter Syndrome: A Rare Cause of Hypoglycemia in a Patient without Diabetes

Abstract

Hypoglycemia in patients without diabetes is a diagnostic challenge for the endocrinologist. Sometimes it is related to rare causes such as Doege–Potter Syndrome (DPS). DPS is caused by an abnormal insulin-like grow factor 2(IGF-2) that retains part of the E domain during the production process, resulting in a longer peptide called “big-IGF-2”. We present a case report of DPS with emphasis on the diagnosis and especially on the difficulties in interpreting the biochemical findings. An elderly patient with an intrathoracic neoplasm and hypoglycemia underwent various tests: insulin autoantibodies and fasting test were both negative. She had low values of IGF-1 and normal values of IGF-2 that apparently excludes a diagnosis of DPS. The evaluation of the IGF-2/IGF-1 ratio is the most important test because a ratio >10 is widely considered to be indicative of non-islet cell tumor hypoglycemia (NICTH). Glucose infusion and steroid therapy were used to control the hypoglycemia, but the definitive treatment was surgery, which almost immediately reversed the hypoglycemia. The differential diagnosis of hypoglycemia should include rare causes such as DPS, and the IGF-2/IGF-1 ratio is a useful tool.

Addressing recurrent hypoglycaemia through thoracic surgical intervention: understanding Doege-Potter syndrome, a rarity in syndromes

“…The surplus circulating IGF-2 activates the insulin receptor and insulin-like growth factor 1 receptor (IGF-1R) in an autocrine or paracrine manner. This activation promotes glucose uptake into tissues, enhances glycogen synthesis, inhibits gluconeogenesis, and stimulates cell growth and proliferation [ 4 ]. The elevated IGF-2 levels result in increased glucose utilisation and storage in peripheral tissues, leading to hypoglycaemia due to depleted circulating glucose.…”

“…The elevated IGF-2 levels result in increased glucose utilisation and storage in peripheral tissues, leading to hypoglycaemia due to depleted circulating glucose. This dysregulated glucose metabolism and heightened glucose utilisation are the primary contributors to the observed hypoglycaemic episodes in DPS [ 4 , 20 ].…”

“…For sustained management, pharmacological approaches may include corticosteroids, growth hormone, octreotide, or glucagon [ 20 , 31 ]. Recombinant human growth hormone (GH) administered at doses ranging from 3 to 12 mg/day has demonstrated efficacy in managing hypoglycaemia [ 4 ].…”

“…While surgical intervention is recognised in cases like pancreatic insulinoma for addressing recurrent hypoglycaemia, this review focuses on an uncommon condition, Doege-Potter Syndrome (DPS), in which comprehensive surgical resection acts as both an oncologic and therapeutic intervention for recurrent hypoglycaemia. This syndrome presents diagnostic complexities due to multifaceted clinical manifestations and its elusive nature [ 4 ].…”