Year: 2025
Type: conference-abstract
Abstract: Disclosure: D.R. Ávila Salcedo: None. J.A. Ayala San Pedro: None. L.M. Martínez Borja: None. P.A. Montes Hernández: None. R.S. Sánchez García: None. Background: Paraneoplastic hypoglycemia or Doege-Potter syndrome was first described in 1930. It is the clinical manifestation of mesenchymal tumors that produce IGF-II. The most frequent localization is thorax, pelvis, liver and retroperitoneum. The clinical data in addition to hypoglycemia are; hypertrophic osteoarthropathy, seborrheic hyperkeratosis, acanthosis nigricans. The IGF-I/IGF-II molar index in 79% of cases is >10 and in the rest between 3 and 10. Case: 31-year-old female with a history of uterine myomatosis. She started suffering with alterations in mental status, periodic confusion and disorientation, and was diagnosed with psychiatric disease. As the symptoms persisted, hypoglycemia was documented, which was more frequent during fasting. Physical examination showed pachyonychia, gray dyschromia, longitudinal striae and distal lysis of the 1st and 2nd fingers. Paraclinical: TSH1.68 mU/ml, T4t 6.28 µg/dl, T4l 2.7pg/ml, T3t 1.2ng/ml, T3l 2.7pg/ml, Anti-thyroglobulin and anti-TPO antibodies negative. HbA1c 4.9%, glucose 51 mg/dl, creatinine 0.28 mg/dl, total cholesterol 215 mg/dl, triglycerides 102 mg/dl, c-LDL 136 mg/dl, total bilirubin 0.50 mg/dl, albumin 3.6 mg/dl , AST 32 UI/L, ALT 27 U/L. Fasting test was performed, presenting hypoglycemia of 22 mg/dl showing diaphoresis, hunger, dysarthria and somnolence. C-peptide levels 0.01 ng/ml, insulin 0.20µUI/ml, proinsulin <0.5 pmol/L, serum cortisol >18 µg/dl and serum sulfonylureas were negative. Negative anti-insulin and anti-insulin receptor antibodies, GH 0.066 ng/ml, IGF-I 18 ng/ml, IGF-II 184 (267-616 ng/ml). Ratio IGF-I/IGF-II 1:9 (<3), suspecting aberrant IGF-II production, without having that reagent in our country. CT: Heterogeneous right-sided pelvic cavity tumor measuring 133x91x132mm compressing the uterus, bladder and rectum, causing mild ectasia of the right ureter. Treatment was partial resection due to its high vascularity and surgical bleeding. Histology: brown and smooth tissue of pelvic fibrothecoma, proliferation index <10%, Ki67 <10%, Wt-1+, negative for: alpha-inhibin, progesterone receptor, STAT-6, CD56, estrogen receptor, CD10 and CD34. After 24 hours the hypoglycemia subsided and 3 months later the nail changes reversed. Clinical lessons: Despite its low prevalence worldwide, it should be considered as a differential diagnosis of non-insulin-mediated hypoglycemia. The review of the medical literature has not described the nail manifestations presented in this case. In addition, she presented normal serum IGF-II levels and IGF I/IGF II molar index between 3 and 10 contrary to most cases. The patient is being followed up for a second intervention or radiotherapy. References: 1.Torres Gómez, A.(2023). Doege-Potter syndrome. doi.org/10.1016/j.endien.2023.06.0052.Han, G.(2017). Doege-Potter syndrome. doi.org/10.1097/MD.0000000000007417 Presentation: Saturday, July 12, 2025
Source: Journal of the Endocrine Society
Authors: Dylani Rosa Ávila Salcedo, Jorge Alejandro Ayala San Pedro, Livia Magdalena Martínez Borja, Pavel Antonio Montes Hernández, Rodrigo Santiago Sánchez García
Language: English
Subfield: Endocrinology, Diabetes and Metabolism
Field: Medicine
Domain: Health Sciences
Open Access status: gold
Estimated APC paid: $3,255
OpenAlex ID: W7093375944
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