Session: SUN 29-49-Congenital Adrenal Hyperplasia & Ectopic Cushing's
Poster Board SUN-37
Pregnancy is uncommon in women with Cushing’s syndrome (CS). Both clinical and biochemical diagnoses of CS are difficult due to overlapping features and physiological hypercortisolism of pregnancy. Adrenal adenomas underlie a disproportionately high proportion of CS cases in pregnancy. Therefore, anovulation may be less prevalent or possibly presence of unrecognized ectopic luteinizing hormone (LH) /human chorionic gonadotropin (hCG) receptors stimulated by high hCG levels in pregnancy may be contributory
Retrospective review of 2 patients with CS during pregnancy and 6 non-pregnant patients with CS were selected for comparison from database. Pathological analysis of surgically resected adrenal adenomas was carried out to assess the LH/hCG receptor status via immunohistochemical (IHC) staining. Hematoxylin and eosin stained microscopic slides were reviewed and a representative area within each tumor was chosen. IHC studies were performed on 4 micron formalin-fixed, paraffin-embedded tissue sections using Ventana Benchmark XT. Following pretreatment with Protease 2 (LH) or CC1 (hCG), primary antibodies against hCG (polyclonal) and LH (monoclonal) were applied for incubation period of 32 minutes at 37 °C using Ventana Ultravision (LH) or Optiview (hCG) with Ventana DAB chromogen.
Mean age (yrs.) of pregnant vs non-pregnant 6 female patients was 22.5±2.1vs 40.1±14. The index pregnant patient presented at 23 weeks gestation, the 2ndpatient noted symptoms in pregnancy but was evaluated after delivery. Laboratory data in pregnant vs non-pregnant patients included mean adrenocorticotropic hormone (ACTH)(pg/ml) of 4.5±0.7 vs 7.8±2.6, mean morning serum cortisol(ug/dl) of 29±14 vs 21.5±6, mean 24-hr urine free cortisol(mcg) of 611±703 vs 193±156.The index patient underwent abdominal MRI, remainder of patients had CT imaging. All 8 cases were treated via laparoscopic adrenalectomy. Mean tumor size (cm) in pregnant vs non-pregnant patients was 3.25±1.2 vs 3.5 ±0.9. Both pregnant patients delivered healthy babies albeit pre-term. Postoperatively, glucocorticoid replacement therapy (days) was required for 322 ±60.1 in pregnant patients and 110 ±103 in non-pregnant patients. IHC analysis of both groups of patients was negative for LH and hCG staining.
LH/hCG receptor analysis via IHC was negative in adrenal tumors causing CS. Possible considerations would be to further evaluate the receptor status using mRNA analysis and evaluation of additional possible ectopic receptors.
Disclosure: DZE: Advisory Group Member, Ipsen. Nothing to Disclose: NWG, LE, GBT
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