Adrenocortical carcinoma and pregnancy: clinical and biological features and prognosis

Abstract

Objective: Adrenocortical carcinoma (ACC) is a rare, severe disease. Pregnancy-associated ACC has rarely been reported. We wished to evaluate the characteristics and prognosis of ACC diagnosed in patients during pregnancy or in the postpartum period, comparing them with those for ACC diagnosed in nonpregnant women. Design: Clinical presentation, hormonal secretion, staging, survival, and obstetric data are reported. Patients were included between 1963 and 2007. Mean follow-up was 48 months. Patients and methods: This is a retrospective cohort study carried out at a referral center. All female patients aged 16-49 years diagnosed with ACC during the observation period were included (nZ110). Twelve of these women were pregnant or in the first 6 months after delivery. Hormonal secretion, staging, obstetric data, and survival were analyzed. For the survival analysis, pregnant patients were compared with a subgroup of nonpregnant women matched for age, stage, and year of diagnosis (1 pregnant patient/2 controls). Results: Adrenocortical tumors diagnosed during pregnancy or in the postpartum period tend to be more often cortisol-secreting tumors (PZ0.06) and to be discovered at a more advanced stage than those in nonpregnant women, although the differences were not significant. Fetal outcome was poor. Overall survival of the mother was worse than that of matched controls (hazard ratio of death: 3.98, confidence intervalZ1.34-11.85, PZ0.013). Conclusion: ACC diagnosed during pregnancy or in the postpartum period is associated with a poor fetal outcome and a poorer prognosis than ACC diagnosed in nonpregnant women.

A Pregnant Patient with Adrenocortical Carcinoma: Case Report

“…Tumors strictlylocatedwithintheadrenalglandaredefinedasstageI andII,dependingontumorsize(>5cmforstageII).Stage III means the neoplasm infiltrates the surrounding tissue or lymphnodesorbloodvessels,andstageIVisdeterminedby thepresenceofdistantmetastases.Tumorstageatdiagnosisis the best prognostic factor for overall survival [8]. The available literature shows that at the time of diagnosis 33% of pregnantpatientshadstageIIdisease,42%hadstageIII,and 25%hadstageIV [9].OurpatientwasconsideredasstageII ACC(5.9-cmmass),althoughlymphnodesamplingwasnot available.…”

“…With no adequate, wellcontrolledstudiesonpregnantpatients,seriousconsideration shouldbegivenpriortotheadministrationofthesedrugs [15][16][17][18]. Pregnancy outcomes for a mother with ACC and her fetus may be complicated by hypertension and diabetes mellitus in the former and by preterm birth and low birth weight,stillbirth,orabortioninthelatter [9].Despitepreterm birth,theyoungboyinourcasehasshownnolong-termproblemstodate.…”

“…Hypercortisolemia cannot be considered a factor affecting the results since survival remained grim even after tumor excision. As already mentioned, 10% of pregnant women with CS are found to have ACC.Hence,onemaywellspeculatethatpregnancyisarisk factor for ACC, or, in other words, pregnancy probably createsanenvironmentinwhichACCbehavesmoreaggressively [9].ThecareofapregnantwomanwithACCinvolves evaluationofcompetingmaternalandfetalrisksandbenefits, whichmustbeundertakenbyamultidisciplinaryteamincludingoncologists,endocrinologists,surgeons,andgynecologists. Early diagnosis and surgery are essential so that better outcomesmaybeachieved.…”

Adrenal cortical carcinoma mimicking early severe preeclampsia

“…The largest review series of 12 women (three diagnosed during pregnancy and nine diagnosed within six months of delivery) revealed that all 12 tumours secreted cortisol and nine tumours co-secreted androgens. 6 Pregnancy was uncomplicated in only four of the 12 pregnancies. Premature birth occurred in five pregnancies, intrauterine growth retardation in three pregnancies, one pregnancy was terminated because of severe preeclampsia with haemolysis, elevated liver enzymes and low platelets (HELLP) at 12 weeks gestation, and there was one intrauterine foetal death.…”

Management of Adrenal Tumors in Pregnancy

“…[92][93][94] More than half of all ACCs are hormone secreting, with cortisol and androgen secretion being the most common. 95,96 ACC is more common in women than in men. There is a bimodal age distribution with the first peak occurring in childhood and a second during the fourth to fifth decades.…”