Multi-organ resection for locally advanced adrenocortical cancer: surgical strategy and literature review.

Adrenocortical carcinoma (ACC) is a rare and aggressive endocrine malignancy with an estimated worldwide incidence of 0.5-2 per million/year. Complete surgical removal of ACC represents the current treatment of choice for this tumor. A disease-free resection margin (R0) is an important predictor of long-term survival: surgery is demanding and must be performed by a highly experienced surgical team. We report the surgical strategy adopted in a patient with locally advanced ACC and virilization to obtain a R0 resection.

Mature teratoma, a rare cause of virilization in adolescence.

Virilization in an adolescent patient can occur for multiple reasons (ovarian, suprarenal or exogenous reasons). We describe a 14-year-old patient with 1-year secondary amenorrhea, who had an ovarian mature teratoma as a cause of her clinical history.

A case of hypertension with virilisation during pregnancy.

This case report describes the onset of hypertension and virilisation during pregnancy due to a Brenner tumour.

[Maternal virilization due to mucinous ovarian tumour: a case report]. / Tumeur ovarienne mucineuse virilisante et grossesse: à propos d'un cas.

Virilization in pregnancy is rare and mostly due to luteoma or to hyper-reactio luteinalis. We present a rare case of a virilization borderline mucinous ovarian tumour on a gravida 1 patient. The tumour was responsible for a clinical hyperandrogenism and for an increased level of testosterone. This patient was treated by ovariectomy at 31 weeks of gestation. The surgery was completed one month after delivery. There was no fetal consequence and the clinical and biological signs of virilization totally disappeared after surgery.

[Virilisation due to an ovarian mucinous cystadenoma]. / Virilisatie door een ovarieel mucineus cystadenoom.

An 82-year-old postmenopausal woman presented with severe clinical hyperandrogenism related to testosterone overproduction, possibly as a result of a mucinous cystadenoma. The cystadenoma was successfully removed in toto. The patient was discharged in good health. Plasma testosterone levels normalised 6 weeks after surgery. Ovarian mucinous cystadenomas are a rare cause ofhyperandrogenism.

Disappearance of a virilizing adrenal tumor following therapy with cyproterone acetate.

A 57-year-old woman presented with an apparently obvious diagnosis of iatrogenic virilization. At the age of 51, she began a 4-year treatment with prednisone or cyclosporine, which are known to promote hair growth, for Behçet disease. At the age of 56, osteoporosis was overtreated with the anabolic steroid nandrolone. Insignificant inhibition by dexamethasone of the extremely high serum concentrations of testosterone and less high concentrations of weak androgens prompted us to search for a virilizing tumor. Computed tomography showed a 2.3 x 1.5 cm nodule in the right adrenal gland. As the patient refused surgery, virilization was treated with the antiandrogen cyproterone acetate (CPA), but for only 4 months because clinical and hormone abnormalities reversed and the tumor was no longer visible. The patient remains symptom-free. This first report of a curative effect of CPA on a purely virilizing adrenal tumor opens new avenues in the management of such tumors.

Fetal endocrine therapy for congenital adrenal hyperplasia should not be done.

Prenatal treatment of congenital adrenal hyperplasia by administering dexamethasone to a woman presumed to be carrying an at-risk fetus remains a controversial experimental treatment. Review of data from animal experimentation and human trials indicates that dexamethasone cannot be considered safe for the fetus. In animals, prenatal dexamethasone decreases birth weight, affects renal, pancreatic beta cell and brain development, increases anxiety and predisposes to adult hypertension and hyperglycemia. In human studies, prenatal dexamethasone is associated with orofacial clefts, decreased birth weight, poorer verbal working memory, and poorer self-perception of scholastic and social competence. Numerous medical societies have cautioned that prenatal treatment of adrenal hyperplasia with dexamethasone is not appropriate for routine clinical practice and should only be done in Institutional Review Board approved, prospective clinical research settings with written informed consent. The data indicate that this treatment is inconsistent with the classic medical ethical maxim to 'first do no harm'.

Maternal side effects of prenatal dexamethasone therapy for fetal congenital adrenal hyperplasia.

UNLABELLED: Prenatal treatment of virilizing congenital adrenal hyperplasia in female fetuses via maternal dexamethasone (Dex) therapy (1-1.5 mg/day) from first trimester to birth was associated with excessive weight gain (47-56 pounds at 35-37 weeks gestation), Cushingoid facial features, severe striae resulting in permanent scarring, and hyperglycemic response (8-11.7 nmol/L) to oral glucose administration in all our experience (three cases). Other symptoms included hypertension, gastrointestinal intolerance, or extreme irritability. Previous pregnancies were uncomplicated by these problems. In each case, first or second trimester amniotic fluid 17-hydroxyprogesterone (17OHP, 17-41 nmol/L; normal less than 0.4 nmol/L), androstenedione (22-31 nmol/L, normal less than 5 nmol/L), and testosterone levels (0.54-0.7 nmol/L, normal less than 0.4 nmol/L) during Dex treatment were elevated regardless of the newborn genital outcome. Maternal serum estriol (E3) levels in one mother (normal newborn genitalia) were consistently low (less than 0.2 nmol/L) during the second and third trimester. In two mothers (partially virilized newborn genitalia) initial second trimester E3 levels were unsuppressed (11, 17.4 nmol/L) and suppressed (less than 1.4 nmol/L) following short-term increased dose. CONCLUSION: prenatal Dex treatment of virilizing congenital adrenal hyperplasia at a dose of 1-1.5 mg daily throughout gestation is associated with significant maternal side effects. Parents should be informed of these side effects before initiation of treatment. Careful monitoring for signs of side effects, medical intervention when necessary, and lowering of Dex dose during the second half of gestation would minimize the side effects. Maternal serum E3 levels appear useful for prediction of fetal outcome while amniotic fluid steroid levels may not.

Pregnancy luteomas: clinical presentations and management strategies.

UNLABELLED: Pregnancy luteomas are rare, benign, neoplasms of the ovary thought to be caused by the hormonal effects of pregnancy. They are usually asymptomatic and found incidentally during imaging or surgery. However, they may present with virilization of the mother or infant or cause complications due to a mass effect or hemorrhage secondary to torsion. Luteomas spontaneously regress postpartum. We present a case of a woman with the classic presentation of a pregnancy luteoma and the common challenges of subsequent management. Pregnancy luteomas present a diagnostic and management challenge in that they can mimic the presentation of malignant ovarian tumors. There have been fewer than 200 case reports of pregnancy luteomas and a relative paucity of data to provide guidance for clinical management. However, certain general principles emerged from a review of modern cases. Management of pregnancy luteomas depends on the clinical situation. Luteomas present most commonly in the second half of pregnancy, with a solid ovarian mass that is frequently bilateral, elevated testosterone levels and some aspects of virilization. With high clinical suspicion for pregnancy luteoma, clinical monitoring and postpartum radiologic follow-up may be an appropriate management strategy to avoid unnecessary surgery. However, in some cases with atypical presentation or with complications from the mass, surgical intervention may be necessary for diagnostic or management purposes. Patients who present in the first half of pregnancy generally have more severe symptoms and are more likely to require surgical intervention for management of mass effect. When there is a high clinical suspicion for pregnancy luteoma, conservative management is appropriate since these tumors will usually regress spontaneously. After completing this CME activity, obstetrician/gynecologists should be better able to evaluate clinical presentations of pregnancy luteomas, examine the complexities involved in diagnosing neoplasms suspicious for pregnancy luteoma, and counsel patients about appropriate management and treatment options. TARGET AUDIENCE: Obstetricians & Gynecologists, Family Physicians, Pediatricians Learning Objectives: After completion of this article, the reader should be better able to evaluate the clinical presentations of pregnancy luteomas; examine the complexities involved in the diagnosis of neoplasms suspicious for pregnancy luteoma; and counsel patients on appropriate management and treatment for suspected luteomas.

Ovarian sex cord-stromal tumor, unclassified: case report of endocrinologic findings and review of the literature.

Endocrinologic findings and a successful pregnancy in a 39-year-old woman with significant androgen excess due to a biologically active, virilizing ovarian sex cord-stromal tumor, unclassified, are described. She had secondary amenorrhea, infertility, and hirsutism. Her basal plasma testosterone level was high at 187 ng/mL (normal range: 10-85 ng/mL). Tumor resection resulted in resumption of ovulation, pregnancy, and delivery.

Congenital adrenal hyperplasia due to 11 beta-hydroxylase deficiency: final diagnosis in adult age in three patients.

Three patients, one male and two females, in whom the diagnosis of congenital adrenal hyperplasia (CAH) was made in early childhood were studied. The two females were treated with cortisone acetate from the age of 2 and 4 years, respectively, and later they both proved to be fertile. The male patient was only treated sporadically with cortisone acetate until the age of 33 years. He also became fertile when a more consistent treatment with prednisolone was started. The two female patients had a slight hypertension in childhood before the treatment was initiated but became normotensive on treatment. The male patient revealed a blood pressure of180/130 mm Hg at the age of 33 years. In this patient the treatment with prednisolone produced a moderate decrease in the blood pressure, but additional treatment with antihypertensive drugs was needed to make him normotensive. All three patients were originally thought to have a 21-hydroxylase deficiency and the correct diagnosis of an 11 beta-hydroxylase deficiency was first established between the age of 26 and 33 years. It is concluded that an early diagnosis and an uninterrupted treatment with glucocorticoids are important in order to prevent hypertension and infertility problems.

Chlormadinone acetate as a possible effective agent for congenital adrenal hyperplasia to suppress elevated ACTH and antagonize masculinization.

We report two cases of congenital adrenal hyperplasia (CAH) in which administration of chlormadinone acetate (CMA), a substituted progestational agent for prostatic disease, suppressed ACTH hypersecretion and lowered plasma testosterone levels. Case 1 was 83-year-old male with advanced prostatic carcinoma and CAH due to 21-hydroxylase deficiency. His plasma testosterone did not decrease in spite of a bilateral orchiectomy. Case 2 was 40-year-old female with CAH due to 21-hydroxylase deficiency suffering from virilization after the cessation of cortisol supplement therapy because of her breast carcinoma. In these two cases, oral administration of CMA at a daily dose of 75-100 mg suppressed ACTH and cortisol to subnormal levels and reduced testosterone levels. With the suppressive effect on ACTH excess and antiandrogenic action, CMA may be suitable for patients with CAH suffering from symptoms due to overproduced ACTH or adrenal androgen.

Recurrent adrenocortical carcinoma in a 4 year old girl.

Adrenocortical tumours are rare in the paediatric age group. This is a report of a 4 year old girl with recurrent adrenocortical carcinoma emphasizing the role of surgery as the definitive form of therapy even in recurrent cases.