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An eleven-year experience with adrenocortical carcinoma.

BACKGROUND: Key issues in the treatment of adrenocortical carcinoma are the value of adjuvant therapy, the value of reoperation, and the search for effective chemotherapeutic agents. The present series was reviewed to evaluate these issues. METHODS: We present a retrospective series of 73 patients with adrenocortical carcinoma treated at a single institution. RESULTS: Twenty patients had carcinomas that were unresectable, and 53 patients underwent complete resections. Ten patients received adjuvant therapy (mitotane, seven patients; radiation, three patients). Forty-five (85%) patients had recurrence, including all who received adjuvant therapy. Mean disease-free intervals for those who did and did not receive adjuvant therapy were equivalent at 2.4 years. Nineteen patients with recurrent disease received chemotherapy, and 26 patients underwent 51 reoperations to resect recurrent and metastatic disease. The overall 5-year survival rate, which was 35%, was 47% for patients with complete resection. Stage and resectability were prognostic factors. Mean survival time for patients with recurrent disease treated medically was 19 months compared with 56 months for patients who underwent reoperation. Mitotane had a 24% partial response rate. Other chemotherapeutic agents were ineffective. CONCLUSIONS: We conclude that an aggressive surgical approach to recurrent and metastatic disease should be adopted and that patients should be resected free of disease whenever possible. Currently no effective chemotherapy exists, and the value of adjuvant therapy remains unproved.

Adrenal Cortex Hormones↗

[Adrenal incidentaloma in a patient operated upon for colon carcinoma].

The extensive use of visualization techniques has allowed the finding of adrenal masses without clinical symptoms in a growing number of patients affected by diseases of different nature. The challenge of these masses, so called incidentalomas, is particularly diagnostic but also prognostic and therapeutic. A flow-chart of proper investigations utilized in a tumoral patient harbouring a left adrenal mass, permitted the correct diagnosis and prognosis.

Adenoma↗

[Study of a virilizing adrenal cortical tumor. Analysis of urinary steroids by gas-liquid chromatography and mass spectrometry].

The C19 and C21 urinary steroids from a virilizing adrenal tumour with high levels of plasma 17alpha-progesterone and its urinary metabolites have been identified and quantitated by gas chromatography and mass spectrometry of sephadex fractions of the total urinary extract. Of the fifty five identified steroids thirteen were new compounds or known compounds not found before in such a case. The actiology of the apparent 21-steroid hydroxylase deficiency is discussed at the light of these analytical results and of the hormonogenesis enzymatic induction of the tumour biopsy.

Adenoma↗

[A case of non-functioning adrenocortical tumor].

A resected case of a non-functioning adrenocortical tumor is reported. The patient's chief complaint was fever and general fatigue. Computed tomography and ultrasonography revealed the suspected presence of a tumor on the right lobe of the liver, though a definite diagnosis of a right adrenal tumor was reached after hepatic angiography. An endocrinal examination, however, showed no abnormality. The tumor was dissected sharply from the right hepatic lobe and completely removed with a part of the diaphragm. It measured 10 x 12 x 18 cm and weighed 1,280 g. Hemorrhagic and necrotic changes were noted in the cut section, and a subsequent pathological examination revealed an adenocarcinoma of the adrenal cortex. An administration of 5-FU was given orally and the patient's progress has gone well without a recurrence or a metastasis for 4 years and a month since the surgery.

Adenocarcinoma↗

Gynaecomastia and azoospermia as sole presenting symptoms of feminizing adrenal tumor.

Adrenal feminizing tumours are rare but frequently malignant. A case of adrenal feminizing tumour in a 35-year-old man, which presented with gynaecomastia and infertility due to azoospermia, without loss of libido and sexual potency is reported in this paper. Our patient represents a relatively unusual case. The long free interval from the excision allows us to consider the patient cured as despite the pathological malignant features of the neoplasm.

Adrenal Cortex Hormones↗

Pregnancy and Cushing's syndrome: example of endocrine interaction.

Pregnancy rarely occurs in women with Cushing's syndrome, and when it does, fetal mortality and morbidity are very high. We describe a 30-year-old woman who was found to have severe Cushing's syndrome in the 22nd week of her first pregnancy, after a year of unsuccessful attempts to conceive. The patient had the majority of the symptoms and signs characteristic of the syndrome. Laboratory examinations revealed hypokalemia of 2.7 mEq/l, serum cortisol 39.5 micrograms/dl without diurnal variation, free urinary cortisol 1,850 to 3,500 micrograms/24 h, 17-hydroxycorticosteroids (OHCS) 52.5 mg/24 h, 17-ketosteroids (KS) 12 mg/24 h, and ACTH 29 pg/ml. No suppression was observed upon dexamethasone administration (2 and 8 mg). Ultrasound examination of the adrenal glands revealed a left adrenal tumor with a diameter of 4.2 cm. An adrenocortical adenoma was successfully excised in the 24th week of pregnancy. During the 37th week of pregnancy, she delivered a normal baby girl. Postoperatively, the patient was put on maintenance therapy. One year after delivery, mother and child are in perfect health.

Adenoma↗

Cecal perforation and adrenocortical adenoma in a dog.

Cecal perforation was diagnosed in a dog with a history of acute vomiting. The dog also had an adrenocortical adenoma. Intestinal perforation can be a serious complication of cortico-steroid treatment in the dog, but has not been attributable to hyperadrenocorticism. Fever and an inflammatory CBC were not observed, which could have been secondary to adrenal-dependent hyperadrenocorticism. The acute abdominal crisis associated with peritonitis required quick resolution in an attempt to save the dog, but also precluded any further diagnostic procedures for possible hyperadrenocorticism. The signs that suggested hyper-adrenocorticism in this dog included alopecia, lymphopenia, eosinopenia, high liver enzyme activities, hypercholesterolemia, and one large and one small adrenal gland. This latter finding presumably indicated negative feedback suppression and atrophy attributable to a functional adrenocortical adenoma.

Adenoma↗