Adrenocortical carcinoma is a rare disease, its estimated incidence being 12/million per year in general population. Age distribution shows two peaks: the first in early childhood (70% of patients having a benign evolution), and the second between 40 and 50 years of age (with a dismal prognosis).
The 38-years-old male patient (C A) hospitalized in our Clinic in September 2008 presented hypertension (to 250/150 mmHg) for about 3 months, lumbar pain with irradiation into left fossa iliaca, weight loss, anorexia and anxiety. Both abdominal US and CT-scan showed a great tumor mass (124×111 mm) in the left adrenal gland that dislocated the pancreas and the left kidney, so we supposed firstly a phaeochromocytoma or an adrenal adenoma. The investigations (normal urinary AVM and metanephrines, slightly elevated basal cortisolaemia, responding normal to the suppression with DXM 1 mg, normal ACTH, DHEA-S, aldosterone) did not confirm our presumptions. 17-OH-progesterone was slightly elevated (3.82 ng/ml, normal range: 0.52.1 ng/ml). After an adequate cardiovascular stabilization the patient underwent operation in December 2008. The histology completed with immunohistochemistry showed a primary adrenocortical carcinoma with sarcomatous transformation, a very rare form of adrenocortical malignant tumors. The abdominal CT-scan did not find local recurrence or metastases in April 2009, and the patient did not receive chemotherapy. In October 2009 a second surgery was needed for a local recurrence (190×140×100 mm) and pancreatic metastases. In November 2009 the CT-scan showed a tumor mass of 27 mm in diameter in the left suprarenal region and peritoneal microlymph nodes (34 mm). Post-surgical chemotherapy was recommended.
In the accessible literature only seven cases have been detailed until 2007, three of them with osteo-, chondro- or rhabdomyosarcoma components, and four others only with sarcomatous spindle cell component. The case is discussed under different aspects (nosological evaluation, differential diagnosis, management and prognosis), based on recent references.
Prague, Czech Republic
24 - 28 Apr 2010
European Society of Endocrinology