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.
24 - 28 Apr 2010
European Society of Endocrinology