Endocrine Abstracts

Introduction: Angiosarcomas account for < 1% of all sarcomas, and are highly agressive neoplasms whose clinical course is striking: local recurrence, metastasis, and a high mortality rate. Primary angiosarcoma of the adrenal gland was first described in 1988 by Kareti et al. and is very rare with, so far, only 51 reported cases.

Case report: A 49-year-old male, without prior malignancy, presented with a 4.9x5.9 cm right adrenal nodule and a 2.4 cm lesion at L1 both found incidentally on a chest CT. His mother died with lung cancer. Physical examination was unremarkable. Routine lab tests were within the normal range. Serum aldosterone, renin, DHEAS, cortisol post 1 mg dexametasone supression test, 24H urinary catecholamines and metanefrines were normal; 24H urinary normetanephrine slightly elevated. An abdominal MRI confimed an heterogeneous lesion 5.5x4.7x4.4 cm on the right adrenal gland with areas of possible hemorraghe. An MRI of the column revelead a 2.1x1.3x2.2 cm lesion at L1 without agressive features. No uptake was shown at MIBG scan. Right laparoscopic adrenalectomy was done. The adrenal gland (76 g and 8.2x5.8x3.4 cm) disclosed a 4.0x2.6x5.0 cm well defined mass with hemorraghic areas. On microscopic examination, the mass consisted mostly of hematoma and scattered small agregates of cytologically atypical epithelioid cells with amphophilic cytoplasm, irregular vesicular nuclei with variably prominent nucleoli and some mitoses; the cells were diffusely positive for CD31 and ERG. The features are characteristic of epihelioid angiosarcoma. After surgery, a FDG-PET scan disclosed 2 foci of uptake, one on the right thyroid lobe (SUV max 3.7) and the other at the L1 lesion (SUV max 3.2). The cervical US revealed a heterogeneous thyroid gland with characteristics of thyroiditis. Anti microssomal antibodies positive, TSH normal. A biopsy of the L1 lesion did not reveal malignancy. Because there were complete resection margins and no metastic foci were found no adjuvant chemotherapy was done. The patient has been kept under clinical and imagiological surveillance for three years with no further evidence of disease.

Discussion: Adrenal angiosarcoma is a very rare clinical entity with a propensity for local recurrence and metastasis and a median survival of 18 months. Noteworthy the prognosis is unpredictable but some times quite good as in this case.