Primary adrenal angiosarcoma and functioning adrenocortical adenoma: an exceptional combined tumor

Abstract

Context: Primary adrenal angiosarcoma is an extremely rare neoplasm, as are combined tumors within a given adrenal lesion. Clinical presentation and intervention: A 35-year-old man presented with hypokalemic hypertension leading to the discovery of a 6 cm diameter malignant-appearing right adrenal tumor. The lesion displayed marked 18 F-fluorodeoxyglucose uptake on positron emission tomography scanning. Endocrine investigations revealed secretion of both cortisol and aldosterone by the neoplasm. The entire right adrenal gland along with the periadrenal fat tissue was removed during laparoscopic surgery. Results: Histological examination revealed two intermingled tumor cell proliferations, namely an angiosarcoma and an adrenocortical adenoma. An extensive post-operative search revealed no other primary tumor site, nor metastases. The lesion was then considered to be a primary adrenal angiosarcoma combined with a secreting adrenocortical adenoma. The patient received four cycles of chemotherapy (adriamycin/ifosfamide). At 2-year follow-up, he is alive and well, with no sign of relapse. Conclusion: To the best of our knowledge, this is the first case of an adrenal neoplasm combining a primary angiosarcoma and a functioning adrenocortical adenoma.

Uterine Epithelioid Angiosarcoma on F-18 FDG PET/CT

“…[4] F-18 FDG PET/CT experiences in imaging angiosarcoma at anterior mediastinum, vessel, epicardium and adrenal gland were reported. [14][15][16][17] These reports have described variable F-18 FDG avidity of epithelioid angiosarcoma, which were mild to intense in F-18 FDG uptake (SUVmax up to 9.7). To our knowledge, the F-18 FDG PET/CT findings of primary uterine angiosarcoma have not been previously reported.…”

Adrenal Gland Hemangiosarcoma in a Patient with Chronic Myeloid Leukaemia

“…According to Rosen et al (1988), MeisKindblom and Kindblom (1998) and Deyrup et al (2009) (Kareti et al, 1988;Livatidou et al, 1991;Bosco et al, 1991;Wenig et al, 1994;Otal et al, 1999;Ben Izhak et al, 1999;Krüger et al, 2001;Croitoru et al, 2001;Invitti et al, 2001;Pasqual et al, 2002;Lepoutre-Lussey et al, 2012;Derlin et al, 2012).…”

“…More extensive initial surgery was considered in a case reported by Lepoutre-Lussey et al in 2012. They presented a 35 year-old man with combined PAA and functioning adrenocortical adenoma.…”

Fine-needle aspiration accuracy in the diagnosis of primary epithelioid angiosarcoma of the adrenal gland: a case report and review of the literature

“…No increase in adrenal gland hormones production is noted in most of the reported cases, even though two patients had Cushing's syndrome [9,15] and in three patients the disease was discovered for the sudden onset of hypertension, despite none of them had increased plasmatic aldosterone or metanephrines [26,31,33].…”

“…The etiology of EA remains unknown: apart from a case of exposure to arsenic [6] and a case of exposure to vynil chloride [29], the published case reports didn't succeed in identifying a clear correlation with the patients' exposure to carcinogens or with a significant incidence of other diseases, even though three patients had concomitant adrenal cortical adenoma [26,31,32] (Table I). The disease occurs more frequently in males (M/F= 28/9), ranges from 34 to 85 years of age, with a mean peak at 57 years.…”