Unité d'Hypertension Artérielle, Assistance Publique-Hôpitaux de Paris, Hôpital Européen Georges Pompidou, 20-40 rue Leblanc, F-75015 Paris, France.
Eur J Endocrinol. 2012 Jan;166(1):131-5. doi: 10.1530/EJE-11-0791. Epub 2011 Nov 2.
Primary adrenal angiosarcoma is an extremely rare neoplasm, as are combined tumors within a given adrenal lesion.
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.
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.
To the best of our knowledge, this is the first case of an adrenal neoplasm combining a primary angiosarcoma and a functioning adrenocortical adenoma.
原发性肾上腺血管肉瘤是一种极其罕见的肿瘤，而在同一肾上腺病变中出现的混合性肿瘤也非常罕见。
一名 35 岁男性因低钾性高血压就诊，检查发现一个 6 厘米直径的恶性右肾上腺肿瘤。该病变在正电子发射断层扫描（PET）扫描中显示出明显的（18）F-氟脱氧葡萄糖摄取。内分泌检查显示肿瘤同时分泌皮质醇和醛固酮。在腹腔镜手术中，整个右肾上腺连同肾上腺周围脂肪组织被切除。
组织学检查显示两种交织在一起的肿瘤细胞增生，即血管肉瘤和肾上腺皮质腺瘤。广泛的术后检查未发现其他原发性肿瘤部位或转移灶。因此，该病变被认为是原发性肾上腺血管肉瘤合并分泌性肾上腺皮质腺瘤。患者接受了四个周期的化疗（阿霉素/异环磷酰胺）。在 2 年的随访中，他情况良好，无复发迹象。
据我们所知，这是首例同时合并原发性血管肉瘤和功能性肾上腺皮质腺瘤的肾上腺肿瘤病例。
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