Angiosarcoma of the adrenal gland with concurrent contralateral advanced renal cell carcinoma: A diagnostic and management dilemma

Abstract

Angiosarcoma is a rare high-grade malignant neoplasm with poor clinical outcome and survival rates, occurring most commonly in the skin and soft tissue. It is composed of neoplastic cells that demonstrate endothelial differentiation. The diagnosis of angiosarcoma can be difficult due to its pathohistologic presentation as a poorly differentiated neoplasm with associated secondary changes. We report a case of angiosarcoma of the adrenal gland with concurrent contralateral renal cell carcinoma (RCC) and renal vein thrombus. The presumptive clinical diagnosis was metastatic renal cell carcinoma to the contralateral adrenal gland. Pathohistologic evaluation demonstrated massive hematoma associated with intravascular papillary endothelial hyperplasia (IPEH)-like features. We discuss the pathohistological features used to ascertain a diagnosis of angiosarcoma in the presence of IPEH-like changes and differentiate it from reactive vascular proliferation seen in IPEH (Masson's tumour).

A case report of primary adrenal angiosarcoma as depicted on magnetic resonance imaging

“…Previous researches showed that adrenal angiosarcoma tends to be a round or lobulated, heterogeneously low attenuation mass on unenhanced CT images, in which the hyper-attenuating area corresponds to acute hemorrhage or calcification, while the hypo-attenuating area represents necrosis which does not enhance after contrast administration. [ 4 , 5 , 11 , 14 – 16 ] The case reported here also has a round shape with clear boundary, which may be explained by the complete capsule in pathology. Heterogeneous intensity was demonstrated on unenhanced MRI images.…”

“…[ 2 , 3 ] Advanced age, increased tumor size, a retroperitoneal primary site, and a proliferative index >10% are considered to be adverse prognostic factors, and low-grade angiosarcoma has a better prognosis than high-grade angiosarcoma. [ 2 , 3 , 5 ]…”

“…In view of the aggressive nature of angiosarcoma in all sites, adjuvant paclitaxel-based chemotherapy or radiotherapy appears justified for patients in whom complete surgical extirpation cannot be ensured. [ 2 , 3 , 5 ]…”

Angiosarcoma of the Adrenal Gland Treated Using a Multimodal Approach

“…Patients present in a variety of ways including flank or abdominal pain, palpable mass, fatigue, weight loss, fever, or clinically asymptomatic with a mass detected on incidental imaging. Table 1 summarizes the age, sex, type of presentation, treatment, and follow-up for previously reported cases 2, 3, 4, 5, 6, 7, 8, 9, 10, 11, 12, 13, 14, 15, 16, 17, 18, 19, 20, 21, 22, 23, 24, 25, 26, 27. Patients who present with systemic symptoms tend to have worse outcomes than those with localized symptomatology.…”

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].…”