Angiosarcoma of the Adrenal Gland Treated Using a Multimodal Approach

Abstract

Angiosarcoma of the adrenal gland is an extremely rare malignancy. We report a case of a 59-year-old female who presented with abdominal pain and profound anemia. A 7 cm enhancing, lipid-poor adrenal mass with calcifications that extended posterior to the vena cava was identified on imaging. Patient underwent right adrenalectomy with retroperitoneal lymph node dissection. Pathology demonstrated angiosarcoma of the adrenal gland. Consolidative treatment included adjuvant radiation and chemotherapy. Patient remains disease free 1.5 years following treatment. Prior reported literature on the diagnosis and management of adrenal angiosarcoma is reviewed.

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.…”

Adrenal Angiosarcoma: A Diagnostic Dilemma

“…It was first described by Kareti et al in 1988 [5]. It is more common in men, and occurs most frequently in individuals in the sixth and seventh decades of life [6]. Patients typically present with abdominal pain and/or flank pain, though symptoms can also include weight loss, fatigue or weakness or those who are asymptomatic with incidental findings on imaging.…”

A case of angiosarcomas which occurred in an adrenal gland and spleen synchronously

“…Primary adrenal angiosarcoma is extremely rare and was first reported by Kareti et al in 1988 [ 4 ]. Since then, an additional 40 cases have been reported in the literature with a median age at presentation of 60 (range, 34–85) years [ 5 ]. The most common symptom associated with adrenal angiosarcoma is an abdominal mass-accompanied pain.…”

“…Reportedly, the median survival for patients with localized disease is only 7 months [ 1 ]. Previously reported multimodal treatment approaches included doxorubicin-based chemotherapy and adjuvant radiation therapy [ 5 ]. Although we recommended adjuvant therapy for our patient, she refused it.…”

“…Hematogenous spread is considered the most probable route of spread, resulting in spleen metastasis [ 10 ]. Given the rarity of the disease, there is no consensus on the best treatment modality for adrenal and spleen angiosarcomas [ 5 ]; however, surgery is still the main stay of treatment [ 11 ] and it was considered that we might not be reluctant to perform surgery if patients consent to undergo surgery.…”