Primary Adrenal Angiosarcoma: A Rare and Potentially Misdiagnosed Tumor

Abstract

Summary A man aged 69 years presented with acute right flank pain secondary to a hemorrhagic large adrenal tumor. En bloc resection was performed to repair the inferior vena cava. Immunoperoxidase levels in the tumor were positive for factor VIII and CD31 and negative for S100, protein Melan-A, CD34, synaptophysin, chromogranin, desmin, muscle specific actin, ETFA (EMA), KRT20 (CK20), CDX2, TTF1, LNPEP (PLAP), inhibin, α-fetoprotein, CD30, hepatocyte paraffin, and aberrant expression of cytokeratin 7 and pankeratin. The pathological diagnosis was consistent with adrenal angiosarcoma. Obtaining appropriate immunoperoxidase stains and multidisciplinary evaluation helped make the diagnosis of this rare adrenal tumor and determine its management. The patient had an uneventful postoperative course and completed 4 cycles of adjuvant chemotherapy with doxorubicin/ifosfamide and adequately tolerated the treatment. However, positive surgical margins were found, so he was referred to radiation oncology specialists for possible adjuvant radiotherapy to the surgical bed. Weeks after the first initiation of therapy, the patient presented to the emergency department complaining of shortness of breath, fatigue, and generalized weakness for 3 days. He was admitted and found to have new-onset anemia and a new-onset, large, right pleural effusion. Thoracentesis performed showed sanguinolent fluid that, after microscopic evaluation, was suggestive of recurrent malignancy. Thoracic aortography performed with subselective catheterization to several arteries (right bronchial, right phrenic, and right renal arteries) did not show any active bleeding. However, the right inferior intercostal and adrenal arteries were presumed to be the reason for the bleeding event, so they were embolized until stasis. The patient remained hemodynamically unstable but eventually experienced multiorgan failure. In spite of aggressive measures, he died 10 days after his admission to the hospital.

Adrenal Angiosarcoma: A Diagnostic Dilemma

“…Predisposing risk factors for angiosarcomas include a history of familial angiodysplasia, chronic lymphedema, anabolic steroid use and exposure to thorium dioxide or radiation [2]. While the etiology of the majority of case reports of adrenal angiosarcoma is unknown, there have been a few case reports of adrenal angiosarcoma associated with abdominal fibromatosis and exposure to arsenic-containing insecticides and vinyl chloride [3]. CT or magnetic resonance imaging and biopsy with appropriate immunohistochemical staining is used to arrive at the diagnosis.…”

“…Finally, an incorrect diagnosis can be made if a wide immunohistochemical panel is not performed. These tumors typically stain positive for cytokeratin, an epithelial tumor marker, which can be seen in metastatic epithelial tumors or other mesenchymal neoplasms [3]. Positive endothelial markers such as CD31, CD34, Factor 8, and FLI1 performed on a wider immunohistochemical panel are needed to confirm the diagnosis of primary adrenal angiosarcoma (Figures 3A-3D) [3,9].…”

“…It accounts for less than 1% of all soft tissue sarcomas [1]. Skin and soft tissue angiosarcomas are more common than sarcomas that arise in the visceral organs or bone, which are typically sites of metastatic disease [2-3]. Primary angiosarcoma of the adrenal gland is an exceptionally uncommon malignancy with less than 50 cases reported in the literature.…”

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

“…Notably, none of the reported cases included hyperfunctioning tumors. Adrenal angiosarcoma often includes high-grade tumors possessing a potential to infiltrate and metastasize [ 2 ]. Adrenalectomy serves diagnostic and therapeutic purposes.…”

“…Angiosarcoma, a malignant endothelial-derived soft-tissue sarcoma, develops from blood or lymph vessels, with the most common locations being the head, neck, breast, and extremities [ 1 ]. Although surgical resection is the standard treatment for angiosarcoma, the 5-year survival rate remains at a dismal 30% [ 2 ]. Adrenal and spleen angiosarcomas are sporadic diseases, and adjuvant therapy such as chemotherapy or radiotherapy has been employed to address its aggressive biology.…”