Epithelioid angiosarcoma of the adrenal gland with metastasis: a case report and literature review

Abstract

Primary adrenal epithelioid angiosarcoma is an exceptionally rare condition, with only 51 cases reported in the literature since 1988.

Case presentation: We report a case of a 59-year-old male patient who presented with a 4-month history of left flank pain and anemia. Radiographic imaging identified a 14 cm mass lesion in the left suprarenal region, which showed heterogeneous enhancement. The patient underwent total adrenalectomy with tumor-free surgical margins. Histological sections showed features consistent with an epithelioid tumor, and immunohistochemical staining confirmed the diagnosis of epithelioid angiosarcoma of the left adrenal gland.

Discussion: Primary adrenal epithelioid angiosarcoma is a very rare entity. It was first described by Kareti et al. in 1998. The most common presentation is an abdominal mass associated with pain. As there are no specific imaging findings for this tumor, histology combined with immunohistochemistry is the most definitive diagnostic method. Surgery with adjuvant chemotherapy is the management reported for previous cases.

Conclusion: In cases of rare malignancies, interdisciplinary collaboration is crucial for determining the optimal management strategy.

Keywords: adjuvant therapy; adrenal gland; angiosarcoma; case report; immunohistochemistry.

Figure 1

Sagittal, axial, and coronal computed tomography (CT) images showing heterogenous left apical mass. Sagittal (A), axial (B), and coronal (C) CT images show a solid tumor measuring 17 cm in diameter at the upper pole of the left kidney, with peripheral calcifications. It was found to be abutting the spleen and displacing the left kidney inferiorly, as well as the tail of the pancreas anteriorly.

Figure 2

Macroscopic examination. An encapsulated mass involving the entire adrenal gland measuring 14×13×10 cm. Step-sectioning revealed a friable cut surface and grayish-white areas with regions of hemorrhage and necrosis.

Figure 3

Histological examination. (A) Histological images (4× magnification) of the formalin-fixed paraffin-embedded sections from adrenal gland tissue infiltrated by malignant vascular tumor, that is angiosarcoma. (B) Histological images (10× magnification) of the formalin-fixed paraffin-embedded sections from adrenal gland tissue infiltrated by malignant vascular tumor, that is angiosarcoma. (C) Histological images (10× magnification) of the formalin-fixed paraffin-embedded sections from the tumor showing malignant proliferation formed of intercommunicating vascular spaces lined by malignant epithelioid cells with interstitial hemorrhage.

Figure 4

Immunohistochemistry slides. (A) Histological images (10× magnification) of immunohistochemically stained slide of CD31 showing the diffuse positive reaction of tumor cells. (B) Histological images (10× magnification) of immunohistochemically stained slide of FLI-1 showing the diffuse positive reaction of tumor cells. (C) Histological images (10× magnification) of immunohistochemically stained slide of Ki67 showing a high (more than 50%) proliferative index of tumor cells. (D) Histological images (10× magnification) of immunohistochemically stained slide of inhibin showing positive residual adrenal tissue with negative staining of tumor cells.

Figure 5

Positron emission tomography images at the time of diagnosis. (A) The mass exhibited minimal nonspecific heterogeneous fluorodeoxyglucose (FDG) activity and measured ~7×4.5 cm in its maximal axial dimensions. (B) The scan revealed multiple bilateral pulmonary nodules throughout both lung fields, showing variable degrees of FDG avidity, suggesting a metastatic process.

Figure 6

Positron emission tomography after treatment. (A) No definitive hypermetabolic lesion at the anatomical area of the primary lesion. There is slight fluorodeoxyglucose (FDG) activity which is consistent with postoperative changes. (B) Complete resolution of the previously seen hypermetabolic pulmonary nodules.