J Pathol Transl Med.  2022 May;56(3):161-166. 10.4132/jptm.2021.12.28.

Adrenal hemangioblastoma

• Koo JY
• Lee KH
• Choi JH
• Chung HS
• Choi C

• KMID: 2529808
• DOI: http://doi.org/10.4132/jptm.2021.12.28

Abstract

Hemangioblastoma (HB) is a rare benign tumor that most commonly occurs in the cerebellum. HB is composed of neoplastic stromal cells and abundant small vessels. However, the exact origin of stromal cells is controversial. Extraneural HBs have been reported in a small series, and peripheral HBs arising in the adrenal gland are extremely rare. Herein, we report a case of sporadic adrenal HB in a 54-year-old woman. The tumor was a well-circumscribed, yellow mass measuring 4.2 cm in diameter. Histologically, the tumor was composed of small blood vessels and vacuolated stromal cells with clear cytoplasm. On immunohistochemical stain, the stromal cells were positive for S-100 protein, neuron-specific enolase, and synaptophysin. The tumor did not reveal mutation of VHL alleles. We herein present a case of HB of the adrenal gland and review of the literature.

Keyword

Hemangioblastoma; Adrenal glands; von Hippel-Lindau disease; Immunohistochemistry; Sequencing

Figure

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  Fig. 1 (A) Magnetic resonance imaging reveals a 4.2 × 3.6 × 4.2 cm mass in the left adrenal gland. (B) Gross examination shows an encapsulated nodular mass.

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  Fig. 2 The tumor is encapsulated by a fibrous wall (A) and is composed of alternating hypercellular and hypocellular areas (B). Vascular structures with vacuolated stromal cells (C, D) are observed.

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  Fig. 3 Immunohistochemical staining shows that the vascular endothelial cells are positive for CD31 (A). The stromal cells are positive for NSE (B), S-100 protein (C), and synaptophysin (D). However, they are negative for chromogranin A (E) and GATA3 (F).

Reference

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