Anastomosing Hemangioma of the Ovary With Leydig Cell Hyperplasia: A Clinicopathologic Study of 12 Cases

Abstract

Anastomosing hemangioma is a recently described vascular neoplasm, initially identified in the male genitourinary tract. Since its first description, it has been reported at multiple anatomic sites, including rare cases in the female genital tract, most in the ovary. Herein we report the largest series to date of 12 ovarian anastomosing hemangiomas identified at our institution over a 15-yr period. The patients’ age at the time of resection ranged from 50 to 76 yr (median: 62 yr), 3 patients presented with symptomatic pelvic masses, 3 tumors were identified by imaging studies, and the remaining 6 were incidentally discovered in ovaries removed for other indications. All tumors were unilateral, occurred at the ovarian hilum, and contained a vaguely lobulated architecture with sinusoidal-like vessels lined by hobnail endothelial cells with minimal to no cytologic atypia. A rim of luteinized/Leydig cells with abundant, eosinophilic cytoplasm and round, centrally placed nuclei surrounding the hemangioma was present in 9/12 tumors. Reinke crystals were observed in 3 of these 9 tumors. The volume of luteinized cells relative to the vascular proliferation ranged from 2% to 30%. All tumors with luteinized/Leydig cells also displayed numerous small eosinophilic, globular intracytoplasmic inclusions within the endothelial cells. The 3 tumors without luteinized/Leydig cells were exclusively intravascular lesions. Despite the frequent presence of luteinization/Leydig cells none of the patients experienced hormonal manifestations. Awareness of this rare benign ovarian entity is important, as its association with luteinized cells/Leydig cell hyperplasia (often exuberant) may be misinterpreted as a steroid cell tumor, Leydig-cell tumor, or as a mixed stromal-vascular tumor.

Relevance of Molecular Pathology for the Diagnosis of Sex Cord–Stromal Tumors of the Ovary: A Narrative Review

“…The stroma between tumor cells may be hyalinized, edematous, or hemangiomatous. The latter may be predominant, and an association with anastomosing hemangiomas has been recently described in a series of Leydig cell tumors [97], which is consistent with the endocrine nature of this tumor. Association with contralateral Leydig cell hyperplasia is common [8,94].…”

“…Generally, one key feature of Leydig cell tumors is their hilar location. Tumors are usually small with a mean size of 2.1 cm and well-circumscribed [95,97]. This tumor is typically unilateral, although bilateral cases and/or an association with hilar Leydig cell hyperplasia have been reported [8,95,97].…”

“…Tumors are usually small with a mean size of 2.1 cm and well-circumscribed [95,97]. This tumor is typically unilateral, although bilateral cases and/or an association with hilar Leydig cell hyperplasia have been reported [8,95,97]. Leydig cell tumors are solid, with a yellowish or brownish and often lobulated cut surface [8].…”

A case report of anastomosing hemangioma of the ovary

“…In some cases, an intracapillary fibrin thrombus is also observed. [ 7 , 9 ] AH cases with adipocyte metaplasia [ 10 ] and extramedullary hematopoiesis have also been reported. [ 11 ] According to the fifth edition of the World Health Organization classification of soft tissue tumors, the diagnosis of AH requires the presence of specific criteria, including the presence of hobnail-shaped endothelial cells that line the vascular lumen and form anastomoses with each other, hyaline droplets and extramedullary hematopoiesis.…”

“…However, these crystals may not always be present, and their absence does not rule out a diagnosis of AH. [ 9 , 11 , 13 ] Other common histological features reported in most of the literature reports include (21/31) significant interstitial luteinization and eosinophilic or vacuolated cytoplasm. These features can be easily confused with other types of ovarian tumors, such as steroid cell tumors, Leydig cell tumors, or other mixed stromal vascular tumors.…”