Periorbital Masson's Tumor: A Case Series

Abstract

Intravascular papillary endothelial hyperplasia (IPEH) or Masson's tumor is an unusual variety of benign vascular tumor. Involvement of periorbital area is uncommon. We report 2 cases of periorbital IPEH and review relevant literature.

Masson's tumor in the ulnar artery

“…1 Masson's tumors are found more frequently in women than in men, but they show no age or race predilection. 5,6 The female predominance may be a result of the tumor's response to estrogen and progesterone, as demonstrated by increased growth during pregnancy and immunohistochemical evidence of increased estrogen receptor and progesterone receptor expression. 7 Masson's tumors have also been reported to occur more commonly in veins than in arteries.…”

“…Others suggest that it may be a reactive process that results in endothelial hyperplasia. 6 The decision for surgical intervention in this patient was driven by the patient's neurologic symptoms. Our patient presented with numbness and weakness that impaired daily activity, but she had no evidence of vascular impairment.…”

Intravascular Papillary Endothelial Hyperplasia of the Orbit: A Case of Masson’s Tumor

“…IPEH was first described by Masson in 1923 as “vegetate intravascular hemangioendothelioma”[5]. It is characterized by a reactive hyperplastic proliferation of the vascular endothelium [1]. It has been speculated that the primary process is endothelial proliferation followed by thrombus formation; however, others conjecture that endothelial papillary proliferation is a response to thrombus formation [1-3].…”

“…It is characterized by a reactive hyperplastic proliferation of the vascular endothelium [1]. It has been speculated that the primary process is endothelial proliferation followed by thrombus formation; however, others conjecture that endothelial papillary proliferation is a response to thrombus formation [1-3]. There is no age, race, or sex predilection for the generalized IPEH; however, in their case series, Wagh et al found that the average age for periorbital IPEH was 41.2 years with a male to female ratio of 2:1 [1,2].…”

“…Masson’s tumor, also known as intravascular papillary endothelial hyperplasia (IPEH), is a rare benign neoplasm that affects the head, neck, and upper extremities. There are few (less than 20) periorbital cases that have been reported in ophthalmologic literature [1-4]. Masson’s tumor can often be difficult to distinguish between other entities including malignant angiosarcoma; its prompt diagnosis is important to begin optimal treatment as soon as possible.…”