Sonographic features of intravascular papillary endothelial hyperplasia (Masson's tumor) in the forearm

Abstract

Intravascular papillary endothelial hyperplasia, also known as Masson's tumor, is a benign, vascular lesion in which there is papillary proliferation of endothelial cells. The lesion presents as a palpable soft-tissue mass, often located within normal or dilated vascular spaces, and may be mistaken for a sarcomatous tumor on imaging. We present the case of an intravascular papillary endothelial hyperplasia in the forearm, with a remarkable appearance on color Doppler sonography, and suggest that this entity will be encountered more frequently by sonologists in the future.

Masson's tumor in the ulnar artery

“…2 Our patient presented with an aneurysmal ulnar artery and the primary form of Masson's tumor, which is the most common form of Masson's tumor constituting more than 50% of the cases seen in the literature. 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.…”

“…9 On sonography, Lysyy et al describe a pure form of Masson's tumor that appears as a well-defined as echogenic lesion. 1 Other reports suggest the use of color Doppler imaging in demonstrating the hypervascularity of these masses. 9,10 However, these nonspecific findings do not differentiate Masson's tumors from other vascular neoplasms, so there is little preoperative predictive value in radiographic studies.…”

“…8 Preoperative imaging has been used in only a few cases. 1 On computed tomography and magnetic resonance imaging scans, Masson's tumors appear as homogeneous, contrast-enhancing masses. 9 On sonography, Lysyy et al describe a pure form of Masson's tumor that appears as a well-defined as echogenic lesion.…”

“…In addition, re- ports of imaging are limited because few cases require radiographic investigation given the superficial location and small size of most of these tumors. 1 Masson's tumors are only diagnosed definitively by immunohistochemistry. Specific markers include factor VIII, CD31, CD34, and platelet endothelial cell adhesion molecule which confirm a vascular origin of pathology.…”

“…1 Despite comprising only 2% to 4% of all skin and soft tissue vascular tumors, multiple case reports have described Masson's tumors arising in the hand, forearm, neck, oral mucosa, and salivary glands as well as in both arteries and veins. [1][2][3][4] However, the diagnosis of Masson's tumor can be difficult with a broad differential diagnosis, including intravascular fasciitis, intravascular pyogenic granuloma, and angiosarcoma. 4 We describe a patient with intravascular epithelial papillary hyperplasia presenting as an ulnar artery aneurysm.…”