Intravascular papillary endothelial hyperplasia of the vulva: report of a patient with Masson tumor of the vulva and literature review.
BACKGROUND
Intravascular papillary endothelial hyperplasia -- also known as Masson tumor -- is a rare, benign vascular condition that manifests on the skin as a firm, blue-black colored nodule or papule. Lesions range in size from 0.25 to 5 centimeters in diameter and may be tender or painless. In some individuals, nodules appear red colored, mimicking hemangioma or pyogenic granuloma. Histologically, intravascular papillary endothelial hyperplasia is characterized by the presence of an organizing thrombus in the vascular lumen with accompanying hyperplastic endothelial cell proliferation. Common sites of presentation include the head, neck, and extremities. However, albeit rarely, lesions may also appear in the genital region.


OBJECTIVE
We describe the clinical and pathologic findings of a woman who developed intravascular papillary endothelial hyperplasia of the vulva. We also review the characteristics of other patients with intravascular papillary endothelial hyperplasia of the vulva and summarize the differential diagnosis and treatment options for this condition.


METHODS
The features of a woman with intravascular papillary endothelial hyperplasia of the vulva are presented. Using PubMed, the following terms were searched and relevant citations assessed: intravascular papillary endothelial hyperplasia, IPEH, labia majora, Masson hemangioma, Masson pseudoangiosarcoma, Masson tumor, and vulva. In addition, the literature on intravascular papillary endothelial hyperplasia is reviewed.


RESULTS
A 32-year-old woman presented with a 3 x 2 millimeter painless, black colored submucosal papule on her left labia majora. The lesion was removed by excisional biopsy. Microscopic examination revealed a re-canalizing thrombus and a proliferation of erythrocytes within a dilated vascular structure. Based on correlation of the clinical presentation and histopathologic findings, a diagnosis of intravascular papillary endothelial hyperplasia was established. The patient applied mupirocin 2% ointment to the biopsy site, which subsequently healed without complication or recurrence.


CONCLUSIONS
Intravascular papillary endothelial hyperplasia -- also known as Masson tumor -- is a rare, benign vascular eruption. Cutaneous lesions typically present as red colored or blue-black colored nodules ranging in size from 0.25 to 5 centimeters in diameter. The most common sites of presentation include the head, neck, and extremities. However, albeit rarely, lesions may also appear in the genital region. The histologic hallmark of intravascular papillary endothelial hyperplasia is an organizing thrombus in the vascular lumen with associated hyperplastic endothelial cell proliferation. Lesions often appears similar to other neoplastic and non-neoplastic cutaneous tumors, including hemangiomas, pyogenic granulomas, and cutaneous angiosarcomas. Therefore, pathologic examination is required to confirm the suspected diagnosis and exclude malignancy. The condition can usually be treated with simple local excision.
University of Nevada School of Medicine. brycebeutler@hotmail.com.
Bryce David
BD
Beutler
Philip R
PR
Cohen
D000328:Adult; D005260:Female; D006390:Hemangioendothelioma / Q000175:diagnosis / Q000473:pathology*; D006801:Humans; D049590:Postpartum Period; D011247:Pregnancy; D011252:Pregnancy Complications, Neoplastic / Q000175:diagnosis / Q000473:pathology*; D012867:Skin / Q000473:pathology; D014846:Vulvar Neoplasms / Q000175:diagnosis / Q000473:pathology*