PracticalDermatology

CASE REPORT: EXCISION OF AN INTRAVASCULAR PAPILLARY ENDOTHELIAL HYPERPLASIA

Doug Richley, DO; Christine Sickles, DO, Stephen J Plumb, DO; Jonathan Cleaver, DO, FAOCD; and Lloyd Cleaver, DO, FAOCD

A 37-year-old Caucasian male presented for evaluation of a lesion located on his left superior mucosal lip (Fig. 1). He reported that the lesion was asymptomatic and had been enlarging over a six-month period. He admitted to mild pain with manipulation. He first noticed this lesion approximately two years before his visit. He presented to the office wanting to have the lesion removed, secondary to him being concerned due to the fact that it has been enlarging, as well as the mild pain he experiences with manipulation.

Based on the clinical presentation and history, our initial differential diagnosis included venous lake, dysplastic nevus, and blue nevus. The changing nature of the lesion, as well as it being mildly tender to palpation warranted that the lesion be removed.

The decision was made to perform an excisional biopsy. The defect created by the excision was approximated using 5.0 Vicryl absorbable suture. A total of four interrupted sutures were used to close the defect (Fig. 2). We instructed the patient to follow-up with his primary physician five to seven days after excision for evaluation of the wound and to have the sutures removed. The lesion was sent for hemotoxylin and eosin (H & E) staining and histologic review. The dermatopathology report showed a proliferation of vessels with papillary endothelial projections (Fig. 3), consistent with the diagnosis of intravascular papillary endothelial hyperplasia (IPEH) or Masson’s tumor. The benign nature of this entity did not warrant further treatment, and the patient was advised to follow-up in one year.

DISCUSSION

Intravascular papillary endothelial hyperplasia (IPEH) or Masson’s tumor was first described by Pierre Masson in 1923 as a neoplastic process. This was due to the proliferation of endothelial cells and fibrin deposition into the vessel of the lumen that lead to obstruction and degeneration. It was later described as a reactive process and is now regarded as a benign, non-neoplastic vascular lesion that can occur post-trauma.^1,2 In 1976, Clearkin and Enzinger proposed the name intravascular papillary endothelial hyperplasia (IPEH).³ The lesion may also be referred to as Masson’s lesion, Masson’s tumor, Masson’s hemangio-endotheliome vegetant intravasculaire, Masson’s pseudoangiosarcoma, intravascular angiomatosis, intravascular or extravascular papillary endothelial hyperplasia.⁴

Masson’s tumor occurs in both males and females, showing a slight female predominance with a 1.0:1.2 male to female ratio. A possible hormonal role has been suggested based on this gender difference, and local angiogenic growth factors may contribute to endothelial proliferation.5 The average age of presentation is 34 years old.³ Vascular lesions are common in the oral region, but Masson’s tumor is an uncommon lesion that comprises approximately two percent of vascular tumors.²

Clinically, a Masson’s tumor presents as a single firm, papule, nodule or mass. Patients may complain of pain or tenderness.¹ The lesions are red or purple in color and ranges in size from 5mm to 5cm. It typically involves the fingers, head, neck, trunk, lower extremities, and upper extremities.⁵ Rarely these lesion arise on mucosal membranes, including the upper lip, lower lip, tongue, buccal mucosa, mandibular vestibule, and angle of the mouth.⁶

The pathogenesis of Masson’s tumor is not completely understood, but it is thought that these lesions develop as a reactive process during the organization and recanalization of a thrombus. This lesion is considered to be a result from trauma or continuous stimulation.² In the early stage of formation, endothelial cells embed themselves into the thrombus. The endothelial cells cause a separation within the thrombus leading to irregular digestion by collagenase. Papillary structures then develop within the irregular thrombus and anastomose with adjacent vascular structures. The anastomosis and recanalization may convert the thrombus to a mass of vascularized connective tissue and cause subendothelial vascular wall swelling.⁷

There are three different types of lesions described. The first type is the primary form and it arises within an isolated dilated blood vessel, most commonly a vein and occurs on the fingers, head, and neck. The secondary type is found in a preexisting vascular malformation, such as pyogenic granuloma or cavernous hemangioma and arises intramuscularly. The third type is the least common and is found extravascularly, and usually arises in the setting of a hematoma.^3,8 The differential diagnosis of benign oral lesions includes hemangioma, mucocele, pyogenic granuloma, endothelioma, and the malignant neoplasms diagnosis includes angiosarcoma.⁵

Histologically, Masson’s tumor is characterized by papillary structures with a central hypocellular hyaline core lined by endothelial cells and associated thrombi. The histology is usually distinct enough to make the diagnosis, but immunohistochemical confirmation may be needed to ensure the lesion is endothelial in origin. Von Willebrand factor, CD 31, factor XIIIa, and CD 43 are markers that can be used to highlight the endothelial lining.⁹ The histologic features that distinguish Masson’s tumor from angiosarcoma are a well circumscribed intravascular papillary hyperplasia, a proliferative process that is confined to the intravascular space, and absence of nuclear atypia or mitotic figures.5 The prognosis of IPEH is excellent and surgical excision is considered curative. Malignant lesions require more aggressive treatment than surgical excision because of their ability to metastasize, therefore differentiation is critical.⁹

TOP 10 RESIDENT PRESENTATIONS FROM THE 2014 COSMETIC SURGERY FORUM

A significant point of emphasis at Cosmetic Surgery Forum (CSF), which was most recently held in December in Las Vegas, is the education future generations of dermatologists. Residents are given the opportunity to conduct and present case studies, and every year, CSF acknowledges the top 10 presentations. The winning presentations given by dermatology residents at the 2014 CSF meeting include:

1. Kerry Shaughnessy: “A Chronic Pruritic Plaque on the Left Shoulder”
2. Amy Thorne: “The Extracutaneous Effects of Benign Stigmata”
3. Divya Shokeen: “Rhabdomyolysis in Patients on Isotretinoin Therapy”
4. David Rayhan: “Bilateral Peri-Ocular Primary Mucinous Carcinoma Treated with Mohs Micrographic Surgery”
5. Tatyana Petukhiva: “Significant Inter-rater Variability in Acne Scarring”
6. Bonita Kozma: “Hidradenitis Suppurtiva”
7. Cerrene Giordano: “Ulcerations of Striae Distensae”
8. Michael Graves: “Defining the Absorption Spectrum of Dihydroxyacetone, a Popular Sunless Tanner, Using Reflectance Photospectrometry”
9. Conor Dolehide: “An Adolescent with Solid Facial Edema (Morbihan Disease)
10. Tanya Khan: “Retained Dermal Filler Masquerading as Periorbital Edema”

ASK AN EXPERT: MANAGING MOLLUSCUM

Sheila Fallon Friedlander, MD discussed her current approach to the management of molluscum with host Adam Friedman, MD in a recent issue of NewDermMD.

Dr. Friedlander says the first step to treating molluscum is to find out how long the child has had it, if anyone else in the family also has it, and how much treatment they’ve already tried. Explaining that many patients who present to her with this condition are typically frustrated because they’ve already been to a pediatrician and maybe even another dermatologist, she says education about the disease and treatment options is very important. Treatment advice can range from watchful waiting to treatment with cantharidin, if the patient is the right candidate. To hear more about how and when she uses cantharidin, as well as how she treats molluscum on the face, visit http://newdermmd.com/2014/12/ask-an-expert

Doug Richley, DO is a Dermatology Resident, 2nd year, Northeast Regional Medical Center/ ATSU, Kirksville, Missouri. Christine Sickles, DO is a Traditional Intern, Lewis-Gale Medical Center, Blacksburg, VA. Stephen J Plumb, DO is a Dermatopathologist, Cleaver Dermatology, Kirksville, Missouri. Jonathan Cleaver, DO, FAOCD, FAOCD is Attending Dermatologist, Northeast Regional Medical Center/ATSU, Kirksville, Missouri. Lloyd Cleaver, DO, FAOCD is Program Director, Northeast Regional Medical Center/ ATSU, Kirksville, Missouri.

The authors have no relevant disclosures.

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