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Intravascular papillary endothelial hyperplasia (Masson's tumor) manifesting as a lateral neck mass.

Abstract

Intravascular papillary endothelial hyperplasia is a benign lesion of vascular origin that is caused by an excessive proliferation of endothelial cells in normal blood vessels or vascular malformations. We report the case of a 26-year-old man who had such a lesion deep within the soft tissues of his neck. Imaging studies revealed a 6-cm-diameter mass, with its epicenter in the right retromandibular space. The mass extended into the right parapharyngeal space and compressed the pharynx. The mass was excised, and the patient recovered uneventfully. We discuss the management of this lesion, with emphasis on radiologic and histologic assessment and the differential diagnosis.

Introduction

Unexplained swelling in the head and neck region poses a diagnostic dilemma for clinicians. One such difficult-to-diagnose entity is intravascular papillary endothelial hyperplasia (IPEH), also known as intravascular angiomatosis, Masson's pseudoangiosarcoma, and vegetant intravascular hemangioendothelioma. IPEH is a benign lesion of vascular origin that is caused by an excessive proliferation of endothelial cells in normal blood vessels or vascular malformations.

Although it is ubiquitous in nature, [1] IPEH has a predilection for the head and neck region. [2,3] Most of the reported head and neck cases have involved only the skin and the subcutaneous tissues of the face and scalp. [4-6] Less common sites include the buccal mucosa, [7,8] tongue, [9,10] pharynx, [11] masseter muscle, [12] and parotid gland. [2] IPEH of the maxillary sinus, [13] mandible, [14] orbit, [15] and ocular adnexa [16] has also been observed. Only five IPEH patients have been reported to have an anterior neck mass; one was in the external jugular vein, [17] one in the vasculature beneath the platysma muscle, [18] and three in organizing hematomas of the thyroid gland. [9,20] One case of IPEH in the superior vena cava that caused obstructive symptoms has also been reported. [21]

In this article, we report the case of a patient who had an IPEH deep within the soft tissues of the neck, which manifested as a lateral neck mass. We also describe its radiologic appearance on computed tomography (CT) and magnetic resonance imaging (MRI). In most previously reported cases, radiologic investigations either were not performed or their significance was not noted. The purpose of this report is to alert clinicians to the potential for misdiagnosing IPEH at the clinical, radiologic, and histologic levels, particularly those who are unfamiliar with this lesion.

Case report

A 26-year-old man came to the hospital 2 days after he had developed a painful swelling on the right side of his neck. He had no history of trauma or recent dental or oropharyngeal infection, and his medical history was unremarkable. On clinical examination, his oral temperature was 100[degrees]F (37.8[degrees] C) and his pulse rate was 90 beats per minute. General and systemic examinations were normal.

On his neck, a tender, noncystic, nonpulsatile mass measuring 5 x 4 cm extended from the angle of the right mandible into the parapharyngeal space. The mass appeared to be fixed to deeper tissues. The overlying skin was normal, and there were no palpable cervical nodes. There was no bruit, trismus, or intraoral sign of infection. The mass protruded into the pharynx and pushed the right tonsil and soft palate medially. The nose, nasopharynx, hypopharynx, larynx, and ears were normal on examination, and the cranial nerves were not involved. Complete blood count, serum electrolyte, and renal function measurements were normal, as was the chest x-ray. The patient tested HIV-negative.

After 3 days of treatment with a broad-spectrum antibiotic, indomethacin, and an antiseptic mouthwash, the patient's pain and temperature subsided, but the swelling persisted. CT was ordered, and it revealed a 6-cm-diameter mass. Its epicenter was located in the right retromandibular space. The mass extended into the right parapharyngeal space and compressed the pharynx. The lesion was clearly separate from the parotid gland. The mass was of low density on precontrast CT, and it displayed a peripheral enhancement on postcontrast scanning. Calcification was notably absent.

MRI confirmed that a well-defined nonhomogenous mass had abutted and eroded the medial side of the right mandibular ramus (figure 1). The mass exhibited a patchy central enhancement after the administration of gadolinium. Fine-needle aspiration on two occasions was not helpful because the aspirate consisted entirely of blood.

At surgery, a poorly encapsulated vascular mass was encountered in the region of the right tall of the parotid gland. The mass extended up toward the pterygoid plates and muscles. The surgeon ligated all small feeding vessels and excised the mass in its entirety.

Microscopic examination disclosed the presence of a myriad of papillary structures that were characterized by central collagenized cores, which were surrounded by an attenuated single layer of endothelium (figure 2). Dilated vessels, focal hemorrhage with hemosiderin deposition, and organizing thrombi were noted. The surrounding stroma featured a mononuclear inflammatory cell infiltrate. There was no cytologic atypia or necrosis. Examination of the adjacent parotid gland revealed a chronic sialadenitis with interstitial fibrosis. Postoperatively, the patient was left with a mild right mandibular facial palsy. One year later, he was well without any evidence of recurrence.

Discussion

Clinically, IPEH usually manifests as a firm and sometimes tender mass that imparts a reddish-blue color to the overlying skin or mucous membrane. The age distribution is wide (9 mo-80 yr), [22] and most series report a slightly higher incidence among females. [23,24]

Because its clinical signs and symptoms are nonspecific, IPEH, like most other vascular malformations, poses a difficult diagnostic challenge. [25] This is particularly true with the deeper intramuscular lesions. [22] In our case, the patient's pain might have been caused by the compression of the parotid gland and other adjacent structures.

Radiologic evaluation. Radiologic investigations are integral to a rational approach to evaluating most lateral neck masses, particularly when the etiology is unclear. In our case, the patient's tumor displayed peripheral enhancement on postcontrast CT. On MRI, it produced a nonhomogenous signal postcontrast, with patchy central enhancement. An abscess can also show a central low-attenuation area and a contrast-enhancing rim on CT. Previous studies have shown that some IPEH masses are homogenous on CT and MRI, whereas others are nonhomogenous as well as contrast-enhancing. [26-30] Such signals are probably related to the fact that lesions are likely to have varying amounts of both solid "parenchymal" tissue areas (papillary structures and thrombi) and anastomotic, stagnant, or low-flow vascular channels.

Areas of microcalcification have been noted in IPEH lesions. [31,32] Phleboliths, however, are also common in patients with hemangiomas. Likewise, calcification can occur in hematomas and tumors, and they can follow necrosis of soft tissues. Moreover, on angiography, IPEH can manifest as either a vascular or avascular mass. [33] MRI is usually superior as the initial diagnostic test for vascular malformations, and further investigations are usually not necessary in low-flow lesions. [34] The CT, MRI, and angiographic patterns of IPEH can all simulate other benign (e.g., hemangioma [32]) and malignant (e.g., angiosarcoma [27]) conditions.

When Masson's tumor causes bone erosion as a result of pressure effects, its radiologic features can be even more suggestive of malignancy.[15,35] Therefore, while radiologic investigations can contribute to management, they are not sufficiently characteristic by themselves to make a diagnosis of IPEH. The diagnosis is best established with a histologic examination--or cytologically if the aspirate contains sufficient cells in addition to blood and fibrinoid material. [36,37]

Histologic evaluation. Microscopically, IPEH is a well-circumscribed lesion made up of stromal papillae that are lined with endothelial cells. These papillary structures can fuse to form an anastomosing vascular network, such as that associated with thrombosis. Inflammatory cells are usually few in number. In rare instances, a rupture of the vessel of origin permits the spilling over of proliferating endothelial cells into surrounding soft tissue, a phenomenon that should not be equated with malignancy. [38]

Pathogenesis. There are three types of JPEH: (1) a pure (primary) form that arises de novo in dilated vascular spaces, (2) a mixed (secondary or reactive) form that occurs focally in pre-existing varices, hemangiomas, and arteriovenous malformations, and (3) a rare extra vascular form that arises in hematomas. [19] The pathogenesis of IPEH remains speculative. Masson, in 1923, regarded this condition as a true neoplasm with secondary thrombosis. [39] However, its form of presentation, its association with other lesions, and its pattern of growth are more suggestive of a benign reactive process, perhaps related to trauma. [40, 41] Our patient had no such significant history.

The frequency with which thrombi are found in these lesions have led some authors to propose that IPEH represents an unusual form of thrombus organization. [42, 43] It is thought that once the thrombus develops, it serves as a matrix for the ingrowth of papillary structures. [44] Ultrastructurally, these papillary structures appear to closely resemble granulation tissue, which further suggests a reparative origin. [45] In further support of this view is the fact that the endothelium-lining cells seem to originate in histiocytes. [46] The exuberant endothelial proliferation involves an autocrine loop of endothelial secretion of basic fibroblast growth factor. [47] Reports of occasional cases that did not involve thrombosis in addition to reports of the presence of a lymphatic counterpart have led some authors to support Masson's original theory that JPEH is a benign tumor marked by primary endothelial proliferation and secondary thrombus formation. [48]

Differential diagnosis. We emphasize the importance of the differential diagnosis, particularly with respect to other conditions that have a predilection for the head and neck. Most significantly, IPEH can be mistaken for an angiosarcoma. [49,50] Other differential diagnoses include mucocele, [51] pyogenic granuloma, [23] Kaposi' s sarcoma, [52] and other vascular conditions such as hemangioma, angioendothelioma, papular angioplasia, Kimura's disease, bacillary angiomatosis, and intravenous atypical vascular proliferation. [53]

Microscopically, angiosarcoma usually exhibits an invasive growth pattern, cellular pleomorphism, frequent mitoses, and necrotic foci that are not seen in IPEH. [54] Although endothelial cells are also a prominent feature in pyogenic granuloma, no surrounding vascular wall is evident. Histologically, Kaposi's sarcoma has no papillary fronds; rather, it displays anastomosing slit-like vessels associated with a fascicular pattern of spindle-shaped cells, a plasma cell infiltrate, and hemosiderin pigment deposition. Detection of Bartonella organisms in histologic specimens distinguishes bacillary angiomatosis from IPEH. [55]

The importance of this report is that it stresses IPEH's likeness to, and therefore the difficulty in differentiating it from, similar benign and malignant conditions clinically, radiologically, and histologically. Conservative surgical excision is the treatment of choice. Invariably, the prognosis appears to be excellent, except for patients with intracranial lesions, which can be fatal. [56] Recurrence is extremely rare and is thought to occur only when the underlying vascular lesion is not excised completely. [57]

From the Department of Anatomical Pathology, South African Institute for Medical Research (Dr. Pantanowitz and Dr. Muc); the Department of Radiology, Chris Hani Baragwanath Hospital (Dr. Spanger); the Department of Anatomical Pathology, Chris Hani Baragwanath Hospital and the South African Institute for Medical Research (Dr. Sonnendecker); and the Department of Otorhinolaryngology, Johannesburg Teaching Hospitals (Dr. McIntosh) and the University of the Witwatersrand, Johannesburg, South Africa.

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Comment:Intravascular papillary endothelial hyperplasia (Masson's tumor) manifesting as a lateral neck mass.
Author:McIntos, William A.
Publication:Ear, Nose and Throat Journal
Geographic Code:6SOUT
Date:Oct 1, 2000
Words:3155
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