Intravascular papillary endothelial hyperplasia (Masson's tumor) manifesting as a lateral neck mass.Abstract Intravascular papillary papillary /pap·il·lary/ (pap´i-lar?e) pertaining to or resembling a papilla, or nipple. papillary, adj similar to a small, nipple-shaped elevation or projection. 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 In human anatomy, the masseter is one of the muscles of mastication. It is particularly powerful in herbivores to assist when they are chewing plants. Origin and insertion of the two heads , [12] and parotid gland. [2] IPEH of the maxillary sinus, [13] mandible, [14] orbit, [15] and ocular adnexa adnexa /ad·nexa/ (ad-nek´sah) [L., pl.] appendages or accessory structures of an organ, as the appendages of the eye (a. o´culi), including the eyelids and lacrimal apparatus, or of the uterus (a. [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 external jugular vein n. A vein that is formed by the junction of the posterior auricular and the retromandibular veins, passes down the side of the neck superficial to the sternocleidomastoid muscle, and empties into the subclavian 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 superior vena cava n. Abbr. SVC A large vein formed by the union of the two brachiocephalic veins and the azygos vein that receives blood from the head, neck, upper limbs, and chest, and empties into the right atrium of the heart. 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 magnetic resonance imaging (MRI), noninvasive diagnostic technique that uses nuclear magnetic resonance to produce cross-sectional images of organs and other internal body structures. (MRI 1. (application) MRI - Magnetic Resonance Imaging. 2. MRI - Measurement Requirements and Interface. ). 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 oropharyngeal /oro·pha·ryn·ge·al/ (-fah-rin´je-al) 1. pertaining to the mouth and pharynx. 2. pertaining to the oropharynx. 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 beats per minute Cardiac pacing The unit of measure for the frequency of heart depolarizations or contractions each minute–or pulse rate . 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 overlying suffocation of piglets by the sow. The piglets may be weak from illness or malnutrition, the sow may be clumsy or ill, the pen may be inadequate in size or poorly designed so that piglets cannot escape. skin was normal, and there were no palpable cervical nodes. There was no bruit bruit (brwe) (brldbomact) 1. a sound or murmur heard in auscultation, especially an abnormal one. 2. sound (3). , trismus trismus /tris·mus/ (triz´mus) motor disturbance of the trigeminal nerve, especially spasm of the masticatory muscles, with difficulty in opening the mouth (lockjaw); a characteristic early symptom of tetanus. , or intraoral sign of infection. The mass protruded into the pharynx and pushed the right tonsil tonsil Small mass of lymphoid tissue in the wall of the pharynx. The term usually refers to the palatine tonsils on each side of the oropharynx. They are thought to produce antibodies to help prevent respiratory and digestive tract infection but often become infected 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 mouthwash /mouth·wash/ (mouth´wosh) a solution for rinsing the mouth. mouth·wash n. A medicated liquid for cleaning the mouth and treating diseased mucous membranes. , 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 ramus /ra·mus/ (ra´mus) pl. ra´mi [L.] a branch, as of a nerve, vein, or artery. ramus articula´ris (figure 1). The mass exhibited a patchy central enhancement after the administration of gadolinium gadolinium (gădəlĭn`ēəm), metallic chemical element; symbol Gd; at. no. 64; at. wt. 157.25; m.p. 1,312°C;; b.p. 3,233°C;; sp. gr. 7.898 at 25°C;; valence +3. . Fine-needle aspiration on two occasions was not helpful because the aspirate as·pi·rate v. To take in or remove by aspiration. n. A substance removed by aspiration. Aspirate The removal by suction of a fluid from a body cavity using a needle. 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 pterygoid /pter·y·goid/ (ter´i-goid) shaped like a wing. pter·y·goid adj. 1. Of, relating to, or located in the region of the sphenoid bone. 2. 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 hemosiderin /he·mo·sid·er·in/ (he?mo-sid´er-in) an insoluble form of tissue storage iron, visible microscopically both with and without the use of special stains. he·mo·sid·er·in n. deposition, and organizing thrombi thrombi /throm·bi/ (throm´bi) plural of thrombus. were noted. The surrounding stroma stroma /stro·ma/ (stro´mah) pl. stro´mata [Gr.] the matrix or supporting tissue of an organ.stro´malstromat´ic stro·ma n. pl. stro·ma·ta 1. featured a mononuclear inflammatory cell infiltrate. There was no cytologic atypia or necrosis. Examination of the adjacent parotid gland revealed a chronic sialadenitis sialadenitis /si·al·ad·e·ni·tis/ (si?al-ad?e-ni´tis) inflammation of a salivary gland. si·a·lad·en·i·tis or si·a·lo·ad·e·ni·tis n. Inflammation of a salivary gland. 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 CT and MRI Two high technology methods of creating images of internal organs. Computerized axial tomography (CT or CAT) uses x rays, while magnetic resonance imaging (MRI) uses magnet fields and radio-frequency signals. Both construct images using a computer. , 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 avascular /avas·cu·lar/ (a-vas´ku-ler) not vascular; bloodless. a·vas·cu·lar adj. Not associated with or supplied by blood vessels. 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 hemangioma Congenital benign tumour made of blood vessels in the skin. Capillary hemangioma (nevus flammeus, port-wine stain), an abnormal mass of capillaries on the head, neck, or face, is pink to dark bluish-red and even with the skin. Size and shape vary. [32]) and malignant (e.g., angiosarcoma angiosarcoma /an·gio·sar·co·ma/ (an?je-o-sahr-ko´mah) a malignant neoplasm arising from vascular endothelial cells; the term may be used generally or may denote a subtype, such as hemangiosarcoma. [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 fibrinoid /fi·brin·oid/ (fi´bri-noid) 1. resembling fibrin. 2. a homogeneous, eosinophilic, relatively acellular refractile substance with some of the staining properties of fibrin. material. [36,37] Histologic evaluation. Microscopically, IPEH is a well-circumscribed lesion made up of stromal Stromal A type of tissue that is associated with the support of an organ. Mentioned in: Wilms' Tumor 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 neoplasm or tumor, tissue composed of cells that grow in an abnormal way. Normal tissue is growth-limited, i.e., cell reproduction is equal to cell death. 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 ingrowth /in·growth/ (-groth) an inward growth; something that grows inward or into. in·growth n. Something that grows inward or into a part of the body. 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 autocrine /au·to·crine/ (-krin) denoting a mode of hormone action in which a hormone binds to receptors on and affects the function of the cell type that produced it. au·to·crine adj. loop of endothelial secretion of basic fibroblast growth factor Basic fibroblast growth factor, also known as bFGF or FGF2, is a member of the fibroblast growth factor family. In normal tissue, basic fibroblast growth factor is present in basement membranes and in the subendothelial extracellular matrix of blood . [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 papular characterized by the development of epidermal or oral mucosal papules. bovine papular stomatitis a benign stomatitis caused by a poxvirus in the genus Parapoxvirus. angioplasia, Kimura's disease, bacillary angiomatosis, and intravenous atypical vascular proliferation. [53] Microscopically, angiosarcoma usually exhibits an invasive growth pattern, cellular pleomorphism pleomorphism /pleo·mor·phism/ (-mor´fizm) the occurrence of various distinct forms by a single organism or within a species.pleomor´phicpleomor´phous ple·o·mor·phism n. 1. , 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 fascicular /fas·cic·u·lar/ (fah-sik´u-lar) 1. pertaining to a fasciculus. 2. fasciculated. fas·cic·u·lar or fas·cic·u·late or fas·cic·u·lat·ed adj. 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 Chris Hani Baragwanath Hospital is the largest hospital in the world[1], occupying 173 acres, with 3200 beds and 6760 staff members. The hospital is in Soweto, South Africa - just outside Johannesburg. (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 otorhinolaryngology /oto·rhi·no·lar·yn·gol·o·gy/ (-ri?no-lar?ing-gol´ah-je) the branch of medicine dealing with the ear, nose, and throat. o·to·rhi·no·lar·yn·gol·o·gy n. , Johannesburg Teaching Hospitals (Dr. McIntosh) and the University of the Witwatersrand Due to the 1959 Extension of University Education Act the school was only allowed to register a small number of black students for most of the apartheid era, even though several notable black anti-apartheid leaders graduated from the university. , Johannesburg, South Africa. References (1.) Enzinger FM, Weiss SW. Soft Tissue Tumors. St Louis: Mosby, 1995:614-9. (2.) Corio RL, Brannon RB, Tarpley TM. Intravascular papillary endothelial hyperplasia of the head and neck. Ear Nose Throat J 1982;61:88-91. (3.) 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