28 August 2022: Articles 
Intravascular Papillary Endothelial Hyperplasia (Masson’s Tumor) of the Supraclavicular Region: Management of a Rare Case
Challenging differential diagnosis, Rare disease
Eva Aurora Massimilla1ADEF*, Gabriella Panetti1BEF, Giovanni Motta1B, Alfonso Fiorelli2B, Marco Montella3BD, Domenico Testa1AD, Gaetano Motta1ADDOI: 10.12659/AJCR.936402
Am J Case Rep 2022; 23:e936402
Abstract
BACKGROUND: Masson’s tumor, also known as IPEH (intravascular papillary endothelial hyperplasia), is a rare benign vascular process, comprising approximately 2% of vascular tumors of the skin and subcutaneous tissue. IPEH presenting as a neck mass is rare, with only 5 reports of anterior neck mass and 7 cases of lateral neck lesions, of which 1 was in an external jugular vein aneurysm. To the best of our knowledge, the localization of intravascular papillary endothelial hyperplasia in the supraclavicular region is rarely reported.
CASE REPORT: We describe our management of a non-traumatic and non-painful mass on the right supraclavicular region in 24-year-old woman. Ultrasound revealed a heterogeneously hypoechoic mass with intense vascularization. Magnetic resonance imaging (MRI) showed a formation with lobulated contours and closely related to muscular layers of serratus anterior muscle. The findings of ultrasound-guided biopsy (FNA) were inconclusive. Complete removal of the mass was performed. Histopathological examination showed a well-circumscribed lesion with numerous small papillary structures. The papillae had hyalinized hypocellular cores covered by flattened endothelium. Immunohistochemical analysis showed endothelial positivity for CD34 and CD31. These features are typical of IPEH. No recurrence had occurred at 12 months after surgery.
CONCLUSIONS: The differential diagnosis of malignant tumors like angiosarcoma is important because the prognosis significantly differs. Surgical excision is the treatment of choice. In our experience, the size of the lesion and its critical landmarks have not influenced the outcomes of the surgical procedure in terms of potential functional damages.
Keywords: Vascular Neoplasms, Head and Neck Neoplasms, Subcutaneous Tissue, Neck, Antigens, CD34, Vascular Diseases, Adult, Diagnosis, Differential, Female, Humans, Hyperplasia, Magnetic Resonance Imaging, Skin, young adult
Background
Intravascular papillary endothelial hyperplasia (IPEH) is a rare vascular lesion comprising approximately 2% of benign and malignant vascular tumors of skin and subcutaneous tissues [1–4]. It can occur in all age groups and has no race or ethnic group preferences [5] but there is a slight female predominance, which can be related to involvement of hormonal factors [6]. It can affect any blood vessel in the body but has a predilection for the head and neck region, fingers, trunk, and upper and lower extremities [7]. Most reported head and neck cases have involved skin and subcutaneous tissues of face and scalp [8–10]; less common sites are buccal mucosa [4,11], tongue [12,13], pharynx [14], larynx and hypopharynx [15], masseter muscle [16] and parotid gland [17], maxillary sinus [18], mandible [19], orbit [20], and ocular adnexa [21]. IPEH presenting as a neck mass is rare, with only 5 reports of anterior neck mass [22–25] and 7 cases of lateral neck lesions [26–32], of which 1 was in an external jugular vein aneurysm [30].
To the best of our knowledge, the localization of intravascular papillary endothelial hyperplasia in the supraclavicular region has rarely been reported [33,34]. Only 1 case in close proximity of serratus anterior muscular fibers was reported in the English language literature and treated by a multidisciplinary team.
Here, we report a clinical case of IPEH located in subcutaneous soft tissue of the supraclavicular region that was successfully managed by otolaryngologists without postoperative complications and with no recurrence of the disease 12 months after the surgical procedure.
Case report
A 24-year-old woman presented with non-traumatic and non-painful swelling on the right supraclavicular region that was noticed 1 year ago and was progressively increasing in size. At the dermatological examination, the lesion appeared mobile, non-tender, non-compressible, with fibroelastic consistency, without alterations of the overlying skin and pulsatility. Ultrasound revealed a heterogeneously hypoechoic mass and Doppler US recognized in it an intense vascularization.
T1-weighted and T2-weighted magnetic resonance imaging (MRI) was conducted, showing a 43×30×49 mm formation with lobulated contours and closely related to muscular layers of serratus anterior muscle (Figure 1A, 1B). The signal is mostly fluid (likely serohematic content) and few calcifications are described. After intravenous administration of contrast (gadobutrol) there is a septal enhancement.
An ultrasound-guided biopsy was performed, but the findings were inconclusive.
The presence of a lymphangioma was hypothesized and the mass was radically excised for histopathological evaluation.
Intraoperative neurophysiologic monitoring has been used. The mass was completely removed. Macroscopically, the mass was 5.5×3×3.5 cm in size (Figures 2, 3). After surgery, a drainage tube has been placed. The patient had a fast and uncomplicated recovery. There was no evidence of recurrence after 12 months.
Under pathologic examination, the lesion appears well-circumscribed and presents numerous small papillary structures. The papillae have a hyalinized hypocellular cores covered by flattened endothelium (Figure 4). Immunohistochemical analysis showed endothelial positivity for CD34 and CD31 (Figure 5). These features are typical of IPEH.
Discussion
The complexity of the anatomy of the supraclavicular fossa allows for an extensive differential diagnosis for regional pathology, which categorizes into neoplastic, congenital, and infectious etiologies. According to some authors, the majority of supraclavicular masses biopsied via FNA were found to be the result of a malignant process, with metastatic spread being far more likely than lymphoma [35,36].
IPEH was first described in 1923 by Pierre Masson [37]. He described an intravascular papillary proliferation in the lumen of an inflamed hemorrhoidal plexus in a 68-yarr-old man and named it “hémangioendothéliome végétant intravasculaire”.
In 1932, Folke Henschen [38] reported a similar case and named it “endovasculite proliférante thrombopoiétique”, describing it as a reactive process instead of a neoplasm. The name of “intravascular papillary endothelial hyperplasia” (IPEH), reported by Clearkin and Enzinger in 1976 [39], is currently the most commonly used, since it underlines the non-neoplastic character of this disease.
Three types of IPEH were described: a primary type, the most common, which is a pure endovascular form that originates in dilated vascular spaces; a mixed or secondary form that occurs in pre-existing vascular abnormalities; and the undetermined type, which is a rare extravascular hemangioma form that arises from hematomas [25,29]. The pathogenesis of IPEH seems to be multifactorial [10]. In 30% of cases, it is related to trauma or vascular abnormalities (eg, hemangiomas, vascular malformations, pyogenic granulomas, blood stasis) that induce a perivascular inflammation and favor the onset of a reactive process by endothelial cells through a consistent secretion of FGF-β [5]. However, the trigger stimulus is still not clear, so the etiology remains unknown [5,15,40]. Salyer et al found a peculiar pattern of thrombus organization [28,41]. CD 31 and CD 34 were found to be the most efficient immunohistological markers to identify the vascular origin of IPEH, in comparison to FVIII, type IV collagen, SMA and MSA antibodies, CD105, and Ki-67 [28,42]. Clinical and radiological features are not adequate to guide the diagnosis; therefore, histological characterization of the lesion is necessary.
IPEH can be mistaken for other benign subcutaneous tumors, such as lipomas, angiomas, and cysts, but the differential diagnosis is more important with malignant tumors like angiosarcoma and Kaposi’s sarcoma [7], since the prognosis will change.
Angiosarcoma is distinguished from Masson’s tumor by the presence at histopathological examination of solid and necrotic areas, mitotic figures, or pleomorphic cells and by the absence of confined intralaminar location [10,26]. Other differential diagnoses to consider are pyogenic granuloma, mucocele, the intravascular form of hemangioma, and malignant endovascular papillary angioendothelioma [25,29].
Surgical excision is the treatment of choice, generally with a good prognosis. Recurrences are rare and mostly associated with incomplete excision [25,26].
The size in combination with the site of the lesion gave us some concerns in terms of potential complications, due to the uncertain histology and vascularization of the mass.
Intraoperative neurophysiologic monitoring is crucial since several nerve structures such as the brachial plexus, cervical plexus, and phrenic nerve are potentially at risk.
Bleeding control has a fundamental role too, since IPEH has a conspicuous vascularization and the vasculature within the region of the supraclavicular triangle is complex, with presence of major vessels like subclavian, transverse cervical, and suprascapular arteries and veins, as well as external and internal jugular veins and their branches.
Conclusions
To the best of our knowledge, the localization of intravascular papillary endothelial hyperplasia in the supraclavicular region is rarely reported. The differential diagnosis with malignant tumors like angiosarcoma is fundamental since the subsequent prognosis will significantly change. Surgical excision is the treatment of choice. In our experience, the size of the lesion and its critical landmarks do not influence the outcomes of the surgical procedure in terms of potential functional damages.
Figures
References:
1.. Sasso SE, Naspolini AP, Milanez TB, Suchard G, Masson’s tumor (intravascular papillary endothelial hyperplasia): An Bras Dermatol, 2019; 94(5); 620-21
2.. Almarghoub MA, Shah Mardan QNM, Alotaibi AS, Masson’s tumor involving the hand: A case report: Int J Surg Case Rep, 2020; 70; 223-26
3.. Carta F, Sionis S, Ledda V, Parotid Masson’s tumor: Case report: Braz J Otorhinolaryngol, 2018; 84(4); 523-25
4.. Tosios K, Koutlas IG, Papanikolaou SI, Intravascular papillary hyperplasia of the oral soft tissues: Report of 18 cases and review of the literature: J Oral Maxillofacial Surg, 1994; 52; 1263-68
5.. Cagli S, Oktar N, Dalbasti T, Intravascular papillary endothelial hyper-plasia of the central nervous system – four case reports: Neurol Med Chir (Tokyo), 2004; 44; 302-10
6.. Pins MR, Rosenthal DI, Springfield DS, Rosenberg AE, Florid extravascular papillary endothelial hyperplasia (Masson’s pseudoangiosarcoma) presenting as a soft-tissue sarcoma: Arch Pathol Lab Med, 1993; 117; 259-63
7.. Hong SG, Cho HM, Chin HM, Intravascular papillary endothelial hyper-plasia (Masson’s hemangioma) of the liver: A new hepatic lesion: J Korean Med Sci, 2004; 19(2); 305-8
8.. Amerigo J, Matilla A, Gonzalez-Campora R, Galera-Davidson H, Vegetant intravascular hemangioendothelioma of the skin. Report of 3 cases with a critical pathogenetic study: Dermatologica, 1979; 159; 476-81
9.. Cozzutto C, Guarino M, Dodera P, Nattera G, Intravascular endothelial praliferations in children: Am J Clin Pathol, 1979; 71; 247-52
10.. Luce EB, Montgomery MT, Redding SW, Aufdemorte TB, Intravascular angiomatosis (Masson’s lesion): J Oral Maxillofac Surg, 1988; 46; 736-41
11.. Bodner L, Dayan D, Intravascular papillary endothelial hyperplasia of the mandibular mucosa: Int J Oral Maxillofac Surg, 1991; 20; 273-74
12.. Torne Escasany R, Umbert Millet P, Masson’s pseudoangiosarcoma of the tongue: Report of two cases: J Cutan Pathol, 1985; 12; 66-71
13.. Stern Y, Braslavsky D, Shpitzcr T, Feinmesser R, Papillary endothelial hyper-plasia in the tongue: A benign lesion that may be mistaken for angiosarcoma: J Otolaryngol, 1994; 23; 81-83
14.. Ejeckam GC, Orizaga M, Gerin-Lajoie J, Castilio G, Intravascular angiomatosis of the pharynx (Masson’s vegetant intravascular haemangioendothelioma) – a case report: Br J Oral Surg, 1978; 16; 105-10
15.. Tedla M, Bežová M, Biró C, Intravascular papillary endothelial hyper-plasia of larynx: Case report and literature review of all head and neck cases: Otolaryngol Pol, 2014; 68(4); 200-3
16.. Rodriguez Campo FJ, Monje Gil F, Chayes Benito A, [Intravascular endothelial papillary hyperplasia in the masseter muscle]: Rev Stomatol Chir Maxillofac, 1991; 92; 420-24
17.. Corio RL, Brannon RB, Tarpley TM, Intravascular papillary endothelial hyperplasia of the head and neck: Ear Nose Thraat J, 1982; 61; 88-91
18.. Lancaster JL, Alderson DJ, Sherman IW, Clark AH, Papillary endothelial hyperplasia (Masson’s tumour) of the maxillary sinus: J Larnyngol Oto, 1998; 112; 500-2
19.. Komori A, Koike M, Kinjo T, Central intravascular papillary endothelial hyperplasia of the mandible: Virchows Arch A Pathol Anat Histopathol, 1984; 403; 453-59
20.. Weber FL, Babel J, Intravascular papillary endothelial hyperplasia of the orbit: Br J OphlhalmoI, 1981; 65; 18-22
21.. Werner MS, Hornblass A, Reifler DM, Intravascular papillary endothelial hyperplasia: Collection of four cases and a review of the literature: Ophthal Plast Reconstr Surg, 1997; 13; 48-56
22.. Pye JK, A case of Masson’s vegetant, intravascular haemangio-endotheliorna presenting in a large neck vein: Clin Oncol, 1983; 9; 57-59
23.. Youngerman JS, Intravascular papillary endothelial hyperplasia: Arch Otolaryngol, 1984; 110; 274-75 : 277
24.. Chen KT, Extravascular papillary endothelial hyperplasia: J Surg Oncol, 1987; 36; 52-54
25.. Hashimoto H, Daimaru Y, Enjoji M, Intravascular papillary endothelial hyperplasia. A clinicopathologic study of 91 cases: Am J Derrnat opathol, 1983; 5; 539-46
26.. Pantanowitz L, Muc R, Spanger M, Intravascular papillary endothelial hyperplasia (Masson’s tumor) manifesting as a lateral neck mass: Ear Nose Throat J, 2000; 79(10); 806 [809–10, 812 passim]
27.. Yücesoy C, Coban G, Yilmazer D, Intravascular papillary endothelial hyperplasia (Masson’s hemangioma) presenting as a lateral neck mass: JBR-BTR, 2009; 92(1); 20-22
28.. Alotaibi M, Geisler V, Olze H, Knopke S, Masson tumor as a neck mass: Eur Ann Otorhinolaryngol Head Neck Dis, 2020; 137(3); 217-18
29.. da Silva Leal M, Amado C, Paracana B, Masson’s tumour: A rare cause of cervical mass: Eur J Case Rep Intern Med, 2021; 8(12); 003078
30.. El Husseiny M, Benhaiem N, Vayssairat M, Allaire E, Masson’s vegetant intravascular hemangioendothelioma in an external jugular vein aneurysm with recurrent thrombosis: J Vasc Surg, 2011; 53(6); 1723-25
31.. Suh KS, Shin KS, Park IA, Intravascular papillary endothelial hyperplasia of the neck masquerading as malignancy on fine-needle aspiration cytology: Diagn Cytopathol, 2003; 29(1); 14-17
32.. Nangia A, Sehgal S, Agarwal K, Masson’s tumor masquerading as a papillary adenocarcinoma on fine needle aspiration cytology: A case report: Turk Patoloji Derg, 2018; 34(2); 179-81
33.. Shenton C, Sarwar S, Goswamy J, Cervicothoracic ‘no man’s land’: An unusual location of intravascular papillary endothelial hyperplasia in the supraclavicular fossa presenting a surgical challenge: BMJ Case Rep, 2021; 14; e240479
34.. Gutiérrez Sánchez AM, Calabuig Adobes A, Carmen Marcén G, Hiperplasia endotelial papilar intravascular en la región supraclavicular: Rev Pediatr Aten Primaria, 2021; 23; 87-90
35.. Franzen A, Günzel T, Buchali A, Coordes A, Etiologic and differential diagnostic significance of tumor location in the supraclavicular fossa: Laryngoscope, 2018; 128(3); 646-50
36.. Ellison E, LaPuerta P, Martin SE, Supraclavicular masses: Results of a series of 309 cases biopsied by fine needle aspiration: Head Neck, 1999; 21(3); 239-46
37.. Masson P, [Intravascular vegetative hemangioendothelioma.]: Bull Mem Soc Anat, 1923; 93; 517-23 [in French]
38.. Henschen P, [Thrombopoietin proliferative endovasculitis in local vascular lesion.]: Ann Anat Pathol, 1932; 9; 113-21
39.. Clearkin KP, Enzinger FM, Intravascular papillary endothelial hyperplasia: ArchPathol Lab Med, 1976; 100(8); 441-44
40.. Giannitto C, Mercante G, Spriano G, CT and MRI findings of head and neck Masson’s tumor: A rare case report and systematic review of the literature: Rep Med Imaging, 2021; 14; 53-64
41.. Salyer WR, Salyer DC, Intravascular angiomatosis: Development and distinctionfrom angiosarcoma: Cancer, 1975; 36(3); 995-1001
42.. Akdur NC, Donmez M, Gozel S, Intravascular papillary endothelial hyperplasia: Histomorphological and immunohistochemical features: Diagn Pathol, 2013; 8(1); 167
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