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 Table of Contents  
Year : 2016  |  Volume : 6  |  Issue : 1  |  Page : 33-35

Intravascular papillary endothelial hyperplasia (Masson's tumor) as a nasal mass: a case report and review of the literature

Department of Otolaryngology-Head and Neck Surgery, College of Medicine, King Abdul Aziz University Hospital, King Saud University, Riyadh, Kingdom of Saudi Arabia

Date of Submission23-Feb-2016
Date of Acceptance09-Mar-2016
Date of Web Publication26-Jul-2016

Correspondence Address:
Khalid H Al-Qahtani
Department of Otolaryngology-Head and Neck Surgery, College of Medicine, King Abdul Aziz University Hospital, King Saud University, PO Box 245, Riyadh 11411
Kingdom of Saudi Arabia
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/2090-7540.183731

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Intravascular papillary endothelial hyperplasia (Masson's tumor) is a rare non-neoplastic vascular proliferative process in a normal blood vessel or vascular malformation. Nose and paranasal sinuses are extremely rare location for this type of tumor. We report a case of Masson's tumor in a 33-year-old female, presented with an obstructing nasal mass in the right side, repeated epistaxis and rhinorrhea for eight months associated with anosmia, frontal headache and proptosis.

Keywords: intravascular papillary endothelial hyperplasia, Masson's tumor, soft tissues

How to cite this article:
Al-Qahtani KH. Intravascular papillary endothelial hyperplasia (Masson's tumor) as a nasal mass: a case report and review of the literature. Pan Arab J Rhinol 2016;6:33-5

How to cite this URL:
Al-Qahtani KH. Intravascular papillary endothelial hyperplasia (Masson's tumor) as a nasal mass: a case report and review of the literature. Pan Arab J Rhinol [serial online] 2016 [cited 2020 May 25];6:33-5. Available from: http://www.PAJR.eg.net/text.asp?2016/6/1/33/183731

  Introduction Top

Intravascular papillary endothelial hyperplasia (Masson's tumor) is a non-neoplastic vascular proliferative process in a normal blood vessel, or vascular malformation characterized by papillary proliferation of the vascular endothelial cells that mimic angiosarcoma clinically, radiologically, and histopathologically. Although commonly presented in the head and neck region, its occurrence in the nose and paranasal sinuses is extremely rare. This paper describes a case of intravascular papillary endothelial hyperplasia presenting as an obstructing nasal mass extending into paranasal sinuses with successful endoscopic excision. Awareness of this benign lesion and its distinguished histopathological features is essential for correct management.

  Report of Case Top

A 33-year-old female patient, who was otherwise healthy, presented with an obstructing nasal mass in the right side, associated with repeated epistaxis and rhinorrhea for 8 months and associated with anosmia, frontal headache, and proptosis. There was no history of trauma or other related otolaryngological illnesses, and there were no other ophthalmological problems.

Physical examination showed an extensive reddish swelling in the right nostril, producing a hump over the nasal bone, with unremarkable ear, throat, and head and neck examination.

Computed tomography scan showed a large heterogeneously enhancing mass lesion seen in the right nasal cavity measuring about 4.28 × 2.7 × 3.7 cm. There was involvement of maxillary, ethmoid, frontal, and sphenoid sinuses with evidence of pressure erosions in the surrounding bone structures.

Histopathological report revealed hemangioma with foci consistent with papillary endothelial hyperplasia with no evidence of malignancy.

Excision was performed through an endoscopic approach, and the patient recovered with no complications or symptoms and was followed up for 4 years with no evidence of recurrence ([Figure 1], [Figure 2], [Figure 3]).
Figure 1: Axial computed tomography scan showing soft tissue densification occupying the right nasal cavity.

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Figure 2: Axial computed tomography scan showing the soft tissue lesion after the surgical removal.

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Figure 3: Histopathological appearance showing hemangioma consistent with intravascular papillary endothelial hyperplasia.

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  Discussion Top

Intravascular papillary endothelial hyperplasia is a peculiar benign reactive condition caused by an excessive proliferation of endothelial cells in blood vessels [1]. It presents in three different settings: as a primary or pure form involving an isolated dilated blood vessel; as a secondary focal change in a variety of pre-existing vascular lesions such as hemangiomas, hemorrhoidal veins, and varices; and, rarely, it can present in an extravascular location in association with a hematoma [2],[3].

It was first described by Masson in 1923 as a 'hemangioendothelioma vegetant intravasculaire' that was formed within the lumen of a hemorrhoidal vein believing it to be a true neoplasm [1].

The pathogenesis of this lesion continues to remain unclear; most researchers believe that it is an unusual type of organizing thrombus [4],[5],[6].

Although these lesions have the predilection to occur in the skin and subcutaneous soft tissue of the hand, head and neck, and trunk, these tumors can occur in any blood vessel [7]. A variety of locations have been described in the literature for the intravascular papillary endothelial hyperplasia. However, its occurrence in the nasal cavity and paranasal sinuses is extremely rare. To the best of our knowledge, there are only three cases of nose involvement and four cases of paranasal sinuses that have been reported. These reports are summarized in [Table 1]. Our case differs from other nasal cases in that it extends into a wide range of paranasal sinuses.
Table 1 Reported cases of Intravascular Papillary Endothelial Hyperplasia

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The lesion has been clinically mistaken most commonly for angiosarcoma [15]. Other lesions that are to be considered in differential diagnosis include intravenous pyogenic granuloma, mucocele, Kaposiís sarcoma, and other vascular conditions such as hemangioma, papular angioplasia, angioendothelioma, Kimuraís disease, bacillary angiomatosis, and intravenous atypical vascular proliferation [16],[17],[18].

Histopathological examination is the confirmatory diagnosis and it is described by the presence of a single layer of attenuated endothelial cells covering multiple small papillary structures with little or no atypia. Mitosis is usually absent. The papillary core is made up of hypocellular, hyaline collagen in association with occasional small capillaries. It should be differentiated from angiosarcoma in that the latter is usually an extravascular process characterized by an extensive infiltrative or dissecting pattern that exhibits cytologic atypia, endothelial multilayering, mitotic activity, and irregular anastomosing blood vessels [19]. Immunohistochemically, the Masson's tumor tends to show variably positive staining for CD34, CD31, and smooth muscle actin [20],[21],[22]. They also show positive staining for ferritin, type VIII collagen, and vimentin [4].

The prognosis is excellent as intravascular papillary endothelial hyperplasia does not transform into malignancy or recur after adequate excision. Its possible location in the sinonasal cavity should be borne in mind and careful histopathological examination is essential to avoid misdiagnosis and inappropriate therapy.

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Conflicts of interest

There are no conflicts of interest.

  References Top

Kuo T, Sayers CP, Rosai J. Masson's 'vegetant intravascular hemangioendothelioma': a lesion often mistaken for angiosarcoma: study of seventeen cases located in the skin and soft tissues. Cancer 1976; 38(3):1227–1236.  Back to cited text no. 1
Hashimoto H, Daimaru Y, Enjoji M. Intravascular papillary endothelial hyperplasia. A clinicopathologic study of 91 cases. Am J Dermatopathol 1983; 5(6):539–546.  Back to cited text no. 2
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(3):259–263.  Back to cited text no. 3
Albrecht S, Kahn HJ. Immunohistochemistry of intravascular papillary endothelial hyperplasia. J Cutan Pathol 1990; 17(1):16–21.  Back to cited text no. 4
Salyer WR, Salyer DC. Intravascular angiomatosis: development and distinction from angiosarcoma. Cancer 1975; 36(3):995–1001.  Back to cited text no. 5
Eusebi V, Fanti PA, Fedeli F, Mancini AM. Masson's intravascular vegetant hemangioendothelioma. Tumori 1980; 66(4):489–498.  Back to cited text no. 6
Sickler GK, Langford LA. Intracranial tumor-forming papillary endothelial hyperplasia – a case report. Clin Neuropathol 1990; 9(3):125–128.  Back to cited text no. 7
Moon WS, Chung GH, Hong KH. Intravascular papillary endothelial hyperplasia in a vascular lesion of the paranasal sinus. Arch Pathol Lab Med 2000; 124(8):1224–1227.  Back to cited text no. 8
Hooda S, Humphreys MR, Wong SW, Evans AS. Masson's pseudotumour of the ethmoid sinus – a case report. J Laryngol Otol 2008; 122(9):990–992.  Back to cited text no. 9
Lancaster JL, Alderson DJ, Sherman IW, Clark AH. Papillary endothelial hyperplasia (Masson's tumour) of the maxillary sinus. J Laryngol Otol 1998; 112(5):500–502.  Back to cited text no. 10
Stern Y, Braslavsky D, Segal K, Shpitzer T, Abraham A. Intravascular papillary endothelial hyperplasia in the maxillary sinus. A benign lesion that may be mistaken for angiosarcoma. Arch Otolaryngol Head Neck Surg 1991; 117(10):1182–1184.  Back to cited text no. 11
Stevens DJ. Papillary endothelial hyperplasia in the nose. J Laryngol Otol 1988; 102(10):935–937.  Back to cited text no. 12
Safneck JR, Alguacil-Garcia A, Dort JC. Intranasal papillary endothelial hyperplasia. Otolaryngol Head Neck Surg 1995; 113(6):766–770.  Back to cited text no. 13
Wang ZH, Hsin CH, Chen SY, Lo CY, Cheng PW. Sinonasal intravascular papillary endothelial hyperplasia successfully treated by endoscopic excision: a case report and review of the literature. Auris Nasus Larynx 2009; 36(3):363–366.  Back to cited text no. 14
Barr RJ, Graham JH, Sherwin LA. Intravascular papillary endothelial hyperplasia. A benign lesion mimicking angiosarcoma. Arch Dermatol 1978; 114(5):723–726.  Back to cited text no. 15
Tosios K, Koutlas IG, Papanicolaou SI. Intravascular papillary endothelial hyperplasia of the oral soft tissues: report of 18 cases and review of the literature. J Oral Maxillofac Surg 1994; 52(12):1263–1268.  Back to cited text no. 16
Kawashima A, Johsen T, Murayama S, Russell WJ. Intravascular papillary endothelial hyperplasia of the adrenal gland. Br J Radiol 1986; 59(702):610–613.  Back to cited text no. 17
Rosai J, Akerman LR. Intravenous atypical vascular proliferation. A cutaneous lesion simulating a malignant blood vessel tumor. Arch Dermatol 1974; 109(5):714–717.  Back to cited text no. 18
Meis-Kindblom JM, Kindblom LG. Angiosarcoma of soft tissue: a study of 80 cases. Am J Surg Pathol 1998; 22(6):683–697.  Back to cited text no. 19
Shih CS, Burgett R, Bonnin J, Boaz J, Ho CY. Intracranial Masson tumor: case report and literature review. J Neurooncol 2012; 108(1):211–217.  Back to cited text no. 20
Zhang R, Zhou LF, Mao Y, Wang Y. Papillary endothelial hyperplasia (Masson tumor) of the petrous and jugulare region: case report and literature review. Surg Neurol 2005; 64(1):55–60.  Back to cited text no. 21
Soares AB, Altemani A, Furuse C, Demasi AP, Gati C, Nunes N, de Araújo VC Intravascular papillary endothelial hyperplasia: report of 2 cases and immunohistochemical study. Oral Surg Oral Med Oral Pathol Oral Radiol Endod 2008; 106(5):708–711.  Back to cited text no. 22


  [Figure 1], [Figure 2], [Figure 3]

  [Table 1]

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