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 Table of Contents    
Year : 2022  |  Volume : 15  |  Issue : 3  |  Page : 370-372  

Masson's tumor of the ocular surface – A rare clinical entity

1 Department of Orbit and Oculoplasty, Giridhar Eye Institute, Cochin, Kerala, India
2 Department of Pathology, Vision Research Foundation, Chennai, Tamil Nadu, India

Date of Submission21-May-2021
Date of Acceptance17-Oct-2021
Date of Web Publication02-Nov-2022

Correspondence Address:
R Sruthi
Giridhar Eye Institute, Kochi - 682 020, Kerala
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/ojo.ojo_162_21

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Masson's tumor or intravascular papillary endothelial hyperplasia (IPEH) is a benign vascular lesion usually involving the head-and-neck region. On histopathological examination, it consists of reactive proliferation of endothelial cells with papillary formations which is the key to diagnosis. This rare entity was first described in 1923 by Pierre Masson. Lesions involving orbit and eyelids have been reported before. Here, we report a case of Masson's tumor which occurred in the lid margin and later in the conjunctiva which regressed completely after excision.

Keywords: Conjunctiva, excisional biopsy, hemangioma, histopathology, Masson's tumor

How to cite this article:
Pauly M, Sruthi R, Subramanian K, Giridhar A. Masson's tumor of the ocular surface – A rare clinical entity. Oman J Ophthalmol 2022;15:370-2

How to cite this URL:
Pauly M, Sruthi R, Subramanian K, Giridhar A. Masson's tumor of the ocular surface – A rare clinical entity. Oman J Ophthalmol [serial online] 2022 [cited 2023 Jan 27];15:370-2. Available from: https://www.ojoonline.org/text.asp?2022/15/3/370/360397

   Introduction Top

Intravascular papillary endothelial hyperplasia (IPEH) is a benign vascular lesion usually involving the head-and-neck region and the upper extremities. IPEH is alternatively known as Masson's hemangioma and Masson's intravascular hemangioendothelioma. It consists of reactive proliferation of endothelial cells with papillary formations related to a thrombus involving the skin and subcutaneous tissue mostly.[1] Eyelid and orbital involvement have been reported in various series.[1],[2] To our knowledge, no case with a conjunctival involvement has been reported yet.

Here, we report a case of Masson's tumor which occurred in the lid margin and later in the conjunctiva after 1 year which mimicked ocular surface squamous neoplasia.

   Case Report Top

A 56-year-old gentleman presented with a history of recurrent episodes of bleeding from a lesion in the left upper eyelid for 2 years. Excision and cauterization of the base was done multiple times in the past. The best-corrected visual acuity was 6/6 N6 in both eyes. On examination, he had a vascular lesion of size 5 mm × 3 mm involving the medial part of the left upper eyelid. He also had two blackish elevated skin lesions of size 2 mm × 1 mm involving the right side of the forehead. With the provisional diagnosis of hemangioma/pyogenic granuloma of the lid, we excised the lesion under local anesthesia and the base was cauterized. The histopathology showed sinusoidal channels with papillary projection into the lumen with prominent endothelial cells in the dermis. Areas of hemorrhages and siderophages were seen. In the deep dermis, there was an area of hemorrhage which was suggestive of IPEH.

One year later, the patient presented with a lesion in the conjunctiva [Figure 1]a with a similar appearance of the previous lesion. It was a reddish elevated friable lesion of size 4 mm × 2 mm × 2 mm involving the medial bulbar conjunctiva near the limbus in the left eye. The lesion was excised under local anesthesia and the base was cauterized. Histopathology [Figure 1]b showed IPEH possibly in a venous lake. There were no recurrences at 1-year follow-up.
Figure 1: (a) Clinical picture showing conjunctival and skin lesions. (b) Histopathology

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   Discussion Top

IPEH was first described in 1923 by Pierre Masson.[3] He called this lesion “hemangioendotheliome vegetant intra-vasculaire” which was found as an atypical papillary endothelial hyperplasia in a 68-year-old patient with infected hemorrhoids.[4] He suggested that proliferation of endothelial cells is the primary event and the thrombus arises secondary to this. In 1932, Henschen[5] described that the endothelial proliferation in IPEH is secondary to an organization of a preexisting thrombus. This was supported by Clearkin and Enzinger[6] in 1976. They identified 44 cases of IPEH and they found the presence of thrombus in all cases by pathological examination. Currently, it is considered to be a vascular prolieration following a traumatic vascular stasis.[7] IPEH formation is proposed to be due to the release of basic fibroblast growth factor by the invading macrophages to the trauma site with the proliferation of endothelial cells.[8]

IPEH usually occurs in the skin and subcutaneous tissue with more predilection to head-and neck-region and extremities. It presents as a small, firm, superficial mass with red to bluish discoloration of the overlying skin.[7]

In 1983, Hashimoto et al.[9] described a clinical classification by their study of 91 cases of IPEH – a pure form that occurs within a dilated vascular space (most frequently situated in the subcutis of the fingers, of the head and neck, and in the region between the elbows and hands), a mixed form that appears as a focal change in a hemangioma (mostly intramuscular with no particular predictive sites), and a third form that belongs to neither of the first two.

The diagnosis is confirmed on microscopic examination. The endothelial cell proliferation is histopathologically characterized by confinement to the intravascular space, absence of anaplasia or multiple mitosis, lack of true fibrous papillary fronds, and the presence of papillae consisting of fibrohyalinized tissue covered by pleomorphic endothelium.[10]

Periocular and orbital lesions are rare. In 1981, Weber and Babel[11] first reported a case of IPEH in the orbit. They reported a case of 20-year-old girl with unilateral progressive exophthalmos. The tumor described in their report was said to be probably developed in a dilated intraorbital vascular malformation. There have been many cases of orbital IPEH since then. Fasina et al.[12] reported a case of orbital IPEH with associated vision loss. In 1983, Font et al.[1] reported a case series of orbital and adnexal IPEH lesions.

The first reported case of IPEH in eyelid was by Daicker and Raffa[13] in 1972. In 1974, Wolter and Lewis[14] reported a case of IPEH in a 25-year-old man with an upper lid lesion which he was provisionally diagnosed as hemangioma. Sorenson et al.[15] in 1983 reported IPEH in a 20-year-old lady which arose in a preexisting vascular anomaly. In 1997, Werner et al.[2] published a case series with three cases of Masson's tumor in the eyelid and one in the orbital margin.

In our case, there was a lid margin lesion of IPEH which on excision was completely resolved. However, the same pathology recurred in the conjunctiva. This is the first reported case where sequential involvement of eyelid and conjunctiva has occurred. The conjunctival lesion was mimicking a case of ocular surface squamous neoplasia. But, the color of the lesion, absence of feeder vessels and the lack of surface keratin were the features pointing towards the diagnosis of IPEH. However, histopathology is the key in diagnosis. He also had similar lesions in the skin of the forehead region.

The histopathological differential diagnosis is angiosarcoma. However, the presence of extravascular extension, more than one or two layers of endothelium covering the papillary formations, and malignant features in cytology are the key features of angiosarcoma which differentiates it from IPEH.[16]

Surgical excision is the treatment of choice, and complete resection prevents recurrence in most of the cases.[1] Adjuvant chemotherapy and radiotherapy also have been described in cases with incomplete resection.[17] Gamma knie radiosurgery is said to be useful in smaller lesions, which are not amenable to complete resection.[18]

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form, the patient has given his consent for his images and other clinical information to be reported in the journal. The patient understands that his name and initials will not be published and due efforts will be made to conceal identity, but anonymity cannot be guaranteed.

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Conflicts of interest

There are no conflicts of interest.

   References Top

Font RL, Wheeler TM, Boniuk M. Intravascular papillary endothelial hyperplasia of the orbit and ocular adnexa. A report of five cases. Arch Ophthalmol 1983;101:1731-6.  Back to cited text no. 1
Werner MS, Hornblass A, Reifler DM, Dresner SC, Harrison W. Intravascular papillary endothelial hyperplasia: Collection of four cases and a review of the literature. Ophthalmic Plast Reconstr Surg 1997;13:48-56.  Back to cited text no. 2
Steffen C. The man behind the eponym: C. L. Pierre Masson. Am J Dermatopathol 2003;25:71-6.  Back to cited text no. 3
Masson P. Intravascular vegetative hemangioendothelioma. Bull Soc Anat (Paris) 1923;93:517.  Back to cited text no. 4
Henschen F. Thrombopoietic proliferative endovasculitis in local vascular injury. Ann Anath Pathl (Paris) 1932;9:113.  Back to cited text no. 5
Clearkin KP, Enzinger FM. Intravascular papillary endothelial hyperplasia. Arch Pathol Lab Med 1976;100:441-4.  Back to cited text no. 6
Mahapatra QS, Sahai K, Malik A, Mani NS. Intravascular papillary endothelial hyperplasia: An unusual histopathological entity. Indian Dermatol Online J 2015;6:277-9.  Back to cited text no. 7
[PUBMED]  [Full text]  
Levere SM, Barsky SH, Meals RA. Intravascular papillary endothelial hyperplasia: A neoplastic “actor” representing an exaggerated attempt at recanalization mediated by basic fibroblast growth factor. J Hand Surg Am 1994;19:559-64.  Back to cited text no. 8
Hashimoto H, Daimaru Y, Enjoji M. Intravascular papillary endothelial hyperplasia. A clinicopathologic study of 91 cases. Am J Dermatopathol 1983;5:539-46.  Back to cited text no. 9
Kuo TT, Sayers P, Rosai J. Masson's 'vegetant intra-vascular hemangioendothelioma': A lesion often mistaken for angiosarcoma. Cancer 1976;38:1227-36.  Back to cited text no. 10
Weber FL, Babel J. Intravascular papillary endothelial hyperplasia of the orbit. Br J Ophthalmol 1981;65:18-22.  Back to cited text no. 11
Fasina O, Adeoye A, Akang E. Orbital intravascular papillary endothelial hyperplasia in a Nigerian child: A case report and review of the literature. J Med Case Rep 2012;6:300.  Back to cited text no. 12
Daicker B, Raffa H. Endovasale Hämangiome der Vena angularis. Ophthalmologica 1972;165:472-81.  Back to cited text no. 13
Wolter JR, Lewis RG. Endovascular hemangioendothelioma of the eyelid. Am J Ophthalmol 1974;78:727-9.  Back to cited text no. 14
Sorenson RL, Spencer WH, Stewart WB, Miller WW, Kleinhenz RJ. Intravascular papillary endothelial hyperplasia of the eyelid. Arch Ophthalmol 1983;101:1728-30.  Back to cited text no. 15
Schulze S, Hoerle S, Koop G, Barth PJ, Huegens-Penzel M, Strempel I. Intravascular papillary endothelial hyperplasia-A rare finding in the orbital region. Ophthalmologica 2008;222:213-5.  Back to cited text no. 16
Sim SY, Lim YC, Won KS, Cho KG. Thirteen-year follow-up of parasellar intravascular papillary endothelial hyperplasia successfully treated by surgical excision: Case report. J Neurosurg Pediatr 2015;15:384-91.  Back to cited text no. 17
Ohshima T, Ogura K, Nakayashiki N, Tachibana E. Intravascular papillary endothelial hyperplasia at the superior orbital fissure: Report of a case successfully treated with gamma knife radiosurgery. Surg Neurol 2005;64:266-9.  Back to cited text no. 18


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