|Year : 2022 | Volume
| Issue : 3 | Page : 370-372
Masson's tumor of the ocular surface – A rare clinical entity
Marian Pauly1, R Sruthi1, Krishnakumar Subramanian2, Anantharaman Giridhar1
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 Submission||21-May-2021|
|Date of Acceptance||17-Oct-2021|
|Date of Web Publication||02-Nov-2022|
Giridhar Eye Institute, Kochi - 682 020, Kerala
Source of Support: None, Conflict of Interest: None
| Abstract|| |
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
| Introduction|| |
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. Eyelid and orbital involvement have been reported in various series., 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|| |
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|| |
IPEH was first described in 1923 by Pierre Masson. 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. He suggested that proliferation of endothelial cells is the primary event and the thrombus arises secondary to this. In 1932, Henschen described that the endothelial proliferation in IPEH is secondary to an organization of a preexisting thrombus. This was supported by Clearkin and Enzinger 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. 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.
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.
In 1983, Hashimoto et al. 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.
Periocular and orbital lesions are rare. In 1981, Weber and Babel 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. reported a case of orbital IPEH with associated vision loss. In 1983, Font et al. reported a case series of orbital and adnexal IPEH lesions.
The first reported case of IPEH in eyelid was by Daicker and Raffa in 1972. In 1974, Wolter and Lewis 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. in 1983 reported IPEH in a 20-year-old lady which arose in a preexisting vascular anomaly. In 1997, Werner et al. 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.
Surgical excision is the treatment of choice, and complete resection prevents recurrence in most of the cases. Adjuvant chemotherapy and radiotherapy also have been described in cases with incomplete resection. Gamma knie radiosurgery is said to be useful in smaller lesions, which are not amenable to complete resection.
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.
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