Abstract
Although papillary endothelial hyperplasia may occur at almost any site, one of the most common sites is the hand. It is generally regarded as a reactive vascular proliferation i.e. exuberant form of organizing thrombus. Diagnosis of Masson tumor can be challenging due to its close clinical, radiological and even histopathological resemblance to angiosarcoma. We present seven cases of Masson tumor of the hand; wanting to reveal its nature using new vascular markers and discuss the treatment options and expected outcomes, present clinical and radiological features that may aid diagnosis and also offer treatment plans. A multicenter retrospective study was performed between January 2014 and November 2019. Immunohistochemical stains of Glut1, WT1, ERG, CD31 and alpha smooth muscle actin (ASMA) were performed on each cases. We found seven cases during the examined period. 4 out of 7 cases were women. All lesions occurred in the hands. 3 out of 7 cases appeared in a previously present vascular malformation. All cases were treated with surgical excision and the diagnosis of papillary endothelial hyperplasia was made by histology. Pre-operative testing (radiograph/MRI/US/fine needle aspiration biopsy) did not suggest the diagnosis of Masson tumor; however, aspiration cytology could rule out malignancy. The proliferative endothelial cells proved to be Glut1 negative and WT1 positive and the accompanying pericytic cells were ASMA positive in all cases. Though Masson tumor is a rare vascular lesion in the hand among other vascular tumors, it should be considered in the differential diagnostics even in the case of previously existing vascular malformation. WT1 positivity of the endothelial cells and the accompanying pericytic cells raises the question whether the initially reactive endothelial proliferation may transform into a true benign vascular tumor.
Highlights
Masson tumor is a rare malformation of skin and subcutaneous tissues and was first described by Pierre Masson in 1923 [19]
The lesions were analyzed according to whether they formed on a pre-existing vascular lesion, or if they could be considered de novo
Benign vascular tumors can be characterized by the accompanying pericytes, the lack of a pericytic layer is quite specific for the malignant vascular tumors
Summary
Masson tumor is a rare malformation of skin and subcutaneous tissues and was first described by Pierre Masson in 1923 [19]. Intravascular Papillary Endothelial Hyperplasia (IPEH), called Masson’s Tumor/ Masson Tumor, is an entity that is described as appearing as either a reactive or a neoplastic process and as a benign entity [30]. It presents with exuberant organization and recanalization of thrombi [17, 25]. Palpable mass, pain, swelling, and limited range of motion may occur These lesions are treated by orthopaedic, trauma or hand surgeons but it is not rare for patients to be seen by general or plastic surgeons. Though 15% of recurrence rate was noted in recent literature; [8, 28] all of these cases had a benign natural history
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