Papillary Endothelial Hyperplasia (Masson Tumor) of the Hand. Surgical and Pathological Consideration from Seven Cases U
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Papillary Endothelial Hyperplasia (Masson Tumor) of the Hand. Surgical and Pathological Consideration from Seven Cases Using New Vascular Markers Bernadett Bettina Patai 1 & Nora Peterfy 2 & Noemi Szakacs 3 & Zoltan Sapi 4 & Judit Reka Hetthessy 3 Received: 27 January 2020 / Accepted: 10 June 2020 # The Author(s) 2020
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. Keywords Hand . Soft tissue lesion . Vascular tumor . Vascular malformation . Intravascular papillary endothelial hyperplasia . IPEH . Masson tumor
Abbreviations CSM circulation FNAB fine needle aspiration biopsy IPEH Intravascular Papillary Endothelial Hyperplasia
ROM US
Range of motion ultrasound
Introduction * Judit Reka Hetthessy [email protected] 1
Department of Traumatology, Military Health Centre, Budapest, Hungary
2
Department of Traumatology and Hand surgery, Saint John’s Hospital, Budapest, Hungary
3
Department of Orthopedics, Semmelweis University, Budapest, Hungary
4
First Department of Pathology and Experimental Cancer Research, Semmelweis University, Budapest, Hungary
Masson tumor is a rare malformation of skin and subcutaneous tissues and was first described by Pierre Masson in 1923 [19]. Intravascular Papillary
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