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      Intravascular papillary endothelial hyperplasia (Masson's tumor) of the scalp with intracranial extension

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          Abstract

          Intravascular papillary endothelial hyperplasia (IPEH) (Masson's tumor) is an unusual benign vascular lesion of the skin and subcutaneous tissue, consisting of papillary formations related to a thrombus and covered by a single layer of plump endothelial cells. The lesion is often mistaken with angiosarcoma and a group of other benign and malignant vascular lesions. The clinical and radiological findings are not specific, and the diagnosis is based on the histological examination. Intracranial lesions are extremely rare with only 32 cases been reported in the literature. Only two cases of IPEH presenting as scalp swelling have been reported in the literature. We report a case of a 3-month-old boy with IPEH of scalp in the left parietal region, which was involving the skull bone and extending intracranially.

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          Intravascular papillary endothelial hyperplasia. A clinicopathologic study of 91 cases.

          Ninety-one cases of intravascular papillary endothelial hyperplasia were studied clinically and histologically. This peculiar benign process, occasionally resembling hemangiosarcoma, was subgrouped in the following categories: a pure form that occurs within a dilated vascular space (30 cases), a mixed form that appears as a focal change in a hemangioma (55 cases), a third form (six cases) that belongs to neither of the first two. In the pure form, the lesions were most frequently situated in the subcutis of fingers (14 cases), of the head and neck (seven cases), and in the region between the elbows and hands (six cases). In the mixed form, half of the accompanying hemangiomas were intramuscular in no particular predilective sites. Papillary proliferation of endothelial cells was commonly found to be closely associated with thrombotic material and seemed to be an unusual feature of a thrombus undergoing organization.
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            Intravascular papillary endothelial hyperplasia.

            Papillary endothelial hyperplasia is a peculiar benign intravascular process that bears a remarkable resemblance to a hemangiosarcoma. In 44 cases of this lesion studied from the files of the Armed Forces Institute of Pathology, the process manifested as a small tumor-like lesion that occurred most frequently in the subcutis of the fingers (14 cases), the head and neck region (ten), and the trunk (seven). Microscopically, the tuft-like or papillary proliferation of endothelial cells was nearly always intimately associated with a thrombus and seemed to represent a peculiar variant of an organizing process. Features that aided in recognition and differential diagnosis from a hemangio-sarcoma included the intraluminal location of the lesion, the absence of tissue necrosis, and the intimate association of the proliferated tuft-like structures with thrombotic material. Follow-up information obtained in 31 cases indicated a benign clinical course despite the sarcoma-like microscopic appearance of this condition.
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              Masson's "vegetant intravascular hemangioendothelioma:" a lesion often mistaken for angiosarcoma: study of seventeen cases located in the skin and soft tissues.

              The occurrence of Masson's "hemangio-endotheliome vegetant intravasculaire" (Masson's pseudoangiosarcoma) in the skin and soft tissues is illustrated with 17 surgically excised specimens. Two forms are recognized; it may appear either as a pure lesion or as a focal condition in a pre-existing vascular process, such as pyogenic granuloma or hemangioma. The clinical appearance is not specific and the diagnosis can only be established by microscopic examination. It shows a predilection for the head and extremities. Its characteristic morphologic appearance makes possible its differentiation from a group of benign and malignant vascular proliferations. The key microscopic feature is the presence of a papillary growth composed of hyperplastic endothelial cells supported by delicate fibrous stalks entirely confined within the vascular lumen. The lesion should not be mistaken for angiosarcoma, since its clinical behavior is invariably benign.
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                Author and article information

                Journal
                J Pediatr Neurosci
                J Pediatr Neurosci
                JPN
                Journal of Pediatric Neurosciences
                Medknow Publications & Media Pvt Ltd (India )
                1817-1745
                1998-3948
                Sep-Dec 2014
                : 9
                : 3
                : 260-262
                Affiliations
                [1]Department of Neurosurgery, B. J. Medical College and Civil Hospital, Ahmedabad, Gujarat, India
                Author notes
                Address for correspondence: Dr. Divik H. Mittal, 2, Vithal Nagar, Civil Camp Road, Shahibaug, Ahmedabad - 380 004, Gujarat, India. E-mail: drdivik@ 123456yahoo.com
                Article
                JPN-9-260
                10.4103/1817-1745.147584
                4302549
                25624932
                86510cb4-8083-4ef5-8bab-2789d0e15c62
                Copyright: © Journal of Pediatric Neurosciences

                This is an open-access article distributed under the terms of the Creative Commons Attribution-Noncommercial-Share Alike 3.0 Unported, which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.

                History
                Categories
                Case Report

                Neurosciences
                intravascular papillary endothelial hyperplasia,masson's tumor,scalp swelling

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