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      Intravascular Papillary Endothelial Hyperplasia (Masson’s Tumor) of the Supraclavicular Region: Management of a Rare Case

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          Abstract

          Patient: Female, 24-year-old

          Final Diagnosis: Hemangioendothelioma

          Symptoms: Swelling

          Medication: —

          Clinical Procedure: —

          Specialty: Otolaryngology • Surgery

          Objective:

          Rare disease

          Background:

          Masson’s tumor, also known as IPEH (intravascular papillary endothelial hyperplasia), is a rare benign vascular process, comprising approximately 2% of vascular tumors of the skin and subcutaneous tissue. IPEH presenting as a neck mass is rare, with only 5 reports of anterior neck mass and 7 cases of lateral neck lesions, of which 1 was in an external jugular vein aneurysm. To the best of our knowledge, the localization of intravascular papillary endothelial hyperplasia in the supraclavicular region is rarely reported.

          Case Report:

          We describe our management of a non-traumatic and non-painful mass on the right supraclavicular region in 24-year-old woman. Ultrasound revealed a heterogeneously hypoechoic mass with intense vascularization. Magnetic resonance imaging (MRI) showed a formation with lobulated contours and closely related to muscular layers of serratus anterior muscle. The findings of ultrasound-guided biopsy (FNA) were inconclusive. Complete removal of the mass was performed. Histopathological examination showed a well-circumscribed lesion with numerous small papillary structures. The papillae had hyalinized hypocellular cores covered by flattened endothelium. Immunohistochemical analysis showed endothelial positivity for CD34 and CD31. These features are typical of IPEH. No recurrence had occurred at 12 months after surgery.

          Conclusions:

          The differential diagnosis of malignant tumors like angiosarcoma is important because the prognosis significantly differs. Surgical excision is the treatment of choice. In our experience, the size of the lesion and its critical landmarks have not influenced the outcomes of the surgical procedure in terms of potential functional damages.

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          Most cited references42

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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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              Florid extravascular papillary endothelial hyperplasia (Masson's pseudoangiosarcoma) presenting as a soft-tissue sarcoma.

              Papillary endothelial hyperplasia (also known as Masson's pseudoangiosarcoma) is a hyperplastic proliferation of endothelial cells that is usually an incidental finding within thrombosed dilated blood vessels or vascular tumors. Rare extravascular forms occur and can histologically closely mimic angiosarcoma. We report a case of extravascular papillary endothelial hyperplasia that extensively involved a large, traumatic, deep soft-tissue hematoma in a 19-year-old man and clinically presented as a soft-tissue sarcoma. We also summarize characteristics of 314 cases of papillary endothelial hyperplasia reported in the literature and identify 13 likely cases of the rare extravascular form.
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                Author and article information

                Journal
                Am J Case Rep
                Am J Case Rep
                amjcaserep
                The American Journal of Case Reports
                International Scientific Literature, Inc.
                1941-5923
                2022
                28 August 2022
                : 23
                : e936402-1-e936402-5
                Affiliations
                [1 ]ENT Unit, Department of Mental Health and Public Medicine, University of Campania “L. Vanvitelli”, Naples, Italy
                [2 ]Thoracic Surgery Unit, Department of Cardio-Thoracic and Respiratory Sciences, University of Campania “L. Vanvitelli”, Naples, Italy
                [3 ]Pathology Unit, Department of Mental Health and Physical and Preventive Medicine, University of Campania “L. Vanvitelli”, Naples, Italy
                Author notes
                Corresponding Author: Eva Aurora Massimilla, e-mail: evamassimilla@ 123456gmail.com , evaaurora.massimilla@ 123456unicampania.it

                Authors’ Contribution:

                [A]

                Study Design

                [B]

                Data Collection

                [C]

                Statistical Analysis

                [D]

                Data Interpretation

                [E]

                Manuscript Preparation

                [F]

                Literature Search

                [G]

                Funds Collection

                Financial support: None declared

                Conflict of interest: None declared

                Article
                936402
                10.12659/AJCR.936402
                9438939
                36030377
                26395ee2-a9d5-492c-942c-478b75ff7f3c
                © Am J Case Rep, 2022

                This work is licensed under Creative Common Attribution-NonCommercial-NoDerivatives 4.0 International ( CC BY-NC-ND 4.0)

                History
                : 15 February 2022
                : 30 June 2022
                : 26 July 2022
                Categories
                Articles

                vascular neoplasms,head and neck neoplasms,subcutaneous tissue,neck,antigens, cd34,vascular diseases

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