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      Unresectable Retiform Hemangioendothelioma Treated with External Beam Radiation Therapy and Chemotherapy: A Case Report and Review of the Literature

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          Abstract

          Retiform hemangioendothelioma (RH) is an infrequently encountered vascular neoplasm of intermediate or borderline malignancy. Treatment of RH is controversial. We present a case of a 44-year-old Asian male presenting with an unresectable RH of the pelvis. The patient was treated with concurrent low-dose Cisplatin and External beam Radiation (4140cGy in 180cGy per fraction). This is the first report of a clinical complete response and a long-term local control of this rare tumor. This has significant clinical implication, since it gives the first evidence of treatment of this rare tumor using concurrent low-dose chemotherapy and radiation.

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          Retiform hemangioendothelioma. A distinctive form of low-grade angiosarcoma delineated in a series of 15 cases.

          E Calonje, Menna Jones, Paul C. Fletcher … (1994)
          Fifteen cases of a distinctive type of low-grade angiosarcoma of the skin are described. Most tumors presented in the second to fourth decades of life, the youngest patient being 9 years old and the oldest 78 (mean age, 36 years). There was no sex predilection. Six tumors arose on the lower limb, four on the upper limb, three on the trunk, and one each on the penis and the scalp. One case arose in the setting of chronic lymphedema and another following radiotherapy for carcinoma of the uterine cervix. Distinctive morphologic features were the presence of long arborizing blood vessels arranged in a retiform pattern (reminiscent of normal rete testis) lined by monomorphic hobnail endothelial cells, a very prominent lymphocytic infiltrate in most cases, and the focal presence of papillae with hyaline collagenous cores, similar to those seen in malignant endovascular papillary angioendothelioma (Dabska's tumor). With a median follow-up of 7.25 years in 14 cases, retiform hemangioendothelioma has proved to be a low-grade neoplasm that recurs frequently but has a very low metastatic rate. The single regional lymph node metastasis in this series was from a case with a biphasic pattern in which only the spindle cell component was represented in the metastasis. There have been no tumor-related deaths, underlining the importance of accurate distinction from conventional angiosarcoma. This distinction is facilitated principally by the absence of dissection between individual collagen bundles and the absence of endothelial atypia or mitotic activity. The precise relationship between retiform hemangioendothelioma and Dabska's tumor is uncertain, possibly because cases of the latter may not be homogeneous.
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            Retiform hemangioendothelioma: a case report and review of the literature.

            Thaer Khoury, Dongfeng Tan, R Cheney … (2005)
            Retiform hemangioendothelioma (RH) is a rare, recently described vascular neoplasm of low malignant potential. We report a case of RH of the foot of a 19-year-old white female. Histologically, the tumor grew as numerous elongated vessels resembling the shape of rete testis with involvement of the skin adnexal structures and subcutaneous adipose tissue. No dissection of collagen by small groups of endothelial cells was seen. Wide local excision was performed and the patient was healthy with no metastasis at 14 months follow-up. Our case is discussed in the context of previously reported cases of RH; we also include a review of all cases of RH published to date.
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              Retiform Hemangioendothelioma

              Edward Wilson-Jones, Eduardo Calonje, Juan Rosai … (1994)
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                Author and article information

                Journal
                Journal ID (nlm-ta): Sarcoma
                Journal ID (publisher-id): SRCM
                Title: Sarcoma
                Publisher: Hindawi Publishing Corporation
                ISSN (Print): 1357-714X
                ISSN (Electronic): 1369-1643
                Publication date (Print): 2010
                Publication date (Electronic): 26 September 2010
                Volume: 2010
                Electronic Location Identifier: 756246
                Affiliations
                1Stich Radiation Center, Weill Cornell Medical Center, New York, NY 10065, USA
                2Department of Hematology/Oncology, New York Hospital Queens, NY 11355, USA
                Author notes

                Academic Editor: Alberto Pappo

                Article
                DOI: 10.1155/2010/756246
                PMC ID: 2948930
                PubMed ID: 20936126
                SO-VID: 4ee3d8e0-1300-4523-a33c-79b47fcc767b
                Copyright © Copyright © 2010 Alina Z. Hirsh et al.
                License:

                This is an open access article distributed under the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.

                History
                Date received : 19 April 2010
                Date revision received : 15 August 2010
                Date accepted : 16 August 2010
                Categories
                Subject: Case Report

                ScienceOpen disciplines: Oncology & Radiotherapy
                Data availability:
                ScienceOpen disciplines: Oncology & Radiotherapy

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