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Table of Contents
Year : 2020  |  Volume : 38  |  Issue : 2  |  Page : 125-126

A case of composite hemangioendothelioma associated with lymphangioma-like feature

1 Department of Dermatology, Taichung Veterans General Hospital, Taichung, Taiwan
2 Department of Pathology and Laboratory Medicine, Taichung Veterans General Hospital; Medical Laboratory Science and Biotechnology, Central Taiwan University of Science and Technology, Taichung, Taiwan

Date of Submission02-Jul-2019
Date of Decision28-Nov-2019
Date of Acceptance06-Dec-2019
Date of Web Publication29-May-2020

Correspondence Address:
Dr. Chi-Shun Yang
Department of Pathology and Laboratory Medicine, Taichung Veterans General Hospital, No. 1650, Section 4, Taiwan Boulevard, Taichung 40705
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/ds.ds_47_19

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How to cite this article:
Liao CY, Yang CS, Kuei JC. A case of composite hemangioendothelioma associated with lymphangioma-like feature. Dermatol Sin 2020;38:125-6

How to cite this URL:
Liao CY, Yang CS, Kuei JC. A case of composite hemangioendothelioma associated with lymphangioma-like feature. Dermatol Sin [serial online] 2020 [cited 2023 Mar 21];38:125-6. Available from: https://www.dermsinica.org/text.asp?2020/38/2/125/285356

Dear Editor,

Composite hemangioendothelioma (CHE), a rare vascular tumor with intermediate malignant potential, is characterized by an admixture of benign, low-grade malignant, and malignant vascular components. Fewer than 40 cases of CHE have been reported in the English-language literature thus far; only three cases of CHE have included a lymphangioma-like component.[1],[2],[3],[4],[5],[6],[7],[8] Here, we present a unique case of CHE in a 28-year-old female with lymphangioma-like feature.

This 28-year-old female intermittently developed several discrete asymptomatic skin-colored papules and tiny clear hemorrhagic vesicles over the chest wall approximately 20 years since childhood. Among these papules and vesicles, one newly developed well-demarcated erythematous to violaceous papule, sized 1 cm × 1.2 cm [Figure 1]a, with overlying smooth surface and peripheral erythematous change [Figure 1]b, was noted 3 months ago. Excisional biopsy of the papule was performed at our hospital. The pathological findings revealed several vascular tumor components, including epithelioid hemangioendothelioma, retiform hemangioendothelioma, low-grade angiosarcoma-like area, and lymphangioma [Figure 1]c. The epithelioid hemangioendothelioma component was characterized by cords and nests of epithelioid cells with eosinophilic cytoplasm embedded in hyalinized stroma. Some tumor cells contained intracytoplasmic lumina with erythrocyte retention [Figure 2]a. The retiform hemangioendothelioma component exhibited elongated, arborizing, and dilated blood vessels lined with hobnail endothelial cells with intraluminal projection [Figure 2]b. The low-grade angiosarcoma-like component was composed of slit-like and anastomosing vascular channels lined with mildly atypical endothelial cells [Figure 2]c. The lymphangioma component was characterized by dilated and thin-walled lymphatic vessels lined with flattened endothelial cells throughout the dermis [Figure 2]d. Immunohistochemical staining of all tumor cells, except for the lymphangiomatous component, was positive for CD31 and ETS related gene (ERG), but negative for D2-40. All tumor cells were nonreactive for CD34 and human herpesvirus (HHV)-8 stains. The Ki-67 index staining was <1% [Figure 1]d. Therefore, a diagnosis of CHE was rendered. Subsequent chest magnetic resonance imaging with contrast enhancement over the lower chest and upper abdominal wall demonstrated dilated and serpiginous cystic spaces, 15 cm × 10 cm in size, but without regional lymph node or solid-organ metastasis. Wide local excision with a safe margin of approximately 2 cm was performed subsequently. The residual tumor exhibited pathologic findings similar to the previous biopsy; the relative proportion of the four distinct components after total excision was approximately 90% lymphangioma, 5% retiform hemangioendothelioma, 5% low-grade angiosarcoma-like area, and <1% epithelioid hemangioendothelioma. Neither local recurrence nor distant metastasis was observed 8 months after surgery.
Figure 1: (a) Well-demarcated erythematous to violaceous papule (size: 1 cm × 1.2 cm), with several adjacent discrete translucent to hemorrhagic vesicles on the chest wall. (b) Close-up view. (c) Pathologic findings revealed an ill-defined tumor involving dermis and subcutis with various vascular components (H and E, ×10).). : Low-grade angiosarcoma-like area, : Lymphangioma area, : Epithelioid hemangioendothelioma area, and : Retiform hemangioendothelioma area. (d) Ki-67 index staining (<1%)

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Figure 2: (a) Cords and nests of epithelioid cells with eosinophilic cytoplasm accompanying occasional intracytoplasmic lumina and erythrocyte retention (H and E, ×200). (b) Arborizing, elongated, and dilated blood vessels lined with hobnail endothelial cells resembling rete testis (H and E, ×100). (c) Slit-like and anastomosing blood vessels lined with mildly atypical endothelial cells (H and E, ×200). (d) Dilated and thin-walled lymphatic vessels lined with flattened endothelial cells (H and E, ×40)

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CHE was first described by Nayler et al.[1] It features frequent local recurrence, but rarely demonstrates lymph node or distant metastasis. Moreover, it predominantly occurs in younger to middle-aged patients with a slight female preponderance.[1],[2],[3],[4],[5],[6],[7],[8] Although its etiology remains unknown, its association with underlying vascular abnormalities, such as arteriovenous malformation, lymphangioma circumscriptum, Maffucci syndrome, and Kasabach–Merritt syndrome, has been reported.[1],[3],[5],[7],[8] Clinically, most CHEs present ill-defined solitary or multiple asymptomatic erythematous to violaceous papulonodules with sizes ranging from 0.7 to 30 cm on the distal extremities. They can develop at other sites, including the head, neck, scalp, oral cavity, hypopharynx, mandibular vestibule, back, mediastinum, Achilles tendon, kidney, and spleen.[1],[4],[5],[6],[7],[8] Histopathologically, CHE is a poorly circumscribed and infiltrative dermal and subcutaneous neoplasm containing variable numbers of vascular tumors. Its benign components may include cavernous hemangioma, capillary hemangioma, spindle cell hemangioma, lymphangioma, and angiomatosis. The most common vascular components harboring intermediate malignant potential in CHEs are retiform and epithelioid hemangioendotheliomas. With respect to the malignant counterparts, a moderately differentiated to well-differentiated angiosarcoma-like area is present in nearly half of CHE cases and comprises a minority of the main tumor region.[3],[7] Immunohistochemically, the tumor cells present variable positivity for CD31, CD34, and von Willebrand factor, but rarely for D2-40. The tumor cells are nonreactive to cytokeratin, S-100, epithelial membrane antigen, desmin, actin, and HHV-8.[3],[6],[7] The high rate of local CHE recurrence is attributable to its multicentric origin and incomplete excision due to a deep infiltrative margin. The best management strategy for CHE remains debatable. Wide local excision beyond the clinical margin is the currently accepted primary treatment for CHE. Although no patient has died due to this rare tumor thus far, metastasis to regional lymph nodes, soft tissues, bones, the liver, and the lungs can occur. Careful preoperative evaluation and postoperative imaging surveys with computed topography or magnetic resonance imaging are warranted.[1],[2],[3],[4],[5],[6],[7],[8]

Thus, we described the first case in Taiwan of a patient with CHE with lymphangioma-like component. Regarding CHE prognosis, the presence of satellite lesions around the primary CHE may indicate aggressive behavior,[6],[8] whereas the presence of angiosarcoma-like area in the CHE indicates better prognosis than that of conventional angiosarcoma,[7] which is irrelevant to its recurrence or metastasis.[5] Given that CHE has a high local recurrence rate (approximately 50%; varying with time) and a low metastasis risk,[1],[2],[3],[4],[5],[6],[7],[8] regular follow-up after surgery is imperative.

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent form. In the form, the patient has given her consent for her figures and other clinical information to be reported in the journal. The patient understands that her name and initial will not be published and due efforts will be made to conceal her identity, but anonymity cannot be guaranteed.

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Conflicts of interest

There are no conflicts of interest.

  References Top

Nayler SJ, Rubin BP, Calonje E, Chan JK, Fletcher CD. Composite hemangioendothelioma: A complex, low-grade vascular lesion mimicking angiosarcoma. Am J Surg Pathol 2000;24:352-61.  Back to cited text no. 1
Biagioli M, Sbano P, Miracco C, Fimiani M. Composite cutaneous haemangioendothelioma: Case report and review of the literature. Clin Exp Dermatol 2005;30:385-7.  Back to cited text no. 2
Fukunaga M, Suzuki K, Saegusa N, Folpe AL. Composite hemangioendothelioma: Report of 5 cases including one with associated Maffucci syndrome. Am J Surg Pathol 2007;31:1567-72.  Back to cited text no. 3
Aydingöz IE, Demirkesen C, Serdar ZA, Mansur AT, Yaşar S, Aslan C. Composite haemangioendothelioma with lymph-node metastasis: An unusual presentation at an uncommon site. Clin Exp Dermatol 2009;34:e802-6.  Back to cited text no. 4
McNab PM, Quigley BC, Glass LF, Jukic DM. Composite hemangioendothelioma and its classification as a low-grade malignancy. Am J Dermatopathol 2013;35:517-22.  Back to cited text no. 5
Mahmoudizad R, Samrao A, Bentow JJ, Peng SK, Bhatia N. Composite hemangioendothelioma: An unusual presentation of a rare vascular tumor. Am J Clin Pathol 2014;141:732-6.  Back to cited text no. 6
Shang Leen SL, Fisher C, Thway K. Composite hemangioendothelioma: Clinical and histologic features of an enigmatic entity. Adv Anat Pathol 2015;22:254-9.  Back to cited text no. 7
Bhat A, Chowdappa V. Composite hemangioendothelioma: Report of a rare case. J Clin Diagn Res 2016;10:ED01-3.  Back to cited text no. 8


  [Figure 1], [Figure 2]


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