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Year : 2020  |  Volume : 63  |  Issue : 1  |  Page : 122-124
Retiform hemangioendothelioma: An uncommon vascular neoplasm

Department of Pathology, IPGME and R, Kolkata, West Bengal, India

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Date of Web Publication31-Jan-2020


Retiform hemangioendothelioma is a rare vascular neoplasm of intermediate grade, the diagnosis of which can be challenging. We report a case of 35-year-old man with swelling in the postauricular region. He had undergone FNAC which had revealed blood only. Microscopic examination showed narrow, arborizing, vascular channels resembling normal rete testis. Evidence of mitoses or cytological atypia were lacking. Immunohistochemistry showed diffuse and strong staining for CD34 along with CD31 positivity. Immunostains for D240 and GLUT1 were negative. A diagnosis of retiform hemangioendothelioma was made. Histologically, it should be distinguished from Kaposiform hemangioendothelioma, Dabska tumor, epithelioid hemangioendothelioma, and angiosarcoma.

Keywords: CD 31, CD 34, retiform hemangioendothelioma

How to cite this article:
Mondal A, Das M, Chatterjee U, Datta C. Retiform hemangioendothelioma: An uncommon vascular neoplasm. Indian J Pathol Microbiol 2020;63:122-4

How to cite this URL:
Mondal A, Das M, Chatterjee U, Datta C. Retiform hemangioendothelioma: An uncommon vascular neoplasm. Indian J Pathol Microbiol [serial online] 2020 [cited 2020 Sep 21];63:122-4. Available from: http://www.ijpmonline.org/text.asp?2020/63/1/122/277370

   Introduction Top

Retiform hemangioendothelioma is a unusual vascular tumor of intermediate grade malignancy, first described in the year 1994 and only 35 cases are described so far.[1],[2],[3],[4] Here, we take the opportunity to report a case of retiform hemangioendothelioma in a 35-year-old man along with its histological differentials and discuss the panel of immunohistochemical markers useful for the diagnosis.

   Case Report Top

A 35-year-old male presented with two years of swelling in the postauricular region [Figure 1]a. The patient was asymptomatic and there was no other relevant medical history. On gross examination, the swelling was 3 cm. The patient had undergone FNAC from the swelling which revealed only blood. Later, it was surgically excised and was sent for histopathological examination. On gross examination, it was tan colored and measured 2.5 cm across. Microscopic examination, revealed narrow, arborizing, vascular channels resembling normal rete testis. Monomorphic endothelial cells with protuberant nuclei with hobnail appearance were lining the blood vessels. Adjoining stroma was sclerotic with focal lymphocytic infiltration. Evidence of mitotic figures or cytologic atypia were lacking [Figure 1]b[Figure 1]c, [Figure 1]d. Diffuse staining for CD 34 was positive along with strong positivity for CD 31 whereas immunostains for D240 and GLUT1 were negative [Figure 2].[5],[6] A diagnosis of retiform hemangioendothelioma was made. The patient is doing well, on a one-year follow up.
Figure 1: (a): Right sided postauricular swelling. (b): Photomicrograph showing lobules of narrow, arborizing, vascular channels. (H and E stain, ×40). (c): Curvilinear vessels resembling rete testis. (H and E stain, ×100). (d): High power view of vessels showing hobnail pattern of endothelial cells. Inset shows sprinkling of lymphocytes. (H and E stain, ×400)

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Figure 2: (a): Diffuse and strong staining of endothelial cells for CD 34. (b): Diffuse and strong staining for CD31. (c): Staining for GLUT1 is negative. (d): Staining for D240 is negative

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   Discussion Top

Retiform hemangioendothelioma is a rare vascular tumor. It affects mainly the adults in the second to fourth decade of life with the mean age being 36 years.[1],[2] Both males and females are equally affected. It presents clinically as a slow-growing asymptomatic nodule or plaque usually either on extremities or trunk.[1],[2] The head and neck region is a distinct, unusual site.[1],[4] Retiform hemangioendothelioma shows elongated narrow, arborizing, vascular channels which bears similarity to normal rete testis. If these vascular channels are small or collapsed, the identification of the retiform pattern may be difficult. Uniform, hyperchromatic, endothelial cells with protuberant nuclei which have characteristic “tombstone” or “hobnail” appearance are seen lining the blood vessels. Pleomorphism is not seen and mitotic figures are also rare. Lymphocytic infiltrate is present in around half of cases in the stroma.

Histologically, it must be differentiated from Kaposiform hemangioendothelioma, epithelioid hemangioendothelioma, Dabska tumor, benign tumors such as cellular hemangioma, spindle cell hemangioma, hobnail hemangioma, and malignant forms like angiosarcoma and Kaposi sarcoma.

Epithelioid hemangioendothelioma is a highly cellular tumor in which the sclerotic pattern is common. These strands of tumor cells proliferate in a myxohyaline stroma. The vascular nature may be betrayed by formation of large vacoules which are frequently septate. The tumor cells are positive for CD31 and ERG.

Dabska tumor consists of matchstick like, columnar or hobnail endothelial cell proliferation into lymphatic channel lumina.[7] The intraluminal endothelial tufting can have hyaline cores and intermixed intraluminal lymphocytes. The lymphatic phenotype is known by positivity for podoplanin/(D240) and VEGFR3. The endothelial marker CD31 is more strongly positive than CD34.

In Kaposiform hamangioendothelioma, the tumour nodules are surrounded by desmoplastic stroma. The nodules have areas resembling both capillary haemangioma as well as Kaposi sarcoma. The distinguishing feature is a tightly formed glomeruloid structure comprising tightly coiled capillary vessels.[8] The lymph vessel differentiation markers PROX1, LYVE1, and D240 are positive in Kaposi-like areas but the glomeruloid areas are negative for the these markers. These areas also show positivity for endothelial markers such as CD34 and CD31. Negativity for GLUT 1 helps in its differentiation from infantile hemangioma. Clinically, Kaposiform hemangioendothelioma can be associated Kasabach–Merritt phenomenon which is not seen in retiform hemangioendothelioma.

Cellular hemangioma often shows sheets of plump polygonal cells, devoid of lumina. Numerous mitotic figures may be seen but these lesions are clearly benign and they are self-remitting unlike retiform hemangioendothelioma. Infantile hemangioma shows positivity for GLUT1. In spindle cell hemangioma, bland spindle cells proliferating between the vascular lumina and presence of extravasated red blood cells is the feature. Hobnail hemangioma consists of an irregular, dilated, thin-walled, proliferation of vascular channels lined by prominent hobnail endothelial cells with occasional papillary projections. The extravasated red blood cells and inflammatory cells may be seen in the adjoining stroma.

In angiosarcoma, freely anatomizing vascular channels lined with atypical endothelial cells are found and collection of lymphocytes and clumps of hemosiderin are common. Significant cytologic atypia, mitotic figures, and collagen bundle infiltration by tumor cells and absence of hobnail endothelial cell are the main differentiating features with retiform hemangioendothelioma.[9],[10]

Kaposi sarcoma is a cellular tumor with fascicles and sheets of elongated spindle cells forming slit like spaces which contain erythrocytes. Lymphocytes, hemosiderin-laden macrophages, and other inflammatory cells are often found admixed in these tumors. HHV 8 positivity in Kaposi sarcoma helps to differentiate it from Kaposiform hemangioendothelioma.

An extended panel of immunomarkers is helpful in the accurate diagnosis retiform hemangioendothelioma. The CD34 marker is helpful in confirming its vascular nature and the tumor cells are also positive for CD31. While negativity for GLUT 1 rules out hemangioma, cellular hemangioma, and juvenile hemangioma, D240 negativity rules out Kaposiform hemangioendothelioma and Dabska tumor.

Local recurrence is common and is seen in approximately half of the cases.[4] Regional lymph node metastasis and local soft tissue spread has been reported in one patient each. However, distant metastasis has not been reported with this condition.

   Conclusion Top

Here, we take the opportunity to describe a case of retiform hemangioendothelioma along with its morphologic differentials and immunohistochemical pattern. Positivity for CD 31 and CD34 along with negative stain for D240 and GLUT 1 is useful to distinguish it from other vascular tumors which are its histological mimickers.

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.

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Conflicts of interest

There are no conflicts of interest.

   References Top

Calonje E, Fletcher CD, Wilson-Jones E, Rosai J. Retiform haemangioendothelioma. A distinctive form of low-grade angiosar- coma delineated in a series of 15 cases. Am J Surg Pathol 1994;18:115-25.  Back to cited text no. 1
Albertini AF, Brousse N, Bodemer C, Calonje E, Fraitag S. Retiform haemangioedothelioma develpoed on a site of an earlier cystic lymphangioma in a 6 year old girl. Am J Dermapathol 2011;33:e84-7.  Back to cited text no. 2
Fakunaga M, Endo Y, Masui F, Yoshikawa T, Ishikawa E, Ushigome S. Retiform haemangioendothelioma. Virchows Arch 1996;428:301-4.  Back to cited text no. 3
Tan D, Kraybill W, Cheney RT, Khoury T. Retiform haemangioendothelioma: A case report and review of the literature. J Cutan Pathol 2005;32:634-7.  Back to cited text no. 4
Emberger M, Laimer M, Steiner H, Zelger B. Retiform haemangioendothelioma: Presentation of a case expresing D2-40. J Cutan Pathol 2009;36:987-90.  Back to cited text no. 5
Parsons A, Sheehan DJ, Sangueza OP. Retiform haemangioendotheliomas usually do not express D2-40 and VEGFR-3. Am J Dermatopathol 2008;30:31-3.  Back to cited text no. 6
Fanburg-Smith JC, Michal M, Parteanen TA, Alitalo K, Miettinen M. Papillary intrlaymphatic angioendothelioma (PILA): A report of twelve cases of a distinctive vascular tumour with phenotypic features of lymphatic vessels. Am J Surg Pathol 1999;23:1004-10.  Back to cited text no. 7
Lyons LL, North PE, Mac Moune Lai F, Stoler MH, Folpe AL, Weiss SW. Kaposiform haemangioendothelima: A study of 33 cases emphasizing its pathologic, immunophenotypic and biologic uniqueness from juvenile hemangioma. Am J Surg Pathol 2004;28:559-68.  Back to cited text no. 8
Cooper PH. Angiosarcomas of skin. Semin Diagn Pathol 1987;4:2-17.  Back to cited text no. 9
Alessi E, Sala F, Berti E. Angiosarcomas in lymphadematous limbs. Am J Dematopathol 1986;8:371-8.  Back to cited text no. 10

Correspondence Address:
Mou Das
Department of Pathology, IPGME and R, Kolkata, West Bengal
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/IJPM.IJPM_237_19

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