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 Table of Contents  
CASE REPORT
Year : 2017  |  Volume : 7  |  Issue : 4  |  Page : 135-138

Composite Hemangioendothelioma of the Scalp: An unusual presentation of a rare vascular tumor of low malignant potential


Department of Pathology, Ahmadu Bello University Teaching Hospital, Zaria, Nigeria

Date of Web Publication29-Oct-2018

Correspondence Address:
Dr. Adoke Kasimu Umar
Department of Pathology, Ahmadu Bello University Teaching Hospital, Zaria
Nigeria
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/ais.ais_43_17

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  Abstract 


Composite hemangioendothelioma (CHE) is a recently described new entity classified under the hemangioendothelioma group of vascular tumors. It is a rare vascular neoplasm of low malignancy that exhibits a composite of hemangioendothelioma variants which was first described in 2000 by Nayler et al. It is a complex heterogenous vascular tumor with infiltrative margins. We report a case of CHE of the scalp with spindle cell hemangioma-like areas comprising 10% of tumor, papillary intralymphatic angioendothelioma (Dabska) comprising 70% of tumor, and retiform hemangioendothelioma-like areas comprising 20% in a 9-year-old boy who presented with a 2 × 2 × 2 cm scalp mass on the parietal area with no regional lymph node metastasis. Immunohistochemical stain was positive for CD34 and vimentin in the all areas, and EMA was negative in all areas of tumor. Mitosis was less than 2 per 10 high-power fields.

Keywords: Composite hemangioendothelioma, Dabska tumor, retiform hemangioendothelioma, spindle cell hemangioma


How to cite this article:
Umar AK, Suleiman DE. Composite Hemangioendothelioma of the Scalp: An unusual presentation of a rare vascular tumor of low malignant potential. Arch Int Surg 2017;7:135-8

How to cite this URL:
Umar AK, Suleiman DE. Composite Hemangioendothelioma of the Scalp: An unusual presentation of a rare vascular tumor of low malignant potential. Arch Int Surg [serial online] 2017 [cited 2018 Nov 18];7:135-8. Available from: http://www.archintsurg.org/text.asp?2017/7/4/135/244415




  Introduction Top


Composite hemangioendothelioma (CHE) is a new entity classified under the hemangioendothelioma group of tumors. It is an extremely rare vascular neoplasm of low-to-moderate malignancy.[1] It usually presents on the skin and soft tissue of upper and lower extremities, especially the lower leg and foot. Other sites are the head and neck region chiefly the tongue, mandibular vestibule, cheek mucosa, scalp, nose, and hypopharynx.[2] Histopathologic diagnosis requires the presence of at least two hemangioendothelioma variants. Epithelioid and retiform are the most commonly observed variants.[3]


  Case Presentation Top


A 9-year-old boy presented to the clinic with a solitary nodular mass on the scalp around the right parietofrontal region for 6 months. The mass was painless and freely mobile. It was diagnosed as a scalp lipoma by the surgeon who performed an excision biopsy and sent the sample for histopathological confirmation. The gross examination revealed a dark brownish tissue measuring 2 × 2 × 2 cm with no skin covering. Cut sections showed brownish areas.

Histopathological examination showed a vasoformative tumor composed of three components. The first component was composed of spindle cell hemangioma areas lined by plump endothelial cells, Comprising 10% of tumour [Figure 1]. The second area was composed of Dabska area consisting of papillary endothelial structures lined by hobnail cells with glumeruloid-like structures and Comprising 70% of the tumour [Figure 2]. The third area was composed of retiform-like areas characterized by arborizing elongated blood vessels lined by hobnail cells embedded in a hyalinized stroma and comprising Approximately 20% of the tumour [Figure 3]. Focal areas of inflammatory cells were seen; mitosis was less than 2 per 10 high-power fields. Immunohistochemical stains were Positive in all areas with CD 34 stain [Figure 4] and [Figure 5] and were strongly positive for Vimentin [Figure 6] but negative for EMA in all areas [Figure 7] and [Figure 8].
Figure 1: Spindle cell hemangioma areas composed of dilated vessels in a hyalinized stroma. H&E×100

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Figure 2: Papillary like structures in lined by hobnail cells in Dabska tumour a component of CHE

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Figure 3: Retiform areas in CHE compose of arborizing blood vessels in a hyalinized stroma. H&E×40

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Figure 4: CD 34 in Dabska area. ×100

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Figure 5: CD 34 retiform areas. ×40

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Figure 6: Retiform areas vimentin positive. ×100

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Figure 7: Retiform areas EMA negative stainin

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Figure 8: Dabska area EMA negative staining. ×40

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  Discussion Top


CHE is the most recently described entity of the hemangioendothelioma spectrum. It is a locally aggressive, rarely metastasizing neoplasm with vascular differentiation, containing an admixture of histologically benign, intermediate, and malignant components.[3] It commonly occurs in young-to-middle aged adults, but can present at any age from birth to the elderly. CHE contains areas that resemble at least two of the following tumors – spindle cell hemangioma, epithelioid hemangioendothelioma, retiform hemangioendothelioma, Kaposiform hemangioendothelioma, papillary intralymphatic angioendothelioma, or angiosarcoma-like areas.[3],[4] The unique presentation in this case of retiform and Dabska areas is another reason to suggest that these lesions are related, especially for some authors who regard Dabska tumor as a heterogenous group of lesions.[5] It also shows variability in presentation of CHE. Most components reported in the literature were composed of epithelioid and retiform variants;[3] spindle cell hemangioma areas, Dabska areas, and retiform areas, as seen in our index patient, have been rarely reported in the literature.

Spindle cell hemangioma (hemangioendothelioma) is a distinct vascular lesion which was initially considered to be a low-grade angiosarcoma when first described in 1986.[6] Spindle cell hemangioma may be associated with anomalies such as Mafucci's syndrome, Klippel–Trenaunay syndrome, lymphedema, and early-onset varicose vein, which were not present in our patient. The lesion is usually located in the dermis or subcutis of the distal extremities, particularly the hands. Microscopically, it consists of three main areas – a vascular component displaying gaping vessels containing organizing thrombi, solid areas of spindle cells, and plump endothelial cells. This feature was clearly seen in the index patient and could be mistaken for cavernous hemangioma or Kaposi's sarcoma. It comprises approximately 10% of this lesion. On the other hand, malignant endovascular papillary angioendothelioma (Dabska tumor), a rare but distinctive vascular tumor of childhood, was first described in 1969 by Maria Dabska.[7] According to some authors, endovascular papillary angioendothelioma may not be a distinct entity and may well include a heterogeneous group of lesions.[5] It mostly occurs in infants and children. Microscopically, it is composed of prominent endothelial intraluminal papillary tufts lined by hobnail cells with hyaline cores surrounded by lymphocytes. Vascular endothelial growth factor receptor-3 (VEGFR-3), a recently introduced marker for lymphatic endothelia, is strongly expressed in Dabska tumor.[8] In the index patient, Dabska tumor constituted the majority of the lesion (70%). Retiform hemangioendothelioma was first described by Calonje et al. in 1994.[9] It is usually located in the dermis or subcutis and characterized by long arborizing vessels with features reminiscent of rete-testis and vessels lined by hobnail endothelial cells with prominent stromal lymphocytic infiltrate and occasional intraluminal papillae with hyaline collagenous cores and lymphocytes within vascular lumen. This lesion constituted approximately 20% of the lesion. Immunohistochemical stains show all components positive for CD34 and vimentin but negative for EMA stain. CHE has been shown to recur locally and metastasis is rare; few cases of metastasis have been reported to regional lymph node.[2],[10] No history of recurrence or metastasis was seen in the index patient after 18 months of follow-up.

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.


  Conclusion Top


We present a rare case of CHE with an unusual composite of hemangioendothelioma variants. We propose that hobnail hemangioendotheliomas i.e., papillary intralymphatic angioendothelioma (Dabska) and retiform hemangioendothelioma are probably related tumors that can coexist in a composite lesion.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.



 
  References Top

1.
Amoolya B, Vijaya C. Composite hemangioendothelioma: Report of a case. J Clin Diagn Res 2016;10:ED01-3.  Back to cited text no. 1
    
2.
Zhang J, Wu B, Zhou GQ, Zhang RS, Wei X, Yu B, et al. Composite hemangioendothelioma arising from the kidney: Case report with review of the literature. Int J Clin Exp Pathol 2013;6:1935-41.  Back to cited text no. 2
    
3.
Rod M, Aman S, Jason JB, Shi-Kaung P, Neil B. Composite hemangioendothelioma: An unusual presentation of a rare vascular tumour. Am J Clin Pathol 2014;141:732-6.  Back to cited text no. 3
    
4.
Chu YC, Choi SJ, Park IS, Kim L, Han JY, Kim JM. Composite hemangioendothelioma- a case report. Korean J Pathol 2006;40:142-7.  Back to cited text no. 4
    
5.
Sanz-Trelles A, Rodrigo-Fernandez I, Ayala-Carbonero A, Contreras-Rubio F. Retiform hemangioendothelioma. A new case in a child with diffuse endovascular papillary endothelial proliferation. J Cutan Pathol 1997;4:440-4.  Back to cited text no. 5
    
6.
Weiss SW, Ezinger FM. Spindle cell hemangioendothelioma. A low grade angiosarcoma resembling hemangioma and kaposi's sarcoma. Am J Surg Pathol 1986;10:521-30.  Back to cited text no. 6
    
7.
Dabska M. Mlignant endovascular papillary angioendothelioma of the skin in childhood. Clinicopathologic study of 6 cases. Cancer 1969;24:503-10.  Back to cited text no. 7
    
8.
Fanburg-Smith JC, Michal M, Partanen TA, Alitalo K, Miettinen M. Papillary intralymphatic angioendothelioma (PILA): A report of twelve cases of a distinctive vascular tumor with phenotypic features of lymphatic vessels. Am J Surg Pathol 1999;23:1004-10.  Back to cited text no. 8
    
9.
Calonje E, Fletcher CD, Wilson-Jones E, Rosai J. Retiform hemangioendothelioma. A distinctive form of low-grade angiosarcoma delineated in a series of 15 cases. Am J Surg Pathol 1994;18:115-25.  Back to cited text no. 9
    
10.
Requena L, Luis Diaz J, Manzarbeitia F, Carrillo R, Fernández-Herrera J, Kutzner H. Cutaneous composite hemangioendothelioma with satellitosis and lymph node metastases. J Cutan Pathol 2008; 35:225-30.  Back to cited text no. 10
    


    Figures

  [Figure 1], [Figure 2], [Figure 3], [Figure 4], [Figure 5], [Figure 6], [Figure 7], [Figure 8]



 

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Introduction
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