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 Table of Contents  
CASE REPORT
Year : 2017  |  Volume : 29  |  Issue : 1  |  Page : 47-49

Epithelioid hemangioendothelioma of upper lip: A rare entity


1 Department of Oral Medicine and Radiology, Pushpagiri College of Dental Sciences, Kerala, India
2 Department of General Surgery, Believers Church Medical College, Kerala, India
3 Department of Oral and Maxillofacial Pathology, Pushpagiri College of Dental Sciences, Kerala, India

Date of Submission27-Oct-2016
Date of Acceptance13-Jul-2017
Date of Web Publication04-Aug-2017

Correspondence Address:
Anuna L Mathew
Department of Oral Medicine and Radiology, Pushpagiri College of Dental Sciences, Kerala
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/jiaomr.JIAOMR_130_16

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   Abstract 

Epithelioid hemangioendothelioma (EHE) is an uncommon vascular neoplasm intermediating hemangioma and angiosarcoma characterized by the proliferation of epithelioid or histiocytoid endothelial cells with vacuolated cytoplasm and occasional eosinophilic spindle cells. Tumors of intermediate behavior may recur locally and metastasize but with less frequency compared to malignant ones. It rarely affects the oral cavity. Literature review shows only a few cases of this entity in the oral mucosa. Due to its rarity, we report a case of EHE on the upper lip.

Keywords: Epithelioid hemangioendothelioma, intraoral, tumor, upper lip, vascular neoplasm


How to cite this article:
Mathew AL, Daniel MP, Babu SS, Nair SS. Epithelioid hemangioendothelioma of upper lip: A rare entity. J Indian Acad Oral Med Radiol 2017;29:47-9

How to cite this URL:
Mathew AL, Daniel MP, Babu SS, Nair SS. Epithelioid hemangioendothelioma of upper lip: A rare entity. J Indian Acad Oral Med Radiol [serial online] 2017 [cited 2020 Jan 24];29:47-9. Available from: http://www.jiaomr.in/text.asp?2017/29/1/47/212079


   Introduction Top


The term “hemangioendothelioma” was introduced by Borrmann in 1899 who proposed it as an intermediate or low-grade malignant vascular neoplasm. Epithelioid hemangioendothelioma (EHE) was first described by Weiss and Enzinger in 1982 as an angiocentric neoplasm characterized by neoplastic proliferation of cells showing eosinophilic vacuolated cytoplasm and occasional fusiform cells.[1] The lesions of EHE are quite small, superficial, red to brown, angioma-like nodules, usually seen intramucosally or subcutaneously, and are often multifocal. It usually occurs in young adults, with a mean age of 30 years. Lesion often persist but may resolve spontaneously.[1] The tumor is primarily committed to the soft tissues of the extremities, however, cases have been reported in the lung, liver, bone, skin, and head and neck regions, including the oral cavity. The EHE is rare in the head and neck region and even more rare in the oral cavity. Here, we report a case of EHE in a 30-year-old male on the upper lip.


   Case Report Top


A 30-year-old male patient reported with the complaint of a slowly growing painless swelling on the left side of the upper lip of 1 year duration. There was no history of trauma. Extraoral examination revealed a diffuse swelling on the left side of the upper lip, with obliteration of the nasolabial fold and elevation of the philtrum. Intraoral examination revealed a well-defined, smooth-surfaced, bean-shaped sessile nodule with same color as the adjacent mucosa, measuring 2.5 × 1.5 cm [Figure 1]. Lesion was firm and nontender on palpation. A provisional diagnosis of benign minor salivary gland neoplasm was made. After routine blood investigations, excisional biopsy was done [Figure 2]. Histopathological examination revealed a nonkeratinized stratified squamous epithelium with underlying moderately collagenous connective tissue showing proliferation of vascular channels, epithelioid cells and extravasated red blood cells. Proliferating epithelioid cells appeared plump, with vesicular nucleus, inconspicuous nucleolus, and moderate eosinophilic cytoplasm. No pleomorphism and increased mitosis were seen [Figure 3] and [Figure 4]. Diffuse collection of chronic inflammatory cells was also noted. Based on the histopathological features, a final diagnosis of EHE involving the left side of the upper lip was made. Regular follow-up was done for 1 year and no recurrence was reported.
Figure 1: Clinical photograph showing the swelling on the inner aspect of upper lip

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Figure 2: Photograph showing excision of the lesion

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Figure 3: Photomicrograph showing moderately collagenous connective tissue and moderate vascularity, H and E stain, ×10

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Figure 4: Photomicrograph showing proliferating vascular channels and epithelioid/endothelial cells, H and E stain, ×40

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   Discussion Top


EHE is a rare benign neoplasm. Its etiology and pathogenesis are not yet clarified. Some authors believe that EHE represents an unusual manifestation of hypersensitivity, whereas others suggest that it is a reactive vascular disorder, which may be caused by deregulated expression of vascular factors or traumatic phenomena or a malformation.[2] In the past, various synonyms have been given to this entity such as atypical or pseudopyogenic granuloma, angioblastic lymphoid hyperplasia with eosinophilia, and eosinophilic lymphofolliculosis of the skin. The term “angio-lymphoid hyperplasia with eosinophilia’” was proposed by Wells and Whimster in 1969. It is seen mainly in the soft tissues of extremities but has also been reported within the bone and other parts of the body.[1] EHE occurs rarely in the head and neck region and was first reported in the oral cavity by Ellis and Kratochvil in 1986.[3]

Considering the clinical presentation of the lesion as a slowly growing painless swelling on the lip, a provisional diagnosis of pleomorphic adenoma was made. Differential diagnosis includes lipoma, neurofibroma, fibrosed mucocele, calcified hematoma, oral myofibroma, and leiomyoma. Oral lipomas frequently have a yellowish tinge and are soft in consistency. Neurofibroma is also a possibility, and the most common location is tongue. Leiomyoma rarely affects lips.[4],[5] Fibrosed mucocele and calcified hematoma were excluded as no history of trauma was reported. Rarely, oral myofibroma occurs in adults.

Microscopically, EHE consists of vascular and inflammatory cellular components. Vascular channels are lined by epithelioid or histiocytoid endothelial cells which extend considerably into the lumen, imparting a “tombstone” effect to the vessel walls. Cells have rounded nuclei and abundant eosinophilic cytoplasm. A mixed chronic inflammatory cell infiltrate, including eosinophils and occasional germinal centers, is seen surrounding the lesional vessels of epithelioid hemangioma.[4],[5] Lymphoid follicle may be present. Oral EHE must be histologically differentiated from metastatic carcinoma, melanoma, and epithelioid angiosarcoma.[1] Lack of degree of nuclear atypia and mitotic figures ruled out epithelial malignancies such as metastatic carcinomas and melanomas. Epithelioid angiosarcoma usually shows solid nests of endothelial cells with high degree of mitotic activity along with necrosis.[2],[6],[7],[8],[9]

Oral EHE is usually treated by surgical excision.[7],[8],[10],[11],[12] Recurrences are quite common and have been successfully treated with re-excision.[7],[8] Epithelioid hemangioma may become malignant or recur or may metastasize to regional lymph nodes.[7],[8] If diagnosed as EHE, complete surgical excision with wide margins and long-term follow-up is necessary.[5],[7],[8],[9] It is important to know the characteristics of this rare condition in an attempt to consider it in the differential diagnosis of such lesions.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.

 
   References Top

1.
Weiss SW, Enzinger FM. Epithelioid hemangioendothelioma. A vascular tumor often mistaken for carcinoma. Cancer 1982;50:970-81.  Back to cited text no. 1
    
2.
Manjunatha BS, Kumar GS, Vandana R. Intraoral epithelioid hemangioendothelioma: An intermediate vascular tumor – A case report. Dent Res J 2009;6:99-102.  Back to cited text no. 2
    
3.
Enzinger FM, Weiss SW. In: Soft Tissue Tumors. 3rd ed. Philadelphia: Mosby. 1995; pp. 891-900.  Back to cited text no. 3
    
4.
Gordon-Nunez MA, Silva LM, Lopes MF, de Oliveira-Neto SF, Maia AP, Galvao HC. Intra oral epithelioid hemangioendothelioma-A case report and review of literature. Med Oral Patol Oral Cir Bucal 2010;15:340-6.  Back to cited text no. 4
    
5.
Ellis GL, Kratochvil FJ. Epithelioid hemangioendothelioma of the head and neck: A clinicopathologic report of twelve cases. Oral Surg Oral Med Oral Pathol 1986;61:61-8.  Back to cited text no. 5
    
6.
Orsini G, Fioroni M, Rubini C, Piattelli A. Epithelioid hemangioendothelioma of the oral cavity: Report of case. J Oral Maxillofac Surg 2001;59:334-7.  Back to cited text no. 6
    
7.
Mohtashan N, Kharrazi AA, Jamshidi S, Jafarzadeh H. Epithelioid hemangioendothelioma of the oral cavity: A case report. J Oral Sci 2008;50:2.  Back to cited text no. 7
    
8.
Molina Palma MI, Cervantes Gongora JA, Garcia de la Torre E, Conde Perez de la Blanca I, Ramirez Tortosa CL. Primary intra oral epithelioid hemangioma: Case report and review of literature. Acta Otorhinolaryngol Esp 2002;53:21.  Back to cited text no. 8
    
9.
Orsini G, Fioroni M, Rubini C, Piattelli A. Epithelioid hemangioendothelioma of the oral cavity; Report of case. J Oral Maxillofac Surg 2001;59:334-7.  Back to cited text no. 9
    
10.
Marrogi AJ, Boyd D, EI-Mofty S, Waldron C. Epithelioid hemangioendothelioma of the oral cavity: Report of two cases and review of literature. J Oral Maxillofac Surg 1991;49:633-8.  Back to cited text no. 10
    
11.
Chi AC, Weathers DR, Folpe AL, Dunlap DT, Rasenberger K, Neville BW. Epithelioid hemangioendothelioma of the oral cavity: A report of two cases and review of literature. Oral Surg Oral Med Oral Pathol Oral Radiol Endod 2005;100:717-24.  Back to cited text no. 11
    
12.
Sun ZJ, Zhang L, Zhang WF, Chen XM, Lai FM, Zhao YF. Epithelioid hemangioendothelioma of the oral cavity. Oral Dis 2007;13:244-50.  Back to cited text no. 12
    


    Figures

  [Figure 1], [Figure 2], [Figure 3], [Figure 4]



 

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