epithelioid hemangioendothelioma ( ehe ) is an uncommon vascular tumour of soft tissue arising from the endothelium and it was first reported by weiss and enzinger in 1982 . the histological and clinical features of tumour are intermediate between benign vascular tumours such as haemangiomas and frank malignant tumours like angiosarcomas . it is most commonly found in soft - tissues , but can arise in any organ . an exceptionally rare case of primary cutaneous ehe on  the left iliac region without systemic involvement in a child . a 9-year - old boy presented with an asymptomatic gradually enlarging nodular lesion since 5 months . on palpation , the lesion was non - tender , freely mobile and firm in consistency . it measured 3 cm  2 cm and was situated in left iliac region [ figure 1 ] . based on the clinical examination , dermatofibroma , hemangioendothelioma and arteriovenous malformation were considered as differentials . swelling of 3 cm  2 cm with violaceous hue , located on left iliac region microscopic examination revealed well - circumscribed tumour localised in the dermis , composed of mass of plump epithelioid shaped endothelial cells arranged in  nest - like  pattern with minimal vascular differentiation . the epithelioid cells showed intracytoplasmic vacuole ( primitive lumina ) and erythrocytes were seen in some of the lumina . on immunohistochemistry , cd 31 was positive , other endothelial markers were not done due to lack of facilities and our patient was not affordable . other investigations such as chest x - ray , ultrasound abdomen and a whole - body bone scan were performed to exclude multicentric location and distant metastasis , but were found to be normal . patient is on regular follow - up for the last 10 months with no signs of local recurrence or metastasis . mass of plump endothelial cells distributed in myxohyaline stroma with minimal vascular differentiation ( h and e , 10 ) plump endothelial cells with intracytoplasmic vacuole ( primitive lumen ) arranged in nests , few lumen show red blood cells ( h and e , 40 ) ehe is an uncommon , well - differentiated endothelial vascular neoplasm of low grade malignancy . the epithelioid haemangiomas represent the benign end of the spectrum , whereas epithelioid angiosarcomas represent the malignant end . it presents frequently during the second and third decades of life , but rarely occurs during childhood . both sexes are equally affected , though liver and lung lesions are more common in females . the neoplasm usually presents as a solitary , rarely multiple , erythematous papule , nodule , plaque or nonhealing ulcers . it not only involves soft - tissue , but also involves other sites such as liver , lung , bone , skin , lymph nodes , brain and heart . ehe of the skin is extremely rare , it can be isolated or associated with systemic involvement . the tumour arises from a medium - sized vessel mainly in superficial or deep soft - tissue , about 10% , arise within dermis . the chromosomal translocation involving chromosomes 1 and 3 ( t[1;3][p36.3;q25 ] ) is known to occur in ehe . there are no predisposing factors , but possible associations with trauma , therapeutic radiation and hormonal factors have been postulated . ehe shows multifocal involvement in 10% of cases , but isolated skin involvement is rarely described . clinically it has to be differentiated from a haemangioma , pyogenic granuloma , arteriovenous malformation and hamartomas . the tumour is composed histologically of epithelioid , round , to slightly spindle shaped endothelial cells with abundant eosinophilic cytoplasm and round vesicular nucleus . tumour cells are arranged in cords , short strands , small nests with minimal vascular differentiation and are embedded in fibromyxoid stroma . in one - fourth of cases , there can be significant atypia , mitotic activity , focal splindling of cells and necrosis , which correlates with aggressiveness of tumour . ultrastructural study has revealed the endothelial nature of the tumour cells surrounded by basal lamina , dotted with surface pinocytotic vesicles , intracytoplasmic lumina and occasionally containing weibel - palade bodies . immunohistochemical staining of tumour cell is positive for vascular endothelial markers ( cd 31 , cd 34 and factor viii , vimentin and uea-1 and mostly negative for cytokeratin ) . cases are always positive for at least one vascular endothelial marker , but not usually all . cd31 is positive in kaposiform hemangioendothelioma , composite hemangioendothelioma , epithelioid sarcoma - like hemangioendothelioma , retiform hemangioendotheliomas , epithelioid haemangioma , tufted angioma , reactive angioendotheliomatosis and angiosarcoma . about 19 cases of ehe with skin involvement have been reported in the literature [ table 1 ] and the majority of them show systemic localisation and most of them are adult patients . ( 2000 ) , reported isolated cutaneous ehe in an 8-year - old female child , who presented with erythematous plaque on the left frontal area of the scalp . there was no evidence of recurrence or metastasis after a follow - up of 20 months . to the best of our knowledge , our case is the second case of ehe involving skin without systemic involvement in paediatric age group . uses , advantages and disadvantages of tissue adhesives the detection of skin lesions should lead to a complete assessment of the extension of the disease to detect any internal localisation . thus wide excision is recommended with evaluation of regional lymph nodes and if they occur here , further surgery may be curative . adjuvant treatment with interferon therapy and radiotherapy or chemotherapy may be used to restrain growth of incompletely removed tumours or in patients with multi system involvement . ehe displaying features of cellular atypia , mitotic activity ( > 1 mitotic figure per 10 high - power fields ) , necrosis and extensive spindling favours an aggressive course and correlates with poor prognosis . . showed local recurrence in 3 cases and systemic metastases in 21% of patients , of which 17% died of their tumours ; hence the authors suggested that ehe of the soft tissue should be better regarded as a fully malignant , rather than borderline , vascular neoplasm . patients with cutaneous ehe have a favourable outcome than their more centrally located counterparts and isolated ehe of the skin probably behaves in a more benign fashion . however , in virtue of its malignant potential and infiltrative growth pattern , a close follow - up is recommended . in our case , there was no evidence of distant metastasis or multifocal involvement , so after wide surgical excision and favourable histopathology report , we proceeded with close follow - up of patient clinically , once in 6 weeks for 6 months and every 3 months later on . as it was isolated ehe , literature says it probably behaves as benign lesion and so we are assessing him clinically for local recurrence , lymph node examination and any systemic complains . ehe is a unique vascular neoplasm of intermediate aggressiveness and unpredictable prognosis , which usually appears in adults . it generally affects soft - tissue and less frequently , the viscera such as lungs and liver . this is the second reported case of childhood onset ehe arising from skin without systemic involvement . we would like to point out that clinicians should be aware of this rare presentation in paediatric age group .
epithelioid hemangioendothelioma ( ehe ) is a rare tumour arising from the vascular endothelium of soft - tissue , bone and viscera . 
 skin involvement is rare . 
 the disease has an indolent course , with the potential for recurrence and often associated with multi - systemic localisations . 
 we present a rare case of cutaneous ehe without systemic involvement in 9-year - old boy . 
 the tumour was completely excised and histopathologic diagnosis was consistent with ehe . 
 complete systemic assessment showed no internal localisation . 
 wide excision is the mainstay of therapy and regular follow - up is suggested due to the potential for recurrence . 
 the majority of documented cases of ehe is in adults and has developed in association with an underlying systemic involvement . to the best of our knowledge , 
 this is the second reported case of isolated cutaneous ehe in paediatric age group .