Volume 5, Issue 10, October – 2020 International Journal of Innovative Science and Research Technology

ISSN No:-2456-2165

The Diagnostic Role of Ultrasound and

MRI with IHC in a Rare Case of Epithelioid
Hemangioendothelioma of Soft
Tissue and Management
Dr. Divya Y G¹, Dr. Vivek Chail²
¹Post- Graduate, Department of Radio- Diagnosis, Dr. B. R. Ambedkar Medical College, Bangalore – 560045, India.
²Assistant Professor, Department of Radio-Diagnosis, Dr. B. R. Ambedkar Medical College, Bangalore – 560045, India.

Abstract:- Epithelioid Hemangioendothelioma (EH) is a Keywords:- CT Computed Tomography, Epithelioid

rare malignant vascular tumour that is considered to be Hemangioendothelioma, Immunohistochemistry, Magnetic
intermediate grade between benign Hemangioma and Resonance Imaging, Out Patient Department, T1 Weighted
malignant Angiosarcoma originates from vascular Images, T2 Weighted Images, Ultrasound.
endothelial or Pre endothelial cells. EH can occur
anywhere in the body most commonly affects the Liver, I. INTRODUCTION
Lungs, Bones, and although involves the Pleura,
Mediastinum, Spleen, Skin, Breast, Head and neck area, The term "EH" was proposed by Weiss and Enzinger,
Brain and Meninges and Lymph nodes. It often involves to explain a category ofsoft tissue vascular tumours
either superficial or deep soft tissue, visceral organs and composed of epithelioid appearance endothelial cells with
less commonly, medium-size or large veins. The aetiology intermediate clinical course between benign hemangioma
is unknown and is usually diagnosed at young adult, and malignant angiosarcoma[1].
being rare in children. EH is locally aggressive,
heterogenous and represents less than 1% of all the EH, which is a rare vascular tumour with an epithelioid
vascular tumours and capable of metastasis. Prevalence and histiocytoid appearance, originating from vascular
is 1 in 1 million. endothelial or pre-endothelial cells[2].

A 45Years old male presented with soft to firm It has a prevalence of 1 in a million . It is often
subcutaneous swelling since 7years later it progressed misdiagnosed and not suitably treated leading to a poor
rapidly in size from 1year in the Right upper anterior prognosis in many cases[6]. It usually affects middle-aged
chest. On examination: A non-tender, multilobulated patients, although cases in children and elderly people.
exophytic lesion in the subcutaneous plane showing few
erythematous nodules of varying size and were firm in Moreover, many patients are asymptomatic at the time
consistency. High frequency Ultrasound.(US) of Right of diagnosis[4].
Supraclavicular Mass, and MRI Thorax modalities
suggest features of Supraclavicular soft tissue neoplasm The aetiology of EH remains a dilemma. At the
showing vascular components and concern for molecular level, various angiogenic stimulators may act as
Hemangioendothelioma. promoters of endothelial cell proliferation. Recently it had
been reported that monocyte chemo-attractant protein-1 is
Management includes wide excision of the soft required for EH proliferation and might promote the event of
tissue lesion in the supraclavicular region and followed those lesions by stimulating the angiogenic behaviour of
by excisional biopsy and histopathological confirmation. endothelial cells[3].
US and most sensitiveMagnetic Resonance
Imaging(MRI) modality features along with It occurs in any a region of the body are often affected,
pathological techniques of Histopathology and but the foremost common sites are liver alone 21%, liver
Immunohistochemistry(IHC) techniques confirms the plus lungs 18%, lungs alone 12%, and bone alone 14%[5]
vascular nature of tumour. Followed by the wide excision but the prognosis on visceral organs EH is worse.
the patient has undergone adjuvant radiation therapy to
decrease the risk of local recurrence and distant The definitive diagnosis of EH requires
metastasis. Histopathological correlation. The pattern of solid growth
and thus the epithelioid appearance of the endothelium
frequently leads to the mistaken diagnosis of metastatic
carcinoma. The tumour are often distinguished from a
carcinoma by the shortage of pleomorphism and mitotic

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Volume 5, Issue 10, October – 2020 International Journal of Innovative Science and Research Technology
ISSN No:-2456-2165
activity in most instances and by the presence of focal
vascular channels[1].

II. CASE PRESENTATION

2.1 Clinical History

A 45year old male presented to Surgery OPD, with
soft to firm subcutaneous swelling since 7years later it
progressed rapidly in size from 1year in the right upper
anterior chest with no history of pain.

On physical examination showed non-tender, multilobulated

exophytic lesion in the subcutaneous plane showed
erythematous nodules of varying size and were firm in (b)
consistency(Fig. 1). Cutaneous changes over the lesion Fig. 2 (a) and (b) Grey scale Ultrasound images showing
noted. lobulated soft tissue mass anteriorly to and about the right
clavicle.

2.3 On Colour doppler, lesion was hypervascular showed

low resistance flow in the arteries. Veins showed normal
flows. No admixture flow are seen in Fig. 3.US revealed the
features of differentials of Hemangioendothelioma or Soft
tissue Sarcoma, however in view of lack of infiltration of
underlying muscle planes,Sarcoma was unlikely. However
there was no admixture flow hence angiosarcoma was ruled
out.

Fig. 1:Multilobulated exophytic lesion in the subcutaneous

plane of Right Supraclavicular region.

2.2 On High frequency Ultrasound of Right

Supraclavicular Region showed well defined Lobulated
nodular heterogenous predominantly hypoechoic
subcutaneous soft tissue mass noted anteriorly to and about
the right clavicle extending to the skin surface measures
about 7.6x5.9x6.6cm(Fig. 2).There was no evidence of
infiltration of the lesion into the underlying pectoral
muscles. (a)

(a) (b)

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Volume 5, Issue 10, October – 2020 International Journal of Innovative Science and Research Technology
ISSN No:-2456-2165

(c)
Fig. 3(a), (b) and (c): Colour Doppler images of Right (b)
Supraclavicular mass showing hypervascular lesion with no Fig. 5: MRI THORAX showing (a) Hypointense lesion on
admixture flow.
T1 WI and (b) Hyperintense on T2 WI with no extension
into the underlying structures clavicle and pectoralis major
2.4 On Computed Tomography(CT) Thorax(Plain) muscle.
showed well defined soft tissue attenuated lesion was seen in
Fig. 4 involving the right supraclavicular region extending to
On Post-contrast T1WI the lesion showed “Target
the skin surface. No evidence of calcification within the Pattern” of hypointense centrally with thick enhancing inner
lesion. peripheral rim and thin non enhancing outer peripheral
rim(Fig.6).

Tubular like hypointense are noted within the lesion on

Post Contrast T1WI suggestive of Intratumoral flow voids
(Fig.6).

Fig. 4 : CT Thorax showing no calcification within the

lesion.

2.5 On MRI Thorax with Contrast

On T1 Weighted Images(T1WI) the lesion was seen in
Fig. 5(a) a large well defined multilobulated Hypointense to
the muscle and in Fig. 5(b) on T2 Weighted Images(T2WI)
there was central hyperintense with peripheral thin (a) (b)
hypointense rim measures 66 x 63 x 77mm (AP x TR x CC)
which was anteriorly extending into the skin surface and
posteriorly lies anterosuperior to the underlying pectoralis
major muscle and medial one third of the clavicle.

(c) (d)
Fig. 6(a), (b), (c) and (d) : MRI THORAX of Axial and
Sagittal sections of Post-contrast T1 Weighted Images
showing Non homogenous enhancement, “Target Pattern” of
hypointense centrally with thick enhancing inner peripheral
rim and thin non enhancing outer peripheral rim with
(a) intratumoral flow voids.

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No evidence of extension of lesion into the underlying
structures clavicle and pectoralis major muscle. Lung
parenchyma appeared normal without mass lesion or
infiltration. There was no mediastinal mass and mediastinal
or hilar lymphadenopathy.

2.6 Histopathological findings:

After wide excision of a tumour the sample sent to the
HPE which showed the deep dermis were comprised of
epithelial and endothelial cells arranged in cords and small
nest pattern. Individual tumour cells shows abundant
eosinophilic cytoplasm with some intracytoplasmic (c)
vacuolesFig. 7. All margins of the excised lesion was free Fig. 9(a), (b) and (c) : Immunohistochemical staining shows
from tumour deposits. The features were suggestive of EH tumour cells express CD31, CD34 and ERG.
of Anterior Chest wall.
After the 2 weeks of wide excision of the
supraclavicular soft tissue the patient was referred to the
radiotherapy unit and advised to take the local radiotherapy
to reduce the risk of local recurrence after the primary
surgery and the patient underwent the PET CT scan in which
there was no uptake of FDG in the previously operated right
supraclavicular region, suggestive of no residual tumour
with no distant metastasis. Then Patient underwent the
adjuvant radiation therapy to decrease the risk of local
recurrence.

III. DISCUSSION
(a) (b)
EH may be a rare vascular tumour with an
epithelioid and histiocytoid appearance, originating from
vascular endothelial or pre-endothelial cells[2].
Epithelioid hemangioendothelioma of soft tissue is most
often a solitary lesion, in either the superficial or deep
tissue with uncertain behaviour and prognosis.

The tumour impacts on both sexes equally and no

predisposing factors are recognized. The neoplasm
usually presents as solitary, rarely multiple, slightly
painful erythematous papules, nodules, plaque and
(c) nonhealing ulcer[7].
Fig. 7(a), (b) and (c): Histopathological slides of the
obtained tissue from the wide excision of lesion. Although epithelioid hemangioendothelioma is
capable of causing regional and distant metastasis, it
2.7 IHC : The cells were immunoreactive for CD31, CD34 does thus far less frequently than the typical
endothelial markers and ERG revealed the endothelial nature angiosarcoma. In a recent study the rate of metastases in
of the cells(Fig. 8). epithelioid hemangioendothelioma is found to be
22%[8].

Because of its rarity, EH has no standard treatment.

The available treatment options are surgical resection,
adjuvant chemotherapy and/or radiotherapy[8],[9].
Histopathological examination remains the mainstay of
diagnosis for this rare tumour. The final diagnosis to be
assisted by the use of immunocytochemical techniques.
The more commonly used antisera are CD 31, CD34,
ERG and factor VIII-related antigen. Radiotherapy after
surgical resection is chosen for localized EH to control
residual disease[8],[9].
(a) (b)

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ISSN No:-2456-2165
The prognosis of EH is uncertain as the mortality and Immunohistochemistry and to arrive at a confirmation
rate for EH of the liver is 35% and lung is 65%. It would of diagnosis followed which patient underwent the wide
seem that the prognosis in primary cutaneous lesions excision with adjuvant radiation therapy to decrease the risk
may be good[7]. of local recurrence and distant metastasis.

In diagnosing the tumour in this case, ultrasonography REFERENCES

demonstrates tumour characterisation and vascularity which
helps in making differential diagnosis. [1]. Weiss SW, Enzinger FM et al;. Epithelioid
Hemangioendothelioma / Soft tissue hemangioendothelioma: a vascular tumor often
Sarcoma/Angiosarcoma, however in view of lack of mistaken for a carcinoma. Cancer 1982;50:970-81.
infiltration of underlying muscle planes, Sarcoma is [2]. Angela Sardaro, Lilia Bardoscia, Maria Fonte
unlikely. There was no admixture flow oncolour doppler, Petruzzelli, and Maurizio Portaluri et al; Epithelioid
Angiosarcoma had been ruled out. Suggest features of Hemangioendothelioma: An Overview and Update on
Supraclavicular soft tissue neoplasm showing vascular a Rare Vascular Tumor 2014 Sep 23; 8(2): 259.
components and concern for Hemangioendothelioma. [3]. Gordillo GM, Onat D, Stockinger M, et al. A key
Magnetic Resonance Imaging(MRI) Thorax of contrast angiogenic role of monocyte chemoattractant protein-1
modality has major role in diagnosing epithelioid in hemangioendothelioma proliferation. Am J Physiol
hemangioendothelioma showing the characteristic Target Cell Physiol 2004;4:C866-73.
pattern of the lesion with minimal delayed capsule [4]. A. LlodioUribeetxebarria, A. Carballeira Álvarez, S.
enhancement and intralesional flow voids. Correa García, M. Esnaola Albizu, J. Vega Eraso, K.
BiurrunMancisidor; Donostia/ES et al; Epithelioid
However CT Thorax is done as the ancillary to the hemangioendothelioma: a diagnostic challenge CT and
MRI has the MRI is most sensitive modality to evaluate the MRI appearance: from typical to bizarre.
soft tissue structures and the soft tissue lesions to delineate [5]. Lau K, Massad M, Pollak C, Rubin C, Yeh J, Wang J,
its characteristics by the extent of the lesion and its et al. Clinical patterns and outcome in epithelioid
relationship to the adjacent structures and provides an hemangioendothelioma with or without pulmonary
excellent contrast resolution. involvement: Insights from an internet registry in the
study of a rare cancer. Chest 2011;140:1312-8.
The patient underwent the wide excision of the right [6]. Friday Titus Nyaku1, Theophilus Maksha Dabkana1,
supraclavicular lesion with skin grafting and the sample sent Haruna A Nggada2, Joasaih Miner Njem3, Stanley
to the HPE showing deep dermis are comprised of epithelial Tella Bwala1, Yakubu Mohammed Gana1, et al
and endothelial cells with abundant eosinophilic cytoplasm Epithelioid haemengioendothelioma: A report of two
with some intracytoplasmic vacuoles consistent with cases 2019.
Epithelioid Hemangioendothelioma and was confirmed by [7]. Enzinger FM, Weiss SW., et al
IHC in which tumour cells were immunoreactive for CD31, Hemangioendothelioma: Vascular tumors of
CD34 endothelial markers and ERG, revealed the intermediate malignancy. In: Enzinger FM, Weiss SW,
endothelial nature of the cells. editors. Soft tissue tumors. 3 rd ed. St. Louis: CV
Mosby 1995. p. 627-31.
Wide excision of the supraclavicular soft tissue was [8]. Deyrup AT, Tighiouart M, Montag AG, Weiss SW, et
done and after 2 weeks the patient was referred to the al Epithelioid haemangioendothelioma of soft tissue: A
radiotherapy unit and advised to take the local radiotherapy proposal for risk stratification based on 49 cases. Am J
to reduce the risk of local recurrence after the primary SurgPathol 2008;32:924-7.
surgery and the patient underwent the PET CT scan in which [9]. Mascarelli PE, Iredell JR, Maggi RG, Weinberg G,
there was no uptake of FDG in the previously operated right Breitschwerdt EB, et alBartonella species bacteremia
supraclavicular region suggestive of no residual tumour with in two patients with epithelioid
no distant metastasis. Then Patient underwent the adjuvant hemangioendothelioma. J Clin Microbiol
radiation therapy to decrease the risk of local recurrence. 2011;49:4006-12.
[10]. Gaur S, Torabi A, O'Neill TJ, et al Activity of
IV. CONCLUSION angiogenesis inhibitors in metastatic epithelioid
hemangioendothelioma: A case report. Cancer Biol
The importance of distinguishing epithelioid vascular Med 2012;9:133-6.
tumor on suspecting malignant epithelioid vascular tumor on [11]. Sumana Mukherjee, Jayati Mallick, Prabir C Pal,
imaging and further characterization into subgroups, using Sarbani Chattopadhyay, et al Hemangioendothelioma
Ultrasound and most sensitive MRI modality features are of soft tissue: Cytological dilemma in two cases at
confirmed with pathological techniques of Histopathology unusual sites 2012;29;89-91.

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