SM Journal of Sarcoma Research

Case Report © Green C. et al. 2021

Epithelioid Angiosarcoma in the Inguinal Canal

Region of an 81-year-old man. Case Report of a Rare
Tumor with Challenging Diagnosis and Review of the
Literature
Christian Green*, Malina Mohtadi, Joseph Russo, Brandon Watkins, Siovan Hope-Ross, Zachary
Elder, Jacqueline Nicholas, Mohamed Aziz
Department of Pathology- American University of the Caribbean, School of Medicine, USA

Abstract
Angiosarcomas, which are endothelial in origin, make up less than 1% of all soft tissue sarcomas. For every million people in the
United States, one will be diagnosed with an angiosarcoma. Most angiosarcomas are found in the breast, deep soft tissue of the head and
neck, and skin, though they may rarely arise within other soft tissue sites. Epithelioid angiosarcomas represent a diagnostically challenging
subset of angiosarcomas due to their non-specific imaging features, non-specific clinical presentation and rarity of the neoplasm. Epithelioid
angiosarcomas should always be included in the differential diagnosis in patients with vascular neoplasms. Early detection combined with
complete resection of the mass, as well as adjuvant therapy if possible, yields the highest favorable prognosis for patients with EAs.

Keywords: Epithelioid, angiosarcoma, carcinoma, Immunohistochemistry, vascular

ABBREVIATION [8]. Angiosarcomas can develop at any age, however the median
age is reported to be between 60 and 71 years old [15,12]. The
EA: Epithelioid angiosarcoma, AS: Angiosarcoma, IHC: head and neck account for the majority in location [16,12]. Risk
Immunohistochemistry, EM: electron microscopy factors for epithelioid angiosarcoma include toxic chemical
INTRODUCTION exposure, Thorotrast exposure, use of Dacron vascular grafts,
chronic lymphedema, and previous irradiation, though the
Angiosarcomas (ASs) are endothelial in origin and make etiology has not been completely confirmed [4]. Certain familial
up less than 1% of all soft tissue sarcomas [1,2]. Endothelial syndromes are associated with AS such as neurofibromatosis
malignancies are derived from mesenchymal origin, which can type-1, Maffucci syndrome, bilateral retinoblastoma, von
undergo blood vessel or lymphatic differentiation, though they Recklinghausen syndrome, hemochromatosis, and Klippel-
may follow both endothelial cell lines [26]. Trenaunay syndrome and appear with other lesions like port-
Classically, angiosarcomas develop in the breast, head, neck wine stains, and hemangiomas [11,17,10,18].
or skin of patients who are 60-70 years of age [1,12,15]. The EAs represent a problematic subset of endothelial neoplasms
range of primary sites is credited to the universality of lymphatics due to non-specific radiological signs of malignancy, as well as
and blood vessels throughout the body. Deep soft tissues have an ambiguous clinical presentation, rarity of the neoplasm, and
an extensive lymphovascular supply and thus are at the highest histomorphologic resemblance when compared to more prevalent
risk for developing ASs. Angiosarcomas vary and involve varied benign and malignant neoplasms. Immunohistochemistry (IHC)
patterns of growth, including spindled, papillary and epithelioid is paramount in diagnosing EA. In conjunction with IHC, electron
morphology [4]. Epithelioid angiosarcomas (EAs) typically form microscopy (EM) can provide further identifying information
spongy, hemorrhagic masses due to their vascular characteristics for EAs [23]. Patients usually experience pain and the presence
of a mass, followed by fever, weakness, and weight loss [7]. In
Submitted: 20 August, 2021 | Accepted: 12 September, 2021 | Published: some studies, up to one-third of patients with EA experience a
15 September, 2021 hypocoagulable state, including ecchymosis, gastrointestinal and
peritoneal bleeding, and persistent hematomas [10].
*Corresponding author(s): Christian Green, Department of Pathology,
American University of the Caribbean School of Medicine, 1 University Preventative measures for EA include avoiding Thorotrast
Drive at Jordan Road Cupecoy, St. Maarten, Tel: +16013071393
exposure or use of Dacron vascular grafts, though most patients
Copyright: © 2021 Green C, et al. This is an open-access article develop EA without an association with these risk factors [4].
distributed under the terms of the Creative Commons Attribution Complete resection is considered the current standard of care
License, which permits unrestricted use, distribution, and reproduction in in patients with EA, though the complexity of the small area of
any medium, provided the original author and source are credited.
the head and neck make negative margins difficult to achieve.
Citation: Green C, Mohtadi M, Russo J, Watkins B, Hope-Ross S, et Paclitaxel and Bevacizumab used in conjunction with surgical
al. (2021) Epithelioid Angiosarcoma in the Inguinal Canal Region of an intervention has been shown to improve prognosis [24,25]. We
81-year-old man. Case Report of a Rare Tumor with Challenging Diagnosis present a case of epithelioid angiosarcoma in the inguinal canal
and Review of the Literature. SM J Sarcoma Res 5: 4.
region in an 81-year-old man and we review the literature.

SM J Sarcoma Res 5 : 4 1/4

CASE PRESENTATION CK 5/6, 34 BE12, CK19, HMB45, S100, CD21, CD35, LCA (CD45),
SMA, PAX-2, B-Catenin, CD34, WT-1, CD68, and CD99. Additional
An 81-year-old man presented with a large painful soft tissue negative IHC studies included Desmin, H-Caldesmon, Myogenin,
mass at the left inguinal region. He also reported a weight loss PLAP, AFB, CD138, PSA, Synaptophysin and Chromogranin.
of 15 Ib during the year before current presentation. Physical This negative IHC studies were sufficient to rule out epithelial,
examination noted two adjacent large soft tissue masses in the myoepithelial, lymphoid, dendritic, histiocytic, mesothelial or
left groin region. CT scan studies revealed two soft tissue masses melanocytic origin. The tumor cells were, however, strongly and
with irregular enhancement and decreased heterogeneous diffusely positive for Vimentin, which in absence of other specific
density infiltrating the surrounding soft tissue structures. markers supported mesenchymal origin of the tumor. As muscle
MRI scan demonstrated a heterogeneous enhancement of and neural mesenchymal origin were also ruled out, vascular IHC
the two masses, with ill-defined demarcation with infiltrated markers were tested. The tumor cells were strongly positive for
surrounding structures and non-enhanced necrosis inside the CD31, Fli-1, and Cytokeratin AE1/AE3 (Focal scattered positive
lesions. Patient reported history of controlled type-II diabetes, cells), in addition to Vimentin (Figure 1 D-E-F).
controlled hypertension and controlled hypercholesterolemia.
The tumor masses measured 5.5x 3.5x 3 cm and 4X 3x 2.5 cm. The histomorphologic features and the IHC profile were
He also reported localized prostatic adenocarcinoma treated by consistent with the diagnosis of epithelioid angiosarcoma in the
prostatectomy 10 years prior to current presentation. Inguinal canal region. Patient underwent staging investigations,
and none were shown to have distant metastasis. The case
A fine needle aspiration and core biopsy were obtained was discussed in a multidisciplinary tumor board and it was
from the larger inguinal mass. Microscopic examination recommended to undergo total surgical excision followed by
demonstrated a tumor composed of solid sheets of high- adjuvant radiotherapy. Patient refused surgery or radiation
grade pleomorphic epithelioid neoplastic cells with abundant therapy and chemotherapy was initiated with six courses of
amphophilic to lightly eosinophilic cytoplasm, large vesicular combination chemotherapy with paclitaxel, epirubicin and
nuclei, and prominent nucleoli. The tumor was set in fibrotic carboplatin. Patient tolerated the treatment well and showed no
stroma with scattered complex anastomosing vascular channels evidence of recurrence or metastasis for 8 months after which he
and abundant abnormal mitosis exceeding 15 mitosis/10 HPF. was lost to follow up.
Prominent tumor necrosis was also noted (Figure 1 A-B-C). The
histomorphologic features were non-specific and could be seen DISCUSSION
in various carcinomas, epithelioid variants of mesenchymal
Angiosarcomas, which are endothelial in origin, make up
tumors, anaplastic tumors as well as other malignant tumors.
less than 1% of all soft tissue sarcomas.1,2 Angiosarcomas have
Although the tumor displayed prominent epithelioid features,
a propensity for breast, deep soft tissue of the head and neck,
immunohistochemistry studies were essential to determine the
and skin, accounting for 5.4% of all skin sarcomas.1,3 Rarely,
line of cell differentiation. The tumor cells were negative for
angiosarcomas may arise within other soft tissue malignancies.6
numerous lineage specific markers including Cam5.2, EMA, P63,

Figure 1 Histomorphology and immunohistochemistry profile of the excised angiomatoid angiosarcoma

1A: The tumor is set in fibrotic stroma with scattered complex anastomosing vascular channels (H&E stain X20 magnification)
1B: Low power view of the tumor showing masses of cellular epithelioid tumor cells (H&E stain X40 magnification)
1C: High power view of the tumor showing high-grade pleomorphic epithelioid neoplastic cells with abundant amphophilic to lightly eosinophilic
cytoplasm, large vesicular nuclei, prominent nucleoli and multiple abnormal mitosis (H&E stain X60)
1D: Tumor cells positive for Vimentin
1E: Tumor cells positive for CD-31
1F: Tumor cells positive for Fli-1

SM J Sarcoma Res 5: 4 2/4

 EAs can resemble many types of neoplasms: metastatic is found to have a 5-year survival rate ranging from 12%-35%
melanoma, anaplastic large cell lymphoma, epithelioid sarcoma, [19,12]. The overall mean survival was seen to be 36 months
epithelioid hemangioma, epithelioid hemangioendothelioma, with adjuvant radiotherapy versus a 9 months survival without
metastatic carcinoma, proximal-type epithelioid sarcoma, adjuvant therapy [20,12]. This case report and literature review
epithelioid rhabdomyosarcoma and epithelioid-variant seek to shed light on the variability and diagnostic challenges
nerve sheath tumors. Low grade EA tumors often resemble a in patients with epithelioid angiosarcoma. The non-specific
hemangioma, and high grade EA tumors may appear as various radiological signs of malignancy, as well as an ambiguous clinical
anaplastic neoplasms [14,10,12]. On contrast CT soft tissue EA presentation, rarity of the neoplasm, and histomorphologic
can be seen as an irregular enhanced mass, occasionally with resemblance when compared to more prevalent benign and
underlying bone or organ invasion [13,12]. EA tumors, such as malignant neoplasms pose a threat to epithelioid angiosarcomas
those in the soft tissue of the extremities, abdomen, chest wall, being mis-diagnosed. The definitive diagnosis of EA is made
and peritoneum or retroperitoneum are found to display differing through biopsy and meticulous evaluation through histological
characteristics [10,12]. EAs in the extremities are fast growing and specific immunohistochemical analysis, and possibly EM
and palpable, compared to those in the peritoneum which studies. Epithelioid angiosarcomas should always be included in
are associated with increased pain due to mass effect [11,12]. the differential diagnosis in patients with vascular neoplasms.
Epithelioid hemangiomas are generally found in a younger
patients, usually does not express factor VIII, and demonstrates
ACKNOWLEDGMENT
normal nuclei well-formed vascularity, demonstrating its benign Special thanks to Grecia Corrada, Jacob Selph, and Lorenzo
nature.21 Negative staining for S-100 and HMB-45 excludes Russo, MD candidates, American University of the Caribbean for
melanoma from the differential diagnosis.4 Carcinoma will not their assistance in reviewing the final version of this manuscript.
stain positive for endothelial markers, including CD31, CD34,
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