INTRODUCTION
The term “sarcoma” is derived from the Greek “sarkos” (flesh) and “oma” (tumor). Sarcomas
comprise a heterogeneous group of mesenchymal neoplasms and are classified into primary
bone sarcomas and soft tissue (cutaneous) sarcomas1.
Among soft tissue sarcomas, cutaneous angiosarcomas deserve special attention and
can be divided into:
Idiopathic angiosarcoma: the most common type, especially in the elderly, characterized by ecchymotic plaques
and/or friable violaceous nodules, ulcerated or not, usually on the head and neck;
Angiosarcoma secondary to chronic lymphedema: a violaceous nodule or infiltrated plaque more common in mastectomized patients who
underwent axillary clearance (Stewart-Treves syndrome);
Post-radiation angiosarcoma: a rare sarcoma associated with conservative treatment of breast cancer usually presenting
as infiltrated plaques or nodules adjacent to the irradiated area; and
Low-grade angiosarcomas1. Cutaneous angiosarcoma is a rare tissue soft tissue sarcoma with an incidence of
approximately 2% and a poor prognosis2.
This disease is most common in the age group >60 years and in men (2:1 ratio)3. Its clinical forms include bruises, violaceous nodules and plaques, and diffuse
hemorrhagic lesions with infiltrative growth3. These lesions are histologically indistinguishable and comprise a network of dermal
vascular channels varying in size from small capillaries to sinusoidal spaces interspersed
with normal endothelium4. One study showed that clinical outcomes were not favorable even after complete tumor
resection, as for other sarcomas5.
OBJECTIVE
To describe the surgical approach and other treatment options for cutaneous angiosarcoma.
CASE REPORT
An 80-year-old man presented with a lesion present in the upper third of the ear for
more than 1 year (Figures 1, 2, and 3). The patient reported no itching or pain. He reported that the lesion growth was
slow and progressive, which motivated him to seek a specialized evaluation.
Figure 1 - Lesion in the upper third of the right ear.
Figure 1 - Lesion in the upper third of the right ear.
Figure 2 - Lesion in the upper third of the right ear.
Figure 2 - Lesion in the upper third of the right ear.
Figure 3 - Lesion in the upper third of the right ear.
Figure 3 - Lesion in the upper third of the right ear.
The examination showed a hyperchromic, nodular, and hard lesion in the upper third
of the right ear. The lesion had regular borders but no inflammatory signs or secretions.
There was no evidence of enlarged cervical lymph nodes.
Surgical resection of the upper third of the right ear was performed under local anesthesia
(Figures 4 and 5) and the wound edges were sutured. Surgical reconstruction was scheduled for a later
date (Figure 6) because of the uncertain nature of the lesion and a high suspicion of malignancy.
Figure 4 - Surgical specimen (anterior view).
Figure 4 - Surgical specimen (anterior view).
Figure 5 - Surgical specimen (posterior view).
Figure 5 - Surgical specimen (posterior view).
Figure 6 - Aspect at 6 months after surgery.
Figure 6 - Aspect at 6 months after surgery.
A histopathological examination showed the presence of tumors with poorly differentiated
mesenchymal cells compatible with cutaneous angiosarcoma. An immunohistochemical examination
showed positive human anti-CD34.
DISCUSSION
Cutaneous angiosarcoma should be treated by a multidisciplinary team according to
the lesion extent, anatomical location, and patient preferences. Surgical resection
alone or in combination with radiotherapy is used for early lesions, although wide-margin
resection is not always feasible.
Chemotherapy is indicated for disseminated tumors and can be combined with radiotherapy
for the locoregional treatment of extensive lesions or as neoadjuvant therapy. The
most commonly used chemotherapeutic agents are doxorubicin, cyclophosphamide, methotrexate,
and vincristine. The concomitant use of alpha-interferon and 13-cis-retinoic acid
in advanced disease is reportedly effective.
Treatment should be aggressive in previously irradiated areas. Despite this wide range
of treatment options, recurrence is common and the prognosis is poor.
Angiosarcoma originating from chronic lymphedema is aggressive, and complete amputation
of the affected limb increases patient survival.
Among soft tissue sarcomas of the head and neck, angiosarcoma has a high rate of lymph
node and distant metastases, corresponding to 50% of cases; the most commonly affected
organ is the lung. The prognosis is poor, and the 5-year survival rate is less than
10–30%.
CONCLUSION
Cutaneous sarcomas are rare; however, aggressive treatment and adequate follow-up
are crucial in suspected cases. Complete tumor resection, chemotherapy, and radiotherapy
are the primary treatments for angiosarcoma depending on patient preference.
COLLABORATIONS
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SSC
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Analysis and/or data interpretation, Data Curation, Final manuscript approval, Realization
of operations and/or trials, Supervision, Visualization, Writing - Review & Editing
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VPB
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Analysis and/or data interpretation, Realization of operations and/or trials, Writing
- Original Draft Preparation, Writing - Review & Editing
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NCNC
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Writing - Original Draft Preparation, Writing - Review & Editing
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AMM
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Writing - Original Draft Preparation
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DMAP
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Writing - Original Draft Preparation, Writing - Review & Editing
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ARD
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Writing - Original Draft Preparation, Writing - Review & Editing
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REFERENCES
1. Fernandes S, Pinto GM, Moura C, Afonso A, Cardoso J. Sarcomas cutâneos – do diagnóstico
ao tratamento. J Port Soc Dermatol Venereol. 2012;70(3):319.
2. Ishida Y, Otsuka A, Kabashima K. Cutaneous angiosarcoma: update on biology and latest
treatment. 2018;30(2):107-12. DOI: https://doi.org/10.1097/CCO.0000000000000427
3. Kim JDU, Santos ABO, Kulcsar MAV, Cernea CR, Brandão LG. Tumores cutâneos raros em
cabeça e pescoço: experiência de 4 anos em uma instituição terciária. Rev Bras Cir
Cabeça Pescoço. 2014;43(2):63-71.
4. Fleury Junior LFF, Sanches JA. Sarcomas cutâneos primários. An Bras Dermatol. 2006;81(3):207-21.
5. Fujisawa Y, Yoshino K, Fujimura T, Nakamura Y, Okiyama N, Ishitsuka Y, et al. Cutaneous
angiosarcoma: the possibility of new treatment options especially for patients with
large primary tumor. Front Oncol. 8:46. DOI: https://doi.org/10.3389/fonc.2018.00046
1. Universidade Federal de Uberlândia, Uberlândia, MG, Brazil.
Corresponding author: Victor Parreira Bizinoto Avenida Mato Grosso, 3395, Apto 202, Umuarama, Uberlândia, MG, Brazil. Zip Code:
38402-043. E-mail: carlosgoye.m@gmail.com
Article received: February 13, 2019.
Article accepted: July 8, 2019.
Conflicts of interest: none.
