Exuberant angiosarcoma^*

Dear Editor,

We report the case of a white, female patient, 78 years of age, presenting an erythematous rash on the face for the past two months, which was asymptomatic but was gradually increasing in size. She had used antibiotics and acyclovir, with no improvement in the lesion. Dermatological examination revealed an erythematous-violaceous plate, with revealed an, and accentuated follicular ostia, affecting the scalp, the upper eyelid, and preauricular, frontal, and mandibular areas, on the left side (Figure 1a). An incisional biopsy performed revealed preserved epidermis and dermis with diffuse infiltration of vascular clefts slits, which dissect collagen fibers, corresponding to pleomorphic ectasic vascular channels with atypical endothelial cells (Figure 2). The immunohistochemical pattern revealed positive CD31, CD34, Vimentin, and Ki67, confirming angiosarcoma (Figure 3).

Figure 1
A- Erythematous-violaceous plaque. B- Full remission of the lesion after six week of chemotherapy. C- Erythematous nodules: recrudescence one month after chemotherapy end

Figure 2
Histopathology: ectasic vascular channels dissecting collagen fibers, 40 HE

Figure 3
Immunohistochemistry: A- Positive vimentin, 200x. B- Positive Ki 67, 100x. C- Positive CD34, 100x. D- Positive CD31, 100x

We chose to start chemotherapy with a weekly 80mg/m² dose of paclitaxel. After six weeks, a nearly full remission of the skin lesion was observed, leaving only residual hypochromia areas (Figure 1b). Nevertheless, the patient completed twelve weeks of treatment. One month after chemotherapy had ended, however, the tumor recrudesced, and erythematous nodules appeared on the same topography as before, with an exulceration area and a purulent discharge (Figure 1c). Treatment with paclitaxel was restarted, with no response after three weeks. Therefore, we chose to switch medication and started the patient on liposomal doxorubicin, 55mg/m², every four weeks, but without clinical improvement. Palliative care was started for the patient, evolved to death 14 months after diagnosis.

Four skin angiosarcoma variants are recognized nowadays: scalp and face angiosarcoma, lymphedema angiosarcoma (Stewart-Treves syndrome), radiation induced angiosarcoma, and epithelioid angiosarcoma.¹ Scalp and face angiosarcoma, the variant exhibited by the patient in the study, is the most common subtype, usually presents an insidious onset, is usually displayed as a violaceous rash, or as nodules, plaques or infiltrate areas. In the most advanced phases, it may bleed or ulcerate. Clinical differential diagnoses include seborrheic dermatitis, ecchymosis, hemangioma, cellulitis, erysipela, rosacea, angioedema, melanoma and Kaposi's sarcoma.² In histopatholo-gy, pleomorphic malignant endothelial cells may be noticed, forming vascular sinusoids that dissect collagen fibers. Architecture becomes chaotic, when the disease is more aggressive, with undefined vascular spaces.³ In this case, some immunohistochemical markers are useful in reaching a diagnosis, such as the Von Willebrand factor, Ulex europaeus agglutinin-1, laminin, CD31, CD34, vascular endothelial growth factor, and vimentin.^1,3,4 Metastases are not common in the initial examination, although a third of patients develop them, and the lung is the most frequently affected.³ Currently, there are no standard guidelines for treating skin angiosarcoma. When the tumor is in the initial stage, radical surgical excision constitutes the first therapeutic option and may be accompanied by adjuvant radiation therapy, as the tumor may display clinically undetectable local dissemination.² However, when the disease is extensive, radiation therapy or chemotherapy may be considered.^2,5 For metastatic disease cases, cytotoxic chemotherapy is the preferred treatment. Standard palliative chemotherapy for soft tissue sarcoma has been performed with doxorubicin. However, taxanes, especially paclitaxel, stand out as promising agents in treating this class of tumors.⁵

• Financial support: None
• *
  Work developed at the Tropical Dermatology Service at the Hospital Central do Exército (HCE), Rio de Janeiro, RJ, Brazil.

References

• ¹
  Dhanasekar P, Karthikeyan VS, Rajkumar N, Chandra Sistla S, Manwar Ali S, Basu D, et al. Cutaneous Angiosarcoma of the scalp masquerading as a squamous cell carcinoma: case report and literature review. J Cutan Med Surg. 2012;16:187-90.
• ²
  Kim JE, Kim BJ, Kang H. A recurrent angiosarcoma isolated to the eyelid without the recurrence on the primary lesion of the Forehead. Ann Dermatol. 2014;26:231-5.
• ³
  Young RJ, Brown NJ, Reed MW, Hughes D, Woll PJ. Angiosarcoma. Lancet Oncol. 2010;11:983-91.
• ⁴
  Farias T P, Câmara MVM, Dias FL, Maia Filho PC, Rangel LG, Peryassu BC, Costa RM. Evolução dos angiossarcomas da cabeça e pescoço. Rev Bras Cir Cabeça Pescoço. 2008;37:104-8.
• ⁵
  Wollina U, Hansel G, Schönlebe J, Averbeck M, Paasch U, Uhl J et al. Cutaneous angiosarcoma is a rare aggressive malignant vascular tumour of the skin. J Eur Acad Dermatol Venereol. 2011;25:964-8.

Publication Dates

• Publication in this collection
  Mar-Apr 2017

History

• Received
  02 Apr 2016
• Accepted
  01 Aug 2016