Case report
A 56-year-old female patient reported the appearance of a nodule on the posterior region of the right arm six months before. During this period, she reported that the lesion showed progressive growth, pain, and friability, with frequent local bleeding. This patient had been submitted to a radical mastectomy and right axillary dissection, with adjuvant chemotherapy and radiotherapy 11 years before, and since then, she had chronic lymphedema in the right upper limb.
Dermatological examination revealed a hyperchromic tumor, measuring approximately 5.0 × 4.0 cm, with central areas of ulceration and slight local bleeding on the posterior region of the right arm. Lymphedema was also observed in the right upper limb and violaceous-brown satellite nodules, measuring up to 0.5cm (Fig. 1).
(A), Lymphedema in the right upper limb. (B), Tumor with ulcerated areas in the posterior region of the right arm, accompanied by satellite nodules.
An incisional biopsy of the tumor and satellite nodules was performed. The patient returned for consultation after one month with a histopathological result that showed a neoplasm characterized by vascular formations permeated by atypical epithelioid cells infiltrating the superficial and deep dermis, with perineural permeation (Fig. 2).
Proliferation of epithelioid and spindle cells, permeating malformed vascular channels and spaces, (Hematoxylin & eosin, ×100).
At this consultation the tumor was slightly larger and there was an increase in the number of satellite lesions (Fig. 3).
Significant increase in the number of satellite lesions and in the ulcerated area of the central tumor.
What is your diagnosis?
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a) Stewart-Treves syndrome
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b) Squamous cell carcinoma
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c) Melanoma
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d) Merkel carcinoma
Immunohistochemistry examination was requested, which showed CD31 positivity, confirming the hypothesis of Stewart-Treves syndrome (Fig. 4).
Discussion
Stewart-Treves syndrome (STS) is a rare entity, with a poor prognosis, consisting of the appearance of cutaneous angiosarcoma in areas of chronic lymphedema and accounts for approximately 5% of angiosarcomas.11 Wang LL, Cui LF, Gao Y, Jiang ZC. Clinicopathologic features of Stewart-Treves syndrome. Int J Clin Exp Pathol. 2019;12:680–8.,22 Sharma A, Schwartz RA. Stewart-Treves syndrome: pathogenesis and management. J Am Acad Dermatol. 2012;67:1342–8.,33 Degrieck B, Crevits I. Cutaneous angiosarcoma postmastectomy (Stewart-Treves Syndrome). J Belg Soc Radiol. 2018;102:60.,44 Murgia RD, Gross GP. Stewart-Treves syndrome [publicação na web]. In: StatPearls. Treasure Island (FL): Statpearls publishing; 2020. Accessed 12 August 2021. Available from: https://www.ncbi.nlm.nih.gov/books/NBK507833/
https://www.ncbi.nlm.nih.gov/books/NBK50...
Its occurrence is more commonly observed after a radical mastectomy with axillary dissection, with a latency period that varies from five to 11 years.22 Sharma A, Schwartz RA. Stewart-Treves syndrome: pathogenesis and management. J Am Acad Dermatol. 2012;67:1342–8.,44 Murgia RD, Gross GP. Stewart-Treves syndrome [publicação na web]. In: StatPearls. Treasure Island (FL): Statpearls publishing; 2020. Accessed 12 August 2021. Available from: https://www.ncbi.nlm.nih.gov/books/NBK507833/
https://www.ncbi.nlm.nih.gov/books/NBK50...
However, it can also appear in areas of chronic lymphedema due to venous stasis, morbid obesity, post-surgical procedures, lymphatic malformations, and chronic infections, among others.22 Sharma A, Schwartz RA. Stewart-Treves syndrome: pathogenesis and management. J Am Acad Dermatol. 2012;67:1342–8.,55 Benmansour A, Laanaz S, Bougtab A. Stewart–Treves syndrome: a case report. PAMJ One Health. 2014;19:2.
The etiology and pathophysiology of the syndrome remain unknown. It is debated whether the lymphedema would favor oncogenesis due to the lymphatic drainage failure and interstitial fluid accumulation and stasis, in addition to the possible occurrence of neoplastic transformation during angiogenesis.44 Murgia RD, Gross GP. Stewart-Treves syndrome [publicação na web]. In: StatPearls. Treasure Island (FL): Statpearls publishing; 2020. Accessed 12 August 2021. Available from: https://www.ncbi.nlm.nih.gov/books/NBK507833/
https://www.ncbi.nlm.nih.gov/books/NBK50...
They initially present as macules and papules, developing into nodules and tumors that can reach large volumes.66 Pereira ESP, Moraes ET, Siqueira DM, Santos MAS. Síndrome de Stewart-Treves. An Bras Dermatol. 2015;90:226–8.,77 Veiga RRG, Nascimento BAM, Carvalho AH, Brito AC, Bittencourt MJS. Síndrome de Stewart-Treves no membro inferior. An Bras Dermatol. 2015;90:229–31. Satellite lesions, pain, and local bleeding are common.66 Pereira ESP, Moraes ET, Siqueira DM, Santos MAS. Síndrome de Stewart-Treves. An Bras Dermatol. 2015;90:226–8.
The diagnosis is based on the clinical picture, along with the biopsy. Histopathology may be suggestive, with irregular vascular spaces lined by mitotic hyperchromatic pleomorphic tumor endothelial cells.44 Murgia RD, Gross GP. Stewart-Treves syndrome [publicação na web]. In: StatPearls. Treasure Island (FL): Statpearls publishing; 2020. Accessed 12 August 2021. Available from: https://www.ncbi.nlm.nih.gov/books/NBK507833/
https://www.ncbi.nlm.nih.gov/books/NBK50...
Immunohistochemistry helps in the diagnostic confirmation, with both CD31 and CD34 markers being positive.44 Murgia RD, Gross GP. Stewart-Treves syndrome [publicação na web]. In: StatPearls. Treasure Island (FL): Statpearls publishing; 2020. Accessed 12 August 2021. Available from: https://www.ncbi.nlm.nih.gov/books/NBK507833/
https://www.ncbi.nlm.nih.gov/books/NBK50...
The treatment is based on surgical resection with wide margins, and chemotherapy and radiotherapy can also be used.22 Sharma A, Schwartz RA. Stewart-Treves syndrome: pathogenesis and management. J Am Acad Dermatol. 2012;67:1342–8.,33 Degrieck B, Crevits I. Cutaneous angiosarcoma postmastectomy (Stewart-Treves Syndrome). J Belg Soc Radiol. 2018;102:60.,66 Pereira ESP, Moraes ET, Siqueira DM, Santos MAS. Síndrome de Stewart-Treves. An Bras Dermatol. 2015;90:226–8.,77 Veiga RRG, Nascimento BAM, Carvalho AH, Brito AC, Bittencourt MJS. Síndrome de Stewart-Treves no membro inferior. An Bras Dermatol. 2015;90:229–31. In the present case, the oncology service chose to use chemotherapy with docetaxel, and the oncology surgery team, so far has not indicated surgical treatment. The occurrence of distant metastases is not uncommon,33 Degrieck B, Crevits I. Cutaneous angiosarcoma postmastectomy (Stewart-Treves Syndrome). J Belg Soc Radiol. 2018;102:60. with the lungs being the most commonly affected organs; the patient in the present case was submitted to CT scans of the chest, abdomen, and pelvis, which did not disclose the presence of metastases.
Although it is rare, knowledge of STS is necessary because, due to its aggressiveness, only its early diagnosis can help to increase patient survival.
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Financial supportNone declared.
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☆
Study conducted at the Department of Dermatology, Faculdade de Medicina de Jundiaí, Jundiaí, SP, Brazil.
References
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1Wang LL, Cui LF, Gao Y, Jiang ZC. Clinicopathologic features of Stewart-Treves syndrome. Int J Clin Exp Pathol. 2019;12:680–8.
-
2Sharma A, Schwartz RA. Stewart-Treves syndrome: pathogenesis and management. J Am Acad Dermatol. 2012;67:1342–8.
-
3Degrieck B, Crevits I. Cutaneous angiosarcoma postmastectomy (Stewart-Treves Syndrome). J Belg Soc Radiol. 2018;102:60.
-
4Murgia RD, Gross GP. Stewart-Treves syndrome [publicação na web]. In: StatPearls. Treasure Island (FL): Statpearls publishing; 2020. Accessed 12 August 2021. Available from: https://www.ncbi.nlm.nih.gov/books/NBK507833/
» https://www.ncbi.nlm.nih.gov/books/NBK507833/ -
5Benmansour A, Laanaz S, Bougtab A. Stewart–Treves syndrome: a case report. PAMJ One Health. 2014;19:2.
-
6Pereira ESP, Moraes ET, Siqueira DM, Santos MAS. Síndrome de Stewart-Treves. An Bras Dermatol. 2015;90:226–8.
-
7Veiga RRG, Nascimento BAM, Carvalho AH, Brito AC, Bittencourt MJS. Síndrome de Stewart-Treves no membro inferior. An Bras Dermatol. 2015;90:229–31.
Publication Dates
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Publication in this collection
30 Sept 2022 -
Date of issue
Sep-Oct 2022
History
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Received
14 Oct 2021 -
Accepted
14 Dec 2021 -
Published
08 July 2022