Table of Contents  
CASE REPORT
Year : 2021  |  Volume : 8  |  Issue : 2  |  Page : 187-189

Angiosarcoma secondary to primary upper limb lymphedema


Department of Vascular Surgery and Pathology, Faculty of Medicine and Pharmacy of Oujda, Mohammed First University, Oujda, Morocco

Date of Submission20-May-2019
Date of Acceptance31-May-2020
Date of Web Publication13-Apr-2021

Correspondence Address:
Husam Bashir
Department of Vascular Surgery and Pathology, Faculty of Medicine and Pharmacy of Oujda, Mohammed First University, Oujda
Morocco
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/ijves.ijves_65_20

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  Abstract 


Stewart–Treves syndrome (STS) is a rare entity with a poor prognosis defined as an angiosarcoma appearing in a specific clinical setting, in 90% of cases in a context of breast neoplasia. Herein, we report a rare case of STS of the upper limb as a complication of primary lymphedema. A 55-year-old male was referred for a large, reddish, necrotic multinodular tumor of rapid extension on the anterior aspect of the right forearm. A biopsy of the lesions was performed, confirming the diagnosis of angiosarcoma. The evaluation of tumor extension showed no distant metastasis. An amputation of the arm was performed. Angiosarcoma as a complication of primary lymphedema is a very rare entity with very poor prognosis.

Keywords: Angiosarcoma, lymphedema, Stewart–Treves syndrome


How to cite this article:
Bashir H, alzaarir H, Abdellah R, Benzirar A, El Mahi O. Angiosarcoma secondary to primary upper limb lymphedema. Indian J Vasc Endovasc Surg 2021;8:187-9

How to cite this URL:
Bashir H, alzaarir H, Abdellah R, Benzirar A, El Mahi O. Angiosarcoma secondary to primary upper limb lymphedema. Indian J Vasc Endovasc Surg [serial online] 2021 [cited 2021 Jun 24];8:187-9. Available from: https://www.indjvascsurg.org/text.asp?2021/8/2/187/313561




  Introduction Top


In 1948, Stewart and Treves reported for the first time a series of six cases of cutaneous angiosarcoma developed in lymphedema of the upper limb after mastectomy with axillary dissection for breast cancer.

These tumors are rare, less than 1% of all sarcomas,[1] and have high clinical aggressiveness and poor prognosis. The mechanism and pathophysiology of this neoplasia remain unknown at present.


  Case Report Top


This was a 55-year-old male followed by epilepsy from childhood treated with Gardenal, who has had chronic lymphedema of the right upper limb, evolving for 5 years. There were no similar cases in the family. The patient consulted in June 2019, after 4 months of appearance of a nodular lesion on the anterior aspect of the right forearm gradually increasing in size. The clinical examination on admission objectifying a multinodular erythematous-violet skin lesion [Figure 1]. This lesion is located at the level of the upper 1/3 of the forearm, which is edematous measuring 9 cm × 7.5 cm × 11 cm. The evolution was marked by infection and ulceration of the lesion [Figure 2]. Magnetic resonance imaging of the limb demonstrated the lesion extension in the cutaneous and subcutaneous soft parts, anterior and external of the forearm, poorly limited compared to the muscular structures of the anterior compartment, hypointense in T1, hypo- and hyperintense in T2, and hyperintense in diffusion-weighted sequence with intense and heterogeneous enhancement after injection of gadolinium, without affecting the bony structures of the forearm [Figure 3]. A biopsy of the lesion confirmed the diagnosis of cutaneous angiosarcoma on chronic lymphedema.
Figure 1: Large red necrotic and multinodular lesion of the forearm

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Figure 2: Infection and ulceration of lesions (3 weeks later)

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Figure 3: Magnetic resonance imaging of forearm T1 sequence showing: An hypointense lesion of the soft tissue

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  Discussion Top


Stewart–Treves syndrome (STS) or angiosarcoma secondary to chronic lymphatic obstruction is a rare and fatal disease, representing approximately 1%–2% of soft tissue sarcomas and <1% of malignant tumors.[1] It is most commonly seen in the upper limb of breast cancer patients who underwent mastectomy and excision of the axillary lymph nodes.[2] It has been rarely reported in other situations of lymphatic stasis such as congenital hereditary or nonhereditary and posttraumatic or postinfectious lymphedema.[3],[4] Angiosarcoma in primary idiopathic lymphedema has been reported especially in the lower limbs, while its occurrence in the upper limb without a history of mastectomy with axillary dissection is extremely rare. The condition predisposing the development of SST is the lack of lymphatic drainage induced by surgical treatment or which can be indirectly aggravated by radiotherapy.[5] Blockage of lymphatic circulation stimulates the growth of lymphatic vessels probably via cytokines, such as the vascular endothelial growth factor. Radiation therapy may increase the risk of lymphedema by increasing sclerosis of the tissue or, in some cases, inducing direct DNA damage. Other cofactors are likely to be necessary: the mutation of a tumor suppressor gene such as p53 or BRCA1 could favor the appearance of neoplasia in the context of lymphatic drainage defect.[6] Lymphatic stasis can also be responsible for abnormal circulation of immunocompetent cells in the affected area. The existence of an immune dysfunction in chronic lymphedema.

The histopathological diagnosis is difficult due to the double vascular and lymphatic component, hence the importance of immunohistochemical confirmation. The histologic findings were a tumor proliferation of irregular vascular channels anastomosed and bordered by a layer of endothelial cells well delimited by a basement membrane and surrounded by pericytes. Immunohistochemical study showed positive labeling for certain markers: CD-31, CD-34, antifactor VIII Ab, and KI67 at 50%. Treatment of obesity hypoventilation syndrome is based on surgery and radiotherapy. Early +++ amputation or broad ± local excision offers the best chance for long-term survival.[6],[7],[8] Radiation therapy has also demonstrated some effectiveness in the treatment of SST. Some authors propose it as a first line before surgery. Systemic chemotherapy is reserved for locally advanced nonoperable forms and metastatic forms. Given the aggressive nature of SST and the high risk of local and distant recurrences, long-term survival is poor with a median survival between 19 and 30 months after diagnosis.[8] It seems that survival is better in the case of nontumor origin of chronic lymphedema as is the case of our patient. The prevention and treatment of chronic lymphedema remains necessary and mandatory in the fight against this syndrome, which remains of poor prognosis despite its rarity.


  Conclusion Top


STS-type angiosarcoma is a rare, aggressive tumor that currently has a poor prognosis. Therefore, it is necessary to emphasize the importance of regular clinical monitoring in patients with chronic lymphedema by the various preventive measures and performing biopsies of suspicious lesions in order to be able to make an early diagnosis and good therapeutic management of cancer and improve survival.

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.



 
  References Top

1.
Stewart FW, Treves N. Lymphangiosarcoma in postmastectomy lymphedema; a report of six cases in elephantiasis chirurgica. Cancer 1948;1:64-81.  Back to cited text no. 1
    
2.
Morel M, Taïeb S, Penel N, Mortier L, Vanseymortier L, Robin YM, et al. Imaging of the most frequent superficial soft-tissue sarcomas. Skeletal Radiol 2011;40:271-84.  Back to cited text no. 2
    
3.
Wendt I, Kietzmann H, Schubert C, Kaiserling E. Progressive lymphangiokeratoma and angiosarcoma (Stewart et Treves syndrome in congenital lymphedema). Hautartzt 1988;39:155-60.  Back to cited text no. 3
    
4.
Laguerre B, Lefeuvre C, Kerbrat P, Hassel M. Syndrome de stewart-treves sur lymphèdème chronique post-traumatique. Bulletin du Cancer 1999;86:279-82.  Back to cited text no. 4
    
5.
Taghian A, de Vathaire F, Terrier P, Le M, Auquier A, Mouriesse H, et al. Long-term risk of sarcoma following radiation treatment for breast cancer. Int J Radiat Oncol Biol Phys 1991;21:361-7.  Back to cited text no. 5
    
6.
Petitjean A, Mathe E, Kato S, Ishioka C. Impact of mutant p53 functional properties on TP53 mutation patterns and tumor phenotype: Lessons from recent developments in the IARC TP53 database. Hum Mutat 2007;28:622-9.  Back to cited text no. 6
    
7.
Kazerooni E, Hessler C. CT appearance of angio-sarcoma associated with chronic Lymphedema. AJR 1991;156:543-4.  Back to cited text no. 7
    
8.
Chopra S, Ors F, Bergin D. MRI of angiosarcoma associated with chronic lymphoedema: Stewart Treves syndrome. Br J Radiol 2007;80:e310-3.  Back to cited text no. 8
    


    Figures

  [Figure 1], [Figure 2], [Figure 3]



 

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