British Journal of Radiology (2007) 80, e310-e313
© 2007 British Institute of Radiology
doi: 10.1259/bjr/19441948
MRI of angiosarcoma associated with chronic lymphoedema: Stewart–Treves syndrome
S Chopra, MD
F Ors, MD
and
D Bergin, MD
Thomas Jefferson University Hospital, Philadelphia, PA 19107, USA
Correspondence: Dr Diane Bergin, Thomas Jefferson University Hosptial, 111 South 10th Street, Philadelphia PA, USA. E-mail: diane.bergin{at}jefferson.edu
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Abstract
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Cutaneous angiosarcoma is a rare complication of a chronic lymphedematous extremity (Stewart-Treves syndrome). Early recognition of these aggressive tumours is important, as they have a high incidence of local recurrence and metastasis. This report describes the MRI appearance of Stewart-Treves syndrome in a patient with a background of lumpectomy, axillary node dissection and radiotherapy for breast carcinoma. MRI revealed a lobulated cutaneous mass of intermediate signal on T1 weighted imaging, and low signal on T2 weighted imaging, with diffuse enhancement following contrast.
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Introduction
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Cutaneous angiosarcoma is a lethal complication of a chronic lymphoedematous extremity. Stewart and Treves first described the entity of angiosarcoma in a chronically lymphoedematous extremity following mastectomy [1]. This entity is now recognized as Stewart–Treves syndrome (STS). Angiosarcoma is a rare dermal-based malignancy of the vascular endothelium arising in achronically oedematous extremity [2, 3]. Though the majority of reported cases have occurred in association with post-mastectomy lymphoedema, a few cases have been reported after chronic lymphoedema due to other causes such as congenital, post-traumatic and filarial lymphoedema [2].
Stewart and Treves and other authors [1, 2] have described these vascular sarcomas as lymphangiosarcomas, believing that they arose from chronically dilated lymphatic vessels. However, these vascular sarcomas appear to be more consistent with haemangiosarcomas than the lymphangiosarcomas [4]. The distinction between the two types is difficult and arbitrary, especially as the cell of origin in both is endothelial and therefore the term "angiosarcoma" is preferable to either of the more specific terms [4].
In this report, we describe the MRI appearance of cutaneous angiosarcoma in a patient with chronic lymphoedema of her arm following breast-conserving surgery, axillary lymph node dissection and radiotherapy. We also review the imaging literature regarding this rare entity.
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Case report
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A 60-year-old Caucasian woman underwent lumpectomy and axillary lymph node dissection on the left side for breast carcinoma. Post-operatively, the patient received radiotherapy. There was no history of any post-operative complication, infection or thrombosis. The patient subsequently developed lymphoedema of the ipsilateral upper limb a few months following surgery. Eight years after the surgery, the patient presented with a massively swollen left upper limb and a bleeding cutaneous ulcer. On clinical examination, the patient's left arm was swollen and had an ill-defined nodular area on the skin that had purplish discolouration and was ulcerated (Figure 1a
). Angiosarcoma was suspected. The larger lesion on the forearm was biopsied and histology demonstrated a tumour consisting of a mixture of spindle cells and sparse vessels deformed by endothelial cells on a background of dense fibrous stroma consistent with diagnosis of angiosarcoma (Figure 1b). Prior to surgery the patient was referred for MRI to assess the extent and depth of the lesion.
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Figure 1. A 60-year-old woman treated for breast carcinoma with chronic lymphoedema and cutaneous angiosarcoma of upper limb. (a) Ulcerated lesion on the dorsum of forearm (arrow) with multiple surrounding satellite lesions (arrowhead) on a background of indurated lymphoedema. (b) Haematoxylin–eosin-stained photomicrograph, original magnification x200, shows the tumour with sparse vessels (arrowhead) and diffuse fibrous stroma (arrow).
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MRI of the left upper extremity was performed using a 1.5 T MR unit (Sigma, General Electric Medical Systems, Milwaukee, WI) with an extremity coil (Medrad, Indianola, PA), 15 to 16 cm field of view with 5 mm slice thickness and 256 x 192 matrix. Sequences used were axial T1 (500/8; echo-train length, 8), axial T2 FSE (fast spin-echo) (7400/98: echo-train length, 8) with fat suppression, and two-dimensional (2D) gradient fat suppression T1 weighted images (300/1.6; slice thickness of 5 mm) before and after administrating 0.1 mmol kg–1 of body weight of gadopentetate dimeglumine (Magnevist; Schering, Berlin, Germany) intravenously. 2D gradient fat-suppressed T1 weighted images following contrast were also obtained in the sagittal and coronal planes.
MRI showed lymphoedema of the arm as a diffuse increased signal on T2 weighted images in the fascial planes, skin and subcutaneous fat of the arm with low T2 signal dermal-based nodular tumour (Figure 2a). The tumour was intermediate signal on T1 (Figure 2b) measuring 13 mm in depth along the volar aspect of the forearm. There was diffuse enhancement of the lesion following contrast (Figure 2c,d). This lesion did not invade the underlying muscle or bone. The extent of skin involvement by the tumour and presence of satellite nodules was determined using the low signal on T2 of the tumour relative to the high signal surrounding oedematous tissues. The tumour was also delineated by relative increased enhancement following contrast compared with adjacent subcutaneous fat and muscle. There was no evidence of metastatic disease on other imaging, including CT of the chest. The patient subsequently had an above-elbow upper-limb amputation without post-operative complications. Pathology confirmed that there was no tumour invasion of adjacent muscle or bone. Three months following surgery, the patient has had no local recurrence or metastases but will be routinely monitored.
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Figure 2. A 60-year-old woman with a nodular dermal-based mass of the arm and history of chronic lymphoedema. (a) Axial fast spin-echo (FSE) T2-weighted image with fat suppression shows increased T2 signal of the subcutaneous tissues consistent with lymphoedema (arrowheads) and low T2 signal dermal-based nodular tumour (arrow). (b) Coronal T1 weighted image shows intermediate signal dermal-based nodular tumour (arrow) of the forearm. (c) Two-dimensional (2D) gradient fat suppression T1 weighted pre-contrast image of the arm and (d) 2D gradient fat suppression T1 weighted image of post-intravenous gadolinium image shows diffuse increased enhancement of the mass (between arrowheads) compared with adjacent muscle and subcutaneous fat.
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Discussion
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Angiosarcoma is an aggressive dermal-based malignant tumour that can occur as a rare complication of chronic lymphoedema. The incidence of angiosarcoma is estimated to be 0.45% in patients living 5 years after radical mastectomy. Approximately 90% of reported cases of cutaneous angiosarcoma occur in the upper limb after mastectomy [4]. Although this tumour is not directly related to breast cancer or radiotherapy, the common factor is chronic lymphoedema, and therefore other causes of chronic lymphoedema can lead to the development of this vascular sarcoma [3, 5, 6]. The median time from mastectomy to the development of angiosarcoma is 10 years, ranging from 5 years to 27 years [5]. The time interval between breast surgery and diagnosis of tumour in our patient was 8 years. The survival of the patients with this type of angiosarcoma is poor, ranging from 19 months to 31 months [5, 7].
Typically, the tumour has a bluish slightly raised macular or polypoid appearance on a lymphoedematous extremity on clinical presentation [2, 4]. The primary site is in the skin of the arm. These lesions tend to grow, coalesce, ulcerate and spread proximally and distally to involve the entire arm. The disease tends to metastasize through the blood to distant organs, particularly the lungs [5]. The treatment of choice is amputation [6]. Radiation and chemotherapy may have some role when combined with radical surgery or in the treatment of metastatic disease [3]. Nonetheless, despite treatment, the overall prognosis remains poor.
The pathogenesis of this entity remains unclear. Stewart and Treves believed that a systemic carcinogen was involved in the aetiology [1]. Schreiber et al [2] postulated that local immunodeficiency was the causative factor, as the oedematous region responds in a manner similar to "immunologically privileged site" allowing sarcomatous degeneration to go unnoticed by the immune system. The histopathological appearance of this neoplasm varies greatly, depending on the degree of cellular differentiation, although, by definition, these tumours consist of vascular cavities lined with spindle-shaped endothelial cells containing large nuclei and prominent nucleoli [3, 5] on a background of fibrous stroma. A number of other conditions resembling cutaneous angiosarcoma should be differentiated, including Kaposi's sarcoma [2].
To date, there is little in the imaging literature regarding this rare complication of chronic lymphoedema. There are two case reports to date with pathological proof describing the MRI appearance of Stewart–Treves syndrome. Our investigations serve to support the findings of previous authors [7, 8]. MRI is well described for its utility in evaluating lymphoedema. Subfascial fluid accumulation is considered a sign of severe lymphoedema. The subcutaneous oedema on MRI has decreased signal intensity on T1 weighted images either isointense or hypointense to muscle and high signal intensity on T2 weighted images [9].
On MRI this dermal-based tumour is recognized by cutaneous thickening and nodularity isointense to muscle on T1 and by decreased signal on T2 weighted images in a lymphoedematous extremity. These lesions demonstrate avid enhancement following contrast. The low signal on T2 and avid enhancement on MR of these lesions distinguishes the tumour from background lymphoedematous tissues, which is of high signal on T2 and has reduced enhancement following contrast. The MRI signal characteristics that we describe in this report concur with previous MRI findings reported by Nakazona et al [7] and Schindera et al [8]. The specific MRI signal characteristic of this rare but potentially lethal tumour, as described in this and previous cases, may allow MRI to have an important role in early detection of this tumour with chronic lymphoedema [7, 8]. Early detection may help to improve patient prognosis.
For surgical planning and to optimize biopsy yield as in our patient, MRI can determine the extent of the tumour and the depth of invasion as well as identify satellite lesions. The low T2 signal of this lesion is unusual for a malignant tumour and probably reflects dense cellularity and prominent fibrous stroma as postulated by Nakazone et al [7].
Early recognition of suspicious dermal lesions on a lymphoedematous extremity, immediate biopsy and prompt treatment are important if the prognosis of this entity is to be improved. Roy et al [10] report that early diagnosis and treatment with radical ablative surgery may serve to improve prognosis of this aggressive disease.
Radiologists need to be aware of the characteristic features of this rare entity as MRI can play an important role in diagnosis, planning biopsy and ultimate management. Cammarato et al [11] suggest that high-frequency ultrasound may be useful for the morphological manifestation of skin lesions. Its specific role in diagnosis of this entity remains to be elucidated. Because of the specific MRI characteristic of this tumour and multiplanar imaging ability, MRI may potentially be used to screen patients with chronic lymphoedema for this rare but lethal complication.
Received for publication May 16, 2006.
Revision received August 24, 2006.
Accepted for publication September 4, 2006.
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References
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- Schindera ST, Streit M, Kaelin U, Steinbach L, Anderson SE. Stewart–Treves syndrome: MR imaging of a postmastectomy upper-limb chronic lymphedema with angiosarcoma. Skeletal Radiol 2005;34:156–60.[CrossRef][Medline]
- Case TC, Witte CL, Witte MH, Unger EC, Williams WH. Magnetic resonance imaging in human lymphedema: comparison with lymphangioscintigraphy. Mag Res Im 1992;10:549–58.[CrossRef]
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- Cammarota T, Pinto F, Magliaro A, Sarno A. Current uses of disgnostic high frequency US in dermatology. Eur J Radiol 1998;27(Suppl 2):S215–23.
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Abstract
Introduction
