Angiosarcoma arising in massive localized lymphedema
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CASE REPORT
Angiosarcoma arising in massive localized lymphedema Samantha Dyroff 1 & Lester J. Layfield 1 & Julia Crim 1 Received: 10 November 2019 / Revised: 30 December 2019 / Accepted: 2 January 2020 # ISS 2020
Abstract We report a case of a 70-year-old woman with a BMI of 58 who developed cellulitis refractory to treatment, within an area of massive localized lymphedema. Biopsy showed angiosarcoma. MRI showed multiple lobulated, low T1, high T2 masses within a background of prominent soft tissue septal stranding, dilated lymphatic channels, and skin thickening. CT also showed the mass well, within the background lymphedema. Massive localized lymphedema is increasing in prevalence due to the worsening obesity epidemic. Radiologists should be aware that the presence of a nodule within an area of massive localized lymphedema is suspicious for sarcoma. Keywords Angiosarcoma . Lymphedema . Massive localized lymphedema . Stewart–Treves syndrome . Adiposis dolorosa
Introduction Stewart–Treves syndrome is a rare malignant phenomenon in which angiosarcomas arise within the background of chronic lymphedema. This association was first recognized and most often seen in the setting of treated breast cancer [1–3]. Although most cases are seen in the upper arm after axillary node dissection, any area with chronic lymphedema is at risk of malignant change to sarcoma, perhaps due to the localized immunosuppression induced by the lymphedema [4]. In a recent case series of five patients presenting over a 25-year period, Shon et al. described the clinical and histologic findings of Stewart–Treves syndrome in the setting of the relatively newly described disorder, massive localized lymphedema (MLL) [5]. MLL is a disorder of localized lymphedema forming a pedunculated mass, most commonly in the upper medial thigh of obese, postmenopausal females [6–9]. Four cases of angiosarcomas arising in the presence of severe obesity, without the classification of MLL, but presumed to be secondary to lymphatic dysfunction have also been reported [5, 10–12]. An increasing number of reports of MLL, and increasing variety in locations of the entity, may reflect increasing rates of morbid obesity [13–15]. In the original case series of angiosarcoma arising from MLL, patients presented
* Julia Crim [email protected] 1
University of Missouri, 1 Hospital Dr, Columbia, MO 65212, USA
with signs highly concerning for malignancy, such as dramatic weight loss. Imaging findings have not been previously reported. Here, we will present the MRI, CT, and pathology findings of a case of angiosarcoma arising in the background of MLL, in a patient who did not have any systemic signs of malignancy. This case is being presented to show the imaging findings of angiosarcoma superimposed on MLL. Given the rising prevalence of MLL, radiologists should be aware of the possibility of sarcomatous degeneration in MLL. Nodules are not a feature of MLL, and therefore the presence of a nodule within a region of MLL should raise concern for sarcoma. It is hoped tha
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