Alveolar Soft Part Sarcoma Arising from the Kidney: Imaging and Clinical Features
- Affiliations
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- 1Department of Radiology, Seoul St. Mary's Hospital, College of Medicine, The Catholic University of Korea, Seoul 137-701, Korea. saim@catholic.ac.kr
- 2Department of Pediatrics, Seoul St. Mary's Hospital, College of Medicine, The Catholic University of Korea, Seoul 137-701, Korea.
- KMID: 2078649
- DOI: http://doi.org/10.3348/kjr.2014.15.3.381
Abstract
- Alveolar soft part sarcoma (ASPS) is an extremely rare malignant soft tissue sarcoma primarily affecting young patients. It usually occurs in the lower extremities, although it can occur in soft tissue anywhere in the body. However, to our knowledge, there has been no case of primary ASPS originating from the kidney in the literature. We herein present the imaging and clinical features of an ASPS which occurred in a 16-year-old male presented as a palpable mass in the left side of the abdomen.
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Figure
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Fig. 1 Imaging and pathologic findings of alveolar soft part sarcoma arising from kidney in 16-year-old boy. Two-dimensional (A) and Doppler (B) ultrasonographic images show heterogeneously hyperechoic mass in left kidney with internal and peritumoral dilated vascular structures. C, D. Contrast-enhanced CT scan of abdomen. Axial (C) and coronal (D) images show huge enhancing mass with internal necrotic foci, occupying almost whole left kidney. Numerous collateral vessels in addition to renal artery and vein show variceal changes. E. On chest CT with lung window setting, multiple different sized pulmonary nodules and diffuse interlobular septal thickening are noted in both lung fields, suggesting metastasis. F. PET-CT shows inhomogeneous FDG uptakes in large mass of left kidney (max SUV = 6.4) and in mass of small bowel mesentery (max SUV = 3.9). Multiple pulmonary nodules in both lungs and lymphadenopathies of left paraaortic, aortocaval and retrocrural regions, right supraclavicular region, mediastinum and in both perihilar regions showed FDG uptakes. FDG = fluorodeoxyglucose, max SUV = maximum standardized uptake value, PET = positron emission tomography. G. Epithelioid tumor cells arranged in nests contain abundant eosinophilic cytoplasm (H&E, × 200). H. Tumor cells exhibit prominent periodic acid-Schiff (PAS)-positive, rod-shaped crystals (arrow) in cytoplasm (PAS Stain with diastase, × 1000).
Reference
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