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Symptomatic Dysrhythmia Caused By a Posterior Mediastinal Angiomyolipoma

¹ Department of Radiology, University of Pennsylvania Medical Center, 1st Fl., Founders Bldg., 3400 Spruce St., Philadelphia, PA 19104-4283.
² Department of Surgery, University of Pennsylvania Medical Center, Philadelphia, PA 19104.
³ Department of Pathology and Laboratory Medicine, University of Pennsylvania Medical Center, Philadelphia, PA 19104.
⁴ Department of Medicine, University of Pennsylvania Medical Center, Philadelphia, PA 19104.

View larger version (105K): [in a new window]   Fig. 1A. —35-year-old woman with history of lymphangioleiomyomatosis and prior resection of right renal—retroperitoneal angiomyolipoma presented with intermittent palpitations and tachycardia. Axial proton density—weighted fast spin-echo image (TR/effective TE, 3529/18) at level of left atrium shows posterior mediastinal mass (M) that mildly compresses left atrium (A) and pulmonary veins (arrow), mildly displaces heart anteriorly, and partially encases descending thoracic aorta (asterisk).

View larger version (116K): [in a new window]   Fig. 1B. —35-year-old woman with history of lymphangioleiomyomatosis and prior resection of right renal—retroperitoneal angiomyolipoma presented with intermittent palpitations and tachycardia. Axial T1-weighted conventional spin-echo image (TR/TE, 577/14) at level of aortic hiatus shows that mass (M) extends from periceliac location in retroperitoneum posteriorly through diaphragmatic crura (large arrow) into retrocrural space and posterior mediastinum (small arrow). Mass is predominantly high in signal intensity but is hypointense relative to subcutaneous fat and retroperitoneal fat (F) with scattered low-signal-intensity internal septations.

View larger version (155K): [in a new window]   Fig. 1C. —35-year-old woman with history of lymphangioleiomyomatosis and prior resection of right renal—retroperitoneal angiomyolipoma presented with intermittent palpitations and tachycardia. Coronal T1-weighted conventional spin-echo images (577/15) show retroperitoneal (R) and posterior mediastinal (M) components of mass separated by diaphragmatic crus (black arrow). Mass extends superiorly to level of carina (C). Susceptibility artifact (white arrow) is seen from prior right nephrectomy.

View larger version (147K): [in a new window]   Fig. 1D. —35-year-old woman with history of lymphangioleiomyomatosis and prior resection of right renal—retroperitoneal angiomyolipoma presented with intermittent palpitations and tachycardia. Coronal T1-weighted conventional spin-echo images (577/15) show retroperitoneal (R) and posterior mediastinal (M) components of mass separated by diaphragmatic crus (black arrow). Mass extends superiorly to level of carina (C). Susceptibility artifact (white arrow) is seen from prior right nephrectomy.

View larger version (119K): [in a new window]   Fig. 2A. —Gross and histopathologic findings of excised fatty posterior mediastinal mass in 35-year-old woman. Photograph of sectioned gross specimen appears as oval well-circumscribed fatty mass (M) with homogenous shiny golden-yellow interior.

View larger version (186K): [in a new window]   Fig. 2B. —Gross and histopathologic findings of excised fatty posterior mediastinal mass in 35-year-old woman. Photomicrograph of histologic specimen shows mature adipocytes (A), plump spindle cells (arrow), and vessels (V). (H and E, x10)

View larger version (168K): [in a new window]   Fig. 2C. —Gross and histopathologic findings of excised fatty posterior mediastinal mass in 35-year-old woman. Photomicrographs of histologic specimens with immunohistochemical staining show positivity of spindle cells for smooth-muscle markers (arrows), confirming muscle differentiation. These findings are diagnostic of posterior mediastinal angiomyolipoma. (HHF-35 stain, [muscle common actin] x40 [C] and HMB-45, [human melanoma, black-45] x40 [D])

View larger version (106K): [in a new window]   Fig. 2D. —Gross and histopathologic findings of excised fatty posterior mediastinal mass in 35-year-old woman. Photomicrographs of histologic specimens with immunohistochemical staining show positivity of spindle cells for smooth-muscle markers (arrows), confirming muscle differentiation. These findings are diagnostic of posterior mediastinal angiomyolipoma. (HHF-35 stain, [muscle common actin] x40 [C] and HMB-45, [human melanoma, black-45] x40 [D])

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