Abstract
Lymphangiomyomatosis is an extremely rare benign disease characterized by extensive proliferation of smooth muscle cells within lymphatic vessels and lymph nodes. While 10-15% of all patients show concomitant renal angiomyolipomas, no association so far has been reported with renal cell carcinoma. We present a case with coincident lymphangiomyomatosis, renal cell carcinoma and renal angiomyolipoma. The possible underlying pathogenesis is discussed. Classifying lymphangiomyomatosis as a "forme fruste" of tuberous sclerosis allows postulation of a common pathogenesis for all three entities.
Publication types
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Case Reports
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English Abstract
MeSH terms
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Adrenalectomy
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Angiomyolipoma / diagnosis*
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Angiomyolipoma / pathology
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Angiomyolipoma / surgery
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Carcinoma, Renal Cell / diagnosis*
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Carcinoma, Renal Cell / pathology
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Carcinoma, Renal Cell / surgery
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Female
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Humans
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Kidney / pathology
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Kidney Neoplasms / diagnosis*
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Kidney Neoplasms / pathology
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Kidney Neoplasms / surgery
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Lymph Node Excision
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Lymphangioleiomyomatosis / diagnosis*
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Lymphangioleiomyomatosis / pathology
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Lymphangioleiomyomatosis / surgery
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Middle Aged
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Neoplasms, Multiple Primary / diagnosis*
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Neoplasms, Multiple Primary / pathology
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Neoplasms, Multiple Primary / surgery
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Nephrectomy
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Tomography, X-Ray Computed