Sporadic lymphangioleiomyomatosis. Presentation of a case of pulmonary disease with retroperitoneal lymphangioleiomyomas

Linfangioleiomiomatosis esporádica. Presentación de un caso con enfermedad pulmonar y linfangioleiomiomas retroperitoneales

Published 2016-07-04

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Vol. 27 No. 2 (2015)
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Presentación de casos
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Sporadic lymphangioleiomyomatosis. Presentation of a case of pulmonary disease with retroperitoneal lymphangioleiomyomas.
rev. colomb. neumol. [Internet]. 2016 Jul. 4 [cited 2024 Nov. 24];27(2). Disponible en: https://doi.org/10.30789/rcneumologia.v27.n2.2015.31
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https://doi.org/10.30789/rcneumologia.v27.n2.2015.31
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Claudia Patricia Zuluaga Gómez, MD.
Hospital Universitario Mayor Mederi. Universidad del Rosario.
    Astrid Arroyave, MD.
    Universidad de Antioquia.
      Diana Carolina Moreno O., MD.
      Universidad del Rosario
        Juan Ricardo Lutz, MD.
        Hospital Universitario Mayor Mederi. Universidad del Rosario.
          Jorge Alberto Carrillo Bayona, MD.
          Hospital Universitario Mayor Méderi. Universidad del Rosario.
            Show authors biography

            Claudia Patricia Zuluaga Gómez, MD.,

            Médica Radióloga. Hospital Universitario Mayor Mederi. Universidad del Rosario.

            Astrid Arroyave, MD.,

            Fellow de Imagen Corporal. Universidad de Antioquia.

            Diana Carolina Moreno O., MD.,

            Residente de Medicina Interna. Universidad del Rosario.

            Juan Ricardo Lutz, MD.,

            Médico Neumólogo. Hospital Universitario Mayor Mederi. Universidad del Rosario.

            Jorge Alberto Carrillo Bayona, MD.,

            Médico Radiólogo. Hospital Universitario Mayor Méderi. Universidad del Rosario.

            Lymphangioleiomyomatosis (LAM) is a multisystemic disease associated with mutations of the TSC 2 gene. It has two presentations: sporadic (S-LAM) and associated with the tuberous sclerosis complex (TSC-LAM). LAM runs together with pulmonary parenchymal cystic disease. However, patients with S-LAM can develop mediastinal, retroperitoneal and renal alterations, with adenomegaly, lymphangioleiomyomas, and angiomyolipomas.

            We present the case of a female patient with S-LAM and cystic lung disease associated with retroperitoneal and pelvic lymphangioleiomyomas.

             

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