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de Campos FPF, Ferreira CR, Simões AB, et al. Unilateral giant renal angiomyolipoma and pulmonary lymphangioleiomyomatosis. Autops Case Rep. 2013;3(4):53-62doi: 10.4322/acr.2013.040.
de Campos, F. P. F., Ferreira, C. R., Simões, A. B., de Alcântara, P. S. M., Martines, B. M., da Silva, A. F., Azzi-Nogueira, D., de Britto, L. R. G., Dufner-Almeida, L. G., & Haddad, L. A. (2013). Unilateral giant renal angiomyolipoma and pulmonary lymphangioleiomyomatosis. Autopsy & case reports, 3(4), 53-62. https://doi.org/10.4322/acr.2013.040
de Campos, Fernando Peixoto Ferraz, et al. "Unilateral giant renal angiomyolipoma and pulmonary lymphangioleiomyomatosis." Autopsy & case reports vol. 3,4 (2013): 53-62. doi: https://doi.org/10.4322/acr.2013.040
de Campos FPF, Ferreira CR, Simões AB, de Alcântara PSM, Martines BM, da Silva AF, Azzi-Nogueira D, de Britto LRG, Dufner-Almeida LG, Haddad LA. Unilateral giant renal angiomyolipoma and pulmonary lymphangioleiomyomatosis. Autops Case Rep. 2013 Dec 31;3(4):53-62. doi: 10.4322/acr.2013.040. eCollection 2013. PMID: 28584808; PMCID: PMC5453662.
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Autops Case Rep. 2013 Dec 31;3(4):53-62. doi: 10.4322/acr.2013.040. eCollection 2013.

Unilateral giant renal angiomyolipoma and pulmonary lymphangioleiomyomatosis.

Autopsy & case reports

Fernando Peixoto Ferraz de Campos, Cristiane Rúbia Ferreira, Angélica Braz Simões, Paulo Sergio Martins de Alcântara, Brenda Margatho Martines, Adriano Ferreira da Silva, Deborah Azzi-Nogueira, Luiz Roberto Giorgetti de Britto, Luiz Gustavo Dufner-Almeida, Luciana Amaral Haddad

Affiliations

  1. Department of Internal Medicine - Hospital Universitário - Universidade de São Paulo, São Paulo/SP - Brazil.
  2. Anatomic Pathology Service - Hospital Universitário - Universidade de São Paulo, São Paulo/SP - Brazil.
  3. Department of Surgery - Hospital Universitário - Universidade de São Paulo, São Paulo/SP - Brazil.
  4. Diagnostic Imaging Service - Hospital Universitário - Universidade de São Paulo, São Paulo/SP - Brazil.
  5. Department of Genetics and Evolutionary Biology - Instituto de Biociências - Universidade de São Paulo, São Paulo/SP - Brazil.
  6. Department of Physiology and Biophysics - Instituto de Ciências Biomédicas - Universidade de São Paulo, São Paulo/SP - Brazil.

PMID: 28584808 PMCID: PMC5453662 DOI: 10.4322/acr.2013.040

Abstract

Angiomyolipomas (AMLs) are mesenchymal neoplasms, named so because of the complex tissue composition represented by variable proportions of mature adipose tissue, smooth muscle cells, and dysmorphic blood vessels. Although AMLs may rise in different sites of the body, they are mostly observed in the kidney and liver. In the case of renal AMLs, they are described in two types: isolated AMLs and AMLs associated with tuberous sclerosis (TS). While most cases of AMLs are found incidentally during imaging examinations and are asymptomatic, others may reach huge proportions causing symptoms. Pulmonary lymphangioleiomyomatosis (LAM) is a rare benign disease characterized by cystic changes in the pulmonary parenchyma and smooth muscle proliferation, leading to a mixed picture of interstitial and obstructive disease. AML and LAM constitute major features of tuberous sclerosis complex (TSC), a multisystem autosomal dominant tumor-suppressor gene complex diagnosis. The authors report the case of a young female patient who presented a huge abdominal tumor, which at computed tomography (CT) show a fat predominance. The tumor displaced the right kidney and remaining abdominal viscera to the left. Chest CT also disclosed pulmonary lesions compatible with lymphangioleiomyomatosis. Because of sudden abdominal pain accompanied by a fall in the hemoglobin level, the patient underwent an urgent laparotomy. The excised tumor was shown to be a giant renal AML with signs of bleeding in its interior. The authors call attention to the diagnosis of AML and the huge proportions that the tumor can reach, as well as for ruling out the TSC diagnosis, once it may impose genetic counseling implications..

Keywords: Angiomyolipoma; Hemorrhage; Kidney Diseases; Lymphangioleiomyomatosis; Nephrectomy; Tuberous Sclerosis

Conflict of interest statement

Conflict of interest: None

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