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Case Reports
. 2023 Nov 12;13(11):1598.
doi: 10.3390/jpm13111598.

Lymphangioleiomyomatosis with Tuberous Sclerosis Complex-A Case Study

Aleksandra Marciniak  1 , Jolanta Nawrocka-Rutkowska  1 , Agnieszka Brodowska  1 , Andrzej Starczewski  1 , Iwona Szydłowska  1
Affiliations
Case Reports

Lymphangioleiomyomatosis with Tuberous Sclerosis Complex-A Case Study

Aleksandra Marciniak et al. J Pers Med. .

Abstract

Lymphangioleiomyomatosis (LAM) is characterized by lung cysts that cause lung deterioration, changes in the lymphatic system, and tumors in the kidneys. It mainly affects women of reproductive age and is a progressive disease. LAM can occur as an isolated disease or coexist with tuberous sclerosis (TSC). The source of LAM cells is unknown. Patients with confirmed LAM should be treated with an mTOR inhibitor, sirolimus, or everolimus. We present a case of LAM with TSC in a patient whose symptoms, including those in the lymph nodes and chyaloperitoneum, mainly concern the abdominal cavity.

Keywords: benign metastasizing leiomyomatosis; lymphangioleiomyomatosis; tuberous sclerosis complex.

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Conflict of interest statement

The authors declare no conflict of interest.

Figures

Figure 1
Figure 1
CT of the lungs in presented case.
See this image and copyright information in PMC

References

    1. Prizant H., Hammes S. Minireview: Lymphangioleiomyomatosis (LAM): The “Other” Steroid-Sensitive Cancer. Endocrinology. 2016;157:3374–3383. doi: 10.1210/en.2016-1395. - DOI - PMC - PubMed
    1. McCarthy C., Gupta N., Johnson S.R., Yu J.J., McCormack F.X. Lymphangioleiomyomatosis: Pathogenesis, clinical features, diagnosis, and management. Lancet Respir. Med. 2021;9:1313–1327. doi: 10.1016/S2213-2600(21)00228-9. - DOI - PubMed
    1. Esposito A.J., Imani J., Shrestha S., Bagwe S., Lamattina A.M., Vivero M., Goldberg H.J., Rosas I.O., Henske E.P., El-Chemaly S.Y. Lymphangioleiomyomatosis: Circulating Levels of FGF23 and pulmonary diffusion. J. Bras. Pneumol. 2023;49:e20220356. doi: 10.36416/1806-3756/e20220356. - DOI - PMC - PubMed
    1. Shah J.M., Patel J.T., Shah H., Dadigiri H., Alla A., Cheriyath P. The epidemiology and clinical features of Lymphangioleiomyomatosis (LAM): A descriptive study of 33 case reports. Cureus. 2023;15:e43513. doi: 10.7759/cureus.43513. - DOI - PMC - PubMed
    1. Ağaçkiran Y., Ertürk A., Yeşıller F.I., Hoca N.T., Ustün L.N., Capan N. Pulmonary lymphangioleiomyomatosis: A rare case. Turk. Patoloji Derg. 2014;30:233–236. - PubMed

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