Lymphangioleiomyomatosis

Doubková, Martina; Štefániková, Marianna; Čan, Vladimír; Merta, Zdeněk; Svoboda, Marek

doi:10.14735/amko2019367

Cited by 5 publications

(3 citation statements)

References 21 publications

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“…23 One link between TSC and development of spontaneous meningiomas that we identified within the literature occurred in patients with lymphangioleiomyomatosis (LAM), a cystic lung disease associated with progressive respiratory failure occurring predominantly in women of childbearing age; that effects 30% to 40% of patients with TSC. [24][25][26] LAM patients in general have an increased risk of meningiomas; however, our patient did not have this condition.…”

Section: Discussioncontrasting

confidence: 63%

Innumerable Meningiomas Arising in a Patient With Tuberous Sclerosis Complex Decades After Radiation Therapy

Gilani

Lee

Kleinschmidt‐DeMasters

2021

Pediatr Dev Pathol

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Meningioma is the most common radiation-induced brain neoplasm, usually occurring after a latency of 20 - 35 years, with multiplicity in 10% of cases. Radiation-induced meningiomas (RIMs) have not previously been reported in patients with tuberous sclerosis complex (TSC), unlike their well-known occurrence in other familial tumor predisposition syndrome patients. We report a TSC patient who developed numerous intracranial meningiomas twenty five year after radiation therapy for subependymal giant cell astrocytoma (SEGA). Autopsy examination showed innumerable, coalescent, benign, meningothelial meningiomas, WHO grade 1, ranging in size from 0.2 cm to 3.3 cm. Autopsy also showed small residual SEGA, radiation-induced cerebral vasculopathy, and classic TSC features including several small subependymal nodules (“candle gutterings”), white matter radial heterotopia, facial angiofibromas, dental enamel pitting, one ash leaf spot, and multiple hepatic and renal angiomyolipomas. Next-generation sequencing analysis utilizing a 500+ gene cancer panel demonstrated chromosomal loss involving the majority of chromosome 22, including the NF2 gene locus, as well as a truncating nonsense mutation in TSC1 p. R509*. While TSC patients rarely require radiation therapy, this striking case suggests that patients with TSC should be monitored closely if cranial therapeutic radiation is administered.

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Section: Discussioncontrasting

confidence: 63%

Innumerable Meningiomas Arising in a Patient With Tuberous Sclerosis Complex Decades After Radiation Therapy

Gilani

Lee

Kleinschmidt‐DeMasters

2021

Pediatr Dev Pathol

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“…Obstruction of the bronchioles creates air cysts and excessive distension of the cysts combined with destruction of the cyst wall or pneumothorax. Most LAMs are found to be due to recurrent pneumothorax of unknown etiology [1 , 3 , [6] , [7] , [8] .…”

Section: Introductionmentioning

confidence: 99%

Pulmonary lymphangioleiomyomatosis (LAM): A literature overview and case report

Cong

Anh

Duc

2022

Radiology Case Reports

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“…Lymphatic blockage can occur as a result of the enlargement of lymph nodes in the abdominal and pelvic regions. LAM is linked to the development of renal angiomyolipomas [4].…”

Section: Introductionmentioning

confidence: 99%

Pregnancy-Associated Events in Prognosis and Outcome of Lymphangioleiomyomatosis: A Single-Center Retrospective Analysis

Jingye,

Shihua,

Xinping

et al. 2024

Preprint

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Background: Lymphangiomyomatosis (LAM) is an uncommon condition that primarily impacts women in their reproductive years. Current guidelines discourage pregnancy behavior in women with LAM because to the belief that it raises the risk and does not result in favorable pregnancy outcomes. Pregnancy events have been linked to a higher likelihood of developing pneumothorax and chylothorax, as well as an acceleration in the deterioration of lung function. Currently, there is a deficiency of extensive clinical trials investigating the impact of pregnancy on LAM.Methods: The study conducted an integrative analysis by collecting data from the LAM patient registry at the First Hospital of Guangzhou Medical University in Guangzhou, China, as well as from LAM patient organizations in South China. Additionally, the study searched the MEDLINE, Scopus, Embase, and Cochrane Central databases for six relevant clinical articles using the keywords "lymphangiomatosis" and "pregnancy". Retrieve scholarly publications for systematic assessment.Results: The study had a total of 133 patients diagnosed with lymphangiomatosis. Out of the total number of patients, 91 individuals (68.4%) experienced irregularities in their menstrual cycles. A single patient was identified with lymphangioleiomyomatosis (LAM) after experiencing pneumothorax during pregnancy. Out of the 12 instances of pregnancy, LAM was identified before conception.One case of dyspnea, one case of renal abnormalities, and one case of tubal pregnancy were identified. Abnormal pregnancy events had place in 25 out of the 173 pregnancies (14.5%) that happened before the diagnosis of LAM. The analysis included six more studies, which reported an incidence of 13.47% for pneumothorax during pregnancy with LAM, 8.30% for dyspnea, and 3.32% for other respiratory abnormalities. Among the five studies that documented aberrant pregnancies, a total of 73 miscarriages (14%), 83 abortions (16%), and 12 other pregnancy outcomes (2%) were reported.Conclusions: The elevated incidence of monthly irregularities in individuals with lymphangiomatosis may be attributed to the use of sirolimus and the variable levels of estrogen in patients with lymphangiomatosis. Pregnancy significantly increases the likelihood of experiencing a sudden worsening of lymphangiomatosis-associated pulmonary disease (LAM). Pregnancy might cause a sudden worsening of LAM. LAM exhibits a substantial prevalence of unfavorable reactions during pregnancy, resulting in unfavorable pregnancy results. Consequently, it is advised against getting pregnant in individuals with LAM.

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Lymphangioleiomyomatosis

Cited by 5 publications

References 21 publications

Innumerable Meningiomas Arising in a Patient With Tuberous Sclerosis Complex Decades After Radiation Therapy

Innumerable Meningiomas Arising in a Patient With Tuberous Sclerosis Complex Decades After Radiation Therapy

Pulmonary lymphangioleiomyomatosis (LAM): A literature overview and case report

Pregnancy-Associated Events in Prognosis and Outcome of Lymphangioleiomyomatosis: A Single-Center Retrospective Analysis

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