Two Kinds of Cystic Lung Lesions with Pulmonary Lymphangioleiomyomatosis in a Male

Yamanaka, Sumitaka; Mizobuchi, Teruaki; Kurihara, Masatoshi

doi:10.5761/atcs.cr.16-00095

Cited by 5 publications

(3 citation statements)

References 7 publications

Supporting

Mentioning

Contrasting

Order By: Relevance

“…Recurrent pneumothorax is considered to be associated with PLAM disease with tuberous sclerosis (TSC-LAM). TSC-LAM is extremely rare; small bullae in early LAM may lead to missed diagnosis in some cases, so they are often diagnosed as spontaneous pneumothorax [ 15 ]. In high-resolution computed tomography (HRCT), a 37-year-old male patient had left pneumothorax and a large number of collapsed lungs with extensive parenchymal cysts in both lungs.…”

Section: Introductionmentioning

confidence: 99%

A Systematic Review of Lymphangioleiomyomatosis on Diagnosis and Molecular Mechanism

Dong

Jin

Wang³

et al. 2021

BioMed Research International

View full text Add to dashboard Cite

Objective. Lymphangioleiomyomatosis (LAM) is a rare low-grade metastatic tumor; however, LAM patients were always found in young age with difficulty for diagnosis. Our study is aimed at observing the clinical characteristics of patients with lymphangiomatosis, including the clinical manifestations, imaging findings, histopathological features, and immunophenotype. Methods. We did a systematic review on LAM/PLAM cases, especially on male cases, and collected the clinical features and molecular mechanisms of PLAM based on previous findings. Results. Diagnosis criteria were summarized by combining CT scans, MRI, immunohistochemistry results, and gene sequencing results for effectively distinguishing between PLAM and similar diseases. Moreover, our study illustrated the molecular mechanism of PLAM as well as the signaling pathway involved in the disease initials. In addition, a male case was reported with differential diagnosis on the clinical manifestations, microscopic features, immunophenotypes, and genotypes. Conclusion. Our review will definitely improve the understanding of diagnosis and treatment in PLAM cases.

show abstract

Section: Introductionmentioning

confidence: 99%

A Systematic Review of Lymphangioleiomyomatosis on Diagnosis and Molecular Mechanism

Dong

Jin

Wang³

et al. 2021

BioMed Research International

View full text Add to dashboard Cite

show abstract

“…Finally, 26 case reports and 36 participants were included in the pooled analysis. [7,13–15,19–40] As shown in Table 1, all screened patients were male. The median age of the participants was 34 years (IQR: 1–79).…”

Section: Resultsmentioning

confidence: 99%

Clinical features and outcomes of male patients with lymphangioleiomyomatosis: A review

Zhang

Wang

et al. 2022

Medicine

View full text Add to dashboard Cite

Background: Lymphangioleiomyomatosis (LAM) is a rare disease involving multiple systems, which is divided into sporadic LAM (S-LAM) and tuberous sclerosis complex-LAM, mostly affecting women who are in childbearing age stage. Data on male patients are limited and scattered. Therefore, it is necessary to conduct a systematic review to investigate the clinical features, diagnosis, treatment, and outcomes of LAM in male. Methods: We performed a literature review by searching for all the published reported cases of LAM in male during the past 35 years (April 1986–October 2021). Results: 36 male patients described in 26 references were included in this article. The median age of onset was 34 years (interquartile range: 1–79). The most common initial manifestations were cough, dyspnea, respite, and hemoptysis, with pulmonary complications such as pneumothorax and chylothorax. Five patients (13.9%) were asymptomatic at admission. Nearly half of the 36 male patients had thin-walled air-filled cysts that were visible throughout both lungs. Considering the abovementioned atypical clinical features, misdiagnosis was committed in 8 patients (22.2%). In addition, patients with tuberous sclerosis complex lymphangioleiomyomatosis often have no pulmonary manifestations at onset but present multiple extrapulmonary manifestations and have higher rates of renal angiomyolipomas than patients with S-LAM ( P < 0.01). Eventually, 4 patients with S-LAM eventually died. Conclusion: Physicians should increase the awareness of LAM in male. Early monitoring of various systems should be recommended to ensure early management and active follow-up. Tuberous sclerosis complex patients should immediately be tracked for the onset of LAM disease to improve prognosis.

show abstract

“…Clinical manifestations in men with LAM are similar to those in females, except the prevalence of symptomatic disease is much higher in women. [45][46][47][48][49][50][51][52] Diagnostic Evaluation…”

Section: Lam In Malesmentioning

confidence: 99%

Lymphangioleiomyomatosis

Liu

et al. 2020

Semin Respir Crit Care Med

View full text Add to dashboard Cite

Lymphangioleiomyomatosis (LAM) is a slow albeit progressive rare neoplastic disease featured with diffuse thin-walled cysts in lungs and angiomyolipomas in kidneys. LAM affects almost exclusively women and has one of the strongest gender predispositions of any extragenital human disease. Two forms of LAM present clinically, sporadic (S-LAM) and tuberous sclerosis complex-associated (TSC-LAM). TSC is an autosomal dominant genetic multisystems neoplastic disease. A high prevalence of LAM can be detected in adult female TSC patients. Tremendous progress has been made in our understanding and management of this rare disease. Both LAM and TSC are TSC2 or TSC1 mutated diseases that result in overactivation of the mechanistic target of rapamycin (mTOR) pathway. Sirolimus, an mTOR inhibitor, has been approved for LAM treatment in the United States and many other countries. Therapies targeting female sex hormones have shown preclinical efficacy in animal and cell culture-based experiments, but have not been properly investigated clinically. In this review, we summarize current recommendations in the diagnosis and treatment of LAM.

show abstract

Two Kinds of Cystic Lung Lesions with Pulmonary Lymphangioleiomyomatosis in a Male

Cited by 5 publications

References 7 publications

A Systematic Review of Lymphangioleiomyomatosis on Diagnosis and Molecular Mechanism

A Systematic Review of Lymphangioleiomyomatosis on Diagnosis and Molecular Mechanism

Clinical features and outcomes of male patients with lymphangioleiomyomatosis: A review

Lymphangioleiomyomatosis

Contact Info

Product

Resources

About