Lymphangioleiomyomatosis in a Male

Wakida, Kazuhiro; Watanabe, Yui; Kumasaka, Takashi; Seyama, Kuniaki; Mitani, Keiko; Hiraki, Takao; Kamimura, Go; Nagata, Toshiyuki; Nakamura, Yoshihiro; Sato, Masami

doi:10.1016/j.athoracsur.2014.11.069

Cited by 13 publications

(4 citation statements)

References 7 publications

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“…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

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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.

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Section: Resultsmentioning

confidence: 99%

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

Zhang

Wang

et al. 2022

Medicine

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“…7) Most of the reported cases of LAM in male including ours were early stage with mild symptoms and their cystic lesions were small. [4][5][6] Cyst size is significantly associated with pneumothorax in LAM. Patients with cyst size of more than 0.5 cm are more likely to have pneumothorax than patients with those of less than 0.5 cm.…”

Section: Discussionmentioning

confidence: 99%

“…Pulmonary LAM in men is extremely rare, and there have been reported only five cases in the past. [2][3][4][5][6] Clinical manifestations in LAM are pneumothorax, dyspnea on effort, hemosputum, chylothorax, and abdominal effusion. Among them, pneumothorax tends to show frequent recurrences.…”

Section: Discussionmentioning

confidence: 99%

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

Yamanaka

Mizobuchi

Kurihara

2017

ATCS

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A 34-year-old male with frequent recurrence of right pneumothorax was admitted to our hospital. He was a current smoker and outwardly male without genital aplasia. He was diagnosed as tuberous sclerosis complex (TSC) at 2 year-old and underwent transcatheter arterial embolization for right renal hemorrhage due to renal tumor 2 years ago. Chest Computed Tomography showed that he had multiple tiny round cystic lesions with thin wall in both lungs. The recurrent pneumothorax was expected to be associated with TSCLymphangioleiomyomatosis (LAM). Video-assisted thoracic surgery was successfully performed. The operative and histological findings revealed that the bullae were classified into two groups; emphysematous bullae and bullae due to LAM. His postoperative course was uneventful. TSC-LAM is extremely rare, but in some cases the clinical recognition might be escaped due to subtle findings of bullae in early LAM, resulting in diagnosis as spontaneous pneumothorax.

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“…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

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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.

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Lymphangioleiomyomatosis in a Male

Cited by 13 publications

References 7 publications

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

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

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

Lymphangioleiomyomatosis

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