Lymphangiomyoma

A tumorlike condition characterized by SMOOTH MUSCLE and ENDOTHELIUM proliferation of LYMPHATIC VESSELS and LYMPH NODES in the MEDIASTINUM and retroperitoneum, also in the lung. It may be manifested by chylous PLEURAL EFFUSION and ASCITES.

Also Known As:

Networked: 57 relevant articles (5 outcomes, 0 trials/studies)

Relationship Network

Disease Context: Research Results

Related Diseases

1.	Angiomyolipoma
2.	Chylothorax
3.	Lymphangioleiomyomatosis (Lymphangiomyomatosis)
4.	Neoplasms (Cancer)
5.	Lymphadenopathy

Experts

1.	Moss, Joel: 18 articles (01/2018 - 02/2005)
2.	Taveira-DaSilva, Angelo M: 8 articles (01/2018 - 03/2010)
3.	Stylianou, Mario: 5 articles (01/2018 - 06/2011)
4.	Glasgow, Connie G: 5 articles (10/2015 - 05/2009)
5.	Pacheco-Rodriguez, Gustavo: 5 articles (03/2015 - 12/2009)
6.	Avila, Nilo A: 4 articles (04/2011 - 02/2005)
7.	Jones, Amanda M: 3 articles (01/2018 - 12/2014)
8.	Julien-Williams, Patricia: 3 articles (01/2018 - 12/2014)
9.	Steagall, Wendy K: 3 articles (03/2015 - 12/2009)
10.	Cai, Xiong: 3 articles (01/2014 - 12/2009)

Drugs and Biologics

Drugs and Important Biological Agents (IBA) related to Lymphangiomyoma:

1.	Sirolimus (Rapamycin)FDA Link 06/01/2015 - "All three patients had a remarkable response to sirolimus, with resolution of effusions, improvement in lung function and shrinking of abdominal lymphangioleiomyomas. " 01/01/2022 - "Rapid Shrinkage of Retroperitoneal Lymphangioleiomyoma after Sirolimus Initiation." 07/01/2019 - "Low-dose sirolimus in retroperitoneal lymphangioleiomyomas." 01/01/2017 - "Treatment with sirolimus led to the complete resolution of PLE within several months; additionally, marked shrinkage was observed in the lymphangioleiomyomas of both cases. " 05/01/2015 - "After treatment with sirolimus for 6 months, the remaining patient had a significant reduction in the volume of a massive retroperitoneal lymphangioleiomyoma. "
2.	lipoarabinomannan (LAM)IBA 10/01/2015 - "Conversely, a combination of food intake and decreased chyle flow through lymphatics partially obstructed by LAM cells may account for increases in lymphangioleiomyoma size. " 06/01/2014 - "A localized form of LAM can manifest as extrapulmonary lesions; this form is referred to as extrapulmonary lymphangioleiomyoma (E-LAM). " 12/01/2011 - "Here, we report two women with LAM whose abdominal lymphangioleiomyomas increased in size with hormonal changes of the menstrual cycle. " 12/01/2009 - "The axial lymphatics appear to be infiltrated by LAM cells, which may result in obstruction and formation of chyle-filled lymphangioleiomyomas. " 08/20/2009 - "Fourteen cases of LAM from Zhongshan Hospital, Fudan University are reviewed, twelve were confirmed by lung biopsy, one by retroperitoneal lymphangioleiomyoma resection, and one by autopsy. "
3.	MTOR InhibitorsIBA 05/01/2015 - "Our findings confirm that mTOR inhibitors are beneficial for patients with LAM, especially those with extrapulmonary manifestations, such as renal angiomyolipoma, lymphangioleiomyomas, and chylous effusions. " 01/01/2016 - "mTOR inhibitors, (e.g., sirolimus) are effective in stabilizing lung function, and reducing the size of chylous effusions, lymphangioleiomyomas, and angiomyolipomas. "
4.	trans-sodium crocetinate (crocetin)IBA 08/26/2021 - "We highlight a rare case of TSC-LAM and the first report of a mediastinum lymphangioleiomyoma associated with TSC-LAM." 09/01/2009 - "In one TSC-LAM patient with a biopsy-documented malignant uterine PEComa, robust uptake was noted in metastatic nodules in the lung but not in the LAM-involved lung parenchyma or the patient's massive abdominal lymphangioleiomyomas. " 01/01/2014 - "Lymphangioleiomyomatosis (LAM), sporadic or in women with tuberous sclerosis complex (TSC), is characterized by cystic lung destruction, lymphatic involvement (eg, chylous pleural effusions, lymphangioleiomyomas), and renal angiomyolipomas (AMLs). " 08/01/2009 - "The connection between angiomyolipoma (AML) of the kidney, clear cell sugar tumor (CCST) of the lung, and pulmonary lymphangioleiomyoma (LAM), was progressively discovered because of the histologic and immunophenotypic similarities between the three tumors and their frequent association with tuberous sclerosis complex (TSC). " 02/01/2013 - "The optimal cut-off point for definite LAM diagnosis was 850.7 pg/mL. In probable LAM group, the majority of patients (92.9%) had serum VEGF-D level over 850.7 pg/mL. The serum levels of VEGF-D in LAM patients with pulmonary cystic lesions only were lower than that in patients with any of evidences of AML, chylous effusions, adenopathy, lymphangioleiomyomas, or TSC, but higher than that in the health control. "
5.	Vascular Endothelial Growth Factor DIBA 09/01/2016 - "But serum VEGF-D levels in LAM patients with/without pneumothorax, AML, and lymphangioleiomyomas were not significantly changed. " 12/01/2009 - "Consistent with a lymphangiogenic process, levels of VEGF-D, a lymphangiogenic factor, were higher in sera of patients with LAM and lymphatic involvement (i.e., lymphangioleiomyoma, adenopathy) than in healthy volunteers or LAM patients with cystic disease limited to the lung. " 02/01/2013 - "The optimal cut-off point for definite LAM diagnosis was 850.7 pg/mL. In probable LAM group, the majority of patients (92.9%) had serum VEGF-D level over 850.7 pg/mL. The serum levels of VEGF-D in LAM patients with pulmonary cystic lesions only were lower than that in patients with any of evidences of AML, chylous effusions, adenopathy, lymphangioleiomyomas, or TSC, but higher than that in the health control. " 01/01/2018 - "Sirolimus reduces serum levels of vascular endothelial growth factor D (VEGF-D); the size of chylous effusions, lymphangioleiomyomas, and angiomyolipomas; and stabilizes lung function in patients with lymphangioleiomyomatosis (LAM). "
6.	SugarsIBA 05/01/2013 - "Included in this broad category are angiomyolipoma, clear-cell "sugar" tumours and lymphangioleiomyoma. " 05/01/2010 - "They constitute a spectrum of lesions in different organs including angiomyolipoma of the kidney and liver, sugar tumor of the lung, lymphangiomatosis, and lymphangiomyoma. " 02/01/2006 - "Perivascular Epithelioid Cell Tumour (PEComa) also known as myelomelanocytic tumours are uncommon, recently described mesenchymal tumours that include angiomyolipoma, clear cell "sugar" tumour of the lung, lymphangioleiomyoma and tumours composed predominantly of epithelioid cell morphology. " 06/01/2001 - "These cases further support the concept of a family of systemic HMB-45 positive tumors that include sugar tumors, angiomyolipoma of kidney or liver, lymphangiomyomas, and clear-cell myomelanocytic tumors of the falciform ligament/ligamentum teres."
7.	Hormones (Hormone)IBA 12/01/2011 - "This is the first description of abdominal lymphangioleiomyomas exhibiting hormone responsiveness in LAM. " 01/01/2006 - "The second case illustrates the hormone dependent growth of lymphangiomyoma and LAM development in young women. "
8.	Melanoma-Specific AntigensIBA 03/01/2015 - "Lymphangioleiomyomatosis (LAM) is characterized by the proliferation in the lung, axial lymphatics (eg, lymphangioleiomyomas), and kidney (eg, angiomyolipomas) of abnormal smooth muscle-like LAM cells, which express melanoma antigens such as Pmel17/gp100 and have dysfunctional tumor suppressor tuberous sclerosis complex (TSC) genes TSC2 or TSC1. "
9.	Vascular Endothelial Growth Factor Receptor-3 (Flt 4)IBA 04/01/2010 - "Lymphangioleiomyoma cells and lymphatic endothelial cells: expression of VEGFR-3 in lymphangioleiomyoma cell clusters."
10.	TamoxifenFDA LinkGeneric 09/01/2006 - "Retroperitoneal lymphangioleiomyoma mimicking ovarian tumor emerging after tamoxifen therapy."

Therapies and Procedures

1.	Therapeutics 05/01/2019 - "Highly effective sirolimus therapy for abdominal lymphangioleiomyoma." 06/21/2011 - "After beginning sirolimus therapy, 12 patients with chylous effusions and 11 patients with lymphangioleiomyomas experienced almost complete resolution of these conditions. " 06/21/2011 - "Lung function and the size of chylous effusions and lymphangioleiomyomas before and during sirolimus therapy. " 12/01/2014 - "Sirolimus therapy stabilizes lung function and reduces the size of chylous effusions and lymphangioleiomyomas in patients with lymphangioleiomyomatosis. " 09/01/2006 - "Retroperitoneal lymphangioleiomyoma mimicking ovarian tumor emerging after tamoxifen therapy."
2.	Aftercare (After-Treatment) 05/01/2015 - "After treatment with sirolimus for 6 months, the remaining patient had a significant reduction in the volume of a massive retroperitoneal lymphangioleiomyoma. " 05/01/2015 - "After treatment with sirolimus for 12 months, one patient presented resolution of severe chylothorax; one had a significant reduction in renal angiomyolipoma volume; and one showed significant regression of retroperitoneal lymphangioleiomyomas and abdominal lymph node enlargement. " 07/01/2019 - "Sirolimus therapy is currently recommended for LAM patients with abnormal or declining lung function with an adjusted dose to maintain a serum trough level of 5-15 ng/mL. We describe a significant reduction of retroperitoneal lymphangioleiomyomas after treatment with low-dose sirolimus therapy (serum trough level <5 ng/mL) in a patient with sporadic LAM."
3.	Hysterectomy 10/01/1977 - "A case report is presented of the incidental finding of a lymphangiomyoma in a 50-year-old woman who underwent total hysterectomy for squamous cell carcinoma of the cervix. " 10/01/1995 - "In both, the uterine lesions were grossly inapparent and were discovered during microscopic examination of hysterectomy specimens removed during surgical treatment for a primary ovarian adenocarcinoma with peritoneal and lymph node metastases in one case and a retroperitoneal lymphangiomyoma in the other. "
4.	Fat-Restricted Diet (Diet, Fat Restricted) 08/01/2016 - "A young woman received a diagnosis of abdominal, sporadic lymphangioleiomyomatosis (LAM) and multiple abdominal lymphangioleiomyomas and was referred for recurrent chylous ascites responding only to a fat-free diet. "
5.	Pleurodesis 04/01/2011 - "Sequelae of pleurodesis, acutely hemorrhagic renal angiomyolipomas, and lymphatic involvement with lymphangioleiomyomatosis including enlarged lymph nodes and lymphangioleiomyomas are common benign conditions seen in patients with lymphangioleiomyomatosis that may be misdiagnosed on CT for malignancy and may prompt unnecessary biopsy and surgery. "