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: 40 relevant articles (3 outcomes, 0 trials/studies)

Relationship Network

Disease Context: Research Results

Related Diseases

1.	Angiomyolipoma
2.	Chylothorax
3.	Lymphangioleiomyomatosis (Lymphangiomyomatosis)
4.	Neoplasms (Cancer)
5.	Perivascular Epithelioid Cell Neoplasms

Experts

1.	Moss, Joel: 15 articles (10/2015 - 02/2005)
2.	Taveira-DaSilva, Angelo M: 6 articles (10/2015 - 03/2010)
3.	Glasgow, Connie G: 5 articles (10/2015 - 05/2009)
4.	Pacheco-Rodriguez, Gustavo: 5 articles (03/2015 - 12/2009)
5.	Stylianou, Mario: 4 articles (10/2015 - 06/2011)
6.	Avila, Nilo A: 4 articles (04/2011 - 02/2005)
7.	Steagall, Wendy K: 3 articles (03/2015 - 12/2009)
8.	Cai, Xiong: 3 articles (01/2014 - 12/2009)
9.	El-Chemaly, Souheil: 3 articles (09/2012 - 12/2009)
10.	Dwyer, Andrew J: 3 articles (04/2011 - 02/2005)

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. " 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. " 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. " 12/01/2014 - "Sirolimus therapy stabilizes lung function and reduces the size of chylous effusions and lymphangioleiomyomas in patients with lymphangioleiomyomatosis. "
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.	Vascular Endothelial Growth Factor DIBA 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. " 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. "
4.	HormonesIBA 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. "
5.	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. "
6.	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."
7.	TamoxifenFDA LinkGeneric 09/01/2006 - "Retroperitoneal lymphangioleiomyoma mimicking ovarian tumor emerging after tamoxifen therapy."
8.	Progesterone Receptors (Progesterone Receptor)IBA 04/01/2010 - "Hamartomatous lymphangiomyoma is not expressed by markers for secondary lymphangiomyoma of lymphangioleiomyoma, including human-melanoma-black-45 and progesterone receptor. "
9.	Glycoproteins (Glycoprotein)IBA 08/01/1998 - "HMB-45, an antibody directed against a premelanosome glycoprotein, although used predominantly for the diagnosis of MM, has shown consistent staining in angiomyolipoma (AML), lymphangiomyoma/lymphangiomyomatosis (LAM), and clear cell sugar tumor (CCST), a group of tumors proposed to be related on the basis of their common perivascular epithelioid cells, which exhibit various degrees of smooth muscle differentiation, melanogenesis, and intracytoplasmic membrane bound granules. "
10.	Carbon MonoxideIBA 09/01/2000 - "The perivascular epithelioid cell family of tumors (PEComas), defined by their co-expression of melanocytic and muscle markers, includes angiomyolipoma, lymphangioleiomyoma, and clear cell "sugar" tumors of the lung, pancreas, and uterus. "

Therapies and Procedures

1.	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. "
2.	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. "
3.	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. "
4.	Nephrectomy 01/01/2007 - "Other findings assessed included noncalcified pulmonary nodules, pleural effusion, thoracic duct dilatation, hepatic and renal angiomyolipomas (AMLs), lymphangioleiomyoma (LALM), ascites, nephrectomy, and renal embolization. "