Journal of Nippon Medical School: Vol.67 No.5 page.311

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ArticleTitle Lymphangioleiomyomatosis (LAM) (A Review of Clinical and Morphological Features)

AuthorList Victor J. Ferrans¹, Zu-Xi Yu¹, William K. Nelson¹, Julio C. Valencia¹, Atsushi Tatsuguchi¹, Nilo A. Avila², William Riemenschneider¹, Kazuhiro Matsui¹, William D. Travis³ and Joel Moss⁴

Affiliation ¹Pathology Section, National Heart, Lung, and Blood Institute, NIH; ²Diagnostic Radiology, Warren Magnuson Clinical Center, NIH; ³Department of Pulmonary and Mediastinal Pathology, Armed Forces Institute of Pathology and ⁴Pulmonary and Critical Care Medicine Branch, National Heart, Lung, and Blood Institute, NIH, Bethesda, MD

Language EN

Volume 67

Issue 5

Year 2000

Page 311-329

Received July 17, 2000

Accepted July 21, 2000

Keywords lymphangioleiomyomatosis, lung, radiology, pathology, histochemistry

Abstract A review is presented of the clinical and morphological manifestations of lymphangioleiomyomatosis (LAM), a systemic disorder of unknown etiology that affects women. The clinical features include dyspnea, hemoptysis, recurrent pneumothorax, chylothorax, and chylous ascites. It is characterized by: 1) proliferation of abnormal smooth muscle cells (LAM cells) in pulmonary interstitium and along the axial lymphatics of the thorax and abdomen; 2) thin-walled pulmonary cysts, and 3) a high incidence of angiomyolipomas. The pulmonary cystic lesions have a characteristic appearance on high resolution computed tomography. The most specific method for diagnosing LAM is lung biopsy to demonstrate the presence of LAM cells, either by their characteristic histological appearance or by specific immunostaining with HMB-45 antibody. LAM cells differ in several important respects from the types of smooth muscle cells normally present in lung. Their reactivity with HMB-45 antibody is localized in stage I and stage II melanosomes. LAM cells show additional evidence of incomplete melanogenesis, and the significance of these observations remains to be determined. Two types of LAM cells are recognized: 1) small, spindle-shaped cells that are centrally located in the LAM nodules and are highly immunoreactive for matrix metalloproteinase-2 (MMP-2), its activating enzyme (MT-1-MMP), and proliferating cell nuclear antigen (PCNA), and 2) large, epithelioid cells that are distributed along the periphery of the nodules and show a high degree of immunoreactivity with HMB-45 antibody and with antibodies against estrogen and progesterone receptors. Types of treatment used for LAM include oophorectomy, administration of Lupron or progesterone and in very severe cases, pulmonary transplantation (following the onset of respiratory insufficiency, not relieved by O₂).

Correspondence to Victor J. Ferrans, M. D. , PhD, Chief, Pathology Section, Bldg. 10/2N240, National Heart, Lung, and Blood Institute, National Institutes of Health, 10 Center Dr. MSC-1518, Bethesda, MD 20892-1518. TEL: 301/402-0908, FAX: 301/402-4127
vf10e@nih.gov

ArticleTitle	Lymphangioleiomyomatosis (LAM) (A Review of Clinical and Morphological Features)
AuthorList	Victor J. Ferrans¹, Zu-Xi Yu¹, William K. Nelson¹, Julio C. Valencia¹, Atsushi Tatsuguchi¹, Nilo A. Avila², William Riemenschneider¹, Kazuhiro Matsui¹, William D. Travis³ and Joel Moss⁴
Affiliation	¹Pathology Section, National Heart, Lung, and Blood Institute, NIH; ²Diagnostic Radiology, Warren Magnuson Clinical Center, NIH; ³Department of Pulmonary and Mediastinal Pathology, Armed Forces Institute of Pathology and ⁴Pulmonary and Critical Care Medicine Branch, National Heart, Lung, and Blood Institute, NIH, Bethesda, MD
Language	EN
Volume	67
Issue	5
Year	2000
Page	311-329
Received	July 17, 2000
Accepted	July 21, 2000
Keywords	lymphangioleiomyomatosis, lung, radiology, pathology, histochemistry
Abstract	A review is presented of the clinical and morphological manifestations of lymphangioleiomyomatosis (LAM), a systemic disorder of unknown etiology that affects women. The clinical features include dyspnea, hemoptysis, recurrent pneumothorax, chylothorax, and chylous ascites. It is characterized by: 1) proliferation of abnormal smooth muscle cells (LAM cells) in pulmonary interstitium and along the axial lymphatics of the thorax and abdomen; 2) thin-walled pulmonary cysts, and 3) a high incidence of angiomyolipomas. The pulmonary cystic lesions have a characteristic appearance on high resolution computed tomography. The most specific method for diagnosing LAM is lung biopsy to demonstrate the presence of LAM cells, either by their characteristic histological appearance or by specific immunostaining with HMB-45 antibody. LAM cells differ in several important respects from the types of smooth muscle cells normally present in lung. Their reactivity with HMB-45 antibody is localized in stage I and stage II melanosomes. LAM cells show additional evidence of incomplete melanogenesis, and the significance of these observations remains to be determined. Two types of LAM cells are recognized: 1) small, spindle-shaped cells that are centrally located in the LAM nodules and are highly immunoreactive for matrix metalloproteinase-2 (MMP-2), its activating enzyme (MT-1-MMP), and proliferating cell nuclear antigen (PCNA), and 2) large, epithelioid cells that are distributed along the periphery of the nodules and show a high degree of immunoreactivity with HMB-45 antibody and with antibodies against estrogen and progesterone receptors. Types of treatment used for LAM include oophorectomy, administration of Lupron or progesterone and in very severe cases, pulmonary transplantation (following the onset of respiratory insufficiency, not relieved by O₂).
Correspondence to	Victor J. Ferrans, M. D. , PhD, Chief, Pathology Section, Bldg. 10/2N240, National Heart, Lung, and Blood Institute, National Institutes of Health, 10 Center Dr. MSC-1518, Bethesda, MD 20892-1518. TEL: 301/402-0908, FAX: 301/402-4127 vf10e@nih.gov