The role of vascular endothelial growth factor-D in diagnosis of lymphangioleiomyomatosis (LAM).

Nov 2012

Xu KF, Zhang P, Tian X, Ma A, Li X, Zhou J, Zeng N, Gui YS, Guo Z, Feng R, Zhang W, Sun W, Cai B.

Source

Department of Respiratory Medicine, Peking Union Medical College Hospital, Peking Union Medical College & Chinese Academy of Medical Sciences, Beijing 100730, China. Electronic address: kaifeng.xu@gmail.com.

Abstract

BACKGROUND:

Definite diagnosis of lymphangioleiomyomatosis (LAM) depends on either transbronchial lung biopsy or video-assisted thoracic surgery, unless there is a history of chylothorax, kidney angiomyolipoma (AML), or tuberous sclerosis complex (TSC). Vascular endothelial growth factor-D (VEGF-D) was recently considered as a novel diagnostic marker for LAM. Herein, we evaluated diagnostic value of serum VEGF-D in LAM patients.

METHODS:

Serum samples were obtained from 78 cases of LAM (50 definite and 28 probable LAM based on European Respiratory Society guidelines), and 40 healthy female volunteers. VEGF-D was measured using enzyme-linked immunosorbant assay according to product instruction (R&D).

RESULTS:

Serum VEGF-D was significantly increased in definite LAM group, compared with that of health control (median: 3841.9 pg/mL vs 405.5 pg/mL respectively, p < 0.001). 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. In addition, VEGF-D levels were correlated with disease severity measured as LAM CT grade, and presentations of chylous effusions and/or lymphatic involvement (p < 0.05).

CONCLUSION:

Serum VEGF-D should be added to the current diagnosis algorithm to enhance definitive diagnosis for LAM.

Elsevier

Primary retroperitoneal lymphangioleiomyomatosis in a postmenopausal woman

Primary retroperitoneal lymphangioleiomyomatosis in a postmenopausal woman: a case report and review of the literature.
Int J Gynecol Cancer. 2007 Mar-Apr
Kebria M,
Black D,
Borelli C,
Modica I,
Hensley M,
Chi DS.
Gynecology Service, Department of Surgery, Memorial Sloan-Kettering Cancer Center, New York, New York.

Lymphangioleiomyomatosis (LAM) is a rare progressive disease of unknown etiology that typically affects women of childbearing age. It is characterized by an abnormal proliferation of smooth muscle cells causing gradual obstruction of small airways, frequently resulting in respiratory failure and death.

While LAM is predominantly a lung disorder, we report a case of retroperitoneal LAM in a patient who had no evidence of pulmonary involvement. A 59-year-old female presented with postmenopausal bleeding and no other complaints. She was found to have a low attenuation retroperitoneal mass on abdominal and pelvic computed tomography (CT) scan suspicious for lymphoma. CT-guided biopsy was nondiagnostic. Laparoscopic resection of some of the enlarged retroperitoneal lymph nodes confirmed the diagnosis of LAM. This case is an atypical presentation of LAM.

The disease typically presents in premenopausal women, with the initial site of involvement being the lungs and mediastinum. In rare cases, such as this of extrapulmonary LAM, patients typically present with a palpable abdominal mass, abdominal pain, or chylous ascites. As in our case, radiographic findings can mimic malignancies such as lymphoma. Laparoscopic lymph node biopsy is a valuable tool in these situations of diagnostic dilemma.

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