|Year : 2015 | Volume
| Issue : 5 | Page : 138-139
Pulmonary lymphangioleiomyomatosis - a case report
Chun-Jie Li1, Guo-Zheng Gao2, Meilin Xu2, Guan-Wei Fan3
1 Department of Emergency Medicine, Tianjin Chest Hospital, Tianjin, China
2 Department of Pathology, Tianjin Chest Hospital, Tianjin, China
3 Institute of Traditional Chinese Medicine Research, Tianjin State Key Laboratory of Modern Chinese Medicine, Tianjin University of Traditional Chinese Medicine, Tianjin 300193, China
|Date of Web Publication||31-Aug-2015|
Dr. Guan-Wei Fan
Institute of Traditional Chinese Medicine Research, Tianjin State Key Laboratory of Modern Chinese Medicine, Tianjin University of Traditional Chinese Medicine, Tianjin 300193
Prof. Meilin Xu
Department of Pathology, Tianjin Chest Hospital, Tianjin
Source of Support: None, Conflict of Interest: None
Pulmonary lymphangioleiomyomatosis (PLAM) is a rare disease, occurs in 16-68-year-old women, especially in women of childbearing age. High-resolution computed tomography would be useful for diagnosis of PLAM. Immunohistochemistry of smooth muscle actin (SMA) and HMB-45 smooth muscle cells was positive for smooth muscle cells. Progesterone receptor and estrogen receptor in some smooth muscle cells were positive for some smooth muscle cells. HMB-45-positive diagnosis of the disease is more important.
Keywords: Diagnosis, pathology, pulmonary lymphangioleiomyomatosis
|How to cite this article:|
Li CJ, Gao GZ, Xu M, Fan GW. Pulmonary lymphangioleiomyomatosis - a case report. J Can Res Ther 2015;11, Suppl S1:138-9
| > Case Report|| |
Pulmonary lymphangioleiomyomatosis (PLAM) is a rare disease, occurs in 16-68-year-old women, especially in women of childbearing age, with the incidence rate of about 0.029-1 part/million. Lung is most likely involved, with diffusing interstitial lung disease. The etiology and pathogenesis of PLAM are not exactly known.  Here on our department diagnostic report one case is as follows.
A female patient, aged 23, complained of the activities of shortness of breath after 10 more than a month, adding to more than 1-week. Computed tomography (CT) showed left outside the hospital limitations pneumothorax, diffuse lung thin-walled cystic changes, distribution of lesions in the whole lung. Then, conscious patient airway symptoms were progressively increased not be systematic check-ups. After admission, blood routine examination: White blood cell: 8.70 × 10 9 /L, red blood cell count: 5.54 × 10 12 /L, hemoglobin determination: Blood tumor markers were generally normal. Blood gas analysis showed: pH: 7.388, PaCO 2 : 36.3 mmHg, and PaO 2: 58.9 mmHg. Admission CT showed bilateral pulmonary multiple size ranging [Figure 1]. Pulmonary function suggests that moderate restrictive and obstructive ventilatory dysfunction, small airway function in severe obstruction, diffuse function severely reduced bronchodilator test (−), consider not except lymphatic leiomyoma disease. The patient agreed to the decision to be transferred to the department of thoracic surgery video-assisted thoracoscopic surgery lung biopsy and other further diagnosis and treatment.
|Figure 1: Computed tomography: Two lung visible diffuse size of the thin-walled gas chamber|
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General: Local lung wedge resection specimen size 5 cm × 1.2 cm × 0.5 cm, alveolated section.
High alveolar expansion, part of the integration, the alveolar wall gap widened spindle lymphatic smooth muscle cells around small vessels and small airway wall growth, some were tumor-like hyperplasia, the formation of small nodules, smooth muscle cell differentiation, mildly abnormal, and nonmitotic [Figure 2]. Immunohistochemistry showed: The expression of tumor cells: Vimentin [Figure 3], smooth muscle actin (SMA) [Figure 4] were positive, CD34 and CD31 are vascular positive. Pathology diagnosis: PLAM.
|Figure 2: Smooth muscle cells of the lung, smooth muscle cells nodular hyperplasia H and E, ×100|
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| > Discussion|| |
PLAM was first reported by Von Stossel in 1937, with pulmonary lymph node cell lung smooth muscle hyperplasia. It is a rare disease affect multiple systems,  which can be sporadic (sporadic LAM) or with tuberous sclerosis (tuberous sclerosis complex-associated LAM). Occurs in women of childbearing age, the patient condition may worsen during pregnancy, oral contraceptives or receiving estrogen therapy, and therefore concluded that this tumor and estrogen, but sometimes also the incidence of postmenopausal women.  The etiology and pathogenesis are unclear. WHO classification of soft-tissue tumors (2007 Edition) will be classified as PLAM perivascular epithelioid cell differentiation of the tumor. PLAM would have the histological and immunological phenotype unique performance perivascular epithelioid cells, the cells expressing melanoma cell markers HMB-45. High-resolution CT would be useful for diagnosis of PLAM. Immunohistochemistry of smooth muscle actin (SMA) and HMB-45 smooth muscle cells was positive for smooth muscle cells. Progesterone receptor and estrogen receptor in some smooth muscle cells were positive for some smooth muscle cells. HMB-45-positive diagnosis of the disease is more important.
Although this disease is a benign, it is often with a poor prognosis. Patients are generally within 5-10 years died of respiratory failure. Ovariectomy and progesterone for some patients could provide clinical benefits. Some patients need to have a lung transplant operation.
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Conflicts of interest
There are no conflicts of interest.
| > References|| |
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Ellender CM, Williams TJ, Gooi J, Snell GI, Whitford HM. Management of refractory chylothorax in pulmonary lymphangioleiomyomatosis. Respirol Case Rep 2015;3:72-4.
[Figure 1], [Figure 2], [Figure 3], [Figure 4]