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Linfangioleiomiomatosi polmonare

Marcatori immunoistologici consigliati per la tipizzazione della lesione

Caso 1. Linfangioleiomiomatosi polmonare. 

Proliferazione interstiziale di cellule muscolari lisce che coesprimono AsMA, S100 e HMB45 

 

                 E.E.                cheratina 8/18              
  

HMB45                S100

 

Actina Muscolo liscio specifica (a-SMA). 

 

Intensa espressione nella proliferazione leiomiomatosa

References

 

Am J Surg Pathol 1993 Nov;17(11):1092-102

Transbronchial biopsy in lymphangiomyomatosis of the lung. HMB45 for diagnosis.

Bonetti F, Chiodera PL, Pea M, Martignoni G, Bosi F, Zamboni G, Mariuzzi GM

Istituto di Anatomia Patologica, Universita di Verona, Italia.

We have investigated the presence of smooth muscle cells with immunoreactivity for melanoma-related marker HMB45 in pulmonary lymphangiomyomatosis (PLAM). Of 75 lung specimens, including a variety of lesions (obtained with both transbronchial and open lung biopsies), only PLAM (six specimens from three patients) showed the presence of HMB45-positive cells. In addition, 20 specimens of normal lung were unreactive with this monoclonal antibody. It is concluded that the smooth muscle cells of PLAM regularly exhibit a peculiar phenotype, characterized by HMB45 immunoreactivity, distinct from other smooth muscle proliferations; and the histological diagnosis of PLAM can be made when only a transbronchial biopsy is available.

 

Histopathology 1993 May;22(5):445-55

Lymphangiomyomatosis and angiomyolipoma: closely related entities characterized by hamartomatous proliferation of HMB-45-positive smooth muscle.

Chan JK, Tsang WY, Pau MY, Tang MC, Pang SW, Fletcher CD

Institute of Pathology, Queen Elizabeth Hospital, London, UK.

Angiomyolipoma is a hamartomatous condition which can occur as a component of the tuberous sclerosis complex. Lymphangiomyomatosis, another hamartomatous lesion occurring predominantly in the lungs, has long been suspected to be related to angiomyolipoma and tuberous sclerosis because of occasional clinical associations. We undertook this study to provide further support for the close relationship between these two entities. Five cases of lymphangiomyomatosis and 20 case of angiomyolipoma were retrieved for histological review and immunohistochemical studies. The antibodies used were anti-muscle specific actin (HHF-35), anti-desmin (D33) and anti-melanoma (HMB-45). Lesions featuring smooth muscle proliferation were used as controls. The proliferated smooth muscle cells in both lymphangiomyomatosis and angiomyolipoma were much plumper and paler or even clear, when compared with the deeply eosinophilic cytoplasm of the normal spindly smooth muscle cells and those of leiomyomas. Their nuclei were round to oval and pale rather than elongated and dark. Cells with bizarre nuclei were commoner in angiomyolipoma (18/20 cases) than lymphangiomyomatosis (1/5). In 12 cases of angiomyolipoma there were foci indistinguishable from lymphangiomyomatosis, i.e. plump spindle cells arranged in short fascicles around ramifying endothelium-lined spaces. All five cases of lymphangiomyomatosis stained for muscle-specific actin, desmin and HMB-45. For angiomyolipomas, the positivity rates for these markers were: 20/20, 17/20 and 18/20, respectively, including one case that was negative for both desmin and HMB-45. The various smooth muscle proliferations and tumours selected as controls were uniformly HMB-45 negative. The distinctive cytological features, morphological overlap and immunophenotypic profile all support a close relationship between lymphangiomyomatosis and angiomyolipoma, which probably represent different morphological manifestations of hamartomatous proliferation of a peculiar form of HMB-45-positive smooth muscle.

 

Arch Pathol Lab Med 1994 Aug;118(8):846-9

Pulmonary lymphangioleiomyomatosis. Diagnosis based on results of transbronchial biopsy and immunohistochemical studies and correlation with high-resolution computed tomography findings.

Guinee DG Jr, Feuerstein I, Koss MN, Travis WD

Laboratory of Pathology, National Cancer Institute, National Institutes of Health, Bethesda, MD.

The diagnosis of lymphangioleiomyomatosis was established in a 35-year-old woman with hemoptysis, mild cough, and dyspnea based on histologic review of results of a transbronchial biopsy correlated with high-resolution computed tomographic scan findings. A chest x-ray film revealed diffuse interstitial lung disease, and a high-resolution computed tomographic scan showed diffuse cystic changes throughout both lungs. The transbronchial biopsy specimen revealed cystic changes and a patchy, sometimes nodular proliferation of smooth muscle that focally expanded the interstitium suggestive of lymphangioleiomyomatosis. The smooth-muscle nature of the cells was confirmed by positive immunohistochemical stains for actin and desmin; positive staining for HMB-45 was also observed. Although the diagnosis of lymphangioleiomyomatosis usually requires an open lung biopsy, this case shows that rarely, in the appropriate clinical setting, the diagnosis may be rendered based on results of a transbronchial biopsy in conjunction with findings from ancillary immunohistochemical studies and high-resolution computed tomography.