Association of D2-40 and MMP-1 expression with cyst formation in lung metastatic lesions of cutaneous angiosarcoma on the scalp: immunohistochemical analysis of 23 autopsy cases

• Published in: Human pathology 2013-12, Vol.44 (12), p.2751-2759
• Main Authors: Masuzawa, Mamiko, MD, Mikami, Tetuo, MD, PhD, Numata, Yoshiko, Tokuyama, Wataru, MD, PhD, Masuzawa, Mikio, MD, PhD, Murakumo, Yoshiki, MD, PhD, Okayasu, Isao, MD, PhD, Katsuoka, Kensei, MD, PhD
• Format: Article
• Language: English
• Subjects: Aged; Aged, 80 and over; Angiosarcoma; Antibodies, Monoclonal, Murine-Derived - metabolism; Cell Line, Tumor; Chemotherapy; Cysts; Cysts - complications; Cysts - metabolism; Cysts - pathology; Endothelium; Enzymes; Female; Head and Neck Neoplasms - complications; Head and Neck Neoplasms - metabolism; Head and Neck Neoplasms - pathology; Hemangiosarcoma - complications; Hemangiosarcoma - metabolism; Hemangiosarcoma - secondary; Humans; Lung - metabolism; Lung - pathology; Lung metastasis; Lung Neoplasms - complications; Lung Neoplasms - metabolism; Lung Neoplasms - secondary; Male; Matrix Metalloproteinase 1 - metabolism; Matrix metalloproteinase-1; Middle Aged; Pathology; Podoplanin; Proteins; Rodents; Scalp - metabolism; Scalp - pathology; Skin Neoplasms - complications; Skin Neoplasms - metabolism; Skin Neoplasms - pathology; Thin-walled cyst
• ISSN: 0046-8177; 1532-8392
• DOI: 10.1016/j.humpath.2013.07.022

Summary Cutaneous angiosarcoma of the scalp can rapidly develop into pulmonary metastasis. The pulmonary metastatic lesions display a unique appearance, so-called thin-walled cysts, which cause a fatal relapsed pneumothorax by rupturing. We analyzed 23 autopsy cases of angiosarcoma with pulmonary metastasis to elucidate the mechanism of the thin-walled cyst development. Of the 23 cases of cutaneous angiosarcoma of the scalp with pulmonary metastasis, radiological examination revealed pulmonary metastatic lesions as thin-walled cysts (39%), nodules (39%), mixed cysts and nodules (13%), and ground-glass opacity (9%). All the cases but one with cystic metastases were complicated by pneumothorax. The cystic lesions were accompanied by podoplanin (D2-40)–positive tumor cells in the luminal surface of the cysts. In both primary cutaneous lesions and pulmonary metastatic lesions, the D2-40 expression was positive for angiosarcoma cells in 100% and 92% of the cases, respectively. While the estrogen-regulated gene (ERG) expression was also positive for most of the primary and metastatic pulmonary angiosarcomas, D2-40 was a more useful marker to differentiate tumor cells from the background than was the ERG expression of the vascular endothelium. Matrix metalloproteinase-1 (MMP-1) expression was also predominant in primary lesions (95%) and pulmonary metastatic lesions (82.6%). Proteinases, like MMP-1, might be associated with a developing thin-walled cyst, although there were no differences in the MMP-1 expression in either the cystic or nodular metastasis. Two extremely aggressive cases showed cystic metastasis with central necrosis that was not observed in other cases. These results suggest a pathogenesis of thin-walled cysts in some progressive cases.