Pneumonectomy of Primary Pulmonary Angiosarcoma with Malignant Effusion and Intrapleural Hypotonic Hyperthermic Chemotherapy: Case Report and Review of the Literature

Kunihide  Nakamura; Katsuya  Kawagoe; Eiichi  Chosa; Masaki  Tomita; Takanori  Ayabe

doi:10.4236/jct.2015.62025

Takanori Ayabe^*, Masaki Tomita, Eiichi Chosa, Katsuya Kawagoe, Kunihide Nakamura

Abstract: Background: Primary pulmonary angiosarcoma is extremely rare and the prognosis is very poor. We report a combination therapy of pneumonectomy and intrapleural hypotonic hyperthermic chemotherapy (IPHHC). Case report: A 48-year-old male with exertional dyspnea was found to have a left massive pleural effusion. Bronchoscopic examination displayed endobronchial stenosis of the left lower bronchus B8,9,10 and diagnosed with pulmonary angiosarcoma. Chest computed-tomographic scanning revealed a 5-cm mass in the left inferior lobe, which invaded the left upperbronchus. Intrapleural dissemination and malignant pleural effusion were also suspected (cT3N1M1a, c-stage IV). As a palliative initial therapy, we performed a pneumonectomy. On the 7th postoperative day, under general anesthesia, we performed video-assisted IPHHC (43°C, 60 min, 200 mg/m2 of CDDP). After the IPHHC, there was no major adverse event (more than Grade 3) during the postoperative course. Histological examination of the resected specimen revealed a highly-cellular growth of atypical spindle cells with a storiform pattern. By immunohistochemical testing, the tumor cells stained positive for markers including CD31 and the factor VIII related antigen, and the diagnosis of pulmonary angiosarcoma was made. No adjuvant chemoradiotherapy was given, and the postoperative clinical course was uneventful. Although there had been a recurrence in the chest wall, the patient eventually died twenty-one months later. Conclusion: As a new therapeutic option, we performed IPHHC after the pneumonectomy for an advanced pulmonary angiosarcoma with malignant pleural effusion. Although the patient had a recurrence in the chest wall, he had a reasonable postoperative outcome, that is, he returned to a work, retained a good quality of life and had a longer survival in spite of the poor prognosis of the pulmonary angiosarcoma.

Keywords: Pneumonectomy, Intrapleural, Hypotonic, Hyperthermic, Chemotherapy, Pulmonary Angiosarcoma

Cite this paper: Ayabe, T. , Tomita, M. , Chosa, E. , Kawagoe, K. and Nakamura, K. (2015) Pneumonectomy of Primary Pulmonary Angiosarcoma with Malignant Effusion and Intrapleural Hypotonic Hyperthermic Chemotherapy: Case Report and Review of the Literature. Journal of Cancer Therapy, 6, 227-236. doi: 10.4236/jct.2015.62025.

References

[1] Patel, A.M. and Ryu, J.H. (1993) Angiosarcoma in the Lung. Chest, 103, 1531-1535.
http://dx.doi.org/10.1378/chest.103.5.1531

[2] Chen, Y.B., Guo, L.C., Yang, L., et al. (2010) Angiosarcoma of the Lung: 2 Cases Report and Literature Reviewed. Lung Cancer, 70, 352-356. http://dx.doi.org/10.1016/j.lungcan.2010.09.002

[3] Matsuzaki, Y., Shibata, K., Yoshioka, M., Inoue, M., Sekiya, R., Onitsuka, T., Iwamoto, I. and Koga, Y. (1995) Intra-pleural Perfusion Hyperthermo-Chemotherapy for Malignant Pleural Dissemination and Effusion. Annals of Thoracic Surgery, 59, 127-131. http://dx.doi.org/10.1016/0003-4975(94)00614-D

[4] Matsuzaki, Y., Edagawa, M., Shimizu, T., Hara, M., Tomita, M., Ayabe, T. and Onitsuka, T. (2004) Intrapleural Hyperthermic Perfusion with Chemotherapy Increases Apoptosis in Malignant Pleuritis. Annals of Thoracic Surgery, 78, 1769-1772. http://dx.doi.org/10.1016/j.athoracsur.2004.05.025

[5] Ichinose, Y., Tsuchiya, R., Koike, T., Yasumitsu, T., Nakamura, K., Tada, H., Yoshimura, H., Mitsudomi, T., Nakagawa, K., Yokoi, K. and Kato, H. (2002) A Prematurely Terminated Phase III Trial of Intraoperative Intrapleural Hypotonic Cisplatin Treatment in Patients with Resected Non-Small Cell Lung Cancer with Positive Pleural Lavage Cytology: The Incidence of Carcinomatous Pleuritis after Surgical Intervention. The Journal of Thoracic and Cardiovascular Surgery, 123, 695-699.
http://dx.doi.org/10.1067/mtc.2002.120712

[6] Ichinose, Y., Hara, N., Ohta, M., Asoh, H., Yano, T., Maeda, K. and Yagawa, K. (1993) Hypotonic Cisplatin Treatment for Carcinomatous Pleuritis Found at Thoracotomy in Patients with Lung Cancer. In Vitro Experiments and Preliminary Clinical Results. Journal of Thoracicand Cardiovascular Surgery, 105, 1041-1046.

[7] Kakegawa, S., Kawashima, O., Ibe, T., Ujita, M., Iwashina, M., Nakano, T. and Shimizu, K. (2012) A Case of Primary Angiosarcoma of the Lung Presenting as a Hemorrhagic Bronchial Tumor. Annals of Thoracic and Cardiovascular Surgery, 18, 347-351. http://dx.doi.org/10.5761/atcs.cr.11.01716

[8] Obeso Carillo, G.A., García Fontán, E.M., Ca?izares Carretero, M.á. and Pérez Pedrosa, A. (2013) Primary Pulmonary Angiosarcoma, an Exceptional Neoplasm with a Poor Prognosis: Reports of Two Cases and Review of the Literature. General Thoracic and Cardiovascular Surgery, 61, 643-647.
http://dx.doi.org/10.1007/s11748-012-0200-6

[9] Case 40191 (1954) Case Records of the Massachusetts General Hospital: Weekly Clinicopathological Exercises. New England Journal of Medicine, 250, 837-841.
http://dx.doi.org/10.1056/NEJM195405132501909

[10] Tralka, G.A. and Katz, S. (1963) Hemangioendothelioma of the Lung. American Review of Respiratory Disease, 87, 107-115.

[11] Spragg, R.G., Wolf, P.L., Haghighi, P., Abraham, J.L. and Astarita, R.W. (1983) Angiosarcoma of the Lung with Fatal Pulmonary Hemorrhage. The American Journal of Medicine, 74, 1072-1076.
http://dx.doi.org/10.1016/0002-9343(83)90821-5

[12] Ott, R.A., Eugene, J., Kollin, J., Kanas, R.J., Conston, D.E. and Chi, J.C.H. (1987) Primary Pulmonary Angiosarcoma Associated with Multiple Synchronous Neoplasms. Journal of Surgical Oncology, 35, 269-276. http://dx.doi.org/10.1002/jso.2930350413

[13] Palvio, D.H., Paulsen, S.M. and Henneberg, E.W. (1987) Primary Angiosarcoma of the Lung Presenting as Intractable Hemoptysis. The Thoracic and Cardiovascular Surgeon, 35, 105-107.
http://dx.doi.org/10.1055/s-2007-1020207

[14] Segal, S.L., Lenchner, G.S., Cichelli, A.V., Promisloff, R.A., Hofman, W.I. and Baiocchi, G.A. (1988) Angiosarcoma Presenting as Diffuse Alveolar Hemorrhage. Chest, 94, 214-216.
http://dx.doi.org/10.1378/chest.94.1.214

[15] Sheppard, M.N., Hansell, D.M., Du Bois, R.M. and Nicholson, A.G. (1997) Primary Epithelioid Angiosarcoma of the Lung Presenting as Pulmonary Hemorrhage. Human Pathology, 28, 383-385.
http://dx.doi.org/10.1016/S0046-8177(97)90140-4

[16] Junge, K., Toens, C., Peiper, C., Hermanns, B. and Schumpelick, V. (2001) Primary Angiosarcoma of the Lung. Der Chirurg, 72, 969-972. http://dx.doi.org/10.1007/s001040170098

[17] Atasoy, C., Fitoz, S., Yigit, H., Atasoy, P., Erden, I. and Akyar, S. (2001) Radiographic, CT, and MRI Findings in Primary Pulmonary Angiosarcoma. Clinical Imaging, 25, 337-340.
http://dx.doi.org/10.1016/S0899-7071(01)00302-3

[18] Kojima, K., Okamoto, I., Ushijima, S., Yoshinaga, T., Kitaoka, M., Suga, M. and Sasaki, Y. (2003) Successful Treatment of Primary Pulmonary Angiosarcoma. Chest, 124, 2397-2400.
http://dx.doi.org/10.1378/chest.124.6.2397

[19] Maglaras, G.C., Katsenos, S., Kakadelis, J., Katsanos, C., Metafratzi, Z., Stefanou, D.G., Vassiliou, M.P. and Constantopoulos, S.H. (2004) Primary Angiosarcoma of the Lung and Pleura. Monaldi Archives for Chest Disease, 61, 234-236.

[20] Pandit, S.A., Fiedler, P.N. and Westcott, J.L. (2005) Primary Angiosarcoma of the Lung. Annals of Diagnostic Pathology, 9, 302-304. http://dx.doi.org/10.1016/j.anndiagpath.2005.04.001

[21] Corpa-Rodríguez, M.E., Mayoralas-Alises, S., García-Sánchez, J., Gil-Alonso, J.L., Díaz-Agero, P. and Casillas-Pajuelo, M. (2005) Postoperative Course in 7 Cases of Primary Sarcoma of the Lung. Archivos de Bronconeumología, 41, 634-637. http://dx.doi.org/10.1157/13081253

[22] Bouhaouala, M.H., Charfi, M.R., Fajraoui, N., Bouaziz, N., Boukhris, A. and Kilani, T. (2005) Angiosarcome Pulmonaire Primitive. Primary Pulmonary Angiosarcoma. Revue de Pneumologie Clinique, 61, 115-118. http://dx.doi.org/10.1016/S0761-8417(05)84798-4

[23] Ozcelik, C., Onat, S., Yaldiz, M. and Ozcelik, Z. (2006) Primary Epithelioid Angiosarcoma of the Lung Presenting as Pulmonary Hemorrhage. Asian Cardiovascular and Thoracic Annals, 14, 69-71. http://dx.doi.org/10.1177/021849230601400118

[24] Herrak, L., Alaziz, S. and Benosmane, A. (2007) Primary Pulmonary Angiosarcoma. Revue des Maladies Respiratoires, 24, 73-76. http://dx.doi.org/10.1016/S0761-8425(07)91015-3

[25] Wilson, R., Glaros, S., Brown, R.K., Michael, C. and Reisman, D. (2008) Complete Radiographic Response of Primary Pulmonary Angiosarcomas Following Gemcitabine and Taxotere. Lung Cancer, 61, 131-136. http://dx.doi.org/10.1016/j.lungcan.2007.12.006

[26] Kuroda, N., Hamaguchi, N., Inoue, K., Ohara, M., Mizuno, K., Hayashi, Y. and Lee, G.H. (2009) Application of Immunocytochemistry to the Diagnosis of Primary Epithelioid Angiosarcoma of the Lung. Medical Molecular Morphology, 42, 250-253. http://dx.doi.org/10.1007/s00795-008-0417-8

[27] Wan Musa, W.R., Abdulwakil Elraied, M.A., Phang, K.S., Kwah, Y.G., Tan, S.P., Harun, R. and Ban, A.Y. (2010) Primary Epithelioid Angiosarcoma of the Lung Presenting as Left-Sided Shoulder Pain. Annals of the Academy of Medicine, Singapore, 39, 658-659.

[28] Weissferdt, A. and Moran, C.A. (2010) Primary Vascular Tumors of the Lungs: A Review. Annals of Diagnostic Pathology, 14, 296-308. http://dx.doi.org/10.1016/j.anndiagpath.2010.03.001

[29] Sasaki, R., Soejima, T., Kishi, K., Imajo, Y., Hirota, S., Kamikonya, N., Murakami, M., Kawabe, T., Ejima, Y., Matsumoto, A. and Sugimura, K. (2002) Angiosarcoma Treated with Radiotherapy: Impact of Tumor Type and Size on Outcome. International Journal of Radiation Oncology, Biology, Physics, 52, 1032-1040. http://dx.doi.org/10.1016/S0360-3016(01)02753-5

[30] Fayette, J., Martin, E., Piperno-Neumann, S., Le Cesne, A., Robert, C., Bonvalot, S., Ranchère, D., Pouillart, P., Coindre, J.M. and Blay, J.Y. (2007) Angiosarcomas, a Heterogeneous Group of Sarcomas with Specific Behavior Depending on Primary Site: A Retrospective Study of 161 Cases. Annals of Oncology, 18, 2030-2036. http://dx.doi.org/10.1093/annonc/mdm381

[31] Donghi, D., Dummer, R. and Cozzio, A. (2010) Complete Remission in a Patient with Multifocal Metastatic Cutaneous Angiosarcoma with a Combination of Paclitaxel and Sorafenib. British Journal of Dermatology, 162, 697-699. http://dx.doi.org/10.1111/j.1365-2133.2009.09607.x

[32] Penel, N., Bui, B.N., Bay, J.O., Cupissol, D., Ray-Coquard, I., Piperno-Neumann, S., Kerbrat, P., Fournier, C., Taieb, S., Jimenez, M., Isambert, N., Peyrade, F., Chevreau, C., Bompas, E., Brain, E.G. and Blay, J.Y. (2008) Phase II Trial of Weekly Paclitaxel for Unresectable Angiosarcoma: The ANGIOTAX Study. Journal of Clinical Oncology, 26, 5269-5274. http://dx.doi.org/10.1200/JCO.2008.17.3146

[33] Yamada, M., Hatta, N., Mizuno, M., Oishi, N. and Takehara, K. (2005) Weekly Low-Dose Docetaxel in the Treatment of Lung Metastases from Angiosarcoma of the Head. British Journal of Dermatology, 152, 811-812. http://dx.doi.org/10.1111/j.1365-2133.2005.06503.x

[34] Girard, N., Barbareschi, M., Cordier, J.F. and Murer, B. (2007) What Is a Rare Tumour and How Should It Be Dealt with Clinically? Pathology of the Lung by W. Timens and H. H. Popper. European Respiratory Society Monographs, 86-134. http://dx.doi.org/10.1183/1025448x.00039005