Abstract
Intimal sarcoma of pulmonary artery (PAIS) is a rare disease with no characteristic symptoms. No standard therapeutic guidelines have been established so far. A 55-year-old woman who underwent pulmonary endarterectomy in 2005 with a pathological and immunohistochemical result of PAIS was re-admitted to our hospital on August 24, 2009, presenting with cough and dyspnea. The thoracic computed tomography (CT) scan revealed a 12.5 cm × 9 cm well-defined mass in the right lobe, considered a local neoplasm recurrence after a 44 month symptom-free period. As surgical resection was not proposed, she received combined chemotherapy consisting of doxorubicin, cisplatin, and ifosfamide for two cycles. Because of intolerable side effects, she received vinorelbine and cisplatin for the next four cycles. CT scans after six cycles showed remarkable regression of the tumor. After that, she began to take oral cyclophosphamide (50 mg t.i.d) everyday as a maintenance therapy. As of the time of writing, 19 months after the onset of the recurrence, she remains stable. Several antineoplastic drugs have been reported to be used in PAIS, with poor effects in most cases. To our knowledge, this is the first case of PAIS that has been successfully treated by a vinorelbine-based regimen used as second-line chemotherapy. Vinorelbine is a promising drug that may be relatively well tolerated in heavily pretreated patients with PAIS.
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Mandelstamm M (1923) Uber primare neubildungen des herzen. Virchows Arch Pathol Anat 245:43–54 (in Germany)
Mattoo A, Fedullo PF, Kapelanski D et al (2002) Pulmonary artery sarcoma: a case report of surgical cure and 5-year follow-up. Chest 122:745–747
Eisenhauer EA, Therasse P, Bogaerts J et al (2009) New response evaluation criteria in solid tumours: revised RECIST guideline (version 1.1). Eur J Cancer 45:228–247
Bode-lesniewska B, Komminoth P (2002) Pathology and genetics of tumors of soft tissue and bone. In: Fletcher CDM, Unni KK, Mertens F (eds) World Health Organization classification of tumors. Oxford University Press, Oxford, pp 223–224
Burke A, Virami R (1996) Tumors of the great vessels. In: Burke A, Virami R (eds) Atlas of tumor pathology. AFIP, Washington, DC, pp 221–227
Nonomura A, Kurumaya H, Kono N et al (1998) Primary pulmonary artery sarcoma. Report of two autopsy cases studied by immunohistochemistry and electron microscopy, and review of 110 cases reported in the literature. Acta Pathol Jpn 38:883–896
Furest I, Marin M, Escribano P et al (2006) Intimal sarcoma of the pulmonary artery: a rare cause of pulmonary hypertension. Arch Bronconeumol 42:148–150
Baker PB, Goodwin RA (1985) Pulmonary artery sarcomas: a review and report of a case. Arch Pathol Lab Med 109:35–39
Naoki N, Chikao Y, Hatsue IU et al (2000) Histopathological characterization of aortic intimal sarcoma with multiple tumor emboli. Pathol Int 50:923–927
Cox JE, Chiles C, Aquino SL et al (1997) Pulmonary artery sarcoma: a review of clinical and radiologic features. J Comput Assist Tomogr 21:750–755
Genoni M, Biraima AM, Bode B et al (2001) Combined resection and adjuvant therapy improves prognosis of sarcomas of the pulmonary trunk. J Cardiovasc Surg 42:829–833
Majeski J, Crawford ES, Majeski EI et al (1998) Primary aortic intimal sarcoma of the endothelial cell type with long-term survival. J Vasc Surg 27:555–558
Hirose T, Ishikawa N, Hamada K et al (2009) A case of intimal sarcoma of the pulmonary artery treated with chemoradiotherapy. Intern Med 48:245–249
Uchida A, Tabata M, Kiura K et al (2005) Successful treatment of pulmonary artery sarcoma by a two-drug combination chemotherapy consisting of ifosfamide and epirubicin. Jpn J Clin Oncol 35:417–419
Hsing JM, Thakkar SG, Borden EC et al (2007) Intimal pulmonary artery sarcoma presenting as dyspnea: case report. Int Semin Surg Oncol 4:14
Penel N, Taieb S, Ceugnart L et al (2008) Report of eight recent cases of locally advanced primary pulmonary artery sarcomas: failure of doxorubicin-based chemotherapy. J Thorac Oncol 38:907–911
Hongqing L, Qin Q, Conghua X (2008) Response of pulmonary artery intimal sarcoma to surgery, radiotherapy and chemotherapy: a case report. J Med Case Rep 2:217
Brennan MF, Singer S, Maki RG et al (2005) Soft tissue sarcoma. In: DeVita VT, Hellman S, Rosenberg SA (eds) Cancer: principles and practice of oncology, 7th edn. Lippincott Williams & Wilkins, Philadelphia, pp 1581–1637
Antman KH, Elias AD (1988) Chemotherapy of advanced soft-tissue sarcomas. Semin Surg Oncol 4:53–58
Svancarova L, Blay JY, Judson IR et al (2002) Gemcitabine in advanced adult soft-tissue sarcomas: a phase II study of the EORTC Soft Tissue and Bone Sarcoma Group. Eur J Cancer 38:556–559
Hensley ML, Maki RG, Venkatraman E et al (2002) Gemcitabine and docetaxel in patients with unresectable leiomyosarcoma: results of a phase II trial. J Clin Oncol 20:2824–2831
Gottlieb JA, Benjamin RS, Baker LH et al (1976) Role of DTIC (NSC-45388) in the chemotherapy of sarcomas. Cancer Treat Rep 60:199–203
Sibyl EA, Mary LK, David RD et al (2006) Retrospective analysis of vinorelbine chemotherapy for patients with previously treated soft-tissue sarcomas. Sarcoma. doi:10.1155/SRCM/2006/15947
Casanova M, Ferrari A, Spreafico F et al (2002) Vinorelbine in previously treated advanced childhood sarcomas: evidence of activity in rhabdomyosarcoma. Cancer 94:3263–3268
Nasti G, Errante D, Talamini R et al (2000) Vinorelbine is an effective and safe drug for AIDS-related Kaposi’s sarcoma: results of a phase II study. J Clin Oncol 18:1550–1557
Fidias P, Demetri G, Harmon D (1998) Navelbine shows activity in previously treated sarcoma patients: phase II results from MGH/Dana Farber/Partner’s Cancer Care Study. Proc Am Soc Clin Oncol 17:1977
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Y. Xu and K. Wang contributed equally to this work.
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Xu, Y., Wang, K., Geng, Y. et al. A case of intimal sarcoma of the pulmonary artery successfully treated with chemotherapy. Int J Clin Oncol 17, 522–527 (2012). https://doi.org/10.1007/s10147-011-0338-8
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DOI: https://doi.org/10.1007/s10147-011-0338-8