Abstract
Myofibrosarcoma is a rare neoplasm that occurs mainly in the head and neck region and extremities of middle-aged patients. It often appears as a low-grade sarcoma and rarely metastasizes. We report the case of a 47-year-old male patient with a malignant mesenchymal pulmonary tumor affecting almost the entire lower left lobe. Clinically suggestive for a lung carcinoma, the tumor showed typical features of a myofibrosarcoma. A major spindle cell component was observed being positive for smooth-muscle actin, calponin, and vimentin, while stainings for desmin, h-caldesmon, alkaline phosphatase (ALK), and extensively studied cytokeratins were negative. Striking was a strong infiltrate with neutrophilic and eosinophilic granulocytes. DNA cytometry revealed aneuploidy with a peak in the near triploid range. Comparative genomic hybridization demonstrated multiple DNA gains and losses correlating with an aggressive clinical course. Shortly after resection of the primary tumor, the patient showed multiple distant metastases in the contralateral lung, the mediastinal lymph nodes, the left adrenal gland, and the pectoral and deltoid muscle, which responded well to chemotherapy. The case report will discuss the evidence for the final diagnosis of a primary pulmonary myofibrosarcoma and the differential diagnosis of sarcomatoid tumors of the lung.
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References
Bahadori M, Liebow AA (1973) Plasma cell granulomas of the lung. Cancer 31:191–208
Bocking A, Nguyen VQ (2004) Diagnostic and prognostic use of DNA image cytometry in cervical squamous intraepithelial lesions and invasive carcinoma. Cancer 102:41–54
Brooks JSJ (2004) Disorders of soft tissue. In: Mills SE (ed) Sternberg's diagnostic surgical pathology. Lippincott, Philadelphia, p139
Chang SE, Choi JH, Sung KJ et al (2001) A case of cutaneous low-grade myofibroblastic sarcoma. J Dermatol 28:383–387
Donner LR, Trompler RA, White RR IV (1996) Progression of inflammatory myofibroblastic tumor (inflammatory pseudotumor) of soft tissue into sarcoma after several recurrences. Human Pathol 27:1095–1098
Essary LR, Vargas SO, Fletcher CD (2002) Primary pleuropulmonary synovial sarcoma: reappraisal of a recently described anatomic subset. Cancer 94:459–469
Eyden BD (1993) Brief review of the fibronexus and its significance for myofibroblastic differentiation and tumor diagnosis. Ultrastruct Pathol 17:611–622
Fisher C (2004) Myofibrosarcoma. Virchows Arch 445:215–223
Fletcher CD (1998) Myofibroblastic tumours: an update. Verh Dtsch Ges Pathol 82:75–82
Garber ME, Troyanskaya OG, Schluens K et al (2001) Diversity of gene expression in adenocarcinoma of the lung. Proc Natl Acad Sci U S A 98:13784–13789
Guillou L, Coindre JM, Bonichon F et al (1997) Comparative study of the National Cancer Institute and French Federation of Cancer Centers Sarcoma Group grading systems in a population of 410 adult patients with soft tissue sarcoma. J Clin Oncol 15:350–362
Kuhnen C, Homann HH, Mentzel T (2003) Myofibroblastic sarcoma of the thoracic wall. Change in appearance in tumor recurrence. Pathologe 24:128–135
Li XQ, Hisaoka M, Shi DR, Zhu XZ, Hashimoto H (2004) Expression of anaplastic lymphoma kinase in soft tissue tumors: an immunohistochemical and molecular study of 249 cases. Human Pathol 35:711–721
Majno G (1997) The story of the myofibroblast. Am J Surg Pathol 3:535–542
Mentzel T, Fletcher JA (2002) Low grade myofibroblastic sarcoma. In: Fletcher CDM, Unni KK, Mertens F (eds) World Health Organization classification of tumours. Pathology and genetics. Tumours of soft tissue and bone. IARC, Lyon, pp 94–95
Mentzel T, Dry S, Katenkamp D et al (1998) Low-grade myofibroblastic sarcoma: analysis of 18 cases in the spectrum of myofibroblastic tumors. Am J Surg Pathol 22:1228–1238
Montgomery E, Goldblum JR, Fisher C (2001) Myofibrosarcoma: a clinicopathologic study. Am J Surg Pathol 25:219–228
Nappi O, Glasner SD, Swanson PE et al (1994) Biphasic and monophasic sarcomatoid carcinomas of the lung. A reappraisal of 'carcinosarcomas' and 'spindle-cell carcinomas'. Am J Clin Pathol 102:331–340
Petersen I, Langreck H, Wolf G et al (1997) Small-cell lung cancer is characterized by a high incidence of deletions on chromosomes 3p, 4q, 5q, 10q, 13q and 17p. Br J Cancer 75:79–86
Schurch W, Seemayer TA, Gabbiani G (1998) The myofibroblast: a quarter century after its discovery. Am J Surg Pathol 22:141–147
Taccagni G, Rovere E, Masullo M et al (1997) Myofibrosarcoma of the breast: review of the literature on myofibroblastic tumors and criteria for defining myofibroblastic differentiation. Am J Surg Pathol 21:489–496
Sekido Y, Sato M, Usami N et al (2002) Establishment of a large cell lung cancer cell line (Y-ML-1B) producing granulocyte colony-stimulating factor. Cancer Genet Cytogenet 137:33–42
Watanabe K (2002) Leiomyosarcoma versus myofibrosarcoma. Am J Surg Pathol 26:393–394
Watanabe K, Ogura G, Tajino T et al (2001) Myofibrosarcoma of the bone: a clinicopathologic study. Am J Surg Pathol 25:1501–1507
Acknowledgements
The technical assistance of Manuela Pacyna-Gengelbach and Karl-Heinz Friedrich is gratefully acknowledged. Martina Eickmann provided excellent secretarial help in preparing the manuscript. We thank Christa Scholz for the macroscopic photographs.
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Morawietz, L., Kuhnen, C., Katenkamp, D. et al. Unusual sarcomatoid neoplasm of the lung suggesting a myofibrosarcoma. Virchows Arch 447, 990–995 (2005). https://doi.org/10.1007/s00428-005-0043-y
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DOI: https://doi.org/10.1007/s00428-005-0043-y