Alcaraz-García P, Díaz-Palacios S, Castillo-Canto C, Gatica-Pérez A, Sánchez-González JA

Language: Spanish
References: 28
Page: 67-71
PDF size: 427.77 Kb.

ABSTRACT

Background: Primary synovial sarcomas of the lung are extremely rare, constituting 0.1% to 0.5% of lung cancers. The first case was reported by Gaertner in 1996.
Clinical case: We present the case of a 15-year-old female who presented with cough, white-colored secretions, right-sided chest pain and progressive dyspnea. Physical examination revealed increased exertion during breathing with no cyanosis. The presence of right scapular pulmonary condensation syndrome was observed. Chest x-ray demonstrated homogeneous opacity occupying the lower two thirds of the right hemithorax. Posterolateral thoracotomy with right lower and medium lobectomy was performed. Because there was evidence of upper lobe tumor, it was decided to carry out a right pneumonectomy. Histopathological diagnosis was biphasic synovial sarcoma.
Conclusions: The presentation of pulmonary synovial sarcoma generally shows a peripheral location with a nonencapsulated and well-circumscribed tumor. Size ranges from 0.6 to 17 cm (mean: 5 cm). Histology is often characterized by a monophasic pattern. Diagnosis is difficult except for a uniform spindle cell pattern. Most synovial sarcomas show immunoreactivity for cytokeratin and/or epithelial membrane antigen. Cytogenetic characteristic of synovial sarcoma are t(X; 18)(p11, q11). Patient prognosis for pulmonary synovial sarcoma is poor with an overall 5-year survival rate of 50%.

REFERENCES

1. Bégueret H, Galateau-Salle F, Guillou L, Chetaille B, Brambilla E, Vignaud JM, et al. Primary intrathoracic synovial sarcoma: a clinicopathologic study of 40 t(x;18)-positive cases from the French Sarcoma Group and the Mesopath Group. Am J Surg Pathol 2005;29:339-346.

2. Robbins S, Cotran R, Kumar V. El sistema osteomuscular. En: Patología Humana. 8th ed. Spain: Elseiver, 2008. p. 758.

3. Spillane AJ, A’Hern R, Judson IR, Fisher C, Thomas JM. Synovial sarcoma: a clinicopathologic, staging, and prognostic assessment. J Clin Oncol 2000;18:3794-3803.

4. Hosono T, Hironaka M, Kobayashi A, Yamasawa H, Bando M, Ohno S, et al. Primary pulmonary synovial sarcoma confirmed by molecular detection of SYT-SSX1 fusion gene transcripts: a case report and review of the literature. Jpn J Clin Oncol 2005;35:274-279.

5. Nishiyama N, Iwata T, Izumi N, Tsukioka T, Nagano K, Suehiro S. Aggressive repeat surgery for a recurrent synovial sarcoma in the pleura. Ann Thorac Cardiovasc Surg 2009;15:46-49.

6. Keel SB, Bacha E, Mark EJ, Nielsen GP, Rosenberg AE. Primary pulmonary sarcoma: a clinicopathologic study of 26 cases. Mod Pathol 1999;12:1124-1131.

7. Russell WO, Cohen J, Enzinger F, Hajdu SI, Heise H, Martin RG, et al. A clinical and pathological staging system for soft tissue sarcomas. Cancer 1977;40:1562-1570.

8. Gladish G, Sabloff B, Munden R, Truong M, Erasmus J, Chasen M. Primary thoracic sarcomas. Radiographics. 2002;22:621-637.

9. Travis WD, Brambila E, Muller-Hermelink HK, Harris CC. World Health Organization Classification of Tumours. Pathology and Genetics of Tumors of the Lung, Pleura, Thymus and Heart. Lyon: IARC Press, 2004. p. 111-112.

10. Travis WD, Travis LB, Devesa SS. Lung cancer. Cancer 1995;75:191-202.

11. Gaertner E, Zeren EJ, Fleming MV, Colby TV, Travis WD. Biphasic synovial sarcomas arising in the pleural cavity: a clinicopathologic study of five cases. Am J Surg Pathol 1996;20:36-45.

12. Essary LR, Vargas SO, Fletcher CD. Primary pleuropulmonary synovial sarcoma: reappraisal of a recently described anatomic subset. Cancer 2002;94:459-469.

13. Zaring RA, Roepke JE. Pathologic quiz case. Pulmonary mass in a patient presenting with a hemothorax. Diagnosis: primary pulmonary biphasic synovial sarcoma. Arch Pathol Lab Med 1999;123:1287-1289.

14. Okamoto S, Hisaoka M, Daa T, Hatakeyama K, Iwamasa T, Hashimoto H. Primary pulmonary synovial sarcoma: a clinicopathologic, immunohistochemical, and molecular study of 11 cases. Hum Pathol 2004;35:850-856.

15. Corson JM, Weiss LM, Banks-Schlegel SP, Pinkus GS. Keratin proteins and carcinoembryonic antigen in synovial sarcomas. An immunohistochemical study of 24 cases. Hum Pathol 1984;15:615-621.

16. Miettinen M. Keratin subsets in spindle cell sarcomas. Keratins are widespread but synovial sarcoma contains a distinctive keratin polypeptide pattern and desmoplakins. Am J Pathol 1991;138:505-513.

17. Fisher C, Schofield JB. S-100 protein positive synovial sarcoma. Histopathology 1991;19:375-377.

18. Dei Tos AP, Wadden C, Calonje E. Immunohistochemical demonstration of glycoprotein p30/32MIC2 (CD99) in synovial sarcoma: a potential cause of diagnostic confusion. Appl Immunohistochem 1995;3:168-173.

19. Rossi S, Orvieto E, Furlanetto A, Laurino L, Ninfo V, Dei Tos A. Utility of the immunohistochemical detection of FLI-1 expression in round cell and vascular neoplasm using a monoclonal antibody. Mod Pathol 2004;17:547-552.

20. Guillou L, Wadden C, Kraus MD, Dei Tos AP, Fletcher CDM. S100 protein activity in synovial sarcoma. A potentially frequent diagnostic pitfall. Immunohistochemical analysis of 100 cases. Appl Immunohistochem 1996;4:167-175.

21. Terasaki H, Niki T, Hasegawa T, Yamada T, Suzuki K, Kusumoto M, et al. Primary synovial sarcoma of the lung: a case report confirmed by molecular detection of SYT-SSX fusion gene transcripts. Jpn J Clin Oncol 2001;31:212-216.

22. Yano M, Toyooka S, Tsukuda K, Dote H, Morimoto Y, Ohata N, et al. SYT-SSX fusion genes in synovial sarcoma of the thorax. Lung Cancer 2004;44:3871-391.

23. Tvrdík D, Povýsil C, Svatosová J, Dundr P. Molecular diagnosis of synovial sarcoma: RT-PCR detection of SYT-SSX1/2 fusion transcripts in paraffin-embedded tissue. Med Sci Monit 2005;11:1-7.

24. Lee HK, Kwon HJ, Lee HB, Jin GY, Chung MJ, Lee YC. Radiofrequency thermal ablation of primary pleural synovial sarcoma. Respiration 2006;73:250-252.

25. Dennison S, Weppler E, Giacoppe G. Primary pulmonary synovial sarcoma: a case report and review of current diagnostic and therapeutic standards. Oncologist 2004;9:339-342.

26. Paulino AC. Synovial sarcoma: Prognostic factors and patterns of failure. Am J Clin Oncol 2004;27:122-127.

27. de Silva MV, McMahon AD, Reid R. Prognostic factors associated with local recurrence, metastases, and tumor-related death in patients with synovial sarcoma. Am J Clin Oncol 2004;27:113-121.

28. Trassard M, Le Doussal V, Hacene K, Terrier P, Ranchère D, Guillou L, et al. Prognostic factors in localized primary synovial sarcoma: a multicenter study of 128 adult patients. J Clin Oncol 2001;19:525-534.