Lung (2014) 192:211–214 DOI 10.1007/s00408-013-9521-1

BRIEF REPORT

Primary Pulmonary Synovial Sarcoma: A Rare Primary Pulmonary Tumor Roger Fei Falkenstern-Ge • Martin Kimmich • Andreas Grabner • Heike Horn • Godehard Friedel German Ott • Martin Kohlha¨ufl



Received: 27 July 2013 / Accepted: 2 October 2013 / Published online: 30 October 2013 Ó Springer Science+Business Media New York 2013

Abstract Introduction Pulmonary sarcomas overall are very uncommon and comprise only 0.5 % of all primary lung malignancies. The diagnosis is established only after sarcoma-like primary lung malignancies and a metastatic extrathoracic sarcoma have been excluded. Synovial sarcoma accounts for *8 % of soft-tissue sarcomas. Synovial sarcoma arising from the pleura has rarely been reported. Methods We report a case of a 58-year-old woman who complained of right-sided chest pain and shortness of breath. Chest CT scan revealed a large heterogeneous mass, occupying most of the right hemithorax. Histologic diagnosis was supplemented by interphase cytogenetic (FISH) analysis. Results Computed tomography guided Tru-cut biopsy was suspicious for a sarcomatous or fibrous malignancy. However, intraoperative frozen-section diagnostics confirmed the

diagnosis of a sarcoma. Immunohistochemistry showed that tumor cells expressed epithelial membrane antigen, CD99 and BCL2. Based on immunohistochemistry, the diagnosis of synovial sarcoma was suspected and was confirmed by FISH analysis. The patient was treated with right upper bilobectomy. Due to R1-resection status, postsurgical systemic chemotherapy was administered. Conclusions Primary pulmonary synovial sarcoma is a rare primary lung tumor. Due to extensive size of the tumor with pleural and mediastinal invasion only a R1-resection status could be achieved by thoracic surgery.

R. F. Falkenstern-Ge (&)  M. Kimmich  M. Kohlha¨ufl Division of Pulmonology, Klinik Schillerhoehe, Center for Pulmonology and Thoracic Surgery, Teaching Hospital of the University of Tuebingen, Solitude Str. 18, 70839 Stuttgart-Gerlingen, Germany e-mail: [email protected]; [email protected]

H. Horn Dr. Margarete Fischer-Bosch Institute of Clinical Pharmacology IKP, Auerbachstrasse 112, 70376 Stuttgart, Germany e-mail: [email protected]

M. Kimmich e-mail: [email protected] M. Kohlha¨ufl e-mail: [email protected]

Keywords Immunohistochemistry  Lung mass  Primary pulmonary synovial sarcoma

G. Friedel Division of Thoracic Surgery, Klinik Schillerhoehe, Center for Pulmonology and Thoracic Surgery, Teaching Hospital of the University of Tuebingen, Solitude Str. 18, 70839 StuttgartGerlingen, Germany e-mail: [email protected]

A. Grabner  G. Ott Department of Clinical Pathology, Robert Bosch Krankenhaus, Teaching Hospital of the University of Tuebingen, Auerbachstrasse 110, 70376 Stuttgart, Germany e-mail: [email protected] G. Ott e-mail: [email protected]

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Fig. 1 Chest CT scan shows a large tumor of the right middle lobe with pleural and mediastinal infiltration

Introduction A 58-year-old nonsmoking woman was complaining of gradually increasing dyspnea for 2 months. In the referring hospital, contrast-enhanced chest CT scan revealed a huge heterogeneous tumor mass in the right middle/lower field of the lung, with multiple areas of necrosis occupying almost 50 % of the whole right hemithorax and infiltration of the pleura (Fig. 1). The patient was referred to our thoracic center for further diagnostic evaluation. The patient’s past medical history was unremarkable. Her initial physical examination and laboratory tests were all within normal limits. Spirometry revealed no restrictive or obstructive pattern. The diffusion capacity was reduced (DLCO-SB 50 %). Bronchoscopic intervention rendered no clear histological diagnosis. Therefore, CT-guided Tru-cut biopsy was performed. Histological workup revealed a spindle-cell neoplasm without further differentiation, which was considered suspicious of a soft-tissue malignancy. Surgical resection was performed. Histopathological workup rendered the final diagnosis of a primary pulmonary synovial sarcoma. At thoracotomy, the mass proved to obviously arise from the pleura and adhere to the right chest wall. A right upper/middle bilobectomy with partial chest wall resection and muscle flap reconstruction were performed. Due to R1-resection status a postoperative systemic chemotherapy with doxorubicin and ifosfamide was to be initiated. An initially performed CT-guided fine-needle biopsy failed to yield a conclusive result. Histopathological workup of the resected mediastinal mass showed a sarcomatous tumor with a proliferation of spindle cells arranged in interlacing bundles (Fig. 2). No epithelialglandular tumor component was recognized, and CK7 staining identified only few positive intermingled epithelioid cells (Fig. 3).

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Fig. 2 Histopathology of the sarcomatous tumor with a proliferation of spindle cells (H&E 9200)

On immunohistochemistry, the spindled tumor cells strongly expressed vimentin and were positive for epithelial membrane antigen (EMA), BCL2 and CD99. FISH analysis using a SS18 break-apart probe disclosed a signal constellation indicative of a rearrangement of the gene (Fig. 4). A final diagnosis of monophasic synovial sarcoma was eventually made.

Discussion Synovial sarcoma is a rare mesenchymal tumor accounting for less than 2 % of soft tissue sarcomas in patients older than age 50 years [1]. It occurs most commonly in deep soft tissues of the extremities of adolescents and young adults, but other rare locations, such as the lungs also can be involved [1]. It is a highly aggressive neoplasia, which is more common in men. The tumor is not related to cigarette smoking. The diagnosis of primary pulmonary synovial sarcoma requires clinical, radiological, pathological, and immunohistochemical

Lung (2014) 192:211–214

Fig. 3 Staining with CK7 identified only few positive intermingled epithelioid cells (9200)

Fig. 4 FISH analysis using a SS18 break-apart probe disclosed a rearrangement of the gene (91,000)

investigations to exclude alternative primary tumors and metastatic sarcoma. Synovial sarcomas grow in four patterns: monophasic fibrous (spindle-cell), monophasic epithelial, biphasic, and the poorly differentiated monophasic subtype [2, 3]. The differential diagnoses of the monophasic subtypes comprise fibrosarcoma, hemangiopericytoma, leiomyosarcoma, and the spindle cell variant of squamous cell carcinoma. Therefore, immunohistochemistry is essential for arriving at a definitive diagnosis of the monophasic subtype of synovial cell sarcoma. Overall, pulmonary sarcomas are rare, accounting for less than 0.5 % of all lung cancers and most malignant mesenchymal tumors of the lung are metastases of extrathoracic tumors [4]. Leiomyosarcomas, fibrosarcomas, and hemangiopericytomas are the most common types of primary

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pulmonary sarcomas [4]. Primary pulmonary sarcomas are extremely rare with only few case reports in the literature [4]. Most of the published cases of primary pulmonary synovial sarcomas have reported centrally located tumors, which present with symptoms of postobstructive pneumonia (cough, dyspnea, fever) and hemoptysis. Peripheral tumors are less common and initially asymptomatic but may infiltrate adjacent tissues, i.e., pleura, thoracic wall, and mediastinum, or give rise to distant metastases [4]. Bronchoscopy may reveal endobronchial polypoidal growth in some cases [5]. Surgery is the mainstay of therapy, and free surgical margins are critical for the prevention of local recurrence. However, these tumors are highly aggressive with an often large size already at presentation. Therefore, it is difficult to achieve tumor-free surgical margins. The adjuvant chemotherapy may increase disease-free survival. The overall prognosis is poor in primary pulmonary synovial sarcoma [6]. Most synovial sarcomas show immunoreactivity for cytokeratin and/or EMA. The cytogenetic hallmark of synovial sarcoma is the t(X;18)(p11;q11) chromosomal translocation, constituting the most important criterion of diagnosis and leading to the rearrangement of the SS18 and one of the SSX genes [7]. The prognosis of pulmonary synovial sarcoma does not differ from sarcomas of other locations. The overall 5-year survival rate is 50 %, and poor prognostic risk factors include age older than 20 years, female sex, incomplete resection, tumor size [ 5 cm, extensive tumor necrosis, high number of mitoses ([10 per 10 high-power fields), neurovascular invasion, and recently, SYT–SSX1 variant [8]. Our patient had all poor prognostic risk factors, especially a tumor measuring more than 10 cm in diameter. Systemic chemotherapy is widely used in the treatment of nonresectable advanced disease, primarily with palliative intention. Initial standard chemotherapy for advanced or metastatic synovial sarcoma consists of single-agent anthracycline (mainly doxorubicin) or an anthracycline-based combination with for example ifosfamide and dacarbazine [9]. Regimens containing doxorubicin, cyclophosphamide, cisplatin, vincristine, dacarbazine, and other agents have been shown to be effective as preoperative and/or postoperative treatment of synovial sarcoma [9]. Based on the R1 status after surgical resection, our patient was treated with systemic chemotherapy consisting of doxorubicin and ifosfamide. Conflict of interest interest.

The author(s) indicated no potential conflicts of

References 1. Suurmeijer et al. (2013) Synovial sarcoma. In: Fletcher DM et al. (eds) WHO classification of tumors of soft tissue and bone, 4th edn. IARC, Lyon

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214 2. Okamoto S, Hisaoka M, Daa T, Hatakeyama K, Iwamasa T, Hashimoto HA et al. (2004) Primary pulmonary synovial sarcoma: a clinicopathologic, immunohistochemical, and molecular study of 11 cases. Hum Pathol 35:850–856 3. Essary LR, Vargas SO, Fletcher CD (2002) Primary pleuropulmonary synovial sarcoma: reappraisal of a recently described anatomic subset. Cancer 94:459–469 4. Etienne-Mastroianni B, Falchero L, Chalabreysse L, Loire R, Ranchere D, Souquet PJ et al. (2002) Primary sarcomas of the lung: a clinicopathologic study of 12 cases. Lung Cancer 38:283–289 5. Kumar R, Menon S, Desai SB, Pramesh CS, Menon H, Jambhekar NA (2009) Primary endobronchial synovial sarcoma confirmed by SYT-SSX1 fusion gene transcript by reverse transcriptase polymerase chain reaction. Indian J Pathol Microbiol 52:520–523

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Lung (2014) 192:211–214 6. Dennison S, Weppler E, Giacoppe G (2004) Primary pulmonary synovial sarcoma: a case report and review of current diagnostic and therapeutic standards. Oncologist 9:339–342 7. Alcaraz-Garcı´a P, Dı´az-Palacios S, Castillo-Canto C, Gatica-Pe´rez A, Sa´nchez-Gonza´lez JA (2012) Primary pulmonary biphasic synovial sarcoma: a case report and literature review. Cir Cir 80(1):67–71 8. Trassard M, Le Doussal V, Hacene K, Terrier P, Ranche`re D, Guillou L et al. (2001) Prognostic factors in localized primary synovial sarcoma: a multicenter study of 128 adult patients. J Clin Oncol 19:525–534 9. Miser JS, Kinsella TJ, Sriche TJ et al. (1987) Ifosfamide with mesna uroprotection and etoposide: an effective regimen in the treatment of recurrent sarcomas and other tumors of children and young adults. J Clin Oncol 5:1191–1198

Primary pulmonary synovial sarcoma: a rare primary pulmonary tumor.

Pulmonary sarcomas overall are very uncommon and comprise only 0.5 % of all primary lung malignancies. The diagnosis is established only after sarcoma...
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