Ann Thorac Surg 2009;87:1927-1928. doi:10.1016/j.athoracsur.2008.10.077
© 2009 The Society of Thoracic Surgeons
Case Reports
Primary Giant Clear Cell Sarcoma (Soft Tissue Malignant Melanoma) of the Sternum
Gaetano Rocco, MD, FRCS(Ed)a,*,
Anna Rosaria de Chiara, MDc,
Flavio Fazioli, MDb,
Francesco Scognamiglio, MDa,
Antonello La Rocca, MDa,
Gaetano Apice, MDb,
Carla Riva, PhDd
a Department of Thoracic Surgery and Oncology, National Cancer Institute, Pascale Foundation, IRCCS, Naples, Italy
b Sarcoma Multidisciplinary Team, National Cancer Institute, Pascale Foundation, IRCCS, Naples, Italy
c Department of Pathology, National Cancer Institute, Pascale Foundation, IRCCS, Naples, Italy
d Department of Pathology, National Cancer Institute, IRCCS, Milan, Italy
Accepted for publication October 27, 2008.
* Address correspondence to Dr Rocco, Department of Thoracic Surgery and Oncology, National Cancer Institute, Pascale Foundation, Via Semmola 81, Naples, 80131, Italy (Email: gaetano.rocco{at}btopenworld.com).
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Abstract
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One case of a primary clear cell sarcoma of the sternum (also called soft tissue melanoma) is reported. This neoplasm represents a rare occurrence, and as a rule, differential diagnosis with melanoma often requires detailed immunohistochemistry and cytogenetic analysis (ie, rearrangement of EWS gene localized on 22q12 chromosome). Because wide resection is recommended, chest wall reconstruction may pose challenging technical issues. In our patient, we elected not to proceed to clavicular stabilization. Nevertheless, acceptable shoulder girdle mobility was observed after surgery.
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Introduction
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Reportedly, clear cell sarcomas account for less than 1% of soft tissue sarcomas [1], typically involving tendons and aponeuroses of young adults. Primary clear cell sarcomas of the bone are exceedingly rare [2]. Before 2005, a review of the literature had outlined five such cases with one report concerning possible sternal involvement [2]. Interestingly, only one case of clear cell sarcoma of the sternum was included in a 10-year series at the University of Texas M.D. Anderson Cancer Center [3]. Given the often controversial interpretation of pathologic findings, the differential diagnosis with melanoma must be taken into consideration and ruled out by immunohistochemistry [4].
A 53-year-old man was referred for the detection of a bulging mass located at the manubrium, with suspected involvement of both sternoclavicular joints as later confirmed by a chest CT (Fig 1). Past history was positive only for major bipolar depression for which he had been currently under treatment. General examination, as well as positron emission tomography and bone scans were negative for any other sites of disease. Given the clinical suspicion of a chondrosarcoma of the sternum, the patient was offered a surgical option that entailed removal of the sternal manubrium and body, and the costochondral junctions from rib 5 upward bilaterally (Fig 2a). No preoperative biopsy was obtained [5]. At surgery, the mass appeared capsulated, albeit displacing overlying muscle fibers. In addition, mammary pedicles were divided along with the median segments of the first ribs, thus allowing for elevation of the specimen for the bilateral resection of the medial clavicles. No involvement of the brachiocephalic venous or arterial trunks was noted. The chest wall defect was reconstructed with a sandwich of methylmethacrylate, whereas no effort was made to reconstruct the clavicles (Fig 2b). The patient made an uneventful recovery with a complete range of mobilization obtained at more than 3 months of surgery. At postoperative work-up, no evidence of melanoma was detected elsewhere. Final pathology showed a clear cell sarcoma (also termed soft tissue malignant melanoma) presenting as a fleshy white (10 x 7 cm) mass, only focally bordered by a thin shell of residual bone, infiltrating the surrounding muscles (Fig 3a).
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Fig 2. Intraoperative view showing (a) the sternal mass elevated (left = head of the patient); (b) reconstruction of the chest wall defect with methylmethacrylate.
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Fig 3. (a) Pathology specimen; (b) Hematoxylin & eosin stain showing nests of polygonal neoplastic cells with clear cytoplasm (in the center) and more elongated neoplastic cells with eosinophilic cytoplasm (on the left), surrounded by residual bone (x20).
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Histologically, the tumor was quite polymorphous in different areas, showing both nested and fascicular growth patterns. The neoplastic cells were both polygonal and spindle shaped; the cytoplasm was abundant and deeply eosinophilic, or clear, or rather scarce (Fig 3b). Solid round aspects and myxoid stroma were also present. Immunohistochemistry showed that S100 (often positive in tumors with melanocytic phenotype) was strong and diffuse, whereas other melanoma antigens (such as HMB45 and MART1) were negative. In addition, TFE3 was also positive, whereas cytokeratins, epithelial membrane antigen, renal cell carcinoma, CD10, and endocrine markers, such as chromogranins and synaptophysin, inhibin, and calretinin were all negative, thereby ruling out a carcinoma. Fluorescence in situ hybridization assay based on break-apart probes showed rearrangement of EWS gene localized on 22q12 chromosome, which was in line with a clear cell sarcoma, but not with the other possible conditions in the differential diagnosis based on morphological and immunohistochemical grounds [4].
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Comment
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One controversial technical issue may reside in the need for stabilizing the anterior chest wall when both sternoclavicular joints and medial clavicles are removed. In our case, we were able to preserve an acceptable shoulder girdle stability without clavicular stabilization [6]. In conclusion, radical resection of clear-cell sarcomas of the chest wall seems to yield prolonged survival, whereas given the rarity of the condition, the value of concurrent treatments, either in the neoadjuvant or adjuvant setting, is unclear [1, 7, 8]. However, too few cases have been observed and treated to outline a conclusive prognostic outlook.
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References
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- Gelczer RK, Wenger DE, Wold LE. Primary clear cell sarcoma of bone: a unique site of origin Skeletal Radiol 1999;28:240-243.[Medline]
- Hersekli MA, Ozkoc G, Bircan S, et al. Primary clear cell sarcoma of rib Skeletal Radiol 2005;34:167-170.[Medline]
- Soysal O, Walsh GL, Nesbitt JC, McMurtrey MJ, Roth JA, Putnam JB. Resection of sternal tumors: extent, reconstruction, and survival Ann Thorac Surg 1995;60:1353-1358.[Abstract/Free Full Text]
- Langezaal SM, Graadt van Roggen JF, Cleton-Jansen AM, Baelde JJ, Hogendoorn PC. Malignant melanoma is genetically distinct from clear cell sarcoma of tendons and aponeurosis (malignant melanoma of soft parts) Br J Cancer 2001;84:535-538.[Medline]
- Walsh GL, Davis BM, Swisher SG, et al. A single-institutional, multidisciplinary approach to primary sarcomas involving the chest wall requiring full-thickness resections J Thorac Cardiovasc Surg 2001;121:48-60.[Medline]
- Oishi H, Matsumura Y, Ishida I, et al. Sternal resection and chest wall reconstruction for primitive neuroectodermal tumor of the sternum Kyobu Geka 2008;61:836-840.[Medline]
- Suehara Y, Yazawa Y, Hitachi K, Terakado A. Clear cell sarcoma arising from the chest wall: a case report J Orthop Sci 2004;9:171-174.[Medline]
- Fujiwara K, Nanjou S, Ishihara S. Clear cell sarcoma of the chest wall; report of a case Kyobu Geka 2006;59:1119-1122.[Medline]
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Abstract
Introduction
