Ann Thorac Surg 2002;74:565-567
© 2002 The Society of Thoracic Surgeons
Case report
Extraskeletal osteosarcoma of the diaphragm presenting as a chest mass
Howard K. Song, MD, PhDa,
T. Mark Leibold, MDa,
Anthony A. Gal, MDa,
Joseph I. Miller, Jr, MD*a
a Division of Cardiothoracic Surgery and Department of Pathology and Laboratory Medicine, Emory University School of Medicine, Atlanta, Georgia USA
Accepted for publication February 18, 2002.
* Address reprint requests to Dr Miller, Division of Cardiothoracic Surgery, Emory University School of Medicine, 1365 Clifton Rd, NE, Atlanta, GA 30322 USA
e-mail: jmill10{at}emory.edu
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Abstract
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Extraskeletal osteosarcoma is a rare malignancy that has not been previously reported to arise from the diaphragm. We describe the case of a 47-year-old woman who had a large thoracic mass found at operation to be an extraskeletal osteosarcoma arising from the diaphragm. The tumor was completely resected en bloc with a margin of surrounding diaphragm, which necessitated a diaphragmatic reconstruction. The literature concerning this rare tumor is reviewed.
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Introduction
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Extraskeletal osteosarcoma is a rare malignancy that accounts for approximately 1% of all soft tissue sarcomas. By definition these tumors arise from tissues unattached to the skeleton and are composed of malignant cells of an osteoblastic phenotype producing osseous matrix [1]. Although they have been reported to occur in many locations throughout the body, they present most commonly as an enlarging soft tissue mass in the extremities. The retroperitoneum is the second most common site of occurrence [2, 3]. Extraskeletal osteosarcomas occurring within the thorax are unusual and have not previously been reported to arise from the diaphragm. Here we describe the case of a 47-year-old woman who presented with a large thoracic mass found at operation to be an extraskeletal osteosarcoma arising from the diaphragm.
A 47-year-old woman complained of left-sided chest and abdominal pain that was achy in nature and occasionally involved her left shoulder. She had no cough, shortness of breath, or hoarseness and denied constitutional symptoms including fevers, chills, and weight loss. There was no history of smoking or environmental exposures. On physical examination she had no cervical, supraclavicular, or axillary lymphadenopathy and her chest was clear to auscultation and percussion bilaterally. A chest roentgenogram showed a large, well-circumscribed mass with calcifications in the left side of the chest obscuring the diaphragm (Fig 1).
Computed tomography of the chest demonstrated the lesion to be 14 x 10 cm and arising either from the left lower lobe of the lung or the diaphragm with compression of the remaining uninvolved lung (Fig 2).
The mass was thought most likely to represent a primary osteochondroma or chondrosarcoma of the lung and exploratory thoracotomy for resection was recommended. The remainder of the patient’s preoperative evaluation was normal.
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Fig 1. Preoperative frontal chest roentgenogram demonstrating a large, well-circumscribed left-side chest mass with calcifications obscuring the diaphragm.
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Fig 2. Computed tomography of the chest demonstrating a 14 x 10 cm mass with calcifications in the left inferior chest arising either from the left lower lobe of the lung or the left hemidiaphragm.
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The patient was explored initially through the sixth rib interspace. A large, smooth white mass measuring approximately 20 x 14 x 10 cm was encountered in the inferior chest emanating from the central portion of the left hemidiaphragm (Fig 3A).
The lung was not directly invaded but was compressed by the mass, which also had a minor subdiaphragmatic component. A second incision was made in the ninth interspace to improve exposure of the diaphragm and retroperitoneum. The mass was then completely resected en bloc with a margin of surrounding diaphragm. The diaphragm was reconstructed with a patch of 2 mm thick Gore-Tex (W. L. Gore & Assoc, Flagstaff, AZ) sewn in with a heavy running Gore-Tex suture. This gave good stabilization of the diaphragm. The patient tolerated the procedure well and was discharged from the hospital on the sixth postoperative day. Pathologic examination of the specimen revealed a grade III extraskeletal osteosarcoma with a prominent chondroblastic component (Fig 3B). There was extensive necrosis involving as much as 10% of the mass and angiolymphatic invasion was identified. The patient continues to do well at mid-term follow-up with no evidence of local or regional recurrence.
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Fig 3. (A) Photograph of the gross specimen after resection and (B) photomicrograph of a representative section at x50 after staining with hematoxylin and eosin. The tumor was found to be a giant (20 x 14 x 10 cm) extraskeletal osteosarcoma. Histologic examination demonstrated a high-grade neoplasm with areas of osteoid formation.
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Comment
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Extraskeletal osteosarcoma is a high-grade malignant tumor with 5-year survival rates reported to be as low as 25% to 37% [1, 4]. Radical resection appears to offer the greatest chance for local control. Distant metastases occur most commonly in the lung and resection of pulmonary metastases has occasionally led to long-term survival [1]. Extraskeletal osteosarcomas arising within the thorax are unusual and typically occur within the mediastinum. Mediastinal extraskeletal osteosarcomas presenting late after mantle irradiation for Hodgkin’s disease have been described [5].
In the patient described the tumor arose from the diaphragm and presented as a large chest mass. While there was a minor infradiaphragmatic component, the majority of the tumor mass grew into the chest cavity compressing the left lower lobe of the lung without directly invading it. This pattern of growth limited the patient’s symptomatology until the tumor achieved significant size and likely led to the large size of the tumor at the time of presentation.
Large tumor size with extensive regional involvement is a common presentation for primary malignant tumors of the diaphragm. These tumors are rare and typically represent sarcomas of mesenchymal origin. A variety of histologies have been reported including leiomyosarcoma, malignant fibrous histiocytoma, and fibrosarcoma [6–8]. This case report establishes extraskeletal osteosarcoma as an additional histologic variant of sarcoma that may occur within the diaphragm.
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References
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- Lee J.S., Fetsch J.F., Wasdhal D.A., et al. A review of 40 patients with extraskeletal osteosarcoma. Cancer 1995;76:2253-2259.[Medline]
- Bane B.L., Evans H.L., Ro J.Y., et al. Extraskeletal osteosarcoma. A clinicopathologic review of 26 cases. Cancer 1990;65:2762-2770.[Medline]
- Chung E.B., Enzinger F.M. Extraskeletal osteosarcoma. Cancer 1987;60:1132-1142.[Medline]
- Lidang Jensen M., Schumacher B., Myhre Jensen O., et al. Extraskeletal osteosarcomas: a clinicopathologic study of 25 cases. Am J Surg Pathol 1998;22:588-594.[Medline]
- Catanese J., Dutcher J.P., Dorfman H.D., et al. Mediastinal osteosarcoma with extension to lungs in a patient treated for Hodgkin’s disease. Cancer 1988;62:2252-2257.[Medline]
- Parker M.C. Leiomyosarcoma of the diaphragm—a case report. Eur J Surg Oncol 1985;11:171-175.[Medline]
- Tanaka F., Sawada K., Ishida I., et al. Prosthetic replacement of the entire left hemidiaphragm in malignant fibrous histiocytoma of the diaphragm. J Thorac Cardiovasc Surg 1982;83:278-284.[Abstract]
- Sbokos C.G., Salama F.D., Powell V., et al. Primary fibrosarcoma of the diaphragm. Br J Dis Chest 1977;71:49-52.[Medline]
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Introduction
