Ann Thorac Surg 1999;67:1154-1155
© 1999 The Society of Thoracic Surgeons
Case Reports
Leiomyosarcoma of the diaphragm
Justus T. Strauch, MDa,
Ivan Aleksic, MDa,
Bernd Schorn, MD, PhDa,
Harald Dalichau, MD, PhDa
a Department of Thoracic and Cardiovascular Surgery, University Hospital of Göttingen, Göttingen, Germany
Accepted for publication September 22, 1998.
Address reprint requests to Dr Strauch, Department of Thoracic and Cardiovascular Surgery, Georg-August-University Göttingen, Robert-Koch-Str. 40, D-37075 Göttingen, Germany
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Abstract
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An asymptomatic, intrathoracic mass was found on imaging studies in a 53-year-old man. After excision it proved to be an extrapulmonary leiomyosarcoma, a rare malignancy of smooth muscle, originating from the right diaphragm. The location of this malignant tumor is rare in an adult. The tumor was resected, the diaphragm was reconstructed, and the patient should have a long-term cure and good quality of life.
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Introduction
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Asymptomatic, intrathoracic, low-grade leiomyosarcomas, pedunculated from the right diaphragm, are rarely found in adults [1]. Leiomyosarcomas are common malign neoplasms of soft tissue. The leiomyosarcoma subtype comprises about 5% of all tissue sarcoma subtypes [2]. Soft tissue sarcomas comprise 0.7% of adult malignancies [3]; the most frequent site is the uterus. Other locations are the intestinum, the retroperitoneum, and the vascular system. They metastasize generally to lungs and liver.
A 53-year-old man who did not smoke had a single mass in the right side of the chest on routine chest radiography (Fig 1). The patient was completely asymptomatic and physical examination was otherwise unremarkable. Clinical history revealed a weight loss of 5 kg in 2 months. Lung function showed a high restriction, with a vital capacity (VC) of 44% and a forced expiratory volume in 1 second of 51%. Bronchoscopy revealed a torqued right bronchus system. Right middle and lower lobe bronchi could not be inspected completely. Contrast-enhanced computed tomography showed a giant, smooth-bordered mass in the lower right chest region. It was not evident whether the tumor mass was seperated from the lung parenchyma (Fig 2). No evidence of metastatic disease was found on computed tomographic scan of the abdomen and brain. Results of bone scan were negative. Extensive surgical resection was considered the treatment of choice.
The tumor was approached through a right anterolateral thoracotomy, but it was not clearly visible whether the tumor mass grew up from the diaphragm or perhaps from the liver or the adrenal gland. Later the thoracotomy was combined with a right paramedian laparotomy after transsecting three ribs. A hard, well-circumscribed mass measuring about 29 x 25 x 14 cm filled the whole lower right pleura (Fig 3). There was no contact to the liver or retroperitoneal organs. The tumor was pedunculated from the diaphragm. There was a complete atelectatic lower lobe of the right lung but no infiltration of the lung parenchyma. The tumor was resected with its base on the diaphragm, and the diaphragm was reconstructed. There were no enlarged thoracic lymph nodes. The postoperative course was uneventful, and the patient was discharged home well on the 12th day. Freedom from recurrence or metastatic disease is more than 2 years.
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Fig 3. Macroscopic view of the resected tumor, showing the leiomyosarcoma with the peduncle from the diaphragm.
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Histologic examination showed a low-grade leiomyosarcoma (grade 1) [4] that was well-differentiated, had hemorrhagic and necrotic areas, and originated in the diaphragm. The tumor was composed of spindle cells with eosinophilic cytoplasm and fusiform nuclei organized into bundles. Pleomorphic and giant cells and 2 to 3 mitoses per high-power field were also present. The grading system used was consistent with that defined by Enzinger and Weiss [4]. Immunocytochemical results showed strong diffuse staining for smooth muscle actin and patchy, strong staining for KiM1P. None of the lymph nodes contained tumor cells (Fig 4).
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Fig 4. Histologic appearance of the tumor, showing bundles of smooth muscle fibers containing uniform spindle-shaped nuclei (hematoxylin and eosin, original magnification x100).
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Comment
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Smooth muscle tumors of the diaphragm are rare, with most being leiomyomas. To the best of our knowledge, this is the fifth report of a leiomyosarcoma originating in the diaphragm [1]. Leiomyosarcomas metastasize by vascular embolization to liver, lung, and brain and seed to the peritoneal cavity. Lymphatic spread is uncommon [3]. When such a smooth muscle tumor originating in the diaphragm is suspected, surgical resection should include a wide segmental resection of normal-looking bowel. Surgical resection is the best treatment option for patients with a solitary mass. No therapeutic benefit has been derived from radiotherapy, chemotherapy, or hormonal treatment [3]. Little is known about the long-term prognosis. The few follow-up studies that have been published deal with leiomyosarcomas of the jejunum and ileum. The low rate of mitoses per high-power field and primary surgical resection are considered reasons for the long survival of patients with solitary leiomyosarcomas.
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References
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Blondeel P.N., Christiaens M.R., Thomas J., Gruwez J.A., Sciot R. Primary leiomyosarcoma of the diaphragm. Eur J Surg Oncol 1995;21:429-431.[Medline]
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Chang A.E., Rosenberg S.A., Glatstein E.J. Sarcomas of soft tissue. In: De Vita V.T., Hellman S., eds. . Cancer: principles and practice of oncology, 3rd ed. Philadelphia, Pennsylvania: Lippincott Co, 1989:1345-1398.
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Oakley R.H., Carty H., Cudmore R.E. Multiple mesenchymatoma of the chest wall. Pediatr Radiol 1985;15:58-60.[Medline]
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Enzinger F.M., Weiss S.W. Leiomyosarcoma. In: Gay S.M., ed. Soft tissue tumors. St Louis, Missouri: Mosby, 1995.
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Abstract
Introduction
