Ann Thorac Surg 2008;85:1802-1805. doi:10.1016/j.athoracsur.2007.11.028
© 2008 The Society of Thoracic Surgeons
Case Reports
A Rare Tumor of the Diaphragm: Pleomorphic Rhabdomyosarcoma
Pelin Pinar Deniz, MDa,*,
Nilgun Kalac, MDa,
Gulnur Onde Ucoluk, MDa,
Belgin Samurkasoglu, MDa,
Abdullah Irfan Tastepe, MDb,
Erkmen Gulhan, MDb,
Funda Demirag, MDc
a Department of Chest Disease, Atatürk Chest Disease and Chest Surgery Center, Ankara, Turkey
b Department of Chest Surgery, Atatürk Chest Disease and Chest Surgery Center, Ankara, Turkey
c Department of Pathology, Atatürk Chest Disease and Chest Surgery Center, Ankara, Turkey
Accepted for publication November 9, 2007.
* Address correspondence to Dr Deniz, Fellow of Pulmonology and Chest Diseases, Atatürk Chest Diseases and Chest Surgery Center, Kecioren, Ankara, Turkey (Email: pelinpinarw{at}hotmail.com).
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Abstract
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We present the case of an extremely rare tumor of the diaphragm. The patient was a middle-aged woman with a 1-month history of dyspnea, fatigue, and cough. Chest roentgenogram and computed tomography scan of the thorax revealed a mass located at the dome of the right diaphragm. Pathologic examination of the biopsy specimen revealed pleomorphic rhabdomyosarcoma.
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Introduction
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Rhabdomyosarcoma is a rare primary diaphragmatic tumor. Rhabdomyosarcoma has four histologic subtypes: pleomorphic, alveolar, botryoid, and embryonal. We present a case of diaphragmatic pleomorphic rhabdomyosarcoma in a female patient.
A 58-year-old woman was admitted to Atatürk Chest Disease and Chest Surgery Center with a 1-month history of dyspnea, fatigue, and cough. Our physical examination showed a blood pressure of 110/70 mm Hg, heart rate of 88 beats/min, temperature of 36.6°C, and respiratory rate of 20 breaths/min. The respiratory system examination detected crackles at the right lower lung field. Results of cardiovascular and abdominal examinations were normal. The result of routine laboratory tests were as follows: hemoglobin, 13.5 mg/dL; leukocyte number, 8100/mm3; erythrocyte sedimentation rate, 40 mm/h. Results of biochemical tests on serum and tests of the urine were normal. Results of spirometric tests were forced vital capacity (FVC), 62%; forced expiratory volume in 1 second (FEV1), 64%; FEV1/FVC, 87%; and peak expiratory flow, 72%.
A chest roentgenogram showed a mass located on the right diaphragm (Fig 1), and a thorax computed tomography (CT) scan revealed 15 x 10 x 13 cm mass located at the dome of the diaphragm. Multiple metastatic masses with the biggest size of approximately 3 cm were also found at the right lung middle lobe lateral, lower lobe superior, and laterobasal segments as well as a left lung lower lobe posterobasal segment with subpleural localization (Fig 2). Results of abdominal ultrasonography and cranial CT were found to be normal.
A CT-guided needle biopsy was planned, but the lesion was highly vascularized; therefore, it was thought to have high risk of hemorrhage. The patient underwent open lung biopsy under general anesthesia. The result of the frozen specimen was reported as malignant mesenchymal tumor. It was decided to perform staged curative surgery. The operation was continued with right thoracotomy. Extrapulmonary mass resection, right-sided wedge resection of metastases, and partial right diaphragm resection were performed. The tumor was subsequently diagnosed as a pleomorphic rhabdomyosarcoma of the diaphragm.
The patient's postoperative course was uneventful. She rejected any further treatment, including chemoradiotherapy and the second-stage procedure, and was discharged from the hospital on postoperative day 7.
On macroscopic examination, the tumor consisted of multiple masses. The biggest mass measured 11 x 10 x 8 cm. The cut surface was solid and gray-tan in color. The predominant histologic appearance of the tumor was pleomorphic tumor giant cells. Mitoses were numerous (Fig 3). Immunohistochemical analysis showed the cytoplasm of the tumor cells to have diffuse strong positivity for desmin (Fig 4). Actin was positive in focal areas. Results for epithelial membrane antigen, S100 protein, and CD34 were all negative. These histopathologic findings revealed that the tumor was pleomorphic rhabdomyosarcoma.
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Comment
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Primary tumors of the diaphragm are very rare and may be benign or malignant. Most primary malignant tumors are of fibrous tissue origin or are undifferentiated sarcomas [1]. Diaphragmatic rhabdomyosarcoma is an extremely uncommon tumor. Even though diaphragmatic rhabdomyosarcomas are very rare, they should be included in the differential diagnosis of upper quadrant abdominal masses. They can occur at any age, and although rare in adults, most adult cases occur in the fourth and fifth decades of life [2].
Rhabdomyosarcoma is a malignant tumor arising in skeletal muscle and is the most common soft-tissue sarcoma in children and adolescents, accounting for 15% of soft-tissue sarcomas in the general population and for 4% to 8% of all childhood malignancies [3]. Rhabdomyosarcoma may occur at any site, but primary tumors are more commonly located in head and neck (42%), urogenital tract (34%), and extremities (11%). Alveolar tumors tend to occur primarily in the trunk and extremities, whereas embryonal tumors are more often found in the head and neck or genitourinary and paratesticular sites [4]. We present a case of diaphragmatic pleomorphic rhabdomyosarcoma in an adult woman.
Some patients with diaphragmatic tumors may be asymptomatic. The symptoms may include chest pain, cough, dyspnea, and dysphagia. In addition, tumors on the left side may cause anorexia, nausea, and vomiting due to gastric compression. Our patient had dyspnea, fatigue, and cough. Abdominal or chest wall mass, atelectasis, or pleural effusion may be detected at the physical examination. A chest roentgenogram may reveal an elevated hemidiaphragm, a localized diaphragmatic hump, or pleural effusion. Computed tomography and ultrasonography usually demonstrate a heterogeneous and enhanced diaphragmatic mass and its extension into the contiguous organs [5]. For the diagnosis, these findings are not characteristic for rhabdomyosarcoma, histologic examination is still the gold standard.
Rhabdomyosarcoma is a highly malignant tumor that originates from embryonal mesenchyme with the potential of differentiating into skeletal muscle cell. Rhabdomyosarcomas are divided into four histologic subtypes: embryonal, alveolar, pleomorphic, and botryoid. Embryonal rhabdomyosarcoma is the most common type (58%) [4]. Our case represents pleomorphic rhabdomyosarcoma of the diaphragm. In contrast with the pediatric population, rhabdomyosarcoma in adults is rare, with the alveolar subtype predominating in adult patients younger than age 25 and the pleomorphic subtype predominating in adult patients older than age of 25 [6]. Pleomorphic rhabdomyosarcoma is believed to be very rare, occurs predominantly in the extremities of adults, and its prognosis is generally poor. The tumor may contain necrotic, hemorrhagic, and microcystic areas owing to its extensive vascularity [7].
According to the Intergroup Rhabdomyosarcoma Study Group, rhabdomyosarcomas are divided into four surgical-histopathologic groups according to the extent of disease, surgical decision, and extent of resection. Group 1 has a localized disease that is completely resected, group 2 has microscopic disease remaining with or without completely resected regional nodal disease, group 3 has incomplete resection or biopsy with gross residual disease, and group 4 has distant metastases [3]. By these criteria, our patient was classified as group 4.
The treatment of rhabdomyosarcoma requires the application classic oncologic principles for mesenchymal tumors, including sufficient surgical excision, if possible, and adjuvant chemoradiotherapy. The tumor is usually large, infiltrative, and metastatic at the time of diagnosis, and metastases involve pleura and lung parenchyme [8]. Cross-sectional imaging can assess the presence of metastasis and the resectability of these tumors and thus provides important data for therapy planning.
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References
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- Merrick SH, Fishman NH. Tumors of the mediastinum, pleura, chest wall, and diaphragmIn: Baum GL, Wolinsky E, editors. 5th ed.. Textbook of pulmonary diseases. II. New York, NY: Little, Brown and Co; 1994. pp. 1415-1441.
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- Wijnaendts LC, van der Linden JC, van Unnik AJ, Delemarre JF, Voute PA, Meijer CJ. Histopathological classification of childhood rhabdomyosarcomas: relationship with clinical parameters and prognosis Hum Pathol 1994;25:900-907.[Medline]
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- Raney RB, Anderson JR, Andrassy RJ, Crist WM, Donaldson SS, Maurer HM, Intergroup Rhabdomyosarcoma Study Group Soft-tissue sarcomas of the diaphragm: a report from the Intergroup Rhabdomyosarcoma Study Group from 1972 to 1997 J Pediatr Hematol Oncol 2000;22:510-514.[Medline]
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Abstract
Introduction
