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J Thorac Cardiovasc Surg 2002;124:196-197
© 2002 The American Association for Thoracic Surgery

Brief Communications

Primary myxofibrosarcoma of the esophagus

From the Division of Cardiothoracic Surgery, Emory University School of Medicine, Atlanta, Ga.

Received for publication Dec 26, 2001. Accepted for publication Jan 11, 2002. Address for reprints: Joseph I. Miller, MD, Division of Cardiothoracic Surgery, Emory University School of Medicine, 1365 Clifton Rd, NE, Atlanta, GA 30322 (E-mail: jmill10{at}emory.edu).

Primary sarcomatous lesions of the esophagus are unusual. Of these lesions, leiomyosarcoma is the most common primary sarcoma of the esophagus, whereas rhabdomyosarcoma is rare. ¹ Other reported histopathologic diagnoses include fibrosarcoma, chondrosarcoma, and liposarcoma. ^2,3 Here we describe the first reported case of a primary myxofibrosarcoma of the esophagus presenting in a woman with a long-standing history of scleroderma.

Clinical summary

The patient is a 40-year-old white woman with a medical history of scleroderma who reported a several year history of dysphagia. She described this as intermittent sticking, primarily of solid foods, after swallowing. Results of physical examination of the patient were unremarkable. The patient's lung fields were clear to auscultation, and there was no cervical or supraclavicular adenopathy. A barium swallow was performed and demonstrated a smooth 5 x 3-cm mass with intact mucosa and well-defined margins involving the posterolateral wall of the distal esophagus (Figure 1). The mass was located 8 cm proximal to the gastroesophageal junction. The remainder of the examination was unremarkable. The esophagus exhibited normal motility and mucosal pattern, with no esophagitis or strictures. Computed tomography of the chest also demonstrated the lesion, which was hypoattenuating and well circumscribed. No associated lymphadenopathy or metastatic lesions were detected. Esophagoscopy with endoscopic ultrasonography demonstrated a submucosal mass at 32 cm, which appeared to arise from the muscularis propria. The ultrasonographic image was heterogeneous. A primary leiomyoma of the esophagus was suspected because of the radiographic appearance of the submucosal lesion. An exploratory thoracotomy for planned local resection was recommended, and the patient agreed to proceed.

View larger version (100K): [in this window] [in a new window]   Fig. 1. Barium swallow demonstrating smooth, well-circumscribed 5 x 3-cm submucosal mass in right posterolateral distal esophagus. Motility and overlying mucosal pattern were normal, and there was no evidence of esophagitis or stricture.

On gross examination, the specimen was a red-tan mass measuring 5 x 4 x 3 cm and weighing 28 g (Figure 2). Sectioning of the specimen revealed a white, glistening surface with myxoid changes. Under microscopic examination, the tumor was found to be composed of spindle cells staining positive for CD34 and smooth muscle actin. Stains for S-100, C-kit, and desmin were negative. The histopathologic diagnosis for the tumor was grade I to II myxofibrosarcoma, with the closest margin being 0.2 cm from the tumor.

View larger version (56K): [in this window] [in a new window]   Fig. 2. Photograph of gross specimen after resection. Excised tumor was a red-tan mass measuring 5 x 4 x 3 cm and weighing 28 g. Sectioning of specimen revealed white, glistening surface with myxoid changes. Final histopathologic diagnosis of tumor was grade I to II myxofibrosarcoma.

Myxofibrosarcoma is a distinct entity within the category of myxoid mesenchymal sarcomas with fibroblastic differentiation. It occurs most frequently within the extremities of older patients, usually in the subcutaneous tissues rather than in deep soft tissue compartments. ⁴ It has not to our knowledge been previously reported to occur within the esophagus. Low-grade myxofibrosarcoma, as seen in this case, typically has an indolent course and low metastatic potential, although it does have a tendency to recur locally. ⁵ The relationship between the patient's tumor and her scleroderma is unclear; they may simply be coincidental findings, because the preoperative evaluation of the esophagus did not demonstrate significant involvement with a chronic inflammatory process.

Long-term survival has been reported after resection of esophageal sarcomas, and this appears to be the treatment of choice for patients without disseminated disease. ¹ In this patient, because the tumor was initially suspected to be an esophageal leiomyoma, local excision was carried out at the time of the patient's thoracotomy. Because of the indolent behavior of low-grade myxofibrosarcomas, close observation was offered to the patient as an alternative to repeated thoracotomy for esophagectomy. If the tumor recurs locally, the patient will require a formal resection, because low-grade myxofibrosarcomas may progress to a higher grade with greater metastatic potential. ⁵

References

1. Postlethwait RW. Surgery of the esophagus. 2nd ed. New York: Appleton-Century-Crofts; 1986.
   
2. Yaghmai I, Ghahremani GG. Chondrosarcoma of the esophagus. Am J Roentgenol. 1976;126:1175-7.[Abstract]
   
3. Mansour KA, Fritz RC, Jacobs DM, Vellios F. Pedunculated liposarcoma of the esophagus: a first case report. J Thorac Cardiovasc Surg. 1983;86:447-50.[Abstract]
   
4. van Roggen JF, McMenamin ME, Fletcher CD. Cellular myxoma of soft tissue: a clinicopathological study of 38 cases confirming indolent clinical behaviour. Histopathology. 2001;39:287-97.[Medline]
   
5. Mentzel T, Calonje E, Wadden C, Camplejohn RS, Beham A, Smith MA, et al. Myxofibrosarcoma: clinicopathologic analysis of 75 cases with emphasis on the low-grade variant. Am J Surg Pathol. 1996;20:391-405.[Medline]
   

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This Article Full Text (PDF) Alert me when this article is cited Alert me if a correction is posted Services Email this article to a friend Similar articles in this journal Similar articles in PubMed Alert me to new issues of the journal Add to Personal Folders Download to citation manager Author home page(s): Joseph I. Miller Permission Requests Citing Articles Citing Articles via HighWire Citing Articles via Google Scholar Google Scholar Articles by Song, H. K. Articles by Miller, J. I. Search for Related Content PubMed PubMed Citation Articles by Song, H. K. Articles by Miller, J. I. Related Collections Esophagus - cancer