Giant Esophageal Leiomyoma

John C. Deutsch, M.D.

 

Keywords

Esophagus, leiomyoma, endosonography

Methods for EUS Capture

Olympus GFUM 30 at 7.5 and 12 MHz at radius of 4-9 cm using water filled balloon

Case/Body

Figure 1A

Figure 2A

Figure 3

Case Report History: A 25 year-old male presented to his physician with an 8-year history of dysphagia. The patient had lost 40 lbs over this time. Recently he had been experiencing dyspnea and blackout spells during heavy physical labor. His physician obtained a chest x-ray, revealed a large mediastinal mass. The patient was referred for thoracic surgery. The thoracic surgeon requested an endoscopic ultrasound (EUS) to further define the lesion prior to surgery. Physical: The physical examination revealed a somewhat obese, but healthy appearing male. His lungs were clear to auscultation and his cardiac exam was unremarkable. No supraclavicular adenopathy was appreciated. A fine needle aspiration was performed under EUS guidance using Pentax linear array equipment. There was no evidence of malignancy with the fine needle aspiration, but a definitive diagnosis was not made. Surgery: A large tumor was dissected from the esophageal muscularis. The mucosal and submucosal esophagus was left intact, with the hope that the remaining vasculature would support esophageal viability. Pathologic evaluation: Three specimens of resected tumor were sent to the pathology laboratory. Two smaller samples were 1.3 x 0.8 x 0.4 cm and 3.5 x 1.5 x 1.4 cm. The largest tumor specimen was 15 x 11.5 x 7 cm in size and weighed an estimated 800 grams. All consisted of bland swirls of spindle cells without evidence of mitotoic activity. During the histologic evaluation, the tumor was found to be S-100 and MSA negative The final diagnosis was benign leiomyoma. Follow-up: The patient developed a postoperative leak of the mucosal esophagus into the thorax. On surgical re-exploration the patient was found to have necrosis of his intrathoracic esophagus. Diversion of the esophagus was performed with an external cervical esophagostomy and a separate gastrostomy. Upon resolution of the inflammation and infection that had resulted from the leakage of esophageal contents, the patient underwent esophageal repair. The patient had a gastric pull up procedure with a cervical esophagogastrostomy and was discharged on a pureed diet with further recovery expected.

Discussion/Summary Statement

Discussion: Esophageal leiomyomas are usually small submucosal lesions found incidentally at endoscopy. Biopsy of a suspected leiomyoma does not have to be performed, and could potentially compromise surgical removal. Less invasive surgical techniques are the procedure of choice for therapy (1). The EUS evaluation yields a relatively characteristic picture, showing a hypoechoic lesion arising from the hypoechoic musularis propria (2). The case described above is an extremely unusual presentation in which cardiac outflow appeared to be compromised, and the vascular integrity of the esophagus was compromised. Previously, a report of EUS evaluation of a giant esophageal leiomyoma has been reported (3). In the case presented by Izumi et al, the tumor bulk was one-sixth that seen in this case, highlighting the massive size in our patient. In both cases, EUS evaluation showed some characteristics of the tumor that were useful in making the diagnosis, including the fact that the tumor appeared to originate in the muscular layers. EUS was additionally useful in that our patient had a biopsy that did not support the diagnosis of a high-grade malignancy. If biopsy had shown a high-grade malignancy, chemotherapy with or without radiation therapy would have been the primary modality of choice. EUS is the preferred modality to be used in the evaluation of submucosal lesion of the esophagus (2), and should be employed early in the evaluation of these type of lesions.

References

1. Bonavina L, Segalin A, Rosati R, Pavanello M, Peracchia A. Surgical therapy of esophageal leiomyoma J Am Coll Surg 1995:181(3):257-262.

2. Tio TL, Tygat GN, den Hartog Jager FC. Endoscopic ultrasonography for the evaluation of smooth muscle tumors in the gastrointestinal tract: an experience with 42 cases Gastrointest Endosc 1990;36(4):342-350.

3. Izumi H, Nakahara K, Mikami K, Obata K, Danbara T, Okazaki S, Masuda S A resected case of esophageal leiomyoma 15 cm in long diameter diagnosed by transesophageal ultrasonic endoscopy. Kyobu Geka 1994;47(13):1075-1077

 

 




Editorial Board:
Manoop S. Bhutani, M.D.
Galveston, TX
William R. Brugge, M.D.
Boston, MA
Peter R. McNally, D.O.
Denver, CO
Iqbal S. Sandhu, M.D.
Salt Lake City, UT
Thomas J. Savides, M.D.
San Diego, CA

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