Multiple Leiomyomas of the

Diagnostic Radiology

Esophagus 1

Hubert A. Shaffer, Jr., M.D. Multiple leiomyomas of the esophagus are rare. These benign intramural, extramucosal tumors present a sufficiently characteristic appearance during a barium examination to suggest a specific roentgenologic diagnosis. Although leiomyomas may become quite large and cause considerable distortion of the esophageal lumen, they produce surprisingly few symptoms in most patients. Since these tumors have little if any malignant potential, surgical excision is necessary only in patients with significant symptoms. Four patients with leiomyomas of the esophagus are reported here, and the clinical and roentgenologic features of these tumors are reviewed. INDEX TERMS:

Esophagus, neoplasms. Myoma

Radiology 118:29-34, January 1976

• leiomyoma of the esophagus is a relatively uncommon lesion, it is by far the most frequent benign tumor which involves this structure (3). In a review of 19,982 consecutive autopsies, Plachta (10) found 504 tumors of the esophagus. Of these, 414 (82%) were malignant and 90 (18%) were benign. Of the benign esophageal tumors, 49 (54 % ) were leiomyomas. All were solitary lesions. More than 800 cases of leiomyoma of the esophagus have been reported in the world literature to date (13), but I could find only 24 instances in which multiple tumors existed (1,3, 13, 14). The purpose of this paper is to report 4 additional cases of multiple leiomyomas of the esophagus and to review the salient clinical and roentgenologic features.

Reviews of surgically treated cases suggest that the average tumor is roughly oval in shape and measures about 2-8 cm in diameter (13, 14). There are isolated reports of giant esophageal leiomyomas weighing 1,000-5,000 g (15). Symptoms: More than 50 % of patients remain asymptomatic, a figure attested to by the large number of tumors found incidentally at autopsy (1, 9, 10). Many patients exhibit great distortion of the esophageal lumen as the tumor increases in size, yet they have no difficulty swallowing. Symptoms, when present, vary in type and severity in relationship to the size and location of the tumor and to the degree to which it encircles the esophagus. The commonest presentation is dysphagia, usually mild and periodic, slowly progressive, and worse when eating solid food hurriedly. Pain is infrequent and usually retrosternal or epigastric. Weight loss and vomiting occur only in the presence of obstruction (12). Bleeding is not characteristic, since the tumor is a predominantly intramural lesion and rarely causes ulceration of the overlying mucosa (3). Malignant Potential: Esophageal leiomyomas are benign smooth-muscle tumors which have little if any tendency to undergo malignant transformation. To my knowledge, there has been only one report of a benign tumor, diagnosed histologically as a fibroleiomyoma, which contained a small focus of leiomyosarcoma (3). Associated Conditions: Esophageal hiatal hernia is reported in 4.5 % of patients with leiomyoma, 45 times the incidence in the general population (15). The coexistence of a hiatal hernia with a leiomyoma at the lower end of the esophagus predisposes to reflux esophagitis and subsequent ulceration and bleeding (6). Hypertrophic osteoarthropathy (Marie-Bamberger syndrome) has been reported in 2 patients with esophageal leiomyoma (1, 16). Both patients had clubbing of the fingers and toes, painful and swollen joints, and sub-

ALTHOUGH

H

CLINICAL FEATURES

Age and Sex Incidence: Approximately 90 % of esophageal leiomyomas are discovered in patients between the ages of 20 and 60 years. This peak incidence is about 20 years earlier than that for esophageal malignancies (1, 14). The tumors have a slight predilection for men, with some authors reporting ratios as high as 2:1 (6, 14). Location and Size: Only 5-10% of leiomyomas of the gastrointestinal tract are found in the esophagus (9). Approximately 60 % occur in the lower third, 33 % in the middle third, and 6 % in the upper third (14). Their relative rarity in the upper esophagus reflects its smaller amount of smooth muscle. The vast majority of esophageal leiomyomas are located intramurally; only 1 % present intraluminally as a polypoid lesion (13). The intramural mass is usually localized and grows eccentrically in the muscle of the esophagus. Less commonly, the tumor may surround the gullet like a collar or may be elongate and involve a long segment of the esophagus (1). 1

From the Department of Radiology, University of Virginia School of Medicine, Charlottesville, Va. Accepted for publication in March 1975. elk

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HUBERT

A.

SHAFFER

January 1976

Fig. 1. CASE I. Multiple esophageal leiomyomas. Chest roentgenograms. In the postero-anterior projection, the trachea is deviated to the right of the midline. At least three rounded soft-tissue masses protrude from the upper portion of the right mediastinum. The left middle mediastinal contour is abnormally prominent. A round mass projects into the cardia of the stomach (arrow). In the lateral projection, increased soft-tissue density in the area of the upper esophagus is associated with anterior displacement of the trachea. A left retrocardiac mass can be identified in both projections (arrows). Fig. 2. CASE I. Multiple esophageal leiomyomas. The smoothly rounded filling defects in the barium column conform to the companion soft-tissue masses (arrows). The masses moved freely during swallowing, indicating a lack of adhesions or mediastinal infiltration.

periosteal new bone formation along the long bones of the extremities. The one patient who survived had prompt and complete remission of symptoms after removal of the tumor.

A new syndrome of esophagogastric and vulvar leiomyomatosis (possibly with clitoral hypertrophy and uterine leiomyoma) has recently been described (11). The smooth-muscle tumors presented in late childhood

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Fig. 3. CASE II. Multiple esophageal leiomyomas. Postero-anterior and lateral chest roentgenograms show prominence of the right side of the mediastinum in the region of the ascending aorta and a large softtissue retrocardiac mass contiguous with the diaphragm. The trachea is deviated to the right. Fig. 4. CASE II. Multiple esophageal leiomyomas. The barium esophagograms show multiple sharply-circumscribed filling defects ln the thoracic esophagus. The edge of each tumor meets the uninvolved wall of the esophagus at a sharp angle without evidence of infiltration or undercutting at the tumor margin. The large tumor encircling the lower end of the esophagus produces mild proximal dilatation.

at multiple sites in both reported cases. This multicentric origin of histologically identical tumors in multiple organ systems lends credence to the theory that leiomyoma

may represent a hamartomatous malformation. The hamartoma theory is also a likely explanation for the simultaneous development of multiple esophageal leiomy-

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HUBERT A. SHAFFER

January 1976

Fig. 5. CASE III. Multiple esophageal leiomyomas. Postero-anterior and lateral chest roentgenograms of a man with chronic hypertensive cardiovascular disease. The round nodule (arrow) above the aortic arch in the lateral projection stimulated further investigation. Fig. 6. CASE III. Multiple esophagealleiomyomas. The esophagogram demonstrates many characteristic rounded intramural, extramucosal filling defects in the thoracic esophagus. The arrow indicates the tumor seen on the lateral chest roentgenogram.

omas which may represent hyperplasia of aberrant nodes of embryonic muscle tissue (9). Esophagoscopy: The results of esophagoscopic examination may be completely negative due to easy passage of the instrument past the tumor. More typicalIy, a firm submucosal mass with normal or flattened overlying mucosa may be seen to indent the lumen of the esophagus (3). Because of the risk of complication, biopsy through an intact, healthy mucosa is contraindi-

cated. The chief value of esophagoscopy is, therefore, not to make a histological diagnosis but to exclude a mucosal tumor (3, 7, 14). Treatment: In cases where surgery is necessary, thoracotomy and simple enucleation is the procedure of choice for the majority of esophageal leiomyomas (3, 7, 13). Occasionally a tumor will be so extensive or adherent as to require resection of a portion of the esophagus. Esophageal resection, however, carries a much

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Diagnostic Radiology

higher morbidity and mortality risk than does simple enucleation (13). Tumor recurrence following surgery is extremely rare (3). ROENTGENOLOGIC FEATURES

Because the clinical presentation of esophageal leiomyomas is quite variable, they are usually first discovered by the radiologist. The roentgenologic features of benign intramural tumors described by Schatzki and Hawes (12) and by Harper and Tiscenco (5) apply to any benign intramural tumor of the esophagus. They are not specific for leiomyoma when a solitary lesion is encountered. On the other hand, when multiple intramural tumors are present in the esophagus, the appearance is sufficiently distinctive to allow the radiologist to suggest the diagnosis of multiple leiomyomas with a high degree of confidence. Plain Chest Radiograph: If their size is sufficient to permit identification on a routine posteroanterior chest roentgenogram, these tumors produce smoothly rounded or lobulated masses projecting to one or both sides of the mediastinum (Figs. 1 and 3). Smaller tumors tend to be obscured by the normal mediastinal shadow. A lateral view usually reveals the masses to be situated in the region of the chest normally occupied by the esophagus (Figs. 1, 3, and 5). Calcification within these tumors is unusual but not rare (1, 4, 6). Esophagogram: The barium swallow is the most helpful of all studies for diagnosis of esophageal leiomyomas. When viewed in profile, the tumors classically produce smoothly rounded filling defects in the margins of the barium column with no irregularity of the overlying mucosa. The edge of each tumor meets the uninvolved wall of the esophagus at a sharp angle. There is no evidence of infiltration or undercutting at the tumor margin (Figs. 4 and 6). At the level of each filling defect, a companion soft-tissue shadow should be seen projecting beyond the esophageal outline. The long diameter of the mass conforms to that of the esophageal filling defect. The masses ascend freely with swallowing and move with peristalsis, indicating a lack of adhesions or infiltration (Fig. 2). When studied en face, the tumors appear as round or lobulated filling defects sharply outlined in relief by barium streaming around each side (Fig. 7). Although the esophageal lumen often appears narrowed, there is rarely any evidence of obstruction to the flow of barium. The mucosal folds overlying each mass are stretched, flattened, or obliterated. Normal mucosal folds may be visible, but they are folds of the wall opposite the tumor and not the folds running across the lesion. CASE MATERIAL

The roentgenograms which illustrate this article were obtained during diagnostic evaluation of four previously unreported cases of multiple leiomyomas of the esophagus. The pertinent information related to these cases

Fig. 7. CASE IV. Two leiomyomas are demonstrated in the thoracic esophagus during a barium swallow. The barium column splits and passes around each tumor when viewed en face, producing a forked-stream appearance.

is summarized in TABLE I. In this small series, ail patients are younger than the average age of patients with esophageal malignancy. There is no sex predilection. All are Caucasian. Only one had symptoms significant enough to require surgical excision of the esophageal tumors. The correct diagnosis was suggested by the radiologist in every case. Endoscopy confirmed that all lesions were of submucosal origin. Diagnosis was confirmed in 2 cases by biopsy through the intact esophageal mucosa during endoscopy. Although this was successfully performed in our patients, the general opinion expressed in the literature is that this is a risky procedure which may result in complications (3,7, 14). CASE II involves a young woman who has been followed up for 12 years without progression of symptoms while periodic barium swallows indicated a definite increase in the size and number of her esophageal lesions. It is most interesting that she had multiple uterine leiomyomas removed because they were also rapidly enlarging during this interval. The simultaneous occurrence of esophageal and uterine leiomyomas in this pa-

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HUBERT

Table I:

Age, Race, Case and Sex

Symptoms

Related Associated Conditions

A.

SHAFFER

January 1976

Summary of Cases

Correct Diagnosis Suggested Radiologically

Esophagoscopic Findings

Treatment

1

22 WF

None

None

Yes

2

28WF

Life-long moderate dysphagia

Uterine leiomyomas

Yes

3

54WM

None

None

Yes

None

4

43WM

Severe dysphagia for 3 months

None

Yes

Thoracotomy with tumor enucleation

tient supports the theory that these tumors may represent hyperplasia of aberrant nodes of embryonic muscle tissue and may, therefore, be hamartomas (9). ACKNOWLEDGMENTS: I am grateful to Drs. James L. Lynde. Johnsey L. Leef. William L. Crawford. and Anne C. Brower for their assistance in obtaining case material for this article.

REFERENCES 1. Barrett NR: Benign smooth muscle tumours of the oesophagus. Thorax 19:185-194; Mar 1964 2. Glanville IN: .Leiomyomata of the oesophagus. Clin Radiol 16:187....:190. Apr 19,65 3. Godard JE, McCranie D: Multiple leiomyomas of the esophagus. Am J RoehtgenoI117:259-26~. Feb 1973 4. Gutman E: Posterior mediastinal calcification due to esophageal ieiomyoma. GastroenteroI63:665-666. Oct 1972 5. Harper RA, Tiscenco E: Benign tumour of the oesophagus and its differential diagnosis. Br J RadioI18:99-107, Apr 1945 6. Huddy P, Griffiths G: Leiomyoma of the oesophagus with calcification. Br J Surg 59:239-242. Mar 197~ 7. Kostlalnen S, Virkkula L, Teppo L: Smooth muscle tumours of the oesophagus. Scand J Thorac Oardlovasc Surg 7:98-103. 1973 8. Nahmad M. Clatworthy HW Jr: Leiomyoma of the entire esophagus. J Pediatr Surg 8:829-839, Oct 1973

None Multiple firm submucosal masses covered by normal mucosa in all cases

None

Pathologic Diagnosis Biopsy during mediastinoscopy Biopsy through esophageal mucosa

Biopsy through esophageal mucosa Surgical specimen

9. Piacentini L: Leiomyoma of the esophagus. J Thorac Surg 29:296-316,Mar 1955 10. Plachta A: Benign tumors of the esophagus. Review of literature and report of 99 cases. Am J Gastroenterol 38:639-652, Dec 1962 . 11. Schapiro RL, Sandrock AR: Esophagogastric and VUlvar leiomyomatosis: a new radiologic syndrome. J Can Assoc Radiol 24:184-187, Jun 1973 12. Schatzki R, Hawes LE: The roentgenological appearance of extramucosal tumors of the esophagus. Am J Roentgenol 48: 115, Jul 1942 13. Seremetis MG, De Guzman VC. Lyons WS, et al: Leiomyoma of the esophagus. A report of 19 surgical cases. Ann Thorac Surg 16:308-316. Sep 1973 14. Storey CF, Adams WC: Leiomyoma of the esophagus; report of 4 cases and review of surgical literature. Am J SUfg 91:3-23, Jan 1956 15. Tsuzuki T, Kakegawa T, Arimori M, et al: Giant leiomyoma of the esophagus and cardia weighing more than 1.000 grams. Chest 60:396:-399, Oct 1971 16. Ullal SR: Hypertrophic osteoarthropathy and leiomyoma of the esophagus. Am J Surg 123:356-358, Mar 1972

Department of Radiology University of Virginia School of Medicine Charlottesville. Va. 22901

Multiple leiomyomas of the esophagus.

Multiple leiomyomas of the esophagus are rare. These benign intramural, extramucosal tumors present a sufficiently characteristic appearance during a ...
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