Scand J Thor Cardiovasc Surg 7: 98-103, 1973

SMOOTH-MUSCLE TUMOURS OF THE OESOPHAGUS

Seppo Kostiainen, Lauri Virkkula and Lyly Teppo

From the Department of Thorncic Surgery, University Central Hospital, and rhe Second Department of Pathology, Universiry of Helsinki, Firtlund

Abstract. A series of 11 patients with leiomyoma and one with leiomyosarcoma of the oesophagus is presented. Dysphagia and pain were the main symptoms both in the group of leiomyomas and in the case of leiomyosarcoma. Plain chest roentgenograms revealed the leiomyosarcoma and the leiomyoma in 6 patients. Marked calcification was demonstrated both roentgenologically and histologi- cally in one leiomyoma. One leiomyoma was intra- luminal and pedunculated, thc others were intramural and sessile. There was no mortality after surgical re- moval of the tumours. The patients with leiomyomas were asymptomatic 6 months to 18 years after operation. T h e patient with leiomyosarcoma died 1 year after opera- tion from a recurrence of the tumour.

Smooth-muscle tumours of the oesophagus are rare. Up to 1959, a total of 389 cases, of which 351 were leiomyomas and 38 leiomyosarcomas, had been reported in the literature (Gray et al., 1961). 207 of the leiomyomas and 29 of the leio- myosarcomas belonged to surgical materials and the rest were autopsy findings. The ratio between treated oesophageal leiom yomas and carcinomas in clinical materials varies from 1 : 38 to 1 : 128 (Table I). Oesophageal leiom yomas have been encountered in 0.07-0.4% of autopsy materials (Piacentini, 1955; Plachta, 1962; Attah & Hajdu, 1 968).

MATERIAL Eleven patients were treatcd for oesophageal leiomyoma and 1 patient for leiomyosarcoma at our clinic during the period 1945-1970.

Leiom yomas

There were 3 females and 8 males among the patients with leiomyoma. Ages were ranged from 22 to 61 years, mean 44 years.

Symptoms. The only patient without symptoms was a 22-year-old man; the tumour was observed at an X-ray

Scand J Thor Cardiowsc Surg 7

examination of the lungs for a respiratory infection (Fig. 1).

The symptoms were usually dysphagia and substernal or epigastric pain (Table 11). The dysphagia was mild and only 2 patients had lost a little weight. When the tumour was localised in the middle third of the oe- sophagus, the pain was substernal and radiated dorsally at the height of the scapulae. Tumours of the lower third of the oesophagus caused pain in the upper epigastrium and in the region of the left costal margin. The pain was intermittent and often occurred postprandially. In 3 patients with regurgitation the tumour was close to the cardia. The tumour of the 2 patients with dyspnoea was at the height of the arch of the aorta and displaced the bifurcation of the trachea. A general characteristic of the symptoms was that they were mild, of long duration and that no definite aggravation of the symptoms occurred even in the course of several years. The duration of the symptoms was &-15 years, mean 5 years.

One patient had a 2-3-year history of recurrent anae- mia. She had been admitted to the local hospital for melaena and anaemia (Hb 63 g/l) 2 months before the operation. The patient was found at operation to have a pedunculated turnour measuring 2 x 2 x 3 cm in the car- dia; its narrow peduncle was fixed on the side of the oesophagus. At the distal end of the tumour there was a 3 x 5 mm ulcer, which had probably caused the inter- mittent bleeding and anaemia.

Clinical findings. Plain chest roentgenogram revealed the tumour in 6 patients as a rounded mediastinal mass (Fig. 1); in one case marked calcification was seen in the tumour (the symptoms of this patient had lasted for 15 years). The turnour was visualised on oesophagography as a typical filling defect (Fig. 2) in all patients except the onc with a pedunculated tumour extending to the cardia and another whose tumour was localized in an extensive epiphrenic diverticulum. No noteworthy dilata- tion was seen in the oesophageal lumen on the oral side of the tumour and the passage of the contrast medium past the tumour was unobstructed, although it seemed at oesophagography to obstruct the oesophagus.

In 6 patients no turnour was seen oesophagoscopically. In the other 5 patients a sm2oth-surfaced tumour push- ing into the lumen was detected. The mucosa on the tu- mour appeared to be intact. In one case the turnour at the height of the arch of the aorta moved in rhythm with

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Smooth-muscle tumours of the oesophagus 99

Table I. Number of leiomyomas and carcinomas in four clinical series of oesophageal tumours. The figures indicate the number of patients treated during a certain period

Leio- Carci- Leiomyomas: Authors myomas nomas Carcinomas

Petrovsky & Vantsian, 1967 28 1065 1:38

Johnston et al., 1953 18 2312 1:128 Dillow et al., 1970 11 430 1:39 Our series 11 530 1:48

aortic pulsation, leading to suspicion of aneurysm of the aorta. This possibility was eliminated by aortography.

Localization and macroscopic findings of the tumours. All the tumours were situated in the middle or lower third of th3 oesophagos (Table 111). Seven were lobulated and partially surrounded the lumen of the oesophagus. The other four were round or oval. The tumours ranged in length irom 3 to 10 cm, and weighed between 10 and 100 g. All the tumours were solid, clearly demarcated from the surroundings and the cut-surfaces were grey or reddish.

Hrktologic findings. The histologic specimens of 8 pa- tients were available for re-evaluation. In all the tumours, the overall structure was that of a typical leiomyoma: interlacing bundles of spindle-shaped cells with elongated nuclei and without any cellular or nuclear atypia (Fig. 3). In one tumour, slight fibrosis was observed, and this was the only one which had a definite collagenous

Table 11. Clinical symptoms of patients with leiomy- oma of the oesophagus in the series of I66 surgically treatedpatients reported by Gray et al. (1961) and in our series of I 1 patients

Our series

Gray et al. No. of Symptoms (% of cases) cases %

Dysphagia 48 8 73

Regurgitation - 3 27

Haemorrhagia 3 1 9 Asymptomatic 13 1 9

Pain 48 7 64

Loss of weight 20 2 18 Dyspnoea - 2 18

capsule. Degenerative myxoid areas were seen in two tu- mours, and one of these also exhibited necrotic focuses without inflammatory reaction (this tumour was the largest in our series). Inflammatory cells were encoun- tered in three tumours. In 2 cases the cells were mono- nuclear (plasma cells and lymphocytes) and were situated predominantly along the small vessels in the tumour. In the third there were large amounts of eosinophils, both in the perivascular areas and within the tumour tissue proper. No special features could be observed in the history or clinical findings of these 3 patients. Calcifica- tion was observed in two tumours. One was markedly cal- cified, while in the other there were only some minute deposits in the periphery of the tumour.

Fig. 1. Clearly demarcated round shadow caused by an oesophageal leiomyoma in the lower mediastinum in the plain chest roentgeno- gram.

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100 S . Kostiainen et al.

Table 111. Localization of the tumow in the series of 345 patients reported by Gray et al. (1961) and in our series of I1 patients with leiomyoma of the oesophagus

Our series

Gray et al. No. of Localization (% of cases)a cases %

- - Upper third 7 Middle third 35 6 55 Lower third 55 5 45

a Localization not indicated: 3 24.

Plain chest rJentgenogram revealed a fist-sized dense infiltrate in the mediastinum behind the shadow of th: heart. The bifurcation cf the trachea had spread and was displaced ventrally and cranially. The tumour was found at oesophagography to displace and obstruct the oe- sophagus (Fig, 4). Oescphagoscopy showed that the mu- cusa on the tumour pushing into the lumen was intact.

Fig. 2. Typical semilunar filling defect caused by leio- myoma, seen ;it oesophagography.

Treafment onti prognosis. Enucleation of the tumour was performed in 9 cases. &SophagOgdStnC resection Was performed on the patient whose tumour was in a n epi- phrenic diverticulum. The pedunculated turnour extending to the cardia was removed through the stomach by exci- sion at the base of the peduncle. The oesophageal mu- cosa was opened in two enucleations, but this did not affect the recovery of the patient.

The patients made a normal postoperative recovery with the exception of a mild infection of the thoracotomy wound in one case. The only late complication was post- operative diaphragmatic hernia in the patient undergoing oesophagogastric resection. The hernia was repaired 18 years after the primary operation. The patients were asymptomatic in the oesophagus at follow-up &18 years after the enucleation of the tumour.

Leiom yosarcoma

Report of a Case The patient was a 73-year-old woman who had had pain substernally and in the scapular region, difficulty in Fig. 3. Leiomyoma of the oesophagus: spindle-shaped swallowing, slight loss of weight and dyspnoea for 3 cells with elongated nuclei, no atypia. There is fibrous months. tissue between the smooth-muscle cells. H.-E., x 440.

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Smooth-muscle tumours of the oesophagus 101

Fig. 4. Ossophagography reveals a large leiomyosarcoma which displaces and obstructs the oesophagus.

Puncture of the tumsur was performed via the trachea at bronchoscopy. Histologic study showed malignant tumour tissue of mesenchymal origin in the specimen.

At operation, an oval, nodular, grey tumour was seen to be attached to the wall of the oesophagus with a pedicle the size of a finger. The solid tumour measuring 5 x 5 x 8 cm was level with the middle third of the oe- sophagus. Neither infiltrative growth into the environ- ment nor mediastinal metastases were encountered. The tumour was removed by excision down to healthy tissue. A more radical operation was not regarded as indicated in view of the patient’s age and poor general condition; also the tumour appeared on gross examination to have been enucleated completely.

At microscopy, the tumour was highly cellular, com- posed of spindle-shaped cells with moderate variation in the size and shape of the nuclei and numerous, some- times pathologic mitoses (Fig. 5). No clear-cut infiltration could be demonstrated. Necrosis was not observed. The diagnosis leiomyosarcoma was based on the pleomorphisrn of the tumour and the number of the mitoses.

The patient made an uneventful postoperative recovery and felt well for half a year. Dysphagia and pain felt substernally and in the scapular region then began again. A fast-growing node appeared at this juncture in the left supraclavicular fossa; it looked like a metastasis. Oe- sophagography revealed an obstruction caused by a local

Fig. 5. Leiomyosarcoma of the oesophagus: highly cel- lular tumour tissue with numerous mitoses (3 in the fig- ure). Cellular atypism is not very marked in this area. H.-E., x 440.

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102 S . Kostiainen et al.

Table IV. Histologic types in a series of 432 benign tiimours and tumour-like lesions of the oesophagus reported by Plachta (1962)

No. of 01 Histologic type tumours /a

Myoma Polyp cyst Papilloma Fibroma Hemangioni Lipoma Adenoma Others

Total

225 108 34 14 13

7 4

18

432

d 9

52.1 25.0

1.9 3.2 3.0 2.1 1.6 0.9 4.2

100.0

tumour recurrence. The mucosa at the obstruction was found by biopsy taken at the oesophagoscopy to be nor- mal. The patient died 1 year after the operation from re- currence of the tumour.

DISCUSSION

Leiomyoma is the commonest benign tumour of the oesophagus. There were 225 (52%) myomas in the material of 432 benign oesophageal tu- mours reported up to 1960 which Plachta col- lected. They were, with a few exceptions, leio- myomas (Table IV). 5-10% of leiomyomas of the gastrointestinal tract are localized in the oe- sophagus (Piacentini, 1955). In the large collected material published by Gray et al. (1961), the tu- mour was in a typical case intramural, solitary and spherical, oval or rod-like in shape. When rod-like it partly encircled the oesophagus. Only 4% of the tumours were pedunculated and intra- luminal, like the case in our own series.

The age distribution and symptoms of the pa- tients (Table 11) in the material described by Gray and his co-workers agreed with those ob- served in our own material, but the proportion of men (1.8 : 1.0) in their work was smaller than in our series (2 7 : 1.0). Attention is attracted by the rare occurrence of bleeding in connection with oesophageal leiomyoma compared with its fre- quency in association with other leiomyomas of the gastrointestinal tract. Haemorrhage has been established %hen the tumour is close to the cardia and damages its sphincter mechanism, resulting in regurgitation from the stomach causing in- flammation and ulceration on the oesophageal mucosa. Scand J Thor Cardiovasc Surg 7

The tumour was visualised in plain chest roentgenograms in 6 (55%) of our patients. This is a considerably higher proportion than in larger materials in which visualisation of the tu- mour has been reported in 8-18% (Johnston et al., 1953; Gray et al., 1961; Deverall, 1968). An interesting finding was the appearance of numer- ous calcified foci in the plain chest roentgeno- gram. This can be regarded as a differential diagnostic sign: calcification of the tumour speaks against malignancy. A typical oesophagographic finding is a round, sharply demarcated filling defect, semilunar in the lateral exposure, which moves with the oesophagus during the act of swallowing. There is usually no dilatation above the tumour and the mucosa over the tumour is stretched smooth (Schatzki & Hawes, 1950; Glanville, 1965). Unlike most epithelial tumours, oesophagoscopy does not provide a histologic diagnosis in leiomyomas. A biopsy specimen from the intramural tumour taken through the mucosa is seldom diagnostic and the risk of complication in the stretched oesophageal wall is great (Pera- salo & Laustela, 1955; Gray et al., 1961). Hence, no biopsy specimen is taken as a rule when the mucosa is of normal appearance.

The first to remove a leiomyoma was Sauer- bruch (1932). The treatment was oesophagogastric resection, depending on the size and localisation of the tumour. I t is usually possible to remove the tumour by enucleation, such as was first per- formed by Ohsawa (1933). The mortality rate in the enucleated cases has ranged from 1 to 2 % , in all surgically-treated cases from 4 to 5 % (Gray et al., 1961; Watson et al., 1967; Dillow et al., 1970).

The majority of the patients in the material of 38 oesophageal leiomyosarcomas published up to 1959, and collected by Gray et al. (1961), were 5&58 years old, 60% of them men. One-third of the tumours were intraluminal and 54% were in the lower thud of the oesophagus. The com- monest symptoms were dysphagia (75 %), weight loss (50%) and pain (45%). The symptoms were characterized by short duration (1 month to 1 year) and fast deterioration. Oesophagography revealed in eight cases infiltration of the mucosa by the tumour; the finding in the other cases resembled that made in leiomyoma, as in our own case. A histologic diagnosis was established in six of the 15 biopsies made at oesophagoscopy;

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Smooth-muscle tumours of the oesophagus 103

the tumour infiltrated the mucosa in these cases. In our own case, diagnosis of malignant mesen- chymal tumour was arrived at by puncture of the tumour at bronchoscopy. Twenty-one of the 38 cases described by Gray et al. were treated opera- tively (16 oesophagogastric resections, two resec- tions and three local excisions). The prognosis of operable leiomyosarcomas has been fairly good, but some of the tumours regarded as sarcoma may have been leiomyomas, as it is sometimes extremely difficult to distinguish histologically between a benign and malignant muscle tissue tu- mour. Fourteen (67%) of the 21 patients oper- ated on had been followed up for 1-7 years. The patient followed up for 7 years without any sign of recurrence had undergone local excision only.

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Dillow, B. M., Neis, D. D. & Sellers, R. D. 1970. Leio- myoma of the esophagus. Amer J Surg 120, 615.

Glanville, J. N. 1965. Leiomyomata of the oesophagus. Clin Radio1 16, 187.

Gray, S. W., Skandalakis, J. E. & Shephard, D. 1961. Smooth muscle tumors of the esophagus. Znt Abstr Surg 113, 205.

Johnston, J. B., Clagett, 0. T. & McDonald, J. R. 1953. Smooth-muscle tumours of the oesophagus. Thorax 8, 251.

Ohsawa, T. 1933. Surgery of the oesophagus. Arch Jap Chir 10, 605.

Perasalo, 0. & Laustela, E. 1955. Benign muscle wall tu- mours of the oesophagus. Ann Chir Gyn Fenn 44, 145.

Petrovsky, B. V. & Vantsian, E. N. 1967. Our experience in the surgical treatment of malignant and benign esophageal tumors. Surgery 62, 833.

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