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Smooth muscle tumours of the upper gastrointestinal tract
Clin. Radiol. (1978) 29, 407-414 SMOOTH MUSCLE TUMOURS OF THE UPPER GASTROINTESTINAL TRACT PAUL M. DAVIES
The Department of Radiology, The Royal Surrey County Hospital, Guild¢brd The clinical and radiological features and endoscopic findings in 13 patients with smooth muscle tumours of the upper gastrointestinal tract are reviewed. The study emphasises the non-specific nature of the symptomatology and exemplifies a number of aspects of the radiological picture of these disorders. It also illustrates some of the problems that can arise in their investigation, by radiological and endoscopic methods, and gives some indication of the favourable prognosis that may obtain when complete surgical removal of the tumour is possible.
Smooth muscle tumours of the gastrointestinal tract are uncommon, but can develop in any site. They are more often found in the stomach than in the oesophagus and occur much more rarely in the duodenum and elsewhere in the bowel. Some, especially when small and benign, do not produce any symptoms and their presence may only be revealed incidentally at autopsy, surgery or radiological investigations of other conditions. In other instances symptoms may be produced which, although often initially ill-defined and mild may be the precursors of serious disorder.
Jensen (1967) describing 'spindle-cell sarcoma of the stomach' (a term he uses collectively for fibrosarcoma, neurosarcoma and leiomyosarcoma) refers to opinions that a malignant tumour of this type may run a clinically benign course and that sometimes the reverse may also obtain. Faegenburg et al. (1975) describe leiomyoblastomas as generally pursuing a benign course,
As they enlarge, smooth muscle tumours may expand into the lumen of the gastrointestinal tract or they may extend outside this structure. Intraluminal growth may be associated with the development of ulceration in the mucosa overlying the turnout. The mode of growth and the presence of PATHOLOGY mucosal changes are important factors which influence The tumours usually originate in the muscle wall the degree of difficulty with which these neoplasms and only rarely from the muscularis mucosae. Leio- can be demonstrated by radiological and endoscopic myomas and leiomyosarcomas show a spindle-cell methods. Structure, but leiomyoblastomas (bizarre or epithelioid leiomyomas), the most uncommon type, are MATERIAL AND METHODS characterised by areas of round and polygonal cells The present study is concerned with 13 patients (Morson and Sobin, 1976). Considerable difficulty is often experienced in differentiating histologically with smooth muscle tumours arising in either the between the benign and malignant types of smooth oesophagus, stomach or duodenum. They were muscle tumour. Indeed, Morson and Dawson (1972) unselected and included all those with histological discussing gastric tumours state that the only reliable proof of a leiomyomatous tumour of the upper evidence of leiomyosarcoma is the presence of meta- gastrointestinal tract, investigated and treated during stases. They also indicate it may not be possible to a 12-year period (April 1965 March 1977) in the distinguish a neurllemmoma (schwannoma) from a three major hospitals of what is now the South West leiomyomatous growth. Opinions differ as to whether Surrey Health District. The study was directed all leiomyosarcomas are malignant from the outset, or principally to the clinical and radiologically aspects if they sometimes occur as a result of a malignant of the disorders investigated, but endoscopic and transformation of a leiomyoma. According to Glanz follow-up findings were also recorded. and Grtinebaum (1977) the question remains open. All the patients were treated by surgery except Leiomyosarcomas are often of extremely slow for one (case 4) who had evidence of metastatic growth and low grade malignancy. On occasion, spread from an oesophageal leiomyosarcoma at the however, they may show rapid local invasiveness and time of referral. In this instance the diagnosis was also early blood stream metastases and then develop made from a biopsy taken durir~g oesophagoscopy general signs and symptoms of malignant disease. and treatment was by radiotherapy and chemotherapy.
408
CLINICAL
RADIOLOGY
Fig. 1 - Case 11. Large gastric leiomyoma. A barium meal seven years after resection of this tumour showed no evidence of recurrence.
RESULTS
The average age of the patients in the series was 58.8 years and thus accords with the findings in other series of a peak incidence in middle age. In contradistinction to the usual absence of any marked difference ~in sex incidence 11 of the 13 subjects were males. Histological findings indicated that five of the tumours were leiomyomas and eight were leiomyosarcomas; one of these latter being of anaplastic type. All the smooth muscle tumours discovered were single, but one was found lying in close proximity to a gastric adenocarcinoma. One oesophageal leiomyoma was found in association with a hiatus hernia, thus serving as a reminder
that hiatus hernia has been stated to predispose to the formation of smooth muscle tumours in the lower third of the oesophagus (Gray et al., 1961). No polypoid tumours or growths showing radiologically demonstrable calcification were encountered (Huddy and Griffiths, 1972); neither were any giant tumours of the type described by Tsuzuki et al. (1971) although the very large leiomyoma in case 11 appeared to approach this category (Fig. 1). The results of the study are summarised in Tables 1 - 4 wherein it will be noted that the tumours are classified as oesophageal, gastric and duodenal. Case 5 who had a tumour with both oesophageal and gastric components presented with dysphagia and was thus classified with the oesophageal group.
Table 1 - Oesophageal tumours- clinical features Case No. His t ology
Sex Age
Dysphagia
1
M
Leiomyoma
40
2 Leiomyoma
M 53
3 Leiomyoma
M 49
4 Leiomyosarcoma
M 57
+
5 Leiomyosarcoma
F 76
+
Pain
+
Other dyspeptic symptoms
Lassitude
Cough
Weight loss
+
+
+
+
+
+
Haemoglobin (g/d O
14.8 +
Not known
-
15.7
+
10.0 13.0
SMOOTH MUSCLE TUMOURS
OF THE UPPER GASTROINTESTINAL
TRACT
409
Table 2 - Gastric and duodenal tumours - clinical features
Case No. His tology
Sex Age
Melaena
Pain
Other dyspep tie symptoms
Lassitude
Weigh t loss
6 Leiomyoma
M 44
+
7 Leiomyosarcoma (gastric)
F 66
+
+
+
8 Leiomyosarcoma (gastric)
M 61
+
+
-
9 Leiomyosarcoma (gastricanaplastic)
M 64
+
+
+
10 Leiomyosarcoma (gastric and carcinoma)
M 72
-
-
+
11" Leiomyoma (gastric)
M 60
-
+
+
-
12 Leiomyosarcoma (duodenal)
M 69
+
+
+
+
-
13 Leiomyosarcoma
M 64
+
+
-
Vomiting
+
-
+
Haemoglobin (g/dO
8.1 7.9
7.5
+
-
10.7
. +
+
10.7
8.9
-
6.0
10.1
* This p a t i e n t also had a palpable mass and was the only p a t i e n t to e x h i b i t this clinical sign. He was also the only pa t i e nt with a h isto ry o f previous gastric surgery, having had a g a s t r o t o m y and removal of a small benign a d e n o m a five years before the present referral.
Table 3 - Oesophageal t u m o u r s - radiological features and endoscopic findings
Case No. His tology
Preoperative X-ray findings
Oesophagoscopy
Chest radiograph
Barium studies
Case 1 Leiomyoma
Mediastinal mass
Reflux. Narrowing lower oesophagus. Slight hold up. ? extrinsic t umour (Ba. swallow elsewhere ? achalasia)
No tumour. Findings compatible with achalasia
Case 2 Leiomyoma
Normal
Reflux + hiatus hernia only
Normal
Case 3 Leiomyoma
Mediastinal mass
Filling defect mid-oesophagus. ? benign t u m o u r
? Leiomyoma. ? gastrogenic cyst
Case 4 Leiomyosarcoma
Mediastinal mass
Filling defect mid-oesophagus. ? glandular mass or l e i omyos a rc oma
Tumour. Biopsy = leiomyosarcoma (also b r o n c h o s c o p y = mass bulging at carina)
Case 5 Leiomyosarcoma (oesophagogastric)
Normal
Obstruction at cardia shown on Ba. swallow done elsewhere - urgent oesophagoscopy suggested appearances confused by retained food and fluid. Possible carcinoma or achalasia. Soft tissue swelling noted below diaphragm nature uncertain
Probable stricture lower oesophagus b u t e xa mi na t i on difficult. Negative biopsy
410
CLINICAL RADIOLOGY
Table 4 - Gastric and duodenal tumours - radiological features and endoscopic findings
Case No. Histology
Preoperative barium studies
Endoscopy
Case 6 Leiomyoma
Hiatus hernia and reflux. Antral filling defect. ? benign neoplasm (see text - 'Radiological Features')
Not performed
Case 7 Leiomyosarcoma (gastric)
Fundal filling defect. ? malignant tumour
Not performed
Case 8 Leiomyosarcoma (gastric)
Filling defect pars media of stomach, ? benign neoplasm
Not performed
Case 9 Leiomyosarcoma (anaplastic)
Stomach - n.a.d. Deformed duodenal bulb with ulcer crater
Not performed
Case 10 Leiomyosarcoma and adenocarcinoma (gastric)
Pyloric stenosis - cause not apparent
Not performed
Case 11 Leiomyoma (gastric)
Filling defect fundus and pars media of stomach. Probable leiomyoma
Not performed
Case 12 Leiomyosarcoma (duodenal)
Large barium filled cavity ? connected with second part of duodenum (at operation this was a duodenal diverticulum)
Negative - but instrument could not be introduced past duodenal bulb
Case 13 Leiomyosarcoma (duodenal)
Before first operation (gastrotomy, fundal
Before first operation. Oesophagos-
plication and pyloroplasty) - (see text 'Some Diagnostic Problems'). Barium meal-hiatus hernia and reflux and mucosal irregularity lower end of oesophagus. Stomach and duodenum n.a.d. (also colonic polyp at barium enema) Before second operation (resection of second part of duodenum) Coeliac angiogram - vascular lesion in duodenum of head of pancreas. Second barium meal - filling defect in descending duodenum suggests leiomyosarcoma
DISCUSSION
copy and gastroscopy - ulcer lower oesophagus with evidence of recent bleeding
Before second operation. Duodenoscopy - tumour and blood clot in descending duodenum
oesophageal group was found to be anaemic, whereas
anaemia, with a haemoglobin of less than 12 g/dl was Clinical Features. - Pain, in the chest or epigast- found in all eight patients with gastric or duodenal rium, and other dyspeptic symptoms (e.g. heartburn, lesions. Melaena was a complaint in six of these, b u t regurgitation, epigastric discomfort, flatulence)were no haematemesis was reported. The tendency of gastric and duodenal leiomyocommon to all three groups; eight patients complainmatous lesions to ulceration and bleeding is well ing of pain and 10 of other dyspeptic symptoms. Two subjects suffered attacks of pain of such known. This is not, however, a characteristic of severity as to lead to their urgent admission to hospital leiomyomas of the oesophagus which rarely cause with a subsequently unconfirmed diagnosis of ulceration of the overlying mucosa (Shaffer, 1976). Less common symptoms were as indicated in myocardial infarction in the one (case 11) and acute angina in the other (case 12). Dysphagia, said to be Tables 1 and 2. The clinical features of this small series serve to the main symptom of oesophageal leiomyoma (Seremetis et al., 1972; Shaffer, 1976) was reported illustrate the non-specific nature of the clinical in three patients with oesophageal lesions, being picture of smooth muscle tumours and indicate why characteristically mild in two. Only one patient in the those of the upper gastrointestinal tract enter into
SMOOTH MUSCLE TUMOURS
OF THE UPPER GASTROINTESTINAL
the differential diagnosis of a variety of other disorders arising in organs within the thorax and upper -abdomen, e.g. hiatus hernia and reflux oesophagitis, oesophageal stricture, peptic ulceration, carcinoma of the oesophagus, stomach or pancreas, etc. Radiological F e a t u r e s . - Mediastinal masses were shown on the plain radiographs of three patients and two of these masses were associated with filling defects and one with a concentric narrowing of the lower end of the oesophagus. The masses showed no specific features, but both the filling defects resembled those included by Glanz and Gr(inebaum (1977)when describing the major radiological signs of leimyosarcoma. Thus both were oval with long axis within 'the shadow' of the barium filled oesophagus. One of them, however, showed a fine irregularity along its free border correctly suggesting it had been produced by a leiomyosarcoma (Fig. 2.). A mass below the diaphragm in one patient (case 5) denoted the gastric component of an oesophagogastric leiomyosarcoma (Fig. 3) but although the mass was noted during the preoperative barium swallow its significance was not appreciated. Four of the patients with gastric tumours showed large rounded filling defects with evidence of central ulceration in two and mucosal abnormality in all of them. The defects were of the type considered as
Fig. 2 - Case 4. Oesophageal leiomyosarcoma with irregularity of anterior aspect of filling defect.
TRACT
411
Fig. 3 - Case 5. Oesophagogastric leiomyosarcoma showing partial obstruction o f the cardia and soft tissue mass due to gastric c o m p o n e n t o f the tumour.
characteristic of leiomyomas and certain benign gastric tumours (Shanks, 1969). Two of them were in fact caused by leiomyosarcomas, but did not show any distinguishing features from the two produced by leiomyomas. One patient (case 13) showed initially a minor mucosal abnormality and subsequently a filling defect in the descending duodenum. This defect was of nonspecific type and did not show any central ulceration or sinus tract formation, such as may be seen in Idling defects due to duodenal leiomyosarcomas (Meyers, 1971). This patient will be referred to when discussing some problems in diagnosis as will three patients (cases, 2, 9 and 12) without preoperative radiological evidence of a tumour. Indirect evidence of the presence of a tumour was obtained in one patient (case 10) by the demonstration of pyloric stenosis. There was, however, no indication of the cause of the obstruction, as radiological diagnosis in this instance, as in the patient with the oesophagogastric leiomyosarcoma who had marked obstruction at the cardia (case 5) was rendered difficult by retained secretions and food residue. Subsequently both an adenocarcinoma and a teiomyosarcoma were found in the distal part of the pyloric antrum. Two patients were considered as being of special interest. The first (case 3) had an attack of severe 'gastritis' during an overseas tour of duty. When he returned home four months later, although completely symptomless, he was requested to have a health checkup. This included a barium examination, which to the surprise of the radiologist revealed a large leiomyoma in the mid-third of his oesophagus. The second
412
CLINICAL RADIOLOGY
years after operation there was no clinical evidence of recurrence. Endoscopic Findings. - All the patients with either oesophageal or duodenal lesions were examined by endoscopy. This procedure was not carried out in any of the patients with gastric neoplasms because they were investigated before a gastroscopy service was available. Thus seven patients were subjected to endoscopy on one or more occasions and in only three was a tumour demonstrated. Of the only two biopsies carried out during endoscopy, that in case 4 (the leiomyosarcoma with metastatic spread) was extremely helpful in enabling a histological diagnosis to be made. Both Seremetis e t al. (1973) and Shaffer (1976) indicate barium swallow as being more useful than oesophagoscopy in the demonstration of oesoFig. 4 Case 6. Gastric leiomyoma in pyloric antrum. phageal leiomyomas and the latter states that the oesophagoscopic examination may be completely negative due to easy passage of the instrument past (case 6) illustrated that not all benign smooth muscle neoplasms are of slow growth. This patient refused the tumour. Endoscopy is, however, generally regarded treatment when his gastric leiomyoma was first as being complementary to barium studies in the diagrevealed as a small filling defect on a barium meat nosis of leiomyomatous neoplasms in the upper (Fig. 4). Consequently when, as a result of persisting gastrointestinal tract and of particular value in showing melaena he changed his mind 18 months later, a mucosal changes. Biopsy is contraindicated in some further barium examination showed that during this instances and also likely to be negative unless there is time the filling defect had approximately tripled in mucosal involvement or the lesion lies immediately size and also developed evidence of ulceration (Fig. 5). below the mucosa. Some Diagnostic Problems. - As stated by Kavlie This lesion on histological examination did not show any evidence of malignancy and at follow-up seven and White (1972) diagnosis may be elusive. Problems met with in the present series arose in the main, from the absence of any pathognomonic clinical features of the lesions, the difficulty or interpreting radiological signs in some instances and the absence of such signs in others and limitations in endoscopic methods. Another important factor was the infrequency with which these lesions are encountered in hospital practice. The statement made in their classic paper on smooth muscle tumours of the oesophagus by Gray et al. (1961) that these tumours 'belong to the large class of diseases that are too infrequent in any one surgical practice to give much personal experience yet common enough to warrant consideration in the differential diagnosis of more widely encountered lesions' may be considered equally applicable to all leiomyomatous neoplasms of the upper gastrointestinal tract. As a result of the radiological findings, supported in three instances by those of endoscopy, it was possible to indicate the likelihood of a tumour being present in 10 patients and in five of these suggest this was of leiomyomatous or benign nature. HowFig. 5 - Case 6. Eighteen months after barium meal shown in ever, the diagnosis was not always straightforward Fig. 4. The patient initially refused treatment and the filling defect has greatly increased in size and developed evidence and alternative diagnoses were advanced in a number of instances. o f ulceration.
SMOOTH
MUSCLE
TUMOURS
OF THE UPPER
Moreover, in three patients (cases 2, 9 and 12), no radiological evidence o f a tumour was found. Two of these were examined by endoscopy which also produced negative results. In case 2 the absence of radiological and endoscopic signs of the oesophageal leiomyoma, stated to be about the size of a 'large bean', appeared to be related to its intramural form; and in case 9 the absence of demonstrable abnormality of the stomach at barium meal, to the predominantly exogastric type of enlargement of the leiomyosarcoma found at operation (the operation in this patient was undertaken for suspected bleeding duodenal ulcer). In the third patient; case 12, the site of the duodenal leiomyosarcoma was completely hidden during the barium meal by the filling with contrast medium of a very large overlying duodenal diverticulum, some 7 cm long. This patient was also examined by duodenoscopy, but the instrument could not be passed far enough to reach the tumour site in the lower part of the descending duodenum. Problems in cases 1 and 5 arose initially as a result of narrowing of the lower end of the oesophagus simulating an achalasia (cardiospasm). In case 1, where the tumour ensheathed the lower end of the oesophagus, the suggestion o f achalasia which had been made on a barium swallow performed elsewhere received some support from the appearances at oesophagoscopy. Further plain films and barium swallow, however, suggested a tumour, although this was thought to be extrinsic. In retrospect the radiographic appearances were such that a diagnosis of idiopathic diffuse spasm or muscular hypertrophy of the lower third of the oesophagus (Shanks, 1969) would not have been unreasonable. Achalasia was suggested, also as a possible cause of the oesophageal obstruction in case 5 and it is interesting to note that Shanks (1969) indicates that leiomyomas can produce marked oesophageal dilatation similar to that caused by achalasia. This marked dilatation was not, however, observed in either of the Cases in the present study. Diagnostic problems may also arise from the X-ray appearances of the mediastinal masses seen on plain films as these lack any specific features to distinguish them from glandular mediastinal swellings, mediasfinal turnouts and cysts, etc. The masses shown on the plain films of cases 1 (Fig. 6.) 3 and 4 were all smooth in outline and did not show any lobulation. In case 4, however, a diagnosis of malignant lymphadenopathy due to reticulosarcoma or other cause with secondary oesophageal involvement was suggested as an alternative to that of leiomyosarcoma. Another patient in whom diagnostic difficulties were encountered was case 13, who was eventually found to have a duodenal leiomyosarcoma. This
GASTROINTESTINAL
TRACT
413
patient had severe melaena of only three weeks duration and the oesophagoscopy and radiographic findings suggested that this was due to a small ulcer at the gastro-oesophageal junction, associated with reflux and a small hiatus hernia. Surgery failed to cure the melaena so that further investigations were carried out to determine the major source of the
Fig. 6 Case 1. Non-specific appearance of mediastinal mass due to oesphageal leiomyoma ensheathing lower end of oesophagus.
Fig. 7 - Case 13. Duodenal leiomyosarcoma - filling defect in descending duodenum shown on second barium meal.
414
CLINICAL RADIOLOGY
bleeding. These illustrated the great hetp that can be obtained from coeliac angiography in this type o f situation as this examination produced evidence o f a vascular lesion in the region of the duodenum or head o f the pancreas. Duodenoscopy and a second barium meal then identified this lesion as a turnout in the descending duodenum. The tumour must have been very rapid in its growth as at this later examination, only two months after the first barium study, there was a large filling defect and marked mucosal changes in a part o f the duodenum, where the mucosa had previously been considered as showing no significant abnormality (Fig. 7.). In retrospect, however, some changes in direction of the mucosal folds could be seen on the first barium meal in the area where the filling defect was later demonstrated. Incidentally this patient was also found to have a haemorrhagic duodenal papilloma and also a small colonic polyp. Follow-up Findings. - Three patients, all with leiomyosarcomas were known to have died (two within a few weeks, and one nearly two years after operation). Enquiries were made in respect of those other patients who were operated upon before 1974, thus permitting a follow-up of three years and longer. These were six in number of whom two could not be traced. The remaining four had survived without evidence o f recurrence for the following periods: Case 1 (oesophageal leiomyoma) - 10 years. Cases 6 and 11 (gastric l e i o m y o m a s ) - seven years. Case 8 (gastric l e i o m y o s a r c o m a ) - seven years. (Note: the evidence o f non-recurrence was clinical and radiological in case 11 and clinical only in the other three cases.)
CONCLUSIONS Previous studies have built up a comprehensive picture of the clinical and radiological features of smooth muscle tumours o f the oesophagus, stomach and duodenum. The present study exemplifies many important aspects o f this established picture and also arise in the investigation o f these disorders by radiology and endoscopy. It is, however, likely that the present increasing employment of double contrast
techniques in barium studies and of modern endoscopic methods may facilitate the demonstration of many of these tumours. The follow-up findings are o f interest as, although the number o f patients investigated was very small, they are in accord with the favourable prognosis carried b y these lesions when their complete local excision can be achieved. Acknowledgements. - The author is grateful to his clinical and radiological colleagues for permission to use their notes and reports; to Dr J. L. Price and Professor N. Gibbs for much helpful advice concerning the study; and to Mrs Iris Bates, Miss Fiona Brown, Mrs Fiona Wood and Mrs Caroline Sawers for assistance with records and references and Mr J. Butt for the illustrations. REFERENCES Faegenburg, D., Farman, J., Dallemand, S., Schecter, L. S., Rosen, Y. & Chiat, H. (1975). Leiomyoblastoma of stomach. Radiology, 117, 297-300. Glanz, I. & Griinebaum, M. (1977). Clinical Radiology, 28, 197-200. Gray, S. W., Skandalakis, J. E., & Shepard, D. (1961). Smooth Muscle tumours of the oesophagus. International Abstracts of Surgery, 3,205-219. Huddy, P. & Griffiths, G. (1972). British Journal of Surgery, 59, 239-242. Jensen, F B. (1967) Primary gastric sarcoma. Acta chirurgica scandinavica, 133,139-151. Kavlie, H. & White, T. T. (1972). Leiomyomas of the upper gastrointestinal tract. Surgery, 71,842-848. Meyers, M. A. (1971). Leiomyosarcoma of the duodenum. Clinical Radiology, 22, 257-260. Morson, B. C. & Dawson, I. M. P. (1972). Gastrointestinal Pathology. Blackwood Scientific Publications, Oxford, London & Edinburgh. Morson, B. C. & Sobin, L. H. (1976). International Histological Classification of Turnouts, No. 15. World Health Organisation, Geneva. Seremetis, M. G., de Guzman, V. C., Lyons, W. S. & Peabody, J. W. (1973). Leiomyoma of the oesophagus. Annals of" Thoracic Surgery, 16,308-316. Shaffer, H. A. (1976) Multiple leiomyomas of the oesophagus. Radiology, 118, 29-34. Shanks, S. C. (1969). A Text Book of X-ray Diagnosis, Vol. IV. H. K. Lewis, London. Tsuzuki, T., Kakegawa, T., Arimor, M., Veda, M., Watenake, H., Okamoto, T. & Akakura, I. (1971). Giant leiomyoma of the oesophagus and cardia weighing more than 1,000 grams. Chest, 60, 396 399.
The Department of Radiology, The Royal Surrey County Hospital, Guild¢brd The clinical and radiological features and endoscopic findings in 13 patients with smooth muscle tumours of the upper gastrointestinal tract are reviewed. The study emphasises the non-specific nature of the symptomatology and exemplifies a number of aspects of the radiological picture of these disorders. It also illustrates some of the problems that can arise in their investigation, by radiological and endoscopic methods, and gives some indication of the favourable prognosis that may obtain when complete surgical removal of the tumour is possible.
Smooth muscle tumours of the gastrointestinal tract are uncommon, but can develop in any site. They are more often found in the stomach than in the oesophagus and occur much more rarely in the duodenum and elsewhere in the bowel. Some, especially when small and benign, do not produce any symptoms and their presence may only be revealed incidentally at autopsy, surgery or radiological investigations of other conditions. In other instances symptoms may be produced which, although often initially ill-defined and mild may be the precursors of serious disorder.
Jensen (1967) describing 'spindle-cell sarcoma of the stomach' (a term he uses collectively for fibrosarcoma, neurosarcoma and leiomyosarcoma) refers to opinions that a malignant tumour of this type may run a clinically benign course and that sometimes the reverse may also obtain. Faegenburg et al. (1975) describe leiomyoblastomas as generally pursuing a benign course,
As they enlarge, smooth muscle tumours may expand into the lumen of the gastrointestinal tract or they may extend outside this structure. Intraluminal growth may be associated with the development of ulceration in the mucosa overlying the turnout. The mode of growth and the presence of PATHOLOGY mucosal changes are important factors which influence The tumours usually originate in the muscle wall the degree of difficulty with which these neoplasms and only rarely from the muscularis mucosae. Leio- can be demonstrated by radiological and endoscopic myomas and leiomyosarcomas show a spindle-cell methods. Structure, but leiomyoblastomas (bizarre or epithelioid leiomyomas), the most uncommon type, are MATERIAL AND METHODS characterised by areas of round and polygonal cells The present study is concerned with 13 patients (Morson and Sobin, 1976). Considerable difficulty is often experienced in differentiating histologically with smooth muscle tumours arising in either the between the benign and malignant types of smooth oesophagus, stomach or duodenum. They were muscle tumour. Indeed, Morson and Dawson (1972) unselected and included all those with histological discussing gastric tumours state that the only reliable proof of a leiomyomatous tumour of the upper evidence of leiomyosarcoma is the presence of meta- gastrointestinal tract, investigated and treated during stases. They also indicate it may not be possible to a 12-year period (April 1965 March 1977) in the distinguish a neurllemmoma (schwannoma) from a three major hospitals of what is now the South West leiomyomatous growth. Opinions differ as to whether Surrey Health District. The study was directed all leiomyosarcomas are malignant from the outset, or principally to the clinical and radiologically aspects if they sometimes occur as a result of a malignant of the disorders investigated, but endoscopic and transformation of a leiomyoma. According to Glanz follow-up findings were also recorded. and Grtinebaum (1977) the question remains open. All the patients were treated by surgery except Leiomyosarcomas are often of extremely slow for one (case 4) who had evidence of metastatic growth and low grade malignancy. On occasion, spread from an oesophageal leiomyosarcoma at the however, they may show rapid local invasiveness and time of referral. In this instance the diagnosis was also early blood stream metastases and then develop made from a biopsy taken durir~g oesophagoscopy general signs and symptoms of malignant disease. and treatment was by radiotherapy and chemotherapy.
408
CLINICAL
RADIOLOGY
Fig. 1 - Case 11. Large gastric leiomyoma. A barium meal seven years after resection of this tumour showed no evidence of recurrence.
RESULTS
The average age of the patients in the series was 58.8 years and thus accords with the findings in other series of a peak incidence in middle age. In contradistinction to the usual absence of any marked difference ~in sex incidence 11 of the 13 subjects were males. Histological findings indicated that five of the tumours were leiomyomas and eight were leiomyosarcomas; one of these latter being of anaplastic type. All the smooth muscle tumours discovered were single, but one was found lying in close proximity to a gastric adenocarcinoma. One oesophageal leiomyoma was found in association with a hiatus hernia, thus serving as a reminder
that hiatus hernia has been stated to predispose to the formation of smooth muscle tumours in the lower third of the oesophagus (Gray et al., 1961). No polypoid tumours or growths showing radiologically demonstrable calcification were encountered (Huddy and Griffiths, 1972); neither were any giant tumours of the type described by Tsuzuki et al. (1971) although the very large leiomyoma in case 11 appeared to approach this category (Fig. 1). The results of the study are summarised in Tables 1 - 4 wherein it will be noted that the tumours are classified as oesophageal, gastric and duodenal. Case 5 who had a tumour with both oesophageal and gastric components presented with dysphagia and was thus classified with the oesophageal group.
Table 1 - Oesophageal tumours- clinical features Case No. His t ology
Sex Age
Dysphagia
1
M
Leiomyoma
40
2 Leiomyoma
M 53
3 Leiomyoma
M 49
4 Leiomyosarcoma
M 57
+
5 Leiomyosarcoma
F 76
+
Pain
+
Other dyspeptic symptoms
Lassitude
Cough
Weight loss
+
+
+
+
+
+
Haemoglobin (g/d O
14.8 +
Not known
-
15.7
+
10.0 13.0
SMOOTH MUSCLE TUMOURS
OF THE UPPER GASTROINTESTINAL
TRACT
409
Table 2 - Gastric and duodenal tumours - clinical features
Case No. His tology
Sex Age
Melaena
Pain
Other dyspep tie symptoms
Lassitude
Weigh t loss
6 Leiomyoma
M 44
+
7 Leiomyosarcoma (gastric)
F 66
+
+
+
8 Leiomyosarcoma (gastric)
M 61
+
+
-
9 Leiomyosarcoma (gastricanaplastic)
M 64
+
+
+
10 Leiomyosarcoma (gastric and carcinoma)
M 72
-
-
+
11" Leiomyoma (gastric)
M 60
-
+
+
-
12 Leiomyosarcoma (duodenal)
M 69
+
+
+
+
-
13 Leiomyosarcoma
M 64
+
+
-
Vomiting
+
-
+
Haemoglobin (g/dO
8.1 7.9
7.5
+
-
10.7
. +
+
10.7
8.9
-
6.0
10.1
* This p a t i e n t also had a palpable mass and was the only p a t i e n t to e x h i b i t this clinical sign. He was also the only pa t i e nt with a h isto ry o f previous gastric surgery, having had a g a s t r o t o m y and removal of a small benign a d e n o m a five years before the present referral.
Table 3 - Oesophageal t u m o u r s - radiological features and endoscopic findings
Case No. His tology
Preoperative X-ray findings
Oesophagoscopy
Chest radiograph
Barium studies
Case 1 Leiomyoma
Mediastinal mass
Reflux. Narrowing lower oesophagus. Slight hold up. ? extrinsic t umour (Ba. swallow elsewhere ? achalasia)
No tumour. Findings compatible with achalasia
Case 2 Leiomyoma
Normal
Reflux + hiatus hernia only
Normal
Case 3 Leiomyoma
Mediastinal mass
Filling defect mid-oesophagus. ? benign t u m o u r
? Leiomyoma. ? gastrogenic cyst
Case 4 Leiomyosarcoma
Mediastinal mass
Filling defect mid-oesophagus. ? glandular mass or l e i omyos a rc oma
Tumour. Biopsy = leiomyosarcoma (also b r o n c h o s c o p y = mass bulging at carina)
Case 5 Leiomyosarcoma (oesophagogastric)
Normal
Obstruction at cardia shown on Ba. swallow done elsewhere - urgent oesophagoscopy suggested appearances confused by retained food and fluid. Possible carcinoma or achalasia. Soft tissue swelling noted below diaphragm nature uncertain
Probable stricture lower oesophagus b u t e xa mi na t i on difficult. Negative biopsy
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Table 4 - Gastric and duodenal tumours - radiological features and endoscopic findings
Case No. Histology
Preoperative barium studies
Endoscopy
Case 6 Leiomyoma
Hiatus hernia and reflux. Antral filling defect. ? benign neoplasm (see text - 'Radiological Features')
Not performed
Case 7 Leiomyosarcoma (gastric)
Fundal filling defect. ? malignant tumour
Not performed
Case 8 Leiomyosarcoma (gastric)
Filling defect pars media of stomach, ? benign neoplasm
Not performed
Case 9 Leiomyosarcoma (anaplastic)
Stomach - n.a.d. Deformed duodenal bulb with ulcer crater
Not performed
Case 10 Leiomyosarcoma and adenocarcinoma (gastric)
Pyloric stenosis - cause not apparent
Not performed
Case 11 Leiomyoma (gastric)
Filling defect fundus and pars media of stomach. Probable leiomyoma
Not performed
Case 12 Leiomyosarcoma (duodenal)
Large barium filled cavity ? connected with second part of duodenum (at operation this was a duodenal diverticulum)
Negative - but instrument could not be introduced past duodenal bulb
Case 13 Leiomyosarcoma (duodenal)
Before first operation (gastrotomy, fundal
Before first operation. Oesophagos-
plication and pyloroplasty) - (see text 'Some Diagnostic Problems'). Barium meal-hiatus hernia and reflux and mucosal irregularity lower end of oesophagus. Stomach and duodenum n.a.d. (also colonic polyp at barium enema) Before second operation (resection of second part of duodenum) Coeliac angiogram - vascular lesion in duodenum of head of pancreas. Second barium meal - filling defect in descending duodenum suggests leiomyosarcoma
DISCUSSION
copy and gastroscopy - ulcer lower oesophagus with evidence of recent bleeding
Before second operation. Duodenoscopy - tumour and blood clot in descending duodenum
oesophageal group was found to be anaemic, whereas
anaemia, with a haemoglobin of less than 12 g/dl was Clinical Features. - Pain, in the chest or epigast- found in all eight patients with gastric or duodenal rium, and other dyspeptic symptoms (e.g. heartburn, lesions. Melaena was a complaint in six of these, b u t regurgitation, epigastric discomfort, flatulence)were no haematemesis was reported. The tendency of gastric and duodenal leiomyocommon to all three groups; eight patients complainmatous lesions to ulceration and bleeding is well ing of pain and 10 of other dyspeptic symptoms. Two subjects suffered attacks of pain of such known. This is not, however, a characteristic of severity as to lead to their urgent admission to hospital leiomyomas of the oesophagus which rarely cause with a subsequently unconfirmed diagnosis of ulceration of the overlying mucosa (Shaffer, 1976). Less common symptoms were as indicated in myocardial infarction in the one (case 11) and acute angina in the other (case 12). Dysphagia, said to be Tables 1 and 2. The clinical features of this small series serve to the main symptom of oesophageal leiomyoma (Seremetis et al., 1972; Shaffer, 1976) was reported illustrate the non-specific nature of the clinical in three patients with oesophageal lesions, being picture of smooth muscle tumours and indicate why characteristically mild in two. Only one patient in the those of the upper gastrointestinal tract enter into
SMOOTH MUSCLE TUMOURS
OF THE UPPER GASTROINTESTINAL
the differential diagnosis of a variety of other disorders arising in organs within the thorax and upper -abdomen, e.g. hiatus hernia and reflux oesophagitis, oesophageal stricture, peptic ulceration, carcinoma of the oesophagus, stomach or pancreas, etc. Radiological F e a t u r e s . - Mediastinal masses were shown on the plain radiographs of three patients and two of these masses were associated with filling defects and one with a concentric narrowing of the lower end of the oesophagus. The masses showed no specific features, but both the filling defects resembled those included by Glanz and Gr(inebaum (1977)when describing the major radiological signs of leimyosarcoma. Thus both were oval with long axis within 'the shadow' of the barium filled oesophagus. One of them, however, showed a fine irregularity along its free border correctly suggesting it had been produced by a leiomyosarcoma (Fig. 2.). A mass below the diaphragm in one patient (case 5) denoted the gastric component of an oesophagogastric leiomyosarcoma (Fig. 3) but although the mass was noted during the preoperative barium swallow its significance was not appreciated. Four of the patients with gastric tumours showed large rounded filling defects with evidence of central ulceration in two and mucosal abnormality in all of them. The defects were of the type considered as
Fig. 2 - Case 4. Oesophageal leiomyosarcoma with irregularity of anterior aspect of filling defect.
TRACT
411
Fig. 3 - Case 5. Oesophagogastric leiomyosarcoma showing partial obstruction o f the cardia and soft tissue mass due to gastric c o m p o n e n t o f the tumour.
characteristic of leiomyomas and certain benign gastric tumours (Shanks, 1969). Two of them were in fact caused by leiomyosarcomas, but did not show any distinguishing features from the two produced by leiomyomas. One patient (case 13) showed initially a minor mucosal abnormality and subsequently a filling defect in the descending duodenum. This defect was of nonspecific type and did not show any central ulceration or sinus tract formation, such as may be seen in Idling defects due to duodenal leiomyosarcomas (Meyers, 1971). This patient will be referred to when discussing some problems in diagnosis as will three patients (cases, 2, 9 and 12) without preoperative radiological evidence of a tumour. Indirect evidence of the presence of a tumour was obtained in one patient (case 10) by the demonstration of pyloric stenosis. There was, however, no indication of the cause of the obstruction, as radiological diagnosis in this instance, as in the patient with the oesophagogastric leiomyosarcoma who had marked obstruction at the cardia (case 5) was rendered difficult by retained secretions and food residue. Subsequently both an adenocarcinoma and a teiomyosarcoma were found in the distal part of the pyloric antrum. Two patients were considered as being of special interest. The first (case 3) had an attack of severe 'gastritis' during an overseas tour of duty. When he returned home four months later, although completely symptomless, he was requested to have a health checkup. This included a barium examination, which to the surprise of the radiologist revealed a large leiomyoma in the mid-third of his oesophagus. The second
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years after operation there was no clinical evidence of recurrence. Endoscopic Findings. - All the patients with either oesophageal or duodenal lesions were examined by endoscopy. This procedure was not carried out in any of the patients with gastric neoplasms because they were investigated before a gastroscopy service was available. Thus seven patients were subjected to endoscopy on one or more occasions and in only three was a tumour demonstrated. Of the only two biopsies carried out during endoscopy, that in case 4 (the leiomyosarcoma with metastatic spread) was extremely helpful in enabling a histological diagnosis to be made. Both Seremetis e t al. (1973) and Shaffer (1976) indicate barium swallow as being more useful than oesophagoscopy in the demonstration of oesoFig. 4 Case 6. Gastric leiomyoma in pyloric antrum. phageal leiomyomas and the latter states that the oesophagoscopic examination may be completely negative due to easy passage of the instrument past (case 6) illustrated that not all benign smooth muscle neoplasms are of slow growth. This patient refused the tumour. Endoscopy is, however, generally regarded treatment when his gastric leiomyoma was first as being complementary to barium studies in the diagrevealed as a small filling defect on a barium meat nosis of leiomyomatous neoplasms in the upper (Fig. 4). Consequently when, as a result of persisting gastrointestinal tract and of particular value in showing melaena he changed his mind 18 months later, a mucosal changes. Biopsy is contraindicated in some further barium examination showed that during this instances and also likely to be negative unless there is time the filling defect had approximately tripled in mucosal involvement or the lesion lies immediately size and also developed evidence of ulceration (Fig. 5). below the mucosa. Some Diagnostic Problems. - As stated by Kavlie This lesion on histological examination did not show any evidence of malignancy and at follow-up seven and White (1972) diagnosis may be elusive. Problems met with in the present series arose in the main, from the absence of any pathognomonic clinical features of the lesions, the difficulty or interpreting radiological signs in some instances and the absence of such signs in others and limitations in endoscopic methods. Another important factor was the infrequency with which these lesions are encountered in hospital practice. The statement made in their classic paper on smooth muscle tumours of the oesophagus by Gray et al. (1961) that these tumours 'belong to the large class of diseases that are too infrequent in any one surgical practice to give much personal experience yet common enough to warrant consideration in the differential diagnosis of more widely encountered lesions' may be considered equally applicable to all leiomyomatous neoplasms of the upper gastrointestinal tract. As a result of the radiological findings, supported in three instances by those of endoscopy, it was possible to indicate the likelihood of a tumour being present in 10 patients and in five of these suggest this was of leiomyomatous or benign nature. HowFig. 5 - Case 6. Eighteen months after barium meal shown in ever, the diagnosis was not always straightforward Fig. 4. The patient initially refused treatment and the filling defect has greatly increased in size and developed evidence and alternative diagnoses were advanced in a number of instances. o f ulceration.
SMOOTH
MUSCLE
TUMOURS
OF THE UPPER
Moreover, in three patients (cases 2, 9 and 12), no radiological evidence o f a tumour was found. Two of these were examined by endoscopy which also produced negative results. In case 2 the absence of radiological and endoscopic signs of the oesophageal leiomyoma, stated to be about the size of a 'large bean', appeared to be related to its intramural form; and in case 9 the absence of demonstrable abnormality of the stomach at barium meal, to the predominantly exogastric type of enlargement of the leiomyosarcoma found at operation (the operation in this patient was undertaken for suspected bleeding duodenal ulcer). In the third patient; case 12, the site of the duodenal leiomyosarcoma was completely hidden during the barium meal by the filling with contrast medium of a very large overlying duodenal diverticulum, some 7 cm long. This patient was also examined by duodenoscopy, but the instrument could not be passed far enough to reach the tumour site in the lower part of the descending duodenum. Problems in cases 1 and 5 arose initially as a result of narrowing of the lower end of the oesophagus simulating an achalasia (cardiospasm). In case 1, where the tumour ensheathed the lower end of the oesophagus, the suggestion o f achalasia which had been made on a barium swallow performed elsewhere received some support from the appearances at oesophagoscopy. Further plain films and barium swallow, however, suggested a tumour, although this was thought to be extrinsic. In retrospect the radiographic appearances were such that a diagnosis of idiopathic diffuse spasm or muscular hypertrophy of the lower third of the oesophagus (Shanks, 1969) would not have been unreasonable. Achalasia was suggested, also as a possible cause of the oesophageal obstruction in case 5 and it is interesting to note that Shanks (1969) indicates that leiomyomas can produce marked oesophageal dilatation similar to that caused by achalasia. This marked dilatation was not, however, observed in either of the Cases in the present study. Diagnostic problems may also arise from the X-ray appearances of the mediastinal masses seen on plain films as these lack any specific features to distinguish them from glandular mediastinal swellings, mediasfinal turnouts and cysts, etc. The masses shown on the plain films of cases 1 (Fig. 6.) 3 and 4 were all smooth in outline and did not show any lobulation. In case 4, however, a diagnosis of malignant lymphadenopathy due to reticulosarcoma or other cause with secondary oesophageal involvement was suggested as an alternative to that of leiomyosarcoma. Another patient in whom diagnostic difficulties were encountered was case 13, who was eventually found to have a duodenal leiomyosarcoma. This
GASTROINTESTINAL
TRACT
413
patient had severe melaena of only three weeks duration and the oesophagoscopy and radiographic findings suggested that this was due to a small ulcer at the gastro-oesophageal junction, associated with reflux and a small hiatus hernia. Surgery failed to cure the melaena so that further investigations were carried out to determine the major source of the
Fig. 6 Case 1. Non-specific appearance of mediastinal mass due to oesphageal leiomyoma ensheathing lower end of oesophagus.
Fig. 7 - Case 13. Duodenal leiomyosarcoma - filling defect in descending duodenum shown on second barium meal.
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CLINICAL RADIOLOGY
bleeding. These illustrated the great hetp that can be obtained from coeliac angiography in this type o f situation as this examination produced evidence o f a vascular lesion in the region of the duodenum or head o f the pancreas. Duodenoscopy and a second barium meal then identified this lesion as a turnout in the descending duodenum. The tumour must have been very rapid in its growth as at this later examination, only two months after the first barium study, there was a large filling defect and marked mucosal changes in a part o f the duodenum, where the mucosa had previously been considered as showing no significant abnormality (Fig. 7.). In retrospect, however, some changes in direction of the mucosal folds could be seen on the first barium meal in the area where the filling defect was later demonstrated. Incidentally this patient was also found to have a haemorrhagic duodenal papilloma and also a small colonic polyp. Follow-up Findings. - Three patients, all with leiomyosarcomas were known to have died (two within a few weeks, and one nearly two years after operation). Enquiries were made in respect of those other patients who were operated upon before 1974, thus permitting a follow-up of three years and longer. These were six in number of whom two could not be traced. The remaining four had survived without evidence o f recurrence for the following periods: Case 1 (oesophageal leiomyoma) - 10 years. Cases 6 and 11 (gastric l e i o m y o m a s ) - seven years. Case 8 (gastric l e i o m y o s a r c o m a ) - seven years. (Note: the evidence o f non-recurrence was clinical and radiological in case 11 and clinical only in the other three cases.)
CONCLUSIONS Previous studies have built up a comprehensive picture of the clinical and radiological features of smooth muscle tumours o f the oesophagus, stomach and duodenum. The present study exemplifies many important aspects o f this established picture and also arise in the investigation o f these disorders by radiology and endoscopy. It is, however, likely that the present increasing employment of double contrast
techniques in barium studies and of modern endoscopic methods may facilitate the demonstration of many of these tumours. The follow-up findings are o f interest as, although the number o f patients investigated was very small, they are in accord with the favourable prognosis carried b y these lesions when their complete local excision can be achieved. Acknowledgements. - The author is grateful to his clinical and radiological colleagues for permission to use their notes and reports; to Dr J. L. Price and Professor N. Gibbs for much helpful advice concerning the study; and to Mrs Iris Bates, Miss Fiona Brown, Mrs Fiona Wood and Mrs Caroline Sawers for assistance with records and references and Mr J. Butt for the illustrations. REFERENCES Faegenburg, D., Farman, J., Dallemand, S., Schecter, L. S., Rosen, Y. & Chiat, H. (1975). Leiomyoblastoma of stomach. Radiology, 117, 297-300. Glanz, I. & Griinebaum, M. (1977). Clinical Radiology, 28, 197-200. Gray, S. W., Skandalakis, J. E., & Shepard, D. (1961). Smooth Muscle tumours of the oesophagus. International Abstracts of Surgery, 3,205-219. Huddy, P. & Griffiths, G. (1972). British Journal of Surgery, 59, 239-242. Jensen, F B. (1967) Primary gastric sarcoma. Acta chirurgica scandinavica, 133,139-151. Kavlie, H. & White, T. T. (1972). Leiomyomas of the upper gastrointestinal tract. Surgery, 71,842-848. Meyers, M. A. (1971). Leiomyosarcoma of the duodenum. Clinical Radiology, 22, 257-260. Morson, B. C. & Dawson, I. M. P. (1972). Gastrointestinal Pathology. Blackwood Scientific Publications, Oxford, London & Edinburgh. Morson, B. C. & Sobin, L. H. (1976). International Histological Classification of Turnouts, No. 15. World Health Organisation, Geneva. Seremetis, M. G., de Guzman, V. C., Lyons, W. S. & Peabody, J. W. (1973). Leiomyoma of the oesophagus. Annals of" Thoracic Surgery, 16,308-316. Shaffer, H. A. (1976) Multiple leiomyomas of the oesophagus. Radiology, 118, 29-34. Shanks, S. C. (1969). A Text Book of X-ray Diagnosis, Vol. IV. H. K. Lewis, London. Tsuzuki, T., Kakegawa, T., Arimor, M., Veda, M., Watenake, H., Okamoto, T. & Akakura, I. (1971). Giant leiomyoma of the oesophagus and cardia weighing more than 1,000 grams. Chest, 60, 396 399.