Annals of the Royal College of Surgeons of England (1990) vol. 72, 316-320
Smooth
muscle tumours of the alimentary
tract T Diamond BSc FRCS Senior Surgical Registrar
T G Parks
MCh FRCS Professor of Surgical Science
M H D Danton MB BCh Senior House Officer Department of Surgery, The Queen's University of Belfast
Key words: Leiomyoma; Leiomyosarcoma; Alimentary
tract
Neoplasms arising from smooth muscle of the gastrointestinal (GI) tract are uncommon, comprising only 1% of gastrointestinal tumours. A total of 51 cases of smooth muscle tumour of the GI tract were analysed; 44 leiomyomas and 7 leiomyosarcomas. Lesions occurred in all areas from the oesophagus to the rectum, the stomach being the commonest site. Thirty-six patients had clinical features referable to the tumour. The tumour was detected during investigation or management of an unrelated disease process in 15 patients. The clinical presentation varied depending on tumour location, but abdominal pain and GI bleeding were the commonest presenting symptoms. The lesion was demonstrated preoperatively, mainly by endoscopy and barium studies, in 27 patients. Surgical excision was the treatment of choice, where possible. There was no recurrence in the leiomyoma group but four patients died in the leiomyosarcoma group. Although rare, smooth muscle tumours should be considered in situations where clinical presentation and investigations are not suggestive of any common GI disorder. The preoperative assessment and diagnosis is difficult because of the variability in clinical features and their inaccessibility to routine GI investigation. It is recommended that, where possible, the lesion, whether symptomatic or discovered incidentally, should be excised completely to achieve a cure and prevent future complications.
Neoplasms arising from smooth muscle of the alimentary tract are uncommon, comprising only 1% of gastrointestinal tumours. The symptoms of such tumours, whether benign or malignant, are non-specific, often resulting in late presentation and difficulty with preoper-
This paper reviews the management of 51 cases of smooth muscle tumours of the alimentary tract over a 20year period. The purpose of the review was to elucidate further the natural history of these uncommon lesions, to determine their incidence, distribution and clinical presentation, and the appropriate investigation and management in various parts of the GI tract.
Patients and methods The medical records of all patients treated at the Royal Victoria and Belfast City Hospitals over a 20-year period (1968-1988) with a pathological diagnosis of smooth muscle neoplasm of the GI tract were reviewed. Patient details including age, sex, tumour site, clinical presentation, surgical management and pathological description were recorded in each case.
Results Age and sex distribution A total of 51 patients underwent surgical treatment for a smooth muscle neoplasm of the gastrointestinal tract in the 20-year study period. The mean age at presentation was 64 years (range 26-88 years). Leiomyomas were six times more common than leiomyosarcomas (benign 44:malignant 7-Table I). Mean age at presentation was Table I. Sex distribution of benign and malignant tumours
ative diagnosis.
Correspondence to: Mr T Diamond, Department of Surgery, The Queen's University of Belfast, University Floor, Belfast City Hospital, Belfast BT9 7AB, Northern Ireland
Leiomyoma Leiomyosarcoma Total
Male
Female
19
25 2 27
5 24
Smooth muscle tumours of the alimentary tract Number of patients 20 18
-
16
-
14
-
12
-
10
-
=
Leiomyoma
E
Leiomyosarcoma
86
4
-
2
-
O-
-l-
HF I-L I
H
P11_
317
Oesophagus Oesophageal smooth muscle tumours were symptomatic in two patients presenting with weight loss and dysphagia. A smooth muscle tumour was detected during surgery for oesophageal carcinoma in three asymptomatic patients. Stomach Tumours of the stomach tended to be symptomatic (21 out of 29 patients-72%), the main clinical features being abdominal pain, gastrointestinal bleeding, weight loss and a palpable abdominal mass.
0-10 10-20 20-30 30-40 40-50 50-60 60-70 70-80 80-90 90-100 Age
Figure 1. Age distribution of patients with benign and malignant tumours.
higher in the benign group compared with the malignant group (65:59 years-Fig. 1). Overall there were 27 female and 24 male patients (female:male ratio 1.12:1). However, male patients had a higher incidence of malignant tumours (5 male:2 female-Table I). Tumour location Tumour location is detailed in Fig. 2. Tumours were more common in the upper GI tract, particularly the stomach (29 patients-57% of the total). Tumours occurred more frequently in the duodenum than elsewhere in the small intestine, on a length for length basis. The rectum was the main site for tumours within the large intestine (four out of six patients). Clinical features The patients were divided into two groups, asymptomatic and symptomatic (Table II), when evaluating the clinical presentation. The asymptomatic group consisted of tumours detected in the process of investigation or management of an unrelated disease process. Site
Leiomyoma
Oesophagus (Total)
S
1/3 middle 1/3 lower 1/3
0
upper
24
fundus
9 12
Small Intestine (Total)
duodenum
O
4
Stomach (Total)
body pylorus
Letomnyosarcoma
3
10 2
jejunum/ileum Large Bowel (Total) colon rectum
2
0 *
*
Leiomyona Leiomyosarcona
Figure 2. Distribution of benign and malignant tumours within the alimentary tract.
Small intestine All three duodenal tumours were symptomatic, causing obstruction (two patients) and abdominal pain (one patient). Only four (50%) of the jejunal/ileal lesions were symptomatic; gastrointestinal bleeding, abdominal pain and abdominal mass being the clinical features. One ileal lesion which caused an intussusception and resultant gangrene presented with acute abdominal signs. Large intestine All large bowel tumours were symptomatic, presenting with abdominal pain and rectal bleeding. In addition two patients with rectal lesions complained of a change in bowel habit and two patients had a mass palpable on
rectal examination. Investigations and diagnosis In 27 patients, preoperative investigotion demonstrated a lesion, whereas in 24 patients the tumour was only identified at operation (Table III). Proportionately, an intraoperative diagnosis was more common in the oesophagus and jejunal/ileal region. Contrast studies and endoscopy were the investigations used most frequently. Superior mesenteric arteriography revealed one tumour at the duodenojejunal region, computed tomography (CT) demonstrated two gastric lesions and ultrasound four upper GI lesions (two stomach, two duodenal). Treatment and outcome All lesions were resected surgically except for two leiomyosarcomas which were inoperable at laparotomy. Two patients died postoperatively (hospital mortality = 4%); both had a leiomyoma of the stomach. Death in one case was due to sepsis and in the other to anastomotic leakage. There were no hospital deaths in the malignant group. There was no recurrence of tumour in the benign group with a follow-up ranging from 1 to 14 years. In the malignant group, four patients died at 3 weeks, 4 months, 5 months and 19 months postoperatively (three
318
T Diamond et al.
Table II. Clinical presentation Jrejunuml Oesophagus
Stomach
Duodenum
ileum
Colon
Rectum
3 2
8 21 10 12 7
0 3
4 4 2 2
0 2 1 2
0 4 1 2
Asymptomatic Symptomatic Bleeding Pain Weight loss Dysphagia Altered bowel habit Anaemia Mass Obstruction Peritonitis
2 2
3
5 6
2 1 2
1 2 2
1
Table III. Tumour identification
jejunumi Intraoperative Preoperative Barium studies Endoscopy USS CT scan Arteriogram
Oesophagus
Stomach
Duodenum
ileum
Colon
Rectum
4 1 1 1
14 15 14 13 1 2 1
0 3 2 1 2
6 2
0 2 1 2
0 4
gastric, one duodenal lesion). Three patients are well at 2, 8 and 10 years postoperatively (two stomach, one rectal lesion).
4
1
1
asymptomatic or present with non-specific symptoms and signs (6,7). Furthermore, the presentation varies depending on the location within the alimentary tract (3). In this series, 36 patients had clinical features referable to the tumour.
Discussion Mesenchymal tumours account for 1% of gastrointestinal neoplasms (1). They can present at almost any age, but the majority do so in the 5th and 6th decades (2,3), their peak incidence being earlier than that of mucosal derived neoplasms. This was confirmed in the present series. Moreover, as with other reports (4), malignant tumours presented at an earlier mean age than benign tumours. Leiomyosarcoma was more common in male patients. This is in agreement with most reports, which cite a male preponderance in the malignant group (3-5), but at variance with Evans who found an equal sex distribution for benign and malignant tumours (2). The present study reflects the distribution of these lesions in the gastrointestinal tract as documented by others (2). Baker and Good (6) analysed 150 cases of smooth muscle tumours, 66% of which were in the stomach, 24% in the small bowel, 7% oesophageal and 3% large bowel, which corresponds to our own tumour distribution. It is difficult to establish a preoperative diagnosis of smooth muscle tumours. Clinically the lesions are often
Oesophageal lesions present typically with dysphagia and heartburn (8,9), features recorded in the present series. All three asymptomatic oesophageal leiomyomas were found in association with oesophageal carcinoma. This association, although rare, has been documented previously (10,11). In addition, the possibility that a preexisting leiomyoma may induce an oesophageal carcinoma through irritation with stenosis has been proposed (10). Gastrointestinal bleeding and abdominal pain are features of stomach and small bowel lesions as documented in this series (3-5,12,13). Rarer presentations include perforation (7,14), intussusception and obstruction (15). Abdominal pain, bleeding and altered bowel habit are the commonest presentations with large bowel lesions. Rectal tumours are often palpable (3). All these features were recorded in this series. In this review, 27 patients (53%) had a lesion demonstrated by a preoperative investigation. Barium studies and endoscopy demonstrated most of these lesions. Contrast studies and endoscopy may miss the mural lesion due to the fact that smooth muscle tumours tend to grow in exophytic fashion (16). This was the case in four gastric leiomyomas in the present review.
Smooth muscle tumours of the alimentary tract
Figure 3. Macroscopic appearance of a large gastric leiomyoma with central ulceration.
Occasionally a large gastric ieiomyoma may contain a centrally ulcerated area as in one of our cases (Fig. 3) and produce a classic appearance at endoscopy or on barium meal investigation (Fig. 4). These techniques may demonstrate the lesion, but a specific diagnosis of smooth muscle tumour is obtained rarely as the biopsy may not penetrate deep enough to reach the lesion (1 7). Coeliac and superior mesenteric arteriography may prove to be a useful aid in investigation, especially for small bowel lesions, as these are often missed on barium studies and
III 11 sdi..;...
Flur 4 Banu mcasonall.
mea deosrtn
ag
al lagegaticleo
ati eoym
a
oti
319
are beyond the reach of conventional endoscopy (14,17). The characteristic angiographic picture is of a wellcircumscribed, hypervascular tumour with irregular vessels, venous lakes and some large feeding vessels. Arteriography identified one jejunal and one gastric lesion in the present study. A CT scan may be helpful in the investigation of these tumours as it can demonstrate the complete lesion and its relation to surrounding structures (8). It may also help to differentiate between benign and malignant lesions by demonstrating specific tumour appearance (16) or the presence of distant metastases. In this series CT scan identified two stomach leiomyomas. The treatment of choice is surgery. Symptoms were relieved and there was no tumour recurrence in the benign group. Two of the five patients have survived 5 years where a potentially curative resection was possible in the malignant group. A 50% 5-year survival is documented by others for resectable leiomyosarcoma (3,5,12). It is recommended that as much as possible of the tumour should be resected where a complete resection is not possible as this may improve the prognosis in terms of both palliation and survival (5). We feel that, where feasible, all lesions should be resected, as it is difficult or impossible to differentiate between benign and malignant lesions before or during surgery. Tumour size and frozen section histology are not conclusive (2,12,15) and, although rare, an initial benign lesion may proceed to malignancy. The present study confirms that symptoms may arise at any age, and removal of the lesion at an early stage may prevent a future life-threatening complication. Finally, a small, asymptomatic lesion may be easily enucleated from the bowel wall without disturbing the overlying mucosa or necessitating a bowel resection, this being of particular advantage in the oesophagus (8-10).
References I Skandalakis JE, Grey SW, Shepard D. Smooth muscle tumours of the small intestine. Am 7 Gastroenterol 1964;42: 172-90. 2 Evans HL. Smooth muscle tumours of the gastrointestinal tract. Cancer 1985;56:2242-50. 3 Akwari OE, Dozois RR, Weiland LH, Beahrs OH. Leiomyosarcoma of the small and large bowel. Cancer 1978;42: 1375-84. 4 Appelman HD, Helwig EB. Gastric epithelial leiomyoma
and leiomyosarcoma. Cancer 1976;38:708-28. 5 Starr GF, Dockerty MB. Leiomyomas and leiomyosarcomas of the small intestine. Cancer 1955;8:101-11. 6 Baker HL, Good CA. Smooth muscle tumours of the alimentary tract. AJR 1955;74:246-55. 7 Lawsen E, Lyng KM. Leiomyomas of the small intestine: a rare cause of abdominal emergency. Acta Chir Scand
1987;153:391-3. 8 Abrescia F, Montresor E, Saggin P et al. Leiomyomata of the oesophagus: report of four surgically treated cases. Eur J Surg Oncol 1985;11:333-6. 9 Deverall PB. Smooth muscle tumours of the oesophagus.
BrJZ Surg 1968;55:457-61.
320
T Diamond et al.
10 Toshifumi Iizuka, Hoichi Kato, Hiroshi Watanabe, Masayuki Itabashi, Teruyuki Hirota. Superficial carcinoma of the esophagus co-existing with esophageal leiomyoma: a case report and review of Japanese literature. Jpn J Clin Oncol 1984;14(1):115-22. 11 Nakamura Y, Kakegawa T, Shimoyamada K, Kosaka Y, Watanabe H. Leiomyoma of the esophagus associated with esophageal carcinoma. Geka Shinryo 1964;6:763 (in Japanese). 12 Shiu MH, Farr GH, Papachristou DN, Steda H, Hajdu SI. Myosarcomas of the stomach: natural history, prognostic factors and management. Cancer 1982;49:177-87. 13 Cathcart PM, Cathcart RS, Yarbrough DR. Tumours of gastric smooth muscle. South Med J 1980;73;18-20. 14 Stothert JC, Riaz MA, Joyce PF, Kaminski DL. Pre-operative angiographic diagnosis of small bowel leiomyomas. Arch Surg 1978;113:643-5.
15 Pacella RA, Caleel RT. Leiomyosarcoma of the jejunum. Report of a case and review of the literature. j Am Osteopath Assoc 1985;85:107-11. 16 Megibon AJ, Balthazar EJ, Hulnick PH, Naidich DP, Bosniak MA. CT evaluation of gastrointestinal leiomyomas and leiomyosarcomas. AJR 1985;144:727-31. 17 Fagin RR, Levison JD, Kobayoshi S et al. Myogenic tumours of the stomach (gastroscopic and pathological observations). Gastrointest Endosc 1970;17:30-3. 18 Lezhak Y, Adar R, Mozes M, Deutsch V. Angiographic diagnosis of leiomyomatous small bowel tumours. Angiologia 1973;10:310-17. Received 22 February 1990
Notes on books Bone and Joint Imaging edited by Donald Resnick. 1330 pages, illustrated. W B Saunders Company, Philadelphia. 1989. £125.00. ISBN O72162215 1 Despite the very large size of this book the editor writes in his preface that it contains only essential information necessary in the interpretation of imaging studies of patients with musculoskeletal disorders. The volume is a condensation of a multivolume series that contains even more detail. The present compendium is directed specifically to trainees in orthopaedic surgery, rheumatology and radiology and there is no doubt that this audience will find the contents helpful. Established consultants might also benefit from thumbing the pages.
Second Vienna Shock Forum edited by G Schlag and H RedI. 1142 pages, illustrated. Alan R Liss Inc., New York. 1989. $196.00. ISBN 0 8451 5158 4 Printed from camera-ready copy this volume contains the papers given at the Second Vienna Shock Forum held in May 1988. An international team of contributors, many from departments of surgery. Sadly, not a single British surgical department is represented, highlighting the lack of interest shown in this field by surgeons in this country.
Arthritis: The Painfil Challenge by David Freedman. 18 pages, paperback. Arthritis Care, London. 1989. No price given. A report commissioned by the charity Arthritis Care detailing the enormity of the problem of arthritis, the large proportion of the population involved and the consequences to them as individuals and as families. It is suggested that the medical profession could focus more attention on the control and management of pain.
Diseases of the Gastrointestinal Tract and Liver edited by David J C Shearman, Niall D Finlayson and David C
Carter. 2nd edition. 1336 pages, illustrated. Churchill Livingstone, Edinburgh. 1989. £95.00. ISBN 0 443 03110 X The first edition of this comprehensive textbook was very successful when it was published in 1982. Seven years later the editors have updated throughout and introduced new contributors. Of special importance is the appointment of a surgical editor, thus acknowledging the importance of surgery in the development of gastroenterology and hepatology. Well produced, adequately illustrated, extensively referenced, this volume will need to be accessible to all gastroenterologists and surgeons with an interest in this specialty.
Reconstruction of the Thumb edited by A Landi, S De Luca and G De Santis. 344 pages, illustrated. Chapman and Hall Medical, London. 1989. £75.00. ISBN 0 412 29390 0 The thumb is the key digit for many everyday activities and its absence or deformity will cause extreme difficulty in the performance of many tasks. This book covers the history of thumb reconstruction and gives details of surgical and microvascular techniques. Comprehensive, with many illustrations and references.
Cancer Management in Man edited by L Goldson. 296 pages, illustrated. Kluwer Academic Publishers, Dordrecht. 1989. £59.95. ISBN 0 89838 998 4 Volume 9 in the series Cancer Growth and Progression covering detection, diagnosis, surgery, radiology, chronobiology and endocrine therapy.
Cancer Management in Man edited by P V Woolley. 248 pages, illustrated. Kluwer Academic Publishers, Dordrecht. 1989. £59.95. ISBN 0 89838 999 2 Volume 10 in the series Cancer Growth and Progression. This volume covers biological response modifiers, chemotherapy, antibiotics, hyperthermia and supporting measures such as nutrition and pain management.
Smooth
muscle tumours of the alimentary
tract T Diamond BSc FRCS Senior Surgical Registrar
T G Parks
MCh FRCS Professor of Surgical Science
M H D Danton MB BCh Senior House Officer Department of Surgery, The Queen's University of Belfast
Key words: Leiomyoma; Leiomyosarcoma; Alimentary
tract
Neoplasms arising from smooth muscle of the gastrointestinal (GI) tract are uncommon, comprising only 1% of gastrointestinal tumours. A total of 51 cases of smooth muscle tumour of the GI tract were analysed; 44 leiomyomas and 7 leiomyosarcomas. Lesions occurred in all areas from the oesophagus to the rectum, the stomach being the commonest site. Thirty-six patients had clinical features referable to the tumour. The tumour was detected during investigation or management of an unrelated disease process in 15 patients. The clinical presentation varied depending on tumour location, but abdominal pain and GI bleeding were the commonest presenting symptoms. The lesion was demonstrated preoperatively, mainly by endoscopy and barium studies, in 27 patients. Surgical excision was the treatment of choice, where possible. There was no recurrence in the leiomyoma group but four patients died in the leiomyosarcoma group. Although rare, smooth muscle tumours should be considered in situations where clinical presentation and investigations are not suggestive of any common GI disorder. The preoperative assessment and diagnosis is difficult because of the variability in clinical features and their inaccessibility to routine GI investigation. It is recommended that, where possible, the lesion, whether symptomatic or discovered incidentally, should be excised completely to achieve a cure and prevent future complications.
Neoplasms arising from smooth muscle of the alimentary tract are uncommon, comprising only 1% of gastrointestinal tumours. The symptoms of such tumours, whether benign or malignant, are non-specific, often resulting in late presentation and difficulty with preoper-
This paper reviews the management of 51 cases of smooth muscle tumours of the alimentary tract over a 20year period. The purpose of the review was to elucidate further the natural history of these uncommon lesions, to determine their incidence, distribution and clinical presentation, and the appropriate investigation and management in various parts of the GI tract.
Patients and methods The medical records of all patients treated at the Royal Victoria and Belfast City Hospitals over a 20-year period (1968-1988) with a pathological diagnosis of smooth muscle neoplasm of the GI tract were reviewed. Patient details including age, sex, tumour site, clinical presentation, surgical management and pathological description were recorded in each case.
Results Age and sex distribution A total of 51 patients underwent surgical treatment for a smooth muscle neoplasm of the gastrointestinal tract in the 20-year study period. The mean age at presentation was 64 years (range 26-88 years). Leiomyomas were six times more common than leiomyosarcomas (benign 44:malignant 7-Table I). Mean age at presentation was Table I. Sex distribution of benign and malignant tumours
ative diagnosis.
Correspondence to: Mr T Diamond, Department of Surgery, The Queen's University of Belfast, University Floor, Belfast City Hospital, Belfast BT9 7AB, Northern Ireland
Leiomyoma Leiomyosarcoma Total
Male
Female
19
25 2 27
5 24
Smooth muscle tumours of the alimentary tract Number of patients 20 18
-
16
-
14
-
12
-
10
-
=
Leiomyoma
E
Leiomyosarcoma
86
4
-
2
-
O-
-l-
HF I-L I
H
P11_
317
Oesophagus Oesophageal smooth muscle tumours were symptomatic in two patients presenting with weight loss and dysphagia. A smooth muscle tumour was detected during surgery for oesophageal carcinoma in three asymptomatic patients. Stomach Tumours of the stomach tended to be symptomatic (21 out of 29 patients-72%), the main clinical features being abdominal pain, gastrointestinal bleeding, weight loss and a palpable abdominal mass.
0-10 10-20 20-30 30-40 40-50 50-60 60-70 70-80 80-90 90-100 Age
Figure 1. Age distribution of patients with benign and malignant tumours.
higher in the benign group compared with the malignant group (65:59 years-Fig. 1). Overall there were 27 female and 24 male patients (female:male ratio 1.12:1). However, male patients had a higher incidence of malignant tumours (5 male:2 female-Table I). Tumour location Tumour location is detailed in Fig. 2. Tumours were more common in the upper GI tract, particularly the stomach (29 patients-57% of the total). Tumours occurred more frequently in the duodenum than elsewhere in the small intestine, on a length for length basis. The rectum was the main site for tumours within the large intestine (four out of six patients). Clinical features The patients were divided into two groups, asymptomatic and symptomatic (Table II), when evaluating the clinical presentation. The asymptomatic group consisted of tumours detected in the process of investigation or management of an unrelated disease process. Site
Leiomyoma
Oesophagus (Total)
S
1/3 middle 1/3 lower 1/3
0
upper
24
fundus
9 12
Small Intestine (Total)
duodenum
O
4
Stomach (Total)
body pylorus
Letomnyosarcoma
3
10 2
jejunum/ileum Large Bowel (Total) colon rectum
2
0 *
*
Leiomyona Leiomyosarcona
Figure 2. Distribution of benign and malignant tumours within the alimentary tract.
Small intestine All three duodenal tumours were symptomatic, causing obstruction (two patients) and abdominal pain (one patient). Only four (50%) of the jejunal/ileal lesions were symptomatic; gastrointestinal bleeding, abdominal pain and abdominal mass being the clinical features. One ileal lesion which caused an intussusception and resultant gangrene presented with acute abdominal signs. Large intestine All large bowel tumours were symptomatic, presenting with abdominal pain and rectal bleeding. In addition two patients with rectal lesions complained of a change in bowel habit and two patients had a mass palpable on
rectal examination. Investigations and diagnosis In 27 patients, preoperative investigotion demonstrated a lesion, whereas in 24 patients the tumour was only identified at operation (Table III). Proportionately, an intraoperative diagnosis was more common in the oesophagus and jejunal/ileal region. Contrast studies and endoscopy were the investigations used most frequently. Superior mesenteric arteriography revealed one tumour at the duodenojejunal region, computed tomography (CT) demonstrated two gastric lesions and ultrasound four upper GI lesions (two stomach, two duodenal). Treatment and outcome All lesions were resected surgically except for two leiomyosarcomas which were inoperable at laparotomy. Two patients died postoperatively (hospital mortality = 4%); both had a leiomyoma of the stomach. Death in one case was due to sepsis and in the other to anastomotic leakage. There were no hospital deaths in the malignant group. There was no recurrence of tumour in the benign group with a follow-up ranging from 1 to 14 years. In the malignant group, four patients died at 3 weeks, 4 months, 5 months and 19 months postoperatively (three
318
T Diamond et al.
Table II. Clinical presentation Jrejunuml Oesophagus
Stomach
Duodenum
ileum
Colon
Rectum
3 2
8 21 10 12 7
0 3
4 4 2 2
0 2 1 2
0 4 1 2
Asymptomatic Symptomatic Bleeding Pain Weight loss Dysphagia Altered bowel habit Anaemia Mass Obstruction Peritonitis
2 2
3
5 6
2 1 2
1 2 2
1
Table III. Tumour identification
jejunumi Intraoperative Preoperative Barium studies Endoscopy USS CT scan Arteriogram
Oesophagus
Stomach
Duodenum
ileum
Colon
Rectum
4 1 1 1
14 15 14 13 1 2 1
0 3 2 1 2
6 2
0 2 1 2
0 4
gastric, one duodenal lesion). Three patients are well at 2, 8 and 10 years postoperatively (two stomach, one rectal lesion).
4
1
1
asymptomatic or present with non-specific symptoms and signs (6,7). Furthermore, the presentation varies depending on the location within the alimentary tract (3). In this series, 36 patients had clinical features referable to the tumour.
Discussion Mesenchymal tumours account for 1% of gastrointestinal neoplasms (1). They can present at almost any age, but the majority do so in the 5th and 6th decades (2,3), their peak incidence being earlier than that of mucosal derived neoplasms. This was confirmed in the present series. Moreover, as with other reports (4), malignant tumours presented at an earlier mean age than benign tumours. Leiomyosarcoma was more common in male patients. This is in agreement with most reports, which cite a male preponderance in the malignant group (3-5), but at variance with Evans who found an equal sex distribution for benign and malignant tumours (2). The present study reflects the distribution of these lesions in the gastrointestinal tract as documented by others (2). Baker and Good (6) analysed 150 cases of smooth muscle tumours, 66% of which were in the stomach, 24% in the small bowel, 7% oesophageal and 3% large bowel, which corresponds to our own tumour distribution. It is difficult to establish a preoperative diagnosis of smooth muscle tumours. Clinically the lesions are often
Oesophageal lesions present typically with dysphagia and heartburn (8,9), features recorded in the present series. All three asymptomatic oesophageal leiomyomas were found in association with oesophageal carcinoma. This association, although rare, has been documented previously (10,11). In addition, the possibility that a preexisting leiomyoma may induce an oesophageal carcinoma through irritation with stenosis has been proposed (10). Gastrointestinal bleeding and abdominal pain are features of stomach and small bowel lesions as documented in this series (3-5,12,13). Rarer presentations include perforation (7,14), intussusception and obstruction (15). Abdominal pain, bleeding and altered bowel habit are the commonest presentations with large bowel lesions. Rectal tumours are often palpable (3). All these features were recorded in this series. In this review, 27 patients (53%) had a lesion demonstrated by a preoperative investigation. Barium studies and endoscopy demonstrated most of these lesions. Contrast studies and endoscopy may miss the mural lesion due to the fact that smooth muscle tumours tend to grow in exophytic fashion (16). This was the case in four gastric leiomyomas in the present review.
Smooth muscle tumours of the alimentary tract
Figure 3. Macroscopic appearance of a large gastric leiomyoma with central ulceration.
Occasionally a large gastric ieiomyoma may contain a centrally ulcerated area as in one of our cases (Fig. 3) and produce a classic appearance at endoscopy or on barium meal investigation (Fig. 4). These techniques may demonstrate the lesion, but a specific diagnosis of smooth muscle tumour is obtained rarely as the biopsy may not penetrate deep enough to reach the lesion (1 7). Coeliac and superior mesenteric arteriography may prove to be a useful aid in investigation, especially for small bowel lesions, as these are often missed on barium studies and
III 11 sdi..;...
Flur 4 Banu mcasonall.
mea deosrtn
ag
al lagegaticleo
ati eoym
a
oti
319
are beyond the reach of conventional endoscopy (14,17). The characteristic angiographic picture is of a wellcircumscribed, hypervascular tumour with irregular vessels, venous lakes and some large feeding vessels. Arteriography identified one jejunal and one gastric lesion in the present study. A CT scan may be helpful in the investigation of these tumours as it can demonstrate the complete lesion and its relation to surrounding structures (8). It may also help to differentiate between benign and malignant lesions by demonstrating specific tumour appearance (16) or the presence of distant metastases. In this series CT scan identified two stomach leiomyomas. The treatment of choice is surgery. Symptoms were relieved and there was no tumour recurrence in the benign group. Two of the five patients have survived 5 years where a potentially curative resection was possible in the malignant group. A 50% 5-year survival is documented by others for resectable leiomyosarcoma (3,5,12). It is recommended that as much as possible of the tumour should be resected where a complete resection is not possible as this may improve the prognosis in terms of both palliation and survival (5). We feel that, where feasible, all lesions should be resected, as it is difficult or impossible to differentiate between benign and malignant lesions before or during surgery. Tumour size and frozen section histology are not conclusive (2,12,15) and, although rare, an initial benign lesion may proceed to malignancy. The present study confirms that symptoms may arise at any age, and removal of the lesion at an early stage may prevent a future life-threatening complication. Finally, a small, asymptomatic lesion may be easily enucleated from the bowel wall without disturbing the overlying mucosa or necessitating a bowel resection, this being of particular advantage in the oesophagus (8-10).
References I Skandalakis JE, Grey SW, Shepard D. Smooth muscle tumours of the small intestine. Am 7 Gastroenterol 1964;42: 172-90. 2 Evans HL. Smooth muscle tumours of the gastrointestinal tract. Cancer 1985;56:2242-50. 3 Akwari OE, Dozois RR, Weiland LH, Beahrs OH. Leiomyosarcoma of the small and large bowel. Cancer 1978;42: 1375-84. 4 Appelman HD, Helwig EB. Gastric epithelial leiomyoma
and leiomyosarcoma. Cancer 1976;38:708-28. 5 Starr GF, Dockerty MB. Leiomyomas and leiomyosarcomas of the small intestine. Cancer 1955;8:101-11. 6 Baker HL, Good CA. Smooth muscle tumours of the alimentary tract. AJR 1955;74:246-55. 7 Lawsen E, Lyng KM. Leiomyomas of the small intestine: a rare cause of abdominal emergency. Acta Chir Scand
1987;153:391-3. 8 Abrescia F, Montresor E, Saggin P et al. Leiomyomata of the oesophagus: report of four surgically treated cases. Eur J Surg Oncol 1985;11:333-6. 9 Deverall PB. Smooth muscle tumours of the oesophagus.
BrJZ Surg 1968;55:457-61.
320
T Diamond et al.
10 Toshifumi Iizuka, Hoichi Kato, Hiroshi Watanabe, Masayuki Itabashi, Teruyuki Hirota. Superficial carcinoma of the esophagus co-existing with esophageal leiomyoma: a case report and review of Japanese literature. Jpn J Clin Oncol 1984;14(1):115-22. 11 Nakamura Y, Kakegawa T, Shimoyamada K, Kosaka Y, Watanabe H. Leiomyoma of the esophagus associated with esophageal carcinoma. Geka Shinryo 1964;6:763 (in Japanese). 12 Shiu MH, Farr GH, Papachristou DN, Steda H, Hajdu SI. Myosarcomas of the stomach: natural history, prognostic factors and management. Cancer 1982;49:177-87. 13 Cathcart PM, Cathcart RS, Yarbrough DR. Tumours of gastric smooth muscle. South Med J 1980;73;18-20. 14 Stothert JC, Riaz MA, Joyce PF, Kaminski DL. Pre-operative angiographic diagnosis of small bowel leiomyomas. Arch Surg 1978;113:643-5.
15 Pacella RA, Caleel RT. Leiomyosarcoma of the jejunum. Report of a case and review of the literature. j Am Osteopath Assoc 1985;85:107-11. 16 Megibon AJ, Balthazar EJ, Hulnick PH, Naidich DP, Bosniak MA. CT evaluation of gastrointestinal leiomyomas and leiomyosarcomas. AJR 1985;144:727-31. 17 Fagin RR, Levison JD, Kobayoshi S et al. Myogenic tumours of the stomach (gastroscopic and pathological observations). Gastrointest Endosc 1970;17:30-3. 18 Lezhak Y, Adar R, Mozes M, Deutsch V. Angiographic diagnosis of leiomyomatous small bowel tumours. Angiologia 1973;10:310-17. Received 22 February 1990
Notes on books Bone and Joint Imaging edited by Donald Resnick. 1330 pages, illustrated. W B Saunders Company, Philadelphia. 1989. £125.00. ISBN O72162215 1 Despite the very large size of this book the editor writes in his preface that it contains only essential information necessary in the interpretation of imaging studies of patients with musculoskeletal disorders. The volume is a condensation of a multivolume series that contains even more detail. The present compendium is directed specifically to trainees in orthopaedic surgery, rheumatology and radiology and there is no doubt that this audience will find the contents helpful. Established consultants might also benefit from thumbing the pages.
Second Vienna Shock Forum edited by G Schlag and H RedI. 1142 pages, illustrated. Alan R Liss Inc., New York. 1989. $196.00. ISBN 0 8451 5158 4 Printed from camera-ready copy this volume contains the papers given at the Second Vienna Shock Forum held in May 1988. An international team of contributors, many from departments of surgery. Sadly, not a single British surgical department is represented, highlighting the lack of interest shown in this field by surgeons in this country.
Arthritis: The Painfil Challenge by David Freedman. 18 pages, paperback. Arthritis Care, London. 1989. No price given. A report commissioned by the charity Arthritis Care detailing the enormity of the problem of arthritis, the large proportion of the population involved and the consequences to them as individuals and as families. It is suggested that the medical profession could focus more attention on the control and management of pain.
Diseases of the Gastrointestinal Tract and Liver edited by David J C Shearman, Niall D Finlayson and David C
Carter. 2nd edition. 1336 pages, illustrated. Churchill Livingstone, Edinburgh. 1989. £95.00. ISBN 0 443 03110 X The first edition of this comprehensive textbook was very successful when it was published in 1982. Seven years later the editors have updated throughout and introduced new contributors. Of special importance is the appointment of a surgical editor, thus acknowledging the importance of surgery in the development of gastroenterology and hepatology. Well produced, adequately illustrated, extensively referenced, this volume will need to be accessible to all gastroenterologists and surgeons with an interest in this specialty.
Reconstruction of the Thumb edited by A Landi, S De Luca and G De Santis. 344 pages, illustrated. Chapman and Hall Medical, London. 1989. £75.00. ISBN 0 412 29390 0 The thumb is the key digit for many everyday activities and its absence or deformity will cause extreme difficulty in the performance of many tasks. This book covers the history of thumb reconstruction and gives details of surgical and microvascular techniques. Comprehensive, with many illustrations and references.
Cancer Management in Man edited by L Goldson. 296 pages, illustrated. Kluwer Academic Publishers, Dordrecht. 1989. £59.95. ISBN 0 89838 998 4 Volume 9 in the series Cancer Growth and Progression covering detection, diagnosis, surgery, radiology, chronobiology and endocrine therapy.
Cancer Management in Man edited by P V Woolley. 248 pages, illustrated. Kluwer Academic Publishers, Dordrecht. 1989. £59.95. ISBN 0 89838 999 2 Volume 10 in the series Cancer Growth and Progression. This volume covers biological response modifiers, chemotherapy, antibiotics, hyperthermia and supporting measures such as nutrition and pain management.
