Ausr. N . Z . J . Surg.
607
1990, 60, 607-611
GASTRIC LEIOMYOSARCOMAS: A GENERAL SURGICAL EXPERIENCE SEOW CHOEN AND
ABU RAUFF
Universio Department of Surgery, Singapore General Hospital, Singapore Leiomyosarcomas of the stomach are rare malignancies. In Singapore, they account for only 0.7% of all gastric tumours. Nine cases of gastric leiomyosarcomas were treated in this department over a 20 year period from 1968 to 1987. Pre-operative diagnosis was difficult. All our cases required laparotomy for histological diagnosis. Of the nine cases, there were eight gastrectomies (two repeated). None of the resected cases showed lymph node spread. There was only one long-term survival (8 years). Key word: gastric leiomyosarcomas.
Introduction Gastric leiomyosarcomas are very rare tumours, the reported incidence ranging from 0.25 to 3% of gastric tumours. 1-6 In Singapore, leiomyosarcomas account for 0.7% of all gastric neoplasm^.^
A general surgical experience MATERIALS AND METHODS
The records of the University Department of Surgery at Singapore General Hospital were examined for the period 1968- 1987. All cases of gastric leiomyosarcomas were reviewed, while leiomyomas were excluded. Nine cases of gastric leiomyosarcomas were found, accounting for 0.4% of all gastric malignancies in our department. Five patients were male and four were female. All were Chinese. The ages ranged from 47 to 87 years, the mean age being 61 years for females and 64 for males.
No preferred primary sites were evident in our series, but four of our nine cases arose from the proximal stomach. Histology revealed malignant leiomyosarcomas in eight cases, the ninth case being an epithelioid leiomyosarcoma. The liver and diaphragm were the most common organs to be involved in secondary spread. The patient with a skin secondary had had a previous laparotomy for leiomyosarcoma of the stomach. Of eight gastrectomies (two cases were operated on twice), no lymph node spread was found in any. Of the three unresectable cases (one at a second laparotomy), enlarged nodes were found in only two. Emergency laparotomies were performed in three patients, two because of massive gastrointestinal haemorrhage, and in the third patient following colonic perforation during colonoscopy . The only patient to have survived more than 5 years was a woman who had a total gastrectomy after an initial open gastric polypectomy showed leiomyosarcoma (Table 3).
RESULTS
The major symptoms seen in our patients were epigastric pain, associated with anorexia, loss of weight, lassitude, epigastric masses and various degrees of gastro-intestinal bleeding (Table 1). All of our cases required a laparotomy for definite diagnosis. In two cases, this took more than 10 months from the time of presentation. Computerized tomography (CT) scans and barium meal examinations showed some form of abnormality, but were misinterpreted as showing hepatic and splenic enlargements. Gastroscopy clearly missed the lesion completely in two out of four cases (Table 2). Correspondence: Mr Seow Choen, University Depmment of Surgery, Singapore General Hospital, Outrarn Road, Singapore 03 16. Accepted for publication 20 December 1989.
Discussion Early authors regarded gastric leiomyosarcomas as being of low grade malignancy.'-" This was subsequently thought to be due to the inclusion of leiomyoblastomas and cellular leiomyomas as leiomyosarcomas. 14-" Leiomyoblastomas, also known as epithelioid leiomyosarcomas, are now regarded as having an appreciable malignant potential. 14.16-21 Gastric leiomyosarcomas show no definite sexual p r e d i ~ e c t i o n , ~ . 3 , 6 . 8 . 1 6 . 1 7 . ~ 2 ~ ~although 4 some authors have found a male preponderance. ' 8 2 - 2 7 Leiomyosarcomas are usually asymptomatic when small, and even large ones present only after complications have occurred. 14.1h.'72s.28.29 There are, however, no definitive clinical features pathognomonic of leiomyosarcoma. Symptoms
CHOEN AND RAUFF
608
However, leiomyosarcomas may be intramural or of anorexia, weight loss, lethargy, epigastric pain, epigastric mass and gastro-intestinal haemorrhage even exogastric, and are easily missed on gastroscopy, I .23.45.46 It is important therefore that the are common and should arouse suspicion, espegastroscopist visualizes the stomach adequately. cially in an otherwise healthy person.5~'4~'7~24~27~30-38 Biopsies should be obtained from within any Other unusual symptoms and signs which may be mucosal ulcer to secure an adequate specimen. Any seen include haemoperit~neum,'~-~' abdominal abscess and peritonitis, '6.39 symptoms mimicking intraluminal bulging will indicate the need for further investigations, so that lesions will not be uterine pathology4' and Carney's triad.13 Clinical suspicion of a gastric lesion will often missed. If there is doubt, a barium study should lead to a gastroscopy. Characteristic endoscopic be undertaken. Barium meal has been noted to pick up 98% of all abnormalities in gastric leiomyosarfindings include a bulging extragastric mass with smooth effacement of the overlying gastric mucosa, comas, but precise diagnosis is rare. 1.3,4.'4~".3"25.3'' and central mucosal ulcerations. The gastric Helpful radiological features may include an inucosa usually retains normal extragastric mass with a gastric f i s t ~ l a . " , ' ~ . ~A~ Table 1. Leiomyosarcornas of the stomach: demography, symptomatology and timing of surgery Patient No. Sex
Age (years)
female male male female female male male female male
87 50 62 49 62 72 59 47 75
Duration of symptoms before presentation
Symptoms
Time between presentation and surgery
3 months 1 day I week 6 months days 1 year 1 day 2 days 3 months
Anorexia, weight loss, lethargy, pain Bleeding GIT" Anorexia, weight loss. lethargy, pain Anorexia, lethargy, pain, epigastric mass Epigastric mass Epigastric pain Bleeding GIT, anorexia Bleeding GIT Change of bowel habits
< 1 month Emergency 10 months 4 months < I month I2 months < 1 month Emergency < I month emergency
* GIT Gastrointestinal tract.
Table 2. Diagnostic modalities Patient number 5
Investigation I
2
3
4
Chest X-ray C
C
C
C*
Abdominal N D ultrasound Liver ND scintiscan Gastroscopy Benign gastric ulcer Fine-needle N D aspiration Barium Large meal ulcer '? carcinoma
ND
ND:i:
ND
C
?Hepatoma ? Hepatonia ND
'? Carcinoma ND
ND
ND
ND
Inconchive
Angiography N D
ND
Extrinsic compression N D ? liver '? spleen ND ND
CATscan
ND
ND
ND
Colonoscopy N D
ND
ND
'C: Clear and normal; .''ND: Not donc.
? Hepatoma ND
6
7
8
9
C
C
C
C
ND
ND
ND
ND
Lung secondary ND
C
ND
ND
ND
ND
ND
C
C
ND
ND
ND
ND
ND
ND
ND
Leiomyosarcoma
Lesser curve deformity ? liver '? Benign leiom yoma
Extrinsic N D mass '? spleen
ND
ND
'? Liver
'? RetroND peritoneal tuinour ND ND
ND
ND
Traumatic perforation
ND
Leioinyosarcolna ND
ND
ND
609
GASTRIC LEIOMYOSARCOMAS
laparatomy should be closed, no matter how extenconstant fixed angle between the extragastric mass sive the tumour spread, without a Biopand the adjacent gastric mucosa indicates a submucosal mass and may point to l ei o my ~ s ar co ma. ~ ~ sies of gastric lesions should be from the tumour itself rather than from lymph nodes, as enlarged Ultrasound-guided percutaneous fine-needle biopsy nodes are often reactive and not malignant. Several may sometimes secure the diagnosis, even when biopsies should be taken, as one may not be repregastroscopic biopsy has failed.29 ~ e n t a t i v e .Single ~ ~ biopsies are prone to sampling SURGICAL MANAGEMENT errors, as even tumours diagnosed as leiomyomas 7 7 ~4.25.34.35.39.53 on histology have metastasized. 8 ’--.Surgical excision of the tumour is the treatment The differentiation between benign and malignant of choice for gastric leiomyosarcomas. Chemosmooth muscle tumour may be quite difficult intratherapy and radiotherapy have had no demonstrable benefit,1.3,4.6.8.25.27.29,31.49.50 E~~~large turnours or operatively. Haemorrhage, cystic degeneration and central neurosis are not absolute criteria for maligtumours with secondary spread, or recurrences can nancy. Tumours larger than 8 cm, however, should be treated vigorously with good palliation. 1,4-6,x317, 22.2S.25.26.30.3I ,34,35,39.45.47.50,5 I be strongly suspected of being malignant.’” EnuCombinations of cleation of suspected leiomyomas is therefore not high grade, large size, high mitotic index, with recommended. 16. 17,31s4 adjacent organ involvement or secondary spread, Radical surgery is seldom indicated, as lymph however, are indicators of a poor prognosis,1,6.8,23.26.31.49although selected cases have bennode spread is rare and occurs only in advanced disease,~,3.4.6.14,2S,26,Sl,~4.5’ Total or subtotal gasefited from h e p a t e c t o r n i e ~ . ’ ~ ~ ~ ~ trectomies have been recommended by some All sub-mucosal gastric tumours should be resecauthors on the basis that microscopic spread of leioted, regardless of size.6 No tumour of the stomach myosarcoma may be beyond the level of the gross should be classed as inoperable or beyond therapy lesion, and that this procedure results in the best until a laparotomy had been performed, and no Table 3. Site of the tumour, extent of spread, primary and repeat procedures, and result
Patient no. Primary site
Extent of spread
Histology
Procedure
High lesser curve Cardia
Pancreas
Leiomyosarcoma
LTS*
Leiomyoblastoma
Cardiooesophagectomy Cardiooesophagectomy
Proximal stomach
Liver, spleen, diaphragm
Leiomyosarcoma
Liver Body and lesser curve Greater curve Transverse colon
Pancreas
Body
Leiom yosarcoma Leiomyosarcoma
Leiom yosarcoma
peritoneum Pancreas, spleen Leiomyosarcoma diaphragm, lung, skin, spleen
Proximal stomach Lesser curve
LTS
Greater curve Liver
* LTS: Limited
to
stomach
Leiomyosarcoma Leiom yosarcoma
Subsequent procedure -
Total gastrectomy, 4 years later
Cardiooesophagectomy , left lateral segmentectomy , splenectomy , partial diaphragm resection Subtotal gastrectomy, left lateral segmentectomy Subtotal gastrectomy omentectomy, and transverse colectomy Laparotomy and biopsy Proximal gastrectomy Laparotomy and biopsy 9 months later Laparotomy and Total gastrectomy pol ypectomy 2 weeks later Wedge resection -
Postop. result Well, 14th month Well, 14th month Died, 3rd year
Well, 15th month Died, 9th month Died, 4th
week Died, 4th month Well, at 8 years Died, 7th month
610
CHOEN AND RAUFF
survival.'.'"'' Other authors, however, argue for wedge resections with a 2 cm margin, after frozen sections of the margins, I.S.6.14,17.21.24,~5.2~,31.3S The choice of operation should be tailored to the location of the tumour on the stomach, the extent of local spread and the presence or otherwise of secondary tumour.
References I. LINDSAY P. C., ORDONEZ N. & RAAFJ . H. (1981)
2. 3. 4.
5. 6. 7. 8.
9. 10. II.
12. 13. 14.
15. 16. 17. 18. 19. 20. 21.
Gastric leiomyosarcoma: Clinical and pathological review of fifty patients. J . Surg. Oncol. 18, 399-421. JENSEN F. B . (1967) Primary gastric sarcoma: A review of the literature and report of nine personal cases. Acfa Chir. Scund. 133, 139-5 I , REMISEW. H. (1970) Gastric sarcomas. Amer. J . Surg. 120, 320-3. C~IATTERJEE D. & P O W ~ LA.L (1982) Mesenchymal tumours of the stomach: report of cases, review of literature, and analysis of leiomyosarcomas. Brit. J . Clin. Prac. 36, 26-33. Boss B . & CANDY J . (1970) Gastric leiomyoblastoma. Cur 11, 875-80. NAuhfcr T. C., ZORNOZA J . & O R D O NN~.Z (1982) Gastric leiomyosarcomas. Amer. J . Roentgenol. 139, 291-1. Llit H. P., DAYN.E. & SIIANMUGARAINAM K . (1988) Trends in cancer incidence in Singapore, 1968-82. IARC Scientific Pubhcdtions, No. 9 I (Lyon). BERGJ . & MCNEERG. (1960) Leiomyosarcoma of the stomach: A clinical and pathological study. Cuncer 13, 25-33. S . F. (1955) Gastric tuniours other than MARSHALL carcinoma: Report of unusual cases. Surg. Clin. N . Amer. 35, 693-702. CAMERON A. L., BRESLICH P. J. & MINOT P. (1941) Primary sarcoma of the stomach. Surgery 9,916-30. MARSHALL S . F. & MEISSNER W. A. (1950) Sarcoma of the stomach. Ann. Surg. 131, 824-37. R A B ~ N ~ ~ I IJC. H et a/. (1950) Sarcomas of the stomach. Amer. J . Surg. SO, 550-5. COWDELL R. H. (1950) Smooth muscle tumours of the gastrointestinal tract. B . J . S. 38, 3- I 1 . SMITHWICK W . , BIESECKEK J . L. & LEANI)P. M . ( 1969) Leiomyoblastoma: Behaviour and prognosis. Cmcer 24, 996- 1003. SCHOPIELD P. F. & Fox H. (1965) Leiomyoblastoma of the stomach. B . J . S. 52, 928-30. ABRAMSON D. J . ( I 973) Gastric leiomyoblastomas: Report of three cases, one malignant. Ann. SUQ. 178, 625-30. ABKAMSON D. J . (1973) Leiomyoblastomas of the stomach. S. G. 0. 136, 118-25. STOUT A . P. (1962) Bizarre smooth-muscle tumours of the stomach. Cancer 15, 400-9. LAWNP., HAJIXS. & Foo.rE F. W. (1972) Gastric and extragastric leiomyoblastomas: clinicopathologic study of 44 cases. Cancer 29, 305- II . FOURNIEK P. & MACNER D. (1966) Atypical smoothmuscle tumours of the stomach. Can. J . Surg. 9 , 262-5. HEKKINCTON J . L. (1968) Gastric leiomyoblastoma. Amer. Surg. 34, 681-6.
22. PIZIDCENJ . E. (1961) Leiomyosarcoma of the stomach. Ann. Surg. 153, 971-8. S (1984) Ad23. CELIKC . , LOPELC. & D O ~ A HS. 0. vanced leiomyosarcoma of the stomach. J . Surg. Oncol. 26, 83-5. 24. CROCKER D. W . (1969) Smooth-muscle tumours of the stomach. Ann. Surg. 170, 239-43. 25. G A R VW. I ~ H. H. (1965) Leiomyosarcoma of the stomach. B . J . S.52, 32-7. 26. SHACKLEFORD R . T., Wool, S. & B o r r w r r J . K . (1961) Primary sarcomas of the stomach. Amer. J . Surg. 101, 292-300. 27. SKANUALAKIS J . E . , GRAY S . W. & SIIEPEKI) D. (1960) Smooth-muscle tumours of the stomach (Collective Review). Int. Ahstr. Surg. 110, 209-26. 28. WRIGI-IT W.. WALLIS P. & HuNI-P. S . (1985) Haemorrhage [rom smooth-muscle tuniours of the upper gastrointestinal tract. Aust. N.Z. J . Sui-g. 55, 365-68. 29. GRAHAM S . M . , BALLANTYNE G. H. & MOI)I.IN I. M. ( 1987) Gastric epitheloid leiomyosarcoma: A curable gastric neoplasm. Amer. J . Gastroeiiferol. 87, 82-5. 30. JORDAN G. L. e f trl. (1955) Sarcomas of the stomach. s. c.0. 453-7. 3 I . SHIU M. H. et a/. (1982) Myosarconias of thc stomach: Natural history, prognostic [actors and management. Cancer 49, 177-87. 32. TIIOKBJAKNAKSON B . , PEARCE J . M. & B ~ A JL. M . (1959) Sarcoma of the stomach. A m r r . J . Surg. 97, 36-42. 33. HACGARII W . D. (1920) Sarcoma of the stomach. S. G. 0. 31, 505-1 I. 34. G I B E I Z SR~. NG., DOCKI-IIIY M . B . & GKAYH. K . ( 1954) Leiomyosarcoma of the stomach: Clinicopathologic study of 40 cases. S. G. 0. 98, 186-96. 35. APPBLMAN H. D. & HELwic E. B. (1977) Sarcomas of the stomach. Amer. J . Clin. Path. 67, 2-10. 36. O C H ~ Ns. E R& OCiisNEit A. (1955) Sarcoma o f the stomach: Analysis of 17 cases. Ann. Surg. 142, 804-9. 37. PARRY R. G. (1974) Smooth-niuscle tumours of the stomach and associated gastro-intestinal ulceration. Amer. Surg. 40, 2-585. 38. MARKS J . H. (1952) Leiomyosarcoma or the stomach with excavation of the centre of the tumour. Amer. J. Roentgenol. 67, 16-9. 39. SALMELA H. (1968) Smooth-muscle turnours of the stomach: A clinical study of I12 cases. Actci Chir. Scand. 134, 384-91. 40. RAMOS S . M. & MITSUDO S. (1984) Hemoperitoneuni secondary to exogastric leiomyosarcomas and leiomyoblastomas. Amer. J . Gci.stroentero1. 79, 637-4 I . 41. KELSEY J . R. (1966) Leiomyoblastoma of the stomach presenting as acute intraperitoneal haeniorrhage. Gustroenferology 51, 539-4 I . 42. SINNREICH M . , FRIEDMAN R . , DACWM . R . & SnxN W. (1966) Bizarre gastric leiomyoblastoma simulating a pedunculated uterine Fibromyoma. Ohs. Gynnecol. 27, 690-704. 43. R A A F A .F. ~ et nl. (1986) Carney's triad: Gastric leiomyosarcoma, pulmonary chondroma, and extra adrenal paraganglioma in young females. Histopathol. 10, 1325-33. 44. NELSONR. S . & LANZA F. L. (1968) Endoscopy in the diagnosis of gastric lymphoma and sarcoma. Amer. ' J . Gastroenterology 50, 31-46.
GASTRIC LEIOMYOSARCOMAS
45. SCHINDLER et al. (1946) Leiomyosarcoma of the stomach: Its roentgenologic and gastroscopic diagnosis and its possible relation to pernicious anaemia. S. G. 0. 82, 239-52. 46. MORRISSEY K., CHOE. S., GRAYG. F. & THORBJARNARSON B . (1978) Muscular tumours of the stomach: Clinical and pathological study of 113 cases. Ann. Surg. 178, 148-55. J. W. & KENDALL J. A. (1970) 47. PHILLIPS J . C., LINDSAY Gastric leiomyosarcoma: Roentgenologic and clinical findings. Digestive Diseases 15, 239-46. J. S., HERTZI. & KELLER R. J. (1981) Exogas48. TRAIN tric smooth-muscle tumours. Amer. J . Gastroenterol. 76, 544-50.
611
49. BEDIKIAN et al. (1980) Sarcoma of the stomach: Clinicopathologic study of 43 cases. J . Surg. Oncol. 13, 121-7. 50. HE L. J., WANGB. S . & CHENC. C . (1988) Smoothmuscle tumours of the digestive tract: Report of 160 cases. B . J . S. 75,184-6. 51. TAYLOR P. H. (1969) Leiomyosarcoma of the stomach. B . J . S. 56, 187-92. 52. TRIMBLE I. R. & HARKINS G. A. (1960) Sarcoma of the stomach. S. G. 0. 110, 437-42. 53. KAVLIE H. & WHITE T. T. (1972) Leiomyomas of the upper gastrointestinal tract. Surgery 71, 842-8. 54. CORNOG J. L. (1974) Gastric leiomyoblastoma: A clinical and ultra-structural study. Cancer 34, 71 1-9.
607
1990, 60, 607-611
GASTRIC LEIOMYOSARCOMAS: A GENERAL SURGICAL EXPERIENCE SEOW CHOEN AND
ABU RAUFF
Universio Department of Surgery, Singapore General Hospital, Singapore Leiomyosarcomas of the stomach are rare malignancies. In Singapore, they account for only 0.7% of all gastric tumours. Nine cases of gastric leiomyosarcomas were treated in this department over a 20 year period from 1968 to 1987. Pre-operative diagnosis was difficult. All our cases required laparotomy for histological diagnosis. Of the nine cases, there were eight gastrectomies (two repeated). None of the resected cases showed lymph node spread. There was only one long-term survival (8 years). Key word: gastric leiomyosarcomas.
Introduction Gastric leiomyosarcomas are very rare tumours, the reported incidence ranging from 0.25 to 3% of gastric tumours. 1-6 In Singapore, leiomyosarcomas account for 0.7% of all gastric neoplasm^.^
A general surgical experience MATERIALS AND METHODS
The records of the University Department of Surgery at Singapore General Hospital were examined for the period 1968- 1987. All cases of gastric leiomyosarcomas were reviewed, while leiomyomas were excluded. Nine cases of gastric leiomyosarcomas were found, accounting for 0.4% of all gastric malignancies in our department. Five patients were male and four were female. All were Chinese. The ages ranged from 47 to 87 years, the mean age being 61 years for females and 64 for males.
No preferred primary sites were evident in our series, but four of our nine cases arose from the proximal stomach. Histology revealed malignant leiomyosarcomas in eight cases, the ninth case being an epithelioid leiomyosarcoma. The liver and diaphragm were the most common organs to be involved in secondary spread. The patient with a skin secondary had had a previous laparotomy for leiomyosarcoma of the stomach. Of eight gastrectomies (two cases were operated on twice), no lymph node spread was found in any. Of the three unresectable cases (one at a second laparotomy), enlarged nodes were found in only two. Emergency laparotomies were performed in three patients, two because of massive gastrointestinal haemorrhage, and in the third patient following colonic perforation during colonoscopy . The only patient to have survived more than 5 years was a woman who had a total gastrectomy after an initial open gastric polypectomy showed leiomyosarcoma (Table 3).
RESULTS
The major symptoms seen in our patients were epigastric pain, associated with anorexia, loss of weight, lassitude, epigastric masses and various degrees of gastro-intestinal bleeding (Table 1). All of our cases required a laparotomy for definite diagnosis. In two cases, this took more than 10 months from the time of presentation. Computerized tomography (CT) scans and barium meal examinations showed some form of abnormality, but were misinterpreted as showing hepatic and splenic enlargements. Gastroscopy clearly missed the lesion completely in two out of four cases (Table 2). Correspondence: Mr Seow Choen, University Depmment of Surgery, Singapore General Hospital, Outrarn Road, Singapore 03 16. Accepted for publication 20 December 1989.
Discussion Early authors regarded gastric leiomyosarcomas as being of low grade malignancy.'-" This was subsequently thought to be due to the inclusion of leiomyoblastomas and cellular leiomyomas as leiomyosarcomas. 14-" Leiomyoblastomas, also known as epithelioid leiomyosarcomas, are now regarded as having an appreciable malignant potential. 14.16-21 Gastric leiomyosarcomas show no definite sexual p r e d i ~ e c t i o n , ~ . 3 , 6 . 8 . 1 6 . 1 7 . ~ 2 ~ ~although 4 some authors have found a male preponderance. ' 8 2 - 2 7 Leiomyosarcomas are usually asymptomatic when small, and even large ones present only after complications have occurred. 14.1h.'72s.28.29 There are, however, no definitive clinical features pathognomonic of leiomyosarcoma. Symptoms
CHOEN AND RAUFF
608
However, leiomyosarcomas may be intramural or of anorexia, weight loss, lethargy, epigastric pain, epigastric mass and gastro-intestinal haemorrhage even exogastric, and are easily missed on gastroscopy, I .23.45.46 It is important therefore that the are common and should arouse suspicion, espegastroscopist visualizes the stomach adequately. cially in an otherwise healthy person.5~'4~'7~24~27~30-38 Biopsies should be obtained from within any Other unusual symptoms and signs which may be mucosal ulcer to secure an adequate specimen. Any seen include haemoperit~neum,'~-~' abdominal abscess and peritonitis, '6.39 symptoms mimicking intraluminal bulging will indicate the need for further investigations, so that lesions will not be uterine pathology4' and Carney's triad.13 Clinical suspicion of a gastric lesion will often missed. If there is doubt, a barium study should lead to a gastroscopy. Characteristic endoscopic be undertaken. Barium meal has been noted to pick up 98% of all abnormalities in gastric leiomyosarfindings include a bulging extragastric mass with smooth effacement of the overlying gastric mucosa, comas, but precise diagnosis is rare. 1.3,4.'4~".3"25.3'' and central mucosal ulcerations. The gastric Helpful radiological features may include an inucosa usually retains normal extragastric mass with a gastric f i s t ~ l a . " , ' ~ . ~A~ Table 1. Leiomyosarcornas of the stomach: demography, symptomatology and timing of surgery Patient No. Sex
Age (years)
female male male female female male male female male
87 50 62 49 62 72 59 47 75
Duration of symptoms before presentation
Symptoms
Time between presentation and surgery
3 months 1 day I week 6 months days 1 year 1 day 2 days 3 months
Anorexia, weight loss, lethargy, pain Bleeding GIT" Anorexia, weight loss. lethargy, pain Anorexia, lethargy, pain, epigastric mass Epigastric mass Epigastric pain Bleeding GIT, anorexia Bleeding GIT Change of bowel habits
< 1 month Emergency 10 months 4 months < I month I2 months < 1 month Emergency < I month emergency
* GIT Gastrointestinal tract.
Table 2. Diagnostic modalities Patient number 5
Investigation I
2
3
4
Chest X-ray C
C
C
C*
Abdominal N D ultrasound Liver ND scintiscan Gastroscopy Benign gastric ulcer Fine-needle N D aspiration Barium Large meal ulcer '? carcinoma
ND
ND:i:
ND
C
?Hepatoma ? Hepatonia ND
'? Carcinoma ND
ND
ND
ND
Inconchive
Angiography N D
ND
Extrinsic compression N D ? liver '? spleen ND ND
CATscan
ND
ND
ND
Colonoscopy N D
ND
ND
'C: Clear and normal; .''ND: Not donc.
? Hepatoma ND
6
7
8
9
C
C
C
C
ND
ND
ND
ND
Lung secondary ND
C
ND
ND
ND
ND
ND
C
C
ND
ND
ND
ND
ND
ND
ND
Leiomyosarcoma
Lesser curve deformity ? liver '? Benign leiom yoma
Extrinsic N D mass '? spleen
ND
ND
'? Liver
'? RetroND peritoneal tuinour ND ND
ND
ND
Traumatic perforation
ND
Leioinyosarcolna ND
ND
ND
609
GASTRIC LEIOMYOSARCOMAS
laparatomy should be closed, no matter how extenconstant fixed angle between the extragastric mass sive the tumour spread, without a Biopand the adjacent gastric mucosa indicates a submucosal mass and may point to l ei o my ~ s ar co ma. ~ ~ sies of gastric lesions should be from the tumour itself rather than from lymph nodes, as enlarged Ultrasound-guided percutaneous fine-needle biopsy nodes are often reactive and not malignant. Several may sometimes secure the diagnosis, even when biopsies should be taken, as one may not be repregastroscopic biopsy has failed.29 ~ e n t a t i v e .Single ~ ~ biopsies are prone to sampling SURGICAL MANAGEMENT errors, as even tumours diagnosed as leiomyomas 7 7 ~4.25.34.35.39.53 on histology have metastasized. 8 ’--.Surgical excision of the tumour is the treatment The differentiation between benign and malignant of choice for gastric leiomyosarcomas. Chemosmooth muscle tumour may be quite difficult intratherapy and radiotherapy have had no demonstrable benefit,1.3,4.6.8.25.27.29,31.49.50 E~~~large turnours or operatively. Haemorrhage, cystic degeneration and central neurosis are not absolute criteria for maligtumours with secondary spread, or recurrences can nancy. Tumours larger than 8 cm, however, should be treated vigorously with good palliation. 1,4-6,x317, 22.2S.25.26.30.3I ,34,35,39.45.47.50,5 I be strongly suspected of being malignant.’” EnuCombinations of cleation of suspected leiomyomas is therefore not high grade, large size, high mitotic index, with recommended. 16. 17,31s4 adjacent organ involvement or secondary spread, Radical surgery is seldom indicated, as lymph however, are indicators of a poor prognosis,1,6.8,23.26.31.49although selected cases have bennode spread is rare and occurs only in advanced disease,~,3.4.6.14,2S,26,Sl,~4.5’ Total or subtotal gasefited from h e p a t e c t o r n i e ~ . ’ ~ ~ ~ ~ trectomies have been recommended by some All sub-mucosal gastric tumours should be resecauthors on the basis that microscopic spread of leioted, regardless of size.6 No tumour of the stomach myosarcoma may be beyond the level of the gross should be classed as inoperable or beyond therapy lesion, and that this procedure results in the best until a laparotomy had been performed, and no Table 3. Site of the tumour, extent of spread, primary and repeat procedures, and result
Patient no. Primary site
Extent of spread
Histology
Procedure
High lesser curve Cardia
Pancreas
Leiomyosarcoma
LTS*
Leiomyoblastoma
Cardiooesophagectomy Cardiooesophagectomy
Proximal stomach
Liver, spleen, diaphragm
Leiomyosarcoma
Liver Body and lesser curve Greater curve Transverse colon
Pancreas
Body
Leiom yosarcoma Leiomyosarcoma
Leiom yosarcoma
peritoneum Pancreas, spleen Leiomyosarcoma diaphragm, lung, skin, spleen
Proximal stomach Lesser curve
LTS
Greater curve Liver
* LTS: Limited
to
stomach
Leiomyosarcoma Leiom yosarcoma
Subsequent procedure -
Total gastrectomy, 4 years later
Cardiooesophagectomy , left lateral segmentectomy , splenectomy , partial diaphragm resection Subtotal gastrectomy, left lateral segmentectomy Subtotal gastrectomy omentectomy, and transverse colectomy Laparotomy and biopsy Proximal gastrectomy Laparotomy and biopsy 9 months later Laparotomy and Total gastrectomy pol ypectomy 2 weeks later Wedge resection -
Postop. result Well, 14th month Well, 14th month Died, 3rd year
Well, 15th month Died, 9th month Died, 4th
week Died, 4th month Well, at 8 years Died, 7th month
610
CHOEN AND RAUFF
survival.'.'"'' Other authors, however, argue for wedge resections with a 2 cm margin, after frozen sections of the margins, I.S.6.14,17.21.24,~5.2~,31.3S The choice of operation should be tailored to the location of the tumour on the stomach, the extent of local spread and the presence or otherwise of secondary tumour.
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