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- W2321761081 abstract "Although rare in children, smooth muscle tumours (SMTs) have been reported to originate from any part of the gastrointestinal tract, with the stomach being the usual site. Although well recognised in adults (1,2), the relative rarity of gastric leiomyosarcomas in children is reflected in only 34 cases reported in the scientific literature (3). These tumours are characterised by wide variation of microscopic features from one tumour to another and within the same tumour (4). Similarly, the clinical behaviour of these SMTs is frequently at variance with their histologic appearances, so that biopsy results are a poor guide to either treatment or prognosis. There is considerable variation in their microscopic and immunohistologic characteristics. Uncertainties about histogenesis, especially about whether some are of neural origin, has led to the labelling of many of these tumours as simply gastrointestinal stromal tumours (5). Smooth muscle tumours are usually asymptomatic during childhood, but they can cause abdominal pain, gastrointestinal haemorrhage, and anaemia. We report the case of a teenage girl who had severe, sudden-onset iron-deficiency anaemia, the underlying cause of which was found to be a leiomyosarcoma of the stomach. CASE REPORT A 14-year-old girl was admitted to our hospital with a history of pallor and lethargy. Eighteen months before admission, she had received a 3-month course of iron supplements for an episode of anaemia (haemoglobin, 6.4 g/dl). Apart from vague upper abdominal discomfort in the past, she had had no symptoms. In particular, there was no history of vomiting or melaena, and her appetite had always been good. She had not reached menarche at the time of initial presentation. On admission, the only abnormal physical finding was marked pallor. Initial investigations produced normal findings in a blood coagulation screen and haemoglobin of 3.1 g/dl. The serum iron levels, blood film, and other haematologic indices were all indicative of severe iron-deficiency anaemia. After blood transfusion, haemoglobin rose to 12.3 g/dl. Two days later, however, the patient had an episode of haematemesis associated with a further decrease in haemoglobin (to 7.4 g/dl). This further episode prompted an upper gastrointestinal endoscopy that showed a large mass in the lumen of the stomach with active bleeding (Fig. 1). The apex of the lesion was ulcerated and was clearly the source of the bleeding, and therefore no biopsies were performed at endoscopy. The gross appearance of the lesion was suggestive of leiomyoma or leiomyosarcoma.FIG. 1: Endoscopic view of the lobulated tumour on the lesser curve of the stomach seen from the gastric antrum with the pylorus in the background.At laparotomy, a mass measuring 11 × 7 cm was seen to involve most of the lesser curvature of the stomach (Fig. 2). There were numerous projections through the serosal surface, and the bulk of the tumour protruded into the lumen of the stomach in the prepyloric region. At the apex of the mass there was a large ulcer about 3 cm in diameter (Fig. 2). A subtotal gastrectomy with Polya-type anastomosis was performed because of the extent of the growth (Fig. 3).FIG. 2: Detailed view of the resected specimen showing the 3-cm ulcer crater first seen at endoscopy.FIG. 3: Projections of the gastric tumour seen at the serosal surface after partial gastrectomy.Pathology showed an ulcerated multinodular tumour that had invaded, and in places penetrated, the muscularis propria. Histology showed an extremely cellular tumour with a predominantly round cell, epithelioid appearance with some spindle cell areas. Necrosis was extensive, and mitoses varied from two to eight per 30 high-power fields (Fig. 4). Immunohistology showed positive staining with smooth muscle actin in spindle cell areas, together with some neurofilament positivity and focal S-100 positivity. Q-bend (CD-34) was strongly positive in both spindle and epithelioid areas. Other markers that showed very focal positivity included cytokeratin, vimentin, and neurone-specific enolase (NSE). Leukocyte common antigen, carcino-embryonic antigen (CEA), and desmin were negative. This immunocytologic profile is typical of this particular tumour, in which features are consistent with smooth muscle origin, but there are also features that suggest some neural differentiation.FIG. 4: High-power view of a typical histologic section of the tumour, showing numerous mitoses.The patient had an uneventful postoperative recovery. A follow-up computed tomographic scan of the abdomen and chest 6 months later showed no evidence of recurrence. She underwent 6-month gastroscopic examinations for 2 years and subsequently, 6-monthly computed tomographic scanning of the abdomen to monitor for recurrence. She was free of disease 30 months after surgery at the time of this report. DISCUSSION The tumour reported here was first described as an SMT by Martin et al. in 1960 (6). In 1962, Stout (7) published a series of 69 cases with detailed description of pathologic and clinical courses. The tumours arise from the muscularis propria. Their size varies considerably. When small, they retain an intramural position, but as they grow they project into the lumen or the serosal surface. Ulceration through the mucosa with resultant haemorrhage can occur with both the benign and malignant forms (4). In recent years, the wide range of appearance of these tumours has caused considerable controversy concerning both the histogenesis and the reliability of the histology in predicting behaviour. Immunohistology has frequently failed to demonstrate a convincing smooth muscle origin for many of these tumours, with some showing neural characteristics or a mixed neural and smooth muscle phenotype. A recent tendency has therefore been to label these tumours gastrointestinal stromal tumours. In this case, the actin positivity, even though patchy, provided some evidence of smooth muscle differentiation. A further difficulty is that the malignant potential of these tumours is not assessed reliably by histology. Newman et al. (8) have provided a means by which these tumours can be assessed that takes into account the cellular appearances and mitotic rate. In this case, the presence of between 2 and 8 mitoses per high-power field in the presence of an epithelioid appearance indicates that this should be regarded as a tumour with malignant potential-that is, a leiomyosarcoma when the immunohistology is taken into account. Other factors that may herald an unfavourable prognosis include size greater than 5 cm in diameter, tumour necrosis, and a tumour that has already invaded the serosa or surrounding structures at the time of initial examination (9). Because of this difficulty in assessing malignant potential histologically, prognosis is also difficult to assess. On a retrospective study of 41 patients the 5-year survival after surgery was 32% for those with high-grade neoplasms, 67% for those with large tumours, and 0% for those with local invasion (10). A similar study has shown a 25% to 30% 5-year survival rate (9). The cause of intestinal SMTs is unknown. One fifth of oesophageal tumours are known to have a familial tendency or association with other conditions such as Alport's syndrome. Similarly, gastric tumours have been reported to occur synchronously with SMTs of other parts of the intestine or malignancies outside the gastrointestinal tract (10). The coexistence of gastric leiomyosarcoma, pulmonary chondroma, and functioning extraadrenal paraganglioma is known as Carney's triad (11). This condition affects a younger age group with a predilection for female patients. Malignant SMTs are not uncommon in adults, accounting for 1% to 2% of gastric malignancies, but they are rare in children. They may spread locally to the lymph glands and liver or metastasize, usually to the lungs. Thirty-four paediatric cases have been published in the literature (3). The incidence of these tumours, however, has increased since acquired immune deficiency syndrome emerged as a worldwide health problem in the 1980s. There have been several recent reports about the occurrence of leiomyomas and leiomyosarcomas in children with HIV infection. Some of these cases have superadded Epstein-Barr virus infection (12-14). Most SMTs are asymptomatic. Autopsy data suggest that small growths (<2 cm) may be present in nearly half of all people over the age of 50 (15). When symptomatic, they usually cause symptoms of abdominal pain, bleeding, or iron-deficiency anaemia. Less frequently, they give rise to obstructive symptoms. In their review, Wurlitzer et al. showed that gastrointestinal bleeding and/or anaemia were the initial symptoms in more than 85% of the cases (3). Approximately 80% of gastric tumours are detected by barium study, usually as filling defects with or without ulceration (4). Computed tomography and flexible endoscopy, however, have been advocated as better diagnostic aids. The latter has the advantage of direct visualisation and the ability to perform multiple biopsies. It also enables the examiner to rule out coincidental lesions such as peptic ulcer. The current treatment of gastric leiomyosarcomas is surgical. Although subtotal or partial gastrectomy are recommended, wide local excision, when feasible, has shown comparable overall survival rates to those obtained after gastrectomy (4,16). Both radiotherapy and adjuvant chemotherapy have negligible effects. Their use, particularly in the management of gastrointestinal leiomyosarcomas, has been unrewarding (9,17). Gastrointestinal bleeding is an important cause of iron-deficiency anaemia in children but rarely is caused by malignant tumours. A thorough history and clinical examination may help to eliminate a few of the possible causes, but signs and symptoms do not usually correlate well with the source of bleeding in children (18). Advances in diagnostic techniques and their safety have improved the ability to localise the site of haemorrhage in 80% to 90% of cases (19). Finally, children with gastric leiomyosarcoma should be kept under close observation, to identify by regular chest radiographs and blood pressure monitoring the possible coexistence of other tumours or the evolution to Carney's triad. Acknowledgment: The authors thank Mr. Michael G. W. Kettlewell for permission to photograph the patient during surgery and Dr. Bryan F. Warren for advice on histopathologic aspects of this case." @default.
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- W2321761081 title "Gastric Leiomyosarcoma Presenting as Severe Iron-Deficiency Anaemia" @default.
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