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Elastofibroma of the pylorus presenting as gastric outlet obstruction: a case report and review of literature Elastofibroma is a very rare benign tumour that is very rarely reported within the gastrointestinal tract. We hereby report the only case of elastofibroma presenting as a gastric outlet obstruction. A 61-year-old woman presented to the emergency department with a sudden onset of epigastric pain radiating to her back. It was associated with nausea and vomiting. She had experienced similar episodes over the previous 5 months. No weight loss was noted. Her past medical history was of a duodenal ulcer 20 years ago, asthma, hypertension and ischaemic heart disease. Upon examination, a mildly distended abdomen with mild tenderness in epigastrium was noted.
Fig. 1. Computed tomography scan of abdomen with irregular mass at the pylorus/first part of duodenum (white arrow) distally to a dilated stomach.
Fig. 2. Microscopy of elastin stains of the submucosal elastofibroma showing the beaded and partly fragmented elastin fibres lying within the fibrous tissue of low cellularity in 4× (a) and 40× (b) magnifications (mucosal side indicated by black arrow).
© 2014 Royal Australasian College of Surgeons
(a)
A computed tomography scan of the abdomen showed a dilated stomach with an irregular thickening of the pylorus/first part of duodenum (Fig. 1) with several prominent regional lymph nodes suggestive of a gastric malignancy. On gastroscopy, a deformed and stenosed pylorus was noted. The overlying mucosa was irregular with ulcerations in the duodenum; however, the mucosal biopsies were normal. The patient underwent a distal gastrectomy following staging investigations. Intraoperatively, a smooth rubbery mass was felt in pylorus/first part of the duodenum, which could easily be resected with a distal gastrectomy. The patient had an uneventful postoperative recovery. Macroscopic examination revealed a 3 × 2 cm firm grey/white nodular submucosal tumour above the muscularis propria protruding into and obstructing the lumen of the pylorus. On microscopy, after haematoxylin and eosin staining and elastin staining, an area of thickened beaded and partly fragmented elastin fibres lying within the fibrous tissue of low cellularity was observed within the submucosa. Additional immunohistochemistry ruled out a malignancy or a gastrointestinal stromal tumour (Fig. 2). Elastofibromas are rare tumours with about 350 cases reported since 1980, with the vast majority described in the subscapular region – elastofibroma dorsi. There are only single reports in areas outside the subscapular region in hand, foot, ocular, ventricular and intra-spinal locations.1 Gastrointestinal elastrofibromas are rare with single reports in the oral cavity, stomach, sigmoid colon and omentum. There has been no report of an elastofibroma causing gastrointestinal obstruction. The pathogenesis of an elastofibroma remains unknown. It is postulated that elastofibroma dorsi might be a response to repetitive strain and is derived from fibroblasts or even the periosteum, although this theory does not apply to the gastrointestinal tract.1 Electron microscopy studies have suggested an abnormal elastin
(b)
ANZ J Surg •• (2014) ••–••
2
deposition1 and immunohistology has shown active neovascularization within the elastofibroma.2 Also, additional perivascular elastin deposits outside the main lesion have been observed in both stomach and in elastofibroma dorsi specimens.2,3 In reviewing 170 cases of elastofibroma dorsi, an increased appearance within families was found, suggesting a hereditary component.4 Only four cases of gastric elastofibroma have been reported so far. Two of these were incidental findings in gastrectomies for ulcer disease and gastric adenocarcinoma.2,3 The other two patients experienced atypical epigastric discomfort and had stomach wall thickening on imaging.5,6 Both were operated on with the suspicion of a gastric malignancy, as was the case in our patient.
References 1. Kumaratilake JS, Krishnan R, Lomax-Smith J, Cleary EG. Elastofibroma: disturbed elastic fibrillogenesis by periosteal-derived cells? An immunoelectron microscopic and in situ hybridization study. Hum. Pathol. 1991; 22: 1017–29. 2. Kai K, Kusano K, Sakai M et al. Active neovascularization and possible vascular-centric development of gastric and periscapular elastofibromas. Virchows Arch. 2009; 454: 181–8.
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3. Enjoji M, Sumiyoshi K, Sueyoshi K. Elastofibromatous lesion of the stomach in a patient with elastofibroma dorsi. Am. J. Surg. Pathol. 1985; 9: 233–7. 4. Nagamine N, Nohara Y, Ito E. Elastofibroma in Okinawa. A clinicopathologic study of 170 cases. Cancer 1982; 50: 1794–805. 5. Lau KN, Sindram D, Ahrens WA, Agee N, Martinie JB, Iannitti DA. Gastric elastofibroma. Am. Surg. 2010; 76: 1446–8. 6. Saint-Paul MC, Musso S, Cardot-Leccia N et al. Elastofibroma of the stomach. Pathol. Res. Pract. 2003; 199: 637–9.
Edwin Beenen,* MD Michael Brown,† FRCPA Sivakumar Gananadha,* FRACS Charles Mosse,* FRACS *Upper Gastro-Intestinal and Hepato-Biliary Unit, The Canberra Hospital, Canberra, Australian Capital Territory, Australia and †Department of Pathology, The Canberra Hospital, Canberra, Australian Capital Territory, Australia doi: 10.1111/ans.12793
© 2014 Royal Australasian College of Surgeons
Elastofibroma of the pylorus presenting as gastric outlet obstruction: a case report and review of literature Elastofibroma is a very rare benign tumour that is very rarely reported within the gastrointestinal tract. We hereby report the only case of elastofibroma presenting as a gastric outlet obstruction. A 61-year-old woman presented to the emergency department with a sudden onset of epigastric pain radiating to her back. It was associated with nausea and vomiting. She had experienced similar episodes over the previous 5 months. No weight loss was noted. Her past medical history was of a duodenal ulcer 20 years ago, asthma, hypertension and ischaemic heart disease. Upon examination, a mildly distended abdomen with mild tenderness in epigastrium was noted.
Fig. 1. Computed tomography scan of abdomen with irregular mass at the pylorus/first part of duodenum (white arrow) distally to a dilated stomach.
Fig. 2. Microscopy of elastin stains of the submucosal elastofibroma showing the beaded and partly fragmented elastin fibres lying within the fibrous tissue of low cellularity in 4× (a) and 40× (b) magnifications (mucosal side indicated by black arrow).
© 2014 Royal Australasian College of Surgeons
(a)
A computed tomography scan of the abdomen showed a dilated stomach with an irregular thickening of the pylorus/first part of duodenum (Fig. 1) with several prominent regional lymph nodes suggestive of a gastric malignancy. On gastroscopy, a deformed and stenosed pylorus was noted. The overlying mucosa was irregular with ulcerations in the duodenum; however, the mucosal biopsies were normal. The patient underwent a distal gastrectomy following staging investigations. Intraoperatively, a smooth rubbery mass was felt in pylorus/first part of the duodenum, which could easily be resected with a distal gastrectomy. The patient had an uneventful postoperative recovery. Macroscopic examination revealed a 3 × 2 cm firm grey/white nodular submucosal tumour above the muscularis propria protruding into and obstructing the lumen of the pylorus. On microscopy, after haematoxylin and eosin staining and elastin staining, an area of thickened beaded and partly fragmented elastin fibres lying within the fibrous tissue of low cellularity was observed within the submucosa. Additional immunohistochemistry ruled out a malignancy or a gastrointestinal stromal tumour (Fig. 2). Elastofibromas are rare tumours with about 350 cases reported since 1980, with the vast majority described in the subscapular region – elastofibroma dorsi. There are only single reports in areas outside the subscapular region in hand, foot, ocular, ventricular and intra-spinal locations.1 Gastrointestinal elastrofibromas are rare with single reports in the oral cavity, stomach, sigmoid colon and omentum. There has been no report of an elastofibroma causing gastrointestinal obstruction. The pathogenesis of an elastofibroma remains unknown. It is postulated that elastofibroma dorsi might be a response to repetitive strain and is derived from fibroblasts or even the periosteum, although this theory does not apply to the gastrointestinal tract.1 Electron microscopy studies have suggested an abnormal elastin
(b)
ANZ J Surg •• (2014) ••–••
2
deposition1 and immunohistology has shown active neovascularization within the elastofibroma.2 Also, additional perivascular elastin deposits outside the main lesion have been observed in both stomach and in elastofibroma dorsi specimens.2,3 In reviewing 170 cases of elastofibroma dorsi, an increased appearance within families was found, suggesting a hereditary component.4 Only four cases of gastric elastofibroma have been reported so far. Two of these were incidental findings in gastrectomies for ulcer disease and gastric adenocarcinoma.2,3 The other two patients experienced atypical epigastric discomfort and had stomach wall thickening on imaging.5,6 Both were operated on with the suspicion of a gastric malignancy, as was the case in our patient.
References 1. Kumaratilake JS, Krishnan R, Lomax-Smith J, Cleary EG. Elastofibroma: disturbed elastic fibrillogenesis by periosteal-derived cells? An immunoelectron microscopic and in situ hybridization study. Hum. Pathol. 1991; 22: 1017–29. 2. Kai K, Kusano K, Sakai M et al. Active neovascularization and possible vascular-centric development of gastric and periscapular elastofibromas. Virchows Arch. 2009; 454: 181–8.
Images for surgeons
3. Enjoji M, Sumiyoshi K, Sueyoshi K. Elastofibromatous lesion of the stomach in a patient with elastofibroma dorsi. Am. J. Surg. Pathol. 1985; 9: 233–7. 4. Nagamine N, Nohara Y, Ito E. Elastofibroma in Okinawa. A clinicopathologic study of 170 cases. Cancer 1982; 50: 1794–805. 5. Lau KN, Sindram D, Ahrens WA, Agee N, Martinie JB, Iannitti DA. Gastric elastofibroma. Am. Surg. 2010; 76: 1446–8. 6. Saint-Paul MC, Musso S, Cardot-Leccia N et al. Elastofibroma of the stomach. Pathol. Res. Pract. 2003; 199: 637–9.
Edwin Beenen,* MD Michael Brown,† FRCPA Sivakumar Gananadha,* FRACS Charles Mosse,* FRACS *Upper Gastro-Intestinal and Hepato-Biliary Unit, The Canberra Hospital, Canberra, Australian Capital Territory, Australia and †Department of Pathology, The Canberra Hospital, Canberra, Australian Capital Territory, Australia doi: 10.1111/ans.12793
© 2014 Royal Australasian College of Surgeons
