ClinicalRadiology(1990) 42, 356-358
Case Report: Recurrent Liposarcoma of the Oesophagus S. P. Y A T E S
a n d M . C. C O L L I N S
X-ray Department, Royal Hallamshire Hospital, Sheffield A case of recurrent liposarcoma of the oesophagus is presented. The clinical and radiological findings are discussed. Our case displays the characteristic features and behaviour of a c o m m o n sarcoma arising in a rare site. Y a t e s , S.P. & C o l l i n s , M . C . ( 1 9 9 0 ) Clinical Radiology 42, 3 5 6 358. C a s e R e p o r t : R e c u r r e n t L i p o s a r c o m a
CASE
of the Oesophagus.
REPORT
A 49-year-old Italian waiter presented with a 6 month history of progressive dysphagia to solid foods. He also suffered retro-sternal pain on swallowing with radiation through to the back. There was weight loss of 6 kg in spite of a good appetite. The patient recalled an episode prior to the onset of dysphagia when he vomited after a meal and for a while had the sensation of having 'two tongues' in his mouth. Barium swallow demonstrated a large lobulated intraluminal oesophageal tumour extending from immediately below the cricopharyngeus to 4 cm above the gastro-oesophageal junction (Fig. 1). The chest radiograph taken at presentation demonstrated the smooth contour of the tumour, outlined by air within the dilated oesophagus. Appearances at rigid oesophagoscopy suggested a leiomyoma on a broad pedicle. The shape and extent of the attachment of the pedicle to the oesophageal wall could not be accurately determined either by endoscopy or on the barium study. Biopsy revealed only squamous epithelium, with no evidence of malignancy. Thoracotomy and oesophagotomy revealed a large fleshy tumour attached to the oesophageal wall by a wide pedicle at its upper end. Histological examination showed the lesion to be a myxoid liposarcoma of very low grade malignancy. There was tumour at the margin of the resected specimen. The patient was followed up for more than 3 years with regular endoscopy but no evidence of stricture or recurrent tumour was found. Six and a half years after resection of the tumour the patient was rereferred with dysphagia of gradual onset which had become so severe that he could take only fluids. Barium swallow demonstrated recurrence of a turnout similar in appearance but smaller than the original lesion (Fig. 2). CT of the chest showed the tumour to be of predominantly fatty density with a few strands of soft tissue density internally (Fig. 3). The recurrent tumour extended from the level of the T1/2 disc to 2 cm below the level of the carina. The tumour was attached by a broad pedicle at its upper end (Fig. 4). There was marked compression of the trachea and major bronchi. A three stage oesophagectomy was performed. The tumour was excised to 3 cm above its macroscopic extent. Histological features were the same as those of the original tumour, and again there was tumour at the resected margin. Post-operative recovery was uncomplicated. Check endoscopy after 7 months showed no evidence of recurrent tumour, though there was a mild anastomotic stricture which was dilated uneventfully. The patient remained well for 2 years after his oesophagectomy, with no significant dysphagia. He then began to lose weight and developed a cough, chest pain and a right-sided pleural effusion. The clinical diagnosis of bronchial carcinoma had not been confirmed by bronchoscopic or other means when the patient died after a short period of very rapid deterioration. There was no post-mortem.
DISCUSSION I t is u n u s u a l t o see a l a r g e f l e s h y i n t r a l u m i n a l c o m p o n e n t i n o e s o p h a g e a l t u m o u r s , b u t t h i s is a n o c c a s i o n a l finding with a variety of both benign and malignant histological types. These include leiomyoma and leiomyosarcoma, lipoma, carcinosarcoma and melanoma (Laufer Correspondence to: Dr S. P. Yates, X-ray Department, Royal Hallamshire Hospital, Glossop Road, Sheffield S10 2JF.
Fig. 1 -Barium swallow at initial presentation demonstrates a large intraluminal oesophageal tumour.
a n d A k i y o s h i , 1979; H a r e l l , 1986). I t is u n u s u a l for carcinoma of the oesophagus to present as a polypoid intraluminal mass. A l t h o u g h a r a r e t u r n o u t , l i p o s a r c o m a is t h e most c o m m o n o f t h e s o f t t i s s u e s a r c b m a s , a n d o c c u r s mostly b e y o n d 30 y e a r s . V a r y i n g d e g r e e s o f d i f f e r e n t i a t i o n are seen. M y x o i d l e s i o n s w i t h l i p o b l a s t s e m b e d d e d in a g e l a t i n o u s m a t r i x a c c o u n t f o r a p p r o x i m a t e l y h a l f the c a s e s ( A n d e r s o n , 1985). T h e r e m a i n i n g t u m o u r s a r e well d i f f e r e n t i a t e d f a t t y l e s i o n s , a n d f i r m e r , r o u n d cell or pleomorphic lesions which are more aggressive. Neither t h e f a t t y n o r m y x o i d l e s i o n s m e t a s t a s i z e f r e q u e n t l y , but
RECURRENT LIPOSARCOMA OF THE OESOPHAGUS
357
Fig. 3 - CT at the level of D5 after ingestion of oral contrast medium. Soft tissue strands are seen within the fatty tumour. Note compression of the trachea.
(a)
Fig. 4 - CT at D2 showing the root of the pedicle (arrow).
(b) Fig. 2 - Barium swallow (i00 mm format) demonstrates recurrent tumour. (a) Upper end. (b) Lower end.
there is a well recognized tendency for repeated recurrence following excision, sometimes over a period of several years. The myxoid tumours occasionally recur in a less well differentiated round cell form, showing more aggressive histological and clinical features. Hadju (1979) studied a large series of 242 patients with liposarcoma at various sites, and found a 12% incidence of a histologically different but co-existent primary tumour.
Liposarcomas occur most commonly in the soft tissues of the thigh or in the retroperitoneum. Involvement of the mediastinum is also well recognized (Prohm et al., 1981). Lesions of the gastro-intestinal tract are extremely rare, and we know of only one other case of oesophageal liposarcoma (Mansour et al., 1983). Mansour's case was a 53-year-old man who developed a squamous bronchial carcinoma l0 months prior to the liposarcoma. Our case exhibits many typical features of this rare tumour. The lesion is slow growing and bulky, but being pedunculated it is also mobile. Our patient shares with the previously reported case the unusual symptom of intermittent regurgitation of the tumour into the oral cavity (Mansour et al., 1983). It is noteworthy that, in spite of
358
CLINICAL RADIOLOGY
severe compression of the tracheobronchial tree, our patient remained free from respiratory problems due to the liposarcoma. We believe that the rapid deterioration and death of our patient was due to a malignant primary bronchial neoplasm rather than recurrent liposarcoma. Although not pathologically proven, development of such a tumour would not be unusual in a patient with liposarcoma, and represents a further similarity with the previously reported case. There is considerable difficulty assessing the total extent of the lesion, both by radiological means (barium swallow and CT) and also at surgery. On two occasions tumour has been found at the margin of the resected specimen in the absence of any radiological or macroscopic evidence of spread of the tumour. Slow local recurrence is typical, and the clinical course in this patient emphasizes the value of long term follow up. Acknowledgements. We thank Mr C. J. Stoddard for allowing us to report on his patient, Dr R. A. Nakielny for interpreting the CT scan, and Mrs Y. Steel for typing the manuscript.
REFERENCES Anderson, JR (Ed.) (1985). Muir's Textbook of Pathology, 12th edition, 23.67. Edward Arnold, London. Hadju, SI (1979). Pathology of Soft Tissue Tumours. Lea & Febiger, Philadelphia. Harell, GS (1986). The oesophagus. In Diagnostic Radiology, eds, Grainger, RG & Allison, DJ, pp. 743-781. Churchill Livingstone, London. Laufer, I & Akiyoshi, Y (1979). Tumors of the esophagus. In Double Contrast Gastrointestinal Radiology, ed. Laufer, I, pp. 129-153. WB Saunders, Philadelphia. Mansour, KA, Fritz, RC~ Jacobs, DM & Vellios, F (1983). Peduncu. lated liposarcoma of the esophagus: a first case report. Journal of Thoracic and Cardiovascular Surgery, 86, 447 450. Prohm, P, Winter, J & Watowski, L (1981). Liposarcoma of the mediastinum. Case report and a review of the literature. Journalof Thoracic and Cardiovascular Surgery, 29, 119-121.
Case Report: Recurrent Liposarcoma of the Oesophagus S. P. Y A T E S
a n d M . C. C O L L I N S
X-ray Department, Royal Hallamshire Hospital, Sheffield A case of recurrent liposarcoma of the oesophagus is presented. The clinical and radiological findings are discussed. Our case displays the characteristic features and behaviour of a c o m m o n sarcoma arising in a rare site. Y a t e s , S.P. & C o l l i n s , M . C . ( 1 9 9 0 ) Clinical Radiology 42, 3 5 6 358. C a s e R e p o r t : R e c u r r e n t L i p o s a r c o m a
CASE
of the Oesophagus.
REPORT
A 49-year-old Italian waiter presented with a 6 month history of progressive dysphagia to solid foods. He also suffered retro-sternal pain on swallowing with radiation through to the back. There was weight loss of 6 kg in spite of a good appetite. The patient recalled an episode prior to the onset of dysphagia when he vomited after a meal and for a while had the sensation of having 'two tongues' in his mouth. Barium swallow demonstrated a large lobulated intraluminal oesophageal tumour extending from immediately below the cricopharyngeus to 4 cm above the gastro-oesophageal junction (Fig. 1). The chest radiograph taken at presentation demonstrated the smooth contour of the tumour, outlined by air within the dilated oesophagus. Appearances at rigid oesophagoscopy suggested a leiomyoma on a broad pedicle. The shape and extent of the attachment of the pedicle to the oesophageal wall could not be accurately determined either by endoscopy or on the barium study. Biopsy revealed only squamous epithelium, with no evidence of malignancy. Thoracotomy and oesophagotomy revealed a large fleshy tumour attached to the oesophageal wall by a wide pedicle at its upper end. Histological examination showed the lesion to be a myxoid liposarcoma of very low grade malignancy. There was tumour at the margin of the resected specimen. The patient was followed up for more than 3 years with regular endoscopy but no evidence of stricture or recurrent tumour was found. Six and a half years after resection of the tumour the patient was rereferred with dysphagia of gradual onset which had become so severe that he could take only fluids. Barium swallow demonstrated recurrence of a turnout similar in appearance but smaller than the original lesion (Fig. 2). CT of the chest showed the tumour to be of predominantly fatty density with a few strands of soft tissue density internally (Fig. 3). The recurrent tumour extended from the level of the T1/2 disc to 2 cm below the level of the carina. The tumour was attached by a broad pedicle at its upper end (Fig. 4). There was marked compression of the trachea and major bronchi. A three stage oesophagectomy was performed. The tumour was excised to 3 cm above its macroscopic extent. Histological features were the same as those of the original tumour, and again there was tumour at the resected margin. Post-operative recovery was uncomplicated. Check endoscopy after 7 months showed no evidence of recurrent tumour, though there was a mild anastomotic stricture which was dilated uneventfully. The patient remained well for 2 years after his oesophagectomy, with no significant dysphagia. He then began to lose weight and developed a cough, chest pain and a right-sided pleural effusion. The clinical diagnosis of bronchial carcinoma had not been confirmed by bronchoscopic or other means when the patient died after a short period of very rapid deterioration. There was no post-mortem.
DISCUSSION I t is u n u s u a l t o see a l a r g e f l e s h y i n t r a l u m i n a l c o m p o n e n t i n o e s o p h a g e a l t u m o u r s , b u t t h i s is a n o c c a s i o n a l finding with a variety of both benign and malignant histological types. These include leiomyoma and leiomyosarcoma, lipoma, carcinosarcoma and melanoma (Laufer Correspondence to: Dr S. P. Yates, X-ray Department, Royal Hallamshire Hospital, Glossop Road, Sheffield S10 2JF.
Fig. 1 -Barium swallow at initial presentation demonstrates a large intraluminal oesophageal tumour.
a n d A k i y o s h i , 1979; H a r e l l , 1986). I t is u n u s u a l for carcinoma of the oesophagus to present as a polypoid intraluminal mass. A l t h o u g h a r a r e t u r n o u t , l i p o s a r c o m a is t h e most c o m m o n o f t h e s o f t t i s s u e s a r c b m a s , a n d o c c u r s mostly b e y o n d 30 y e a r s . V a r y i n g d e g r e e s o f d i f f e r e n t i a t i o n are seen. M y x o i d l e s i o n s w i t h l i p o b l a s t s e m b e d d e d in a g e l a t i n o u s m a t r i x a c c o u n t f o r a p p r o x i m a t e l y h a l f the c a s e s ( A n d e r s o n , 1985). T h e r e m a i n i n g t u m o u r s a r e well d i f f e r e n t i a t e d f a t t y l e s i o n s , a n d f i r m e r , r o u n d cell or pleomorphic lesions which are more aggressive. Neither t h e f a t t y n o r m y x o i d l e s i o n s m e t a s t a s i z e f r e q u e n t l y , but
RECURRENT LIPOSARCOMA OF THE OESOPHAGUS
357
Fig. 3 - CT at the level of D5 after ingestion of oral contrast medium. Soft tissue strands are seen within the fatty tumour. Note compression of the trachea.
(a)
Fig. 4 - CT at D2 showing the root of the pedicle (arrow).
(b) Fig. 2 - Barium swallow (i00 mm format) demonstrates recurrent tumour. (a) Upper end. (b) Lower end.
there is a well recognized tendency for repeated recurrence following excision, sometimes over a period of several years. The myxoid tumours occasionally recur in a less well differentiated round cell form, showing more aggressive histological and clinical features. Hadju (1979) studied a large series of 242 patients with liposarcoma at various sites, and found a 12% incidence of a histologically different but co-existent primary tumour.
Liposarcomas occur most commonly in the soft tissues of the thigh or in the retroperitoneum. Involvement of the mediastinum is also well recognized (Prohm et al., 1981). Lesions of the gastro-intestinal tract are extremely rare, and we know of only one other case of oesophageal liposarcoma (Mansour et al., 1983). Mansour's case was a 53-year-old man who developed a squamous bronchial carcinoma l0 months prior to the liposarcoma. Our case exhibits many typical features of this rare tumour. The lesion is slow growing and bulky, but being pedunculated it is also mobile. Our patient shares with the previously reported case the unusual symptom of intermittent regurgitation of the tumour into the oral cavity (Mansour et al., 1983). It is noteworthy that, in spite of
358
CLINICAL RADIOLOGY
severe compression of the tracheobronchial tree, our patient remained free from respiratory problems due to the liposarcoma. We believe that the rapid deterioration and death of our patient was due to a malignant primary bronchial neoplasm rather than recurrent liposarcoma. Although not pathologically proven, development of such a tumour would not be unusual in a patient with liposarcoma, and represents a further similarity with the previously reported case. There is considerable difficulty assessing the total extent of the lesion, both by radiological means (barium swallow and CT) and also at surgery. On two occasions tumour has been found at the margin of the resected specimen in the absence of any radiological or macroscopic evidence of spread of the tumour. Slow local recurrence is typical, and the clinical course in this patient emphasizes the value of long term follow up. Acknowledgements. We thank Mr C. J. Stoddard for allowing us to report on his patient, Dr R. A. Nakielny for interpreting the CT scan, and Mrs Y. Steel for typing the manuscript.
REFERENCES Anderson, JR (Ed.) (1985). Muir's Textbook of Pathology, 12th edition, 23.67. Edward Arnold, London. Hadju, SI (1979). Pathology of Soft Tissue Tumours. Lea & Febiger, Philadelphia. Harell, GS (1986). The oesophagus. In Diagnostic Radiology, eds, Grainger, RG & Allison, DJ, pp. 743-781. Churchill Livingstone, London. Laufer, I & Akiyoshi, Y (1979). Tumors of the esophagus. In Double Contrast Gastrointestinal Radiology, ed. Laufer, I, pp. 129-153. WB Saunders, Philadelphia. Mansour, KA, Fritz, RC~ Jacobs, DM & Vellios, F (1983). Peduncu. lated liposarcoma of the esophagus: a first case report. Journal of Thoracic and Cardiovascular Surgery, 86, 447 450. Prohm, P, Winter, J & Watowski, L (1981). Liposarcoma of the mediastinum. Case report and a review of the literature. Journalof Thoracic and Cardiovascular Surgery, 29, 119-121.
