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Case report: Infiltrative fibromatosis: A rare cause of fatal haemorrhage
ClinicalRadiology (1986) 37, 193-I94 © 1986 Royal College of Radiologists
0009-9260/86/598193502.00
Case Report: Infiltrative Fibromatosis: A Rare Cause of Fatal Haemorrhage M. A. ASHBY,
C. L. H A R M E R ,
J. A . M c K I N N A
a n d S. C. L E N N O X *
Joint Sarcoma Unit, Royal Marsden Hospital and *The Brompton Hospital, Fulham Road, London
A case of infiltrative fibromatosis in a 25-year-old man is described. This is a rare, recurring tumour most commonly seen in the shoulder and pelvic girdles, whose behaviour seems to justify the opinion that it is a nonmetastasing, low-grade fibrosarcoma.
The terms 'extra-abdominal desmoid tumour' and ' i n f i l t r a t i n g f i b r o m a t o s i s ' r e f e r to a n i n v a s i v e f i b r o b l a s tic p r o l i f e r a t i o n w h i c h has n e o p l a s t i c f e a t u r e s , i n c l u d i n g a t e n d e n c y to r e c u r a f t e r w h a t w a s r e g a r d e d as a c o m p l e t e e x c i s i o n ( M a c k e n z i e , 1972). D e s c r i b e d b y P a g e t in 1856 as a r e c u r r i n g a n t e r i o r a b d o m i n a l w a l l t u m o u r in p o s t - p a r t u m w o m e n , it is n o w r e c o g n i s e d at m a n y sites, t h e s h o u l d e r a n d p e l v i c g i r d l e s b e i n g t h e c o m m o n e s t . It is n o t e n c a p s u l a t e d , i n f i l t r a t e s a l o n g f a s c i a l p l a n e s , a n d m a y b e an i n t r a c t a b l e l o c a l p r o b l e m . S u c h b e h a v i o u r j u s t i f i e s t h e o p i n i o n h e l d b y s o m e p a t h o l o g i s t s t h a t it s h o u l d b e r e g a r d e d as a n o n - m e t a s t a s i s i n g , l o w - g r a d e fibrosarcoma.
CASE REPORT A 25-year-old male student presented in October 1981 complaining of coughing and difficulty in breathing. On examination he was found to have a right Horner's syndrome associated with a diffusely indurated mass in the right supraclavicular fossa. Chest radiography and computed tomography showed right-sided intrathoracic extension with deviation and compression of the trachea (Figs 1-3). Biopsy was undertaken and the material was described as moderately cellular but highly differentiated fibrous tissue featuring abundant collagen formation. There was no nuclear aberration and mitotic figures were scanty. Lymph nodes showed reactive changes only. The histological diagnosis was one of infiltrative fibromatosis. Full surgical exploration was then undertaken with thoracotomy. Much of the tumour was removed but the intrathoracic component could not be resected because of the infiltration in and around the major vessels in the superior mediastinum at the thoracic outlet on the right. Because of this he received a course of radiotherapy to the right side of the neck and the mediastinum: a dose of 60 Gy modal was delivered in 30 fractions over 71 days, a split course incorporating a 4-week gap. A volume measuring 19 cmx12.5 cm was treated with a three-field plan comprising two anterior and one posterior oblique fields. He tolerated this treatment well and the intrathoracic residual disease regressed. Repeat chest radiographs showed only some right apical pulmonary fibrosis from which he was asymptomatic. Two years later he returned with a history of increasing pain and swelling in the right arm and neck. A chest radiograph showed regrowth of the right apical and mediastinal disease (Fig. 4). Whilst an in-patient for assessment of this, he suddenly collapsed with chest pain and was found to be severely hypovolaemic as a result of a large right haemothorax. After resuscitation an arteriogram was performed which showed leakage from a branch of the subclavian artery. This was embolised under angiographic control. This manoeuvre stopped the bleeding temporarily but he succumbed after a further massive haemorrhage a few hours later. Post-mortem examination showed the right hemithorax to be filled with blood and the right lung was collapsed. A turnout
Fig. 1 Fig. 2 Fig. 1 - Close-up of penetrated chest radiograph at presentation showing the trachea displaced to the left by a right upper zone mass. Fig. 2 - Barium swallow at presentation, showing the oesophagus displaced to the left.
Fig. 3 - Computed tomogram of the chest at presentation showing infiltration around the mediastinal contents, particularly posterior to the trachea (L +46, W 256).
194
CLINCAL RADIOLOGY
DISCUSSION
The primary treatment for this rare turnout is surgical excision. However, this is often not possible and two small series from the USA suggest that regression of residual disease is observed after high doses of radiotherapy (Greenberg et al., 1981; Leibel et al., 1983; Keil et al., 1984). A few cases of recurrent disease have also been successfully treated with chemotherapy (Leibel et al., 1983). In our case, post-surgical residual disease was controlled for 2 years, until he developed an in-field recurrence. Only one other case of fatal haemorrhage due to large-vessel erosion has been reported previously (Leibel et al., 1983). REFERENCES
Fig. 4 - Chest radiograph showing right upper zone recurrence and fibrosis.
measuring 16 cmx 10 cmx8 cm occupied the right thoracic inlet and mediastinum. This was composed of rubbery white tissue, much of which was haemorrhagic in nature. The tumour was infiltrating widely into the superior mediastinum, right lobe of the thyroid and also into the axilla and lateral chest wall along a subcutaneous plane.
Greenberg, H. M., Goebel, R., Weichselbaum, R. R., Greenberger, J. S., Chaffey, J. T. & Cassady, J. R. (198l). Radiation therapy in the treatment of aggressive fibromatosis. International Journal of Radzation Oncology, Biology and Physics, 7, 305-330. Keil, K. D. & Suit, H. D. (1984). Radiation therapy in the treatment of aggressive fibromatosis (desmoid tumours). Cancer, 54, 20512055. Leibel, S. A., Wara, W. M., Hill, D. R., Bovill Jr, E. G., De Lorimer, A. A., Beckstead, J. H. et al. (1983). Desmoid tumours: local control and patterns of relapse following radiation therapy. International Journal of Radiation Oncology, Biology and Physics, 9, 1167-1171. Mackenzie, D. H. (1972). The Fibromatoses: a clinicopathological concept. British Medical Journal, iv, 277-281. Paget, J. (1856). Fibronucleated tumour of the abdomen of 14 years' growth: removal. Lancet, i, 625.
0009-9260/86/598193502.00
Case Report: Infiltrative Fibromatosis: A Rare Cause of Fatal Haemorrhage M. A. ASHBY,
C. L. H A R M E R ,
J. A . M c K I N N A
a n d S. C. L E N N O X *
Joint Sarcoma Unit, Royal Marsden Hospital and *The Brompton Hospital, Fulham Road, London
A case of infiltrative fibromatosis in a 25-year-old man is described. This is a rare, recurring tumour most commonly seen in the shoulder and pelvic girdles, whose behaviour seems to justify the opinion that it is a nonmetastasing, low-grade fibrosarcoma.
The terms 'extra-abdominal desmoid tumour' and ' i n f i l t r a t i n g f i b r o m a t o s i s ' r e f e r to a n i n v a s i v e f i b r o b l a s tic p r o l i f e r a t i o n w h i c h has n e o p l a s t i c f e a t u r e s , i n c l u d i n g a t e n d e n c y to r e c u r a f t e r w h a t w a s r e g a r d e d as a c o m p l e t e e x c i s i o n ( M a c k e n z i e , 1972). D e s c r i b e d b y P a g e t in 1856 as a r e c u r r i n g a n t e r i o r a b d o m i n a l w a l l t u m o u r in p o s t - p a r t u m w o m e n , it is n o w r e c o g n i s e d at m a n y sites, t h e s h o u l d e r a n d p e l v i c g i r d l e s b e i n g t h e c o m m o n e s t . It is n o t e n c a p s u l a t e d , i n f i l t r a t e s a l o n g f a s c i a l p l a n e s , a n d m a y b e an i n t r a c t a b l e l o c a l p r o b l e m . S u c h b e h a v i o u r j u s t i f i e s t h e o p i n i o n h e l d b y s o m e p a t h o l o g i s t s t h a t it s h o u l d b e r e g a r d e d as a n o n - m e t a s t a s i s i n g , l o w - g r a d e fibrosarcoma.
CASE REPORT A 25-year-old male student presented in October 1981 complaining of coughing and difficulty in breathing. On examination he was found to have a right Horner's syndrome associated with a diffusely indurated mass in the right supraclavicular fossa. Chest radiography and computed tomography showed right-sided intrathoracic extension with deviation and compression of the trachea (Figs 1-3). Biopsy was undertaken and the material was described as moderately cellular but highly differentiated fibrous tissue featuring abundant collagen formation. There was no nuclear aberration and mitotic figures were scanty. Lymph nodes showed reactive changes only. The histological diagnosis was one of infiltrative fibromatosis. Full surgical exploration was then undertaken with thoracotomy. Much of the tumour was removed but the intrathoracic component could not be resected because of the infiltration in and around the major vessels in the superior mediastinum at the thoracic outlet on the right. Because of this he received a course of radiotherapy to the right side of the neck and the mediastinum: a dose of 60 Gy modal was delivered in 30 fractions over 71 days, a split course incorporating a 4-week gap. A volume measuring 19 cmx12.5 cm was treated with a three-field plan comprising two anterior and one posterior oblique fields. He tolerated this treatment well and the intrathoracic residual disease regressed. Repeat chest radiographs showed only some right apical pulmonary fibrosis from which he was asymptomatic. Two years later he returned with a history of increasing pain and swelling in the right arm and neck. A chest radiograph showed regrowth of the right apical and mediastinal disease (Fig. 4). Whilst an in-patient for assessment of this, he suddenly collapsed with chest pain and was found to be severely hypovolaemic as a result of a large right haemothorax. After resuscitation an arteriogram was performed which showed leakage from a branch of the subclavian artery. This was embolised under angiographic control. This manoeuvre stopped the bleeding temporarily but he succumbed after a further massive haemorrhage a few hours later. Post-mortem examination showed the right hemithorax to be filled with blood and the right lung was collapsed. A turnout
Fig. 1 Fig. 2 Fig. 1 - Close-up of penetrated chest radiograph at presentation showing the trachea displaced to the left by a right upper zone mass. Fig. 2 - Barium swallow at presentation, showing the oesophagus displaced to the left.
Fig. 3 - Computed tomogram of the chest at presentation showing infiltration around the mediastinal contents, particularly posterior to the trachea (L +46, W 256).
194
CLINCAL RADIOLOGY
DISCUSSION
The primary treatment for this rare turnout is surgical excision. However, this is often not possible and two small series from the USA suggest that regression of residual disease is observed after high doses of radiotherapy (Greenberg et al., 1981; Leibel et al., 1983; Keil et al., 1984). A few cases of recurrent disease have also been successfully treated with chemotherapy (Leibel et al., 1983). In our case, post-surgical residual disease was controlled for 2 years, until he developed an in-field recurrence. Only one other case of fatal haemorrhage due to large-vessel erosion has been reported previously (Leibel et al., 1983). REFERENCES
Fig. 4 - Chest radiograph showing right upper zone recurrence and fibrosis.
measuring 16 cmx 10 cmx8 cm occupied the right thoracic inlet and mediastinum. This was composed of rubbery white tissue, much of which was haemorrhagic in nature. The tumour was infiltrating widely into the superior mediastinum, right lobe of the thyroid and also into the axilla and lateral chest wall along a subcutaneous plane.
Greenberg, H. M., Goebel, R., Weichselbaum, R. R., Greenberger, J. S., Chaffey, J. T. & Cassady, J. R. (198l). Radiation therapy in the treatment of aggressive fibromatosis. International Journal of Radzation Oncology, Biology and Physics, 7, 305-330. Keil, K. D. & Suit, H. D. (1984). Radiation therapy in the treatment of aggressive fibromatosis (desmoid tumours). Cancer, 54, 20512055. Leibel, S. A., Wara, W. M., Hill, D. R., Bovill Jr, E. G., De Lorimer, A. A., Beckstead, J. H. et al. (1983). Desmoid tumours: local control and patterns of relapse following radiation therapy. International Journal of Radiation Oncology, Biology and Physics, 9, 1167-1171. Mackenzie, D. H. (1972). The Fibromatoses: a clinicopathological concept. British Medical Journal, iv, 277-281. Paget, J. (1856). Fibronucleated tumour of the abdomen of 14 years' growth: removal. Lancet, i, 625.