Lymphangioleiomyomatosis Uiagnoslic imagingapproach lo a rare disease in women Ry Jylle H e d e 1 nnd Anne Crethe Jurik'
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Key words . .. Lyrnphangioleiomyomatosis imaging
Schlüsselwörter
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Diagnostic
P -
Case Reporl
I
Lymphangioleiomyornatose - Bildgeben-
/
de Diagnostik
I
lnlroduclion In adults chylothorax mostly iollows chest traiirna ur is associated with malignnnt tumours. Chylous effusions rnay. however. also be due to the rare. benigii disease lyniphansoIeioiriyoniatosis (LAM). It has been reported in less thari 100 cases and is exclusively Seen in wornen, mainly of childbearing age. Al-
Fig. 1
lhough thc lcsion is benign and does not rnetastasizc,. the prognosis is often very poor. We hereby presenta typical case i r i wliicli tlie pntient bad both pulmonaiy and rctropcritoncal LAM.
A 34-yrnr-old wornen was iidmitted to thc Municipal Hospital. March 1986. due to fatiaie. cougli aiid iiicreasirig brrathlcssness during 3 rnonths Plain chest radiograph showed left-sided pleural effusion. Pleuraceiitesis revealod chylc without rnalignant cells aiid bacterial cultures were negative. CT of t h s rnediastinum and othcr investigations for a rnalignant tumour did iiot explain tlie chylothorax. lnitinlly the dnily loss of chyle through a pleural d r a i n a g ~was about orie litte. She was t,reated with parantcral nutrition and recovercd when the pleural effusion ceased orie inontli Inter. The patient was quite wrll I'or thc subscqucnt two months. after which she gradiially siifTered from iiicreasing dyspnoca. pain in thc right side of her chest, a swelling on the neck. and iricreasiiig abdoniiiial distension. Plain chest X-ray showcd bilateral pleural effiisioris arid basal bilateral iriterstitial pulrnonary fibrocis (I:ig. 1). Cl' examination of the thorax a n d abdomen revealed bilateral pleural and peritorieal fluid accuniulations. pul-
Posteroanterior(a1and lateral (b)chest radiographsshowing basal reticularabnormalitiesand bilateral pleuraleffusion, mainly on the right side. -
Fortschr.Röntgcnstr. 155.6(1991)582 584 C) Ceorg ThieiiieVerlng Stuttgart - NAWYork
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Dieses Dokument wurde zum persönlichen Gebrauch heruntergeladen. Vervielfältigung nur mit Zustimmung des Verlages.
I Dcpai~mciitol'1)iagnostic Hadiology. Coiiril I lospilal c i f Randers and .' I)epartmeiif of I)iagiioslic Rndiologv R. Muriicipal liospital. I!niversity of Aarhiis. Iknmark
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Fig. 2 CT-scanof the thorax demonstrating bilateral fibrosis and numerous cystic air spaces of vatying sizes. In addition,there are pleural effusions.
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Fortschr Honlgcnslr 755.6 583 P -
Fig. 3 CT-scanof the abdomen showinga poorly demarcated retroperitoneal space due to a tumour rnass surrounding the aorta and the inferior caval vein.
Fig. 4 Posteroanterior (a)and lateral (b)chest radiographs,three years after the onset of symptoms, revealing increased retrosternal air space and flatteningof the diaphragm. lrregularopacities are present mainly in the basal parts of both lungsas well as cystic air spaces resemblinghoneycornbing
moiiary cystic air spaces and librosis. and a retroperitoneal tumour mass (Fig. 2 and 3). 'The Iindings were confirmed by ultrasonography, wliich also showed bilateral subclavian vein thrombosis. An exploratory laparotomy was perhrmed and a poorly demarcated para-aortic tumour was found. Complete removal was not possible. Microscopic diagnosis: I.AM. Postoperatively there were increasing pleural and peritoneal chylous effusions. A chylovenous shunt was installed. but had to be removed because ofclotting. She was then treated with medroxyprogesterone and bilateral oophorectomy. without any obvious effect. Since then it has been necessnry to perforrn pleural and peritoneal drainage of about 7 litres of chyle each week, and her pulrnonary fibrosis lias gradually increased (Fig. 4). At present. 3 yenrs after surgery. the patient is severely underweight, suffering frorn chronic fatigue. aiid is unable to work physically.
Discussion ..
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LAM is a rare disease ofwornen rnainly in the reproductive years of life. The etiology is unknown, and there is no familial aggregation. but an association o l pulmonary LAM with tuberous sclerosis has been noticed (2.6.13. 14). LAM ischaracterised by a hamartomatous proliferation of aiypical srnooth inuscle cells along the lymphatics. This hyperplasia may occur within lung parpnchyma and/or extrapulmonary lymphatic structures of the thorax, and in the abdornen. When the lungs are involved the prognosis is very poor; rnost patients die within 1 to 10 years after the onset of Symptoms (4, 6). Complications such a s pneumothorax, chylous effiisions. haemoptysis. and phlebothrornbosis are attribiitable to bronchiolnr. Iymphatic. and venous obstructions caused by proliferating muscleielis (6). In the lungs. this causes srnall airway obstruction leading to poor diffusion and air-trapping with progressive emphysema
Dieses Dokument wurde zum persönlichen Gebrauch heruntergeladen. Vervielfältigung nur mit Zustimmung des Verlages.
1.i/nipt1cl1rgioleir~ni!lornrrtosis
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Jytte Hede andAnne Grethe Jurik: I.ymphangiobiom~jomatosis P
~
Hadiographic pulnionary abnormalities ofteii develop relatively late in the course of the djsease 14). Characteristic are reticular markings. suggesting honeycornbing, but with a more delicate appearance than Seen in end-stage interstitial fibrosis. Concomitant progressive enlargement of the lungs may occur. The gradual descent of the diaphragm and elongation of the rncdiastinum may be overlooked without scrial films (4. 6). This combination of a diffuse infiltrative Pattern and cystlike spaces together with an increased lung volume is very unusual. and when Seen in womcn the diagnosis of LAM should always be considered. Recently, the CT appearance of pulmonary LAM has been described (10-12). The main findings are nurnerous thin-walled cystic air spaces of various sizes often with normal lung parenchynia between the cysts. These air spaces vary in size with the extent oi disease. and in patients with more than 80% pulmonary involvement the predominant cysts tend to be larger than one cm in dianieter (1 1). Furthermore, CT has shown that all parts of the lungs are equally affected. In the present case, plain radiographs and C'1' of the lungs showed rather extensive abnormalities relatively early i i i ths coursc of the disease. Llltrasonography and CI' of the abdomen was fouiid to be useful in the diagnosis of retroperitoneal LAM .
Diverse modes of treatment Iiave been rccommcnded. Hormonal therapy with a progestational agent may lead to objective regression of LAhl (7). and bilateral oophorectorny sometimes inhibits progression of the disease (1.8). The basis for thesc treatments are that LAM tends to show a milder course in postmenopausal wornen (1, 2, 5). and that exacerbation o i the disease may be incited by oestrogen sti~nulatione. g. during pregnancv and use oforal contraceptives (3.8.9). Furtherrnore, pulmonary LAhl-tissue contains oestrogeii and progesterone receptors. whereas normal lung tissuc does not (3). Dietary I'at rcstriction and nicdium chairi triglyceride f~cdingsmay reduce the amount of chysucccssful therapy lous ~ffusions(8).'l'he only coriiplrtely . - has been total excisiori of a localised lesion.
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References P
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Banner, A. S . . C. B. Currington. W. B. Bnzory, 1.'. Kittle, G. Leonard, J. Hingus. P. Iaylor, W. W. Addinglon: EfTicacy of oophorectorny in lymphangiolciomyomatosis and benigri inetastasizingleiornyoma. N . Engl. .I. Med. 305 (1981) 204-209 " Bhattacharyya, A. K., K. Balogh: Retroperitoneal lymphangioleiornyomatosis. A 36-year benign course in a postmenopausal women. Cancer 56 (1985) 1144-1 146 Brentani. M. M., C. R. R. Caruafho,P. H. Saldiua, M. M . Pacheco, C. T. F. Oshima: Steroid receptors iii pulmonary lymphangiornyomatosis. Chest 85 (1984) 96-99 Carrington. C. B..D. W. Cugell. E. A. (;acnsler, A. Marks. R. A. Redding, J. T. Schau5 A. Tomasian: L.ymphangioleiomyomatosis. Am. Rev. Respir. Dis. 116 (1977) 977-995 Cornog, J. L., H. T. Enterlir~et Lyrnphangiomyomit. a benign lesion of chyliferous lymphatics synonymous with lymphangiopericytoma. Cancer 19 (1966) 1909-1929 Corrin. B., A. A. Liebow. P. J. Friedman: I'ulmonary Lympliarigiomyomatosis. Am. J. Pathol. 79 (1975) 347-382 ßishner. W., B. M. Cordasco, J. Blackburn. S. ßemeter. 11. Lcuirz, W. D. Carey: Pulnionary Lyrnphangiomyomatosis. Chest 85 (1984) 796-799 Kitzsteiner, K. A., R. G. Mallen: Pulrnonary 1,yrnphangiornyomatosis: Treatnient with Castration. Cancer 4 6 (1980) 2248-2249 Lack, E. E.. M. F. Dolan. J. Finisio, G. Grouer. M. Singh, T. J. Triche: Pulrnonary and extrapulrnonary lymphangioleiomyomatosis. Am. J . Surg. Pathol. 10 (9) (1986) 650-657 'Qenoir. S.. P. Grenier, M. W. Brauner, J. Frija, M. Remy-Jardin, D. Reuel, J.-F. Cordier: Pulmonary lymphangiomyornatosis arid tuberous sclerosis: Comparison of radiographic and thin-section CT findings. Radiology 175 (1990) 329-334 I ] Müller, IV. L.. C. Chiles, P. Kullnig: Pulrnonary lyrnphangiomyornatosis: Correlation of CT with radiographic and functional findings. Radiology 175 (1990) 335-339 Sherrier, R. H.. C. Chiles, V. Roggli: Pulmonary lymphangioleiornyomatosis: CT findings. AIR 153 (1989) 937-940 l 3 Teglhjcerg, P. S., V. Suendsen: Lyrnphangiomyoma of the thoracic duct with previously undcscribed epithelial tubules. Tumori 6 4 (1978) 63-70 l4 W o l g M.: Lyrnphangiomyoma: clinicopathologic study and ultrastruclural conlirrnation of its histiogenesis. Cancer 31 (1973) 988-1007
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Jytte Hede Departrnent of diagnostic radiology Municipal Hospital llniversity ofAarhus DK-8000 AarhusC Deninark
Dieses Dokument wurde zum persönlichen Gebrauch heruntergeladen. Vervielfältigung nur mit Zustimmung des Verlages.
584 Fortschr. Röntgenstr. 155.6 -
I
--
Key words . .. Lyrnphangioleiomyomatosis imaging
Schlüsselwörter
-
-
Diagnostic
P -
Case Reporl
I
Lymphangioleiomyornatose - Bildgeben-
/
de Diagnostik
I
lnlroduclion In adults chylothorax mostly iollows chest traiirna ur is associated with malignnnt tumours. Chylous effusions rnay. however. also be due to the rare. benigii disease lyniphansoIeioiriyoniatosis (LAM). It has been reported in less thari 100 cases and is exclusively Seen in wornen, mainly of childbearing age. Al-
Fig. 1
lhough thc lcsion is benign and does not rnetastasizc,. the prognosis is often very poor. We hereby presenta typical case i r i wliicli tlie pntient bad both pulmonaiy and rctropcritoncal LAM.
A 34-yrnr-old wornen was iidmitted to thc Municipal Hospital. March 1986. due to fatiaie. cougli aiid iiicreasirig brrathlcssness during 3 rnonths Plain chest radiograph showed left-sided pleural effusion. Pleuraceiitesis revealod chylc without rnalignant cells aiid bacterial cultures were negative. CT of t h s rnediastinum and othcr investigations for a rnalignant tumour did iiot explain tlie chylothorax. lnitinlly the dnily loss of chyle through a pleural d r a i n a g ~was about orie litte. She was t,reated with parantcral nutrition and recovercd when the pleural effusion ceased orie inontli Inter. The patient was quite wrll I'or thc subscqucnt two months. after which she gradiially siifTered from iiicreasing dyspnoca. pain in thc right side of her chest, a swelling on the neck. and iricreasiiig abdoniiiial distension. Plain chest X-ray showcd bilateral pleural effiisioris arid basal bilateral iriterstitial pulrnonary fibrocis (I:ig. 1). Cl' examination of the thorax a n d abdomen revealed bilateral pleural and peritorieal fluid accuniulations. pul-
Posteroanterior(a1and lateral (b)chest radiographsshowing basal reticularabnormalitiesand bilateral pleuraleffusion, mainly on the right side. -
Fortschr.Röntgcnstr. 155.6(1991)582 584 C) Ceorg ThieiiieVerlng Stuttgart - NAWYork
--
~
Dieses Dokument wurde zum persönlichen Gebrauch heruntergeladen. Vervielfältigung nur mit Zustimmung des Verlages.
I Dcpai~mciitol'1)iagnostic Hadiology. Coiiril I lospilal c i f Randers and .' I)epartmeiif of I)iagiioslic Rndiologv R. Muriicipal liospital. I!niversity of Aarhiis. Iknmark
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-- --
Fig. 2 CT-scanof the thorax demonstrating bilateral fibrosis and numerous cystic air spaces of vatying sizes. In addition,there are pleural effusions.
.-P
-
Fortschr Honlgcnslr 755.6 583 P -
Fig. 3 CT-scanof the abdomen showinga poorly demarcated retroperitoneal space due to a tumour rnass surrounding the aorta and the inferior caval vein.
Fig. 4 Posteroanterior (a)and lateral (b)chest radiographs,three years after the onset of symptoms, revealing increased retrosternal air space and flatteningof the diaphragm. lrregularopacities are present mainly in the basal parts of both lungsas well as cystic air spaces resemblinghoneycornbing
moiiary cystic air spaces and librosis. and a retroperitoneal tumour mass (Fig. 2 and 3). 'The Iindings were confirmed by ultrasonography, wliich also showed bilateral subclavian vein thrombosis. An exploratory laparotomy was perhrmed and a poorly demarcated para-aortic tumour was found. Complete removal was not possible. Microscopic diagnosis: I.AM. Postoperatively there were increasing pleural and peritoneal chylous effusions. A chylovenous shunt was installed. but had to be removed because ofclotting. She was then treated with medroxyprogesterone and bilateral oophorectomy. without any obvious effect. Since then it has been necessnry to perforrn pleural and peritoneal drainage of about 7 litres of chyle each week, and her pulrnonary fibrosis lias gradually increased (Fig. 4). At present. 3 yenrs after surgery. the patient is severely underweight, suffering frorn chronic fatigue. aiid is unable to work physically.
Discussion ..
.
-
LAM is a rare disease ofwornen rnainly in the reproductive years of life. The etiology is unknown, and there is no familial aggregation. but an association o l pulmonary LAM with tuberous sclerosis has been noticed (2.6.13. 14). LAM ischaracterised by a hamartomatous proliferation of aiypical srnooth inuscle cells along the lymphatics. This hyperplasia may occur within lung parpnchyma and/or extrapulmonary lymphatic structures of the thorax, and in the abdornen. When the lungs are involved the prognosis is very poor; rnost patients die within 1 to 10 years after the onset of Symptoms (4, 6). Complications such a s pneumothorax, chylous effiisions. haemoptysis. and phlebothrornbosis are attribiitable to bronchiolnr. Iymphatic. and venous obstructions caused by proliferating muscleielis (6). In the lungs. this causes srnall airway obstruction leading to poor diffusion and air-trapping with progressive emphysema
Dieses Dokument wurde zum persönlichen Gebrauch heruntergeladen. Vervielfältigung nur mit Zustimmung des Verlages.
1.i/nipt1cl1rgioleir~ni!lornrrtosis
--
Jytte Hede andAnne Grethe Jurik: I.ymphangiobiom~jomatosis P
~
Hadiographic pulnionary abnormalities ofteii develop relatively late in the course of the djsease 14). Characteristic are reticular markings. suggesting honeycornbing, but with a more delicate appearance than Seen in end-stage interstitial fibrosis. Concomitant progressive enlargement of the lungs may occur. The gradual descent of the diaphragm and elongation of the rncdiastinum may be overlooked without scrial films (4. 6). This combination of a diffuse infiltrative Pattern and cystlike spaces together with an increased lung volume is very unusual. and when Seen in womcn the diagnosis of LAM should always be considered. Recently, the CT appearance of pulmonary LAM has been described (10-12). The main findings are nurnerous thin-walled cystic air spaces of various sizes often with normal lung parenchynia between the cysts. These air spaces vary in size with the extent oi disease. and in patients with more than 80% pulmonary involvement the predominant cysts tend to be larger than one cm in dianieter (1 1). Furthermore, CT has shown that all parts of the lungs are equally affected. In the present case, plain radiographs and C'1' of the lungs showed rather extensive abnormalities relatively early i i i ths coursc of the disease. Llltrasonography and CI' of the abdomen was fouiid to be useful in the diagnosis of retroperitoneal LAM .
Diverse modes of treatment Iiave been rccommcnded. Hormonal therapy with a progestational agent may lead to objective regression of LAhl (7). and bilateral oophorectorny sometimes inhibits progression of the disease (1.8). The basis for thesc treatments are that LAM tends to show a milder course in postmenopausal wornen (1, 2, 5). and that exacerbation o i the disease may be incited by oestrogen sti~nulatione. g. during pregnancv and use oforal contraceptives (3.8.9). Furtherrnore, pulmonary LAhl-tissue contains oestrogeii and progesterone receptors. whereas normal lung tissuc does not (3). Dietary I'at rcstriction and nicdium chairi triglyceride f~cdingsmay reduce the amount of chysucccssful therapy lous ~ffusions(8).'l'he only coriiplrtely . - has been total excisiori of a localised lesion.
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.-
p
References P
'
Banner, A. S . . C. B. Currington. W. B. Bnzory, 1.'. Kittle, G. Leonard, J. Hingus. P. Iaylor, W. W. Addinglon: EfTicacy of oophorectorny in lymphangiolciomyomatosis and benigri inetastasizingleiornyoma. N . Engl. .I. Med. 305 (1981) 204-209 " Bhattacharyya, A. K., K. Balogh: Retroperitoneal lymphangioleiornyomatosis. A 36-year benign course in a postmenopausal women. Cancer 56 (1985) 1144-1 146 Brentani. M. M., C. R. R. Caruafho,P. H. Saldiua, M. M . Pacheco, C. T. F. Oshima: Steroid receptors iii pulmonary lymphangiornyomatosis. Chest 85 (1984) 96-99 Carrington. C. B..D. W. Cugell. E. A. (;acnsler, A. Marks. R. A. Redding, J. T. Schau5 A. Tomasian: L.ymphangioleiomyomatosis. Am. Rev. Respir. Dis. 116 (1977) 977-995 Cornog, J. L., H. T. Enterlir~et Lyrnphangiomyomit. a benign lesion of chyliferous lymphatics synonymous with lymphangiopericytoma. Cancer 19 (1966) 1909-1929 Corrin. B., A. A. Liebow. P. J. Friedman: I'ulmonary Lympliarigiomyomatosis. Am. J. Pathol. 79 (1975) 347-382 ßishner. W., B. M. Cordasco, J. Blackburn. S. ßemeter. 11. Lcuirz, W. D. Carey: Pulnionary Lyrnphangiomyomatosis. Chest 85 (1984) 796-799 Kitzsteiner, K. A., R. G. Mallen: Pulrnonary 1,yrnphangiornyomatosis: Treatnient with Castration. Cancer 4 6 (1980) 2248-2249 Lack, E. E.. M. F. Dolan. J. Finisio, G. Grouer. M. Singh, T. J. Triche: Pulrnonary and extrapulrnonary lymphangioleiomyomatosis. Am. J . Surg. Pathol. 10 (9) (1986) 650-657 'Qenoir. S.. P. Grenier, M. W. Brauner, J. Frija, M. Remy-Jardin, D. Reuel, J.-F. Cordier: Pulmonary lymphangiomyornatosis arid tuberous sclerosis: Comparison of radiographic and thin-section CT findings. Radiology 175 (1990) 329-334 I ] Müller, IV. L.. C. Chiles, P. Kullnig: Pulrnonary lyrnphangiomyornatosis: Correlation of CT with radiographic and functional findings. Radiology 175 (1990) 335-339 Sherrier, R. H.. C. Chiles, V. Roggli: Pulmonary lymphangioleiornyomatosis: CT findings. AIR 153 (1989) 937-940 l 3 Teglhjcerg, P. S., V. Suendsen: Lyrnphangiomyoma of the thoracic duct with previously undcscribed epithelial tubules. Tumori 6 4 (1978) 63-70 l4 W o l g M.: Lyrnphangiomyoma: clinicopathologic study and ultrastruclural conlirrnation of its histiogenesis. Cancer 31 (1973) 988-1007
"
'
Jytte Hede Departrnent of diagnostic radiology Municipal Hospital llniversity ofAarhus DK-8000 AarhusC Deninark
Dieses Dokument wurde zum persönlichen Gebrauch heruntergeladen. Vervielfältigung nur mit Zustimmung des Verlages.
584 Fortschr. Röntgenstr. 155.6 -
