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Case report: Papillary adenoma of the gall-bladder in a child of 9 years
Clinical Radiology(1993)47, 432-433
Case Report: Papillary Adenoma of the Gall-bladder in a Child of 9 Years S. M U L L I C K ,
R. G O T H I * a n d A. M U K E R J E E ' ~
Safdarjang Hospital, New Delhi, *South Delhi Ultrasound and X-Ray Clinic and Dr Diwan Chand Aggarwal Imaging Research Centre, New Delhi, and ~lnstitute of Pathology, Safdarjang Hospital, New Delhi, India Gall-bladder neoplasms are unusual in childhood and are often not suspected clinically. We wish to report this unusual case of papillary adenoma of the gall-bladder in a 9-year-old male child who had presented with clinical features of acute choleeystitis. Mullick, S., Gothi, R. & M u k e r j e e , A. (1993). Clinical Radiology 47, 4 3 2 - 4 3 3 . C a s e R e p o r t : P a p i l l a r y A d e n o m a o f the G a l l - b l a d d e r i n a C h i l d o f 9 Y e a r s
CASE REPORT
DISCUSSION
A 9-year-old male child presented with pain in the right hypochondrium and fever. On examination, there was a tender lump in the gallbladder region. A clinical diagnosis of acute cholecystitis was made and the patient referred for an ultrasound examination. On ultrasonography, the gall-bladder was distended. There was an echogenic soft tissue lesion attached to its anterior wall (Fig. 1), which showed localized thickening in this region. The lesion was nonshadowing and immobile: No other abnormality was seen in the gallbladder. In view of the patient's history, a sonographic diagnosis of acute acalculous cholecystitis with viscid sludge was made. The patient was managed conservatively and discharged on the 10th day, by which time the lump had regressed considerably. A follow-up ultrasound and oral cholecystogram (OCG) was performed after 6 weeks. The soft tissue lesion was still present and appeared unchanged in size on sonography. The oral cholecystogram showed non-opacification of the gall-bladder. Based on these observations, the patient underwent a cholecystectomy. The gall-bladder was distended, greenish blue in colour and measured 10-12 cm in length. On exposing its lumen, a yellowish soft tissue mass, about 10-12 mm in size, was found attached to the body close to the neck. The rest of the gall-bladder was unremarkable. A bistopathologieal examination of this lesion revealed it to be a papillary adenoma (Fig. 2). The patient has been followed up for the last 1 1/2 years and is now completely asymptomatic.
A n a d e n o m a o f the g a l l - b l a d d e r is a rare n e o p l a s m a n d is f o u n d in a b o u t 1.2% o f all c h o l e c y s t e c t o m i e s [1]. T h e a v e r a g e age o f the p a t i e n t s is 50.5 y e a r s _ 16.3 years a n d a m a l e to f e m a l e ratio, 1 : 3.5 [2]. T h e u n u s u a l f e a t u r e o f this case is the y o u n g age o f the p a t i e n t . T o the best o f o u r k n o w l e d g e , there has b e e n j u s t o n e p r e v i o u s r e p o r t o f a s i m i l a r case in a girl o f 8 years [3]. B e n i g n t u m o u r s o f the g a l l - b l a d d e r are f r e q u e n t l y d e s c r i b e d as p a p i l l o m a s . H o w e v e r , these t u m o u r s c a n be n o n - p a p i l l a r y o r p a p i l lary. T h u s , the t e r m a d e n o m a a p p e a r s m o r e a p p r o p r i a t e [2]. T h e s e r e p r e s e n t 2 8 % o f all b e n i g n g a l l - b l a d d e r tumours with nearly half of them (43%) having a p a p i l l a r y c o n f i g u r a t i o n [4]. T h e h i s t o p a t h o l o g i c a l a p p e a r a n c e o f the lesion i n o u r case was c o m p a t i b l e w i t h a p a p i l l a r y a d e n o m a . M o s t o f these a d e n o m a s d o n o t p r o d u c e s y m p t o m s a n d are detected i n c i d e n t a l l y o n c h o l e c y s t e c t o m y . H o w e v e r , there m a y b e signs a n d s y m p t o m s o f c h r o n i c g a l l - b l a d d e r disease s u c h as colic p a i n , p o s t - p r a n d i a l distress, v o m i t i n g , fever a n d occas i o n a l l y j a u n d i c e . O u r p a t i e n t h a d s y m p t o m s suggestive o f a c u t e cholecystitis. T h e s e features c o u l d b e a t t r i b u t e d to the l o c a t i o n o f the lesion, for a l t h o u g h it was a r i s i n g f r o m the b o d y o f the g a l l - b l a d d e r , it was p r o b a b l y
Fig. 1 - Ultrasonographic image of the gall-bladder demonstrating an echogenic nonshadowinglesion arising from an area of focal thickening. Correspondence to: Dr S. M ullick, D2/141. West Kidwai Nagar, New Delhi, India.
Fig. 2 - Photomicrograph (HAE x 200) showing details of the adenoma. Well-formed glandular structures are seen. The lesion does not show any evidence of malignancy.
PAPILLARY ADENOMA OF GALL-BLADDER
sufficiently close to the neck to cause obstruction and produce symptoms of cholecystitis. However, this might not have been the case. In these patients, symptoms can occur even in the absence of associated cholecystitis or cholelithiasis [5]. Ultrasonography is highly accurate in demonstrating these lesions. It also has some predictive value in the surgical management of these patients. In a recent study of 40 cholecystectomy specimens that were pre-operatively examined by sonography [6], 95% of the benign tumours were found to be less than 1 cm in size while 88% of malignant lesions were larger. A prior sonographic estimation of the size of these lesions could, therefore, help in deciding the appropriate approach to these patients. Cholecystectomy would be justified in lesions larger than 1 cm. Nevertheless in symptomatic patients, even smaller lesions will need removal [3], because o f the possible occurrence of carcinoma in situ in many cases of adenomas [7]. Sonographically, these lesions may mimic other conditions such as cholesterolosis, non-shadowing calculi, blood clots, parasitic infestation and aggregate material containing pus or sludge [8]. Highly viscous material may temporarily clump due to bile stasis and give a mass effect [9,10]. However, these 'pseudotumours' will disappear on reexamination [10]. Therefore, if a lesion persists on repeated examinations, the possibility of a neoplasm should be considered. In conclusion, we report a case of a young male symptomatic patient, who was clinically and sonographi-
433
cally suspected of having acute cholecystitis, but at surgery was found to have a papillary adenoma of the gall-bladder which is extremely unusual at this age. Acknowledgements. We are grateful to Dr P. C. Rai, Medical Superintendent, Safdarjang Hospital, New Delhi, for permitting us to go through the hospital records. Thanks are also due to Dr Sudershan Aggarwal of Dr Diwan Chand Aggarwal Imaging Research Centre for allowing us to use his magnificent radiology library. REFERENCES 1 Arbab AA, Brasfield R. Benign tumours of the gall-bladder. Surgery 1967;61:535 540. 2 Hulten J, Johansson H, Olding L. Adenomas of the gall bladder and extrahepatic bile ducts. Acta Chirurgica Scandinavica 1970; 136:203 207. 3 Mogilner JG, Dharan M, Siplovich L. Adenoma of the gall bladder in childhood. Journal of Pediatric Surgery 1991;26(2):223-224. 4 Carter SJ, Rutledge J, Hirsch JH, Vracko R, Chikos PM. Papillary adenoma of the gall bladder: ultrasonic demonstration. Journal of Clinical Ultrasound 1978;6(6):433-435. 5 Christensen AH, Ishak KG. Benign tumours and pseudotumours of the gall bladder. Archives of Pathology 1970;90:423432. 6 Koga A, Watanabe K, Fukuyama T. Diagnosis and operative indications for polypoid lesions of the gall bladder. Archives of Surgery 1988; 123:26-29. 7 Tobah E J, MacNeer G. Papilloma of the gall bladder. Surgery 1953;34:57-71. 8 Jeanty P, Ammann W, Cooperberg PL, Gooding GA, Kunstlinger F et al. Mobile intraluminal masses of the gallbladder. Journal of Ultrasound in Medicine 1983;2(2):65-71. 9 Anastasi B, Sutherland GR. Biliary sludge - ultrasonic appearance simulating neoplasm. British Journal of Radiology 1981;54:679. 10 Fakhry J. Sonography of tumefactive biliary sludge. American Journal of Roentgenology 1982; 139:717.
Case Report: Papillary Adenoma of the Gall-bladder in a Child of 9 Years S. M U L L I C K ,
R. G O T H I * a n d A. M U K E R J E E ' ~
Safdarjang Hospital, New Delhi, *South Delhi Ultrasound and X-Ray Clinic and Dr Diwan Chand Aggarwal Imaging Research Centre, New Delhi, and ~lnstitute of Pathology, Safdarjang Hospital, New Delhi, India Gall-bladder neoplasms are unusual in childhood and are often not suspected clinically. We wish to report this unusual case of papillary adenoma of the gall-bladder in a 9-year-old male child who had presented with clinical features of acute choleeystitis. Mullick, S., Gothi, R. & M u k e r j e e , A. (1993). Clinical Radiology 47, 4 3 2 - 4 3 3 . C a s e R e p o r t : P a p i l l a r y A d e n o m a o f the G a l l - b l a d d e r i n a C h i l d o f 9 Y e a r s
CASE REPORT
DISCUSSION
A 9-year-old male child presented with pain in the right hypochondrium and fever. On examination, there was a tender lump in the gallbladder region. A clinical diagnosis of acute cholecystitis was made and the patient referred for an ultrasound examination. On ultrasonography, the gall-bladder was distended. There was an echogenic soft tissue lesion attached to its anterior wall (Fig. 1), which showed localized thickening in this region. The lesion was nonshadowing and immobile: No other abnormality was seen in the gallbladder. In view of the patient's history, a sonographic diagnosis of acute acalculous cholecystitis with viscid sludge was made. The patient was managed conservatively and discharged on the 10th day, by which time the lump had regressed considerably. A follow-up ultrasound and oral cholecystogram (OCG) was performed after 6 weeks. The soft tissue lesion was still present and appeared unchanged in size on sonography. The oral cholecystogram showed non-opacification of the gall-bladder. Based on these observations, the patient underwent a cholecystectomy. The gall-bladder was distended, greenish blue in colour and measured 10-12 cm in length. On exposing its lumen, a yellowish soft tissue mass, about 10-12 mm in size, was found attached to the body close to the neck. The rest of the gall-bladder was unremarkable. A bistopathologieal examination of this lesion revealed it to be a papillary adenoma (Fig. 2). The patient has been followed up for the last 1 1/2 years and is now completely asymptomatic.
A n a d e n o m a o f the g a l l - b l a d d e r is a rare n e o p l a s m a n d is f o u n d in a b o u t 1.2% o f all c h o l e c y s t e c t o m i e s [1]. T h e a v e r a g e age o f the p a t i e n t s is 50.5 y e a r s _ 16.3 years a n d a m a l e to f e m a l e ratio, 1 : 3.5 [2]. T h e u n u s u a l f e a t u r e o f this case is the y o u n g age o f the p a t i e n t . T o the best o f o u r k n o w l e d g e , there has b e e n j u s t o n e p r e v i o u s r e p o r t o f a s i m i l a r case in a girl o f 8 years [3]. B e n i g n t u m o u r s o f the g a l l - b l a d d e r are f r e q u e n t l y d e s c r i b e d as p a p i l l o m a s . H o w e v e r , these t u m o u r s c a n be n o n - p a p i l l a r y o r p a p i l lary. T h u s , the t e r m a d e n o m a a p p e a r s m o r e a p p r o p r i a t e [2]. T h e s e r e p r e s e n t 2 8 % o f all b e n i g n g a l l - b l a d d e r tumours with nearly half of them (43%) having a p a p i l l a r y c o n f i g u r a t i o n [4]. T h e h i s t o p a t h o l o g i c a l a p p e a r a n c e o f the lesion i n o u r case was c o m p a t i b l e w i t h a p a p i l l a r y a d e n o m a . M o s t o f these a d e n o m a s d o n o t p r o d u c e s y m p t o m s a n d are detected i n c i d e n t a l l y o n c h o l e c y s t e c t o m y . H o w e v e r , there m a y b e signs a n d s y m p t o m s o f c h r o n i c g a l l - b l a d d e r disease s u c h as colic p a i n , p o s t - p r a n d i a l distress, v o m i t i n g , fever a n d occas i o n a l l y j a u n d i c e . O u r p a t i e n t h a d s y m p t o m s suggestive o f a c u t e cholecystitis. T h e s e features c o u l d b e a t t r i b u t e d to the l o c a t i o n o f the lesion, for a l t h o u g h it was a r i s i n g f r o m the b o d y o f the g a l l - b l a d d e r , it was p r o b a b l y
Fig. 1 - Ultrasonographic image of the gall-bladder demonstrating an echogenic nonshadowinglesion arising from an area of focal thickening. Correspondence to: Dr S. M ullick, D2/141. West Kidwai Nagar, New Delhi, India.
Fig. 2 - Photomicrograph (HAE x 200) showing details of the adenoma. Well-formed glandular structures are seen. The lesion does not show any evidence of malignancy.
PAPILLARY ADENOMA OF GALL-BLADDER
sufficiently close to the neck to cause obstruction and produce symptoms of cholecystitis. However, this might not have been the case. In these patients, symptoms can occur even in the absence of associated cholecystitis or cholelithiasis [5]. Ultrasonography is highly accurate in demonstrating these lesions. It also has some predictive value in the surgical management of these patients. In a recent study of 40 cholecystectomy specimens that were pre-operatively examined by sonography [6], 95% of the benign tumours were found to be less than 1 cm in size while 88% of malignant lesions were larger. A prior sonographic estimation of the size of these lesions could, therefore, help in deciding the appropriate approach to these patients. Cholecystectomy would be justified in lesions larger than 1 cm. Nevertheless in symptomatic patients, even smaller lesions will need removal [3], because o f the possible occurrence of carcinoma in situ in many cases of adenomas [7]. Sonographically, these lesions may mimic other conditions such as cholesterolosis, non-shadowing calculi, blood clots, parasitic infestation and aggregate material containing pus or sludge [8]. Highly viscous material may temporarily clump due to bile stasis and give a mass effect [9,10]. However, these 'pseudotumours' will disappear on reexamination [10]. Therefore, if a lesion persists on repeated examinations, the possibility of a neoplasm should be considered. In conclusion, we report a case of a young male symptomatic patient, who was clinically and sonographi-
433
cally suspected of having acute cholecystitis, but at surgery was found to have a papillary adenoma of the gall-bladder which is extremely unusual at this age. Acknowledgements. We are grateful to Dr P. C. Rai, Medical Superintendent, Safdarjang Hospital, New Delhi, for permitting us to go through the hospital records. Thanks are also due to Dr Sudershan Aggarwal of Dr Diwan Chand Aggarwal Imaging Research Centre for allowing us to use his magnificent radiology library. REFERENCES 1 Arbab AA, Brasfield R. Benign tumours of the gall-bladder. Surgery 1967;61:535 540. 2 Hulten J, Johansson H, Olding L. Adenomas of the gall bladder and extrahepatic bile ducts. Acta Chirurgica Scandinavica 1970; 136:203 207. 3 Mogilner JG, Dharan M, Siplovich L. Adenoma of the gall bladder in childhood. Journal of Pediatric Surgery 1991;26(2):223-224. 4 Carter SJ, Rutledge J, Hirsch JH, Vracko R, Chikos PM. Papillary adenoma of the gall bladder: ultrasonic demonstration. Journal of Clinical Ultrasound 1978;6(6):433-435. 5 Christensen AH, Ishak KG. Benign tumours and pseudotumours of the gall bladder. Archives of Pathology 1970;90:423432. 6 Koga A, Watanabe K, Fukuyama T. Diagnosis and operative indications for polypoid lesions of the gall bladder. Archives of Surgery 1988; 123:26-29. 7 Tobah E J, MacNeer G. Papilloma of the gall bladder. Surgery 1953;34:57-71. 8 Jeanty P, Ammann W, Cooperberg PL, Gooding GA, Kunstlinger F et al. Mobile intraluminal masses of the gallbladder. Journal of Ultrasound in Medicine 1983;2(2):65-71. 9 Anastasi B, Sutherland GR. Biliary sludge - ultrasonic appearance simulating neoplasm. British Journal of Radiology 1981;54:679. 10 Fakhry J. Sonography of tumefactive biliary sludge. American Journal of Roentgenology 1982; 139:717.