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Tumor of the organ of Zuckerkandl
Tumor of the Organ of Zuckerkandl EMILE *[INTIENS, M.D., H:\RVF.V BERNARD, M.D. AND *'I'ALCOLM PE'rERSON, M.D., Sl. Louis, J[issouri
From the tI@ital Civil de Beni-Messous, Alger, Algeria, and The Departments of Medicine and Surgery, ll"ash-
ington University S(hool of Medicine, &)tint Louis, Missouri. HXS is a r e p o r t of the n i n e t e e n t h p a t i e n t to T suffer from a f u n c t i o n i n g t u m o r of the organ of Z u c k e r k a n d l and the sixth t r e a t e d successfully by surgical resection with relief of h y p e r tension. T h e r e p o r t is p r e s e n t e d because of the c o m p a r a t i v e r a r i t y of the t u m o r , t h e u n u s u a l historical and x-ray findings a n d t h e circumstances associated with the t r e a t m e n t . CASE REPORT
A. B., a twenty 3"ear old Algerian, was adnfitted in September 1962 to the H6pital Civil de BeniMessous near Algiers for bilateral puhnonary tubercuh)sis. The patient also complained of periodic epigastric pain that radiated into the right shoulder and the right lower quadrant of the abdomen. The onset of pain was followed by palpitations, profuse perspiration and headache. These episodes lasted for two to three days, occurred every two to three months and began two ),ears before. The patient was very nervous. There was a line tremor of the hands, tachycardia and prominent eves but no goiter. There was tenderness in the epigastrium and the right lower quadrant. The blood pressure on admission wag 80/60 ram. Hg. X-ray exmnination of the chest showed bilateral cavitary tuberculosis and tubercle bacilli were seen in the sputum. X-ray examination of the upper gastrointestinal tract showed a defornfity of the first portion of the duodenum. The patient was treated for duodenal ulcer. The abdominal pain and perspiration increased remarkably. There was no fever. The blood pressure was 200/170 ram. Hg. Thereafter the blood pressure measurements varied strikingly but usually were elevated. Many attacks of upper abdominal pain were noted over the next eight months, Studies of renal function were normal and intravenous pyelography showed no abnormality. An immediate and perslstant reduction of the blood pressure was noted upon the intravenous adVol. 109, February 1905
FIG. l. This roet3tgenogram shows displacement of the duodenum by the tumor.
ministration of ifllentolamine (Regitine®). Examination of the urine showed 1,480 rag. of eatecholamines/24 hours and 23 nag. 9VMA/24 hours. Presacral air studies were performed at the Mustapha Hospital in Algiers. Both adrenal glands were outlined and no tumor was demonstrable. The gastrointestinal x-ray examination was repeated. The third portion of the duodenum was displaced anteriorly and superiorly. (Fig. I.) The patient was operated upon on August 15, 1963. A tumor S cm. in diameter was found anterior to the aorta and inferior vena eava displacing the superior mesenteric vessels and the third and fourth portions of the duodenmn anteriorly. The blood vessels supplying the tumor were numerous and of small caliber. There were no other tumors
233
Minticns, Bernard and Petcrson demonstrable within the abdomen, The bhmd pressure remained stable during inductim~ of anesthesia and mobilization of the tumor. After excision of the tumor the blood i)ressure fell precipitously fronl 2aa/la0 ram. Hg and stabilized at 80/50 ram. I lg with the aid of a constant drip of L-norei)inephrine which was discontinued after twenty-two hours. At this time the blood pressure was 120/80 ram. Ilg and has remained so until the present (six months postoperatively). There has been no recurrence of the abdominal pain, sweats or emotional instability. X-ray examinations of the chest indicate that healing of the tuberculosis is taking place, The t)atient has gained 10 pounds and generally feels much improved. The microscopic sections were reviewed by the Department of Surgical Pathology at Washington University School of Medicine, A diagnosis of pheoehromoeytoma was made.
siblc in this patient. This indicates the imp o r t a n c e of careful examination of the gastrointestinal ~t r a c t especially in the diagnosis of extra-adrenal tumors. A n o t h e r interesting sidelight was t h a t the diagnosis of p h e o c h r o n l o c y t o u m was made b y a Belgian internist serving in Algeria as a p a r t of Belgium's p r o g r a m of serving the needs of developing c o u n t r i e s the p h e n t o l a m i n c was o b t a i n e d b y the C A R E - M e d i c o organization from G e r m a n y ; the examination of the urine for catecholamines was performed b y the Bio-Science L a b o r a t o r i e s in California (gratis); and the o p e r a t i o n was performed b y a t e a m of surgeons, physicians and a n e s t h e t i s t s from the United S t a t e s sponsored b y C A R E - M e d i c o and the United S t a t e s S t a t e D e p a r t m e n t .
COMMENTS
H u e b n e r and R e e d [1] recently reported the eighteenth d o c u m e n t e d t u m o r of the organ of Zuckerkandl and the fifth to be surgically extirpated. T h e y h a v e reviewed the historical, clinical and statistical aspects of secreting tumors of chromaffin tissue. T h e diagnosis and location of these t m n o r s is a l w a y s difficult. T h e history of a b d o m i n a l pains associated with the episodic a t t a c k s of s y m p a t h e t i c a c t i v i t y coupled w i t h the x-ray evidence of d i s p l a c e m e n t of the retroperitoneal d u o d e n u m m a d e p r e o p e r a t i v e localization pos-
2~4
SUMMARY
This is a report of the surgical excision of a t u m o r of the organ of Z u c k e r k a u d l whictl had p r o d u c e d s y m p t o m s of atxlominal pain simulating d u o d e n a l ulcer in association with hypertensive episodes. The t u m o r displaced the d u o d e n u m and was e v i d e n t on x-ray examination of the u p p e r g a s t r o i n t e s t i n a l tract. REFERENCE 1. I{UEItNER, G. l). ~Alld REED, P. A. Secreting t u m o r of c h r o m a f l i n tissue. A n n . 2,'urg., 158:216, 1963
A mo'ica. Journal of Surgery
From the tI@ital Civil de Beni-Messous, Alger, Algeria, and The Departments of Medicine and Surgery, ll"ash-
ington University S(hool of Medicine, &)tint Louis, Missouri. HXS is a r e p o r t of the n i n e t e e n t h p a t i e n t to T suffer from a f u n c t i o n i n g t u m o r of the organ of Z u c k e r k a n d l and the sixth t r e a t e d successfully by surgical resection with relief of h y p e r tension. T h e r e p o r t is p r e s e n t e d because of the c o m p a r a t i v e r a r i t y of the t u m o r , t h e u n u s u a l historical and x-ray findings a n d t h e circumstances associated with the t r e a t m e n t . CASE REPORT
A. B., a twenty 3"ear old Algerian, was adnfitted in September 1962 to the H6pital Civil de BeniMessous near Algiers for bilateral puhnonary tubercuh)sis. The patient also complained of periodic epigastric pain that radiated into the right shoulder and the right lower quadrant of the abdomen. The onset of pain was followed by palpitations, profuse perspiration and headache. These episodes lasted for two to three days, occurred every two to three months and began two ),ears before. The patient was very nervous. There was a line tremor of the hands, tachycardia and prominent eves but no goiter. There was tenderness in the epigastrium and the right lower quadrant. The blood pressure on admission wag 80/60 ram. Hg. X-ray exmnination of the chest showed bilateral cavitary tuberculosis and tubercle bacilli were seen in the sputum. X-ray examination of the upper gastrointestinal tract showed a defornfity of the first portion of the duodenum. The patient was treated for duodenal ulcer. The abdominal pain and perspiration increased remarkably. There was no fever. The blood pressure was 200/170 ram. Hg. Thereafter the blood pressure measurements varied strikingly but usually were elevated. Many attacks of upper abdominal pain were noted over the next eight months, Studies of renal function were normal and intravenous pyelography showed no abnormality. An immediate and perslstant reduction of the blood pressure was noted upon the intravenous adVol. 109, February 1905
FIG. l. This roet3tgenogram shows displacement of the duodenum by the tumor.
ministration of ifllentolamine (Regitine®). Examination of the urine showed 1,480 rag. of eatecholamines/24 hours and 23 nag. 9VMA/24 hours. Presacral air studies were performed at the Mustapha Hospital in Algiers. Both adrenal glands were outlined and no tumor was demonstrable. The gastrointestinal x-ray examination was repeated. The third portion of the duodenum was displaced anteriorly and superiorly. (Fig. I.) The patient was operated upon on August 15, 1963. A tumor S cm. in diameter was found anterior to the aorta and inferior vena eava displacing the superior mesenteric vessels and the third and fourth portions of the duodenmn anteriorly. The blood vessels supplying the tumor were numerous and of small caliber. There were no other tumors
233
Minticns, Bernard and Petcrson demonstrable within the abdomen, The bhmd pressure remained stable during inductim~ of anesthesia and mobilization of the tumor. After excision of the tumor the blood i)ressure fell precipitously fronl 2aa/la0 ram. Hg and stabilized at 80/50 ram. I lg with the aid of a constant drip of L-norei)inephrine which was discontinued after twenty-two hours. At this time the blood pressure was 120/80 ram. Ilg and has remained so until the present (six months postoperatively). There has been no recurrence of the abdominal pain, sweats or emotional instability. X-ray examinations of the chest indicate that healing of the tuberculosis is taking place, The t)atient has gained 10 pounds and generally feels much improved. The microscopic sections were reviewed by the Department of Surgical Pathology at Washington University School of Medicine, A diagnosis of pheoehromoeytoma was made.
siblc in this patient. This indicates the imp o r t a n c e of careful examination of the gastrointestinal ~t r a c t especially in the diagnosis of extra-adrenal tumors. A n o t h e r interesting sidelight was t h a t the diagnosis of p h e o c h r o n l o c y t o u m was made b y a Belgian internist serving in Algeria as a p a r t of Belgium's p r o g r a m of serving the needs of developing c o u n t r i e s the p h e n t o l a m i n c was o b t a i n e d b y the C A R E - M e d i c o organization from G e r m a n y ; the examination of the urine for catecholamines was performed b y the Bio-Science L a b o r a t o r i e s in California (gratis); and the o p e r a t i o n was performed b y a t e a m of surgeons, physicians and a n e s t h e t i s t s from the United S t a t e s sponsored b y C A R E - M e d i c o and the United S t a t e s S t a t e D e p a r t m e n t .
COMMENTS
H u e b n e r and R e e d [1] recently reported the eighteenth d o c u m e n t e d t u m o r of the organ of Zuckerkandl and the fifth to be surgically extirpated. T h e y h a v e reviewed the historical, clinical and statistical aspects of secreting tumors of chromaffin tissue. T h e diagnosis and location of these t m n o r s is a l w a y s difficult. T h e history of a b d o m i n a l pains associated with the episodic a t t a c k s of s y m p a t h e t i c a c t i v i t y coupled w i t h the x-ray evidence of d i s p l a c e m e n t of the retroperitoneal d u o d e n u m m a d e p r e o p e r a t i v e localization pos-
2~4
SUMMARY
This is a report of the surgical excision of a t u m o r of the organ of Z u c k e r k a u d l whictl had p r o d u c e d s y m p t o m s of atxlominal pain simulating d u o d e n a l ulcer in association with hypertensive episodes. The t u m o r displaced the d u o d e n u m and was e v i d e n t on x-ray examination of the u p p e r g a s t r o i n t e s t i n a l tract. REFERENCE 1. I{UEItNER, G. l). ~Alld REED, P. A. Secreting t u m o r of c h r o m a f l i n tissue. A n n . 2,'urg., 158:216, 1963
A mo'ica. Journal of Surgery