A Case of Pheochromocytoma with Severe Paralytic Ileus Masakuni NOGUCHI*'**,Takao TANIYA**,Keiichi UENO**, Masao YAGI**, Ryohei IztrMi**, Koji KONISHI** and Itsuo MIYAZAKI** ABSTRACT: We report herein a rare case o f a 26 year old woman with pheochromocytoma complicated by paralytic ileus. She presented with remarkable abdominal distension and respiratory difficulty but was effectively treated by surgical removal o f the tumor with preoperative and operative management using 0~ and fl adrenergic blocking agents. An excessive and persistent catecholamine production from large tumors or massive metastases characterizes this rare complication, and a review o f the English and Japanese literature is given following this report. KEY WORDS:

pheochromocytoma, paralytic ileus

INTRODUCTION D e c r e a s e d gastrointestinal motility and resultant constipation are u n c o m m o n symptoms of p h e o c h r o m o c y t o m a . 1 Moreover, paralytic ileus is a rare complication of the diseasC with a review of the English and Japanese literature revealing only 16 previously described casesY -17 We report herein a case we experienced o f pheochromocytoma with severe paralytic ileus.

CASEREPORT A 26 year old J a p a n e s e female was admitted to Kanazawa University Hospital in January, 1986, with severe abdominal distension, palpitation and hypertension. At the age o f 23 years, she had sometimes experienced nausea and headaches and at 24,

*The Operation Center, Kanazawa University Hospital **The Second Department of Surgery, School of Medicine, Kanazawa University Reprint requests to: Masakuni Noguchi, MD, The Operation Center, Kanazawa University Hospital, Takara-machi, 13-1, Kanazawa 920,Japan

following some episodes of palpitation was found to have high blood pressure. She also developed leg stiffness when walking with a cold sensation in her legs, as well as constipation and a loss o f appetite. At the end of 1985, she developed severe abdominal distension and respiratory distress for which she was admitted to a local hospital on January 1st 1986, at the age of 26 years. Abdominal plain X-ray showed remarkable intestinal distension (Fig. 1) and computed tomography showed a huge mass in the right upper abdomen and a distended gallbladder (Fig. 2). She was transferred to our hospital on January 9th. O n examination, she was 161 cm tall and weighed 45 kg. H e r blood pressure was 150/110 mmHg, pulse rate 110/min and body temperature 37.5~ Her abdomen was remarkably distended and abdominal sounds hypoactive, however, no abdominal mass could be felt. Erythema was f o u n d on h e r knees and feet. Laboratory data showed leucocytosis of 26100, an elevated CRP of 15.9 mg/dl, ferritin: 328 ng/dl, GOT: 82 IU/1, LDH: 824 IU/1, CPK: 1454 IU/I, FDP: 19.1 #g/ml, FBS: 191 mg/dl, urinary glucose: 22.8 g/dl and a decreased PO 2 in the arterial blood (59 mmHg). At this

JAPANESEJOURNALOFSURGERY,VOL.20, No. 4 pp. 448-452, 1990

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Pheochromocytoma with severe paralytic ileus

Fig. 1. Abdominal plain X-ray showing remarkable intestinal distension.

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a laparotomy was performed at which time the intestine was found to be remarkably distended, although no intestinal obstruction was seen. Intestinal fluid was aspirated through a tube into the appendical stump following an appendectomy. An abdominal mass was found but not considered to be resected because of her poor general condition, however, shortly after the operation, the abdominal distension developed again. Tachycardia and hypertension were still present and she moreover developed blindness due to retinal abrasion. By now her plasma norepinephrine concentration was found to be 213.1 ng/ml, epinephrine 13.8 ng/ml, urinary excretion of norepinephrine 46599.3 pg/day and epinephrine 7521.9 pg/day. Under the diagnosis of pheochromocytoma, phentolamine hydrochloride therapy was started, subsequently followed by propranolol hydrochloride and prazocin hydrochloride therapy, but her high blood pressure and tachycardia did not improve. Thus, on January 17th, the tumor mass was removed, immediately after which the blood pressure fe~llto 60/20 mmHg. and the small intestine activity became hyperactive. Two days after the operation, the abdominal distension, blindness and erythema also disappeared. The tumor was 12 X 16 X 12 cm in diameter and the microscopical appearance showed it to be made up of in'egularity

Fig. 2. Computed tomogTaphy showed a huge mass in the right upper abdomen and a distended gallbladder.

time, no data was yet available on the plasma and urinary catecholamines. Under the diagnosis of ileus, she was treated by a small intestinal drainage tube, however, the abdominal distension did not resolve and she subsequently developed abdominal pain and respiratory difficulty again. On January 10th,

Fig. 3. Microphotograph of a pheochromocytoma arising in the area of the right adrenal gland. (HE X200)

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s h a p e d large cells, in places syncytial, with large vesicular nuclei, most possessing a deeply vacuolated, eosinophilic cytoplasm. Mitoses were i n f r e q u e n t (Fig. 3). Since this histology was typical o f a b e n i g n p h e o c h r o m o c y t o m a a n d n o metastasis was found, it was t h e r e f o r e d i a g n o s e d as a b e n i g n p h e o c h r o m o c y t o m a . T h e patient was disc h a r g e d after an uneventful recovery a n d is n o w alive a n d well at the time o f this report.

DTSCUSS[ON A l t h o u g h c h r o n i c constipation has b e e n r e p o r t e d in up to 13 p e r c e n t o f patients with p h e o c h r o m o c y t o m a ) s o t h e r gastrointestinal manifestations are far less c o m m o n a n d have b e e n the subject o f a n u m b e r o f case reports. T h e most severe gastrointestinal manifestations o f p h e o c h r o m o c y t o m a include gastrointestinal bleeding, intestinal ischemia a n d necrosis, bowel p e r f o r a t i o n a n d peritonitis, a n d paralytic ileus. T h e s e are

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all extreme a n d fortunately rare complications o f the disease, but the h i g h morbidity a n d f r e q u e n t mortality make recognition and p r o m p t t r e a t m e n t o f the utmost importance) Most o f these complications are manifestations o f the excess c a t e c h o l a m i n e production in p h e o c h r o m o c y t o m a , a n d the varied p a t h o p h y s i o l o g y can be u n d e r s t o o d by the fact that a a n d fl c a t h e c h o l a m i n e receptors are p r e s e n t in varying concentrations in v a s c u l a r s m o o t h m u s c l e a n d intestinal s m o o t h muscle. I f sustained vascular smooth muscle contraction o r spasm occurs, mucosal ischemia with h e m o r r h a g e , ulceration and p e r f o r a t i o n m a y o c c u r TM a n d if the catec h o l a m i n e levels r e m a i n at a persistently elevated level, such motility abnormalities as resistant constipation or paralytic ileus, as n o t e d in o u r patient, m a y manifest. However, paralytic ileus is a rare complication o f the disease 2 with a review o f the English and J a p a n e s e literature revealing only 16 previously described cases 3-17 (Table 1). These

Table 1. Reported Cases of Pheochromocytoma with Ileus Tumor Dimensions (era)

Source

Age

Sex

Weight (gin)

1) Brown s 2) Hughes 4 3) Duffy5

37 44 30

F F M

22 26

F F

62 29 47 61 41 47 39 37 72 67 55 26

F M M F M M F M F F M F

Massive metastases 999 7XSX3 148 9X7X4.5 (rt) 69 7X5X4 (it) -. 9 13 1,449 (rt) 9 .. 440 (It) 50 . . . . . 999 12X10X3.8 88 6X5X4 71 Massive metastases Massive metastases Massive metastases Massive metastases 800 645 9.5X12.5X9 55 2X5X8 12X16X12

4) Huston 6 5) Bernstein 7 6) 7) 8) 9) 10) 11) 12) 13) 14) 15) 16) t7)

Fred ~ Gilliland ~ Cruz ~ Berkenboom t~ Baba n Okuno TM Ishizuka is Sugawara ~4 SumiyaTM Terashima TM Obara 1' Noguchi*

*: Present case **: Urinary vanilylmandelic acid, mg/gm creatinine.

.

Urinary Catechols (/tg/24 hr)

Outcome

4,500 1,800 3,240

Death Death Alive

..1,000"*

Death

.

Death . . . . . . 5,900 Alive Alive Death Death Death 3,330 Death 3,240 Alive 2,050 Death 34,700 Death 54,120 Alive

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Pheochromocytoma with severeparalytic ileus

findings suggest that large tumors or metastases which release massive amounts o f c a t h e c h o l a m i n e s characterize the subject with ileus and a p o o r prognosis is apparent. Pharmacological studies in dogs ~~ and rabbits ~ support this clinical observation. The infusion o f low doses o f levarterenol into dogs (0.8 # g / k g / m i n ) had no effect on the gastrointestinal tract, while a larger dose (2.6 ktg/kg/min) resulted in anorexia, nausea and ischemic lesions of the gastrointestinal tract. Similarly, studies o f rabbit intestines have shown that segmental intestinal distension occurs only with high concentrations of levarterenol. The pheochromocytomas are slow growing and unresponsive to radiation or chemotherapy 22with surgical resection of the tumor being the only effective treatment, so long as the tumor localizes within an area resectable by surgery. However, resection is frequently impossible in patients with ileus because of massive metastases, and surgical mortality has been high. The induction o f anesthesia is also potentially hazardous and special preparations are r e c o m m e n d e d for preoperative and operative m a n a g e m e n t using a and fl adrenergic blocking agents. In approximately 10 per cent o f all cases, the pheochromocytoma is considered malignant, 18 although the malignancy is difficult to establish by histology, and is usually only made when metastatic i n v o l v e m e n t o f l y m p h nodes, liver, lungs, or b o n e is noted. ~3 Moreover, histologically, the presence of capsular or v e n o u s i n v a s i o n by t u m o r cells, the absence o f a capsule, p l e o m o r p h i s m or hyperchromatism o f cells and bizzare or frequent mitotic figures can be seen in benign as well as malignant p h e o c h r o m o cytomas2~ Thus, histologic pattern does not accurately predict biologic behavior. In our department, there have been five cases o f pheochromocytoma, including the present case, one of whom was diagnosed as malignant because of liver metastases, and the others diagnosed as benign. We evaluated the tumor tissues by using a fluorescence

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cytophotometer, ~5 however, there was no definite difference f o u n d between malignant and benign p h e o c h r o m o c y t o m a with DNA analysis, indicating the need for all patients with pheochromocytoma to have detailed follow-up for at least 15 years, n,26,2~ (Received for publication o n J u n . 14, 1989)

REFERENCES 1. Mullen JP, Cartwright RC, Tisherman SE, Misage JR, Shapiro AP. Case report: pathologenesis and pharmacologic management of pseudo-obstruction of the bowel in pheochromocytoma.AmerJ Med Scie 1985;290: 155-158. 2. Cruz SR, ColwellJA. Pheochromocytomaand ileus. JAMA 1972;219: 1050-1051. 3. Brown R, BorowskyM. Further observations on the intestinal lesions of pheochromocytoma.Ann Surg 1960; 151:683-692. 4. Hughes LE, Bolt DE, Hobson JQ. Pheochromocytoma presenting as ileus. Proc Roy Soc Med 1962; 55: 998-999. 5. Duffy TJ, Erickson EE, Jordan GL, Bennett HD. Megacolon and bilateral pheochromocytomata. AmerJ Gastroent 1962;38: 555-574. 6. HustonJR, StewartWR.Hemorrhagicpheochromocytoma with shock and abdominal pain. AmerJ Med 1965; 39: 502-504. 7. Bernstein A, Wright AC. Pheochromocytomaas a cause of gastrointestinal distention. Postgrad Med J 1957;43: 180-183. 8. Fred HL, Allred DP, Garber HE, Retiene K, Lipscomb H. Pheochromocytomamasquerading as overwhelminginfection. Amer HeartJ 1967;73: 149-154. 9. Gilliland IC, Daniel O. Phaeochromocytornapresenting as an abdominal emergency. Brit Med J 1951; 2: 275-277. 10. Berkenboom G, Kraytman M, Frederic N. Pulmonary oedema after propranolol therapy and ileus in a patient with phaeochromocytoma. Acta Clinica Belgica 1979;34: 296-297. 11. Baba T, Machida K, Ozaki I, Okushima T, Murabayashi S, Kamata Y, Imamura K, Takebe K. A malignant pheochromocytomawith ileus, polyuria and hypercalcemia:A case of recurrence 17 years after the initial operation. EndocrinolJapon 1985; 32: 337-345. 12. Okuno T, Nishino M, Kageyama H, Nakamura R, Ohguro T, Saruta T, Mural K. A case of malignant pheochromocytoma.Horumon to Rvnshou (Clinical Endocrinology) 1981; 29: ~96-299. (in Japanese)

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13. Ishizuka Y, Miura Y, Adachi M, Seino H, Adachi M, Kimura S Yoshinaga K, Ohuchi H Iwai K, Sasano H, Kimura N, Morinaga S. A malignant pheochromocytoma observed for 14 years with extensive bone metastases. Igaku no Ayumi (J Clin Exp Med) 1983; 127: 213-226. (in Japanese) 14. Sugawara T, M!ura Y, Kimura S, Toriyabe S, Ishizuka Y, Noshiro T, Takahashi M, Ohashi H, Yoshinaga IL A case of malignant pheochromocytoma complicated with severe paralytic ileus: in special reference to the long-term treatment with parenteral alpha and beta adrenoreceptor blockers. Nippon Naibunpitu Gakkai Kaishi (J Jpn Soc Intern Med) 1986; 75: 951-956. (in Japanese) 15. Sumiya T, Harada K, Mishima S, Goto Y, Shin O, Saji Y. Management of huge phaeochromocytoma with severe ileus. Rynshou Masui (J Clinical Anesthesia) 1986; 10: 757-761. (in Japanese) 16. Terashima M, Aoki Y, Suzuki I, Itoh T, Takasawa H, Shirahata M, Kamura R. Paralytic ileus due to pheochromocytoma. Fukubu Kyukyu Shinryou no Shinpo (Progress in Acute Abdominal Medicine) 1987; 7: 911-914. (in Japanese) 17. Obara T, Ito Y, Akiba M, Yashiro T, Kanaji Y, Ito K, Kodama T, Fujimoto Y, Hirayama A. Malignant extraadrenal pheochromocytoma associated with marked hypertension and paralytic ileus. Naibunpitu Geka (Endocrine Surgery) 1988; 5: 101-106. (in Japanese) 18. Manger WM, Gifford RW. Pheochromocytoma. New

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York: Springer-Verlag 1979; 88-94. 19. Turner CE. Gastrointestinal pseudo-obstruction due to pheochromocytoma. Am J Gastroenterol 1983; 78: 214-217. 20, Szakacs J, Cannon A. Norepinephrine myocarditis. AmerJ Clin Path 1958; 30: 425-434. 2L Blackett RB, Picketing GW, Wilson GM. The effects of prolonged infusion of noradrenaline and adrenaline on the arterial pressure of the rabbit. Clin Sci 1950; 9: 247-251. 22. Scott HWJr, OatesJA, Nies AS, Bucho H, Page PL, Rhamey RIL Pheochromocytoma: Present diagnosis and management. Ann Surg 1976; 183: 537-593. 23. Priestly JT, Kvale WF, Gifford RW. Pheochrom0cytoma. Clinical aspects and surgical treatment. Arch Surg 1963; 86: 778-790. 24. Scott HW, Reynolds V, Green N, Page D, OatesJA, Robertson D, Roberts S. Clinical experience with malignant pheochromocytomas. SGO 1982; 154: 801-818~ 25. Aretxabala X, Yonemura Y, Sugiyama K, Kamata T, Konishi K, Miwa K, Miyazaki I. DNA ploidy in early gastric cancer and its relationship to prognosis. Br J Cancer 1988; 58: 81-84. 26. Mahoney EM, Harrison JH. Malignant pheochr0mocytoma (clinical course and treatment). J Ur01 1980; 118: 225-229. 27. Sparagana M. Late recurrence of benign phe0chromocytomas: the necessity for long term follow-up.J Surg Oncol 1988; 37: 140-146.

A case of pheochromocytoma with severe paralytic ileus.

We report herein a rare case of a 26 year old woman with pheochromocytoma complicated by paralytic ileus. She presented with remarkable abdominal dist...
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