Cancer of Bile Duct With Situs Inversus Brian C.

Organ, MD;

Lee J.

Skandalakis, MD; Stephen

present a case of adenocarcinoma of the distal combile duct in a 68-year-old woman with total situs inver-

\s=b\ We

mon sus.

Endoscopic retrograde cholangiopancreatography prior

to surgery demonstrated segmental obstruction of the distal

common bile duct. Obstructive jaundice was observed and the results of liver function studies were abnormal. All biopsy specimens obtained in the operating room were negative. The case met all the criteria of Child and Frey, but at the end of the procedure the tumor was found to involve the superior mesenteric vein, a factor not appreciated during the operative evaluation of the patient. The situs inversus did not produce any technical problems. The patient did not have any surgical complications, but she died 18 months later of the cancer.

(Arch Surg. 1991;126:1150-1153) of total inversus present with adenocarcinoma of the distal We duct. unrelated in both a case

situs

in

a

patient

common

bile

Although origin, pathologic enti¬ ties are extremely unusual and challenging to the surgeon.

Situs inversus, in and of itself, is a rare condition; accord¬ ing to Varano and Merklin1 the reported incidence is es¬ timated at 1:5000 to 10 000 adults. Situs inversus demands total reorientation of the surgeon's perspective so that the step-by-step operation will proceed smoothly. According to Sako et al,2 malignancy of the bile ducts is a very rare lesion (0.01% to 0.46% of reported autopsies) that de¬ mands timely decisions in the operating room (ie, pallia¬ tive or radical resection); however, despite the fact that the surgeon may make the correct decision, overall prognosis in any case is very poor, anatomical complications are very common,3 and morbidity and mortality parallel those of pancreatic carcinoma. With regard to cancer, only the distal bile duct will be discussed in this report. REPORT OF A CASE A

68-year-old

woman was

examined in the emergency de¬

partment at the Piedmont Hospital, Atlanta, Ga, for possible in-

Accepted

for publication May 19, 1991. From the Thalia and Michael Carlos Center for Surgical Anatomy and Technique and the Alfred A. Davis Research Center for Surgical Anatomy and Technique (Drs Lee Skandalakis, Gray, and John Skandalakis) and the Department of Surgery (Dr Organ), Emory University School of Medicine, Atlanta, Ga, and the Department of Surgery, Piedmont Hospital, Atlanta (Dr Lee Skandalakis). Reprint requests to the Centers for Surgical Anatomy and Technique, Emory University School of Medicine, Suite 303 Dental Bldg, 1462 Clifton Rd, Atlanta, GA 30322 (Dr John Skandalakis).

W.

Gray, PhD; John

E.

Skandalakis, MD, PhD

juries sustained in a fall. No significant injury was found. The patient had a history of heavy alcohol consumption and was jaundiced. Her biochemical profile was remarkable for elevated levels of the following components: bilirubin, 135 fimol/L; aspartate aminotransferase, 218 U/L; alkaline phosphatase, 905 U/L; and 7-glutamyltransferase, 2754 U/L. The diagnosis on ad¬ mission was alcoholic hepatitis.

Chest roentgenograms obtained on admission demon¬ strated dextrocardia (Fig 1), which was known to the patient. Abdominal ultrasonography revealed total situs inversus, with the liver and inferior vena cava on the left side, the aorta on the right side, and the pancreas not visualized. Liver/spleen scan confirmed situs inversus with the liver on the left side and the spleen on the right side. Abdominal computed tomographic scan reaffirmed the diagnosis of situs inversus and demonstrated extrahepatic biliary and pancreatic duct obstruction (Fig 2). Endoscopie retrograde cholangiopancreatography outlined a 2-cm high-grade stricture of the distal common bile duct with prominent proximal dilation (Fig 3). The pancreatic duct could not be cannulated. At laparotomy, the diagnosis of situs inversus totalis was con¬ firmed. Moderately well-differentiated adenocarcinoma was di¬ agnosed by frozen section analysis from tissues behind the head of the pancreas. No identifiable extension was observed outside the pancreas. Two lymph nodes from the posterior peripancreatic area and portal vein were sent for frozen section analysis and demonstrated no evidence of metastatic disease. The portal vein, superior mesenteric vein, and root of the mesocolon were free of tumor. The tumor was deemed resectable, according to the criteria of Child and Frey.4 A Whipple procedure was performed, and gastrointestinal continuity was restored with end-to-end pancreaticojejunostomy with invagination of the pancreatic stump, end-to-side choledochojejunostomy with transanastomotic T tube, and antecolic gastrojejunostomy without vagotomy (Fig 4). At the end of the dissection, however, a tumor was found to have invaded the posterior surface of the superior mesenteric vein, a factor that was not appreciated during the exploration. Pathologic review of the resected specimen revealed a moderately welldifferentiated adenocarcinoma of the distal common bile duct with four peripancreatic lymph nodes positive for metastatic ad¬ enocarcinoma.

The patient received postoperative adjuvant radiation ther¬ apy and fluorouracil chemotherapy. She did well for 17 months, then developed lower-extremity edema, nonmalignant ascites, and Serratia pneumonia. She died 18 months after the operation.

COMMENT

This patient had two separate problems that warrant discussion: situs inversus totalis and adenocarcinoma of the distal common bile duct.

Downloaded From: http://archsurg.jamanetwork.com/ by a University of Pennsylvania User on 06/16/2015

Fig

Dextrocardia visible

\.

on

admission chest roentgenogram.



2.—Abdominal computed tomographic scan. 1 indicates di¬ lated pancreatic duct; 2, superior mesenteric vein; 3, superior mesenteric artery; 4, aorta; 5, mass; 6, dilated distal common bile duct in front of mass; 7, gallbladder; and 8, dilated intrahepatic common bile duct.

Fig 3. —Endoscopie retrograde cholangiopancreatography strating dilated common bile duct.

demon¬

Fig

Situs Inversus Left-to-right transposition of the normally asymmetrical organs of the body is termed situs inversus viscerum. The opposite, normal condition is termed situs solitus. Situs in¬ versus may be total or partial, complicated or simple. In the total form, the heart lies to the right of the midline, with complete reversal of its chambers. The aorta turns to the right. The left lung is divided into three lobes, and the right lung has only two lobes. In the abdomen, the liver and stomach are reversed, and the gallbladder lies to the left of the midline. The spleen and pancreas lie to the right. The colonie flexures are reversed. In partial situs inversus, the inversion may be limited to the thoracic organs or to the cardiac chambers. There are three well-known associated embryonic defects in-

Fig 4. —Diagram of the biopsy specimen, showing the distal com¬ hepatic duct, the gallbladder and cystic duct, the common bile duct and tumor, the proximal aspect of the pancreas, the distal as¬

mon

pect of the stomach, and the duodenum.

volving total

or partial situs inversus: Kartagener's syn¬ drome (familial) involves situs inversus totalis with symp¬ toms of bronchiectasis and chronic sinusitis; asplenia syndrome involves situs inversus partialis with symp¬ toms of severe cardiac defects; and polysplenia syndrome involves situs inversus partialis with symptoms of severe cardiac defects. A patient with situs inversus totalis should have iden¬ tically functioning organ systems as patients with situs solitus, and, therefore, characteristics of lesions should be the same as a comparable normal patient. In this patient, the inversion seems to have been silent for 63 years and had the adenocarcinoma not manifested itself, she most likely would have lived a normal, healthy life.

Downloaded From: http://archsurg.jamanetwork.com/ by a University of Pennsylvania User on 06/16/2015

Adenocarcinoma of the Distal Common Bile Duct The adenocarcinoma in our patient was a primary can¬ cer of the lower common bile duct not originating from the duodenum, the papilla of Vater, the gallbladder, or the liver. There was no extension of the lesion either from the adjacent lymph nodes with metastatic disease or to the common bile duct distal to the insertion of the cystic duct. Blumgart,5 in agreement with Klatskin,6 van Heerden et al,7 and Weinbren and Mutum,8 classified the malignancy of the bile duct in three locations: the upper third, involv¬ ing the common hepatic and hepatic ducts; the middle third, involving the common bile duct between the cystic and upper part of the duodenum; and the lower third, involving the area between the upper border of the duo¬ denum and the papilla of Vater. The common bile duct is divided anatomically into four portions: supraduodenal, retroduodenal, pancreatic, and intramural.9 In our case, the last three segments of the common bile duct were involved. Part of the supraduo¬ denal portion may also have been involved, but we can¬ not be certain since the local anatomy was distorted due to the cancer. The three types of adenocarcinoma of the bile ducts in¬ clude papillary, nodular, and diffuse. As reported by Weinbren and Mutum,8 the papillary type is multiple and more common at the distal common bile duct. The mid¬ dle and upper ducts are hosts of the nodular type. In the diffuse type, the wall of the ducts is thickened, and the thickness occupies a great part of the wall. According to Weinbren,10 the malignant tumors of the distal 2 cm of the common bile duct are masses of papil¬ lary adenocarcinomas that are friable and pink or grayishwhite. Alexander et al11 reported that lesions of the distal common bile duct are fewer in number (13%) than tumors in the proximal bile ducts. Soreide et al12 agreed, report¬ ing 94 cases with 15 distal malignant lesions. We know very little of the etiology of carcinoma of the bile ducts, although Yeo et al13 presented some plausible theories. From time to time, there have been reports of several associated conditions, perhaps as a link with ad¬ enocarcinoma of the bile ducts. Hou14 and Lin15 have re¬ ported associations with parasitic infections, such as Clonorchis sinensis or Opisthorchis. Welton et al16 reported the association of typhoid carriers with adenocarcinoma. Blumgart et al17 reported gallstones existing concur¬ rently with adenocarcinoma. Ross et al18 and RobertsThompson et al19 found associative ulcerative colitis. Irwin and Morison20 reported malignant degeneration of a choledochal cyst and adenocarcinoma. Sell and Dunsford21 believe that hepatocarcinogens may influence liver stem cells. Thompson et al22 reported sclerosing cholangitis in association with adenocarcinoma. Several authors have reported associations of adeno¬ carcinoma with drugs. Lowenfels and Norman23 reported the association of cancer with isoniazid. The presence of adenocarcinoma and methyldopa was noted by Broden and Bengtson.24 In addition, Yen et al25 an asso¬ ciation between oral contraceptive use and the incidence of adenocarcinoma. We believe that the presence of situs inversus with bile duct carcinoma in our patient was a coincidental phe¬ nomenon. A few other cases have been reported in the literature of concurrent situs inversus and bile duct carci¬ noma. In 1989, Kim et al26 reported hepatocellular and stomach carcinoma in a 66-year-old woman with situs in-

reported

Similar cases were reported by Wada et al27 and Kanematsu et al.28 Renal cell carcinoma and situs inversus totalis were reported by Bertini and Boileau.29 Transverse colon carcinoma and situs inversus were noted by Yoshida et al,30 as was carcinoma of the head of the pan¬ creas with situs inversus viscerum totalis by Sakaguchi et al.31 Talerman,32 Abdel-Dayem et al,33 and Ibrahim et al34 reported cases of embryonal ovarian carcinoma. Detailed preoperative evaluation of the patient is es¬ sential. Computed tomography, sonography, and arteriography will help define the strategy to follow in the operating room. Knowing whether multiple lymph node biopsy specimens are negative or positive will certainly assist the surgeon in making decisions. Once the criteria of Child and Frey4 for contraindica¬ tions for pancreaticoduodenectomy have been applied and a tumor is deemed resectable, the treatment of choice is surgery, either palliative or radical resection of the area, performed with a typical Whipple procedure. The site, extent, metastasis, age, and general condition of the pa¬ tient should be considered when deciding the extent of resection. The Child and Frey contraindications include the following: (1) liver métastases or carcinomatous im¬ plants on the hepatic serosa; (2) invasion of the base of the colonie mesentery; (3) invasion of the hepatoduodenal ligament; (4) invasion of the gastroduodenal, hepatic, or superior mesenteric arteries; (5) involvement of the porta hepatis; (6) métastases to the portal vein; (7) tumor fixa¬ tion of the duodenum and pancreas to underlying struc¬ tures; and (8) métastases to the aorta or vena cava. Positive lymph nodes are not a contraindication for and Hermann and Cooperman35 believed that localized masses in the head of the pancreas should be resected even in the absence of histo¬ logie proof of malignancy. They suggested that "the smallest tumors, and perhaps those with the most favor¬ able outcome, may never be resected for lack of histological proof of the diagnosis."35 The site and proximal extension of the tumor is another problem. Most tumors involve the union of the right and left hepatic ducts and most likely extend within the hepatic parenchyma. The extension of the tumor to the head of the pancreas and peripancreatic tissue should be investigated. The index fingers of the surgeon should be placed behind the pancreatic neck and should easily sep¬ arate the head of the pancreas and the distal common bile duct from the retroperitoneal space, portal vein, and su¬ perior mesenteric vessels. Under these circumstances, a Whipple procedure may be undertaken. Prognosis is poor with or without radiation therapy and chemotherapy. Liver transplantation provides very poor results in pa¬ tients with bile duct cancer.36 When operating on a patient with situs inversus tota¬ lis, the surgeon must reposition himself or herself to af¬ ford adequate access and orientation. The ideal position is always on the side of the body that contains the diseased organ. This repositioning should compensate for any operative confusion the situs inversus creates, disal¬ lowing other variations or incidental abnormalities. versus.

pancreaticoduodenectomy,

References 1. Varano NR, Merklin RJ. Situs inversus: review of the literature, report of four cases and analysis of the clinical implications. J Coll Surg. 1960;33:131-148. Int 2. Sako S, Seitzinger GL, Garside E. Carcinoma of the extra\x=req-\

Downloaded From: http://archsurg.jamanetwork.com/ by a University of Pennsylvania User on 06/16/2015

hepatic

bile ducts: review of the literature and report of six

Surgery. 1957;41:416-437. 3. Skandalakis JE, Gray SW, Rowe JS Jr. Anatomical Complications in General Surgery. New York, NY: McGraw-Hill Intercases.

national Book Co; 1983:125-145. 4. Child CG, Frey CF. Pancreaticoduodenectomy. Surg Clin North Am. 1966;46:1201-1213. 5. Blumgart LH, ed. Surgery of the Liver and Biliary Tract. New York, NY: Churchill Livingstone Inc; 1988:829. 6. Klatskin GK. Adenocarcinoma of the hepatic duct at its bifurcation with the porta hepatis. Am J Med. 1965;38:241-256. 7. van Heerden JA, Judd ES, Dockerty MB. Carcinoma of the extrahepatic bile ducts. Am JSurg. 1967;13:49-55. 8. Weinbren K, Mutum SS. Pathological aspects of cholangiocarcinoma. J Pathol. 1983;139:217-238. 9. Skandalakis JE, Gray SW, Rowe JS, Skandalakis LJ. Surgical anatomy of the pancreas. Contemp Surg. 1979;15:17-40. 10. Weinbren K. Tumours of the bile duct: pathological asand pects. In: Blumgart LH, ed. Surgery of the Liver Biliary Tract. New York, NY: Churchill Livingstone Inc; 1988:795. 11. Alexander F, Rossi RL, O'Bryan M, Khettriy U, Braasch JW, Watkins E. Biliary carcinoma: a review of 109 cases. Am J

Surg. 1984;147:503-509. 12. Soreide O, Kelley

Czerniak CJ,

A,Hadjis NS, Edwards WH Jr, Blumgart LH. Clinical spectrum and diagnostic strategies in non-calculous lower bile duct obstruction. Dig Surg.

1986;2:209-221. 13. Yeo CJ, Pitt HA, Cameron JL. Clin North Am. 1990;70:1429-1447.

Cholangiocarcinoma. Surg

14. Hou PC. The relationship between primary carcinoma of the liver and infestation with Clonorchis sinensis. J Pathol Bacteriol. 1956;72:239-246. 15. Lin TY. Malignant tumour of the liver. In: Bockus HL, ed. Gastroenterology. 3rd ed. Philadelphia, Pa: WB Saunders Co; 1976:522. 16. Welton

hepatobiliary

JC, Marr JS, Freidman SM. Association between

1979;1:791-794.

cancer

and

typhoid

carrier

state.

Lancet.

17. Blumgart LH, Hadjis NS, Benjamin IS, Beazley RM. Surgical approaches to cholangiocarcinoma at confluence of hepatic ducts. Lancet. 1984;1:66-70.

J Surg. 1944;32:319-321. 21. Sell S, Dunsford HA. Evidence for the

hepatocellular

carcinoma and

Pathol. 1989;134:1347-1363. 22.

Thompson HH,

Pitt

stem cell

origin of

cholangiocarcinoma.

Am

J

HA, Tompkins RK, Longmire WP Jr.

Primary sclerosing cholangitis:

a

Surg. 1982;196:127-136.

heterogeneous disease.

Ann

23. Lowenfels AB, Norman J. Isoniazid and bile duct cancer. JAMA. 1978;240:434-435. 24. Broden G, Bengtson L. Biliary carcinoma associated with methyldopa therapy. Acta Chir Scand Suppl. 1980;500:7-12. 25. Yen S, Hsieh CC, MacMahon B. Extrahepatic bile duct cancer and smoking, beverage consumption, past medical

history,

and

2116. 26. Kim

oral-contraceptive

use.

Cancer.

1987;59:2112\x=req-\

Yl, Tada I, Kuwabara A, Kobayashi M. Double cancer of the liver and stomach with situs inversus totalis: a case report. Jpn J Surg. 1989;19:756-759. 27. Wada I, Nambu M, Sakita R,

et al. Metastasis of hepatocellular carcinoma to the duodenum: an autopsy case with triple primary carcinoma on heterotaxia (situs inversus transversus viscerum totalis). Nippon Shokakibyo Gakkai Zasshi.

1983;80:228-231.

28. Kanematsu T, Matsumata T, Kohno H, Sugimachi K, Inokuchi K. Hepatocellular carcinoma with situs inversus. Cancer. 1983;51:549-552. 29. Bertini JE Jr, Boileau MA. Renal cell carcinoma in a patient with situs inversus totalis. J Surg Oncol. 1987;34:29-31. 30. Yoshida J, Tsuneyoshi M, Nakamura K, Murakami T, Akamine Y. Primary biliary dyskinesia with transverse colon carcinoma. Am J Clin Pathol. 1986;85:101-104. 31. Sakaguchi O, Kamio H, Sakurai H, Kumagai K, Kobayashi E. Pancreas head carcinoma associated with situs inversus viscerum totalis. Nippon Geka Gakkai Zasshi. 1985;86:111-115. 32. Talerman A. A patient with situs inversus totalis and embryonal carcinoma of the ovary. Gynecol Oncol. 1984;19:371\x=req-\

372. 33. Abdel-Dayem HM, Motawi S, Jahan S, Kubasik H. Liver metastasis from embryonal carcinoma of the ovary with complete situs inversus: first reported case and review of literature.

AP, Braasch JW, Warren KW. Carcinoma of the proximal bile ducts. Surg Gynecol Obstet. 1973;136:923-928. 19. Roberts-Thompson IC, Strickland RJ, Mackay IR. Bile

Clin Nucl Med. 1984;9:558-560. 34. Ibrahim EM, Al-Idrissi H, Al-Faraq A, Al-Tamimi D, Perry W. Situs inversus totalis with embryonal cell carcinoma of ovaries. Gynecol Oncol. 1984;18:270-273.

1973;3:264-267.

cer.

18. Ross

duct carcinoma in chronic ulcerative colitis. Aust N Z JMed.

20. Irwin ST, Morison JE. Congenital cyst of common bile duct containing stones and undergoing cancerous change. Br

35. Hermann RE, Cooperman AM. Current concepts in canN Engl JMed. 1979;301:482-485. 36. Calne RY, Williams R. Orthotopic liver transplantation: the first 60 patients. Br Med J.1977;1:471-476.

Downloaded From: http://archsurg.jamanetwork.com/ by a University of Pennsylvania User on 06/16/2015

Cancer of bile duct with situs inversus.

We present a case of adenocarcinoma of the distal common bile duct in a 68-year-old woman with total situs inversus. Endoscopic retrograde cholangiopa...
3MB Sizes 0 Downloads 0 Views