Abstract
We report a case of successful resection of hepatocellular carcinoma (HCC) in a patient with situs ambiguous with polysplenia (PS), a rare congenital anomaly. A 62-year-old Japanese man was admitted to our hospital for investigation and treatment of a hepatic tumor. Imaging studies revealed two HCCs, 2.5 and 0.7 cm in diameter, in the anterior section and segment 3 of the reversed liver, respectively. He also had multiple spleens in the right side of the abdomen, right side of the stomach, and duodenum, as well as azygous continuation of the inferior vena cava (IVC) with absence of the retro-hepatic IVC, a truncated pancreas, rare variation of the hepatic artery, and dextrocardia. We performed anterior sectionectomy of the liver and partial resection of segment 3. We found only two previous reports of HCC in patients with situs ambiguous with PS, but our patient is the first to have undergone anatomical hepatic resection of the reversed liver. Accurate anatomical assessment is essential during surgery on such patients.
References
Applegate KE, Goske MJ, Pierce G, Murphy D. Situs revisited: imaging of the heterotaxy syndrome. Radiographics. 1999;19:837–52.
Fulcher AS, Turner MA. Abdominal manifestations of situs anomalies in adults. Radiographics. 2002;22:1439–56.
Ghosh S, Yarmish G, Godelman A, Haramati LB, Spindola-Franco H. Anomalies of visceroatrial situs. AJR Am J Roentgenol. 2009;193:1107–17.
Tawfik AM, Batouty NM, Zaky MM, Eladalany MA, Elmokadem AH. Polysplenia syndrome: a review of the relationship with viscero-atrial situs and the spectrum of extra-cardiac anomalies. Surg Radiol Anat. 2013;2013:19.
Sugimachi K, Ikeda Y, Taketomi A, Tomikawa M, Kawasaki K, Korenaga D, et al. Extended hemi-hepatectomy with portal vein reconstruction in a patient with situs ambiguous. Case Rep Gastroenterol. 2008;2:76–82.
Li T, Wang L, Chen RX, Ye QH, Sun HC, Qin LX, et al. Hepatocellular carcinoma with situs inversus totalis and polysplenia syndrome. Liver Int. 2007;27:1430–1.
Matsuda M, Fujii H, Kono H, Matsumoto Y. Surgical treatment of recurrent hepatocellular carcinoma based on the mode of recurrence: repeat hepatic resection or ablation are good choices for patients with recurrent multicentric cancer. J Hepatobiliary Pancreat Surg. 2001;8:353–9.
Low JP, Williams D, Chaganti JR. Polysplenia syndrome with agenesis of the dorsal pancreas and preduodenal portal vein presenting with obstructive jaundice—a case report and literature review. Br J Radiol. 2011;84:e217–20.
Peoples WM, Moller JH, Edwards JE. Polysplenia: a review of 146 cases. Pediatr Cardiol. 1983;4:129–37.
Winer-Muram HT, Tonkin IL. The spectrum of heterotaxic syndromes. Radiol Clin North Am. 1989;27:1147–70.
Matsusue S, Kashihara S, Koizumi S. Pancreatectomy for carcinoma of the head of the pancreas associated with multiple anomalies including the preduodenal portal vein. Jpn J Surg. 1984;14:394–8.
Hirohata S, Isobe H, Mitamura T, Yoshinoya S, Miyamoto T, Motoki T, et al. Situs ambiguous with polysplenia complicated by renal adenocarcinoma. Arch Intern Med. 1985;145:1134–7.
Curran JG, Ryan MJ. Hodgkin’s disease in a patient with polysplenia. Br J Radiol. 1987;60:929–31.
Iwamura T, Shibata N, Haraguchi Y, Hisashi Y, Nishikawa T, Yamada H, et al. Synchronous double cancer of the stomach and rectum with situs inversus totalis and polysplenia syndrome. J Clin Gastroenterol. 2001;33:148–53.
Chirica M, Vullierme MP, Sibert A, Cazals-Hatem D, Gaudin B, Belghiti J, et al. Major hepatectomy for peripheral papillary cholangiocarcinoma with hilar extension in a patient with situs ambiguous. Gastroenterol Clin Biol. 2005;29:456–60.
Park SS, Min BW, Kim WB, Han HJ, Yong HS, Kim SJ, et al. Double cancer of the stomach and oesophagus with situs ambiguus with polysplenia: the importance of preoperative evaluation. Dig Liver Dis. 2005;37:799–802.
Tsutsumi R, Nagata Y, Enjoji A, Ohno Y, Kamito H, Kanematsu T. Situs ambiguous with gastric cancer: report of a case. Surg Today. 2007;37:676–9.
Byun JR, Jahng JH, Song JC, Yu JS, Lee DK. Supine position endoscopic retrograde cholangiopancreatography in a patient with situs ambiguous with polysplenia. Dig Endosc. 2010;22:322–4.
Mimatsu K, Oida T, Kano H, Kawasaki A, Fukino N, Kida K, et al. Preduodenal portal vein, intestinal malrotation, polysplenia, and interruption of the inferior vena cava: a review of anatomical anomalies associated with gastric cancer. Surg Radiol Anat. 2012;34:179–86.
Choi MG, Kim JY, Noh JH, Bae JM, Kim S, Sohn TS. Laparoscopy-assisted distal gastrectomy in a patient with early gastric cancer and polysplenia. Surg Laparosc Endosc Percutan Tech. 2012;22:e15–7.
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Masanori Matsuda and his co-authors have no conflicts of interest to report.
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Matsuda, M., Amemiya, H., Hosomura, N. et al. Hepatic resection for hepatocellular carcinoma in a patient with situs ambiguous with polysplenia: report of a case. Surg Today 45, 765–771 (2015). https://doi.org/10.1007/s00595-014-0997-y
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DOI: https://doi.org/10.1007/s00595-014-0997-y