Int Canc Conf J (2014) 3:72–76 DOI 10.1007/s13691-013-0118-6
CASE REPORT
Neuroendocrine tumor in the Glissonean pedicle of the hepatic hilum Takayoshi Kaida • Akira Chikamoto • Yuki Kiyozumi • Hideaki Nitta Katsunori Imai • Osamu Nakahara • Yoshiaki Ikuta • Koichi Doi • Takatoshi Ishiko • Hiroshi Takamori • Toru Beppu • Hideo Baba
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Received: 25 April 2013 / Accepted: 18 June 2013 / Published online: 12 July 2013 Ó The Japan Society of Clinical Oncology 2013
Abstract Neuroendocrine tumors are neoplasms with various pathologic features. They occur in many areas such as the pituitary gland, digestive tract, and lung. Here, we report the case of a 68-year-old man who developed a neuroendocrine tumor in the Glissonean pedicle of the hepatic hilum. At a routine medical screening, the patient was found to have a hepatic tumor adjacent to the portal bifurcation measuring 2.0 cm in diameter. Results of laboratory and imaging studies were highly suggestive of well-differentiated hepatocellular carcinoma. The patient underwent excision of the tumor, which was located within the Glissonean pedicle. On immunohistochemical examination, the tumor was positive for synaptophysin, chromogranin A, and CD56 immunostaining. The final pathologic diagnosis was low-grade neuroendocrine tumor, according to the World Health Organization’s WHO Classification of Tumours of the Digestive System. The patient is doing well, with no evidence of recurrence, more than 1 year after the operation. Keywords Neuroendocrine tumor Glissonean pedicle Porta hepatis
T. Kaida (&) A. Chikamoto Y. Kiyozumi H. Nitta K. Imai O. Nakahara Y. Ikuta K. Doi T. Ishiko H. Takamori T. Beppu H. Baba (&) Department of Gastroenterological Surgery, Graduate School of Medical Sciences, Kumamoto University, Honjo 1-1-1, Kumamoto 860-8556, Japan e-mail:
[email protected] H. Baba e-mail:
[email protected]
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Introduction Neuroendocrine tumors (NETs) occur in many areas, including the gastrointestinal tract (73.7 %) and the bronchopulmonary system (25.1 %) [1]. Within the gastrointestinal tract, most occur in the small bowel (28.7 %), appendix (18.9 %), and rectum (12.6 %) [1]. NETs of the extrahepatic bile duct are extremely rare, accounting for 0.1–2 % of all gastrointestinal NETs [2]. Development of a NET in the Glissonean pedicle (GP) has not been reported in the English-language literature. Here, we report a NET in the GP of the hepatic hilum.
Case report During a routine medical screening, a 68-year-old man showed the development of a tumor in the hepatic portal region. The tumor was 2.0 cm in diameter, with no findings indicative of invasion. It was left untreated for almost 5 years, during which time it increased in size. The patient was referred to Kumamoto University Hospital for treatment. He had no subjective or objective symptoms on the first visit. On laboratory examination, liver enzyme levels were within normal limits, and the patient was negative for hepatitis B or C infection. Although the alpha-fetoprotein level was slightly elevated, the des-gamma-carboxy prothrombin level was within normal limits. Results of contrast-enhanced abdominal computed tomography revealed a round tumor with a clear border, which had increased to 2.8 cm in diameter over 5 years, and which was gradually enhanced (Fig. 1). Results of magnetic resonance imaging (MRI) showed that the tumor was low intensity on T1weighted images and high intensity on T2-weighted images. On the magnetic resonance cholangiopancreatography
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Fig. 1 Results of contrastenhanced abdominal computed tomography. a Initial examination of the tumor. The tumor diameter was 2.0 cm. b Follow-up 5 years after the first examination. The tumor had grown to 2.8 cm in diameter. c Image of early arterial phase of dynamic liver CT, d coronal image of liver CT
(MRCP) image, there was no dilation of any bile duct (Fig. 2). On dynamic study of MRI with gadoliniumethoxybenzyl-diethylenetriamine pentaacetic acid, the tumor was gradually enhanced, and the contrast medium was washed out at a late phase. Apparent diffusion coefficient (ADC) values derived from diffusion-weighted imaging (DWI) were high. [18F]-2-Fluoro-2-deoxy-D-glucose positron emission tomography (FDG-PET) revealed no abnormal accumulation of FDG in the lesion. The results of laboratory and imaging studies were highly suggestive of well-differentiated hepatocellular carcinoma. We therefore scheduled partial liver resection as surgery for excision of the tumor. Although preoperative imaging indicated that the tumor was located in the region of the hepatic hilum and originated from liver parenchyma, cranially adjacent to the portal bifurcation, intraoperative findings did not show the tumor in the region of the hepatic hilum and the tumor was free of liver parenchyma. We thought that the tumor was located within the right GP, and so we opened the capsule. The tumor was deep inside the right GP, surrounded by the right hepatic artery and the right hepatic duct (Figs. 3, 4). No continuity between the tumor and the hepatic parenchyma or hepatic duct was
observed. The tumor was resected completely without residual tumor (Fig. 5). On pathology examination, the tumor was diagnosed as a NET measuring 2.0 cm in diameter. Microscopic examination showed that the tumor cells possessed oval-shaped nuclei, were acidophilic and possessed a clear cytoplasm. On immunochemical examination, the tumor was positive for chromogranin A, synaptophysin, and CD56 staining. The MIB-1 index was less than 1 % (Fig. 6). The final pathologic diagnosis was NET, G1 according to the World Health Organization’s WHO Classification of Tumours of the Digestive System. The patient was discharged 13 days after the operation. Results of subsequent systemic MRI and capsule endoscopy of the small intestine showed no primary lesion. The patient is doing well, with no evidence of recurrence, more than 1 year after the operation.
Discussion Neuroendocrine tumors can occur in any part of the body, and most occur in the digestive tract. They are commonly classified by embryonic origin as foregut, midgut, and
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Fig. 4 Schematic of the location of the tumor. The tumor was surrounded by the hepatic artery, portal vein, and hepatic duct
Fig. 2 On the magnetic resonance cholangiopancreatography (MRCP) image, there is no dilation of any bile duct
Fig. 3 Intraoperative findings. The tumor was located within the right Glissonean pedicle
hindgut. The proportion of foregut origin in Japan is 30.4 %, that of midgut is 9.6 %, and that of hindgut is 60.0 % [3]. Primary hepatic NETs or primary extrahepatic bile duct NETs are particularly rare [4, 5]. No Englishlanguage reports of NET within the GP have been published. The preoperative diagnosis of this tumor was well-differentiated hepatocellular carcinoma because of the incidence and increase in size over 5 years. However,
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Fig. 5 a Resected tumor has no liver parenchyma, b cut surface of resected tumor
operative findings showed that the tumor did not originate in the liver parenchyma and that it was located within the right GP. The tumor was free of invasion to adjacent
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Fig. 6 Microscopic examination showed that the tumor cells possessed ovalshaped nuclei, were acidophilic and possessed a clear cytoplasm. Immunohistochemical results showed positivity for synaptophysin (b), chromogranin A (c), and CD56 (d). Nuclear fusion of tumor cells was observed in less than 1 in 10 high-power fields (MIB-1 index \1 %) (e)
anatomic structures, such as the hepatic artery, portal vein, and hepatic duct, and was resected completely and safely without injury to surrounded structures. Because no local recurrence was observed 1 year after the operation, this operative technique appears to be valid with respect to tumor clearance. A precise preoperative diagnosis for NET is difficult. NETs are classified as functioning or nonfunctioning according to the presence of hormone secretion. In Japan, most NETs are nonfunctioning. The incidence of functioning tumors is approximately 3.4 % [3]. In the present case, ADC values derived from DWI of MRI showed positive results. Although high ADC values often indicate malignancy, they are not definitive for diagnosis.
Kwekkeboom and associates reported the usefulness of somatostatin receptor scintigraphy for the diagnosis of preoperative metastasis or postoperative recurrence [6]. This method has high sensitivity and specificity for the diagnosis of NET [7, 8]. Although NETs can arise throughout of body, most originate in the gastrointestinal tract or lung. In addition, small primary lesions are not detected with various diagnostic modalities. Accordingly, definitive diagnosis of NET arising in the GP is very difficult. Capsule endoscopy cannot detect primary lesions in the digestive tract. The absence of emergence of a primary lesion during a 5-year observation period may support the diagnosis of NET in the GP. However, long-term follow-up is required because of slow growth of low-grade NETs.
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76 Conflict of interest of interest.
Int Canc Conf J (2014) 3:72–76 The authors declare that they have no conflict
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