Primary hepatic carcinoid tumors: A case report and literature review

Adel Johari; Nisar H Zaidi; Abdulmalik Altaf; Abdel N. Y. Kibeida

doi:10.4103/2320-3846.118154

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CASE REPORT

Year : 2013 | Volume : 1 | Issue : 1 | Page : 24-27

Primary hepatic carcinoid tumors: A case report and literature review

Adel Johari¹, Nisar H Zaidi¹, Abdulmalik Altaf², Abdel N. Y. Kibeida³
¹ Department of Surgery, King Abdulaziz University Hospital, Jeddah, Saudi Arabia
² Faculty of Medicine, King Abdulaziz University, Jeddah, Saudi Arabia
³ International Medical Center, Jeddah, Saudi Arabia

Date of Web Publication

13-Sep-2013

Correspondence Address:
Adel Johari
Department of Surgery, King Abdulaziz University Hospital, King Abdulaziz University, Jeddah 21589
Saudi Arabia

Source of Support: None, Conflict of Interest: None

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DOI: 10.4103/2320-3846.118154

Abstract

Primary hepatic carcinoid is a rare disease and requires meticulous workup for the search of primary in other organs. A 56-year-old male, known hypertensive, presented in November 2007 with epigastric discomfort and indigestion on and off for 2 months. There was no history of diarrhea, flushing attacks, or cardiac symptoms. He had no family history of carcinoids or any other chronic disease. On examination, he was afebrile, pulse rate was 83/min, and BP was 166/95 mm Hg. Per-abdominal examination revealed mild tenderness in the epigastrium and enlarged liver. Computerized tomography abdomen showed a capsulated huge liver lesion (11 × 10 × 13 cm) in the left lobe of the liver, displacing the left hepatic artery, while rest of the liver was normal. There was no evidence of carcinoid tumor in any other organ. He was operated and left hepatic lobectomy was done. Histology showed primary hepatic carcinoid. He is being followed up in our OPD till date and has shown no recurrence. Primary hepatic carcinoid is a rare disease which is not associated with the symptoms of carcinoid syndrome in majority of patients, and therefore should be diagnosed after extensive search for primary in the other organs. Ultimate diagnosis should be made by histology and immunohistochemistry.

Keywords: Carcinoid syndrome, primary hepatic carcinoid, tumor

How to cite this article:
Johari A, Zaidi NH, Altaf A, Kibeida AN. Primary hepatic carcinoid tumors: A case report and literature review. Saudi Surg J 2013;1:24-7

How to cite this URL:
Johari A, Zaidi NH, Altaf A, Kibeida AN. Primary hepatic carcinoid tumors: A case report and literature review. Saudi Surg J [serial online] 2013 [cited 2023 Mar 30];1:24-7. Available from: https://www.saudisurgj.org/text.asp?2013/1/1/24/118154

Introduction

Carcinoids are rare tumors originating from Kulchitsky cells in the crypts of Lieberkuhn in the gut and are the most common gastrointestinal neuroendocrine tumors. ^[1] The term "carcinoid" originated from karzinoide, which means carcinoma-like, as given by Siegfried Obendorfer in 1907. ^[2] Carcinoids behave like benign tumors, but microscopically resemble carcinomas and comprise 0.66% of all malignancies. ^[3] The incidence rates of carcinoids are 55% and 30% in the gastrointestinal tract and bronchopulmonary system, respectively, and within the gastrointestinal tract, they occur most commonly in the small intestine (45%), rectum (20%), appendix (17%), colon (11%), and stomach (7%). ^[4] Carcinoid tumors secrete 5-hydroxytryptamine (5-HT), a tryptophan derivative that causes carcinoid syndrome, characterized by loose motion, flushing, bronchoconstriction, and occasional valvular heart disease. ^[5] Carcinoids may be classified as typical [well differentiated with a clear histological pattern (e.g., insular, trabecular, glandular, or mixed)] or atypical (nuclear atypia, necrotic foci, and high mitotic figures). ^[6] The diagnosis of carcinoid tumors is made by determining the urine levels of 5-hydroxyindoleacetic acid (5-HIAA) (specificity, 100%; sensitivity, 73%). ^[7] Chromogranin A (CgA) levels are increased in 85-100% of carcinoid tumors (specificity, 98.4%; sensitivity, 62.9%). ^[8],[9],[10]

Primary carcinoid tumors of the liver are extremely rare; only 94 cases have been reported thus far, ^[11] with no apparent association with preexisting liver disease. The first case of a liver carcinoid was described by Edmondson in 1958. ^[12] Liver carcinoids usually progress to a large size on diagnosis. ^[13] We present the case of a huge primary liver carcinoid in a 56-year-old man who presented at our hospital with non-specific abdominal discomfort.

Case Report

In November 2007, a 56-year-old man with hypertension presented to our hospital because of having epigastric discomfort and indigestion since sometime. He had no history of diarrhea, flushing, cardiac symptoms, or diabetes mellitus, and no family history of carcinoids or other chronic diseases. Upon examination, he was afebrile with pulse rate 83/min and blood pressure 166/95 mm Hg. A per-abdominal examination revealed hepatomegaly and mild tenderness in the epigastrium. Further test results were: hemoglobin level and white blood cell count were normal; albumin level, 4.94 g/dl; and total protein level, 8.32 g/dl. Other liver function tests were normal. α-Fetoprotein (AFP) and carcinoembryonic antigen (CEA) levels were within normal ranges. The 5-HIAA level was 4.8 mg/24 h. Abdominal magnetic resonance imaging (MRI) revealed a solitary intrahepatic lesion with a cephalocaudal diameter of 13 cm and a maximum diameter of 11 × 11 cm. Vascular invasion and lymphadenopathy were not observed. Rest of the liver was normal. Abdominal computerized tomography (CT) [Figure 1] and [Figure 2] scan showed a huge, capsulated liver lesion (11 × 10 × 13 cm) in the left lobe, which had displaced the left hepatic artery; rest of the liver was normally oriented. There was no evidence of carcinoid tumors in any other organ.

Figure 1: CT demonstrating a large mass originating from the left hemi-liver

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Figure 2: CT scan showing a huge, capsulated liver lesion (11 × 10 × 13 cm) in the left hemi-liver, which had displaced the left hepatic artery

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The patient had a hepatic carcinoid and was therefore scheduled for laparotomy. Pre-operatively, a huge tumor was found to occupy most of the lobe of the liver [Figure 3]a, b, thus a left hepatic lobectomy was performed. Macroscopically, the 20 × 12 cm tumor [Figure 4]a, b had a gray/tan cut surface and hemorrhagic foci. Microscopically, the left lobe of the liver had an encapsulated tumor comprising cells arranged in nests, trabeculae, and small glands. The tumor had focal fibrosis with prominent retraction artifacts. The cells exhibited mildly pleomorphic round oval nuclei, inconspicuous nucleoli, and moderate amounts of pale eosinophilic cytoplasm. Rare mitotic figures (< 2/10 high-power fields) were noticed. Few necrotic foci were identified. The tumor had invaded the surrounding capsule and focally extended to the surrounding liver tissue. Vascular invasion foci were noted. The tumor was located 1.5 mm from the surgical margin. Rest of the liver showed mild periportal lymphocytic cell infiltration without fibrosis or cirrhosis. The immunohistochemistry analysis was strongly positive for CgA, AFP, and synaptophysin; weakly positive for cytokeratin (CK) 20; and negative for CK7, carcinoembryonic antigen (CEA), and vimentin. These findings were consistent with a primary hepatic carcinoid tumor [Figure 5]a-e.

Figure 3: (a) Huge carcinoid liver per-operatively. (b) Per-operative view of carcinoid

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Figure 4: (a) Macroscopic view. (b) Macroscopic view after bisecting specimen

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Figure 5: (a) Low power magnification of carcinoid tumor (left upper aspect) separated from liver tissue (right lower aspect) by a fibrous capsule (H and E, 10X). (b) The tumor is formed of nests with retraction artifacts. Desmoblastic reaction is noticed (right aspect), (H and E, 10X). (c)Higher magnification showing the nesting pattern of the tumor (H and E, 20X). (d) High magnification showing the salt and pepper appearance of nuclear chromatin (H and E, 40X). (e) Immunostain for chromogranin showing patchy cytoplasmic positivity (40X). (f) Immunostain for synaptophysin showing strong cytoplasmic positivity (40X)

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Discussion

Primary liver carcinoid is a rare entity, and most neuroendocrine tumors metastasize to the liver. ^[14] Carcinoid syndrome occurs in < 10% of carcinoids. Primary liver carcinoid with carcinoid syndrome is extremely rare. ^[15] Primary hepatic carcinoids can arise from dispersed neuroendocrine cells in the intrahepatic biliary epithelium and possibly from chronic inflammation in the biliary system or ectopic adrenal or pancreatic tissues in the liver. ^[16] Imaging analyses such as abdominal ultrasound, a non-invasive method, are used for detecting solid lesions with cystic liver components. Dynamic abdominal CT scan showed enhanced and low-density masses in the early and late phases, and MRI showed low and high intensities in the T1 and T2 images, respectively. Hepatic carcinoid was finally diagnosed from histological and immunohistochemical results. Carcinoid localization is performed with an octreoscan [somatostatin receptor scintigraphy is superior to CT or MRI (sensitivity, 90%)]. ^[17] Metaiodobenzylguanidine scintigraphy is an alternative to an octreoscan.

In a review of 94 primary hepatic carcinoids, the carcinoids occurred commonly in middle-aged patients (mean age, 49.8 years) and slightly more commonly in women (58.5%). The most common complaint was abdominal pain (44%); 13.1% were asymptomatic, and the overall mortality rate was 25%. ^[11] Carcinoids require a multidisciplinary approach with surgery as the primary treatment modality, hepatic chemoembolization, and medical therapy. Surgical resection of the primary hepatic carcinoid is the treatment of choice in approximately 85% patients. In our patient, a left lobectomy was performed as the primary treatment. An extensive search for carcinoids was performed, but no carcinoids were found outside the liver. Radiofrequency ablation and hepatic chemoembolization significantly improved hepatic metastases or unresectable tumors in some studies. ^[18] Radiolabeled somatostatin analogs, which act as vehicles to guide radioactivity to carcinoid metastases, have also shown improvement. Five-year survival rates of 88.3% for rectal, 73.2% for bronchopulmonary, and 71% for appendicular carcinoids have been reported in 13,715 carcinoid cases. ^[19] Knox et al. reported postoperative 1-, 5-, and 10-year survival rates for primary hepatic carcinoid patients of 88%, 80%, and 68%, respectively. ^[20] Our patient has been followed up for recurrence or carcinoid development in other organs until date, but none was detected.

Conclusion

Primary hepatic carcinoid is a rare disease that is not commonly associated with carcinoid syndrome symptoms; therefore, it should be diagnosed after an extensive search for primary in other organs. The ultimate diagnosis should be made after reviewing histological and immunohistochemical results.

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