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CASE REPORT Table of Contents   
Year : 2010  |  Volume : 53  |  Issue : 4  |  Page : 760-762
Primary hepatic neuroendocrine tumor: An unusual cystic presentation

1 Department of Pathology, Bhagwan Mahaveer Jain Hospital, Bangalore, India
2 Department of Surgical Gastroentrology, Bhagwan Mahaveer Jain Hospital, Bangalore, India
3 Department of Radiology, Bhagwan Mahaveer Jain Hospital, Bangalore, India

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Date of Web Publication27-Oct-2010


Primary neuroendocrine tumors of the liver are exceedingly rare and unlike metastatic neuroendocrine tumor rarely cause carcinoid syndrome. There are only about 60 such cases reported in the current literature. We present a case of a 57-year-old male with a primary hepatic neuroendocrine tumor which presented as cystic mass and successfully resected. The case presented required meticulous radiological, histopathological, and immunohistochemical work-up to rule out an occult extrahepatic malignancy with hepatic metastasis to confirm the primary nature of hepatic tumors. Here we intend to put forward a review of the current literature regarding the diagnosis, pathology, and management of this disease.

Keywords: Computerized tomography scan, immunohistochemistry, liver, neuroendocrine tumor

How to cite this article:
Shetty PK, Baliga SV, Balaiah K, Gnana PS. Primary hepatic neuroendocrine tumor: An unusual cystic presentation. Indian J Pathol Microbiol 2010;53:760-2

How to cite this URL:
Shetty PK, Baliga SV, Balaiah K, Gnana PS. Primary hepatic neuroendocrine tumor: An unusual cystic presentation. Indian J Pathol Microbiol [serial online] 2010 [cited 2021 Jul 25];53:760-2. Available from: https://www.ijpmonline.org/text.asp?2010/53/4/760/72078

   Introduction Top

Primary neuroendocrine tumors of the liver are rare and ususally do not cause carcinoid syndrome. Only 60 such tumors have been reported. [1],[2]

   Case Report Top

A 57-year-old male presented with right upper quadrant abdominal pain, intermittent dull in nature with tightness of abdomen and mild fever on and off since 1 month. His past medical history revealed known case of hypertension since 12 years. On physical examination eyes were icitric, no abdominal scars, normal bowel sounds on auscultation, minimal right upper quadrant tenderness on palpation, no rebound tenderness or guarding, and no hepatomegaly. His laboratory studies were significant for bilirubin 1.2 mg/dl (normal 0.3-1.0 mg/dl) and Gamma glutamyl transferase of 147 U/L (normal 5-80 U/L). Ultrasound of the abdomen revealed multiple cysts in the right lobe of liver with floating membranes suggestive of hydatid cysts. An abdominal computerized tomography (CT) scan with contrast was obtained, which revealed multiple hypodense space-occupying lesions in the right lobe of liver [Figure 1] with no other intra-abdominal masses or lymphadenopathy. Laprotomy was performed and since cyst was vascular and adherent to the liver tissue, partial cystectomy was performed. Grossly partial cystectomy tissue was in multiple bits, together measuring 7.5x5 cm, and outer surface and cut surface were brownish in color. Microscopically, the tumor consisted predominantly of cyst with large areas of hemorrhage, and solid component consisted of tumor cells arranged in glandular, trabacular, and organoid nests of uniform, intermediate-sized, polyhedral cells in a vascular stroma. The tumor cells had uniform round to oval nucleus having vesicular fine granular chromatin with moderate amount of eosinophilic cytoplasm. There were no areas of necrosis and mitoses were infrequent [Figure 2]. A diagnosis of metastatic carcinoma was made and subjected for immunohistochemical work up. First panel of immunohistochemical markers performed could not rule out metastatic occult primary as tumor cells were positive for Cytokeratin (CK)7 and CK-19 and negative for CK-20, Carcinoembryonic Antigen (CEA), Thyroid Transcription Factor (TTF)1, Prostate Specific Antigen (PSA), and Alpha Fetoprotein (AFP). When second panel of immunohistochemical markers for synaptophysin and chromogranin were performed on the biopsy specimen, the tumor cells expressed synaptophysin and chromogranin consistently with a neuroendocrine tumor [Figure 3]. Further workup to rule out metastatic sites included a negative CT scan of the chest, upper and lower gastrointestinal endoscopy. An uncomplicated right hepatic lobectomy (segment V, VI, VII, VIII) and segment IV resection were performed. No peritoneal carcinomatosis was noted upon exploration. The postoperative course was uneventful. Grossly right hepatic lobectomy with segment IV resection tissue measured 19 × 14 × 11 cm, outer surface was smooth gray yellow, and cut surface showed multiple well circumscribed hemorrhagic cystic masses with gray white solid areas forming 20% of the lesion [Figure 4]. Microscopically it showed similar features as described earlier with surgical margins free of tumor. The histomorphologic features coupled with the immunohistochemical results confirmed the diagnosis of a primary neuroendocrine tumor.
Figure 1: CT scan showing mulitple hypodense space occupying lesion liver

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Figure 2: Photomicrograph showing tumor cells arranged in glandular and trabacular pattern with vascular storma (H and E, ×100)

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Figure 3: (a) Tumor cells showing immunopositivity for CK-7 (IHC, ×200), (b) Tumor cells showing immunopositivity for CK-19 (IHC, ×200) (c) Tumor cells showing immunopositivity for synaptophysin, (d) Tumor cells showing immunopositivity for chromogranin. (IHC, ×200)

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Figure 4: Gross photograph of liver showing a multiple cystic tumor

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   Discussion Top

Primary neuroendocrine tumor of liver is rare and was first described by Edmonson in 1958. It represents 0.3% of all carcinoids and a total of 60 cases of primary hepatic neuroendocrine tumor have been reported in the literature. [1],[2] Females are affected slightly more often than males (1.4:1) and the age group of affected patients ranges from 18 to 84 with an average age of 54 years. [3]

Primary hepatic neuroendocrine tumors may be an incidental finding or can present with severe symptoms including abdominal pain, jaundice, palpable right upper quadrant mass, carcinoid syndrome, carcinoid heart disease, and Cushings syndrome. Interestingly, the carcinoid syndrome is rarely present in primary hepatic neuroendocrine tumors because hepatic enzymatic degradation of neoplastic-derived products spill directly into the portal circulation. [4],[5],[6] Our patient presented only with abdominal pain.

Grossly, hepatic carcinoids can vary widely in size ranging from 1 cm to 20 cm. [3],[7] They are well demarcated from the surrounding liver parenchyma and their cut surfaces are generally gray-yellow in color with multiple irregular hemorrhagic areas. However in our case it was an atypical cystic presentation with hemorrhagic areas. Microscopy of neuroendocrine tumor commonly shows varied pattern of solid, nested, trabecular, and microacinar arrangements. [7] In our case latter two patterns predominated. The immunohistochemical characteristics of hepatic carcinoid include positivity for the neurosecretory markers: chromogranin, synaptophysin, and neuron-specific enolase. Cytokeratin tends to impart a granular perinuclear staining pattern. Stains for CK20, CEA, TTF-1, PSA, and AFP are negative. [3] In our case immunohistochemical tests showed similar pattern. The overall 5-year survival rate for primary hepatic carcinoids is excellent, averaging 92%, while the metastasis rate is 45%. [3]

In conclusion, on histopathology when a hepatic cystic neuroendocrine tumor is diagnosed, extensive immunohistochemical work up should be carried out to rule out unknown primary along with whole body CT scan and upper and lower gastrointestinal endoscopy. [8]

   Acknowledgment Top

We thank Bangalore Institute of Oncology for there help with Immunohistochemistry.

   References Top

1.Caplin ME, Buscombe JR, Hilson AJ, Jones AL, Watkinson AF, Burroughs AK. Carcinoid tumor. Lancet 1998;352:799-805.  Back to cited text no. 1
2.Mehta DC, Warner RR, Parnes I, Weiss M. An 18-year follow-up of primary hepatic carcinoid with carcinoid syndrome. J Clin Gastroenterol 1996;23:60-2.  Back to cited text no. 2
3.Soga J. Primary hepatic endocrinomas (carcinoids and variant neoplasms). A statistical evaluation of 126 reported cases. J Exp Clin Cancer Res 2002;21:457-68.   Back to cited text no. 3
4.Tohyama T, Matsui K, Kitagawa K. Primary hepatic carcinoid tumor with carcinoid syndrome and carcinoid heart disease: A case report of a patient on long-term follow-up. Intern Med 2005;44:958-62.   Back to cited text no. 4
5.Shah NA, Urusova IA, DAgnolo A, Colquhoun SD, Rosenbloom BE, Vener SL, et al. Primary hepatic carcinoid tumor presenting as Cushings syndrome. J Endocrinol Invest 2007;30:327-33.  Back to cited text no. 5
6.Komatsuda T, Ishida H, Furukawa K, Miyauchi T. Heianna Primary carcinoid tumor of the liver: Report of a case with an emphasis on contrast-enhanced ultrasonographic findings. J Clin Ultrasound 2005;33:302-4.  Back to cited text no. 6
7.Roskams T, De Vos R, van den Oord JJ, Desmet V. Cells with neuroendocrine features in regenerating human liver. APMIS Suppl 1991;23:32-9.  Back to cited text no. 7
8.Pilichowska M, Kimura N, Ouchi A, Lin H, Mizuno Y, Nagura H. Primary hepatic carcinoid and neuroendocrine carcinoma: Clinicopathological and immunohistochemical study of five cases. Pathol Int 1999;49:318-24.  Back to cited text no. 8

Correspondence Address:
Prasad K Shetty
Department of Pathology, Bhagwan Mahaveer Jain Hospital, Vasanthnagar, Bangalore - 560 052
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/0377-4929.72078

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  [Figure 1], [Figure 2], [Figure 3], [Figure 4]

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