Primary carcinoid tumors of the liver
World Journal of Surgical Oncology
Primary carcinoid tumors of the liver
Gary Schwartz 2
Agnes Colanta 1
Harold Gaetz 1
John Olichney 0
Fadi Attiyeh 2
0 Department of Hematology-Oncology, St. Luke's-Roosevelt Hospital Center , 350 West 58th Street, New York, NY, 10019 , USA
1 Department of Pathology, St. Luke's-Roosevelt Hospital Center , 1000 10th Avenue, 1st Floor, New York, NY, 10019 , USA
2 Department of Surgery , 1000 10th Avenue, Suite 2B, New York, NY, 10019 , USA
Background: Primary carcinoid tumors of the liver are uncommon and rarely symptomatic. The diagnosis of primary hepatic etiology requires rigorous workup and continued surveillance to exclude a missed primary. Case Presentation: We present a case of a 62-year-old female with a primary hepatic carcinoid tumor successfully resected, now with three years of disease-free follow-up. We present a review of the current literature regarding the diagnosis, pathology, management, and natural history of this disease entity. Conclusion: Primary carcinoid tumors of the liver are rare, therefore classifying their nature as primary hepatic in nature requires extensive workup and prolonged follow-up. All neuroendocrine tumors have an inherent malignant potential that must be recognized. Management remains surgical resection, with several alternative options available for non-resectable tumors and severe symptoms. The risk of recurrence of primary hepatic carcinoid tumors after resection remains unknown.
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Background
Although carcinoid tumors can be found throughout the
body, 90% occur within the gastrointestinal tract [1]. They
preferentially metastasize to the liver and occasionally (<
10%) cause the carcinoid syndrome by secretion of
serotonin and its precursors, as well as other vasoactive
substances [2]. Primary carcinoid tumors of the liver are
exceedingly rare, with only about 60 cases reported in the
current literature. Meticulous follow-up is necessary to
rule out an occult extrahepatic malignancy with hepatic
metastasis to confirm the primary nature of hepatic
carcinoids.
Case presentation
EG is a 62-year-old female who presented with right upper
quadrant abdominal pain, intermittent in timing and dull
in nature, not related to oral intake and not associated
with nausea or vomiting. Her past medical history
included hypertension, irritable bowel syndrome,
osteoarthritis, and a history of recurrent bilateral lower
extremity deep venous thrombosis on Warfarin. On
physical exam there were no abdominal scars, normal bowel
sounds on auscultation, minimal right upper quadrant
tenderness to palpation, no rebound tenderness or
guarding, no hepatomegaly and a negative Murphy's sign. Her
laboratory studies were significant for a GGT of 162 U/L
(normal 580 U/L), with otherwise normal liver function
tests. Tumor markers were negative, with an AFP of 3.1 ng/
ml.
Diagnostic imaging included an abdominal ultrasound
(Figure 1) which revealed a heterogeneous solid mass in
the lateral segment of the left hepatic lobe measuring 6.3
5.3 5.0 cm. A CT scan with intravenous contrast was
obtained which revealed a 4.9 4.9 cm enhancing, poorly
marginated mass in segment II of the liver, with no other
intra-abdominal masses or lymphadenopathy (Figure 2).
A CT-guided biopsy was performed which yielded scant
tissue with poorly cohesive cells arranged in papillae.
PASD stain showed focal, small mucin droplets in some cells.
Immunohistochemistry was positive for CEA and CK-7
and negative for calretinin, CDX-2, CK-20, Muc-2 and
Muc-6. The limited sample was diagnosed as papillary
adenocarcinoma, favoring metastasis, on the basis of
morphology, special stain results and immunoprofile.
However, a second panel of immunohistochemical stains for
synaptophysin, CD56 and chromogranin were performed
on the biopsy specimen. The tumor cells were negative for
chromogranin but expressed synaptophysin and CD56,
consistent with the immunoprofile of a neuroendocrine
tumor (NET).
Further workup for a primary tumor or other metastatic
sites included a negative CT scan of the chest, upper and
lower gastrointestinal endoscopy, and a Technetium-99m
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latUltrasound of the abdomen; Ultrasound of the
abdomen depicting a 6.3 5.3 5.0 heterogenous solid
mass in the lateral segment of the left lobe of the
liver.
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CT scan of the abdomen and pelvis; CT scan of the
abdomen and pelvis with IV contrast demonstrates a
4.9 4.9 cm enhancing, poorly marginated mass in
segment II of the liver.
An uncomplicated left lateral segmentectomy (II & III)
and cholecystectomy were performed. No peritoneal
carcinomatosis was noted upon exploration. The
postoperative course was uneventful and she was discharged home
on the fourth postoper (...truncated)