Primary carcinoid tumors of the liver

World Journal of Surgical Oncology, Aug 2008

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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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. - 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 edFUreilgatprluiacsrsteeoignumg1neadn6ot.3fotfhteh5e.a3bledfot5ml.o0ebhnee;tUoeflrttorhageseonliuvoneudrs osofltidhemaabsdsoinmtehne 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. aeFCniTgdhuaspncreacelnivn2iogs,fwpthiotehoraIlVbydcmoomanrtegrniansattneddepmmeolavnsiss;tiCrnaTtsesgcmane4n.o9tf ItIho4ef.9atbhcdemolmiv ern 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)


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Gary Schwartz, Agnes Colanta, Harold Gaetz, John Olichney, Fadi Attiyeh. Primary carcinoid tumors of the liver, World Journal of Surgical Oncology, 2008, pp. 91, 6, DOI: 10.1186/1477-7819-6-91