Abstract
We present the case of a 46 year old woman with a giant, 23-centimeter, atypical carcinoid of the liver. A primary site for this neoplasm could not be identified despite multiple radiographic imaging studies, including a somatostatin scan, and a thorough inspection of the bowel during surgical resection of the lesion. Histologically, the tumor displayed mild cytologic atypia, abundant necrosis, and intravascular metastases, the last feature of which was identified by immunohistochemical markers for chromogranin and synaptophysin. Also described is the unusual sinusoidal infiltration, or "spillage," of tumor cells into the surrounding liver parenchyma, a feature that has not been described as far as we are aware but may suggest an aggressive clinical course. Even though an exact definition of atypia for these lesions apparently does not exist at this point, the multiple atypical features in this case strongly suggest the diagnosis of atypical carcinoid of the liver, thus far an altogether rare and vaguely reported entity. As more cases arise in the medical literature, it may be worthwhile to establish a set of guidelines to define atypical hepatic carcinoids and other gastrointestinal carcinoids, although survivorship data thus far indicates no significant difference in the prognosis between typical versus atypical variants.
Highlights
Primary hepatic carcinoid tumor is an incredibly rare entity but must be distinguished from other lesions such as hepatocellular carcinoma because of its different treatment and prognostic implications
We present the case of a giant atypical carcinoid tumor that, as far as we can determine, is primary to the liver and displays the unusual histopathologic phenomenon of sinusoidal infiltration throughout the surrounding liver parenchyma
We have presented the case of giant primary hepatic carcinoid tumor with a number of atypical features including an enormous size, mild cytologic atypia, abundant necrosis, and intravascular metastases
Summary
Primary hepatic carcinoid tumor is an incredibly rare entity but must be distinguished from other lesions such as hepatocellular carcinoma because of its different treatment and prognostic implications At this time about 125 cases have been reported, but many of these may have been metastases or a neuroendocrine component of another neoplasm [2]. Magnetic resonance imaging showed a 23 × 16 × 14 cm intraparenchymal hepatic mass virtually replacing the right lobe of the liver while medially displacing the portal vein and inferior vena cava. Ingrowth into these vascular structures could not be identified. Based upon the pathologic and radiologic findings, the surgical team decided to perform a right hepatic lobectomy
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