J Liver Cancer.  2021 Sep;21(2):187-193. 10.17998/jlc.2021.09.17.

Primary multifocal cystic signet ring neuroendocrine tumor of liver: a case report

• Bansal N
• Satapathy B

• KMID: 2520907
• DOI: http://doi.org/10.17998/jlc.2021.09.17

Abstract

Primary signet ring neuroendocrine tumors of the liver are extremely rare tumors. Morphologically, they mimic signet ring cell adenocarcinomas; however, the absence of mucin by special stains and the expression of neuroendocrine markers help to diagnose these tumors. We herein report a case of a 47-year-old female who presented with multiple solid and cystic lesions in both liver lobes, which were initially suggested to be biliary cystadenocarcinoma on imaging. Liver biopsy of the lesion revealed the presence of a signet ring neoplasm with diffuse expression of synaptophysin and pan-cytokeratin. The case was subsequently diagnosed as a primary hepatic signet ring neuroendocrine tumor. The patient was offered 3 cycles of chemotherapy and is well preserved after 14 months of diagnosis. Although this is an extremely rare entity, its possibility should be considered in the differential diagnosis of neoplasms characterized by signet ring cell morphology.

Keyword

Neuroendocrine neoplasm; Signet ring cell; Pathology; Case report

Figure

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  Figure 1 Contrast-enhanced computed tomography findings. There are cystic lesions in both lobes of liver with peripheral solid component with heterogeneous enhancement on arterial phase (A). On venous phase images, the solid components remain heterogeneous (B). Some of the solid components show calcification (C).

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  Figure 2 Positron emission tomography computed tomography shows multiple liver lesions with uptake in the solid components (A), and intraabdominal lymph nodes (B).

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  Figure 3 Microscopic features of the tumor. Low power magnification demonstrates fragments of tumor along with the non-neoplastic hepatic parenchyme (A). The tumor demonstrates a papillary architecture, with the tumor cells centered around microvasculature (B). High power magnification demonstrates the details of the tumor cells (C): the tumor cells demonstrate eccentrically located nuclei and intracytoplasmic vacuoles with a signet ring cell-like appearance. (A) Hematoxylin-eosin, original magnification ×100, (B) ×200, (C) ×400. Mucicarmine stain demonstrates the absence of mucin in the cytoplasm (D) (original magnification, ×400).

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  Figure 4 Immunohistochemical stain results of the tumor. The tumor cells express pan-cytokeratin (A), synaptophysin (C), CD56 (D), chromogranin A (E) and are negative for thyroid transcription factor-1 (B). The Ki-67 labeling index was 2–3% (F). (original magnification x100 [A–D], x200 [E], x400 [F]).

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