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Case Reports
. 2023 Aug 30;23(1):186.
doi: 10.1186/s12902-023-01439-7.

Diagnostic dilemma in a patient with history of medullary thyroid carcinoma and abnormal serum liver enzymes; a case report with six years follow up

Fatemeh Rahmani  1 , Maryam Tohidi  2 , Farid Azmoudeh-Ardalan  3 , Amir Sadeghi  4 , Farzad Hadaegh  1
Affiliations
Case Reports

Diagnostic dilemma in a patient with history of medullary thyroid carcinoma and abnormal serum liver enzymes; a case report with six years follow up

Fatemeh Rahmani et al. BMC Endocr Disord. .

Abstract

Background: Medullary thyroid carcinoma (MTC) is a neuroendocrine tumor that originates from parafollicular C-cells. Calcitonin (Ctn) and carcinoembryonic antigen (CEA) are useful biomarkers for monitoring MTC cases.

Case presentation: Here, we describe a 48-year-old woman, who presented in 2014 with bilateral thyroid nodules. Report of fine needle aspiration was suspicious for MTC; initial laboratory evaluation showed serum Ctn level of 1567 pg/mL. After excluding type 2 multiple endocrine neoplasia syndrome clinically, total thyroidectomy and neck lymph node dissection were performed. The final histopathological diagnosis was right lobe MTC with neither vascular invasion nor lymph node involvement. On regular follow-up visits, Ctn and CEA levels have been undetectable, and repeated cervical ultrasonographic exams were unremarkable from 2014 to 2021. As liver enzymes became elevated in 2016, the patient was further evaluated by a gastroenterologist. Abdominopelvic ultrasonography revealed a coarse echo pattern of the liver parenchyma with normal bile ducts. A liver fibroscan showed a low fibrosis score (7kPa). The patient was recommended to use ursodeoxycholic acid. According to the progressive rise of liver enzymes with a cholestatic pattern in October 2020, a liver biopsy was performed that showed tiny nests of neuroendocrine-like cells with a background of primary biliary cholangitis (PBC). Immunohistochemical stainings were positive for chromogranin A (CgA), and synaptophysin and negative for Ctn, CEA, and thyroglobulin. Further imaging investigations did not reveal any site of a neuroendocrine tumor in the body. Considering normal physical exam, imaging findings, as well as normal serum levels of Ctn, CEA, CgA, and procalcitonin, the patient was managed as a PBC.

Conclusion: In follow-up of a patient with MTC, we reported progressively increased liver enzymes with a cholestatic pattern. Liver biopsy revealed nests of neuroendocrine-like cells with a background of PBC, the findings that might suggest acquiring neuroendocrine phenotype by proliferating cholangiocytes.

Keywords: Case report; Histopathology; Medullary thyroid carcinoma; Neuroendocrine tumor; Primary biliary cholangitis.

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Conflict of interest statement

The authors declare that they have no competing interests.

Figures

Fig. 1
Fig. 1
Trend of basal serum Ctn and CEA levels from 2014 to 2021. Ctn: calcitonin, CEA: carcinoembryonic antigen
Fig. 2
Fig. 2
Trend of serum liver enzymes levels. ALT: Alanine aminotransferase, AST: Aspartate aminotransferase, ALP: alkaline phosphatase
Fig. 3
Fig. 3
Histopathologic sections of the liver biopsy (×100 and × 400 magnifications): A and B, Liver tissue with cholestatic pattern of injury and bile duct damage (H&E stain); C and D, Fibrous expansion of portal tract with occasional p-p bridges (Trichrome stain); E and F Nests of neuroendocrine-like cells (H&E stain)
Fig. 4
Fig. 4
Immunohistochemistry (IHC) stained sections of the liver biopsy (×100 and × 400 magnifications): A and B. IHC staining for Chromogranin A; C and D, IHC staining for synaptophysin
See this image and copyright information in PMC

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