Primary hepatic paraganglioma with megacolon: A case report

Bo,Jin-Peng; Zhou,Nan; Sun,Meng-Xue; Zhou,Jian

doi:10.3892/ol.2023.13769

Primary hepatic paraganglioma with megacolon: A case report

Authors:
- Jin-Peng Bo
- Nan Zhou
- Meng-Xue Sun
- Jian Zhou
View Affiliations

Affiliations: Department of Radiology, Beijing Tiantan Hospital, Capital Medical University, Beijing 100070, P.R. China, Department of Pathology, Beijing Tiantan Hospital, Capital Medical University, Beijing 100070, P.R. China
Published online on: March 22, 2023 https://doi.org/10.3892/ol.2023.13769
Article Number: 183
Copyright: © Bo et al. This is an open access article distributed under the terms of Creative Commons Attribution License.

Metrics: Total Views: 0 (Spandidos Publications: | PMC Statistics: )

Metrics: Total PDF Downloads: 0 (Spandidos Publications: | PMC Statistics: )

Cited By (CrossRef): 0 citations Loading Articles...

Abstract

Primary hepatic paraganglioma (PGL) is a rare neuroendocrine tumor characterized by clinical manifestations including paroxysmal hypertension, palpitation, abdominal pain and constipation. In the present study, the case of a 21‑year‑old woman with pathologically confirmed hepatic PGL with megacolon following surgery is reported. The patient initially visited Beijing Tiantan Hospital (Beijing, China) for hypoferric anemia. A triple‑phase CT scan of the whole abdomen showed a large hypodense mass with a solid periphery and strong arterial enhancement of the peripheral solid portion of the liver. The sigmoid colon and rectum were obviously distended, filled with gas and intestinal contents. The patient was preoperatively diagnosed with iron deficiency anemia, liver injury and megacolon and then underwent partial hepatectomy, total colectomy and enterostomy. Microscopically, the liver cells exhibited an irregular zellballen pattern. In addition, immunohistochemical staining revealed that liver cells were positive for CD56, chromogranin A, vimentin, S‑100, melan‑A and neuron‑specific enolase. Therefore, the diagnosis of primary PGL of the liver was confirmed. These findings suggested that primary hepatic PGL should not be excluded when megacolon occurs and comprehensive imaging evaluation is of great importance for its diagnosis.

View Figures

View References

1	Lloyd RV, Osamura YR, Kloppel G and Rosa J: WHO classification of tumours of endocrine organs. WHO Classification of Tumours. 10. 4th edition. World Health Organization; Geneva: 2017
2	Liao W, Ding ZY, Zhang B, Chen L, Li GX, Wu JJ, Zhang B, Chen XP and Zhu P: Primary functioning hepatic paraganglioma mimicking hepatocellular carcinoma: A case report and literature review. Medicine (Baltimore). 97:e02932018. View Article : Google Scholar : PubMed/NCBI
3	Thosani S, Ayala-Ramirez M, Román-González A, Zhou S, Thosani N, Bisanz A and Jimenez C: Constipation: an overlooked, unmanaged symptom of patients with pheochromocytoma and sympathetic paraganglioma. Eur J Endocrinol. 173:377–387. 2015. View Article : Google Scholar : PubMed/NCBI
4	Corti B, D'Errico A, Pierangeli F, Fiorentino M, Altimari A and Grigioni WF: Primary paraganglioma strictly confined to the liver and mimicking hepatocellular carcinoma: An immunohistochemical and in situ hybridization study. Am J Surg Pathol. 26:945–949. 2002. View Article : Google Scholar : PubMed/NCBI
5	Khan MR, Raza R, Jabbar A and Ahmed A: Primary non-functioning paraganglioma of liver: A rare tumour at an unusual location. J Pak Med Assoc. 6:814–816. 2011.PubMed/NCBI
6	Lin CS and Hsu YH: A primary paraganglioma of the liver mimicking hepatocellular carcinoma. Ci Ji Yi Xue Za Zhi. 31:286–288. 2019.PubMed/NCBI
7	Vella I, De Carlis R, Lauterio A and De Carlis L: Extremely rare presentation of primary nonfunctioning hepatic paraganglioma. Dig Liver Dis. 54:838–839. 2022. View Article : Google Scholar : PubMed/NCBI
8	Bachmeier CAE, Haque M, Barrett HL and Morton A: Hepatic paraganglioma hiding as a slowly growing lesion for 24 years: A diagnostic conundrum. BMJ Case Rep. 12:e2289472019. View Article : Google Scholar : PubMed/NCBI
9	Koh PS, Koong JK, Westerhout CJ and Yoong BK: Education and imaging. Hepatobiliary and pancreatic: A huge liver paraganglioma. J Gastroenterol Hepatol. 28:10752013. View Article : Google Scholar : PubMed/NCBI
10	Miller ME, Vietor NO, Park EJ, Sweeney SP, Katz M and Vietor RC: Paraganglioma masquerading as a primary liver lesion: A rare entity discovered during surgery. Clin Case Rep. 10:e053102022. View Article : Google Scholar : PubMed/NCBI
11	Chang H, Xu L and Mu Q: Primary functioning hepatic paraganglioma: A case report. Adv Ther. 23:817–820. 2006. View Article : Google Scholar : PubMed/NCBI
12	You Z, Deng Y, Shrestha A, Li F and Cheng N: Primary malignant hepatic paraganglioma mimicking liver tumor: A case report. Oncol Lett. 10:1176–1178. 2015. View Article : Google Scholar : PubMed/NCBI
13	Corssmit EP and Romijn JA: Clinical management of paragangliomas. Eur J Endocrinol. 171:R231–R243. 2014. View Article : Google Scholar : PubMed/NCBI
14	Gunawardane PTK and Grossman A: Phaeochromocytoma and paraganglioma. Adv Exp Med Biol. 956:239–259. 2017. View Article : Google Scholar : PubMed/NCBI
15	Szakacs JE and Cannon A: L-Norepinephrine myocarditis. Am J Clin Pathol. 30:425–434. 1958. View Article : Google Scholar : PubMed/NCBI
16	Sweeney AT, Malabanan AO, Blake MA, de las Morenas A, Cachecho R and Melby JC: Megacolon as the presenting feature in pheochromocytoma. J Clin Endocrinol Metab. 85:3968–3972. 2000. View Article : Google Scholar : PubMed/NCBI
17	Carrasquillo JA, Chen CC, Jha A, Ling A, Lin FI, Pryma DA and Pacak K: Imaging of pheochromocytoma and paraganglioma. J Nucl Med. 62:1033–1042. 2021. View Article : Google Scholar : PubMed/NCBI
18	Baez JC, Jagannathan JP, Krajewski K, O'Regan K, Zukotynski K and Ramaiya NH: Pheochromocytoma and paraganglioma: Imaging characteristics. Cancer Imaging. 12:153–162. 2012. View Article : Google Scholar : PubMed/NCBI
19	Jain A, Baracco R and Kapur G: Pheochromocytoma and paraganglioma-an update on diagnosis, evaluation, and management. Pediatr Nephrol. 35:581–594. 2020. View Article : Google Scholar : PubMed/NCBI
20	Tanaka S, Ito T, Tomoda J, Higashi T, Yamada G and Tsuji T: Malignant pheochromocytoma with hepatic metastasis diagnosed 20 years after resection of the primary adrenal lesion. Intern Med. 32:789–794. 1993. View Article : Google Scholar : PubMed/NCBI
21	Crona J, Taïeb D and Pacak K: New perspectives on pheochromocytoma and paraganglioma: Toward a molecular classification. Endocr Rev. 38:489–515. 2017. View Article : Google Scholar : PubMed/NCBI
22	Fishbein L: Pheochromocytoma and paraganglioma: Genetics, diagnosis, and treatment. Hematol Oncol Clin North Am. 30:135–150. 2016. View Article : Google Scholar : PubMed/NCBI
23	Neumann HPH, Young WF Jr and Eng C: Pheochromocytoma and paraganglioma. N Engl J Med. 381:552–565. 2019. View Article : Google Scholar : PubMed/NCBI
24	Karimi P, Islami F, Anandasabapathy S, Freedman ND and Kamangar F: Gastric cancer: Descriptive epidemiology, risk factors, screening, and prevention. Cancer Epidemiol Biomarkers Prev. 23:700–713. 2014. View Article : Google Scholar : PubMed/NCBI