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Mucin-producing hepatic cystic neoplasms: an uncommon but challenging disease often misdiagnosed and mismanaged
- Source :
- Acta Chirurgica Belgica, Vol. 120, no.1, p. 1-10 (2018), Acta Chirurgica Belgica, Vol. 120, no. 1, p. 6-15 (2020)
- Publication Year :
- 2018
-
Abstract
- Background: Mucin-producing hepatic cystic neoplasms (MHCN) are uncommon and potentially malignant. Methods: Nine MHCN were encountered in our centre for over 32 years. Patients’ clinical, biological, radiological and pathological features were reviewed. Lesions were classified into Mucinous Cystic Neoplasms (MCN) and Intraductal Papillary Neoplasms of the Bile duct (IPNB) (WHO 2010 classification). Results: Five MCN and 4 IPNB were reviewed. Serum and intracystic tumour markers were insufficient to diagnose malignancy. Complications were encountered in five out of nine patients (56%), mean symptom duration was 26 months (range: 1–132). Three patients were mismanaged pre-referral. Radiological features enabled preoperative diagnosis in eight out of nine patients (89%). Greater tumour size, unilocular lesion and mural nodularity indicated malignancy. Radical tumour excision was achieved in eight patients. One IPNB patient was misdiagnosed and underwent unroofing. For 103 months median follow-up, five out of six patients with benign tumours were alive and disease-free, whereas the misdiagnosed IPNB recurred with fatal malignant transformation seven years later. Among the three patients with malignancies (median follow-up: 77 months), two IPNB died, one from cancer recurrence and one from unrelated causes, whereas the malignant MCN was alive and disease-free. Conclusions: Appropriate MHCN diagnosis is crucial, yet it is often misdiagnosed and mismanaged. The prognosis after complete excision is favourable
- Subjects :
- Adult
Male
medicine.medical_specialty
Disease
Malignancy
cystic tumour
Malignant transformation
Lesion
Diagnosis, Differential
03 medical and health sciences
0302 clinical medicine
medicine
Humans
mucinous cystic neoplasm
Liver surgery
Pathological
Aged
Retrospective Studies
Bile duct
business.industry
Papillary Neoplasm
Mucin
Liver Neoplasms
General Medicine
Middle Aged
medicine.disease
Adenocarcinoma, Mucinous
medicine.anatomical_structure
Treatment Outcome
intraductal papillary neoplasm of the bile duct
Bile Duct Neoplasms
030220 oncology & carcinogenesis
030211 gastroenterology & hepatology
Surgery
Female
Radiology
medicine.symptom
business
Subjects
Details
- ISSN :
- 00015458
- Volume :
- 120
- Issue :
- 1
- Database :
- OpenAIRE
- Journal :
- Acta chirurgica Belgica
- Accession number :
- edsair.doi.dedup.....4906f86bef79b231382fef412e21320d