Aggressive intrahepatic cholangiocarcinoma in pregnancy: Case report and literature review

Abstract

Introduction and Objectives

Cholangiocarcinoma is a very rare type of hepatobiliary cancer and extremely rare reported during pregnancy. Its early and timely diagnosis is complicated. To report a rare and poorly studied case of aggressive intrahepatic cholangiocarcinoma during pregnancy in a 30-year-old patient.

Material and Patients

Female patient, 30 years old, with antecedent of 2 cesarean sections, one 2 years ago and the second one 1 and a half year ago, without complications and occupational exposure to unspecified pesticides. The clinical picture begins at 32 weeks of gestation characterized by nausea and vomiting of gastric contents, dull pain in the right hypochondrium and weight loss of 7 kg in 2 months, to which generalized jaundice, choluria, acholia, pruritus, nocturnal diaphoresis and ecchymosis; A simple magnetic resonance image was performed and a large liver lesion was identified at the level of liver segments IV and VIII with a maximum diameter of 10.3 cm, suggestive of malignancy associated with the presence of satellite lesions suggestive of infiltration to the rest of the liver parenchyma. It was decided to resolve the pregnancy at 35 weeks of gestation by cesarean section without apparent complications. During the mid-surgical postpartum period simple and contrasted tomography of the abdomen is performed where hepatic, pulmonary, pleural and bone tumor activity and dilation of the intrahepatic bile duct are reported; tumor markers ACE 1.91, CA 19-9 30.89, AFP 149.4; liver biopsy reports metastasis of moderately differentiated adenocarcinoma (g2) consistent with primary bile duct (cholangiocarcinoma); Immunohistochemistry with positivity for ck7, ck19, negative for ck20, gata 3, cdx2, pax8 and hepar1.

Results

During his in-hospital stay, she presented sinus tachycardia evidenced by ECG, associated with risk factors, and pulmonary thromboembolism was suspected. The ICU service was consulted and they accepted the case, evaluated by cardiology performing an echocardiogram discarding the diagnosis. The general surgery, oncological surgery and oncology services were consulted and commented that she was not a candidate for surgical or systemic treatment for advanced disease clinical stage IV.

She was discharged from the hospital with palliative measures and two weeks later she was re-admitted to the emergency department due to generalized tonic-clonic seizures advanced airway manage was performed and vasopressor support was decided; simple skull tomography without metastatic activity; presented clinical deterioration and progression of the disease leading to multiple organ failure. The patient died 4 days later. The baby is being monitored by ophthalmology for a diagnosis of retinopathy of prematurity.

Conclusions

Cholangiocarcinoma is the second most common liver neoplasm, it encompasses neoplasms that depend on the bile duct. It has an incidence in pregnancy of 10 cases/10,000 pregnancies, making it a very uncommon pathology and only 12 cases reported from 1998 to 2023 are known. Its prognosis is lethal due to its aggressiveness and diagnosis in advanced stages. The treatment is only surgical, however the procedure carries high rates of morbidity and mortality.