Extrahepatic cholestasis associated with paracoccidioidomycosis: Challenges in the differential diagnosis of biliopancreatic neoplasia

José Sebastião Dos Santos; Vitor de Moura Arrais; William José Rosseto Ferreira; Ricardo Ribeiro Correa Filho; Mariângela Ottoboni Brunaldi; Rafael Kemp; Ajith Kumar Sankanrakutty; Jorge Elias Junior; Fernando Bellissimo-Rodrigues; Roberto Martinez; Edson Zangiacomi Martinez; José Celso Ardengh

doi:10.4251/wjgo.v16.i6.2531

Extrahepatic cholestasis associated with paracoccidioidomycosis: Challenges in the differential diagnosis of biliopancreatic neoplasia

World J Gastrointest Oncol. 2024 Jun 15;16(6):2531-2540. doi: 10.4251/wjgo.v16.i6.2531.

Affiliations

¹ Faculdade de Medicina de Ribeirão Preto da Universidade de São Paulo (FMRP-USP), Departamento de Cirurgia e Anatomia, Ribeirão Preto 14049-900, São Paulo, Brazil.
² Universidade de São Paulo, Faculdade de Medicina de Ribeirão Preto da Universidade de São Paulo (FMRP-USP), Departamento de Patologia, Ribeirão Preto 14048900, São Paulo, Brazil.
³ Universidade de São Paulo, Faculdade de Medicina de Ribeirão Preto da Universidade de São Paulo (FMRP-USP), Departamento de Imagens Médicas, Hematologia e Oncologia Clínica, Ribeirão Preto 14048900, São Paulo, Brazil.
⁴ Universidade de São Paulo, Faculdade de Medicina de Ribeirão Preto da Universidade de São Paulo (FMRP-USP), Departamento de Medicina Social , Ribeirão Preto 14015-010, São Paulo, Brazil.
⁵ Universidade de São Paulo, Faculdade de Medicina de Ribeirão Preto da Universidade de São Paulo (FMRP-USP), Departamento de Clínica Médica, Ribeirão Preto 14015-010, São Paulo, Brazil.
⁶ Universidade de São Paulo, Faculdade de Medicina de Ribeirão Preto da Universidade de São Paulo (FMRP-USP), Departamento de Cirurgia e Anatomia, Ribeirão Preto 14049-900, São Paulo, Brazil and Hospital Moriah, Serviço de Endoscopia Digestiva, São Paulo 04084-002, São Paulo, Brazil. jcelso@uol.com.br.

Abstract

Background: Paracoccidioidomycosis (PCM) may involve the hepatic pedicle and peripancreatic lymph nodes, cause damage to the bile duct and manifest, exceptionally, in combination with extrahepatic cholestasis (EHC), making investigation and treatment challenging.

Aim: To investigate the management of patients with visceral PCM admitted with EHC.

Methods: All patients diagnosed with PCM treated in a public, tertiary teaching hospital between 1982 and 2020 were retrospectively evaluated. Those also identified with EHC were allocated to two groups according to the treatment approach for the purpose of comparing clinical, laboratory, and imaging findings, resources used for etiological diagnosis, treatment results, and prognosis. Statistical analyses were performed using the linear mixed-effects model (random and fixed effects), which was adjusted using the PROC MIXED procedure of the SAS^® 9.0 software, and Fisher's exact test.

Results: Of 1645 patients diagnosed with PCM, 40 (2.4%) had EHC. Of these, 20 (50.0%) lived in the rural area and 29 (72.5%) were men, with a mean age of 27.1 years (3-65 years). Jaundice as first symptom and weight loss of at least 10 kg were observed in 16 patients (40.0%), and a mass in the head of the pancreas was observed in 8 (20.0%). The etiological diagnosis was made by tissue collection during surgery in 4 cases (10.0%) and by endoscopic methods in 3 cases (7.5%). Twenty-seven patients (67.5%) received drug treatment alone (Group 1), whereas 13 (32.5%) underwent endoscopic and/or surgical procedures in combination with drug treatment (Group 2). EHC was significantly reduced in both groups (40.7% in Group 1, with a mean time of 3 months; and 38.4% in Group 2, with a mean time of 7.5 months), with no statistically significant difference between them. EHC recurrence rates, associated mainly with treatment nonadherence, were similar in both groups: 37% in Group 1 and 15.4% in Group 2. The mortality rate was 18.5% in Group 1 and 23% in Group 2, with survival estimates of 71.3% and 72.5%, respectively, with no statistically significant difference.

Conclusion: Although PCM-related EHC is rare, it needs to be included in the differential diagnosis of malignancies, as timely treatment can prevent hepatic and extrahepatic sequelae.

Keywords: Blastomycosis; Cholestasis; Diagnosis; Jaundice; Obstructive; Paracoccidioides; Treatment.