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Polycystic echinococcosis

Infectious Disease MONDO:0000288 Echinococcosis Liver disorder Digestive system infectious disorder

Polycystic echinococcosis is a zoonotic helminth infection caused by the larval stage of Echinococcus vogeli, producing multilocular hydatid cysts that typically involve the liver and can infiltrate adjacent organs.

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Mappings
0
Definitions
0
Inheritance
3
Pathophysiology
0
Histopathology
3
Phenotypes
0
Genes
2
Treatments
0
Subtypes
3
Differentials
0
Datasets
0
Trials
⚙

Pathophysiology

3
Hepatic metacestode implantation
Oncospheres establish multilocular metacestode vesicles within the liver parenchyma, anchoring infection to hepatic tissue.
liver link
Show evidence (1 reference)
PMID:33553405 SUPPORT
"Computed tomography of the abdomen revealed a huge polycystic lesion in left liver lobe, without reinforcement after enhanced scanning and sized about 16.9 cm × 12.2 cm, which was considered a type II hydatid cyst."
The CT finding localizes the polycystic hydatid lesion to hepatic parenchyma, consistent with metacestode implantation.
Daughter cyst proliferation and matrix remodeling
Metacestodes bud daughter cysts that coalesce into a multilocular mass, expanding through hepatic parenchyma while remodeling surrounding extracellular matrix.
extracellular matrix organization link
liver link
Show evidence (1 reference)
PMID:33553405 SUPPORT
"Computed tomography of the abdomen revealed a huge polycystic lesion in left liver lobe, without reinforcement after enhanced scanning and sized about 16.9 cm × 12.2 cm, which was considered a type II hydatid cyst."
The multilocular hepatic mass on imaging reflects proliferating daughter cysts and associated matrix remodeling.
Mass effect and local invasion
The enlarging multilocular cyst complex compresses adjacent hepatic tissue and can invade neighboring structures, generating space-occupying effects.
liver link
Show evidence (1 reference)
PMID:33553405 SUPPORT
"Computed tomography of the abdomen revealed a huge polycystic lesion in left liver lobe, without reinforcement after enhanced scanning and sized about 16.9 cm × 12.2 cm, which was considered a type II hydatid cyst."
The large polycystic hepatic lesion demonstrates mass effect consistent with local compression and invasive growth.
●

Phenotypes

3
Digestive(2) Immune(1)
Digestive 2
Polycystic hepatic lesion Hepatic cysts (HP:0001407)
Show evidence (1 reference)
PMID:33553405 SUPPORT
"Computed tomography of the abdomen revealed a huge polycystic lesion in left liver lobe, without reinforcement after enhanced scanning and sized about 16.9 cm × 12.2 cm, which was considered a type II hydatid cyst."
The CT finding documents a large hepatic polycystic hydatid lesion consistent with the phenotype.
Hepatic abscess complication Liver abscess (HP:0100523)
Show evidence (1 reference)
PMID:41431593 SUPPORT
"We describe a rural patient with hepatitis B virus (HBV)-related cirrhosis and neotropical echinococcosis who developed a hepatic abscess that fistulized into the right hemithorax."
The report documents hepatic abscess formation as a complication of neotropical echinococcosis.
Immune 1
Thoracic abscess from hepatic fistula Lung abscess (HP:0025044)
Show evidence (1 reference)
PMID:41431593 SUPPORT
"We describe a rural patient with hepatitis B virus (HBV)-related cirrhosis and neotropical echinococcosis who developed a hepatic abscess that fistulized into the right hemithorax."
The case describes a transdiaphragmatic fistula leading to a thoracic abscess, aligning with a lung abscess complication.
💊

Treatments

2
Albendazole therapy MAXO:0000058
Benzimidazole antiparasitic used for smaller or inoperable hydatid cysts and as adjunct around surgery.
Show evidence (1 reference)
PMID:34034501 SUPPORT
"Treatment with albendazole, whether combined or not with praziquantel, is useful for smaller, uncomplicated cysts (< 5 cm)."
Review guidance highlights albendazole as standard medical therapy for hydatid cysts.
Radical hepatic resection MAXO:0000004
Surgical removal of involved hepatic segments for large polycystic echinococcosis lesions or when malignancy is suspected.
Show evidence (1 reference)
PMID:33553405 SUPPORT
"According to Romic classification, the case belongs to type IIb, and radical left hemi-hepatectomy was performed."
The case demonstrates radical liver resection as operative management for polycystic echinococcosis.
🌍

Environmental Factors

2
Bush dog–paca sylvatic cycle and domestic dog bridging
Transmission amplified when bush dogs or domestic dogs ingest paca viscera, sustaining E. vogeli eggs that contaminate food or water for humans.
Show evidence (2 references)
PMID:41431593 SUPPORT
"Its definitive hosts are Speothos venaticus (bush dog) and Canis familiaris (domestic dog), while Cuniculus paca (paca) serves as the intermediate host... Humans become accidental hosts by consuming food or water contaminated with eggs of this helminth."
The case report outlines the Amazon sylvatic cycle (bush dog–paca) and how domestic dogs bridge to humans in forest settings.
PMID:37549288 SUPPORT
"PE is sustained in Pan-Amazonia by a complex sylvatic cycle. The hunting of its infected intermediate hosts (especially the lowland paca Cuniculus paca) enables the disease to further transmit to humans, when their viscera are improperly handled."
The Pan-Amazon sylvatic cycle centered in tropical forest biome links pacas to bush or domestic dogs and onward human exposure.
Climate stability and hunting patterns driving spillover
Temperature stability and ENSO-linked hunting changes in Pan-Amazonia increase sylvatic circulation and human spillover risk.
Show evidence (1 reference)
PMID:37549288 SUPPORT
"Our findings indicate that temperature stability promotes the sylvatic circulation of the disease... In a scenario where climate extremes are projected to intensify, climate change at regional level appears to be indirectly driving the spillover of E. vogeli."
Modeling shows climate stability and extremes modulate sylvatic transmission and spillover risk.
🔀

Differential Diagnoses

3

Conditions with similar clinical presentations that must be differentiated from Polycystic echinococcosis:

Cystic echinococcosis MONDO:0018408
Overlapping Features Unilocular hydatid disease caused by E. granulosus that presents with solitary liver cysts rather than multilocular lesions.
Distinguishing Features
  • Unilocular cyst architecture and typical imaging classification distinguish it from multilocular polycystic lesions.
Show evidence (1 reference)
PMID:39099980 SUPPORT
"The review highlights the challenges associated with diagnosing the different echinococcosis types, including cystic echinococcosis, alveolar echinococcosis, and polycystic echinococcosis."
Differential diagnosis among echinococcosis subtypes is emphasized as a clinical challenge.
Alveolar echinococcosis Not Yet Curated MONDO:0017282
Overlapping Features Invasive hepatic lesions from E. multilocularis that mimic tumors and require distinction from polycystic hydatid disease.
Distinguishing Features
  • Microvesicular infiltrative pattern and often absent daughter cysts contrast with the larger multilocular cysts of polycystic echinococcosis.
Show evidence (1 reference)
PMID:39099980 SUPPORT
"The review highlights the challenges associated with diagnosing the different echinococcosis types, including cystic echinococcosis, alveolar echinococcosis, and polycystic echinococcosis."
The review notes diagnostic overlap among echinococcosis forms, supporting the need for differentiation.
Hepatocellular carcinoma MONDO:0007256
Overlapping Features Primary liver malignancy that can coexist with or mimic hydatid lesions, requiring imaging and pathology to distinguish.
Distinguishing Features
  • Arterial enhancement and washout patterns and malignant cytology separate HCC from parasitic cyst architecture.
Show evidence (1 reference)
PMID:33553405 SUPPORT
"After a multidisciplinary team discussion, the diagnosis of co-occurrence of hepatic CE and HCC was made... radical left hemi-hepatectomy was performed."
The case report shows that hydatid lesions can coexist with HCC, making HCC an important differential consideration.
{ }

Source YAML

click to show 
name: Polycystic echinococcosis
creation_date: '2026-01-15T17:46:03Z'
updated_date: '2026-02-17T21:53:14Z'
category: Infectious Disease
description: >-
  Polycystic echinococcosis is a zoonotic helminth infection caused by the larval
  stage of Echinococcus vogeli, producing multilocular hydatid cysts that typically
  involve the liver and can infiltrate adjacent organs.
disease_term:
  term:
    id: MONDO:0000288
    label: polycystic echinococcosis
  preferred_term: Polycystic echinococcosis
parents:
- Echinococcosis
- Liver disorder
- Digestive system infectious disorder
infectious_agent:
- name: Echinococcus vogeli
  infectious_agent_term:
    preferred_term: Echinococcus vogeli
    term:
      id: NCBITaxon:6213
      label: Echinococcus vogeli
  description: The cestode species responsible for polycystic echinococcosis in
    humans.
  evidence:
  - reference: PMID:40705460
    supports: SUPPORT
    snippet: We describe a case of E. vogeli echinococcosis in a woman,
      diagnosed through PCR, mitochondrial DNA sequencing, and molecular
      characterization.
    explanation: Molecular confirmation links human infection to Echinococcus
      vogeli.
transmission:
- name: Food or water contaminated by dog or bush dog feces
  description: Human infection occurs after ingestion of E. vogeli eggs shed by
    definitive hosts that fed on infected paca viscera.
  evidence:
  - reference: PMID:41431593
    supports: SUPPORT
    snippet: Humans become accidental hosts by consuming food or water
      contaminated with eggs of this helminth.
    explanation: The case report describes the fecal-oral route from definitive
      hosts as the human exposure pathway.
prevalence:
- population: Pan-Amazonia
  percentage: Unknown; focal endemic with documented human cases and identified
    hotspots
  evidence:
  - reference: PMID:37549288
    supports: SUPPORT
    snippet: We compiled a unique dataset of host occurrences (~86000 records)
      and disease infections (~400 cases) covering the entire Pan-Amazonia and
      employed different modeling and statistical tools to unveil the spatial
      distribution of PE's key animal hosts.
    explanation: The modeling study documents hundreds of human infections
      across Pan-Amazonia, indicating focal endemicity.
has_subtypes: []
pathophysiology:
- name: Hepatic metacestode implantation
  description: Oncospheres establish multilocular metacestode vesicles within
    the liver parenchyma, anchoring infection to hepatic tissue.
  locations:
  - preferred_term: liver
    term:
      id: UBERON:0002107
      label: liver
  evidence:
  - reference: PMID:33553405
    supports: SUPPORT
    snippet: Computed tomography of the abdomen revealed a huge polycystic
      lesion in left liver lobe, without reinforcement after enhanced scanning
      and sized about 16.9 cm × 12.2 cm, which was considered a type II hydatid
      cyst.
    explanation: The CT finding localizes the polycystic hydatid lesion to
      hepatic parenchyma, consistent with metacestode implantation.
- name: Daughter cyst proliferation and matrix remodeling
  description: Metacestodes bud daughter cysts that coalesce into a multilocular
    mass, expanding through hepatic parenchyma while remodeling surrounding
    extracellular matrix.
  locations:
  - preferred_term: liver
    term:
      id: UBERON:0002107
      label: liver
  biological_processes:
  - preferred_term: extracellular matrix organization
    term:
      id: GO:0030198
      label: extracellular matrix organization
  evidence:
  - reference: PMID:33553405
    supports: SUPPORT
    snippet: Computed tomography of the abdomen revealed a huge polycystic
      lesion in left liver lobe, without reinforcement after enhanced scanning
      and sized about 16.9 cm × 12.2 cm, which was considered a type II hydatid
      cyst.
    explanation: The multilocular hepatic mass on imaging reflects proliferating
      daughter cysts and associated matrix remodeling.
- name: Mass effect and local invasion
  description: The enlarging multilocular cyst complex compresses adjacent
    hepatic tissue and can invade neighboring structures, generating
    space-occupying effects.
  locations:
  - preferred_term: liver
    term:
      id: UBERON:0002107
      label: liver
  evidence:
  - reference: PMID:33553405
    supports: SUPPORT
    snippet: Computed tomography of the abdomen revealed a huge polycystic
      lesion in left liver lobe, without reinforcement after enhanced scanning
      and sized about 16.9 cm × 12.2 cm, which was considered a type II hydatid
      cyst.
    explanation: The large polycystic hepatic lesion demonstrates mass effect
      consistent with local compression and invasive growth.
phenotypes:
- name: Polycystic hepatic lesion
  description: Large multilocular hydatid cysts occupying the liver.
  phenotype_term:
    preferred_term: Hepatic cysts
    term:
      id: HP:0001407
      label: Hepatic cysts
  evidence:
  - reference: PMID:33553405
    supports: SUPPORT
    snippet: Computed tomography of the abdomen revealed a huge polycystic
      lesion in left liver lobe, without reinforcement after enhanced scanning
      and sized about 16.9 cm × 12.2 cm, which was considered a type II hydatid
      cyst.
    explanation: The CT finding documents a large hepatic polycystic hydatid
      lesion consistent with the phenotype.
- name: Hepatic abscess complication
  description: Secondary infection of hepatic hydatid lesions leading to abscess
    formation.
  phenotype_term:
    preferred_term: Liver abscess
    term:
      id: HP:0100523
      label: Liver abscess
  evidence:
  - reference: PMID:41431593
    supports: SUPPORT
    snippet: We describe a rural patient with hepatitis B virus (HBV)-related
      cirrhosis and neotropical echinococcosis who developed a hepatic abscess
      that fistulized into the right hemithorax.
    explanation: The report documents hepatic abscess formation as a
      complication of neotropical echinococcosis.
- name: Thoracic abscess from hepatic fistula
  description: Extension of the hepatic abscess through the diaphragm into the
    thoracic cavity, resulting in a secondary thoracic abscess.
  phenotype_term:
    preferred_term: Lung abscess
    term:
      id: HP:0025044
      label: Lung abscess
  evidence:
  - reference: PMID:41431593
    supports: SUPPORT
    snippet: We describe a rural patient with hepatitis B virus (HBV)-related
      cirrhosis and neotropical echinococcosis who developed a hepatic abscess
      that fistulized into the right hemithorax.
    explanation: The case describes a transdiaphragmatic fistula leading to a
      thoracic abscess, aligning with a lung abscess complication.
biochemical: []
genetic: []
environmental:
- name: Bush dog–paca sylvatic cycle and domestic dog bridging
  description: Transmission amplified when bush dogs or domestic dogs ingest
    paca viscera, sustaining E. vogeli eggs that contaminate food or water for
    humans.
  environment_context:
    preferred_term: rural settlement
    term:
      id: ENVO:01000763
      label: rural settlement
  evidence:
  - reference: PMID:41431593
    supports: SUPPORT
    snippet: Its definitive hosts are Speothos venaticus (bush dog) and Canis
      familiaris (domestic dog), while Cuniculus paca (paca) serves as the
      intermediate host... Humans become accidental hosts by consuming food or
      water contaminated with eggs of this helminth.
    explanation: The case report outlines the Amazon sylvatic cycle (bush
      dog–paca) and how domestic dogs bridge to humans in forest settings.
  - reference: PMID:37549288
    supports: SUPPORT
    snippet: PE is sustained in Pan-Amazonia by a complex sylvatic cycle. The
      hunting of its infected intermediate hosts (especially the lowland paca
      Cuniculus paca) enables the disease to further transmit to humans, when
      their viscera are improperly handled.
    explanation: The Pan-Amazon sylvatic cycle centered in tropical forest biome
      links pacas to bush or domestic dogs and onward human exposure.
- name: Climate stability and hunting patterns driving spillover
  description: Temperature stability and ENSO-linked hunting changes in
    Pan-Amazonia increase sylvatic circulation and human spillover risk.
  evidence:
  - reference: PMID:37549288
    supports: SUPPORT
    snippet: Our findings indicate that temperature stability promotes the
      sylvatic circulation of the disease... In a scenario where climate
      extremes are projected to intensify, climate change at regional level
      appears to be indirectly driving the spillover of E. vogeli.
    explanation: Modeling shows climate stability and extremes modulate sylvatic
      transmission and spillover risk.
treatments:
- name: Albendazole therapy
  description: Benzimidazole antiparasitic used for smaller or inoperable
    hydatid cysts and as adjunct around surgery.
  treatment_term:
    preferred_term: pharmacotherapy
    term:
      id: MAXO:0000058
      label: pharmacotherapy
  evidence:
  - reference: PMID:34034501
    supports: SUPPORT
    snippet: Treatment with albendazole, whether combined or not with
      praziquantel, is useful for smaller, uncomplicated cysts (< 5 cm).
    explanation: Review guidance highlights albendazole as standard medical
      therapy for hydatid cysts.
- name: Radical hepatic resection
  description: Surgical removal of involved hepatic segments for large
    polycystic echinococcosis lesions or when malignancy is suspected.
  treatment_term:
    preferred_term: surgical procedure
    term:
      id: MAXO:0000004
      label: surgical procedure
  evidence:
  - reference: PMID:33553405
    supports: SUPPORT
    snippet: According to Romic classification, the case belongs to type IIb,
      and radical left hemi-hepatectomy was performed.
    explanation: The case demonstrates radical liver resection as operative
      management for polycystic echinococcosis.
datasets: []
differential_diagnoses:
- name: Cystic echinococcosis
  description: Unilocular hydatid disease caused by E. granulosus that presents
    with solitary liver cysts rather than multilocular lesions.
  distinguishing_features:
  - Unilocular cyst architecture and typical imaging classification distinguish
    it from multilocular polycystic lesions.
  disease_term:
    preferred_term: cystic echinococcosis
    term:
      id: MONDO:0018408
      label: cystic echinococcosis
  evidence:
  - reference: PMID:39099980
    supports: SUPPORT
    snippet: The review highlights the challenges associated with diagnosing the
      different echinococcosis types, including cystic echinococcosis, alveolar
      echinococcosis, and polycystic echinococcosis.
    explanation: Differential diagnosis among echinococcosis subtypes is
      emphasized as a clinical challenge.
- name: Alveolar echinococcosis
  description: Invasive hepatic lesions from E. multilocularis that mimic tumors
    and require distinction from polycystic hydatid disease.
  distinguishing_features:
  - Microvesicular infiltrative pattern and often absent daughter cysts contrast
    with the larger multilocular cysts of polycystic echinococcosis.
  disease_term:
    preferred_term: alveolar echinococcosis
    term:
      id: MONDO:0017282
      label: alveolar echinococcosis
  evidence:
  - reference: PMID:39099980
    supports: SUPPORT
    snippet: The review highlights the challenges associated with diagnosing the
      different echinococcosis types, including cystic echinococcosis, alveolar
      echinococcosis, and polycystic echinococcosis.
    explanation: The review notes diagnostic overlap among echinococcosis forms,
      supporting the need for differentiation.
- name: Hepatocellular carcinoma
  description: Primary liver malignancy that can coexist with or mimic hydatid
    lesions, requiring imaging and pathology to distinguish.
  distinguishing_features:
  - Arterial enhancement and washout patterns and malignant cytology separate
    HCC from parasitic cyst architecture.
  disease_term:
    preferred_term: hepatocellular carcinoma
    term:
      id: MONDO:0007256
      label: hepatocellular carcinoma
  evidence:
  - reference: PMID:33553405
    supports: SUPPORT
    snippet: After a multidisciplinary team discussion, the diagnosis of
      co-occurrence of hepatic CE and HCC was made... radical left
      hemi-hepatectomy was performed.
    explanation: The case report shows that hydatid lesions can coexist with
      HCC, making HCC an important differential consideration.
references:
- reference: DOI:10.1128/cmr.00050-07
  title: New Aspects of Neotropical Polycystic ( <i>Echinococcus vogeli</i> )
    and Unicystic ( <i>Echinococcus oligarthrus</i> ) Echinococcosis
  findings: []
- reference: DOI:10.1128/cmr.00075-18
  title: 'Echinococcosis: Advances in the 21st Century'
  findings: []
- reference: DOI:10.1590/s1678-9946202567069
  title: Pathological characterization of hepatic and mesenteric neotropical
    Echinococcosis in Brazilian Amazonian patients using light and scanning
    electron microscopy
  findings: []