Onco Wiki / Disease

Pleuropulmonary blastoma (PPB)

Deterministic view of the source YAML entity. Clinical authority remains with the cited source IDs and reviewer sign-off state.

IDDIS-PLEUROPULMONARY-BLASTOMA
TypeDisease
Aliases
DICER1-associated pleuropulmonary blastomaPPBPPB Type I (cystic)PPB Type II (mixed cystic/solid)PPB Type III (solid)Pleuropulmonary blastomaПлевропульмональна бластома (PPB)
Statusreviewed 2026-05-20 | pending_clinical_signoff
DiseasesNone declared
SourcesSRC-COG SRC-NCCN-PEDIATRIC-SARCOMA

Informational support. All recommendations must be verified by a qualified oncologist. Engineering checks are not clinical validation or treatment approval.

Source YAML

icd_o_3_morphology8973/3
icd_o_3_topography["C34"]
icd_10C34
who_classificationWHO Thoracic Tumours 5th ed (2021): Pleuropulmonary blastoma — pediatric embryonal lung-pleural tumour with three morphologic types (I, II, III) representing a continuum of progression from cystic (Type I) → mixed (Type II) → solid (Type III)

Capabilities

Notes

Pleuropulmonary blastoma is the canonical DICER1-syndrome cancer — PPB diagnosis in any child should trigger DICER1 germline testing AND cascade-testing of parents + siblings. The Type I → Type II → Type III progression model is unique among pediatric cancers in offering a true surveillance-curable precursor lesion (Type I PPB is histologically benign-appearing cystic disease that, if completely resected, has excellent prognosis; if left in situ, may progress to the higher-mortality Type II/III). Treatment by type: Type I — complete surgical resection; close follow-up imaging for new cysts or solid transformation. Type II/III — surgical resection (often combined lobectomy / pneumonectomy with CNS prophylaxis consideration); IVADo (ifosfamide, vincristine, actinomycin-D, doxorubicin) or similar sarcoma-style regimens; CNS-directed therapy for Type III given high CNS-relapse risk. Distinguishing PPB Type I from congenital pulmonary airway malformation (CPAM) — the most common pediatric cystic lung lesion — is the major early-life diagnostic challenge: family history of DICER1-related malignancies + multi-locular or septated cysts argue for PPB over CPAM and should prompt referral to...

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Disease

Sources