Pleuro-pulmonary blastoma

Classification according to ICD-10
C34.9	Malignant neoplasm of the bronchi and lungs, unspecified
ICD-10 online (WHO version 2019)

The pleuro-pulmonary blastoma (PPB) is a rare tumor of the lung ( lung tumor ) or pleura with onset in early childhood.

Synonyms are: English cystic mesenchymal hamartoma; mesenchymal cystic hamartoma; pediatric pulmonary blastoma; pneumoblastoma; pulmonary rhabdomyosarcoma; rhabdomyosarcoma in lung cyst; CPAM / CCAM Type 4

The name was coined by the first authors of the first description from 1988.

distribution

The frequency is not known. 90% of blastomas occur in the first two years of life.

root cause

The disease are about two-thirds of mutations in DICER1 - gene on chromosome 14 locus q32.13 basis.

classification

The following classification is common:

• Type I PPB cystic, frequency 14%, occurrence prenatally or in the first 10 to 24 months of life, comparatively favorable prognosis with a cure probability of 89%, possibility of relapse
  • Type Ir (regressive), microscopic without detectable malignant cells, possible in every age group
• Type II PPB cystic-solid, frequency 48%, occurrence on average at 34 months
• Type III PPB solid, frequency 38%, occurrence on average after 44 months

Healing probability for types II and III about 50-70%

Clinical manifestations

Clinical criteria are:

• Occurrence in newborns and toddlers
• Shortness of breath ( Acute Respiratory Distress Syndrome )
• pneumonia

There is an association with cystic-adenomatoid malformation of the lung type IV. In 10%, a multilocular cystic nephroma can also occur.

Differential diagnosis

The following are to be distinguished:

Compared to type I.

• Bronchogenic cyst
• Cystic adenomatoid malformation of the lungs

Compared to types II and III

• Soft tissue sarcoma
• Primitive neuroectodermal tumor
• FLIT (fetal lung interstitial tumor)

therapy

The treatment consists of the surgical removal and, depending on the classification type, further measures, if necessary.

Individual evidence

1. ↑ ^{a b c} Pleuro-pulmonary blastoma. In: Orphanet (Rare Disease Database).
2. ↑ JC Manivel, JR Priest, J. Watterson, M. Steiner, WG Woods, MR Wick, LP Dehner: Pleuropulmonary blastoma. The so-called pulmonary blastoma of childhood. In: Cancer. Vol. 62, No. 8, October 1988, pp. 1516-1526, PMID 3048630 (Review).
3. ↑ ^{a b c d} Radiopaedia
4. ↑ Pleuropulmonary blastoma.  In: Online Mendelian Inheritance in Man . (English)
5. ↑ ^{a b c} Rare Diseases

Web links

• Genetics Home Reference

This article is about a health issue. It is not used for self-diagnosis and does not replace a diagnosis by a doctor. Please note the information on health issues !