Home » Courses » Paediatric Cytology (Recently updated) » VI - LIVER » Embryonal sarcoma

Embryonal sarcoma

Clinical features

Usually occurs between 6-10 years of age
Presentation: abdominal mass, fever and pain
Serum alpha-fetoprotein levels are normal

Fig 80 - Embryonal Sarcoma- Cellular smear with mesenchymal clusters in a myxoid matrix (Giemsa)

Fig 81 - Embryonal Sarcoma- Heterogeneous population of neoplastic cells showing atypical cells with bizarre nuclei and lobulated contours. On the bottom a fragment of myxoid tissue (Giemsa)

Fig 82 - Embryonal Sarcoma- stripped bizarre nuclei and necrotic material (H&E)

Cellular smears
Mesenchymal clusters
Heterogeneous population of tumour cells (spindle, oval, pleomorphic and multinucleated giant cells)
Moderate amount of cytoplasm with poorly defined limits
Nuclei with coarse chromatin
Myxoid background
Hyaline globules in the background or intracytoplasmic
Haemorrhage and necrosis

Immunocytochemistry

Anti-chymotrypsin: positive
Alpha-1-antitrypsin: positive
Desmin: variable
Muscle-specific actin: variable
Vimentin: variable
Alpha-fetoprotein: negative (hyaline globules)
Myogenin: negative

Genetic studies

Rearrangements of chromosomal band 19q13.4,

t (11; 19) (q13; q13.4)

Differential diagnosis

Mesenchymal hamartoma
- In patients under one year of age
- Bland tumour cells
- No cellular pleomorphism
Embryonal rhabdomyosarcoma
- No hyaline globules
- Rhabdomyoblastic differentiation
- Myogenin : positive
Hepatoblastoma (mixed and embryonal)
- Lack of myxoid stroma
- Lack of cellular pleomorphism
- Lack of hyaline globules
- Presence of extra medullary haematopoiesis

Main points

Occasionally arising in mesenchymal hamartoma,
Primitive mesenchymal neoplasm
Treatment: complete surgical resection
Death within two years due to direct extension
Metastasis to lung and bone