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Liver tumors in neonates and very young infants: diagnostic pitfalls and therapeutic problems
D von Schweinitz1, S Glüer, H Mildenberger
1Department of Pediatric Surgery, Medical School Hannover.
Insights
Pediatric liver tumors like infantile hemangioendothelioma (inf HE) and hepatoblastoma (HB) in infants require accurate diagnosis. Some inf HEs show spontaneous regression, while others may relapse, highlighting the need for precise differentiation.
Area of Science:
- Pediatric Oncology
- Hepatology
- Neonatal Medicine
Background:
- Liver tumors are rare in infants under 3 months.
- Accurate diagnosis and differentiation of pediatric liver tumors are critical for appropriate management.
- Infantile hemangioendothelioma (inf HE) and hepatoblastoma (HB) are common types.
Purpose of the Study:
- To analyze the clinical presentation, diagnosis, and outcomes of various liver tumors in infants.
- To evaluate the reliability of diagnostic methods in differentiating pediatric liver tumors.
- To report on the natural history and treatment responses of infantile hemangioendothelioma.
Main Methods:
- Retrospective analysis of 26 infants with liver tumors treated since 1977.
- Review of clinical data, imaging (ultrasonography, CT, angiography), and histology.
- Correlation of tumor type with clinical features, complications, and treatment outcomes.
Main Results:
- Infantile hemangioendothelioma (17 cases) and hepatoblastoma (7 cases) were most common.
- Complications included polyhydramnios, preterm delivery, and congestive heart insufficiency.
- Diagnostic imaging and tumor markers were not always definitive; histological revision was sometimes necessary.
- Spontaneous regression observed in 7 non-resected infantile hemangioendotheliomas (Dehner type I).
Conclusions:
- Accurate differentiation between infantile hemangioendothelioma and hepatoblastoma is challenging but crucial.
- Some infantile hemangioendotheliomas exhibit spontaneous regression, suggesting a conservative approach may be warranted.
- Misdiagnosis can lead to inappropriate initial treatment, emphasizing the need for careful evaluation.
Abstract:
We report on 26 infants under 3 months of age with various liver tumors, who were treated in our hospital since 1977 and/or registered in the German Cooperative Pediatric Liver Tumor Study HB-89. 17 of these had an infantile hemangioendothelioma (inf HE), 7 a hepatoblastoma (HB), one a mesenchymal hamartoma (mes H) and one a neuroblastoma stage IV-S (Nbl). Polyhydramnios occurred in 5 cases of inf HE, in one associated with hydrops fetalis. This led to a preterm delivery of 4 infants. 7 infants with a large inf HE suffered from high output congestive heart insufficiency, 2 additionally from a Kasabach-Merritt-syndrome. Serum alpha-fetoprotein was within normal range of age in all infants except in one HB patient. Neuron-specific enolase was clearly elevated in the Nbl patient and slightly above normal range in one inf HE and 3 HB patients. Other tumor markers and platelet counts were not indicative of diagnosis. Also imaging techniques as ultrasonography, CT and angiography were not always reliable in differentiating the tumors. In 2 cases the initial histological diagnosis of an HB had to be corrected for an inf HE. In 6 children the tumors were initially treated under a wrong assumption: 4 tumors as an HB instead of an inf HE, one as an inf HE instead of a Nbl IV-S and one as a focal nodular hyperplasia of the liver instead of an HB. 7 not resected inf HEs (Dehner type I) showed spontaneous regression after 6 months to 3 years. One inf HE type II relapsed twice after resection.(ABSTRACT TRUNCATED AT 250 WORDS)