Akademik Veri Yönetim Sistemi
36th European Congress of Pathology , Florence, İtalya, 7 - 15 Eylül 2024, sa.15028, ss.358
Background & objectives: Fibrous hamartoma of infancy (FHI) is a
benign rare neoplasm affecting infants. FHI is characterized by triphasic morphology consisting of fibroblastic fascicles, nodules of primitive cells and mature adipose tissue. In this case, we report FHI in a
9-months-old girl.
Methods: A lumbosacral mass specimen, measuring in total of 7x4x3
cm, on cut surface with a tan-yellow color consisting of haemorrhagic,
fibrotic and lipomatous areas was sampled. Paraffin-embedded tissue
sections of this specimen were evaluated for the histopathological characteristics. Demographic, clinical and imaging data were collected.
Results: A 9-months-old girl admitted to the hospital with complaint
of a congenital lumbosacral mass which was initially thought to bemultiple Mongolian blue spots. Mother described the mass as it had
been growing as the baby grew, but the growth rate has increased in the
last few months. Her examination showed a moveable, painless mass
with bluish color at the lumbosacral region. Magnetic resonance imaging revealed a subcutaneous mass with 5,5 cm in greatest width showing contrast enhancement. Histopathological examination revealed a
characteristic organoid triphasic morphology with bland fibroblastic
fascicles, mature adipose tissue and primitive looking oval-to-stellate
shape cell nodules with myxoid stroma. Diagnosis of FHI was made.
Conclusion: FHI is a rare benign soft tissue neoplasm affecting mostly
infants under 2 years of age. Rapidly growing nature and difficulties
in making diagnosis may lead to misdiagnosis of this entity. While
excision is curative, it is known that local recurrences could be encountered. Also, recent studies have shown that FHI cases often harbor
EGFR exon 20 insertion/duplication mutations. Therefore, FHI should
be considered in the differential diagnosis of a rapidly growing soft
tissue mass in an infant.