Presenter Status

Resident/Psychology Intern

Abstract Type

Case report

Primary Mentor

Heather Von Bevern, MD

Start Date

2-5-2022 11:30 AM

End Date

2-5-2022 1:30 PM

Presentation Type

Poster Presentation

Description

Case report abstract: Fibrous hamartoma of infancy (FHI) is a rare, benign lesion characterized as a tumor of myofibroblastic origin that has characteristic features of triphasic histology. FHI was first described in 1956 by Reye and formally named by Enzinger in 1965 (1, 2). The lesion is defined as a hamartoma due to the histologic presentation of disorganized mesenchymal, fibrous, and adipose tissue with absence of mitotic figures; this combination of derived tissue without evidence of anaplasia is diagnostic for FHI. These lesions typically arise as a single, solitary mass, are most commonly located on the extremities, trunk, sacrum, or scrotum and are typically 0.5 to 9.0 centimeters in size (3, 4). Only roughly 200 cases have been reported in the literature (3). The majority of cases occur in young children; 91% of cases arise within the first year of life (4). Males are more often affected in a ratio of 2.4:1 (4). Roughly 20% of cases have been documented as congenital (3). Treatment is surgical excision, which is often curative; local recurrence is rare and incidence decreased by obtaining negative margins (8). We present a case of congenital FHI identified at birth.

Conclusion: FHI is a benign, soft tissue lesion presenting in early infancy and even the neonatal period that carries diagnostic histopathology and supportive MR imaging and immunostaining that can differentiate this tumor from other pediatric soft tissue masses. However, due to its rarity, it is important to recognize its characteristic diagnostic criteria and ensure its place on the differential diagnosis because it is largely benign and does not warrant aggressive therapy.

MeSH Keywords

hamartoma, fibrous hamartoma, neonatal musculoskeletal lesion, benign neonatal lesion, neonatal mass

Additional Files

A Case of Fibrous Hamartoma in Term Neonate.pdf (132 kB)
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May 2nd, 11:30 AM May 2nd, 1:30 PM

A Case of Fibrous Hamartoma in Term Neonate

Case report abstract: Fibrous hamartoma of infancy (FHI) is a rare, benign lesion characterized as a tumor of myofibroblastic origin that has characteristic features of triphasic histology. FHI was first described in 1956 by Reye and formally named by Enzinger in 1965 (1, 2). The lesion is defined as a hamartoma due to the histologic presentation of disorganized mesenchymal, fibrous, and adipose tissue with absence of mitotic figures; this combination of derived tissue without evidence of anaplasia is diagnostic for FHI. These lesions typically arise as a single, solitary mass, are most commonly located on the extremities, trunk, sacrum, or scrotum and are typically 0.5 to 9.0 centimeters in size (3, 4). Only roughly 200 cases have been reported in the literature (3). The majority of cases occur in young children; 91% of cases arise within the first year of life (4). Males are more often affected in a ratio of 2.4:1 (4). Roughly 20% of cases have been documented as congenital (3). Treatment is surgical excision, which is often curative; local recurrence is rare and incidence decreased by obtaining negative margins (8). We present a case of congenital FHI identified at birth.

Conclusion: FHI is a benign, soft tissue lesion presenting in early infancy and even the neonatal period that carries diagnostic histopathology and supportive MR imaging and immunostaining that can differentiate this tumor from other pediatric soft tissue masses. However, due to its rarity, it is important to recognize its characteristic diagnostic criteria and ensure its place on the differential diagnosis because it is largely benign and does not warrant aggressive therapy.

 

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