A rare case of calcaneoplanovalgus foot deformity in a toddler with aggressive lipofibromatosis

Abstract

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Maxillary Lipofibromatosis in a pediatric patient. Case report.

“…Patients have ranged in age from newborns to teenagers (1,7,9) . These tumors have a male-to-female predominance of a 2.7:1 ratio (3,4) , but other studies have reported no gender predilection (9) . Even though numerous theories have been proposed, the etiology is still uncertain (7,10) .…”

“…In this case, initial imaging includes MRI, which is better suited to distinguish soft tissue, obtains a better cleavage plane image, and is considered the imaging modality of choice (2,4,9,11) . It helps evaluate the tumor extension and characterization with hyperintense signaling that isointense to fat on T1-and T2-weighted imaging (1,3,4,8) ; even though this cannot definitively diagnose LPF, it can exclude other diagnoses in preoperative planning (4) .…”

“…Immunohistochemistry is not required to establish the diagnosis as it is variable and non-specific (1,2,3,4,5) . The spindle cell element of the tumor may exhibit immunoreactivity for CD99, CD34, alfa smooth muscle actin (SMA), BCL-2, S-100, muscle-specific actin (MSA), and rarely epithelial membrane antigen (EMA) (1,3,5,8) . Stains for desmin and keratin are usually negative (1,5,8) .…”

“…Lipofibromatosis (LPF) is an uncommon benign soft tissue tumor of childhood first described by Fetsch et al in 2000 and was previously named infantile/juvenile fibromatosis (1,3) . In 2002 LF was included in the World Health Organization (WHO) under the fibroblastic/myofibroblastic tumors and afterward reclassified based on the biological behavior as an intermediate-locally aggressive lesion1.…”

“…Therefore, the primary treatment is complete surgical resection, and only in cases where the lesion is diffusely infiltrating could partial resection be considered to reduce the patient's postoperative morbidity (4) . Fortunately, there is no metastatic potential (3) .…”