Infantile lipofibromatosis of the upper limb

Abstract

The imaging features of extensive lipofibromatosis presenting in a 1-day-old female infant are reported. This lesion involved her entire right upper limb, extending from the axilla to the palm of the hand. Radiographs showed marked deformity and thinning of all the right upper-limb bones due to pressure effect of soft-tissue enlargement, especially affecting the distal humerus and proximal forearm bones. Magnetic resonance imaging showed a huge soft-tissue mass infiltrating most of the muscles of the entire upper limb, with bony erosion. The mass was largely T1-isointense, moderately T2-hyperintense and showed marked enhancement. There were intra-lesional signal changes consistent with fatty elements. A lesion debulking procedure was performed and the histology was that of lipofibromatosis. The limb was found to be non-viable after the procedure and a subsequent above-elbow amputation was performed. Although the resection margins were not clear, she had no further recurrence over a subsequent 3-year follow-up period.

Soft Tissue Ankle Mass in a 15-month-old Girl

“…It is described as occurring exclusively in children from infancy to early second decade [7]. MRI features of this lesion include high signal on T1 and T2 imaging that shows loss of signal with fat suppression, indicating that the lesion contains fat, which is consistent with this case [1,2,11,12]. Contrast enhancement on MRI has been described as heterogenous, primarily in the internal septations.…”

“…Contrast enhancement on MRI has been described as heterogenous, primarily in the internal septations. The lesion did not contain nonfatty nodular areas, as can be seen with a liposarcoma [1,11].…”

Pigmented lipofibromatosis in unusual location: case report and review of the literature

“…Lipofibromatosis is a rare, recently reported, fibrofatty pediatric tumor, which affects mainly extremities [1,6]. Histopathological features of this tumor must be distinguished from fibrous hamartoma of child, fibromatosis, calcifying aponevrotic fibroma, and lipoblastoma [1].…”

“…Lipofibromatosis is different and must be equally distinguished from the entity designated as "lipofibromatosis" observed in cases of congenital macrodactyly and local gigantism [1]. The tumor commonly occurs in extremities, rarely in the hands, arms, legs, and feet [1,6]. Rarely, chest, abdomen, or head and neck are affected [1][2][3][4][5].…”

“…Macroscopically, it is yellowish or tan-whitish appearance with a prominent fatty component. Very rare tumor have exceeded 5 cm [1,[3][4][5][6]. Histologically, the tumor is poorly marginated, often composed of more than 70% of adipose tissue.…”