Proteus syndrome presenting with hemihypertrophy consisting of lipomatous
tumor is similar to macrodystrophia lipomatosa.
This case shows a predominantly lipomatous
tumor component, with morphologic features supporting pleomorphic liposarcoma, high grade.
Enzi et al(13) reported an insensitivity to the lipolytic effect of norepinephrine on the lipomatous
tissue, suggesting a biochemical defect in adrenergic-stimulated lipolysis.
Benign tumors and tumorlike conditions of the breast that may display an adipocytic component include mammary hamartoma, lipomatous
adenosis, myofibroblastoma, and lipoma variants.
Multiple variants of MFB have been described, including cellular, infiltrative, epithelioid, deciduoid-like, lipomatous
, collaginized/fibrous and myxoid.
In addition, severe muscle atrophy and lipomatous
changes were observed in both thighs.
Bonvalot et al., "Adjuvant radiotherapy for extremity and trunk wall atypical lipomatous
tumor/ well-differentiated LPS (ALT/WD-LPS): a French Sarcoma Group (GSF-GETO) Study," Annals of Oncology, vol.
Cytogenetic studies have demonstrated rearrangements of 11q13; however, amplification of MDM2 should prompt consideration of an atypical lipomatous
tumor/well differentiated liposarcoma.
When the AML component lacks significant lipomatous
areas, radiological features may not be typical, leading to misinterpretation as RCC alone, as occurred in our case.
These observations suggest that expression of DDIT3 protein could be a common phenotype determining factor for several types of lipomatous
Another theory known as "metaplasia theory" suggests that lipomatous
development occurs due to aberrant differentiation of in situ mesenchymal cells into lipoblast, since fatty tissue can be derived from mutable connective tissue cells almost anywhere in the body .
(2) The difference between lipohyperplasia and other types of parathyroid hyperplasia is that the increase in size is due to lipomatous
tissue and not other stromal components.
hypertrophy of the interatrial septum (LHAS), characterized with lipid deposition in the interatrial septum, is thought to be benign and rarely associated with clinical manifestations.
The diagnosis was a lipomatous
lesion and excision was advised for histopathological examination.
On cranial MRI, posterior and splenium of corpus callosum were not present; in addition there was 50x36 mm hyperdense lipomatous
mass at the anterior of corpus callosum on T1-weighted images.