Benign symmetrical lipomatosis (BSL), or Madelung's disease, is a rare disease characterized by he progressive growth of diffuse, painless, non-enveloped symmetric lipomas at typical sites in the body. The etiology of this disease remains unknown. A disturbance of lipid metabolism is involved, and there is a proven connection with chronic alcoholism. Problems with the differential diagnosis of the characteristic clinical picture arise from the fact that this disease is not widely recognized. Surgical lipectomy in several sessions can relieve pressure and provide satisfactory patient functional recovery as well as cosmetic disfiguration.
Alcoholism ; complications ; Diagnosis, Differential ; Humans ; Lipectomy ; Lipomatosis, Multiple Symmetrical ; complications ; diagnosis ; surgery

We report a case of thymic hyperplasia accompanied by pericardial lipomatosis and right facial hemihypertrophy in an 8-year-old boy. On imaging studies, the hyperplastic thymus had prominent curvilinear and nodular fatty areas simulating a fat-containing anterior mediastinal mass, which is an unusual finding in children. To our knowledge, this is the first report on a child with a combination of thymic hyperplasia, pericardial lipomatosis, and right facial hemihypertrophy. The radiologic findings are presented with a brief discussion.
Child ; Diagnosis, Differential ; Facial Asymmetry/complications/*diagnosis ; Heart Diseases/complications/*diagnosis/surgery ; Humans ; Hypertrophy/pathology ; Lipomatosis/complications/*diagnosis/surgery ; Magnetic Resonance Imaging ; Male ; Pericardium/*pathology ; Thymus Hyperplasia/complications/*diagnosis/surgery ; Tomography, X-Ray Computed

OBJECTIVE: To introduce recent progress on diagnosis and treatment of benign symmetric lipomatosis (BSL). METHOD: Detailed clinical data of 6 patients with BSL were reviewed and analyzed. We present a summary of the clinical symptoms, physical sign, diagnosis and therapeutic methods of BSL. And related literatures were discussed together. RESULT: All of 6 patients have excessive subcutaneous fat deposit predominantly around neck. One patients had upper extremity localizations. Six patients had the complication of left ventricular diastolic function changes, glucose intolerance or diabetes mellitus, chronic hepatopathy, hyperuricemia and sleep apnea syndrome in one or more. One patients with several symptoms occur simultaneously, another one female patient was accompanied by all symptoms but chronic hepatopathy. Five male patient were alcohol abusers. Total neck lipectomy and abstinence from alcohol were performed on 5 patients. One patient refused treatment. During a follow-up of 3 months to 4 years, one patients was relapsed again, and no recurrence was seen in another 4 patients. All patho logical results were nonencapsulated fat. CONCLUSION BSL is a lipodystrophy caused by diffuse fatty tissue, symmetry deposition in the neck and shoulder subcutaneous fascia space or deep fascial space. The highest incidence favors to middle-aged man who is alcoholic. Chronic alcohol addiction and typical clinical symptoms help to diagnosis BSL. Lipectomy represents a successful procedure in treating BSL.
Adult ; Alcoholism ; complications ; Fascia ; pathology ; Female ; Humans ; Lipectomy ; Lipomatosis, Multiple Symmetrical ; complications ; diagnosis ; surgery ; Male ; Middle Aged ; Neck ; pathology ; Shoulder ; pathology

OBJECTIVETo study the clinicopathologic features of lipomatosis of nerve (NLS).

METHODSThe clinical, radiologic and pathologic features were analyzed in 15 cases of NLS.

RESULTSThere were a total of 10 males and 5 females. The age of patients ranged from 4 to 42 years (mean age = 22.4 years). Eleven cases were located in the upper limbs and 4 cases in the lower limbs. The median nerve was the most common involved nerve. The patients typically presented before 30 years of age (often at birth or in early childhood) with a soft and slowly enlarging mass in the limb, with or without accompanying motor and sensory deficits. Some cases also had macrodactyly and carpal tunnel syndrome. MRI showed the presence of fatty tissue between nerve fascicles, resembling coaxial cable in axial plane and assuming a spaghetti-like appearance in coronal plane. On gross examination, the affected nerve was markedly increased in length and diameter. It consisted of a diffusely enlarged greyish-yellow lobulated fusiform beaded mass within the epineural sheath. Histologically, the epineurium was infiltrated by fibrofatty tissue which separated, surrounded and compressed the usually normal-appearing nerve fascicles, resulting in perineural septation of nerve fascicles and microfascicle formation. The infiltration sometimes resulted in concentric arrangement of perineural cells and pseudo-onion bulb-like hypertrophic changes. The perineurial cells might proliferate, with thickening of collagen fibers, degeneration and atrophic changes of nerve bundles. Immunohistochemical study showed that the nerve fibers expressed S-100 protein, neurofilament and CD56 (weak). The endothelial cells and dendritic fibers were highlighted by CD34. The intravascular smooth muscle cells were positive for muscle-specific actin.

CONCLUSIONSNLS is a rare benign soft tissue tumor of peripheral nerve. The MRI findings are characteristic. A definitive diagnosis can be made with histologic examination of tissue biopsy.

Adolescent ; Adult ; Antigens, CD34 ; metabolism ; CD56 Antigen ; metabolism ; Carpal Tunnel Syndrome ; complications ; pathology ; Child ; Child, Preschool ; Diagnosis, Differential ; Extremities ; innervation ; Female ; Hand Deformities, Congenital ; complications ; pathology ; Humans ; Lipoma ; pathology ; Lipomatosis ; complications ; diagnosis ; metabolism ; pathology ; surgery ; Magnetic Resonance Imaging ; Male ; Median Nerve ; metabolism ; pathology ; Nerve Sheath Neoplasms ; pathology ; Neurofibroma ; pathology ; Neurofilament Proteins ; metabolism ; Neuroma ; pathology ; Peripheral Nervous System Diseases ; complications ; diagnosis ; metabolism ; pathology ; surgery ; Peripheral Nervous System Neoplasms ; pathology ; Retrospective Studies ; S100 Proteins ; metabolism ; Vimentin ; metabolism ; Young Adult