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At least 379 records · Page 21Linked to original sources

PubMed1998

Benign symmetric lipomatosis Launois-Bensaude successfully treated with extensive plastic surgery.

Benign symmetric lipomatosis is a rare condition predominantly seen in male patients. The etiology is unknown, and therapy is difficult, although liposuction has had effect. We describe a 38-year-old male patient who had suffered from severe adipositas since the age of 7. Due to an ulceration in the groin, he later needed intensive care, and in order to normalize his condition, extensive surgical treatment became necessary.

Adult↗

PubMed1992

An unusual cause of carotid sinus syndrome: multiple symmetric lipomatosis.

A 45-year-old man with multiple symmetric lipomatosis suffered recurrent syncope attributed to carotid sinus syndrome caused by extrinsic compression of the carotid body by the lipomatous masses. Surgical removal reduced but did not stop syncope, which was then controlled by implantation of a DDD pacemaker.

Carotid Sinus↗

PubMed1995

Encephalocraniocutaneous lipomatosis with a mutation in the NF1 gene.

Encephalocraniocutaneous lipomatosis (ECCL) is a congenital hamartomatous disorder characterised by unilateral skin lesions, lipomas, and ipsilateral ophthamological and cerebral malformations. The disorder is thought to represent a localised form of Proteus syndrome. In this report, a child is described with ECCL and a de novo nonsense mutation in exon 29 (S1745X) of the neurofibromatosis type 1 (NF1) gene. Although it is possible that both ECCL and NF1 occur coincidentally in this patient, we favour the hypothesis that in exceptional cases a mutation in the NF1 gene might give rise to severe congenital malformations such as ECCL. Possible pathogenetic mechanisms for these malformations are discussed.

Base Sequence↗

PubMed2002

Intramuscular lipomatosis of tongue.

A rare case of intramuscular lipomatosis of the tongue with intramuscular invasion in a 42 year old man is presented. The literature is reviewed and the clinical features, pathology, and treatment are discussed briefly.

Adult↗

PubMed1978

Mediastinal lipomatosis. CT confirmation of a normal variant.

Mediastinal lipomatosis is a common benign cause of mediastinal widening. While it may be associated with exogenous obesity, steroid ingestion, or Cushing's syndrome, these factors often are not present. Evaluation of the mediastinum by CT should be the initial diagnostic study in the work-up of patients with abnormalities of the mediastinal contour; indeed, in many cases it can eliminate the need for other procedures.

Adult↗

PubMed1982

Computed tomography of deep fat masses in multiple symmetrical lipomatosis.

Deep fat masses were evaluated by computed tomography (CT) in 15 patients with multiple symmetrical lipomatosis. In 4 patients, peritracheal accumulations of fat were observed. In 3 of them, tracheal compression by lipomatous tissue was demonstrated: 2 were asymptomatic and the third had severe respiratory insufficiency secondary to blockage of the airway by the vocal cords as the result of recurrent nerve palsy. In 6 patients, lipomatous tissue occupied the potential space between the spinal scapulae and the trapezius, supraspinatus, and infraspinatus muscles. In 2, calcification of lipomatous masses was observed. There was no relationship between extension of subcutaneous fat and accumulation at deep sites. CT facilitates early detection of peritracheal lipomatous tissue and is helpful in follow-up when deep fat accumulation is responsible for space-occupying lesions requiring surgery.

Adult↗

PubMed2002

Pelvic lipomatosis in a child.

We report a 10-year-old boy with pelvic lipomatosis causing chronic urinary retention. CT scan features, therapy and review of the literature are discussed.

Biopsy, Needle↗

PubMed1984

Encephalocraniocutaneous lipomatosis: a recently described neurocutaneous syndrome.

The authors report a 1-year-old Japanese boy, who was noted to have unilateral alopecia and a cloudy cornea since birth. Additional features included mild spasticity and mental retardation, DQ being 70. A non-communicating cystic lesion was found in the right middle cerebral fossa by metrizamide CT scan, and resection of the lesion by craniotomy revealed an arachnoid cyst. The case was diagnosed as encephalocraniocutaneous lipomatosis, a recently described neurocutaneous syndrome.

Arachnoid↗

PubMedDate not supplied

Multiple symmetrical lipomatosis: no longer just a Mediterranean disease?

Multiple symmetrical lipomatosis is a rare condition, of which the etiology remains unclear. Most reported cases have been from the Mediterranean countries, and it is generally thought of as a disorder characteristic of that region. However, there have been 11 cases reported in Japan since 1978, suggesting that this condition is no longer confined to Mediterranean countries.

Adult↗

PubMed1991

Pelvic lipomatosis in a female: diagnosis and initial therapy.

The extremely rare case of pelvic lipomatosis in a women is reported (4 cases in the literature). The patient suffered from symptoms of irritable bladder. Treatment included hysterectomy and extension of the bladder resulting in a significant reduction of frequency. Prognostic criteria of the progressive benign disease and therapeutic modalities are discussed.

Adult↗

PubMed1987

Renal hilus lipomatosis simulating carcinoma of the kidney.

We report a case of lipomatosis of the renal hilus which presented with macroscopic painless haematuria. The appearance of the pyelocalyceal system on the intravenous urogram set the diagnosis of renal carcinoma, a diagnosis that both ultrasound and computed tomography did not refute. Final diagnosis was established histologically.

Aged↗

PubMed1979

Pelvic lipomatosis: a nonsurgical diagnosis.

Pelvic lipomatosis is rare in Europe. Preoperative diagnosis is seldom reached and subsequent explorative procedures are necessary. Typical roentgenographic findings are presented. The possibility of an early presurgical diagnosis is emphasized.

Adult↗

PubMed1977

Multiple symmetric lipomatosis: a defect in adrenergic-stimulated lipolysis.

The cellularity of normal and lipomatous adipose tissue and its response to different lipolytic agents have been studied in a group of 10 patients with multiple symmetric lipomatosis (MSL). In MSL patients, fat cells from lipomatous tissue are smaller than normal, uninvolved adipocytes. Fat cells from lipomata show minimal variations in size following conspicuous increase of lipomatous masses. These findings suggest that the growth of lipomata can be attributed to the neoformation of adipocytes rather than to an enlargement in the single fat cells. The incidence of reduced glucose tolerance and of hyperlipoproteinemia is similar in MSL patients and in controls. A significant reduction in plasma free fatty acids was observed in MSL patients after a 24-h fast as well as after noradrenaline infusion. A specific insensitivity of lipomatous tissue to the lipolytic effect of noradrenaline and isoprenaline was observed in vitro, as indicated by glycerol release in the medium, whereas response to theophylline and to dibutyryl cyclic AMP was retained. The lipolytic response to catecholamines was retained. The lipolytic response to catecholamines was normal in the nonlipomatous adipose tissue of MSL patients. In basal conditions ATP concentrations were similar in normal and in lipomatous adipose tissue. However, incubation with noradrenaline induced a significant fall in intracellular ATP levels in normal tissue, whereas no variations were observed in lipomatous tissue. Theophylline, instead, induced a prompt and significant decrease in intracellular ATP levels in lipomatous tissue. These observations indicate that the block in catecholamine-stimulated lipolysis in lipomatous tissue of MSL patients can be localized at a level preceding the formation of cyclic AMP.

Adenosine Triphosphate↗

PubMedDate not supplied

Computed tomography of pelvic lipomatosis. Report of a case.

Pelvic lipomatosis is a non-malignant condition of unknown etiology characterized by an overgrowth of non-encapsulated fatty tissue in the perirectal and perivesical spaces of the pelvis. The symptoms are generally vague and the condition is often diagnosed accidentally. It may cause obstruction of the ureters, inferior vena cava and pelvic veins and may be associated with cystitis glandularis. The typical conventional radiographic findings, though not pathognomonic, are a high-positioned and pear-shaped bladder, tubular narrowing of the rectum and distal sigmoid colon and reduced attenuation of the pelvic soft tissues. Computed tomography demonstrates a non-encapsulated fatty mass surrounding the pelvic organs symmetrically and with an attenuation similar to that of subcutaneous fat. The fatty tissue may contain strands with a higher attenuation than that of fat. The findings at computed tomography seem to be pathognomonic for this condition and eliminate the need for routine surgical biopsies.

Adult↗

PubMed2003

Encephalocraniocutaneous lipomatosis: neurologic manifestations.

We report a new case of encephalocraniocutaneous lipomatosis, a rare neurocutaneous syndrome of unknown etiology with involvement of tissues arising from the mesoderm and ectoderm: skin, eye, adipose tissue, and brain. We also review the neurologic manifestations of the syndrome, the most frequent of which include seizures, ventricular enlargement, calcifications, mental retardation, and cerebellopontine angle tumor. Our patient had an extensive extradural spinal cord lipomatous lesion, emphasizing the importance of screening for spinal abnormalities in asymptomatic patients with this condition.

Brain↗

PubMed1987

Encephalocraniocutaneous lipomatosis.

Encephalocraniocutaneous lipomatosis is a distinct clinical syndrome characterized by unilateral cerebral malformations and ipsilateral scalp, face, and eye lesions. The cutaneous lesions are confined to the head. The central nervous system lesions consist in part of cerebral hemiatrophy, porencephaly, and defective opercularization of the insula. The intracranial lesions and clinical symptomatology are progressive, and appear, in part, to have a vascular pathogenesis. This suggests that a primary defect in the formation of tissues derived from embryonic mesenchyme may be responsible for the syndrome and that tissues derived from ectoderm may be secondarily affected.

Adolescent↗

PubMed1979

Multiple symmetric lipomatosis (Launois-Bensaude syndrome) - adipose tissue insensitivity to cyclic AMP.

Adipocytes prepared from lipomatous tissue of a patient with multiple symmetric lipomatosis have been incubated in the presence of norepinephrine, or insulin, or both. No lipolytic response to norepinephrine could be detected. However, the cellular content of cyclic AMP doubled in response to norepinephrine. Insulin reversed this. The data suggest that fat mobilization from the lipoma is impaired, and that this is due to resistance to the effect of cyclic AMP on hormone sensitive lipase.

Adipose Tissue↗

PubMed2001

Adipose tissue metabolism in benign symmetric lipomatosis.

Type 2 benign symmetric lipomatosis (BSL) is characterized by abnormal growth of adipose tissue in the upper back, deltoid region, upper arms, hips, and upper thigh region. Studies of lipomatous tissue in vitro have suggested that defective lipolysis may account for excess fat accumulation; however, in vivo adipose tissue metabolism has not been evaluated. We measured systemic adipose tissue lipolysis and regional adipose tissue fatty acid uptake in a patient with type 2 BSL scheduled for elective brachioplasty. We found increased, rather than decreased, rates of systemic free fatty acid release coupled with normal fatty acid oxidation. The uptake of fatty acids was 19% greater in deltoid region lipomatous tissue than in abdominal sc fat, whereas in control studies the relative uptake of fatty acids in deltoid fat averaged 29% less than that in abdominal fat. Adipocyte size was smaller than expected in lipomatous tissue. These results suggest that type 2 BSL is a hyperplastic adipose tissue abnormality that does not impair systemic lipolysis. The pathophysiology appears similar to what has been termed hyperplastic obesity. A better understanding of this condition could lead to insights into the mechanisms of hyperplastic obesity.

Adipose Tissue↗