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Launois Bensaude disease is a widespread symmetrical lipomatosis with a head-neck-trunk predominance. Its pathogeny is unknown and its treatment, basically surgical, is aggressive, difficult and even exasperating as a result of the large amount of noticed relapses.
The results of clinical roentgenological investigations of 52 patients with cervical lipoma were analyzed. The authors describe the radiographic appearance and the investigation techniques for circumscript lipoma in the subcutaneous tissue, in the muscles and for a large diffuse symmetric lipomatosis. In the diagnostics of cervical lipoma x-ray investigations have a leading role.
A case of invagination caused by lipomatosis on a median loop of the small intestine is presented. Certain unusual features of the case--the rarity of the lesion and its location, the absence of symptoms before the occlusion occurred--are highlighted.
Described are rare thyroid lesions which either contain mature adipocytes or consist of lipid-rich follicle cells. Of the former, diffuse lipomatosis and adenolipoma of the thyroid are most likely to be derived from heterotopic islands of fat cells included in the gland during embryogenesis. On the other hand, metaplastic origin of fat cells from stromal fibroblasts due to chronic tissue hypoxia is presumed responsible for the presence of fatty tissue in amyloid goiters. Massive steatosis of follicle cells, as seen in the lipid-rich variant of clear cell thyroid adenomas and carcinomas, is, however, claimed to most likely result from metaplastic transformation of neoplastic thyrocytes. Pathomorphological and clinical features of the aforementioned lesions are discussed.
We have evaluated the relationships between adipose tissue lipoprotein-lipase activity (AT-LPL) or post-heparin plasma lipolytic activity (PHLA) and the composition of circulating lipoproteins in 7 patients with multiple symmetric lipomatosis (MSL). In addition we have investigated the behaviour of serum triglycerides after an oral fat load (1 g triglycerides/kg body weight) in 5 MSL patients and in 5 age and sex matched controls. We found significantly higher values of HDL, HDL2 and HDL3 cholesterol in MSL patients than in controls. Moreover, HDL2/HDL3 cholesterol ratio was higher in MSL patients than in controls, which indicate a predominant increase in HDL2 subfraction. The mean values of AT-LPL in lipomatous tissue was significantly higher than in control tissue. A positive statistically significant correlation was found between AT-LPL activity and HDL, HDL2 and HDL3 cholesterol values. After the fat load the maximal increments of serum triglyceride levels and the triglyceride areas over 8 and 12 h are lower in MSL patients than in controls; there was an inverse, statistically significant correlation between the total PHLA or the extrahepatic PHLA values and the triglyceride areas after lipid load. We conclude that MSL represents a useful naturally occurring model for the study of the role of AT-LPL in the metabolism of triglyceride-rich lipoproteins and in the clearance of lipoproteins in the post-prandial phase.
From a review of 2,000 autopsies of children, 16 cases of extensive necrosis and scarring fibrosis of the myocardium were found. These lesions involved mainly the left ventricle and spared the endocardium, the pericardium, and the coronary vessels. These necrotic of fibrotic heart lesions were found to be closely associated with various pancreatic diseases: cystic fibrosis (11 cases), pancreatic lipomatosis (2 cases), extensive small bowel resection (3 cases, 2 of which were associated with acute interstitial pancreatitis). To explain these unexpected associations, two hypotheses can be put forth: (1) The lack of absorption of some presently undetermined substances indispensable for the correct trophicity of the myocardium, and (2) the release in the blood of proteolytic enzymes with consecutive activation of phlogistic substances such as kinins.
This work is based on the separate study of a triple material: axial gross body sections from cadavers (4); normal and conventional radiographs with or without opacification; in vivo scans (210). Authors have selectionned thriteen scans patterns according to several regional levels: supra-aortic scans (n08 1, 2, 3); scan through the horizontal part of aortic arch (n0 4B); the scan of the aorto-pulmonary window (n0 4B); supra-cardiac scans through intra and extra-pericardic parts of great vessels: vena cava, aorta and pulmonary artery (n08 5, 6, 7); heart and cardiac cavities scans (n08 8, 9, 10, 11); scan through the retro-crural space (n0 12). Knowing these regional scan patterns, makes it possible to the radiologist to manage a topographic approach quite adapted to the technical mode of direct scannography through the area of interest, already tested by some constructors. The non pathologic variations are numerous. Mediastinal lipomatosis, dilated and tortuous great vessels in the older and atheromatic individual, constitutional variations of the vessels topography. Limits found out during this work concern heart examination and hilum study; great strides are to be achieved in these fields because of the unceasing technical improvments.
It is reported on a 42-year-old female patient with panniculitis nodularis non suppurativa (Weber-Christian's syndrome). Anamnesis and histological findings are discussed and the therapy is explained. In the discussion the finally still unknown etiology (allergic, autoimmunological) as well as the differential diagnosis (focal panniculitis, lipomatosis subcutanea, erythema nodosum and others) are explained.
Multiple symmetric lipomatosis has been proposed to be associated with impaired catecholamine-responsiveness of hypertrophic adipose tissue at the level of beta-adrenergic receptors or adenylate cyclase respectively. We have studied the regulation of the adenylate cyclase by guanine nucleotides and adrenaline in 5 subjects suffering from multiple encapsulated lipomata. In the presence of GTP (0.1 mmol/l) basal adenylate cyclase activity averaged 0.5 +/- 0.3 nmol cAMP/mg protein/10 minutes in normal adipose tissue and 1.0 +/- 0.4 nmol cAMP/mg protein/10 minutes in hypertrophic adipose tissue respectively. The synthetic GTP-analogue GMP(PNP) (0.1 mmol/l) increased non-stimulated activity by about 100% in both tissues. Adrenaline (1 mumol/l-1 mmol/l) caused a dose-dependent increase of enzymic activity in both tissues which had a maximum of 130% above basal levels in the presence of GTP and of 300% in the presence of GMP(PNP) respectively. In one of the six subjects suffering from gluteal lipomata normal adipose tissue was obtained from the gluteal as well as the abdominal region on two occasions. Maximally effective concentrations of adrenaline (1 mmol/l) induced a 3-fold increase of enzymic activity in abdominal membranes compared with about a 1.7- and 1.75-fold increase in normal and lipomatous tissue from the gluteal region. The results show that encapsulated lipomata contain a normally reactive adenylate cyclase system.
A survey of roentgenological findings in muscular alterations of extremities based on the author's experiences and on the literature is presented. Following a description of the normal roentgen anatomy, the alterations in different diseases of interstitial lipomatosis are demonstrated. By roentgenological examinations different muscular lesions of the extremities can be differentiated and the clinical follow-up verified.
40 duodenal stumps formed following gastric resections according to Billroth II were studied. The most recent stump was 6 hours old, the oldest 35 years. With some exceptions the torus on the stump---due to the suture---disappears within one year and is replaced by a wide strip of scar tissue. Chronic inflammations due to foreign bodies, some with acute exacerbation, were proved histologically to persist even decades after the resection. These inflammations are closely related with the suture material and other foreign matter. In patients submitted to the Billroth II operation, lipomatosis in the pancreas occurred more rarely, whereas fibrosis set in more often.
Two cases of Madelung disease (benign symmetrical lipomatosis) are presented. The MR findings in this striking condition are demonstrated. Short-repetition-time/short-echo time sequences nicely show the relationship of the cervical lipomatous accumulations to the airway and major neurovascular structures in the carotid spaces. Fat-suppression techniques add no additional information in the radiologic evaluation of these patients.
Symmetrical benign lipomatosis (Madelung's disease) is extremely rare. Approximately 200 cases have been reported in the literature. SLB is a syndrome characterized by the occurrence of symmetric lipomas of the neck. No clear etiology has been recognized while a frequent association with systemic metabolic abnormalities has been described. The only effective therapy is the palliative surgical removal of the fatty tissue by lipectomy or liposuction. Experience with surgically treated patients shows the tendency of this disease to recur.
Lipomatous tumors with intramuscular infiltration are uncommon in the head and neck. Oral lipomas have been recorded in the literature; however, infiltration and recurrence have rarely been documented. We describe a case of an oral lipoma that did not have microscopic characteristics of malignancy, lipoblastomatosis, or atypia, but which showed intramuscular invasion and recurred twice after surgery. Although certain characteristics distinguish our case from congenital lipomatosis, the clinical picture is quite similar. Lipomas with intramuscular invasion uniformly tend to recur when they are not widely resected, whether in the trunk and extremities or in the head and neck. These lesions can rapidly enlarge and infiltrate local tissues, and they require wide resection with an attempt at preservation of important structures. Preoperatively, computed tomographic analysis allows a lipoma to be diagnosed by its low attenuation, and a grossly infiltrating tumor can be distinguished from the ordinary well-encapsulated lesion.
The concept of autosomal lethal genes surviving only in a mosaic state was proposed by Happle to explain the genetic basis of several syndromes characterized by (almost always) sporadic occurrence, distribution of lesions in a scattered or asymmetrical pattern, variable extent of involvement, lack of diffuse involvement of entire organs, and equal sex ratio. The mosaic may either arise from a gametic half-chromatid mutation or from an early postzygotic mutation. The purpose of this article is to review current knowledge of the genetics and cutaneous manifestations of some of the birth defects to which the lethal gene concept is thought to apply: the Schimmelpenning (Feuerstein-Mims) syndrome, Proteus syndrome, encephalocraniocutaneous lipomatosis, Sturge-Weber and Klippel-Trenaunay syndrome, cutis marmorata teleangiectatica congenita (van Lohuizen syndrome), and neurocutaneous melanosis.
Effective therapies are now available that can stop the progression of HIV infection and significantly delay the onset of AIDS. The "highly active antiretroviral therapy" (HAART) is a combination of potent antiretroviral drugs such as viral protease inhibitors or nucleoside-analogue reverse-transcriptase inhibitors, that has a variety of serious side effects, including lipodystrophy, a pathology characterized by accumulation of visceral fat, breast adiposity, cervical fat-pads, hyperlipidemia, insulin resistance as well as fat wasting in face and limbs. There is still an open debate that concerns the precise responsibility of HAART as well as metabolic pathways and mechanisms that are involved in the onset of lipodystrophy. The similarities with multiple symmetric lipomatosis (MSL), in which mitochondria impairment plays a crucial role, lead to the hypothesis that drug-induced damages to mitochondrial DNA are able to alter mitochondria functionality to an extent that is similar to what occurs in MSL. In addition, several evidences indicate that HAART is also linked to a deregulated production of tumour necrosis factor-alpha, which uses mitochondria as intracellular targets. In this paper, we review data concerning the role of mitochondria in the pathogenesis of lipodystrophy, and advance a unifying hypothesis involving either direct or indirect effects of the drugs employed during HAART.
The anterior neck is an unusual location for lipomas. Cervical lipomas can be mistaken for non-functioning thyroid nodules. We report eight cases from our files diagnosed by fine needle aspiration (FNA). Our purpose is to call attention to this simple technique in establishing an accurate diagnosis and how it can contribute to better patient management by avoiding unnecessary thyroid suppressive therapy and/or surgery. We advocate using FNA as the initial diagnostic test on palpable masses in the neck. The differential diagnosis must include thyroid lipomatosis, thyrolipoma or adenolipoma, amyloid goiter with fatty infiltration, and fat-containing thyroidal neoplasms (papillary carcinoma and follicular neoplasms).
PTEN, on 10q23.3, encodes a major lipid phosphatase which signals down the phosphoinositol-3-kinase/Akt pathway and effects G1 cell cycle arrest and apoptosis. Germline PTEN mutations have been found to occur in 80% of classic Cowden syndrome (CS), 60% of Bannayan-Riley-Ruvalcaba syndrome (BRRS), up to 20% of Proteus syndrome (PS), and approximately 50% of a Proteus-like syndrome (PSL). CS is a heritable multiple hamartoma syndrome with a high risk of breast, thyroid, and endometrial carcinomas. BRRS is a congenital autosomal dominant disorder characterized by megencephaly, developmental delay, lipomatosis, and speckled penis. PS and PSL had never been associated with risk of malignancy. Finding germline PTEN mutations in patients with BRRS, PS, and PSL suggests equivalent risks of developing malignancy as in CS with implications for medical management. The mutational spectra of CS and BRRS overlap, with many of the mutations occurring in exons 5, 7, and 8. Genotype-phenotype association analyses have revealed that the presence of germline PTEN mutations is associated with breast tumor development, and that mutations occurring within and 5' of the phosphatase motif were associated with multi-organ involvement. Pooled analysis of PTEN mutation series of CS and BRRS occurring in the last five years reveals that 65% of CS-associated mutations occur in the first five exons encoding the phosphatase domain and the promoter region, while 60% of BRRS-associated mutations occur in the 3' four exons encoding mainly the C2 domain. Somatic PTEN mutations occur with a wide distribution of frequencies in sporadic primary tumors, with the highest frequencies in endometrial carcinomas and glioblastoma multiform. Several mechanisms of PTEN inactivation occur in primary malignancies derived from different tissues, but a favored mechanism appears to occur in a tissue-specific manner. Inappropriate subcellular compartmentalization and increased/decreased proteosome degradation may be two novel mechanisms of PTEN inactivation. Further functional work could reveal more effective means of molecular-directed therapy and prevention.