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Results for “LIPOMATOSIS”

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PubMed1982

[Renal sinus lipomatosis, a pseudotumorous transformation].

A case of longstanding chronic pyelonephritis associated with severe recurrent nephrolithiasis followed by excessive lipomatosis, ureteritis cystica and xanthogranulomatous pyelonephritis is presented. In addition to the definition and morphologic description of lipomatosis its differential diagnosis is discussed. This also applies to the pathogenesis of these diseases. The special role of lipomatosis in the perpetuation of a circulus vitiosus is stressed. In special cases it seems to be justified to speak of clinical malignant lipomatosis.

Female↗

PubMed1994

Sporadic multiple lipomatosis: a case report and review of the literature.

The finding of multiple lipomas, or lipomatosis, can be a marker for several clinical or familial syndromes. Familial multiple lipomatosis is a benign hereditary disorder of adipose regulation associated with hyperlipidemia. Multiple symmetric lipomatosis involves the local infiltration of adipose tissue of the neck, upper torso and mediastinum. This condition is often found in alcoholics and has been associated with diabetes mellitus. An afflicted patient's family history is important both to reveal occult pathology and to help determine the disease's prevalence in the population. In this article, we report the case of a patient with sporadic multiple lipomatosis and provide a brief review of the literature.

Genes, Dominant↗

PubMed1996

Multiple familial lipomatosis with polyneuropathy, an inherited dominant condition.

A 22-year-old man had polyneuropathy, facial dysmorphia, atopia and multiple lipomatosis. His mother had neuropathy but not lipomatosis as two of her first cousins. The proband's grandmother had multiple lipomatosis as her own mother and a sister of her mother, but they didn't have neuropathy. This family is an example of a dominant syndrome the principle features of which are polyneuropathy. Variable expression could account for the phenotypic differences, combined with multiple lipomatosis.

Adult↗

PubMedDate not supplied

Lymphomatous meningitis and steroid-induced epidural lipomatosis: CT evaluation.

A case of spinal cord compression secondary to steroid-induced epidural lipomatosis is reported. The epidural lipomatosis developed in a setting of spinal lymphomatous leptomeningitis secondary to adult T-cell leukemia/lymphoma, an unusual type of non-Hodgkin lymphoma with a high frequency of leptomeningeal involvement. Computed tomography was invaluable in diagnosing epidural lipomatosis, despite the concomitant spinal lymphomatous leptomeningitis. To our knowledge this is the first case of epidural lipomatosis following chemotherapy for a malignancy.

Adrenal Cortex Hormones↗

PubMed1992

Crossed renal ectopia with pelvic lipomatosis: a new syndrome involving chromosome 1.

An 18-year-old male patient is described who possesses both kidneys on one side (crossed renal ectopia), together with pelvic lipomatosis. In general, lipomatosis is benign, but here the tissue shows the rare feature of malignancy. Chromosomally, the patient is typically characterised by somatic translocations involving chromosome 1 (37% metaphases); these almost always exhibit a whole chromosome translocation with chromosome 6 (35%), although involvement of chromosome 1 with chromosome 8 is present in 2% of metaphases. Other chromosomal features encountered in Giemsa-stained and G-banded preparations from lymphocyte cultures include the prevalence of a small Y chromosome in 25% of metaphases, the presence of marker dots in 20%, and acrocentric associations in 8%-10% of metaphases. However, more than 50% of metaphases have a normal 46XY karyotype with a normal-sized Y-chromosome. Crossed renal ectopia with pelvic lipomatosis can therefore be assigned to a new syndrome characterised by a whole-chromosome translocation involving chromosomes 1 and 6.

Adolescent↗

PubMed1995

Shoulder girdle lipomatosis.

Shoulder girdle lipomatosis is a distinct type of lipomatosis which has unique clinical and radiographic features. The MR findings reflect the pathologic findings and, in our opinion, allow the differentiation of shoulder girdle lipomatosis from other lipomatous masses on the basis of imaging findings alone.

Female↗

PubMed1995

Diffuse colonic lipomatosis with giant hypertrophy of the epiploic appendices and diverticulosis of the colon. Report of a case and review of the literature.

PURPOSE: A case of diffuse colonic lipomatosis, dolichosigmoid, hypertrophy of the epiploic appendices and diverticulosis of the colon producing constipation, recurrent sub-occlusive episodes with diarrhea and vomiting, and weight loss is reported. METHODS: Careful preoperative assessment of clinical, radiologic, and endoscopic findings suggested the diagnosis. RESULTS: A total colectomy, followed by a lateroterminal ileoproctostomy, gave complete relief from symptoms. CONCLUSIONS: Of the 12 cases of colonic lipomatosis previously reported, only 2 have isolated involvement of the entire organ, but they do not present lipomatosis of the epiploic appendices.

Adult↗

PubMed2003

Epidural, paraspinal, and subcutaneous lipomatosis.

A unique case of idiopathic diffuse lipomatosis is reported. The patient was an 11-year-old boy with diffuse lipomatosis in the epidural space, paraspinal muscles, and thoracolumbar subcutaneous regions. Epidural lipomatosis involved the entire thoracolumbar spine and was associated with filar thickening and lipoma. In addition, paraspinal muscles, especially the erector spinae group, had diffuse fatty infiltration. The ultimate clinical effect of this fatty tissue was urinary dysfunction, radicular pain and hypoesthesia in both legs and difficulty walking.

Child↗

PubMed2000

Congenital infiltrating lipomatosis of the face with cerebral abnormalities.

The aim of this study was to describe a possible variant of encephalo-craniocutaneous lipomatosis syndrome. Three cases of congenital infiltrating lipomatosis of the face, associated with cutaneous, subcutaneous, and cerebral abnormalities, are presented. This neurocutaneous syndrome appears very similar to encephalo-craniocutaneous lipomatosis syndrome but lacks the typical eye lesions.

Abnormalities, Multiple↗

PubMed2000

Esophageal lipomatosis: another consequence of the use of steroids.

After we incidentally found on CT extensive esophageal fat accumulations in a patient with long-term use of steroids, we prospectively evaluated during a 6-month period all CT studies of the chest for esophageal lipomatosis and related the findings to the possible use of steroids. The diagnosis of esophageal fat on CT was made by density measurements or if too small for reliable density measurements by comparison with mediastinal fat. In 21 of 1,320 exclusively older male patients the diagnosis of esophageal lipomatosis was definite in 7 and likely in 14 patients. All fat accumulations were located in the upper third of the esophagus (mean length 22 +/- 6 mm) and presented ring-like (n = 10), irregular (n = 3), or as a horseshoe sparing the posterior border (n = 8). In 20 patients there was an unequivocal history of steroid treatment. Associated centripetal fat infiltration was found in 11 patients. None of the patients had swallowing problems. Prolonged use of steroids, either orally or inhalationally administered, is associated with esophageal lipomatosis. The predisposition for the upper esophagus might be related to the presence of striated muscle cells in this part of the esophagus; moreover, inhalational steroid therapy may adversely affect the upper esophagus.

Aged↗

PubMed1999

Renal replacement lipomatosis: ultrasonography and computed tomography findings.

Replacement lipomatosis of the kidney is the result of severe atrophy or destruction of the renal parenchyma often caused by calculous disease with secondary marked proliferation of renal sinus, renal hilus, and perirenal fatty tissue. The diagnosis is difficult to establish with conventional radiographic methods. Although ultrasonography may show highly suggestive findings, computed tomography seems to be the most accurate method for demonstrating the distinctive features of replacement lipomatosis. Ultrasonographic and computed tomographic features in three cases of replacement lipomatosis of the kidney are reported.

Adult↗

PubMed1998

Jejunal lipomatosis with diverticulosis: report of a case.

We herein report the very rare case of a 68-year-old Japanese man with multiple jejunal lipomatosis and diverticulosis. He was admitted to our hospital with the chief complaint of melena and anemia. A barium study of the small bowel showed multiple lipomatosis and diverticulosis. An approximately 200-cm length of the jejunum was therefore resected. Thereafter, two diverticula and 215 lipomas were recognized in the resected specimen. A pathological examination showed mature adipose tissue with fibrous septa in the submucosal and muscularis propria. These findings were thus suggested to be due to the attenuation of the muscularis propria. The complications of lipomatosis are also discussed.

Aged↗

PubMed1979

Computed tomography and ultrasound in diagnosis of pelvic lipomatosis.

Computed tomography (CT) and ultrasound are emerging as useful diagnostic adjuvants in the confirmation of pelvic lipomatosis. A case of pelvic lipomatosis studied by CT and sonography is presented. These two techniques offer greater precision in the demonstration of fatty tissue density within the true pelvis. The findings appear characteristic and unique. CT and ultrasound confirmation of pelvic lipomatosis provide added confidence in an accurate clinical diagnosis and may obviate the need for diagnostic surgical exploration.

Humans↗

PubMed1994

Lumbar epidural lipomatosis causing neurogenic claudication in two obese patients.

Two obese patients suffering from neurogenic claudication caused by lumbar epidural lipomatosis are described. Although lumbar epidural lipomatosis is most often related to prolonged use of steroid drugs, obesity has also been reported as a possible cause. Both CT and MRI can demonstrate excess epidural fat; because of the possibility of sagittal views MRI is to be preferred. In one of our patient with neurogenic claudication the excess epidural fat normalised completely after considerable weight reduction and symptoms resolved. Therefore weight reduction might be the initial therapy in an obese patient with symptomatic epidural lipomatosis. When weight reduction fails or when there are urgent clinical reasons, surgical removal of the excess amount of epidural fat should be considered.

Adult↗

PubMedDate not supplied

Spinal cord involvement in encephalocraniocutaneous lipomatosis.

Encephalocraniocutaneous lipomatosis is a rare hamartomatosis involving the craniofacial region and the central nervous system. The most prominent clinical features are large areas of scalp alopecia, soft subcutaneous craniofacial masses, lipomas, connective tissue nevi of the eyelids and surrounding areas, pterygium-like choriostoma of the ocular conjunctiva, mental retardation, motor deficit, and seizures. Of the eight patients reported previously, three had spinal cord evaluations and two had evidence of lipomatosis. We report the third patient with this association, review the literature of encephalocraniocutaneous lipomatosis, and stress the importance of spinal cord evaluation during the newborn period.

Adolescent↗

PubMedDate not supplied

Lumbar epidural lipomatosis: the "Y" sign of thecal sac compression.

PURPOSE: We have identified a specific sign of lumbar thecal sac compression seen in patients with symptomatic lumbar epidural lipomatosis. Recognition of this sign will aid with proper diagnosis of this unusual disorder. METHODS: Computerized Tomography (CT) and Magnetic Resonance Imaging (MRI) examinations of the lumbar spine performed in the evaluation of low back pain over a 24 mo period demonstrated nine patients with excessive fat in the epidural space. Myelography was performed on five of these patients. Detailed analysis of the shape and degree of thecal sac compression was made. RESULTS: Eight of the nine patients with epidural lipomatosis demonstrated a characteristic type of thecal sac compression not seen with other spinal disorders. On cross sectional imaging, the thecal sac has a striking stellate appearance with three rays emanating from a central core. This produces a trifid shape resembling the letter "Y". In two patients scanned following subsequent surgical removal of the epidural fat, the thecal sac had resumed its normal configuration. CONCLUSION: A seemingly specific and easily recognized type of compression of the thecal sac may occur with lumbar epidural lipomatosis. This creates a trifid appearance of the thecal sac; we have termed this the "Y" sign.

Adult↗

PubMedDate not supplied

The importance of re-evaluation in patients with cystitis glandularis associated with pelvic lipomatosis: a case report.

Pelvic lipomatosis is a rare, proliferative disease involving an overgrowth of normal fat in the pelvic retroperitoneal space. Cystitis glandularis, cystitis cystica, or cystitis follicularis can be observed in 75% of patients with pelvic lipomatosis. We describe a 58-yr-old man with pelvic lipomatosis in whom adenocarcinoma of the bladder was diagnosed in the second transurethral resection of the bladder 1 month after the first operation. This proliferative disease can cause obstruction of the bladder drainage leading to a proliferative cystitis because of an altered environment rich in protein fluid. Because the association of this proliferative disease with adenocarcinoma of the bladder is frequent, we recommend close follow-up of these patients to detect the associated adenocarcinoma of the bladder.

Adenocarcinoma↗

PubMed1994

Ocular and systemic manifestations of encephalocraniocutaneous lipomatosis.

Encephalocraniocutaneous lipomatosis is a congenital neurocutaneous syndrome with epibulbar choristomas and connective tissue nevi of the eyelids as common ophthalmic manifestations. Systemic manifestations occur ipsilateral to the ocular lesions and include lipomas of the cranium and central nervous system, alopecia of the scalp, and abnormalities of the central nervous system. We treated a child with encephalocraniocutaneous lipomatosis who required removal of an epibulbar choristoma. Pathologic evaluation of the epibulbar choristoma in our patient showed the presence of ectopic lacrimal gland tissue and cartilage. Encephalocraniocutaneous lipomatosis should be considered, together with Goldenhar's syndrome and sebaceous nevus syndrome, in the differential diagnosis of conditions associated with epibulbar choristomas.

Cartilage↗