SEARCH · PubMed Health
Explore indexed PubMed citations for clinical trials, systematic reviews and public health research. Read source abstracts and follow each citation to its original PubMed record.
Quote a phrase for an exact phrase match. Source license links do not imply unrestricted reuse.
Replacement lipomatosis of the kidney in a case of long-standing renal tuberculosis is reported. The radiologic and pathologic findings are described and the differential diagnosis is discussed. A hypothesis is given to explain the association of renal tuberculosis and replacement lipomatosis of the kidney.
UNLABELLED: We report a 7-year-old boy with the typical features of encephalocraniocutaneous lipomatosis (ECCL) including unilateral skin, eye, bone, and asymptomatic central nervous system involvement. The presenting sign were numerous ipsilateral odontomas which have been described in only two previous patients so far. We reviewed all 32 cases reported to date and focus on the dermatological hallmark of the condition, namely a fatty tissue naevus of the scalp for which the term naevus psiloliparus was recently coined. Sporadic occurrence, patchy type of organ involvement and roughly balanced sex ratio in ECCL are compatible with the concept of a lethal autosomal mutation only surviving in a mosaic state. CONCLUSION: In children presenting with a congenital smooth hairless lesion on the scalp, the diagnosis of naevus psiloliparus should be considered and other signs of encephalocraniocutaneous lipomatosis should be looked for.
Although not a common condition, benign lipomatosis of the neck (Madelung's disease) is well documented in the literature and to date we have failed to trace any reference to malignant change developing in this tissue. We wish therefore to report the case of a patient with benign lipomatosis of the neck in which malignant degeneration appeared after an interval of 6 years and in which this transformation from a benign to a malignant lesion was verified by histological examination.
A 34-year-old man presented with progressive myelopathy 4 months after cadaveric renal transplant for endstage renal disease. Radiographic evaluation gave findings consistent with epidural lipomatosis and compression of the thoracic thecal sac. Decompressive laminectomy resulted in dramatic improvement of his neurologic deficit. This case is unusual in the brevity of steroid treatment prior to onset of the myelopathy, as well as the relatively small dose. The 10 previous cases of epidural lipomatosis are also reviewed.
Pelvic lipomatosis has a classic roentgen triad which includes (1) pelvic radiolucency, (2) elevation of an intact rectosigmoid, and (3) elevation of the urinary bladder. This triad, in the absence of other abnormal clinical and roentgen findings, is pathognomonic of pelvic lipomatosis, and surgical biopsy is not necessary to confirm the diagnosis.
Pelvic lipomatosis is a disease characterized by abnormal deposition of mature adipose tissue within the confines of the pelvis surrounding the bladder, prostate, and rectosigmoid. The presenting symptoms are vague and nonspecific, and it has typical radiologic features. Computed tomography is extremely useful in the differential diagnosis because of its ability to differentiate fatty infiltration from other conditions which can cause elevation and compression of the urinary bladder and sigmoid colon. Computed tomographic confirmation of the presence of fat surrounding the bladder and rectum eliminates the need for surgical biopsy, unless indicated for other reasons. The course of this disease is usually benign and indolent, however a close follow-up is mandatory to rule out those cases in which ureteral obstruction may develop requiring surgical relief of the obstruction. When cystitis glandularis is found in association with pelvic lipomatosis, periodic cystoscopic examinations are recommended because of the potential risk of malignant transformation.
This is the first case reported of adenocarcinoma of the urinary bladder in a patient with pelvic lipomatosis and cystitis glandularis. The abnormal pelvic fat hampered endoscopic evaluation and transurethral biopsy, confused clinical staging, and complicated preoperative irradiation and cystectomy. The development of adenocarcinoma in this patient indicates the potential for malignant transformation of cystitis glandularis and underscores the necessity for endoscopic follow-up of persons with pelvic lipomatosis.
Forty-two cases of renal sinus lipomatosis were diagnosed by intravenous urography and nephrotomography. The differential diagnosis from a malignant process necessitated selective renal angiography in 9 cases. With the aid of computed tomography (CT), an invasive technique such as renal angiography appears to be unnecessary in the differential diagnosis of renal sinus lipomatosis. CT seems to be more accurate since it revealed a concomitant cyst in 2 of 4 cases.
Retroperitoneal lipomatosis is a condition in which excessive fat deposits in the retroperitoneum can exert a mass effect, with displacement of normal structures. In the case presented, it displaced lymph nodes and simulated adenopathy in nonopacified nodes. The specific diagnosis of retroperitoneal lipomatosis as the cause of the mass effect can be made by computerized tomography.
Three cases of infiltrating congenital lipomatosis of the face are presented. These lesions shared the following morphologic criteria: 1) nonencapsulated congenital fatty tumor; 2) infiltration of adjacent muscle and soft tissue; 3) absence of lipoblast and malignant characteristics; 4) presence of fibrous elements with numerous nerve bundles and vessels; and 5) hypertrophy of subjacent bone. Although these tumors are benign, all three recurred after surgical excision. Extensive specimen sampling is advisable to exclude infiltrating lipoma, diffuse angiomatosis, infiltrating angiolipoma, and well-differentiated liposarcoma. Relationship with lipoblastomatosis and histogenesis of infiltrating congenital lipomatosis of the face are discussed.
Epidural lipomatosis is most frequently seen in patients on chronic steroid treatment. Only twelve cases of idiopathic spinal epidural lipomatosis have been described. In this report we present an additional case of this condition in a middle-aged male presenting with neurogenic claudication.
INTRODUCTION: Encephalocraniocutaneous lipomatosis is a rare congenital disorder characterized by an abnormal development of adipose tissue, and is of unknown pathogenesis. Catherine Haberland and Maurice Perou first diagnosed this disorder in 1970. To our knowledge, approximately 25 patients have been reported with this diagnosis but so far, only 1 patient has been reported in Poland by Roszkowski and Dabrowski in 1997. At that time she was a 13-year-old girl, who was neurosurgically treated. CASE REPORT: The authors followed the same patient (now 21 years old), who was referred to the Department of Cranio-Maxillofacial Surgery of the Medical University of Lodz for osteoma of the mandible. Partial resection of the mandible was performed on account of the size of the lesion. The mandible was reconstructed by an iliac crest bone graft fixed by 2 titanium plates. The surgical procedure is described. CONCLUSION: Encephalocraniocutaneous lipomatosis is a very rare syndrome. Most of the cases described in the literature presented lipomas of the skin with neurological and ophthalmological disturbances. Only a few authors described jaw tumours and no information was found on such big osteomas to this syndrome.
INTRODUCTION: Madelung's disease is characterized by benign, non-encapsulated accumulations of fat in a symmetrical manner. Although rare, symmetrical lipomatosis involving the tongue exclusively has been previously reported. Here another case is presented of Madelung's disease involving the tongue. CASE REPORT: A 57-year-old woman was referred with the diagnosis of Madelung's disease, with slight difficulty in swallowing, dysarthria and dyspnoea while sleeping due to tongue swelling. Clinical examination revealed multiple, large disfiguring soft masses involving neck, upper arms and hips. There was enlargement of the tongue with bilateral protrusions and buccal displacement of the teeth. The masses were soft and non-tender. Bilateral partial glossectomy was performed under general anaesthesia. Histology revealed adipose tissue, interspersed within the lingual musculature. DISCUSSION: Lipomas in Madelung's disease are characterized by multiplicity, non-encapsulation and invasiveness. Over the years, the fat deposits enlarge significantly, become cosmetically deforming, and cause dyspnoea and dysphagia in advanced cases. The aetiology of benign symmetrical lipomatosis is still unknown. It is often accompanied by liver dysfunction probably due to alcoholism. Treatment is limited to surgical removal of the fatty tissue, in patients with a severe cosmetic deformity causing psychological stress, and in patients with dyspnoea or dysphagia if there is restriction of the aerodigestive tract.
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. Different diagnostic tools have been used to define this entity, with ultrasonography (US), i.v. pyelography (IVP) and computed tomography (CT) used most commonly. We report MR urography and MR appearance of replacement lipomatosis in a 38-year-old man. We think that it combines the advantages of IVP, US and CT.
From 1950 through 1985, 18 patients 26 to 65 years old (mean age 44.5 years) presented with pelvic lipomatosis. Followup ranged from 6 months to 17 years, with a mean of 7.5 years. Of these 18 patients 16 (89 per cent) are alive, 1 (6 per cent) died of uremia and 1 (6 per cent) was lost to followup after 6 months. During this period 7 patients (39 per cent) required operative procedures for urinary obstruction. Although pelvic lipomatosis is a benign proliferation of fat in the true pelvis that is known to lead to urinary tract, fecal and even vena caval obstruction, it also may remain dormant and cause no significant symptoms for many years.
Pelvic lipomatosis may increase significantly the difficulty and morbidity of many urologic procedures. Radical prostatectomy in this setting has not been described previously. Such a case is presented and the reported surgical experience in pelvic lipomatosis is reviewed. Considerations in the management of prostatic cancer in patients with this condition are discussed.
Pelvic lipomatosis is a rare, benign disease in which the characteristic roentgenographic findings may mimic those of pelvic malignancy. In the past more than two-thirds of reported cases have been explored surgically for diagnosis. We present 2 cases to demonstrate how the definitive diagnosis of pelvic lipomatosis can be made with the aid of computed tomography to avoid invasive techniques.
A 54-year-old black male with a 15-year history progressive lower extremity weakness was evaluated with a thoracic MRI that revealed epidural lipomatosis extending from T1-T10 with an associated syrinx at T1-3. He was neither overweight nor taking steroids. A multilevel thoracic laminectomy with resection of lipoma was performed without directly addressing the syrinx. Postoperatively, his symptoms improved and an MRI obtained 1 year after surgery demonstrated resolution of the syrinx. This is the only case of epidural lipomatosis with an associated syrinx that we have seen reported in the world literature.