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Congenital lumbosacral lipomas: pitfalls in analysing the results of prophylactic surgery.

OBJECTIVES: Our objectives were to examine the assumption that the natural history of congenital lumbosacral lipomas in children presenting early and those presenting late is the same, and to determine whether there are factors that might predict the post-operative outcome of surgery among the presenting features of children with lumbosacral lipomas. METHODS: The authors present a clinical series of 50 consecutive cases of congenital lumbosacral lipoma that had been treated surgically. All patients underwent both pre- and post-operative urological assessment and pre-operative imaging with MRI. The case notes, urology assessments and MRI films were studied to provide data on the clinical presentation, follow-up history and urological outcome of the children, as well as on the anatomical features of their lipomas. RESULTS: Analysis of the data revealed a predominance in girls and a bimodal distribution of age at presentation with peaks at 0-2 and 7-8 years. Skin stigmata were more frequent in children below 6 years of age than in older children (P=0.035). The MRI scans demonstrated that the conus lay within the sacral canal more often in girls than boys (P=0.025). Severe bladder dysfunction was significantly more frequent in those presenting at =3 years of age (P=0.017) and there were more normal outcomes in patients operated upon before the age of 3 than in those operated upon at or after this age ( P<0.0001). Eight patients who had been operated upon had severe urological problems at the time of their last assessment (median age of 8 years and 2 months). They were predominantly girls with major pre-operative urological dysfunction. CONCLUSIONS: Formal pre-operative urological assessment proved to be the strongest determinant of final urological outcome. Other predictors of poor urological outcome were large lipoma size and sacral position for the lipoma. This series demonstrates how early and late presenting children may represent different patient groups, follow different natural histories and may therefore not be reliable for comparison purposes. Future studies of prophylactic untethering need to follow cohorts beyond the age of deterioration found in untreated cases (8 years) and must report the salient MRI features that we suggest are predictive of poor outcome.

Adolescent↗

Lumbosacral lipomas: in utero diagnosis and prognosis.

INTRODUCTION: For the purposes of prenatal counselling and prognostication, it is essential to keep in mind the key diagnostic criteria allowing recognition of the diverse forms of spinal dysraphism. DIAGNOSIS: It is most crucial not to confuse lipomas and myelomeningoceles, the former carrying a usually favourable prognosis, while the latter a high risk of serious neurological deficits. In our experience, these two conditions can be clearly distinguished on prenatal ultrasound and by fetal biology. Lipomas are skin-covered, which explains the normality of the amniotic fluid and probably that of the intracranial content (no Chiari, no hydrocephalus). These features are distinct from those observed in the case of myelomeningoceles. The distinction between a lipoma and a meningocele is more difficult. The prenatal ultrasound presentation of many lipomas actually resembles that of a meningocele. In a few cases, the diagnosis of lipoma can, however, be suspected by the presence of an abnormally thick skin covering and intradural echoic structures that evoke a low-lying cord. CONCLUSIONS: However, the consequences of confusing a lipoma and most of the other types of occult dysraphism are minimal. Most of these lesions carry a favourable outcome.

Female↗

Factors in neurological deterioration and role of surgical treatment in lumbosacral spinal lipoma.

The purpose of this study was to determine factors that might be involved in neurological deterioration and the role of surgical treatment in patients with lumbosacral spinal lipoma. Pre- and postoperative courses of 34 patients were retrospectively analyzed. The age at surgery ranged from 1 month to 47 years. The records of preoperative neurological status indicated that older patients had more severe deficits, while all 8 asymptomatic patients were under 5 years of age. Motor deficits were noted in 9 patients, in 7 of whom the lipoma extended cranially beyond the L5 level. Transitional-type lipomas were accompanied by more severe deficits (asymptomatic 1, symptomatic 17) than other types (asymptomatic 7, symptomatic 9). Postoperative follow-up periods ranged from 5 months to 13 years. During these periods, 7 of the 8 asymptomatic patients remained neurologically intact. Nine of the 26 symptomatic patients improved. Age, extension of the lipoma in the spinal canal and type of lipoma will influence the preoperative neurological status of the patients. Early untethering surgery is recommended in patients with large lipomas extending beyond the L5 level.

Adolescent↗

Minilaparotomy approach for removal of a large colonic lipoma: report of two cases.

A minimally invasive surgical approach should be employed to resect symptomatic colonic lipomas whenever possible. We report two cases of large colonic lipomas that were successfully removed using a minimally invasive minilaparotomy approach. Patient 1 was a 53-year-old man with a 3.8-cm symptomatic submucosal lipoma in the ascending colon and patient 2 was a 57-year-old woman with a 4.2-cm symptomatic submucosal lipoma in the transverse colon. Both lipomas were successfully removed through a 5-7-cm minilaparotomy. Normal bowel function returned quickly without any postoperative complications. These case reports demonstrate that the minilaparotomy approach is a suitable alternative to conventional laparotomy to remove a large colonic lipoma.

Colonic Neoplasms↗

Computed tomography of infiltrating benign lipoma.

Lipomas are frequently encountered benign tumors of adipose tissue origin. A lipoma generally develops in the superficial subcutaneous tissues but may occasionally be deep seated, growing between or within muscles. Deep-seated lipomas (excluding body cavities and viscera) have been termed infiltrating benign lipomas, of which there are two varieties--lipoma and angiolipoma. We wish to report three cases of infiltrating benign lipomas. Computed tomography scanning is the diagnostic modality of choice in the evaluation of these tumors.

Aged↗

Supernumerary ring marker chromosome as a secondary rearrangement in a parapharyngeal lipoma with t(10;12)(q25;q15) as the primary karyotypic abnormality.

Cytogenetic analysis of a large parapharyngeal lipoma, a rare tumor at this site, showed the karyotype 46,XY,t(10;12)(q25;q15)/47,XY,t(10;12)(q25;q15),+r. The primary abnormality must have been t(10;12), whereas the +r developed secondarily as an additional aberration. Although a similar translocation between chromosomes 10 and 12 has not been described previously, that the tumor had a rearrangement of the 12q13-15 region, the most frequently involved genomic region in lipogenic tumors, indicates that the tumorigenetic mechanisms are identical in parapharyngeal lipomas and lipomas of other locations. Supernumerary ring markers are not usually detected in lipomas with 12q rearrangements but are common in atypical lipomas and well-differentiated liposarcomas. The parapharyngeal lipoma we describe recurred after initial resection, and we hypothesize that the more aggressive tumor phenotype thus demonstrated may be causally related to the clonal evolution it had undergone.

Chromosomes, Human, Pair 10↗

Lipomas treated with subcutaneous deoxycholate injections.

BACKGROUND: Lipomas are benign neoplasms of mature fat cells. Current treatments are invasive and carry the risk of scarring. Injections of phosphatidylcholine solubilized with deoxycholate, a bile salt, have been used to reduce unwanted accumulations of fat. Recent in vitro and ex vivo investigations indicate that deoxycholate alone causes adipocyte lysis. OBJECTIVE: We sought to report our experience treating lipomas using subcutaneous deoxycholate injections. METHODS: A total of 6 patients presenting with 12 lipomas were treated with intralesional injections of sodium deoxycholate (1.0%, 2.5%, and 5.0%) at intervals of 2 to 20 weeks. Tumor size, cutaneous reactions, and patients' subjective responses were recorded before and after treatment. RESULTS: All lipomas decreased in size (mean area reduction, 75%; range, 37%-100%) as determined by clinical measurement (with ultrasound confirmation in one lipoma) after an average of 2.2 treatments. Several lipomas fragmented or became softer in addition to decreasing in volume. Adverse effects, including transient burning, erythema, and local swelling, were associated with higher deoxycholate concentrations but resolved without intervention. There was no clear association between deoxycholate concentration and efficacy. CONCLUSIONS: Our clinical experience supports our laboratory investigations demonstrating that deoxycholate, rather than phosphatidylcholine, is the active ingredient in subcutaneously injected formulas used to treat adipose tissue. This small series suggests that low concentration deoxycholate may be a relatively safe and effective treatment for small collections of fat. However, controlled clinical trials will be necessary to substantiate these observations.

Adult↗

Meningioma associated with intraosseous lipoma.

Intraosseous lipomas of the skull are quite rare and only eight cases have been reported. The lipoma in our case was different from that in others in that it showed remarkable proliferation of collagen tissue. This is the first report of meningioma occurring after removal of an intraosseous lipoma of the skull. This report concerns a rare case of a 32-year-old female who developed a huge convexity meningioma 11 years after successful removal of intraosseous lipoma of the left frontoparietal bone. The meningioma originated from the convexity where the lipoma had been, and extended intracranially to the sphenoid ridge. The patient underwent surgical exploration showing that the meningioma had invaded the frontoparietal convexity bone and was firmly attached to the coronal suture with a large dural defect. This rare coincidence suggests that proliferative changes of the intraosseous lipoma and meningeal neoplasm may be the result of the same, as yet unknown, mechanism.

Adult↗

Congenital infiltrating lipoma of the upper limb in a patient with von Willebrand disease.

Infiltrating lipoma is a rare variety of lipoma, characterized by an infiltration of the adipose tissue of the muscles. Infiltrating lipomas are usually classified in two groups: intermuscular infiltrating lipoma and intramuscular infiltrating lipoma. Most are acquired, and they usually appear in middle-aged individuals. Exceptionally, they are congenital. In such cases they are not related to other diseases. We report an 8-year-old boy with a congenital infiltrating lipoma of the upper limb and von Willebrand disease. Both diseases are linked to an alteration in chromosome 12, but this clinical association seems to be random rather than causal.

Arm↗

Arthroscopic treatment of an intra-articular lipoma of the knee joint.

Intra-articular lipoma is an exceedingly rare diagnosis. There have been less than 15 documented cases of an intra-articular lipoma of the knee joint. This report presents the first description of an intra-articular knee lipoma treated entirely by arthroscopic methods. Preoperative history, examination, and imaging studies are reviewed. Intraoperative findings, treatment, and postoperative evaluation are discussed as well. Symptomatic intra-articular lipoma of the knee joint can be successfully treated by arthroscopic resection. Differentiating intra-articular lipoma from lipoma arborescens, a similar but more common condition, is important with regards to optimal treatment.

Adult↗

CNS lipoma in patients with epidermal nevus syndrome.

Epidermal nevus syndrome (ENS) is a congenital neurocutaneous disorder characterized by linear nevus with a significant involvement of the nervous, ophthalmological and skeletal systems. Clinical manifestations of ENS include neurological features such as mental retardation, seizures, and movement disorders which are caused by a wide range of neuropathological lesions. We describe three patients with ENS, all of whom had in addition to the characteristic features of ENS intracranial and/or intraspinal lipomas. In one patient the lipoma extended from the thoracal vertebra 8 to the 4th ventricle; in the second patient it was localized on T9, and in the third patient an intracranial lipoma was located at the right cerebellopontine angle. The intraspinal lipomas caused a significant spastic movement disorder. So far, CNS lipomas have not been described as typical neuropathological findings in ENS. The differential diagnosis to encephalocraniocutaneous lipomatosis with the typical finding of CNS lipoma is discussed.

Adolescent↗

MR imaging of lipoma and liposarcoma.

PURPOSE: To evaluate whether lipoma, atypical lipomatous tumors, and liposarcomas can be differentiated by MR images. MATERIAL AND METHODS: The MR images of 59 lipomatous lesions and liposarcomas were retrospectively reviewed. Apart from size, surgical site, location and margins, the percentage of fat of the tumor volume was assessed as none, 1-75%, 75-95%, or 95-100%. RESULTS: None of the 18 liposarcomas contained fat that could be recognized by MR imaging. The 3 atypical lipomatous tumors all contained fat but less than 75% of the tumor volume. In 32 of 38 ordinary lipomas, the percentage of fat was 95-100%, and in 4 less than 95% of the tumor volume. Two lipomas did not contain fat that could be recognized by MR imaging. CONCLUSION: A lesion which predominantly has a fat signal is, in all probability, an ordinary lipoma. Lesions with less fat, but still mostly fatty, may either be lipoma or atypical lipomatous tumor. In this group, the discrimination between these two entities cannot be based upon imaging features. In the absence of a fat signal, liposarcoma or lipoma cannot be differentiated from other soft tissue tumors.

Adipose Tissue↗

Orbital lipoma.

A patient who had a huge orbital lipoma with extension to the muscle cone is described. The lipoma was 42 x 34 x 32 mm in size, and there was no visual disturbance following removal of the tumor and iliac bone grafting. Diagnosis of orbital lipoma is difficult to make. This is particularly true of a lipoma with an obvious capsule, which cannot be easily differentiated from a dermoid cyst. However, computed tomography scanning is useful in ascertaining the location, size, and relationship with orbital contents. A review of the literature concerning orbital lipoma revealed only a few reports, and the features of the orbital lipoma are summarized. In addition, the possible mechanism of orbital enlargement and the approach to the muscle cone are described.

Adult↗

Retropharyngeal lipoma causing obstructive sleep apnea: case report including five-year follow-up.

OBJECTIVES/HYPOTHESIS: Lipomas of the retropharyngeal space are rare and do not cause symptoms until they reach a large size. Although retropharyngeal lipoma is an uncommon entity, several reports of it appear in the literature, and the treatment has routinely been surgical excision. Such fatty tumors also carry the rare possibility of being liposarcomas, which further warrants their excision. We present the case of a lipoma of the retropharyngeal space extending from the nasopharynx to the superior mediastinum causing symptoms of obstructive sleep apnea. The patient had multiple medical problems and was on a regimen of anticoagulation therapy; therefore, he opted against surgical treatment. He has used continuous positive airway pressure and has been followed clinically and radiographically for 5 years. Radiographic follow-up of a retropharyngeal lipoma after a needle biopsy confirming its benign nature is a legitimate means of management of this rare condition. STUDY DESIGN: Case report of a 64-year-old man presenting with this rare lesion. METHODS: Computed tomography-guided needle biopsy of the mass was performed to obtain tissue diagnosis. Thereafter, the patient has been followed for 5 years with annual magnetic resonance imaging scans to determine growth or changes of the retropharyngeal mass. RESULTS: Fine-needle aspiration of the mass revealed mature adipose tissue intermixed with fibroconnective tissue, consistent with lipoma. Based on this result, we opted to follow this patient with serial magnetic resonance imaging scans, which have shown no change in size. There has also been no change in the patient's symptoms. CONCLUSIONS: Large lipomas warrant excision, especially when their location produces pressure symptoms. However, when surgical morbidity is high, fine-needle aspiration biopsy and serial magnetic resonance imaging scans are a safe alternative.

Biopsy, Needle↗

Spindle cell lipoma of the oral cavity.

Spindle cell lipoma is typically seen in the neck/trunk region of middle-aged and older men. Rare cases of oral spindle cell lipoma have been reported. An entity described as myxoid lipoma of the oral cavity has rarely been reported but appears to be more properly classified as spindle cell lipoma. We describe the largest series yet of oral spindle cell lipoma involving the tongue (4), buccal mucosa (1), floor of mouth (1), and lip (1). The patients (3M; 4F) ranged from 31 to 88 years old. All presented with mass lesions. All were circumscribed and composed of mature adipocytes admixed with bland spindled cells. In two cases the adipocytes appeared atrophic, imparting a pseudo-lipoblastic appearance. No true lipoblasts were seen and none had the characteristic vasculature of a myxoid liposarcoma or the characteristic hyperchromatic cells of well-differentiated liposarcoma. The stromal background of all cases contained characteristic wiry collagen and myxoid ground substance. The myxoid ground substance was prominent in four cases. Immunohistochemical stains for CD34 highlighted the bland spindle cells in all cases. The combination of the histologic features and the immunoreactivity for CD34 confirmed the diagnosis. Spindle cell lipoma should be considered in the differential diagnosis of oral cavity mesenchymal tumors.

Adult↗

The treatment of atypical lipoma with liposuction.

Large lipomas may best be treated with liposuction. Once a lipoma enlarges to 4 cm or more, liposuction has several advantages over conventional surgery. A well-demarcated lipoma 15 cm in diameter and deep to muscle was treated by liposuction. Biopsy showed an atypical lipoma. Because the entire tumor was removed by liposuction, we feel that the prognosis is excellent. The cosmesis and morbidity results were far superior to those anticipated with conventional excisional surgery. Small superficial lipomas may be treated by suction lipectomy and the surrounding area can be contoured symmetrically at the same time, whereas large ones can be liposuctioned completely without extensive surgical extirpation and morbidity. Because such big lesions may represent atypical lipomas or liposarcomas, care must be taken to remove the entire tumor.

Adult↗

Giant lipomas: a series of 12 consecutive cases and a giant liposarcoma of the thigh.

BACKGROUND: The clinical presentation and character of giant lipomas create diagnostic problems because of their close relationship with other mesenchymal benign and malign tumors. It is therefore of primary importance for the clinician to be familiar with epidemiologic and anatomic attributes of these lesions. OBJECTIVE: To evaluate and compare epidemiologic and clinical characteristics of giant lipomas operated in our clinic. METHODS: Twelve giant lipomas and one giant liposarcoma are included in the study. RESULTS: There were seven females and six males. The patients ranged in age between 22 and 75. The tumor duration was between 2 months and 40 years. The smallest lipoma size was 10x5x3 cm, and the largest was 19x9x5 cm. Two of the lipomas were located intermuscular. Mild hematoma formation was observed in two patients. Histopathologic evaluation was done for all specimens, and one myxoid liposarcoma was encountered. CONCLUSION: When dealing with giant lipomas, liposarcomas should always be included in the differential diagnosis.

Adult↗

Fibrohistiocytic differentiation in subcutaneous fatty tumors. Study of spindle cell, pleomorphic, myxoid, and atypical lipoma and dedifferentiated liposarcoma cases composed in part of CD34+ fibroblasts and FXIIIa+ histiocytes.

Subsets of dendritic cells, fibroblasts which express the human progenitor cell antigen CD34 or histiocytes which express coagulation factor XIIIa (FXIIIa), are present in fat and in collagenous connective tissue. As components of the microvascular unit, these fibrohistiocytic cell subsets may interact during stromal remodeling, repair, and neoplasia. We studied white fat and subcutaneous fatty tumors to determine if CD34 and/or FXIIIa+ "fibrohistiocytic" dendritic cell subsets are involved in their morphogenesis. Three lipomas (L), 1 intramuscular lipoma (IL), 1 myxoid lipoma (ML), 2 pleomorphic lipomas (PL), 2 spindle cell lipomas (SCL), 8 angiolipomas (AN) in 4 patients, 1 atypical lipoma/well-differentiated liposarcoma (AL), 1 de novo dedifferentiated liposarcoma (DL), and 1 recurring atypical myxoid signet ring lipomatous tumor were examined for CD34, FXIIIa and in some cases for CD31, desmin, Ki 67, or S-100. Normal fat has scattered CD34+ dendritic cells and small FXIIIa+ dendritic histiocytes among variably S-100+ adipocytes. The CD34 and FXIIIa+ dendritic cells are more numerous near vessels and within fibrovascular septae. In L and IL, CD34 and FXIIIa+ dendritic cells are activated and some adipocytes express CD34. Mesenchymal areas of SCL, PL, ML, and AL and DL are composed of CD34+ dendritic cells with CD34+ but FXIIIa-negative floret cells in PL or atypical cells in AL and DL. FXIIIa+ dendritic cells are numerous in these lesions, comprising 30-40% of cells in SCL and PL, and 50% in ML, AL, and DL. AN have focal CD34+ interstitial cells and plump FXIIIa+ cells that in one case resembled multivacuolated lipoblasts. The myxoid signet ring lipomatous tumor was CD34 negative with few FXIIIa+ cells. We conclude that subsets of CD34+ and FXIIIa+ dendritic microvascular cells are present in normal fat and proliferate together in various types of lipomas and in at least some dedifferentiated liposarcomas.

Aged↗