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Central nervous system lipomas.

The lipomas of the central nervous system are rare lesions of congenital origin and are located in the medial line and especially in corpus callosum. Intramedullary spinal lipomas can be seen in the life span of 30 years of age and most frequently coincide with initial puberty period. Magnetic resonance imaging and computerized tomography together with clinical trials are of crucial importance for diagnosis. The first case: A two-year-aged girl who had lipoma in quadrigeminal cistern and who suffered from encephalocraniocutaneous lipomatosis has been clinically studied. The fascial lipoma of the patient has been excised subtotally by the Plastic Surgeons; then the patient has underwent supracerebellar infratentorial operation where the intracranial lipoma has been excised by our team. The histopathology has been reported to be consisting of peripheric nerve tissue and calcification. The second case: A twenty-year-aged man with intramedullary lipoma localized between T1-T4 has been given our clinical trials. T1-T5 total laminectomy and subtotal excision were made for this patient. Due to the fact that the lipomas of central nervous system are rarely seen and are involved in nervous and calcific tissues except for fatty tissues they can be mistaken for hamartomatous masses. The total excision of the lipomas of central nervous system and especially the spinal intramedullary lipomas are quite difficult to be excised since they are tightly entangled with the neural tissue. So any attempt for total excision would be dangerous. Operation for decompression and biopsy is of primary concern.

Adult↗

Differential diagnosis of benign peripheral lipoma from well-differentiated liposarcoma on MR imaging: is comparison of margins and internal characteristics useful?

OBJECTIVE: Our objective was to evaluate the reliability of MR imaging in distinguishing between benign lipoma and well-differentiated liposarcoma. MATERIALS AND METHODS: The MR images of 35 pathologically proven benign lipomas in 35 patients and 23 well-differentiated liposarcomas in 17 patients were retrospectively reviewed. T1-, T2-, and fat-suppressed T1-weighted images were obtained after administration of gadopentetate dimeglumine. Margins and internal characteristics revealed on the MR images and the degree of contrast enhancement of septa were evaluated. These MR imaging findings were compared for well-differentiated liposarcomas and benign lipomas. RESULTS: Completely irregular margins were recognized only in benign lipomas with a pathologic diagnosis of infiltrating lipoma. All tumors without a recognizable nonadipose component were benign lipomas (p < 0.05). As for the well-differentiated liposarcomas, thick septa and nodular or patchy nonadipose components were present more frequently in deep and retroperitoneal lesions than in subcutaneous lesions (p < 0.01). No cases showed only thin septa in the deep lesions of well-differentiated liposarcoma, and all cases showed thick septa or nodular or patchy nonadipose components. The septa in well-differentiated liposarcomas enhanced more strongly than did those in benign lipomas. The septa showed no enhancement relative to muscle in 11 of 19 benign lipomas, whereas the septa showed moderate or marked enhancement in all well-differentiated liposarcomas (p < 0.01). CONCLUSION: Careful assessment of margins and internal characteristics on MR imaging can be a useful aid in further distinguishing between biologically different benign lipoma and well-differentiated liposarcoma.

Adult↗

[Echography in the study of lipomas of superficial soft tissues].

High-resolution sonography (US) was used to evaluate 47 superficial soft tissue lipomas. Forty-one lesions were found in subcutaneous tissues and 6 in superficial muscles. The lipomas were classified by location, shape, echotexture, homogeneity and US beam attenuation values. Moreover, 21 lipomas underwent histology and histologic patterns were correlated with echogenicity. Most lesions were elongated and their greatest diameter was parallel to the skin. Eighty-four percent exhibited well-defined margins. Twenty-six percent of the lipomas were hypoechoic, 24% were isoechoic, 34% hyperechoic and 16% exhibited a mixed pattern. In 27% of cases, a hypoechoic capsule was present. Superficial soft tissue lipomas exhibit unsteady echotexture: most of them are hyperechoic. The correlation between echotexture and histology showed that no typical and steady patterns can be identified, since lipomas vary from a histologic point of view. The clinical diagnosis of superficial lipomas is based on clinical history and the palpation of a well-defined, mobile and soft superficial mass. When palpation is not diagnostic, US can be used to rule out a cyst. An elongated isoechoic or hyperechoic mass in the subcutaneous tissue should suggest a lipoma, whereas a hypoechoic mass is associated with a broader range of differential diagnoses, including malignant tumors. However, malignant masses are not likely to have an elongated or flattened shape. Even though tissue characterization is less specific with US than with CT and MRI, the former method is quick, easy and less expensive and, thanks to high-frequency transducers, is also well-suited to diagnose soft tissue lipomas.

Adolescent↗

Correlation of morphologic and biochemical observations in human lipomas.

Our studies of 107 patients with 133 lipomata showed that the male to female ratio for single lipoma was 1.2:1, and for multiple lipoma it was 3.5:1. More than 40% of the men and 30% of women with multiple lipoma had a family history of lipoma. Specimens from 23 patients were analyzed for lipid and protein content. Adipose tissue from lipoma had somewhat more lipid per gram of wet weight than adjacent normal tissue (75.0 versus 72.8%) or 16 additional control adipose tissue samples, from patients undergoing elective abdominal surgery (71.5%), but the differences were not statistically significant (p = 0.085). There were no differences in protein content. In 13 men the lipoprotein lipase activity of lipoma was markedly higher compared with the adjacent normal adipose tissue (40.4 +/- 15.5 versus 14.0 +/- 11.7 nmoles/gm/minute at 37 degrees C, p = 0.001) or to control adipose tissue (9.6 +/- 7.2 nmoles of free fatty acid/gm/minute at 37 degrees C, p = 0.001). It is likely that the high lipoprotein lipase activity of lipoma contributes to the growth of the tumor. Morphologically, there were no clear distinguishing features between the adipocytes of lipoma, adjacent normal adipose tissue, or control adipose tissue. However, the numbers of more immature type or preadipocytes (mesenchymal cells) appeared to be more numerous in lipomata compared with the control tissues. No differences in immunologic reactivity could be detected using antisera to fat cell membranes from lipoma or control adipose tissue.

Adipose Tissue↗

Multiple symmetric lipomas with high levels of mtDNA with the tRNA(Lys) A-->G(8344) mutation as the only manifestation of disease in a carrier of myoclonus epilepsy and ragged-red fibers (MERRF) syndrome.

We have investigated the morphology, cytogenetics, and the fraction of mtDNA with the tRNA(Lys) A-->G(8344) mutation in three lipomas in a carrier of this mutation. The son of the patient had myoclonus epilepsy and ragged-red fibers syndrome. The fraction of mtDNA with the tRNA(Lys) mutation varied between 62% and 80% in cultured skin fibroblasts, lymphocytes, normal adipose tissue, and muscle. In the three lipomas the mean fraction of mutated mtDNA was 90%, 94%, and 94%. Ultrastructural examination of the lipomas revealed numerous mitochondria with changes such as electron-dense inclusions in some adipocytes. When considered cytogenetically, the lipomas were characterized by a mixture of karyotypically abnormal and normal cells. An identical del(6)(q24) was found in two tumors. The fraction of mutated mtDNA in cultured lipoma cells was the same as in the lipoma in situ, indicating that the cultured cells were representative of the primary tumor. These findings indicate that the lipomas have originated with a grossly normal stem line and subsequently have developed the 6q deletion. We conclude that the lipomas represent clonal growth of adipocytes with a high content of mtDNA with the tRNA(Lys) mutation. The tRNA(Lys) mutation may be either the direct or the indirect cause of pertubation of the maturation process of the adipocytes, leading to an increased risk of lipoma formation.

Adenine↗

LHFP, a novel translocation partner gene of HMGIC in a lipoma, is a member of a new family of LHFP-like genes.

A major cytogenetic subgroup among human lipomas is characterized by translocations involving the HMGIC gene at 12q15. In the context of an ongoing research program aiming at the elucidation of the functional consequences of HMGIC translocations in the etiology of lipomas, we have isolated a novel human gene, LHFP (lipoma HMGIC fusion partner), that acts as a translocation partner of HMGIC in a lipoma with t(12;13). The LHFP gene was mapped to the long arm of chromosome 13, a region recurrently targeted by chromosomal aberrations in lipomas. By Northern blot analysis, a transcript of 2. 4 kb was detected in a variety of human tissues. We assembled a cDNA contig containing the entire coding region of LHFP. Nucleotide sequence analysis of the composite LHFP cDNA revealed an open reading frame encoding a protein of 200 amino acids. The predicted human LHFP protein is almost identical to a translated mouse EST that covers almost the entire LHFP coding region. In addition, BLAST searches revealed that the LHFP protein belongs to a new protein family consisting of at least four or five members. In the lipoma studied, the expressed HMGIC/LHFP fusion transcript encodes the three DNA binding domains of HMGIC followed by 69 amino acids encoded by frame-shifted LHFP sequences. LHFP is the second translocation partner of HMGIC identified in lipomas and represents a candidate target gene for lipomas with 13q aberrations.

Adult↗

Lipoma of the corpus callosum associated with frontal and facial anomalies.

Seven patients with a corpus callosum lipoma associated with a frontal cranial defect with or without a superficial lipoma or a fronto-nasal dysplasia, were encountered over a period of 12 years. This group of patients is reported as these associated lesions may provide a lead to the cause of lipomas of the corpus callosum as well as of the different lesions in the fronto-facial region. From our experience and that of others, the frontal lesion may be an extracranial lipoma, a frontal bone defect, with or without an external lipoma, a lipomeningocele connecting the extracranial lipoma through a frontal defect with the corpus callosum lipoma, a frontal encephalocele. Fronto-nasal dysplasia associated with a corpus callosum lipoma, seems to form an independent group although some of these patients may have other extracranial lesions as well. It is suggested by the authors that as the primitive mesenchyme gives rise to the meninx primitiva as well as to the fronto-facial skeleton a disturbance of the neural crest may give rise to these combined lesions. Further analysis of similar cases may indicate the timing of these events, and perhaps reveal a common causative factor.

Adult↗

Septum-like structures in lipoma and liposarcoma: MR imaging and pathologic correlation.

OBJECTIVE: To investigate the septum-like structures in predominantly lipomatous tumors, by correlating fat-suppressed MR images with histopathologic findings. DESIGN AND PATIENTS: The MR findings of three cases of well-differentiated liposarcoma (atypical lipoma), one case of lipoma-like component of dedifferentiated liposarcoma, and nine cases of lipoma were analyzed. T1-, T2-, and fat-suppressed T1-weighted images after Gd-DTPA administration were obtained. Surgical specimens from five patients (four with liposarcoma and one with lipoma) were also scanned with a MR unit, and compared with the pathologic findings. RESULTS AND CONCLUSIONS: Enhancement features of lipoma and liposarcoma were well visualized on fat-suppressed T1-weighted images after Gd-DTPA administration. The septum-like structures of liposarcoma are thick and enhanced considerably, while septa of lipoma are thin and enhanced only slightly. Pathologically, the septum-like structures of liposarcoma contained muscle fibers and the septa of lipoma represented fibrous capsule. Identification of well-enhanced septa in a predominantly lipomatous tumor helps to differentiate malignant tumors from lipomas. As the septum-like structures of liposarcoma contain a skeletal muscle component the tumor might need more extensive surgical procedures including resection of adjacent muscles.

Aged↗

Antenatal diagnosis of spinal lipomas.

OBJECT: The purpose of this study was to highlight the key prenatal diagnostic features of fetal spinal lipomas. METHODS AND RESULTS: All fetuses in whom a spinal lipoma was suspected prenatally or diagnosed postnatally were included in the study. Lumbosacral lipoma was diagnosed in 16 fetuses (group 1), but at birth 3 of these were found to have other forms of occult dysraphism. In 2 other fetuses (group 2) a meningocele was suspected but at birth these lesions were found to be lumbosacral lipomas. The lipomas were covering large meningoceles in 13 fetuses and filling the cul-de-sac in 2 others. Three sets of parents decided on termination. Of the 13 children born with a spinal lipoma, 9 were operated on and 4 were managed conservatively. At the last follow-up, 7 were neurologically intact. CONCLUSION: The distinction between meningomyelocele and spinal lipoma is possible antenatally. The differential diagnosis between lipoma and the other forms of occult dysraphism is more difficult.

Diagnosis, Differential↗

Thoracic intradural extramedullary lipomas. Report of three cases and review of the literature.

BACKGROUND: Indication for surgery and the surgical strategy for treatment of spinal intradural lipomas are still discussed controversially. Among spinal lipomas the cervicothoracic region is rarely affected. We report on our experience with spinal decompression for the treatment of three intradural thoracic lipomas. FINDINGS: Three adult patients with extramedullary intradural lipomas of the thoracic spinal cord demonstrated progressive neurological symptoms and signs without evidence of growth of the lipoma. All patients underwent surgery with decompression of the affected spinal levels. No attempt was made to reduce the size of the lipomas. Postoperatively, each patient demonstrated significant clinical improvement. Interpretation. Attempts to remove lipomas are associated with significant risks of surgical morbidity. Lipomas are hamartomas which change their size according to alterations of body fat. Therefore, decompression of the affected spinal levels is sufficient to achieve significant neurological improvement.

Adult↗

Lipoma of the breast: a diagnostic dilemma.

Lipoma of the breast often causes diagnostic and therapeutic uncertainty. Clinically it may be difficult to distinguish a lipoma from other conditions. Fine-needle aspiration cytology (FNAC) is often not helpful. Both mammography and ultrasound scanning are often negative. For the present study, 108 women with a clinical diagnosis of lipoma were enrolled prospectively. The clinical diagnosis of lipoma was found to be incorrect in 25.0% of these cases. Mammography and ultrasound revealed a lipoma in only 3.0% and 21.0%, respectively. FNAC revealed only fat cells in 74.0% of cases. In all, only 9 patients (11.4%) fulfilled the triple diagnostic criteria, theoretically making tumour excision mandatory in the remaining cases. Our proposal for management is for any clinical diagnosis of lipoma to be confirmed by either FNAC revealing fat cells or a core biopsy consistent with a lipoma. The mammogram and the ultrasound need not necessarily demonstrate a lipoma, but obviously must not show anything to raise the suspicion of malignancy at the site. If these criteria are met it is not necessary to excise the tumour.

Adult↗

Intracranial lipomas--a clinical study.

OBJECTIVE: This study aimed to investigate the localization of the lipoma, as well as associated intracranial and extracranial lesions in 14 patients immediately following hospital admission. The pathological findings from the neurological examinations of these patients are also investigated. METHODS: Fourteen patients who were admitted to our clinic with a variety of symptoms and diagnosed with intracranial lipoma were included in the study. Problems presented upon admission, neurological findings, and other existing system abnormalities were evaluated. Localization of the lipomas and accompanying pathologies were determined by using computerised tomography and magnetic resonance imaging. RESULTS: The most frequent reasons for admission of patients with intracranial lipomas were: headache 7 (50%), trauma 3 (21.5%), epilepsy 3 (21.5%) and one with symptoms due to the local mass effect of tumor (7%). Although the pericallosal region is accepted as the region where lipomas commonly occur, this study found the most frequent occurrence in the quadrigeminal cistern. Intracranial lipoma calcification was only evident in 1 of the 14 patients. In addition, contrary to the expectations, intracranial and extracranial lesions accompanying lipomas were rare. All patients received systematic treatment. CONCLUSION: This study showed that intracranial lipomas are more frequent in the quadrigeminal region of the brain; most are asymptomatic, generally caught incidentally; and accompanying intracranial and extracranial pathologies are less common than expected.

Adolescent↗

Expression of reciprocal fusion transcripts of the HMGIC and LPP genes in parosteal lipoma.

Parosteal lipomas are rare benign neoplasms of adipose tissue that exhibit a contiguous relationship with the periosteum. These lipomas of the bone share some histopathologic features with their commonly occurring soft tissue counterparts. The latter are well-characterized cytogenetically, primarily by rearrangements involving chromosome region 12q13-q15. In particular, translocations involving 12q13-q15 are prominent, with chromosomal region 3q27-q28 as the most frequent translocation partner. Recently, we established that the genes HMGIC at 12q15 and LPP at 3q27-28 are affected by the 3;12-translocation and demonstrated that, as a direct result, HMGIC/LPP and LPP/HMGIC fusion transcripts are expressed in soft tissue lipomas. In this study, cytogenetic and molecular analyses revealed similar findings in a parosteal lipoma. Specifically, a t(3;12)(q28;q14) was detected cytogenetically in a parosteal lipoma from a 51-year-old female and subsequently confirmed by FISH utilizing a chromosome 3 breakpoint spanning YAC probe and chromosome 12 breakpoint flanking cosmid probes. RT-PCR analysis showed expression of HMGIC/LPP and LPP/HMGIC fusion transcripts in this parosteal lipoma; nucleotide sequence analysis revealed that these transcripts are identical to those expressed in soft tissue lipomas characterized by a 3;12-translocation. These findings lend further support to a common histopathogenesis between lipomas of soft tissue and parosteal origin.

Bone Neoplasms↗

Lipomas and fibrolipomas of the oral cavity.

The clinical and histological characteristics of 19 lipomas and fibrolipomas of the oral cavity are presented. Data on age, sex, location, clinical appearance, duration, recurrence and histological diagnosis are reviewed. The data gave a slightly higher incidence of oral lipomas in comparison with lipomas of the whole body (2.2%), and with benign tumours of the oral cavity (2.4%) than reported in the literature. As with many tumours the aetiology of lipomas remains obscure. It is though that repeated mild trauma may trigger the proliferation of fatty tissue and finally causes a lipoma. In 1971 Hatziotis reviewed the literature from 1945 to 1967 and found 145 cases of intraoral lipomas. The literature up to 1981 has been surveyed and 80 additional cases were found. These 225 oral lipomas and fibrolipomas were reviewed. Sixty-six per cent of the tumours occurred in patients in the fifth to the seventh decade. The male-female ratio for lipomas was 1.5:1, and for fibrolipomas 1:1,3. In most cases the only symptom was a painless, palpable tumour. The time between the initial discovery and removal was on average 4 years and 9 months. The cheek was the most favoured site, followed by the tongue, floor of mouth and buccal sulcus and vestibule equally, lip, palate, gingiva and retromolar area. Surgical excision is the treatment of choice and, as far as is known, recurrence has only once been noticed.

Adult↗

Sequence analysis of the MEN I gene in two patients with multiple cutaneous lipomas and endocrine tumors.

The discovery of mutations of the menin gene in a few multiple endocrine neoplasma type 1 (MEN I)-associated lipomas and loss of heterozygosity (LOH) on chromosome 11q13 in some sporadic lipomas has stimulated the hypothesis that lipomas may belong to the group of sporadic tumors caused by defects of the gene responsible for MEN I. Since it is unclear if the above hypothesis applies to all patients with lipoma or just to specific subsets, we searched to enlarge the database on this topic. For this purpose, we identified two patients with multiple cutaneous lipomas. One had an additional pituitary adenoma and familial presentation of multiple lipomas, the other had recurrent goiter in the setting of a family history of adenomatous goiter. Deoxyribonucleic acid (DNA) was analyzed by complete direct DNA sequencing of all coding exons and splice junctions of the MEN I gene. No mutation was identified in the coding exons of the menin gene. In contrast to former data on sporadic lipomas, these data are the first to render evidence that mutations of the MEN I gene may not be responsible for the formation of multiple lipomas, even if they appear in the context of other endocrine tumors.

Adenoma↗

Endoscopic removal of large colonic lipomas.

BACKGROUND: Colonic lipomas are benign adipose tumors that rarely cause symptoms. Removal of lipomas 2 cm or greater in diameter has been associated with a greater risk of perforation. Experience with the evaluation and removal of large colonic lipomas with the assistance of EUS to reduce the risk of perforation is reported. METHODS: Four patients with large colonic lipomas were evaluated and treated with endoscopic methods. EUS was performed to confirm that the lesion was a lipoma superficial to the muscularis propria. Saline or epinephrine solution was injected at the base of the tumor, which was then resected electrosurgically with a snare. OBSERVATIONS: All 4 lesions were successfully removed and histopathologically confirmed to be lipomas (mean maximal diameter 2.8 cm). There were no complications of any procedure. One patient was hospitalized overnight because of abdominal pain that resolved without complication. CONCLUSIONS: Large colonic lipomas can be removed safely by electrosurgical snare resection after injection of the base with epinephrine or saline solution. EUS should be used to ensure that the lipoma does not extend into the muscularis propria. Clinical judgment is important in assessing the need to remove these lesions.

Adult↗

Pathogenesis of intracranial lipoma: an MR study in 42 patients.

Intracranial lipomas are uncommon lesions whose development remains poorly understood. To clarify the anatomic and embryologic features of intracranial lipomas, we retrospectively reviewed the MR scans of 42 patients with 44 intracranial lipomas. Interhemispheric lipomas were the most common, accounting for 45% of cases. The remainder of the lesions were clustered in the quadrigeminal/superior cerebellar (25%), suprasellar/interpeduncular (14%), cerebellopontine angle (9%), and sylvian (5%) cisterns. Fifty-five percent of the lesions were associated with brain malformations of varying degrees. Intracranial vessels and nerves were noted to course through 16 (36%) of the lesions. The relative frequencies of the locations of the lipomas correspond to the temporal sequence of dissolution of the meninx primitiva, the mesenchymal anlage of the meninges. This finding supports the concept of lipoma formation as a result of abnormal persistence and maldifferentiation of the meninx. This embryologic concept of the development of intracranial lipomas explains the high frequency of callosal and other brain hypoplasias. Intracranial lipomas are neither hamartomas nor true neoplasms; rather, they are congenital malformations.

Adolescent↗

Spinal lipomas.

Lipomas of the spinal cord are among the most fascinating lesions encountered by the pediatric neurosurgeon. An understanding of spinal lipomas may, however, be difficult because the terminology used to describe the accumulations of spinal fat is confusing, inconsistently applied, and at times contradictory. An anatomical characterization of lipomas may assist in understanding these lesions. Lipomas of the spinal cord are very rare and cause symptoms related to mass effect and secondary compressive myelopathy. Lipomas of the conus medullaris (or lipomyelomeningocele) are the most common form of fatty masses in the spine and can be divided into dorsal, caudal, and transitional forms. These lesions are a manifestation of occult spinal dysraphism and a common cause of the tethered cord syndrome (TCS). The natural history of untreated lipomyelomeningocele, although incompletely understood, appears to be progressive neurological deterioration with loss of bladder control. Timely, careful surgical intervention may prevent significant neurological deterioration and progressive disability in the majority of children harboring these lesions. In surgical intervention the surgeon seeks to disrupt the connection between the fibrofatty mass and underlying cord as well as to reestablish normal anatomical planes. Several intraoperative video segments illustrating lipomyelomeningocele resection are included in this paper. Lipomas of the terminal filum (fatty filum) are truly occult and are also associated with TCS. Surgical treatment of filum lipomas carries significantly lower risk than that for lipomas of the conus medullaris. Again, the goal of surgery is to disrupt the connection between the abnormal fibrofatty tissue and the underlying spinal cord.

Biomechanical Phenomena↗