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The case of a 78-year-old patient suffering from a liposarcoma of the uterus is described. This malignant tumour which belongs to the group of mesenchymal tumours is very rare. The patient underwent an abdominal hysterectomy with adnexectomy on the left. Since then she has been regularly controlled for more than three years now and no recurrence or metastasis has been found.
A case of liposarcoma of the mediastinum in reported and a review of the literature is given. To our knowledge 53 cases have been described in the literature. The most common symptoms are dyspnea, chest-pain, a cough and loss of weight. There is no relation between operability, the duration of clinical symptoms and the size of the tumor. The final diagnosis was made by histological examination in all cases. A clinical classification of the tumor is not possible, because there are no characteristic clinical findings. The prognosis in general depends on the histological type of the malignant. Although recurrence of the tumor appears in more than 40% of cases, radical surgical resection is the only method of successful treatment. The significance of the five-year survival rate is doubtful, because recurrent tumors were reported up to 14 years after the initial surgical procedure.
The concept that selective transfer of high density lipoprotein (HDL)-derived cholesteryl esters (CE) does not require lipoprotein internalization has been challenged recently by evidence that implicates HDL recycling during the selective uptake process. This has prompted us to examine the role of the low density lipoprotein receptor-related protein (LRP) in selective uptake. LRP is an endocytic receptor for lipoprotein lipase (LpL) and apolipoprotein E (apoE) ligands that are able to mediate selective uptake. We report that molecules that interfere with ligand binding to LRP, such as the receptor-associated protein (RAP), suramin, alpha(2)-macroglobulin, or lactoferrin, inhibit HDL-CE selective uptake by human primary adipocytes and SW872 liposarcoma cells by 35-50%. This partial inhibition of selective uptake from total HDL was not due to preferential inhibition of the HDL(2) or HDL(3) subfractions. Selective uptake by the scavenger receptor BI was not inhibited by RAP, excluding its involvement. Furthermore, in SW872 cells in which LRP was reduced to 14% of control levels by stable antisense expression, selective uptake was attenuated by at least 33%, confirming a role for LRP in this process. RAP, alpha(2)-macroglobulin, lactoferrin, and suramin (individually or in paired combinations) also attenuated selective uptake of HDL-CE by primary human adipocytes by about 40%. On the other hand, human skin fibroblasts express LRP abundantly but lack the capacity for selective uptake, demonstrating that other molecules are required. In SW872 cells, exogenous apoE or LpL can facilitate selective uptake but only the apoE-enhanced uptake can be inhibited by RAP, implicating apoE as a likely co-mediator. We discuss the possible mechanisms by which the endocytic receptor, LRP, can mediate selective uptake.
A specific TLS-CHOP fusion gene derived from the t(12;16) is present in at least 95% of myxoid/round cell liposarcomas (MLS). Rare cases of MLS show a variant t(12;22) translocation, resulting in EWS-CHOP fusion gene. The CHOP gene encodes a leucine-zipper transcription factor, which is implicated in both oncogenic transformation and inhibition of adipogenesis. To examine whether the molecular variability of TLS-CHOP or EWS-CHOP fusion transcript structure is associated with the degree of inhibition of adipogenesis, a comparative ultrastructural and RT-PCR-based analysis of 14 MLS was performed. The specimens consisted of 9 primary, 2 locally recurrent tumors, and one representative sample each from 3 patients with multifocal soft tissue metastases. Histologically, there were 8 high-grade and 6 low-grade MLS using 5% round cell (RC) component as the cutoff point. By RT-PCR assay there were 13 cases with TLS-CHOP fusion transcripts: 7 cases of type 5-2 (known as type II), 4 cases of type 7-2 (known as type I),1 case of type 8-2 (known as type III), and 1 unique case of type 6-2. The remaining 1 case showed an EWS-CHOP fusion transcript. Ultrastructural examination revealed that tumor cells were composed of a moderate-to-predominant proportion of well-formed lipoblasts in 4 cases, while in 6 cases such lipoblasts were very scant. The remaining 4 tumors were arrested in the stage of transitional cells. The heterogeneity of TLS-CHOP fusion transcript showed no apparent impact on adipogenesis, since both TLS-CHOP type I and II cases could randomly display various levels of lipoblastic differentiation. Furthermore, the 4 cases without definite lipoblasts showed no preference for any specific fusion variants and consisted of one each of TLS-CHOP subtypes. In addition, the fusion transcript variants did not correlate with other ultrastructural features, such as the presence and amount of glycogen, mitochondria, rough endoplasmic reticulum, vimentin-like intermediate filaments, and external lamina. However, there appeared to have a trend suggesting the predilections of glycogen particles and vimentin-like intermediate filaments in primitive mesenchymal cells and/or transitional cells. These findings cannot substantiate the hypothesis that the molecular variability of fusion transcripts has a biological impact on adipogenesis of MLS, and other factors might be implicated in their level of differentiation.
Malignant lipomatous tumours of the hands are extremely rare. A 62-year-old man complained of an enlarging, soft, painless mass in his left hand. Magnetic resonance imaging showed a fatty tumour with irregular septa, and an incisional biopsy showed it to be a well-differentiated liposarcoma. The tumour was resected en bloc and he had a course of external beam radiotherapy. Apart from a slight reduction in grip strength he was well 15 months later with no sign of recurrence.
A retrospective observational study was performed on a series of 12 consecutive pediatric patients treated over a 20-year period at the Istituto Nazionale Tumori, Milano. Conservative surgery was the treatment of choice in all patients; radical excision was obtained at diagnosis in 9 cases and after primary chemotherapy in 1 case. Five patients were subjected to surgery alone, and one to postoperative radiotherapy. After a median follow-up of 11 years (range 1-20), all the patients were alive without evidence of disease, 11 in first complete remission, and 1 after local relapse. In agreement with other reports, the authors underline the unquestionable pivotal role of radical surgery in the treatment of liposarcoma. The high proportion of resectable tumors accounts for the excellent survival of the patients in this study. The role of both adjuvant chemotherapy and radiotherapy is uncertain and awaits multicentric cooperative prospective studies.
Two cases in which myxoid liposarcomas were examined by use of light and electron microscopy are presented. The relationship of this tumor to the development of normal adipose tissue is discussed. Most of the ultrastructural features were identical to those of developing white adipose tissue. Features not typical of developing white adipose tissue, such as congeries of 60-Angstrom filaments and large masses of glycogen, are also illustrated and discussed.
A case of recurrent sarcoma arising from the stroma of the thymus and having the microscopic features of well-differentiated and pleomorphic liposarcoma is presented. No previous reference was found in the literature to this tumor type, which could be viewed as the malignant counterpart of thymolipoma.
A benign massive retroperitoneal lipomatous tumor is described. The tumor, from a 72-year-old woman with increasing abdominal girth, consisted of a mixture of mature lipocytes, smooth muscle cells, and thick-walled medium-size blood vessels. Although the tumor focally involved the uterine serosa, suggesting the possible diagnosis of uterine leiomyoma with fatty change, the authors think that, because the bulk of the tumor was located in the retroperitoneum and because the tumor contained characteristic thick-walled blood vessels from which smooth muscle cells radiated, this tumor would be best classified as a retroperitoneal angiomyolipoma. Although both angiomyolipomas and leiomyomas with fatty change presenting as large retroperitoneal tumors are rare, and therefore are not well-recognized by surgical pathologists, they are benign and must be distinguished from liposarcomas.
We report a case of a rapidly progressing isolated giant metastatic myxoid liposarcoma to the heart in a 54-year-old man, who presented with acute symptoms of cardiac tamponade. Surgery remains the best treatment option for this rare condition.
Myxoid liposarcoma (ML) has a high predilection for extrapulmonary sites of metastases, including intra-abdominal metastases, but pancreatic involvement is extremely rare. Here, we report the case of a 66-year-old male patient, who underwent pancreaticoduodenectomy for isolated pancreatic metastasis of ML of the left lower extremity that had been excised 6 years before. Completion pancreatectomy was necessitated afterwards for a delayed haemorrage associated with pancreatic fistula. Currently the patient is alive with no evidence of disease. Highly selected patients with isolated pancreatic metastasis of soft tissue sarcoma may benefit from a curative surgical resection.
Little is known about the regulation of apolipoprotein (apo) C-I production by human adipocytes. The aim of the present study, therefore, was to investigate the effect of different tissue culture conditions on the synthesis and secretion of apoC-I and apoE in human SW872 liposarcoma cells. After 3-4 d in culture (0.5 x 10(6) cells/well, DMEM/F-12 medium with 10% fetal calf serum), cells reached confluence and became growth arrested. The molar ratio of apoE:apoC-I in the cell was 8.9 +/- 0.6 and in the medium was 6.6 +/- 0.5. After 17 d in culture, SW872 cells contained significantly more cholesterol (100%) and triglyceride (3-fold) and secreted more apoC-I [4 vs. 17 d: 0.11 +/- 0.01 vs. 0.23 +/- 0.01 pmol/(10(6) cells . 24 h), P < 0.001] and apoE [0.7 +/- 0.1 vs. 3.1 +/- 0.3 pmol/(10(6) cells . 24 h), P < 0.001]. Cellular apoC-I increased 7-fold and apoE increased 16-fold. Cell maturation was associated with significantly higher levels of apoE mRNA but not apoC-I mRNA. Increases in cell lipids, apoC-I, and apoE were not dependent on the presence of extracellular lipids because similar changes occurred in cells incubated with lipoprotein-deficient serum or in cells incubated without serum. Treatment (7 d) of cells during maturation with insulin (10 or 1000 nmol/L) significantly reduced the secretion of apoC-I and apoE. These results demonstrate that in maturing SW872 cells, cholesterol and triglyceride accumulation in the presence or absence of extracellular lipids, is associated with increased apoC-I and apoE production. Furthermore, apoC-I and apoE production are differentially regulated at the transcriptional level, and long-term treatment with insulin has an inhibitory rather than stimulatory effect on apoC-I and apoE production.
Epidermal inclusion cysts are an exceedingly common entity seldom seen in association with a malignant tumor. Herein, we report a unique case of an epithelial inclusion cyst seen in association with an atypical lipomatous tumor/well-differentiated liposarcoma. The epidermal inclusion cyst was delimited to the dermis and circumferentially enveloped by an atypical adipocyte tumor containing myxoid foci and comprised of lipoblasts. This case underscores the importance of scrutinizing the entirety of cysts and other ostensibly trivial dermal entities to avoid the pitfall of misdiagnosing a potentially serious tumor.
Metastatic soft-tissue sarcoma of the central nervous system (CNS) is exceedingly rare. We report a case of a 56-year-old male treated for a right lower extremity liposarcoma at the age of 30 years, whose first recurrence was an intracerebral metastasis occurring 26 years after resection of the primary tumor. Initial treatment of the metastasis with surgical resection and adjuvant radiotherapy was followed rapidly by CNS recurrence in 3 months. Further debulking and interstitial brachytherapy were unsuccessful in controlling disease progression. Clinical presentation and treatment of brain metastases in soft-tissue sarcoma are discussed.
Soft-tissue sarcomas of the digit are uncommon. We herein report on a patient with a de-novo subungual right thumb liposarcoma with subsequent failure in the brain. The pertinent literature and recommendations for management are presented.
A liposarcoma arose in the kidney of a 52-year-old man. After 13 years, it recurred, was resected, and recurred again. The second recurrence was inoperable.
We studied 28 cases of anterior mediastinal liposarcoma occurring in 16 males and 12 females with a mean age of 43 years (range, 14-72). Presenting symptoms included dyspnea (four cases) and chest pain (four cases), although 11 tumors were detected incidentally by routine chest radiography. Seven cases were believed to be located within the thymus. Most (i.e., 25) of the cases were of low grade, with the well-differentiated lipoma-like or sclerosing subtypes constituting 60% and the myxoid subtype constituting 28%; the remaining 12% exhibited mixed features. Three cases were pleomorphic type. Several low-grade tumors exhibited widespread, dense aggregates of mature-appearing lymphocytes and plasma cells, which occasionally obscured the mesenchymal nature of the neoplasm, suggesting instead a lymphoid neoplasm or a reactive fibroinflammatory condition. The three high-grade tumors showed combinations of pleomorphic and round cell patterns, with focal myxoid areas. Of the cases grossly arising within the thymus, only one showed extensive thymic tissue within the lesion ("thymoliposarcoma"); six others exhibited residual thymus peripheral to the tumor. Clinical follow-up in 23 cases revealed recurrence in seven patients (31.8%), with a mean interval to recurrence of 3 years. Eight patients died (mean survival, 2.6 years), one postoperatively and three following a recurrence. Fifteen patients were alive (mean survival, 2 years), four with recurrent tumor. The myxoid tumors had a somewhat more aggressive course than the well-differentiated tumors. Metastases were not observed in any of the patients.
We report 14 cases of a soft tissue lesion in the limbs of morbidly obese adults that presents as a large mass and histologically simulates well-differentiated liposarcoma (WDL). Based on its distinctive clinical setting and morphologic identity to diffuse lymphedema we have termed this process massive localized lymphedema (MLL). All cases occurred in morbidly obese adults (mean weight 372 lbs; mean age 47 years). Women predominated (9 women; 5 men). The lesions affected the proximal medial aspect of the extremities (12 thigh; 2 arm) and were unilateral in all but two patients. Etiologically significant antecedent events include ipsilateral axillary lymphadenectomy in both patients with arm lesions, chronic lymphedema resulting from vein-stripping 10 years prior in one patient. inguinal lymphadenectomy for anal carcinoma in another patient, and significant blunt trauma to the inner thigh during a motor vehicle accident in a third patient. The tumors were long standing ( I-IO years) and extremely large (mean size 33.4 cm, 7408 g). Clinically, they were diffuse, ill-defined masses that histologically consisted of lobules of mature fat interrupted by expanded connective tissue septa. The constituents of the septa were fine, fibrillary collagen, edema fluid, and uniformly distributed fibroblasts. Clusters of capillaries were frequently found at the interface between fat and connective tissue. The widened septa simulated the fibrous bands of sclerosing WDL, but MLL lacks the degree of nuclear atypia seen in the former. The consistent clustering of reactive vessels at the interface between the fat and fibrous tissue also contrasted with WDL. Six patients experienced persistent or recurrent lesions within 10 months to 10 years. No aggressive growth or histologic progression was observed during this time, however. Awareness of the features of MLL is important to avoid misclassification of this reactive lesion with WDL.