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A group of patients with retroperitoneal liposarcoma is presented and general guidelines of management reviewed. It is suggested that aggressive, radical total surgical excision followed by adjuvant postoperative irradiation be used as the treatment of choice. Although the management of recurrent disease may be discouraging in terms of ultimate cure, reasonable palliation and prolonged survival may result from aggressive therapy.
A previously healthy 35-year-old man experiencing slowly progressive, painless proptosis of the right eye. Visual function was normal, but supraduction was limited. Computed tomography revealed a superior, extraconal orbital mass. Subtotal excision was performed, and a diagnosis of liposarcoma was rendered only with expert analysis. Despite subsequent orbital exenteration and postoperative radiation, a local recurrence developed 5 years later. The clinical features that predict recurrence, and management options that may promote longevity, are discussed.
CASE REPORT: A 56-year-old man with widely metastatic liposarcoma, after left Tower extremity amputation, complained of severe right lower extremity pain. Trials of systemic opioids had resulted in poor pain control while introducing intolerable dose-limiting side effects. METHODS AND RESULTS: Initial inpatient management consisted of a lumbar epidural infusion of a dilute local anesthetic and preservative-free morphine. This provided satisfactory relief but was discontinued because of recrudescence of phantom limb pain. A lumbar epidural infusion of preservative-free morphine sulfate was associated with poor pain relief, central nervous system (CNS) side effects, and severe urinary retention resulting in acute renal failure. A repeated trial of parental opioids provided marginal pain relief with persistent CNS side effects. Chemical neurolysis of the lumbar plexus was performed with 10 ml of 10% aqueous phenol injected into the psoas muscle sheath. The pain gradually resolved over a 2-day period without apparent side effects. Motor function was preserved, pain was resolved, and as systemic opioids were reduced, cognitive function and overall well-being were improved.
The effectiveness of Tc-99m bleomycin (BLM) and Tc(V)-99m DMSA are compared with that of Ga-67 citrate, which is currently the most widely used agent. In four patients with lipomatous tumors, the clinical significance of tumor imaging with each of these three agents is discussed and compared. Results indicate that both Tc-99m BLM and Tc(V)-99m DMSA are superior in detecting the extension or localization of liposarcomas.
Two cases of pregnancy associated with myxoid liposarcoma are presented. Both patients were treated with hyperthermoradiotherapy after the induced delivery of healthy infants and by surgical excision of the tumor 5 to 7 weeks after delivery. No local recurrence or distant metastasis occurred; the children have progressed normally for 4 and 5 years, respectively, after surgery. Because coexistence of pregnancy and sarcoma is rare, it is difficult for the clinician to develop an appropriate approach to the management of bone and soft tissue sarcoma during pregnancy. The literature was reviewed regarding the effect that pregnancy may have on the clinical behavior of sarcoma and the effect treatment for sarcoma may have on the fetus.
Encapsulated fat necrosis is a degenerative process involving various mature adipose tissues. We report on a case of abdominal encapsulated fat necrosis which mimicked abdominal liposarcoma, and we discuss its imaging findings on computed tomography (CT).
We present a report of a patient with bilateral lower extremity lipodystrophy and lymphedema who underwent excision of a large extremity liposarcoma. Total excision of the tumor was performed with no evidence of recurrence to date. The natural history, characteristics, and management of this tumor are discussed. A high index of suspicion and awareness among surgeons and pathologists should allow accurate diagnosis and treatment of this condition.
Myxoid liposarcoma of the head and neck is an extremely rare entity. The scalp region represents a risk factor to the patient because the diagnosis is usually made late, and the surgeon must have a high index of suspicion for this entity because suctioning the tumor without taking a biopsy further delays an accurate diagnosis.
Liposarcoma of the spermatic cord is rare and easily masquerades as a more common benign neoplasm of the spermatic cord. If neglected, these malignancies may grow to tremendous size and become formidable management problems. The treatment of this malignancy is orchiectomy and en bloc removal of the tumor. More radical operative therapy has not generally proved beneficial. Adjunctive therapy may be helpful in selected cases.
Two patients with metastatic liposarcoma to the spine developed neurologic deficits that were successfully reversed by laminectomy and partial tumor resections. Recurrences at later dates were treated similarly with comparable outcomes. Paraplegia was forestalled for approximately two years by each of the surgical interventions.
The fusion oncoproteins, TLS-CHOP and EWS-CHOP, are characteristic markers for myxoid and round cell liposarcomas (MLS/RCLS). Especially, the peptide sequence of 26 amino acids corresponding to the normally untranslated CHOP exon 2 and parts of exon 3 (5'-UTR) is a unique structure for these chimeric proteins. In this report, we have generated monoclonal antibodies against the unique peptide sequence of TLS/EWS-CHOP oncoproteins. These antibodies reacted with TLS-CHOP fusion protein, but not reacted with normal TLS and CHOP proteins by Western blot analysis. In addition, one of the antibodies also recognized the chimeric oncoprotein in archival paraffin-embedded tissue samples of MLS/RCLS. The oncoprotein was detectable by the antibody even in the paraffin-embedded tissue samples whose mRNAs were too degraded to be detected by a nested reverse transcription-polymerase chain reaction-based assay. Thus, the molecular assay using the novel antibody is expected to be one of the most sensitive diagnostic assays for MLS/RCLS.
OBJECTIVE: To determine the prognostic significance of histologic subtype in a large series of patients with primary liposarcoma (LS) and to construct a LS-specific postoperative nomogram for disease-specific survival (DSS). SUMMARY BACKGROUND DATA: Nomograms, used to define and predict outcome following operative intervention, may contain variables not conventionally used in standard staging systems. A 12-year DSS postoperative nomogram for all sarcomas has already been established. METHODS: From a single-institution prospective sarcoma database, patients with primary extremity, truncal, or retroperitoneal LS treated between 1982 and 2005 were identified. Histology was reviewed by a sarcoma pathologist and divided into 5 subtypes. A nomogram predictive of 5- and 12-year DSS was developed. RESULTS: Of 801 patients with primary LS resected with curative intent, 369 (46%) presented with well-differentiated, 143 (18%) dedifferentiated, 144 (18%) myxoid, 81 (10%) round cell, and 64 (8%) pleomorphic histology. The median tumor burden was 15 cm (range, 1-139 cm). At last follow-up, 560 patients were alive with a median follow-up time of 45 months (range, 1-264 months) and 51 months for surviving patients. The 5- and 12-year DSS rates were 83% (95% confidence interval [CI], 80%-86%) and 72% (95% CI, 67%-77%), respectively. The nomogram was drawn on the basis of a Cox regression model. The independent predictors of DSS were age, presentation status, histologic variant, primary site, tumor burden, and gross margin status. The nomogram was internally validated using bootstrapping and shown to have excellent calibration. The concordance index was 0.827 compared with 0.776 for the general sarcoma postoperative nomogram for 12-year DSS. CONCLUSION: The LS-specific nomogram based on histologic subtype provides more accurate survival predictions for patients with primary LS than the previously established generic sarcoma nomogram. DSS nomograms aid in more accurate counseling of patients, identification of patients appropriate for adjuvant therapy, and stratification of patients for clinical trials and molecular analysis.
Following intradermal inoculation of bovine papillomavirus type 4 (BPV-4) into a Syrian hamster, a liposarcoma developed at the inoculation site 20 months later. The DNA of this tumour contained multiple copies of the BPV-4 genome which existed in a free unintegrated state. Unintegrated viral DNA and viral DNA isolated from virus particles from bovine alimentary tract papillomas revealed identical cleavage patterns with CpG methylation-resistant and -sensitive restriction enzymes: apparently there was no gross methylation of CpG sites in either case. The entire BPV-4 genome appeared to be represented in the tumour DNA.
Cytogenetic and gene analyses were performed in a child with myxoid liposarcoma (MLS). A reciprocal chromosome translocation t(12;16)(q13;p11) was found in the tumor cells. This result, combined with the previous reports of a similar translocation in adult MLS strongly suggests that this translocation may be a characteristic cytogenetic marker in MLS. The human int-1 gene has been reported to be located close to the 12q13 breakpoint associated with MLS. Therefore, we examined the rearrangement of the human int-1 gene by Southern blotting analysis. When genomic DNAs from the tumor cells were digested with KpnI, EcoRI and BamHI, no difference was seen compared to peripheral blood leukocytes (PBL) DNAs from a normal individual. However, with HindIII digestion there appeared a 3.1 kb fragment in tumor cell DNA as compared to a 2.8 kb fragment in DNAs prepared from normal PBL and the patient's PBL. These findings suggest that the int-1 gene may be implicated in tumorigenesis of MLS with t(12;16)(q13;p11).
The cytological features of 18 myxoid liposarcomas from 12 patients were analysed. The most consistent cytological features were: abundant myxoid matrix, several small tissue fragments with uniform small, round or stellate cells and a network of branching thin-walled capillaries. The presence of univacoulated or multivacuolated lipoblasts was noted frequently, but not in all aspirates. The diagnostic importance of vacuolated lipoblasts and the main problems of differential cytodiagnosis are discussed.
A case of the unusual condition of liposarcoma of the hypopharynx extending submucosally down the oesophagus in a 64 year old male is reviewed. The clinical, radiological and pathological features are presented and the role of various radiological modalities is discussed.
A 57-year-old female with a large retroperitoneal tumor was atutopsied. She showed through the entire course of illness marked leukocytosis with the appearance of immature marrow cells, and this finding was hematologically interpreted as granulocytic leukemoid reaction. An intermittent pyrexia was also seen without infective etiology. The histologic diagnosis of the tumor was liposarcoma of the pleomorphic type. The tumor showed extensive necrosis, but no metastatic invasion was found. So-called leukemoid reaction appears sometimes together with malignant neoplasms. The majority of these tumors show widespread bone marrow metastases, and without bone marrow invasion they only rarely induce this reaction. Besides it is not common for leukemoid reaction to be evoked by the development of malignant mesenchymal tumors except for malignant lymphomas. The pathophysiologic process of the present of the present case is thought to be a rare occurrence.
An electron-microscopic study of a myxoid liposarcoma is presented in which amorphous electron-dense material and crystalline inclusions in the granular endoplasmic reticulum were observed. These inclusions showed transversal striations and were associated with round electrodense bodies. The previous finding of similar structures in normal cells from vertebrates and plants without relationship to adipose tissue discards the direct association of these inclusions with this particular tumor and suggests a possible functional origin.