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Dedifferentiated liposarcoma of the cheek.

Liposarcomas of the head and neck region are rare; only a few cases have been reported to arise in the cheek or buccal mucosa. Dedifferentiated liposarcoma has rarely been reported in the head and neck region and, to the best of our knowledge, this is the first reported case of dedifferentiated liposarcoma of the cheek. Dedifferentiated liposarcoma is a mixed histologic subtype defined by the association of an atypical lipomatous tumor or well-differentiated liposarcoma and a nonlipogenic sarcoma. The patient was a 61-year-old man who presented with a soft-tissue mass of the left cheek and a presumptive diagnosis of salivary neoplasm based on a fine needle aspiration. The tumor was excised and consisted histologically of an atypical lipomatous tumor/well-differentiated liposarcoma composed a well-differentiated lipomatous neoplasm with atypical cells and rare lipoblasts. The tumor recurred in the same location 5 months after surgery. The recurrent tumor was primarily composed of a nonlipogenic spindle sarcoma with focal rhabdomyoblastic differentiation associated with areas of an atypical lipomatous tumor/well-differentiated liposarcoma.

Biomarkers, Tumor↗

[Magnetic resonance tomography of liposarcoma].

PURPOSE: Liposarcoma is the most common soft tissue sarcoma in adults. The purpose of the study was to correlate MR imaging features with the four ICD subclasses. METHODS: 28 patients with liposarcoma were examined preoperatively on a 1.0 T MRI system, using spin echo sequences, gradient echo imaging and STIR sequences. In all patients, classification of the liposarcomas was made by histologic diagnosis (ICD-10-M-classification). RESULTS: Well differentiated liposarcomas (9 patients) showed a number of MRI features consistent with lipoma (well defined mass high signal intensity (SI) on T1 images, low signal on T2 images, no or only little signal increase of tumour tissue after contrast material application, no necrosis). Not well differentiated liposarcomas (myxoid 14 patients, pleomorphic three patients, round cell two patients) revealed the largest part of the mass to be of low SI on T1-weighted images. Only scattered fatty islands and septa (9 patients with myxoid liposarcoma had more SI in T1-weighted images. T2-weighted image showed most of the mass to be of intermediate to high SI are the fatty island of relatively low SI. The probability of tumor necrosis increased with lower tumour grading. CONCLUSION: MRI seems to be valuable to identify well differentiated liposarcomas.

Adult↗

Heterologous elements in the dedifferentiated component of dedifferentiated liposarcoma.

Five cases of dedifferentiated liposarcoma in which heterologous elements were present in the dedifferentiated component are presented. Two patients, men 54 and 66 years of age, had retroperitoneal atypical lipomatous tumors (well-differentiated liposarcomas) that recurred (after 2.5 and 12 years, respectively) as dedifferentiated liposarcomas with rhabdomyosarcomatous elements in the dedifferentiated components. The third patient, a 63-year-old woman, had a retroperitoneal dedifferentiated liposarcoma whose dedifferentiated component demonstrated focal osteoid formation. The fourth patient, a 36-year-old woman, had two seemingly separate retroperitoneal masses, one composed of atypical lipomatous tumor with foci of smooth muscle and the other of leiomyosarcoma; despite the apparent separation, it was thought more logical to consider this a dedifferentiated liposarcoma with leiomyosarcoma as the dedifferentiated component than two unrelated neoplasms. The fifth patient, a 45-year-old man, had a mediastinal dedifferentiated liposarcoma with angiosarcomatous areas in the dedifferentiated component. The relatively short follow-up thus far available on these cases has not demonstrated tumor behavior significantly different from that of dedifferentiated liposarcoma as a whole.

Adult↗

Upper aerodigestive tract liposarcoma: report on four cases and literature review.

OBJECTIVE: To report on the clinical behavior, histopathology, treatment, and prognosis of laryngeal, hypopharyngeal, and cervical esophageal liposarcomas. STUDY DESIGN: Retrospective reviews of pathology files and hospital records at a tertiary care hospital and a retrospective search of the English-language literature. METHODS: Cases of upper aerodigestive tract (UADT) liposarcoma with adequate histopathologic documentation and clinical information were included for review. RESULTS: Four cases of UADT liposarcomas were identified. The literature review revealed 26 cases of laryngeal liposarcomas, 7 cases of hypopharyngeal liposarcomas, and 6 cases of esophageal liposarcomas: the mean age at presentation was 55.8 years, the male:female ratio was 5:1, and 60% of the patients presented with dysphagia. Eighty-six percent of tumors had low-grade histologic findings. The recurrence rate after primary resection was 50%. Recurrence correlated with surgical procedure rather than with histologic subtype; 94.7% of recurrences happened after simple excision. Distant metastases occurred in three patients; two of them died of the disease. CONCLUSIONS: The literature supports that UADT liposarcomas are rare and usually of low-grade histologic type. The rate of metastatic disease and tumor-related mortality is low. However, high recurrence rates have been noted, particularly when less radical surgery is employed.

Adult↗

Biochemical uncovering of mdm2/p53 complexes in liposarcomas parallels their immunohistochemical detection.

Recent observations indicate the existence of pathogenetically distinct groups of well-differentiated (WD) dedifferentiated (DD) liposarcomas. In the retroperitoneal WD-DD liposarcomas, the predominant phenotype is represented by the aberrant (overexpressed) mdm2+/p53+ wild-type profile. At the nonretroperitoneal site, the WD liposarcomas present a wider association of MDM2/P53 gene expression; i.e., mdm2+/p53+, mdm2+/p53-, mdm2-/p53+ and mdm2-/p53-, and TP53 mutations seem to correlate with the dedifferentiation process. A biochemical study of mdm2-p53 association in 11 tumor samples characterized by the presence of different mdm2 and p53 immunophenotypes was performed. Immunoprecipitation assays using a p53-specific antibody were performed on tumor tissue and surrounding normal tissue; the immunoprecipitated material was then investigated for the presence of p53 (control) and of coimmunoprecipitated mdm2. This biochemical analysis showed that, in mdm2+/p53+/wild-type retroperitoneal liposarcomas, a band corresponded to mdm2 protein in the cellular lysates immunoprecipitated with a p53-directed antibody. In contrast, the mdm2+/p53- liposarcoma did not evidence the presence of mdm2 protein nor was p53 protein available to direct immunoprecipitation, as in the p53 mutant tumor samples with mdm2-/p53+ and mdm2-/p53- phenotypes. From the normal counterpart of retroperitoneal liposarcoma lysates, no p53 protein was immunoprecipitated. The findings in this study agree with the molecular data and they show the physical association of mdm2 and p53 in fresh liposarcoma surgical specimens.

Gene Amplification↗

Gene expression profile identifies a rare epithelioid variant case of pleomorphic liposarcoma carrying FUS-CHOP transcript.

AIMS: To describe a tumour with morphological and immunophenotypic characteristics of epithelioid variant of pleomorphic liposarcoma. Pleomorphic liposarcoma is a very rare variant of liposarcoma defined morphologically by the presence of pleomorphic lipoblasts showing peculiar epithelial-like features that can be confused with primary or metastatic carcinoma. METHODS AND RESULTS: Molecular analysis demonstrated for the first time the presence of FUS-CHOP transcript in this liposarcoma variant. Microarray analysis revealed a gene expression profile related to a more aggressive tumour type when compared with other myxoid/round cell liposarcomas. CONCLUSIONS: The present data show that the epithelioid variant of pleomorphic liposarcoma represents a further variant of myxoid liposarcoma sharing the FUS-CHOP fusion transcript but carrying a distinct expression profile, in keeping with its aggressive clinical course.

Antigens, CD34↗

Myxoid liposarcoma: appearance at MR imaging with histologic correlation.

Although myxoid liposarcoma is a subtype of liposarcoma, it may be difficult to establish the correct diagnosis with magnetic resonance (MR) imaging due to the lack of fat signal intensity. Without the administration of gadolinium contrast material, the tumor may even mimic a cystic tumor. A spectrum of MR imaging abnormalities occur in myxoid liposarcoma, depending on the amount of fat and myxoid material, the degree of cellularity and vascularity, and the presence of necrosis. Most myxoid liposarcomas have lacy or linear, amorphous foci of fat. Some myxoid liposarcomas appear to be cystic at nonenhanced MR imaging, although they enhance like other solid masses at contrast material-enhanced MR imaging. The enhancing areas within the tumor represent increased cellularity and vascularity; the nonenhancing areas represent necrosis, reduced cellularity, and accumulated mucinous material. Gadolinium-enhanced imaging is important in differentiating myxoid liposarcoma from benign cystic tumors. Characterization of the tumor with MR imaging plays an important role in the management of myxoid liposarcoma.

Adipose Tissue↗

Dedifferentiated liposarcoma of the pyriform sinus: report of a case and review of the literature.

Laryngeal and hypopharyngeal liposarcomas are extraordinarily infrequent tumors. To the best of our knowledge there are fewer than 40 well-documented cases reported to date. Almost all of them are well-differentiated liposarcomas, with only 2 laryngeal-hypopharyngeal dedifferentiated liposarcomas. Dedifferentiated liposarcoma is defined as a well-differentiated liposarcoma with areas of high-grade spindle cell nonlipogenic sarcoma. The well-differentiated areas may be of a lipoma-like, sclerosing, or mixed type, and the dedifferentiated areas most frequently are of malignant fibrous hystiocytoma-like type. Despite its commonly pleomorphic histology, dedifferentiated liposarcoma does not behave as aggressively as most pleomorphic sarcomas of adulthood; however, it has the capacity to metastasize, in contrast to its well-differentiated counterpart. We present a case of dedifferentiated liposarcoma arising in the pyriform sinus, an event only twice reported previously in the literature.

Aged↗

Liposarcomas of the larynx and hypopharynx: a clinicopathologic study of eight new cases and a review of the literature.

Laryngeal and hypopharyngeal liposarcomas were studied in seven men and one woman. Patient age ranged from 25 to 81 years, with a median of 64 years. Symptoms included dysphagia, airway obstruction, and the sensation of a foreign body in the back of the throat. Histologically, seven of the tumors were of the biologically favorable types, either representing well-differentiated (lipoma-like) liposarcomas or myxoid liposarcomas. One tumor was a pleomorphic liposarcoma. Six of the eight patients had one or more episodes of recurrent tumor. Surgery is the treatment of choice and can include conservative (organ-sparing) procedures. However, to eradicate the tumor completely and thereby prevent recurrent disease, open surgical approaches (i.e., lateral pharyngotomy), rather than endoscopic techniques should be employed. The morbidity rate for laryngeal and hypopharyngeal liposarcomas is high because these tumors tend to recur over extended periods of time. In this study, however, there were no instances of metastatic disease, and no deaths were attributed to liposarcoma. Prospectively, laryngeal and hypopharyngeal well-differentiated (lipoma-like) liposarcoma is a difficult clinical and histopathologic diagnosis to establish. Often, this diagnosis is made only after one or more episodes of recurrent disease.

Adult↗

Dedifferentiated liposarcoma of the extremities: imaging findings in four patients.

OBJECTIVE: The term "dedifferentiated" liposarcoma is used to describe a distinctive lesion in which a well-differentiated liposarcoma is juxtaposed with a high-grade nonlipogenic sarcoma. Dedifferentiated liposarcoma is probably the most common of all the dedifferentiated sarcomas, occurring almost exclusively in the mediastinum, the retroperitoneum, and the inguinal/paratesticular regions. We report the imaging findings in four cases of dedifferentiated liposarcoma of the lower extremities. MATERIALS AND METHODS: The radiologic images and clinical histories of four patients with histologically verified dedifferentiated liposarcoma were retrospectively studied. The mean age of the patients was 61 years (range, 33-79 years). All lesions occurred in the thigh. Spin-echo MR images were available for review in two cases and CT scans in the two remaining cases. Plain radiographs were available in all cases. RESULTS: All lesions were large, with a mean maximum size of 24 cm (range, 12-30 cm), and had a significant fatty component radiologically. Both MR and CT showed a well-delineated fatty component of the lesions and a closely apposed nonfatty region. Radiographs showed well-defined bone within one lesion, bone and amorphous calcification in another, and a single punctate calcification in a third. Two patients had a history of excision of a lipomatous lesion in the region of the mass. One patient had pulmonary metastases. CONCLUSION: The potential for deep well-differentiated fatty tumors of the extremities to dedifferentiate is not generally recognized. Although the different types of liposarcoma cannot be reliably distinguished with imaging studies, a well-defined nonlipomatous mass juxtaposed with a predominantly fatty tumor is suggestive of a dedifferentiated liposarcoma.

Adult↗

Prognostic relevance of p53 alterations and Mib-1 proliferation index in subgroups of primary liposarcomas.

For prognostic analyses of p53 alterations (p53 gene mutations + p53 immunopositivity) and Mib-1 proliferation index, we investigated 42 primary malignant lipomatous tumors for which complete clinical data and a long follow-up were available. p53 gene mutations were investigated by PCR-single strand conformation polymorphism-sequencing analysis, and immunohistochemistry was used to determine p53 protein expression and Mib-1 proliferation index. We found a mutation frequency of 14.3%. Nine liposarcomas (21%) were p53 immunopositive, and 11 (26.2%) had at least one p53 alteration. In myxoid liposarcomas, p53 alterations are not relevant to the presence or absence of round cell components. Pleomorphic liposarcomas showed a significantly higher proliferation index and more p53 alterations than myxoid or well-differentiated variants (P<0.001). When the Cox's regression analysis tumors of grade III histology (P = 0.005) was performed, the pleomorphic subtype (P = 0.016) and liposarcomas of retroperitoneal localization (P = 0.015) showed a significantly poorer prognosis. Moreover, we found that p53 alterations and high proliferation index correlated significantly with reduced overall survival. Their prognostic value seemed to be higher in myxoid than in pleomorphic liposarcomas. The metastasis-free survival was reduced in patients who had liposarcomas with p53 alterations (P = 0.171) or elevated proliferation index (P<0.016), reflecting a more aggressive behavior. In conclusion, the determination of p53 alterations and/or Mib-1 proliferation index is useful for assessing the prognosis of patients with liposarcomas and may especially be helpful in dividing different prognostic groups for patients with myxoid variants.

Adolescent↗

Liposarcoma a clinicopathologic, radiographic and prognostic study.

The present investigation is a clinicopathologic, radiologic and prognostic study of 122 liposarcomas registered in the Swedish Cancer Registry between 1958 and 1966. The incidence of liposarcoma in Sweden was estimated to be 2.5 cases per million inhabitants per year. The liposarcomas were divided into 4 main subtypes according to WHO's recommended classification (1969): well-differentiated (33 cases), myxoid (22 cases), rundcell (14 cases) and pleomorphic types (21 cases). When predominantly well-differentiated or myxoid liposarcomas contained areas of round-cell or pleomorphic types (24 and 8 cases, respectively), they were classified separately, thus adding 2 further subtypes to our classification. The validity of this histologic classification was emphasized by the prognostic study, based on a 10 year follow-up. The differential diagnosis for each of the 4 main subtypes is discussed. Three well-differentiated, lipoma-like liposarcomas which contained highly vascular hibernoma-like areas are described. Two well-differentiated liposarcomas situated in the neck and upper part of the back, respectively, of elderly patients included area similar to "spindle-cell lipoma" (Enzinger). An intracytoplasmic network of birefringent fibers with the staining reaction of collagen was found in 2 well-differentiated fibrosing liposarcomas...

Adolescent↗

Intussusception secondary to metastasis from a low-grade retroperitoneal liposarcoma.

Liposarcomas are the most prevalent soft tissue sarcomas in adults. Low-grade liposarcomas are the most frequent and least aggressive and are noted as having a low risk of metastasis. We present a case of low-grade myxoid liposarcoma that metastasized to the small bowel resulting in intussusception. This case involves a 44-year-old woman with a recurrent retroperitoneal liposarcoma. Approximately 30 months after initial diagnosis, the patient was found to have a solitary metastasis to the small bowel that presented as a jejunal intussusception. Interestingly, there was no indication of a round cell component in either the metastasis or recurrent tumor, although the original tumor did contain a small round cell component. This is the first report in the English literature of liposarcoma metastasizing to the small bowel. We suggest that atypical presentations of liposarcoma metastases should always be considered in the management of patients with a history of liposarcoma.

Adult↗

[Correlation between pathology and image characteristics of retroperitoneal liposarcoma].

OBJECTIVE: To investigate the correlation between the pathological and image features of different subtypes of retroperitoneal liposarcoma. METHODS: Pathological and image characteristics of 21 retroperitoneal liposarcomas were retrospectively analyzed comparing their different histopathological subtypes. RESULTS: This series consisted of 11 well-differentiated, 4 myxoid, 3 round-cell, 2 pleomorphic and 1 dedifferentiated liposarcoma. Well-differentiated liposarcomas were predominantly composed of lipoma-like and sclerosing components. On CT and MRI image, the attenuation and signal intensity of lipoma-like components resembled those of fat, whereas the signal intensity of sclerosing components was similar to those of muscle. Unenhanced CT image of the myxoid subtype showed density resembling that of water. On contrast-enhanced CT image, it showed gradual reticular or sheet-like enhancement. The CT and MRI image appearance of the round cell and pleomorphic subtypes resembled those of nonfatty soft tissue masses with foci of necrosis. The dedifferentiated liposarcoma showed the image feature of a well-differentiated component clearing enhanced soft tissue mass. CONCLUSION: Different subtypes of retroperitoneal liposarcoma show different CT and MRI features relating to their major histologic components. A good understanding of the relation between their radiological and pathological features is helpful to arrive at a correct diagnosis for retroperitoneal liposarcoma.

Adult↗

Liposarcoma of soft tissue: MRI findings with pathologic correlation.

OBJECTIVE: To evaluate the MRI findings of liposarcomas of different histologic types and correlate these with the histopathologic features. DESIGN: The MR images of seven liposarcomas were reviewed retrospectively to assess the tumor size, location, margination, signal characteristics and enhancement patterns in different histologic types. PATIENTS: Seven liposarcomas comprising three well-differentiated, two myxoid and two pleomorphic types were evaluated. RESULTS AND CONCLUSION: All tumors showed well-defined and mostly lobulated margins. The well-differentiated liposarcomas were composed mainly of fat with septations or nodules, were hyperintense on T2-weighted images, and demonstrated faint enhancement or no enhancement following intravenous contrast. Myxoid liposarcomas were homogeneous or mildly heterogeneous and a pseudocapsule was present in one case. Pleomorphic types showed a markedly heterogeneous internal structure. Both myxoid and pleomorphic lesions-showed moderate or marked heterogeneous enhancement after contrast administration. Well-differentiated liposarcomas may be differentiated from other types of the tumor by their largely lipomatous appearance. The malignancy grade increases in parallel with tumor heterogeneity and contrast enhancement.

Adult↗

Unusual spread of liposarcoma.

BACKGROUND: The aim of our study was to investigate the unusual pattern of metastatic spread of liposarcoma. MATERIALS AND METHODS: We retrospectively studied thirty-two patients with liposarcoma (in seventeen located in the retroperitoneum and in fifteen in the extremities) who were managed at our institution over a ten-year period. Six patients with extremity liposarcoma developed local recurrence and or distal metastases and in three the site of the distal spread was other than the lungs. In only one patient with retroperitoneal liposarcoma we observed extra-pulmonary spread. The histologic type of all four cases with unusual spread was myxoid, with small areas of round cell differentiation in two cases. RESULTS: The survival of the patients after the initial recurrence was relatively prolonged (6, 1.5, 8 and 3 years with the last patient alive and well). CONCLUSION: Physicians should maintain a low threshold for initiating evaluation of relatively minor symptoms in patients with liposarcoma, as these symptoms may reflect the first manifestation of an extrapulmonary metastasis. Such an approach should result in earlier detection and hopefully improve survival for patients with liposarcoma.

Abdominal Neoplasms↗

Chimeric TLS/FUS-CHOP gene expression and the heterogeneity of its junction in human myxoid and round cell liposarcoma.

Myxoid liposarcomas have a unique and specific t(12;16)q13;p11) chromosomal translocation. The breakpoint has recently been identified and shown to involve the TLS/FUS gene on chromosome 16 and the CHOP gene on chromosome 12. This translocation causes fusion of these genes resulting in the expression of a novel chimeric TLS/FUS-CHOP message. Using the polymerase chain reaction with primer sets derived from sequences of TLS/FUS and CHOP cDNAs, we could amplify three types of the fusion transcripts from seven of seven samples of myxoid and round cell liposarcomas. In six of the seven positive samples, two kinds of chimeric messenger RNAs were found that have been reported previously. However, the last sample had a novel chimeric message that had an extra sequence of 33 bp derived from the TLS/FUS gene. Thus, it was shown that these fusion transcripts had a varying extent of the sequence of TLS/FUS gene incorporated at the site of the fusion. However, the TLS/FUS-CHOP fusion transcripts were not detected in two pleomorphic liposarcomas or in three myxoid variants of malignant fibrous histiocytomas. Our findings indicate that in liposarcomas TLS/FUS-CHOP fusion transcripts have variations at the junction of chimeric messages, which was the case for Ewing's sarcoma. Detection of the chimeric message by reverse transcription polymerase chain reaction was also suggested to be a useful approach for the diagnosis of myxoid and round cell liposarcomas that have (12;16) translocation, and for distinguishing them from pleomorphic liposarcoma and myxoid variant of malignant fibrous histiocytomas.

Adult↗

Fine-needle aspiration cytology of well-differentiated inflammatory liposarcoma: a case report with histologic follow-up.

Inflammatory liposarcoma is an unusual variant of well-differentiated liposarcoma. We report on the fine-needle aspiration (FNA) cytology findings of a retroperitoneal well-differentiated inflammatory liposarcoma from a 63-yr-old white female. The smears showed numerous dispersed inflammatory cells, with the majority being reactive lymphoid cells and plasma cells. There were scattered, large, atypical cells containing multiple or hyperlobated nuclei with coarse chromatin and abundant ill-defined cytoplasm. The large atypical cells, as well as the inflammatory cells, were also found within the fibrous tissue fragments. The follow-up surgical resection of the tumor demonstrated a well-differentiated inflammatory liposarcoma with coexistent dedifferentiated areas and lipoma-like, well-differentiated liposarcoma. With the appropriate anatomic and radiographic settings, the FNA cytology findings of abundant, reactive inflammatory cells and scattered, large, atypical tumor cells that are CD15-, CD30-, and cytokeratin-negative are highly suggestive of a well-differentiated inflammatory liposarcoma.

Biopsy, Needle↗