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[Liposarcoma in the head-neck area].

Between 1977 and 1992 four patients with liposarcomas of the head and neck region were treated and regularly reexamined at the ENT department of the University of Tubingen. Our patients were aged 19, 64, 66, and 73 years at primary diagnosis. Three patients were female and one was male. The tumors were located in cheek/parotid gland, the neck, the tracheoesophageal region and larynx/hypopharynx. In three patients complete primary resection was planned. In three cases further surgery was necessary due to recurrences. Two patients are free of tumor for seven and nine years, respectively. One patient was irradiated with 70 Gy four years after primary surgery since she had developed an unresectable recurrence. She died eleven years later aged 79 without residual liposarcoma (autopsy). One patient aged 76 rejected a radical resection and underwent a functional tumor reduction and is alive in fairly good condition. Histologically we found one myxoid and three well-differentiated liposarcomas. Metastatic spread was not observed. Wide tumor excision is the therapy of choice. Unresectable tumors can be irradiated successfully (up to 70 Gy are necessary). Representative studies on the value of chemotherapy have not yet been presented. The main factor in clinical behavior and prognosis of this rare tumor is the histological subtype. While well-differentiated and myxoid liposarcomas show almost no metastasis and good prognosis, the pleomorphic and round cell type do metastasis, recur more often, and have a significantly worse prognosis.

Adult↗

Induction of solid tumor differentiation by the peroxisome proliferator-activated receptor-gamma ligand troglitazone in patients with liposarcoma.

Agonist ligands for the nuclear receptor peroxisome proliferator-activated receptor-gamma have been shown to induce terminal differentiation of normal preadipocytes and human liposarcoma cells in vitro. Because the differentiation status of liposarcoma is predictive of clinical outcomes, modulation of the differentiation status of a tumor may favorably impact clinical behavior. We have conducted a clinical trial for treatment of patients with advanced liposarcoma by using the peroxisome proliferator-activated receptor-gamma ligand troglitazone, in which extensive correlative laboratory studies of tumor differentiation were performed. We report here the results of three patients with intermediate to high-grade liposarcomas in whom troglitazone administration induced histologic and biochemical differentiation in vivo. Biopsies of tumors from each of these patients while on troglitazone demonstrated histologic evidence of extensive lipid accumulation by tumor cells and substantial increases in NMR-detectable tumor triglycerides compared with pretreatment biopsies. In addition, expression of several mRNA transcripts characteristic of differentiation in the adipocyte lineage was induced. There was also a marked reduction in immunohistochemical expression of Ki-67, a marker of cell proliferation. Together, these data indicate that terminal adipocytic differentiation was induced in these malignant tumors by troglitazone. These results indicate that lineage-appropriate differentiation can be induced pharmacologically in a human solid tumor.

Adult↗

Atypical lipoma: well-differentiated liposarcoma of the orbit with dedifferentiated areas.

Primary orbital liposarcoma is a very rare orbital tumor and dedifferentiation in the liposarcomas occurs rarely. Dedifferentiation is defined by development, in a well-differentiated tumor, of poorly differentiated, high-grade areas. The dedifferentiated liposarcomas (DDLS) have a better prognosis than pleomorphic sarcomas as a whole. The treatment of DDLS patients proves to be complicated due to the fact that the histopathological diagnosis usually becomes evident only after the surgery. In the present paper, the clinical presentation, histopathological features, radiological characteristics and clinical treatment of one case of well-differentiated liposarcoma with dedifferentiated areas, abruptly delimited from the well-differentiated adipocytic component, are reported.

Combined Modality Therapy↗

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↗

Controversial issues in diagnosis of liposarcomas of the thigh. A report of 18 cases.

Liposarcoma is a common soft tissue neoplasm mainly found in the thigh. Early diagnosis and appropriate treatment are essential for survival, but clinical diagnosis may not be easy, and the importance of imaging is controversial as it often fails to indicate the presence of liposarcoma so that treatment is delayed. We report our experience of 18 cases of liposarcoma (16 of which in the thigh). This experience has led us to revise our opinions about imaging, clinical, and histopathological diagnosis, and the surgical and medical treatment. We now suggest that any hard, subfascial mass in an adult should be considered as a soft tissue neoplasm. Of these neoplasms, liposarcoma is the most common type, after malignant fibrous histiocytoma. Diagnosis by imaging techniques is rarely possible, so that integration with clinical data is essential. Among the various imaging techniques, computed tomography and ultrasound seem to be most useful.

Adult↗

Surgery for dedifferentiated liposarcoma, presenting two radiologically and pathologically distinctive patterns.

In this paper, we report three cases with dedifferentiated liposarcoma in the lower extremities, presenting two patterns that are distinctively different radiologically and pathologically. The first case was a 67-year-old male who had discovered a mass in his left thigh, which was shown as a low and iso density mass between the muscles on computed tomography (CT). The second case was a 61-year-old male who had suffered from an intramuscular tumor with a non-fatty sarcomatous area within the fatty components as shown on magenetic resonance imaging (MRI). The third case was a 52-year-old female who had presented with an intramuscular tumor of the left thigh, consisting of a mass with fat and another soft tissue tumor adjacent to the lipomatous component as detected with CT and MRI. The final pathological diagnoses of the resected specimens in all three cases were concluded to be dedifferentiated liposarcoma, composed of well-differentiated liposarcomas and spindle and/or pleomorphic sarcomas, compatible with malignant fibrous histiocytoma. In all three cases, wide resection of the tumor was successfully carried out, and all patients have been continuously disease-free up to the most recent follow-up. Evaluation of the surgical margins for the resected specimens indicated that the safety margin for a dedifferentiated lesion should be accomplished as an adequate margin or more, and a marginal margin or more could be considered as safe only for the confined part of a well-differentiated liposarcoma.

Aged↗

Well-differentiated liposarcoma (atypical lipoma) of deep soft tissue of the extremities, retroperitoneum, and miscellaneous sites. A follow-up study of 92 cases with analysis of the incidence of "dedifferentiation".

Ninety-two cases of well-differentiated liposarcoma of deep soft tissue of the extremity, retroperitoneum, and groin with follow-up information of at least 2 years and no evidence of dedifferentiation at the time of diagnosis were studied to determine their long-term behavior. The tumors occurred most commonly in the muscles of the extremity (46 cases), followed by the retroperitoneum (23 cases), groin (14 cases), and miscellaneous sites (nine cases). Tumors in the retroperitoneum recurred in nearly all cases (21 of 23 cases), occasionally caused patient death, and dedifferentiated in four cases (median time to dedifferentiation, 8 years). Tumors in the groin had a similar high recurrence rate (11 of 14 cases), caused death of patients (two of 14 cases), and dedifferentiated (four of 14 cases). In contrast, those in the extremity recurred less frequently (20 of 46 cases) and had no disease-related mortality. Three of 46, however, underwent dedifferentiation (median time to dedifferentiation, 7 years). Of the 11 cases that underwent dedifferentiation, the interval between diagnosis and dedifferentiation ranged from 2 to 18 years (median time, 9 years; average time, 11 years). Six of the 11 patients showed dedifferentiated foci in the first recurrence, and three died of metastatic disease. Our study indicates that the behavior of well-differentiated liposarcomas is strongly influenced by location. Although the prevailing view is that dedifferentiation is restricted to tumors of the retroperitoneum, our study indicates that it is not a site-specific phenomenon, but is more likely a time-dependent phenomenon seen in situations with a high likelihood for clinical persistence of disease for a long period. Dedifferentiation identifies a tumor that is potentially more aggressive; yet the progression of the disease following dedifferentiation may be highly variable and probably depends on a number of factors, including the amount of dedifferentiation and type of therapy. Although atypical lipoma is an acceptable term for well-differentiated liposarcomas of the subcutis, it fails to convey the potentially life-threatening properties of retroperitoneal tumors. For these lesions as well as those in the deep soft tissues of the extremity, retention of the term well-differentiated liposarcoma is advocated.

Adult↗

A well-differentiated giant liposarcoma originating from the buccal fat pad.

Although liposarcoma is one of the most common soft-tissue sarcomas, facial localization is extremely rare. The buccal fat pad is an important anatomic structure located in the face that recently gained interest as a result of increasing research on facial anatomy. In this paper, we report a case of giant liposarcoma originating from the buccal fat pad. The precise localization of the tumor was determined preoperatively with computed tomography examination. The liposarcoma that invaded the body and the extensions of the buccal fat pad was resected completely. The pathological examination revealed a sclerosing, well-differentiated liposarcoma, which is known to be very rare in the head and neck region. Chemotherapy and radiotherapy were not necessary because of the favourable histological type of the tumor and the advanced age and poor general condition of the patient. Local recurrence and distant metastasis were not observed during the 1-year follow-up.

Aged↗

Liposarcoma associated with multiple intramuscular lipomas. A case report.

A 71-year-old slender, previously healthy man was admitted to the authors' institution because of a huge painless mass in his left scapular area. Physical examination revealed a second soft tissue mass in his right scapular region and a third soft tissue mass in the anterior side of his right shoulder. Surgical treatment including marginal resection of the second and third small masses followed by wide local resection of the huge tumor was performed. Histologic examination showed that the first mass was a well differentiated lipomalike liposarcoma, whereas the second and third lesions were identified as intramuscular lipomas. A review of the literature showed two cases of retroperitoneal liposarcoma associated with multiple subcutaneous lipomas and two cases of liposarcoma involving an extremity associated with multiple subcutaneous lipomas. There is no previous report in which intramuscular liposarcoma was associated with multiple intramuscular lipomas.

Aged↗

Liposarcoma (atypical lipomatous tumors) of the vulva: a clinicopathologic study of six cases.

Liposarcoma, the most common soft tissue sarcoma, usually arises in the limbs, trunk, or abdomen, but very rarely in the vulva. Six cases of liposarcoma of the vulva are described here. They were characterized by their occurrence in predominantly middle-aged women (median age 52), variable size, and focally infiltrative margin. The preoperative clinical diagnosis for all patients was of a benign lesion, with a diagnosis of lipoma in two. Four of six cases had the usual histologic appearance of a well-differentiated liposarcoma/atypical lipomatous tumor with variation in adipocyte size, adipocytic nuclear atypia, and occasional lipoblasts. Two of the six cases had a very unusual histologic appearance, not previously described, with an admixture of neoplastic bland spindle and round cells along with adipocytes showing variation in size as well as numerous mainly bivacuolated lipoblasts. Liposarcomas of other types were not identified. Follow-up data were available for five of the six patients and all were treated by excision with no additional treatment. Four showed no sign of recurrence at 12, 14, 18, and 84 months. One tumor was incompletely excised, regrew over a 10-year period, was re-excised, and showed no further recurrence 31 months thereafter. Liposarcoma can occur in the vulva, is predominantly of the well-differentiated subtype, can show an unusual histologic appearance with an admixture of spindle cells and numerous lipoblasts, and seems to have a behavior similar to these tumors in other anatomic locations.

Adult↗

Morphologic-cytogenetic analysis of dedifferentiated liposarcomas with an extensive misleading leiomyosarcomatous component.

This report describes two cases of recurrent retroperitoneal dedifferentiated liposarcoma characterized by an extensive leiomyomatous component that prevented the correct diagnosis before the last recurrence. Strong immunoreactivity with smooth muscle and desmin antibodies and ultrastructural features consistent with leiomyosarcoma were observed in the spindle-cell and/or myxoid-like components in all four recurrences in case 1, and in the spindle-cell component of the primary tumor and the first recurrence in case 2. In case 1, the correct diagnosis was suggested by the cytogenetic evidence of ring markers, a hallmark of well-differentiated/dedifferentiated liposarcoma. In case 2, tumor type was yielded mainly by the morphology of the second recurrence, which consisted entirely of a well-differentiated liposarcoma, a sclerosing inflammatory variant, as confirmed by the karyotype. Reevaluation of the first two surgical specimens of each case revealed small areas consistent with well-differentiated liposarcoma that had been previously overlooked. Despite the smooth-muscle antigen profile, both cases retained an mdm2+/p53+/cdk4+ immunophenotype consistent with the genotype.

Aged↗

The impact of chemotherapy on the survival of patients with high-grade primary extremity liposarcoma.

OBJECTIVE: To determine if chemotherapy offers a survival benefit to patients with large, high-grade, primary extremity liposarcoma. SUMMARY BACKGROUND DATA: The impact of chemotherapy on the survival of patients with primary extremity soft tissue sarcoma is controversial and its effect on individual histologic subtypes is not defined. PATIENT AND METHODS: Two prospectively collected sarcoma databases were used to identify all patients with >5 cm, high-grade, primary extremity liposarcoma that underwent surgical treatment of cure from 1975 to 2003 (n = 245). Clinical, pathologic and treatment variables were analyzed for disease-specific survival (DSS), distant recurrence-free survival (DRFS) and local recurrence-free survival (LRFS). RESULTS: Sixty-three (26%) patients were treated with ifosfamide based chemotherapy (IF), 83 (34%) with doxorubicin based chemotherapy (DOX) and 99 (40%) received no chemotherapy (NoC). To assess the impact of DOX, a contemporary cohort analysis of patients treated from 1975 to 1990 was performed. The 5 year DSS of the DOX treated patients was 64% (53%-74%) compared with 56% (51%-79%) for the NoC patients (log-rank P value = 0.28). To assess the impact of IF, a contemporary cohort analysis of patients treated from 1990 to 2003 was performed. The 5 year DSS of the IF treated patients was 92% (84%-100%) compared with 65% (51%-79%) for the NoC patients (log-rank P value = 0.0003). Independent prognostic factors for improved DSS were smaller size (HR = 0.7, P = 0.01), myxoid/round cell histologic subtype (HR = 0.3, P = 0.03) and treatment with IF (HR = 0.3, P = 0.01). The five-year DRFS of the IF treated patients was 81% (70%-92%) compared with 63% (50%-76%) for the NoC patients (log-rank P value = 0.02). The 5 year LRFS of the IF treated patients was 86% (76%-96%) compared with 87% (77%-97%) for the NoC patients (log-rank P value = 0.99). CONCLUSIONS: In patients with large, high-grade, primary extremity liposarcoma; DOX is not associated with improved DSS and IF is associated with an improved DSS. Treatment with IF should be considered in patients with high-risk primary extremity liposarcoma.

Adult↗

Paratesticular liposarcoma: coexistence with urinary bladder cancer.

A 58-year-old man with liposarcoma originating from the right paratesticular area was reported. Histologically, liposarcoma showed a well-differentiated sclerosing type. Coexistence of transitional cell carcinoma of the urinary bladder and liposarcoma was also revealed. The cases of paratesticular liposarcoma in the literature are briefly reviewed.

Carcinoma in Situ↗

Dedifferentiated liposarcoma with an inflammatory malignant fibrous histiocytoma-like component presenting a leukemoid reaction.

A rare case of dedifferentiated liposarcoma (well-differentiated liposarcoma with an inflammatory malignant fibrous histiocytoma (MFH)-like anaplastic component) occurring in a 69-year-old male is presented. The patient had noticed a dull pain in his left loin and thigh for about 1 month. Computed tomography examination revealed a low-density mass lesion, measuring about 6 cm in diameter, in the left iliopsoas muscle, and it was surgically removed. Grossly, the lesion was composed of an encapsulated, soft, whitish mass and an adjacent, well-demarcated, yellowish hard nodule, measuring about 2.5 cm in diameter. Microscopically, both lesions showed features of an inflammatory variant of MFH and a sclerosing type of well-differentiated liposarcoma, respectively. To our knowledge, only two cases of dedifferentiated liposarcoma combined with inflammatory MFH as a dedifferentiated component have been recorded in the literature. The salient feature of the present case is a systemic inflammatory reaction, as shown by prominent leukocytosis (up to 73,900/mm3) and the elevated serum value of C reactive protein (up to 26.0 mg/dL), which were transiently reduced after surgery. The inflammatory reaction was suggested to be induced by cytokines, such as granulocyte colony-stimulating factor and interleukin-6, which were probably produced by the tumor cells in the present case, because the elevated serum values of those cytokines were decreased after surgery.

Aged↗

Case report of vulvar liposarcoma.

Vulvar liposarcoma is very rare with only 11 cases reported since 1966. Primary vulvar sarcomas constitute 1-2% of all vulvar malignancies. Vulvar liposarcoma may be misdiagnosed as benign lesions because of its location and appearance. The case presented in this paper is a 65-year-old-woman referred with a vulvar mass. The mass was clinically similar to lipoma. After total excision the pathologic report showed a well-differentiated sclerosing liposarcoma. Since a favorable prognosis in vulvar liposarcoma is dependent on the early diagnosis, all clinicians who face patients with vulvar mass should have this diagnosis in their minds.

Aged↗

Recurrent retroperitoneal myxoid liposarcoma during pregnancy: a case report and literature review.

Retroperitoneal liposarcoma in pregnancy is rare, and only a few cases of primary liposarcoma during pregnancy have been reported. To the best of our knowledge, there is no published report of retroperitoneal liposarcoma that was previously treated and recurred during pregnancy. Our patient was diagnosed with a pelvic mass on ultrasound at 12-weeks' gestation. The mass was found to be a retroperitonal, well-differentiated myxoid liposarcoma and was radically excised at the time of cesarean delivery at 36-weeks' gestation. However, the tumor recurred soon and progressed rapidly, and the patient eventually died of the disease. A thorough sonographic investigation and timing of surgery may be critical in terms of finding a surgically resectable lesion and leading to a more favorable prognosis.

Adult↗

Uterine liposarcoma in a young woman: a case report.

Liposarcoma of uterus is a very rare neoplasm encountered generally in the sixth or seventh decade of life. We reported the eighth case of uterine liposarcoma with extension to broad ligament in a 23-year-old woman who presented with pelvic pain, dysuria, and rapidly enlarging pelvic mass. During laparotomy, the mass was detected to be originated from the uterine cervix. In frozen section, no definitive diagnosis was established, and for preservation of fertility, extirpation of the mass was performed. The histopathologic examination of the specimen revealed liposarcoma. The tumor cells were diffusely and intensively immune reactive for vimentin and S-100, and negative for smooth muscle actin, desmin, h-caldesmon, pan cytokeratin, and CD10. Staining with periodic acid and Schiff, alcian blue, mucicarmine, and epithelial membrane antigen (EMA) were negative. No recurrence was detected in the postoperative seventh month. Present case is the youngest patient with uterine liposarcoma and also the first case detected in reproductive period.

Adult↗

Use of Oil Red O stain in the cytologic diagnosis of canine liposarcoma.

BACKGROUND: Oil Red O, a stain commonly used to demonstrate lipid in frozen tissue, also may be used to stain air-dried cytologic specimens. OBJECTIVE: The purpose of this study was to prospectively evaluate the value of Oil Red O in identifying lipid to aid in the differentiation of liposarcomas from other types of sarcoma. METHODS: Twelve tumor specimens from dogs were evaluated. The tumors were included in the study if initial cytologic evaluation indicated a sarcoma, and if histologic confirmation was available. Oil Red O was applied to all cytologic specimens. RESULTS: Tumor specimens were diagnosed histologically as liposarcoma (3 well-differentiated, 1 pleomorphic), hemangiopericytoma (n = 3), fibrosarcoma (n = 3), malignant fibrous histiocytoma (n = 1), and undifferentiated sarcoma (n = 1). Cytologic specimens from all liposarcomas showed strong positive staining of cytoplasmic vacuoles for lipid. Specimens from other sarcomas stained negative for Oil Red O, with the exception of weak, irregular positive staining in 1 hemangiopericytoma. CONCLUSION: Our results suggest that Oil Red O staining may be an easy, inexpensive, and useful diagnostic tool for the differentiation of liposarcoma from other mesenchymal neoplasms.

Animals↗