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PubMed2005

Multiple mechanisms of telomere maintenance exist in liposarcomas.

PURPOSE: Telomeres are specialized nucleoprotein complexes that protect and confer stability upon chromosome ends. Loss of telomere function as a consequence of proliferation-associated sequence attrition results in genome instability, which may facilitate carcinogenesis by generating growth-promoting mutations. However, unlimited cellular proliferation requires the maintenance of telomeric DNA; thus, the majority of tumor cells maintain their telomeres either through the activity of telomerase or via a mechanism known as alternative lengthening of telomeres (ALT). Recent data suggest that constitutive telomere maintenance may not be required in all tumor types. Here we assess the role and requirement of telomere maintenance in liposarcoma. EXPERIMENTAL DESIGN: Tumor samples were analyzed with respect to telomerase activity, telomere length, and the presence of ALT-specific subcellular structures, ALT-associated promyelocytic leukemia nuclear bodies. This multi-assay assessment improved the accuracy of categorization. RESULTS: Our data reveal a significant incidence (24%) of ALT-positive liposarcomas, whereas telomerase is used at a similar frequency (27%). A large number of tumors (49%) do not show characteristics of telomerase or ALT. In addition, telomere length was always shorter in recurrent disease, regardless of the telomere maintenance mechanism. CONCLUSIONS: These results suggest that approximately one half of liposarcomas either employ a novel constitutively active telomere maintenance mechanism or lack such a mechanism. Analysis of recurrent tumors suggests that liposarcomas can develop despite limiting or undetectable activity of a constitutively active telomere maintenance mechanism.

Adult↗
PubMed2003

Solitary bone metastasis from myxoid liposarcoma.

BACKGROUND: Extrapulmonary metastasis is more common in liposarcoma than in other soft tissue sarcomas. However, osseous metastases are rare. CASE REPORT: We report the case of a 61-year-old woman with a solitary histologically proven bone metastasis of the right femur from primary myxoid liposarcoma of the left thigh. In September 2000 resection of the primary tumor was performed. Histology showed a high-grade liposarcoma with round cell differentiation. Postoperative radiotherapy with 60 Gy was performed. In a follow-up MRI examination 11 months after initial diagnosis, a suspicious formation in the marrow space of the right proximal femur was detected. CT-guided biopsy was performed and histology showed a metastasis from liposarcoma. PET examination confirmed the metastasis as solitary. Curettage of the metastasis was performed followed by radiotherapy with 60 Gy. The patient was without evidence of disease in the last follow-up. CONCLUSIONS: No standard treatment exists for the management of solitary extrapulmonary metastases from soft tissue sarcoma. Interdisciplinary cooperation is advised and the therapy concept should be chosen individually.

Biopsy↗
PubMedDate not supplied

Fine needle aspiration cytology of well-differentiated liposarcoma. A report of two cases.

BACKGROUND: Well-differentiated liposarcoma is difficult to diagnose on fine needle aspiration cytology (FNAC) smears and may create considerable diagnostic problems. CASES: Males aged 60 and 45 years presented with a swelling in the groin and retroperitoneal region, respectively. FNAC showed large cells with multilobulated nuclei and mature-looking fat tissue. A soft tissue tumor with bizarre cells was diagnosed cytologically in case 1 and liposarcoma in case 2. Histologically, both cases were diagnosed as well-differentiated sclerosing liposarcoma. CONCLUSION: The cytologic diagnosis of well-differentiated liposarcoma should be done with caution, and the sites should be taken into consideration. Deep-seated tumors with large, bizarre, giant cells should have wide excision as they recur more frequently.

Abdominal Neoplasms↗
PubMedDate not supplied

Fine needle aspiration of primary pleomorphic liposarcoma of the breast. A case report.

BACKGROUND: Primary liposarcoma of the breast is an extremely rare lesion. Only two cases describing the aspiration biopsy findings have been reported in the literature. We report the cytologic findings in an additional case, stressing the cytologic clues necessary to distinguish this neoplasm from a primary adenocarcinoma. CASE: A 53-year-old female presented to the emergency room with bleeding from a 20-cm, ulcerating mass in the right breast. Four months earlier she had been seen at another institution, where a diagnosis of poorly differentiated carcinoma was made by aspiration biopsy. Computed tomography had been negative for metastatic disease, and the patient refused further evaluation. Aspiration biopsy of the breast mass was repeated at our institution and interpreted as consistent with a poorly differentiated carcinoma. Histologic, immunophenotypic and ultrastructural evaluation of the mastectomy specimen revealed a pleomorphic liposarcoma. CONCLUSION: With increasing utilization of fine needle aspiration to evaluate breast lesions, it can be anticipated that unusual entities, including liposarcomas, will be encountered increasingly in breast aspirates. Therefore, it is important to consider liposarcoma in the differential diagnosis of aspirates showing isolated spindle and polygonal cells with vacuolated cytoplasm, nuclear scalloping and pleomorphism to avoid a misdiagnosis of carcinoma.

Adenocarcinoma↗
PubMed1994

Primary liposarcoma of the larynx. Case report and literature review.

Laryngeal liposarcoma is an unusual tumor. Only 16 cases have been previously reported. We describe a new case of well-differentiated laryngeal liposarcoma and review the literature. The tumor was treated by surgical removal. Immunohistochemical study showed an immunophenotype consistent with liposarcoma. The case described is of the oldest patient (83 years old), the second to be found in a woman, and the second to be localized to one true vocal cord (according to the literature reviewed). The patient is still free of tumor 2 years later. The treatment and prognosis of liposarcoma of the larynx are discussed.

Aged↗
PubMed2004

Focal divergent chondrosarcomatous differentiation in a primary pleomorphic liposarcoma and expression of transforming growth factor beta.

A rare case of primary pleomorphic liposarcoma of the thigh with a myxoid component, in which divergent differentiation to a well-differentiated chondrosarcoma was focally present, is described. Presence of heterologous elements has mainly been recognized in the context of dedifferentiated liposarcomas. Few cases of benign mesenchymal tissue have also been reported in well-differentiated and myxoid liposarcomas, while divergent sarcomatous differentiation in liposarcomas appears to be also rare in the absence of dedifferentiation. Positive immunostaining of transforming growth factor-beta, which seems to play a role in the formation of bone and cartilage, was demonstrated in our case. Review of the existing literature on the subject has been carried out.

Aged↗
PubMed1988

Metastatic liposarcoma of the brain with response to chemotherapy: case report.

Metastatic liposarcomas to the brain are rare. The authors describe a patient with a 20-year history of liposarcoma originating in the thigh and metastatic to the brain 18 years later. The brain metastasis was removed by surgery. Nine months later, the patient developed metastases to the retroperitoneum and liver. At that same time, she had recurrent brain metastasis. She was then treated with chemotherapy. The abdominal mass shrank considerably in the beginning, and the recurrent brain metastases totally disappeared. The patient eventually succumbed to widespread liposarcoma. Autopsy revealed extensive liposarcoma involving the retroperitoneum, liver, and lung, but no trace of tumor was found in the brain.

Brain Neoplasms↗
PubMed1977

Liposarcoma of the larynx: a multicentric or a metastatic malignancy.

The third case of liposarcoma of the larynx, this one arising from the preepiglottic space, is presented. The report is unique in so far as the patient previously had been treated for a liposarcoma in another anatomic region. The diagnosis in this case was in doubt until electron microscopic studies were done. Since the histopathology of the laryngeal and the previous liposarcoma were different, the question of metastasis versus multicentricity was raised. Other discussions of liposarcomas over the past three decades have not resolved this aspect of their behavior completely. In this instance, after considering avenues of metastatic spread in conjuction with the histopathology, it would appear that the laryngeal lesion was a second primary and not a metastasis.

Aged↗
PubMed2001

Retroperitoneal liposarcoma presenting a indirect inguinal hernia.

A 60-year-old man was admitted to our hospital with a right inguinal swelling that had been growing in size without any pain for 7 months. We diagnosed the growth as a right inguinal hernia and operated on him. The growth, however, was found to be a tumor it situated along the spermatic cord and testicular vessels. We diagnosed it as a lipoma. The tumor was resected near part of the internal inguinal ring. Histopathological diagnosis showed well-differentiated liposarcoma of the sclerosing type. Postoperative computed tomography (CT) revealed a large residual tumor in the retroperitoneum. We believed that the tumor was a retroperitoneal liposarcoma and that it developed in the inguinal region. The residue of the liposarcoma was resected onto the right inguinal tract. A periodic follow up has been performed and no evidence of recurrence or metastasis has been seen in the 4 years and 9 months since the second surgery. No adjuvant therapy was performed. Inguinal liposarcomas are relatively rare and in most cases these tumors are thought to originate in the spermatic cord. The origin of the tumor is believed to be the retroperitoneum.

Hernia, Inguinal↗
PubMed2006

Primary liposarcoma of esophagus: a case report.

Liposarcoma is the most common soft tissue sarcoma in adult life while esophageal liposarcoma is an extremely rare tumor. In the world literature, only 14 cases of esophageal liposarcomas have been described. We report a 72-year old male patient who was urgently admitted to our hospital for acute epigastric pain with a burning retrosternal sensation, persistent nausea, vomiting and dysphagia. Barium swallow, upper gastrointestinal (GI) endoscopy, esophageal manometry and CT scan, failed to accurately diagnose the lesion. After surgical resection of an esophageal polypoid tumor, the histological examination revealed a well-differentiated grade I liposarcoma. Diagnostic and therapeutic tools were discussed and the results of literature were reviewed.

Aged↗
PubMed1999

No microsatellite instability, but frequent LOH in liposarcomas.

To evaluate the significance of microsatellite instability (MI) and loss of heterozygosity (LOH) in the development of different histological subgroups of liposarcomas, we examined 28 tissue-samples from 21 patients and the corresponding non-neoplastic reference tissues. We investigated nine microsatellite loci and detected no MI. LOH for at least one marker was observed in 11 of 28 tumours (39%). Widespread allelic losses were a common characteristic of pleomorphic liposarcomas. Well-differentiated variants did not show LOH (p<0.003). Our findings support the idea that liposarcoma subgroups are defined by different spectra of genetic alterations. Inefficient DNA mismatch repair does not seem to be involved in the oncogenesis of liposarcomas.

DNA Repair↗
PubMed2006

[Paratesticular liposarcoma. Clinical and pathological study of three cases and review of the literature].

OBJECTIVES: Paratesticular liposarcomas are rare; most published cases are single cases. We report the clinical, histological and immunohistochemical characteristics of three cases of paratesticular liposarcomas. METHODS: We performed clinical, histological and immunohistochemical studies with expression of ki 67 and p 53 markers in three cases of tumors of the spermatic cord. RESULTS: Patient age ranged from 36 to 67 years, with a mean of 54 years. All tumors were big, multinodular and predominant histological type was well-differentiated (2/3), being one case undifferentiated (1/3). All cases were treated by surgical excision; one of them had a relapse five years later (33%) as a low-grade undifferentiated liposarcoma. None of the patients have developed metastases after a long follow-up (10, 7, and 5 yr.). CONCLUSIONS: Paratesticular liposarcomas have favourable prognostic markers (histological type, low proliferation index Ki 67 and p 53). The evaluation of this masses should be based on imaging tests, but a biopsy is necessary to determine their nature both in the case of primary tumors and recurrences. Surgical treatment should include complete excision with free margins to avoid recurrences.

Adult↗
PubMed2001

Primary liposarcoma of the liver: a case report and review of literature.

Liposarcoma is a rare mesenchymal malignant tumor, which usually originates in the retroperitoneum and the extremities. Seven cases of primary liposarcoma of the liver have been previously reported. We present the eighth case, which occurred in an adult female patient. Primary liposarcoma of the liver, although extremely rare, must be considered in the differential diagnosis of a hepatic mass that develops in a noncirrhotic liver, especially in patients who are potential candidates for orthotopic liver transplantation. Liposarcoma is an absolute contraindication for liver transplantation.

Contraindications↗
PubMed2004

Dedifferentiated liposarcoma with a paraganglioma-like histologic pattern: a case report and review of the literature.

A 53-year-old man presented with a 4-month history of increasing abdominal discomfort and distension. A large retroperitoneal mass was found on imaging. Image-guided needle core biopsy demonstrated a poorly differentiated malignant neoplasm. A 30 x 32 x 33-cm soft tissue mass was removed. Microscopically, the tumor consisted of predominantly epithelioid malignant cells arranged in a paraganglioma-like growth pattern. Immunohistochemically, these cells were strongly positive for neuron-specific enolase. Stains for synaptophysin and chromogranin, however, were negative. There was no ultrastructural evidence of neuroendocrine differentiation. Adjacent sarcomatous areas were composed of spindled cells arranged in storiform and fibrosarcoma-like growth patterns. A small area of well-differentiated liposarcoma was identified, and a diagnosis of dedifferentiated liposarcoma was established. To the best of our knowledge, this represents the first reported case of dedifferentiated liposarcoma with a paraganglioma-like histologic pattern. A brief review focusing on the morphologic variations of dedifferentiated liposarcoma is also presented.

Diagnosis, Differential↗
PubMed1993

[Retroperitoneal liposarcoma with intrarenal metastases which was difficult to distinguish from angiomyolipoma. A case report].

A 40-year-old man was admitted to our hospital with a fat-density extrarenal tumor and intrarenal nodules which were detected by ultrasonography (US) and CT scan obtained the general health check. After his admission, US, CT, MRI and angiography were performed. As the final diagnostic procedure, we performed a US-guided percutaneous needle biopsy of the extrarenal tumor, because this tumor was difficult to distinguish from liposarcoma and angiomyolipoma (AML). The pathological study revealed a well-differentiated liposarcoma, and the extrarenal tumor and the left kidney were removed en bloc. Pathologically, the tumor not only invaded into the renal parenchyma directly but also have metastasized independently to the intrarenal region. Postoperatively, he received a tumor dose of irradiation of 50 Gy. Liposarcoma arising in the perirenal space should be considered in the differential diagnosis of exophytic AML. If radiological studies can not confirm the appropriate diagnosis, we consider that biopsy of the tumor should be selected as an alternative diagnostic approach. And, we think that this is the first case report dealing with intrarenal metastases of liposarcoma.

Adult↗
PubMed1995

[A case of retroperitoneal dedifferentiated liposarcoma with tumor thrombus in the inferior vena cava].

We experienced a case of retroperitoneal dedifferentiated liposarcoma with tumor thrombus in the inferior vena cava. A 45-year-old man was pointed out a retroperitoneal mass on ultrasound examination. He was referred to the Department of Urology, Tochigi Cancer Center for critical evaluation. Image diagnostic procedures showed a retroperitoneal mass surrounded by loose tissue and large thrombus in the inferior vena cava. The tumor was removed surgically with right lobe of the liver and segment of the inferior vena cava. Histological examination revealed the tumor composed of the peripheral well differentiated liposarcoma and the central dedifferentiated liposarcoma. Characteristic radiological findings of retroperitoneal liposarcoma with dedifferentiation could be pointed out.

Humans↗
PubMed1999

[A case of metastatic liposarcoma originating in the retroperitoneum successfully treated with combination chemotherapy].

We reported a 36-year-old woman with metastatic liposarcoma originating in the retroperitoneum, which responded well to adjuvant chemotherapy. The primary tumor was removed by surgery. Two months later, the patient developed metastasis to the brain, and to the lung four months later. Metastatic liposarcomas to the brain generally are extremely rare. The patient was treated with combination chemotherapy using cyclophosphamide, vincristine, adriamycin, and dacarbazine (CYVADIC). After she was examined, the former two drugs were alternated with vindesine and ifosfamide, and another regimen with cisplatin and etoposide was given after a three-week interval. As a result, both of the metastases totally disappeared. No recurrent lesion has been noted for two years. Although the role of chemotherapy for liposarcoma has not been well defined and little data support its use in an adjuvant setting, this combination chemotherapy seemed to be effective for advanced liposarcoma.

Adult↗
PubMed1999

[Well differentiated inflammatory subcutaneous liposarcoma (inflammatory atypical lipomatous tumor)].

Well differential inflammatory liposarcoma is a rare type of low grade liposarcoma. Its microscopical features may simulate benign inflammatory pseudotumor. A case of this lesion was described occurring in the subcutis of the right supraclavicular region in 61-year-old woman. The tumor was round well circumscribed and measured 3 cm in diameter. Histologically, it contained a 1.5 cm inflammatory nodule, which was surrounded by otherwise typical lipoma-like liposarcoma. The inflammatory nodule was composed of lymphocytic aggregates and spindle to stellate cells with mild atypism. No lipoblasts were seen in this area which mimicked an inflammatory pseudotumor. Correct diagnosis was based on the recognition of the lipoma-like liposarcoma area in this lesion. Our patient had no recurrence six years after the tumor excision.

Female↗