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Clinico-molecular study of dedifferentiation in well-differentiated liposarcoma.

Well-differentiated liposarcoma (WD) acquires fully malignant potential when the histological progression named dedifferentiation occurs. This progression is supposed to occur in a time-dependent manner but this is still a debated issue. Clinically, the prediction of dedifferentiation for WD is very important from the therapeutic point of view. To identify genes that are predictive of dedifferentiation and to understand the mechanism of dedifferentiation, we investigated clinical information of 50 cases and studied the gene expression profiles of 36 lipomatous tumors using cDNA microarray. The clinical study showed that the dedifferentiation did not always seem to occur in a time-dependent manner. Interestingly, from the gene expression study, unsupervised hierarchical clustering analysis of well-differentiated lesions obtained from dedifferentiated liposarcoma (DD) cases that were indistinguishable from WD pathologically showed a clearly distinct gene expression pattern from WD. Using the pattern-matching program, 1687 genes including 487 known genes were identified, which discriminated WD cases from well-differentiated lipomatous lesions obtained from DD cases. These results suggest that the dedifferentiation may arise from different types of WD that could be distinguished from gene expression profiling but could hardly be classified by the pathological studies.

Base Sequence↗

A novel type of EWS-CHOP fusion gene in myxoid liposarcoma.

The cytogenetic hallmark of myxoid type and round cell type liposarcoma consists of reciprocal translocation of t(12;16)(q13;p11) and t(12;22)(q13;q12), which results in fusion of TLS/FUS and CHOP, and EWS and CHOP, respectively. Nine structural variations of the TLS/FUS-CHOP chimeric transcript have been reported, however, only two types of EWS-CHOP have been described. We describe here a case of myxoid liposarcoma containing a novel EWS-CHOP chimeric transcript and identified the breakpoint occurring in intron 13 of EWS. Reverse transcription-polymerase chain reaction and direct sequence showed that exon 13 of EWS was in-frame fused to exon 2 of CHOP. Genomic analysis revealed that the breaks were located in intron 13 of EWS and intron 1 of CHOP.

Adult↗

Retroperitoneal liposarcoma weighing over one hundred pounds with review of the literature.

BACKGROUND: Rarely, excessively large, slow growing tumors are found to be malignant. One exception includes retroperitoneal tumors, which if considered a mass of pelvic origin, may be encountered by the gynecologic oncologist. CASE: A post-menopausal female was referred for evaluation of a huge mass thought to arise from the pelvis. The patient underwent exploratory laparotomy and had resected a 50x48x45 cm, 103.6 lb. liposarcoma arising from the right retroperitoneum. DISCUSSION: Rarely, large slow growing abdomino-pelvic masses may be malignant, and one should be prepared to perform an appropriate surgical resection. This case represents removal of the largest retroperitoneal liposarcoma reported.

Diagnosis, Differential↗

[Giant retroperitoneal liposarcoma].

Liposarcoma is a malignancy of fat cells and is the most frequent soft tissue sarcoma localized in the retroperitoneum. It can reach substantial proportions. It is a slow-growing tumor, and the most frequent symptom is nonspecific abdominal pain and diffuse abdominal enlargement. Treatment is radical surgery and complete resection is essential for local control of the disease. We present a case of giant right retroperitoneal liposarcoma, which was well-encapsulated and could be completely excised. The patient is currently in follow up and at 2 years is disease-free.

Adult↗

Spermatic cord liposarcoma in a young boy.

We report on a spermatic cord liposarcoma arising in a 16-year-old boy. Treatment consisted of radical orchiectomy without retroperitoneal lymph node dissection, and adjuvant radiation and chemotherapy. The patient has survived 14 months without evidence of recurrence. Paratesticular localization of liposarcoma in patients less than 20 years old is rare. The tumor has no particular characteristic. Treatment consists of radical orchiectomy and the role of retroperitoneal lymphadenectomy is controversial.

Adolescent↗

The management of spermatic cord liposarcomas.

We review 3 cases of spermatic cord liposarcoma that have been followed for 12 months to 5 years after diagnosis. Inguinal orchiectomy rendered adequate treatment. Preoperative diagnosis of liposarcoma of the spermatic cord is unusual although gray scale sonography may be of some assistance. A review of the literature reveals that adjunctive medical or surgical treatment appears to be superfluous.

Adult↗

Perineal liposarcoma: diagnosis and management.

Perineal liposarcoma is excessively rare. Its clinical and pathological behavior can only be predicted by comparison with the behavior of liposarcoma in other parts of the body. We describe the management of a man who presented with symptoms related to bladder outlet obstruction and was found to have a prostatic mass on digital examination of the rectum. A needle biopsy was performed and the tissue was histologically consistent with sarcoma of the prostate. Computerized tomography suggested that the mass extended through the urogenital diaphragm and was contiguous with the corpus spongiosum and corpora cavernosa of the penis. Complete surgical extirpation was achieved through a perineal approach, although local extension of the growth beneath the internal anal sphincter and the urogenital diaphragm necessitated reconstruction of these structures. The surgical margins were free of tumor. We advocate local surgical extirpation rather than pelvic exenteration in these cases.

Adult↗

Myxoid liposarcoma of the bladder.

We report a case of myxoid liposarcoma of the bladder dome. Treatment consisted of partial cystectomy and pelvic lymphadenectomy. The patient had no evidence of recurrence 30 months postoperatively. This is the first reported case of primary bladder localization of liposarcoma.

Adult↗

Liposarcoma of the pleura.

Pleural liposarcomas are extremely rare; only eight cases have been reported in the literature. Herein we describe a 38-year-old man who had dyspnea on exertion and chest pain. Computed tomography of the chest revealed a large mass in the right pleural cavity. Thoracotomy was performed, and the patient recovered with no complications. Postoperative adjuvant irradiation was successful. In addition to the current case, we review the previously reported cases of pleural liposarcomas and discuss the treatment outcomes.

Adult↗

Ultrastructural distinctions between adult pleomorphic rhabdomyosarcomas, pleomorphic liposarcomas, and pleomorphic malignant fibrous histiocytomas.

The ultrastructural features of five pleomorphic rhabdomyosarcomas, five high-grade malignant fibrous histiocytomas, and five pleomorphic liposarcomas were studied. Electron microscopy was found to be consistently useful in distinguishing between these tumors. The rhabdomyosarcomas showed thick and thin filaments in complexes and consistently contained glycogen. The malignant fibrous histiocytomas had numerous lysosomes, often in cells with ruffled borders, and contained cells showing "myofibroblastic" differentiation. The liposarcomas showed abundant and coalescing lipid droplets, sparse stroma with condensation of amorphous granular materials surrounding plasma membranes, and prominent vascularity. Fourteen of the 15 tumors could be identified on the basis of ultrastructure; thus, electron microscopic examination is an important diagnostic tool for pleomorphic tumors.

Cytoskeleton↗

Primary liposarcoma of the right atrium.

Primary intracardiac liposarcomas are among the rarest tumors of the heart, with a total of six reported cases. The previous reports have focused on clinical features with minimal histologic documentation. We report a primary right atrial liposarcoma showing extracardiac spread and three types of histologic differentiation. This case demonstrated that certain clinical and pathologic features are common to all intracavitary cardiac neoplasms, emphasizing the need for careful histologic examination.

Heart Atria↗

Perirenal liposarcoma containing extramedullary hematopoiesis associated with renal cell carcinoma.

We present the first reported case of a perirenal well-differentiated liposarcoma containing extramedullary hematopoiesis associated with an ipsilateral T2N0M0 renal cell carcinoma. This patient, a seventy-one-year-old white male, had no known hematologic disorder and on preoperative evaluation had similar radiographic findings identified within the contralateral perirenal space. Pathologic evaluation after radical nephrectomy reported the perirenal mass to consist only of myelolipomatous change, however, further review identified liposarcoma.

Aged↗

Combined myxoid liposarcoma and angiolipoma of the spermatic cord.

To date, only 14 cases of myxoid liposarcoma of the spermatic cord have been reported in the literature. The growth is a rare, usually highly differentiated malignancy that tends to recur locally, rarely metastasizes, and has a good prognosis following complete removal. We describe a new case of a highly differentiated myxoid liposarcoma of the spermatic cord, combined with an angiolipoma. Left inguinal orchiectomy with high ligation of the cord was performed. Staging revealed no evidence of regional or distal metastases. By the latest visit, at 30 months, the tumor had not recurred. The diagnosis, management, and prognosis of this oncologic entity are discussed in the light of the literature.

Angiolipoma↗

Dedifferentiation of a well-differentiated liposarcoma to a highly malignant metastatic osteosarcoma: amplification of 12q14 at all stages and gain of 1q22-q24 associated with metastases.

Well-differentiated liposarcomas (WDLPS), especially those located in the retroperitoneum, may occasionally undergo dedifferentiation. Although this process is associated with a more aggressive clinical course, dedifferentiated liposarcomas rarely produces metastases. The case reported here is rather uncommon: A retroperitoneal WDLPS gave lung metastases that were diagnosed as highly malignant osteosarcomas. We used comparative genomic hybridization (CGH), fluorescence in situ hybridization (FISH), and Southern blot analyses to characterize the copy number changes and genetic aberrations occurring at different stages of the disease. In the primary tumor, the only detectable aberration was amplification of 12q13-q14, which was present only in a fraction of the cells and revealed by FISH analysis. High-level amplification of 12q13-q14, involving CDK4, MDM2, and HMGIC, was seen both in the relapse and the metastases. The second most common change, gain or high-level amplification of 1q22-q24, was detectable by CGH only in the osteogenic metastases, as was loss of the distal 2q. FISH analyses revealed considerable heterogeneity in the samples, and the percentage of cells showing aberrations was significantly higher in the metastatic samples. In particular, increased copy numbers of 789f2, a marker for 1q21 amplification in sarcomas, was observed in more than 65% of the cells in the metastatic samples, but in less than 10% of the cells from the recurrent samples. These observations could indicate that 1q amplification, in particular, may be indicative of a more malignant phenotype and ability of metastasis in WDLPS, as has also been suggested by others.

Adult↗

Cytogenetic analysis of a case of myxoid liposarcoma with cartilaginous differentiation.

The cytogenetic analysis of a patient with a myxoid liposarcoma exhibiting cartilaginous differentiation is presented. A complex translocation involving chromosome 12, 16, and 19 was found, instead of the t(12;16), specific for myxoid liposarcoma. The involvement of 19q13 in a tumor with cartilaginous differentiation, and the assignment of TGF beta 1 to 19q13.1-13.2, which appears to play a role in the formation of bone and cartilage, suggest a possible relation between both.

Adult↗

[Mediastinal liposarcoma. A report of 2 cases].

Liposarcomas are malignant mesenchymal tumors whose primary location in the mediastinum is rare. We report two cases of mediastinal liposarcoma, describing the clinical signs and radiologic features found and the diagnostic procedures performed. The significance of histologic variability as a factor that conditions treatment and prognosis is underlined.

Adult↗

Liposarcoma of the buccal mucosa.

An extremely rare tumor of the oral cavity, liposarcoma, is reported. The tumor was located in the submucosal layers of the cheek and protruded into the mouth. To the best of our knowledge this is the fourth reported instance of liposarcoma of the cheek in the world literature. Combined surgical excision and radiotherapy proved a success as judged by a 3-year follow-up.

Adult↗

Gastric liposarcoma. A case report.

Gastric localization of a liposarcoma is exceptional. A 67 year old patient with repeated hematemesis was found to have an ulcer on the dorsal wall of the antrum on radiology. Surgery revealed an ulcerated tumour, 5 X 2 X 1.5 cm, involving the tunica muscularis up to the serosa. Histologic examination showed a liposarcoma with intricate myxomatous zones, round cells, pleomorphous clearly differentiated lipoblastic aspects, and haemorrhagic areas.

Aged↗