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[Retroperitoneal liposarcoma with reactive pleural effusion: a case report].

A case of retroperitoneal liposarcoma with reactive pleural effusion is reported. A 58-year-old male who complained of weight gain and edema had visited another clinic in May, 2002. Computed tomography revealed a retroperitoneal tumor, 11 x 9 x 9.5 cm in size, surrounding his right kidney and bilateral pleural effusion. Complete resection of the tumor and right kidney was performed with a transperitoneal approach. Though the tumor was limited within Gerota's fascia, we noted two liters of abdominal ascites. Surgical specimen was histologically diagnosed as well differentiated liposarcoma. The post operative course was uneventful. The pleural effusion disappeared, and serum total protein level was normalized. He has remained free of disease for one year.

Humans↗

Well-differentiated liposarcoma associated with benign lipoma.

The objective of this study was to review the clinicopathological features of seven patients presenting with well-differentiated liposarcoma (WDL), which was associated with subcutaneous lipoma. From 1980 through 2002, 34 individuals displaying WDL were treated in our institutions. Lipoma was observed in seven of these 34 patients (five men and two women, mean age of 66.7 years). The rate of co-existence of lipoma in WDL [20.6% (7/34)] cases was significantly higher than the corresponding rate in the other liposarcoma subtypes [2.5% (1/40)]. Immunohistochemically, cdk4 was positive in all WDLs (100%). ki-67 was positive in 57.1% (4/7) and mdm2 and p53 were positive in 14.5% (1/7) of the WDL cases. Weak cdk4 immunoreactivity was detected in two lipomas. All lipomas were negative for mdm2, p53 and ki-67. Comparison of the expression profile in these malignant and benign tumors, which had arisen in identical genetic backgrounds, confirmed the involvement of these proteins, especially cdk4, in the tumorigenesis process of WDL.

Aged↗

Expression of DOL54 is not restricted to myxoid liposarcomas with the FUS-DDIT3 chimera but is found in various sarcomas.

The DOL54 gene [also known as megakaryocyte stimulating factor, articular superficial zone protein (SZP) or proteoglycan 4 (PRG4)], was cloned as a downstream target gene of the FUS-DDIT3 chimera, which is the fusion gene that characterizes myxoid liposarcoma (MLS). Activation of DOL54 was found to require an intact DNA binding domain of the DDIT3 protein and to be dependent on the presence of the N-terminal part of the FUS protein. Although originally suggested to be of oncogenic significance, expression analysis of DOL54 in tumors has so far been limited to a few cases of liposarcoma and malignant fibrous histiocytoma (MFH). In the present study we were interested to evaluate whether DOL54 expression can be associated with other fusion genes in which FUS is the 5'-partner. Thus, we investigated the expression of DOL54 in low grade fibromyxoid sarcoma (LGFMS) carrying the FUS-BBF2H7 chimeric transcript. We also included synovial sarcomas (SS), Ewing tumors (ET), extraskeletal myxoid chondrosarcomas (EMC) and MFH. The first 3 of these tumor types are characterized by chromosomal translocations that give rise to fusion genes not involving FUS, while no specific chimeric genes have been reported in MFH. DOL54 expression was found in 8/12 LGFMS carrying the FUS-BBF2H7 chimera but also in 8/10 of the examined MFH, 5/7 SS, 2/5 ET and 7/7 examined EMC. The results of our study clearly show that expression of DOL54 is not only a characteristic feature of MLS with the FUS-DDIT3 chimera but that this is a frequent finding also in various other sarcomas.

Cloning, Molecular↗

Giant mixed type retroperitoneal liposarcoma.

Retroperitoneal liposarcoma is a rare tumour that often presents late due to its indolent nature and large potential growing space. Total resection is the aim, including adjacent organs if necessary. Recurrence is common, and 5-year disease-free interval is not regarded as a cure. We present a case of a 57 year old man who had a 17 kg liposarcoma surgically excised.

Humans↗

[Liposarcoma of the laryngeal region. Case report and literature review].

The case is reported of a 64-year-old man in whom a pedunculated tumor of the Vallecula epiglottica occurred and was excised. Histologically it proved to be a dedifferentiated liposarcoma. The patient is well and free of disease 18 months later. The literature is reviewed and 20 other cases of liposarcoma in the laryngeal region are discussed.

Epiglottis↗

C-kit expression in dedifferentiated and well-differentiated liposarcomas; immunohistochemistry and genetic analysis.

BACKGROUND: c-kit expression by immunohistochemistry has been utilized to identify cancer patients who can be treated with imatinib-mesylate. In gastrointestinal stromal tumors (GISTs), an activating mutation in c-kit predicts treatment response; its presence in other soft tissue tumors is unexplored. MATERIALS AND METHODS: We evaluated seven cases of dedifferentiated liposarcomas (DDLS) and compared those with seven well-differentiated liposarcomas (WDLS). Immunohistochemical staining for c-kit was performed using a polyclonal antibody. Using PCR, exons 9, 10-11, 12-13 and 17 of c-kit were amplified and direct DNA sequencing performed. RESULTS: Two out of 7 (30%) DDLS showed focal weak immunoreactivity with c-kit; no (0%) WDLS stained with c-kit. Seven out of 7 (100%) DDLS showed an allelic variation in exon 10, with a single base pair substitution (A >C) at codon 541; 3/7 (43%) WDLS showed the same change. CONCLUSION: c-kit immunoreactivity did not correlate with the change in DNA sequence; DDLS showed a consistent allelic variation in c-kit that may have significant prognostic, diagnostic and therapeutic implications.

Adult↗

[Giant retroperitoneal liposarcoma--case report].

Retroperitoneal liposarcomas are the most frequent soft tissue sarcomas and the second most frequent retroperitoneal tumours. They represent less then 0.1% of all human malignancies. Hereby we describe the treatment of our patient where of a 15-kg giant retroperitoneal liposarcoma was successfully removed and a local recurrence two years later was operated on as well. This was the 3rd largest retroperitoneal malignant tumor that was successfully removed according to the available literature of the last five decades. These typically symptom-free tumors usually grow extreme size before diagnosed. The "gold-standard" of treatment remains surgical total excision, but the high local recurrence rate (50-60%) hopefully can be reduced by adjuvant radio- and chemotherapy. Multidisciplinary treatment and long-time follow-up can provide as high as 40% 5-year survival rate. We summarize the recent clinical, diagnostic and therapeutic methods of this rare condition.

Humans↗

Retroperitoneal liposarcoma with metastasis to both orbits: an unusual metastatic site.

Liposarcoma is among the most common adult histotypes of soft tissue sarcoma. It usually arises from the deep soft tissue of the extremities or the retroperitoneum. Despite the high content of fat in the orbit, both benign and malignant tumors of adipose tissue are extremely rare at this site; reports describing liposarcoma metastatic to the orbit are rare. Our report is a new case and the appropriate methods for diagnosis and treatment of this unusual metastatic site in this type of cancer are discussed.

Humans↗

[Well differentiated paratesticular liposarcoma in adults].

The authors report a case of well differentiated paratesticular liposarcoma in a 41-year-old patient. This is a rare tumour (about one hundred cases have been reported in the literature), which essentially arises from the spermatic cord. Clinical and radiological signs are nonspecific and the diagnosis is generally based on histological examination of the operative specimen. Due to the histological similarities, all specimens of benign lipoma must be examined for the presence of well differentiated liposarcoma. Treatment consists of transinguinal radical orchidectomy, sometimes with resection of adjacent structures. The prognosis is generally better than that of other paratesticular sarcomas. Adjuvant radiotherapy may be indicated in locally advanced masses or in the case of incomplete resection.

Adult↗

[Giant retroperitoneal liposarcoma].

The authors report a case of giant retroperitoneal liposarcoma. The diagnosis was suspected after scanography and magnetic resonance imaging and confirmed by the histological analysis of the extracted piece after surgical treatment. Postoperative evolution was favourable after one year without recurrence or distant metastasis. The authors discuss the pathologic and therapeutic aspects and the prognosis of retroperitoneal liposarcoma.

Aged↗

Lower-extremity liposarcoma metastatic to the larynx: case report.

Distant metastases to the larynx are rare. We describe the case of a 46-year-old man who was referred to our head and neck surgery clinic with a 6-week history of sore throat and otalgia. He was found to have a laryngeal lesion that was consistent with a primary myxoid liposarcoma that had been extirpated from a lower extremity earlier To the best of our knowledge, no case of myxoid liposarcoma metastatic to the larynx has been previously reported in the English-language literature.

Humans↗

[Retroperitoneal liposarcoma: a case report].

We report a case of retroperitoneal liposarcoma. A 66-year-old male was referred to our hospital for a retroperitoneal tumor that was detected during gallbladder stone examination. Computed tomography (CT) and magnetic resonance imaging (MRI) revealed an extrarenal lipomatous tumor, 20 x 14 x 8 cm in size, in the right retroperitoneal space. He underwent surgical excision of the tumor with concomitant resection of the right kidney and adrenal gland. The resected tissue weighed 730 g. Histological examination revealed a well differentiated liposarcoma. He received no adjuvant therapy.

Adrenalectomy↗

[A case of liposarcoma of spermatic cord].

An 84-year-old male was referred to our hospital with the chief complaint of a painless inguinal mass. An elastic hard mass was palpable in the right inguinal region next to the spermatic cord. Ultrasonography and computed tomography showed an inguinal homogeneous mass which was slightly enhanced. Since the operation appearance indicated the tumor was arising from the right spermatic cord, right radical orchiectomy was performed. Histopathological examination revealed a well-differentiated liposarcoma of the right spermatic cord. This is the 70th case of liposarcoma of the spermatic cord reported in Japan.

Aged, 80 and over↗

[Thoracic wall metastasis in liposarcoma].

Myxoid liposarcoma often metastasizes to extrapulmonar sites; however, osseous metastases are rare. We report the case of a 52-year-old male with a single histologically proven bone metastasis of the thoracic wall from a primary myxoid liposarcoma with a round cell component of the right thigh.

Humans↗

[Liposarcoma of the spermatic cord].

Liposarcoma of the spermatic cord is rare. It usually presents as a painless slowly-growing scrotal mass of consistency like lipoma. Inguinal orchiectomy is an adequate surgical approach. Retroperitoneal lymphadenectomy is not indicated owing to the low malignancy potential of the sarcomas. Value of adjuvant radiotherapy/chemotherapy remains uncertain. Recurrences are frequent, owing to incomplete surgical removal of the tumor. We report on a 62 year old male who presented with a half a year history of a soft painless mass in the left scrotum extending from the groin up to the testis. Clinical examination suggested inguinal hernia. Radical orchiectomy was performed. Histological examination revealed a well-differentiated liposarcoma of sclerosing subtype. No evidence of recurrence or metastases has been noted during the 6-month and one year follow-up without any postoperative adjuvant therapy.

Genital Neoplasms, Male↗

Paratesticular liposarcoma--a case report.

A case of paratesticular liposarcoma in a 60 year male is presented for its rarity. The tumour was a dedifferentiated liposarcoma (DDL) seen in the original excision.

Diagnosis, Differential↗

[Retro-peritoneal liposarcoma. About 5 cases and revue of literature].

Liposarcomas are malignant mesenchymatous tumors developed from elements constituting the greasy tissue in various stages of differentiation. It is one of the most frequent mesenchymatous sarcomas. Retro-peritoneum is a privileged site of development in 12 to 15% of cases. During 9 years, from 1994 till 2002, we have colligated and operated 5 cases of retro-peritoneal liposarcomas. Average age was 50 years (extremes 34 to 79 years) with a sex-ratio of 1.5. Diagnosis was late beyond 6 months. Abdominal pain and the appearance of an enormous mass (20 cm) were the revealing signs. No imagery method allowed to evoke the diagnosis. The cyto-puncture realized once, was not able to determine with precision the histological type. Immunohistochimy occupies nowadays an important place to classify a sarcoma. Large surgical resection realized in 5 cases did not allowed the complete ablation of the tumour in 3 cases considering the advanced loco-regional extension, which was at the origin of 3 recurrences. Well differentiated histological type was noted in 3 cases, myxoid type in a case and not differentiated type in a case.

Abdominal Pain↗

Repeated resection for intra-abdominal and retroperitoneal liposarcomas: long-term experience in a single cancer center in Japan.

Intra-abdominal or retroperitoneal liposarcomas are huge, and local recurrence is a common event. There is still no consensus concerning debulking or repeated resection. Twelve patients who had surgery for intra-abdominal or retroperitoneal liposarcomas at the Cancer Institute Hospital (Tokyo, Japan) during 1981-2002 were enrolled. Eight patients underwent complete resection, for a resectability rate of 67%. Combined resection of adjacent organs was necessary to obtain clear margins in 10 of the 12 patients (83%). A patient who underwent R2 resection survived 13.2 years in a dormant state without tumor regrowth. Resection of second (five patients), third (three patients), or seventh (one patient) for local recurrence or regrowth tumors was successfully performed. A patient survived 21.5 years after four R0 resections and three R2 resections. Our experience suggests that both near-total and repeated resection might improve survival benefit.

Abdominal Neoplasms↗