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Retroperitoneal liposarcoma in a child.

Retroperitoneal liposarcoma is a rare tumor in children and has been reported previously on only three occasions. This is the fourth such case, presenting with a large mass on the left side which displaced the sigmoid and ureters to the right. The patient was treated with surgery, radiotherapy, and chemotherapy. She is alive and well more than four years after therapy was instituted. A review of the pertinent literature cites the need for radio- and chemotherapy especially when surgical removal is incomplete.

Antineoplastic Agents↗

Liposarcoma of spermatic cord.

Four patients with myxoid liposarcoma of the spermatic cord are presented, three of whom have survived longer than five years. Wide local excision is usually the treatment of choice with radiotherapy reserved for control of either extensive local disease or following incomplete removal of the lesion.

Aged↗

Paratesticular liposarcoma.

The fifteenth reported case of paratesticular liposarcoma is presented, together with a review of the literature. The prognostic and therapeutic implications of this particular neoplasm are discussed.

Adult↗

Spermatic cord liposarcoma.

The clinical and pathologic data in 3 cases of well-differentiated liposarcoma of the spermatic cord are presented. Radical orchiectomy appears to be adequate treatment. A regional lymph node dissection is usally not advocated since the pattern of spread is hematogenous.

Aged↗

Retroperitoneal liposarcoma presenting as spermatic cord tumor.

A well-differentiated liposarcoma of the retroperitoneum and spermatic cord, contiguous through the inguinal canal, presented clinically as a scrotal mass. Radical orchiectomy was performed with removal of the spermatic cord tumor. The retroperitoneal portion was not discovered until one month postoperatively when a computerized tomographic scan of the abdomen was done. A recommendation is made for preoperative evaluation of the retroperitoneal space prior to extirpation of supratesticular spermatic cord masses.

Diagnosis, Differential↗

Liposarcoma of spermatic cord.

Liposarcoma of the spermatic cord is a rare tumor. Most are of a low-grade malignancy, and treatment is wide local excision with radical orchiectomy. At present there is no clear indication for chemotherapy. Because recurrence, especially local recurrence, is a possibility, periodic followup is necessary. Local recurrences are treated with repeat wide local excision and radiotherapy.

Adult↗

Computed tomography in pelvic liposarcoma: a case report.

A case of well-differentiated pelvic liposarcoma with unusual presentation of low-back pain and sciatica, due to the herniation through the greater sciatic notch, is described. Computed tomography is invaluable, not only in predicting the histologic diagnosis, but also in outlining the location, extent, and size of the tumor. This allows accurate planning of the surgical approach.

Back Pain↗

Putative apolipoprotein receptor gene (LRP, A2MR) is not rearranged in either myxoid liposarcoma or lipomas with translocations in 12q13-14.

The APR, also known as LRP, gene is highly homologous to the low-density lipoprotein (LDL)-receptor and encodes a cell surface molecule with biochemical properties consistent with function as a lipoprotein receptor. This gene has been mapped to human chromosomal bands 12q13-q14, a region commonly altered in tumors of adipose cells. The proximity of APR to these breakpoints, coupled with its presumed role in lipid metabolism and possible affect on cell proliferation, suggest it as a candidate gene for adipose tissue tumor formation. Pulsed-field gel analysis was used to develop a physical map covering 750 kilobases (kb) surrounding this gene. Examination of myxoid liposarcomas and lipomas bearing the characteristic translocations (12;16)(q13;p11) or (12;variable)(q14;variable), respectively, excluded the breakpoints from within a 750-kb region surrounding the APR gene. These results suggest that APR is not involved directly in the genetic changes that underlie development or progression of these tumors.

Apolipoproteins↗

Analysis of a giant marker chromosome in a well-differentiated liposarcoma using cytogenetics and fluorescence in situ hybridization.

Well-differentiated liposarcomas (LPS) are cytogenetically very complex, characterized by giant marker chromosomes, ring chromosomes, and telomeric associations. We report a case of well-differentiated LPS in which the only cytogenetic anomaly was an additional giant marker. In an attempt to identify the origin of this marker, centromeric probes (chosen on the basis of the morphology of the marker) to chromosomes 1,2,3,4,6,7,8,9,10,11,12,16,17, and X and a shared satellite probe for chromosomes 1,5, and 19, were used with fluorescence in situ hybridization (FISH). This was successful at eliminating certain chromosomes as candidates for centromeric trisomy but could not identify the origin of the marker. This case is unusual in that it does not conform to the typical cytogenetic pattern for well-differentiated LPS and is the first known example with an apparently normal diploid karyotype with only one additional change.

Aged↗

Isolation of a yeast artificial chromosome clone that spans the (12;16) translocation breakpoint characteristic of myxoid liposarcoma.

Cytogenetic analysis of liposarcomas has demonstrated that translocation (12;16) (q13.3;p11.2) is characteristic of the myxoid subtype of this adipose tissue tumor. Our previous results suggested that the GLI gene is close to the translocation breakpoint on chromosome 12. We now describe a yeast artificial chromosome (YAC) that contains GLI and spans the chromosome 12 region involved in the t(12;16) breakpoint. This clone will permit rapid definition of the genetic region surrounding the breakpoint and allow isolation of the gene presumably affected by the translocation.

Chromosomes, Fungal↗

Liposarcoma arising in the cheek: report of a case and review of the literature.

The following points can be made about liposarcoma of the oral cavity: 1) it is rare and slow growing; 2) it is often mistaken for a benign lesion; 3) there is a direct correlation of microscopic appearance with biological behavior and prognosis; 4) treatment is primarily surgical, with radiation used for selected cases; and 5) the better-differentiated tumors seem to respond more favorably to radiation.

Cheek↗

Translocated in liposarcoma (TLS) is a substrate for fibroblast growth factor receptor-1.

Binding of fibroblast growth factor (FGF) to the high affinity receptor-1 (FGFR-1) leads to activation of its endogenous tyrosine kinase activity. A number of substrates for the FGFR-1 kinase have been identified. Among those, FGF receptor-substrate-2 (FRS-2) was identified by virtue of its interaction with p13suc, a yeast protein involved in cell cycle regulation. We have used immobilized p13suc to identify a new substrate for FGRF-1, which is identical to "translocated in liposarcoma" (TLS). TLS is a RNA/DNA-binding protein which occurs in fusion products with different transcription factors in a variety of solid tumours. We show that TLS is tyrosine phosphorylated in intact cells by a number of different growth factors, indicating a role in growth regulation.

Adaptor Proteins, Signal Transducing↗

Radiation response: an additional unique signature of myxoid liposarcoma.

PURPOSE: Historically, myxoid liposarcoma (MLS) has been reported to show a marked clinical response to radiotherapy (RT), but objective data to support that contention have been lacking. We performed a retrospective analysis of the response of a group of MLS tumors to preoperative RT using tumor dimensions calculated from pretreatment imaging and the subsequent surgical specimen obtained approximately 1 month after RT. METHODS AND MATERIALS: Data were abstracted from the Princess Margaret Hospital prospective database on a series of patients with MLS who had undergone preoperative RT between 1991 and 1999. A group of malignant fibrous histiocytoma (MFH) patients treated on the same protocol served as controls. Pretreatment and posttreatment three-dimensional measurements were obtained; comparison tumor volume measurements were estimated using the ellipsoid formula, and the analysis was also repeated using the maximal tumor dimension. Identical measurement procedures were used for the MLS (experimental) and MFH (control) cases. RESULTS: A total of 16 MLS tumor specimens were available for analysis, and 16 MFH cases were randomly chosen for comparison. The mean pretreatment and posttreatment volume of the MLS tumors was 415 and 199 cm(3), respectively (P = <0.0001). The mean pretreatment and posttreatment volume of the MFH tumors was 264 and 273 cm(3), respectively (p = 0.804). The proportional reduction in the median tumor volume was 59% and -7% for MLS and MFH tumors, respectively. Both the absolute and the proportional reduction in volume for MLS tumors vs. MFH tumors was statistically significant (p = 0.006 and p = 0.002, respectively). CONCLUSION: These results provide the first objective data to support the idea that MLS tumors show a statistically significant reduction in size when treated with RT and that this response is greater than that of MFH tumors given the same RT. These results may be relevant to the management of MLS with combined RT and surgery in difficult anatomic situations in which adequate surgical excision is not feasible. They also confirm another unique characteristic of this unusual subtype of soft tissue sarcoma.

Adult↗

Intensity modulated radiation-therapy for preoperative posterior abdominal wall irradiation of retroperitoneal liposarcomas.

PURPOSE: Preoperative external-beam radiation therapy (preop RT) in the management of Retroperitoneal Liposarcomas (RPLS) typically involves the delivery of radiation to the entire tumor mass: yet this may not be necessary. The purpose of this study is to evaluate a new strategy of preop RT for RPLS in which the target volume is limited to the contact area between the tumoral mass and the posterior abdominal wall. METHODS AND MATERIALS: Between June 2000 and Jan 2005, 18 patients with the diagnosis of RPLS have been treated following a pilot protocol of pre-op RT, 50 Gy in 25 fractions of 2 Gy/day. The Clinical Target Volume (CTV) has been limited to the posterior abdominal wall, region at higher risk for local relapse. A Three-Dimensional conformal (3D-CRT) and an Intensity Modulated (IMRT) plan were generated and compared; toxicity was reported following the National Cancer Institute (NCI) Common Terminology Criteria for Adverse Events v3.0. RESULTS: All patients completed the planned treatment and the acute toxicity was tolerable: 2 patients experienced Grade 3 and 1 Grade 2 anorexia while 2 patients developed Grade 2 nausea. IMRT allows a better sparing of the ipsilateral and the contralateral kidney. All tumors were successfully resected without major complications. At a median follow-up of 27 months 2 patients developed a local relapse and 1 lung metastasis. CONCLUSIONS: Our strategy of preop RT is feasible and well tolerated: the rate of resectability is not compromised by limiting the preop CTV to the posterior abdominal wall and a better critical-structures sparing is obtained with IMRT.

Abdominal Wall↗

Liposarcoma of the tongue: clinico-pathologic correlations of a possible underdiagnosed entity.

Liposarcoma (LS), one of the most common malignant tumors, is considered as a rarity in the oral cavity, the tongue being the most frequent site for its occurrence. The purpose of the present study was to review and analyze the clinico-pathologic correlations of 32 cases of LS of the tongue, including four new cases from the files of our department. A total of 32 cases of LS of the tongue were collected, 29 from a MEDLINE search of the English language literature (1966-2004). Since the histopathologic features of one of the four new cases have been presented previously, this case was counted only once. LS of the tongue is a tumor of adult and old age, with a mean age of approximately 62 years and a peak incidence in the seventh and eighth decades. Clinically, it is a long-standing tumor that commonly presents as a solitary nodular mass, but can also present as a multi-nodular lesion. The most common histopathologic type is that of atypical lipomatous tumor (ALT)/well-differentiated LS (75%). LS can be easily misdiagnosed with both benign and malignant lipomatous and non-lipomatous tumors. LS of the tongue was commonly treated by local excision, however, there were cases of (multiple) recurrences and dedifferentiation. When a lipomatous lesion is encountered in the oral cavity, and especially in the tongue, LS should certainly be among the lesions that top the list of differential diagnosis. The follow-up must be on a long-term basis since this tumor can recur years after initial surgical treatment and can also undergo dedifferentiation.

Adult↗

Epithelioid variant of pleomorphic liposarcoma: report of a case.

A case of an epithelioid variant of pleomorphic liposarcoma (PL) arising in the back of a 72-year old male is presented. The lesion was a well-circumscribed but unencapsulated, yellowish white mass measuring 11 x 10 x 9 cm in the subcutis and muscle. Histologically, the tumor consisted of three elements; an epithelioid cell element occupying about 50%, a lipogenic and mixed lipogenic and epithelioid cell element occupying 40%, and a malignant fibrous histiocytoma-like element. The lipogenic area was composed of uni- and multivacuolated pleomorphic lipoblasts. Immunohistochemically, the tumor was positive for vimentin, epithelial membrane antigen, and CD10. It was negative for desmin, alpha-smooth muscle actin, muscle actin, S-100 protein, and cytokeratins 8 and 18. The epithelioid variant of PL should be differentiated from metastatic renal cell carcinoma and adrenal cortical adenocarcinoma.

Aged↗