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[Liposarcoma of the hypopharynx].

Liposarcomas (LS) of the hypopharynx are uncommon tumors. All the literature considers laryngeal and hypopharyngeal liposarcomas together, and they represent 10-15% of all LS of the head and neck. Obstruction of the upper respiratory tract and dysphagia are usually the initial symptoms. The histological subtype is the most important prognostic factor. Surgery is the treatment of choice. Recurrences are frequent. We present a case about a liposarcoma in a 81 years-old man. Diagnosis, evolution and treatment are evaluated.

Aged↗

[Giant retroperitoneal liposarcoma: report of a case].

Liposarcoma is a rare and primary malignancy developed from mesenchymal tissue. We report the case of a 32-year-old woman who had a complete exeresis of a 15 cm retroperitoneal myxoid liposarcoma. Three months later, a 9 cm local recurrence was excised and radiotherapy was performed. In 2000, a local recurrence and an intestinal localisation of the liposarcoma were observed. The retroperitoneal tumor could not be removed and she had an intestinal resection followed by chemotherapy. With the literature data, we review the pathological, therapeutic and prognostic aspects of this tumor.

Adult↗

Surgical resection of recurrent bilateral mediastinal liposarcoma through the clamshell approach.

Primary mediastinal liposarcoma is an unusual variant of mediastinal neoplasms. We describe a long-term survivor who underwent repeated operations. After resections through a posterolateral thoracotomy and median sternotomy, a third operation was performed for recurrent bilateral huge tumors through a clamshell incision, and both tumors were removed en bloc. Results of pathologic examination showed that both tumors were well-differentiated liposarcoma. The patient is currently well 16 years after the first operation. Aggressive surgical intervention whenever possible appears to improve the quality of life and prolong the survival of patients with mediastinal liposarcoma.

Female↗

Tumor karyotype differentiates lipoblastoma from liposarcoma.

Lipoblastoma is a rare benign pediatric soft tissue tumor that may be difficult to distinguish from a myxoid liposarcoma clinically or histologically. The authors present a case of a progressively growing, locally invasive soft tissue tumor in a child. Tissue culture for cytogenetic study showed a breakpoint in the long arm of chromosome 8. A review of the literature showed seven case reports of lipoblastoma karyotype, six of which had a similar breakpoint in chromosome 8. This distinguishes it from the histologically alike myxoid liposarcoma, the karyotype of which typically contains the clonal anomaly t(12;16)(q13:p11). The authors recommend that when performing a biopsy of a childhood adipose tumor with unusual features, such as progressive or invasive growth, that fresh tissue be submitted for cell culture. The tumor karyotype will, in most cases, aid in differentiating lipoblastoma from myxoid liposarcoma.

Chromosomes, Human, Pair 8↗

Giant pelvic retroperitoneal liposarcoma.

BACKGROUND: Pelvic retroperitoneal liposarcomas are rare in young women (under 30 years old). We present a case of a giant well-differentiated liposarcoma of the retroperitoneum in a young woman. CASE: A 27-year-old nulligravida presented with rapid abdominal enlargement. Pelvic examination found a huge mass extending from the left adnexa to the epigastric region. At surgery, the pelvic organs were displaced to the right side by a retroperitoneal mass that marginally involved the left fallopian tube. The left ovary, uterus, and right adnexa were not involved and were not removed. No adjuvant irradiation was given. The patient was alive and well 2 years after the operation and was 23 weeks pregnant. CONCLUSION: Gynecologists should be familiar with pelvic retroperitoneal liposarcomas and their treatment.

Adult↗

Primary liposarcoma of the orbit. Problems in the diagnosis and management of five cases.

The clinicopathologic features and the results of diagnostic studies were reviewed in five patients with primary orbital liposarcoma. The major clinical complaints were diplopia and proptosis; visual function was typically well maintained. In three cases, computed tomographic (CT) scans displayed lesions with a central fat density rimmed by a variably radiodense pseudocapsule, leading to the erroneous clinical impression that the lesions were cystic in character. Ultrasonography ruled out a truly cystic tumor by demonstrating internal acoustic interfaces. Magnetic resonance imaging (MRI) in one case showed hyperintense signals in T-1-weighted images, suggesting the presence of fat within the lesional tissue. In the other two cases, CT scans showed reticulated densities accentuating the septa of the orbital fat in the absence of clear-cut circumscription. A potentially useful feature pointing to a neoplasm was bowing of an involved extraocular muscle, which is usually straightened and splinted in either Graves' disease or inflammatory pseudotumor. Histopathologically, two lesions were well-differentiated liposarcomas and three were myxoid liposarcomas, all featuring univacuolar signet ring lipoblasts. Scattered bizarre hyperchromatic mesenchymal cells without prominent lipidization were present in the lesions. None of the patients has experienced regional or distant metastases with follow-ups from onset of 1 to 7 years (mean, 5.2 years); three required orbital exenteration after local recurrence; and two who refused exenteration received only orbital radiotherapy.

Adult↗

Overrepresentation of 1q21-23 and 12q13-21 in lipoma-like liposarcomas but not in benign lipomas: a comparative genomic hybridization study.

Twenty lipomatous tumors, including eight lipoma-like liposarcomas and 12 benign lipomas, were analyzed using comparative genomic hybridization (CGH). DNA sequence copy number changes detected in five lipoma-like liposarcomas (mean, 1.1 aberrations/tumor; range, 0-2) consisted of gains of 12q13-21 (five tumors) and 1q21-23 (four tumors). Two of the tumors showed high-level amplification at 12q14-21 and one tumor at 1q21-22. No copy number changes were found in lipomas. Overrepresentation of 1q and 12q sequences was a recurrent finding in lipoma-like liposarcomas but not in lipomas. Thus, CGH may help in the differential diagnosis of low-grade or borderline adipose neoplasms.

Adult↗

Primary lung liposarcoma.

Liposarcoma of the lung is extremely rare with only 11 cases previously reported. A case of a locally advanced pulmonary pleomorphic liposarcoma in a 49-year-old male is presented. Surgery was performed but radical resection was not possible. Post-operative radiotherapy (40 Gy) was given concurrently with cisplatin (12.5 mg daily for 10 days). A partial response of short duration was obtained. The patient died 8 months following surgery with disseminated disease. Based on reports in the literature, liposarcoma of the lung must be classified as an aggressive highly metastatic disease responding poorly to both chemotherapy and radiotherapy.

Chemotherapy, Adjuvant↗

[Dedifferentiated liposarcoma of spermatic cord: degeneration of lipoma previously resected].

Spermatic cord sarcomas are rare tumours. Dedifferentiated liposarcoma accounts for only 10% of all spermatic cord sarcomas. These are usually large-sized tumours histologically characterised for being well-differentiated liposarcomas with some high grade sarcoma areas. Volume, location, mass homogeneity as well as presence of pelvic and retroperitoneal adenopathies are reported by CT and ultrasound techniques. These are useful for post-treatment follow-up. This paper presents one spermatic cord, dedifferentiated liposarcoma from which lipomas from the same spermatic cord had been previously removed in three occasions. We believe this is a degeneration of the earlier resected lipoma. Management is by extended inguinal radical orchiectomy. Value of adjuvant radio- and chemotherapy is uncertain. Post-surgery local relapses are common, and haematogenous and pelvic nodes metastasis likely. Survival at 5 and 10 years is 75% and 63% respectively.

Genital Neoplasms, Male↗

[Giant retroperitoneal liposarcoma].

Liposarcoma is a malignant tumor that has an embryologic origin from mesodermal tissue depending on fatty tissue. Although liposarcoma is only 0.1% of all human neoplasms, it is the most common histology subtype of retroperitoneal soft tissue sarcomas. This tumor grows slowly. Diffuse abdominal pain is its most frequent symptom and abdominal mass is the most common sign. Aggressive surgical treatment is basic to get a complete resection and a local disease control. This objective is difficult because of the large tumor size it gets in the retroperitoneal location and the multiorgan involvement that require the resection of a high percentage of contiguous organs. We report a case of a giant retroperitoneal liposarcoma presenting like continuous left hemiabdominal pain because of the visceral compression. The right kidney was involved and suffering from renal vessel enlargement without renal function. There is a high probability of microscopic residual disease and a good follow-up of the patients is necessary as well as an adjuvant radiation therapy in some cases.

Abdominal Pain↗

[Spermatic cord liposarcoma. Association with prostate cancer. Report of a case and review of literature].

Spermatic Cord Liposarcoma are uncommon soft tissue neoplasm. Association with others tumors is so exceptional. We describe and relation between liposarcoma and prostate cancer in a 66 years old patient who had a left paratesticular tumor with low speed growth and 12 cm of length; nodule in prostate gland was detected. Ecography demostrate an hipoecoic tumor in the spermatic cord; Prostate Specific Antigen (PSA) was 1276 ng./ml. and bone gammagraphy reported metastatic lesions. We made an radical orquiectomy and pathological diagnosis including inmunohistoquimical process was sclerosing dedifferenciated liposarcoma. We discuss clinical and pathologic behaviour of this lesions and diagnosis and treatment options.

Adenocarcinoma↗

Late recurrence of liposarcoma simulating adenoma of the duodenum.

A large pedunculated, polypoid mass in the duodenum of a patient with asymptomatic anaemia, with mucosal biopsies indicating a villous adenoma, turned out to be a liposarcoma during laparotomy. The patient had had a completely resected retroperitoneal liposarcoma 8 years before. Liposarcoma recurrence should be highly suspected even in case of atypical presentation and long disease free interval.

Adenocarcinoma↗

Myxoid liposarcoma with t (12; 16)(q 13; p 11). Possible usefulness of chromosome analysis in a poorly differentiated sarcoma.

A chromosomal study was used to establish diagnosis of a poorly differentiated soft-tissue sarcoma occurring in the right thigh of a 57-year-old Japanese female. Histopathologically the excised tumor consisted of a poorly differentiated myxoid neoplasm, without specific features to enable the identification of neoplastic cells. Although a tentative diagnosis of poorly differentiated myxoid liposarcoma was made, ultrastructural examination and Oil Red O fat stain failed to demonstrate the evidence of lipoblastic differentiation, except that occasional cells possessed a small number of fine fat droplets. The diagnosis of liposarcoma was suggested by chromosome analysis of the fresh tumor tissue after short time culture and trypsin-Giemsa banding technique. The tumor cells demonstrated a clonal abnormality characterized by a reciprocal translocation, t(12; 16)(q 13; p 11), which is known as a specific aberration in myxoid liposarcoma. Thus, chromosome study seems to be useful for identifying undifferentiated mesenchymal tumors, which lack morphologic evidence of any specific differentiation, as in the present case.

Cell Differentiation↗

Foci of signal intensities different from fat in well-differentiated liposarcoma and lipoma: correlation between MR and histological findings.

To investigate the histological features of foci, which showed signal intensities different from fat by magnetic resonance (MR) imagings in well-differentiated lipoma-like liposarcomas and a case of lipoma, a retrospective review of these lipomatous tumors was performed to correlate MR and histological findings. Microscopic findings revealed that these foci were also composed of lipomatous tissue. Well-differentiated liposarcoma and benign lipoma associated with such foci should be differentiated from dedifferentiated liposarcoma based on the histological findings.

Adipose Tissue↗

Successful removal of a giant recurrent mediastinal liposarcoma involving both hemithoraces.

Primary liposarcomas of the mediastinum are unusual tumors. We report herein a case of a 52-year-old woman, who was found to have a mediastinal tumor involving both hemithoraces and radiologically showing non-resectable-invasive features to the adjacent vital structures. She had a history of left thoracotomy for mediastinal schwannoma 14 years previously. The patient underwent an exploratory thoracotomy following a preoperative misdiagnosis of an ancient schwannoma. Complete removal of the tumor was accomplished through a right posterolateral thoracotomy with a subsequent histological diagnosis of a recurrent low-grade liposarcoma. A resectable liposarcoma should be considered in the differential diagnosis of a mediastinal tumor, although radiologically, the tumor presents with invasive features.

Diagnostic Errors↗

[Liposarcoma of the larynx. Review of the literature apropos of a case].

Liposarcoma of the larynx is an uncommon tumor. Only 27 cases have been described in literature. We report a new case occurring in a 50-year-old patient. The treatment consisted of a total laryngectomy with lymph node dissection followed by adjuvant irradiation. Prognosis for laryngeal liposarcoma is better than that of non laryngeal liposarcoma. This tumor is at high risk of local recurrence and seldom has metastatic potential.

Combined Modality Therapy↗

Translin binds to the sequences adjacent to the breakpoints of the TLS and CHOP genes in liposarcomas with translocation t(12;6).

Myxoid and round-cell liposarcomas share the translocation t(12;16)(q13;p11) creating the TLS-CHOP fusion gene as a common genetic alteration. We previously reported several unique characteristics of genomic sequences around the breakpoints in the TLS and CHOP loci, and among them was the presence of consensus recognition motifs of Translin, a protein that associates with chromosomal translocations of lymphoid neoplasms. We further extended our search for Translin binding motifs in sequences adjacent to breakpoints and investigated whether Translin binds to these sequences in vitro by mobility-shift assay. Computer-assisted search found sequences highly homologous (>70%) with Translin binding motifs adjacent to the breakpoints in 10 out of 11 liposarcomas with the TLS-CHOP fusion genes. All of 13 oligonucleotides corresponding to the putative binding sequences in these cases bind to Hela cell extract and also recombinant Translin protein, although the binding affinity of each motif showed considerable differences. The DNA-protein complex formation was inhibited by non-labeled competitor or anti-Translin antibody, suggesting the specificity of the complex formation. Considering the high incidence and specific binding property, the presence of Translin binding motif may be one of the important determinants for the location of breakpoints in the TLS and CHOP genes in liposarcomas.

Binding Sites↗

Paratesticular liposarcoma with smooth muscle differentiation mimicking angiomyolipoma.

AIMS: To discuss the differential diagnosis of a case of well-differentiated liposarcoma which had areas resembling angiomyolipoma-a feature which, to our knowledge, has not been reported previously. METHODS AND RESULTS: A tumour in the paratesticular region had apparently been present for 40 years, but had grown recently. A fat component containing lipoblasts was admixed with areas resembling angiomyolipoma, i.e. desmin positive, but HMB45-negative smooth muscle proliferation with atypia and thick-walled blood vessels devoid of elastin. CONCLUSION: The diagnosis of liposarcoma, rather than angiomyolipoma with adipose atypia, in this case is based on the fact that smooth muscle differentiation is documented in liposarcoma, lack of HMB45 staining and recent clonality studies which suggest that the fat in angiomyolipoma is not neoplastic.

Angiomyolipoma↗