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At least 19 recordsLinked to original sources

Malignant mesenchymoma of median nerve: combined nerve sheath sarcoma and liposarcoma.

A malignant mesenchymoma consisting of nerve sheath sarcoma and liposarcoma occurred in the right median nerve of a 29-year-old man who did not have stigmata of von Recklinghausen neurofibromatosis. It produced pain at the affected part, referred pain to the palm, and weakness of muscles supplied by branches of this nerve. The tumor was first excised 1 month after its initial manifestation, but recurred twice and eventually metastasized to the lung within 19 months. It is suggested that aberrant differentiation of the mesenchymal component of the neural crest, the ectomesenchymal structure, has created this combined sarcoma of nerve sheath and liposarcoma, forming a malignant mesenchymoma.

Adipose Tissue↗

Nonangiogenic and nonlymphomatous sarcomas of the canine spleen: 57 cases (1975-1987).

The case records of and histopathologic findings in 57 dogs with nonangiogenic and nonlymphomatous splenic sarcomas were reviewed. Splenic neoplasms in these dogs included leiomyosarcoma, fibrosarcoma, undifferentiated sarcoma, liposarcoma, osteosarcoma, chondrosarcoma, myxosarcoma, rhabdomyosarcoma, and fibrous histiocytoma. The clinical signs associated with splenic sarcoma included anorexia or decreased appetite, abdominal distention, polydipsia, lethargy, vomiting, weight loss, and weakness. An abdominal mass was detected in 86% of the dogs by use of abdominal palpation (63%), and/or abdominal radiography (74%). The diagnosis was based on histopathologic findings in the spleen. Abdominal exploratory surgery was performed on 43 of the 57 dogs. Twenty-seven dogs were treated by splenectomy, and 16 were euthanatized at the time of surgery because of widespread metastatic lesions. Of the 14 dogs on which surgery was not performed, 11 were euthanatized on the basis of results of preoperative diagnostic tests, and the remaining 3 dogs had splenic neoplasms that were incidental findings at necropsy. Of the 27 surgically treated dogs, 5 died in the immediate postoperative period, 12 died or were euthanatized within 1 year after splenectomy, and only 5 dogs survived greater than or equal to 1 year. Three dogs were lost to follow-up evaluation, and 2 were still alive 6 and 7 months after surgery. The median survival time of the 22 dogs for which survival was known was 2.5 months. The median survival time for 11 dogs with no obvious metastasis at the time of splenectomy was 9 months.(ABSTRACT TRUNCATED AT 250 WORDS)

Animals↗

[liposarcoma. Aspects of pathomorphology--an analysis of 209 tumos].

BACKGROUND: Among the heterogeneous group of adult soft tissue sarcomas, liposarcomas represent the largest entity along with malignant fibrous histiocytomas (MFH). PATIENTS AND METHODS: This article summarizes the results of pathomorphological data on 209 liposarcomas resected over a 10-year period. RESULTS: The most common tumor site was the thigh, and the peak age incidence was in the 5th and 6th decades. In general, three major subtypes of liposarcoma can be distinguished in terms of pathomorphology: well-differentiated/dedifferentiated liposarcoma, myxoid/round cell liposarcoma, and pleomorphic liposarcoma. Well-differentiated liposarcomas represent malignancy grade 1 tumors without biological potential to metastasize, but which are able to relapse locally in cases of incomplete resection. When a local relapse has occurred, the liposarcoma may show dedifferentiation and may metastasize. CONCLUSIONS: In the pathologic-anatomical diagnosis of liposarcomas, conventional light-microscopic findings are decisive. Additional methods of molecular pathology may help in single cases to gain further insights.

Adult↗

Hypermethylation of the APC promoter but lack of APC mutations in myxoid/round-cell liposarcoma.

The adenomatous polyposis coli (APC) protein is a key component of the WNT signalling pathway wherein it acts as a scaffolding protein in controlling the level of the proto-oncoprotein beta-catenin. Although APC has been shown to be genetically or epigenetically inactivated in a variety of carcinomas, little is known about its role in sarcoma. Liposarcomas (LPSs) are the second most common soft tissue sarcoma in adults. Despite different histology and malignancy, the myxoid and round-cell LPSs belong to one tumour entity characterized by a specific chromosomal translocation. We assessed the extent of genetic and epigenetic inactivation of the APC gene in myxoid/round-cell LPS. Sequencing of the mutation cluster region, the protein truncation test and a loss of heterozygosity (LOH) analysis did not reveal any genetic alterations of the APC gene in all of the liposarcoma samples. Methylation of the APC promoter was detected by methylation-specific PCR in 9 of 20 (45%) tumours. Analysis of APC expression by semiquantitative RT-PCR in a subset of the samples demonstrated that tumours with a methylated APC promoter showed a downregulation of the APC transcript. However, APC downregulation was not correlated with a stabilisation of the beta-catenin protein. Thus, the epigenetic regulation of the APC gene might play an important role in the pathogenesis of myxoid/round-cell LPS. However, the impact of APC methylation on liposarcoma development is quite likely not mediated through WNT signalling.

Adult↗

[Pathology and histology of soft tissue sarcomas in the upper extremity: adults].

PURPOSE/BACKGROUND: Clinicopathologic characterization and demonstration of histologic findings in six common soft tissue sarcomas of the upper extremity in adult patients. RESULTS/CONCLUSION: Clinicopathologic findings and histologic features of the following malignant soft tissue tumours are compiled: epithelioid sarcoma, synovial sarcoma, clear cell sarcoma, liposarcoma, fibrosarcoma, and malignant fibrous histiocytoma (MFH). All these tumours show typical clinical presentations and characteristic histological findings.

Adolescent↗

Sternal reconstruction with synthetic mesh and metallic plates for high grade tumours of the chest wall.

OBJECTIVE: To present our experience of reconstruction of the chest wall after sternectomy for high grade tumours. DESIGN: Retrospective study. SETTING: University hospital, Italy. SUBJECTS: 18 patients who required sternectomy for high grade tumours. INTERVENTIONS: After wide resection of the tumour the sternum and chest wall were reconstructed with polypropylene (Marlex) mesh and mouldable titanium plates. Pedicled muscle flaps were used to complete the reconstruction. MAIN OUTCOME MEASURES: Morbidity, mortality, and outcome. RESULTS: The 18 tumours were chondrosarcomas (n = 7); osteosarcomas, radiation-induced sarcomas, and local recurrences after breast cancer (n = 2 each); and angiosarcoma, Ewing sarcoma, liposarcoma, malignant fibrous histiocytoma, and metastatic renal carcinoma (n = 1 each). One patient died in hospital. Two patients developed wound infections, one required repeated aspirations of a pleural effusion, and one patient later developed loosening of the plate that had been sutured to the clavicular stump. At the time of follow-up (mean 32 months, range 12-74) 16 patients were alive, all of whom had returned to their normal life style. CONCLUSIONS: The technique is easy to follow and has the advantages of a short hospital stay and good local control. It obviates the need for postoperative mechanical ventilation, and means that patients are not prevented from working because of incapacity.

Adult↗

Frequent beta-catenin abnormalities in bone and soft-tissue tumors.

We have screened mutations of the beta-catenin gene by using the polymerase chain reaction-single strand conformation polymorphism (PCR-SSCP) method in 62 malignant bone and soft-tissue tumors, including malignant fibrous histiocytomas (MFHs), osteosarcomas, synovial sarcomas, liposarcomas, malignant schwannomas, and other types of tumors, as well as 11 benign tumors. beta-Catenin-activating missense mutations were found in two malignant tumors. One found in MFH occurred at codon 45 and caused an amino acid substitution from serine (one of the GSK3 beta-targeted phosphorylation sites) to phenylalanine. The other, detected in synovial sarcoma at codon 32, resulted in an amino acid change from aspartic acid (located adjacent to the phosphorylation target, serine, encoded by codon 33) to tyrosine. Furthermore, we found accumulation of beta-catenin by western-blotting analysis in 12 of 19 malignant tumors in which we found no mutation involving exon 3. Our results suggested the possible involvement of beta-catenin activation, by beta-catenin gene mutation or alteration of other factor(s), in the formation and/or progression of various types of bone and soft-tissue tumors.

Bone Neoplasms↗

Pattern of primary musculoskeletal sarcomas referred to Institute of Radiotherapy and Oncology, Hospital Kuala Lumpur, 1995-1999.

An epidemiological study of 101 consecutive musculoskeletal sarcomas seen at the Institute of Radiotherapy and Oncology between 1995 and 1999 inclusive was carried out. The commonest sarcomas seen were osteosarcoma, rhabdomyosarcoma, Ewing's Sarcoma, liposarcoma, leimyosarcoma, malignant fibrous histiocytoma and chondrosarcoma; which collectively accounted for 84.2% of the group. Thirty patients (29.7%) presented with metastases. The commonest site of occurrence was lower extremity with 47.5%, followed by 34.7% in the trunk and peritoneum/axial skeleton, 9.9% in the head and neck region; and 7.90 in the upper extremity. We found no apparent relationship between race and incidence osteosarcoma and Ewing's sarcoma, as was reported by previous workers.

Adolescent↗

Expression of p21WAF1/CIP1 in soft tissue sarcomas: a comparative immunohistochemical study with p53 and Ki-67.

The p53 gene controls the cell cycle by transactivating p21WAF1/CIP1, a cyclin dependent kinase (cdk) inhibitor. By inhibiting cdks, p21WAF1/CIP1 regulates the cell cycle by blocking the G1 to S phase transition. In this study, we analyzed the immunohistochemical expression of p21WAF1/CIP1 in 66 soft tissue sarcomas and its relationship to p53 and the cell cycle proliferation antigen Ki-67. Expression of p21WAF1/CIP1, was detected in 76% of the tumors and p53 in 26%. All malignant schwannomas, synovial sarcomas, leiomyosarcomas and gastrointestinal stromal tumors expressed p21WAF1/CIP1. The majority of angiosarcomas, dermatofibrosarcomas, and fibrosarcomas showed low expression or were negative for p21WAF1/CIP1. Ewing's sarcomas, liposarcomas, and malignant fibrous histiocytomas were heterogeneous in their expression of p21WAF1/CIP1. Combining p53 and p21WAF1/CIP1 staining, the following four patterns were observed: 23% of the tumors showed the p53+/p21+ pattern; 53% showed the p53-/p21+ pattern; 3% showed the p53+/p21- pattern and 21% were negative for both p53 and p21WAF1/CIP1. There was no correlation between Ki-67 and p21WAF1/CIP1 or p53 staining. Our results show that soft tissue sarcomas, independent of their histologic subtype, frequently express p21WAF1/CIP1 which is probably important in their tumorigenesis. Additionally, p21WAF1/CIP1 may play a role in determining the efficacy of various cell cycle-directed therapies in soft tissue sarcomas.

Cyclin-Dependent Kinase Inhibitor p21↗

Small round cell neoplasms: can electron microscopy and immunohistochemical studies accurately classify them?

The answer to the question posed in the title, "Small Round Cell Neoplasms: Can Electron Microscopy and Immunohistochemical Studies Accurately Classify Them?", is obviously "yes"; but a qualified yes--generally yes, perhaps with expertise usually yes, but never just plain yes. Some cases certainly will defy the best attempts even of the most expert in the application of these "special" techniques. And embarrassing as it may be for those of us infatuated with the latest technology to admit, it is with the difficult case especially that old-fashioned technology so often must be depended upon. In his excellent recent appraisal of the role of a variety of special techniques in this application, Triche offers the following comment: "Overall, electron microscopy is probably the most universally useful of all diagnostic techniques other than light microscopy in round cell tumors." The data from our studies certainly point to the same conclusion. With each of the tumors, electron microscopy demonstrated itself to be more reliable than immunohistochemistry. Electron microscopy offers not only greater sensitivity and specificity, but also greater versatility. Immunohistochemistry allows hypothesis testing only. Electron microscopy, on the other hand, can provide answers even when the right questions are not being asked. For example, if a particular small round cell tumor under investigation happens in actuality to represent something other than the neuroblastoma which it is being considered (e.g., a granulocytic sarcoma, liposarcoma, Wilm's tumor, etc.), electron microscopy can reveal this fact, but a neuron-specific enolase stain cannot. Parenthetically, it should also be said that electron microscopy has proven particularly well suited to the examination of fine-needle aspiration specimens. The two spare many patients in our institution the need for a major operative procedure to establish a secure tissue diagnosis. Immunohistochemistry does have a role to play but it is, at least in our opinion, clearly secondary to that of electron microscopy. The concept of replacing electron microscopy with a battery of immunostains has often been advocated as an economic measure, but this argument begins quickly to lose its weight as the number stains included in the battery is increased to cover the diagnostic possibilities. Giving consideration to the capriciousness of some of these stains, there exists with this also an increasing possibility of a spurious or misinterpreted result leading to an errant diagnosis.(ABSTRACT TRUNCATED AT 400 WORDS)

Carcinoma, Small Cell↗

Comparative diagnostic aspects of herpes simplex virus tumor-associated antigens.

Sera from cancer patients and healthy individuals, obtained from two independent sources, were examined for their abilities to react with herpes simplex virus-associated tumor antigens, AG-4 and NVA-TAA (nonvirion antigen-tumor-associated antigen). Both antigens were prepared by infection of HEp-2 cells with herpes simplex virus type 2, and all antigen-antibody interactions were measured by the micro-complement fixation test. Of sera from 16 patients with cancer of the uterine cervix, 81% (P less than 0.01) reacted with NVA-TAA, whereas 78% (P less than 0.001) of 18 sera examined reacted with AG-4. These values differed significantly from those for normal sera, of which 14% reacted with NVA-TAA and 13% with AG-4. Of sera for 8 patients with squamous cell carcinoma of head and neck or vulva, 75% (P less than 0.02) reacted with NVA-TAA, whereas 63% (P less than 0.05) reacted with AG-4. As a group, other cancers (including adenocarcinoma of lung, breast, ovary, and cervix; liposarcoma; sarcoma; melanoma; and carcinoma of the endometrium) did not differ significantly from controls in reactive patterns with AG-4 or NVA-TAA. These studies partly supported the reported preferential reactivity of AG-4 and NVA-TAA with sera of patients with squamous cell carcinoma, especially of the uterine cervix.

Adult↗

Aggressive angiomyxoma of the female pelvis and perineum. Report of nine cases of a distinctive type of gynecologic soft-tissue neoplasm.

Nine case of a distinctive soft tissue tumor of the female pelvis and perineum are described. They were characterized by their occurrence in young women (ages 21-38), large size (up to 60 X 20 cm), locally infiltrative nature, and grossly gelatinous appearance. The initial clinical impression was usually that of a Bartholin gland cyst. The microscopic appearance was that of spindle or stellate cells widely separated by a loose myxoid stroma focally rich in collagen fibrils, a prominent vascular component, including many large thick-walled vessels without an arborizing pattern, and foci of proliferating glandular elements in two cases. Mitotic activity was exceedingly low. Ultrastructural study of the spindle cells showed features consistent with myofibroblastic differentiation. Four patients developed large local recurrences; one tumor recurred twice, 14 and 15 years after initial excision. No distant metastases have been documented to date, and all patients are alive and well. The differential diagnosis of this unusual tumor includes myxoma, myxoid liposarcoma, sarcoma botryoides, myxoid variant of malignant fibrous histiocytoma, nerve sheath myxoma, and other soft tissue tumors with secondary myxoid changes. We have chosen the term "aggressive angiomyxoma" for this neoplasm to emphasize the neoplastic nature of the blood vessels and its locally infiltrative and recurrent nature.

Adult↗

Reaction of antigens isolated from herpes simplex virus-transformed cells with sera of squamous cell carcinoma patients.

Antigens isolated from herpes simplex virus type 1, herpes simplex virus type 2, or cytomegalovirus-transformed hamster cells were tested against 66 sera from non-cancer individuals or patients with different types of cancer. By use of the microcomplement fixation procedure to quantify all antigen-antibody interactions, it was observed that 94% (p less than 0.001) of all sera from patients with squamous cell carcinoma reacted with antigens from herpes simplex virus type 1-transformed cells, while 84% (p less than 0.001) of the same sera reacted with antigen preparations from herpes simplex virus type 2-transformed cells. When sera from patients with adenocarcinoma, sarcoma, liposarcoma, and melanoma were tested against these antigens, there was no significant difference in their reactivity from sera of noncancer patients. When sera from all individuals (normal and cancer) were tested against antigens from cytomegalovirus-transformed cells, no significant reaction pattern developed. These studies are the first to describe the isolation of a reactive tumor-associated protein from herpes simplex virus-transformed cells.

Adult↗

[The treatment of retroperitoneal soft tissue sarcomas an analysis or 110 cases].

We analyzed the treatment results of 110 patients with retroperitoneal soft tissue sarcomas. Liposarcoma (31.8%) was most common followed by leiomyosarcoma and malignant peripheral nerve sheath tumors (11.8% and 11.8%, respectively). Complete resection was performed in 56 patients (50.9%), of which 18 underwent reoperation for 2-4 times. Adjacent organs were included in 13 patients in the en bloc rejection. Overall 5-year survival rate was 55.6% for those undergoing complete resection, compared to 11.1% and 6.6% respectively for those undergoing partial resection and biopsy only (P < 0.001). 37.5% of patients survived 5 years after their recurrent tumors were completely resected. Adjuvant radiation therapy or chemotherapy failed to have significant impact on survival.

Adolescent↗

Pleomorphic Liposarcoma: Comprehensive Genomic Analysis of 39 Cases With Comparison to Other Genomically Complex Sarcomas.

Pleomorphic liposarcoma (PLPS) is an aggressive high-grade sarcoma that often shows diverse morphological features and can mimic high-grade undifferentiated pleomorphic sarcoma (UPS)/spindle cell sarcoma or myxofibrosarcoma (MFS), especially when pleomorphic lipoblasts are sparse. The molecular profile of PLPS is distinct from well differentiated/dedifferentiated liposarcoma and myxoid liposarcoma. In this study, we investigate 39 cases of PLPS by comprehensive genomic profiling, occurring in 32 patients with available molecular data. Cases were reviewed and morphologic parameters-lipoblastic component, UPS-like, and MFS-like areas were estimated. The genomic findings were collected and compared to UPS and MFS groups studied using the same platform. The cohort included 15 females and 17 males, with a median age of 56.5 (range, 34-78). The lower extremity (n&#x2009;=&#x2009;17) was the most common site involved, followed by upper extremity (n&#x2009;=&#x2009;5) and pelvis (n&#x2009;=&#x2009;5). UPS-like and MFS-like patterns were the most common morphologic variants, ranging from 15% to 95% and 20% to 90%, respectively. TP53 (87%) and RB1 (51%) mutations and copy number alterations were the most common alterations seen, followed by ATRX (36%). Compared to UPS and MFS, TP53 and RB1 gene alterations were significantly more common in PLPS. Conversely, CDKN2A/B deletions were infrequent in PLPS. Survival analysis showed that MYC amplification was associated with significantly shorter overall survival in PLPS. Among histologic variants, CYSLTR2 alterations were found to be highest in cases with predominantly pleomorphic lipoblasts; additionally, strong correlations were found between gene alteration frequencies of MFS and MFS-like PLPS, and between UPS and UPS-like PLPS. RB1 allele-specific copy number analysis showed loss of heterozygosity in 82% of cases. Our cohort of PLPS showed a complex molecular landscape with distinct genetic alterations, histologic correlations, and clinical outcomes, highlighting its unique position among genomically complex sarcomas and providing insights that may inform future diagnostic and therapeutic approaches.

Humans↗

Dedifferentiated liposarcoma. Clinicopathologic analysis of 32 cases suggesting a better prognostic subgroup among pleomorphic sarcomas.

Dedifferentiated liposarcoma is uncommon, and only a small number of cases have been documented. We describe the clinicopathologic features in a series of 32 cases. All patients were adults (age range, 37-83 years; median, 67 years); 20 were men and 12 were women. Commonest site was the retroperitoneum (fifteen cases); six cases arose in the limbs, four in the paratesticular region, three in the peritoneal cavity, two on the trunk, and one each in the buttock and larynx. One primary tumor was subcutaneous. Thirty tumors arose de novo (i.e., combined with well-differentiated tumor in the primary lesion), while two developed in recurrences of a previously well-differentiated liposarcoma. The well-differentiated component was most often lipoma-like and typically there was a histologically abrupt transition to spindle celled nonlipogenic tumor. The dedifferentiated component most often resembled either storiform "malignant fibrous histiocytoma" ("MFH") with limited pleomorphism or myxofibrosarcoma (myxoid "MFH"); the latter pattern is rarely otherwise seen in the retroperitoneum. A small number of cases showed appearances reminiscent of myxoid embryonal rhabdomyosarcoma. An unusual feature in three cases was the occurrence of a discontinuous micronodular pattern of dedifferentiation. Average follow-up of 5.6 years (range, 3 months to 33 years) in 27 cases have revealed local recurrence in 14 patients and systemic metastases in only four patients. The primary sites of the metastasising cases were upper back, thigh, retroperitoneum, and paratesticular region. There have been only seven tumor-related deaths. Good prognosis in de novo dedifferentiated liposarcomas seems unrelated to the extent or morphologic pattern of dedifferentiation. However, high mitotic activity in the dedifferentiated component was associated with a more aggressive clinical course. Our study underlines that dedifferentiation in peripherally located or even subcutaneous liposarcomas does occur, albeit rarely, and that dedifferentiated liposarcomas of the limbs may metastasize. The results suggest that dedifferentiated liposarcomas, as a subgroup among the "MFH-like" sarcomas, have a better prognosis than pleomorphic sarcomas as a whole.

Adult↗