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PubMed1989

[Primary retroperitoneal tumors].

The Authors report 7 cases of primary retroperitoneal tumors observed in a five-year period. On the basis of the literature data, they examine the diagnostic, therapeutic and prognostic features, emphasizing the need for a surgical treatment supported, only when useful, by radio- and chemotherapy. The importance of an early diagnosis, thanks to modern technologic progress, is also stressed, echography in particular.

Adult↗
PubMed1989

[Retrospective analysis of 31 cases of soft tissue tumors. Comparison of MR and surgical and anatomo-pathological findings].

Twenty-five patients (16 with malignant soft-tissue tumors and 9 with suspected recurrence), previously studied with conventional radiography and Computed Tomography (CT), were examined with Magnetic Resonance (MR) imaging. MR findings were surgically and anatomo-histopathologically confirmed. Six more patients with benign lesions were examined for differential diagnosis. MR capabilities are here evaluated to detect and eventually characterize the lesion, and to assess its exact location. No false positives were found, and there was only one false negative, in a case of recurrent Malignant Fibrous Histiocytoma (MFH). In another case of MFH the infiltration of the adjacent vessels and nerves was not imaged. After a careful review of all the examinations, MR imaging appears to be able to provide much information for surgical planning about tumor extent and margins, thanks to its contrast resolution between tumor and surrounding anatomical structures. Therefore, MR imaging can be reasonably employed as the examination of choice when a soft-tissue tumor is clinically suspected, because of the multi-planar images it provides with and of its differential imaging parameters.

Adolescent↗
PubMed1993

[Sarcomatous degeneration of a giant scrotal lipoma. Presentation of a clinical case].

This paper presents a case of giant scrotal lipoma with areas of sarcomatous degeneration which were evident on anatomico-pathological examination; the authors considered it worth-while reporting this case given that giant lipomas with sarcomatous degenerations are rare. It is important to underline that in comparison to other malignant tumours of the scrotum the frequency of liposarcomas is very low. In addition to the liposarcoma reported here, at the level of the scrotum it is worth recalling neurofibrosarcoma and fibrosarcoma and some rare sarcomas of the spermatic cord among the malignant sarcomas of sarcomatous origin reported in the literature. Difficulties may arise from a diagnostic point of view due to the scarcity of symptoms. Among the most frequent symptoms is the presence of a hard mass on palpation of the scrotum; the volume of this mass may increase very rapidly. It is important to note that this tumour may sometimes originate as malignant and then degenerate in toto or in some isolated areas. Instrumental tests include abdominal radiography without the use of contrast medium in order to exclude the presence of herniated abdominal viscera at the level of the scrotum. Ultrasonography may also be extremely useful since it is capable of providing information regarding the presence, inside the scrotal sack, of liquid retention or solid forms. At all events histological diagnosis is carried out in the majority of cases following the removal of the operated part. The exeresis of the tumour therefore becomes both diagnostic and therapeutic. Surgery consists in the simple exeresis of the neoplasia; in some cases orchiectomy is required in addition to exeresis. Some authors also recommend the dissection of inguinal lymph nodes following extemporary anatomo-pathological tests. In other cases when histological tests confirm the malignant degeneration with a high degree of biological aggressiveness, the patient has to undergo postoperative radiotherapy and/or chemotherapy.(ABSTRACT TRUNCATED AT 250 WORDS)

Aged↗
PubMed2006

[Soft tissue sarcomas: update on molecular data].

Soft tissue sarcomas are rare and may be a source of problems for diagnosis and treatment. Four types of genetic disorders can be distinguished: translocations, gene amplifications, mutations and complex genetic imbalances. Detection of these disorders may help in diagnosis and in determining prognosis. Detection of specific translocation is recommended in synovial sarcoma, alveolar rhabdomyosarcoma or PNET diagnosis because of therapeutic consequences; in case of rarer histologic type (low grade fibromyxoid sarcoma, clear cell sarcoma, infantile fibrosarcoma...), it may confirm the diagnosis. In some cases, some translocations have a prognostic value (alveolar rhabdomyosarcoma) whereas it is discussed in others (synovial sarcoma). The techniques used to detect these translocations are very sensitive so it may be used to detect microscopical metastasis (bone marrow metastasis of alveolar rhabdomyosarcoma for example). Detection of MDM2 and CDK4 genes amplifications (FISH or quantitative PCR) may be sometimes useful in well differentiated and dedifferentiated liposarcomas diagnosis. Mutation detection of KIT or PDGFRA may help in GIST diagnosis and type of mutation is predictive of response to treatment. Study of complex genomic imbalances in sarcomas is not used in routine practice but remains useful in research.

Humans↗
PubMed1972

Human sarcomas contain RNA related to the RNA of a mouse leukemia virus.

Labeled DNA complementary to the RNA of the Rauscher leukemia virus was hybridized with RNA from the polysome fraction of human sarcomas. Eighteen out of 25 specimens contained RNA possessing homology to the RNA of the mouse leukemia virus but not to that of the unrelated viruses causing mammary tumors in mice or myeloblastosis in chickens. Further, no normal adult or fetal tissues showed significant amounts of RNA specific to mouse leukemia virus. It appears that human sarcomas contain RNA sequences homologous to those found in an agent related to a virus known to cause sarcomas in mice.

Animals↗
PubMed2001

Well-differentiated inflammatory liposarcoma presenting as pyrexia of unknown origin.

Liposarcomas have diverse histological appearance and clinical manifestations. Well-differentiated inflammatory liposarcoma is an uncommon sub-type, which often causes diagnostic difficulty. We report here a young female patient who presented with prolonged pyrexia and sub-diaphragmatic mass and was detected to have this uncommon soft tissue sarcoma on laparotomy.

Adult↗
PubMed1998

On telomere shortening in soft-tissue tumors.

PURPOSE: Specific simple DNA repeats occur at the telomeric ends of mammalian chromosomes. Loss of (G + C)-rich repeats can result in genetic instability, associated with tumorigenesis. So far, data on telomere shortening have not been available for different types of soft-tissue tumors. METHODS: Using tumor material and the blood of the corresponding patient, high-molecular-mass DNA was prepared by digestion with proteinase K and extraction with phenol/chloroform. A 10-microg sample of DNA was digested with the restriction enzyme HinfI. DNA fragments were separated in a 0.7% agarose gel, and in-gel hybridization was performed with the telomere-specific repeat probe (TTAGGG)3. RESULTS: Shortening of the telomere repeat was observed in 14/30 soft-tissue tumors; 5 tumors showed elongated telomere repeats, whereas the telomeres appeared unchanged in 11 tumors. Decreased telomere repeat length correlated with advanced age, DNA ploidy, and a higher proliferation index. There was no association between telomere repeat length and tumor grade. Interestingly, in contrast to other entities, all malignant schwannomas and leiomyosarcomas showed significantly reduced telomere lengths. An explanation for the telomere heterogeneity in liposarcomas may include differential telomerase reactivation in well and poorly differentiated tumors. CONCLUSIONS: Telomere shortening is frequent but not a uniform phenomenon in different types of soft-tissue tumor. Studies on telomerase activity should be performed in the same cohort of sarcomas.

Adult↗
PubMed2004

Sarcomas of the oral and maxillofacial region: a review of 32 cases in 25 years.

Thirty-two cases of sarcomas involving the oral and maxillofacial region over a period of 25 years were reviewed. The age range was from 5 months to 77 years with a mean age of 42. The male to female ratio was 3:1. The sarcomas were located in the maxilla including the maxillary sinus (n= 13), mandible (n= 13), buccal mucosa (n= 3), temporomandibular fossa (n= 2), and submandibular region (n= 1). Histologically sarcomas were classified as osteosarcoma (n= 9), malignant fibrous histiocytoma (n= 7), rhabdomyosarcoma (n= 5), fibrosarcoma (n= 3), plasmacytoma (n= 2), leiomyosarcoma (n= 2), angiosarcoma (n= 2), liposarcoma (n= 1), and ameloblastic fibrosarcoma (n= 1). Surgical resection was performed in 29 cases. Local recurrence was found in 10 patients and metastasis in 11 patients. Metastases included five regional lymph node metastases and eight distant metastases. The survival of patients with local recurrence or metastasis was poor. Surgery is the most reliable treatment for sarcomas of the oral and maxillofacial region. Adequate excision with safety surgical margin as the initial therapy is important for better survival. The value of radiation therapy and/or chemotherapy is uncertain. The 5-year survival rate of primary cases was 61%.

Adult↗
PubMed2004

[Clinical analysis of 48 cases sarcoma in nasal cavity and sinuses].

OBJECTIVE: To enhance the level of diagnose and treatment of sarcoma in nasal cavity and sinuses by studying the clinical features, diagnosis and treatment of 48 cases sarcoma in nasal cavity and sinuses. METHOD: Forty-eight cases sarcoma in nasal cavity and sinuses treated from 1995 to 2000 were analyzed retrospectively. RESULT: Fibrosarcoma in 4 cases, malignant fibrous histiocytoma in 3 cases, liposarcoma in 4 cases, leiomyosarcoma in 5 cases, rhabdomyosarcoma in 5 cases, osteosarcoma in 2 cases, chondrosarcoma in 3 cases, malignant melanoma in 4 cases, non-Hogkin's in 4 cases, extramedullary plasmacytoma in 1 case, Schwannoma in 5 cases, esthesioneuroblastoma in 5 cases, angiosarcoma in 3 cases. Following-up after synthetic treatment, the 1, 3, 5 year survival rates were 62.5%, 46.7%, 35.7% respectively. CONCLUSION: Sarcoma in nasal cavity and sinuses is not easy to diagnose definitely, immunohistochemistry is helpful for pathological diagnose. It's easy to recurrent and metastasis and it's beneficial to improve prognosis with synthetic treatment.

Adolescent↗
PubMed1992

Pleomorphic malignant fibrous histiocytoma: fact or fiction? A critical reappraisal based on 159 tumors diagnosed as pleomorphic sarcoma.

Pleomorphic malignant fibrous histiocytoma (MFH) is regarded as the most common soft tissue sarcoma of adulthood, but no definable criteria exist for its diagnosis. Possibly its only distinctive feature is its apparent lack of specific differentiation. To determine the validity of pleomorphic MFH, 159 tumors diagnosed as pleomorphic sarcomas have been reassessed morphologically, immunohistochemically, and ultrastructurally, where possible. Of these 97 cases (63%) proved to be specific sarcomas other than MFH, 20 proved to be nonmesenchymal neoplasms, and 42 were unclassifiable (of which 21 were either small biopsies or subtotally necrotic). Only 13% of these cases were eligible for consideration as MFH, but these showed no reproducible histological differences from the other tumors studied, nor was this group morphologically consistent. These tumors showed no evidence of true monocyte/macrophage differentiation. It is postulated that pleomorphic MFH is a noncohesive heterogeneous group of poorly differentiated neoplasms, a term that has become a meaningless diagnosis of convenience. With sufficient effort, a specific line of differentiation can be identified in the majority of pleomorphic malignant soft tissue tumors; with advances in investigative technology, the proportion that remain unclassifiable is very likely to diminish further in the future.

Adolescent↗