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[Acute ischemia of the hand as a complication of radial artery catheterization. Apropos of 2 cases following abdominal sarcoma surgery].

Arterial catheterization is a simple technique that yields great benefits, such as continuous monitoring of arterial pressure and the possibility of taking repeated samples for analysis. However, it is not free of complications, the main ones being limb ischemia and gas embolism. To reduce the risk of complications, guidelines for insertion and maintenance of arterial catheters have been established. We report two cases of acute hand ischemia secondary to arterial catheterization. Both patients were undergoing surgery for sarcoma-type abdominal cancer and developed acute ischemia of the hand lasting several hours. The predisposing factor in both cases was the existence of a highly advanced sarcoma-type abdominal tumor, probably related to a state of hypercoagulability.

Acute Disease↗

Cytochrome P450 expression is a common molecular event in soft tissue sarcomas.

Two major xenobiotic metabolizing sub-families of cytochrome P450 (cytochrome P450 1A and cytochrome P450 3A) have been identified in soft tissue sarcomas. Cytochrome P450 1A was present in 70 per cent and cytochrome P450 3A was present in 78 per cent of tumours, respectively. A high proportion (86 per cent) of those tumours which contained cytochrome P450 1A or cytochrome P450 3A demonstrated co-expression of both sub-families. In each tumour, cytochrome P450 immunoreactivity was identified in all tumour cells and there was no intra-tumour heterogeneity. These results indicate that expression of cytochrome P450 is a common molecular event in soft tissue sarcomas and that the presence of different sub-families of cytochrome P450 has implications both for the pathogenesis and for the treatment of these tumours. Cytochrome P450 expression may influence the intrinsic drug resistance of these tumours and also provide a molecular target for anti-cancer drugs which can be activated by cytochrome P450.

Aged↗

Lymph nodes as sites of metastases from sarcomas of soft tissue.

Records of 323 patients with TNM Stage M0 sarcoma of soft tissue treated by the Radiation Medicine Service of the Massachusetts General Hospital over a 14-year period were reviewed to study the incidence and the implication of regional lymph node involvement. Nineteen patients (5.9%) had evidence of sarcoma metastatic to draining lymph nodes, zero of 63 (0%) were Grade 1 sarcomas, two of 118 (2%) were Grade 2, and 17 of 142 (12%) were Grade 3 sarcomas. Among patients with Grade 3 sarcomas, rhabdomyosarcoma (five of 14), vascular sarcoma (two of five), and epithelioid sarcoma (four of five) were associated with a higher incidence of lymph node involvement than synovial sarcomas (zero of four), fibrosarcomas (zero of 16), malignant fibrohistiocytomas (one of 29), neurofibrosarcomas (one of eight), liposarcomas (one of 14), and leiomyosarcomas (one of ten). From the 19 patients who had evidence of metastatic nodes, six (32%) were alive more than 58 months after the treatment of the nodes; four of six patients were without further tumor. The data of this study are compared with those cited in a review of the literature.

Humans↗

Necrosis observed on CT enhancement is of prognostic value in soft tissue sarcoma.

Fifty-one patients with deep-seated soft tissue sarcoma of the extremities and trunk wall were examined with contrast-enhanced CT for presence of nonenhanced tumor areas (CT necrosis). After a median follow-up time of 3 years, 19 of the 41 patients with CT necrosis had developed metastases, compared to none of the 10 patients who had tumors without CT necrosis. Tumors with CT necrosis were larger than tumors without, but in tumors of similar size, absence of CT necrosis was a favorable prognostic sign.

Adult↗

Telomere lengths of translocation-associated and nontranslocation-associated sarcomas differ dramatically.

Sarcomas can be divided into those with specific translocations displaying monotonous cytomorphology, and those with complex karyotypes and marked cellular pleomorphism. Telomeres contain terminal DNA sequence repeats that maintain chromosomal stability. Telomeres shorten with cell division and may become dysfunctional leading to chromosomal instability. Using a fluorescence in situ hybridization/immunofluorescence method to assess telomere lengths in archival tissues we analyzed these two types of sarcomas using paraffin-embedded primary tumor specimens. Tissues from nine sarcomas with characteristic translocations (two synovial sarcomas, two alveolar rhabdomyosarcomas, two desmoplastic round cell tumors, and one each of infantile fibrosarcoma, myxoid liposarcoma, cellular congenital mesoblastic nephroma) and nine without (four malignant fibrous histiocytomas, two leiomyosarcomas, one pleomorphic rhabdomyosarcoma, one dedifferentiated chondrosarcoma, and one malignant peripheral nerve sheath tumor) were analyzed. In all (nine of nine) cases with specific translocations, which generally have few karyotypic abnormalities, telomere lengths were similar to or reduced compared to surrounding nonneoplastic tissues. In contrast, telomeres in cases lacking specific translocations, which generally contain complex karyotypes, were often found to be dramatically lengthened and heterogeneous. In addition to markedly elongated telomeres, seven of nine (78%) complex cases exhibited large brightly stained regions corresponding to a specific type of promyelocytic leukemia nuclear body found in immortalized cells that maintain telomeres in a telomerase-independent manner [alternative lengthening of telomeres (ALT) pathway]. This phenotype is unlike that of epithelial neoplasms that typically display complex karyotypes with abnormally short telomeres maintained by the enzyme telomerase. The discovery of heterogeneous telomere lengths and evidence of the ALT pathway in the majority of sarcomas with complex karyotypes supports the existence of a telomere maintenance pathway incapable of full karyotypic stabilization in pleomorphic sarcomas. These findings provide additional molecular-genetic evidence supporting the dichotomous grouping of sarcomas into those with characteristic signature translocations without extensive additional karyotypic abnormalities, and those without such signature translocations that typically display very complex karyotypes, and point to telomere dysfunction as a plausible contributor to the chromosomal aberrations found in complex sarcomas.

Cell Nucleus↗

Detection of TLS/FUS-CHOP fusion transcripts in myxoid and round cell liposarcomas by nested reverse transcription-polymerase chain reaction using archival paraffin-embedded tissues.

The reciprocal translocation t(12;16)(q13;p11) has been shown to be highly characteristic of myxoid and round cell subtypes of liposarcoma, and the TLS/FUS-CHOP fusion gene that resulted from the translocation is expected to be a diagnostic molecular marker of these sarcomas. In this study, we conducted a nested reverse transcription-polymerase chain reaction (RT-PCR)-based assay to detect the TLS/FUS-CHOP fusion gene transcripts using archival formalin-fixed, paraffin-embedded tumor specimens. Of 18 paraffin-embedded specimens from 16 myxoid and round cell liposarcoma cases, the fusion transcripts could be identified in 16 (89%) specimens from 15 (94%) cases. A sequence analysis using the PCR products confirmed that the detected messages were derived from either type I or type II TLS/FUS-CHOP fusion gene, the latter of which was predominant (80%). The results were consistent in primary and recurrent lesions of the same patients and in paraffin-embedded and snap-frozen samples from the same tumors. In two negative specimens, transcripts of the beta-actin gene could not be detected by RT-PCR, and intact mRNA including the fusion messages might have been degraded. No fusion transcripts were detected in snap-frozen or paraffin-embedded material of other types of tumors with myxoid morphology (seven myxoid malignant fibrous histiocytomas and four lipomas with myxoid change). These results indicate that this molecular assay can be applied to formalin-fixed, paraffin-embedded tumor tissues as a diagnostic aid for these subtypes of liposarcoma.

Adolescent↗

Collision tumor of the thyroid: a case report of metastatic liposarcoma plus papillary thyroid carcinoma.

BACKGROUND: The term collision tumor represents the proximal coexistence of two histologically distinct tumors. Collision tumors within the thyroid are extremely rare, and reported cases are usually of mixed histologies of papillary and medullary carcinomas. Metastatic disease to the thyroid is also an uncommon scenario but may develop after long disease-free intervals. Carcinomas, most commonly renal cell carcinoma, are the likeliest group of neoplasms to metastasize to the thyroid. Soft tissue sarcomas metastasize to the thyroid with a much lower frequency. METHODS: We are reporting this highly unusual case of metastatic liposarcoma from the thigh to thyroid, which harbored a collision tumor of papillary thyroid carcinoma. This 86-year-old woman had undergone an initial resection for liposarcoma of the thigh two decades before being seen with a thyroid mass. RESULTS: Histologically, the thyroid mass was a metastatic myxoid liposarcoma, identical to the primary neoplasm. Papillary thyroid carcinoma was present within this metastasis and in the adjacent thyroid. CONCLUSION.: For any patient seen with a thyroid mass with a history of prior malignancy, no matter how remote, metastatic disease should be considered in the differential diagnosis.

Aged↗

[Perineo-scrotal liposarcoma. Apropos of 3 cases].

The authors report 3 rare cases of liposarcoma occurring in the perineal or paratesticular regions. They review literature data concerning this particular form of soft tissue sarcoma and emphasize on prognosis which depends mainly on its histopathological type.

Adolescent↗

Secondary tumors in bone sarcomas after treatment with chemotherapy.

New oncologic treatments have improved survival in osteosarcoma and Ewing's sarcoma. However, these treatments may cause secondary malignancies after radiotherapy. This study evaluated the incidence of secondary malignancies after neoadjuvant chemotherapy. Between April 1972 and December 1990, 518 osteosarcoma and 299 Ewing's sarcoma patients entered neoadjuvant chemotherapy protocols. Follow-up records of all patients were analyzed and malignant tumors were reported. Nine patients developed another malignancy, including 5 leukemias, 1 astrocytoma, 1 liposarcoma, 1 parotid, and 1 breast carcinoma. Four leukemias were found in patients treated for osteosarcoma with chemotherapy, but not radiotherapy. Only one leukemia developed after Ewing's sarcoma treated with chemotherapy and radiotherapy. The incidence of leukemias is high, while the other tumors can be explained as unrelated cases. Incidence densities for leukemia were calculated for both groups of patients. Treated osteosarcoma patients seem to have a predisposition to develop leukemias, but whether this is chemotherapy induced needs to be investigated.

Adolescent↗

Heterologous sarcomas of the uterus.

Three cases of heterologous sarcomas of the uterus are presented. Two were mixed tumors; one was a lipoleiomyosarcoma; and one was an osteogenic sarcoma with leiomyosarcoma. The third was a rhabdomyosarcoma. These tumors are believed to behave more aggressively than mixed mesodermal tumors. Six months after initial diagnosis, the lipolieomyosarcoma had metastasized to a vertebra. Following radiation therapy, the metastases recurred at the same site. The rhabdomyosarcoma of the uterus produced lung metastases 4 months following surgery, and the patient died 14 months after initial diagnosis despite extensive chemotherapy. The patient with osteogenic sarcoma of the uterus with leiomyosarcoma had been treated with Adriamycin and is alive and well 1 year following initial diagnosis. The histogenesis of these tumors is reviewed.

Aged↗

Limb-sparing therapy of extremity soft tissue sarcomas: treatment outcome and long-term functional results.

The purpose of this study is to assess the long-term success rate and functional results of limb-sparing therapy in a group of 156 patients with soft tissue sarcomas of the extremities in the Netherlands Cancer Institute, treated according to a standard protocol of surgery and radiotherapy, if indicated. The patients (79 females and 77 males) were treated between 1977 and 1983 by an intended wide local excision with a margin of at least 2 cm. Postoperative radiotherapy was applied in 117 patients; 26 patients had surgery only, including 13 patients who had to be treated by amputation. The total dose was 60 Gy, with 40 Gy to a large volume and a boost of 20 Gy to the tumour bed at 2 Gy per fraction, five fractions per week. Most sarcomas were located in the proximal part of the lower extremity (51%). The group comprised 50 liposarcomas, 47 malignant fibrous hystiocystoma (MFH) and 59 other histologies; 69 (44%) had high-grade tumours. Three treatment groups with limb-sparing treatment were defined: group I (n = 26) patients who had a complete excision receiving no further treatment, group II (n = 64) with narrow surgical margins and radiotherapy and group III (n = 53) with incomplete resection and radiotherapy. The 10-year actuarial overall survival and local control rate for all patients was 63 and 81%, respectively. Multivariate analysis showed that histological grade (P < 0.0001), age (P = 0.0005) and location deep to the fascia (P = 0.0008) were independent prognostic factors for survival, while local control was predicted by grade (P = 0.0014) and treatment group (p = 0.028). Patients with surgery only (group I) had 81% 5-year local control as compared to 92% with radiotherapy after narrow surgery (group II) and 74% with incomplete surgery and radiotherapy (group III). Limb preservation when attempted was achieved in 90% of the patients. After limb-sparing treatment, 7% had severe impairment of mobility, 3% had lymph oedema and 16% marked fibrosis. Fractures in the irradiated bone occurred in 6% of the patients. The combination of limited surgery followed by radiotherapy resulted in a high local control rate with good functional results. Ultimately limb sparing treatment was successful in 83% of all patients with extremity sarcomas.

Adolescent↗

Management of primary sarcomas of the retroperitoneum.

We here report on our 10-year experience of surgery in 25 patients with primary retroperitoneal sarcoma and make an appraisal of the more effective available clinical approach. Ten patients had leiomyosarcoma, eight liposarcoma, and seven had other tumor types. Histological grading was G1 in seven patients, G2 in six patients and G3 in 12. Ten patients (40%) underwent wide tumor excision, five (20%) marginal excision, four (16%) partial excision and six (24%) wedge biopsy at laparotomy. Adjacent organ resection was required in 10 of the 15 patients who underwent wide or marginal resection, seven of whom had adjuvant chemoradiotherapy. After wide resection, local recurrences were observed in 2/10 patients; the 10-year survival rate was 33%. Three out of five patients who underwent marginal resection had local recurrences; one is alive 10 years after surgery and chemo-radiotherapy. Histological grading was the most important prognostic factor. Radical resection is the only effective available treatment for retroperitoneal sarcomas, and patients with low-grade tumors benefit most from it with respect to survival and local recurrence rate. The efficacy of adjuvant treatment has yet to be clarified.

Adult↗

Dedifferentiated liposarcoma with chondroblastic osteosarcomatous dedifferentiation.

We describe a rare case of dedifferentiated liposarcoma with features resembling chondroblastic osteosarcoma in the dedifferentiated component. The tumor was removed from the left thigh in a 78-year-old male. It consisted of a well-differentiated liposarcoma and an anaplastic component that contained numerous osteoid and cartilaginous tissues surrounded by high-grade spindle cell sarcoma. To our knowledge, only two cases similar to the divergent chondroblastic osteosarcomatous dedifferentiation of this disease have been reported in the literature.

Aged↗

A case of extraskeletal osteogenic sarcoma in the neck.

A patient with an extraskeletal osteogenic sarcoma in the neck is described. At the site of this extremely rare tumor a liposarcoma had been removed 7 and 14 years previously. The patient had never received radiotherapy. The presence and type of calcification and ossification serve to distinguish between the various conditions of the differential diagnosis.

Aged↗

Classification of positive margins after resection of soft-tissue sarcoma of the limb predicts the risk of local recurrence.

We considered whether a positive margin occurring after resection of a soft-tissue sarcoma of a limb would affect the incidence of local recurrence. Patients with low-grade liposarcomas were expected to be a low-risk group as were those who had positive margins planned before surgery to preserve critical structures. Two groups, however, were expected to be at a higher risk, namely, patients who had undergone unplanned excision elsewhere with a positive margin on re-excision and those with unplanned positive margins occurring during primary resection. Of 566 patients in a prospective database, 87 with positive margins after limb-sparing surgery and adjuvant radiotherapy were grouped according to the clinical scenario by an observer blinded to the outcome. The rate of local recurrence differed significantly between the two low- (4.2% and 3.6%) and the two high-risk groups (31.6% and 37.5%). This classification therefore provides useful information about the incidence of local recurrence after positive-margin resection.

Female↗