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PubMed1989

Expression of the common acute lymphoblastic leukemia antigen (CD10) in mesenchymal tumors.

The expression of the CD10 antigen, formerly designated as common acute lymphoblastic leukemia antigen and recently identified as neutral endopeptidase, was examined immunohistochemically in 26 benign and in 55 malignant mesenchymal tumors. CD10 expression was found in 4 of 4 leiomyomas, 7 of 10 leiomyosarcomas, 1 of 6 rhabdomyosarcomas, 2 of 2 Triton tumors, 1 of 2 aggressive fibromatoses, 1 of 3 fibrosarcomas, 1 of 4 synovial sarcomas, 1 of 1 giant cell tumors of tendon sheath, 4 of 4 malignant fibrous histiocytomas, 3 of 3 Ewing's sarcomas, and 2 of 3 osteosarcomas. Furthermore, CD10 was expressed consistently in the myoepithelial compartment of 12 fibroadenomas and, in 7 of these cases, in a minor stromal cell population, probably of (myo-) fibroblastic origin. Tumors of adipose tissue (4 lipomas, 5 liposarcomas), tumors of autonomic ganglia (2 ganglioneuromas, 1 ganglioneuroblastoma, 2 neuroblastomas), tumors of peripheral nerves with purely schwannian differentiation (7 malignant schwannomas), and tumors of disputed origin were consistently CD10-negative, however, as were single cases of fibroma and chondrosarcoma. These findings indicate that the expression of CD10 is a frequent but not obligatory feature in some mesenchymal tumors. Therefore CD10 is of value in the differential diagnosis of mesenchymal tumors.

Antibodies, Monoclonal↗
PubMed2006

Preoperative radiotherapy for soft tissue sarcoma: the Peter MacCallum Cancer Centre experience.

AIM: Radiotherapy has been shown to improve local control in combination with limb-sparing or conservative surgery in the management of localised soft tissue sarcoma. Our centre's treatment protocol is to offer preoperative external beam radiotherapy (50.4Gy in 28 fractions) followed by surgery four to six weeks later. The aim of this study is to review the treatment outcome and toxicity of patients treated with this protocol. METHODS: Consecutive patients with localised extremity or truncal soft tissue sarcoma who presented between January 1996 and December 2000 and treated with preoperative radiotherapy followed by limb-sparing surgery were reviewed. Patients with recurrent disease or metastatic disease at diagnosis and patients below the age of 16years were excluded. Local and distant recurrence, overall survival and treatment toxicity were analyzed. RESULTS: Sixty-seven cases were identified (41 males and 26 females). The median age was 52years (range 17 to 82). The majority (79%) had tumours located in the lower limb. The most common histological diagnoses were malignant fibrous histiocytoma and liposarcoma. The median follow-up was 4.1years (range 0.6 to 6.9). There were six local recurrences, two of which were successfully salvaged. Twenty patients developed distant metastases. The estimated 5-year actuarial local recurrence free, distant recurrence free and overall survival were 93%, 68% and 73% respectively. Acute radiotherapy toxicity and wound complications were acceptable and late toxicity was uncommon. CONCLUSION: Preoperative radiotherapy followed by surgery provides effective local control in the management of soft tissue sarcoma.

Adolescent↗
PubMed2003

Adult spermatic cord sarcomas: management and results.

BACKGROUND: We present a 20-year surgical experience with spermatic cord sarcoma, describe prognostic features, and evaluate the results of surgical and adjunctive treatments. METHODS: Forty-seven patients were identified from an institutional database. Disease-free survival was calculated, and prognostic factors such as tumor grade, size, extent of operation, and adjuvant therapy were analyzed. RESULTS: The median patient age was 58 years (range, 16-83 years), and the median follow-up was 51 months (range,.5-226 months). The most common tumor types included liposarcoma (51%), leiomyosarcoma (19%), embryonal rhabdomyosarcoma (13%), and malignant fibrous histiocytoma (11%). Twenty-nine (62%) patients had high-grade tumors, 21 (45%) were treated with adjuvant radiation, and 9 (19%) received chemotherapy. The overall 5- and 10-year disease-specific survival was 75% and 55%, respectively. No specific prognostic factors were identified for recurrence or disease-free survival. In 21 patients who underwent reoperative wide resection after a prior incomplete resection, a trend toward improved disease-free survival was noted (P <.059). Of these, six (29%) had residual viable sarcoma. We could not demonstrate a therapeutic effect of adjuvant radiation or chemotherapy. CONCLUSIONS: We demonstrated that aggressive surgical strategies, including reoperative wide resection, significantly decrease local recurrence and may improve disease free-survival in select patients with spermatic cord sarcoma.

Adolescent↗
PubMed1989

Sarcomas of the retroperitoneum and genitourinary tract.

A total of 62 patients with retroperitoneal or genitourinary sarcoma was treated at our institutions during the last 46 years. Of the patients 51 were followed for at least 5 years or until they died (median followup 11 years); 5 patients were lost to followup. The most common site was the retroperitoneum. Liposarcoma, leiomyosarcoma and malignant fibrous histiocytoma were the most common tumors (74 per cent). Tumors were completely resected in 42 patients (68 per cent) and incompletely resected in 11, while a biopsy only was performed in 9. Some patients also received adjuvant radiation therapy and/or chemotherapy. There were no long-term survivors among patients with unresectable tumors. Over-all 3 and 5-year survival rates were 68 and 39 per cent, respectively. The histological type of the tumor appeared to have prognostic significance. The highest 5-year survivals were for liposarcoma (70 per cent), malignant fibrous histiocytoma (33 per cent) and leiomyosarcoma (13 per cent). The mean survival for patients after adjuvant radiation therapy or chemotherapy was similar to that after a radical operation alone. The primary cause of treatment failure was local recurrence (45 per cent of the patients), which was detected within 3 years of complete resection in most cases (82 per cent). Complete extirpation that provided adequate margins free of tumor was the most effective initial treatment and provided the best chance for cure.

Combined Modality Therapy↗
PubMed2002

Characterization and expression of the human gene encoding two translocation liposarcoma protein-associated serine-arginine (TASR) proteins.

Translocation liposarcoma protein (TLS)-associated serine-arginine (TASR)-1 and -2 are two newly identified serine-arginine splicing factors. Our recent studies suggest that disruption of TASR-mediated pre-mRNA splicing is involved in the pathogenesis of human leukemia and sarcomas. The mRNA transcripts for TASR-1 and -2 share an identical sequence at the 5' untranslated region (5' UTR) and in part of the coding region; however the other regions of the transcripts diverge from each other and it was not clear whether the differences resulted from alternative splicing or transcription from two distinct genes. Here we describe the assignment of both TASR cDNAs to the same 16 kb DNA segment located on chromosome 1. Despite the presence of at least three retroposed products of TASR-1 mRNA in the human genome, only the 16 kb structural TASR gene on chromosome 1 is actively transcribed. In addition, multiple polyadenylation sites and a rare U12-type intron were found within the TASR gene. Transcription initiation site of the TASR gene was determined by primer extension; analysis of the TASR promoter revealed that it lacks the TATA box but contains a GC-rich sequence. When cloned into a luciferase reporter and transfected into human cells, the TASR promoter construct generated luciferase activity that was at least 2000 fold greater than the promoterless plasmid. Northern blot analysis showed that at least five different TASR-1 and -2 transcripts are expressed in a broad range of human tissues.

Alternative Splicing↗
PubMed2004

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↗
PubMed2002

Spindle cell lipoma of the alveolar mucosa: a case report.

Spindle cell lipoma of the oral cavity is extremely rare. We report a case occurring on the alveolar mucosa, an oral site that has not been previously reported. A short review of the literature is also presented. The importance of distinguishing this benign lesion from a sarcoma is highlighted. The lesion was excised, and no recurrence has been reported after 2 years.

Aged↗
PubMed1998

Liposarcoma with meningothelial-like whorls: a study of 17 cases of a distinctive histological pattern associated with dedifferentiated liposarcoma.

AIMS: We reviewed 17 cases of liposarcoma with peculiar meningothelial-like whorls to determine the pathological and clinical significance of this distinctive morphological finding. METHODS AND RESULTS: Seventeen liposarcomas with concentric whorls simulating the whorls seen in meningioma, were retrieved from the soft tissue registry of the Armed Forces Institute of Pathology. There were 10 males and seven females with a mean age of 53 years (range 24-76 years). Twelve of the 17 cases were large retroperitoneal tumours (mean 170 mm in greatest dimensions). All 17 cases had whorls. Three cases were classified as well-differentiated liposarcoma with whorls and bone formation. Five cases showed coalescing of whorls into areas which may be interpreted as low to intermediate grade dedifferentiation. Five cases had spindled areas associated with the whorls which correlate with classical intermediate to high-grade dedifferentiation. Finally, four cases had a predominant spindle cell component resembling malignant fibrous histiocytoma with focal residual possible sclerosing well-differentiated liposarcoma (scattered adipocytes, adipocytic atypia and/or floret-type giant cells). The meningothelial-like whorls were clustered or scattered throughout the tumours and ranged from 0.09 mm to over 10 mm in diameter in cases with coalescent whorls. Metaplastic bone was present within the whorls or in their immediate vicinity in 10 of 17 cases. Immunohistochemically, the whorl-forming spindle cells showed alpha-smooth muscle actin reactivity in three of seven cases but were negative for epithelial membrane antigen, CD21, and CD35 indicating lack of relationship with meningioma and dendritic reticulum cell sarcoma, other tumours that may contain whorls. Whorls with bone formation revealed cells adjacent to the bone to be positive for osteocalcin, a marker of osteoblastic phenotype. The spindle cells of the whorls were negative for CD34 and CD31, yet these highlighted numerous capillaries inside the whorls in a concentric manner. The whorl-forming cells showed moderate to high MIB-1-index and showed p53 immunoreactivity similar to the dedifferentiated areas but differed from the areas of well-differentiated liposarcoma, which were p53-negative and showed a low MIB-1-index. Follow-up (available in 65% cases) revealed seven patients with metastases or dead of the disease and five patients with one or more recurrences. CONCLUSIONS: The meningothelial-like whorls represent a mesenchymal proliferation which may undergo pericytic or myofibroblastic, or occasionally osteoblastic, differentiation in liposarcoma. These whorls do not represent dendritic or perineurial/meningothelial differentiation. The significant proliferative activity, p53 reactivity and tendency to coalesce and associate with dedifferentiated liposarcoma suggest that the meningothelial whorls may represent an early sign of dedifferentiation of liposarcoma.

Adult↗
PubMed1993

Limb-preserving treatment for soft tissue sarcomas of the extremities. The significance of surgical margins.

BACKGROUND AND METHODS: Between 1970 and 1990, 74 patients with extremity soft tissue sarcomas began a regimen of limb-sparing surgery and irradiation. Ninety-two percent received postoperative irradiation, 7% received preoperative irradiation, and one patient was treated with radiation after an incisional biopsy only. Radiation was administered with a shrinking-field technique (median total dose, 63 Gy; range, 39.6-71 Gy), with 14 patients receiving interstitial brachytherapy as a component of treatment. Ten patients received adjuvant chemotherapy. RESULTS: Local control of disease was 82% at 5 years, and 95% after salvage therapy. The actuarial risk of distant disease recurrence was 27%, with an overall survival rate of 70% at 5 years. Histologic subtype, tumor grade, size of tumor, location of tumor, status of the surgical margins, use of chemotherapy, radiation dose, interval until initiation of radiation therapy, and use of a brachytherapy boost were analyzed to determine their effect on survival, local control, and freedom from distant metastasis. Microscopic involvement of the surgical margins predicted for local disease recurrence (P = 0.0002), distant disease recurrence (P = 0.008), and poorer overall survival (P = 0.02). Low histologic grade was favorably correlated with freedom from distant disease recurrence (P = 0.004) and survival (P = 0.002). Patients with liposarcomas had superior local control (P = 0.004) compared with patients with tumors of other histologic subtypes. Five patients experienced a complication of therapy, for an actuarial risk of 10% at 5 years. Only two patients required hospital admittance for treatment-related morbidity. CONCLUSIONS: These findings support the continued use of limb-preserving management for extremity sarcomas and emphasize the importance of the status of the surgical margins.

Adolescent↗
PubMed2003

[Recent progress of molecular diagnosis in pediatric malignancies].

Recent progress of molecular and cytogenetic techniques has led to remarkable advances in molecular diagnosis of pediatric malignancies, including malignant bone and soft tissue sarcoma (MSTS). Fusion genes, such as EWS-FLI1 and PAX3-FKHR, were cloned at the chromosome breakpoints of t(11;22) and t(2;13) in Ewing's sarcoma and rhabdomyosarcoma, respectively. Minimal residual disease can be detected by reverse transcriptase-polymerase chain reaction using these translocations. These fusion genes contribute to differential diagnosis of pediatric small round cell tumor, which was difficult to diagnose morphologically. Some of these fusion genes, including SYT-SSX in synovial sarcoma and EWS-FLI1 in Ewing sarcoma, have been reported to be associated with prognosis. Recently, genome-wide searches using microarray and single nucleotide polymorphisms have been performed in pediatric malignancies. These advances have led to the increased importance of molecular diagnosis as well as morphological diagnosis. We review here the recent progress of molecular diagnosis in pediatric malignancies.

Child↗
PubMed1988

Utility of 24 hour bone scan in evaluation of bone involvement by soft tissue sarcoma.

Accumulation of Tc-99m MDP in soft tissue malignancies occurs frequently. When tumor overlaps adjacent bone structures, the bone margins can be indiscernible on a regular bone scan. Additional views should be taken to differentiate bone from tumor, however, separation is sometimes difficult and not always successful. This report presents a patient in whom accumulation of Tc-99m MDP in a liposarcoma of the thigh disappeared on a 24 hour scan and bone margins were seen clearly. A possible mechanism for this phenomenon and its utility are discussed.

Adult↗
PubMed2003

Comparative fine-needle aspiration and pathologic study of malignant fibrous histiocytoma: cytodiagnostic features of 95 tumors in 71 patients.

To determine diagnostic cytomorphologic features of malignant fibrous histiocytoma (MFH) on fine-needle aspiration (FNA) materials, we reviewed the cytologic material and corresponding histologic slides of 95 tumors in 71 patients. Forty-four (46%) tumors were primary, 38 (40%) were recurrent, and 13 (14%) were metastatic. Histological variants of MFH were as follows: 52 (54.7%, 43 patients) were of the storiform/pleomorphic, seven (7.4%, five patients) were giant cells, four (4.2%, four patients) were inflammatory, and 31 (33.7%, 19 patients) were myxoid type. Review of original cytology reports showed that only 23 (24.2%) tumors were diagnosed as MFH and 68 (71.6%) as other types of malignancies. Four (4.2%) cases were reported as unsatisfactory/suspicious. Our findings showed that spindle-shaped, round, giant cells, osteoclastic-like giant, and inflammatory cells were the most consistent features that allow identification of the storiform/pleomorphic, giant cell, and inflammatory variants of MFH. The myxoid tumors had marked myxoid background matrix with spindle-shaped cells and, less frequently, round and giant cells. Pleomorphic leiomyosarcoma and dedifferentiated liposarcoma should be considered in the differential diagnosis of stroriphorm/pleomorphic, giant cells, and inflammatory variants of MFH. However, myxoid MFH may resemble their leiomyosarcoma and liposarcoma counterparts.

Adolescent↗
PubMed1987

"Dedifferentiated" chordoma. A clinicopathologic and immunohistochemical study of three cases.

Three cases of "dedifferentiated" chordoma arising in the sacrococcygeal region are presented. In all three cases, the "dedifferentiated" component arose de novo in conjunction with conventional chordoma. Two of these patients, whose tumors had a prominent malignant fibrous histiocytoma (MFH) component, died within 6 months of diagnosis. Both patients had lung metastases, one of which was histologically documented to be MFH. The third patient, whose initial tumor contained osteosarcoma, died 76 months after diagnosis and multiple recurrences. Most notable in this case was the absence of the "dedifferentiated" component (in this instance, osteosarcoma) in all of the local recurrences as well as the lung metastases. These were composed exclusively of conventional chordoma. None of the patients had a previous history of radiation therapy. The immunohistochemical staining pattern of conventional chordoma was similar to that of previous reports, where the epithelial-like cells stained for cytokeratin and epithelial membrane antigen. In addition, they stained for alpha-1-anti-chymotrypsin and vimentin. These latter two markers were also identified in the "dedifferentiated" component. As with "dedifferentiated" chondrosarcomas and liposarcomas, "dedifferentiation" in a chordoma usually portends an accelerated clinical course.

Adult↗
PubMed2004

Recurrent aggressive angiomyxoma of vagina--a case report.

Aggressive angiomyxoma is a rare tumour, which presents as a painless expanding mass in the vulvo-vaginal region. It usually occurs in 2nd to 3rd decades of life. It behaves like a low-grade sarcoma with high propensity for local spread and recurrence and can involve vulva, perineum, vagina and urinary bladder. It is difficult to differentiate clinically this tumour from other mesenchymal tumours occurring in this region. Microscopically it must be differentiated from malignant tumours with myxoid change, like liposarcoma and myxoid leiomyosarcoma. Hence, histopathologic examination has a central role in diagnosis of this tumour. We are presenting a case of young woman, who came with history of swelling in vulva and perineum. Imaging studies in the perineal region revealed a large pelvic mass. Fine needle aspiration cytology was inconclusive due to scanty material. Enucleation of mass was attempted in first surgery but complete extirpation could not be performed. The swelling recurred within few weeks after surgery and required irradiation. A second surgery, however, was successful in complete removal of the tumour.

Adult↗
PubMed2004

Primary malignant tumors of the iliopsoas compartment.

BACKGROUND AND OBJECTIVES: Primary malignant tumors of the iliopsoas compartment are rare entities that have been infrequently reported. We present our experience of iliopsoas malignancy to better characterize and define the natural history of this condition. METHODS: Primary iliopsoas tumors occurring from January 1990 were identified from the Royal Marsden Hospital's (RMH) Sarcoma Unit prospective database. RESULTS: Nineteen malignant tumors (11 limited to the psoas muscle only, 1 involving only the iliacus muscle and 7 involving both the iliacus and psoas) of the iliopsoas compartment were evaluated and treated at the RMH during this period. Leiomyosarcoma (n = 5) and liposarcoma (n = 3) were the most frequent histologic types. There were 3 G1, 5 G2, 10 G3 tumors and grade could not be assessed in 1 patient. There were 17 T2 tumors and size was not available in 2 patients. Surgery was done in 14 patients (negative microscopic margins-7, positive microscopic margins-3, and positive gross margins-3 and the margin of excision was not known in 1 patient). Five of 14 surgically treated patients had local recurrence and 6 of 19 patients developed metastases. Ten patients died of their disease at a median follow-up of 12 months. The estimated 2 and 5 year survival rates were 44 and 23%, respectively. CONCLUSIONS: Tumors in this location have a poor prognosis due to the lack of early diagnosis, large size at presentation, multiple attachments of the psoas muscle, and being relatively surgically inaccessible.

Chemotherapy, Adjuvant↗
PubMed1995

[Bilateral breast carcinoma after recurrent myxoid liposarcoma of the breast].

The case is presented of a 26-year-old woman with myxoid liposarcoma of the right breast who developed a relapse with anaplastic spindle-cell mesenchymal tumor 7 months after primary therapy. Additionally, 13 months later a bilateral adeno-carcinoma of differing histological grade occurred in the breasts. To the best of our knowledge, no comparable sarcoma-carcinoma sequence in the mammary gland has been reported. For this reason this case is discussed, with special emphasis on possible causative factors and the relationship to the Li-Fraumeni syndrome.

Adenocarcinoma↗
PubMed1992

[67Ga scintigram and MRI in malignant bone tumors and malignant soft tissue tumors].

We have analyzed the characteristics of 67Ga scintigram and MRI in 11 malignant bone tumors and 11 malignant soft tissue tumors. Osteosarcoma showed a high accumulation in 67Ga scintigram and low signal intensity in T1 weighted image. T2 weighted image were not characteristic. Chondrosarcoma showed medium 67Ga accumulation and low signal in T1 weighted image and high signal in T2 weighted image. Ewing sarcoma showed low accumulation in 67Ga scan and medium intensity in MRI. Malignant soft tissue tumors showed rather low 67Ga accumulation compared with malignant bone tumors. Malignant fibrous histiocytoma showed medium accumulation of 67Ga, low signal in T1 weighted image and high signal in T2 weighted image. Liposarcoma showed low 67Ga accumulation and medium signal in T1 weighted image and high signal in T2 weighted image. To summarize these characteristics, three dimensional display is demonstrated.

Bone Neoplasms↗