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At least 253 records · Page 14Linked to original sources
PubMed2002

Intrarenal solitary fibrous tumor of the kidney report of a case with emphasis on the differential diagnosis in the wide spectrum of monomorphous spindle cell tumors of the kidney.

Solitary fibrous tumor (SFT) is a neoplasm that can occur in the urogenital tract, and is also reported occurring in the spermatic cord, seminal vesicles, urinary bladder, prostate, and kidney. Furthermore, it is most important to consider its existence in the kidney, because it is usually diagnosed as renal cell carcinoma pre-operatively. To our knowledge, only 10 cases of SFT have been reported in the kidney to date. We report the clinico-pathological features of an intrarenal SFT occurring in a 31-year-old woman. The tumor, measuring 8.6 cm in its greatest diameter, completely replaced the cortex and the medulla of the middle region of the right kidney, compressing the pelvis. Radiological imaging was consistent with a renal cell carcinoma. Histologically, the tumor was composed of a proliferation of bland-looking vimentin+, CD34+, bcl2+ and CD99+ spindle cells exhibiting a haphazard to storiform growth pattern, pushing borders, and a low mitotic rate (2 mitoses x 10 HPF). We placed emphasis on the differential diagnostic problems, i.e., its differentiation from other primary monomorphous benign and malignant spindle cell tumors of the kidney, such as fibroma, benign fibrous histiocytoma, hemangiopericytoma, inflammatory myofibroblastic (pseudo-)tumor, leiomyoma, angiomyolipoma with predominant spindle cell smooth muscle component, benign peripheral nerve sheath tumors, renal mixed epithelial/stromal tumors, adult type mesoblastic nephroma, fibrous type monophasic synovial sarcoma, malignant peripheral nerve sheath tumors, fibrosarcoma, and low-grade fibromyxoid sarcoma.

Adult↗
PubMedDate not supplied

First impressions of I.V. C. parvum in patients with solid tumours.

6 patients with solid tumours (4 malignant melanomas, one fibrosarcoma, one osteogenic sarcoma) received i.v. C. parvum alone or in combination therapy (radiotherapy, Levamisole and/or vitamin A). The single doses of C. parvum ranged from 5.0-7.5 mg/m2; No. of doses ranged from 1-8; interval between doses ranged from 2-140 days. 2 patients with malignant melanoma had no measurable disease, one of them (stage I) still has no evidence of disease. The patient with fibrosarcoma appeared to have a minor decrease in size of some of the lung metastases for a short time (ca 4 weeks), but soon progressed as well as the other 4 patients. Except for the one patients still having no evidence of disease the other 5 are dead of disease. Survival time did not appear to correlate either with systemic reaction to C. parvum or with No. of doses of C. parvum. The one minor response was observed in a patient receiving a total of 8 doses; but response was seen already after 3 doses, and progression occurred after 6 doses. So this change of lung metastases might have been unrelated to this therapy. Summarizing, there was no evident anti-tumour effect observed after i.v. C. parvum in these patients with solid tumours.

Humans↗
PubMed1999

Sarcomas of the head and neck.

Sarcomas are rare malignancies of mesenchymal origin. Computed tomographic and magnetic resonance imaging characteristics, as well as histologic findings and epidemiology, of sarcomas of the head and neck are reviewed. The sarcomas discussed include rhabdomyosarcoma, fibrosarcoma, osteosarcoma, chondrosarcoma, malignant fibrous histiocytoma, leiomyosarcoma, alveolar soft part sarcoma, Ewing's sarcoma, and synovial sarcoma. The imaging findings of these entities frequently are nonspecific. Imaging, particularly magnetic resonance imaging, has a major role in defining the extent of these tumors. This is important because complete surgical excision is the preferred method of treatment. Imaging also is useful in planning radiation therapy and determining prognosis.

Head and Neck Neoplasms↗
PubMed1988

[Endometrial stromal sarcoma of vaginal and para-vaginal localization. Morphology, diagnosis and differential diagnosis].

Endometrial stromal sarcomas constitute a subgroup of malignant mesenchymal uterine neoplasms and comprise biologically somewhat different variants. Their occurrence in extrauterine tissues is possible but extremely rare and therefore they are quite often mistaken for other tumours. Reported in this paper is a stromal sarcoma in vaginal and paravaginal localization (64-years old woman). It was the purpose of our study to present the histological structure in comparison to uterine neoplasms and to discuss differential diagnosis. It could be demonstrated that histological features of the actual case corresponded precisely to the respective uterine tumours originating within the myometrium. In extrauterine position, haemangiopericytomas, leiomyosarcoma, malignant schwannomas, fibrosarcomas and synovial sarcomas as well as Müllerian adenosarcomas and carcinosarcomas should be taken into account. Important histological features facilitating distinction of endometrial stromal sarcomas from the aforementioned malignant tumours are discussed, and some comments are added with regard to histogenetical interpretation.

Diagnosis, Differential↗
PubMed1988

Regional lymph node metastases in patients with soft tissue sarcomas of the extremities, what are the therapeutic consequences?

In the period 1977-1983, 183 adult patients with soft tissue sarcomas of the extremities were treated in the Netherlands Cancer Institute. One hundred and seventy-one patients had initially operable tumors. Fifteen patients (8.2%) developed regional lymph node metastases (RLNM) during the course of their disease. Only two patients (1.2%) developed RLNM as first site of tumor recurrence. The incidence of RLNM varied according to the histological subtypes: liposarcoma: 1/64, fibrosarcoma: 1/12, tendosynovial sarcoma: 5/24, unclassifiable sarcoma: 3/8. The outcome in patients with RLNM was invariably fatal. In all cases with RNLM distant metastases were present either at the time RLNM were found or shortly afterwards (median 4 months). Based on this experience we now consider RLNM in soft tissue sarcoma an expression of systemic tumor spread, which should be treated as such. We find no indication for elective lymph node dissection as part of the initial treatment of soft tissue sarcoma of any histological subtype. Therapeutic lymph node dissection might be indicated as part of the palliative management.

Adult↗
PubMedDate not supplied

[Laryngeal fibrosarcoma. Report of 2 new cases].

Sarcomas constitute less than one percent of laryngeal neoplasms. Fibrosarcomas are by far the most commonly reported mesenchymal tumors of the larynx. We report two cases of fibrosarcoma of the larynx and discuss clinical aspects, diagnosis and treatment.

Aged↗
PubMed2004

[Soft tissue malignancies in childhood and adolescence. Pathology and clinical relevance based on data from the kiel pediatric tumor registry].

Soft tissue malignancies in childhood and adolescence encompass a wide variety of histologically and genetically different tumor entities. In the files of the Kiel Pediatric Tumor Registry, 4,272 soft tissue malignancies were collected since 1977. Rhabdomyosarcomas are by far the most frequent sarcomas (44.6 % of the cases), followed in decreasing order of frequency by the family of Ewing tumors (peripheral primitive neuroectodermal tumors and extraosseous Ewing's sarcomas; altogether 22.3 %), malignant peripheral nerve sheath tumors (8.1 %), synovial sarcomas (5.0 %), leiomyosarcomas (3.2 %), fibrosarcomas (2.4 %), extrarenal malignant rhabdoid tumors (2.0 %), and alveolar soft tissue sarcomas (1.1 %). A further group (11.3 %) includes rare tumors, intermediate fibrohistiocytic tumors, and unclassified sarcomas. Embryonal rhabdomyosarcomas are 2.5 times more frequent than the alveolar rhabdomyosarcomas, which are prognostically unfavorable and located predominantly in the extremities and the trunk. With regard to clinical findings, histology, molecular biology and prognosis, embryonal and alveolar rhabdomyosarcomas have to be considered as two different tumor types. The family of Ewing tumors includes extraosseous Ewing's sarcoma and peripheral primitive neuroectodermal tumors (synonym: malignant peripheral neuroectodermal tumors), the former tumors without and the latter with neural differentiation. Many cases of infantile malignant peripheral nerve sheath tumors and infantile fibrosarcomas are low-grade malignancies and prognostically more favorable than their "adult" counterparts.

Adolescent↗
PubMed1997

[A clinical analysis of 153 uterine sarcomas].

OBJECTIVE: To evaluate the prognostic factors and treatment methods of 153 uterine sarcomas. METHODS: 153 cases of the uterine sarcoma were eligible for this retrospective study. Of the 153 cases, 48 were leiomyosarcomas, 47 mixed mesodermal sarcomas, 37 endometrial stromal sarcomas, 8 carcinosarcomas, 4 sarcoma botryoides, 1 fibrosarcoma, and 8 malignant lymphomas. 81 cases were in stage I, 11 stage II, 33 stage III and 11 stage IV. 38 cases were treated by surgery alone, 24 by surgery combined with radiation therapy, 50 by surgery plus chemotherapy, 23 by surgery plus radiation therapy and chemotherapy, 4 by radiation therapy alone, 3 by chemotherapy alone, and 11 by radiation therapy plus chemotherapy. RESULTS: The overall 5-year survival rate was 49.0%, and that of leiomyosarcomas, mixed mesodermal sarcomas and endometrial stromal sarcomas was 46.9%, 34.1% and 69.3% respectively (P < 0.01). The 5-year survival rate of lesions limited to the uterus (stage I + II), and that of pelvic cavity invasion (stage III) and distant metastases was 59.6%, 25.6% and 10.0% respectively (P < 0.01. When the uterus was smaller than a 3 months pregnant uterus, the 5-year survival rate was 49.9%. When the uterus size larger than a 3 months pregnant uterus, the survival rate was 18.8% (P < 0.05). Premenopausals surviving 5-year accounted for 56.3% and post-menopausal 28.9% (P < 0.01). CONCLUSIONS: The prognosis of uterine sarcoma is significantly associated with histologic type, clinical and surgico-pathological stage, uterine size and pre- or post-menopausal status. Radiation or chemotherapy alone is palliative. Postsurgical adjuvant radiotherapy significantly decreased vaginal and pelvic recurrences rates. A combination of surgery, radiotherapy and chemotherapy can reduce pelvic recurrence as well as enhance survivals.

Adolescent↗
PubMed2005

A survey of clusterin and fascin expression in sarcomas and spindle cell neoplasms: strong clusterin immunostaining is highly specific for follicular dendritic cell tumor.

Clusterin, a glycoprotein implicated in many cellular functions including apoptosis, has recently been shown to be strongly expressed in follicular dendritic cell tumors, and to be absent or only weakly expressed in other dendritic cell tumors. Fascin has also been investigated as a potential marker of dendritic cell neoplasms. We evaluated staining for antibodies directed against these two antigens in 202 spindle cell tumors, including cases of follicular dendritic cell tumor (n=14), interdigitating dendritic cell tumor (n=7), leiomyosarcoma (n=17), inflammatory myofibroblastic tumor (n=13), inflammatory pseudotumor (n=2), spindle cell thymoma (n=17), synovial sarcoma (n=11), fibrosarcoma (n=14), liposarcoma (n=27), gastrointestinal stromal tumor (n=13), malignant fibrous histiocytoma (n=18), angiomatoid fibrous histiocytoma (n=4), angiosarcoma (n=10), malignant peripheral nerve sheath tumor (n=8), malignant melanoma (n=16), and spindle cell carcinoma (n=11). Among these spindle cell neoplasms, strong diffuse clusterin staining had an overall specificity of 93% and a sensitivity of 100% for follicular dendritic cell tumor. Clusterin staining was least reliable in distinguishing follicular dendritic cell tumor from spindle cell thymoma or malignant fibrous histiocytoma, but these are entities that usually can be distinguished by clinical and morphologic data. Rare cases of leiomyosarcoma, fibrosarcoma and angiosarcoma may show strong clusterin staining. Fascin staining was very nonspecific among spindle cell tumors and thus does not imply a dendritic cell lineage.

Carrier Proteins↗
PubMed1995

Post-irradiation soft tissue sarcoma.

From 1975 to 1993, 11 of 375 patients treated for soft tissue sarcoma presented with post-irradiation sarcoma. The mean time interval between irradiation therapy and onset of the second neoplasm was 15.8 years (4-31 years). The total radiation dosage ranged from 12 to 60 Gy with a mean of 40 Gy. All patients had complete staging including CT or MRI of the tumour site, and CT of the lung. Surgical resection was the treatment of choice. Wide margins could be achieved in 10 patients. One had a marginal resection. Tumours included malignant fibrous histiocytoma, haemangiosarcoma, rhabdomyosarcoma, malignant schwannoma, fibrosarcoma and undifferentiated sarcoma. All patients were reassessed in our outpatient clinic. After a mean follow-up of 4.7 years (1.0-11.5 years), only 1 patient had died because of the tumour. Although post-irradiation sarcomas are rather infrequently observed, these tumours must be suspected when alterations or symptoms occur in a previously irradiated region. Early detection provides the chance of curative, wide margin resection.

Adolescent↗
PubMed1994

Primary sarcoma of the lung: a clinical study with long-term follow-up.

Primary pulmonary sarcoma is an extremely rare tumor. In more than 30 years, only 22 patients with PPS were seen in our hospital; 18 patients (82%) underwent operation. Radical resection is the only curative treatment in patients with primary pulmonary sarcoma. All 4 patients (18%) who did not undergo operation died within 17 months. All 7 patients (32%) in whom no radical resection could be performed died between 10 months and 16 years after operation. Total resection of the tumor could be performed in 11 patients (50%). Of these, 7 are still alive (64%), and 1 patient died of an unrelated cause after 25 years (mean follow-up, 13.5 years). Histologic diagnosis in these patients was leiomyosarcoma in 4, malignant schwannoma in 2, and fibrosarcoma and undifferentiated sarcoma in 1 each. Median survival for all patients was 24 months. Actuarial 5-year survival was 44% for all patients. Small tumor diameter and low-grade malignancy are statistically significant favorable prognostic factors. No patient with grade 1 tumor died; the median survival was 60 months for grade 2 sarcomas, and 17 months for grade 3 sarcomas. No patient with a completely resected small primary pulmonary sarcoma had recurrence or metastasis.

Adolescent↗
PubMed2005

Ifosfamide, carboplatin, and etoposide (ICE) reinduction chemotherapy in a large cohort of children and adolescents with recurrent/refractory sarcoma: the Children's Cancer Group (CCG) experience.

BACKGROUND: The prognosis for children with recurrent/refractory sarcomas is poor. We determined the overall response rate (ORR) and overall survival (OS) of children with recurrent/refractory sarcomas who were given ifosfamide, carboplatin, and etoposide (ICE) in three Children's Cancer Group (CCG) phase I/II trials. PROCEDURE: Children with recurrent/refractory sarcoma were treated with ifosfamide (1,800 mg/m2/day on day 0-4), carboplatin (400 mg/m2/day on day 0-1), etoposide (100 mg/m2/day on day 0-4) and either rhG-CSF (10 microg/kg/day vs. 5 microg/kg/day, CCG-0894, 71 patients), PIXY321 (500-1,000 microg/m2/day, CCG-0924, 14 patients), or rhG-CSF (5 microg/kg/day) and IL-6 (2.5-5 microg/kg/day, CCG-0931, 12 patients). RESULTS: Ninety-seven patients were evaluable for tumor response, 56 male and 41 female, median age 14.1 years (range 2.8-22.5 years). Tumor types were osteosarcoma (OTS) (n = 34), rhabdomyosarcoma (n = 27), Ewing sarcoma (EWS) (n = 21), soft tissue sarcoma-not otherwise specified (n = 5), undifferentiated sarcoma (n = 6), fibrosarcoma (n = 2), peripheral primitive neuroectodermal tumor (n = 1), and extraosseous Ewing (n = 1). The ORR was 51% (27% complete response [CR]). OS at 1 and 2 years was 49% and 28%, respectively. Patients with CR or partial response (PR) had significantly increased 1- and 2-year OS, 71% and 41%, respectively, (P < 0.001). Rhabdomyosarcoma patients with embryonal histology had significant improvement in 1- and 2-year OS: 82% and 46%, respectively, compared with other histologies, (P < 0.005). CONCLUSIONS: The ORR to ICE reinduction chemotherapy in children with recurrent/refractory sarcoma was 51%. OS of 1 and 2 years appeared significantly improved in patients who had CR or PR following ICE reinduction therapy or who had rhabdomyosarcoma with embryonal histology.

Adolescent↗
PubMed1988

Soft-tissue sarcoma: initial characteristics and prognostic factors in patients with and without metastatic disease.

Five hundred and sixty-five patients with soft-tissue sarcoma were admitted to Memorial Sloan-Kettering Cancer Center from July 1, 1982 to July 1, 1985. Clinical and pathologic factors were analyzed to identify initial characteristics and to determine factors associated with prognosis and the development of metastasis. In decreasing order of frequency, the most common sites of primary tumor were the extremity (51.1%), retroperitoneum/viscera (28.8%), trunk (15.9%), head and neck (3.7%) and breast (0.4%). The most common histologic types were liposarcoma (21.4%), malignant fibrous histiocytoma (20.2%), leiomyosarcoma (20.0%), fibrosarcoma (11.0%), and tendosynovial sarcoma (9.6%). One hundred and twenty-eight patients (22.7%) had metastases in a median follow-up period of 19 months. Metastasis was more common in patients with retroperitoneal and visceral sarcomas than in patients with extremity sarcomas. Patients with leiomyosarcomas were more likely to develop metastases than those with other histologic types. Fibrosarcoma and liposarcomas were the least likely to develop metastases in this time frame. Tumors greater than 5 cm in diameter occurred more frequently in patients with metastases. Actuarial survival was related to the site of primary tumor and the histologic type and grade of malignancy. Multivariate analysis indicated that patients with low-grade sarcomas, extremity sarcomas, and fibrosarcomas and patients without metastatic disease demonstrated significantly improved survival. In patients with metastases, disease-free interval (less than 365 or greater than or equal to 365 days) was significantly related to overall survival.(ABSTRACT TRUNCATED AT 250 WORDS)

Actuarial Analysis↗
PubMed2002

Cytohistologic correlations of 24 malignant peripheral nerve sheath tumor (MPNST) in 17 patients: the Institut Curie experience.

Cytomorphological patterns of malignant peripheral nerve sheath tumor (MPNST) are insufficiently documented in the literature. Cytological and histological specimens in 24 tumors in 17 patients were correlated. The review of the original cytology reports showed that four (16.6%) tumors were correctly diagnosed, eight (33.3%) were diagnosed as sarcoma not otherwise specified, four (16.7%) as fibrosarcoma, three (12.5%) as synovial sarcoma, three (12.5%) as leiomyosarcoma, and one (4.2%) case each as malignant fibrous histiocytoma and rhabdomyosarcoma. At the review tumors were histologically reclassified as well-differentiated MPNST in 11 (45.9%) cases, anaplastic MPNST in 11 (45.9%) cases, and epithelioid MPNST and malignant Triton tumor in one (4.2%) case each. Cytologically, well-differentiated MPNST were composed of polymorphous oval to round cells, small spindle-shaped cells with wavy and comma-like naked nuclei, and a fibrillary, delicate stroma. Anaplastic MPNST, moreover, were composed of anaplastic giant and polymorphous cells. The malignant Triton tumor was composed of oval to round rhabdomyoblastic cells with eccentric nuclei and the epithelioid MPNST of polymorphous and round, epithelial-like cells. The cytological diagnosis of MPNST may be difficult, especially in anaplastic tumors. The correlation between the cytological features and the clinical information--origin of the tumor from a nerve trunk, a preexisting neurofibroma, patients with known history of neurofibromatosis 1--could be indicative of an MPNST diagnosis.

Adult↗
PubMed1996

Primary inflammatory fibrosarcoma of the esophagus.

Primary sarcomas of the esophagus are rare. We report the radiologic, surgical, and pathologic findings of a primary inflammatory fibrosarcoma of the esophagus in a 33-year-old woman, and review the prognostic features and management options of this tumor.

Adult↗
PubMed1985

Application of markers in the diagnosis of soft tissue tumours.

In this review we describe the application of markers which are useful for the diagnosis of soft tissue tumours in paraffin sections. Detection of intermediate filament proteins appears to be most useful for first screening of these neoplasms because all, except neuroblastomas, express vimentin; cytokeratin is expressed in synovial sarcomas, epithelioid sarcomas and mesotheliomas; desmin in myogenic tumours and glial fibrillary acidic protein in astrocytomas and gliomas. Tissue-specific markers are: factor VIII--related antigen-endothelial cells; myoglobulin and skeletal muscle myosin--skeletal muscle cells; neuron specific enolase--neurons and cells of the APUD systems; and leukocyte-associated antigen--leukocytes. Markers which are present in a variety of cell types and therefore do not serve as tissue-specific markers are; S-100 proteins, alpha-1-antichymotrypsin, creatine kinase M and actin. The S-100 antigens have been detected in melanomas, granular cell tumours, chondrosarcomas and in some schwannomas and liposarcomas. Alpha-1-antichymotrypsin has been found in fibrohistiocytic and 'true' histiocytic tumours and creatine kinase M and actin in myogenic tumours. No specific markers have, as yet, been described for fibrosarcomas, Ewing's sarcomas and hemangiopericytomas.

Carcinoma↗