No abstract available.
Leiomyosarcoma* ; Myosarcoma* ; Rhabdomyosarcoma*

BACKGROUND AND OBJECTIVES: Sinonasal sarcomas are relatively rare tumors, accounting for only about 1 percent of all sinonasal malignancies. The purpose of this study is to analyze the clinical features, treatment modalities and outcomes, survival and prognostic factors of sinonasal sarcomas. SUBJECTS AND METHOD: We retrospectively reviewed the medical records of 20 patients who were diagnosed and treated for sinonasal sarcomas between July 1993 and June 2006. Potential prognostic factors including age, tumor size, histology and adjuvant treatment were evaluated. RESULTS: According to histologic subtype, twenty cases consisted of 7 rhabdomyosarcomas, each 2 cases of spindle cell sarcoma, Ewing's sarcoma, angiosarcoma, and each 1 case of fibrosarcoma, leiomyosarcoma, hemangiopericytoma, peripheral neuroectodermal tumor, myxoid sarcoma, osteosarcoma, chondrosarcoma. The mean follow-up period was 61 months. Fourteen patients (65%) had undergone surgical approach with adjuvant chemotherapy or radiotherapy. Four patients (20%) had undergone surgical treatment only. Two patients (15%) had undergone chemotherapy or radiotherapy. At 5 years, the overall survival rates of sinonasal sarcomas were 65%. The survival rates according to the treatment modality were 100% (surgery only), 66% (surgery with adjuvant chemotherapy or radiotherapy), and 33% (chemotherapy or radiotherapy), respectively. The overall recurrence rates of the sinonasal sarcomas were 25%. The univariate analysis showed that the treatment modality and surgical margin are important prognostic factors for survival. CONCLUSION: The 5-year survival rate of sinonasal sarcomas was 65%. Early diagnosis and wide excision with safe resection margin are important for the treatment of sinonasal sarcomas.
Accounting ; Chemotherapy, Adjuvant ; Chondrosarcoma ; Early Diagnosis ; Fibrosarcoma ; Follow-Up Studies ; Hemangiopericytoma ; Hemangiosarcoma ; Humans ; Leiomyosarcoma ; Medical Records ; Neuroectodermal Tumors, Primitive, Peripheral ; Osteosarcoma ; Recurrence ; Retrospective Studies ; Rhabdomyosarcoma ; Sarcoma ; Sarcoma, Ewing ; Survival Rate

Carcinosarcoma of the stomach is a rarely occurring malignant biphasic tumor that consists of both carcinomatous and sarcomatous components simultaneously in a single tumor. The common carcinoma component is tubular or papillary adenocarcinoma and the mesenchymal sarcomatous components are variable and these include leiomyosarcoma, rhabdomyosarcoma, osteosarcoma and chondrosarcoma. However, neuroendocrine carcinomatous differentiation in the carcinomatous component is extremely rare. We present here a rare gastric carcinosarcoma that demonstrated neuroendocrine carcinomatous and leiomyosarcomatous differentiation in a 47-year-old man.
Adenocarcinoma, Papillary ; Carcinosarcoma ; Chondrosarcoma ; Humans ; Leiomyosarcoma ; Middle Aged ; Muscle, Smooth ; Neurosecretory Systems ; Osteosarcoma ; Rhabdomyosarcoma ; Stomach

The present study is concerned with angiographic findings of bone tumors in patients who have been admitted to Severance Hospital from April 1968 to March 1971. Angiography of peripheral tumors is a valuabIe procedure in that it provides important information concerning the presence or absence of a tumor, defines its size and extent, and gives indication of malignancy. Angiography as an additional aid in the diagnosis of malignant bone lesions was first presented by dos Santos, Lamas and Caldos (1931). Begg (1955) stated that a normal angiogram of a limb shows that the branches of the main artery follow a slightly curved and undulating course, and that their calibre slowly and progressively decreases. None of these vessels are seen to reach the periosteum, nor is the nutrient artery ever visible. dos Santos (1931) proposed certain angiographic criteria of malignancy, notably the presence of numerous irregular vessels at the transit of the contrast medium from the arteries to the veins. Margulis(1964)stated a large number of malignant neoplasms are highly vascular and present chaotic patterns. Their vessels have irregular lumina and arteriovenous communications are present within them. Areas of intense contrast staining occupy parts of or the entire tumor. This appearance, frequently described, has been assumed to be characteristic of and uniformly encountered in malignant tumors. Angiographies in the present study include 6 cases of Osteogenic sarcoma, 4 cases of Chondrosarcoma, 2 cases of Giant cell tumor, 1 case of Liposarcoma, 1 case of Kaposis sarcoma, 1 case of Hemangioma, 1 case of Metastatic bone tumor, 1 case of Fibrous dysplasia and 1 case of Neurofibromatosis. Angiography was performed 13 times under local and 5 times under the general anesthesis. 20 to 30cc of 50 per cent Hypaque was utilized for examination. Angiographic findings are as follows: 1. HypervascuIarity is seen in osteogenic sarcoma, giant cell tumor, hemangioma and metastatic bone tumor. But neurofibromatosis and fibrous dysplasia are avascular. 2. Irregularity of the vascular net work seems characteristic in osteogenic sarcoma, hemangioma, and metastatic bone tumor. 3. Blood pools are seen prominently in osteogenic sarcoma, giant cell tumor, hemangioma and metastatic bone tumor. but no blood pools in neurofibromatosis and fibrous dysplasia. 4. Arteriovenous shunt is seen in osteogenic sarcoma, chondrosarcoma, hemangioma and metastatic bone tumor. But there is no A-V shunt in giant cell tumor, liposarcoma, Kaposis sarcoma, neurofibromatosis and fibrous dysplasia. 5. Retention of contrast medium is seen prominently in osteogenic sarcoma and hemangioma. But no retention of contrast medium is noted in liposarcoma, neurofibromatosis and fibrous dysplasia. While I do not regard arteriography as a necessary procedure for the differantial diagnosis of all presumptive bone neoplasms, it is indeed valuable in selected cases, in differential diagnosis of bone tumors and in the demonstration of the vascularity and extent of extraosseous soft tissue masses and it can be helpful in selecting an appropriate biopsy site. By indicating the size ar,d vascularity of a lesion and its major blood supply, it may influence the surgical approach.
Angiography ; Arteries ; Biopsy ; Bone Neoplasms ; Chondrosarcoma ; Diagnosis ; Diagnosis, Differential ; Diatrizoate ; Extremities ; Giant Cell Tumors ; Hemangioma ; Humans ; Liposarcoma ; Neurofibromatoses ; Osteosarcoma ; Periosteum ; Sarcoma, Kaposi ; Veins

PURPOSE: Primary sarcoma and SMC (sarcomatous metaplastic carcinoma) of the breast are very rare tumors, accounting for less than 1% of all breast malignancies. There are many controversies concerning the biological characteristics, prognosis and optimal treatment of these tumors owing to the rarity of incidence. The aims of this study were to elucidate the clinicopathologic characteristics of these tumors and to assist in elucidating the optimal treatment plan for the disease. MATERIALS AND METHODS: 13 cases of primary sarcoma and 10 cases of SMC that had been treated at KCCH between 1984 and 2001 were retrospectively reviewed. Phyllodes tumors were excluded from our study. RESULTS: Among the 13 cases of primary sarcoma included, stromal sarcoma occurred in 5 cases, osteosarcoma in 3 cases, angiosarcoma in 3 cases and spindle cell sarcoma in 2 cases. The mean age of the patients with primary sarcoma and SMC was 39.7 years and 55.1 years respectively (p=0.002). When survival rates were compared according to histologic types, size of tumor, histologic grade, type of surgery and use of adjuvant therapy, both size of tumor (p=0.0256) and histologic grade (p=0.0197) were shown to be prognostic factors. CONCLUSION: There were no significantly different features between primary sarcoma and SMC in terms of biologic characteristics or survival rates, with the exception that patients with SMC were older than those with primary sarcoma. Histologic grade and size of tumor were significant prognostic factors of these tumors.
Breast Neoplasms ; Breast* ; Hemangiosarcoma ; Humans ; Incidence ; Osteosarcoma ; Phyllodes Tumor ; Population Characteristics ; Prognosis ; Retrospective Studies ; Sarcoma* ; Survival Rate

BACKGROUNDS: Head and neck sarcomas constitute a heterogenous group of rare malignant tumors occurring at rare sites. The purpose of this retrospective study is to evaluate the pathologic features, treatment modalities, outcome, patterns of failure, survival, and other prognostic factors. MATERIALS AND METHOD: The medical records of 33 patients whose tumors were diagnosed as head and neck sarcomas and treated at the department of otolaryngology in Asan Medical Center between 1990 to 2001 were reviewed. Potential prognostic factors including age, tumor size, histology, and adjuvant treatment were evlauated. RESULTS: All 33 of histologic classification consist of : rhabdomyosarcoma (10), angiosarcoma (4), spindle cell sarcoma (4), osteosarcoma (3), follicular dendritic cell sarcoma (2), synovial sarcoma (2), chondrosarcoma (2), malignant fibrous histiocytoma (2), hemangiopericytoma (1), leiomyosarcoma (1), unclassified (2). The ratio of male to female was 1.2 : 1 (19 : 14). The median age was 42 years (range 4-78 years). The mean follow-up period was 24.7 months. At 3 years, the disease-specific survival rates for patients with rhabdomyosarcoma were 100% and patients with other types of sarcoma had 78.0% mean survival. Twenty-three patients (70%) had undergone surgical approach with adjuvant chemotherapy or radiotherapy. Five patients had undergone surgical treatment only. The univariate analysis showed that tumor size is one of the most important prognostic factors for the survival. CONCLUSION: The rhabdomyosarcoma showed a better survival rate than other sarcomas of head and neck. Tumor size more or less than 5 cm was important prognostic factor for survival. We could not find the proven treatment guidelines, but surgery may be essential to the treatment for head and neck sarcomas.
Chemotherapy, Adjuvant ; Chondrosarcoma ; Chungcheongnam-do ; Classification ; Dendritic Cell Sarcoma, Follicular ; Female ; Follow-Up Studies ; Head* ; Hemangiopericytoma ; Hemangiosarcoma ; Histiocytoma, Malignant Fibrous ; Humans ; Leiomyosarcoma ; Male ; Medical Records ; Neck* ; Osteosarcoma ; Otolaryngology ; Radiotherapy ; Retrospective Studies ; Rhabdomyosarcoma ; Sarcoma* ; Sarcoma, Synovial ; Survival Rate

To study the clinical characteristics and treatment results of childhood soft tissue sarcoma, the retrospective study was performed on 67 patients with soft tissue sarcoma, experienced at the Department of Pediatrics, Seoul National University Hospital from January, 1982 to July, 1990. The median age of 67 soft tissue sarcoma patients was 4 years 5 months and age distribution showed that 0-4 year age group was most common (55.2%). The sex ratio of male to female was 1.2:1. There were 3 cancers among relatives of soft tissue sarcoma patients, including one cancer among first-degree relatives. As for pathological classification, rhabdomyosarcoma (67.1%) was the most common childhood soft tissue sarcoma, followed by malignant Schwannoma (8.9%), extraskeletal Ewing's sarcoma (6.0%), infantile fibrosarcoma (4.5%), malignant fibrous histiocytoma (3.0%), malignant hemangiopericytoma (3.0%), and there were 1 case each of angiosarcoma, leiomyosarcoma, synovial sarcoma, malignant mesenchymoma and mesenchymal chondrosarcoma. The median age of 45 rhabdomyosarcoma patients was 3 years 8 months and age distribution showed that 0-4 year age group was most common (64.5%). Twenty three patients were male and 22 were female. The histologic subtype of rhabdomyosarcoma was embryonal type in 38 patients (84.5%), alveolar type in 5 patients (11.1%) and unclassified type in 2 patients (4.4%). As for primary site of soft tissue sarcomas, the most frequent site was the head and neck region (32.8%) including parameningeal region (13.4%) and orbit (6.0%), followed by extremities (20.9%), trunk (19.4%), retroperitoneum and pelvis (11.9%), urogenital region (7.5%), perineum and perianal region (4.5%) and other region (3.0%). As for primary site of 45 rhabdomyosarcoma cases, the most frequent site was also the head and neck region (37.8%). The most common initial symptom of soft tissue sarcoma patients was mass (68.7%). As for Intergroup Rhabdomyosarcoma Study clinical grouping system of 67 soft tissue sarcoma patients, clinical group III (58.2%) was most common, followed by clinical group II(20.9%), IV (14.9%) and I (6.0%). Of 10 cases of clinical group IV with distant metastasis, lung (8 cases) was the most common metastaic region and other metastatic regions were bone, kidney, liver and bone marrow. As for IRS clinical grouping system of 45 rhabdomyosarcoma patients, clinical group III was most common (68.9%). Of 6 cases of clinical group IV, lung (5 cases) was also the most common metastatic region, followed by kidney and liver. From 1982 to 1985, chemotherapy was done with pulse VAC or pulse VAdrC-VAC regimen based on IRS-I and IRS-II. From 1986, patients in clinical group I and II received vincristine and actinomycin-D for 1 year and patients in clinical group III, IV and II with alveolar histologic subtype(unfavorable histologic group) received vincristine, actinomycin-D, adriamycin, cyclophosphamide and cisplatinum based on IRS-III. Radiation therapy was administered to patients in clinical group II, III and IV. Of 67 cases of soft tissue sarcoma, 54 case were eligible for treatment analysis. The 3 year disease free survival (DFS) of all 54 cases was 54.1%, 3 year DFS of clinical group I and II was 83.9%,3 year DFS of clinical group III and IV before 1986 was 35.7% and after 1986 was 48.2%. Of 45 cases of rhabdomyosarcoma, 41 cases were eligible for treatment analysis. The 3 year DFS of all 41 cases was 49.1%,3 year DFS of clinical group I and II was 87.5%,3 year DFS of clinical group III and IV before 1986 was 27.2% and after 1986 was 45.0%. Patients in clinical group I and II who had no gross residual tumor after primary surgical excision had best prognosis with 3 year DFS approximating 90% with only 2 drugs regimen, significantly better than patients in clinical group III and IV with 3 year DFS below 50% even after intensifying chemotherapy since year 1986. This analysis suggests that total surgical removal is very important for improving prognosis and should be undertaken where possible in all patients without distant metastasis. Treatment results also showed that after year 1986 with intensification of chemotherapy, 3 year DFS of clinical group III and IV as well as early toxic deaths increased, and after lowering doses of chemotherapeutic agents of regimen 35 of IRS-III, treatment results improved much. Therfore to improve prognosis of patients with gross residual tumor after surgical excision of biopsy and patients with distant metastasis at diagnosis, intensified multiagent chemcherapeutic regimen with adequate dose modification should be done to lower early toxic deaths.
Age Distribution ; Biopsy ; Bone Marrow ; Chondrosarcoma, Mesenchymal ; Classification ; Cyclophosphamide ; Diagnosis ; Disease-Free Survival ; Doxorubicin ; Drug Therapy ; Extremities ; Female ; Fibrosarcoma ; Head ; Hemangiopericytoma ; Hemangiosarcoma ; Histiocytoma, Malignant Fibrous ; Humans ; Kidney ; Leiomyosarcoma ; Liver ; Lung ; Male ; Mesenchymoma ; Neck ; Neoplasm Metastasis ; Neoplasm, Residual ; Neurilemmoma ; Orbit ; Pediatrics ; Pelvis ; Perineum ; Prognosis ; Retrospective Studies ; Rhabdomyosarcoma ; Sarcoma* ; Sarcoma, Ewing ; Sarcoma, Synovial ; Seoul ; Sex Ratio ; Survival Rate ; Vincristine

Biomarkers, Tumor ; metabolism ; Hemangioendothelioma, Epithelioid ; metabolism ; pathology ; Hemangiosarcoma ; metabolism ; pathology ; Humans ; Immunohistochemistry ; Leiomyosarcoma ; metabolism ; pathology ; Lung Neoplasms ; metabolism ; pathology ; Nerve Sheath Neoplasms ; metabolism ; pathology ; Pulmonary Blastoma ; metabolism ; pathology ; Sarcoma ; metabolism ; pathology ; Sarcoma, Synovial ; metabolism ; pathology ; Solitary Fibrous Tumors ; metabolism ; pathology

Cutaneous carcinosarcoma is a biphasic, rare tumor composed of malignant epithelial and mesenchymal components. The most common carcinoma component is a squamous cell carcinoma and the most common sarcoma component is an osteosarcoma. A 74-year-old female patient presented to our hospital with a 3.5x3.5 cm-sized exophytic, eroded tumor surrounded by an erythematous plaque on her right flank that had been present for 10 years. On the incisional biopsy specimen, the tumor was composed of atypical malignant squamous cells and epithelioid cells. Furthermore, immunohistochemistry revealed that the tumor cells were positive to cytokeratin AE1/AE3, cytokeratin 5/6, epithelial membrane antigen, p63, and vimentin but were negative to HMB45, S-100, CD31, CD34, factor VIII, smooth muscle actin, and desmin. Finally, based on these histopathological and immunohistochemical findings, we diagnosed this tumor as cutaneous carcinosarcoma consisting of squamous cell carcinoma and epithelioid sarcoma.
Actins ; Aged ; Biopsy ; Carcinoma, Squamous Cell* ; Carcinosarcoma* ; Cytochrome P-450 CYP1A1 ; Desmin ; Epithelioid Cells ; Factor VIII ; Female ; Humans ; Immunohistochemistry ; Keratins ; Mucin-1 ; Muscle, Smooth ; Osteosarcoma ; Sarcoma* ; Vimentin

Malignant mesenchymoma is an interesting but very rare tumor in which malignant differentiation has occurred twice or more. We report a case of retroperitoneal malignant mesenchymoma consisting of osteosarcoma, leiomyosarcoma, liposarcoma and fibrosarcoma. Abdominal CT showed a large retroperitoneal mass with two separate and distinct parts, namely an area of prominent calcification and one of clearly enhancing solid components. The mass contained histologically distinct tumorous components with no histologic admixure at the interfaces. The densely calcified nodule corresponded to osteosarcoma, and the noncalcified clearly enhancing nodules to leiomyosarcoma, liposarcoma and fibrosarcoma.
Case Report ; Fibrosarcoma/*radiography ; Human ; Leiomyosarcoma/*radiography ; Liposarcoma/*radiography ; Male ; Mesenchymoma/*radiography ; Middle Age ; Osteosarcoma/*radiography ; Retroperitoneal Neoplasms/*radiography ; Tomography, X-Ray Computed