National Cancer Institute®
Last Modified: March 1, 2002
UI - 11642491
AU - Shields JA; Shields CL; Brotman HK; Carvalho C; Perez N; Eagle RC Jr
TI - Cancer metastatic to the orbit: the 2000 Robert M. Curts Lecture.
SO - Ophthal Plast Reconstr Surg 2001 Sep;17(5):346-54
AD - Oncology Service, Wills Eye Hospital, Thomas Jefferson University, Philadelphia, Pennsylvania 19107, USA.
PURPOSE: To report the demographics and clinical features of a large series of patients with orbital metastasis. METHODS: Retrospective chart review on 100 consecutive patients and a literature review on orbital metastasis. RESULTS: Of 100 patients, the primary tumor site was breast in 53 (53%), prostate gland in 12 (12%), lung in 8 (8%), skin (melanoma) in 6 (6%), kidney in 5 (5%), gastrointestinal tract in 5 (5%), choroid (melanoma) in 2 (2%), parotid gland in 1 (1%), and adrenal gland (neuroblastoma) in 1 (1%). Of patients in whom a detailed history was available, there was no history of cancer at the time of presentation in 19%. In 10%, the primary tumor remained undetected despite systemic evaluation. There were 36 male patients and 64 female patients whose mean age at diagnosis was 62 years (median 60 years, range 5 to 91 years). Both the right and left orbits were affected equally, and 4 cases (4%) were bilateral. The most frequent clinical findings were limited ocular motility (54%), proptosis (50%), and palpable mass (43%). The diagnoses were established by history, systemic survey, imaging studies, and biopsy. Treatment included chemotherapy, hormone therapy, irradiation, surgical excision, or observation, depending on clinical circumstances. Among patients with sufficient follow-up, 95% died of metastasis, with overall mean survival of 15 months (median 15 months; range 3 to 96 months) after orbital diagnosis. CONCLUSIONS: The most common primary cancers that metastasize to the orbit are breast, prostate gland, and lung cancer. In 19%, there is no history of cancer when the patient presents with ophthalmic symptoms and in 10% the primary site remains obscure despite systemic evaluation. The systemic prognosis is generally poor.
UI - 11845358
AU - Unni KK
TI - Cartilaginous lesions of bone.
SO - J Orthop Sci 2001;6(5):457-72
AD - Mayo Clinic, Department of Laboratory Medicine and Pathology, 200 First Street SW, Rochester, MN 55905, USA.
Cartilaginous lesions of the skeleton are very unusual. It is extremely important to correlate the roentgenographic features, the clinical features, and the histological features to arrive at a definite diagnosis. Most cartilaginous lesions are benign or of low-grade malignancy. However, there are some subtypes of chondrosarcoma that behave in a highly aggressive fashion.
UI - 11723748
AU - Otsuka T; Yonezawa M; Kamiyama F; Matsushita Y; Matsui N
TI - Results of surgery and radio-hyperthermo-chemotherapy for patients with soft-tissue sarcoma.
SO - Int J Clin Oncol 2001 Oct;6(5):253-8
AD - Department of Orthopaedic Surgery, Nagoya City University Medical School, 1 Kawasumi, Mizuho-ku, Nagoya 467-8601, Japan. email@example.com
BACKGROUND: Between 1990 and 1999, we performed radio-hyperthermo-chemotherapy (RHC) in 44 patients with high-grade soft-tissue sarcomas of the limbs. METHODS: Radiotherapy involved the delivery of radiation at a dose of 2 Gy once daily on 16 days, to give a total dose of 32 Gy. Hyperthermia was conducted once a week, with a total of five sessions. Chemotherapy was performed by implanting a reservoir and administering cisplatin (3 mg/kg) three times, and pinorubin (an adriamycin derivative; 1 mg/kg) twice by intra-arterial infusion, at weekly intervals. These drugs were administered alternately during hyperthermia sessions. RESULTS: Tumor shrinkage was observed in 98% (43/44) of the patients. Of the 36 patients with M0 tumors, 30 were disease-free at final follow-up, 2 had no evidence of disease, 1 was alive with disease, and 3 had died of the disease. Amputation was required only in the first patient, and the affected limb was preserved in the other 43 patients. The surgical margin was wide in 9 patients and marginal in 29 patients, and intralesional excision was performed in 5 patients. There was recurrence in only 1 of the 44 patients. CONCLUSION: RHC is currently the most potent and relatively safe treatment method for high-grade soft-tissue sarcomas that is available clinically.
UI - 11870247
AU - Savage DG; Antman KH
TI - Imatinib mesylate -- a new oral targeted therapy.
SO - N Engl J Med 2002 Feb 28;346(9):683-93
AD - Herbert Irving Comprehensive Cancer Center, Columbia University College of Physicians and Surgeons, New York, NY, USA.
UI - 11843853
AU - Pandey M; Mathew A; Kattoor J; Abraham EK; Mathew BS; Rajan B; Nair KM
TI - Malignant phyllodes tumor.
SO - Breast J 2001 Nov-Dec;7(6):411-6
AD - Division of Surgical Oncology, Regional Cancer Center, Medical College PO, Thiruvananthapuram, Kerala, India.
The study aims to evaluate the survival and prognosis of patients with malignant phyllodes tumor. Between 1982 and 1998, 37 women with malignant phyllodes tumor were treated at the Regional Cancer Center, Trivandrum. Twelve patients were recurrent. Survival was estimated using the Kaplan-Meier method. Patient, disease, and treatment factors were compared using log-rank test. The Cox-proportional hazard model was employed to identify the prognostic factors. Thirty-six patients had surgery. Twenty-five patients received postoperative radiotherapy, and 2 received chemotherapy in addition. The median follow-up was 43 months (range 1-170 months). Eight patients failed locally, and 7 of these were successfully salvaged by surgery. The 5-year overall survival was 74.2% (95% CI, 0.44 to 0.89), whereas 5-year disease-free survival was 59.6% (95% CI, 0.39 to 0.7). The margin of surgical excision was found to be the only independent prognostic factor (p=0.003). However, patients with tumor size more than 5 cm (hazard ratio 2.9) were found to have increased hazard, whereas those receiving adjuvant radiotherapy (hazard ratio 0.6), married women (hazard ratio 0.4), and those women over the age of 35 years (hazard ratio 0.7) showed a decreased hazards. Cystosarcoma phyllodes is a rare malignancy of the female breast. Surgery with adequate margins is the primary treatment. Adjuvant radiotherapy appears to improve the disease-free survival.
UI - 11823688
AU - Janjan N; Crane C; Delclos M; Ballo M
TI - Brachytherapy for locally recurrent soft-tissue sarcoma.
SO - Am J Clin Oncol 2002 Feb;25(1):9-15
AD - University of Texas M. D. Anderson Cancer Center, 1515 Holcombe Boulevard, Houston, TX 77030, U.S.A.
Radiation is used to reduce potential risk of local recurrence from microscopic residual disease after surgical resection. Brachytherapy is a clinically established means of providing radiation for soft-tissue sarcomas that recur after surgical resection alone or surgical resection and radiation. Although the total dose of radiation that is prescribed is approximately the same for patients undergoing external beam radiation or brachytherapy, the radiobiologic characteristics of brachytherapy, based on the inverse-square law, provide higher doses of radiation to the surgical bed. This provides a theoretical advantage for the use of brachytherapy as compared with external beam radiation among patients with recurrence after surgical resection. When soft-tissue sarcomas recur in a previously irradiated area, further external beam radiation generally is not possible; therefore, brachytherapy allows a radiotherapeutic alternative in an attempt to reduce the risk of further local recurrence. Recommendations for patient selection, the total dose of radiation, and the radiation dose-rate are outlined. Standard grading systems for response, symptoms, and severity of complications should be used.
UI - 11856160
AU - Erhan Y; Zekioglu O; Ersoy O; Tugan D; Aydede H; Sakarya A; Kapkac M;
TI - Ozdemir N; Ozbal O; Erhan Y p53 and Ki-67 expression as prognostic factors in cystosarcoma phyllodes.
SO - Breast J 2002 Jan-Feb;8(1):38-44
AD - General Surgery Department, Celal Bayar University, School of Medicine, Guzelyali-Izmir, Turkey. firstname.lastname@example.org
We have reviewed the histopathological, clinical outcome and immunohistochemical status in 21 women with cystosarcoma phyllodes (CSP) tumors of the breast. We assessed 12 tumors as histopathologically benign and 9 tumors as malignant. The median patient ages in benign and malignant CSP tumors were 39.6 and 45.4 years of age, respectively. The stromal cellularity, stromal cellular atypism, high mitotic activity, atypic mitoses, stromal overgrowth, infiltrative tumor contour, and heterologous stromal elements were significant features of the malignant CSP tumors. Benign CSP tumors were predominantly of fibroadenomatous architecture with cellular stroma (mild or moderate) and some distortion and elongation of glandular elements. Five malignant CSP tumors were stained positively with p53, and 6 malignant CSP tumors were stained immunohistochemically with Ki-67. All benign CSP tumors were negatively stained for p53 and Ki-67. The patients with benign CSP tumors were treated with local excision ( n=11) and with subcutaneous mastectomy ( n=1). Malignant CSP tumors were treated with wide local excision ( n=1), partial mastectomy ( n=1), simple mastectomy ( n=2), and modified radical mastectomy ( n=5). Two patients with a high mitotic rate and high values of p53 and Ki-67 received additional radiotherapy and chemotherapy. One case had liver metastasis. This tumor had high mitotic figures, stromal overgrowth, severe stromal cellularity, and 20% Ki-67 and mild p53 positivity. We suggest that p53 and Ki-67 can play an important role in predicting prognosis and yielding additional therapy besides conventional prognostic factors in the treatment of the CSP patients.
UI - 11857314
AU - Edmonson JH; Petersen IA; Shives TC; Mahoney MR; Rock MG; Haddock MG;
TI - Sim FH; Maples WJ; O'Connor MI; Gunderson LL; Foo ML; Pritchard DJ; Buckner JC; Stafford SL Chemotherapy, irradiation, and surgery for function-preserving therapy of primary extremity soft tissue sarcomas: initial treatment with ifosfamide, mitomycin, doxorubicin, and cisplatin plus granulocyte macrophage-colony-stimulating factor.
SO - Cancer 2002 Feb 1;94(3):786-92
AD - Mayo Clinic, Rochester, Minnesota 55905, USA. email@example.com
BACKGROUND: Most institutional teams utilize multimodality therapy in their efforts to cure patients with primary high-grade extremity soft tissue sarcomas, although the value of adjuvant systemic chemotherapy is still disputed by some oncologists. This single-institution Phase II study describes an effort to control metastasis by the use of two cycles of chemotherapy as the initial preoperative treatment. METHODS: Between or limb girdle high-grade soft tissue sarcomas were registered to a study of preoperative ifosfamide, mitomycin, doxorubicin, cisplatin (IMAP) plus granulocyte macrophage-colony-stimulating factor (GM-CSF) followed by preoperative irradiation and subsequent limb-sparing surgery. The two sequential monthly cycles of IMAP involved intravenous ifosfamide, 2500 mg/m(2), and mesna, 2500 mg/m(2), on Day 0, followed by identical doses of these agents plus intravenous mitomycin, 4 mg/m(2), doxorubicin, 40 mg/m(2), and cisplatin, 60 mg/m(2), on Day 1. Sargramostim (GM-CSF) 250 microg/m(2) was given subcutaneously every 12 hours for 4 days beginning 6 days before the chemotherapy, and then for 14 more days beginning the day after chemotherapy was completed. At the beginning of the third month, external beam irradiation was administered daily, 5 days each week for 5 consecutive weeks to total preoperative doses of 4500 centigrays (cGy). This was accompanied by reduced doses of MAP chemotherapy (mitomycin, 6 mg/m(2), doxorubicin, 30 mg/m(2), and cisplatin, 45 mg/m(2)) intravenously on Days 0, 21, and 42 of the radiation therapy segment. Approximately 1 month after preoperative irradiation ended, each patient had complete surgical excision with curative intent, using limb-sparing techniques when possible. Radiation to total doses of 5500-6500 cGy was accomplished by delivery of an additional 1000-2000 cGy to the tumor bed via intraoperative electron beam, brachytherapy, or external beam irradiation at the completion of surgery. RESULTS: All except 5 patients had tumors at least 5 cm in diameter. Chemotherapy toxicity grade three or higher consisted primarily of vomiting (23%), leukopenia (54%), and thrombocytopenia (77%). Six patients have died of metastatic sarcoma, and one other died in a motorcycle accident. Kaplan-Meier curves indicate estimated 5-year survival of approximately 80% and freedom from metastasis at 2 years of approximately 85%. CONCLUSIONS: IMAP plus GM-CSF is satisfactory as initial treatment for primary extremity soft tissue sarcomas in two monthly cycles preceding irradiation. The prescribed irradiation was generally tolerable and effective in permitting limb-sparing surgery. Although the outcome of patients treated on this regimen has been favorable, the metastasis problem has not been eliminated. Copyright 2002 American Cancer Society. DOI 10.1002/cncr.10259
UI - 11872294
AU - Petersen IA; Haddock MG; Donohue JH; Nagorney DM; Grill JP; Sargent DJ;
TI - Gunderson LL Use of intraoperative electron beam radiotherapy in the management of retroperitoneal soft tissue sarcomas.
SO - Int J Radiat Oncol Biol Phys 2002 Feb 1;52(2):469-75
AD - Division of Radiation Oncology, Mayo Clinic, Rochester, MN 55905, USA. firstname.lastname@example.org
PURPOSE: To evaluate the disease control, survival results, and tolerance of intraoperative electron beam radiotherapy (IOERT) as a component of treatment for retroperitoneal soft tissue sarcomas. METHODS primary (n = 43) or recurrent (n = 44) retroperitoneal or intrapelvic sarcomas received IOERT as a component of treatment at the Mayo Clinic. The tumors were high grade in 54 patients (62%) and low grade in 33 (38%). The median tumor size was 10 cm (range 2-36). All patients underwent maximal surgical resection with IOERT; in 72 patients, only microscopic or no residual tumor remained. The IOERT doses ranged from 8.75 to 30 Gy (median 15). All primary tumors received external beam irradiation (EBRT) with a median dose of 48.6 Gy. Thirty-four of the 44 recurrent tumors received EBRT to a median dose of 45 Gy. All patients were followed prospectively for outcome and toxicity evaluation. RESULTS: The median follow-up, based on 46 patients (53%) currently alive, was 3.5 years. The overall estimated 5-year survival was 47%. For patients with tumors > or = 10 cm, the 5-year overall survival was significantly poorer (28%) than for those with smaller lesions (60%) (p = 0.01). Neither primary vs. recurrent status nor tumor grade had a significant impact on survival. Patients with gross residual tumor had a marginally significantly poorer survival compared with patients with microscopic or no residual tumor, with a 5-year survival rate of 37% and 52%, respectively (p = 0.08). A total of 49 patients (56%) experienced failure, including 20 local recurrences (23%). The median time to failure was 2.3 years. Four recurrences were within the IOERT field, 3 within the IOERT and EBRT field, and 13 within the EBRT field alone. The 3- and 5-year estimated local control rate was 77% and 59%, respectively. Local control was marginally significantly affected by the amount of residual tumor, with a 5-year local control rate of 41% for those with gross residual tumor, 60% for those with microscopic residual tumor, and 100% for those with no residual tumor (p = 0.09). Gastrointestinal complications were recorded in 12 incidences (Grade 3 or higher toxicity). These complications were believed to be secondary to surgery and/or EBRT in 10 of the 12 cases. Seven patients had fistula formation, and 3 experienced severe proctitis. Grade 3 peripheral neurologic toxicities occurred in 9 patients (10%), but none had pain as a component of their neuropathy. CONCLUSION: Retroperitoneal soft tissue sarcomas can be treated with an aggressive combined approach of EBRT, surgery, and IOERT, with acceptable toxicity. Local control in primary disease appears to be improved in this retrospective series with this approach. Distant disease control and options for recurrent disease needs further definition.
UI - 11776610
AU - Bai P; Zhang W; Sun J
TI - [Combination chemotherapy of uterine sarcomas]
SO - Zhonghua Zhong Liu Za Zhi 2000 Jan;22(1):80-2
AD - Cancer Institute (Hospital), Chinese Academy of Medical Sciences, Peking Union Medical College, Beijing 100021, China.
OBJECTIVE: To study the results of combination chemotherapy of uterine sarcoma after operation and recurrent tumor. METHODS: One hundred seventy-four cases of three major pathological subtypes of uterine sarcomas were treated in the Cancer Hospital from 1960 to 1996. Clinical data were analyzed of 51 cases of uterine sarcomas treated with postoperative adjuvant chemotherapy and 38 cases with recurrent tumors received 98 courses of chemotherapy. They were divided into 4 groups according to the adjuvant chemotherapy regimen: single drug, VAC, VAD, and other regimens. Chemotherapy regimens for recurrent tumors were VAD, PA/PAC, and other combination regimens including etoposide, ifosfamide, cisplatin, adriamycin. RESULTS: The 5-year survival rate of stage I-II uterine sarcoma patients was 54.9% receiving adjuvant chemotherapy. It was 72.7% in VAD group which was significantly higher than that in other regimen groups. The survival rate was related to the number of chemotherapy course. The chemo-sensitivity of various pathological types of recurrent uterine sarcomas was not different. CONCLUSION: The 5-year survival rate does not improve in patients with stage I-II uterine sarcomas given postoperative chemotherapy. VAD is among the best regimens and at least 3 courses should be performed. The results of new treatment regimens such as EPA, IA, etc., must await further clinical observation.
UI - 11828318
AU - Takanami I; Imamura T
TI - The treatment of Askin tumor: results of two cases.
SO - J Thorac Cardiovasc Surg 2002 Feb;123(2):391-2
AD - Department of Surgery, Teikyo School of Medicine, Tokyo, Japan. email@example.com
UI - 11848531
AU - Schwarzbach MH; Dimitrakopoulou-Strauss A; Mechtersheimer G; Hinz U;
TI - Willeke F; Cardona S; Attigah N; Strauss LG; Herfarth C; Lehnert T Assessment of soft tissue lesions suspicious for liposarcoma by F18-deoxyglucose (FDG) positron emission tomography (PET).
SO - Anticancer Res 2001 Sep-Oct;21(5):3609-14
AD - Department of Surgery, University of Heidelberg, Germany. firstname.lastname@example.org
BACKGROUND: F18-deoxyglucose (FDG) positron emission tomography (PET) is a promising imaging technique. The aim of this study was to investigate the use of FDG PET in patients with suspected liposarcomas (LS). PATIENTS AND METHODS: Forty-two masses were studied. The FDG uptake was estimated in tumor (T) and normal tissue (NT). The data were analyzed with respect to pathological findings. RESULTS: Pathology revealed 11 primary LS, 14 locally recurrent LS, 5 other sarcomas, 1 inflammation, 1 lymphoma and 10 benign lesions. FDG uptake (T-to-NT ratio) in 25 LS corresponded with the histological subtype. Pleomorphic, mixed and myxoid LS showed an increased T-to-NT ratio and were thus visualized. Four out of six well-differentiated LS presented a low FDG uptake. Like subtype, the tumor grade also corresponded to FDG uptake. The T-to-NT ratio of higher grade LS, contrary to low grade LS, was uniformly increased. Primary LS were distinguishable from benign tumors, while other sarcomas, inflammation and lymphoma were not. Recurrence was detected with a sensitivity of 86% and a specificity of 100%. False-negative diagnoses occurred only in well-differentiated recurrences. CONCLUSION: FDG uptake in LS correlates with the histological subtype and tumor grade. The diagnostic value of FDG PET in LS, therefore, is influenced by histomorphological parameters. Our data suggest that pleomorphic, mixed and higher-grade LS recurrences are preferentially amenable to FDG PET imaging.
UI - 11869019
AU - Kunisada T; Ngan SY; Powell G; Choong PF
TI - Wound complications following pre-operative radiotherapy for soft tissue sarcoma.
SO - Eur J Surg Oncol 2002 Feb;28(1):75-9
AD - Bone and Soft Tissue Sarcoma Unit, St. Vincent's Hospital and Peter MacCallum Cancer Institute, Level 3 Daly Wing, 41 Victoria Parade, Fitzroy, Victoria 3065, Australia.
AIMS: We analysed wound complications in 43 patients with soft tissue sarcoma who were treated with combined pre-operative radiotherapy and surgery. METHODS: All patients received the same protocol of pre-operative radiotherapy at our institution. RESULTS: Thirty-six (84%) patients developed acute skin toxicity following radiotherapy. After wide local excision, 15 patients required primary soft tissue reconstruction with vascularized muscle transfer and four patients underwent free skin flap to enable wound closure as part of their primary surgery. Nineteen patients (44%) developed post-operative wound complications including 10 (23%) patients who required an additional surgical procedure. Four (27%) patients developed flap necrosis in a group of 15 who underwent primary vascularized soft tissue transfer. All required a second vascularized muscular flap. One elderly patient, who had grade 3 acute radiation skin toxicity, had an arterial graft and total hip arthroplasty for a femoral artery aneurysm and an avascular necrosis of the hip, respectively. In our series, age (> or = 40 years) was the only impact factor influencing wound complication after surgery following radiotherapy (P=0.06). CONCLUSIONS: Site of tumour, radiation field size, surgical resection volume, grade of acute radiation toxicity, co-morbidity, and smoking were not demonstrated to have predictive value in wound complication following pre-operative radiotherapy. Although previous papers suggested that vascularized soft tissue transfer could be useful reducing wound morbidity, our results could not confirm this. Copyright Harcourt Publishers Limited.
UI - 11869020
AU - Begossi G; Gonzalez-Moreno S; Ortega-Perez G; Fon LJ; Sugarbaker PH
TI - Cytoreduction and intraperitoneal chemotherapy for the management of peritoneal carcinomatosis, sarcomatosis and mesothelioma.
SO - Eur J Surg Oncol 2002 Feb;28(1):80-7
AD - The Washington Cancer Institute, Washington Hospital Center, Washington, DC 20005, USA.
Despite new developments in multi-modality treatments, complete resection remains as an absolute requirement for cure of gastrointestinal cancer. We have reported benefits from combined treatment with complete cytoreduction and intraperitoneal chemotherapy. This has been achieved with low morbidity and mortality. Success in the surgical management of peritoneal surface malignancy depends on the surgeon's ability to complete complex cytoreductive procedures so that only microscopic residual disease remains. This paper describes the current strategy that the surgical oncologist should pursue in the treatment of patients with peritoneal carcinomatosis, sarcomatosis and mesothelioma. Technical details required for this surgery include patient position, incision and exposure, complete lysis of adhesion, electroevaporative dissection with irrigation and suction to preserve the translucent quality of tissues, peritonectomy procedures, proper positioning of tubes and drains for intraperitoneal chemotherapy, and reconstructive surgery. Understanding the treatment and mastery of surgical skills to manage the peritoneal surface spread of cancer has led to long-term survival of selected patients. Combination of this treatment strategy with proper patient selection has reduced the mortality and morbidity. The success of cytoreductive surgery and perioperative intraperitoneal chemotherapy depends on a long-term dedication to achieve the full potential of a curative outcome. Our unit has continued to achieve good results over two decades as improved results of treatment have evolved. Copyright Harcourt Publishers Limited.
UI - 11796846
AU - Nuciforo PG; O'Hara CD
TI - Correspondence re: Bonetti F, Martignoni G, Colato C, Manfrin E, Gambacorta M, Faleri M, et al. Abdominopelvic sarcoma of perivascular epithelioid cells. Report of four cases in young women, one with tuberous sclerosis. Mod Pathol 2001;14:563-8. And Tazelaar HD, Batts KP, Srigley JR. Primary extrapulmonary sugar tumor (PEST): a report of four cases. Mod Pathol 2001;14:615-22.
SO - Mod Pathol 2002 Jan;15(1):87-90
UI - 11815866
AU - Wu SS; Collins MH; de Chadarevian JP
TI - Study of the regression process in cardiac rhabdomyomas.
SO - Pediatr Dev Pathol 2002 Jan-Feb;5(1):29-36
AD - Department of Pathology and Laboratory Medicine, MCP Hannemann University School of Medicine and St. Christopher's Hospital for Children, Front Street at Erie Avenue, Philadelphia, PA 19134, USA.
Rhabdomyomas are the most common primary cardiac tumors in children, and have been shown to undergo spontaneous regression. The aim of our study was to investigate morphologically and immunohistochemically some mechanisms that may explain this clinical phenomenon. Eleven tumors from three term newborn girls who had physical and radiographic features pathognomonic of tuberous sclerosis were evaluated. Control specimens were left and right heart sections from five autopsies of age- and sex-matched patients who died of causes unrelated to the cardiovascular system. The tumors had been surgically excised from various regions in the heart, and all had similar "typical" histology. Histomorphologic evaluation with von Kossa and alizarin-red stains and terminal deoxynucleotidyl transferase-mediated deoxyuridine triphosphate nick end-labeling (TUNEL) method were performed to evaluate cell calcifications, necrosis, and apoptosis. Ubiquitin immunohistochemical study was also conducted to evaluate intracytoplasmic protein degradation. In cardiac rhabdomyomas (CR), all myocytes with acidophilic cytoplasmic myofibrils showed strong intracytoplasmic ubiquitin immunoreactivity, compared with the occasional weak cytoplasmic and focal nuclear positivity in control heart sections. Calcified myocyte nuclei were commonly seen in CR tumoral and nontumoral rhabdomyocytes, whereas control nontumoral cardiac myocytes did not show any calcification. The incidence of TUNEL reactivity seen in CR (4.8 nuclei per 100 cardiac rhabdomyocyte nuclei) was higher than that seen in control heart sections (1.7 nuclei per 106 cardiac myocytes, P < 0.005). The data led us to conclude that the cytoplasmic contents in CR were degraded via the ubiquitin pathway, and from our observation of increased TUNEL positivity, the rate of cell death in CR appeared increased. These findings may explain, to some extent, the mechanism of tumor regression.
UI - 11276029
AU - Polgar C; Orosz Z; Fodor J
TI - Is postirradiation angiosarcoma of the breast so rare and does breast lymphedema contribute to its development?
SO - J Surg Oncol 2001 Mar;76(3):239-41
UI - 11836422
AU - Lagunoff M; Bechtel J; Venetsanakos E; Roy AM; Abbey N; Herndier B;
TI - McMahon M; Ganem D De novo infection and serial transmission of Kaposi's sarcoma-associated herpesvirus in cultured endothelial cells.
SO - J Virol 2002 Mar;76(5):2440-8
AD - Department of Microbiology and Medicine, Howard Hughes Medical Institute, University of California-San Francisco, 513 Parnassus Ave., San Francisco, CA 94143-0414, USA.
Infection by Kaposi's sarcoma-associated herpesvirus (KSHV) is central to the pathogenesis of the endothelial neoplasm Kaposi's sarcoma (KS) and is also linked to the rare B-cell tumor known as primary effusion lymphoma (PEL). Latently infected PEL cell lines can be induced to enter the lytic cycle and produce KSHV virions. However, such cells do not support de novo infection or serial propagation of KSHV. These limitations have prevented the development of systems for the genetic analysis of KSHV and have impeded a deeper understanding of KS pathogenesis. Here we show that human dermal microvascular endothelial cells immortalized by expression of telomerase can be readily infected by KSHV virions produced by PEL cells. Infection is predominantly latent, but a small subpopulation enters the lytic cycle spontaneously. Phorbol ester (tetradecanoyl phorbol acetate [TPA]) treatment of latently infected cells leads to enhanced induction of lytic KSHV replication, resulting in foci of cytopathic effect. There is no cytopathic effect or viral DNA expansion when infected TIME cells (telomerase-immortalized microvascular endothelial cells) are TPA induced in the presence of phosphonoacetic acid (PAA), an inhibitor of herpesvirus replication. Supernatants from phorbol-induced cultures transfer latent KSHV infection to uninfected cells, which can likewise be induced to undergo lytic replication by TPA treatment, and the virus can be further serially transmitted. Serial passage of the virus in TIME cells is completely inhibited when TPA treatment is done in the presence of PAA. Latently infected endothelial cells do not undergo major morphological changes or growth transformation, and infection is lost from the culture upon serial passage. This behavior faithfully recapitulates the behavior of spindle cells explanted from primary KS biopsies, strongly supporting the biological relevance of this culture system. These findings suggest that either the stability or the growth-deregulatory potential of the KSHV latency program in endothelial cells is more limited than might be predicted by analogy with other oncogenic viruses.
UI - 11443456
AU - Nakajima J; Takamoto S; Tanaka M; Takeuchi E; Murakawa T; Fukami T
TI - Thoracoscopic surgery and conventional open thoracotomy in metastatic lung cancer.
SO - Surg Endosc 2001 Aug;15(8):849-53
AD - Department of Cardiothoracic Surgery, Faculty of Medicine, University of Tokyo, 7-3-1, Hongo, Bunkyo-ku, Tokyo 113-8655, Japan. email@example.com
BACKGROUND: We performed a retrospective comparison of the oncological outcome of thoracoscopic surgery for pulmonary metastasis with that of conventional open thoracotomy. METHODS: The patient population for our retrospective comparison was comprised of 45 patients undergoing pulmonary resections via video-assisted thoracoscopy (thoracoscopy group) and 55 undergoing similar resections by open thoracotomy (open group) for pulmonary metastases between 1994 and 1999. RESULTS: Solitary metastasis was resected more frequently with thoracoscopy than open thoracotomy. There were no significant intergroup differences in rates of local recurrence from the initial pulmonary resection site. The actuarial 1-year, 2-year, and 3-year survival rates were, respectively, 82.8%, 70.0%, and 62.3% in the thoracoscopy group and 93.6%, 64.6%, and 52.7% in the open group. The rates of pulmonary recurrence and survival also did not differ significantly between the two groups with solitary metastases. CONCLUSION: Thoracoscopic surgery for metastatic lung disease appears to be feasible as long as the preoperative metastatic tumor evaluation using chest computed tomography (CT) is accurate.
UI - 11786573
AU - Ferrari A; Bisogno G; Casanova M; Meazza C; Piva L; Cecchetto G; Zanetti
TI - I; Pilz T; Mattke A; Treuner J; Carli M Paratesticular rhabdomyosarcoma: report from the Italian and German Cooperative Group.
SO - J Clin Oncol 2002 Jan 15;20(2):449-55
AD - Pediatric Oncology and Pediatric Surgery Units, Istituto Nazionale Tumori, Milan, Italy. firstname.lastname@example.org
PURPOSE: We report the experience of the German-Italian Cooperative Group with 216 pediatric patients with paratesticular rhabdomyosarcoma treated over 20 years. PATIENTS AND METHODS: At diagnosis, 198 patients had localized disease and 18 had distant metastases. Among the nonmetastatic patients, complete tumor resection was performed in 83% of cases. Evaluation of the retroperitoneal lymph nodes changed over the years from routine surgical staging to radiologic assessment. All patients received chemotherapy, which was reduced in intensity and duration for patients with low-risk features in subsequent protocols. Radiotherapy was administered to 10% of patients. RESULTS: Among 72 patients with a negative retroperitoneal computed tomography (CT) scan, surgical assessment detected nodal involvement in only one case. Among 23 patients with enlarged nodes on CT scans, surgery confirmed nodal spread in 65% of patients. No differences in the rate of nodal involvement were observed over the years. With a median follow-up of 110 months, 5-year survival was 85.5% for the series as a whole, 94.6% for patients with localized disease, and 22.2% for metastatic cases. Retroperitoneal nodal recurrence was the major cause of treatment failure. Univariate analysis revealed the prognostic value of tumor invasiveness, size, and resectability, as well as of nodal involvement and age, in patients with localized tumor. CONCLUSION: The outcome for patients with localized paratesticular rhabdomyosarcoma is excellent, despite the reduction in chemotherapy over the years: an alkylating agent-free and anthracycline-free regimen is adequate treatment for low-risk patients. Surgical assessment of the retroperitoneum must be reserved for patients with enlarged nodes on CT scans. Children over 10 years old carry a higher risk of nodal involvement and relapse.
UI - 11868476
AU - Palacios E; Friedlander PL
TI - Chondrosarcoma of the larynx.
SO - Ear Nose Throat J 2002 Feb;81(2):83
AD - Department of Radiology, Louisiana State University Health Sciences Center, New Orleans, USA.
UI - 11886321
AU - Andreoni M; Sarmati L; Nicastri E; El Sawaf G; El Zalabani M; Uccella I;
TI - Bugarini R; Parisi SG; Rezza G Primary human herpesvirus 8 infection in immunocompetent children.
SO - JAMA 2002 Mar 13;287(10):1295-300
AD - Department of Public Health and Cellular Biology, University Tor Vergata, Via Montpellier 1, 00133 Rome, Italy. email@example.com
CONTEXT: Human herpesvirus 8 (HHV-8) infection causes Kaposi sarcoma and lymphoproliferative disorders in immunosuppressed adults. Its manifestations in immunocompetent hosts are unknown. OBJECTIVES: To determine whether HHV-8 primary infection is symptomatic in immunocompetent children and to identify the epidemiological and virological correlates of HHV-8 infection. DESIGN AND SETTING: Prospective cohort study conducted in the pediatric emergency department of a hospital in Alexandria, Egypt, between December 1, 1999, and April 30, 2000. PATIENTS: Eighty-six children aged 1 to 4 years who were evaluated for a febrile syndrome of undetermined origin. MAIN OUTCOME MEASURES: Serological assay and polymerase chain reaction of blood and saliva samples for HHV-8. Information on potential risk factors for HHV-8 infection was also collected. RESULTS: Thirty-six children (41.9%) were seropositive; HHV-8 DNA sequences were detected in 14 (38.9%) of these 36 children (detected in saliva in 11 of 14). Significant associations were found between HHV-8 infection and close contact with at least 2 other children in the community (36 of 63 vs 6 of 23 for <2 children; adjusted odds ratio [OR], 3.50; 95% confidence interval [CI], 1.11-12.22) and admission to the emergency department in December or January (28 of 47 vs 14 of 39 for February-April; adjusted OR, 3.15; 95% CI, 1.23-8.58). Six children had suspected primary HHV-8 infection; all but 1 had a febrile cutaneous craniocaudal maculopapular rash, which was more common among these children (5 of 6 vs 10 of 75; P<.001). For 3 of these 6 children, a second blood sample was obtained after the convalescence phase, and all 3 seroconverted for HHV-8. CONCLUSIONS: Primary infection with HHV-8 may be associated with a febrile maculopapular skin rash among immunocompetent children. The finding of HHV-8 DNA sequences in saliva supports the hypothesis that transmission through saliva is the main mode of transmission in the pediatric age group.
UI - 11850069
AU - Nishio J; Iwasaki H; Ohjimi Y; Ishiguro M; Isayama T; Naito M; Iwashita
TI - A; Kikuchi M Overrepresentation of 17q22-qter and 22q13 in dermatofibrosarcoma protuberans but not in dermatofibroma: a comparative genomic hybridization study.
SO - Cancer Genet Cytogenet 2002 Jan 15;132(2):102-8
AD - Department of Pathology, School of Medicine, Fukuoka University, Fukuoka, Japan. firstname.lastname@example.org
Histopathological differentiation between dermatofibrosarcoma protuberans (DFSP) and dermatofibroma (DF) is often difficult, because both neoplasms share some clinical features and the presence of a storiform pattern. In the present study, we investigated the usefulness of comparative genomic hybridization (CGH) in the diagnosis of these entities by examining 12 DFSP and 12 DF cases. The most frequent DNA sequence copy number changes detected in 10 (83%) of 12 DFSP cases (mean, 1.9 aberrations/tumor; range, 0-3) consisted of gains of 17q22-qter (10 tumors), 22q13 (nine tumors), and 8q24.1-qter (three tumors). High-level amplification, which was detected in three tumors, was seen only in chromosome 17, with 17q23-q25 as the minimal common region. Loss of DNA sequences was not found in DFSP cases. In contrast, two (17%) of the 12 DF cases (mean, 0.5 aberrations/tumor; range, 0-4) showed DNA sequence copy number changes, although recurrent gains and losses and high-level amplifications were not observed. Gains were more common than losses in DF. Overrepresentation of 17q and 22q sequences was a common finding in DFSP but not in DF. Thus, CGH seems to be useful for distinguishing DFSP from DF in most cases.
UI - 11850075
AU - Mathew S; Dalton J; Riedley S; Spunt SL; Hill DA
TI - Complex t(X;18)(p11.2;q11.2) with a pericentric inversion of the X chromosome in an adolescent boy with synovial sarcoma.
SO - Cancer Genet Cytogenet 2002 Jan 15;132(2):136-40
AD - Department of Pathology, St. Jude Children's Research Hospital, 332 North Lauderdale, Memphis, TN 38105-2794, USA. email@example.com
Synovial sarcoma is the most common nonrhabdomyosarcomatous soft-tissue sarcoma in children and young adults. It is characterized by the common t(X;18)(p11.2;q11.2) that results in the fusion of SYT on chromosome 18 to one of two closely related and adjacent genes on the X chromosome, SSX1 or SSX2. Here we describe a poorly differentiated, monophasic synovial sarcoma in a 17-year-old adolescent boy. Hyperdiploidy, a t(X;18)(q13;q11), and other structural abnormalities were detected by conventional cytogenetic analysis. Fluorescence in situ hybridization with the PAC probe RP3-519N18, which is specific for the Xp11 region, resulted in a signal on the der(Xq), a finding consistent with a pericentric inversion of the X chromosome that resulted in a t(X;18)(p11.2;q11.2)inv(X)(p11.2q13). Real-time polymerase chain reaction using primer sets specific for SYT-SSX1 and SYT-SSX2 confirmed the presence of an SYT-SSX1 fusion transcript. Our finding of this unique and complex translocation in synovial sarcoma demonstrates the utility of molecular methods in confirming the diagnosis of synovial sarcoma.
UI - 11847031
AU - Bui BN; Tabrizi R; Dagada C; Trufflandier N; St ckle E; Coindre JM
TI - [Update on soft tissue sarcomas]
SO - Bull Cancer 2002 Jan;89(1):100-7
AD - Institut Bergonie, Centre regional de lutte contre le cancer, 229, cours de l'Argonne, 33076 Bordeaux Cedex.
Important refinements have taken place in the diagnosis of soft tissue sarcoma with extensive use of immuno-histochemistry. New entities have been described, while malignant histiocytofibroma, the most diagnosed sarcoma type during the last two decades, has been dismembered. As for prognosis, the new UICC classification is effectively more discriminating in the definition of prognostic groups; but the usefullness of new biological or genetic markers remains to be assessed. Several breakthrough have taken place in the last years in the treatment of soft tissue sarcoma. Isolated limb perfusion with TNF, hyperthermia and melphalan have proven its efficacy, and is now an alternative to preoperative chemotherapy and/or radiotherapy for limb sparing treatment of the primary tumor site or to amputation. For systemic treatments, novel cytostatic drugs have been shown to be active in sarcomas, including ecteinascidine (ET743) and Glivec (STI571). This last drug has been shown to be remarkably active in c-kit+ stromal sarcoma of the gastro-intestinal tract. It can hopefully regarded as an example for targeted therapies, which may come with a better understanding of the molecular mechanisms triggered by the fundamental, specific genetic alterations shown in sarcoma.
UI - 11847032
AU - Bergeron C; Ranchere-Vince D; Berard-Marec P
TI - [Update on rhabdomyosarcomas in children]
SO - Bull Cancer 2002 Jan;89(1):108-12
AD - Departement de pediatrie, Centre Leon-Berard, 28, rue Laennec, 69373 Lyon Cedex 08.
Rhabdomyosarcoma is a rare tumour corresponding to 60-70% of soft tissue sarcomas in children. Significant advances in treatment have been made possible, and will be further obtained, by multicentric treatment protocols conducted in paediatric oncology centres. Overall survival and disease-free survival have been significantly improved over the past 30 years. In the meantime, diagnosis improvements have made the classification of rhabdomyosarcomas more complex. A review of European and American studies has evidenced a number of criteria that should be taken into account for selecting treatment strategy: histological examination (refined with the use of molecular biology) had proved very informative, suggesting a worse prognosis for alveolar rhabdomyosarcomas. Other criteria of interest are the tumour site (favourable or unfavourable), patient age (under or above 10), tumour size ( 5 cm), and disease stage. The number of sub-groups of patients requiring different, more adapted treatment strategies increases with the number of prognostic parameters to be considered. For convenient clinical management, patients will therefore be classified into 4 risk groups for systemic therapy (low, standard, high and very high risk), whereas local treatment strategies will take into account the whole set of prognostic criteria defined above.
UI - 11398160
AU - Wunder JS; Leitch K; Griffin AM; Davis AM; Bell RS
TI - Comparison of two methods of reconstruction for primary malignant tumors at the knee: a sequential cohort study.
SO - J Surg Oncol 2001 Jun;77(2):89-99; discussion 100
AD - University Musculoskeletal Oncology Unit, Mount Sinai Hospital, and the Department of Surgery, University of Toronto, Toronto, Canada. firstname.lastname@example.org
BACKGROUND AND OBJECTIVES: The purpose of this study was to compare the complications and functional outcome associated with the use of an irradiated allograft-implant composite or a bone-ingrowth modular tumor prosthesis for replacement of the knee joint after resection of a bone sarcoma from the distal femur or proximal tibia. METHODS: Eleven patients initially received an allograft reconstruction, followed by 64 treated with a tumor prosthesis. The primary analysis concerned reconstructive failure, defined by the requirement for removal of the original construct. Functional outcome was assessed by using the 1987 Musculoskeletal Tumor Society rating system. RESULTS: Reconstructive failure occurred in 6 of 11 (55%) allograft constructs compared with 10 of 64 (16%) tumor prostheses (P = 0.009). Failures were due to infection (2 of 11 allografts versus 4 of 64 prostheses; P = 0.2) or mechanical complications (4 of 11 allograft fractures versus 5 of 64 broken prosthetic stems and 1 aseptically loose prosthesis; P = 0.03). The limb salvage rate was 95% (61 of 64) for patients with a tumor prosthesis compared with 64% (7 of 11) for those with an allograft (P = 0.007). Patients with a tumor prosthesis had a better functional outcome with a mean score of 75% compared with 57% for those with an allograft reconstruction (P = 0.006). CONCLUSIONS: This comparative study suggests that limb salvage surgery at the knee has a better and more predictable outcome with a tumor prosthesis than with an allograft-implant reconstruction. Copyright 2001 Wiley-Liss, Inc.
UI - 11822026
AU - Shete S; Amos CI; Hwang SJ; Strong LC
TI - Individual-specific liability groups in genetic linkage, with applications to kindreds with Li-Fraumeni syndrome.
SO - Am J Hum Genet 2002 Mar;70(3):813-7
AD - Department of Epidemiology, The University of Texas M. D. Anderson Cancer Center, Houston, TX 77030, USA. email@example.com
In this report, we present a simple and powerful way to incorporate individual-specific liability classes into linkage analysis. The proposed method is applicable to both quantitative and qualitative traits. In linkage studies, we may have information about different covariates. Incorpo