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Accuracy of roentgenograms of the chest in metastases to the lungs.

The findings on roentgenographic and tomographic examination of the chest were compared in 63 instances of suspected pulmonary metastases. These were further compared with the findings at thoracotomy in 41 instances. Of the 60 patients, 30 had soft tissue or bone sarcomas and 30 had carcinomas arising from colon, ovary or breast, and it also included five with malignant melanoma. Three patients with sarcomas had more than one thoracotomy. The diagnosis of pulmonary metastases by roentgenography of the chest was correct in 60 of 63 instances. Tomograms showed more lesions in 14 of 33 instances of sarcomas and 14 of 30 instances of carcinomas. Thoracotomy revealed even more lesions than were detected by tomography in 21 out of 26 instances with sarcomas and eight of 15 instances of carcinomas. Of the 37 patients with a solitary metastasis detected on roentgenograms of the chest, 22 were found to have additional lesions on the tomograms, 11 of 16 sarcomas and 11 of 21 carcinomas. At thoracotomy, however, nine of ten patients with a single metastasis from sarcomas were found to have even more lesions, while, in patients with carcinomas, tomograms were found to be accurate. Routinely, prior to major ablative operations for sarcomas and before excision of pulmonary metastases, it is suggested that tomography be carried out.

Adolescent↗

Surgery for disseminated abdominal sarcoma.

Seventy-two consecutive patients with disseminated soft tissue sarcoma in the abdomen were prospectively placed in a program of debulking surgery. The tumor was completely resectable in 64% of the patients. Following the first exploration, the median survival was 23 months for those with resection of metastases and 9 months for those without resection (p less than or equal to 0.01); the former group had a survival rate of 28% at 3 years, 18% at 4 years, and 4% at 5 years (44%, 37%, and 10%, respectively, for low-grade sarcomas, i.e., grade I or II sarcomas), whereas in the latter group, none survived for 3 years. In the group with resection, patients with grade III tumors had a median survival longer by 6 months, and those with low-grade tumors by 28 months (p less than or equal to 0.001), over the respective median survival of patients with unresectable tumors. Metastasectomy appeared to prolong survival in all patients and significantly so in patients with low-grade tumors and those with long disease-free intervals.

Abdominal Neoplasms↗

A case of classical Kaposi's sarcoma of the penis showing a good response to high-energy pulsed carbon dioxide laser therapy.

Isolated Kaposi's sarcoma (KS) of the glans penis is rare; only 37 cases are reported in the literature. Treatment of KS consists mostly of old modalities, some old methods in updated improved versions, and some new and experimental therapies. We report a case of isolated classical Kaposi's sarcoma involving multiple sites of the penis in a 54-year-old man who was treated with high-energy pulsed carbon dioxide (CO2) laser therapy, resulting in complete resolution of the lesions and no evidence of recurrence.

Carbon Dioxide↗

Radiation-induced sarcoma following curative radiotherapy for testicular seminoma: case report and brief review of the literature.

We report a case of radiation-induced retroperitoneal leiomyosarcoma which developed 37 years after the patient received radiation therapy for testicular seminoma. The sarcoma originated within the para-aortic field, extensively involving neighboring organs, soft tissue and muscle tissues, and could be only partially resected. The absolute number of these secondary sarcomas is low, but the risk of developing such neoplasms calls for awareness in the long-term follow-up of cured seminoma patients.

Humans↗

[Gastric granulocytic sarcoma revealed by a massive digestive hemorrhage].

We report the case of a 37-year-old-man having a chronic myelogenous leukemia, who presented, one month after a splenic acutization, massive gastrointestinal bleeding from ulcerated nodules of the gastric fundus. The histologic examination of one of these nodules showed granulocytic sarcoma. In spite of an endoscopic treatment by sclerotherapy with adrenalined serum, the death occurred during a hemorrhagic recurrence. This observation, which is the third case reported of gastric granulocytic sarcoma during the acutization of a myelogenous chronic leukemia, and the first revealed by fatal gastrointestinal bleeding, shows the particular gravity of gastrointestinal bleeding complicating granulocytic sarcoma.

Adult↗

Primary synovial sarcoma of the gastrocolic ligament.

A case of primary synovial sarcoma of the gastrocolic ligament is reported in a 37-year-old woman. CT showed an encapsulated intraabdominal cystic tumour with an amorphous solid component. Barium studies showed a mass at the gastrocolic ligament which elevated the gastric antrum and displaced the transverse colon downwards. This is the first report of synovial sarcoma formation in the gastrocolic ligament. This tumour may have originated from pluripotential mesenchyme.

Adult↗

Predictors of survival in malignant tumors of the sternum.

From 1930 to 1994, 54 patients with primary malignant tumors of the sternum were seen. Fifty patients were first seen with a mass, and one half of them also had pain in the sternal region. Two patients had no symptoms at presentation. Among 39 solid tumors were 26 chondrosarcomas, 10 osteosarcomas, 1 fibrosarcoma, 1 angiosarcoma, and 1 malignant fibrous histiocytoma. Of these, 25 were low-grade and 14 were high-grade tumors. Among 15 small cell tumors were 8 plasmacytomas, 6 malignant lymphomas, and 1 Ewing's sarcoma. Partial or subtotal sternectomy was done in 37 patients and total sternectomy in 3. Of the remaining 14 patients, 3 had local excision; 10 had external radiation or chemotherapy without operation, or both; and 1 had no treatment. All but one patient treated by wide resection (N = 40) had some form of skeletal reconstruction of the chest wall defect. Thirty-one (78%) underwent repair with Marlex mesh, and in 25 this was combined with methyl methacrylate. The skin edges were closed per primum in 32 patients; 8 required muscle, omentum, or skin flaps. Resection in chondrosarcomas yielded a 5-year survival (Kaplan-Meier) of 80% (median follow-up, 17 years). The 5-year survival in osteosarcomas was 14%. Resection was curative in 64% of low-grade sarcomas but in only 7% of high-grade sarcomas. In small cell tumors, resection and radiation were helpful for local control; all failures were a result of distant metastases. We conclude that primary sarcomas of the sternum though uncommon are potentially curable by wide surgical excision. With rigid prostheses to repair the skeletal defects, the surgical complication rates are low. Overall survival after complete surgical resection is related to tumor histologic type and grade.

Bone Cements↗

Local control and functional results after twice-daily radiotherapy for Ewing's sarcoma of the extremities.

PURPOSE: Radiotherapy (RT) has been the predominant local treatment for Ewing's sarcoma of bone at the University of Florida. Twice-daily hyperfractionated RT was initiated in 1982 to improve local control and functional outcome. This retrospective review compares the results of once-daily vs. twice-daily RT in patients with primary Ewing's sarcoma of an extremity, with emphasis on functional outcome. METHODS AND MATERIALS: Between June 1971 and January 1990, 37 patients were treated at the University of Florida for nonmetastatic Ewing's sarcoma of bone with a primary lesion in an extremity. Three patients underwent amputation. Of 34 patients treated with RT, 31 had RT alone and 3 had a combination of RT and local excision. Before 1982, 14 patients received once-daily RT; since 1982, 17 patients have received twice-daily RT. Doses of once-daily RT varied from 47 to 61 Gy at 1.8-2 Gy per fraction. Doses of twice-daily RT varied, depending on the response of the soft-tissue component of the tumor to chemotherapy, and ranged from 50.4 to 60 Gy at 1.2 Gy per fraction. Some patients in the twice-daily RT group also received total body irradiation 1-3 months after local RT as part of a conditioning regimen before marrow-ablative therapy with stem cell rescue. They received either 8 Gy in two once-daily fractions or 12 Gy in six twice-daily fractions. The six patients who received surgery were excluded from local control analysis. Local control rates were calculated using the Kaplan-Meier (actuarial) method. Fifteen patients had a formal functional evaluation. RESULTS: In the 31 patients treated with RT alone, the actuarial local control rate at 5 years was 81% for patients treated twice daily and 77% for those treated once daily (p = NS). No posttreatment pathologic fractures occurred in patients treated twice daily, whereas five fractures occurred in those treated once daily (p = 0.01). On functional evaluation, less loss in range of motion (15 degrees vs. 28 degrees of loss, p = 0.02) and a lesser degree of muscle atrophy (8% vs. 21% loss in muscle circumference, p = 0.0004) occurred with twice-daily than with once-daily RT. A trend toward less fibrosis and less local alopecia was seen in patients treated twice daily. Patients treated twice daily received a higher Musculoskeletal Tumor Society functional rating (determined by the Department of Orthopaedics) than those treated once daily (29.4 vs. 26.0, p = 0.15). CONCLUSIONS: Local control rates were similar in the two groups (77% vs. 81%), but functional results were superior in the group treated twice daily.

Bone Neoplasms↗

Reconstruction of urinary tract combined with surgical management of locally advanced non-urological cancer involving the genitourinary organs.

INTRODUCTION: The objective of this study was to review our experience with urinary reconstruction in patients undergoing surgical management for locally advanced pelvic cancer, and to evaluate the role of urologists in these procedures. MATERIALS AND METHODS: This study included a total of 37 patients undergoing some type of urinary reconstruction due to invasion of the urological organs by locally advanced pelvic cancers, including 17 rectal cancers, 9 cervical cancers, 4 sigmoid cancers, 4 retroperitoneal sarcomas, 2 ovarian cancers and 1 appendiceal cancer. Among these 37, 18 were recurrent cancers following initial surgery for primary tumors. The clinical outcomes of these approaches were retrospectively analyzed. RESULTS: Of the 37 patients, 9 underwent cystectomy (group A) with the following urinary diversions: ileal neobladder in 3, ileal conduit in 5 and colon conduit in 1, and 12 underwent partial cystectomy (group B), among whom 11 received additional urinary reconstruction as follows: bladder flap repair in 5, psoas hitch in 2, ileal ureter in 2, bladder augmentation in 1 and ureteroureterostomy in 1, while the remaining 16 (group C), in whom complete bladder preservation was possible, underwent the following types of urinary reconstruction: bladder flap repair in 6, psoas hitch in 3, en bloc removal of the rectum with prostate in 3, ileal ureter in 2, and ureteroureterostomy in 2. There were 10 early urological complications, including leakage of urine in 7 and acute pyelonephritis in 3. As a late urological complication, hydronephrosis was observed in 8 patients, but ureteral stent was not required in any of these 8. There were no significant differences in the incidence of postoperative complications, the status of surgical margin and the survival among groups A-C. CONCLUSION: Our experience with extended surgical management of non-urological pelvic cancer with reconstruction of the urinary tract suggests that the urological portion of this procedure can be performed with acceptable morbidity, and that the role of the urological surgeon during this procedure is potentially important.

Adult↗

Estradiol and progesterone receptors in gynecologic sarcomas.

We report the concentrations of estradiol and progesterone receptors found in the tumor tissue of 5 patients with leiomyosarcoma, 4 of uterine and one of ventricular origin, and of 5 patients with uterine or ovarian carcinosarcoma. Steroid receptor positive and negative tumors were present in both groups of sarcoma. One uterine leiomyosarcoma had a high concentration of estradiol and progesterone receptors, 37 fmoles/mg protein and 156 fmoles/mg protein, respectively. We suggest that steroid receptors should be analysed in all gynecologic sarcomas.

Adult↗

Loss of heterozygosity at chromosome 1p in different solid human tumours: association with survival.

The distal half of chromosome 1p was analysed with 15 polymorphic microsatellite markers in 683 human solid tumours at different locations. Loss of heterozygosity (LOH) was observed at least at one site in 369 cases or 54% of the tumours. LOHs detected ranged from 30-64%, depending on tumour location. The major results regarding LOH at different tumour locations were as follows: stomach, 20/38 (53%); colon and rectum, 60/109 (55%); lung, 38/63 (60%); breast, 145/238 (61%); endometrium, 18/25 (72%); ovary, 17/31 (55%); testis, 11/30 (37%); kidney, 22/73 (30%); thyroid, 4/14 (29%); and sarcomas, 9/14 (64%). High percentages of LOH were seen in the 1p36.3, 1p36.1, 1p35-p34.3, 1p32 and 1p31 regions, suggesting the presence of tumour-suppressor genes. All these regions on chromosome 1p show high LOH in more than one tumour type. However, distinct patterns of LOH were detected at different tumour locations. There was a significant separation of survival curves, with and without LOH at chromosome 1p, in the breast cancer patients. Multivariate analysis showed that LOH at 1p in breast tumours is a better indicator for prognosis than the other variables tested in our model, including nodal metastasis.

Analysis of Variance↗

Osteomyelitis caused by Mycobacterium haemophilum: successful therapy in two patients with AIDS.

OBJECTIVE: Difficulties involved in diagnosis and response to antimicrobial therapy are described in detail for two cases of biopsy-proven osteomyelitis caused by Mycobacterium haemophilum in AIDS patients. SETTING: Two large, private teaching hospitals in New York City, New York, USA. PATIENTS, PARTICIPANTS: A 31-year-old woman with previous diagnoses of candida esophagitis and peripheral neuropathy (patient 1), and a 37-year-old man with Kaposi's sarcoma (patient 2). INTERVENTIONS: One patient was treated with a combination of rifampin, ethambutol, clofazimine, and ciprofloxacin, while the other received rifampin, ciprofloxacin and doxycycline. Both patients also received a short course of intravenous amikacin. MAIN OUTCOME MEASURES: Disease activity was monitored clinically by observing resolution of skin ulcers, lymphadenopathy, and pain and swelling in areas affected by osteomyelitis. RESULTS: Both patients experienced complete resolution of signs and symptoms of M. haemophilum infection. Patient 1 was treated for 17 months and remains well after 10 months without therapy. Patient 2 shows no evidence of infection after 14 months of therapy. CONCLUSIONS: M. haemophilum infection must be considered in the differential diagnosis of osteomyelitis in AIDS patients, although specialized culture techniques are required to isolate and identify this pathogen. Excellent clinical response can be achieved with oral antimicrobial therapy.

Acquired Immunodeficiency Syndrome↗

Acquired immune deficiency syndrome. The first cases in Finland.

We report here on the first two patients with acquired immune deficiency syndrome (AIDS) in Finland. The first patient, a 37-year-old man, had Kaposi's sarcoma and the other, a 26-year-old man, had pneumonia due to Pneumocystis carinii. Both patients were homosexuals and showed a marked defect in cell-mediated immunity consistent with the definition of AIDS. Both patients were found in a screening survey of homosexual men in Helsinki.

Acquired Immunodeficiency Syndrome↗

Long-term results of intraoperative electron beam radiotherapy for primary and recurrent retroperitoneal soft tissue sarcoma.

PURPOSE: This study assesses the long-term outcome of patients with retroperitoneal sarcoma treated by preoperative external beam radiotherapy, resection, and intraoperative electron beam radiation (IOERT). METHODS AND MATERIALS: From 1980 to 1996, 37 patients were treated with curative intent for primary or recurrent retroperitoneal soft tissue sarcoma. All patients underwent external beam radiotherapy with a median dose of 45 Gy. This was followed by laparotomy, resection, and IOERT, if feasible. Twenty patients received 10-20 Gy of IOERT with 9-15 MeV electrons. These patients were compared to a group of 17 patients receiving preoperative irradiation without IOERT. RESULTS: The 5-yr actuarial overall survival (OS), disease-free survival, local control (LC), and freedom from distant disease of all 37 patients was 50%, 38%, 59%, and 54%, respectively. After preoperative irradiation, 29 patients (78%) underwent gross total resection. For 16 patients undergoing gross total resection and IOERT, OS and LC were 74% and 83%, respectively. In contrast, these results were less satisfactory for 13 patients undergoing gross total resection without IOERT. For these patients, OS and LC were 30% and 61%, respectively. Four patients experienced treatment-related morbidity. CONCLUSIONS: In selected patients, IOERT results in excellent local control and disease-free survival with acceptable morbidity.

Adolescent↗

Adult head and neck soft-tissue sarcomas.

Adult soft tissue sarcomas of the head and neck are so rare that an accurate diagnosis and appropriate therapy are frequently delayed. A retrospective study of such patients seen at the University of Illinois over the last 15 years has helped to better characterize this disease entity. Patients with sarcoma of the neck, the most common anatomic location (37 per cent), had the highest 5-year disease-free survival rate (67 per cent). Fibrosarcoma was the most common histologic type (25 per cent). Aggressive fibromatosis patients experienced the longest mean survival time (93 months). The disease-free 2-year, 5-year, and 10-year survival rates were found to be 68, 54, and 28 per cent, respectively. Wide excision was the treatment of choice, with adjuvant radiotherapy or chemotherapy, or both, used in selected patients. The sarcomas of all long-term survivors were either well-differentiated or 5.0 cm or less in diameter. It is evident that aggressive therapy of adult head and neck sarcomas can provide good long-term results.

Adult↗

Safety and efficacy of ET-743: the French experience.

Initial evidence of clinical benefit with ecteinascidin-743 (ET-743) in patients with sarcoma was provided during a Phase I pharmacokinetic study in which 52 patients received ET-743 at doses of 50-1800 micrograms/m2 as a 24 h continuous infusion every 3 weeks. Neutropenia and thrombocytopenia were the dose-limiting toxicities; liver toxicity (a severe but transient and reversible increase in transaminase concentrations) was not treatment limiting. In conjunction with results obtained with ET-743 in a compassionate-use program, these indications of activity in heavily pretreated patients with sarcoma prompted initiation of a French multicenter Phase II study of ET-743 in this population. From February 1999 to January 2001, 54 patients with advanced anthracycline-pretreated soft-tissue sarcoma (STS) received ET-743 at a dose of 1500 micrograms/m2 every 3 weeks by continuous 24 h infusion. The main histological subtype was leiomyosarcoma (37%); the majority of primary tumors were visceral (24%) or uterine (19%) sarcomas. In this Phase II population (> or = 25% negative prognostic or predictive factors of response to chemotherapy; > or = 50% anthracycline- and ifosfamide-resistant), safety data were comparable to those obtained in the Phase I and compassionate-use studies. Asymptomatic and reversible neutropenia and transaminitis (grade 3/4) were the most frequent toxicities (approximately 60% of patients); febrile neutropenia was infrequent (< 10%). No mucositis, alopecia, cardiac or neurotoxicity was observed. Two severe cases of rhabdomyolysis occurred. Side effects were non-cumulative, reversible and manageable. Of 52 evaluable patients, three (6%) achieved a long-lasting (8-13 months) partial response, four (8%) achieved a minor response (25-50% tumor reduction) and 22 (42%) achieved disease stabilization. With a 13-month median follow-up, median survival was almost 11 months. Progression-free survival at 6 months was 26.5% and the overall survival rate at 12 months was almost 50%. The response rate was uninfluenced by tumor metastatic site, size or anthracycline sensitivity status. These results, combined with the lack of cumulative toxicity, confirm the role of ET-743 in the treatment of advanced STS.

Antineoplastic Agents, Alkylating↗

[Treatment and prognosis of soft tissue sarcoma].

The treatment and prognosis of 121 patients with primary and locally recurrent soft tissue sarcomas of the extremities and trunk were analyzed. The most common histologic type was malignant fibrous histocytoma (32 patients, 26.4%). Ninety-three patients had high grade sarcomas (76.9%) and 28 had low grade sarcomas (23.1%). Adjuvant radiotherapy and chemotherapy were applied to 37 and 41 patients respectively. In 28 patients who had local recurrence after surgery, the 5-year local control rate was 72.3%. The overall 5-year survival rate was 59.9% for all patients, and 59.1% for 96 patients with locally recurrent lesions. The independent risk factors affecting the overall survival included high grade, tumor of upper extremity, perioperative blood transfusion, and tumor size of > or = 10 cm. We conclude that (1) wide excision is the most accepted operative procedure for treatment of soft tissue sarcoma of the extremity and trunk unless the lesions are locally advanced (In the advanced cases amputation should be performed); (2) in patients with local recurrence only, there is an almost same rate of long-term salvage as that in patients with primary tumors; (3) avoiding unnecessary perioperative blood transfusion may improve survival; and (4) further investigation of more effective regimen of adjuvant radiotherapy and chemotherapy needs to be taken.

Adolescent↗

Sarcoma arising from interdigitating cells. Cytology and cytochemistry.

The cytologic features of a highly malignant sarcomatous tumor in a 37-year-old male, arising from interdigitating cells and localized in the mediastinum, lymph nodes and skin, are described. Cytologically this sarcoma was characterized by large cells with ill-defined, faintly basophilic cytoplasm, monocytoid or multilobulated nuclei and a reticular chromatin structure; very prominent nucleoli were seen in some of the cells. Some of the tumor cells were spindle shaped. The ultrastructurally characteristic invaginations of the cell membrane were not obvious in the cytologic smear, although the nuclear membrane showed deep, narrow, channel-like indentations. The specific ultrastructural, immunologic and cytochemical characteristics of the interdigitating cells were recognized in the tumor cells. Adenosine triphosphatase was present in the tumor cells in large amounts, while acid phosphatase, acid alpha-naphthyl acetate esterase and other enzymes were absent. The described tumor must be considered another tumor of the mononuclear phagocyte system; the proposed name is "interdigitating-cell sarcoma."

Acid Phosphatase↗