Radiotherapy in Ewing's sarcoma and PNET of the chest wall: results of the trials CESS 81, CESS 86 and EICESS 92

PURPOSE: Treatment results and the pattern of relapse were evaluated in the multimodal treatment of Ewing's sarcomas of the chest wall. METHODS AND MATERIALS: In a retrospective analysis, 114 patients with non-metastatic Ewing's sarcoma of the chest wall were evaluated. They were treated in the CESS 81, CESS 86, or EICESS 92 studies between January 1981 and December 1993. The treatment consisted of polychemotherapy (VACA, VAIA, or EVAIA) and local therapy, either surgery alone (14 patients), radiotherapy alone (28 patients) or a combination of both (71 patients). The median follow-up was 46.6 months (range 5-170). A relapse analysis for all patients with local or combined relapses was performed. RESULTS: Overall survival was 60% after 5 years, event-free survival was 50%. Thirty-seven patients had a systemic relapse (32.4%), 11 patients had a local relapse alone (9.6%), and 3 patients had a combined local and systemic relapse (2.6%). The risk to relapse locally after 5 years was 0% after surgery alone, 19% after radiation alone, and 19% after postoperative irradiation. None of the 8 patients with preoperative irradiation have failed locally so far. With the introduction of central radiotherapy planning in CESS 86, local control of irradiated patients improved. Ten of 14 patients with local failure could be evaluated in the relapse analysis: 3 patients had an in-field relapse, 4 patients had a marginal relapse, 2 patients had a relapse outside the radiation fields, and 1 patient failed with pleural dissemination. Six treatment deviations were observed. CONCLUSION: Local control was best after surgery alone in a positively selected group of patients. Local control after radiation or combined radiation and surgery was good. With diligent performance of radiotherapy, it will be possible to further improve the results in the radiotherapy group.     

Tuberculosis of the chest wall: CT findings

To assess the CT features of tuberculosis of the chest wall, the CT findings in four patients with documented tuberculous chest wall infection were reviewed. Two patients were Caucasian and two were of Chinese origin. All had normal immune status. In two cases tuberculosis involved the ribs, in one the costal cartilage, and in one the sternoclavicular joint. Computed tomography demonstrated osseous and cartilaginous destruction (in four), soft tissue masses with calcification (in two), and rim enhancement following intravenous contrast medium administration (in two). Underlying pleuroparenchymal tuberculosis was present in two cases. Tuberculosis of the chest wall is characterized by bone or costal cartilage destruction and soft tissue masses that may demonstrate calcification or rim enhancement with or without evidence of underlying lung or pleural disease.     

Fetal dose estimates for electron-beam treatment to the chest wall of a pregnant patient

The purpose of this report is to discuss dosimetry and shielding for electron-beam radiotherapy of pregnant patients. Specifically, we have determined fetal dose for a pregnant patient considering electron radiotherapy for a chest wall recurrence of breast cancer. The treatment was simulated using an anthropomorphic phantom, and the measured dose to the unshielded fetus for this plan was 5.3 cGy, a level at which risk to the fetus is uncertain. Therefore abdominal shielding, consisting of 6.6 cm of lead, was used to reduce the dose to the fetus to less than 1.5 cGy, a level considered to be of little risk. We further found that using the lower (instead of upper) variable trimmer bars to define the field edge closest to the fetus resulted in approximately 30% lower dose to the fetus. These results show that it is possible to reduce fetal dose to acceptable limits in electron-beam radiotherapy of the chest wall using the general principles recommended for photon-beam radiotherapy.     

Horner's syndrome associated with a solitary bone plasmacytoma of the first rib

A case of solitary plasmacytoma originated in the left first rib in a 45-year-male is presented. The initial symptomatology was a Horner's syndrome. A chest X-ray, gammagraphy, TAC and arteriography showed a tumor shadow in the left chest wall; no evidence of plasmacytoma after clinical study found use where. The treatment was surgical excision and postsurgical radiotherapy. The histological investigation revealed a plasmacytoma of the rib. The patient have been followed for two years. The patient is asymptomatic, without evidence of local recurrence and systemic spread of the disease two years after treatment.     

Cartilaginous disorders of the chest

Cartilaginous disorders of the thorax can arise in the parenchyma, airways, chest wall, and axial skeleton. At radiography, pulmonary hamartoma is characterized by "popcorn" calcification or fat density, either of which is diagnostic. Bronchiectasis is best demonstrated at high-resolution computed tomography (CT) and has a "tramline" or "signet ring" appearance. Tracheopathia osteochondroplastica appears at CT as multiple sessile submucosal nodules with or without calcification along the cartilaginous portion of the trachea. In relapsing polychondritis, the trachea and mainstem bronchi have diffuse or focal thickening with luminal narrowing at radiography. Costochondritis of the chest wall has become more prevalent with increased intravenous drug abuse and may be demonstrated at CT as soft-tissue swelling along with underlying cartilaginous fragmentation and bone destruction. Enchondromas are expansile and may display a calcified cartilaginous matrix at radiography. In osteochondroma, the thickness of the cartilaginous cap determines the likelihood of malignant degeneration. At radiography, chondroblastomas have a round contour, sharp margins, and cortical scalloping, whereas chondrosarcomas are large masses with indistinct margins, cortical breakthrough, and soft-tissue extension. By identifying either a process affecting a cartilage-containing structure or a cartilaginous matrix within a lesion, the chest radiologist may be able to narrow the list of differential diagnostic possibilities substantially.     

Twin pregnancy with one fetus in a rudimentary horn: a case report of a surviving twin

We report herein the case of a 30-year-old man who developed a primary leiomyosarcoma (LMS) 11 years after undergoing a median sternotomy for mediastinal seminoma followed by 50 Gy radiotherapy. He was given two courses of chemotherapy, resulting in 90% tumor regression, after which resection of the tumor with adjacent chest wall structures was carried out. Reconstruction was performed using a methylmethacrylate prosthesis prepared preoperatively. Postoperatively, he received two additional courses of chemotherapy and has had no sign of recurrence for 45 months.     

The existence of "Nederwiet", a new fact in the history of cannabis

Our purpose was to examine the role of radiotherapy in the management of phyllodes tumor of the breast. Eight patients were treated with adjuvant radiotherapy for nonmetastatic phyllodes tumor of the breast at the M.D. Anderson Cancer Center between December 1988-August 1993. Tumors were classified as benign (n=2), borderline (indeterminate; n=1), or malignant (n=5). Median follow-up was 36.5 months. Primary surgery consisted of either lumpectomy in 2 patients or mastectomy in 6 patients. Seven patients received adjuvant radiation therapy to the breast or chest wall to a dose of 60 Gy. One patient received 50 Gy to the breast, followed by an interstitial boost of 20 Gy for a total of 70 Gy. Radiotherapy was administered for a combination of reasons, including bulky tumor volume, positive margins, recurrence, and/or malignant histology. There were no local or distant failures. This retrospective review suggests that adjuvant radiotherapy may be underutilized in the treatment of phyllodes tumor of the breast, particularly in patients with adverse features. Although treatment to the breast or chest wall (not the lymphatics) to a dose of 60 Gy appears effective, a dose-response has not been established, and lower doses (50-60 Gy) may be equally effective.     

Axillary lymph node metastases from bronchogenic carcinoma

BACKGROUND: Axillary lymph node metastases (ALNMs) from bronchogenic carcinoma are rare and their significance may be questioned. A surgical approach may allow a better understanding of the mechanism of their occurrence. METHODS: A retrospective study of 1,486 cases of surgically removed non-small cell lung carcinoma was performed. Twenty-two patients (1.5%) had extrathoracic nodal metastases. Nine of them were ALNMs (<1%). These cases form the basis of this study. RESULTS: In 1 patient ipsilateral ALNM was removed during a lung operation. It was a left non-small cell lung carcinoma invading the chest wall (T3 N2). In the other patients (n = 8) ALNMs were observed during postoperative follow-up; 4 underwent ALNM resection, 2 had radiotherapy, and 2 had symptomatic treatment only. For these 8 patients, in the TNM classification performed after an initial bronchogenic carcinoma operation, the lymph node status was, respectively, N0 in four cases, N1 in three cases, and N2 in one case. Survival ranged from 1 to 10 months, except for one patient who is still alive after more than 5 years. In this case, the ALNM was discovered 4 months after a right lower lobectomy for a T2 N0 adenocarcinoma. CONCLUSIONS: Axillary lymph node metastases may be involved through direct chest wall invasion of bronchogenic carcinoma or retrograde spread from supraclavicular lymphnode block. However, another mechanism seems to be the systemic vascular route.     

Postradiation sarcoma of bone: review of 78 Mayo Clinic cases

Postradiation sarcoma of bone is an uncommon but serious sequela of radiation therapy. Seventy-eight Mayo Clinic patients have been treated for sarcomas arising in irradiated bones. They received their initial radiotherapy for a wide variety of nonneoplastic and neoplastic conditions, both benign and malignant. Thirty-five sarcomas arose in bone that was normal at the time of radiotherapy, and 43 arose in irradiated preexisting osseous lesions. The latent period between radiotherapy and diagnosis of sarcoma averaged 14.3 years. Ninety percent of the postradiation sarcomas were either osteosarcomas or fibrosarcomas; chondrosarcoma, malignant (fibrous) histiocytoma, malignant lymphoma, Ewing's tumor, and metastasizing chondroblastoma also occurred. Prompt radical surgery, when feasible, is usually the treatment of choice for the sarcoma. About 30% of patients with sarcomas of the extremities or craniofacial bones survived 5 years without recurrence; there were no disease-free survivors among patients with tumors of the vertebral column, pelvis, or shoulder girdle. The low risk of sarcoma following radiotherapy for the treatment of cancer should not be a contraindication to its use in these patients; however, radiation therapy for benign bone tumors should be reserved for lesions that are not amenable to surgical treatment. An unusual case is also reported herein in which a fibrosarcoma was discovered in the humerus of a patient who had received radiotherapy 55 years previously for a verified osteosarcoma in the same site.     

Informative value of Hemoccult test according to the number of positive slides in mass screening of colorectal cancer]

Desmoid tumors develop in the muscle or aponeurosis, but rarely in the chest wall. We report a patient with desmoid tumor in the chest wall. A 30-year-old female visited our hospital due to an abnormal shadow in the chest detected at a health examination. No definite diagnosis could be made by chest CT, MRI, or Ga scintigraphy, and finally a biopsy demonstrated desmoid tumor. The left anterior chest and posterolateral chest (lung apex) were opened, and tumors were resected together with the chest wall. No radiotherapy was performed. No recurrence has been observed for 1.5 years after operation. This tumor does not yield distant metastasis, but its recurrence rate is very high. Therefore, we intend to follow this patient for a long period.     

Adjuvant radiotherapy and chemotherapy in node-positive premenopausal women with breast cancer

BACKGROUND: Radiotherapy after mastectomy to treat early breast cancer has been known since the 1940s to reduce rates of local relapse. However, the routine use of postoperative radiotherapy began to decline in the 1980s because it failed to improve overall survival. We prospectively tested the efficacy of combining radiotherapy with chemotherapy. METHODS: From 1978 through 1986, 318 premenopausal women with node-positive breast cancer were randomly assigned, after modified radical mastectomy, to receive chemotherapy plus radiotherapy or chemotherapy alone. Radiotherapy was given to the chest wall and locoregional lymph nodes between the fourth and fifth cycles of cyclophosphamide, methotrexate, and fluorouracil. RESULTS: After 15 years of follow-up, the women assigned to chemotherapy plus radiotherapy had a 33 percent reduction in the rate of recurrence (relative risk, 0.67; 95 percent confidence interval, 0.50 to 0.90) and a 29 percent reduction in mortality from breast cancer (relative risk, 0.71; 95 percent confidence interval, 0.51 to 0.99), as compared with the women treated with chemotherapy alone. CONCLUSIONS: Radiotherapy combined with chemotherapy after modified radical mastectomy decreases rates of locoregional and systemic relapse and reduces mortality from breast cancer.     

A 6p22 reference map of leukocyte DNA: exclusion of rearrangement in four cases of atypical haemochromatosis

Primary angiosarcomas of the chest wall and pleura are extremely rare and carry a dismal prognosis. Two cases are reported. One patient (case 1), presented with massive recurrent haemothorax, was found to have multifocal angiosarcoma of the pleura, treated with surgical de-bulking, chemical pleurodesis and chemotherapy, achieving control of the bleeding. She died 10 months later from complications related to chemotherapy. A full post-mortem examination confirmed this was a primary pleural angiosarcoma with no evidence of disease elsewhere. Another patient (case 2) with a large solitary angiosarcoma of the chest wall, discovered incidentally on a routine physical examination, was successfully treated with surgical excision and subsequent radical radiotherapy, remaining well 15 years post-operatively.     

The epidemiology of Langerhans cell histiocytosis

PURPOSE: During recent years, more intensified systemic and local treatment regimens have increased the 5-year survival figures in localized Ewing's sarcoma to more than 60%. There is, however, concern about the risk of second malignancies (SM) in long-term survivors. We have analyzed the second malignancies in patients treated in the German Ewing's Sarcoma Studies CESS 81 and CESS 86. MATERIALS AND METHODS: From January 1981 through June 1991, 674 patients were registered in the two sequential multicentric Ewing's sarcoma trials CESS 81 (recruitment period 1981-1985) and CESS 86 (1986-1991). The systemic treatment in both studies consisted of a four-drug-regimen (VACA = vincristine, actinomycin D, cyclophosphamide, and adriamycin; or VAIA = vincristine, actinomycin D, ifosfamide, and adriamycin) and a total number of four courses, each lasting nine weeks, was recommended by the protocol. Local therapy in curative patients was either complete surgery (n = 162), surgery plus postoperative radiotherapy with 36-46Gy (n = 274), or definitive radiotherapy with 46-60Gy (n = 212). The median follow-up at the time of this analysis was 5.1 years, the maximum follow-up 16.5 years. RESULTS: The overall survival of all patients including metastatic patients was 55% after 5 years, 48% after 10 years, and 37% after 15 years. Eight out of 674 patients (1.2%) developed a SM. Five of these were acute myelogenic leukemias (n = 4) or MDS (n = 1), and three were sarcomas. The interval between diagnosis of Ewing's sarcoma and the diagnosis of the SM was 17-78 months for the four AMLs, 96 months for the MDS and 82-136 months for the three sarcomas. The cumulative risk of an SM was 0.7% after 5 years, 2.9% after 10 years, and 4.7% after 15 years. Out of five patients with AML/MDS, three died of rapid AML-progression, and two are living with disease. Local therapy (surgery vs. surgery plus postoperative irradiation vs. definitive radiotherapy) had no impact on the frequency of AML/MDS, but local therapy did influence the risk of secondary sarcomas. All three patients with secondary sarcomas had received radiotherapy; however, all three sarcomas were salvaged by subsequent treatment and are in clinical remission with a follow-up of 1 month, 4.3 years, and 7.5 years after the diagnosis of the secondary sarcoma. Thus far, SM contributed to less than 1 % (3/328) of all deaths in the CESS-studies. CONCLUSIONS: The risk of leukemia after treatment for Ewing's sarcoma is probably in the range of 2%. The risk of solid tumors also seems to be low within the first 10 years after treatment and remains in the range of 5 % after 15 years. In the CESS-studies, less than 1% of all deaths within the first 10 years after diagnosis were caused by SM. Effective salvage therapy for secondary sarcomas is feasible.     

Is the chest radiograph a reliable tool in the assessment of tumor response after radiotherapy in nonsmall cell lung carcinoma?

BACKGROUND: The purpose of this study was to evaluate whether the chest radiograph is a reliable tool to assess response to radiotherapy. MATERIALS AND METHODS: Pre- and post-treatment chest radiographs and computed tomographs (CT) of 63 patients with nonsmall cell lung cancer (NSCLC) treated by radiotherapy were reviewed by four observers with regard to suitability for tumor measurement, and response. Suitability for tumor measurement was expressed as the number of measurable diameters. In addition, the consequences to clinical outcome were studied by survival analysis. RESULTS: The CT turned out to be more suited for tumor measurement before as well as after radiotherapy, resulting in an increase of the number of measurable cases. The number of measurable cases with CT was 52 (83%) as compared to 28 (44%) with chest radiography. Especially in case of centrally localized tumors, the presence of an atelectasis, or squamous cell carcinoma, CT contributed to a higher rate of measurable cases. The interobserver agreement with regard to response using chest radiograph was good (mean kappa = 0.74). In 25 of 28 cases (89%) measurable with CT as well as with chest radiograph, response was equally classified. When CT was used, the median survival of the responders was 14.2 months as compared to 6.8 months of the nonresponders. When chest radiograph was used, the median survival of these groups was 12.0 and 6.6 months respectively, which was not significantly different when response was assessed by CT. CONCLUSION: We conclude that CT is more suited for tumor measurement because more measurable lesions can be found and more evaluable lesions on chest radiograph become measurable on CT. The chest radiograph does have a valuable role to play in those lesions that are measurable because of the good interobserver agreement with regard to the response classification, the high overall agreement between CT and chest radiograph in case of measurable cases, and the lack of important differences with regard to survival.     

Surgery of soft tissue sarcoma (with the exception of the retroperitoneum)

The place of surgery in the treatment of soft-tissue sarcoma is defined in the light of a review of the recent litterature. Usual treatment combines conservative surgery and radiotherapy. The essential risk factor of local recurrence is the quality of surgical resection, defined by the definitive resection margins. The addition of radiotherapy after inadequate surgery can improve local control, but cannot ensure that obtained after adequate surgery. Some limited tumours can be treated by surgery alone, but radiotherapy remains essential in more advanced tumours. Under these conditions, local recurrence rates after surgery alone, surgery and systematic radiotherapy and surgery with radiotherapy, as required are 27%, 28% and 30% respectively. The development of a local recurrence appears to affect survival in tumour with a good initial prognosis, early recurrence determining unfavourable outcome. Serious postoperative morbidity is observed in 14% of the cases and is responsible for delayed treatment and functional disorders. The use of muscle flaps to fill the surgical defects can reduce these complications. Evaluation of the functional results must be based on predefined objective criteria.     

Bronchopulmonary Kaposi's sarcoma in 106 HIV-1 infected patients

The objectives of this study were to describe the clinical and radiological features at presentation, and the natural history of HIV-related bronchopulmonary Kaposi's sarcoma. A retrospective review of medical records and chest radiographs was performed in 106 HIV-infected homosexual men with bronchopulmonary Kaposi's sarcoma diagnosed at bronchoscopy between September 1988 and November 1994. The majority of patients had evidence of advanced HIV disease at diagnosis (median CD4 cell count was 15 x 10(6)/l, range 0-288), and 93% had had a diagnosis of cutaneous Kaposi's sarcoma for a median duration of 11 months prior to diagnosis of their bronchopulmonary disease. The most frequent symptoms at presentation were cough (92%), dyspnoea (69%), pleuritic pain (20%), haemoptysis (13%) and wheezing (10%). The most common radiological finding in 73% of our series was of poorly defined and confluent opacities, with predominant middle and lower zone involvement. Median survival was 4 months (range 0-37 months) from diagnosis and 9 months (range 1-25) from the onset of symptoms. Treatment with either chemotherapy or radiotherapy was associated with a significantly reduced risk of death (hazards ratio (HR)=0.48, 95% CI=0.26-0.87). Factors associated with a poor survival, after adjustment for treatment effect were older age (HR=1.79, 95% CI=1.22-2.84) for each 10-year increase in age; a history of pleuritic pain (HR=2.97, 95% CI=1.39-6.32); presence of pleural effusion on X-ray (HR=2.01, 95% CI=1.13-3.59) and a prior diagnosis of cutaneous Kaposi's sarcoma (HR=1.8, 95% CI=1.00, 3.24). Bronchopulmonary Kaposi's sarcoma occurs mainly in patients with advanced HIV disease and a prior history of cutaneous disease. Survival is poor, and adverse prognostic factors include older age at diagnosis and the presence of pleural disease.     

Radiogenic sarcoma of the thyroid (author's transl)

A sarcoma in the region of the thyroid, observed 30 years after radiotherapy in a patient with syringomyelia is presented. Even after low dose radiation in childhood and adolescence tumours of the thyroid frequently can be diagnosed. They rarely occur in adults, because apparently of a long latent period. This case is thought not to be a specific thyroid tumour.     

The type of local treatment conditions the prognosis in patients with nonmetastatic Ewing's sarcoma of the extremities treated with adjuvant chemotherapy

The results obtained in 172 cases of non metastatic Ewing's sarcoma of the extremities are reported. The patients were advised to undergo surgical treatment, followed by radiotherapy (40-45 Gy) in case of inadequate surgical margins. 48 patients who refused surgical treatment, were locally treated with radiotherapy alone (50-65 Gy). With a mean follow-up of 8 years (R. 3-15) 101 patients (58.7%) are free of disease and 68 relapsed with metastases and/or local recurrence. A radio-induced bone sarcoma developed in two patients, one patient died of ADM cardiomyopathy. No differences in terms of risk factors were observed between patients who were or were not treated with surgery. A better DFS was observed in the patients treated with surgery (66.9%) in comparison with those treated with radiotherapy alone. The higher percentage of local recurrences observed in patients treated with radiotherapy alone seems to be responsible for the worse prognosis observed in these patients. The authors' conclusion is that the local control in patients with non metastatic Ewing's sarcoma should always be achieved by means of surgery.     

Alloplastic sternal replacement in malignant sternal tumors. Case report and review of the literature

Although not seldom as a palliative procedure, the preferred treatment of locally recurrent breast cancer or chest wall involvement by metastases is full-thickness chest wall resection. For closure and coverage of the defect various techniques are described. Autoplastic reconstruction is indicated for smaller defects, while larger defects usually require alloplastic materials, especially in case of chest instability after resection. We report the case of a 55-year-old female who developed a locally recurrent breast cancer with infiltration of the sternum 4 years after left sided ablation. En bloc resection of the chest wall including the complete sternum was followed by replacement with a computer-aided custom made polyethylene sternal prosthesis. With this procedure we stabilized the chest wall with protection of the underlying organs, avoided prolonged postoperative ventilation and achieved a satisfying cosmetic result.     

The toxicity of radiotherapy following high-dose chemotherapy with peripheral blood stem cell support in high-risk breast cancer: a preliminary analysis

High-dose chemotherapy with autologous bone marrow and/or peripheral blood stem cell (PBSC) support is increasingly employed in the adjuvant treatment of high-risk breast cancer. Subsequent radiotherapy has been reported to be associated with morbidity and mortality resulting from pulmonary toxicity. In addition, the course of radiation therapy may be hampered by excess myelosuppression. The aim of this study was to investigate the contribution to radiation-induced toxicity of a high-dose chemotherapy regimen (CTC) that incorporates cyclophosphamide, thiotepa and carboplatin, in patients with high-risk breast cancer. In two randomised single institution studies, 70 consecutive patients received anthracycline-containing adjuvant chemotherapy (FEC: 5-fluorouracil, epirubicin and cyclophosphamide) followed by radiotherapy to achieve maximal local control. Of these patients, 34 received high-dose CTC with autologous PBSC support. All patients tolerated the full radiation dose in the planned time schedule. Radiation pneumonitis was observed in 5 patients (7%), 4 of whom had undergone high-dose chemotherapy (P = 0.38). All 5 responded favourably to prednisone. Fatal toxicities were not observed. Myelosuppression did not require interruption or untimely discontinuation of the radiotherapy, although significant reductions in median nadir platelet counts and haemoglobin levels were observed in patients who had received high-dose chemotherapy (P = 0.0001). The median nadir of WBC counts was mildly but significantly decreased during radiotherapy (P = 0.01). Red blood cell or platelet transfusions were rarely indicated. Adequate radiotherapy for breast cancer can be safely administered after high-dose CTC with autologous PBSC support. Radiation-induced myelotoxicity is clearly enhanced following CTC, but this is of little clinical significance. Radiation pneumonitis after high-dose therapy may occur more often in patients with a history of lung disease or after a relatively high radiation dose to the chest wall. Other high-dose regimens, particularly those incorporating drugs with known pulmonary toxicity (such as BCNU), may predispose patients to radiation pneumonitis.