Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0019829 (Hodgkin's disease)
 30,247 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

The purpose of the study was to evaluate the incidence of second malignancies in childhood Hodgkin disease treated with COPP and COPP/ABV (replacing mechlorethamine by cyclophosphamide) chemotherapy. In 212 children with Hodgkin disease who were under 14 years of age and treated at Cancer Institute during the 25-year period of 1970-1994, the occurrence of second malignant neoplasms was analyzed as on 31 December 1999. Eighty-two percent attained complete response. The 5- and 10-year overall survival rate was 91 and 83%, respectively. In this interm report 5 cases of second malignancies were documented. All 5 were solid tumors: one each of soft tissue sarcoma, dermatofibrosarcoma, micropapillary carcinoma of thyroid, malignant phylloides tumor of breast, and chondrosarcoma of ilium. All patients had received combination chemotherapy and radiotherapy. Interestingly, all were splenectomized. All these patients had advanced stage of cancer and were 7-14 years of age at the time of diagnosis of first primary Hodgkin disease. It is significant that there were no secondary hematological malignancies. COPP and COPP/ABV are effective therapeutic regimens. The paucity of secondary hematological malignancies is unique in this series and may be attributed to the substitution of nitrogen mustard with cyclophosphamide in the chemotherapy combination. This is an initial observation, and further follow-up is needed for a firm conclusion.
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PMID:Paucity of hematological neoplasia after treatment of Hodgkin disease: observation after long-term follow-up at Cancer Institute, Chennai, south India. 1193 33

Primary sarcomas of the sternum are infrequent; the most common histological types are chondrosarcoma, plasmacytoma, Ewing's sarcoma and Hodgkin's tumors. Since osteosarcoma of the sternum is a very rare entity, we have thought to explain the principal characteristics and the clinical and therapeutic approach based on our experience of two cases.
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PMID:Osteosarcoma of the sternum: two case reports. 1208 58

Pulmonary surgery is frequently used for the treatment of metastasis or nodules in children with various types of malignancies. However, the indications and effectiveness of pulmonary metastatectomy have not been evaluated recently. Therefore, a retrospective study was conducted to analyse the results of pulmonary metastatectomy in children. Children who underwent pulmonary metastatectomy at our department between 1990 and 2000 were reviewed. Eighteen children consisting of 11 boys and 7 girls (age range, 3 to 18 years) underwent thoracotomy for pulmonary metastasis excision. The primaries were osteosarcoma (n = 2), synovial sarcoma (n = 1), fibrosarcoma (n = 1), Ewing's sarcoma (n = 2), mesenchymal chondrosarcoma (n = 1), Wilms' tumour (n = 4), clear-cell sarcoma (n = 1), Hodgkin lymphoma (n = 3), hepatoblastoma (n = 1), hepatocellular carcinoma (n = 1) and haemangioendotheliosarcoma (n = 1). Pulmonary metastases were encountered either at the time of initial diagnosis (22 %) or occurred within 6 months to 5 years. They were frequently nodular (94 %), unilateral (94 %) and located in the right lung (70 %). The number of metastases were frequently one (56 %) or two (28 %). Excision was done by means of wedge resection (88 %), segmentectomy (6 %), and lobectomy + wedge resection (6 %). The nodules contained tumour cells in most cases (n = 14) (78 %), mature nephrogenic elements (6 %) and no tumour tissue (16 %) in the remaining cases. Histology was similar to that of the original tumour in 12 cases. However, synovial sarcoma was encountered in metastasis in one case with fibrosarcoma primary. Re-thoracotomy was performed in 22 % of cases for the recurrent lesion, which in only one case was a true local recurrence. Overall disease-free survival rate was 56 % during the follow-up period (mean, 36.4 +/- 31.8 months). Pulmonary metastatectomy may increase survival in carefully selected children, though it is unlikely to cure the patient. Therefore combined therapies such as chemotherapy and/or radiotherapy should be continued in the postoperative period.
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PMID:Pulmonary metastases in children: an analysis of surgical spectrum. 1210 95

A multicentre analysis was carried out on bone tumours in Cameroon during a 10-year period. Registers and patient records of five pathology laboratories were consulted, and all patients with a histological report of a bone tumour were included in the study. A total of 268 bone tumours were studied and the average incidence was 27 tumours a year, or two per one million inhabitants. Of these tumours 48% were benign, 45% were primary bone cancers and only 6% were metastatic disease. Among the primary malignant bone tumours, osteosarcoma was the most frequent (39%), followed by non-Hodgkin's primary bone lymphoma, fibrosarcoma, chondrosarcoma, and Ewing's sarcoma. Primary site of the metastatic bone tumours was prostatic adenocarcinoma, breast cancer, hepatocarcinoma and thyroid cancer. In Cameroon many bone tumours are not diagnosed due to lack of medical facilities and little awareness among our medical staff. It is likely that the real incidence is at least ten times higher than that shown in our report.
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PMID:Bone tumours in Cameroon: incidence, demography and histopathology. 1294 93

In two patients, men aged 39 and 66 years, a sternal mass in combination with pain developed. One patient was diagnosed with a non-Hodgkin lymphoma located in the sternum and the other one with a primary chondrosarcoma of the sternum. They both recovered after treatment. The differential diagnosis of disorders of the chest wall is troublesome and includes haematologic, rheumatologic and infectious processes. Tietze's syndrome is a rare cause of pain and non-suppurative swelling of the costosternal joints. However, tumours of the anterior chest wall can also cause these symptoms and these must therefore be excluded if the complaints persist or the swelling progresses. The most common malignant tumours of the chest wall are non-Hodgkin lymphoma, primary chondrosarcoma and metastases. Diagnostics should consist of blood tests and X-rays. CT and MRI scans are more helpful in establishing the diagnosis. A definitive diagnosis can only be determined by biopsy.
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PMID:[Sternal pain: not always harmless]. 1563 92

Treatment of Hodgkin disease (HD) with chemoradiotherapy in children is associated with increased risk for developing secondary neoplasms. Parathyroid adenoma (PTA) and chondrosarcoma (CS) are quite rare types of secondary neoplasms after HD. We describe a 5-year-old boy with stage IV HD, successfully treated with MOPP (mechlorethamine, vincristine, procarbazine, and prednisone)/ABVD (doxorubicin, bleomycin, vinblastine, and dacarbazine) chemotherapy followed by 35 Gy mantle radiotherapy who developed primary hyperparathyroidism because of benign PTA at the age of 20 years, and died of CS in thoracic vertebrae at the age of 22 years. Consecutive occurrence of PTA and CS after treatment of pediatric HD, to the best of our knowledge, has not been reported earlier.
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PMID:Parathyroid adenoma and chondrosarcoma after treatment of pediatric Hodgkin disease. 2073 43

Primary malignant tumours of the clavicle are extremely rare and little is known regarding their clinicopathological characteristics and outcomes of surgical management. The aim of the study is to analyse the clinical, imaging, and histological features of six patients with malignant tumours of the clavicle and present the outcome of cleidectomy in four of them. A review of the literature is also provided. Six cases were included in this series: two plasmocytomas; three PNETs, one non-Hodgkin lymphoma, one high-grade chondrosarcoma and one post-irradiation fibrosarcoma. Apart from one patient with plasmocytoma and another one with non-Hodgkin lymphoma, the remaining four patients underwent partial or complete cleidectomy according to tumour location. At the time of latest follow-up all patients were alive. Neither local recurrence nor metastases were observed in patients that underwent cleidectomy. In this group, the average score was 86.6% of the expected normal function according to the Musculoskeletal Tumour Society (MSTS) evaluation form. The mean Constant-Murley score of the affected side was 80. Patients after cleidectomy were pain free, they had almost full shoulder range of motion and no significant functional deficit was reported. Primary malignant clavicular tumours may be easily undiagnosed due to their insidious clinical onset. Partial or total cleidectomy is associated with adequate shoulder mobility and mild functional deficit. Therefore, the extent of clavicle excision during tumour removal does not seem to determine the functional outcome of the affected shoulder.
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PMID:Primary malignant clavicular tumours: a clinicopathological analysis of six cases and evaluation of surgical management. 2118 96

Small round cell tumors (SRCTs) are a heterogeneous group of neoplasms composed of small, primitive, and undifferentiated cells sharing similar histology under light microscopy. SRCTs include Ewing sarcoma/peripheral neuroectodermal tumor family tumors, neuroblastoma, desmoplastic SRCT, rhabdomyosarcoma, poorly differentiated round cell synovial sarcoma, mesenchymal chondrosarcoma, small cell osteosarcoma, small cell malignant peripheral nerve sheath tumor, and small cell schwannoma. Non-Hodgkin's malignant lymphoma, myeloid sarcoma, malignant melanoma, and gastrointestinal stromal tumor may also present as SRCT. The current shift towards immunohistochemistry and cytogenetic molecular techniques for SRCT may be inappropriate because of antigenic overlapping or inconclusive molecular results due to the lack of differentiation of primitive cells and unavailable genetic service or limited moleculocytogenetic experience. Although usage has declined, electron microscopy (EM) remains very useful and shows salient features for the diagnosis of SRCTs. Although EM is not always required, it provides reliability and validity in the diagnosis of SRCT. Here, the ultrastructural characteristics of SRCTs are reviewed and we suggest that EM would be utilized as one of the reliable modalities for the diagnosis of undifferentiated and poorly differentiated SRCTs.
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PMID:Utility of transmission electron microscopy in small round cell tumors. 2581 30

CD30 is a member of the tumor necrosis factor receptor superfamily, member 8 (TNFRSF8), and its normal expression is restricted to activated T and B cells. In tumor cells, CD30 expression is most commonly associated with lymphoid malignancies (Hodgkin and non-Hodgkin lymphomas) and is a therapeutic target using anti-CD30 antibody. CD30 expression has been reported also in mostly adult non-lymphoid malignancies, raising the possibility of CD30-targeted therapy for additional tumors. In this study, we examined the incidence of CD30 expression in 251 hematopoietic and 334 non-hematopoietic cases of pediatric tumors. As expected, strong and membranous CD30 staining was seen in anaplastic large cell lymphoma, classical Hodgkin lymphoma, and embryonal carcinoma while variable staining was seen in diffuse large B cell lymphoma. In addition, positive CD30 staining was also seen in cases of neuroblastoma (33 of 56), neoplasm with chondroid differentiation (8 of 25), myeloid neoplasms (11 of 120), hemangioma (2 of 12), and mature teratoma (1 of 11). In neuroblastoma, the CD30 expression was generally restricted to cells with ganglion differentiation; staining of ganglion cells was also seen in the one positive case of mature teratoma. In neoplasm with chondroid differentiation, the positive cases were chondrosarcoma (3 of 5), chondroblastic osteosarcoma (2 of 10), and chondroblastoma (3 of 7). In acute myeloid leukemia, the CD30 positive cases were more common in AML with monocytic differentiation but did not correlate with any specific molecular change. We conclude that CD30 expression in pediatric tumors is more general than anticipated and future studies are warranted to understand the biologic and therapeutic significances.
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PMID:CD30 Expression in Pediatric Neoplasms, Study of 585 Cases. 2852 33


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