Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0677930 (primary tumor)
 20,210 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Malignant germ cell tumors account for about 3% of neoplasms in children, and endodermal sinus tumor (EST) is the most common histological subtype. The authors reviewed 22 years' experience (at their institution) in the management of 37 patients with this tumor. Fifteen of them (41%) had a sacrococcygeal primary, 10 had a testicular tumor (27%), 6 had an ovarian tumor (16%), 3 had a vaginal tumor (8%) and 3 had tumors at other sites (8%). Seven (19%) patients presented with metastatic disease, primarily pulmonary. The serum alpha-fetoprotein (AFP) level was elevated in all cases tested. The initial chemotherapy regimen included vincristine, actinomycin, cyclophosphamide (VAC), and Adriamycin (6 patients), but since 1985 the regimen has been changed to include cisplatin or Carboplatin, etoposide, and bleomycin (21 patients). Eight patients with testicular tumors initially were treated with surgical excision alone. Computed tomography results were a poor predictor of recurrence, but AFP surveillance was extremely sensitive. No second-look operation detected residual tumor in the absence of AFP elevation. Initial relapse for all patients tended to occur early (within 2 years), locally, and often with pulmonary metastases. Although historically the prognosis for patients with EST has been poor, the overall 2-year survival rate in this series was 70%. The best prognosis was among the children who had a testicular primary tumor (survival rate, 100%). The 2-year survival rate for patients with ovarian tumors was 67%; for those with sacrococcygeal primaries it was 60%. These results suggest that the prognosis for children with EST has improved significantly over the past decade. Contributing factors include therapy based on cisplatin, etoposide, and bleomycin, and relapse surveillance with serial AFP determinations. Second-look procedures should be reserved for patients who have an increasing level of serum AFP, suspicious computed tomography findings, and no obvious evidence of metastatic disease.
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PMID:Endodermal sinus tumor in children. 886 37

Esthesioneuroblastoma, a malignant neoplasm arising from olfactory epithelium, is unusual in the pediatric age-group. Management has traditionally involved surgery and radiotherapy, alone or in combination, with chemotherapy reserved for recurrent or high grade disease. We report a single institution experience utilizing chemotherapy and radiotherapy as the initial treatment and successful control of the primary tumor in two patients. In one patient, neck dissection and high dose chemotherapy combined with autologous bone marrow transplantation were used as successful salvage therapy of neck metastasis. Both patients are alive and disease free with a mean follow-up of 56 months. These results support the role of chemotherapy in the treatment of esthesioneuroblastoma and suggest that chemotherapy be used as part of the initial combined modality treatment plan.
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PMID:Esthesioneuroblastoma in the pediatric age-group: the role of chemotherapy and autologous bone marrow transplantation. 888 6

Based on our experience and a review of the literature, we conclude that superficial, well- to moderately differentiated T1 cancers of the anal margin may be successfully treated with radiotherapy alone or local excision. Stage T2 lesions have a significant risk of inguinal lymph node metastases and should be treated with radiotherapy to the primary tumor in conjunction with elective inguinal lymph node irradiation. The best treatment for T3 and T4 lesions is radiotherapy to the primary lesion and regional nodes (inguinal and pelvic) combined with concomitant chemotherapy. Abdominoperineal resection (APR) should be reserved for patients who have fecal incontinence at presentation or locally recurrent disease after previous radiotherapy.
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PMID:Squamous cell carcinoma of the anal margin. 915 89

The observed response to [131I]MIBG therapy in advanced neuroblastoma after conventional therapy, the noninvasiveness of the procedure, and the high metabolic activity which is frequently observed in untreated tumors led to the concept of substituting [131I]MIBG therapy for combination chemotherapy at diagnosis prior to surgery in patients with advanced disease/high risk neuroblastoma. The objective of this approach is to reduce the tumor volume, enabling adequate surgical resection of the tumor, and to avoid toxicity and the induction of early drug resistance. Chemotherapy is reserved to treat minimal residual disease postoperatively. Thirty-one children who presented with inoperable neuroblastoma were treated according to this protocol. After an objective response to [131I]MIBG therapy at diagnosis, 19 of 27 evaluable patients (70%) had complete or > 95% resection of the primary tumor or did not require surgery at all. Only mild hematological toxicity was observed. It is concluded that [131I]MIBG therapy of neuroblastoma at diagnosis is feasible; its effectiveness in attaining operability of the primary tumor is at least equal to that of combination chemotherapy, but its toxicity is considerably less.
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PMID:[131I]MIBG as a first line treatment in advanced neuroblastoma. 900 52

Adjuvant chemotherapy (chth): The place of postoperative adjuvant chth after complete resection of a primary tumor (R0) is not well established. 13 adjuvant chemotherapy trials with a variety of combinations and most often doxorubicin alone in different regimens were compared to a control group without chemotherapy. Only 2 reports showed a difference in overall survival and 4 some gain in disease-free survival. New studies began with defined inclusion criteria and a control arm without chemotherapy. There are only some risk factors coming into consideration: deep seated tumors with histologic grade 2 and all grade 3 cases. Adjuvant chemotherapy appears justified only within these studies. The potential side effects of adjuvant chemotherapy outside of these trials are detrimental (e.g. 10% cumulative risk of cardiomyopathy after ADM; risk of secondary leukemias) and a benefit is not known. Primary induction chth: Preoperative neoadjuvant chth has to be reserved for studies. They are performed in case of deep seated large primary tumors, not suitable for R0-resection without mutilation. Palliative Chth: In metastatic or locally not curative resectable disease treatment is palliative. The aim of treatment is the relief of existing symptoms or the avoidance of threatening complaints and possibly prolongation of life. The combination of ADM and Ifosfamide (IFO) is commonly used although its superiority to ADM monotherapy is not proven. As second line chemotherapy in case of tumor resistance high dose IFO-monotherapy can be put up for discussion. Myeloablative high-dose-poly-chth with stem cell transplantation remains an experimental approach.
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PMID:[Adjuvant chemotherapy in early soft tissue sarcoma and palliative chemotherapy in advanced soft tissue sarcoma in adults]. 975 90

The records of 15 patients with metastatic carcinoma to skeletal muscle treated between 1979 and the present were reviewed. Fourteen patients were referred with a diagnosis of soft tissue sarcoma and one with suspected infection. There was a previous diagnosis of carcinoma in eight patients but seven patients had no prior diagnosis of a known malignancy. Primary tumors were lung (eight), melanoma (two), gastrointestinal (one), kidney (one), and bladder (one). No primary tumor could be identified in two patients. Local control of metastatic lesions was achieved by radiotherapy in 11 patients as an initial measure. Two patients underwent wide excision and one declined treatment for local tumor control. Eight patients died within 12 months of presentation and survival analysis indicated a 25% overall survival at 60 months. Two patients remained free of disease at 132 months and 72 months. From this study and a review of 52 cases reported in the literature, the authors are unable to find any clinical or radiographic characteristics that distinguish metastatic carcinoma to muscle from soft tissue sarcomas. Surgical resection can be reserved for cases in which radiation does not provide local control.
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PMID:Metastatic carcinoma to skeletal muscle. A report of 15 patients. 991 13

Glandular carcinomas of the larynx are rare tumors that constitute less than 1% of all laryngeal malignancies. A retrospective case review of 12 patients with glandular carcinomas of the larynx is presented to identify patient and tumor characteristics, therapeutic modalities, and treatment outcomes. Ten patients underwent surgical excision of the primary tumor, by either supraglottic laryngectomy, vertical partial laryngectomy, or total laryngectomy. Seven of these patients also received postoperative radiotherapy. After a median follow-up period of 23 months, 7 of the 12 patients (58.3%) died as a result of uncontrolled locoregional disease or distant metastases. The 5-year survival rate was 57% in patients who underwent combination therapy versus 50% for those patients who received surgery alone. Surgical resection is the primary treatment modality used in the majority of cases. Neck dissection is reserved for patients with clinically apparent adenopathy, adenocarcinoma, or high-grade mucoepidermoid carcinoma. Combination therapy with surgical resection and radiotherapy may be more effective in achieving tumor remission than is surgical resection alone.
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PMID:Glandular carcinoma of the larynx: the UCLA experience. 1033 11

Capromab Pendetide imaging illustrates the successful translation of monoclonal antibody technology from the laboratory to the clinic. It provides a means of identifying otherwise occult soft tissue metastases in patients with adenocarcinoma of the prostate. When utilized with other clinical, pathological and laboratory findings, Capromab Pendetide imaging enables more accurate disease staging and monitoring than is afforded by other imaging modalities such as CT and MRI. In the primary disease setting Capromab Pendetide imaging should be reserved for use in patients with negative bone scans who are at high risk for metastatic disease based on such factors as advanced clinical stage, high Gleason score and significantly elevated serum PSA or alkaline phosphatase. Due to low sensitivity for small-volume disease, a negative Mab scan may not eliminate the need for a staging lymph node dissection but should encourage further consideration of local treatment options. Capromab Pendetide should be used with caution in patients at low risk for metastatic disease. Positive scan findings in low risk patients should be confirmed before altering the treatment plan since some false positive scans should be anticipated in a population with low disease prevalence. Capromab Pendetide imaging has not been shown to be reliable in determining the local extent of the primary tumor but new techniques involving co-registration of SPECT and CT images show promise in this regard. In the patient with recurrent disease following primary therapy, the predictive value of Capromab Pendetide imaging of the prostate or prostate fossa is limited, particularly following RT. Its more important role in this setting is to identify lymph node metastases in the high risk patient with a negative bone scan who might otherwise be a candidate for local salvage therapy. A large prospective study is needed for confirmation, but preliminary data suggest that Capromab Pendetide imaging is helpful in identifying those patients with PSA elevation after radical prostatectomy who are most likely to benefit from salvage RT. As with any imaging technique, Capromab Pendetide has strengths and weaknesses that must be understood to maximize patient benefit by utilizing the scan in clinical settings where it is most likely to be useful and least likely to be misleading. Capromab Pendetide is a technically demanding procedure best performed and interpreted at sites with experience and expertise.
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PMID:Capromab Pendetide imaging of prostate cancer. 1080 17

Current concepts in management of the clinically negative and clinically positive neck in laryngeal cancer are reviewed. Occult disease in the neck not detected by physical and radiographic examination may also be difficult to identify on routine histologic examination. Immunohistochemistry or molecular analysis may detect metastatic involvement not apparent by light microscopy. The surgeon should be aware of the relatively high incidence of micrometastases in patients with laryngeal cancer to establish optimal treatment approaches. Elective treatment of the neck is recommended for supraglottic tumors staged T2 or higher, and glottic or subglottic tumors staged T3 or higher. The neck may be treated electively by either surgery or irradiation, but irradiation is best reserved for cases where that modality is employed for the primary tumor. Elective neck dissection provides important information for prognostic purposes and therapeutic decisions, by establishing the presence, number, location and nature of occult lymph node metastases. The selective lateral neck dissection (levels II, III and IV), unilateral or bilateral, is the procedure of choice for elective treatment. Paratracheal nodes (level VI) should be dissected in cases of advanced glottic and subglottic cancer. Complete radical or functional neck dissections are excessive in extent, as levels I and V are almost never involved. Sentinel lymph node biopsy may fail to detect tumor on frozen section examination or may not reveal 'skip' metastases. The clinically involved neck is usually treated by complete radical or functional neck dissection of levels I through V. Selective neck dissection has been employed successfully in selected cases, particularly for N1 or occasionally N2 nodal involvement. The selective neck dissection can be extended to include structures at risk. More advanced disease has been treated in this manner often in association with adjuvant chemotherapy and/or irradiation. While the benefit of adjuvant treatment is difficult to assess, it appears most useful in cases with extranodal spread of disease, a factor associated with the worst prognosis.
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PMID:Surgical treatment of the neck in cancer of the larynx. 1085 23

Sentinel node biopsy has the potential to provide more accurate staging information than axillary node dissection. Given the considerable morbidity of axillary node dissection this less invasive approach is attractive. However, there are a number of issues to be resolved before the best technique of sentinel node biopsy is determined. When large studies with long-term follow up demonstrate that lymphatic mapping to identify clinically occult lymph node metastases is as effective as we hope, then full axillary node dissection can be reserved to treat patients who indeed have lymph node metastases. Around 60% of the patients could then be spared an axillary node dissection that they do not need because they do not have metastases there. Modern technology is providing more accurate prognostic information based on primary tumor characteristics. Applying these technologies to sentinel lymph nodes may render the lymph node status even more relevant than it currently is.
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PMID:Status of lymph node staging. 1244 69


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