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)

Approximately 80 per cent of all patients with testis cancer will present with low-volume disease. If they are pathologic stage I, there is virtually a 100 per cent cure rate even though 5 to 10 per cent will relapse after node dissection. Those who have relapsed have been salvaged with systemic chemotherapy. If clinical stage I patients are followed on observation protocols, 20 to 30 per cent of the patients will relapse. Unfortunately, a small percentage of these patients will not be salvaged with chemotherapy because they have more advanced disease at the time that the relapse is discovered. The main objection to retroperitoneal lymph node dissection in patients with low-stage disease has been the issue of infertility induced by the procedure. This problem has been alleviated by the development of the prospective nerve-sparing procedures. Long-term follow-up is still required to make certain that the relapse rate is not significantly higher than with a full retroperitoneal lymph node dissection. Experience has demonstrated that patients with stage II disease will achieve a 98 to 99 per cent cure rate regardless of whether they have adjuvant chemotherapy after surgery, or full-course chemotherapy should they relapse (approximately 50 per cent). Advanced disease is best treated initially by systemic chemotherapy. The emphasis in the last few years has been on a reduction in the toxicity of chemotherapy while maintaining the high degree of efficacy. Surgery is used as an adjunct to chemotherapy in those patients who achieve a partial remission. Careful follow-up cannot be over emphasized in patients with any form of treatment for testis cancer. It is especially important that those patients who have teratoma present in the surgical specimens obtained at the time of a postchemotherapy lymph node dissection have prolonged follow-up, because recurrences have been noted as long as 6 to 8 years after the initial event. Although primary retroperitoneal or mediastinal germ-cell tumors do exist, the vast majority arise from the testis. If the diagnosis of germ-cell tumor has been made by some means other than orchiectomy, a careful review of the patient's history and physical findings is needed to guide the physician to the possible testicular site of origin. If no physical findings are present, scrotal ultrasound may be helpful in localizing a primary tumor of the testis. If such localization is possible, then a radical orchiectomy should be performed to prevent future seeding from persistent tumor in the testicle.(ABSTRACT TRUNCATED AT 400 WORDS)
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PMID:Testicular germ-cell neoplasms: curative approaches. 305 95

Testicular intraepithelial neoplasia (TIN; also called carcinoma in situ of the testis) is the uniform precursor of testicular germ cell tumors. There is general agreement on the biological significance of TIN, however, the treatment is still a matter of dispute. The present review summarizes the treatment options currently available. In general, the management of TIN has to be adapted to the particular clinical situation of the patient. Eradication of TIN usually implies the loss of fertility. Therefore, fertility aspects should be considered before any kind of treatment is employed. Usually, patients with TIN have only small residual potential of fertility. Nonetheless, individual patients may qualify for sperm banking or cryopreservation of testicular tissue for future sperm extraction (TESE) and assisted fertilization. The most common clinical situation is the case of contralateral TIN in the presence of unilateral testicular cancer. Low dose radiotherapy to the testis with 18 Gy is the standard management option in these patients. The same procedure may be applied to solitary testicles after partial orchiectomy for germ cell tumors. During follow-up, testosterone levels should be evaluated every six months. If chemotherapy is required due to metastatic disease of the primary tumor management of TIN should be deferred. After chemotherapy 30% of TIN cases will persist and approximately 42% will recur in the later course. Repeat biopsy should be done six months after completion of chemotherapy or later. Only in cases with persistent TIN additional radiotherapy should be administered. If one testicle is afflicted with TIN while the other testis is in healthy condition (conceivable in infertility cases or patients with primary extragonadal germ cell tumors), then the TIN-bearing testis should be excised. Radiotherapy is not feasible in these cases because of shielding problems with the healthy testis.
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PMID:[Management of testicular intraepithelial neoplasia (TIN)--a review based on the principles of evidence-based medicine]. 1123 74

Fallopian tube carcinoma is the rarest of all female genital tract malignancies. It usually occurs in postmenopausal women and is associated with infertility. We present the first reported case of it occurring as a primary tumor in a young primigravida. It presented as a large, rapidly growing adnexal mass at 9 weeks of gestation which was removed and found to be a papillary serous carcinoma of the fallopian tube. The patient continued the pregnancy to term and delivered a live healthy infant by ventouse. A staging laparotomy in the postnatal period showed no spread of tumor, and in view of her age and desire for further pregnancies, her uterus and other ovary and tube were conserved. She remains tumor free 2 years following detection. We discuss the incidence, progress, management, and survival rates of this rare gynecological malignancy.
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PMID:An unusual case of primary fallopian tube carcinoma in pregnancy. 1651 25

Rhabdomyosarcoma is the most common sarcoma of childhood. Fortunately, the goal of cure is realistic for the majority of patients with localized tumors. However, management of these patients remains challenging. The fact that the tumor arises in a wide variety of primary sites, some of which are associated with specific patterns of local invasion, regional lymph node spread, and therapeutic response, requires physicians to be familiar with site-specific staging and treatment details. In addition, rhabdomyosarcoma requires multimodality therapy that can be associated with significant acute toxicities and long-term effects, particularly when administered to young children. These factors sometimes present a dilemma as to the best approach to optimize the chance of cure, minimize toxicity, and respect quality of life. The purpose of this review is to discuss 'optimal' management of this complicated tumor. Since the tumor is relatively rare, requires highly specialized care, and important management questions remain to be answered, optimal management of rhabdomyosarcoma includes enrollment in clinical trials whenever possible. Appropriate management begins with establishing the correct pathologic diagnosis, histologic subtype, primary site, extent of disease (International Society of Pediatric Oncology [SIOP]-TNM-Union Internationale Contre le Cancer stage or Intergroup Rhabdomyosarcoma Study Group [IRSG] stage), and extent of resection (IRSG group). Cooperative groups throughout North America and Europe have defined risk-adapted treatment based on these factors; this treatment requires a coordinated management plan that includes surgery, chemotherapy, and usually radiotherapy. The surgical approach for rhabdomyosarcoma is to excise the primary tumor whenever possible without causing major functional or cosmetic deficits. Wide excision is difficult in some primary sites and can be complicated by the fact that the tumor grows in a locally infiltrative manner so that complete resection is often neither possible nor medically indicated. Incompletely resected tumors are generally treated with radiotherapy. The cooperative groups reduce the dose of radiation based on the response of the tumor to chemotherapy and delayed primary resection to differing degrees. Response-adjusted radiation administration may reduce the long-term effects of radiotherapy, such as bone growth arrest, muscle atrophy, bladder dysfunction, and induction of second malignant neoplasms; however, it may also be associated with an increased risk of tumor recurrence. All patients with rhabdomyosarcoma require chemotherapy. A backbone of vincristine and dactinomycin with either cyclophosphamide (VAC) or ifosfamide (IVA) has been established. Risk-adapted treatment involves reducing or eliminating the alklyating agent for patients with the most favorable disease characteristics. Clinical trials are ongoing to improve outcomes for higher risk patients; newer agents, such as topotecan or irinotecan, in combination with VAC or use of agents in novel ways are being investigated. Acute and long-term toxicities associated with these chemotherapy regimens include myelosuppression, febrile neutropenia, hepatopathy, infertility, and second malignant neoplasms. A 5-year survival rate >70% has been achieved in recent trials for patients with localized rhabdomyosarcoma. However, the outcome for patients who present with metastatic disease remains poor. In the future, risk-adapted classification of rhabdomyosarcoma will likely be based on biologic features, such as the presence of chromosomal translocations or specific gene expression profiles. It is hoped that newer therapies directed at specific molecular genetic defects will benefit all patients with rhabdomyosarcoma.
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PMID:Optimal management strategies for rhabdomyosarcoma in children. 1805 9

Rhabdomyosarcoma (RMS), a malignant tumor of mesenchymal origin, is the third most common extracranial malignant solid tumor in children and adolescents. However, in adults, RMS represents <1% of all solid tumor malignancies. The embryonal and alveolar histologic variants are more commonly seen in pediatric patients, while the pleomorphic variant is rare in children and seen more often in adults. Advances in the research of the embryonal and alveolar variants have improved our understanding of certain genes and biologic pathways that are involved in RMS, but much less is known for the other variants. Multimodality therapy that includes surgery and chemotherapy with or without radiation therapy is the mainstay of treatment for RMS. Improvements in the risk stratification of the pediatric patients based on presurgical (primary tumor site, tumor size, regional lymph node involvement, presence of metastasis) and postsurgical parameters (completeness of resection or presence of residual disease or metastasis) has allowed for the treatment assignment of patients in different studies and therapeutic trials, leading to increases in 5-year survival from 25%-70% over the past 40 years. However, for adult patients, in great part due to rarity of the disease and the lack of consensus on optimal treatment, clinical outcome is still poor. Many factors have been implicated for the differing outcomes between pediatric RMS versus adult RMS, such as the lack of standardized treatment protocols for adult RMS patients and the increased prevalence of advanced presentations. Now that there are increased numbers of survivors, we can appreciate the sequelae from therapy in these patients, such as bone growth abnormalities, endocrinopathies, and infertility. Improvements in risk stratification have led to clinical trials using lower doses of chemotherapy or radiation therapy with the intention of decreasing the incidence of side effects without compromising survival outcome.
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PMID:Rhabdomyosarcoma in adolescent and young adult patients: current perspectives. 2496 11

Neuroblastoma is the most common extracranial solid tumor, arising from primitive sympathetic ganglion cells, in pediatric patients. The unique features of neuroblastoma include variable clinical behaviors, such as rapid progression to death and maturation to benign ganglioneuroma, followed by regression. Radiation therapy (RT) is usually administered to both the primary tumor bed and persistent metastatic sites after induction chemotherapy for high-risk neuroblastoma. RT to the tumor bed after surgical resection contributes significantly to local disease control and prevention of local relapse, confirming the role of RT. Palliative radiotherapy for metastatic neuroblastoma is also effective and safe and mainly provides symptomatic relief. The late side effects of RT in neuroblastoma patients include growth and developmental failure, hypothyroidism, gastrointestinal dysfunction, neurocognitive defects, pulmonary and cardiac abnormalities, infertility, and secondary cancers. In this article, we reviewed the role and toxicity of RT in neuroblastoma patients.
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PMID:Role and toxicity of radiation therapy in neuroblastoma patients: A literature review. 3217 25