Klinická onkologie : casopis Ceské a Slovenské onkologické spolecnosti
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Pheochromocytomas and paragangliomas are tumors arising from chromaffin cells. These tumors produce catecholamines and are typically found with symptoms and signs that may include hypertension (persistent or episodic), palpitations, headache and sweating. So far, 10 different genes have been associated with both tumors and other genes are expected to be detected. ⋯ Patients with SDHB mutations are at high risk to develop malignant disease and unfortunately current therapeutic options for malignant form of disease are poor. Genetic testing plays a key role in the management of these tumors and therefore not only index patients with pheochromocytoma but also relatives should be tested. Management of this disease requires multidisciplinary cooperation and should be performed in the specialized medical centres.
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Case report - 30 months surviving patient with unresectable hepatocellular carcinoma treated with sorafenib.
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Preoperative radiotherapy is considered to be standard treatment for locally advanced rectal cancer. The timing and dosage of radiotherapy with or without preoperative chemotherapy remain controversial issues. The objective of this study was to evaluate relevant clinical outcomes of two preoperative radiotherapy regimens - the short-course and -long-course radiotherapy with or without chemotherapy for patients with locally advanced rectal cancer. ⋯ Our findings provide convincing evidence that in comparison to preoperative short-course radiotherapy, the preoperative long-course radiotherapy in combination with chemotherapy is the most effective treatment modality for patients with operable locally advanced rectal cancer in terms downstaging and pathologic complete response. Increase in overall survival time as well as lower local recurrence rate makes this modality superior to other preoperative radiotherapy alternatives.
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Case Reports
[Paraneoplastic neurological syndrome in 64-year-old patient in association with a small cell lung carcinoma].
Paraneoplastic neurological syndromes are rare conditions manifest as psychiatric/neurological symptoms. They are caused by autoimmunne cross-reaction leading to destruction of the central nervous system. ⋯ Paraneoplastic neurological syndromes can be early signs of malignancy and may considerably precede oncologic diagnosis.
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Birt-Hogg-Dubé syndrome (BHDS, MIM 135150) is an autosomal dominant condition characterized by presence of skin fibrofolliculomas, lung cysts, spontaneous pneumothorax and renal cancer. The disease is caused by germ-line mutations of the FLCN gene, which encodes protein folliculin. ⋯ Clinical recommendations include increased care during general anesthesia due to a higher risk of pneumothorax, and long-term follow-up due to an elevated risk of renal cancer. Diagnostic and predictive DNA tests are available; prenatal and preimplantation diagnosis is possible.