1/2. Treatment policy of neuroendocrine small cell cancer of the cervix.Small cell cancers of the cervix are very rare and aggressive tumours. It is difficult to manage these tumours. They are often diagnosed in an advanced stage and their prognosis is generally poor. There are no clinical trials, due to their rarity, that would suggest optimal treatment. The present report describes a patient with a neuroendocrine small cell cancer of the cervix Stage IB2 with a positive lymph node. The treatment consisted of radical hysterectomy and node dissection, adjuvant chemotherapy, chemoradiation and brachytherapy. Currently, after 52 months, the patient is well and free of disease. Since 1996, there has been a classification for neuroendocrine tumours (NETs) of the cervix in four categories (large cell, small cell, typical carcinoid and atypical carcinoid). The aggressive behaviour of neuroendocrine small cell cancer is demonstrated by the high percentage of early lymphatic node and vessel invasion (68 and 90%). Almost half of the patients with Stage I and II will recur with an estimated 5-year survival from 14% to a maximum of 55%. Multimodal therapy for these tumours appears to give good response but often implies severe side-effects.- - - - - - - - - - ranking = 1keywords = behaviour (Clic here for more details about this article) |
2/2. Obstructive jaundice secondary to neuroendocrine tumour in a patient with von Recklinghausen's disease.Neurofibromatosis type 1 (NF1) is an autosomal dominant genetic disorder, with variable clinical manifestations and unpredictable course, associated with an increased incidence of various tumours. Plexiform neurofibromas are hallmark lesions of NF1; they are slow-growing tumours, which account for substantial morbidity, including disfigurement and functional impairment, and may even be life-threatening. Neuroendocrine tumours (NETs), a rare diverse group of neoplasms, are occasionally associated with neurofibromatosis. Pancreatic NETs are tumours with an incidence of less than 1/100 000 population/year and complex patterns of behaviour, which often need complicated strategies for optimal management. We present the case of a young adult with NF1, having a unique concurrence of plexiform neurofibroma involving the liver with an ampullary NET, and we discuss step by step the management in a specialist centre.- - - - - - - - - - ranking = 1keywords = behaviour (Clic here for more details about this article) |