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The abdomen
Published in James A. Gossage, Matthew F. Bultitude, Steven A. Corbett, Katherine M. Burnand, Rajiv Lahiri, Kevin Burnand, Browse's Introduction to the Symptoms & Signs of Surgical Disease, 2021
James A Gossage, Katherine M Burnand
Solid tumours that can become very large and cause abdominal distension are, in approximate order of frequency: Hepatomegaly (Budd–Chiari syndrome or metastatic liver tumours).Fibroids.Splenomegaly (myelofibrosis).Large cancers of the colon.Polycystic kidneys.Primary carcinoma of the liver.Retroperitoneal sarcoma and lymphadenopathy.Neuroblastoma/ganglioneuroblastoma/ganglioneuroma (in children).Nephroblastoma (in children).Neurofibroma/schwannoma.
Fluids and electrolyte management and nutritional support
Published in Brice Antao, S Irish Michael, Anthony Lander, S Rothenberg MD Steven, Succeeding in Paediatric Surgery Examinations, 2017
Craig R Nemechek, Onyebuchi Ukabiala
VIPoma (Verner–Morrison’s syndrome) was originally described as a rare non-betacell pancreatic tumour. It can be associated with multiple endocrine neoplasia type 1 syndrome. More than 20 cases have been described in association with ganglioneuroma and ganglioneuroblastoma as well as the maturation process often induced by chemotherapy for neuroblastoma. Being a neural crest tumour, this is easily understood since the cells in the APUD (amine precursor uptake and decarboxylation) system are often capable of secretions in common with one another. Prolonged/profuse watery diarrhoea, hypokalaemia with dehydration, lethargy, hypotonia and abdominal pain are common. Occasionally, carcinoid syndrome-type flushing with impaired glucose tolerance is seen. Treatment is directed to correction of the water and the electrolyte abnormalities. Pharmacological suppression with somatostatin is helpful but the only cure is surgical resection, even if the residual tumour is benign.
Neuroblastoma
Published in Demetrius Pertsemlidis, William B. Inabnet III, Michel Gagner, Endocrine Surgery, 2017
The histopathology of neuroblastoma, as defined by the International Neuroblastoma Pathology Classification (INPC) system, also known as the Shimada classification, also plays a role in determining how aggressive the tumor is [7]. The Shimada classification evaluates the tumor specimen, before treatment, for the amount of Schwannian stroma, the degree of neuroblastic maturation, and the mitosis–karyorrhexis index. Combining these features with patient age, the Shimada classification determines whether the histology is favorable or unfavorable, which has an effect on risk grouping and prognosis. The Shimada classification also places the tumor into one of four subtypes of neurogenic tumors. Neuroblastoma is stroma poor, ganglioneuroma is stroma dominant, and ganglioneuroblastoma can be either intermixed or nodular, both of which are stroma rich, with the nodular type having some areas that are stroma poor.
Surgical Treatment of Cervicothoracic Junction Lesions in Children: A Series of 18 Cases
Published in Journal of Investigative Surgery, 2022
Yanzhen Li, Shengcai Wang, Jun Tai, Qi Zeng, Jie Zhang, Yuanhu Liu, Wentong Ge, Yuzhang Huang, Xuexi Zhang, Qiaoyin Liu, Nian Sun, Chenghao Chen, Xin Ni
However, there were 3 malignant cases of ganglioneuroblastoma which were difficult to excise by simple LACA due to its own characteristics. A ganglioneuroblastoma is a tumor arising from the sympathetic nerve in the neck and chest. The nerves and ganglia of the somatic and autonomic nervous systems are visible in the thorax, although they are mainly concentrated in the paravertebral sulcus, commonly referred to as the posterior mediastinum. Before the advent of video-assisted thoracoscopic surgery (VATS), most nerve sheath and autonomic nervous system tumors were best approached by standard posterolateral thoracotomy. However, given the advantages shown by the efficacy of VATS and its less invasive nature, including fewer postoperative pulmonary complications, a more rapid return of respiratory function, a shorter postoperative hospital stay, decreased hospital cost, less postoperative pain and disability and better cosmesis, thoracic surgeons have recently chosen VATS as the procedure of choice to remove intrathoracic neurogenic tumors [26, 27]. In our study, LACA was applied to resect the part of the tumor in the neck and unilateral-side VATS was conducted to remove the part in the throat. The patients occurred pleural effusion after operations, but returned to normal in short time. In one case, however, the tumor proved to be challenging, and there was a risk of damage to the subclavian and vertebral arteries during the procedure; thus, posterolateral thoracotomy was used for lesion removal. Atelectasis and pneumothorax developed postoperatively in the child. Our experience indicates that the minimally invasive technique, LACA combined with VATS, could be considered first for the resection of cervicothoracic malignant lesions extending to T4.