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Mutsushi Yamasaki, Yoshiyasu Sato, Takeo Nomura, Fuminori Sato, Shinya Uchino, Hiromitsu Mimata
Multiple endocrine neoplasia type 2 (MEN2) is an autosomal-dominant cancer syndrome with major components of medullary thyroid carcinoma, pheochromocytoma, and hyperparathyroidism. MEN2B is the most aggressive and rarest of the MEN2 variants. Pheochromocytoma in MEN2 is virtually always located in the adrenal medulla, but MEN2-associated extra-adrenal pheochromocytomas (paraganglioma) are rare. A 59-year-old man who has been diagnosed with MEN2B consulted our hospital for surgical treatment of a 10-mm left adrenal mass and a 30-mm retroperitoneal mass...
October 5, 2016: Asian Journal of Endoscopic Surgery
Santosh Kumar, Shivanshu Singh, Abhishek Chandna
The retroperitoneum is a closed space harbouring vital organs including the great vessels, kidneys and adrenal glands, ureters, and the ascending and descending colon. Surgical management of retroperitoneal pathologies may need multiorgan resection in order to achieve complete surgical resection while preservation of surrounding organs should be attempted, especially in case of benign tumors. We present a case of 15-year-old girl with an 11 × 6 × 5 cm retroperitoneal ganglioneuroma displacing the right kidney, renal vein, and ureter and abutting the IVC which was excised in toto preserving the right kidney and ureter with careful dissection around the great vessels...
2016: Case Reports in Surgery
Monica Ortenzi, Roberto Ghiselli, Maria Michela Cappelletti Trombettoni, Luca Cardinali, Mario Guerrieri
AIM: To analyze the outcomes of transanal endoscopic microsurgery (TEM) in the treatment of rare rectal condition like mesenchymal tumors, condylomas, endometriosis and melanoma. METHODS: We retrospectively reviewed a twenty-three years database. Fifty-two patients were enrolled in this study. The lesions were considered suitable for TEM if they were within 20 cm from the anus. All of them underwent an accurate preoperative workup consisting in clinical examination, total colonoscopy with biopsies, endoscopic ultrasonography, and pelvic computerized tomography or pelvic magnetic resonance imaging...
September 16, 2016: World Journal of Gastrointestinal Endoscopy
Ruthy Shaco-Levy, Kory W Jasperson, Katie Martin, N J Samadder, Randall W Burt, Jian Ying, Mary P Bronner
GOALS: To further characterize the gastrointestinal manifestations of Cowden syndrome in clinically well-annotated patients to improve the diagnosis of this syndrome. BACKGROUND: The gastrointestinal manifestations of Cowden Syndrome, an important heritable and multiorgan cancer syndrome, are not well defined. Proper diagnosis is essential for effective cancer surveillance and prevention in these patients. STUDY: Cowden patients with gastrointestinal polyps were selected for medical record and pathologic slide review...
September 22, 2016: Journal of Clinical Gastroenterology
Delia Proposito, Francesca De Lucia, Alessandra Giannella, Francesca Frangella, Giuseppe Pappalardo, Maria Santangelo, Domenico Magagnano
AIM: Ganglioneuroma (GN) is the most uncommon and the most benign tumor among neuroblastic neoplasms, and in 29.7% of cases it finds in an adrenal gland. Usually asymptomatic, this tumor is detected incidentally in the majority of cases. It is generally challenging to obtain a precise diagnosis of adrenal ganglioneuroma (AGN) before surgery. Misdiagnosis rate of AGN on CT and MRI is 64.7% and clinicians and surgeons are often lacking in knowledge of this rare disease. For this reason, we pointed out the clinical, biochemical, radiologic and pathologic features of AGN in an our experience, with the aim to find out if there are some features able to facilitate a preoperative diagnosis...
2016: Annali Italiani di Chirurgia
Xiaojuan Deng, Jingqin Fang, Qingya Luo, Haipeng Tong, Weiguo Zhang
BACKGROUND: Ganglioneuroma is a rare benign tumor originating from the sympathetic nerves, and its origination from the trigeminal nerves is even rarer. Only 4 cases of ganglioneuroma originating from the trigeminal nerve have previously been reported, and these studies only reported conventional MRI manifestations. To our knowledge, the advanced MRI features of trigeminal ganglioneuroma have not been reported thus far. CASE PRESENTATION: This study reports a case of trigeminal ganglioneuroma in the left cerebellopontine angle...
2016: BMC Cancer
Yasemin Benderli Cihan, Aynur Aytekin, Talha Sarigoz
No abstract text is available yet for this article.
May 2016: Journal of B.U.ON.: Official Journal of the Balkan Union of Oncology
P Nesnídal, J Štulík, R Lischke, M Barna
UNLABELLED: The case of a 15-year-old girl with a large ganglioneuroma in the mediastinum and spinal canal is presented. The tumour initially manifested as scoliosis. Its diagnosis was made on the basis of CT scanning and magnetic resonance imaging, and confirmed by thoracoscopic mediastinal biopsy. Radical tumour excision was indicated. The first stage involved removal of the tumour from the spinal canal through a posterior approach, and transpedicular fixation of the spinal column with correction of the curve...
2016: Acta Chirurgiae Orthopaedicae et Traumatologiae Cechoslovaca
Boris Decarolis, Thorsten Simon, Barbara Krug, Ivo Leuschner, Christian Vokuhl, Peter Kaatsch, Dietrich von Schweinitz, Thomas Klingebiel, Ingo Mueller, Lothar Schweigerer, Frank Berthold, Barbara Hero
BACKGROUND: Ganglioneuroma (GN) and ganglioneuroblastoma intermixed (GNBI) are mature variants of neuroblastic tumors (NT). It is still discussed whether incomplete resection of GN/GNBI impairs the outcome of patients. METHODS: Clinical characteristics and outcome of localized GN/GNBI were retrospectively compared to localized neuroblastoma (NB) and ganglioneuroblastoma-nodular (GNBN) registered in the German neuroblastoma trials between 2000 and 2010. RESULTS: Of 808 consecutive localized NT, 162 (20 %) were classified as GN and 55 (7 %) as GNBI...
2016: BMC Cancer
Christoph Paasch, Anja Harder, Esther Jasmin Gatzky, Ehssan Ghadamgahi, Andreas Spuler, Robert Siegel
BACKGROUND: Ganglioneuroma (GN) of the adult is a rare benign tumour originating from neural crest-derived cells. In most cases, GN is found in the mediastinum or retroperitoneum incidentally and may present with unspecific symptoms caused by space-occupying effects. The correct diagnosis of a retroperitoneal mass is still a challenge. Nevertheless, a preoperatively confirmed diagnosis of GN may support the concept of a less radical approach and may help to prevent unnecessary morbidity or loss of function...
2016: World Journal of Surgical Oncology
Jeremy R Jackson, Hung C Tran, James E Stein, Hiroyuki Shimada, Ankur M Patel, Araz Marachelian, Eugene S Kim
BACKGROUND: Although patients with peripheral neuroblastoma (NB; pelvic and thoracic) typically have better outcomes and less aggressive disease compared with patients with abdominal disease, little has been published with regard to the management and outcomes of patients with cervical NB. Herein, we sought to determine the characteristics of cervical neuroblastic tumors and the effect of extent of resection on survival and outcomes. METHODS: We performed a retrospective review of 325 children with neuroblastic tumors at Children's Hospital Los Angeles over a 15-y period (January 1990-February 2015)...
July 2016: Journal of Surgical Research
Joon-Hyop Lee, Young Jun Chai, Tae-Hyung Kim, June Young Choi, Kyu Eun Lee, Hyun-Young Kim, Yoo-Seok Yoon, Hyeon Hoe Kim
BACKGROUND: Ganglioneuromas are benign tumors that rarely develop from adrenal glands. In this study, we present our clinical experience of patients with adrenal ganglioneuroma (AGN). METHODS: Demographic, diagnostic, surgical, and pathologic findings of patients who were adrenalectomized as a result of AGN were retrospectively reviewed from the database of a tertiary referral hospital. RESULTS: Among 1784 patients who underwent an adrenalectomy between 2002 and 2015, 35 (1...
July 18, 2016: World Journal of Surgery
Halil Ibrahim Serin, Sureyya Burcu Gorkem, Selim Doganay, Saliha Cıracı, Ekrem Unal, Mahmut Guzel, Ahmet Kucuk, Ali Kurtsoy, Abdulhakim Coskun
PURPOSE: Our aim was to assess diffusion weighted imaging (DWI) of neuroblastic tumors and whether apparent diffusion coefficient (ADC) value may have a role in discrimination among neuroblastoma, ganglioneuroblastoma and ganglioneuroma. MATERIAL AND METHODS: The DWIs (b = 0-800 s/mm(2)) of 24 children (13 girls, 11 boys) who were diagnosed neuroblastic tumors on histopathological examination (neuroblastoma = 15, ganglioneuroblastoma = 5, ganglioneuroma = 4) were evaluated retrospectively...
September 2016: Japanese Journal of Radiology
Chao Yang, Chang-Chun Li, Jun Zhang, Xiang-Ru Kong, Zhenzhen Zhao, Xiao-Bin Deng, Liang Peng, Shan Wang
Ganglioneuromas are rare tumors in the neuroblastoma group. Paraneoplastic syndrome (PNS) due to presacral ganglioneuromas was hardly reported in previous literature. Here, we reported that a case of a 5-year-old girl with a presacral ganglioneuroma presented with PNS, who presented with intermittent fever, progressive weight gain, and personality changes. Our report revealed intermittent fever, progressive weight gain, and personality changes may represent rare paraneoplastic syndromes in ganglioneuromas.
2016: Case Reports in Endocrinology
Denys Gibbons, Michael Camilleri, Alfred D Nelson, Deborah Eckert
BACKGROUND: Gastrointestinal symptoms are frequent in multiple endocrine neoplasia (MEN) 2B and may be related to megacolon. OBJECTIVE: The objective of this article is to review the clinical features of patients with MEN 2B, particularly megacolon. METHODS: We used natural language processing of electronic medical records of Mayo Clinic patients over 20 years: Eight patients with definite MEN 2B were identified; of these, four had megacolon...
June 2016: United European Gastroenterology Journal
Naoto Watanabe, Kunihiko Yokoyama, Seigo Kinuya, Hisao Tonami
OBJECTIVE: The purpose of this study was to evaluate the degree of cytological radiation damage to lymphocytes occurring after I-131 metaiodobenzylguanidine (MIBG) therapy as determined by the cytokinesis-blocked micronucleus assay. The chromosomal damage to lymphocytes induced by I-131 in vivo should result in augmentation of the number of cells with micronuclei. METHODS: We studied 15 patients with pheochromocytoma (14/15) or ganglioneuroma (1/15), who were treated initially with 7...
July 9, 2016: Annals of Nuclear Medicine
Antonio Ríos, Luis Felipe Alemón, José Ruiz, Jose Manuel Rodríguez
No abstract text is available yet for this article.
June 27, 2016: Cirugía Española
Zhi-Gang Yao, Xiu-Mei Liu, Ye-Jun Qin
We describe two children with ganglioneuroma (GN) likely originating from incompletely resected neuroblastoma (NB) during infancy, stages 2A and 2B, who did not undergo postoperative adjuvant chemotherapies. Both NB tumors had no MYCN amplification, had TrKA but no TrkB expression, and by TUNEL had apoptosis. These findings may have contributed to spontaneous maturation of the residual primary NB and hence the favorable prognosis, which suggests surgery alone might be the sufficient initial therapy for low-risk patients...
June 30, 2016: Fetal and Pediatric Pathology
M Beth McCarville
This review focuses on the MRI features of neurogenic tumors and rhabdomyosarcoma in children. Neurogenic tumors include those arising from a nerve sheath and neuroblastic tumors that arise from the sympathetic nervous system. Nerve sheath tumors can be benign or malignant and occur sporadically or in association with neurofibromatosis type 1. Neuroblastic tumors comprise a spectrum of tumors ranging from highly malignant neuroblastoma to the benign ganglioneuroma. These neurogenic tumors arise in typical locations within the chest, abdomen and pelvis and have distinctive and characteristic imaging features that should suggest their diagnosis...
May 2016: Pediatric Radiology
Tomoko Iehara, Shigeki Yagyu, Kunihiko Tsuchiya, Yasumichi Kuwahara, Mitsuru Miyachi, Tatsuro Tajiri, Tohru Sugimoto, Tadashi Sawada, Hajime Hosoi
BACKGROUND: It remains unclear whether a residual tumor mass following therapy influences the prognosis of neuroblastoma. METHODS: We retrospectively reviewed 20 patients with intermediate-risk tumors treated at our institution between 1993 and 2012 to elucidate whether additional treatment is required for residual tumors. RESULTS: The patient ages at diagnosis ranged from 0 days to 7 years. The 5-year overall survival rate was 94.4%. Thirteen patients had Stage 3 disease and seven patients had Stage 4 disease...
July 2016: Japanese Journal of Clinical Oncology
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