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Zhu H, Ye J, Luo H, Ni S, Pan Y, Zhao Z, Yang Y. Ultrasonic-Based Radiomics Signature With Machine Learning for Differentiating Prognostic Subsets of Pediatric Peripheral Neuroblastic Tumors: A Retrospective Study. ULTRASOUND IN MEDICINE & BIOLOGY 2025; 51:852-859. [PMID: 39924420 DOI: 10.1016/j.ultrasmedbio.2025.01.013] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Subscribe] [Scholar Register] [Received: 12/04/2024] [Revised: 01/03/2025] [Accepted: 01/21/2025] [Indexed: 02/11/2025]
Abstract
OBJECTIVE To construct and select a better model based on ultrasonic-based radiomics features and clinical characteristics for prognostic subsets of pediatric neuroblastic tumors. METHODS Data from 73 children with neuroblastic tumors were included and divided into a training group and a validation group. Data 1 contained the subjects' radiomics features and clinical characteristics, while data 2 contained radiomics features. With the help of machine learning, five models were constructed for data 1 and data 2, respectively. The model with the highest accuracy and area under the curve was selected as the combined model and radiomics model for data 1 and data 2, respectively. A superior model was then chosen from the models after further comparison. RESULTS The extreme gradient-boosting model for data 1 was chosen as the combined model and the extreme gradient-boosting model for data 2 was chosen as the radiomics model. The area under the curve of the combined and radiomics models in the validation group was 0.941 and 0.918 (p = 0.6906). The balanced accuracy, kappa value and F1 score of the radiomics model (0.9045, 0.8091 and 0.9091, respectively) were higher than those of the combined model (0.8545, 0.7123 and 0.8696, respectively). The top eight features of the radiomics model included five first-order statistical features and three textural features, all of which were high-dimensional features. CONCLUSION Our study proved that the radiomics model outperformed the combined model at differentiating prognostic subsets of pediatric neuroblastic tumors. Additionally, we found that high-dimensional ultrasonic-based radiomics features surpassed other features and clinical characteristics.
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Affiliation(s)
- Hui Zhu
- Department of Ultrasound, The Second Affiliated Hospital of Wenzhou Medical University, Wenzhou, Zhejiang, China; Wenzhou Key Laboratory of Structural & Functional Imaging, Wenzhou, Zhejiang, China
| | - Jiazong Ye
- Department of Medical Imaging & Nuclear Medicine, The Second Affiliated Hospital, Wenzhou Medical University, Wenzhou, Zhejiang, China; Department of Ultrasound, Dongtou District People's Hospital, Wenzhou, Zhejiang, China
| | - Hongxia Luo
- Department of Ultrasound, The Second Affiliated Hospital of Wenzhou Medical University, Wenzhou, Zhejiang, China; Wenzhou Key Laboratory of Structural & Functional Imaging, Wenzhou, Zhejiang, China
| | - Shuangshuang Ni
- Department of Ultrasound, The Second Affiliated Hospital of Wenzhou Medical University, Wenzhou, Zhejiang, China; Wenzhou Key Laboratory of Structural & Functional Imaging, Wenzhou, Zhejiang, China
| | - Yin Pan
- Department of Ultrasound, The Second Affiliated Hospital of Wenzhou Medical University, Wenzhou, Zhejiang, China; Wenzhou Key Laboratory of Structural & Functional Imaging, Wenzhou, Zhejiang, China
| | - Zhilin Zhao
- Department of Ultrasound, The Second Affiliated Hospital of Wenzhou Medical University, Wenzhou, Zhejiang, China; Wenzhou Key Laboratory of Structural & Functional Imaging, Wenzhou, Zhejiang, China
| | - Yan Yang
- Department of Ultrasound, The Second Affiliated Hospital of Wenzhou Medical University, Wenzhou, Zhejiang, China; Wenzhou Key Laboratory of Structural & Functional Imaging, Wenzhou, Zhejiang, China.
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Lipiński Ł, Lipińska J, Kowalczuk M, Kopeć I, Woźniak MM, Mitek-Palusińska J, Mitura-Lesiuk M. Nonspecific Gastrointestinal Symptoms as the First Sign of Ganglioneuroblastoma Intermixed-Case Report and Literature Review. J Clin Med 2023; 12:6092. [PMID: 37763032 PMCID: PMC10531539 DOI: 10.3390/jcm12186092] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 07/20/2023] [Revised: 08/19/2023] [Accepted: 09/19/2023] [Indexed: 09/29/2023] Open
Abstract
Nonspecific gastrointestinal symptoms remain a problem for pediatricians because, out of a thousand trivial cases, there are rare diseases that require in-depth diagnostics and extensive knowledge to identify them. These complaints may be caused by a neoplastic process. We present the case of a 5-year-old boy whose diagnostic pathway lasted about 3 months. He was admitted to hospital due to severe abdominal pain. Physical examination revealed a bloated, hard, and painful abdomen. In the standing X-ray, the features of intestinal obstruction were visualized. An ultrasound examination showed a possible malignant lesion in the location of the left adrenal gland. After the surgical removal of the pathological mass and histopathological examination, the diagnosis of ganglioneuroblastoma intermixed was made. This tumor, along with neuroblastoma, ganglioneuroma, and ganglioneuroblastoma nodular, belongs to neuroblastic tumors (NTs), which originate from primitive cells of the sympathetic nervous system. NTs are quite rare, but they are still the majority of extracranial solid tumors in children, and their symptoms often appear relatively late when the neoplastic process is already advanced. The purpose of this review is to present current information about ganglioneuroblastoma, with a special emphasis on nonspecific gastrointestinal symptoms as first sign of this tumor and its diagnostics.
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Affiliation(s)
- Łukasz Lipiński
- Students’ Scientific Society at the Department of Pediatric Radiology, Medical University of Lublin, 20-093 Lublin, Poland; (Ł.L.); (J.L.); (M.K.); (I.K.)
| | - Justyna Lipińska
- Students’ Scientific Society at the Department of Pediatric Radiology, Medical University of Lublin, 20-093 Lublin, Poland; (Ł.L.); (J.L.); (M.K.); (I.K.)
| | - Maria Kowalczuk
- Students’ Scientific Society at the Department of Pediatric Radiology, Medical University of Lublin, 20-093 Lublin, Poland; (Ł.L.); (J.L.); (M.K.); (I.K.)
| | - Izabela Kopeć
- Students’ Scientific Society at the Department of Pediatric Radiology, Medical University of Lublin, 20-093 Lublin, Poland; (Ł.L.); (J.L.); (M.K.); (I.K.)
| | | | | | - Małgorzata Mitura-Lesiuk
- Department of Pediatric Hematology, Oncology and Transplantation, Medical University of Lublin, 20-093 Lublin, Poland;
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Nezami BG, Modak S, Cardenas FI, Sarungbam J, Sirintrapun SJ, Gopalan A, Chen Y, Al-Ahmadie H, Fine SW, Reuter VE, Tickoo SK. Ganglioneuroblastoma intermixed: Clinicopathological implications of diagnosis at presentation and genomic correlations. Pediatr Blood Cancer 2023; 70:e30434. [PMID: 37243318 PMCID: PMC10810239 DOI: 10.1002/pbc.30434] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Grants] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 03/17/2023] [Revised: 04/13/2023] [Accepted: 05/02/2023] [Indexed: 05/28/2023]
Abstract
BACKGROUND Ganglioneuroblastoma intermixed (GNBI) is classified as "favorable" histology by International Neuroblastoma Pathology Classification system. However, the International Neuroblastoma Risk Group (INRG) stratifies patients using wider clinicopathological and cytogenetic/molecular parameters. While the diagnosis of GNBI is typically made on resected tumor, it may sometimes be rendered on initial biopsy. We studied GNBI noted at diagnosis to evaluate its correlation with INRG staging and other clinicopathological and molecular features. METHODS In this retrospective study, clinical, radiological, pathological, cytogenetic, and molecular information from patients with GNBI at diagnosis seen between 1995 and 2021 was analyzed. INRG staging was performed. RESULTS Of the 15,827 neuroblastoma specimens, GNBI was noted in 237 patients. Of these, 53 had the initial pathological diagnosis of GNBI; median follow-up 3.5 (range: 0.2-14) years. Disease was locoregional in 41 (77%, 16 stage L1 and 25 L2); none relapsed. Twelve (23%) had metastatic disease at presentation; six (50%) relapsed, and two died of disease. MYCN was amplified in two metastatic tumors. Six of 31 (19%) tumors tested had recurrent cytogenetic abnormalities and nonrecurrent somatic gene mutations in 10/23 (43%). The presence of any adverse molecular/cytogenetic findings was associated with metastatic disease (p < .05). For patients with localized GNBI undergoing both biopsy and resection, GNBI was diagnosed in both in 17/19 (90%). CONCLUSIONS Localized GNBI at diagnosis has excellent long-term clinical outcome even without cytotoxic therapy. For localized GNBI, a biopsy sample is adequate to make the diagnosis. When associated with metastasis at diagnosis, prognosis is poorer, possibly due to associated adverse biological features.
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Affiliation(s)
- Behtash G. Nezami
- Department of Pathology, Memorial Sloan Kettering Cancer Center, New York, NY, USA
| | - Shakeel Modak
- Department of Pediatrics, Memorial Sloan Kettering Cancer Center, New York, NY, USA
| | | | - Judy Sarungbam
- Department of Pathology, Memorial Sloan Kettering Cancer Center, New York, NY, USA
| | | | - Anuradha Gopalan
- Department of Pathology, Memorial Sloan Kettering Cancer Center, New York, NY, USA
| | - Yingbei Chen
- Department of Pathology, Memorial Sloan Kettering Cancer Center, New York, NY, USA
| | - Hikmat Al-Ahmadie
- Department of Pathology, Memorial Sloan Kettering Cancer Center, New York, NY, USA
| | - Samson W. Fine
- Department of Pathology, Memorial Sloan Kettering Cancer Center, New York, NY, USA
| | - Victor E. Reuter
- Department of Pathology, Memorial Sloan Kettering Cancer Center, New York, NY, USA
| | - Satish K. Tickoo
- Department of Pathology, Memorial Sloan Kettering Cancer Center, New York, NY, USA
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Xiu WL, Liu J, Zhang JL, Su N, Wang FJ, Hao XW, Wang FF, Dong Q. Computer-assisted rescue of the inferior mesenteric artery in a child with a giant ganglioneuroblastoma: A case report. World J Gastrointest Surg 2023; 15:984-991. [PMID: 37342847 PMCID: PMC10277949 DOI: 10.4240/wjgs.v15.i5.984] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 12/25/2022] [Revised: 01/22/2023] [Accepted: 03/27/2023] [Indexed: 05/26/2023] Open
Abstract
BACKGROUND Ganglioneuroblastoma (GNB) is a peripheral neuroblastoma (NB) with malignant degree between highly malignant NB and benign ganglioma (GN). Pathology is the gold standard of diagnosis. Although GNB is not uncommon in children, biopsy alone may lead to an inaccurate diagnosis, especially for giant tumors. However, surgical resection may be associated with significant complications. Here, we report a case of computer-assisted surgical resection of a giant GNB in a child and successful rescue of the inferior mesenteric artery.
CASE SUMMARY A 4-year-old girl was admitted to our department for a giant retroperitoneal lesion, which was considered to be an NB by her local hospital. The symptoms of the girl disappeared spontaneously without treatment. On physical examination, a mass of about 10 cm × 7 cm could be palpated in her abdomen. Ultrasonography and contrast-enhanced computed tomography performed in our hospital also showed an NB, and there was a very thick blood vessel inside the tumor. However, aspiration biopsy revealed GN. Surgical resection is the best treatment option for this giant benign tumor. For precise preoperative evaluation, three-dimensional reconstruction was performed. It was clear that the tumor was close to the abdominal aorta. The superior mesenteric vein was pushed forward, and the inferior mesenteric artery passed through the tumor. Because GN generally does not invade blood vessels, we split the tumor with a CUSA knife during the operation and found that there was indeed a straight and intact vascular sheath. Arterial pulsation was observed in the completely exposed inferior mesenteric artery. The pathologists interpreting the tissue finally diagnosed it as a mixed GNB (GNBi), which is more malignant than GN. However, both GN and GNBi usually have a good prognosis.
CONCLUSION This was a case of successful surgical resection of a giant GNB, and aspiration biopsy underestimated the pathological staging of the tumor. Preoperative three-dimensional reconstruction assisted with the radical resection of the tumor and rescue of the inferior mesenteric artery.
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Affiliation(s)
- Wen-Li Xiu
- Department of Pediatric Surgery, The Affiliated Hospital of Qingdao University, Qingdao 266000, Shandong Province, China
- Shandong Provincial Key Laboratory of Digital Medicine and Computer-assisted Surgery, Shandong College Collaborative Innovation Center of Digital Medicine Clinical Treatment and Nutrition Health, Qingdao University, Qingdao 266003, Shandong Province, China
| | - Jie Liu
- Department of Pediatric Surgery, Yijishan Hospital of Wannan Medical College, Wuhu 241000, Anhui Province, China
| | - Jing-Li Zhang
- Department of Pediatric Surgery, The Affiliated Hospital of Qingdao University, Qingdao 266000, Shandong Province, China
| | - Nan Su
- Department of Pediatric Surgery, The Affiliated Hospital of Qingdao University, Qingdao 266000, Shandong Province, China
| | - Feng-Jiao Wang
- Shandong Provincial Key Laboratory of Digital Medicine and Computer-assisted Surgery, Shandong College Collaborative Innovation Center of Digital Medicine Clinical Treatment and Nutrition Health, Qingdao University, Qingdao 266003, Shandong Province, China
| | - Xi-Wei Hao
- Department of Pediatric Surgery, The Affiliated Hospital of Qingdao University, Qingdao 266000, Shandong Province, China
| | - Fei-Fei Wang
- Shandong Provincial Key Laboratory of Digital Medicine and Computer-assisted Surgery, Shandong College Collaborative Innovation Center of Digital Medicine Clinical Treatment and Nutrition Health, Qingdao University, Qingdao 266003, Shandong Province, China
| | - Qian Dong
- Department of Pediatric Surgery, The Affiliated Hospital of Qingdao University, Qingdao 266000, Shandong Province, China
- Shandong Provincial Key Laboratory of Digital Medicine and Computer-assisted Surgery, Shandong College Collaborative Innovation Center of Digital Medicine Clinical Treatment and Nutrition Health, Qingdao University, Qingdao 266003, Shandong Province, China
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Montante C, Fabozzi F, Villani MF, D’Andrea ML, Stracuzzi A, Natali GL, Del Baldo G, Del Bufalo F, Garganese MC, Serra A, Tomà P, Alaggio R, Vennarini S, Colafati GS, Mastronuzzi A, De Ioris MA. The Pitfall of Ganglioneuroblastoma-Nodular Diagnosis: Clinical and Imaging Considerations over a Rare Bifocal Sporadic Case. Diagnostics (Basel) 2022; 12:3221. [PMID: 36553228 PMCID: PMC9777706 DOI: 10.3390/diagnostics12123221] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 11/23/2022] [Revised: 12/14/2022] [Accepted: 12/15/2022] [Indexed: 12/23/2022] Open
Abstract
Neuroblastic tumors (NTs) represent the most common extracranial neoplasm occurring in childhood. Although ganglioneuroblastoma intermixed (GNBI) and ganglioneuroma (GN) are classified as very low-risk tumors, neuroblastoma (NB) and ganglioneuroblastoma-nodular (GNBN) may represent a serious risk to survival. Unfortunately, areas of GNBI and GNBN can coexist in the same mass, leading to incorrect risk staging when only biopsy is performed. Herein, we describe a case of multifocal NT (thoracic and abdominal localization) occurring in a 4-year-old male. Different histological subtypes, namely GNBI and GNBN, were revealed in the two lesions. We focus on the difficulties of proper diagnosis and risk stratification, underlining the usefulness of several diagnostic tools for appropriate management and therapeutic choices.
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Affiliation(s)
- Claudio Montante
- Department of Onco-Hematology, Cell and Gene Therapies, Bambino Gesù Children’s Hospital, IRCCS, 00165 Rome, Italy
| | - Francesco Fabozzi
- Department of Onco-Hematology, Cell and Gene Therapies, Bambino Gesù Children’s Hospital, IRCCS, 00165 Rome, Italy
| | | | - Maria Luisa D’Andrea
- Department of Imaging, Bambino Gesù Children’s Hospital, IRCCS, 00165 Rome, Italy
| | - Alessandra Stracuzzi
- Department of Pathology, Bambino Gesù Children’s Hospital, IRCCS, 00165 Rome, Italy
| | - Gian Luigi Natali
- Department of Imaging, Bambino Gesù Children’s Hospital, IRCCS, 00165 Rome, Italy
| | - Giada Del Baldo
- Department of Onco-Hematology, Cell and Gene Therapies, Bambino Gesù Children’s Hospital, IRCCS, 00165 Rome, Italy
| | - Francesca Del Bufalo
- Department of Onco-Hematology, Cell and Gene Therapies, Bambino Gesù Children’s Hospital, IRCCS, 00165 Rome, Italy
| | | | - Annalisa Serra
- Department of Onco-Hematology, Cell and Gene Therapies, Bambino Gesù Children’s Hospital, IRCCS, 00165 Rome, Italy
| | - Paolo Tomà
- Department of Imaging, Bambino Gesù Children’s Hospital, IRCCS, 00165 Rome, Italy
| | - Rita Alaggio
- Department of Pathology, Bambino Gesù Children’s Hospital, IRCCS, 00165 Rome, Italy
| | - Sabina Vennarini
- Pediatric Radiotherapy Unit, Fondazione IRCCS Istituto Nazionale Tumori, 20133 Milan, Italy
| | | | - Angela Mastronuzzi
- Department of Onco-Hematology, Cell and Gene Therapies, Bambino Gesù Children’s Hospital, IRCCS, 00165 Rome, Italy
| | - Maria Antonietta De Ioris
- Department of Onco-Hematology, Cell and Gene Therapies, Bambino Gesù Children’s Hospital, IRCCS, 00165 Rome, Italy
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Chandrahas S, Meller J. Free floating abdominal ganglioneuroblastoma in a 3 year old male. JOURNAL OF PEDIATRIC SURGERY CASE REPORTS 2022; 83:102332. [DOI: 10.1016/j.epsc.2022.102332] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/16/2022] Open
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7
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Gaddipati R, Ma J, Dayawansa S, Shan Y, Huang JH, Garrett D, Qaiser R. Lumbar Ganglioneuroma Presenting With Scoliosis. Cureus 2021; 13:e16794. [PMID: 34513400 PMCID: PMC8405311 DOI: 10.7759/cureus.16794] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 07/18/2021] [Accepted: 07/30/2021] [Indexed: 11/05/2022] Open
Abstract
Ganglioneuromas are rare, benign tumors arising from the sympathetic nervous system. The presentation of the tumor is variable and may be associated with scoliosis. Few reports of ganglioneuroma associated with scoliosis- exist and most involve the thoracic spine. Here, we present a 13-year-old female with scoliosis who was found to have a lumbar ganglioneuroma. The patient was treated with a subtotal resection and lumbar spinal fusion to correct her scoliosis in a single-stage operation. The patient's symptoms and scoliosis markedly improved following treatment without any complications. Additionally, we conducted an up-to-date literature review of ganglioneuromas associated with scoliosis that have been published in the last 20 years. We discuss variations in clinical presentation and surgical approach.
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Affiliation(s)
- Ravi Gaddipati
- Neurosurgery, Baylor Scott and White Medical Center - Temple, Temple, USA
| | - Joanna Ma
- Neurosurgery, Baylor Scott and White Medical Center - Temple, Temple, USA
| | - Samantha Dayawansa
- Neurosurgery, Baylor Scott and White Medical Center - Temple, Temple, USA
| | - Yuan Shan
- Pathology, Baylor Scott and White Medical Center - Temple, Temple, USA
| | - Jason H Huang
- Neurosurgery, Baylor Scott and White Medical Center - Temple, Temple, USA
| | - David Garrett
- Neurosurgery, Baylor Scott and White Medical Center - Temple, Temple, USA
| | - Rabia Qaiser
- Neurosurgery, Baylor Scott and White Medical Center - Temple, Temple, USA
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8
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Ma R, Wang Y, He W. Primary orbital ganglioneuroblastoma: A case report. Open Med (Wars) 2021; 16:1076-1081. [PMID: 34307890 PMCID: PMC8284332 DOI: 10.1515/med-2021-0230] [Citation(s) in RCA: 1] [Impact Index Per Article: 0.3] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 09/30/2020] [Revised: 12/08/2020] [Accepted: 12/19/2020] [Indexed: 02/05/2023] Open
Abstract
Background Ganglioneuroblastoma (GNB) is a neoplasm that arises from the primitive cells of the sympathetic nervous system during childhood. The current case is very unique because of the initial primary tumor manifestation in the orbit and an adrenal tumor being observed later during follow-up. Case presentation A 2-year-old girl presented to the Ophthalmology Department of our hospital complaining of swelling of the left upper eyelid for approximately one month. Orbital computed tomography (CT) revealed a left orbital mass with bone destruction. Thoracic and abdominal CT indicated no abnormalities. The mass was surgically resected, and histopathological analysis confirmed it as GNB. During follow-up, abdominal CT detected an adrenal tumor with internal calcification, a calcified nodule on the left side of the abdominal aorta, and mesenteric lymph nodes. Accordingly, primary orbital GNB and metastatic adrenal GNB were the possible considerations. We removed the adrenal tumor, and the patient underwent chemotherapy. However, the patient died 18 months after the ophthalmic surgery. Conclusion Primary orbital GNB in children is easily misdiagnosed because of its rare occurrence and atypical clinical findings. Imaging methods combined with histopathological examination contribute to the detection and diagnosis of primary and metastatic GNBs. Thus, timely surgery combined with adjuvant chemotherapy and long-term follow-up is essential for controlling the metastasis of GNB and improving the survival rate of patients.
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Affiliation(s)
- Ruixin Ma
- Department of Ophthalmology, Ophthalmic Laboratory, West China Hospital, Sichuan University, Chengdu 610041, Sichuan, China
| | - Yujiao Wang
- Department of Ophthalmology, Ophthalmic Laboratory, West China Hospital, Sichuan University, Chengdu 610041, Sichuan, China
| | - Weimin He
- Department of Ophthalmology, Ophthalmic Laboratory, West China Hospital, Sichuan University, Chengdu 610041, Sichuan, China
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Staged resection of a bilateral thoracic and bilateral adrenal neuroblastoma. JOURNAL OF PEDIATRIC SURGERY CASE REPORTS 2020. [DOI: 10.1016/j.epsc.2020.101599] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/22/2022] Open
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10
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Boumaza K, Michel G, Salaud C, Bossard C, Espitalier F, Malard O. Peripheral neck nerve tumor: A 73-case study and literature review. Eur Ann Otorhinolaryngol Head Neck Dis 2019; 136:455-460. [PMID: 31537489 DOI: 10.1016/j.anorl.2019.09.005] [Citation(s) in RCA: 2] [Impact Index Per Article: 0.3] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 12/18/2022]
Abstract
OBJECTIVES Peripheral neck nerve tumors are rare and mostly benign neoplasms. The exceptional malignant forms are very aggressive, and diagnosis is difficult. The objective of this study was to evaluate diagnostic and therapeutic management and identify possible predictive factors. MATERIAL AND METHODS A retrospective study was conducted of 73 patients treated for peripheral neck nerve tumor between 1995 and 2015. RESULTS Mean age was 44years. The main presenting symptom was a cervical mass, isolated or associated with signs related to the affected nerve structure. Diagnosis was suspected by slow progression of a firm mass, featuring T1 hyposignal and T2 hypersignal on magnetic resonance imaging. Surgery was performed in 99% of cases, completed by adjuvant chemotherapy in case of malignant neuroblastic tumor. Type 1 neurofibromatosis and sudden increase in mass with or without associated pain suggested malignant transformation. Age below 10years suggested neuroblastic tumor. CONCLUSION Neck nerve tumors are very often benign with low degenerative potential. Surgery is the treatment of choice after risk/benefit analysis. However, there is no clearly defined consensus regarding the timing of surgery for these lesions.
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Affiliation(s)
- K Boumaza
- Service d'ORL et de Chirurgie Cervico-Faciale, CHU de Nantes Hôtel-Dieu, 1, place A. Ricordeau, BP 1005, 44093 Nantes Cedex 01, France
| | - G Michel
- Service d'ORL et de Chirurgie Cervico-Faciale, CHU de Nantes Hôtel-Dieu, 1, place A. Ricordeau, BP 1005, 44093 Nantes Cedex 01, France
| | - C Salaud
- Service de Neurotraumatologie/Neurochirugie, Centre Hospitalier Universitaire de Nantes, Hôtel-Dieu, 1, place A. Ricordeau, BP 1005, 44093 Nantes Cedex 01, France
| | - C Bossard
- Service d'Anatomopathologie, Centre Hospitalier Universitaire de Nantes, Hôtel-Dieu, 1, place A. Ricordeau, BP 1005, 44093 Nantes Cedex 01, France
| | - F Espitalier
- Service d'ORL et de Chirurgie Cervico-Faciale, CHU de Nantes Hôtel-Dieu, 1, place A. Ricordeau, BP 1005, 44093 Nantes Cedex 01, France
| | - O Malard
- Service d'ORL et de Chirurgie Cervico-Faciale, CHU de Nantes Hôtel-Dieu, 1, place A. Ricordeau, BP 1005, 44093 Nantes Cedex 01, France.
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Jiang P, Huang M, Qi W, Wang F, Yang T, Gao T, Luo C, Deng J, Yang Z, Zhou T, Zou Y, Gao G, Yang X. FUBP1 promotes neuroblastoma proliferation via enhancing glycolysis-a new possible marker of malignancy for neuroblastoma. JOURNAL OF EXPERIMENTAL & CLINICAL CANCER RESEARCH : CR 2019; 38:400. [PMID: 31511046 PMCID: PMC6737630 DOI: 10.1186/s13046-019-1414-6] [Citation(s) in RCA: 11] [Impact Index Per Article: 1.8] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Subscribe] [Scholar Register] [Received: 05/25/2019] [Accepted: 09/05/2019] [Indexed: 12/16/2022]
Abstract
Background Neuroblastoma (NB) is one of the deadliest paediatric solid tumours due to its rapid proliferative characteristics. Amplified copies of MYCN are considered the most important marker for the prediction of tumour relapse and progression in NB, but they were only detected in 20–30% of NB patients, indicating there might be other oncogenes in the development of NB. The far upstream element binding protein 1 (FUBP1) was first identified as a transcriptional regulator of the proto-oncogene MYC. However, the expression and role of FUBP1 in NB have not been documented. Methods FUBP1 expression was analysed from GEO database and verified by immunohistochemistry (IHC) and western blotting (WB) in NB tissues and cell lines. Cell proliferation and apoptosis were detected by Cell Counting Kit-8, Colony formation assay, EDU, TUNEL staining and flow cytometric analysis. Several glycolytic metabolites production was confirmed by ELISA and oxygen consuming rate (OCR). Luciferase assay, WB, chromatin immunoprecipitation (CHIP) were used to explore the mechanisms of the effect of FUBP1 on NB. Results FUBP1 mRNA levels were increased along with the increase in International Neuroblastoma Staging System (INSS) stages. High expression of FUBP1 with low N-Myc expression accounted for 44.6% of NB patient samples (n = 65). In addition, FUBP1 protein levels were remarkably increased with NB malignancy in the NB tissue microarray (NB: n = 65; ganglioneuroblastoma: n = 31; ganglioneuroma: n = 27). Furthermore, FUBP1 expression was negatively correlated with patient survival rate but positively correlated with ki67 content. In vitro experiments showed that FUBP1 promotes NB cell proliferation and inhibits cell apoptosis via enhancing glycolysis and ATP production. Mechanistically, FUBP1 inhibited the degradation of HIF1α via downregulation of Von Hippel-Lindau (VHL), the E3 ligase for HIF1α, resulting in upregulation of lactate dehydrogenase isoform B (LDHB) expression to enhance glycolysis. Overexpressed or silenced N-Myc could not regulate FUBP1 or LDHB levels. Conclusions Taken together, our findings demonstrate for the first time that elevated FUBP1 promotes NB glycolysis and growth by targeting HIF1α rather than N-Myc, suggesting that FUBP1 is a novel and powerful oncogene in the development of NB independent of N-Myc and may have potential in the diagnosis and treatment of NB.
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Affiliation(s)
- Ping Jiang
- Program of Molecular Medicine, Affiliated Guangzhou Women and Children's Medical Center, Zhongshan School of Medicine, Sun Yat-sen University, Guangzhou, China.,Department of Biochemistry, Zhongshan School of Medicine, Sun Yat-sen University, 74 Zhongshan 2nd Road, Guangzhou, 510080, China
| | - Mao Huang
- Program of Molecular Medicine, Affiliated Guangzhou Women and Children's Medical Center, Zhongshan School of Medicine, Sun Yat-sen University, Guangzhou, China.,Department of Biochemistry, Zhongshan School of Medicine, Sun Yat-sen University, 74 Zhongshan 2nd Road, Guangzhou, 510080, China
| | - Weiwei Qi
- Department of Biochemistry, Zhongshan School of Medicine, Sun Yat-sen University, 74 Zhongshan 2nd Road, Guangzhou, 510080, China
| | - Fenghua Wang
- Program of Molecular Medicine, Affiliated Guangzhou Women and Children's Medical Center, Zhongshan School of Medicine, Sun Yat-sen University, Guangzhou, China
| | - Tianyou Yang
- Program of Molecular Medicine, Affiliated Guangzhou Women and Children's Medical Center, Zhongshan School of Medicine, Sun Yat-sen University, Guangzhou, China
| | - Tianxiao Gao
- Department of Medical Oncology, Sun Yat-sen University Cancer Center, Guangzhou, China
| | - Chuanghua Luo
- Department of Biochemistry, Zhongshan School of Medicine, Sun Yat-sen University, 74 Zhongshan 2nd Road, Guangzhou, 510080, China
| | - Jing Deng
- Department of Biochemistry, Zhongshan School of Medicine, Sun Yat-sen University, 74 Zhongshan 2nd Road, Guangzhou, 510080, China
| | - Zhonghan Yang
- Department of Biochemistry, Zhongshan School of Medicine, Sun Yat-sen University, 74 Zhongshan 2nd Road, Guangzhou, 510080, China
| | - Ti Zhou
- Department of Biochemistry, Zhongshan School of Medicine, Sun Yat-sen University, 74 Zhongshan 2nd Road, Guangzhou, 510080, China
| | - Yan Zou
- Program of Molecular Medicine, Affiliated Guangzhou Women and Children's Medical Center, Zhongshan School of Medicine, Sun Yat-sen University, Guangzhou, China
| | - Guoquan Gao
- Program of Molecular Medicine, Affiliated Guangzhou Women and Children's Medical Center, Zhongshan School of Medicine, Sun Yat-sen University, Guangzhou, China. .,Department of Biochemistry, Zhongshan School of Medicine, Sun Yat-sen University, 74 Zhongshan 2nd Road, Guangzhou, 510080, China. .,Guangdong Engineering & Technology Research Center for Gene Manipulation and Biomacromolecular Products, Sun Yat-sen University, Guangzhou, China.
| | - Xia Yang
- Program of Molecular Medicine, Affiliated Guangzhou Women and Children's Medical Center, Zhongshan School of Medicine, Sun Yat-sen University, Guangzhou, China. .,Department of Biochemistry, Zhongshan School of Medicine, Sun Yat-sen University, 74 Zhongshan 2nd Road, Guangzhou, 510080, China. .,Guangdong Province Key Laboratory of Brain Function and Disease, Zhongshan School of Medicine, Sun Yat-sen University, Guangzhou, China.
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Abstract
INTRODUCTION Neuroblastoma ranks third among pediatric malignancies. CASE REPORT The case of a 3-year-old child is presented, who suddenly had frequent, unproductive, emetic cough; fever; and weight loss. Lung X-ray showed an opacity situated in the posterior superior mediastinum. Thoracic ultrasound revealed a slightly inhomogeneous, hypoechoic mass located in the posterior superior mediastinum. Computed tomography evidenced a tumor mass with homogeneous appearance in the costo-vertebral groove. Histological examination confirmed the diagnosis of ganglioneuroblastoma. CONCLUSION Although history and clinical examination provided few elements, diagnosis was made based on imaging and histopathological examination.
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