Approaches to the Treatment of Children with Esthesioneuroblastoma: Literature Review

2019 ◽  
Vol 6 (2) ◽  
pp. 78-86
Author(s):  
Tatiana V. Gorbunova ◽  
Anastasia D. Rodina ◽  
Ruslan V. Shishkov ◽  
Natalia V. Ivanova ◽  
Igor V. Glekov ◽  
...  
Keyword(s):  
Radiation Therapy ◽  
Surgical Treatment ◽  
Tumor Cells ◽  
Tumor Resection ◽  
Neuron Specific Enolase ◽  
Distant Metastases ◽  
Intravenous Contrast ◽  
Adult Age ◽  
Variable Expression ◽  
Molecular Features

The incidence of esthesioneuroblastoma in children under 15 years of age is 0.1 per 100.000 children. Distinctive histological features of this tumor are diffuse accumulation of neuron-specific enolase, synaptophysin, chromogranin, and variable expression of cytokeratins. Diagnosis of the tumor includes endoscopic examination of the nasal cavity and nasopharynx, magnetic resonance imaging (MRI) and computed tomography (CT) of the skull base, paranasal sinuses with intravenous contrast. PET-CT is advisable to use for the detection of regional and distant metastases, as well as for suspected relapse. In patients of adult age, a negative effect on the outcome of the disease was detected, the detection of metastases in the lymph nodes of the neck, the presence of tumor cells at the edges of tumor resection and a high degree of malignancy of the tumor according to the Hyams system. Therapeutic approaches depend on the stage of esthesioneuroblastoma by Kadish. In the A-stage, surgical treatment is advisable. In the presence of tumor cells at the edges of the resection or residual tumor, radiation therapy is performed. In case of B-stage, surgical treatment is combined with the mandatory irradiation of the primary tumor area. In patients with the C-stage, neoadjuvant chemotherapy or radiation is performed, followed by a surgical treatment, adjuvant chemotherapy and/or radiation therapy. Patients with D-stage chemoradiation therapy is indicated. There is no consensus on an effective drug regimen. Overall 5-year survival varies significantly depending on the design of the study — 55% to 98%. Further study of the features of the clinical picture, morphological and molecular features and the course of the disease will help to improve our understanding of the nature of the tumor.

Phase II trial of preoperative radiation therapy and chemotherapy for patients with localized, resectable adenocarcinoma of the pancreas: an Eastern Cooperative Oncology Group Study.

1998 ◽  
Vol 16 (1) ◽  
pp. 317-323 ◽  
Author(s):  
J P Hoffman ◽  
S Lipsitz ◽  
T Pisansky ◽  
J L Weese ◽  
L Solin ◽  
...  
Keyword(s):  
Radiation Therapy ◽  
Eastern Cooperative Oncology Group ◽  
Tumor Resection ◽  
Distant Metastases ◽  
Entire Group ◽  
Peritoneal Cytology ◽  
Preoperative Radiation ◽  
Group Setting ◽  
Local Progression ◽  
Adenocarcinoma Of The Pancreas

PURPOSE A prospective, multiinstitutional trial was initiated in 1991 to examine the tolerance to and efficacy of a program of preoperative chemoradiotherapy (CTRT) and surgical resection for patients with localized adenocarcinoma of the pancreas. PATIENTS AND METHODS Fifty-three patients were assessable for analysis, with a median follow-up of 52 months for survivors. Radiation therapy (RT) totaling 5,040 cGy in 180 cGy fractions with mitomycin 10 mg/m2 day 2 and fluorouracil (5-FU) 1,000 mg/m2/d continuous infusion days 2 through 5 and 29 through 32 were given as preoperative adjuvant therapy. Twelve patients did not proceed to surgery (one death, one toxicity, three local progression, six distant metastases, one intercurrent illness), whereas 41 patients underwent surgery. Of these, 17 patients did not have resection (11, hepatic and/or peritoneal metastases and six local extension that precluded resection). Twenty-four patients had tumor resection (19 Whipple, four total pancreatectomy, one distal pancreatectomy). RESULTS Treatment toxicity was primarily hematologic, although a comparable number suffered biliary tract complications, either from obstruction or cholangitis as a result of an occluded stent or the primary tumor. There was one postoperative death. Median survival for the entire group and for the 24 patients with resection was 9.7 and 15.7 months. This survival rate reflected the advanced state of most resected cancers (positive peritoneal cytology, three patients; margins within 2 mm, 13 patients; involved lymph nodes, four patients; and need for superior mesenteric vein (SMV) resection, four patients). Tumor progression was most frequent at metastatic sites. CONCLUSION This preoperative CTRT protocol was feasible and safe in a cooperative group setting. Entry of patients with advanced tumors probably accounted for the suboptimal resectability and survival results.

scholarly journals Ossifying Fibromyxoid Tumor: An Update

2016 ◽  
Vol 140 (4) ◽  
pp. 371-375 ◽  
Author(s):  
David Bakiratharajan ◽  
Bharat Rekhi
Keyword(s):  
Tumor Resection ◽  
Distant Metastases ◽  
Uncertain Malignant Potential ◽  
Complete Tumor Resection ◽  
Soft Tissue Neoplasm ◽  
Molecular Features ◽  
Characteristic Imaging ◽  
Ossifying Fibromyxoid Tumor ◽  
Complete Tumor

Ossifying fibromyxoid tumor (OFMT) is a rare soft tissue neoplasm of uncertain differentiation, initially described by Enzinger and colleagues. Until now, nearly 300 such cases have been reported worldwide. The histogenesis of these tumors remains controversial. These tumors show characteristic imaging findings and exhibit a spectrum of histopathologic features, including classical and atypical subtypes. Local recurrences and, occasionally, distant metastases have also been reported. A complete tumor resection forms the preferred treatment modality for these tumors, along with follow-up, as these tumors have an uncertain malignant potential. Lately, certain “molecular signatures” underlying OFMTs have been described that can further aid in reaching an accurate diagnosis for these tumors and unraveling their pathogenesis. This article is a review of the clinical, radiologic, histopathologic, and molecular features of OFMTs.

scholarly journals Applying Single-Cell Technology in Uveal Melanomas: Current Trends and Perspectives for Improving Uveal Melanoma Metastasis Surveillance and Tumor Profiling

2021 ◽  
Vol 7 ◽  
Author(s):  
Mona Meng Wang ◽  
Chuanfei Chen ◽  
Myoe Naing Lynn ◽  
Carlos R. Figueiredo ◽  
Wei Jian Tan ◽  
...  
Keyword(s):  
Single Cell ◽  
Tumor Cells ◽  
Uveal Melanoma ◽  
Vision Loss ◽  
Single Cells ◽  
Chromosomal Rearrangements ◽  
Distant Metastases ◽  
Current Approach ◽  
Melanoma Metastasis ◽  
Molecular Features

Uveal melanoma (UM) is the most common primary adult intraocular malignancy. This rare but devastating cancer causes vision loss and confers a poor survival rate due to distant metastases. Identifying clinical and molecular features that portend a metastatic risk is an important part of UM workup and prognostication. Current UM prognostication tools are based on determining the tumor size, gene expression profile, and chromosomal rearrangements. Although we can predict the risk of metastasis fairly accurately, we cannot obtain preclinical evidence of metastasis or identify biomarkers that might form the basis of targeted therapy. These gaps in UM research might be addressed by single-cell research. Indeed, single-cell technologies are being increasingly used to identify circulating tumor cells and profile transcriptomic signatures in single, drug-resistant tumor cells. Such advances have led to the identification of suitable biomarkers for targeted treatment. Here, we review the approaches used in cutaneous melanomas and other cancers to isolate single cells and profile them at the transcriptomic and/or genomic level. We discuss how these approaches might enhance our current approach to UM management and review the emerging data from single-cell analyses in UM.

LOCAL HYPERTHERMIA IN TREATMENT FOR MALIGNANT BRAIN TUMORS

2018 ◽  
Vol 64 (1) ◽  
pp. 54-61
Author(s):  
A. Ryabova ◽  
O. Gribova ◽  
V. Novikov ◽  
E. Choinzonov ◽  
Zh. Starceva ◽  
...  
Keyword(s):  
Experimental Data ◽  
Radiation Therapy ◽  
Brain Tumors ◽  
Tumor Cells ◽  
Combined Treatment ◽  
Complex Treatment ◽  
Local Hyperthermia ◽  
Malignant Brain Tumors ◽  
Sensitizing Effect ◽  
Methods Of Treatment

Unsatisfactory results of complex treatment for malignant brain tumors stimulate search of new effective methods of treatment. Radiation therapy is an integral part of the combined treatment but often does not influence lethally on resistant tumor cells. Thereby in recent decades there has been an active search for different modifiers, which can increase the sensitivity of tumors to chemotherapy and radiotherapy. One of the universal sensitizers is the local hyperthermia. Experimental data showed that the effect of high temperatures had both a direct damaging effect on tumor cells and a sensitizing effect. The literature review given in the article provides an overview of the existing methods of the local hyperthermia for brain tumors treatment.

scholarly journals NCMP-13. LEPTOMENINGEAL DISEASE FOLLOWING RESECTION OF INTRAVENTRICULAR MELANOMA METASTASIS DIAGNOSED THROUGH CIRCULATING TUMOR CELLS

2020 ◽  
Vol 22 (Supplement_2) ◽  
pp. ii125-ii125
Author(s):  
Christopher Wang ◽  
Melissa Limia ◽  
Peter Forsyth ◽  
James Liu
Keyword(s):  
Early Intervention ◽  
Tumor Cells ◽  
Circulating Tumor Cells ◽  
Surgical Resection ◽  
Tumor Resection ◽  
Leptomeningeal Disease ◽  
Melanoma Metastasis ◽  
Tumor Seeding ◽  
Csf Analysis ◽  
Dismal Prognosis

Abstract Leptomeningeal disease in the setting of malignant melanoma metastatic to the brain provides a dismal prognosis. The relationship between intraventricular metastatic tumor seeding following surgical resection and development of leptomeningeal disease (LMD) is not completely clear, although there appears to be correlation. While the mechanisms that drive the development of LMD is not well understood, monitoring of cerebrospinal fluid (CSF) for circulating tumor cells (CTCs) in high risk patients may allow for early intervention for LMD prior to radiographical diagnosis. This report describes a patient with metastatic melanoma who developed ventricular trapping from an intraventricular melanoma metastasis. The patient underwent endoscopic assisted resection of the tumor. Due to concern for leptomeningeal seeding given the location of the tumor and use of surgical resection, CSF analysis was performed. CTC count was increased shortly after surgical resection along with cytology that was suspicious for malignancy. Due to the increase in CTCs, the patient was treated for LMD with whole brain radiation therapy and intrathecal pembrolizumab. Subsequent CSF analysis revealed clearing of malignant cells in the CSF. The patient developed symptoms consistent with LMD approximately 9 months after the surgery and died 21 months after resection of his brain metastasis. This case illustrates a rare occurrence of an intraventricular melanoma metastasis, and the use of CTC presence within the CSF to diagnose LMD for early intervention. This emphasizes that the risk of developing LMD must be considered with intraventricular metastasis or ventricular exposure during tumor resection, and that CTCs may be an effective factor to monitor for early development of disease with possible prolonged survival benefit.

scholarly journals Cancer of Unknown Primary: Challenges and Progress in Clinical Management

Cancers ◽  
2021 ◽  
Vol 13 (3) ◽  
pp. 451
Author(s):  
Noemi Laprovitera ◽  
Mattia Riefolo ◽  
Elisa Ambrosini ◽  
Christiane Klec ◽  
Martin Pichler ◽  
...  
Keyword(s):  
Current Knowledge ◽  
Distant Metastases ◽  
Cancer Of Unknown Primary ◽  
Unknown Primary ◽  
Diagnostic Strategies ◽  
Molecular Features ◽  
Tissue Of Origin ◽  
Novel Therapeutic Strategies ◽  
Aggressive Clinical Behavior

Distant metastases are the main cause of cancer-related deaths in patients with advanced tumors. A standard diagnostic workup usually contains the identification of the tissue-of-origin of metastatic tumors, although under certain circumstances, it remains elusive. This disease setting is defined as cancer of unknown primary (CUP). Accounting for approximately 3–5% of all cancer diagnoses, CUPs are characterized by an aggressive clinical behavior and represent a real therapeutic challenge. The lack of determination of a tissue of origin precludes CUP patients from specific evidence-based therapeutic options or access to clinical trial, which significantly impacts their life expectancy. In the era of precision medicine, it is essential to characterize CUP molecular features, including the expression profile of non-coding RNAs, to improve our understanding of CUP biology and identify novel therapeutic strategies. This review article sheds light on this enigmatic disease by summarizing the current knowledge on CUPs focusing on recent discoveries and emerging diagnostic strategies.

scholarly journals RONC-19. TWO CASES OF RE-IRRADIATION FOR LATE RECURRENT OR RADIATION-INDUCED TUMOR AFTER RADIATION THERAPY FOR PEDIATRIC BRAIN TUMORS

2020 ◽  
Vol 22 (Supplement_3) ◽  
pp. iii459-iii459
Author(s):  
Takashi Mori ◽  
Shigeru Yamaguchi ◽  
Rikiya Onimaru ◽  
Takayuki Hashimoto ◽  
Hidefumi Aoyama
Keyword(s):  
Radiation Therapy ◽  
Brain Tumors ◽  
Tumor Resection ◽  
Intensity Modulated Radiation Therapy ◽  
Late Recurrence ◽  
Pediatric Brain Tumors ◽  
Suprasellar Region ◽  
Radiation Induced ◽  
Pediatric Brain

Abstract BACKGROUND As the outcome of pediatric brain tumors improves, late recurrence and radiation-induced tumor cases are more likely to occur, and the number of cases requiring re-irradiation is expected to increase. Here we report two cases performed intracranial re-irradiation after radiotherapy for pediatric brain tumors. CASE 1: 21-year-old male. He was diagnosed with craniopharyngioma at eight years old and underwent a tumor resection. At 10 years old, the local recurrence of suprasellar region was treated with 50.4 Gy/28 fr of stereotactic radiotherapy (SRT). After that, other recurrent lesions appeared in the left cerebellopontine angle, and he received surgery three times. The tumor was gross totally resected and re-irradiation with 40 Gy/20 fr of SRT was performed. We have found no recurrence or late effects during the one year follow-up. CASE 2: 15-year-old female. At three years old, she received 18 Gy/10 fr of craniospinal irradiation and 36 Gy/20 fr of boost to the posterior fossa as postoperative irradiation for anaplastic ependymoma and cured. However, a anaplastic meningioma appeared on the left side of the skull base at the age of 15, and 50 Gy/25 fr of postoperative intensity-modulated radiation therapy was performed. Two years later, another meningioma developed in the right cerebellar tent, and 54 Gy/27 fr of SRT was performed. Thirty-three months after re-irradiation, MRI showed a slight increase of the lesion, but no late toxicities are observed. CONCLUSION The follow-up periods are short, however intracranial re-irradiation after radiotherapy for pediatric brain tumors were feasible and effective.

scholarly journals A poor prognostic metastatic nongestational choriocarcinoma of the ovary: a case report and the literature review

2021 ◽  
Vol 14 (1) ◽  
Author(s):  
Kimihiro Nishino ◽  
Eiko Yamamoto ◽  
Yoshiki Ikeda ◽  
Kaoru Niimi ◽  
Toshimichi Yamamoto ◽  
...  
Keyword(s):  
Tumor Cells ◽  
Tumor Resection ◽  
Left Lung ◽  
Str Analysis ◽  
Case Presentation ◽  
Gestational Choriocarcinoma ◽  
Mucosal Cells ◽  
Malignant Germ Cell Tumor ◽  
Brain Tumor Resection ◽  
Short Tandem

Abstract Background Pure ovarian choriocarcinoma can be gestational or nongestational in origin. Nongestational pure ovarian choriocarcinoma is extremely rare and the prognosis is thought to be worse than that of the gestational type in patients with metastatic disease. We present a case of metastatic pure ovarian choriocarcinoma with poor prognosis in which the origin was identified as nongestational by DNA short tandem repeat (STR) analysis. Case presentation A nulliparous woman in her thirties with metastatic choriocarcinoma was referred to our hospital after initial treatment proved unsuccessful. Two months earlier, she had undergone brain tumor resection and histological examination confirmed choriocarcinoma. Serum human chorionic gonadotropin (hCG) concentration at initial diagnosis was 5030 IU/L. Two cycles of a combination chemotherapy regimen of methotrexate, etoposide, and actinomycin-D (MEA therapy), which is commonly used for gestational choriocarcinoma, was administered. However, the disease could not be controlled. Imaging modalities at presentation revealed tumor present in the left ovary and left lung, but not in the uterus, which led us think that the choriocarcinoma was nongestational. Bleomycin, etoposide, and cisplatin (BEP therapy) which is commonly used for nongestational choriocarcinoma (malignant germ cell tumor) and surgical resection of the uterus, bilateral ovaries, and an affected part of the left lung led to the nadir level of hCG, but the tumor relapsed and levels of hCG again increased. To investigate the origin of choriocarcinoma, we performed DNA STR analysis of tumor cells and oral mucosal cells. Analysis revealed the origin of the choriocarcinoma as nongestational, as the genotype of tumor cells entirely corresponded with that of oral mucosal cells. BEP therapy and chemotherapy regimens administered for nongestational choriocarcinoma and gestational choriocarcinoma proved ineffective, and the patient died 21 months after diagnosis of metastatic choriocarcinoma. Conclusion Metastaic nongestational pure choriocarcinoma of ovary is an extremely rare and an aggressive disease, frequently resulting in poor outcome.

scholarly journals Hyperthermia in a pediatric patient with neuroblastoma during anesthesia: a case report

BMC Surgery ◽  
2021 ◽  
Vol 21 (1) ◽  
Author(s):  
Chuan Wang ◽  
Wenqiong Xin ◽  
Yi Ji
Keyword(s):  
Tumor Resection ◽  
Neuron Specific Enolase ◽  
Tracheal Extubation ◽  
Posterior Mediastinum ◽  
Gas Analysis ◽  
Vanillylmandelic Acid ◽  
Management Options ◽  
Paravertebral Space ◽  
Tissue Mass ◽  
The Right

Abstract Background Neuroblastoma is the most common malignant extracranial solid tumor in pediatrics patients. Intraoperative hyperthermia is extremely rare in patients with neuroblastoma and can cause a series of complications. Here, we represent a case of neuroblastoma accompanied by hyperthermia during anesthesia, and propose a rational explanation and management options. Case presentation The patient had gait disturbance and sitting-related pain without fever. Magnetic resonance imaging revealed a soft tissue mass located in the right posterior mediastinum, paravertebral space and canalis vertebralis. Serum tumor marker screening showed that the patient had increased epinephrine, norepinephrine and neuron specific enolase levels, with an increased 24 hour urine vanillylmandelic acid level. Intraspinal tumor resection was conducted. The temperature of the patient rapidly arose to 40.1 °C over 10 minutes when waiting for tracheal extubation. The arterial gas analysis results indicated malignant hyperthermia was less likely, and dantrolene was not administered. Physical cooling methods were used, and the temperature dropped to 38.6 ℃. The trachea was successfully extubated. Histological results confirmed the diagnosis of neuroblastoma. Conclusions Hyperthermia during anesthesia is a serious adverse event. Catecholamines secreted from neuroblatoma cells can lead to hypermetabolism and hyperthermia. Surgeons and anesthesiologists should be aware of the possibility of hyperthermia in patients with neuroblastoma.

Therapy and regeneration-combined bone cements for minimally invasive treatment of neoplastic bone defects

2021 ◽  
Author(s):  
Yu Qu ◽  
Hui Zhuang ◽  
Meng Zhang ◽  
Yufeng Wang ◽  
Dong Zhai ◽  
...  
Keyword(s):  
Calcium Phosphate ◽  
Minimally Invasive ◽  
Tumor Cells ◽  
Bone Tumor ◽  
Tumor Resection ◽  
Bone Defects ◽  
Bone Cements ◽  
Invasive Treatment ◽  
Calcium Phosphate Cements ◽  
Bone Tumor Resection

Although calcium phosphate cements (CPC) have been clinically used to repair bone defects caused by bone tumor resection, traditional CPC cannot kill the remaining tumor cells after surgery and prevent...

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