Lessons Learnt in Health Management: Paediatric Spinal Tumours: A 6-year Retrospective Review from Central India

2021 ◽  
pp. 097206342110504
Author(s):  
Pranati Sharma ◽  
Shrikant V. Rege ◽  
Nilesh Jain
Keyword(s):  
Rare Disease ◽  
Surgical Resection ◽  
Medical Records ◽  
Spinal Metastasis ◽  
Public Awareness ◽  
Survival Rates ◽  
Central India ◽  
Neurological Status ◽  
Histopathological Diagnosis ◽  
Spinal Tumours

Background: From the management perspective, rare medical conditions do not attract enough attention, though their management can often be resource intensive. Increased awareness among health professionals can lead to early diagnosis of cases and prevention of complications leading to improved outcomes. Paediatric spinal tumours are rare disease entities with an annual incidence of approximately 1 per 1 million children. The profile of spinal tumours in the paediatric population is significantly different than that in adults. In this study we retrospectively analyse a heterogenous variety of paediatric spinal tumours which were treated at our institution over the last 6 years, with the goal of contributing to existing knowledge of this relatively rare disease entity. Methods: This retrospective study includes paediatric patients (under 18 years of age) who underwent surgery for primary spinal tumours from 2014 to 2019. The medical records were reviewed retrospectively and the information regarding clinical presentation, tumour location, operative findings, and postoperative status and functional outcome were analysed. The modified McCormick grade was used to assess the neurological status. Patients with spinal metastasis or incomplete medical records were excluded from the study. Results: Of 74 patients with primary spinal tumours operated at our centre between 2014 to 2019, a total of 8 patients (5 males and 3 females) who met the inclusion criteria were identified for the present study. The mean age of the patients included was 12.1±5.3 and the median follow-up period was 24 months. An improvement in the neurological status, as assessed by the modified McCormick grade, was seen in 37.5% of the patients. Three of the patients received adjuvant radiotherapy, post-surgical resection. A unique feature of this series was the wide variety of cases encountered, as each case had a different histopathological diagnosis, despite being limited by its sample size. Conclusion: Primary paediatric spinal cord tumours are rare, and surgical resection remains the treatment of choice. Adjuvant therapy is warranted in cases of high-grade lesions or recurrences. Through this study, we realised that due to its relative rarity and low incidence, a lack of public awareness can often lead to disproportionately increased morbidity and mortality. Prospective multi-centric studies can provide tools to help develop future management strategies for improved survival rates and reduced complications.

Lipomeningocele associated with diplomyelia in a dog

2018 ◽  
Vol 46 (05) ◽  
pp. 323-329 ◽  
Author(s):  
Nele Ondreka ◽  
Sara Malberg ◽  
Emma Laws ◽  
Martin Schmidt ◽  
Sabine Schulze
Keyword(s):  
Spinal Cord ◽  
Neurological Status ◽  
Split Cord Malformation ◽  
Lumbar Spinal Cord ◽  
Histopathological Diagnosis ◽  
Type I ◽  
Thoracic Spinal Cord ◽  
Thoracic Spinal ◽  
Subcutaneous Mass ◽  
Lumbar Spinal

SummaryA 2-year-old male neutered mixed breed dog with a body weight of 30 kg was presented for evaluation of a soft subcutaneous mass on the dorsal midline at the level of the caudal thoracic spine. A further clinical sign was intermittent pain on palpation of the area of the subcutaneous mass. The owner also described a prolonged phase of urination with repeated interruption and re-initiation of voiding. The findings of the neurological examination were consistent with a lesion localization between the 3rd thoracic and 3rd lumbar spinal cord segments. Magnetic resonance imaging revealed a spina bifida with a lipomeningocele and diplomyelia (split cord malformation type I) at the level of thoracic vertebra 11 and 12 and secondary syringomyelia above the aforementioned defects in the caudal thoracic spinal cord. Surgical resection of the lipomeningocele via a hemilaminectomy was performed. After initial deterioration of the neurological status postsurgery with paraplegia and absent deep pain sensation the dog improved within 2 weeks to non-ambulatory paraparesis with voluntary urination. Six weeks postoperatively the dog was ambulatory, according to the owner. Two years after surgery the owner recorded that the dog showed a normal gait, a normal urination and no pain. Histopathological diagnosis of the biopsied material revealed a lipomeningocele which confirmed the radiological diagnosis.

MOLECULAR AND GENETIC SUBTYPES OF OVARIAN CANCER: PROSPECTS FOR FURTHER STUDIES

2019 ◽  
Vol 65 (1) ◽  
pp. 56-62
Author(s):  
Alisa Villert ◽  
Larisa Kolomiets ◽  
Natalya Yunusova ◽  
Yevgeniya Fesik
Keyword(s):  
Ovarian Cancer ◽  
Ovarian Carcinoma ◽  
Molecular Subtypes ◽  
Survival Rates ◽  
Consensus Algorithm ◽  
Histopathological Diagnosis ◽  
Low Grade ◽  
High Grade ◽  
Ovarian Carcinomas ◽  
Genetic Subtypes

High-grade ovarian carcinoma is a histopathological diagnosis, however, at the molecular level, ovarian cancer represents a heterogeneous group of diseases. Studies aimed at identifying molecular genetic subtypes of ovarian cancer are conducted in order to find the answer to the question: can different molecular subgroups influence the choice of treatment? One of the achievements in this trend is the recognition of the dualistic model that categorizes various types of ovarian cancer into two groups designated high-grade (HG) and low-grade (LG) tumors. However, the tumor genome sequencing data suggest the existence of 6 ovarian carcinoma subtypes, including two LG and four HG subtypes. Subtype C1 exhibits a high stromal response and the lowest survival. Subtypes C2 and C4 demonstrate higher number of intratumoral CD3 + cells, lower stromal gene expression and better survival than sybtype C1. Subtype C5 (mesenchymal) is characterized by mesenchymal cells, over-expression of N-cadherin and P-cadherin, low expression of differentiation markers, and lower survival rates than C2 and C4. The use of a consensus algorithm to determine the subtype allows identification of only a minority of ovarian carcinomas (approximately 25%) therefore, the practical importance of this classification requires additional research. There is evidence that it makes sense to randomize tumors into groups with altered expression of angiogenic genes and groups with overexpression of the immune response genes, as in the angiogenic group there is a comparative superiority in terms of survival. The administration of bevacizumab in the angiogenic group improves survival, while the administration of bevacizumab in the immune group even worsens the outcome. Molecular subtypes with worse survival rates (proliferative and mesenchymal) also benefit most from bevacizumab treatment. This review focuses on some of the advances in understanding molecular, cellular, and genetic changes in ovarian carcinomas with the results achieved so far regarding the formulation of molecular subtypes of ovarian cancer, however further studies are needed.

scholarly journals Real-World Palbociclib Use in HR+/HER2− Advanced Breast Cancer in Canada: The IRIS Study

2021 ◽  
Vol 28 (1) ◽  
pp. 678-688
Author(s):  
Katie Mycock ◽  
Lin Zhan ◽  
Gavin Taylor-Stokes ◽  
Gary Milligan ◽  
Debanjali Mitra
Keyword(s):  
Breast Cancer ◽  
Real World ◽  
Medical Records ◽  
Survival Rates ◽  
Growth Factor Receptor ◽  
Metastatic Breast ◽  
Retrospective Chart Review ◽  
Hormone Receptor Positive ◽  
Kaplan Meier ◽  
Epidermal Growth

Background: Palbociclib is a selective cyclin-dependent kinase (CDK) 4/6 inhibitor used in combination with aromatase inhibitors or fulvestrant for patients with hormone receptor-positive (HR+) human epidermal growth factor receptor 2 (HER2)-negative advanced/metastatic breast cancer (ABC/MBC). Palbociclib was the first CDK 4/6 inhibitor approved for HR+/HER2− ABC/MBC treatment in Canada in combination with letrozole (P+L) as an initial endocrine-based therapy (approved March 2016), or with fulvestrant (P+F) following disease progression after prior endocrine therapy (approved May 2017). The Ibrance Real World Insights (IRIS) study (NCT03159195) collected real-world outcomes data for palbociclib-treated patients in several countries, including Canada. Methods: This retrospective chart review included women with HR+/HER2− ABC/MBC receiving P+L or P+F in Canada. Physicians reviewed medical records for up to 14 patients, abstracting demographic and clinical characteristics, treatment patterns, and clinical outcomes. Progression-free rates (PFRs) and survival rates (SRs) at 6, 12, 18, and 24 months were estimated via Kaplan–Meier analysis. Results: Thirty-three physicians examined medical records for 247 patients (P+L, n = 214; P+F, n = 33). Median follow-up was 8.8 months for P+L and 7.0 months for P+F. Most patients were initiated on palbociclib 125 mg/d (P+L, 90.2%; P+F, 84.8%). Doses were reduced in 16.6% of P+L and 14.3% of P+F patients initiating palbociclib at 125 mg/d. The PFR for P+L was 90.3% at 12 months and 78.2% at 18 months; corresponding SRs were 95.6% and 93.0%. For P+F, 6-month PFR was 91.0%; 12-month SR was 100.0%. Conclusions: Dose reduction rates were low and PFR and SR were high in this Canadian real-world assessment of P+L and P+F treatments, suggesting that palbociclib combinations are well tolerated and effective.

scholarly journals Sinonasal mucosal melanoma: treatment strategies and survival rates for a rare disease entity

2021 ◽  
Author(s):  
Alexandros Andrianakis ◽  
Peter Kiss ◽  
Markus Pomberger ◽  
Axel Wolf ◽  
Dietmar Thurnher ◽  
...  
Keyword(s):  
Rare Disease ◽  
Survival Data ◽  
Survival Rates ◽  
Delayed Diagnosis ◽  
Distant Metastases ◽  
Primary Treatment ◽  
Mucosal Melanoma ◽  
Disease Entity ◽  
Improve Outcome ◽  
Initial Symptoms

Summary Background Sinonasal mucosal melanoma (SNMM) is a rare disease entity comprising 0.4–1.3% of all melanomas. Surgery with free margins has been the primary treatment over decades. Neither the addition of radiotherapy nor chemotherapy could significantly improve outcome rates of this devastating malignancy. This study presents our clinical experience with SNMM over a 19-year period and summarizes the current body of literature on SNMM. Methods This retrospective analysis included 12 patients with SNMM treated from 2001 to 2019 at an academic center. Additionally, a literature review of the last 29 years on treatment and survival data of SNMM was conducted. Results Main initial symptoms were epistaxis and nasal obstruction. Of the patients 9 underwent endoscopic surgery, 6 received adjuvant therapy. 3 patients who did not undergo surgery, received chemoradiotherapy, radiotherapy alone, and chemotherapy alone, respectively. At the time of diagnosis 2 patients had distant metastases and 4 patients developed distant metastases during the course of the disease. Mean overall survival (OS) was 30.6 months, 3‑year and 5‑year OS were 25%, and 18.2%, respectively. Conclusion Unspecific symptoms and hidden anatomic locations lead to delayed diagnosis and increased rates of metastatic dissemination. Distant metastasis is the main treatment failure in SNMM. Surgery with free margins remains the primary treatment for SNMM. Adjuvant radiotherapy might improve local control in individual cases but efficient systemic therapy is needed to improve outcome rates. To evaluate and define more effective targeted treatment options and improve outcome rates, homogeneous data and prospective multicentric analysis are needed.

scholarly journals Primary Signet Ring Cell Carcinoma of the Lung with Cerebellar Metastasis Showing Full Response to Cisplatin and Docetaxel Therapy

2014 ◽  
Vol 2014 ◽  
pp. 1-3 ◽  
Author(s):  
Onur Kocas ◽  
Fatih Selcukbiricik ◽  
Ahmet Bilici ◽  
Metin Kanıtez ◽  
Serdar Yildiz ◽  
...  
Keyword(s):  
Cell Carcinoma ◽  
Rare Disease ◽  
Primary Tumor ◽  
Signet Ring Cell Carcinoma ◽  
Signet Ring Cell ◽  
Histopathological Diagnosis ◽  
Pet Ct ◽  
Signet Ring ◽  
Ring Cell ◽  
Cerebellar Metastasis

Introduction. Primary signet ring cell carcinoma (SRCC) of the lung is a very rare disease. We describe a new case of primary SRCC of the lung with cerebellar metastasis, which responded well to the therapeutic approach with cisplatin and docetaxel.Case Report. A 41-year-old female patient (nonsmoker) was consulted to our oncology outpatient clinic after cerebellar metastasectomy. The histopathological diagnosis was SRCC metastasis. The primary tumor was unknown. The PET-CT imaging showed a hypermetabolic mass in the right middle lobe of the lung and hypermetabolic mediastinal lymph node stations. Oesophagogastroduodenoscopy and colonoscopy showed no evidence of gastrointestinal system tumor. The clinical diagnosis of primary SRCC of the lung was made and the administration of six rounds of cisplatin and docetaxel treatment was planned. After the chemotherapy the PET-CT scan to evaluate the therapy response showed full metabolic regression of the primary tumor and the mediastinal lymph nodes. There was no evidence of new metastasis.Conclusion. Primary SRCC of the lung is a very rare disease with poor prognosis. There are not many cases in literature and no standardized chemotherapy protocols. Cisplatin and docetaxel may be a good treatment option.

scholarly journals Posttraumatic Intramuscular Nodular Fasciitis in a 7-Year-Old Boy: Case Report and Review of the Literature

2021 ◽  
pp. 1-4
Author(s):  
Markus Denzinger ◽  
Markus Denzinger ◽  
Sandra Steininger ◽  
Niels Zorger ◽  
Patricia Reis Wolfertstetter ◽  
...  
Keyword(s):  
Case Report ◽  
Rare Disease ◽  
Complete Resection ◽  
Primary Resection ◽  
Histopathological Diagnosis ◽  
Nodular Fasciitis ◽  
Pectoralis Muscle ◽  
Conservative Approach ◽  
Review Of The Literature

Nodular fasciitis is a rare disease, and its diagnosis is difficult. We present a case report of a seven-year-old child with progredient swelling of the left pectoralis muscle three weeks after trauma. After histopathological diagnosis, we performed complete resection. Normally, a conservative approach with regular follow-up is regarded as appropriate since nodular fasciitis does have the capability to regress spontaneously. Since recent publications indicate the possibility of malignant transformation, the complete primary resection also has to be discussed as therapy of choice.

scholarly journals Influence of type and volume of surgical resection on postoperative period in patients with gliomas

2008 ◽  
Vol 7 (5-1) ◽  
pp. 231-235
Author(s):  
B. V. Martynov ◽  
V. Ye. Parfenov ◽  
D. V. Svistov ◽  
G. Ye. Trufanov ◽  
V. A. Fokin ◽  
...  
Keyword(s):  
Postoperative Complications ◽  
Surgical Resection ◽  
Neurological Deficit ◽  
Open Surgery ◽  
Tumor Resection ◽  
Neurological Status ◽  
Tumor Location ◽  
Tumour Resection ◽  
Before And After ◽  
Location Type

283 patients with gliomas were included in this study. Age, sex, neurological status and Karnovsky performance were analyzed before and after surgery, also tumor location, type and volume of surgical resection, postoperative complications were considered. Volume of tumor resection did not depend on glioma localization, excluding deep located tumors, in which case stereotactic cryotomy was performed (p < 0,01). In cases of stereotactic cryotomy postoperative neurological deficit worsening was noted in 12,5%, in patients with open biopsy and partial resection — 10,9%, and in case of total or subtotal tumor resection in 7,0% (p > 0,05). Partial gliom resection often related with postoperative complications and neurological deficit worsening then open surgery total tumour resection. Stereotactic cryotomy does not lead to bigger postoperative complications frequency in comparisons with open surgery.

scholarly journals Lymphadenectomy in the Treatment of Colon Cancer: A Survival Nalysis

2019 ◽  
Vol 4 (12) ◽  
Author(s):  
Romualdo Silva Corrêa ◽  
Luciana Ayres de Oliveira Lima ◽  
Isa Maryana Araújo Bezerra de Macedo ◽  
Amália Cinhtia Meneses Rêgo ◽  
Irami Araújo-Filho
Keyword(s):  
Colon Cancer ◽  
Lymph Nodes ◽  
Medical Records ◽  
Survival Rates ◽  
Primary Treatment ◽  
Term Survival ◽  
Kaplan Meier ◽  
The Impact ◽  
Radiotherapy Treatment

Colon cancer is a curable disease when restricted to the bowel and colectomy, the primary treatment. However, the presence and number of resected lymph nodes influence the therapeutic approach and prognosis of the patient. To evaluate the impact of the number of resected lymph nodes on the overall survival of patients treated for colon cancer at the League of Cancer Hospital - Natal - State of Rio Grande do Norte (RN) - Northeast Brazil. A retrospective observational study of 80 patients with colon cancer from Dr. Luiz Antônio Hospital (Natal-RN / Brazil), considering the period 2007-2014. Data were collected through medical records review. Survival rates were calculated and compared using the non-parametric Kaplan-Meier and Wilcoxon tests, respectively. All patients underwent radical surgical treatment associated or not with chemotherapy and/or radiotherapy treatment. The median survival time for the group of patients who had 12 or more resected lymph nodes was 9.4 years, in contrast to the 3.3 years of those who had less than 12 lymph nodes. Conclusion: It was concluded that a total of 12 or more resected lymph nodes confirmed by histopathology is associated with increased long-term survival in patients with colon cancer undergoing radical colectomy with or without chemotherapy and radiotherapy.

scholarly journals Surgical Resection Significantly Promotes the Overall Survival of Patients with Hepatocellular Carcinoma: A Propensity Score Matching Analysis

2021 ◽  
Author(s):  
Pei-Min Hsieh ◽  
Hung-Yu Lin ◽  
Chao-Ming Hung ◽  
Gin-Ho Lo ◽  
I-Cheng Lu ◽  
...  
Keyword(s):  
Risk Factors ◽  
Hepatocellular Carcinoma ◽  
Overall Survival ◽  
Propensity Score ◽  
Propensity Score Matching ◽  
Surgical Resection ◽  
Cox Regression ◽  
Survival Rates ◽  
Hbv Infection ◽  
Cox Regression Analysis

Abstract Background: The benefits of surgical resection (SR) for various Barcelona Clinic Liver Cancer (BCLC) stages of hepatocellular carcinoma (HCC) remain unclear. We investigated the risk factors of overall survival (OS) and survival benefits of SR over nonsurgical treatments in patients with HCC of various BCLC stages.Methods: Overall, 2316 HCC patients were included, and their clinicopathological data and OS were recorded. OS was analyzed by the Kaplan-Meier method and Cox regression analysis. Propensity score matching (PSM) analysis was performed.Results: In total, 66 (2.8%), 865 (37.4%), 575 (24.8%) and 870 (35.0%) patients had BCLC stage 0, A, B, and C disease, respectively. Furthermore, 1302 (56.2%) of all patients, and 37 (56.9%), 472 (54.6%), 313 (54.4%) and 480 (59.3%) of patients with BCLC stage 0, A, B, and C disease, respectively, died. The median follow-up duration time was 20 (range 0-96) months for the total cohort and was subdivided into 52 (8-96), 32 (1-96), 19 (0-84), and 12 (0-79) months for BCLC stages 0, A, B, and C cohorts, respectively. The risk factors for OS were 1) SR and cirrhosis; 2) SR, cirrhosis, and Child-Pugh (C-P) class; 3) SR, hepatitis B virus (HBV) infection, and C-P class; and 4) SR, HBV infection, and C-P class for the BCLC stage 0, A, B, and C cohorts, respectively. Compared to non-SR treatment, SR resulted in significantly higher survival rates in all cohorts. The 5-year OS rates for SR vs non-SR were 44.0% vs 28.7%, 72.2% vs 42.6%, 42.6% vs 36.2, 44.6% vs 23.5%, and 41.4% vs 15.3% (all p-values<0.05) in the total and BCLC stage 0, A, B, and C cohorts, respectively. After PSM, SR resulted in significantly higher survival rates compared to non-SR treatment in various BCLC stages.Conclusion: SR conferred significant survival benefits to patients with HCC of various BCLC stages and should be considered a recommended treatment for select HCC patients, especially patients with BCLC stage B and C disease.

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