WILMS TUMOR IN ADULTS: CASE REPORT AND REVIEW OF THE LITERATURE

Wilms Tumor (WT) is an extremely rare neoplasia in adults. It represents 95% and 1% of all renal tumors, in adults and children respectively. Clinical symptoms are also distinct between adults and children. In adults, symptoms are usually abdominal pain and hematuria, on the other hand, pediatric patients frequently present with an asymptomatic abdominal mass. The recommended treatment for WT in adults follows protocols established for pediatric patients. Currently, the 5-year overall survival rate for adults with WT is of approximately 90%. The adoption of multimodal therapy systematized, and proposed by current protocols, allowed the results to become much more robust, with a significant improvement from less than 30% to 90%. However, recent reviews suggest a worse outcome for adults when compared to children, even when submitted to the same treatment regimen, and comparable according to histology and tumor stage.We present a case of a 26 years-old male patient, who underwent surgery to treat a solid renal mass. Pathological report demonstrated a Wilms tumor. The patiente also received adjuvant chemotherapy with dactinomycin and vincristine. After 12 months follow-up, patient has no evidence of disease.

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Pediatric Anastomosing Hemangioma: Case Report and Review of Renal Vascular Tumors in Children

Pediatric and Developmental Pathology ◽

10.1177/1093526618809230 ◽

2018 ◽

Vol 22 (3) ◽

pp. 269-275 ◽

Cited By ~ 1

Author(s):

Alvin B Caballes ◽

Agustina D Abelardo ◽

Miguel J Farolan ◽

Januario Antonio D Veloso

Keyword(s):

Case Report ◽

Pediatric Patients ◽

Wilms Tumor ◽

Vascular Lesion ◽

Renal Tumors ◽

Vascular Tumors ◽

Malignant Neoplasms ◽

Therapeutic Implications ◽

Renal Vascular ◽

Anastomosing Hemangioma

The case involves a 10-year-old child who underwent a left radical nephrectomy for what was believed to be a Wilms’ tumor. Histopath examination indicated a benign vascular lesion, subsequently determined to be an anastomosing hemangioma of the kidney. A comparison with the previously cited pediatric patients with renal vascular tumors is provided, and the inconsistent diagnostic terminologies for these conditions are highlighted. The therapeutic implications of these predominantly benign renal tumors, in the context of the much more frequently encountered malignant neoplasms in children, are additionally discussed.

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COVID-19, Various Treatment Options and Special Considerations for Dentistry

Journal of Pharmaceutical Research International ◽

10.9734/jpri/2020/v32i1030494 ◽

2020 ◽

pp. 70-76

Author(s):

Smita Singh Bhardwaj ◽

Sami Alduwayhi ◽

Atul Bhardwaj

Keyword(s):

Health Professionals ◽

Pediatric Patients ◽

Dental Treatment ◽

Clinical Symptoms ◽

Treatment Options ◽

Treatment Protocol ◽

Health Concerns ◽

Dental Clinics ◽

The World ◽

Adults And Children

Objective: The aim of this article is to analyze the epidemiology of COVID-19, comparison of routes of transmission in children and adults, comparing the clinical symptoms in adults and children, treatment protocol to be followed and possible treatment options during this pandemic. Data Sources: Data is collected from Pubmed, Medline and Embase databases. Discussion from Previous References Used: Few studies have been done to analyze its effect on children comparing the symptoms of adults and children. Also very less work is done to analyze the special precautions taken while doing dental treatment during this pandemic. Conclusion: The widespread effect of Coronavirus (COVID-19) or SARS-CoV-2 has created health concerns in the world. Although efforts have been taken to control the disease, it is still increasing due to the community spread. Health professionals may get patients in their practice with this infection and should prevent its spread. Clinical Significance of this Article: It will help us to differentiate the specific clinical symptoms of adult and pediatric patients coming to dental clinics and the special considerations for them including the emergency dental treatment during COVID-19.

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Wilms Tumor

10.1093/med/9780190685157.003.0041 ◽

2018 ◽

Author(s):

Graciela Argote-Romero

Keyword(s):

Wilms Tumor ◽

Incidental Finding ◽

Abdominal Mass ◽

Disease Free Survival ◽

Von Willebrand Disease ◽

Renal Tumors ◽

Intravenous Contrast ◽

Favorable Histology ◽

Disease Free Survival Rate ◽

Von Willebrand

Wilms tumor, known as well as nephroblastoma, is the most common primary malignant renal tumor in children. Over 95% of all renal tumors in patients under the age of 15 are Wilms tumors. The mean age at the time of diagnosis is 3.5 years. Wilms tumors are usually an incidental finding, a large abdominal mass discovered by a family member or pediatrician. Hematuria and hypertension can be present at the time of initial diagnosis. Up to 8% of the patients will have von Willebrand disease; therefore, all patients should have baseline coagulation studies. All patients should have either computed tomography of the abdomen and pelvis with oral and intravenous contrast or magnetic resonance imaging of the abdomen and pelvis with gadolinium. Treatment includes radical nephrectomy, chemotherapy, and, in some cases, radiotherapy. Emergency surgery is rarely. The disease-free survival rate is 86% for favorable-histology tumors and 64% for anaplastic tumors.

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Surgical management of cerebellopontine angle arachnoid cysts associated with hearing deficit in pediatric patients

Journal of Neurosurgery Pediatrics ◽

10.3171/2017.8.peds17341 ◽

2018 ◽

Vol 21 (2) ◽

pp. 119-123

Author(s):

Mario Giordano ◽

Massimo Gallieni ◽

Amir Samii ◽

Concezio Di Rocco ◽

Madjid Samii

Keyword(s):

Hearing Loss ◽

Surgical Treatment ◽

Cerebellopontine Angle ◽

Pediatric Patients ◽

Clinical Symptoms ◽

Complete Recovery ◽

The Other ◽

Arachnoid Cysts ◽

Recent Onset

OBJECTIVEFew cases of cerebellopontine angle (CPA) arachnoid cysts in pediatric patients have been described in the literature, and in only 2 of these cases were the patients described as suffering from hearing deficit. In this article, the authors report on 3 pediatric patients with CPA arachnoid cysts (2 with hearing loss and 1 with recurrent headaches) who underwent neurosurgical treatment at the authors’ institution.METHODSFour pediatric patients were diagnosed with CPA arachnoid cysts at the International Neuroscience Institute during the period from October 2004 through August 2012, and 3 of these patients underwent surgical treatment. The authors describe the patients’ clinical symptoms, the surgical approach, and the results on long-term follow-up.RESULTSOne patient (age 14 years) who presented with headache (without hearing deficit) became asymptomatic after surgical treatment. The other 2 patients who underwent surgical treatment both had hearing loss. One of these children (age 9 years) had recent-onset hypacusia and experienced complete recovery immediately after the surgery. The other (age 6 years) had a longer history (2 years) of progressive hearing loss and showed an interruption of the deficit progression and only mild improvement at the follow-up visit.CONCLUSIONSCPA arachnoid cysts are uncommon in pediatric patients. The indication and timing of the surgical treatment are fundamental, especially when a hearing deficit is present.

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Efficacy and Safety of Rituximab in Chinese Children With Refractory Anti-NMDAR Encephalitis

Frontiers in Neurology ◽

10.3389/fneur.2020.606923 ◽

2020 ◽

Vol 11 ◽

Author(s):

Xiangjun Dou ◽

Dongjing Li ◽

Yan Wu ◽

Zhijing Wang ◽

Le Yang ◽

...

Keyword(s):

Adverse Events ◽

Pediatric Patients ◽

Clinical Symptoms ◽

Complete Recovery ◽

Chinese Children ◽

Second Line ◽

Efficacy And Safety ◽

Treatment Protocols ◽

Nmdar Encephalitis

Purpose: To assess the efficacy and safety of rituximab treatment as second-line immunotherapy in pediatric cases of anti-NMDA receptor (NMDAR) encephalitis.Methods: We retrospectively recruited 8 patients with anti-NMDAR encephalitis who were treated with rituximab as second-line immunotherapy. We evaluated the clinical features, laboratory examination results and treatment protocols of the Chinese children and defined good outcomes based on the modified Rankin scale (mRS) score (0–2) at the last follow-up.Results: A total of eight pediatric patients (median age 6.7 years; four female) with refractory anti-NMDAR encephalitis were recruited to the study. Rituximab was given after a median duration of disease of 57 days (range 50.5–113.75 days). The use of rituximab led to a significant reduction in the mRS and CD19+ B-cells compared to before rituximab infusion (P < 0.05). Five patients (62.5%) had a good outcome (mRS ≤ 2) including four patients (50%) who showed complete recovery (mRS = 0) at the last follow-up. Transient infusion adverse events were recorded in 2 patients (25%). Two patients (25%) had severe infectious adverse events (AEs) and two patients with grade 5 (death). None of the patients developed progressive multifocal leukoencephalopathy (PML).Conclusion: Our study provides evidence that rituximab can efficiently improve the clinical symptoms of anti-NMDAR encephalitis in children. However, due to the risk of adverse infections, rituximab should be restricted in pediatric patients with high rates of mortality and disability.

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Autoantibodies Against Lysosome Associated Membrane Protein-2 (LAMP-2) in Pediatric Chronic Primary Systemic Vasculitis

Frontiers in Immunology ◽

10.3389/fimmu.2020.624758 ◽

2021 ◽

Vol 11 ◽

Author(s):

Kristen M. Gibson ◽

Renate Kain ◽

Raashid A. Luqmani ◽

Colin J. Ross ◽

David A. Cabral ◽

...

Keyword(s):

Membrane Protein ◽

Renal Disease ◽

Disease Activity ◽

Pediatric Patients ◽

Systemic Vasculitis ◽

Small Vessel Vasculitis ◽

Creatinine Concentration ◽

Primary Systemic Vasculitis ◽

Adults And Children

BackgroundAnti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) is a small vessel vasculitis in adults and children that commonly affects the kidneys. Although the frequent antigenic, and presumed pathogenic, targets of ANCA in AAV are proteinase-3 (PR3) and myeloperoxidase (MPO), ANCA against lysosome associated membrane protein-2 (LAMP-2), a lesser known ANCA antigen that is expressed on the glomerular endothelium, are present in some adults with AAV-associated renal disease. LAMP-2-ANCA has not been assessed in children with chronic systemic vasculitis, and, if present, would be a potentially valuable biomarker given that treatment decisions for these pediatric patients at diagnosis are largely informed by kidney function.MethodsA custom ELISA, using commercially available reagents, was designed to detect autoantibodies to human LAMP-2 in serum. Sera obtained from 51 pediatric patients at the time of diagnosis of chronic primary systemic vasculitis (predominantly AAV) were screened. LAMP-2-ANCA titers were evaluated for correlation with clinical metrics of disease activity (pediatric vasculitis activity score [pVAS], C-reactive protein [CRP] concentration, and erythrocyte sedimentation rate [ESR]), MPO- and PR3-ANCA titers, and renal function (glomerular filtration rate [GFR], renal-specific pVAS, and serum creatinine concentration).ResultsLAMP-2-ANCA (>1,000 ng/ml) were detected in 35% (n = 18) of pediatric systemic vasculitis patients, of which, 10 (20% of all patients) were found to have high positive titers (>1,500 ng/ml). Undetectable or negative titres (<500 ng/ml) were identified in 12% (n = 6) of patients, those with titers between 500 and 1,000 ng/ml were considered low with unknown clinical relevance (53%, n = 27). Although LAMP-2-ANCA titers did not significantly differ between patients with AAV versus ANCA-negative vasculitis, only AAV patients had high concentrations (>1,500 ng/ml) of LAMP-2-ANCA. LAMP-2-ANCA titers did not correlate with measures of disease activity (pVAS, CRP, or ESR) at the time of diagnosis. In contrast, for patients with 12-month post diagnosis follow-up, a negative correlation was observed between the change in GFR (from diagnosis to 12-month follow-up) and LAMP-2-ANCA titer at diagnosis.ConclusionsModerate to high LAMP-2-ANCA titers were detected in 35% (18/51) of children with chronic systemic vasculitis affecting small-to-medium vessels. Although the highest concentrations of LAMP-2-ANCA in this population were observed in individuals positive for classic ANCA (MPO- or PR3-ANCA), similar to previous reports on adult patients, LAMP-2-ANCA titers do not correlate with classic ANCA titers or with overall disease activity at diagnosis. Renal disease is a common manifestation in systemic small-medium vessel vasculitis (both in adults and children, though more severe in children) and our preliminary data suggest LAMP-2-ANCA at diagnosis may be a risk factor for more severe renal disease.

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CLINICAL AND SUBCLINICAL FEATURES OF PEDIATRIC PATIENTS WITH HEPATOBLASTOMA AT NATIONAL CHILDREN HOSPITAL

Tạp chí Nghiên Cứu Và Thực Hành Nhi Khoa ◽

10.25073/jprp.v4i4.213 ◽

2020 ◽

Vol 4 (4) ◽

Author(s):

Duy Hien Pham

Keyword(s):

Pediatric Patients ◽

Clinical Symptoms ◽

Abdominal Mass ◽

Malignant Liver Tumor ◽

Average Value ◽

Mean Size ◽

Common Group ◽

Right Lobe ◽

Malignant Liver ◽

The Right

Background: Hepatoblastoma is the most common malignant liver tumor in children. Aims: Study on clinical and subclinical features of pediatric patients with hepatoblastoma at National Children Hospital from 1/2016 to 8/2019. Materials and Methods: A total patients with operated Hepatoblastoma diagnosed by pathologists, at the Viet Nam National Children’s Hospital from January 2016 to August 2019. The study design is retrospective. Resullts: Study subject’s mean of age was 36,8 months (range 4 – 149 months), the common group of age was under 5 years old (76,7%), male’s more than female (56,7% and 43,3%). The most common clinical symptoms were hepatomegaly (50%), a palpable abdominal mass (30%), abdominal pain (13,3%), secondary anemia (13,3%), weight loss (6,7%), jaundice (3,3%). About laboratory test: 83,3% patients with anemia, 36,7% patients with thrombocytosis, 90% patients with hight GOT levels, 53,3% patients with hight GPT levels, 96,7% patients with hight serum AFP levels and the average value of AFP was 217160,7 ng/ml (range 575 – 1686328,6 ng/ml). Mean size of the tumor on computerized tomography was 5,48cm (range 2 – 15cm), 60% size of tumor ≤ 5cm, 96,7% had only one tumor, 70% primary tumor located to the right lobe of the liver, 76,7% were staged PRETEXT II, the most common hepatoblastoma histopathological subtypes was as follows: epithelial (80%). Conclusion: Clinical symptoms of hepatoblastoma are usually nonspecific. A routine check – up strategy for early detection of hepatoblastoma and the role of subclinical aids in diagnosis is very important.

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CT in the prize in charge of wilms 'tumor (monocentric study of 13 cases)

World Journal of Biology Pharmacy and Health Sciences ◽

10.30574/wjbphs.2021.6.1.0033 ◽

2021 ◽

Vol 6 (1) ◽

pp. 054-060

Author(s):

Ousmane Traore ◽

Alassane Kouma ◽

Fousseyni Traore ◽

Issa Cisse ◽

Oungoumba Diarra ◽

...

Keyword(s):

Wilms Tumor ◽

Abdominal Mass ◽

Kidney Tissue ◽

Clinical Information ◽

Childhood Cancers ◽

Single Center Study ◽

The Mean ◽

Histological Confirmation ◽

Lost To Follow Up

Nephroblastoma or Wilms tumor is a malignant tumor that develops at the expense of embryonic kidney tissue. It is the most common kidney tumor in children (> 90%); it represents around 5% to 14% of all childhood cancers. The aim of our work is to recall the contribution of imaging, in particular CT, in the management of this renal tumor. This is a retrospective, single-center study of 136 cases of Wilms tumors diagnosed in our clinic over a 2-year period. The mean age of the patients was 04 years with an extreme of (3 months -12 years) with a female prevalence (8 girls for 5 boys). Abdominal mass dominated in our study in clinical information. Histological confirmation was found in 12 cases, the only non-conforming case was lost to follow-up.

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Current role of imaging in the management of children with Wilms tumor according to the new umbrella protocol

Journal of Advanced Health Care ◽

10.36017/jahc2001-012 ◽

2020 ◽

pp. 51-63

Author(s):

Varchetta Giovanni ◽

Tanzillo Paolina ◽

Mei Sara

Keyword(s):

Wilms Tumor ◽

Abdominal Mass ◽

Epidemiological Data ◽

Renal Tumors ◽

Postoperative Treatment ◽

Malignant Rhabdoid Tumor ◽

Presumptive Diagnosis ◽

Medical Checkup ◽

Abdominal Masses

Wilms tumor is the most common primary renal tumor in childhood. Children with Wilms tumor typically present with an asymptomatic abdominal mass, usually detected on a routine medical checkup or discovered coincidentally by parents. The initial differential diagnosis is with extrarenal abdominal masses; once a tumor of renal origin is established, distinguishing between Wilms tumor and other primary renal neoplasms such as congenital mesoblastic nephroma, clear cell sarcoma, malignant rhabdoid tumor and renal cell carcinoma may not be easy. However, in many cases imaging findings in conjunction with the patient's clinical and epidemiological data, allow the diagnosis of Wilms tumor. Wilms tumor care offers one of the most striking examples of success of pediatric oncology. Over the last decades the European SIOP studies have been the key to developing standardized diagnostic procedures, improved risk stratification, and adjusted treatment recommendations for children with Wilms tumor and this has resulted rate of overall survival is currently greater than 90%. As in previous SIOP trials and studies, the new protocol for the diagnosis and treatment of childhood renal tumors, the UMBRELLA SIOP–RTSG 2016, mandates preoperative chemotherapy without preceding mandatory histological assessment. Therefore, imaging studies are essential to obtain a presumptive diagnosis of WT, to provide disease staging information and to measure the tumor volume after neoadjuvant chemotherapy for the purposes of postoperative treatment stratification. This review describes role of imaging in the management of children with Wilms tumor, according to the current recommendations of the UMBRELLA protocol.

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