scholarly journals 1067 Neoplasia in Association with a Congenital Anomaly: Wilms Tumor in a Horseshoe Kidney

2021 ◽  
Vol 108 (Supplement_6) ◽  
Author(s):  
M Mubarak ◽  
O Alhamdan ◽  
H Alaradi ◽  
S Alhindi
Keyword(s):  
Ct Scan ◽  
Congenital Anomalies ◽  
Wilms Tumor ◽  
Horseshoe Kidney ◽  
Histopathological Analysis ◽  
Multiple Congenital Anomalies ◽  
Wagr Syndrome ◽  
Wilm's Tumor ◽  
Wilm’S Tumor

Abstract Introduction Horseshoe malformation is the most common form of fusion defects involving the kidney, whereas Wilm's tumor is the most common primary malignant renal tumor in children. The co-incidence of these two pathologies gives off an incidence rate of 0.48%. Case Report This is a case of a two-year-old female who presented early in life with multiple congenital anomalies including congenital heart disease, bilateral aniridia, persistent thrombocytopenia, and sickle cell trait in association with a painless abdominal mass. A CT scan showed a horseshoe kidney. The patient was lost for follow up until the age of two years, in which another CT scan revealed a left lower pole focal cystic mass measuring 5.1x5.3x6.3cm. WAGR syndrome was suspected and confirmed through genetic testing. A multidisciplinary approach was necessary to manage her efficiently due to the numerous co-existing congenital pathologies. The patient underwent six cycles of chemotherapy leading to a 35% reduction in tumor size and a left nephrectomy. She was admitted to the pediatric ICU post-operatively for observation. Histopathological analysis revealed features of stage two stromal type nephroblastoma. Her renal function and urine output were satisfactory throughout the duration of her admission as well as on follow up. Conclusions Keeping a high index of suspicion is imperative; individuals with renal anomalies, namely horseshoe kidney in the context of WAGR syndrome, mandate a comprehensive assessment through clinical examination, laboratory workup, and imaging. Though different approaches to standard Wilm's tumor cases have been explored thoroughly, it is crucial to keep in mind the need for multidisciplinary involvement in patients with multiple congenital anomalies.

scholarly journals A Rare Case Report on Therapeutic Management of Wilm’s Tumour

2021 ◽  
pp. 115-118
Author(s):  
Priya Rewatkar ◽  
Manoj Patil
Keyword(s):  
Physical Examination ◽  
Wilms Tumor ◽  
Signs And Symptoms ◽  
Female Child ◽  
The United States ◽  
The Body ◽  
Therapeutic Interventions ◽  
Timely Initiation ◽  
Wilm's Tumor ◽  
Wilm’S Tumor

Introduction: Wilms's tumour was named after Carl Max Wilhelm Wilms, a 19th century German surgeon. Wilm’s tumor is derived from primitive metanephric blastema and is a form of childhood carcinoma that begins in the kidneys (also called nephroblastoma). In infants, it is the most common form of kidney carcinoma. 9 out of 10 kidney cancers in kids is Wilms Tumor. Carcinoma can spread to other parts of the body. The incidence is around seven new cases per million children in the United States, with a peak incidence between the ages of 2 and 3 years. Main symptoms and important clinical findings: A 11 yrs. old female child was admitted in Acharya Vinoba Bhave Rural Hospital, Wardha on 08/02/2021 with chief complaint of breathing difficulty, shortness of breath, blood in the urine, nausea and vomiting. After physical examination and investigations, doctors diagnosed it as a case of Wilm’s tumor. The main diagnoses, therapeutic interventions and outcomes: After physical examination and investigations like MRI, doctor diagnosed it as a case Wilm’s tumour. Tab. Actinomycin D (Dactinomycin), tab. Vincristine (chemotherapy) were started and calcium and multivitamin supplements were given for 7 days to enhance immune function. Tab. Septran 160 mg OD and tab. Acenet 400 mg OD, tab. Bactrim 5mg OD was also prescribed. Patient took all treatment and outcome was good. Her signs and symptoms improved. Conclusion: Accurate diagnosis and timely initiation of treatment speeded up recovery.

The Perlman syndrome: Familial renal dysplasia with Wilms tumor, fetal gigantism and multiple congenital anomalies

1984 ◽  
Vol 19 (1) ◽  
pp. 195-207 ◽  
Author(s):  
Giovanni Neri ◽  
Maria Enrica Martini-Neri ◽  
Ben E. Katz ◽  
John M. Opitz
Keyword(s):  
Congenital Anomalies ◽  
Wilms Tumor ◽  
Renal Dysplasia ◽  
Multiple Congenital Anomalies

Congenital absence of the nasal septum in a Nigerian newborn with multiple congenital anomalies

2020 ◽  
Vol 13 (9) ◽  
pp. e235845
Author(s):  
Isa Abdulkadir ◽  
Sani Musa ◽  
Fatima Lami Abdullahi ◽  
Laila Hassan
Keyword(s):  
Congenital Anomalies ◽  
Nasal Septum ◽  
Relevant Literature ◽  
Congenital Absence ◽  
Multiple Congenital Anomalies ◽  
Nasal Bridge ◽  
Resource Limited ◽  
History Of ◽  
Term Baby

Congenital absence of the nasal septum (hyporhynia) is infrequently reported in the literature. We report a rare case in a newborn with multiple congenital anomalies with the aim to highlight its rarity and compatibility with life in a resource-limited setting. The case notes of the child were summarised and the relevant literature reviewed to give the report a context. A 2-hour-old term baby boy presented with a history of abnormal facie characterised by a single nostril and noisy breathing. On examination, he had stridulous breathing, bilateral proptosis, sloping of the forehead, depressed nasal bridge absent nasal septum, absence of columella and hypoplastic mid-face, among other dysmorphic features. A diagnosis of hyporhynia in a baby with multiple congenital anomalies was made and managed accordingly. The baby was discharged to continue follow-up. Congenital absence of the nasal septum is rare and could present without respiratory distress and may be compatible with life.

scholarly journals The perlman syndrome: Familial renal dysplasia with Wilms tumor, fetal gigantism and multiple congenital anomalies

2013 ◽  
Vol 161 (11) ◽  
pp. 2691-2696 ◽  
Author(s):  
Giovanni Neri ◽  
Maria Enrica Martini-Neri ◽  
Ben E. Katz ◽  
John M. Opitz
Keyword(s):  
Congenital Anomalies ◽  
Wilms Tumor ◽  
Renal Dysplasia ◽  
Multiple Congenital Anomalies

Suspected osseous recurrence visualized on a 68Ga- DOTATATE PET/CT scan during the follow-up of a patient with a resected pulmonary carcinoid tumour

2011 ◽  
Vol 50 (05) ◽  
pp. N57-N59
Author(s):  
S. Geiger ◽  
S. Horster ◽  
A. R. Haug ◽  
A. Hausmann ◽  
M. Schlemmer ◽  
...  
Keyword(s):  
Ct Scan ◽  
Carcinoid Tumour ◽  
Pulmonary Carcinoid ◽  
Pet Ct ◽  
Pet Ct Scan

Wilm’s Tumor: Syndrome-Based and Molecular Diagnostics

2017 ◽  
Vol 4 (4) ◽  
pp. 283-289 ◽  
Author(s):  
S. A. Kulyova ◽  
E. N. Imyanitov
Keyword(s):  
Molecular Diagnostics ◽  
Wilm's Tumor ◽  
Wilm’S Tumor

Individualized cancer follow-up supported by electronic Patient-Reported Outcomes: Development and evaluation (Preprint)

2020 ◽  
Author(s):  
Sissel Ravn ◽  
Henriette Vind Thaysen ◽  
Lene Seibaek ◽  
Victor Jilbert Verwaal ◽  
Lene Hjerrild Iversen
Keyword(s):  
Ct Scan ◽  
Advanced Cancer ◽  
Patient Reported Outcomes ◽  
Cost Benefit Analysis ◽  
Cost Benefit ◽  
Structured Interviews ◽  
European Organization ◽  
Individualized Care ◽  
Patient Reported

BACKGROUND Cancer survivors experience unmet needs during follow-up. Besides recurrence, a follow-up includes detection of late side effects, rehabilitation, palliation and individualized care. OBJECTIVE We aimed to describe the development and evaluate the feasibility of an intervention providing individualized cancer follow-up supported by electronic patient-reported outcomes (e-PRO). METHODS The study was carried out as an interventional study at a Surgical and a Gynecological Department offering complex cancer surgery and follow-up for advanced cancer. The e-PRO screened for a priori defined clinical important symptoms and needs providing individualized follow-up. We included following questionnaires in the e-PRO; the general European Organization for Research and Treatment of Cancer (EORTC) QLQ-C30 and the EORTC validated for colorectal and ovarian cancer patients. To support individualization, we included three prioritized issues of the patient’s preference in each e-PRO. The response-algorithm was aggregated based on the severity of the patient’s response. To ensure the sensitivity of the e-PRO, we performed semi-structured interviews with five patients. All clinicians (surgeons and gynecologists) performing the consultations reviewed the e-PRO. The evaluation was divided in two, 1)The feasibility was assessed by a)Patients’ response rate of the e-PRO, b)Number of follow-up visits documenting the use of e-PRO and c)Patients’ prioritized issues prior to the consultation(‘yes’ / ‘no’), and after the follow-up 2)Patients assessment of a)The need and purpose of the follow-up visit and b)the support provided during the follow-up visit. RESULTS In total, 187 patients were included in the study, of which 73%(n=136/187) patients responded to the e-PRO and were subjected to an individualized follow-up. The e-PRO was documented as applied in 79% of the follow-up visits. In total, 23% of the prioritized issues did not include a response. Stratified by time since surgery, significantly more patients did not fill out a prioritized issue had a follow-up >6 months since surgery. In total, 72 % follow-up visits were evaluated to be necessary in order to discuss the outcome of the CT scan, symptoms, and/or prioritized issues. Contrary, 19% of the follow-up visits were evaluated to be necessary only to discuss the result of the CT scan. A range from 19.3–56.3% of patients assessed the follow-up visit to provide support with respect to physical (42% of patients), mental (56%), sexual (19%) or dietary (27%) issues. Further, a range from 34–60% of the patients reported that they did not need support regarding physical (43% of patients), mental (34%), sexual (63%) or dietary (57%) issues. CONCLUSIONS An individualized follow-up based on e-PRO is feasible, and support most patients surgically treated for advanced cancer. However, results indicate that follow-up based on e-PRO may not be beneficial for all patients and circumstances. A thorough cost-benefit analysis may be warranted before implementation in routine clinic.

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