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Images
Published: 01 March 2023
ISLET HYPERPLASIA IN BECKWITH-WIEDEMANN SYNDROME Figure 15-69 ISLET HYPERPLASIA IN BECKWITH-WIEDEMANN SYNDROME There are enlarged islets and reduced numbers of acinar cells embedded in a loose stroma. More
Book Chapter
Book: Tumors of the Pancreas
Series: Atlases of Tumor and Non-Tumor Pathology, Series 5
Volume: 15
Published: 01 March 2023
DOI:
10.55418/9781933477275-10
ISBN-10: 1-933477-27-X
ISBN: 978-1-933477-27-5
... frequent reporting of these neoplasms in the Asian literature, Asian patients are also overrepresented in a large series of pancreatoblastomas from medical centers in the United States ( 19 ). Pancreatoblastomas are reported in patients with the Beckwith-Wiedemann syndrome (BWS) ( 4 , 25 – 29 ). BWS...
Book Chapter
Book: Tumors of the Pancreas
Series: Atlases of Tumor and Non-Tumor Pathology, Series 5
Volume: 15
Published: 01 March 2023
DOI:
10.55418/9781933477275-15
ISBN-10: 1-933477-27-X
ISBN: 978-1-933477-27-5
... of diabetic mothers, and in patients with Beckwith-Wiedemann syndrome, erythroblastosis fetalis, hereditary tyrosinemia, Zellweger syndrome, and leprechaunism. The much more common aggregation of islets that occurs secondary to chronic pancreatitis (see above) should not be confused with true islet...
Book Chapter
Book: Tumors of the Pancreas
Series: Atlases of Tumor and Non-Tumor Pathology, Series 5
Volume: 15
Published: 01 March 2023
DOI:
10.55418/9781933477275-09
ISBN-10: 1-933477-27-X
ISBN: 978-1-933477-27-5
... , Rodriguez A , Formigo E , et al Pancreatoblastoma associated with incomplete Beckwith-Wiedemann syndrome: case report and review of the literature . J Pediatr Surg 2005 ; 40 : 1341 - 4 . 46. Koh TH , Cooper JE , Newman CL , Walker TM , Kiely EM , Hoffmann EB...
Book Chapter
Series: Atlases of Tumor and Non-Tumor Pathology, Series 5
Volume: 13
Published: 01 November 2022
DOI:
10.55418/9781933477251-5
ISBN-10: 1-933477-25-3
ISBN: 978-1-933477-25-1
... , Bhatti TR , et al . Congenital hyperinsulinism in children with paternal 11p uniparental isodisomy and Beckwith-Wiedemann syndrome . J Med Genet 2016 ; 53 : 53 - 61 . 157. Kapoor RR , Flanagan SE , Arya VB , Shield JP , Ellard S , Hussain K . Clinical...
Book Chapter
Series: Atlases of Tumor and Non-Tumor Pathology, Series 5
Volume: 13
Published: 01 November 2022
DOI:
10.55418/9781933477251-4
ISBN-10: 1-933477-25-3
ISBN: 978-1-933477-25-1
... Secondary and Tertiary Adrenal Insufficiency Unilateral Adrenal Cortical Hyperplasia Beckwith-Wiedemann Syndrome ADRENAL MEDULLARY HYPERPLASIA Aldosterone Excess Due to Adrenocortical Hyperplasia Congenital Adrenal Hyperplasia Adrenocortical Macronodular Hyperplasia Primary Pigmented Nodular...
Book Chapter
Series: Atlases of Tumor and Non-Tumor Pathology, Series 5
Volume: 12
Published: 01 August 2022
DOI:
10.55418/9781933477220-11
ISBN-10: 1-933477-22-9
ISBN: 978-1-933477-22-0
... and Beckwith-Wiedemann syndromes, in prepubertal hypothyroidism, in follicle-stimulating hormone (FSH)-secreting pituitary adenomas, in androgen-producing adrenocortical tumors, and in some cases of aromatase deficiency caused by CYP19A1 gene mutations. As discussed in the “Embryology” section...
Images
Published: 01 November 2021
DYSPLASTIC MEDULLARY RAY NODULE Figure 1-55 DYSPLASTIC MEDULLARY RAY NODULE Left: This lesion is an extension of the malformed “Beckwith medulla” of the kidney in a patient with Beckwith-Wiedemann syndrome. The dysplastic medullary ray is rounded at intermediate power. Right: It contains abnorma... More
Images
Published: 01 November 2021
POTENTIAL FATES OF NEPHROGENIC RESTS Figure 1-49 POTENTIAL FATES OF NEPHROGENIC RESTS In this figure, gray areas represent regression whereas red nodules represent evolution to nephroblastoma. (Modified from fig. 4 in Beckwith JB. Precursor lesions of Wilms tumor: clinical and biological implicat... More
Book Chapter
Series: Atlases of Tumor and Non-Tumor Pathology, Series 5
Volume: 9
Published: 01 November 2021
DOI:
10.55418/9781933477176-1
ISBN-10: 1-933477-17-2
ISBN: 978-1-933477-17-6
... WT1 point mutation >90% Beckwith-Wiedemann Macroglossia, organomegaly, hemihypertrophy, neonatal hyperglycemia, nephroblastoma and other embryonal tumors 11p15 Duplication of paternal allele 10% a Modified from Table 1 in Coppes MJ, Haber DA, Grundy PE. Genetic events...
Images
in Sonographic and Doppler Evaluation of the Placenta with Pathologic Correlation
> Atlas of Placental Pathology
Published: 01 August 2021
PLACENTAL MESENCHYMAL DYSPLASIA Figure 11-1-20 PLACENTAL MESENCHYMAL DYSPLASIA Transabdominal image demonstrating a thickened anterior placenta with multiple lucencies at 14 weeks gestation. This was associated with a sonographically normal-appearing fetus. Prenatal genetic evaluation demonstrate... More
Book Chapter
Series: Atlases of Tumor and Non-Tumor Pathology, Series 5
Volume: 9
Published: 01 November 2021
DOI:
10.55418/9781933477176-2
ISBN-10: 1-933477-17-2
ISBN: 978-1-933477-17-6
... VERSUS PAPILLARY RENAL CELL CARCINOMA AND NEPHROBLASTOMA Metanephric Adenoma Nephroblastoma Papillary Renal Cell Carcinoma Clinical Median age (years) 40-50 3 50-60 Male: female 1:2 1:1 4:1 Associations Polycythemia (10%) WAGR, Denys-Drash, Beckwith-Wiedemann syndrome...
Book Chapter
Series: Atlases of Tumor and Non-Tumor Pathology, Series 5
Volume: 6
Published: 01 August 2021
DOI:
10.55418/9781933477091-appendix01
ISBN-10: 1-933477-09-1
ISBN: 978-1-933477-09-1
... and obstetric provider Rule out infant renal disorders or metabolic disease; high occurrence risk; association with maternal thrombophilia and autoimmune disease Placental mesenchymal dysplasia ( chapter 8-2 ) Pediatrician/neonatologist Association with Beckwith-Wiedemann syndrome and congenital/pediatric...
Book Chapter
Series: Atlases of Tumor and Non-Tumor Pathology, Series 5
Volume: 6
Published: 01 August 2021
DOI:
10.55418/9781933477091-ch11
ISBN-10: 1-933477-09-1
ISBN: 978-1-933477-09-1
... Beckwith-Wiedemann syndrome ( 32 , 33 ). The differential diagnosis of PMD includes partial molar pregnancy and normal pregnancy with a coexisting twin mole. A thin or small placenta is associated with growth restriction and aneuploidy ( 28 ). PLACENTAL MESENCHYMAL DYSPLASIA Figure 11-1-20 PLACENTAL...
Book Chapter
Series: Atlases of Tumor and Non-Tumor Pathology, Series 5
Volume: 6
Published: 01 August 2021
DOI:
10.55418/9781933477091-ch08
ISBN-10: 1-933477-09-1
ISBN: 978-1-933477-09-1
... there was polyspermic or single sperm fertilization). In PMD, the mosaicism is such that the placental androgenetic cell line is restricted to the stroma of the villi whereas the trophoblast remain normally biparental. PMD is associated with Beckwith-Wiedemann syndrome (BWS) in 23 percent of cases ( 43 ). BWS is a rare...
Book Chapter
Series: Atlases of Tumor and Non-Tumor Pathology, Series 5
Volume: 6
Published: 01 August 2021
DOI:
10.55418/9781933477091-ch09
ISBN-10: 1-933477-09-1
ISBN: 978-1-933477-09-1
... the same genetic aberration manifest the clinical syndrome of Beckwith-Wiedemann syndrome. Fetuses, whether or not they have Beckwith-Wiedemann syndrome, are prone to intrauterine growth restriction and stillbirth due to placental dysfunction. Gross Findings . Affected placentas exhibit massive...
Book Chapter
Series: Atlas of Tumor Pathology, Series 4
Volume: 8
Published: 01 December 2007
DOI:
10.55418/9781881041016-02
ISBN-10: 1-881041-01-8
ISBN: 978-1-881041-01-6
