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Images
in Congenital Disorders of the Adrenal Gland
> Tumors of the Adrenal Glands and Extra-Adrenal Paraganglia
Published: 01 December 2024
BECKWITH-WIEDEMANN SYNDROME Figure 2-18 BECKWITH-WIEDEMANN SYNDROME A: Transverse sections of adrenal gland from a 3-week-old infant with Beckwith-Wiedemann syndrome are enlarged and show redundant cerebriform folds of cortex with demarcation between adult or definitive cortex and regressing and ... More about this image found in BECKWITH-WIEDEMANN SYNDROME A: Transverse sections of adrenal gland from a...
Images
in Congenital Disorders of the Adrenal Gland
> Tumors of the Adrenal Glands and Extra-Adrenal Paraganglia
Published: 01 December 2024
BECKWITH-WIEDEMANN SYNDROME Figure 2-19 BECKWITH-WIEDEMANN SYNDROME Chromaffin cells forming a nodule protruding from the fetal cortex are inappropriately mature for this stage of development and have clear cytoplasm. There is a morphologic resemblance of the nodule to normal extra-adrenal paraga... More about this image found in BECKWITH-WIEDEMANN SYNDROME Chromaffin cells forming a nodule protruding f...
Book Chapter
Series: Atlases of Tumor and Non-Tumor Pathology, Series 5
Volume: 21
Published: 01 December 2024
10.55418/9781933477473-02
ISBN-10: 1-933477-47-4
ISBN: 978-1-933477-47-3
... abundant compact eosinophilic cytoplasm. The granular pigment within some cells is lipofuscin. BECKWITH-WIEDEMANN SYNDROME Beckwith-Wiedemann syndrome (BWS) is a congenital human overgrowth disorder associated with dysregulation of the imprinting of genes in chromosome 11p15.5 ( 50 ). BWS...
Book Chapter
Series: Atlases of Tumor and Non-Tumor Pathology, Series 5
Volume: 21
Published: 01 December 2024
10.55418/9781933477473-10
ISBN-10: 1-933477-47-4
ISBN: 978-1-933477-47-3
... gastrointestinal stromal tumor [GIST]) caused by promoter hypermethylation of SDHC ( 11 ), Pacak-Zhuang syndrome (PZS) caused by early postzygotic mutations of EPAS1 ( 12 ), and Beckwith-Wiedemann syndrome ( 13 ) due to abnormal methylation the imprinting centers that regulate several growth...
Book Chapter
Series: Atlases of Tumor and Non-Tumor Pathology, Series 5
Volume: 21
Published: 01 December 2024
10.55418/9781933477473-07
ISBN-10: 1-933477-47-4
ISBN: 978-1-933477-47-3
... to each familial syndrome are depicted in the specific chapters describing adrenal cortical nodular diseases ( chapter 4 ), adenoma ( chapter 5 ), and carcinoma ( chapter 6 ). The Beckwith-Wiedemann syndrome is specifically described in chapter 2 dealing with adrenal congenital anomalies. Table 7-1...
Book Chapter
Series: Atlases of Tumor and Non-Tumor Pathology, Series 5
Volume: 21
Published: 01 December 2024
10.55418/9781933477473-08
ISBN-10: 1-933477-47-4
ISBN: 978-1-933477-47-3
... reported with SDHB, MAX, and TMEM127 germline mutations ( 121 – 124 ) and is seen in patients with Beckwith-Wiedemann syndrome ( 125 ). The signs and symptoms are typically less severe than with pheochromocytoma ( 119 ). Rarely, adrenal medullary hyperplasia, particularly diffuse...
Book Chapter
Series: Atlases of Tumor and Non-Tumor Pathology, Series 5
Volume: 21
Published: 01 December 2024
10.55418/9781933477473-12
ISBN-10: 1-933477-47-4
ISBN: 978-1-933477-47-3
... arteriovenous malformation ( 86 ), endometriosis ( 87 ), polycystic kidney disease ( 88 ), Beckwith-Wiedemann syndrome ( 8 ), neuroblastoma ( 79 ), and teratoma ( 65 ). Adrenal cysts can occur at any age, but are more commonly seen in the 5th and 6th decades of life, with a female predominance ( 82 , 85...
Book Chapter
Series: Atlases of Tumor and Non-Tumor Pathology, Series 5
Volume: 21
Published: 01 December 2024
10.55418/9781933477473-06
ISBN-10: 1-933477-47-4
ISBN: 978-1-933477-47-3
... Cancer 2000 ; 28 : 145 - 2 . 193. Henry I , Jeanpierre M , Couillin P , et al . Molecular definition of the 11p15.5 region involved in Beckwith-Wiedemann syndrome and probably in predisposition to adrenocortical carcinoma . Hum Genet 1989 ; 81 : 273 - 7 . 194. Gicquel...
Book Chapter
Series: Atlases of Tumor and Non-Tumor Pathology, Series 5
Volume: 19
Published: 01 February 2024
10.55418/9781933477428-21
ISBN-10: 1-933477-42-3
ISBN: 978-1-933477-42-8
.... Haas JE , Palmer NF , Weinberg AG , Beckwith JB . Ultrastructure of malignant rhabdoid tumor of the kidney. A distinctive renal tumor of children . Hum Pathol 1981 ; 12 : 646 - 57 . 7. Bittesini L , Dei Tos AP , Fletcher CD . Metastatic malignant melanoma...
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 about this image found in ISLET HYPERPLASIA IN BECKWITH-WIEDEMANN SYNDROME There are enlarged islets...
Book Chapter
Book: Tumors of the Pancreas
Series: Atlases of Tumor and Non-Tumor Pathology, Series 5
Volume: 15
Published: 01 March 2023
10.55418/9781933477275-10
ISBN-10: 1-933477-27-X
ISBN: 978-1-933477-27-5
... Pathology , 3rd Series, Fascicle 20 . Washington DC : American Registry of Pathology ; 1997 : 53 - 64 . 4. Lee CT , Tung YC , Hwu WL , et al Mosaic paternal haploidy in a patient with pancreatoblastoma and Beckwith-Wiedemann spectrum . Am J Med Genet A 2019 ; 179 : 1878 - 83...
Book Chapter
Book: Tumors of the Pancreas
Series: Atlases of Tumor and Non-Tumor Pathology, Series 5
Volume: 15
Published: 01 March 2023
10.55418/9781933477275-15
ISBN-10: 1-933477-27-X
ISBN: 978-1-933477-27-5
... in the diffuse islets are positive. 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. FOCAL NESIDIOBLASTOSIS Figure 15-71 FOCAL NESIDIOBLASTOSIS Clustered...
Book Chapter
Book: Tumors of the Pancreas
Series: Atlases of Tumor and Non-Tumor Pathology, Series 5
Volume: 15
Published: 01 March 2023
10.55418/9781933477275-09
ISBN-10: 1-933477-27-X
ISBN: 978-1-933477-27-5
... 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
10.55418/9781933477251-4
ISBN-10: 1-933477-25-3
ISBN: 978-1-933477-25-1
... reported in up to 69 percent of cases of hydrops fetalis in some series ( 106 , 108 , 110 , 113 ). Adrenal cytomegaly is also a well-known finding in Beckwith-Wiedemann syndrome and is often seen in cases with associated adrenal hemorrhage ( 108 , 110 , 114 , 115 ). Although the pathogenesis of adrenal...
Book Chapter
Series: Atlases of Tumor and Non-Tumor Pathology, Series 5
Volume: 13
Published: 01 November 2022
10.55418/9781933477251-5
ISBN-10: 1-933477-25-3
ISBN: 978-1-933477-25-1
... diazoxide-responsive congenital hyperinsulinism, 24 (42 percent) had GLUD1 mutations, 23 (41 percent) had dominant KATP (19 ABCC8, 4 KCNJ11) mutations, and the rest had mutations of HADH, UCP2, HNF4A, or HNF1a. Beckwith-Wiedemann, Turner, and Kabuki syndromes...
Book Chapter
Series: Atlases of Tumor and Non-Tumor Pathology, Series 5
Volume: 12
Published: 01 August 2022
10.55418/9781933477220-11
ISBN-10: 1-933477-22-9
ISBN: 978-1-933477-22-0
... may occur on an idiopathic basis, in association with hypothyroidism, in patients with fragile X syndrome, in those with McCune-Albright and Beckwith-Wiedemann syndromes, in prepubertal hypothyroidism, in follicle-stimulating hormone (FSH)-secreting pituitary adenomas, in androgen-producing...
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 about this image found in DYSPLASTIC MEDULLARY RAY NODULE Left: This lesion is an extension of the m...
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 about this image found in POTENTIAL FATES OF NEPHROGENIC RESTS In this figure, gray areas represent ...
Book Chapter
Series: Atlases of Tumor and Non-Tumor Pathology, Series 5
Volume: 9
Published: 01 November 2021
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 about this image found in PLACENTAL MESENCHYMAL DYSPLASIA Transabdominal image demonstrating a thick...
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