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congenital
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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
...Congenital adrenal gland abnormalities may manifest anatomically, hormonally, or as a combination of the two, and thus present challenges to pathologists and clinicians. A common occurrence is the need to distinguish ectopic cortical tissue from tumor in cancer staging, abdominal surgery...
Images
in Congenital Disorders of the Adrenal Gland
> Tumors of the Adrenal Glands and Extra-Adrenal Paraganglia
Published: 01 December 2024
ADRENAL CORTICAL TISSUE IN A CONGENITAL DYSPLASTIC KIDNEY Figure 2-6 ADRENAL CORTICAL TISSUE IN A CONGENITAL DYSPLASTIC KIDNEY This dysplastic kidney removed from a 5-week-old girl contains a prominent focus of benign immature adrenal cortex. More about this image found in ADRENAL CORTICAL TISSUE IN A CONGENITAL DYSPLASTIC KIDNEY This dysplastic ...
Images
in Congenital Disorders of the Adrenal Gland
> Tumors of the Adrenal Glands and Extra-Adrenal Paraganglia
Published: 01 December 2024
CONGENITAL ADRENAL HYPERPLASIA Figure 2-10 CONGENITAL ADRENAL HYPERPLASIA This 18-month-old female was mistakenly assigned a male gender. The markedly enlarged clitoris resembles a penis. The hypertrophied clitoris had a urethral opening near the base. The rugosity of the partially fused labio-sc... More about this image found in CONGENITAL ADRENAL HYPERPLASIA This 18-month-old female was mistakenly ass...
Images
in Congenital Disorders of the Adrenal Gland
> Tumors of the Adrenal Glands and Extra-Adrenal Paraganglia
Published: 01 December 2024
CONGENITAL ADRENAL HYPERPLASIA Figure 2-11 CONGENITAL ADRENAL HYPERPLASIA Autopsy of an infant who died of the severe salt-losing form of 21-hydroxylase deficiency shows enlargement of both adrenal glands (arrows). The glands have a convoluted or cerebriform surface and are darker than normal due... More about this image found in CONGENITAL ADRENAL HYPERPLASIA Autopsy of an infant who died of the severe...
Images
in Congenital Disorders of the Adrenal Gland
> Tumors of the Adrenal Glands and Extra-Adrenal Paraganglia
Published: 01 December 2024
CONGENITAL ADRENAL HYPERPLASIA Figure 2-12 CONGENITAL ADRENAL HYPERPLASIA A: Marked expansion of the zona fasciculata in an older child with congenital adrenal hyperplasia (CAH). Many cells have lipid-depleted cytoplasm due to sustained trophic stimulation by ACTH. B: Hyperplastic zona fascicula... More about this image found in CONGENITAL ADRENAL HYPERPLASIA A: Marked expansion of the zona fasciculata...
Images
in Congenital Disorders of the Adrenal Gland
> Tumors of the Adrenal Glands and Extra-Adrenal Paraganglia
Published: 01 December 2024
ADRENAL CORTICAL NEOPLASM ASSOCIATED WITH CONGENITAL ADRENAL HYPERPLASIA Figure 2-13 ADRENAL CORTICAL NEOPLASM ASSOCIATED WITH CONGENITAL ADRENAL HYPERPLASIA A: A large adrenal cortical tumor present on the right is seen next to hyperplastic lipid-depleted adrenal cortex and a normal adrenal medu... More about this image found in ADRENAL CORTICAL NEOPLASM ASSOCIATED WITH CONGENITAL ADRENAL HYPERPLASIA A...
Images
in Congenital Disorders of the Adrenal Gland
> Tumors of the Adrenal Glands and Extra-Adrenal Paraganglia
Published: 01 December 2024
TESTICULAR TUMOR IN CONGENITAL ADRENAL HYPERPLASIA Figure 2-16 TESTICULAR TUMOR IN CONGENITAL ADRENAL HYPERPLASIA Individual cells have distinct cell borders and rounded nuclei, often with one or two eccentric nucleoli. Many cells contain coarse, granular pigment, which is lipofuscin. More about this image found in TESTICULAR TUMOR IN CONGENITAL ADRENAL HYPERPLASIA Individual cells have d...
Images
in Congenital Disorders of the Adrenal Gland
> Tumors of the Adrenal Glands and Extra-Adrenal Paraganglia
Published: 01 December 2024
ADRENAL MYELOLIPOMA IN CONGENITAL ADRENAL HYPERPLASIA Figure 2-17 ADRENAL MYELOLIPOMA IN CONGENITAL ADRENAL HYPERPLASIA A: Huge, bilateral adrenal myelolipomas in an adult male with CAH. The variegated yellow and red coloration results from heterogenous admixture of hematopoietic elements and fat... More about this image found in ADRENAL MYELOLIPOMA IN CONGENITAL ADRENAL HYPERPLASIA A: Huge, bilateral a...
Images
in Other Adrenal Neoplasms and Tumor-Like Lesions
> Tumors of the Adrenal Glands and Extra-Adrenal Paraganglia
Published: 01 December 2024
BILATERAL GIANT ADRENAL MYELOLIPOMAS Figure 12-1 BILATERAL GIANT ADRENAL MYELOLIPOMAS The myelolipomas are from an adult man with poorly controlled congenital adrenal hyperplasia. The red-brown areas contain hematopoietic cells and blood and the yellow patches consist of fat. Yellow-orange bands ... More about this image found in BILATERAL GIANT ADRENAL MYELOLIPOMAS The myelolipomas are from an adult ma...
Book Chapter
Series: Atlases of Tumor and Non-Tumor Pathology, Series 5
Volume: 21
Published: 01 December 2024
10.55418/9781933477473-11
ISBN-10: 1-933477-47-4
ISBN: 978-1-933477-47-3
...-of-function mutations occur, and paired-like homeobox 2B (PHOX2B), in which germline loss-of-function mutations have been described and in which patients may also have central congenital hypoventilation syndrome (Ondine curse) and/or Hirschsprung disease ( 24 – 27 ). Apart from germline mutations...
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
... by the association with congenital adrenal hyperplasia in 10 percent of all patients analyzed, while adrenal hypersecretory disorders, including hypercortisolism, primary hyperaldosteronism, and hyperandrogenism were found in an additional 7.5 percent of cases ( 14 ). These associations are consistent...
Book Chapter
Series: Atlases of Tumor and Non-Tumor Pathology, Series 5
Volume: 21
Published: 01 December 2024
10.55418/9781933477473-04
ISBN-10: 1-933477-47-4
ISBN: 978-1-933477-47-3
... gland, except for congenital adrenal hyperplasia (CAH) ( chapter 2 ) and is accepted for ACTH-dependent adrenal cortical hyperplasia (discussed in chapter 3 ). Within the group of adrenal lesions associated with primary aldosteronism in adults, the term “diffuse hyperplasia” indicates a lesion that may...
Book Chapter
Series: Atlases of Tumor and Non-Tumor Pathology, Series 5
Volume: 21
Published: 01 December 2024
10.55418/9781933477473-03
ISBN-10: 1-933477-47-4
ISBN: 978-1-933477-47-3
... in patients with congenital adrenal hyperplasia ( chapter 2 ) but can also be seen in adrenal cortical neoplasms. Although the production of androgens may be responsible for the hirsutism associated with Cushing syndrome, oversecretion of sex steroid hormones as the main feature of an adrenal cortical...
Book Chapter
Series: Atlases of Tumor and Non-Tumor Pathology, Series 5
Volume: 21
Published: 01 December 2024
10.55418/9781933477473-01
ISBN-10: 1-933477-47-4
ISBN: 978-1-933477-47-3
... be altered by some congenital abnormalities, such as an extremely small size in congenital adrenal hypoplasia and a more rounded contour with ipsilateral renal agenesis. The adrenal glands in anencephalic newborns are small, with an average combined weight that can be less than 1.0 g ( 38 ). In anencephaly...
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
... in pheochromocytoma of multiple endocrine neoplasia type 1 . Endocr Pathol 2023 ; 34 : 156 - 8 . 79. Ladroue C , Carcenac R , Leporrier M , et al . PHD2 mutation and congenital erythrocytosis with paraganglioma . N Engl J Med 2008 ; 359 : 2685 - 92 . 80. Fishbein L...
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...
Images
Published: 01 September 2024
ZIKA CONGENITAL SYNDROME Figure 3-4 ZIKA CONGENITAL SYNDROME Microcephaly can be associated with this disease. (Courtesy of the CDC Zika Virus Webpage https://www.cdc.gov/zika/czs/index.html ) More about this image found in ZIKA CONGENITAL SYNDROME Microcephaly can be associated with this disease....
Images
Published: 01 September 2024
ZIKA VIRUS CONGENITAL SYNDROME Figure 3-7 ZIKA VIRUS CONGENITAL SYNDROME Prominent hydrocephaly (A) and mononuclear inflammatory cell infiltration (B) are seen. (Courtesy of the CDC IDPB) More about this image found in ZIKA VIRUS CONGENITAL SYNDROME Prominent hydrocephaly (A) and mononuclear ...
Images
Published: 01 September 2024
PRIMARY, SECONDARY, AND CONGENITAL SYPHILIS: CLINICAL FEATURES Figure 18-1 PRIMARY, SECONDARY, AND CONGENITAL SYPHILIS: CLINICAL FEATURES Primary syphilis: chancre on the penis (A) and tongue (B). (Courtesy of the Centers for Disease Control and Prevention [CDC] Public Health Image Library [PHIL]... More about this image found in PRIMARY, SECONDARY, AND CONGENITAL SYPHILIS: CLINICAL FEATURES Primary syp...
Images
Published: 01 September 2024
PRIMARY, SECONDARY, AND CONGENITAL SYPHILIS: CLINICAL FEATURES Figure 18-1 PRIMARY, SECONDARY, AND CONGENITAL SYPHILIS: CLINICAL FEATURES Primary syphilis: chancre on the penis (A) and tongue (B). (Courtesy of the Centers for Disease Control and Prevention [CDC] Public Health Image Library [PHIL]... More about this image found in PRIMARY, SECONDARY, AND CONGENITAL SYPHILIS: CLINICAL FEATURES Primary syp...
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