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Book Chapter
Series: Atlases of Tumor and Non-Tumor Pathology, Series 5
Volume: 21
Published: 01 December 2024
10.55418/9781933477473-10
ISBN-10: 1-933477-47-4
ISBN: 978-1-933477-47-3
... tumors is characterized by the production of dopamine, with or without norepinephrine. Mutations of Krebs cycle-related genes, including members of the SDH complex or fumarate hydratase (FH), are implicated primarily in dopamine production, often referred to as “immature biochemical phenotype...
Images
in Pheochromocytoma and Adrenal Medullary Hyperplasia
> Tumors of the Adrenal Glands and Extra-Adrenal Paraganglia
Published: 01 December 2024
PHEOCHROMOCYTOMA: ONCOCYTIC CHANGE IN SDHX-RELATED DISEASE Figure 8-34 PHEOCHROMOCYTOMA: ONCOCYTIC CHANGE IN SDHX-RELATED DISEASE Some pheochromocytomas in patients with SDHx-related disease have abundant granular eosinophilic cytoplasm. More about this image found in PHEOCHROMOCYTOMA: ONCOCYTIC CHANGE IN SDHX-RELATED DISEASE Some pheochromo...
Images
in Pheochromocytoma and Adrenal Medullary Hyperplasia
> Tumors of the Adrenal Glands and Extra-Adrenal Paraganglia
Published: 01 December 2024
PHEOCHROMOCYTOMA: SDHB STAINING Figure 8-43 PHEOCHROMOCYTOMA: SDHB STAINING A: Loss of SDHB immunoreactivity with retained positivity in the stroma serving as an internal control provides strong evidence of SDHX-related disease and should prompt genetic investigation. B: The presence of intact reactivity for SDHB suggests that the tumor is not related to SDHx mutation. More about this image found in PHEOCHROMOCYTOMA: SDHB STAINING A: Loss of SDHB immunoreactivity with reta...
Images
in Pheochromocytoma and Adrenal Medullary Hyperplasia
> Tumors of the Adrenal Glands and Extra-Adrenal Paraganglia
Published: 01 December 2024
VHL-ASSOCIATED PHEOCHROMOCYTOMA: CAIX STAINING Figure 8-45 VHL-ASSOCIATED PHEOCHROMOCYTOMA: CAIX STAINING CAIX positivity is associated with VHL-related disease; it may be diffuse or focal as in this case. More about this image found in VHL-ASSOCIATED PHEOCHROMOCYTOMA: CAIX STAINING CAIX positivity is associat...
Images
in Pheochromocytoma and Adrenal Medullary Hyperplasia
> Tumors of the Adrenal Glands and Extra-Adrenal Paraganglia
Published: 01 December 2024
PHEOCHROMOCYTOMA: INHIBIN STAINING Figure 8-44 PHEOCHROMOCYTOMA: INHIBIN STAINING The presence of inhibin positivity is associated with any hypoxic pathway disease, including SDHx- and VHL-related disease and can be confirmed by staining for SDHB and CAIX. More about this image found in PHEOCHROMOCYTOMA: INHIBIN STAINING The presence of inhibin positivity is a...
Images
in Pheochromocytoma and Adrenal Medullary Hyperplasia
> Tumors of the Adrenal Glands and Extra-Adrenal Paraganglia
Published: 01 December 2024
FUMARATE HYDRATASE (FH)-ASSOCIATED PHEOCHROMOCYTOMA Figure 8-46 FUMARATE HYDRATASE (FH)-ASSOCIATED PHEOCHROMOCYTOMA A,B: The histologic features of FH-related pheochromocytoma are not distinctive. C,D: Immunohistochemical staining for FH shows loss of cytoplasmic reactivity with retained stainin... More about this image found in FUMARATE HYDRATASE (FH)-ASSOCIATED PHEOCHROMOCYTOMA A,B: The histologic fe...
Images
in Pheochromocytoma and Adrenal Medullary Hyperplasia
> Tumors of the Adrenal Glands and Extra-Adrenal Paraganglia
Published: 01 December 2024
FUMARATE HYDRATASE (FH)-ASSOCIATED PHEOCHROMOCYTOMA Figure 8-46 FUMARATE HYDRATASE (FH)-ASSOCIATED PHEOCHROMOCYTOMA A,B: The histologic features of FH-related pheochromocytoma are not distinctive. C,D: Immunohistochemical staining for FH shows loss of cytoplasmic reactivity with retained stainin... More about this image found in FUMARATE HYDRATASE (FH)-ASSOCIATED PHEOCHROMOCYTOMA A,B: The histologic fe...
Images
in Pheochromocytoma and Adrenal Medullary Hyperplasia
> Tumors of the Adrenal Glands and Extra-Adrenal Paraganglia
Published: 01 December 2024
ADRENAL MEDULLARY HYPERPLASIA Figure 8-59 ADRENAL MEDULLARY HYPERPLASIA Adrenal glands from patients with MEN2 (A) and SDHB-related disease (B) show diffuse expansion of the medulla that is much larger than the adrenal cortex on either side. While this is considered to be diffuse hyperplasia, the... More about this image found in ADRENAL MEDULLARY HYPERPLASIA Adrenal glands from patients with MEN2 (A) a...
Images
Published: 01 December 2024
ANCILLARY STAINING IN THE DIFFERENTIAL DIAGNOSIS OF ADRENAL CORTICAL ADENOMA AND CARCINOMA Figure 5-31 ANCILLARY STAINING IN THE DIFFERENTIAL DIAGNOSIS OF ADRENAL CORTICAL ADENOMA AND CARCINOMA A diagnosis of adrenal cortical carcinoma was suspected because of the presence of predominant oncocyti... More about this image found in ANCILLARY STAINING IN THE DIFFERENTIAL DIAGNOSIS OF ADRENAL CORTICAL ADENOM...
Images
in Hereditary Predisposition to Adrenal Cortical Lesions
> Tumors of the Adrenal Glands and Extra-Adrenal Paraganglia
Published: 01 December 2024
ADRENAL CORTICAL TUMOR IN A PATIENT HARBORING ARMC5 GERMLINE VARIANT Figure 7-2 ADRENAL CORTICAL TUMOR IN A PATIENT HARBORING ARMC5 GERMLINE VARIANT The adrenal mass (A) is composed of a peripheral benign macronodular component (A, upper left), and is associated with a central component made of eosinophilic cells (B, lower right) showing malignancy-related features, including increased mitotic activity (B, red circle) for a total Weiss score of 4. More about this image found in ADRENAL CORTICAL TUMOR IN A PATIENT HARBORING ARMC5 GERMLI...
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
... World Health Organization (WHO) nomenclature maintains the term pheochromocytoma for the intra-adrenal location while structurally and functionally related extra-adrenal tumors are classified as paragangliomas ( 4 , 5 ). This arbitrary convention dates to the 1950 Armed Forces...
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
...Adrenal proliferations (including nodular diseases and tumors) can occur in the context of familial or syndromic diseases, with a wide variety of clinical presentations and molecular backgrounds ( Table 7-1 ). The main features and morphofunctional characteristics of pathologic lesions related...
Book Chapter
Series: Atlases of Tumor and Non-Tumor Pathology, Series 5
Volume: 21
Published: 01 December 2024
10.55418/9781933477473-09
ISBN-10: 1-933477-47-4
ISBN: 978-1-933477-47-3
... that these anatomically dispersed structures consisted of cells developmentally, structurally, and functionally related to each other and to sympathetic ganglia. On the basis of that analogy, he coined the term paraganglia ( 1 ). While Kohn’s analogy is still largely viable, it is now recognized that there is more...
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
... variable clinical presentations. It may be an incidental finding, or may cause signs and symptoms related to the presence of an abdominal mass. Some present with clinically overt metastatic disease or features of hormone hypersecretion ( 34 ). These different clinical scenarios require a multidisciplinary...
Book Chapter
Series: Atlases of Tumor and Non-Tumor Pathology, Series 5
Volume: 21
Published: 01 December 2024
10.55418/9781933477473-05
ISBN-10: 1-933477-47-4
ISBN: 978-1-933477-47-3
... is mostly related to the incidental discovery of an adrenal mass on radiologic imaging. As discussed above, a complete physical, radiologic, and endocrinologic evaluation is mandatory to exclude subclinical hormone hypersecretion. Many nonfunctioning tumors are capable of taking up radiolabeled steroid...
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
... differentiated, and differentiating, on the basis of specific histopathologic criteria (see below in Microscopic Findings). Ganglioneuroblastoma (GNBL) is a closely related neoplasm showing variable cytodifferentiation into ganglion cells, accompanied by a spindle cell schwannian stroma. There are two...
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
... embryologic techniques showing progenitors with predetermined fates and separate routes of migration contributing to the development of these two closely related cell types. While cells destined for sympathetic ganglia predominantly migrate to their destinations directly from the neural crest, the adrenal...
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
..., or metastases to the adrenal gland from distant sites. There are also a few case reports of both endocrine and nonendocrine tumors potentially related to the adrenal gland developmentally, either by steroidogenic lineage or by proximity to the adrenogenital/gonadal ridge (see chapter 1 ). Non-neoplastic causes...
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
... oversecretion. Cushing syndrome due to ectopic ACTH/corticotrophin-releasing hormone (CRH) production is a disease of adult men, while most patients with ACTH-dependent Cushing syndrome related to a pituitary tumor are women of reproductive age. In children under the age of 7 years, an adrenal cortical neoplasm...
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
... leiomyomatosis, and renal cancer syndromes, among others ( chapter 7 ). Germline mutations in the ARMC5 gene have been detected in a large proportion (20 to 30 percent) of apparently sporadic cases of PBMAD ( 23 ). ARMC5-related macronodular disease has no gender predominance and often has...
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