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paraganglioma
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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
...Pheochromocytomas and paragangliomas (PPGLs) are a group of tumors with an extraordinarily high genetic predisposition. Approximately 40 percent of patients with these tumors harbor a germline mutation conveying susceptibility to the development of these and other neoplasms ( 1...
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
... paragangliomas in different anatomic locations and hereditary syndromes ( 7 , 8 ). Familiarity with the normal distribution of paraganglia is important because paragangliomas occur with the greatest frequency in areas where paraganglionic tissue is most abundant during development or in adult life, and occur...
Images
Published: 01 December 2024
URINARY BLADDER PARAGANGLIOMA Figure 9-35 URINARY BLADDER PARAGANGLIOMA A: This extensively invasive urinary bladder paraganglioma deep within the bladder wall is a potential mimic of prostatic or urothelial carcinoma. B: Diffuse expression of tyrosine hydroxylase confirms the identity of the tu... More about this image found in URINARY BLADDER PARAGANGLIOMA A: This extensively invasive urinary bladder...
Images
Published: 01 December 2024
ABDOMINAL PARAGANGLIOMA Figure 9-11 ABDOMINAL PARAGANGLIOMA This paraganglioma from the abdomen at the origin of the superior mesenteric artery shows a groove (arrow) where the specimen abutted the artery. Bulging areas of fleshy pink-gray and pink-tan tissue contrast with slightly retracted area... More about this image found in ABDOMINAL PARAGANGLIOMA This paraganglioma from the abdomen at the origin ...
Images
Published: 01 December 2024
MESENTERIC PARAGANGLIOMA Figure 9-14 MESENTERIC PARAGANGLIOMA A: This paraganglioma composed of basophilic and clear cells was incidentally discovered in an asymptomatic patient with no other tumors. Fat invasion, poorly defined irregular cell nests, and diffuse growth are putative risk factors f... More about this image found in MESENTERIC PARAGANGLIOMA A: This paraganglioma composed of basophilic and ...
Images
Published: 01 December 2024
URINARY BLADDER PARAGANGLIOMA Figure 9-18 URINARY BLADDER PARAGANGLIOMA This paraganglioma shows extensive superficial growth beneath the mucosa. The tumor is a potential mimic of urothelial carcinoma because of its growth pattern and pale eosinophilic to clear cytoplasm. More about this image found in URINARY BLADDER PARAGANGLIOMA This paraganglioma shows extensive superfici...
Images
Published: 01 December 2024
URINARY BLADDER PARAGANGLIOMA Figure 9-22 URINARY BLADDER PARAGANGLIOMA A: This paraganglioma extends between muscle bundles in the bladder wall and is a potential mimic of other more aggressive neoplasms because of its growth pattern. B: Higher magnification of the tumor shows that most neoplas... More about this image found in URINARY BLADDER PARAGANGLIOMA A: This paraganglioma extends between muscle...
Images
Published: 01 December 2024
SDH-DEFICIENT ABDOMINAL PARAGANGLIOMA Figure 9-26 SDH-DEFICIENT ABDOMINAL PARAGANGLIOMA This paraganglioma with a known SDHB mutation exhibits small intracytoplasmic vacuoles with flocculent content, which are a well-known characteristic of SDH-deficient renal cell carcinoma but extremely rare in SDH-deficient paraganglioma. More about this image found in SDH-DEFICIENT ABDOMINAL PARAGANGLIOMA This paraganglioma with a known
Images
Published: 01 December 2024
CARDIAC PARAGANGLIOMA Figure 9-31 CARDIAC PARAGANGLIOMA This primary cardiac paraganglioma infiltrates myocardium at lower right. More about this image found in CARDIAC PARAGANGLIOMA This primary cardiac paraganglioma infiltrates myoca...
Images
Published: 01 December 2024
PULMONARY PARAGANGLIOMA Figure 9-32 PULMONARY PARAGANGLIOMA A: This paraganglioma was a solitary lesion initially interpreted as an epithelial pulmonary NET. The patient was found to have a hereditary SDHC mutation. No other primary tumors were found and this tumor contained abundant sustentacular cells. In view of the clinical and histologic findings and the low metastasis frequency associated with SDHC mutation, the tumor was consistent with a primary pulmonary paraganglioma. B: High magnification shows nested architecture and cells of varied sizes. C: S-100 protein highlights sustentacular cells. More about this image found in PULMONARY PARAGANGLIOMA A: This paraganglioma was a solitary lesion initia...
Images
in Familial and Syndrome-Related Pheochromocytoma and Paraganglioma
> Tumors of the Adrenal Glands and Extra-Adrenal Paraganglia
Published: 01 December 2024
SDH-DEFICIENT PARAGANGLIOMA Figure 10-5 SDH-DEFICIENT PARAGANGLIOMA A: This cardiac paraganglioma from a patient with a pathogenic SDHB variant has round nesting architecture, oncocytic cytology with abundant eosinophilic cytoplasm, and focal tendency to form pseudorosettes. B: SDHB immunohistochemistry is negative, with retained positivity in vascular endothelium serving as an internal positive control. More about this image found in SDH-DEFICIENT PARAGANGLIOMA A: This cardiac paraganglioma from a patient w...
Images
Published: 01 December 2024
CAROTID BODY SCLEROSING PARAGANGLIOMA Figure 9-19 CAROTID BODY SCLEROSING PARAGANGLIOMA A: Sclerosing paragangliomas have been reported as a distinct entity that can mimic infiltrating carcinoma. This sharply circumscribed example shows extensive sclerosis, which is very dense at top right. B: S... More about this image found in CAROTID BODY SCLEROSING PARAGANGLIOMA A: Sclerosing paragangliomas have be...
Images
Published: 01 December 2024
SITES OF PARAGANGLIOMA DEVELOPMENT Figure 9-1 SITES OF PARAGANGLIOMA DEVELOPMENT The relationship between the distribution of extra-adrenal chromaffin tissue in neonates (A) and prevalent sites of sympathetic paragangliomas reported up to 1965 (B) in a classic illustration. Arrow added in A indic... More about this image found in SITES OF PARAGANGLIOMA DEVELOPMENT The relationship between the distributi...
Images
in Familial and Syndrome-Related Pheochromocytoma and Paraganglioma
> Tumors of the Adrenal Glands and Extra-Adrenal Paraganglia
Published: 01 December 2024
PHEOCHROMOCYTOMA AND PARAGANGLIOMA ASSOCIATED WITH GERMLINE FUMARATE HYDRATASE (FH) MUTATION Figure 10-6 PHEOCHROMOCYTOMA AND PARAGANGLIOMA ASSOCIATED WITH GERMLINE FUMARATE HYDRATASE (FH) MUTATION A: The pheochromocytoma is associated with an unusual angiomatous proliferation of the cortical vas... More about this image found in PHEOCHROMOCYTOMA AND PARAGANGLIOMA ASSOCIATED WITH GERMLINE FUMARATE HYDRAT...
Images
Published: 01 December 2024
DUODENAL PARAGANGLIOMA Figure 9-33 DUODENAL PARAGANGLIOMA A: This tumor was initially interpreted as an epithelial intestinal NET. However, it was negative for keratins and expressed paraganglioma markers. The absence of keratins also weighed against a diagnosis of composite gangliocytoma/neuroma... More about this image found in DUODENAL PARAGANGLIOMA A: This tumor was initially interpreted as an epith...
Images
Published: 01 December 2024
VAGAL PARAGANGLIOMA Figure 9-28 VAGAL PARAGANGLIOMA Pseudopapillary architecture resulting from intra operative hemorrhage causes this paraganglioma to resemble a papillary carcinoma. More about this image found in VAGAL PARAGANGLIOMA Pseudopapillary architecture resulting from intra oper...
Images
Published: 01 December 2024
CAROTID BODY PARAGANGLIOMA Figure 9-37 CAROTID BODY PARAGANGLIOMA Immunohistochemical stains for S-100 protein and SOX10 outline the nested architecture in carotid body paraganglioma. More about this image found in CAROTID BODY PARAGANGLIOMA Immunohistochemical stains for S-100 protein an...
Images
Published: 01 December 2024
CAROTID BODY PARAGANGLIOMA Figure 9-38 CAROTID BODY PARAGANGLIOMA Immunohistochemical stain for CD163 shows macro phages in a paraganglioma that closely mimic the distribution of sustentacular cells. More about this image found in CAROTID BODY PARAGANGLIOMA Immunohistochemical stain for CD163 shows macro...
Images
Published: 01 December 2024
MIDDLE EAR (JUGULOTYMPANIC) PARAGANGLIOMA Figure 9-23 MIDDLE EAR (JUGULOTYMPANIC) PARAGANGLIOMA The presence of nuclear pleomorphism, together with artifactual cytoplasmic vacuolization and clearing in this paraganglioma, may suggest adenocarcinoma as a diagnosis. More about this image found in MIDDLE EAR (JUGULOTYMPANIC) PARAGANGLIOMA The presence of nuclear pleomorp...
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Published: 01 December 2024
CAROTID BODY PARAGANGLIOMA Figure 9-24 CAROTID BODY PARAGANGLIOMA A: The almost entirely spindle cell composition of this paraganglioma raises the possibility of mesenchymal and other spindle cell neoplasms. B: Reticulin stain highlights the spindle cell pattern. More about this image found in CAROTID BODY PARAGANGLIOMA A: The almost entirely spindle cell composition...
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