ADRENAL CORTICAL CARCINOMA IN A MALE PATIENT WITH BRCA2 GERMLINE MUTATION Figure 7-9 ADRENAL CORTICAL CARCINOMA IN A MALE PATIENT WITH BRCA2 GERMLINE MUTATION The patient had a previous diagnosis of prostate cancer. The adrenal lesion showed predominant features of the myxoid variant. More about this image found in ADRENAL CORTICAL CARCINOMA IN A MALE PATIENT WITH BRCA2 GE...

ADRENAL CORTICAL ADENOMA IN A PATIENT WITH FH GERMLINE MUTATION Figure 7-7 ADRENAL CORTICAL ADENOMA IN A PATIENT WITH FH GERMLINE MUTATION This patient had a previous renal cell carcinoma and a Leydig cell tumor. The adenoma shows a nodular aspect without cytologic atypia or other worrisome cellular or architectural features. Preexistent cortex is present at the top of the image. More about this image found in ADRENAL CORTICAL ADENOMA IN A PATIENT WITH FH GERMLINE MUT...

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...

PRIMARY BILATERAL MACRONODULAR ADRENAL CORTICAL DISEASE IN A PATIENT WITH A KNOWN ARMC5 MUTATION AND SUBCLINICAL CUSHING SYNDROME Figure 7-1 PRIMARY BILATERAL MACRONODULAR ADRENAL CORTICAL DISEASE IN A PATIENT WITH A KNOWN ARMC5 MUTATION AND SUBCLINICAL CUSHING SYNDROME A: Multiple adrenal cortical nodules of varying size are present throughout the adrenal gland (representative sections). B: Higher magnification of two additional sections showing that the residual adrenal cortex away from nodules is atrophic, consistent with autonomous function of the nodular areas. More about this image found in PRIMARY BILATERAL MACRONODULAR ADRENAL CORTICAL DISEASE IN A PATIENT WITH A...

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...

ABDOMINAL PARAGANGLIOMA Figure 9-13 ABDOMINAL PARAGANGLIOMA A: The tumor, from a patient with a hereditary mutation of the SDHB gene (same specimen as in fig. 9-11 ), has mixed nested and trabecular architecture and is composed of monomorphic cells with slightly eosinophilic cytoplasm. This appearance is often associated with SDHB mutation. B: The strong, diffuse expression of synaptophysin highlights a mixed pattern of nested, trabecular, and diffuse histoarchitecture. C: Immunohistochemical stain for tyrosine hydroxylase shows intratumoral heterogeneity at the functional level, with abrupt transition from strong, diffuse expression to absent expression in a small region of the tumor. D: Immunohistochemical stain for S-100 protein shows heterogeneous distribution of sustentacular cells in adjacent areas of tumor. E: Immunohistochemical stain for glial fibrillary acidic protein (GFAP) shows a small focus of GFAP-positive sustentacular cells in a tumor in which S-100 protein-positive cells were much more numerous. More about this image found in ABDOMINAL PARAGANGLIOMA A: The tumor, from a patient with a hereditary mut...

ABDOMINAL PARAGANGLIOMA Figure 9-25 ABDOMINAL PARAGANGLIOMA This tumor is composed of monomorphic cells with slightly eosinophilic cytoplasm and prominent vacuoles creating a pseudoacinar appearance. This appearance is often associated with SDHB mutation. More about this image found in ABDOMINAL PARAGANGLIOMA This tumor is composed of monomorphic cells with s...

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

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...

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 ...