case_images: PMC7249266_01
case_images: Patient pedigree [proband positive for MAX mutation c299G>C (p.Arg100Pro, NM_00.42382)]. She presented clinical signs of multiple PGL and composite PCCs; proband's father had a glioblastoma multiforme, and genetic testing was not performed; proband's son is negative for clinical manifestations, and genetic analyses were not performed.
case_images: fendo-11-00234-g0001.jpg
case_images: PMC7249266_02_fendo-11-00234-g0001.jpg
case_images: F1
case_images: The trend of NE markers over the course of the clinical history is shown in Figure S1.
case_images: Timing of the presentation of multiple and composite NEN-neural tumors in this patient. GNB, ganglioneuroblastoma; CHT, chemotherapy; RT, radiotherapy; PCC, pheochromocytoma; GN, ganglioneuroma; PGL, paraganglioma.
case_images: fendo-11-00234-g0002.jpg
case_images: PMC7249266_01_fendo-11-00234-g0002.jpg
case_images: F2
case_images: PMC7249266_02
case_images: Patient pedigree [proband positive for MAX mutation c299G>C (p.Arg100Pro, NM_00.42382)]. She presented clinical signs of multiple PGL and composite PCCs; proband's father had a glioblastoma multiforme, and genetic testing was not performed; proband's son is negative for clinical manifestations, and genetic analyses were not performed.
case_images: fendo-11-00234-g0001.jpg
case_images: PMC7249266_02_fendo-11-00234-g0001.jpg
case_images: F1
case_images: The trend of NE markers over the course of the clinical history is shown in Figure S1.
case_images: Top: 131I-metaiodobenzylguanidine (MIBG) scintigraphy distinctly shows a hot spot in the inter-aortocaval region, as seen in the (A) transverse, (B) sagittal, and (C) coronal scans. Bottom: Histological appearance of the paraganglioma, cords and nests of cellular elements with abundant granular basophilic cytoplasm and ovoid nuclei with prominent nucleoli. (D) EE 10x. (E) EE 20x.
case_images: fendo-11-00234-g0003.jpg
case_images: PMC7249266_02_fendo-11-00234-g0003.jpg
case_images: F3
case_images: MIBG scintigraphy showed a distinct hot spot in that region (Figure 3). The histological diagnosis was inter-aortocaval PGL (see Figure 3).
case_images: Adrenal CT scan. The two slices (A,B) show four nodules in the left adrenal gland measuring from 10 to 18 mm that are unattenuated after contrast administration (red arrows), compatible with left multifocal adrenal pheochromocytomas.
case_images: fendo-11-00234-g0004.jpg
case_images: PMC7249266_02_fendo-11-00234-g0004.jpg
case_images: F4
case_images: Adrenal washout CT scans showed four non-adenomatous left adrenal masses between 10 and 18 mm (Figure 4).