DOI: 10.1148/rg.262055073
Multiple Endocrine Neoplasia: Spectrum of Radiologic Appearances and Discussion of a Multitechnique Imaging Approach1
Andrew F. Scarsbrook, FRCR,
Rajesh V. Thakker, FRCP,
John A. H. Wass, FRCP,
Fergus V. Gleeson, FRCR and
Rachel R. Phillips, FRCR
1 From the Department of Radiology, Churchill Hospital, Oxford Radcliffe NHS Trust, Oxford, England (A.F.S., F.V.G., R.R.P.), and the Academic Endocrine Unit, Nuffield Department of Medicine (R.V.T.) and the Department of Clinical Endocrinology (J.A.H.W.), Oxford Centre for Diabetes, Endocrinology & Metabolism, University of Oxford, Oxford, England. Recipient of Certificate of Merit and Excellence in Design awards for an education exhibit at the 2004 RSNA Annual Meeting. Received March 30, 2005; revision requested May 3 and received June 22; accepted June 23. R.V.T. is supported by the Medical Research Council of England; all remaining authors have no financial relationships to disclose.
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Figure 1a. Parathyroid imaging in MEN 1: US and computed tomography (CT). (a) Parathyroid adenoma. US image of the neck demonstrates the typical appearance of a parathyroid adenoma in MEN 1: a well-defined, oval hypoechoic mass posterior to the thyroid gland. (b) Parathyroid adenoma in a different patient. Contrast material–enhanced CT scan of the neck shows a right inferior parathyroid adenoma (arrow).
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Figure 1b. Parathyroid imaging in MEN 1: US and computed tomography (CT). (a) Parathyroid adenoma. US image of the neck demonstrates the typical appearance of a parathyroid adenoma in MEN 1: a well-defined, oval hypoechoic mass posterior to the thyroid gland. (b) Parathyroid adenoma in a different patient. Contrast material–enhanced CT scan of the neck shows a right inferior parathyroid adenoma (arrow).
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Figure 2a. Parathyroid imaging in MEN: MR imaging. (a) Parathyroid adenoma. Coronal T2-weighted MR image shows a right parathyroid adenoma with homogeneous high signal intensity (arrow) in the neck. (b, c) Parathyroid adenoma in a patient who presented with persistent hypercalcemia. The patient had previously undergone subtotal parathyroidectomy. (b) Coronal T1-weighted MR image shows a bilobed, low-signal-intensity, left mediastinal parathyroid adenoma (arrow) adjacent to the aortic arch. (c) Corresponding technetium 99m (99mTc)–sestamibi (MIBI) scintigram shows increased radiotracer uptake within the adenoma.
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Figure 2b. Parathyroid imaging in MEN: MR imaging. (a) Parathyroid adenoma. Coronal T2-weighted MR image shows a right parathyroid adenoma with homogeneous high signal intensity (arrow) in the neck. (b, c) Parathyroid adenoma in a patient who presented with persistent hypercalcemia. The patient had previously undergone subtotal parathyroidectomy. (b) Coronal T1-weighted MR image shows a bilobed, low-signal-intensity, left mediastinal parathyroid adenoma (arrow) adjacent to the aortic arch. (c) Corresponding technetium 99m (99mTc)–sestamibi (MIBI) scintigram shows increased radiotracer uptake within the adenoma.
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Figure 2c. Parathyroid imaging in MEN: MR imaging. (a) Parathyroid adenoma. Coronal T2-weighted MR image shows a right parathyroid adenoma with homogeneous high signal intensity (arrow) in the neck. (b, c) Parathyroid adenoma in a patient who presented with persistent hypercalcemia. The patient had previously undergone subtotal parathyroidectomy. (b) Coronal T1-weighted MR image shows a bilobed, low-signal-intensity, left mediastinal parathyroid adenoma (arrow) adjacent to the aortic arch. (c) Corresponding technetium 99m (99mTc)–sestamibi (MIBI) scintigram shows increased radiotracer uptake within the adenoma.
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Figure 3a. Parathyroid imaging in MEN 1: scintigraphy. (a) Parathyroid adenoma. Early (top) and delayed (bottom) 99mTc-MIBI scintigrams show a dominant right superior parathyroid adenoma. All four glands were surgically removed, and the three unaffected glands proved to be hyperplastic. (b) Parathyroid adenomas in a different patient. Early (top) and delayed (bottom) 99mTc-MIBI scintigrams show bilateral parathyroid adenomas.
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Figure 3b. Parathyroid imaging in MEN 1: scintigraphy. (a) Parathyroid adenoma. Early (top) and delayed (bottom) 99mTc-MIBI scintigrams show a dominant right superior parathyroid adenoma. All four glands were surgically removed, and the three unaffected glands proved to be hyperplastic. (b) Parathyroid adenomas in a different patient. Early (top) and delayed (bottom) 99mTc-MIBI scintigrams show bilateral parathyroid adenomas.
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Figure 4a. Pancreaticoduodenal imaging in MEN 1: US. (a, b) Gastrinoma in a patient with hypergastrinemia. (a) Endoscopic US image shows a lesion (arrow) within the duodenal wall. (b) Endoscopic US image shows a focal hypoechoic lesion (arrow) in the pancreatic head. At surgery, the lesion proved to be a gastrinoma. (c) Insulinoma. Intraoperative US image obtained in a patient with a biochemically proved insulinoma but negative cross-sectional imaging findings demonstrates an adenoma (arrow) within the pancreatic body. (Case courtesy of Jane Phillips-Hughes, MD, John Radcliffe Hospital, Oxford, England.)
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Figure 4b. Pancreaticoduodenal imaging in MEN 1: US. (a, b) Gastrinoma in a patient with hypergastrinemia. (a) Endoscopic US image shows a lesion (arrow) within the duodenal wall. (b) Endoscopic US image shows a focal hypoechoic lesion (arrow) in the pancreatic head. At surgery, the lesion proved to be a gastrinoma. (c) Insulinoma. Intraoperative US image obtained in a patient with a biochemically proved insulinoma but negative cross-sectional imaging findings demonstrates an adenoma (arrow) within the pancreatic body. (Case courtesy of Jane Phillips-Hughes, MD, John Radcliffe Hospital, Oxford, England.)
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Figure 4c. Pancreaticoduodenal imaging in MEN 1: US. (a, b) Gastrinoma in a patient with hypergastrinemia. (a) Endoscopic US image shows a lesion (arrow) within the duodenal wall. (b) Endoscopic US image shows a focal hypoechoic lesion (arrow) in the pancreatic head. At surgery, the lesion proved to be a gastrinoma. (c) Insulinoma. Intraoperative US image obtained in a patient with a biochemically proved insulinoma but negative cross-sectional imaging findings demonstrates an adenoma (arrow) within the pancreatic body. (Case courtesy of Jane Phillips-Hughes, MD, John Radcliffe Hospital, Oxford, England.)
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Figure 5a. Pancreaticoduodenal imaging in MEN 1: CT. (a) Insulinoma. Contrast-enhanced CT scan shows a partially cystic insulinoma with enhancing walls within the pancreatic head. (b) Adenoma in a different patient. Contrast-enhanced venous phase CT scan shows a small hypoechoic lesion (arrow) within the pancreatic body. Adenomas typically enhance avidly in the arterial phase following contrast material administration and can be missed if imaging is not performed in all three phases.
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Figure 5b. Pancreaticoduodenal imaging in MEN 1: CT. (a) Insulinoma. Contrast-enhanced CT scan shows a partially cystic insulinoma with enhancing walls within the pancreatic head. (b) Adenoma in a different patient. Contrast-enhanced venous phase CT scan shows a small hypoechoic lesion (arrow) within the pancreatic body. Adenomas typically enhance avidly in the arterial phase following contrast material administration and can be missed if imaging is not performed in all three phases.
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Figure 6a. Pancreaticoduodenal imaging in MEN 1: MR imaging. (a–c) Solitary adenoma (insulinoma). (a) Axial fat-suppressed T1-weighted MR image shows a small low-signal-intensity lesion (arrow) within the pancreatic body. (b) On an axial fat-saturated T2-weighted MR image, the lesion (arrow) demonstrates high signal intensity. (c) Gadolinium-enhanced gradient-echo T1-weighted MR image shows the lesion (arrow) with avid enhancement. The lesion proved to be an insulinoma following surgical excision. (d) Multiple adenomas (insulinomas). Axial T2-weighted MR image obtained in a different patient shows multiple small high-signal-intensity lesions throughout the pancreas. At surgery, these lesions proved to be insulinomas.
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Figure 6b. Pancreaticoduodenal imaging in MEN 1: MR imaging. (a–c) Solitary adenoma (insulinoma). (a) Axial fat-suppressed T1-weighted MR image shows a small low-signal-intensity lesion (arrow) within the pancreatic body. (b) On an axial fat-saturated T2-weighted MR image, the lesion (arrow) demonstrates high signal intensity. (c) Gadolinium-enhanced gradient-echo T1-weighted MR image shows the lesion (arrow) with avid enhancement. The lesion proved to be an insulinoma following surgical excision. (d) Multiple adenomas (insulinomas). Axial T2-weighted MR image obtained in a different patient shows multiple small high-signal-intensity lesions throughout the pancreas. At surgery, these lesions proved to be insulinomas.
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Figure 6c. Pancreaticoduodenal imaging in MEN 1: MR imaging. (a–c) Solitary adenoma (insulinoma). (a) Axial fat-suppressed T1-weighted MR image shows a small low-signal-intensity lesion (arrow) within the pancreatic body. (b) On an axial fat-saturated T2-weighted MR image, the lesion (arrow) demonstrates high signal intensity. (c) Gadolinium-enhanced gradient-echo T1-weighted MR image shows the lesion (arrow) with avid enhancement. The lesion proved to be an insulinoma following surgical excision. (d) Multiple adenomas (insulinomas). Axial T2-weighted MR image obtained in a different patient shows multiple small high-signal-intensity lesions throughout the pancreas. At surgery, these lesions proved to be insulinomas.
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Figure 6d. Pancreaticoduodenal imaging in MEN 1: MR imaging. (a–c) Solitary adenoma (insulinoma). (a) Axial fat-suppressed T1-weighted MR image shows a small low-signal-intensity lesion (arrow) within the pancreatic body. (b) On an axial fat-saturated T2-weighted MR image, the lesion (arrow) demonstrates high signal intensity. (c) Gadolinium-enhanced gradient-echo T1-weighted MR image shows the lesion (arrow) with avid enhancement. The lesion proved to be an insulinoma following surgical excision. (d) Multiple adenomas (insulinomas). Axial T2-weighted MR image obtained in a different patient shows multiple small high-signal-intensity lesions throughout the pancreas. At surgery, these lesions proved to be insulinomas.
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Figure 7. Arterial stimulation and venous sampling in MEN 1. The patient had biochemical evidence of an insulinoma, but no tumor was identified at cross-sectional imaging. Digital subtraction angiogram shows two catheters being used for arterial stimulation and venous sampling. A secretagogue (calcium) is being injected through the catheter on the left side, which lies within the splenic artery. The second catheter lies within the right hepatic vein, where venous sampling is being performed to assess for a rise in hormone concentration following injection. (Courtesy of Philip Boardman, MD, John Radcliffe Hospital, Oxford, England.)
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Figure 8a. Pituitary tumor in MEN 1. (a) Coronal T1-weighted MR image shows a left pituitary microadenoma. (b) Sagittal T1-weighted MR image obtained in a different patient shows a pituitary macroadenoma.
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Figure 8b. Pituitary tumor in MEN 1. (a) Coronal T1-weighted MR image shows a left pituitary microadenoma. (b) Sagittal T1-weighted MR image obtained in a different patient shows a pituitary macroadenoma.
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Figure 9a. Adrenal adenomas in MEN 1. (a) Axial T1-weighted MR image shows bilateral adrenal masses with low signal intensity. (b) Axial out-of-phase MR image shows complete loss of signal intensity within the adrenal masses, a finding that is consistent with benign adrenal cortical adenomas.
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Figure 9b. Adrenal adenomas in MEN 1. (a) Axial T1-weighted MR image shows bilateral adrenal masses with low signal intensity. (b) Axial out-of-phase MR image shows complete loss of signal intensity within the adrenal masses, a finding that is consistent with benign adrenal cortical adenomas.
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Figure 10a. Adrenal carcinoma in MEN 1. (a) CT scan shows a heterogeneous left adrenal mass. The lesion had exhibited progressive enlargement. (b) Contrast-enhanced CT scan reveals multiple enhancing liver lesions, which at biopsy were confirmed to represent metastases from adrenal carcinoma. This neoplasm rarely occurs in MEN 1.
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Figure 10b. Adrenal carcinoma in MEN 1. (a) CT scan shows a heterogeneous left adrenal mass. The lesion had exhibited progressive enlargement. (b) Contrast-enhanced CT scan reveals multiple enhancing liver lesions, which at biopsy were confirmed to represent metastases from adrenal carcinoma. This neoplasm rarely occurs in MEN 1.
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Figure 11a. Thymic carcinoid in MEN 1. Contrast-enhanced chest CT scans obtained in a middle-aged man show an anterior mediastinal mass (a) and multiple pulmonary metastases with concurrent left-sided pneumonia (b).
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Figure 11b. Thymic carcinoid in MEN 1. Contrast-enhanced chest CT scans obtained in a middle-aged man show an anterior mediastinal mass (a) and multiple pulmonary metastases with concurrent left-sided pneumonia (b).
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Figure 12a. Typical bronchial carcinoid in MEN 1. (a) Contrast-enhanced chest CT scan demonstrates an endobronchial carcinoid (straight arrow) within the right main bronchus, a finding that was detected incidentally during pulmonary artery CT for suspected pulmonary embolic disease. Curved arrow indicates a thrombus within the left inferior pulmonary artery. (b) Coronal reformatted CT image of the chest obtained in a different patient shows a small benign carcinoid (arrow) within the left upper lobe adjacent to a bronchus. (c) On 111In-octreotide scintigrams obtained in the same patient as in b, the tumor demonstrates increased radiotracer uptake.
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Figure 12b. Typical bronchial carcinoid in MEN 1. (a) Contrast-enhanced chest CT scan demonstrates an endobronchial carcinoid (straight arrow) within the right main bronchus, a finding that was detected incidentally during pulmonary artery CT for suspected pulmonary embolic disease. Curved arrow indicates a thrombus within the left inferior pulmonary artery. (b) Coronal reformatted CT image of the chest obtained in a different patient shows a small benign carcinoid (arrow) within the left upper lobe adjacent to a bronchus. (c) On 111In-octreotide scintigrams obtained in the same patient as in b, the tumor demonstrates increased radiotracer uptake.
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Figure 12c. Typical bronchial carcinoid in MEN 1. (a) Contrast-enhanced chest CT scan demonstrates an endobronchial carcinoid (straight arrow) within the right main bronchus, a finding that was detected incidentally during pulmonary artery CT for suspected pulmonary embolic disease. Curved arrow indicates a thrombus within the left inferior pulmonary artery. (b) Coronal reformatted CT image of the chest obtained in a different patient shows a small benign carcinoid (arrow) within the left upper lobe adjacent to a bronchus. (c) On 111In-octreotide scintigrams obtained in the same patient as in b, the tumor demonstrates increased radiotracer uptake.
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Figure 13a. Atypical bronchial carcinoid in MEN 1. (a) Contrast-enhanced chest CT scan shows partial right lower lobe collapse due to a calcified atypical endobronchial carcinoid. An associated pleural effusion is also seen. (b, c) CT scans show pleural deposits (b) and multiple liver metastases (c). (d) Corresponding iodine 123 (123I)–metaiodobenzylguanidine (MIBG) scintigram shows increased radiotracer uptake within the right hemithorax and liver, a finding that is consistent with metastatic disease.
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Figure 13b. Atypical bronchial carcinoid in MEN 1. (a) Contrast-enhanced chest CT scan shows partial right lower lobe collapse due to a calcified atypical endobronchial carcinoid. An associated pleural effusion is also seen. (b, c) CT scans show pleural deposits (b) and multiple liver metastases (c). (d) Corresponding iodine 123 (123I)–metaiodobenzylguanidine (MIBG) scintigram shows increased radiotracer uptake within the right hemithorax and liver, a finding that is consistent with metastatic disease.
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Figure 13c. Atypical bronchial carcinoid in MEN 1. (a) Contrast-enhanced chest CT scan shows partial right lower lobe collapse due to a calcified atypical endobronchial carcinoid. An associated pleural effusion is also seen. (b, c) CT scans show pleural deposits (b) and multiple liver metastases (c). (d) Corresponding iodine 123 (123I)–metaiodobenzylguanidine (MIBG) scintigram shows increased radiotracer uptake within the right hemithorax and liver, a finding that is consistent with metastatic disease.
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Figure 13d. Atypical bronchial carcinoid in MEN 1. (a) Contrast-enhanced chest CT scan shows partial right lower lobe collapse due to a calcified atypical endobronchial carcinoid. An associated pleural effusion is also seen. (b, c) CT scans show pleural deposits (b) and multiple liver metastases (c). (d) Corresponding iodine 123 (123I)–metaiodobenzylguanidine (MIBG) scintigram shows increased radiotracer uptake within the right hemithorax and liver, a finding that is consistent with metastatic disease.
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Figure 14a. Gastric carcinoids in MEN 1. (a) Axial T2-weighted MR image obtained in a patient with a known gastrinoma and associated Zollinger-Ellison syndrome shows marked gastric wall thickening. (b) Unenhanced CT scan obtained in a different patient shows a solitary nodule arising from the wall of the stomach. This finding is atypical; usually, there are multiple gastric nodules. Biopsy revealed the nodule to be a gastric carcinoid.
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Figure 14b. Gastric carcinoids in MEN 1. (a) Axial T2-weighted MR image obtained in a patient with a known gastrinoma and associated Zollinger-Ellison syndrome shows marked gastric wall thickening. (b) Unenhanced CT scan obtained in a different patient shows a solitary nodule arising from the wall of the stomach. This finding is atypical; usually, there are multiple gastric nodules. Biopsy revealed the nodule to be a gastric carcinoid.
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Figure 15a. MTC in MEN 2. (a, b) Contrast-enhanced CT scans of the neck show a locally invasive thyroid carcinoma with a soft-tissue metastasis (a) and mediastinal lymphadenopathy (b). (c) T2-weighted MR image obtained in a different patient shows multiple liver metastases.
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Figure 15b. MTC in MEN 2. (a, b) Contrast-enhanced CT scans of the neck show a locally invasive thyroid carcinoma with a soft-tissue metastasis (a) and mediastinal lymphadenopathy (b). (c) T2-weighted MR image obtained in a different patient shows multiple liver metastases.
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Figure 15c. MTC in MEN 2. (a, b) Contrast-enhanced CT scans of the neck show a locally invasive thyroid carcinoma with a soft-tissue metastasis (a) and mediastinal lymphadenopathy (b). (c) T2-weighted MR image obtained in a different patient shows multiple liver metastases.
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Figure 16a. Metastatic MTC in MEN 2. (a) On an 123I-MIBG scintigram, increased radiotracer uptake due to multiple hepatic metastases is seen within the liver. The scan was used as a baseline image prior to initiating radionuclide therapy with 131I-MIBG. (b) On an 111In-octreotide scintigram obtained in a different patient, multiple areas of increased radiotracer uptake are depicted within the neck, mediastinum, liver, and upper abdomen. (c) Posttreatment 131I-MIBG scintigrams obtained in the same patient as in b show increased radiotracer uptake within the liver due to residual disease.
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Figure 16b. Metastatic MTC in MEN 2. (a) On an 123I-MIBG scintigram, increased radiotracer uptake due to multiple hepatic metastases is seen within the liver. The scan was used as a baseline image prior to initiating radionuclide therapy with 131I-MIBG. (b) On an 111In-octreotide scintigram obtained in a different patient, multiple areas of increased radiotracer uptake are depicted within the neck, mediastinum, liver, and upper abdomen. (c) Posttreatment 131I-MIBG scintigrams obtained in the same patient as in b show increased radiotracer uptake within the liver due to residual disease.
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Figure 16c. Metastatic MTC in MEN 2. (a) On an 123I-MIBG scintigram, increased radiotracer uptake due to multiple hepatic metastases is seen within the liver. The scan was used as a baseline image prior to initiating radionuclide therapy with 131I-MIBG. (b) On an 111In-octreotide scintigram obtained in a different patient, multiple areas of increased radiotracer uptake are depicted within the neck, mediastinum, liver, and upper abdomen. (c) Posttreatment 131I-MIBG scintigrams obtained in the same patient as in b show increased radiotracer uptake within the liver due to residual disease.
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Figure 17a. Adrenal pheochromocytomas in MEN 2. (a) US image obtained in a patient with MEN 2A demonstrates a heterogeneous right-sided suprarenal mass, a finding that is consistent with a pheochromocytoma. (b) Contrast-enhanced CT scan obtained in a patient with MEN 2B shows bilateral, partially cystic adrenal masses. At surgery, the masses were confirmed to represent pheochromocytomas. Pheochromocytomas in MEN patients are much more likely to be bilateral (50% of cases) compared with sporadic, nonsyndromal pheochromocytoma (bilateral tumors in 10% of cases).
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Figure 17b. Adrenal pheochromocytomas in MEN 2. (a) US image obtained in a patient with MEN 2A demonstrates a heterogeneous right-sided suprarenal mass, a finding that is consistent with a pheochromocytoma. (b) Contrast-enhanced CT scan obtained in a patient with MEN 2B shows bilateral, partially cystic adrenal masses. At surgery, the masses were confirmed to represent pheochromocytomas. Pheochromocytomas in MEN patients are much more likely to be bilateral (50% of cases) compared with sporadic, nonsyndromal pheochromocytoma (bilateral tumors in 10% of cases).
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Figure 18a. Adrenal lesions in MEN 2. (a) Axial T2-weighted MR image shows a right-sided pheochromocytoma with typical hyperintensity. (b) On an axial in-phase T1-weighted MR image, the lesion is predominantly hypointense. (c) On an out-of-phase MR image, the lesion shows no loss of signal intensity. (d) Axial gadolinium-enhanced T1-weighted MR image demonstrates avid enhancement of the pheochromocytoma, especially at the periphery.
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Figure 18b. Adrenal lesions in MEN 2. (a) Axial T2-weighted MR image shows a right-sided pheochromocytoma with typical hyperintensity. (b) On an axial in-phase T1-weighted MR image, the lesion is predominantly hypointense. (c) On an out-of-phase MR image, the lesion shows no loss of signal intensity. (d) Axial gadolinium-enhanced T1-weighted MR image demonstrates avid enhancement of the pheochromocytoma, especially at the periphery.
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Figure 18c. Adrenal lesions in MEN 2. (a) Axial T2-weighted MR image shows a right-sided pheochromocytoma with typical hyperintensity. (b) On an axial in-phase T1-weighted MR image, the lesion is predominantly hypointense. (c) On an out-of-phase MR image, the lesion shows no loss of signal intensity. (d) Axial gadolinium-enhanced T1-weighted MR image demonstrates avid enhancement of the pheochromocytoma, especially at the periphery.
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Figure 18d. Adrenal lesions in MEN 2. (a) Axial T2-weighted MR image shows a right-sided pheochromocytoma with typical hyperintensity. (b) On an axial in-phase T1-weighted MR image, the lesion is predominantly hypointense. (c) On an out-of-phase MR image, the lesion shows no loss of signal intensity. (d) Axial gadolinium-enhanced T1-weighted MR image demonstrates avid enhancement of the pheochromocytoma, especially at the periphery.
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Figure 19. Pheochromocytoma. 123I-MIBG scintigrams show increased radiotracer uptake within the region of the right adrenal gland, a finding that proved to represent a pheochromocytoma.
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Copyright © 2006 by the Radiological Society of North America.
