INTERESTING IMAGE
An Unusual Case of Ectopic ACTH Syndrome Due to Primary Retroperitoneal Carcinoid Rajeev Kasaliwal, MBBS, MD, DM,* Gaurav Malhotra, MBBS, DRM, DNB,† Anurag Lila, MBBS, MD, DM,* Tushar Bandgar, MBBS, MD, DM,* and Nalini S. Shah, MBBS, MD, DM* Abstract: A 37-year-old woman with clinical and biochemical features of ectopic adrenocorticotropic hormone (ACTH) syndrome underwent 68Ga DOTANOC PET/CT to localize the source of ectopic ACTH secretion. The images showed 35 45-mm retroperitoneal mass with significantly increased tracer uptake. Plasmafree metanephrines and serum chromogranin levels were normal. Whole-body 131 I-MIBG scan including the retroperitoneal mass did not show any abnormal uptake. On excision, histopathology of this paravertebral abdominal mass revealed carcinoid tumor. We suggest the use of 68Ga DOTANOC PET/CT as a first-line imaging modality for localization of source of ACTH secretion in ectopic ACTH syndrome. Key Words: Ectopic ACTH syndrome, 68Ga DOTANOC PET/CT, 131 I-MIBG, retroperitoneal carcinoid (Clin Nucl Med 2015;40: 364–367) Received for publication October 17, 2014; revision accepted November 13, 2014. From the *Department of Endocrinology, Seth G S Medical College; and †Radiation Medicine Centre, Bhabha Atomic Research Centre, Tata Memorial Centre, Mumbai, India. Conflicts of interest and sources of funding: none declared. Reprints: Gaurav Malhotra, MBBS, DRM, DNB, Radiation Medicine Centre, Bhabha Atomic Research Centre, Tata Memorial Centre, Annexe, Jerbai Wadia Rd, Parel, Mumbai-400012, India. E-mail: [email protected]. Copyright © 2015 Wolters Kluwer Health, Inc. All rights reserved. ISSN: 0363-9762/15/4004–0364
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REFERENCES 1. Venkitaraman B, Karunanithi S, Kumar A, et al. Ga-68 DOTATOC PET-CT in the localization of source of ectopic ACTH-dependent Cushing's syndrome. Clin Imaging. 2014;38:208–211. 2. Darr R, Zophel K, Eisenhofer G, et al. Combined use of Ga-68 DOTATATE and F18 FDG PET/CT to localize a bronchial carcinoid associated with ectopic ACTH syndrome. J Clin Endocrinol Metab. 2012;97:2207–2208. 3. Ozkan ZG, Kuyumcu S, Balkose D, et al. The value of somatostatin receptor imaging with In-111 octreotide and/or Ga-68 DOTATATE in localizing ectopic ACTH producing tumors. Mol Imaging Radionucl Ther. 2013;22:49–55. 4. Povedano ST, Pastor CV, Seoane CP, et al. Ectopic ACTH syndrome caused by pulmonary carcinoid tumourlets. Clin Endocrinol (Oxf). 2001;54:839–842. 5. Beddy D, Larson D. Cushing's syndrome cured by resection of an appendiceal carcinoid tumor. Int J Colorectal Dis. 2011;26:949–950. 6. Shah NA, Urusova IA, D'Agnolo A, et al. Primary hepatic carcinoid tumor presenting as Cushing's syndrome. J Endocrinol Invest. 2007;30:327–333. 7. Uecker JM, Janzow MT. A case of Cushing syndrome secondary to ectopic adrenocorticotropic hormone producing carcinoid of the duodenum. Am Surg. 2005;71: 445–446. 8. Schlaghecke R, Kreuzpaintner G, Burrig KF, et al. Cushing's syndrome due to ACTH production of an ovarian carcinoid. Klin Wochenschr. 1989;67:640–644. 9. Chiba S, Numakura K, Satoyoshi K, et al. Primary retroperitoneal carcinoid tumor associated with multiple endocrine neoplasia (MEN) type1: a case report. Nihon Hinyokika Gakkai Zasshi. 2011;102:735–739.
Clinical Nuclear Medicine • Volume 40, Number 4, April 2015 Copyright © 2015 Wolters Kluwer Health, Inc. All rights reserved.
Clinical Nuclear Medicine • Volume 40, Number 4, April 2015
An Unusual Case of Ectopic ACTH Syndrome
FIGURE 1. A 37-year-old woman presented with explosive clinical manifestations of Cushing syndrome (CS) including severe proximal myopathy, resistant hypertension, poorly controlled diabetes mellitus, and severe hypokalemia of relatively short duration (
An Unusual Case of Ectopic ACTH Syndrome Due to Primary Retroperitoneal Carcinoid Rajeev Kasaliwal, MBBS, MD, DM,* Gaurav Malhotra, MBBS, DRM, DNB,† Anurag Lila, MBBS, MD, DM,* Tushar Bandgar, MBBS, MD, DM,* and Nalini S. Shah, MBBS, MD, DM* Abstract: A 37-year-old woman with clinical and biochemical features of ectopic adrenocorticotropic hormone (ACTH) syndrome underwent 68Ga DOTANOC PET/CT to localize the source of ectopic ACTH secretion. The images showed 35 45-mm retroperitoneal mass with significantly increased tracer uptake. Plasmafree metanephrines and serum chromogranin levels were normal. Whole-body 131 I-MIBG scan including the retroperitoneal mass did not show any abnormal uptake. On excision, histopathology of this paravertebral abdominal mass revealed carcinoid tumor. We suggest the use of 68Ga DOTANOC PET/CT as a first-line imaging modality for localization of source of ACTH secretion in ectopic ACTH syndrome. Key Words: Ectopic ACTH syndrome, 68Ga DOTANOC PET/CT, 131 I-MIBG, retroperitoneal carcinoid (Clin Nucl Med 2015;40: 364–367) Received for publication October 17, 2014; revision accepted November 13, 2014. From the *Department of Endocrinology, Seth G S Medical College; and †Radiation Medicine Centre, Bhabha Atomic Research Centre, Tata Memorial Centre, Mumbai, India. Conflicts of interest and sources of funding: none declared. Reprints: Gaurav Malhotra, MBBS, DRM, DNB, Radiation Medicine Centre, Bhabha Atomic Research Centre, Tata Memorial Centre, Annexe, Jerbai Wadia Rd, Parel, Mumbai-400012, India. E-mail: [email protected]. Copyright © 2015 Wolters Kluwer Health, Inc. All rights reserved. ISSN: 0363-9762/15/4004–0364
364
www.nuclearmed.com
REFERENCES 1. Venkitaraman B, Karunanithi S, Kumar A, et al. Ga-68 DOTATOC PET-CT in the localization of source of ectopic ACTH-dependent Cushing's syndrome. Clin Imaging. 2014;38:208–211. 2. Darr R, Zophel K, Eisenhofer G, et al. Combined use of Ga-68 DOTATATE and F18 FDG PET/CT to localize a bronchial carcinoid associated with ectopic ACTH syndrome. J Clin Endocrinol Metab. 2012;97:2207–2208. 3. Ozkan ZG, Kuyumcu S, Balkose D, et al. The value of somatostatin receptor imaging with In-111 octreotide and/or Ga-68 DOTATATE in localizing ectopic ACTH producing tumors. Mol Imaging Radionucl Ther. 2013;22:49–55. 4. Povedano ST, Pastor CV, Seoane CP, et al. Ectopic ACTH syndrome caused by pulmonary carcinoid tumourlets. Clin Endocrinol (Oxf). 2001;54:839–842. 5. Beddy D, Larson D. Cushing's syndrome cured by resection of an appendiceal carcinoid tumor. Int J Colorectal Dis. 2011;26:949–950. 6. Shah NA, Urusova IA, D'Agnolo A, et al. Primary hepatic carcinoid tumor presenting as Cushing's syndrome. J Endocrinol Invest. 2007;30:327–333. 7. Uecker JM, Janzow MT. A case of Cushing syndrome secondary to ectopic adrenocorticotropic hormone producing carcinoid of the duodenum. Am Surg. 2005;71: 445–446. 8. Schlaghecke R, Kreuzpaintner G, Burrig KF, et al. Cushing's syndrome due to ACTH production of an ovarian carcinoid. Klin Wochenschr. 1989;67:640–644. 9. Chiba S, Numakura K, Satoyoshi K, et al. Primary retroperitoneal carcinoid tumor associated with multiple endocrine neoplasia (MEN) type1: a case report. Nihon Hinyokika Gakkai Zasshi. 2011;102:735–739.
Clinical Nuclear Medicine • Volume 40, Number 4, April 2015 Copyright © 2015 Wolters Kluwer Health, Inc. All rights reserved.
Clinical Nuclear Medicine • Volume 40, Number 4, April 2015
An Unusual Case of Ectopic ACTH Syndrome
FIGURE 1. A 37-year-old woman presented with explosive clinical manifestations of Cushing syndrome (CS) including severe proximal myopathy, resistant hypertension, poorly controlled diabetes mellitus, and severe hypokalemia of relatively short duration (
