RADIOFRECENCY ABLATION OF LIVER METASTASES IN ECTOPIC ACTH PRODUCING NEUROENDOCRINE TUMOR RESULTED IN CLINICAL AND BIOCHEMICAL RESOLUTION OF CUSHING’S SYNDROME
AACE ePoster Library. Oertel M. 05/13/15; 97753; 104
Mark Oertel
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RADIOFRECENCY ABLATION OF LIVER METASTASES IN ECTOPIC ACTH PRODUCING NEUROENDOCRINE TUMOR RESULTED IN CLINICAL AND BIOCHEMICAL RESOLUTION OF CUSHING’S SYNDROME
Mark Oertel, MD, Leland Graves, III, MD University of Kansas Medical Center
Objective: Ectopic ACTH secretion (EAS) accounts for 20% of ACTH dependent Cushing’s syndrome. Lung carcinoma and neuroendocrine tumors are the leading causes. Surgical resection of the primary tumor is optimal therapy; however in cases of metastatic tumors a surgical approach may not be feasible. For nonresectable tumors therapeutic options to control the hypercortisolism include adrenal enzyme inhibitors, bilateral adrenalectomy or treatment of metastasis by ablation. We present a case of severe Cushing’s syndrome caused by bronchial carcinoid tumor with liver metastasis with resolution following radiofrequency ablation (RFA).
Case Presentation: A 57 year old male presented with 6 months of uncontrolled hypertension, 50 pound weight gain, new onset diabetes, and edema. Initial laboratory revealed a serum cortisol of 91.5 mcg/dL, ACTH 198 pg/mL, and 24 hour urinary free cortisol of 7820 mcg. Pituitary MRI showed no abnormalities and inferior petrosal sinus sampling confirmed a non-pituitary source of ACTH. Octreotide scan revealed no abnormal uptake. CT scan revealed a left lower lobe peribronchial nodule, 2 right lobe liver masses (3.5 cm and 1.5 cm) all which were metabolically active on PET. Liver biopsy showed moderately differentiated neuroendocrine carcinoma. When ketoconazole 1200 mg/day resulted in persistently high cortisol and no clinical improvement, alternative treatments were considered. Surgery was considered high risk and unlikely curative, therefore RFA of the hepatic metastasis was undertaken. 2 weeks after RFA his serum AM cortisol decreased from 38.8 mcg/dL to 5.0 and ACTH from 232 pg/mL to 59. 3 months after RFA 24 hour urinary free cortisol decreased to 10 mcg. His signs and symptoms of Cushing's syndrome resolved.
Discussion: Curative surgical treatment for EAS has been reported between 12- 46%. Adrenal enzyme inhibitors including ketoconazole and adrenolytic agents are options for cortisol control in preparation for surgery or in patients where surgery is not feasible or curative. Medical therapy response rate has been reported between 53-88%. EAS with high ACTH and cortisol levels may be less likely to respond or normalize with medical therapy. Treatment of the primary tumor or metastasis with RFA or other ablative procedures has shown response rates of 50-96% over 4-18 months.
Conclusion: Treatment of EAS from a non resectable primary tumor or with metastatic disease is challenging. Medical therapy to reduce cortisol may not normalize the cortisol or resolve symptoms. RFA of the metastasis, even if the primary tumor is not treated, may result in control of the serum cortisol and resolution of Cushing’s syndrome. This may be a reasonable alternative to bilateral adrenalectomy.
Mark Oertel, MD, Leland Graves, III, MD University of Kansas Medical Center
Objective: Ectopic ACTH secretion (EAS) accounts for 20% of ACTH dependent Cushing’s syndrome. Lung carcinoma and neuroendocrine tumors are the leading causes. Surgical resection of the primary tumor is optimal therapy; however in cases of metastatic tumors a surgical approach may not be feasible. For nonresectable tumors therapeutic options to control the hypercortisolism include adrenal enzyme inhibitors, bilateral adrenalectomy or treatment of metastasis by ablation. We present a case of severe Cushing’s syndrome caused by bronchial carcinoid tumor with liver metastasis with resolution following radiofrequency ablation (RFA).
Case Presentation: A 57 year old male presented with 6 months of uncontrolled hypertension, 50 pound weight gain, new onset diabetes, and edema. Initial laboratory revealed a serum cortisol of 91.5 mcg/dL, ACTH 198 pg/mL, and 24 hour urinary free cortisol of 7820 mcg. Pituitary MRI showed no abnormalities and inferior petrosal sinus sampling confirmed a non-pituitary source of ACTH. Octreotide scan revealed no abnormal uptake. CT scan revealed a left lower lobe peribronchial nodule, 2 right lobe liver masses (3.5 cm and 1.5 cm) all which were metabolically active on PET. Liver biopsy showed moderately differentiated neuroendocrine carcinoma. When ketoconazole 1200 mg/day resulted in persistently high cortisol and no clinical improvement, alternative treatments were considered. Surgery was considered high risk and unlikely curative, therefore RFA of the hepatic metastasis was undertaken. 2 weeks after RFA his serum AM cortisol decreased from 38.8 mcg/dL to 5.0 and ACTH from 232 pg/mL to 59. 3 months after RFA 24 hour urinary free cortisol decreased to 10 mcg. His signs and symptoms of Cushing's syndrome resolved.
Discussion: Curative surgical treatment for EAS has been reported between 12- 46%. Adrenal enzyme inhibitors including ketoconazole and adrenolytic agents are options for cortisol control in preparation for surgery or in patients where surgery is not feasible or curative. Medical therapy response rate has been reported between 53-88%. EAS with high ACTH and cortisol levels may be less likely to respond or normalize with medical therapy. Treatment of the primary tumor or metastasis with RFA or other ablative procedures has shown response rates of 50-96% over 4-18 months.
Conclusion: Treatment of EAS from a non resectable primary tumor or with metastatic disease is challenging. Medical therapy to reduce cortisol may not normalize the cortisol or resolve symptoms. RFA of the metastasis, even if the primary tumor is not treated, may result in control of the serum cortisol and resolution of Cushing’s syndrome. This may be a reasonable alternative to bilateral adrenalectomy.
RADIOFRECENCY ABLATION OF LIVER METASTASES IN ECTOPIC ACTH PRODUCING NEUROENDOCRINE TUMOR RESULTED IN CLINICAL AND BIOCHEMICAL RESOLUTION OF CUSHING’S SYNDROME
Mark Oertel, MD, Leland Graves, III, MD University of Kansas Medical Center
Objective: Ectopic ACTH secretion (EAS) accounts for 20% of ACTH dependent Cushing’s syndrome. Lung carcinoma and neuroendocrine tumors are the leading causes. Surgical resection of the primary tumor is optimal therapy; however in cases of metastatic tumors a surgical approach may not be feasible. For nonresectable tumors therapeutic options to control the hypercortisolism include adrenal enzyme inhibitors, bilateral adrenalectomy or treatment of metastasis by ablation. We present a case of severe Cushing’s syndrome caused by bronchial carcinoid tumor with liver metastasis with resolution following radiofrequency ablation (RFA).
Case Presentation: A 57 year old male presented with 6 months of uncontrolled hypertension, 50 pound weight gain, new onset diabetes, and edema. Initial laboratory revealed a serum cortisol of 91.5 mcg/dL, ACTH 198 pg/mL, and 24 hour urinary free cortisol of 7820 mcg. Pituitary MRI showed no abnormalities and inferior petrosal sinus sampling confirmed a non-pituitary source of ACTH. Octreotide scan revealed no abnormal uptake. CT scan revealed a left lower lobe peribronchial nodule, 2 right lobe liver masses (3.5 cm and 1.5 cm) all which were metabolically active on PET. Liver biopsy showed moderately differentiated neuroendocrine carcinoma. When ketoconazole 1200 mg/day resulted in persistently high cortisol and no clinical improvement, alternative treatments were considered. Surgery was considered high risk and unlikely curative, therefore RFA of the hepatic metastasis was undertaken. 2 weeks after RFA his serum AM cortisol decreased from 38.8 mcg/dL to 5.0 and ACTH from 232 pg/mL to 59. 3 months after RFA 24 hour urinary free cortisol decreased to 10 mcg. His signs and symptoms of Cushing's syndrome resolved.
Discussion: Curative surgical treatment for EAS has been reported between 12- 46%. Adrenal enzyme inhibitors including ketoconazole and adrenolytic agents are options for cortisol control in preparation for surgery or in patients where surgery is not feasible or curative. Medical therapy response rate has been reported between 53-88%. EAS with high ACTH and cortisol levels may be less likely to respond or normalize with medical therapy. Treatment of the primary tumor or metastasis with RFA or other ablative procedures has shown response rates of 50-96% over 4-18 months.
Conclusion: Treatment of EAS from a non resectable primary tumor or with metastatic disease is challenging. Medical therapy to reduce cortisol may not normalize the cortisol or resolve symptoms. RFA of the metastasis, even if the primary tumor is not treated, may result in control of the serum cortisol and resolution of Cushing’s syndrome. This may be a reasonable alternative to bilateral adrenalectomy.
Mark Oertel, MD, Leland Graves, III, MD University of Kansas Medical Center
Objective: Ectopic ACTH secretion (EAS) accounts for 20% of ACTH dependent Cushing’s syndrome. Lung carcinoma and neuroendocrine tumors are the leading causes. Surgical resection of the primary tumor is optimal therapy; however in cases of metastatic tumors a surgical approach may not be feasible. For nonresectable tumors therapeutic options to control the hypercortisolism include adrenal enzyme inhibitors, bilateral adrenalectomy or treatment of metastasis by ablation. We present a case of severe Cushing’s syndrome caused by bronchial carcinoid tumor with liver metastasis with resolution following radiofrequency ablation (RFA).
Case Presentation: A 57 year old male presented with 6 months of uncontrolled hypertension, 50 pound weight gain, new onset diabetes, and edema. Initial laboratory revealed a serum cortisol of 91.5 mcg/dL, ACTH 198 pg/mL, and 24 hour urinary free cortisol of 7820 mcg. Pituitary MRI showed no abnormalities and inferior petrosal sinus sampling confirmed a non-pituitary source of ACTH. Octreotide scan revealed no abnormal uptake. CT scan revealed a left lower lobe peribronchial nodule, 2 right lobe liver masses (3.5 cm and 1.5 cm) all which were metabolically active on PET. Liver biopsy showed moderately differentiated neuroendocrine carcinoma. When ketoconazole 1200 mg/day resulted in persistently high cortisol and no clinical improvement, alternative treatments were considered. Surgery was considered high risk and unlikely curative, therefore RFA of the hepatic metastasis was undertaken. 2 weeks after RFA his serum AM cortisol decreased from 38.8 mcg/dL to 5.0 and ACTH from 232 pg/mL to 59. 3 months after RFA 24 hour urinary free cortisol decreased to 10 mcg. His signs and symptoms of Cushing's syndrome resolved.
Discussion: Curative surgical treatment for EAS has been reported between 12- 46%. Adrenal enzyme inhibitors including ketoconazole and adrenolytic agents are options for cortisol control in preparation for surgery or in patients where surgery is not feasible or curative. Medical therapy response rate has been reported between 53-88%. EAS with high ACTH and cortisol levels may be less likely to respond or normalize with medical therapy. Treatment of the primary tumor or metastasis with RFA or other ablative procedures has shown response rates of 50-96% over 4-18 months.
Conclusion: Treatment of EAS from a non resectable primary tumor or with metastatic disease is challenging. Medical therapy to reduce cortisol may not normalize the cortisol or resolve symptoms. RFA of the metastasis, even if the primary tumor is not treated, may result in control of the serum cortisol and resolution of Cushing’s syndrome. This may be a reasonable alternative to bilateral adrenalectomy.
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