title: ACTH-secreting pancreatic neuroendocrine carcinoma with ovarian and pelvic metastases causing Cushing's syndrome: a case report.
abstract: Adrenocorticotropin hormone (ACTH)-secreting pancreatic neuroendocrine carcinoma (NEC) with ovarian and pelvic metastases causing Cushing's syndrome is very rare and might be misdiagnosed. We describe a case of ACTH-secreting pancreatic poorly differentiated NEC developing bilateral ovarian and pelvic metastases. A 27-year-old woman presented with thirst, polydipsia, fatigue and poorly controlled hyperglycemia. Laboratory and imaging investigations revealed hypokalemia, hyperglycaemia, ACTH-dependent hypercortisolemia and a 12-cm mass at the junction of body and tail of the pancreas with ovarian and pelvic nodules. The patient underwent partial pancreatectomy and splenectomy, uterectomy, bilateral oophorectomy, and excision of peritoneal nodules. Tumors in pancreas, ovaries and pelvis were diagnosed as poor-differentiated NEC. After 19-month chemotherapy, she developed pelvic metastasis. The tumor in our case is a large, poorly differentiated NEC secreting ACTH and causing CS, with ovarian metastases. To our knowledge, this new additional case of ACTH-secreting pancreatic NEC with ovarian metastases would add to the better understanding of this tumor.
authors.affiliation: Department of Pathology, West China Hospital, Sichuan University Chengdu 610041, China.
authors.forename: Wen-Qing
authors.initials: WQ
authors.lastname: Yao
authors.affiliation: Department of Pathology, West China Hospital, Sichuan University Chengdu 610041, China.
authors.forename: Xia
authors.initials: X
authors.lastname: Wu
authors.affiliation: Department of Pathology, West China Hospital, Sichuan University Chengdu 610041, China.
authors.forename: Gan-Di
authors.initials: GD
authors.lastname: Li
authors.affiliation: Department of Pathology, West China Hospital, Sichuan University Chengdu 610041, China.
authors.forename: Wei-Lu
authors.initials: WL
authors.lastname: Wu
authors.affiliation: Department of Pathology, West China Hospital, Sichuan University Chengdu 610041, China.
authors.forename: Wei-Ya
authors.initials: WY
authors.lastname: Wang
mesh_terms: D000324:Adrenocorticotropic Hormone / Q000378:metabolism*; D000328:Adult; D001706:Biopsy; D018278:Carcinoma, Neuroendocrine / Q000737:chemistry / Q000150:complications* / Q000473:pathology / Q000556:secondary / Q000601:surgery; D002454:Cell Differentiation; D017024:Chemotherapy, Adjuvant; D003480:Cushing Syndrome / Q000175:diagnosis / Q000209:etiology*; D005260:Female; D006801:Humans; D007150:Immunohistochemistry; D010051:Ovarian Neoplasms / Q000737:chemistry / Q000378:metabolism / Q000556:secondary* / Q000601:surgery; D010052:Ovariectomy; D010180:Pancreatectomy; D010190:Pancreatic Neoplasms / Q000737:chemistry / Q000150:complications* / Q000378:metabolism / Q000473:pathology / Q000601:surgery; D010386:Pelvic Neoplasms / Q000737:chemistry / Q000378:metabolism / Q000556:secondary* / Q000601:surgery; D013156:Splenectomy; D014057:Tomography, X-Ray Computed; D016896:Treatment Outcome; D047368:Tumor Burden
chemical_list: D000324:Adrenocorticotropic Hormone
keywords: ACTH; ovarian metastasis; pancreatic neuroendocrine neoplasms
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