High Molecular Weight ACTH-Precursor Presence in a Metastatic Pancreatic Neuroendocrine Tumor Causing Severe Ectopic Cushing's Syndrome: A Case Report
Ectopic ACTH-secretion causing Cushing's syndrome is unusual and its diagnosis is frequently challenging. The presence of high-molecular-weight precursors throughout pro-opiomelanocortin (POMC) translation by these tumors is often not reported. We present the case of a 49-year-old woman with a...
| Autores principales: | , , , , , , , , , , , , |
|---|---|
| Formato: | Artículo |
| Lenguaje: | inglés |
| Publicado: |
Frontiers Research Foundation
2020
|
| Materias: | |
| Acceso en línea: | http://eprints.uanl.mx/23336/1/23336.pdf |
| _version_ | 1824416846044987392 |
|---|---|
| author | Mehta, Roopa Lam Chung, César Ernesto Hinojosa Amaya, José Miguel Roldán Sarmiento, Paola Guillen Placencia, María Fernanda Villanueva Rodríguez, Gerladine Juárez León, Oscar Alfredo León Domínguez, Jefsi Grajales Gómez, Mariana Ventura Gallegos, José Luis León Suárez, Andrés Gómez Pérez, Francisco J. Cuevas Ramos, Daniel |
| author_facet | Mehta, Roopa Lam Chung, César Ernesto Hinojosa Amaya, José Miguel Roldán Sarmiento, Paola Guillen Placencia, María Fernanda Villanueva Rodríguez, Gerladine Juárez León, Oscar Alfredo León Domínguez, Jefsi Grajales Gómez, Mariana Ventura Gallegos, José Luis León Suárez, Andrés Gómez Pérez, Francisco J. Cuevas Ramos, Daniel |
| author_sort | Mehta, Roopa |
| collection | Repositorio Institucional |
| description | Ectopic ACTH-secretion causing Cushing's syndrome is unusual and its diagnosis is frequently challenging. The presence of high-molecular-weight precursors throughout pro-opiomelanocortin (POMC) translation by these tumors is often not reported. We present the case of a 49-year-old woman with a 3-month history of proximal muscular weakness, skin pigmentation, and weight loss. Upon initial evaluation, she had a full moon face, hirsutism, and a buffalo hump. Laboratory workup showed hyperglycemia, hypokalemia and metabolic alkalosis. ACTH, plasma cortisol, and urinary free cortisol levels were quite elevated. Serum cortisol levels were not suppressed on dexamethasone suppression testing. An octreo-SPECT scan showed enhanced nucleotide uptake in the liver and pancreas. Transendoscopic ultrasound-guided biopsy confirmed the diagnosis of a pancreatic ACTH-secreting neuroendocrine tumor (NET). Surgical excision of both pancreatic and liver lesions was carried out. Western blot analysis of the tumor and metastases revealed the presence of a high-molecular-weight precursor possibly POMC (at 30 kDa) but not ACTH (normally 4.5 kDa). ACTH-precursor secretion is more frequent in ectopic ACTH-secreting tumors compared with other causes of Cushing's syndrome. Hence, the measurement of such ACTH precursors warrants further evaluation, especially in the context of ACTH-dependent hypercortisolism. |
| format | Article |
| id | eprints-23336 |
| institution | UANL |
| language | English |
| publishDate | 2020 |
| publisher | Frontiers Research Foundation |
| record_format | eprints |
| spelling | eprints-233362022-06-02T20:28:46Z http://eprints.uanl.mx/23336/ High Molecular Weight ACTH-Precursor Presence in a Metastatic Pancreatic Neuroendocrine Tumor Causing Severe Ectopic Cushing's Syndrome: A Case Report Mehta, Roopa Lam Chung, César Ernesto Hinojosa Amaya, José Miguel Roldán Sarmiento, Paola Guillen Placencia, María Fernanda Villanueva Rodríguez, Gerladine Juárez León, Oscar Alfredo León Domínguez, Jefsi Grajales Gómez, Mariana Ventura Gallegos, José Luis León Suárez, Andrés Gómez Pérez, Francisco J. Cuevas Ramos, Daniel RC Medicina Interna, Psiquiatría, Neurología Ectopic ACTH-secretion causing Cushing's syndrome is unusual and its diagnosis is frequently challenging. The presence of high-molecular-weight precursors throughout pro-opiomelanocortin (POMC) translation by these tumors is often not reported. We present the case of a 49-year-old woman with a 3-month history of proximal muscular weakness, skin pigmentation, and weight loss. Upon initial evaluation, she had a full moon face, hirsutism, and a buffalo hump. Laboratory workup showed hyperglycemia, hypokalemia and metabolic alkalosis. ACTH, plasma cortisol, and urinary free cortisol levels were quite elevated. Serum cortisol levels were not suppressed on dexamethasone suppression testing. An octreo-SPECT scan showed enhanced nucleotide uptake in the liver and pancreas. Transendoscopic ultrasound-guided biopsy confirmed the diagnosis of a pancreatic ACTH-secreting neuroendocrine tumor (NET). Surgical excision of both pancreatic and liver lesions was carried out. Western blot analysis of the tumor and metastases revealed the presence of a high-molecular-weight precursor possibly POMC (at 30 kDa) but not ACTH (normally 4.5 kDa). ACTH-precursor secretion is more frequent in ectopic ACTH-secreting tumors compared with other causes of Cushing's syndrome. Hence, the measurement of such ACTH precursors warrants further evaluation, especially in the context of ACTH-dependent hypercortisolism. Frontiers Research Foundation 2020 Article PeerReviewed text en cc_by_nc_nd http://eprints.uanl.mx/23336/1/23336.pdf http://eprints.uanl.mx/23336/1.haspreviewThumbnailVersion/23336.pdf Mehta, Roopa y Lam Chung, César Ernesto y Hinojosa Amaya, José Miguel y Roldán Sarmiento, Paola y Guillen Placencia, María Fernanda y Villanueva Rodríguez, Gerladine y Juárez León, Oscar Alfredo y León Domínguez, Jefsi y Grajales Gómez, Mariana y Ventura Gallegos, José Luis y León Suárez, Andrés y Gómez Pérez, Francisco J. y Cuevas Ramos, Daniel (2020) High Molecular Weight ACTH-Precursor Presence in a Metastatic Pancreatic Neuroendocrine Tumor Causing Severe Ectopic Cushing's Syndrome: A Case Report. Frontiers in Endocrinology, 11. pp. 1-8. ISSN 1664-2392 http://doi.org/10.3389/fendo.2020.00557 doi:10.3389/fendo.2020.00557 |
| spellingShingle | RC Medicina Interna, Psiquiatría, Neurología Mehta, Roopa Lam Chung, César Ernesto Hinojosa Amaya, José Miguel Roldán Sarmiento, Paola Guillen Placencia, María Fernanda Villanueva Rodríguez, Gerladine Juárez León, Oscar Alfredo León Domínguez, Jefsi Grajales Gómez, Mariana Ventura Gallegos, José Luis León Suárez, Andrés Gómez Pérez, Francisco J. Cuevas Ramos, Daniel High Molecular Weight ACTH-Precursor Presence in a Metastatic Pancreatic Neuroendocrine Tumor Causing Severe Ectopic Cushing's Syndrome: A Case Report |
| thumbnail | https://rediab.uanl.mx/themes/sandal5/images/online.png |
| title | High Molecular Weight ACTH-Precursor Presence in a Metastatic Pancreatic Neuroendocrine Tumor Causing Severe Ectopic Cushing's Syndrome: A Case Report |
| title_full | High Molecular Weight ACTH-Precursor Presence in a Metastatic Pancreatic Neuroendocrine Tumor Causing Severe Ectopic Cushing's Syndrome: A Case Report |
| title_fullStr | High Molecular Weight ACTH-Precursor Presence in a Metastatic Pancreatic Neuroendocrine Tumor Causing Severe Ectopic Cushing's Syndrome: A Case Report |
| title_full_unstemmed | High Molecular Weight ACTH-Precursor Presence in a Metastatic Pancreatic Neuroendocrine Tumor Causing Severe Ectopic Cushing's Syndrome: A Case Report |
| title_short | High Molecular Weight ACTH-Precursor Presence in a Metastatic Pancreatic Neuroendocrine Tumor Causing Severe Ectopic Cushing's Syndrome: A Case Report |
| title_sort | high molecular weight acth precursor presence in a metastatic pancreatic neuroendocrine tumor causing severe ectopic cushing s syndrome a case report |
| topic | RC Medicina Interna, Psiquiatría, Neurología |
| url | http://eprints.uanl.mx/23336/1/23336.pdf |
| work_keys_str_mv | AT mehtaroopa highmolecularweightacthprecursorpresenceinametastaticpancreaticneuroendocrinetumorcausingsevereectopiccushingssyndromeacasereport AT lamchungcesarernesto highmolecularweightacthprecursorpresenceinametastaticpancreaticneuroendocrinetumorcausingsevereectopiccushingssyndromeacasereport AT hinojosaamayajosemiguel highmolecularweightacthprecursorpresenceinametastaticpancreaticneuroendocrinetumorcausingsevereectopiccushingssyndromeacasereport AT roldansarmientopaola highmolecularweightacthprecursorpresenceinametastaticpancreaticneuroendocrinetumorcausingsevereectopiccushingssyndromeacasereport AT guillenplacenciamariafernanda highmolecularweightacthprecursorpresenceinametastaticpancreaticneuroendocrinetumorcausingsevereectopiccushingssyndromeacasereport AT villanuevarodriguezgerladine highmolecularweightacthprecursorpresenceinametastaticpancreaticneuroendocrinetumorcausingsevereectopiccushingssyndromeacasereport AT juarezleonoscaralfredo highmolecularweightacthprecursorpresenceinametastaticpancreaticneuroendocrinetumorcausingsevereectopiccushingssyndromeacasereport AT leondominguezjefsi highmolecularweightacthprecursorpresenceinametastaticpancreaticneuroendocrinetumorcausingsevereectopiccushingssyndromeacasereport AT grajalesgomezmariana highmolecularweightacthprecursorpresenceinametastaticpancreaticneuroendocrinetumorcausingsevereectopiccushingssyndromeacasereport AT venturagallegosjoseluis highmolecularweightacthprecursorpresenceinametastaticpancreaticneuroendocrinetumorcausingsevereectopiccushingssyndromeacasereport AT leonsuarezandres highmolecularweightacthprecursorpresenceinametastaticpancreaticneuroendocrinetumorcausingsevereectopiccushingssyndromeacasereport AT gomezperezfranciscoj highmolecularweightacthprecursorpresenceinametastaticpancreaticneuroendocrinetumorcausingsevereectopiccushingssyndromeacasereport AT cuevasramosdaniel highmolecularweightacthprecursorpresenceinametastaticpancreaticneuroendocrinetumorcausingsevereectopiccushingssyndromeacasereport |