Metastatic Pancreatic Neuroendocrine Tumor that Progressed to Ectopic Adrenocorticotropic Hormone (ACTH) Syndrome with Growth Hormone-releasing Hormone (GHRH) Production

Tadokoro, Rie; Sato, Shotaro; Otsuka, Fumiko; Ueno, Makoto; Ohkawa, Shinichi; Katakami, Hideki; Taniyama, Matsuo; Nagasaka, Shoichiro

doi:10.2169/internalmedicine.55.6827

Cited by 8 publications

(5 citation statements)

References 11 publications

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“…While the most frequent functional PNETs are insulinoma and gastrinoma, ectopic adrenocorticotropic hormone-producing tumours are very rare types of PNET [ 5 , 6 ] and carry a poor prognosis. The survival analysis of all the cases reported in the English/Spanish literature found that only 35% of patients were alive at 5 years after diagnosis [ 7 ].…”

Section: Discussionmentioning

confidence: 99%

Functional pancreatic neuroendocrine tumour causing Cushing’s syndrome: the effect of chemotherapy on clinical symptoms

Divino

Marchetti

Almeida

et al. 2017

ecancer

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BackgroundNeuroendocrine tumours (NETs) are a heterogeneous group of diseases that can originate from any part of the gastrointestinal tract, bronchi, thyroid and pancreas. These tumours may be functioning or not depending on their ability to produce active substances, such as adrenocorticotrophic hormone (ACTH). ACTH-producing pancreatic neuroendocrine tumours are rare, with limited data about effective antitumor therapies.Case ReportA 58-year-old man with a history of type-2 diabetes mellitus and arterial hypertension was diagnosed with Cushing’s syndrome (CS) secondary to an ACTH ectopic production from a well-differentiated neuroendocrine tumour of the pancreas metastatic to the liver. The patient underwent initial body-caudal pancreatectomy, splenectomy and hepatic nodulectomy with subsequent recurrence. Hepatic embolisation and somatostatin analogues were used to control CS but without success. Bilateral adrenalectomy led to CS control, while capecitabine and oxaliplatin (CAPOX) was effective in controlling tumour growth and ACTH production.DiscussionACTH-producing pancreatic neuroendocrine tumours are rare, aggressive and difficult to treat with available therapies. In settings of limited resources, such as in developing countries where targeted therapies are not available, cytotoxic chemotherapy with CAPOX represents a good and inexpensive option to control ACTH-producing pancreatic neuroendocrine tumours. Because of its complexity, the management of this tumour should be performed by multidisciplinary teams.

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Section: Discussionmentioning

confidence: 99%

Functional pancreatic neuroendocrine tumour causing Cushing’s syndrome: the effect of chemotherapy on clinical symptoms

Divino

Marchetti

Almeida

et al. 2017

ecancer

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“…Albeit rare, GHRH secretion in other tumors, including pheochromocytoma, lymphoma, paraganglioma, or thymoma has also been reported ( 4 , 11 , 15 , 34 – 37 ). In some cases, tumor resection preceded possible acromegaly development ( 37 – 39 ). According to gross pathology data available for 72 patients, extracranial tumors measured 6.6 cm on average, ranging from 1 to 25 cm.…”

Section: Methodsmentioning

confidence: 99%

“…Some patients suffered from visual field disturbances and severe headaches due to large masses located in the sellar region, mostly macroadenoma or somatotroph hyperplasia ( 41 , 47 , 59 , 67 – 72 ). In 3 cases, acromegaly signs have not been observed despite elevated growth hormone levels ( 37 – 39 ). Taking the typical long course of acromegaly into consideration, it is possible that the disease was diagnosed in its initial stages based on hormone and imaging results, enabling prompt and effective treatment.…”

Section: Methodsmentioning

confidence: 99%

Acromegaly Caused by Ectopic Growth Hormone Releasing Hormone Secretion: A Review

et al. 2022

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IntroductionEctopic acromegaly is a rare condition caused most frequently by growth hormone releasing hormone (GHRH) secretion from neuroendocrine tumors. The diagnosis is often difficult to establish as its main symptoms do not differ from those of acromegaly of pituitary origin.ObjectivesTo determine most common clinical features and diagnostic challenges in ectopic acromegaly.Patients and MethodsA search for ectopic acromegaly cases available in literature was performed using PubMed, Cochrane, and MEDline database. In this article, 127 cases of ectopic acromegaly described after GHRH isolation in 1982 are comprehensively reviewed, along with a summary of current state of knowledge on its clinical features, diagnostic methods, and treatment modalities. The most important data were compiled and compared in the tables.ResultsNeuroendocrine tumors were confirmed in 119 out of 121 patients with histopathological evaluation, mostly of lung and pancreatic origin. Clinical manifestation comprise symptoms associated with pituitary hyperplasia, such as headache or visual field disturbances, as well as typical signs of acromegaly. Other endocrinopathies may also be present depending on the tumor type. Definitive diagnosis of ectopic acromegaly requires confirmation of GHRH secretion from a tumor using either histopathological methods or GHRH plasma concentration assessment. Hormonal evaluation was available for 84 patients (66%) and histopathological confirmation for 99 cases (78%). Complete tumor resection was the main treatment method for most patients as it is a treatment of choice due to its highest effectiveness. When not feasible, somatostatin receptor ligands (SRL) therapy is the preferred treatment option. Prognosis is relatively favorable for neuroendocrine GHRH-secreting tumors with high survival rate.ConclusionAlthough ectopic acromegaly remains a rare disease, one should be aware of it as a possible differential diagnosis in patients presenting with additional symptoms or those not responding to classic treatment of acromegaly.

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“…CT imaging revealed a mass in the pancreatic tail, for which he received a resection and subsequent normalization of his LH [ 49 ]. PNET secretion of growth-hormone releasing hormone (GHRH) and adrenocorticotropic hormone (ACTH) have been reported in the literature [ 50 , 51 , 52 ], with patients presenting with the characteristic symptoms of acromegaly and Cushing syndrome, respectively.…”

Section: Presentation and Diagnosis Of Pancreatic Neuroendocrine Tumorsmentioning

confidence: 99%

Surgical Management of Pancreatic Neuroendocrine Tumors

Sulciner

Clancy

2023

Cancers

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Pancreatic neuroendocrine tumors (PNETs) are relatively uncommon malignancies, characterized as either functional or nonfunctional secondary to their secretion of biologically active hormones. A wide range of clinical behavior can be seen, with the primary prognostic indicator being tumor grade as defined by the Ki67 proliferation index and mitotic index. Surgery is the primary treatment modality for PNETs. While functional PNETs should undergo resection for symptom control as well as potential curative intent, nonfunctional PNETs are increasingly managed nonoperatively. There is increasing data to suggest small, nonfunctional PNETs (less than 2 cm) are appropriate follow with nonoperative active surveillance. Evidence supports surgical management of metastatic disease if possible, and occasionally even surgical management of the primary tumor in the setting of widespread metastases. In this review, we highlight the evolving surgical management of local and metastatic PNETs.

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Metastatic Pancreatic Neuroendocrine Tumor that Progressed to Ectopic Adrenocorticotropic Hormone (ACTH) Syndrome with Growth Hormone-releasing Hormone (GHRH) Production

Cited by 8 publications

References 11 publications

Functional pancreatic neuroendocrine tumour causing Cushing’s syndrome: the effect of chemotherapy on clinical symptoms

Functional pancreatic neuroendocrine tumour causing Cushing’s syndrome: the effect of chemotherapy on clinical symptoms

Acromegaly Caused by Ectopic Growth Hormone Releasing Hormone Secretion: A Review

Surgical Management of Pancreatic Neuroendocrine Tumors

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