scholarly journals MON-332 Metastatic Pancreatic Neuroendocrine Tumor: A Very Rare Cause of Ectopic ACTH Production (ACTHoma)

2019 ◽  
Vol 3 (Supplement_1) ◽  
Author(s):  
Ghadah Al-Naqeeb ◽  
Sarmed Al Yassin ◽  
Rupa Patel ◽  
Alan Kaell ◽  
Georgia Kulina ◽  
...  
Keyword(s):  
Neuroendocrine Tumor ◽  
Pancreatic Neuroendocrine Tumor ◽  
Ectopic Acth ◽  
Ectopic Acth Production

scholarly journals MON-921 Autonomous Cortisol Secretion Coexisting with Pancreatic Neuroendocrine Tumor: A Rare Presentation

2020 ◽  
Vol 4 (Supplement_1) ◽  
Author(s):  
Fernanda Faro ◽  
Gabriela Karman ◽  
Nathalia Dal-Prá ◽  
Jéssica Loureiro ◽  
Cecilia Kauffman Rutenberg Feder ◽  
...  
Keyword(s):  
Neuroendocrine Tumor ◽  
Pancreatic Neuroendocrine Tumor ◽  
Systemic Hypertension ◽  
Cortisol Secretion ◽  
Ectopic Acth ◽  
Ectopic Acth Secretion ◽  
Adrenocortical Tumors ◽  
Autonomous Cortisol Secretion ◽  
Adrenal Nodule

Abstract Introduction: neuroendocrine tumors (NET) are a very rare and heterogeneous group of malignancies that can be associated with adrenocortical tumors in approximately 20% of the cases, mostly bilateral and non-functioning. Autonomous cortisol secretion occurs in less than 10% of adrenal incidentalomas and the coexistence of pancreatic neuroendocrine neoplasms and autonomous cortisol secretion is not well-described. Clinical case: a 54-year-old man with previous history of systemic hypertension and type 2 diabetes mellitus, presented with left hypochondrium pain in the last 18 months, associated with abdominal distension, constipation and nausea. Physical examination without abnormalities. Abdominal tomography demonstrated dilatated pancreatic duct and a solid heterogeneous nodule in left adrenal, measuring about 2.7 cm. Ecoendoscopy revealed a heterogeneous, hypoechoic and oval nodular lesion, located at the transition of pancreatic head and uncinate process, measuring 1.5x1.1cm. Biopsy was performed, showing a pattern of neuroendocrine neoplasia, with chromogranin and synaptophysin +, Ki67 1%. Gallium-68 dotatate PET revealed two pancreatic nodular formations, one in proximal neck/body (1.5 cm) and the other in pancreatic tail (1 cm), presenting SUV of 20.4 and 21, respectively. Adrenal nodule presented minimal increase in radiopharmaceutical concentration. To exclude the hypothesis of metastasis, PET FDG was performed, showing physiological uptake in adrenal nodule. Pituitary MRI had no abnormalities. Chromogranin A and gastrin values were normal. Pheochromocytoma and primary hyperaldosteronism were excluded. Hypercortisolism investigation presented the following results: 23h salivary cortisol 167ng/dl (NR < 100), 24-hour free urinary cortisol 42.1 mcg/24h (NR 4.2-60), post-1mg and 2mg dexamethasone serum cortisol of 10.8 mcg/dl and 3.8 respectively (serum dexamethasone levels of 193 and 780 ng/dl; NR > 130), ACTH 13 and 11 pg/ml. By these results, coexistence of non-functioning pancreatic neuroendocrine tumor and autonomous cortisol secretion was confirmed. A total pancreatectomy with partial gastrectomy and bileodigestive anastomosis was performed. Pathological anatomical evidence demonstrated a well-differentiated neuroendocrine tumor (NET G1) and immunohistochemistry analyses showed positive chromogranine A, synaptophysine, Ki67 1% and negative ACTH. Clinical follow-up of the adrenal adenoma was preferred. Conclusion: although most adrenocortical tumors associated with NET are nonfunctional, hypercortisolism should be considered. Adrenal metastasis and ectopic ACTH secretion are differential diagnosis. Clinical follow-up is an option when patient is asymptomatic and comorbidities are well-controlled.

scholarly journals Pancreatic Neuroendocrine Tumor with Ectopic Adrenocorticotropin Production upon Second Recurrence

2004 ◽  
Vol 89 (8) ◽  
pp. 3731-3736 ◽  
Author(s):  
Konstanze Miehle ◽  
Andrea Tannapfel ◽  
Peter Lamesch ◽  
Gudrun Borte ◽  
Eva Schenker ◽  
...  
Keyword(s):  
Neuroendocrine Tumor ◽  
Tumor Cells ◽  
Cushing’S Syndrome ◽  
Left Kidney ◽  
Tumor Resection ◽  
Pancreatic Neuroendocrine Tumor ◽  
Cushing's Syndrome ◽  
Tumor Evolution ◽  
Ectopic Acth ◽  
Depressive Episodes

We present a 54-yr-old woman with ectopic corticotropin syndrome caused by a neuroendocrine tumor of the pancreas. At initial presentation, the patient suffered from diarrhea, heartburn, and nonspecific abdominal pain. There was no evidence of Cushing’s syndrome. A neuroendocrine tumor in the head of the pancreas with metastases into peripancreatic lymph nodes was diagnosed and completely resected. Fourteen months later, abdominal computed tomography and scintigraphy with 111In-labeled octreotide suggested relapse of the tumor. The patient again had no evidence of Cushing’s syndrome. A second in toto tumor resection was performed. Another 8 months later, the patient developed forgetfulness, depressive episodes, muscle weakness, new-onset hypertension, hypokalemia, plethora, diabetes mellitus, polyuria, and weight loss. Endocrine testing suggested a source of ectopic ACTH production. An octreotide scan showed an intense uptake ventromedial of the left kidney, an area that showed a mass lateral of the superior mesenteric artery on abdominal magnetic resonance imaging. A complete pancreatectomy with splenectomy and left-sided adrenalectomy were performed. At this second relapse, this neuroendocrine tumor clinically had changed its hormonal profile. Immunohistochemically, in contrast to primary tumor and first relapse, we found strong immunostaining for ACTH in tumor cells of the second relapse and a MIB-1 index greater than 20%. To our knowledge, this is the first report describing a pancreatic neuroendocrine tumor that started to secrete ACTH de novo at the time of the second relapse after two former complete tumor resections. This case underscores the pluripotency of neuroendocrine tumor cells and the importance of keeping in mind a possible shift in hormone production during tumor evolution and progression.

scholarly journals A Case of Acute Psychosis as Manifestation of Ectopic ACTH Producing Pancreatic Neuroendocrine Tumor

2021 ◽  
Vol 5 (Supplement_1) ◽  
pp. A558-A559
Author(s):  
Banafsheh Motazedi ◽  
Laura E Cowen ◽  
Jacqueline Jonklaas
Keyword(s):  
Neuroendocrine Tumor ◽  
Cushing’S Syndrome ◽  
Chromogranin A ◽  
Psychiatric Symptoms ◽  
Pancreatic Head ◽  
Pancreatic Neuroendocrine Tumor ◽  
Ambient Air ◽  
Cushing's Syndrome ◽  
Ectopic Acth ◽  
Free Cortisol

Abstract Introduction: Ectopic adrenocorticotrophic hormone (ACTH) producing Pancreatic Neuroendocrine Tumor (p-NET) are extremely rare with incidence of 1.2% and confer a poor prognosis (5-year survival <20%). Case Report: 31-year old female with diabetes mellitus type 2 (DM-2) and hypertension (HTN) was found to be severely hypokalemic on routine labs and was sent to the Emergency Department for potassium replacement. Vitals; BP:139/87 mmHg, HR:108 bpm, RR:18, T:37°C and saturating 98% on ambient air. Examination revealed cushingoid features; hyperpigmented face, bilateral upper extremity pigmented papules, proximal muscle weakness, acanthosis nigricans, violaceous abdominal striae, truncal obesity, ecchymoses, bilateral lower extremity edema, memory impairment and anxiety. Initial labs; BG: 289 mg/dL, HbA1c: 9.5%, K: 2 mmol/L (N 3.5-5.2), WBC: 12.5 k/μL (N 4-10.8), Cortisol >150 mcg/dL (N 5-23), TSH: 0.325 μIU/mL (N 0.45-4.5), and FT4: 0.69 ng/dL (N 0.82-1.77). Diagnosis of Cushing’s syndrome was made based on elevated cortisol level and confirmed with elevated 24-hour urinary free cortisol: 13,294 μg (N<45), midnight salivary cortisol >15 ug/dL (N 0.09-1.5) and 1 mg dexamethasone suppression test (Cortisol >150 mcg/dL, ACTH: 621 pg/ml). Additional labs showed Chromogranin A: 717 ng/mL (N 0-160). She was started on prophylactic Enoxaparin & Trimethoprim/Sulfamethoxazole and treated with Spironolactone, Basal/Bolus insulin, and Ketoconazole for hypercortisolemia. Pituitary MRI was negative for pituitary adenoma. Abdominal/Pelvic CT scan demonstrated a solid 5 cm pancreatic head mass with innumerable haptic metastases consistent with metastatic p-NET which was confirmed on PET-CT Ga68 Dotatate scan. Liver biopsy was positive for a well differentiated p-NET with ki-67 <5% and negative ACTH staining. Hospital course was complicated by fluctuating cortisol levels with intermittent psychotic manifestations despite up-titration of Ketoconazole, addition of Octreotide as well as anti-psychotics. She later developed transaminitis on maximum dose of Ketoconazole and thus underwent laparoscopic total adrenalectomy for treatment of her hypercortisolemia. Post-operatively, she was started on Hydrocortisone & Fludrocortisone and Spironolactone was discontinued. She was transitioned to monthly Lanreotide and treated with Capecitabine and Temozolomide with adequate therapeutic response and subsequent down trending of her Chromogranin A: 188 ng/mL and ACTH: 228 pg/ml levels. Currently, she is doing well with significant improvement of her DM-2, HTN, psychiatric symptoms and overall clinical status. Conclusions: Symptoms of ectopic Cushing’s syndrome may be delayed, however given the rare and aggressive nature of ACTH producing p-NET, timely diagnosis, prophylaxis and individualized treatment approach is crucial in achieving favorable prognosis.

Adrenocorticotropin Production by a Mammary Carcinoma

1977 ◽  
Vol 35 ◽  
pp. 462-463
Author(s):  
K.S. McCarty ◽  
N.R. Wallace ◽  
W. Litaker ◽  
S. Wells ◽  
G. Eisenbarth
Keyword(s):  
Adrenocorticotropic Hormone ◽  
Small Cell ◽  
Hormone Production ◽  
Carcinoma Of The Breast ◽  
Ectopic Acth ◽  
Ultrastructural Studies ◽  
Subcutaneous Metastases ◽  
Ectopic Acth Production ◽  
The Right ◽  
Pituitary Carcinomas

The production of adrenocorticotropic hormone by non-pituitary carcinomas has been documented in several tumors, most frequently small cell carcinoma of the lung, islet cell carcinomas of the pancreas, thymomas and carcinoids. Electron microscopy of these tumors reveals typical membrane-limited "neurosecretory" granules. Confirmation of the granules as adrenocorticotropin (ACTH) requires the use of OsO4 as a primary fixative to give the characteristic cored granule appearance in conjunction with immunohistochemical demonstration of the hormone peptide. Because of the rarity of ectopic ACTH production by mammary carcinomas and the absence of appropriate ultrastructural studies in the two examples of such ectopic hormone production in the literature of which we are aware (1,2), we present biochemical and ultrastructural data from a carcinoma of the breast with apparent ACTH production.The patient had her primary tumor in the right breast in 1969. The tumor recurred as visceral and subcutaneous metastases in 1976 and again in 1977.

Adrenocortical Ultrastructure in Ectopic Acth Syndrome

1976 ◽  
Vol 34 ◽  
pp. 238-239
Author(s):  
K. Kovacs ◽  
E. Horvath ◽  
W. Singer
Keyword(s):  
Adrenal Cortex ◽  
Zona Glomerulosa ◽  
Ectopic Acth Syndrome ◽  
Pituitary Neoplasms ◽  
Electron Microscopic ◽  
Adrenocortical Cells ◽  
Cell Hyperplasia ◽  
Ectopic Acth ◽  
Ectopic Acth Production ◽  
Microscopic Findings

Secretion of ACTH by non-pituitary neoplasms is recognized with increasing frequency. While the clinical and biochemical changes associated with ectopic ACTH production have been extensively studied recently, relatively little attention was focused on the morphology of the adrenal cortex and, to our knowledge, the fine structure of the adrenocortical cells in cases of ectopic ACTH syndrome has not been described so far. We report here the electron microscopic findings in the adrenal cortex of a 50-year-old man with a pancreatic apudoma. The patient showed the characteristic clinical and biochemical features of ectopic ACTH syndrome and because of extensive hypercorticism, underwent bilateral adrenalectomy.By light microscopy, the adrenal cortices showed extensive compact cell hyperplasia and lipid depletion. The zona glomerulosa was present in small foci and, except for a few places, fasciculata cells were noted under the fibrous capsule.

Sign in / Sign up

Export Citation Format

Share Document