Effective Therapy of Insulinoma by Using Long-Acting Somatostatin Analogue. A Case Report and Literature Review

2011 ◽  
Vol 120 (02) ◽  
pp. 68-72 ◽  
Author(s):  
A. Jawiarczyk ◽  
M. Bolanowski ◽  
J. Syrycka ◽  
G. Bednarek-Tupikowska ◽  
M. Kałużny ◽  
...  
Keyword(s):  
Adrenal Gland ◽  
Somatostatin Receptors ◽  
Subcutaneous Administration ◽  
Pancreatic Neuroendocrine Tumor ◽  
Somatostatin Analogue ◽  
Left Adrenal Gland ◽  
Glucose Levels ◽  
Long Acting ◽  
The Right

AbstractWe are reporting a case of 68-year-old woman with insulinoma, after a non-successful tumor surgery and a long-term diazoxide treatment. She had a lot of hypoglycemia cases, and a weight gain of 50 kg. An abdominal CT scan demonstrated a tumor 28 mm in the diameter, in the head of the pancreas. The patient did not agree for the repeated insulinoma surgery. Furthermore, we found a lesion in the left adrenal gland (14 mm in the diameter) and in the right lung (8 mm in the diameter). Pheochromocytoma was diagnosed on the basis of hypertension, elevated levels of normetanephrine in the 24-h urine collection, and an elevated level of norepinephrine in a plasma sample. After the left adrenal gland removal we observed lower blood pressure. Since we had revealed the presence of somatostatin receptors by the somatostatin receptors scintigraphy, we decided to control hypoglycemia by a monthly subcutaneous administration of the long-acting lanreotide. Because of higher glucose levels (300–400 mg/dl) we started an intense insulin therapy. Nowadays, the patient feels better, she has lost 20 kg of her body weight, and we have observed normal blood glucose levels during the long-term lanreotide treatment. We have noticed neither side effects nor hypoglycemic episodes and we have reduced the dose of insulin. The presented case can be an evidence of the effective treatment of the pancreatic neuroendocrine tumor of insulinoma type, with somatostatin analogue.

BECKWITH'S SYNDROME WITH EXTREME ORGAN HYPERPLASIA

PEDIATRICS ◽  
1973 ◽  
Vol 52 (3) ◽  
pp. 372-381
Author(s):  
Thomas F. Roe ◽  
Ann K. Kershnar ◽  
Jordan J. Weitzman ◽  
Luis Salinas Madrigal
Keyword(s):  
Adrenal Gland ◽  
Abdominal Mass ◽  
Severe Hypoglycemia ◽  
Immunoreactive Insulin ◽  
Left Adrenal Gland ◽  
Adrenal Steroid ◽  
Glucose Levels ◽  
Blood Glucose Levels ◽  
Pedunculated Tumor ◽  
The Right

A large newborn infant with hemihypertrophy, omphalocele, hepatomegaly, left upper quadrant abdominal mass, and zoster-like skin rash had severe hypoglycemia at 4 hours of age. Serum immunoreactive insulin levels were markedly elevated. Hypoglycemia was not controlled by vigorous medical therapy but blood glucose levels returned toward normal following subtotal excision of the markedly hyperplastic pancreas at 24 days of age. At 4½ months of age, a right upper quadrant abdominal mass was noted and urinary adrenal steroid levels were elevated. The right adrenal gland was found to be markedly hyperplastic and it was removed; the left adrenal gland was slightly hyperplastic. Between the ages of 5 and 8 months the umbilical stump enlarged progressively forming a large pedunculated tumor which was removed. This patient presents an unusually severe example of Beckwith's syndrome with both prenatal and postnatal organ overgrowth, severe hypoglycemia and hyperinsulinism.

Long-term treatment of acromegaly with Sandostatin (SMS 201-995)

1987 ◽  
Vol 116 (4_Suppla) ◽  
pp. S9-S18 ◽  
Author(s):  
G. F. F. M. Pieters ◽  
P. A. van Liessum ◽  
A. G. H. Smals ◽  
J. A. van Gennep ◽  
Th.J. Benraad ◽  
...  
Keyword(s):  
Inhibitory Effect ◽  
Term Treatment ◽  
Oral Glucose ◽  
Somatostatin Analogue ◽  
Glucose Loading ◽  
Glucose Levels ◽  
Long Term Treatment ◽  
Strong Inhibitory Effect ◽  
Long Acting

Abstract. Twelve patients with active acromegaly were treated with the long-acting somatostatin analogue SMS 201-995 (SMS), at a dose of 50 μg sc twice daily in the first 2 weeks of treatment and 100 μg sc thereafter. Four h after the first injection of SMS, GH levels became normal in 8 of the 12 patients. Basal glucose levels were significantly lower at the 28th day of treatment. This glucose lowering effect was stronger in the diabetic than in the nondiabetic patients. The post-prandial rise of insulin levels was reversed by SMS, leading to a more pronounced postprandial rise of glucose, whereas the postprandial secretion of glucagon was also reversed by SMS. The rise of glucose levels during oral glucose loading was similar before and during SMS, despite a strong inhibitory effect of the drug on the insulin rise after glucose loading. Basal TSH levels were not influenced by SMS, the TRHinduced TSH response, however, was significantly blunted. Although the basal PRL levels were significantly reduced by SMS, the TRH-induced PRL rise was similar before and during administration of the analogue. Paradoxical GH responses to TRH disappeared in 7 out of 8 patients during SMS. Paradoxical GH responses to GnRH, however, persisted in 4 out of 4 patients. Paradoxical responses of GH after glucose loading disappeared in 2 out of 2 patients. The GH response after GHRH administration was strongly suppressed by SMS. During long-term treatment (up to 2 years), the GH level obtained within 5 h after the last injection of SMS remained normal in the patients whose GH levels normalized at the first day of treatment. There was a good response of the disease to this treatment, and no serious adverse reactions were observed. We conclude that SMS normalizes most anomalous growth hormone kinetics in acromegaly. The drug offers a new tool in the treatment of this disease.

scholarly journals LONG TERM TREATMENT BY A LONG ACTING SOMATOSTATIN ANALOGUE (SMS 201–995) IN A 4 MONTHS BOY PRESENTING WITH PRIMARY PERMANENT HYPERINSULINISM

1988 ◽  
Vol 23 (1) ◽  
pp. 135-135 ◽  
Author(s):  
M T Tauber ◽  
J P Tauber ◽  
A G Harris ◽  
J M Lafitte ◽  
M Keddari ◽  
...  
Keyword(s):  
Term Treatment ◽  
Somatostatin Analogue ◽  
Long Term Treatment ◽  
Long Acting

Adrenal Pheochromocytoma With Contralateral Adrenocortical Adenoma in a Cat

2010 ◽  
Vol 46 (1) ◽  
pp. 36-42 ◽  
Author(s):  
Jon David R. Calsyn ◽  
Rebecca A. Green ◽  
Garrett J. Davis ◽  
Christopher M. Reilly
Keyword(s):  
Adrenal Gland ◽  
Low Dose ◽  
Clinical Signs ◽  
Adrenocortical Adenoma ◽  
Left Adrenal Gland ◽  
Adrenal Pheochromocytoma ◽  
History Of ◽  
Suppression Test ◽  
The Right ◽  
Dexamethasone Suppression

A 7-year-old, neutered male cat was presented with a 6-month history of progressive polyuria, polydipsia, polyphagia, aggression, and weight gain. Previous blood work, urinalysis, and radiographs did not delineate a cause for the clinical signs. An ultrasound revealed bilateral adrenal gland enlargement. A low-dose dexamethasone suppression test was consistent with hyperadrenocorticism. Based on these findings, bilateral adrenalectomy was attempted and successfully performed. Histopathology was consistent with a cortical adenoma in the right adrenal gland and a pheochromocytoma in the left adrenal gland. This association has never been reported in the cat.

scholarly journals The diagnosis and treatment of primary adrenal lipomatous tumors in Chinese patients: a 31-year follow-up study

2014 ◽  
Vol 8 (3-4) ◽  
pp. 132 ◽  
Author(s):  
Fukang Sun ◽  
Juping Zhao ◽  
Xiaolong Jing ◽  
Wenlong Zhou ◽  
Xin Huang ◽  
...  
Keyword(s):  
Adrenal Gland ◽  
Diagnosis And Treatment ◽  
Chinese Patients ◽  
Preoperative Imaging ◽  
Left Adrenal Gland ◽  
Single Centre ◽  
Follow Up Study ◽  
The Right ◽  
Lipomatous Tumors

Introduction: Adrenal lipomatous tumours (ALTs) are rarely encountered in clinical practice and consequently little is known about their clinical features.Methods: We analyze the clinical features, diagnosis and treatment of ALTs based on cases presenting at a single centre over a 31-year period. We reviewed clinical data from patients with primary adrenal tumours treated at the Ruijin Hospital, Shanghai between January 1980 and December 2010.Results: A total of 73 cases of primary ALTs in 22 men and 51 women (mean age 51.1±14.2 years) were reviewed. The ALTs included 65 myelolipomas (89.0%), 3 lipomas (4.1%), 2 angiomyolipomas (2.7%), 2 teratomas (2.7%), and 1 liposarcoma (1.4%). Of the total 73 patients, 24 of them had tumours in the left adrenal gland, 47 in the right gland and 2 had bilateral tumours. In total, 51 patients underwent open surgery and 22 laparoscopic surgery.Conclusion: Myelolipoma is predominant among the various types of lipomatous adrenal gland tumours; it accounts for about 90% of all cases. Surgery is recommended for tumours ≥3.5 cm in diameter, for all cases of symptomatic tumour, and for cases of teratoma or liposarcoma identified by preoperative imaging.

Development of resistance to a long-acting somatostatin analogue during treatment of two patients with metastatic endocrine pancreatic tumours

1988 ◽  
Vol 119 (4) ◽  
pp. 561-566 ◽  
Author(s):  
S. W.J. Lamberts ◽  
G. F. F. M. Pieters ◽  
H.J. Metselaar ◽  
G. L. Ong ◽  
H. S. Tan ◽  
...  
Keyword(s):  
Clinical Effect ◽  
Hormone Secretion ◽  
Somatostatin Receptors ◽  
Inhibitory Effect ◽  
Somatostatin Analogue ◽  
Pancreatic Tumours ◽  
Development Of Resistance ◽  
Clinical Observations ◽  
Transient Improvement ◽  
Long Acting

Abstract. Two patients with metastatic endocrine pancreatic tumours initially responded well to therapy with the long-acting somatostatin analogue SMS 201-995. In the first patient with an insulinoma both the number of hypoglycemic attacks and the increased insulin levels decreased initially, but returned to pretreatment intensity and concentrations within 9 days after the start of therapy with 200–300 μg SMS 201-995 daily. After a short interruption, no effect was observed of re-institution of therapy at a dose of 400 μg SMS 201-995 daily. In the other patient with a metastatic vipoma both diarrhea, hypokalemia and plasma VIP levels reacted initially well to SMS 201-995 treatment with 300 μg per day, but resistance to therapy developed after 2 weeks. An increase in the dose of the analogue to maximally 600 μg/day was followed by a transient improvement, but finally both the volume of diarrhea and the levels of vasoactive intestinal polypeptide were higher than those before the start of therapy. Conclusions: Development of resistance to SMS 201-995 both with regard to the clinical effect and to the inhibitory effect on tumour hormone secretion can be expected in some patients with metastatic endocrine pancreatic tumours. On the basis of our clinical observations down-regulation of somatostatin receptors is suggested to be one of the mechanisms of this development.

scholarly journals SUN-910 High Enhancement Washout by CT Imaging Does Not Exclude Pheochromocytoma/Paraganglioma: Review of Two Cases

2020 ◽  
Vol 4 (Supplement_1) ◽  
Author(s):  
Feyza Erenler ◽  
Ronald M Lechan
Keyword(s):  
Adrenal Gland ◽  
Laparoscopic Adrenalectomy ◽  
Ct Imaging ◽  
Cardiac Resynchronization ◽  
Vanillylmandelic Acid ◽  
Mibg Imaging ◽  
Left Adrenal Gland ◽  
Urinary Catecholamines ◽  
The Right ◽  
Adrenal Nodule

Abstract Background: It is well known that delayed images from contrast-enhanced CT are useful in distinguishing adrenal adenomas from non-adenomas, with an absolute washout that exceeds 60% being most consistent with a lipid rich adenoma. We present two cases of an adrenal mass that met the criteria for a lipid rich adenoma by CT imaging, but found to be a pheochromocytoma (PCC) and paraganglioma (PGL). Clinical Case Case#1 An 82 yo woman presenting with tachycardia was found to have a 2.4 cm heterogeneously attenuating, left adrenal nodule with an absolute washout of 61% and a relative washout of 45%. The right adrenal was normal. Urinary catecholamine levels were elevated with an epinephrine (E) 38 mcg (2–24), norepinephrine (NE) 388 mcg (15–100), dopamine (DOPA) 175 mcg (52–480), metanephrine (MN) mcg 620 (90–315), normetanephrine (NMN) 1553 mcg (122–676) and vanillylmandelic acid 12.5 mg (< 6) on a 24h collection. Due to a cardiac resynchronization therapy device, an MRI could not be obtained. MIBG imaging was obtained and showed increased uptake in left adrenal gland, corresponding to the lesion identified on CT. The patient underwent laparoscopic adrenalectomy and the pathology confirmed a PCC. Case#2: A 74 yo man was found to have an incidental right adrenal nodule on CT imaging measuring 2.4 cm. Absolute washout was 83% and relative washout 68%. The left adrenal gland was normal. A follow up MRI obtained showed slight increase in T2 weighted images and no drop out on out of phase imaging, raising concern for a PCC. Urinary catecholamines were elevated including E 12 mcg (2–24), NE 280 mcg (15–100), DOPA 246 mcg (52–480), MN 175 mcg (90–315) and NMN 1298 mcg (122–676) on a 24-hr. collection. MIBG imaging further confirmed the diagnosis with increased uptake in the right adrenal gland. The patient underwent laparoscopic adrenalectomy then, converted to open right adrenalectomy through an anterior approach due to adherence of the tumor to the renal vein. The pathology revealed a PGL. Conclusion: PCC/PGL are rare but life-threatening neuroendocrine tumors that require early detection to reduce associated morbidities and mortality and improve surgical outcomes. CT is commonly used to characterize adrenal lesions and an absolute washout of >60% is most consistent with an adenoma. However, as demonstrated by these two cases, washout exceeding 60% can also be seen in non-adenomas, perhaps secondary to degeneration of the nodule causing necrotic or cystic changes or uncommonly, the presence of a high lipid content in the tumor [1]. Thus, when clinical suspicion is strong and/or there is a positive biochemical workup, confirmatory imaging should be considered to establish the diagnosis. References: [1] Blake, M. A., Kalra, M. K., Maher, M. M., Sahani, D. V., Sweeney, A. T., Mueller, P. R., ... & Boland, G. W. (2004). Pheochromocytoma: an imaging chameleon. Radiographics, 24(suppl_1), S87-S99.

scholarly journals Ectopic ACTH-Producing Pheochromocytoma as a Rare Cause for Rapid Progressing Cushing Syndrome: A Case Report

2021 ◽  
Vol 5 (Supplement_1) ◽  
pp. A128-A128
Author(s):  
Daniela Salazar ◽  
Cláudia Fernandes Costa ◽  
Joana Oliveira ◽  
Liliana Violante ◽  
Ana Luísa Cunha ◽  
...  
Keyword(s):  
Adrenal Gland ◽  
Cushing Syndrome ◽  
Somatostatin Receptors ◽  
Osteoporotic Fractures ◽  
Urinary Free Cortisol ◽  
Hand Tremor ◽  
Ectopic Acth ◽  
Late Night ◽  
Free Cortisol ◽  
The Right

Abstract Background: Cushing syndrome due to ectopic CRH or ACTH secretion can be rarely caused by pheochromocytoma, commonly as part of genetic conditions. Case: A 21 year-old male, previously healthy, with no usual medication, went to the physician assistant for hematuria. The kidney US revealed, besides lithiasis, a highly vascularized mass in the right adrenal gland with 10 cm. In his first evaluation the patient had no complaints or pheochromocitoma/hypercortisolism stigmata, other than hand tremor and slight rounding of the face. Blood pressure was 149/88 mmHg, and heart rate 86 bpm. There was no family history of endocrine disease. He rapidly developed increased appetite, insomnia, and severe myalgias, with filling of supraclavicular fossae, facial plethora, and cervical and truncal acne. Laboratory analysis showed abnormal overnight dexamethasone suppression test (57.4 µg/dL, N < 1.8), elevated ACTH 378 pg/mL (N 9.0–52.0), 24h-urinary free cortisol (UFC) (5334.0 µg/24h, N 4.3–176.0), and late-night salivar cortisol (1.44 µg/dL, N < 0.32), hypokalemia (2.8 mEq/L, N 3.8–5.0), and leukocytosis (22.4*109/L, N 4.0–11.0); DHEA-S 962 µg/dL (N 80–560), 4-androstenedione 380 ng/dL (N 70–360), 17-OH progesterone 4.5 ng/mL (N 0.59–3.44), cromogranine A 6063 ng/mL (N 0–100), and markedly elevated urinary amines (adrenaline 173 nmol/24h, N 0–109; noradrenaline 5033 nmol/24h, N 89–473; normetanephrine 334605 nmol/24h, N 480–2424; metanephrine 15998 nmol/24h, N 264–1729; dopamine 4808 nmol/24h, N 424–2612). Hypercalcemia with hypophosphatemia and supressed PTH level was also detected. 68Ga-DOTANOC PET revealed a mass of the right adrenal gland with overexpression of somatostatin receptors (likely pheochromocytoma), without evidence of other tumor lesions of neuroendocrine origin. Pituitary MRI showed normal pituitary gland. Potassium supplementation, alpha-blockade with phenoxybenzamine, and metyrapone were initiated. Due to severe back pain, a CT scan of the spine was performed detecting compressive osteoporotic fractures in the mid dorsal and low dorsal segments. The patient was submitted to right adrenalectomy. Histology revealed pheochromocytoma with 11.2*9mm, with capsular and vascular invasion, extra-adrenal extension, necrosis, and atypical mitosis, with Ki67 of 9.5% and PASS score of 16. Postoperative analysis showed ACTH 45.6 pg/mL (N 7.2-63,3), late-night salivar cortisol < 0,0544 µg/dL (N < 0,32) and free urinary cortisol 41.4 µg/24h (N 4.3–176.0). Discussion: Ectopic cushing syndrome caused by pheochromocytoma is a rarely described entity. In this young patient, it caused rapid clinical progression of hypercortisolism with important hydroelectrolytic disturbances and compressive vertebral fractures, requiring prompt surgical intervention for clinical remission and improvement.

Resistant Paediatric Somatotropinomas due to AIP Mutations: Role of Pegvisomant

2018 ◽  
Vol 90 (3) ◽  
pp. 196-202 ◽  
Author(s):  
Kriti Joshi ◽  
Adrian F. Daly ◽  
Albert Beckers ◽  
Margaret Zacharin
Keyword(s):  
Combined Therapy ◽  
Somatostatin Analogues ◽  
Somatostatin Analogue ◽  
Tumour Mass ◽  
Residual Tumour ◽  
Control Disease ◽  
Long Acting ◽  
Analogue Octreotide

Background: Somatotropinomas are rare in childhood and frequently associated with genetic mutations. AIP mutations are found in 20–25% cases and cause aggressive somatotropinomas, often resistant to somatostatin analogues. Aims: To assess responses to multimodal therapy including pegvisomant in 2 children with sporadic somatotropinomas due to AIP mutations. Case Description: We report 2 children, a boy aged 13 and a girl aged 10, with rapid growth, visual impairment, and growth hormone hypersecretion. Magnetic resonance imaging confirmed a pituitary macroadenoma with parasellar extension in both. Despite multiple surgical attempts to debulk tumour mass, residual tumour persisted. Genetic analysis showed two different AIP mutations (patient 1: c.562delC [p.Arg188Glyfs*8]; patient 2: c.140_ 163del24 [p.Gly47_Arg54del8]). They were initially treated with a long-acting somatostatin analogue (octreotide LAR 30 mg/month) and cabergoline as a dopamine agonist, with the later addition of pegvisomant titrated up to 20 mg/day and with radiotherapy for long-term control. Somatostatin analogue was ceased due to patient intolerance and lack of control. Patient 1 had normalization of insulin-like growth factor-1 (IGF-1) after 5 months of combined therapy with pegvisomant and cabergoline. For patient 2, normalization of IGF-1 was achieved after 2 months of cabergoline and pegvisomant. Conclusion: AIP-associated tumours can be resistant to management with somatostatin analogues. Pegvisomant can safely be used, to normalize IGF-1 levels and help control disease.

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