scholarly journals Successful medical management of insulinoma with diazoxide for 27 years

2020 ◽  
Vol 2020 ◽  
Author(s):  
Annabelle M Warren ◽  
Duncan J Topliss ◽  
Peter Shane Hamblin
Keyword(s):  
Case Report ◽  
Adverse Effects ◽  
Medical Management ◽  
List Type ◽  
Successful Management ◽  
Pet Ct ◽  
Effective Option ◽  
Pet Ct Scan ◽  
Learning Points

Summary Despite improvements in localisation techniques and surgical advances, some patients with insulinoma will not be cured by surgery or may not be suitable for surgery. Medical management with diazoxide is an option for such cases. This case report details 27 years of successful management of insulinoma using diazoxide. It has been effective and safe, with only minor adverse effects. Learning points: Long term diazoxide use can be a safe, effective option for insulinoma when it cannot be localised or removed surgically. Common adverse effects include peripheral oedema, hyperuricaemia, and hirsutism. 68Ga-NOTA-exendin-4 PET/CT scan should be considered for insulinoma localisation when other modalities have been unhelpful.

scholarly journals Positive outcome in a high-grade myxofibrosarcoma: a case report

2018 ◽  
Vol 1 (1) ◽  
pp. 27-30
Author(s):  
Mihaela Olaru ◽  
Cornelia Nitipir
Keyword(s):  
Case Report ◽  
Fibrous Histiocytoma ◽  
Tumor Resection ◽  
Pulmonary Nodules ◽  
Positive Outcome ◽  
Pet Ct ◽  
Longus Muscle ◽  
Long Term Follow Up

AbstractMyxofibrosarcoma or myxoid malignant fibrous histiocytoma is one of the most common sarcomas of the limb. It is usually treated multimodally. Most frequent sites of metastasis are the bone, lung and lymph nodes. The present paper is a case report of a 65-year-old male with myxofibrosarcoma of the fibularis longus muscle, for which he first underwent surgery - tumor resection with appropriate margins. The tumor was staged pT2b cN0 cM0. Postoperative PET-CT revealed metabolically inactive pulmonary nodules. Two months after surgery, he underwent adjuvant radiotherapy, a total dose of 60 Gy and 6 courses of chemotherapy (doxorubicin and ifosfamide). Pulmonary nodules have been stationary on all subsequent imagistic studies. He is free of recurrence on long-term follow-up.

scholarly journals Fresh frozen plasma as a source of plasminogen for ligneous conjunctivitis: Case report and a review of the literature

2021 ◽  
pp. 031-035
Author(s):  
Park Benjamin J ◽  
Camoriano David ◽  
Vital Mark C ◽  
Chévez-Barrios Patricia ◽  
Goosey John D ◽  
...  
Keyword(s):  
Case Report ◽  
Fresh Frozen Plasma ◽  
Term Treatment ◽  
Ligneous Conjunctivitis ◽  
Caucasian Girl ◽  
Long Term Treatment ◽  
Effective Option ◽  
Fresh Frozen ◽  
Frozen Plasma

Purpose: To describe management of a patient with ligneous conjunctivitis secondary to plasminogen deficiency and review the literature on treatment with plasminogen. Design: Interventional case report. Methods: A 6-year-old Caucasian girl developed ligneous conjunctivitis recalcitrant to debridement and treatment with topical cyclosporin A and steroids. The literature was reviewed regarding treatment of ligneous conjunctivitis with plasminogen. Results: The patient was treated with plasminogen containing drops derived from her father’s fresh frozen plasma. The ligneous conjunctivitis resolved, and the patient was managed on these drops for the following 8 years, initiating treatment for flares and tapering according to clinical response. Conclusions: Plasminogen drops concentrated from fresh frozen plasma are a safe and effective option for long-term treatment of ligneous conjunctivitis.

scholarly journals Effective long-term temozolomide rechallenge in a macroprolactinoma

2018 ◽  
Vol 2018 ◽  
Author(s):  
Benedetta Zampetti ◽  
Giorgia Simonetti ◽  
Roberto Attanasio ◽  
Antonio Silvani ◽  
Renato Cozzi
Keyword(s):  
Aggressive Behavior ◽  
Acquired Resistance ◽  
Pituitary Tumors ◽  
List Type ◽  
Fractionated Radiotherapy ◽  
Resistance To Treatment ◽  
Aggressive Tumors ◽  
Learning Points ◽  
Treatment Surgery

Summary We describe the 20-year course of a 63-year-old male with a macroprolactinoma that acquired resistance to treatment and aggressive behavior after a 4-year successful treatment with cabergoline. He was submitted to multiple surgical resections by a skilled surgeon, fractionated radiotherapy and was eventually treated with temozolomide. After a first 6-month standard cycle, a relapse occurred and he was treated again successfully. Learning points: Prolactinomas are the most frequent type of pituitary adenoma. They usually have a benign course. In most cases dopamine-agonist drugs, mainly cabergoline, are first-line (and usually only) treatment. Occasionally prolactinomas can have or acquire resistance to treatment and/or aggressive behavior. Temozolomide (TMZ), an oral alkylating drug, can be effective in such aggressive tumors. Multimodal treatment (surgery, radiation, cabergoline and TMZ) is warranted in aggressive pituitary tumors. We describe here successful rechallenge with TMZ after relapse occurring 18 months after a first TMZ cycle.

scholarly journals Pasireotide in an insulin-requiring diabetic acromegalic patient without worsening of hyperglycemia

2017 ◽  
Vol 2017 ◽  
Author(s):  
Murray B Gordon ◽  
Kellie L Spiller
Keyword(s):  
Diabetes Mellitus ◽  
Type 2 Diabetes ◽  
Type 2 Diabetes Mellitus ◽  
List Type ◽  
Effective Treatment Option ◽  
Patients With Diabetes ◽  
Long Acting ◽  
Learning Points

Summary Long-acting pasireotide is an effective treatment option for acromegaly, but it is associated with hyperglycemia, which could impact its use in patients with diabetes. We present a case of a 53-year-old man with acromegaly and type 2 diabetes mellitus (glycated hemoglobin (HbA1c): 7.5%), who refused surgery to remove a pituitary macroadenoma and enrolled in a Phase 3 clinical trial comparing long-acting pasireotide and long-acting octreotide in acromegalic patients. The patient initially received octreotide, but insulin-like growth factor 1 (IGF-1) levels remained elevated after 12 months (383.9 ng/mL; 193.0 ng/mL; reference range: 86.5–223.8 ng/mL), indicating uncontrolled acromegaly. He switched to pasireotide 40 mg and subsequently increased to 60 mg. Within 6 months, IGF-1 levels normalized (193.0 ng/mL), and they were mostly normal for the next 62 months of treatment with pasireotide (median IGF-1: 190.7 ng/mL). Additionally, HbA1c levels remained similar to or lower than baseline levels (range, 6.7% to 7.8%) during treatment with pasireotide despite major changes to the patient’s antidiabetic regimen, which included insulin and metformin. Uncontrolled acromegaly can result in hyperglycemia due to an increase in insulin resistance. Despite having insulin-requiring type 2 diabetes, the patient presented here did not experience a long-term increase in HbA1c levels upon initiating pasireotide, likely because long-term control of acromegaly resulted in increased insulin sensitivity. This case highlights the utility of long-acting pasireotide to treat acromegaly in patients whose levels were uncontrolled after long-acting octreotide and who manage diabetes with insulin. Learning points Long-acting pasireotide provided adequate, long-term biochemical control of acromegaly in a patient with insulin-requiring type 2 diabetes mellitus who was unresponsive to long-acting octreotide. Glycemic levels initially increased after starting treatment with pasireotide but quickly stabilized as acromegaly became controlled. Long-acting pasireotide, along with an appropriate antidiabetic regimen, may be a suitable therapy for patients with acromegaly who also have insulin-requiring type 2 diabetes mellitus.

False positive in pelvis in a PET-CT scan due to ovarian reimplantation: A case report

2010 ◽  
Vol 29 (2) ◽  
pp. 87-90
Author(s):  
M. Simó Perdigó ◽  
P. Pifarre Montaner ◽  
C. Moreno Capdevila ◽  
A. Escudero Rodríguez ◽  
E. Martínez Miralles
Keyword(s):  
Case Report ◽  
Ct Scan ◽  
False Positive ◽  
Pet Ct ◽  
Pet Ct Scan

scholarly journals A black adrenal adenoma with high FDG uptake on PET/CT scan in a patient with esophageal carcinoma: A case report

2018 ◽  
Vol 44 ◽  
pp. 118-121
Author(s):  
Shiro Matsumoto ◽  
Yoshinori Hosoya ◽  
Alan Kawarai Lefor ◽  
Hidenori Haruta ◽  
Takashi Ui ◽  
...  
Keyword(s):  
Case Report ◽  
Esophageal Carcinoma ◽  
Ct Scan ◽  
Adrenal Adenoma ◽  
Fdg Uptake ◽  
Pet Ct ◽  
Black Adrenal Adenoma ◽  
Pet Ct Scan

scholarly journals Paternally inherited ABCC8 mutation causing diffuse congenital hyperinsulinism

2013 ◽  
Vol 2013 ◽  
Author(s):  
Suresh Chandran ◽  
Fabian Yap Kok Peng ◽  
Victor Samuel Rajadurai ◽  
Yap Te Lu ◽  
Kenneth T E Chang ◽  
...  
Keyword(s):  
Ct Scan ◽  
Severe Hypoglycaemia ◽  
Congenital Hyperinsulinism ◽  
List Type ◽  
Focal Lesions ◽  
Hyperinsulinaemic Hypoglycaemia ◽  
Partial Pancreatectomy ◽  
Diffuse Disease ◽  
Pet Ct ◽  
Pet Ct Scan

Summary background: Congenital hyperinsulinism (CHI) is a rare genetic disorder characterised by inappropriate insulin secretion in the face of severe hypoglycaemia. There are two histological subtypes of CHI namely diffuse and focal. Diffuse CHI is most common due to recessive mutations in ABCC8/KCNJ11 (which encode the SUR/KIR6.2 components of the pancreatic β-cell KATP channel) whereas focal CHI is due to a paternally inherited ABCC8/KCNJ11 mutation and somatic loss of heterozygosity for the 11p allele inside the focal lesion. Fluorine-18-l-dihydroxyphenylalanine positron emission tomography/computed tomography (18F-DOPA-PET/CT) is used in the pre-operative localisation of focal lesions prior to surgery. Diffuse CHI if medically unresponsive will require a near total pancreatectomy whereas focal CHI will only require a limited lesionectomy, thus curing the patient from the hypoglycaemia. Aims: To report the first case of genetically confirmed CHI in Singapore from a heterozygous paternally inherited ABCC8 mutation. Methods/Results: A term male infant presented with severe hyperinsulinaemic hypoglycaemia (HH) after birth and failed medical treatment with diazoxide and octreotide. Genetic testing (paternally inherited mutation in ABCC8/p.D1472N) suggested focal disease, but due to the unavailability of 18F-DOPA-PET/CT to confirm focal disease, a partial pancreatectomy was performed. Interestingly, histology of the resected pancreatic tissue showed changes typical of diffuse disease. Conclusion: Heterozygous paternally inherited ABCC8/KCNJ11 mutations can lead to diffuse or focal CHI. Learning points HH is a cause of severe hypoglycaemia in the newborn period. Paternal mutations in ABCC8/KCNJ11 can lead to diffuse or focal disease. 18F-DOPA-PET/CT scan is the current imaging of choice for localising focal lesions. Gallium-68 tetra-aza-cyclododecane-N N′N″N-‴-tetra-acetate octreotate PET scan is not a useful imaging tool for localising focal lesions. The molecular mechanism by which a heterozygous ABCC8 mutation leads to diffuse disease is currently unclear. Focal lesions are curable by lesionectomy and so genetic studies in patients with HH must be followed by imaging using 18F-DOPA-PET/CT scan.

scholarly journals Rituximab: a novel treatment for refractory Riedel’s thyroiditis

2018 ◽  
Vol 2018 ◽  
Author(s):  
Leanne Hunt ◽  
Barney Harrison ◽  
Matthew Bull ◽  
Tim Stephenson ◽  
Amit Allahabadia
Keyword(s):  
Monoclonal Antibody ◽  
Case Report ◽  
Rare Condition ◽  
List Type ◽  
Patient Case ◽  
Riedel’S Thyroiditis ◽  
Novel Treatment ◽  
Tender Mass ◽  
Learning Points ◽  
Riedel's Thyroiditis

Summary This case report reviews the rare condition of Riedel’s thyroiditis via a patient case. The report highlights the difficulties that one may encounter when managing such a case in regards to patient symptoms, side effects of medications and the relapsing nature of the condition. The case report also highlights novel treatment in the treatment of Riedel’s thyroiditis, rituximab, how this works and the resolution of symptoms that we have achieved with our patient on this treatment. Learning points: Riedel’s thyroiditis is characterised by chronic inflammation, which causes dense fibrosis in the thyroid gland. Riedel’s thyroiditis can present with neck pain, dysphagia and dyspnoea with a firm, non-tender mass found on examination. Riedel’s thyroiditis is part of the IgG4-related systemic disorders. Rituximab is a monoclonal antibody that works against the protein CD20.

scholarly journals Isolated hypophosphataemia as an early marker of primary hyperparathyroidism

2021 ◽  
Vol 2021 ◽  
Author(s):  
John Alexander ◽  
Dinesh Nagi
Keyword(s):  
Primary Hyperparathyroidism ◽  
List Type ◽  
Biochemical Abnormality ◽  
Common Presentation ◽  
Long Term Monitoring ◽  
Biochemical Testing ◽  
Term Monitoring ◽  
Learning Points ◽  
Biochemical Abnormalities

Summary Primary hyperparathyroidism (PHPT) is a disease caused by overactive parathyroid glands with consequent hypercalcaemia. The main cause in 85–90% of the cases is the presence of a solitary parathyroid adenoma. The most common presentation is with asymptomatic hypercalcaemia diagnosed on routine biochemical testing. Although low serum phosphate levels are an associated finding in primary hyperparathyroidism, the diagnostic criteria for PHPT remain to be hypercalcaemia, high or inappropriately normal PTH and hypercalciuria. This case report presents a patient who presented with low phosphate levels without any other biochemical evidence of PHPT, who returned several years later with overt primary hyperparathyroidism. This report intends to raise interest among the medical fraternity whether there is a need to consider hypophosphataemia as an early sign of PHPT. Learning points Primary hyperparathyroidism is a relatively common condition with varying clinical and biochemical presentation. The most common presentations still remain as an asymptomatic biochemical abnormality closely related to calcium, PTH and bone metabolism. Not much attention is usually given to associated biochemical abnormalities, and hence they are usually less investigated. Further research is needed to establish if patients need long-term monitoring when no obvious cause for isolated hypophosphataemia has been found.

scholarly journals Sirolimus therapy in a child with partially diazoxide-responsive hyperinsulinaemic hypoglycaemia

2016 ◽  
Vol 2016 ◽  
Author(s):  
Kah-Yin Loke ◽  
Andrew Sng Anjian ◽  
Yvonne Lim Yijuan ◽  
Cindy Ho Wei Li ◽  
Maria Güemes ◽  
...  
Keyword(s):  
Weight Gain ◽  
Side Effects ◽  
Viral Gastroenteritis ◽  
List Type ◽  
Hyperinsulinaemic Hypoglycaemia ◽  
Glucose Levels ◽  
Capillary Glucose ◽  
Clinically Significant ◽  
Learning Points

Summary Hyperinsulinaemic hypoglycaemia (HH), which causes persistent neonatal hypoglycaemia, can result in neurological damage and it’s management is challenging. Diazoxide is the first-line treatment, albeit not all patients will fully respond to it, as episodes of hypoglycaemia may persist and it entails unpleasant adverse effects. Sirolimus, an mTOR inhibitor, has reportedly been successful in treating children with severe diffuse HH, thus obviating the need for pancreatectomy. We report a girl with HH, with a novel heterozygous ABCC8 gene missense mutation (c.4154A>T/ p.Lys1385Thr), who was initially responsive to diazoxide therapy. After 11 months of diazoxide treatment, she developed intermittent, unpredictable breakthrough episodes of hypoglycaemia, in addition to generalized hypertrichosis and weight gain from enforced feeding to avoid hypoglycaemia. Sirolimus, which was commenced at 15 months of age, gradually replaced diazoxide, with significant reduction and abolition of hypoglycaemia. The hypertrichosis resolved and there was less weight gain given the reduced need for enforced feeding. Sirolimus, which was administered over the next 15 months, was well tolerated with no significant side effects and was gradually weaned off. After stopping sirolimus, apart from hypoglycaemia developing during an episode of severe viral gastroenteritis, the capillary glucose concentrations were maintained >3.5 mmol/L, even after a 10 h fast. Sirolimus may have a role in the treatment of partially diazoxide-responsive forms of HH who experience breakthrough hypoglycaemia, but the long-term safety and efficacy of sirolimus are not established. Learning points: Conventional treatment of diffuse HH with diazoxide is not always effective in controlling hypoglycaemia and can be associated with unpleasant side effects. Sirolimus was successfully used to abolish recurrent hypoglycaemia in partially diazoxide-responsive HH, with resolution of unacceptable diazoxide-associated side effects. Sirolimus was well tolerated with no clinically significant side effects. Shortly after stopping sirolimus, the capillary glucose levels remained normoglycemic.

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