Orbital metastasis from neuroendocrine tumour: Case report and literature review

2021 ◽  
pp. 112067212110606
Author(s):  
Giorgio Albanese ◽  
Sreemathi Harieaswar ◽  
Raghavan Sampath

Orbital metastases from neuroendocrine tumour are rare entities. An incidental finding of orbital metastasis originating from neuroendocrine tumour is presented in the context of a comprehensive review of all case reports and series published to date. Demographics, clinical features, diagnostic work-up, treatment and prognostic data from the published literature are discussed. Ninety-five patients with orbital metastases from neuroendocrine tumours have been reported so far. Average age at presentation is 63.8 years (range 25–86), with no significant gender predominance. Gastrointestinal tract is the most common site of primary neuroendocrine tumours (62–85%). Typical presentation includes proptosis (80–85%) and diplopia (27–62%) in patients with known primary neuroendocrine tumour elsewhere. Histological subtype and presence of metastases seem to be important prognostic factors. Incidental finding of neuroendocrine tumour mestastases in asymptomatic patients with unknown primary disease is uncommon. In such cases, orbital biopsy and structural and functional imaging are essential to establish a diagnosis and stage the disease.

2006 ◽  
Vol 126 (5) ◽  
pp. 536-544 ◽  
Author(s):  
Orlando Guntinas-Lichius ◽  
J. Peter Klussmann ◽  
Stephen Dinh ◽  
Mai Dinh ◽  
Matthias Schmidt ◽  
...  

Author(s):  
Rachel L Boal ◽  
James Hughes ◽  
Debbie Matthews ◽  
Helen Johnstone ◽  
Christopher Boot ◽  
...  

Introduction Copeptin concentrations are a useful component of the diagnostic work-up of paediatric patients with polyuria and polydipsia but the value of measuring copeptin in patients with hyponatraemia is less clear. Case Reports We report 5 children with hyponatraemia in the context of different underlying pathologies. Copeptin concentrations were elevated in 4 cases (13.7, 14.4, 26.1, 233pmol/L; reference range 2.4 – 8.6pmol/L) suggesting that non-osmoregulated vasopressin release (syndrome of inappropriate antidiuretic diuretic hormone or SIADH) was the underlying mechanism for the low sodium levels. In one of the patients there was an underlying diagnosis of Schaaf-Yang syndrome (MAGEL 2 gene mutation) with a clinical picture suggestive of dysregulated vasopressin production with inappropriately high and then low copeptin release. In one hyponatraemic patient, low copeptin concentrations indicated that non-osmoregulated AVP release was not the cause of hyponatraemia and oliguria. Discussion Copeptin measurement did not influence management acutely but helped to clarify the mechanism leading to hyponatraemia when the result was available. Relatively high and low copeptin concentrations in association with hypo and hypernatraemia indicates dysregulated vasopressin production in Schaaf-Yang syndrome. Profound copeptin elevation may be a clue to underlying sepsis.


2020 ◽  
Vol 4 (Supplement_1) ◽  
Author(s):  
Ravind Singh Pandher ◽  
Emily Hibbert

Abstract Most phaeochromocytomas and pancreatic neuroendocrine tumours are sporadic in nature however the presence of multiple neuroendocrine tumours raises the suspicion of a hereditary endocrinopathy. Hibernomas, benign tumours that morphologically resemble brown fat, do not possess a clear aetiology and a link with other neuroendocrine tumours remains unclear. We report an unusual case of a concurrent sporadic phaeochromocytoma, pancreatic neuroendocrine tumour and a sacral hibernoma. A 61 year old female presented with a 3 month history of abdominal pain which led to the discovery of a lesion in her right adrenal gland and a soft tissue mass at the pancreatic tail on a CT Abdomen. The adrenal lesion was biochemically suggestive of a phaeochromocytoma (plasma normetanephrine 4930 pmol/L, plasma 3-methoxytyramine 580 pmol/L, urinary noradrenaline 5564 pmol/day, urinary dopamine 4720 nmol/day). A 68Ga-DOTATATE-PET-CT scan revealed DOTATATE avid lesions in the right adrenal gland, tail of pancreas and right sacral ala. Following preoperative medical therapy, the patient underwent a right adrenalectomy and a resection of the distal pancreatic lesion.Histopathology confirmed a phaeochromocytoma with no conscipicouous mitotic activity, and the pancreatic tail lesion was consistent with a well-differentiated neuroendocrine tumour (NET) (Ki-67 score <3%). Following normalisation of the serum catecholamines, a biopsy of the sacral lesion was undertaken, which returned positive for a hibernoma. Genetic testing revealed no identifiable genetic mutations.This case reports the synchronous presence of a phaeochromocytoma, pancreatic NET and sacral hibernoma with no identifiable genetic mutation. To date, the association between hibernomas and neuroendocrine tumours has not been fully established, but a few case reports suggest a possible association between MEN1 and hibernomas.


2007 ◽  
Vol 1 (4) ◽  
pp. 153-164
Author(s):  
Luca Masotti ◽  
Giancarlo Landini ◽  
Fabio Antonelli ◽  
Elio Venturini ◽  
Roberto Cappelli ◽  
...  

Despite modern algorithms have been proposed for diagnosis of pulmonary embolism (PE), it remains understimed and often missed in clinical practice, especially in elderly patients, resulting in high morbidity and mortality when early and correctly untreated. One of the main controversial issue is represented by the role and applicability of D-dimer in the diagnostic work up of geriatric patients. Most recent guidelines in young-adult patients suggest to perform D-dimer assay by ELISA or immunoturbidimetric methods only in non high pre-test clinical probability (PTP) patients; in these patients negative D-dimer can safely rule out the diagnosis of PE. This strategy is safe also in elderly patients; however the percentage of patients with non high PTP and negative D-dimer reduces progressively with age, making difficult its clinical applicability. The Authors, starting from two case reports, up date the diagnostic management of PE underling the limitations of D-dimer assay in elderly patients.


Cancers ◽  
2019 ◽  
Vol 11 (9) ◽  
pp. 1270 ◽  
Author(s):  
Antonella Argentiero ◽  
Antonio Giovanni Solimando ◽  
Oronzo Brunetti ◽  
Angela Calabrese ◽  
Francesco Pantano ◽  
...  

Skeletal metastases of unknown primary (SMUP) represent a clinical challenge in dealing with patients diagnosed with bone metastases. Management of these patients has improved significantly in the past few years. however, it is fraught with a lack of evidence. While some patients have achieved impressive gains, a more systematic and tailored treatment is required. Nevertheless, in real-life practice, the outlook at the beginning of treatment for SMUP is decidedly somber. An incomplete translational relevance of pathological and clinical data on the mortality and morbidity rate has had unsatisfactory consequences for SMUP patients and their physicians. We examined several approaches to confront the available evidence; three key points emerged. The characterization of the SMUP biological profile is essential to driving clinical decisions by integrating genetic and molecular profiles into a multi-step diagnostic work-up. Nonetheless, a pragmatic investigation plan and therapy of SMUP cannot follow a single template; it must be adapted to different pathophysiological dynamics and coordinated with efforts of a systematic algorithm and high-quality data derived from statistically powered clinical trials. The discussion in this review points out that greater efforts are required to face the unmet needs present in SMUP patients in oncology.


2013 ◽  
Vol 2013 ◽  
pp. 1-11 ◽  
Author(s):  
Ayodeji O. Omiyale ◽  
Anthony Kodzo-Grey Venyo

Context. Primary renal carcinoid tumours are rare. Their pathogenesis is unknown and the clinical presentation is similar to other renal tumours thus posing diagnostic dilemmas for clinicians.Objectives. To review the literature for case reports of primary renal carcinoids.Methods. Literature was extensively searched for case reports for primary renal carcinoids. Reports of metastatic carcinoids to the kidneys were excluded.Results. Approximately less than 90 cases of primary carcinoid tumours of the kidney have been reported in the literature. A total of 29 cases of primary renal carcinoids were reviewed. The mean age of presentation was 48 years (range 29–75) with both right kidney (48.3%) and left kidney (44.8%) being equally affected. 28.6% of the cases reviewed were diagnosed as an incidental finding. The mean followup time was 20 months with 73.1% of patients without evidence of disease after surgical treatment (radical or partial nephrectomy). Primary carcinoid tumours of the kidney are often well differentiated tumours. They are often misdiagnosed because of their rarity and similar presentation with other renal tumours.Conclusions. Primary carcinoid tumours of the kidney are rare tumours with an indolent course with frequent metastasis. Metastatic work up and followup is required in their management.


Author(s):  
L. Meijer ◽  
R. H. A. Verhoeven ◽  
I. H. J. T. de Hingh ◽  
A. J. van de Wouw ◽  
H. W. M. van Laarhoven ◽  
...  

Sign in / Sign up

Export Citation Format

Share Document