Programme & Abstracts - Society for Endocrinology

in association with the South East Region Endocrine Club
Friday 10 December 2010
Lansdowne Place Hotel, Brighton, UK
Programme
& Abstracts
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Society for Endocrinology Regional Clinical Cases Meeting
In association with the South East Region Endocrine Club
Friday 10 December 2010
Lansdowne Place Hotel, Brighton, UK
Programme and Abstracts
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Society for Endocrinology Regional Clinical Cases Meeting
in association with the South East Region Endocrine Club
Friday 10 December 2010
Lansdowne Place Hotel, Brighton
The meeting will take place in the Ballroom Suite and all refreshments will be served in the
exhibition area in the Reception Room. All posters will be displayed in the Ballroom Suite.
09:30 Registration
09:55 Chairs Welcome and Introduction
Dr Simon Alywin (London) & Dr Anna Crown (Brighton)
10:00 Lecture 1: What does the future hold for the management of Addison’s
disease?
Dr John Newell-Price (Sheffield)
10:30 Lecture 2: Current issues and future possibilities in the management of
hyper-thyroidism
Professor Colin Dayan (Cardiff)
11:00 Coffee and poster viewing
Clinical Cases I: Pituitary, thyroid and adrenal
Chairs: Professor Colin Dayan (Cardiff) and Dr John Newell-Price (Sheffield)
11:15 Hypophysitis: The diagnostic challenges of an inflammatory pituitary mass
Liu, Y. Boleat,E. Agustsson, T. Kariyawasam, D.
Department of Endocrinology, Guy’s and St Thomas’ Hospital NHS Trust,
London
11:30 Pituitary mycosis complicating a Cushing’s macroadenoma
Edirisinghe, V. 1 Goulden, P. 3 Powrie, J. 2 Kumar, J. 3
1
Watford General Hospital, Vicarage Road, Watford
Guy’s and St Thomas’ Hospital NHS Trust, London
3
Maidstone Hospital, Maidstone, Kent
11:45 Temozolomide treatment of a resistant prolactinoma
Whitelaw, BC. Dworakowska, D. Hampton, T. Thomas, N. Aylwin, SJB.
King’s College Hospital, London
12:00 Straightforward thyrotoxicosis?
Maitland, R. Miell, J.
Department of Diabetes & Endocrinology, University Hospital Lewisham,
London
12:15 21 hydroxylase antibodies in a patient with autoimmune adrenal failure
presenting as mineralocorticoid deficiency
Taylor, DR 1. Aylwin, SJB. 2 Davies, ET. 3 Chan, AOK 4. Zacharia, S. 5
Taylor, NF. 1
Departments of 1 Clinical Biochemistry, 2 Medicine and 3 Immunology, King’s
College Hospital, London, UK. 4 Department of Pathology, Queen Elizabeth
Hospital, Kowloon, Hong Kong. 5 Department of Endocrinology, Princess
Royal University Hospital, Orpington

12:30 Lunch and poster viewing
Reception Room
13:30 Debate: “The NHS can no longer afford to pay for endocrine ‘lifestyle’
conditions”
Introduction: Dr Anna Crown (Brighton)
For: Dr John Quin (Consultant Endocrinologist, Brighton)
Against: Dr Tom Scanlon (Director of Public Health Brighton & Hove)
Clinical cases II: Endocrine neoplasia & neuroendocrine tumours
Chairs: Professor Colin Dayan (Cardiff) and Dr John Newell-Price (Sheffield)
14:15 A case of malignant bladder phaeochromocytoma
Grant ,P. Carroll, P. Agustsson, T.
St. Thomas’ Hospital, London
14:30 RET C620R, the janus mutation
Roplekar, R. 1 , Carroll, PV. 2
1 King’s College London, Guy’s and St Thomas’ Hospital NHS Trust, London
2 Guy’s and St Thomas’ Hospital NHS Trust, London
14:45 Symptomatic hypoglycaemia due to high IGF-2 levels in a patient with
adrenocortical carcinoma
Doumouchtsis,K. Mishra,N. Schulte,K-M. Aylwin, SJB.
King’s College Hospital, London
15:00 A clinically silent invasive adrenocortical tumour: charting steroid excretion
before tumour removal, and after mitotane therapy and repeat surgery
Taylor, N F. 1 , Ghataore, L. 1 , Schulte, K-M . 2 Aylwin, SJB. 3
King’s College Hospital, London
15:15 Pancreatic neuroendocrine tumor secreting glucagon and GLP-1 causing
diabetes and subsequent hyperinsulinaemic hypoglycaemia
Roberts, RE._ Zhao, M. 2 Whitelaw, BC. 3 Ramage, J. 4 Rela, M. 4
Diaz-Cano, SJ. 5 , Quaglia, A. 4 Huang, GC. 2 Aylwin, SJB. 3
1, 3-5 King’s College Hospital London School of Medicine, London
2 King's College London School of Medicine, London
15:30 Tea and poster viewing
16:00 Lecture 3: ‘Pseudo-endocrinology’: unorthodox endocrine remedies
Dr John Miell (London)
Introduction by: Dr Simon Alywin (London)
16:30 Lecture 4: Clinical vignettes from the endocrine cancer to MDMs
Dr Pauline Kane (London) and Dr Simon Aylwin (London)
Introduction by: Dr Anna Crown (Brighton)
17:00 Evening reception and awards ceremony
Reception Room
Two oral presentations will be awarded prizes: £250 for the first prize and
£150 for the runner up prize. The two overall highest scoring posters will also
receive a prize of £100 each.

Hypophysitis: The diagnostic challenges of an inflammatory pituitary mass
Liu,Y. Boleat,E. Agustsson, T. Kariyawasam, D.
Department of Endocrinology, Guy’s and St. Thomas Hospital NHS Foundation Trust
London, Westminster Bridge Road, London SE1 7EH
Case History: A 24 year old student from Pakistan presented to A&E with 6 days of severe headache
and vomiting. He complained of chronic headaches and short-term memory loss 6 months prior to
presentation. There was no visual disturbance or neck stiffness. He denied a history of tuberculosis
(with a negative testing prior to entry to UK), and had no respiratory symptoms, fever or night-sweats.
There was weight loss of approximately 17kg in the preceding months but no symptoms of polyuria or
polydipsia and no nocturia. The past medical history consisted only of congenital nystagmus.
Neurological examination was normal. CT brain raised the concern of a circle of Willis aneurysm;
however a subsequent MRA revealed the abnormality as an enlarged pituitary with compression of
the optic chiasm. On further questioning, he reported a six months history of nausea, vomiting and
reduced energy with lowered libido but intact erectile function.
Investigations and methods: Pituitary screen revealed a TSH of 0.50mIU/L, FT4 6.2pmol/L, FT3
2.5pmol/L, morning cortisol of 60nmol/l, ACTH 20ng/L, IGF-1 17.4nmol/L, GH 1.7ug/L, prolactin 223
mU/L, testosterone 2nmol/l, LH 2.8IU/L, FSH 5.4IU/L. Plasma osmolality was 286 mOsmol and
sodium was 140 mmol/L. Pituitary MRI showed a bulky diffusely avidly enhancing pituitary with
convex upper border and a thickened, enhancing stalk. A right inferotemporal defect was found on
formal visual field testing. Chest X-ray and CT of chest, abdomen and pelvis were normal. He was
considered to have partial hypopituitarism with TSH, gonadotrophin and possibly ACTH deficiency.
Mantoux test was positive at 20mms. ACE levels were normal in serum. CRP was

Pituitary mycosis complicating a Cushing’s macroadenoma
Edirisinghe, V. 1 , Goulden, P. 3 Powrie, J. 2 Kumar, J. 3
1
Watford General Hospital, Vicarage Road, Watford
2
Guy’s and St Thomas’ NHS Foundation Trust, London
3
Maidstone Hospital, Hermitage lane, Maidstone
Case History:
A 59 year old gentleman with a longstanding history of poorly controlled type 2 diabetes mellitus,
obesity, hypertension, obstructive sleep apnoea (OSA), depression and type 2 respiratory failure
was seen in the diabetes review clinic and noted to have truncal obesity, moon facies and wasting of
the proximal muscles.
Investigations, Results and Treatment:
He was investigated for Cushing’s syndrome as follows: Urinary free cortisol was 782 nmol/24 hours
(NR < 200). Midnight sleeping cortisol values were 595 and 532 nmol/l on consecutive days.
Cortisol value was 580 nmol/l with an ACTH of 130 nmol/l after a low dose dexamethasone
suppression test. Cortisol suppressed to159 nmol/l after a high dose dexamethasone suppression
test.
Magnetic resonance imaging (MRI) showed a pituitary macroadenoma occupying and expanding the
pituitary fossa and eroding through the floor of the sella to occupy most of the sphenoid sinus. CT of
the adrenals and CXR were normal. IPSS was not carried out as the pituitary lesion merited removal
in its own right and there was no overt evidence of ectopic ACTH production.
Trans-sphenoidal hypophysectomy was carried out at the National Hospital. Cortisol post-operatively
and pre-hydrocortisone replacement was 60-142 nmol/l. Regular hydrocortisone was prescribed
(20mg am/10mg pm).
Histology of the specimen found strong ACTH staining, with Ki67 index

Temozolomide treatment of a resistant prolactinoma
Whitelaw, BC. Dworakowska, D. Hampton, T. Thomas, N. Aylwin, SJB.
King’s College Hospital, Denmark Hill, London
Case History
A 16 year old man presented in 2007 with bitemporal hemianopia and left sided headaches. MRI
scan showed a sella, suprasellar and left parasellar tumour. Serum prolactin levels were 129,000
mU/l. He was commenced on cabergoline and the dose was titrated up to 1mg daily. Serum prolactin
fell to a nadir of 40,000 mU/l and there was an improvement in visual fields. However his headaches
persisted and neuroimaging did not show any significant shrinkage of the tumour.
Trans-sphenoidal surgery was performed in December 2007. Histology demonstrated a pituitary
adenoma with strongly positive immunohistochemical staining for prolactin. Ki67 labelling index was
4%. MGMT immunostaining was

Straightforward Thyrotoxicosis?
Maitland, R. Miell, J.
University Hospital Lewisham, London
Hyperthyroidism affects a significant proportion of the general population with prevalence of 0.5-3%.
The severity of the clinical presentation often correlates with the degree of biochemical thyroid
dysfunction. The most severe forms such as thyroid storm are rare affecting 1% of all patients
however associated metabolic abnormalities occur much more frequently.
A 49 year old Jamaican with no previous medical history presented to A&E with three week history of
vomiting and progressive dysphagia with intermittent regurgitation. In addition she had developed
profuse diarrhoea and excessive sweating. Initial examination was normal and ECG confirmed atrial
fluter with 2:1block with a ventricular rate of 150 bpm. Admission bloods revealed a mild neutrophilia
and she was commenced on antibiotics for presumed chest sepsis after chest X-ray suggested a
possible ring enhancing lesion.
Over the next few days her condition deteriorated rapidly with the development of bulbar syndrome
affecting cranial nerves VII, IX, X and XI. Swallow became unsafe and she developed global
weakness affecting all limbs with no localizing signs or fatiguability. CT chest excluded any lung
pathology but revealed 3.5x2.3cm soft tissue mass in the anterior mediastinum suggestive of thymic
tissue. In combination with her deteriorating clinical picture a diagnosis of Myasthenia Gravis (MG) or
thyrotoxicosis was raised.
Urgent TFTs confirmed thyrotoxicosis (TSH46 pmol/L
)complicated by severe hypercalcaemia, hypomagnamesia, hyperphosphatemia and abnormal liver
function. (cCa 2.93mmol/L, Mg 0.65mmol/L, phosphate 1.71mmol/L, bilirubin 4umol/L, ALT 101 iu/L,
AST 116 iu/L, alkaline phosphatase 103 iuL).
Initiation of carbimazole 40mg OD led to a slow but gradual improvement in her global myopathy and
bulbar symptoms over a period of four weeks. Following regular speech therapy nasogastric feeding
was weaned down and dysarthria improved dramatically.
Hypercalcaemia persisted despite adequate fluid hydration and therefore exclusion of neoplasm was
sought following the detection of a breast lump. Mammography and ultrasound confirmed a benign
lesion and myeloma screen was negative. Further investigations confirmed a low serum vitamin D
(25 OH-cholecalciferol) 21nmol/L, low PTH 9ng/L (10-65) and low urinary calcium excretion (24hour
urinary Ca 2+ 8.3mmol/d [2.5-7.5]). Anti thyroid peroxidase, acetylcholine receptor and Anti-Musk
antibodies were all negative. Upon discharge both corrected calcium and liver enzymes normalised.
(cCa 2+ 2.49mmol/L, ALT 40iu/L, ALP 121iu/L, bilirubin 5umol/L).
We present this case to highlight the severe metabolic and clinical consequences of thyrotoxicosis
which are detailed in text but seen less frequently in clinical practice. Such abnormalities often lead to
other diagnoses being sought.
Myopathy can present in 60-80% cases of untreated hyperthyroidism however is seldom the
presenting complaint. Bulbar muscle dysfunction is rare and should raise the possibility of MG.
Hypercalcaemia occurs in up to 8% of patients secondary to accelerated bone remodeling. Thyroid
hormone directly stimulates bone resorption via osteoclastic activity however TSH may also have
direct effect on bone resorption mediated via the TSH receptor found on osteoblast and osteoclast
precursors. The hypercalcaemia suppresses PTH secretion, resulting in the protective mechanism of
hypercalciuria. Low serum PTH concentrations reduce the conversion of 25-hydroxyvitamin D to
calcitriol which is compounded by an overall increase in calcitriol metabolism due to the hyperthyroid
state. Over time this translates into reduced bone mineral density and increased fracture rates
however in the acute setting sequelae of hypercalcaemia remain a concern.

21 hydroxylase antibodies in a patient with autoimmune adrenal failure presenting as
mineralocorticoid deficiency
Taylor, DR 1. Aylwin, SJB. 2 Davies, ET. 3 Chan, AOK 4. Zacharia, S. 5 Taylor, NF. 1
Departments of 1 Clinical Biochemistry, 2 Medicine and 3 Immunology, King’s College Hospital, London,
UK. 4 Department of Pathology, Queen Elizabeth Hospital, Kowloon, Hong Kong. 5 Department of
Endocrinology, Princess Royal University Hospital, Orpington, UK
Case history
A 63 year old woman was referred in 2010 to King’s with persistent hyponatraemia and
hyperkalaemia. Her previous medical history was unremarkable until 2006 when weight loss, thirst
and polyuria led to a diagnosis of type 2 diabetes mellitus. She was also hypothyroid and had a longstanding
candidiasis infection. Clinical examination at King’s was normal with the exception of a
widespread tan (no buccal pigmentation) and low blood pressure. She reported a long-standing love
of salty food, dizziness first thing in the morning and a reduction in pubic and axillary hair. Synacthen
tests on two occasions had been normal.
Investigations and method
Measurement of blood ACTH, renin, aldosterone and urine steroid profile. Short synacthen
stimulation test. Testing for gastric parietal cell, thyroid peroxidase, 17- and 21-hydroxylase
autoantibodies. Mutation analysis of AIRE1 and CYP21A2 genes.
Results and treatment
High renin and low aldosterone values alongside the electrolyte disturbances were consistent with
mineralocorticoid deficiency, while the repeat synacthen test showed no increment on a baseline
cortisol of 360 nmol/L, together suggesting early adrenal failure. This, alongside the other clinical
symptoms and positive results for all autoantibodies screened, was strongly suggestive of
autoimmune polyendocrine syndrome type 1 (APS1), but neither of the two mutations in AIRE1 that
are most common in British APS1 patients were found. Surprisingly, urinary steroid metabolites
characteristic of 21-hydroxylase deficiency were present. To explain this, two hypotheses were
explored; 1) that this was a consequence of 21-hydroxylase autoantibody inhibition of 21-
hydroxylase, a phenomenon reported in vitro but not so far in vivo or 2) that the patient had a
previously unrecognised 21-hydroxylase deficiency. Mutation analysis confirmed a c.293-13A>G
mutation in her CYP21A2 gene. The patient is now stabilised with fludrocortisone and hydrocortisone.
Conclusions and points for discussion
Our patient appears to have subclinical 21 hydroxylase deficiency which became evident with
destructive adrenalitis, and therefore presented with predominant mineralocorticoid deficiency. Direct
effects of adrenal autoantibodies on steroidogenesis are plausible but remain unproven.

Metastatic Paraganglioma of the Bladder
Grant, P. Carroll, P. Agustsson, T.
St. Thomas’ Hospital, Westminster Bridge Road, London
Case History
A 23 year old Turkish lady presented to A&E with frank haematuria. Hypertension had been noted at
the age of 17 years & she described headaches & dizziness on micturition but had not sought
medical advice. There was no family history of note.
Cystoscopy revealed a solid lesion in the bladder wall, but this was not felt to be typical of a bladder
cancer.
Investigations
CT scanning confirmed the presence of a vascular, enhancing, infiltrative bladder wall lesion which
was associated with pathological enlargement of a paravesical lymph node. Histology obtained
following a trans-urethral resection of bladder tumour revealed the presence of neuroendocrine
tumour typical of paraganglioma. Plasma metanephrines and urinary catecholamines were elevated
and alpha-adrenergic and beta blocker treatments were commenced.
Results and Treatment
18 FDGPET and MIBG scans confirmed the presence of tracer avidity consistent with paraganglioma
in the bladder but also showed an area of pathological uptake in the right femur. Technetium bone
scan revealed the presence of disease in the right proximal femoral shaft, suspicious for malignancy.
Advice was sought from the Royal National Orthopaedic Hospital for further characterisation of the
bone lesion with a view to intra-lesional bone biopsy and consideration of prophylactic bone pinning
to guard against a pathological fracture.
Conclusion / Discussion
The patient has undergone surgical intervention to the bladder with lymph node clearance to reduce
her tumour burden. Blood pressure is stable and she is symptomatically well post operatively. She is
awaiting genetic testing results to assess for the possibility of an SHD mutation. Follow up with the
oncologists will include consideration of systemic treatment eg. MIBG (+/- topotecan), Yttrium,
Lutetium.
In summary this patient had a bladder phaeochromocytoma which is a rare but well known clinical
entity but there are fewer than 30 cases of metastases in the literature. It accounts for only 0.06% of
all bladder neoplasm’s and

RET C620R, the Janus Mutation
Roplekar, R. 1 Carrol, PV.l 2
1
King’s College London, Guy’s and St Thomas’ Foundation Trust, Westminster Bridge, London.
2
Guy’s and St Thomas’ Foundation Trust, Westminster Bridge Road, London.
Case History
A newborn girl, the patient’s daughter, was identified as having Hirschsprung’s disease following
presentation with meconium ileus. Managed by total colostomy, she underwent genetic screening.
The cause for her Hirschsprung’s disease was identified as a mutation in the RET gene which is
recognised as resulting in both MEN2A and Hirschsprung’s disease.
The child’s father, a 25 year old male, was subsequently found to carry the mutation. Although
clinically asymptomatic, he too had a history of Hirschsprung’s disease, managed at the age of 1 year
by an ileostomy which was later closed. The patient’s father was also identified as carrying the
mutation and found to have bilateral pheochromocytomas and elevated calcitonin. A paternal uncle
had bilateral pheochromocytomas.
Investigations and Method
The patient was investigated as an outpatient and Thyroid ultrasound was performed.
Results and Treatment
In the patient, blood measurements showed an elevated serum calcitonin 78ng/L (NR

Symptomatic hypoglycaemia due to high IGF-2 levels in a patient with adrenocortical
carcinoma
Doumouchtsis, K. Mishra, N. Schulte, K M. Aylwin, S.
King’s College Hospital, Denmark Hill, London
Symptomatic hypoglycaemia can represent a serious complication of malignant neoplasia, usually by
inappropriate insulin secretion, which occurs in pancreatic islet cell tumours or unusually by insulinlike
growth factors (IGF-1 and IGF-2) secreted by tumour cells referred as non islet cell tumour
hypoglycaemia.
We present a case report on a 44 year old Nigerian woman, (living in the United Kingdom for the past
10 years) who was admitted to hospital following an episode of collapse. Paramedics reported low
blood sugar on CBG testing. (1.1mmol/L) The patient reported a 3 week history of facial swelling and
acneiform rash, some chest tightness and abdominal distension. She was overweight, though she
reported recent weight loss. Fasting glucose was 1.9mmol/Lwith undetectable insulin,low C-peptide
and IGF-1 and low IGF BP3 levels of 1.6mg/L (reference range 3.3-6.6). Her potassium levels were
between 3.4-3.8 mmol/L (reference range 3.5-5). Her IGF-2 levels were elevated at 75 nmol/L and
repeat IGF-1 levels were low 4.4 nmol/L (reference range 9 – 40) with a IGF-2/IGF-1 ratio of 17.0
(reference range

A clinically silent invasive adrenocortical tumour: charting steroid excretion before
tumour removal, and after mitotane therapy and repeat surgery
Taylor, N F. 1 Ghataore, L. 1 Schulte, K-M. 2 Aylwin, SJB. 3
Departments of 1 Clinical Biochemistry, 2 Surgery and 3 Medicine, King’s College Hospital,
Denmark Hill, London
Case history:
A 25 year old man with a 2 week history of left sided abdominal pain followed by cough and pleuritic
pain was found to have a firm left upper quadrant mass. A CT scan showed a 19cm left adrenal mass
and 3 lung lesions consistent with metastasis.
Investigations and method
Measurement of blood androstenedione, testosterone, progesterone and cortisol and of urine steroid
profile, dexamethasone suppression test.
Results and treatment
Elevated androstenedione (24.1nmol/L), progesterone (10nmol/L), normal 0900 cortisol, elevated
urine metabolites of DHEA, pregnenolone, progesterone, 17-hydroxypregnenolone and 11-
deoxycortisol were found. After dexamethasone, blood cortisol was 178nmol/L, while the elevated
steroids did not suppress. Taking the ratio of urine cortisol metabolites/tetrahydro-11-deoxycortisol
(FM/THS) as the best marker, this was 3.4 pre and 3.0 post dexamethasone (normal >200). After
radical adrenalectomy and retro-peritoneal en-bloc evisceration, FM/THS was 44.8. Mitotane
treatment resulted (as we have documented) in profound decrease of urinary 5_- and 20_- reduced
metabolites. This would not be predicted to alter production of THS, which is 5_-reduced and 20-oxo.
After removal of a right metastasis 5 months later (not active on histology), FM/THS had decreased
to 9.7. After removal of a left metastasis 6 weeks later (active on histology) FM/THS was 290. This
ratio remained normal for 18 months but has since (over the last year) shown a slow decrease to 46
currently. Symptoms of hypogonadism (a predicted consequence of diminished dihydrotestosterone
production by mitotane) have been effectively treated with nandrolone.
Conclusions and points for discussion
Urinary steroid profiling has proved highly effective in revealing steroid production by a clinically silent
adrenal carcinoma and in checking for recurrence. Effects of mitotane on steroid metabolism can be
taken into account.

Pancreatic neuroendocrine tumor secreting glucagon and GLP-1 causing diabetes
and subsequent hyperinsulinaemic hypoglycaemia.
Roberts, RE_. Zhao, M 2 . Whitelaw, BC 3 . Ramage, J 4 . Rela, M 4 . Diaz-Cano, SJ 6 . Quaglia,
A 4 . Huang, GC 2 . Aylwin, SJB 3 .
_ King’s College London School of Medicine, Henriette Raphael House, Guy’s Campus,
London, UK
2
Diabetes Research Group, King's College London School of Medicine, London, UK
3 Department of Endocrinology, King’s College Hospital, London, UK
4 Institute of Liver studies, King’s College Hospital, London, SE5 9RS, UK,
5
Department of Histopathology, King's College Hospital, London, SE5 9RS, UK
Case History
The association of diabetes and spontaneous hypoglycaemia is rare in the absence of insulin or oral
hypoglycaemic agents. We report a case of symptomatic fasting hypoglycaemia occurring in a 56-year old
Caucasian male with BMI 24 previously diagnosed with diabetes, which persisted despite withdrawal of antidiabetic
medication. Investigations confirmed hyperinsulinaemic hypoglycaeamia and a metastatic pancreatic
neuroendocrine tumour (pNET). Following surgical treatment the patient has had no further episodes of
hypoglycaemia and his blood glucose levels have returned to the diabetic range.
Investigations and method
Initial investigations consisted of ultrasound, CT, MRI, octreotide scan and liver biopsy. Fasting gut hormone
profile, supervised fast and 75 mg oral glucose tolerance test (GTT) were performed. Immunohistochemistry was
performed on surgical specimens. In order to determine the potential effect of tumour cells on human islet cells
ex vivo and in vivo work was conducted. Briefly, 3 groups of cells were cultured; islet cells alone, tumour cells
alone or islet and tumour cells. Semi-quantitative PCR was performed on each group of cells to determine
hormone mRNA expression. 12 male SCID mice were selected as recipients for cultured islet cells (n=4), tumour
cells (n=4) or islet and tumour cells (n=4). Intra-peritoneal GTT was conducted on the mice. On post mortem
analysis at 12 days immunohistochemistry was performed on the mice’s native pancreata.
Results
Imaging revealed multiple liver lesions, left para-aortic lymphadenopathy and a 3 cm pancreatic lesion. Liver
biopsy stained positively for chromogranin, synaptophysin and cytokeratin. Fasting gut hormone profile
demonstrated increased secretion of glucagon, gastrin, and pancreatic polypeptide with raised chromogranin A.
The supervised fast confirmed hyperinsulinaemic hypoglycaemia (glucose, insulin and C-peptide 1.6 mmol/L,
97.3 mU/l and 1800 pmol/l, respectively). Histology from liver metastases stained positively for glucagon,
somatostatin and gastrin but not insulin. Pancreatic islet cell hyperplasia of _-cells was noted. Measurement of
glucagon-like peptide 1 (GLP-1) during an oral GTT revealed a very high peak at 30 minutes (321.37 pmol/l) and
staining of liver metastases for GLP-1 was positive. Ex vivo studies demonstrated significantly reduced insulin
and C-peptide secretion by islet cells when co-cultured with tumour cells compared to islet cells or tumour cells
cultured alone. In vivo studies demonstrated that mice transplanted with tumour cells had an exaggerated
postprandial glucose concentration and impaired glucose tolerance compared to mice transplanted with islet
cells alone. Immunohistochemistry revealed reduced insulin staining in pancreatic islet cells from the mice
transplanted with islet and tumour cells compared to mice transplanted with islet cells alone. Mice transplanted
with tumour cells or islet and tumour cells demonstrated stronger GLP-1 and glucagon expression in pancreatic
islet cells compared to mice transplanted with islets alone.
Treatment
Initially the patient’s endocrine symptoms were controlled with cytoreductive surgery consisting of a distal
pancreatectomy, splenectomy and right hepatectomy. The patient since had a second partial hepatectomy to
remove metastatic lesions. The patient is currently being treated with lutetium octreotate for further liver
metastases.
Conclusions and points for discussion
The results support a conclusion that the patient’s original diabetes was caused by pancreatic NET glucagon
secretion and the following hyperinsulinaemic hypoglycaemia was caused by pancreatic NET GLP-1 secretion
and subsequent islet cell hyperplasia. Ex vivo and in vivo work support the diabetogenic effect of the tumour
cells on islet cells by paracrine and endocrine effect, respectively. A review of publications in Medline, including a
reference search of retrieved articles, identified 37 cases describing hypoglycaemia or insulinoma or islet cell
hyperplasia in patients previously known to have diabetes in 35 publications. We provide the first description of
combined glucagon and GLP-1 secretion from a metastatic pNET causing hyper- and later hypo- glycaemia.
Examination of the literature suggests that this may have occurred previously. In patients with metastatic NET
and hypoglycaemia, GLP-1 secretion should be actively sought.

POSTER PRESENTATIONS
Poster presentations will be chaired and judged by:
Dr John Quin (Brighton) and Dr J Miell (London).
P1. Two cases of Conn’s syndrome presenting following renal transplantation
Kumar, A. Hubbard, J. Moonim, M. Steddon, S. Goldsmith, D.
Guy’s and St. Thomas’ Hospital NHS Trust, Renal and Transplantation Department,
London
P2. Acute hyponatremia - an uncommon complication of ACE inhibitors
Abbas, N. 1 Macleod, C 2 . Mcarthur, Y 3 . Flynn, M 4 .
1 ST3 Diabetes & Endocrinology. 2 F1, 3 GPST2, 4 Consultant Endocrinologist
Kent & Canterbury Hospital
P3. Non-functioning pituitary macroadenoma presenting with hyponatremia
Bansiya, V. Banerjee, R.
Dept of Diabetes and Endocrinology, Luton and Dunstable Hospital, Luton
P4. Interpreting inconsistent investigative findings in Gushing’s syndrome
Agustsson, T 1 . Carroll, P 2 . McGowan, B 1 .
1 Specialist Registrar in Diabetes and Endocrinology, Guy’s and St Thomas’ Hospital NHS
Trust, London
2 Consultant Endocrinologist, Guy’s and St Thomas’ Hospital NHS Trust, London
P5. Pure gonadal dysgenesis and disordered sexual development
Grant, P 1 . Dashora, U 2 .
1
St. Thomas’ Hospital NHS Trust, London
2 Conquest Hospital, Hastings, East Sussex
P6. Severe hypophysitis of unknown cause complicating thyroiditis
Patel, K 1 . Goulden, P 2 . Carroll, P 3 . Kumar, J 2 .
1 F1, Lewisham hospital
2 Consultant Endocrinologist, Maidstone Hospital, Maidstone
3 Consultant Endocrinologist, Guy’s and St. Thomas’ Hospital, London
P7. Pre-conception dilemma in a woman with a pituitary macroadenoma
Lewis, D H. McGowan, B. Powrie, J K.
Guy’s and St Thomas’ NHS Foundation Trust, Diabetes and Endocrine Unit, London
P8. A case of carcinoid syndrome in a post-menopausal woman
Seetho, I W. 1 Jones, G 2 . Idris, I 3 . Fernando, D J S 3 . Thomson, G A 3 .
1 Nottingham University Hospitals NHS Trust, Nottingham
2 Chesterfield Royal Hospital NHS Foundation Trust, Chesterfield
3 Sherwood Forest Hospitals NHS Foundation Trust, Sutton-in-Ashfield
P9. First case report of testosterone assay-interference in a female taking Lepidium
meyenii (Maca)
Srikugan, L 1 . Sankaralingam, A 2 . McGowan, B 1 .
1 Department of Diabetes & Endocrinology, Guy’s and St. Thomas’ Hospital NHS
Trust, London
2 Department of Chemical Pathology, Guy’s and St. Thomas’ Hospital NHS Trust,
London

P10. A case of Graves’ disease unmasked by treatment of low grade Cushing’s disease
Srikugan, L. McGowan, L. Powrie, J.
Department of Diabetes & Endocrinology, Guy’s and St. Thomas’ Hospital NHS
Trust, London
P11. Pituitary incidentaloma revealed by 18 F-FDG PET-CT in a patient treated for B cell
lymphoma
Wale, A. Jacobsberg, S. Singh, N. Dizdarevic, S. Good, CD. Crown, A.
Brighton and Sussex University Hospitals, Royal Sussex County Hospital, Brighton
P12. Is Rituximab the final option in thyroid-eye disease?
Patel, T. Byard, S.
Brighton & Sussex Medical School
P13. A case of Graves’ disease presenting after commencing alemtuzumab treatment for
multiple sclerosis.
Mogford J, Sennik D and Zachariah S.
Department of Diabetes and Endocrinology, East Surrey Hospital, Redhill, Surrey

Contact details of endocrine patient groups:
Addison’s Disease Self-Help Group - http://www.addisons.org.uk/
ALD Life - http://www.aldlife.org/
Androgen Insensitivity Syndrome Support Group - http://www.aissg.org/
Anorchidism Support Group - http://www.asg4u.org/
Association for Multiple Endocrine Neoplasia Disorders - http://www.amend.org.uk/
British Thyroid Foundation - http://www.btf-thyroid.org/
Butterfly Thyroid Cancer Trust - http://www.butterfly.org.uk/
Child Growth Foundation - http://www.childgrowthfoundation.org/
Congenital Adrenal Hyperplasia Support Group - http://www.livingwithcah.com/
Hypoparathyroidism UK - http://www.hypoparathyroidism.org.uk/
Kallmann Syndrome Organisation – http://www.kallmanns.org/
Klinefelter Organisation - http://www.klinefelter.org.uk/
Klinefelter’s Syndrome Association UK - http://www.ksa-uk.co.uk/
National Association for Premenstrual Syndrome - http://www.pms.org.uk/
NET Patient Foundation – http://www.netpatientfoundation.com
Paget’s Association - http://www.paget.org.uk/
Pituitary Foundation - http://www.pituitary.org.uk/
Prader-Willi Syndrome Association - http://www.pwsa.co.uk/
The Gender Trust – http://www.gendertrust.org.uk
Thyroid Eye Disease Charitable Trust - http://www.tedct.co.uk/
Turner Syndrome Support Society - http://www.tss.org.uk/
Verity (PCOS) - http://www.verity-pcos.org.uk/

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