Browse

You are looking at 1 - 10 of 23 items for :

  • Country of Treatment x
  • Case Report Type x
  • Endocrine-related cancer x
  • Publication Details x
  • Unique/unexpected symptoms or presentations of a disease x
  • Clinical Overview x
Clear All
Rikako Nakajima Department of Endocrinology and Metabolism, Tsukuba University Hospital Mito Clinical Education and Training Center, Mito Kyodo General Hospital, Mito, Ibaraki, Japan

Search for other papers by Rikako Nakajima in
Google Scholar
PubMed
Close
,
Daisuke Sato Department of Endocrinology and Metabolism, Tsukuba University Hospital Mito Clinical Education and Training Center, Mito Kyodo General Hospital, Mito, Ibaraki, Japan

Search for other papers by Daisuke Sato in
Google Scholar
PubMed
Close
,
Ichirota Togashi Department of Endocrinology and Metabolism, Tsukuba University Hospital Mito Clinical Education and Training Center, Mito Kyodo General Hospital, Mito, Ibaraki, Japan

Search for other papers by Ichirota Togashi in
Google Scholar
PubMed
Close
,
Hiroto Idesawa Department of Endocrinology and Metabolism, Tsukuba University Hospital Mito Clinical Education and Training Center, Mito Kyodo General Hospital, Mito, Ibaraki, Japan

Search for other papers by Hiroto Idesawa in
Google Scholar
PubMed
Close
,
Jun Ito Department of Endocrinology and Metabolism, Tsukuba University Hospital Mito Clinical Education and Training Center, Mito Kyodo General Hospital, Mito, Ibaraki, Japan

Search for other papers by Jun Ito in
Google Scholar
PubMed
Close
,
Kei Ito Department of Endocrinology and Metabolism, Tsukuba University Hospital Mito Clinical Education and Training Center, Mito Kyodo General Hospital, Mito, Ibaraki, Japan

Search for other papers by Kei Ito in
Google Scholar
PubMed
Close
,
Masanao Fujii Department of Endocrinology and Metabolism, Tsukuba University Hospital Mito Clinical Education and Training Center, Mito Kyodo General Hospital, Mito, Ibaraki, Japan

Search for other papers by Masanao Fujii in
Google Scholar
PubMed
Close
, and
Hiroaki Yagyu Department of Endocrinology and Metabolism, Tsukuba University Hospital Mito Clinical Education and Training Center, Mito Kyodo General Hospital, Mito, Ibaraki, Japan

Search for other papers by Hiroaki Yagyu in
Google Scholar
PubMed
Close

Summary

An 89-year-old woman presented with a 6-year history of occasional episodes of impaired consciousness that were relieved by ingestion of a snack. Three months before presenting to our hospital, she had been hospitalized in a local hospital with subdural hematoma caused by a head contusion, where previously unrecognized hypoglycemia was discovered. Fasting plasma glucose concentration was 37 mg/dL, with a relatively high serum level of insulin (34.9 µU/mL). Computed tomography showed a 14 mm hyperenhancing tumor in the tail of the pancreas and she was referred to our hospital for further investigation. A prolonged fasting test revealed the plasma glucose concentration reduced to 43 mg/dL (2.4 mmol/L) at 8 h after the last meal. Serum insulin, proinsulin, and C-peptide concentrations were 21.1 µU/mL, 16.9 pmol/L, and 2.72 ng/mL, respectively. Subsequent intravenous administration of 1 mg of glucagon increased the plasma glucose concentration to 76 mg/dL (4.2 mmol/L). Moreover, the insulin-to-C-peptide molar ratio was 0.14. These data indicated the presence of insulinoma. Interestingly, serum anti-insulin antibodies were elevated (21.1 U/mL), although she had no history of taking exogenous insulin injection, alpha lipoic acid, or sulfhydryl group-containing agents. Human leukocyte antigen (HLA) typing revealed HLA-DRB1*0407 and HLA-DRB1*1405 alleles. Treatment with diazoxide prevented hypoglycemia, but was discontinued due to weight gain and leg edema. Elevated serum anti-insulin antibodies persisted almost 1 year after the diagnosis of insulinoma. We present a rare case of insulinoma concomitant with serum anti-insulin antibodies.

Learning points

  • Insulinoma presenting with concomitant anti-insulin antibodies appears rare.

  • Insulin/C-peptide molar ratio and serum insulin concentration are useful for differentiating insulinoma and autoimmune syndrome.

  • Flash glucose monitoring systems appear suitable for evaluating treatment outcomes.

Open access
Sarah N Parry Department of Endocrinology, Royal Prince Alfred Hospital, Sydney, Australia
Faculty of Medicine and Health, The University of Sydney, Sydney, Australia

Search for other papers by Sarah N Parry in
Google Scholar
PubMed
Close
and
Namson S Lau Metabolism & Obesity Services, Royal Prince Alfred Hospital, Sydney, Australia
Liverpool Diabetes Collaboration, Ingham Institute of Applied Medical Research, Sydney, Australia
South West Clinical School, University of New South Wales, Sydney, Australia

Search for other papers by Namson S Lau in
Google Scholar
PubMed
Close

Summary

Approximately 80% of adrenal incidentalomas are benign, and development into adrenal cortical cancer is extremely rare. This is a major reason behind clinical guidelines recommending surveillance of incidentalomas for a relatively short duration of up to 5 years. Surveillance of lesions less than 1 cm is not routinely recommended. A 70-year-old lady was diagnosed with a non-hyperfunctioning 8 mm right adrenal lesion. She underwent annual biochemical and radiological assessment for 5 years before surveillance was extended to 2-yearly intervals. The lesion was stable in size, and radiological characteristics were consistent with a benign adenoma. Seven years after the initial detection of the adrenal lesion, she developed acute abdominal pain. Imaging revealed a 7 cm right adrenal lesion, which was surgically resected and histologically confirmed to be adrenal cortical cancer. She died 1 year later. Clinical guidelines have moved towards a shortened duration of surveillance of incidentalomas. Even though malignant transformation is a rare event, it is possible that this will result in a delayed diagnosis of adrenal cortical cancer, a highly aggressive malignancy with a poor prognosis. To our knowledge, this is the first published case of an adrenal lesion of less than 1 cm developing into adrenal cortical cancer.

Learning points

  • Adrenal incidentalomas are increasingly common.

  • Clinical practice guidelines exist to aid in differentiating benign and malignant lesions and assessing functional status.

  • Transformation of adrenal incidentalomas to adrenal cortical carcinomas is a rare but recognised event.

Open access
Chi-Ta Hsieh Department of Internal Medicine, Tungs’ Taichung MetroHarbor Hospital, Taichung, Taiwan

Search for other papers by Chi-Ta Hsieh in
Google Scholar
PubMed
Close
,
Jui-Ting Yu Division of Hematology and Medical Oncology, Department of Internal Medicine, Tungs’ Taichung MetroHarbor Hospital, Taichung, Taiwan

Search for other papers by Jui-Ting Yu in
Google Scholar
PubMed
Close
,
Tang-Yi Tsao Department of Internal Medicine, Tungs’ Taichung MetroHarbor Hospital, Taichung, Taiwan
Department of Post-Baccalaureate Medicine, National Chung Hsing University, Taichung, Taiwan

Search for other papers by Tang-Yi Tsao in
Google Scholar
PubMed
Close
, and
Yao Hsien Tseng Department of Internal Medicine, Tungs’ Taichung MetroHarbor Hospital, Taichung, Taiwan
Division of Endocrinology and Metabolism, Department of Internal Medicine, Tungs' Taichung MetroHarbor Hospital, Taichung, Taiwan
Department of Post-Baccalaureate Medicine, National Chung Hsing University, Taichung, Taiwan

Search for other papers by Yao Hsien Tseng in
Google Scholar
PubMed
Close

Summary

A 69-year-old woman presented with weight loss, fever, dizziness, exertional dyspnea, and drenching night sweats. Imaging showed a thyroid goiter at the left lobe that measured 5.6 × 3.4 × 3.5 cm in size. On computed tomography, she was found to have large adrenal masses. Core needle biopsy of the left thyroid mass revealed the presence of a mucosa-associated lymphoid tissue extranodal marginal zone B cell lymphoma. Non-Hodgkin’s lymphomas (NHL) typically develop in lymph nodes or other lymphatic tissues. There have been cases where the thyroid has been affected, and the secondary involvement of the adrenal gland is common. In reported cases, 7–59% of patients with NHL exhibited symptoms of thyroid dysfunction. Our patient presented no symptoms of thyroid dysfunction or Hashimoto’s thyroiditis. The patient had bilateral adrenal lymphomas that led to adrenal insufficiency. Immunochemotherapy provided a good response in this case, as seen by the rapid improvement in thyroid and adrenal mass on follow-up PET/CT.

Learning points

  • Thyroid lymphoma requires a high index of suspicion for diagnosis in patients with a rapidly growing thyroid tumor, even in the absence of chronic inflammatory thyroid disease.

  • Depending on the extent of involvement, adrenal lymphoma may rapidly cause adrenal insufficiency.

  • In the setting of acute illness, appropriate levels of plasma cortisol are often unclear, necessitating early initiation of glucocorticoid therapy based on clinical suspicion, especially when features like bilateral adrenal masses and elevated ACTH levels are present.

  • Treatment modalities include chemotherapy and radiation therapy for localized lesions, together with hormone replacement for organ dysfunction.

  • The origin of the tumor influences the clinical outcome of patients with lymphoma simultaneously involving the thyroid and adrenal glands.

Open access
Mohammad Alali Kuwait Board of Internal Medicine, Kuwait City, Safat, Kuwait

Search for other papers by Mohammad Alali in
Google Scholar
PubMed
Close
,
Sulaiman Hajji Department of Internal Medicine, Adan Hospital, Kuwait City, Kuwait

Search for other papers by Sulaiman Hajji in
Google Scholar
PubMed
Close
, and
Khalid Aljenaee Department of Internal Medicine, Adan Hospital, Kuwait City, Kuwait

Search for other papers by Khalid Aljenaee in
Google Scholar
PubMed
Close

Summary

Endometrioid carcinomas of the ovary are a subtype of epithelial ovarian tumors, with sertoliform endometrioid carcinomas being a rare variant. We report a case of a previously healthy premenopausal woman presenting with androgenic symptoms in the form of hirsutism and male pattern alopecia. On further testing, she was found to have high levels of luteinizing hormone and total testosterone levels, and imaging revealed a large pelvic abdominal mass in the right ovary. She underwent total hysterectomy with bilateral salpingo-oophorectomy. Microscopy and histopathology confirmed the diagnosis of sertoliform endometrioid carcinoma. Her symptoms improved significantly on follow-up. Androgenic tumors might not be common in premenopausal women; however, it is important to maintain a high level of suspicion in patients presenting with virilizing symptoms especially of rapid progression.

Learning points

  • Our 47-year-old patient presented with virilizing symptoms that were rapidly progressing, which raises the suspicion of an underlying androgen secreting neoplasm.

  • Sertoliform endometrioid carcinoma (SEC) is an extremely rare variant of endometrioid carcinomas and tend to present at an earlier stage as compared to most endometrioid carcinomas of the ovary.

  • Recognition of SEC in virilizing patients is important as it is a well-differentiated, low-grade malignancy with a good prognosis when confined to the ovary.

Open access
Omayma Elshafie Department of Endocrinology, Sultan Qaboos Comprehensive Cancer Care and Research Centre, Muscat, Sultanate of Oman

Search for other papers by Omayma Elshafie in
Google Scholar
PubMed
Close
,
Samir Hussein Department of Radiology, Sultan Qaboos University Hospital, Muscat, Sultanate of Oman

Search for other papers by Samir Hussein in
Google Scholar
PubMed
Close
,
Moza Al Kalbani Department of Gynaecology, Sultan Qaboos Comprehensive Cancer Care and Research Centre, Muscat, Sultanate of Oman

Search for other papers by Moza Al Kalbani in
Google Scholar
PubMed
Close
,
Aisha Al Hamadani Department of Pathology

Search for other papers by Aisha Al Hamadani in
Google Scholar
PubMed
Close
,
Abir Bou Khalil Department of Endocrinology, Sultan Qaboos Comprehensive Cancer Care and Research Centre, Muscat, Sultanate of Oman

Search for other papers by Abir Bou Khalil in
Google Scholar
PubMed
Close
, and
Nicholas Woodhouse Department of Endocrinology, Sultan Qaboos University Hospital, Muscat, Sultanate of Oman

Search for other papers by Nicholas Woodhouse in
Google Scholar
PubMed
Close

Summary

A 33-year-old female presented in 2013 with left flank pain. Ultrasound and MRI pelvis showed a complex mass 9 × 7 cm arising from the left ovary suggestive of ovarian torsion. She underwent a laparoscopic cystectomy, but the patient was lost to follow-up. Three years later, she presented with abdominal distension. Ultrasound and CT scan revealed a solid left ovarian mass with ascites and multiple peritoneal metastasis. Investigations showed elevated CA 125, CA 19-9. Ovarian malignancy was suspected. She underwent total abdominal hysterectomy and bilateral salpingo-oophorectomy on November 2016. The histopathology confirmed a well-differentiated thyroid cancer of ovarian origin with features of a papillary follicular variant without evidence of ovarian cancer and the thyroglobulin (Tg) level was elevated, more than 400 consistent with the diagnosis of malignant struma ovarii. The follow-up post-surgery showed normalization of CA 125, CA 19-9 and Tg. The patient underwent total thyroidectomy on January 2017. The histology was benign excluding thyroid cancer metastases to the ovary. She was started on thyroxine suppression, following which she received two ablation doses 131iodine (131I) each 5.3 GBq. The Tg remains slightly elevated at less than 10. 131I WBS showed no residual neck uptake and no distant avid metastasis. She was planned for molecular analysis which may indicate disease severity. We describe a case of malignant struma ovarii with widespread metastatic dissemination and a good response to surgery and 131I treatment without recurrence after 5 years of follow-up. The Tg remains slightly elevated indicating minimal stable residual disease.

Learning points

  • Malignant struma ovarii is a rare disease; diagnosis is difficult and management is not well defined.

  • Presentation may mimic advanced carcinoma of the ovary.

  • Predominant sites of metastasis are adjacent pelvic structures.

  • Thyroidectomy and 131iodine therapy should be considered. The management should be similar to that of metastatic thyroid cancer.

Open access
Pranav Gupta Division of Endocrinology, Department of Pediatrics, Children’s Healthcare of Atlanta, Emory University School of Medicine, Atlanta, Georgia, USA

Search for other papers by Pranav Gupta in
Google Scholar
PubMed
Close
,
Karen Loechner Division of Endocrinology, Department of Pediatrics, Children’s Healthcare of Atlanta, Emory University School of Medicine, Atlanta, Georgia, USA
Division of Endocrinology, Department of Pediatrics, Connecticut Childrens Medical Center, Farmington, Connecticut, USA

Search for other papers by Karen Loechner in
Google Scholar
PubMed
Close
,
Briana C Patterson Division of Endocrinology, Department of Pediatrics, Children’s Healthcare of Atlanta, Emory University School of Medicine, Atlanta, Georgia, USA
Aflac Cancer and Blood Disorders Center of Children’s Healthcare of Atlanta, Atlanta, Georgia, USA

Search for other papers by Briana C Patterson in
Google Scholar
PubMed
Close
, and
Eric Felner Division of Endocrinology, Department of Pediatrics, Children’s Healthcare of Atlanta, Emory University School of Medicine, Atlanta, Georgia, USA

Search for other papers by Eric Felner in
Google Scholar
PubMed
Close

Summary

Insulinomas are a rare cause of persistent hypoglycemia in a previously healthy child. In addition to symptoms of hypoglycemia, individuals with insulinomas usually present with a history of incessant caloric intake and weight gain due to a constant need to counter hypoglycemia. In addition to an extensive review of the literature, we report the first case of an insulinoma coexisting with reduced appetite secondary to anorexia nervosa in an adolescent female.

Learning points

  • Eliciting a detailed family history is important in hypoglycemia cases.

  • Obtaining a thorough dietary intake, weight history, and menstrual cycles (in females) and considering a psychiatric consultation for an eating disorder when indicated.

  • Although rare in the pediatric population, multiple endocrine neoplasia type 1 syndrome should be considered in the evaluation of children and adolescents with hypoglycemia who also have a family history of pituitary, pancreatic, and/or parathyroid endocrinopathies.

Open access
Said Darawshi Department of Endocrinology, Rambam Health Care Campus, Haifa, Israel
The Faculty of Medicine, Technion, Haifa, Israel

Search for other papers by Said Darawshi in
Google Scholar
PubMed
Close
,
Mahmoud Darawshi Clalit Health Services, Northern District – Arrabah, Israel

Search for other papers by Mahmoud Darawshi in
Google Scholar
PubMed
Close
, and
Deeb Daoud Naccache Department of Endocrinology, Rambam Health Care Campus, Haifa, Israel
The Faculty of Medicine, Technion, Haifa, Israel

Search for other papers by Deeb Daoud Naccache in
Google Scholar
PubMed
Close

Severe hypocalcaemia in breast cancer with bone metastasis is a rare finding usually associated with an advanced stage of the disease. We report a case of a 45-year-old woman with a history of local ductal carcinoma in situ (DCIS) of the breast, who presented with muscle tremors and general weakness. Hypocalcaemia was evident, with a positive Chvostek sign and a serum calcium level of 5.9 mg/dL (1.47 mmol/L), phosphorus 5.9 mg/dL (normal range: 2.3–4.7 mg/dL) with normal levels of albumin, magnesium and parathyroid hormone. High oral doses of alpha calcitriol and calcium with i.v. infusion of high calcium doses were instituted, altogether sufficient to maintain only mild hypocalcaemia. A whole-body CT revealed bone lesions along the axial skeleton. A biopsy from a bone lesion revealed a metastasis of breast carcinoma. With this pathological finding, leuprolide (GNRH analogue) and chlorambucil (alkylating agent) were initiated, followed by prompt tapering of infused calcium down to full discontinuation. Serum calcium was kept stable close to the low normal range by high doses of oral alpha calcitriol and calcium. This course raises suspicion that breast metastases to the skeleton caused tumour-induced hypocalcaemia by a unique mechanism. We assume that hypocalcaemia in this case was promoted by a combination of hypoparathyroidism and bone metastasis.

Learning points

  • Severe hypocalcaemia can a presenting symptom for breast cancer relapse.

Open access
Nynne Emilie Hummelshøj Department of Hepatology and Gastroenterology, Aarhus University, Aarhus, Denmark

Search for other papers by Nynne Emilie Hummelshøj in
Google Scholar
PubMed
Close
,
Gitte Dam Department of Hepatology and Gastroenterology, Aarhus University, Aarhus, Denmark

Search for other papers by Gitte Dam in
Google Scholar
PubMed
Close
,
Lars Henning Pedersen Department of Obstetrics and Gynecology, Aarhus University, Aarhus, Denmark
Department of Clinical Medicine, Aarhus University, Aarhus, Denmark
Department of Biomedicine, Aarhus University, Aarhus, Denmark

Search for other papers by Lars Henning Pedersen in
Google Scholar
PubMed
Close
,
Astrid Hjelholt Department of Endocrinology and Internal Medicine, Aarhus University, Aarhus, Denmark
Department of Clinical Pharmacology, Aarhus University, Aarhus, Denmark

Search for other papers by Astrid Hjelholt in
Google Scholar
PubMed
Close
, and
Gerda Elisabeth Villadsen Department of Hepatology and Gastroenterology, Aarhus University, Aarhus, Denmark

Search for other papers by Gerda Elisabeth Villadsen in
Google Scholar
PubMed
Close

Summary

This rare case describes the course of a pregnancy in a patient with a disseminated small intestinal neuroendocrine tumor. The patient received treatment with first-generation somatostatin ligand receptor (SLR) every 4 weeks and had stable disease for several years before her pregnancy. First-generation SLR treatment was initially paused after detection of the pregnancy. During pregnancy, the patient experienced moderate gastro-intestinal discomfort and fatigue, which was considered predominantly pregnancy related. However, since symptoms could be linked to the patient’s cancer, treatment was resumed after the first trimester. Chromogranin-A measurements remained stable throughout pregnancy and was paralleled by the absence of diarrhea and only minor flushing. She gave birth by elective caesarean section in week 37 to a healthy baby. Subsequent follow up imaging immediately after and 10 months postpartum showed no disease progression. The safety profile of SLR treatment during pregnancy in the context of disseminated neuroendocrine tumors (NET) is discussed.

Learning points

  • Neuroendocrine neoplasms (NEN) are rare cancers often occurring in the gastro-intestinal tract or lungs.

  • Many patients with NEN live for several years with disseminated disease.

  • SLR treatment has been given to pregnant patients before; often patients with acromegaly. Pregnancies are reported uneventful.

  • This patient completed an uneventful pregnancy while receiving SLR treatment for disseminated neuroendocrine disease and gave birth to a healthy baby.

  • More research regarding long term effects and safety signals of SLR treatment during pregnancy are much needed.

Open access
Matthew Seymour Department of Endocrinology and Diabetes, Royal Brisbane and Women’s Hospital, Brisbane, Australia
Faculty of Medicine, The University of Queensland, Brisbane, Australia

Search for other papers by Matthew Seymour in
Google Scholar
PubMed
Close
,
Thomas Robertson Queensland Pathology, Royal Brisbane and Women’s Hospital, Brisbane, Australia
Faculty of Medicine, The University of Queensland, Brisbane, Australia

Search for other papers by Thomas Robertson in
Google Scholar
PubMed
Close
,
Jason Papacostas Department of Neurosurgery, The University of Queensland, Brisbane, Australia

Search for other papers by Jason Papacostas in
Google Scholar
PubMed
Close
,
Kirk Morris Department of Haematology, Royal Brisbane and Women’s Hospital, Brisbane, Australia

Search for other papers by Kirk Morris in
Google Scholar
PubMed
Close
,
Jennifer Gillespie Faculty of Medicine, The University of Queensland, Brisbane, Australia
Department of Radiology, Royal Brisbane and Women’s Hospital, Brisbane, Australia

Search for other papers by Jennifer Gillespie in
Google Scholar
PubMed
Close
,
Debra Norris QML Pathology, Brisbane, Australia

Search for other papers by Debra Norris in
Google Scholar
PubMed
Close
, and
Emma L Duncan Department of Endocrinology and Diabetes, Royal Brisbane and Women’s Hospital, Brisbane, Australia
Faculty of Medicine, The University of Queensland, Brisbane, Australia
Institute of Health and Biomedical Innovation, Faculty of Health, Queensland University of Technology, Translational Research Institute, Princess Alexandra Hospital, Brisbane Australia

Search for other papers by Emma L Duncan in
Google Scholar
PubMed
Close

Summary

A 34-year-old woman presented 18 months post-partum with blurred vision, polyuria, amenorrhoea, headache and general malaise. Comprehensive clinical examination showed left superior temporal visual loss only. Initial investigations revealed panhypopituitarism and MRI demonstrated a sellar mass involving the infundibulum and hypothalamus. Lymphocytic hypophysitis was suspected and high dose glucocorticoids were commenced along with desmopressin and thyroxine. However, her vision rapidly deteriorated. At surgical biopsy, an irresectable grey amorphous mass involving the optic chiasm was identified. Histopathology was initially reported as granulomatous hypophysitis. Despite the ongoing treatment with glucocorticoids, her vision worsened to light detection only. Histopathological review revised the diagnosis to partially treated lymphoma. A PET scan demonstrated avid uptake in the pituitary gland in addition to splenic involvement, lymphadenopathy above and below the diaphragm, and a bone lesion. Excisional node biopsy of an impalpable infraclavicular lymph node confirmed nodular lymphocyte-predominant Hodgkin lymphoma. Hyper-CVAD chemotherapy was commenced, along with rituximab; fluid-balance management during chemotherapy (with its requisite large fluid volumes) was extremely complex given her diabetes insipidus. The patient is now in clinical remission. Panhypopituitarism persists; however, her vision has recovered sufficiently for reading large print and driving. To the best of our knowledge, this is the first reported case of Hodgkin lymphoma presenting initially as hypopituitarism.

Learning points

  • Lymphoma involving the pituitary is exceedingly rare and, to the best of our knowledge, this is the first reported case of nodular lymphocyte-predominant Hodgkin lymphoma presenting as hypopituitarism.

  • There are myriad causes of a sellar mass and this case highlights the importance of reconsidering the diagnosis when patients fail to respond as expected to appropriate therapeutic intervention.

  • This case highlights the difficulties associated with managing panhypopituitary patients receiving chemotherapy, particularly when this involves large volumes of i.v. hydration fluid.

Open access
Kieran Palmer King’s College Hospital National Health Service Foundation Trust, London, UK

Search for other papers by Kieran Palmer in
Google Scholar
PubMed
Close
,
Scott Weerasuriya King’s College Hospital National Health Service Foundation Trust, London, UK

Search for other papers by Scott Weerasuriya in
Google Scholar
PubMed
Close
,
Benjamin Whitelaw King’s College Hospital National Health Service Foundation Trust, London, UK

Search for other papers by Benjamin Whitelaw in
Google Scholar
PubMed
Close
, and
Rajaventhan Srirajaskanthan King’s College Hospital National Health Service Foundation Trust, London, UK

Search for other papers by Rajaventhan Srirajaskanthan in
Google Scholar
PubMed
Close

Summary

We report a rare case of posterior reversible encephalopathy syndrome (PRES), precipitated by ectopic Cushing’s syndrome, in a patient with a metastatic pancreatic neuroendocrine tumour. A 55-year-old female presented as a hypertensive emergency with seizures and severe biochemical disturbance, including alkalosis, hypokalaemia and hyperglycaemia. MRI showed vasogenic oedema in the parieto-occipital region, consistent with a diagnosis of PRES. She had a significantly raised serum cortisol (>6000 nmol/L) which did not suppress with dexamethasone. Plasma adrenocorticotropic hormone (ACTH) concentrations were neither suppressed nor raised but were consistently within the normal reference range. The unexpected finding of a normal ACTH may be explained by either tumour secretion of unmeasured ACTH-related peptides, immunoassay antibody interference or episodic ACTH secretion. PRES is usually reversible with prompt and appropriate treatment. Hypercortisolism associated PRES is rare and may be associated with a worse outcome.

Learning points

  • PRES secondary to ectopic Cushing’s syndrome is very rare.

  • PRES in this context may indicate a worse prognosis.

  • In ectopic Cushing’s syndrome, if the serum ACTH level is normal, consider testing for ACTH-related peptides or interfering antibodies.

  • Further research is required to establish the best treatment approach and to improve patients’ outcomes.

Open access