Browse

You are looking at 1 - 10 of 26 items for :

  • Clinical Overview x
  • Endocrine-related cancer x
Clear All
Evangelos Karvounis Department of Endocrine Surgery, ‘Euroclinic’ Hospital, Athens, Greece

Search for other papers by Evangelos Karvounis in
Google Scholar
PubMed
Close
,
Ioannis Zoupas Department of Endocrine Surgery, ‘Euroclinic’ Hospital, Athens, Greece

Search for other papers by Ioannis Zoupas in
Google Scholar
PubMed
Close
,
Dimitra Bantouna Private Practice, Patras, Greece

Search for other papers by Dimitra Bantouna in
Google Scholar
PubMed
Close
,
Rodis D Paparodis Private Practice, Patras, Greece
Center for Diabetes and Endocrine Research, University of Toledo College of Medicine and Life Sciences, Toledo, Ohio, USA

Search for other papers by Rodis D Paparodis in
Google Scholar
PubMed
Close
,
Roxani Efthymiadou PET-CT Department, Hygeia Hospital, Athens, Greece

Search for other papers by Roxani Efthymiadou in
Google Scholar
PubMed
Close
,
Christina Ioakimidou Department of Pathology

Search for other papers by Christina Ioakimidou in
Google Scholar
PubMed
Close
, and
Christos Panopoulos Department of Medical Oncology, ‘Euroclinic’ Hospital, Athens, Greece

Search for other papers by Christos Panopoulos in
Google Scholar
PubMed
Close

Summary

Large-cell neuroendocrine carcinoma (LCNEC) is a rare neuroendocrine prostatic malignancy. It usually arises after androgen deprivation therapy (ADT), while de novo cases are even more infrequent, with only six cases described. The patient was a 78-year-old man with no history of ADT who presented with cervical lymphadenopathy. Diagnostic approaches included PET/CT, MRI, CT scans, ultrasonography, biopsies, and cytological and immunohistochemical evaluations. Results showed a poorly differentiated carcinoma in the thyroid gland accompanied by cervical lymph node enlargement. Thyroid surgery revealed LCNEC metastasis to the thyroid gland. Additional metastases were identified in both the adrenal glands. Despite appropriate treatment, the patient died of the disease. De novo LCNEC of the prostate is a rare, highly aggressive tumor with a poor prognosis. It is resistant to most therapeutic agents, has a high metastatic potential, and is usually diagnosed at an advanced stage. Further studies are required to characterize this tumor.

Learning points

  • De novo LCNECs of the prostate gland can metastasize almost anywhere in the body, including the thyroid and adrenal glands.

  • LCNECs of the prostate are usually associated with androgen-depriving therapy, but de novo cases are also notable and should be accounted for.

  • Further studies are required to fully understand and treat LCNECs more effectively.

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
Hessa Boharoon Neuroendocrine Tumour Unit, ENETS Centre of Excellence, London, UK

Search for other papers by Hessa Boharoon in
Google Scholar
PubMed
Close
,
Shaunak Navalkissoor Department of Nuclear Medicine, ENETS Centre of Excellence, London, UK

Search for other papers by Shaunak Navalkissoor in
Google Scholar
PubMed
Close
,
Tu Vinh Luong Department of Pathology, Royal Free Hospital, London, UK

Search for other papers by Tu Vinh Luong in
Google Scholar
PubMed
Close
,
Martyn Caplin Neuroendocrine Tumour Unit, ENETS Centre of Excellence, London, UK

Search for other papers by Martyn Caplin in
Google Scholar
PubMed
Close
, and
Ashley Grossman Neuroendocrine Tumour Unit, ENETS Centre of Excellence, London, UK

Search for other papers by Ashley Grossman in
Google Scholar
PubMed
Close

Summary

Insulinomas are rare pancreatic neuroendocrine neoplasms (NENs) that are typically sporadic and solitary, with the majority being <2 cm in diameter at diagnosis. The median duration of symptoms before diagnosis is variable; however, this is usually in the region of 12–18 months. We report on an insulinoma diagnosed some 25 years following initial symptoms, having by that stage attained a diameter of 4 cm. We present a 50-year-old man who was reported with hypoglycaemic symptoms on his wedding 25 years prior to eventual confirmation of an insulinoma. He had since learned to live with the symptoms by eating frequently to manage his hypoglycaemia. However, over recent months, he reported a substantial deterioration in his symptoms, and indeed, had collapsed on two occasions. He had a fasting glucose of 2.9 mmol/L with grossly inappropriate elevated insulin and C-peptide levels. MRI demonstrated a 4.1 cm lesion at the body of pancreas and an indeterminate 9-mm liver lesion with a negative 68Gallium-DOTATATE PET scan. Accordingly, he was initiated on diazoxide and referred to the surgical team for distal pancreatectomy: histology confirmed a 4.4-cm well-differentiated pancreatic NEN of intermediate grade (NEN G2, Grade 2, 2017 World Health Organization (WHO) pancreatic-NEN classification), with positive immunohistochemistry for insulin. His hypoglycaemia episodes have ceased, and he remains under active surveillance. Our case demonstrates the possibility of dietary control of insulinoma-induced hypoglycaemia, and the likelihood that such a prolonged delay in diagnosis has led to the uncommonly large size of the apparently benign tumour which is usually ‘small and indolent’.

Learning points

  • Most patients with insulinomas have lesions that are 1–2 cm in size, with 96% being less than 3 cm.

  • The mean tumour size of insulinomas found in 3 of the largest reported series was 1.5 cm, with a range of 0.1–7.0 cm.

  • It is not uncommon for patients to have symptoms for several months to years before diagnosis; however, no reported cases had the symptoms such long for 25 years, and the large size of the tumour in this case may reflect the very long history.

Open access
Jenny S W Yun Department of Surgical Oncology, Peter MacCallum Cancer Centre, Melbourne, Victoria, Australia

Search for other papers by Jenny S W Yun in
Google Scholar
PubMed
Close
,
Chris McCormack Department of Surgical Oncology, Peter MacCallum Cancer Centre, Melbourne, Victoria, Australia

Search for other papers by Chris McCormack in
Google Scholar
PubMed
Close
,
Michelle Goh Department of Surgical Oncology, Peter MacCallum Cancer Centre, Melbourne, Victoria, Australia

Search for other papers by Michelle Goh in
Google Scholar
PubMed
Close
, and
Cherie Chiang Department of Internal Medicine, Peter MacCallum Cancer Centre, Melbourne, Victoria, Australia
University of Melbourne, Parkville, Victoria, Australia

Search for other papers by Cherie Chiang in
Google Scholar
PubMed
Close

Summary

Acanthosis nigricans (AN) is a common dermatosis associated with hyperinsulinemia and insulin resistance. However, AN has been rarely reported in patients with insulinoma, a state of persistent hyperinsulinemia. We present a case of metastatic insulinoma, in whom AN manifested after the first cycle of peptide receptor radionuclide therapy (PRRT). A 40-year-old man was diagnosed with metastatic insulinoma after 5 months of symptomatic hypoglycemia. Within 1 month post PRRT, the patient became euglycemic but developed a pigmented, pruritic rash which was confirmed on biopsy as AN. We discuss the rare manifestation of AN in subjects with insulinoma, the role of insulin in the pathogenesis of AN, malignant AN in non-insulin-secreting malignancies and association with other insulin-resistant endocrinopathies such as acromegaly.

Learning points

  • Acanthosis nigricans (AN) is a common dermatosis which is typically asymptomatic and associated with the hyperinsulinemic state.

  • Malignant AN can rapidly spread, cause pruritus and affect mucosa and the oral cavity.

  • AN is extremely rare in patients with insulinoma despite marked hyperinsulinemia.

  • Peptide receptor radionuclide therapy might have triggered TGF-α secretion in this subject which led to malignant AN.

  • Rapid spread or unusual distribution of pruritic AN warrants further investigation to exclude underlying malignancy.

Open access
N Ayub Department of Endocrine Oncology, University Medical Center Utrecht, Utrecht, The Netherlands

Search for other papers by N Ayub in
Google Scholar
PubMed
Close
,
A J A T Braat Department of Radiology and Nuclear Medicine, University Medical Center Utrecht, Utrecht, The Netherlands

Search for other papers by A J A T Braat in
Google Scholar
PubMed
Close
,
H J L M Timmers Departments of Endocrinology and Internal Medicine, Radboud University Medical Center, Nijmegen, The Netherlands

Search for other papers by H J L M Timmers in
Google Scholar
PubMed
Close
,
M G E H Lam Department of Radiology and Nuclear Medicine, University Medical Center Utrecht, Utrecht, The Netherlands

Search for other papers by M G E H Lam in
Google Scholar
PubMed
Close
, and
R S van Leeuwaarde Department of Endocrine Oncology, University Medical Center Utrecht, Utrecht, The Netherlands

Search for other papers by R S van Leeuwaarde in
Google Scholar
PubMed
Close

Summary

Von Hippel–Lindau’s disease (VHL) is a hereditary tumor syndrome characterized by its prototype lesions, hemangioblastomas, and renal cell carcinomas. Treatment for renal cell carcinomas can ultimately result in long-term dialysis. Pancreatic neuroendocrine tumors (pNET) can also occur in the course of the disease. Currently, peptide receptor radionuclide therapy (PRRT) is the standard treatment for progressive neuroendocrine tumors. However, little is known about treatment with PRRT in patients on dialysis, an infrequent presentation in patients with VHL. We present a 72-year-old man with VHL on hemodialysis and a progressive pNET. He received four cycles of PRRT with a reduced dose. Only mild thrombopenia was seen during treatments. The patient died 9 months after the last PRRT because of acute bleeding in a hemangioblastoma. Hemodialysis is not a limiting factor for PRRT treatment and it should be considered as it seems a safe short-term treatment option for this specific group.

Learning points

  • Von Hippel–Lindau disease (VHL) is a complex disease in which former interventions can limit optimal treatment for following VHL-related tumors later in life.

  • Metastasized pancreatic neuroendocrine tumors occur as part of VHL disease.

  • Peptide receptor radionuclide therapy seems a safe short-term treatment option in patients on hemodialysis.

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
Ziad Hussein Department of Endocrinology, University College London Hospital, London, UK
Department of Medicine, University College London, London, UK

Search for other papers by Ziad Hussein in
Google Scholar
PubMed
Close
,
Marta Korbonits William Harvey Research Institute, Barts and the London School of Medicine, Queen Mary University of London, London, UK

Search for other papers by Marta Korbonits in
Google Scholar
PubMed
Close
,
Stephanie E Baldeweg Department of Endocrinology, University College London Hospital, London, UK
Department of Medicine, University College London, London, UK

Search for other papers by Stephanie E Baldeweg in
Google Scholar
PubMed
Close
, and
Teng-Teng Chung Department of Endocrinology, University College London Hospital, London, UK

Search for other papers by Teng-Teng Chung in
Google Scholar
PubMed
Close

Summary

We observed a novel therapeutic response with cabergoline in a male patient with a dopamine-secreting head and neck paraganglioma (HNPGL), macroprolactinoma and germline succinate dehydrogenase C mutation (SDHC). The macroprolactinoma was treated with cabergoline which gave an excellent response. He was found to have raised plasma 3-methoxytyramine of 1014 pmol/L (NR: 0–180 pmol/L); but it was unclear if this was a drug-induced phenomenon from dopamine agonist (DA) therapy. Cabergoline was stopped for 4 weeks and the 3-methoxytyramine level increased significantly to 2185 pmol/L, suggesting a biochemical response of his HNPGL. Subsequently, Gallium-68 Dotatate PET and MRI (Gallium-68 Dotatate PET/MRI) demonstrated a second lesion in the sacrum. Both the HNPGL and metastatic sacral deposit received external beam radiotherapy with a good biochemical and radiological response.

Conclusion

Our case report highlights the rare potential of germline SDHC mutations causing metastatic paraganglioma and concurrent pituitary tumours. Cabergoline treatment may lower elevated 3-methoxytyramine levels and, therefore, mask the biochemical evidence of metastatic disease but also may have therapeutic relevance in dopamine-secreting pheochromocytomas/paragangliomas (PPGLs).

Learning points

  • Several neuroendocrine tumours (NETs) express dopamine D2 and D4 receptors. In this case report, cabergoline significantly reduced plasma 3-methoxytyramine level in a patient with functional HNPGL. Cabergoline might have therapeutic relevance in dopamine-secreting PPGLs.

  • Paragangliomas associated with SDHC mutation classically present with asymptomatic non-functional HNPGL and have rare metastatic potential.

  • The association of pheochromocytoma or paraganglioma and pituitary adenoma is now a well-described rare association (<1%), designated as the three P association. While the three P association is most commonly seen with succinate dehydrogenase B and D mutations, it has also been described in patients with SDHA and SDHC mutations.

  • Cabergoline treatment may lower elevated 3-methoxytyramine levels and mask the biochemical evidence of metastatic disease. Regular functional imaging with Gallium-68 Dotatate PET/MRI provides better evidence of metastatic disease.

Open access
Vinaya Srirangam Nadhamuni Department of Endocrinology, Barts and the London School of Medicine and Dentistry, William Harvey Research Institute, Queen Mary University of London, London, UK

Search for other papers by Vinaya Srirangam Nadhamuni in
Google Scholar
PubMed
Close
,
Donato Iacovazzo Department of Endocrinology, Barts and the London School of Medicine and Dentistry, William Harvey Research Institute, Queen Mary University of London, London, UK

Search for other papers by Donato Iacovazzo in
Google Scholar
PubMed
Close
,
Jane Evanson St. Bartholomew’s Hospital, Barts and the London NHS Trust, London, UK

Search for other papers by Jane Evanson in
Google Scholar
PubMed
Close
,
Anju Sahdev St. Bartholomew’s Hospital, Barts and the London NHS Trust, London, UK

Search for other papers by Anju Sahdev in
Google Scholar
PubMed
Close
,
Jacqueline Trouillas Department of Pathology, Groupement Hospitalier Est, Hospices Civils de Lyon, Bron, France

Search for other papers by Jacqueline Trouillas in
Google Scholar
PubMed
Close
,
Lorraine McAndrew St. Bartholomew’s Hospital, Barts and the London NHS Trust, London, UK

Search for other papers by Lorraine McAndrew in
Google Scholar
PubMed
Close
,
Tom R Kurzawinski Division of Endocrine Surgery, University College Hospital, London, UK

Search for other papers by Tom R Kurzawinski in
Google Scholar
PubMed
Close
,
David Bryant Sunderland Royal Hospital, South Tyneside and Sunderland NHS Foundation Trust, South Shields, Tyne and Wear, UK

Search for other papers by David Bryant in
Google Scholar
PubMed
Close
,
Khalid Hussain Division of Endocrinology, Sidra Medicine, Doha, Ad Dawhah, Qatar

Search for other papers by Khalid Hussain in
Google Scholar
PubMed
Close
,
Satya Bhattacharya St. Bartholomew’s Hospital, Barts and the London NHS Trust, London, UK

Search for other papers by Satya Bhattacharya in
Google Scholar
PubMed
Close
, and
Márta Korbonits Department of Endocrinology, Barts and the London School of Medicine and Dentistry, William Harvey Research Institute, Queen Mary University of London, London, UK

Search for other papers by Márta Korbonits in
Google Scholar
PubMed
Close

Summary

A male patient with a germline mutation in MEN1 presented at the age of 18 with classical features of gigantism. Previously, he had undergone resection of an insulin-secreting pancreatic neuroendocrine tumour (pNET) at the age of 10 years and had subtotal parathyroidectomy due to primary hyperparathyroidism at the age of 15 years. He was found to have significantly elevated serum IGF-1, GH, GHRH and calcitonin levels. Pituitary MRI showed an overall bulky gland with a 3 mm hypoechoic area. Abdominal MRI showed a 27 mm mass in the head of the pancreas and a 6 mm lesion in the tail. Lanreotide-Autogel 120 mg/month reduced GHRH by 45% and IGF-1 by 20%. Following pancreaticoduodenectomy, four NETs were identified with positive GHRH and calcitonin staining and Ki-67 index of 2% in the largest lesion. The pancreas tail lesion was not removed. Post-operatively, GHRH and calcitonin levels were undetectable, IGF-1 levels normalised and GH suppressed normally on glucose challenge. Post-operative fasting glucose and HbA1c levels have remained normal at the last check-up. While adolescent-onset cases of GHRH-secreting pNETs have been described, to the best of our knowledge, this is the first reported case of ectopic GHRH in a paediatric setting leading to gigantism in a patient with MEN1. Our case highlights the importance of distinguishing between pituitary and ectopic causes of gigantism, especially in the setting of MEN1, where paediatric somatotroph adenomas causing gigantism are extremely rare.

Learning points

  • It is important to diagnose gigantism and its underlying cause (pituitary vs ectopic) early in order to prevent further growth and avoid unnecessary pituitary surgery. The most common primary tumour sites in ectopic acromegaly include the lung (53%) and the pancreas (34%) (1): 76% of patients with a pNET secreting GHRH showed a MEN1 mutation (1).

  • Plasma GHRH testing is readily available in international laboratories and can be a useful diagnostic tool in distinguishing between pituitary acromegaly mediated by GH and ectopic acromegaly mediated by GHRH. Positive GHRH immunostaining in the NET tissue confirms the diagnosis.

  • Distinguishing between pituitary (somatotroph) hyperplasia secondary to ectopic GHRH and pituitary adenoma is difficult and requires specialist neuroradiology input and consideration, especially in the MEN1 setting. It is important to note that the vast majority of GHRH-secreting tumours (lung, pancreas, phaeochromocytoma) are expected to be visible on cross-sectional imaging (median diameter 55 mm) (1). Therefore, we suggest that a chest X-ray and an abdominal ultrasound checking the adrenal glands and the pancreas should be included in the routine work-up of newly diagnosed acromegaly patients.

Open access
Le Tuan Linh Department of Radiology, Hanoi Medical University Hospital, Hanoi, Vietnam
Department of Radiology, Hanoi Medical University, Hanoi, Vietnam

Search for other papers by Le Tuan Linh in
Google Scholar
PubMed
Close
,
Nguyen Minh Duc Department of Radiology, Pham Ngoc Thach University of Medicine, Ho Chi Minh city, Vietnam
Department of Radiology, Childrent’s Hospital 2, Ho Chi Minh city, Vietnam

Search for other papers by Nguyen Minh Duc in
Google Scholar
PubMed
Close
,
Hoang Tu Minh Department of Radiology, Hanoi Medical University, Hanoi, Vietnam

Search for other papers by Hoang Tu Minh in
Google Scholar
PubMed
Close
,
Nguyen Ngoc Cuong Department of Radiology, Hanoi Medical University Hospital, Hanoi, Vietnam

Search for other papers by Nguyen Ngoc Cuong in
Google Scholar
PubMed
Close
,
Vuong Thu Ha Department of Radiology, Hanoi Medical University Hospital, Hanoi, Vietnam

Search for other papers by Vuong Thu Ha in
Google Scholar
PubMed
Close
,
Dao-Thi Luan Department of Pathology, Hanoi Medical University Hospital, Hanoi, Vietnam

Search for other papers by Dao-Thi Luan in
Google Scholar
PubMed
Close
,
Thieu-Thi Tra My Department of Radiology, Hanoi Medical University, Hanoi, Vietnam

Search for other papers by Thieu-Thi Tra My in
Google Scholar
PubMed
Close
, and
Bui Van Lenh Department of Radiology, Hanoi Medical University Hospital, Hanoi, Vietnam
Department of Radiology, Hanoi Medical University, Hanoi, Vietnam

Search for other papers by Bui Van Lenh in
Google Scholar
PubMed
Close

Summary

Primary hepatic neuroendocrine tumor (PHNET) is a rare type of neuroendocrine tumor (NET) that is also a primary hepatic tumor. Patients are present with almost no specific clinical symptoms and typically present with negative test results and atypical imaging characteristics; therefore, the differentiation of PHNET from other types of primary hepatic masses can be very difficult. In this article, we describe a case of PHNET that mimicked a liver helminth infection in a 57-year-old man. The diagnosis of PHNET in this patient was challenging, and the final diagnosis was based on imaging, histopathology features, and long-term follow-up.

Learning points

  • An uncommon type of neuroendocrine tumor (NET) is a primary hepatic neuroendocrine tumor (PHNET).

  • Primary hepatic neuroendocrine tumors are rare NET lesions found in the liver, characterized by non-specific clinical and imaging results, which can be easily confused with other liver lesions, including HCC and parasitic lesions.

  • To have a conclusive diagnosis and classification, a mixture of many medical assessment techniques, such as imaging, gastrointestinal endoscopy, nuclear medicine, anatomy, including histopathology, and immunohistochemistry, is essential.

Open access
Ziadoon Faisal Department of General Medicine, Royal Victoria Infirmary, Newcastle Upon Tyne Hospitals NHS Foundation Trust, Newcastle Upon Tyne, UK

Search for other papers by Ziadoon Faisal in
Google Scholar
PubMed
Close
and
Miguel Debono Department of Diabetes and Endocrinology, Royal Hallamshire Hospital, Sheffield Teaching Hospitals NHS Foundation Trust, Sheffield, UK

Search for other papers by Miguel Debono in
Google Scholar
PubMed
Close

Summary

In this case report, we describe the management of a patient who was admitted with an ectopic ACTH syndrome during the COVID pandemic with new-onset type 2 diabetes, neutrophilia and unexplained hypokalaemia. These three findings when combined should alert physicians to the potential presence of Cushing’s syndrome (CS). On admission, a quick diagnosis of CS was made based on clinical and biochemical features and the patient was treated urgently using high dose oral metyrapone thus allowing delays in surgery and rapidly improving the patient’s clinical condition. This resulted in the treatment of hyperglycaemia, hypokalaemia and hypertension reducing cardiovascular risk and likely risk for infection. Observing COVID-19 pandemic international guidelines to treat patients with CS has shown to be effective and offers endocrinologists an option to manage these patients adequately in difficult times.

Learning points

  • This case report highlights the importance of having a low threshold for suspicion and investigation for Cushing’s syndrome in a patient with neutrophilia and hypokalaemia, recently diagnosed with type 2 diabetes especially in someone with catabolic features of the disease irrespective of losing weight.

  • It also supports the use of alternative methods of approaching the diagnosis and treatment of Cushing’s syndrome during a pandemic as indicated by international protocols designed specifically for managing this condition during Covid-19.

Open access