Browse

You are looking at 1 - 10 of 62 items for :

  • Patient Demographics 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
Keerthana Haridas PGY2, Internal Medicine, Icahn School of Medicine, Mount Sinai St Luke’s/West, New York, New York, USA

Search for other papers by Keerthana Haridas in
Google Scholar
PubMed
Close

Summary

Human T-cell lymphotropic virus-1 (HTLV-1) causes adult T-cell leukemia and lymphoma (ATLL) and is a rare but important cause of hypercalcemia. A 53-year-old male with HTLV-1-associated myelopathy presented with acute on chronic bilateral lower extremity weakness and numbness. Initial blood work revealed hypercalcemia with corrected calcium of 16.2 mg/dL (8.5–11.5) with normal levels of phosphorus and alkaline phosphatase. Workup for hypercalcemia revealed parathyroid hormone (PTH) of 14 pg/mL (10–65), 25 hydroxy vitamin D at 19.6 ng/mL (30–100), 1,25 dihydroxy vitamin D at 6.7 pg/mL (19.9–79.3), thyroid-stimulating hormone of 1.265 μIU/mL (0.5–5), undetectable PTH-related protein (PTHrP) and lactate dehydrogenase of 433 U/L (100–220). The urine calcium creatinine ratio was 0.388. Reverse transcriptase PCR was positive for HTLV-1 and negative for HTLV-2. Peripheral blood flow cytometry and lymph node biopsy confirmed ATLL. He received treatment with fluids, calcitonin and denosumab after which serum calcium levels fell (nadir: 7.7 mg/dL) and then normalized. Humoral hypercalcemia in this setting is mediated by receptor activator of nuclear factor-kappa B ligand (RANKL), PTHrP and other cytokines. PTHrP levels depend on levels of the TAX gene product, cell type and lymphocyte-specific factors. Thus, a low level, like in our patient, does not rule out HTLV-1 infection/ATLL as the cause of hypercalcemia. Hypercalcemia is known to be responsive to monoclonal antibodies against RANKL given the compound’s role in mediating hypercalcemia in these cases.

Learning points

  • Human T-cell lymphotropic virus-1 infection and adult T-cell leukemia and lymphoma are associated with high rates of hypercalcemia and hypercalcemic crises.

  • Hypercalcemia in these cases is mediated by osteoclastic bone resorption carried out by several agents including receptor activator of nuclear factor-kappa B ligand, parathyroid hormone-related protein (PTHrP), macrophage inflammatory protein 1 alpha, interleukins, etc. A normal PTHRrP does not rule out humoral hypercalcemia of malignancy in this setting, as indicated by this case.

  • Hypercalcemia in such settings is highly responsive to monoclonal antibodies against RANKL given the role the ligand plays in resorptive hypercalcemia.

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
Iris Dirven Department of Endocrinology, Universitair Ziekenhuis Brussel, Vrije Universiteit Brussel, Brussels, Belgium

Search for other papers by Iris Dirven in
Google Scholar
PubMed
Close
,
Bert Bravenboer Department of Endocrinology, Universitair Ziekenhuis Brussel, Vrije Universiteit Brussel, Brussels, Belgium

Search for other papers by Bert Bravenboer in
Google Scholar
PubMed
Close
,
Steven Raeymaeckers Department of Radiology, Universitair Ziekenhuis Brussel, Vrije Universiteit Brussel, Brussels, Belgium

Search for other papers by Steven Raeymaeckers in
Google Scholar
PubMed
Close
, and
Corina E Andreescu Department of Endocrinology, Universitair Ziekenhuis Brussel, Vrije Universiteit Brussel, Brussels, Belgium

Search for other papers by Corina E Andreescu in
Google Scholar
PubMed
Close

Summary

The Covid-19 vaccination has been rapidly implemented among patients with cancer. We present two cases of patients with endocrine tumours who developed lymphadenopathy following a Covid-19 vaccination. In the case of a patient with multiple endocrine neoplasia (MEN) 1 syndrome, an 18-fluorodeoxyglucose (18FDG)-PET/CT showed positive axillary lymph nodes. Further work-up with fine needle aspiration showed a reactive pattern following a Covid-19 vaccination in the ipsilateral arm shortly before the 18FDG-PET/CT. A second patient, in follow-up for thyroid cancer, developed clinical supraclavicular lymphadenopathy after a Covid-19 vaccination. Follow-up ultrasound proved the lesion to be transient. These cases demonstrate lymphadenopathy in response to a Covid-19 vaccination in two patients susceptible to endocrine tumours and metastatic disease. With growing evidence about the pattern and occurrence of lymphadenopathy after mRNA Covid-19 vaccination, recommendations for scheduling and interpretation of imaging among cancer patients should be implemented to reduce equivocal findings, overdiagnosis, and overtreatment, while maintaining a good standard of care in oncological follow-up.

Learning points

  • Reactive lymphadenopathy is very common after an mRNA vaccination against Covid-19 and should be part of the differential diagnosis in patients with endocrine tumours who recently received a Covid-19 mRNA vaccination and present with an ipsilateral lymphadenopathy.

  • A good vaccine history is essential in assessing the risk for lymphadenopathy and if possible, screening imaging in patients with endocrine tumours should be postponed at least 6 weeks after the previous vaccination.

  • For now, a multidisciplinary care approach is recommended to determine the necessary steps in the diagnostic evaluation of lymphadenopathy in the proximity of a Covid-19 vaccination.

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
Adrian Po Zhu Li Department of Endocrinology ASO/EASO COM, King ’s College Hospital NHS Foundation Trust, Denmark Hill, London, UK

Search for other papers by Adrian Po Zhu Li in
Google Scholar
PubMed
Close
,
Sheela Sathyanarayan Department of Endocrinology ASO/EASO COM, King ’s College Hospital NHS Foundation Trust, Denmark Hill, London, UK

Search for other papers by Sheela Sathyanarayan in
Google Scholar
PubMed
Close
,
Salvador Diaz-Cano Departments of Cellular Pathology and Molecular Pathology, Queen Elizabeth Hospital, Birmingham, UK
Division of Cancer Studies, King’s College London, London, UK

Search for other papers by Salvador Diaz-Cano in
Google Scholar
PubMed
Close
,
Sobia Arshad Department of Endocrinology ASO/EASO COM, King ’s College Hospital NHS Foundation Trust, Denmark Hill, London, UK

Search for other papers by Sobia Arshad in
Google Scholar
PubMed
Close
,
Eftychia E Drakou Department of Clinical Oncology, Guy’s Cancer Centre – Guy’s and St Thomas’ NHS Foundation Trust, Great Maze Pond, London, UK

Search for other papers by Eftychia E Drakou in
Google Scholar
PubMed
Close
,
Royce P Vincent Department of Clinical Biochemistry, King’s College Hospital NHS Foundation Trust, Denmark Hill, London, UK
Faculty of Life Sciences and Medicine, School of Life Course Sciences, King’s College London, London, UK

Search for other papers by Royce P Vincent in
Google Scholar
PubMed
Close
,
Ashley B Grossman Oxford Centre for Diabetes, Endocrinology and Metabolism, University of Oxford, Oxford, UK
Barts and the London School of Medicine, Centre for Endocrinology, William Harvey Institute, London, UK
Neuroendocrine Tumour Unit, Royal Free Hospital, London, UK

Search for other papers by Ashley B Grossman in
Google Scholar
PubMed
Close
,
Simon J B Aylwin Department of Endocrinology ASO/EASO COM, King ’s College Hospital NHS Foundation Trust, Denmark Hill, London, UK

Search for other papers by Simon J B Aylwin in
Google Scholar
PubMed
Close
, and
Georgios K Dimitriadis Department of Endocrinology ASO/EASO COM, King ’s College Hospital NHS Foundation Trust, Denmark Hill, London, UK
Obesity, Type 2 Diabetes and Immunometabolism Research Group, Department of Diabetes, Faculty of Life Sciences, School of Life Course Sciences, King’s College London, London, UK
Division of Reproductive Health, Warwick Medical School, University of Warwick, Coventry, UK

Search for other papers by Georgios K Dimitriadis in
Google Scholar
PubMed
Close

Summary

A 49-year-old teacher presented to his general physician with lethargy and lower limb weakness. He had noticed polydipsia, polyuria, and had experienced weight loss, albeit with an increase in central adiposity. He had no concomitant illnesses and took no regular medications. He had hypercalcaemia (adjusted calcium: 3.34 mmol/L) with hyperparathyroidism (parathyroid hormone: 356 ng/L) and hypokalaemia (K: 2.7 mmol/L) and was admitted for i.v. potassium replacement. A contrast-enhanced CT chest/abdomen/pelvis scan revealed a well-encapsulated anterior mediastinal mass measuring 17 × 11 cm with central necrosis, compressing rather than invading adjacent structures. A neck ultrasound revealed a 2 cm right inferior parathyroid lesion. On review of CT imaging, the adrenals appeared normal, but a pancreatic lesion was noted adjacent to the uncinate process. His serum cortisol was 2612 nmol/L, and adrenocorticotrophic hormone was elevated at 67 ng/L, followed by inadequate cortisol suppression to 575 nmol/L from an overnight dexamethasone suppression test. His pituitary MRI was normal, with unremarkable remaining anterior pituitary biochemistry. His admission was further complicated by increased urine output to 10 L/24 h and despite three precipitating factors for the development of diabetes insipidus including hypercalcaemia, hypokalaemia, and hypercortisolaemia, due to academic interest, a water deprivation test was conducted. An 18flurodeoxyglucose-PET (FDG-PET) scan demonstrated high avidity of the mediastinal mass with additionally active bilateral superior mediastinal nodes. The pancreatic lesion was not FDG avid. On 68Ga DOTATE-PET scan, the mediastinal mass was moderately avid, and the 32 mm pancreatic uncinate process mass showed significant uptake. Genetic testing confirmed multiple endocrine neoplasia type 1.

Learning points

  • In young patients presenting with primary hyperparathyroidism, clinicians should be alerted to the possibility of other underlying endocrinopathies.

    In patients with multiple endocrine neoplasia type 1 (MEN-1) and ectopic adrenocorticotrophic hormone syndrome (EAS), clinicians should be alerted to the possibility of this originating from a neoplasm above or below the diaphragm.

  • Although relatively rare compared with sporadic cases, thymic carcinoids secondary to MEN-1 may also be associated with EAS.

  • Electrolyte derangement, in particular hypokalaemia and hypercalcaemia, can precipitate mild nephrogenic diabetes insipidus.

Open access
J M K de Filette Department of Endocrinology, University Hospital Brussels (VUB), Brussels, Belgium

Search for other papers by J M K de Filette in
Google Scholar
PubMed
Close
,
Bastiaan Sol Department of Endocrinology, University Hospital Brussels (VUB), Brussels, Belgium

Search for other papers by Bastiaan Sol in
Google Scholar
PubMed
Close
,
Gil Awada Department of Medical Oncology, University Hospital Brussels (VUB), Brussels, Belgium

Search for other papers by Gil Awada in
Google Scholar
PubMed
Close
,
Corina E Andreescu Department of Endocrinology, University Hospital Brussels (VUB), Brussels, Belgium

Search for other papers by Corina E Andreescu in
Google Scholar
PubMed
Close
,
David Unuane Department of Endocrinology, University Hospital Brussels (VUB), Brussels, Belgium

Search for other papers by David Unuane in
Google Scholar
PubMed
Close
,
Sandrine Aspeslagh Department of Medical Oncology, University Hospital Brussels (VUB), Brussels, Belgium

Search for other papers by Sandrine Aspeslagh in
Google Scholar
PubMed
Close
,
Jan Poelaert Department of Critical Care Medicine, University Hospital Brussels (VUB), Brussels, Belgium
Department of Anesthesiology and Perioperative Medicine, University Hospital Brussels (VUB), Brussels, Belgium

Search for other papers by Jan Poelaert in
Google Scholar
PubMed
Close
, and
Bert Bravenboer Department of Endocrinology, University Hospital Brussels (VUB), Brussels, Belgium

Search for other papers by Bert Bravenboer in
Google Scholar
PubMed
Close

Summary

The pandemic caused by severe acute respiratory syndrome coronavirus 2 is of an unprecedented magnitude and has made it challenging to properly treat patients with urgent or rare endocrine disorders. Little is known about the risk of coronavirus disease 2019 (COVID-19) in patients with rare endocrine malignancies, such as pituitary carcinoma. We describe the case of a 43-year-old patient with adrenocorticotrophic hormone-secreting pituitary carcinoma who developed a severe COVID-19 infection. He had stabilized Cushing’s disease after multiple lines of treatment and was currently receiving maintenance immunotherapy with nivolumab (240 mg every 2 weeks) and steroidogenesis inhibition with ketoconazole (800 mg daily). On admission, he was urgently intubated for respiratory exhaustion. Supplementation of corticosteroid requirements consisted of high-dose dexamethasone, in analogy with the RECOVERY trial, followed by the reintroduction of ketoconazole under the coverage of a hydrocortisone stress regimen, which was continued at a dose depending on the current level of stress. He had a prolonged and complicated stay at the intensive care unit but was eventually discharged and able to continue his rehabilitation. The case points out that multiple risk factors for severe COVID-19 are present in patients with Cushing’s syndrome. ‘Block-replacement’ therapy with suppression of endogenous steroidogenesis and supplementation of corticosteroid requirements might be preferred in this patient population.

Learning points

  • Comorbidities for severe coronavirus disease 2019 (COVID-19) are frequently present in patients with Cushing’s syndrome.

  • ‘Block-replacement’ with suppression of endogenous steroidogenesis and supplementation of corticosteroid requirements might be preferred to reduce the need for biochemical monitoring and avoid adrenal insufficiency.

  • The optimal corticosteroid dose/choice for COVID-19 is unclear, especially in patients with endogenous glucocorticoid excess.

  • First-line surgery vs initial disease control with steroidogenesis inhibitors for Cushing’s disease should be discussed depending on the current healthcare situation.

Open access
Wouter W. de Herder Department of Internal Medicine, Sector of Endocrinology, Rotterdam, The Netherlands

Search for other papers by Wouter W. de Herder in
Google Scholar
PubMed
Close

Summary

The iconic photograph ‘A Jewish giant at home with his parents in the Bronx, N.Y. 1970’ by the famous American photographer Diane Arbus (1923–1971) shows the 2.34 m (7 ft. 8¼ in.) acromegalic giant Eddie Carmel (1936–1972) and his parents in the living room of their New York home. The picture is a typical example of Arbus’ style. The relationship between the artist and the tall subject is described. A growth hormone-secreting pituitary macroadenoma was unsuccessfully treated with two cycles of pituitary radiotherapy achieving a 7000 rad cumulative dose and by incomplete pituitary surgery. Hypopituitarism was treated according to medical standards in the 1960s and 1970s. The giant patient died of increased intracranial pressure and at autopsy a residual acidophil pituitary macroadenoma was found, but also a perisellar meningioma which was most probably induced by the high dose of pituitary radiotherapy. The case report illustrates the possibilities and impossibilities of treating acromegaly 50 years ago and demonstrates the potential risks of high dose pituitary radiotherapy (in acromegaly).

Learning points

  • Acromegaly is a very old disease.

  • Therapy for acromegaly has evolved over the decades.

  • In art museums one can come across artistic impressions of endocrine disorders.

  • People suffering from disfiguring endocrine disorders like acromegaly were pre-WW2 ‘exposed’ in theaters and circuses.

  • High dose pituitary radiotherapy can be associated with secondary brain tumor formation.

Open access
Ray Wang Department of Diabetes and Endocrinology, Royal Melbourne Hospital, Parkville, Victoria, Australia

Search for other papers by Ray Wang in
Google Scholar
PubMed
Close
,
Benjamin Solomon Department of Medical Oncology, Peter MacCallum Cancer Centre, Melbourne, Victoria, Australia
Sir Peter MacCallum Department of Oncology, The University of Melbourne, Victoria, Australia

Search for other papers by Benjamin Solomon in
Google Scholar
PubMed
Close
,
Stephen J Luen Department of Medical Oncology, Peter MacCallum Cancer Centre, Melbourne, Victoria, Australia
Sir Peter MacCallum Department of Oncology, The University of Melbourne, Victoria, Australia

Search for other papers by Stephen J Luen in
Google Scholar
PubMed
Close
,
Owen W.J. Prall Department of Pathology, Peter MacCallum Cancer Centre, Parkville, Victoria, Australia

Search for other papers by Owen W.J. Prall in
Google Scholar
PubMed
Close
,
Christine Khoo Department of Pathology, Peter MacCallum Cancer Centre, Parkville, Victoria, Australia

Search for other papers by Christine Khoo in
Google Scholar
PubMed
Close
,
Anthony J Gill University of Sydney, Sydney, New South Wales, Australia

Search for other papers by Anthony J Gill in
Google Scholar
PubMed
Close
,
Jeremy Lewin Department of Medical Oncology, Peter MacCallum Cancer Centre, Melbourne, Victoria, Australia
Sir Peter MacCallum Department of Oncology, The University of Melbourne, Victoria, Australia

Search for other papers by Jeremy Lewin in
Google Scholar
PubMed
Close
, and
Nirupa Sachithanandan Department of Internal Medicine, Peter MacCallum Cancer Centre, Parkville, Victoria, Australia

Search for other papers by Nirupa Sachithanandan in
Google Scholar
PubMed
Close

Summary

Adrenocortical carcinoma is a rare disease with poor prognosis whose clinical heterogeneity can at times present a challenge to accurate and timely diagnosis. We present the case of a patient who presented with extensive pulmonary lesions, mediastinal and hilar lymphadenopathy and an adrenal mass in whom the oncological diagnosis was initially uncertain. Through the use of immunohistochemistry, biochemistry and genomic testing, an accurate diagnosis of adrenocortical carcinoma was ultimately made which resulted in more directed treatment being administered. The use of multidisciplinary input and genomics to aid in diagnosis and prognosis of adrenocortical carcinoma is discussed.

Learning points

  • Adrenocortical carcinomas can present a diagnostic challenge to clinicians given it is a rare malignancy with significant clinical heterogeneity.

  • Specialist multidisciplinary team input is vital in the diagnosis and management of adrenocortical carcinomas.

  • Hormonal testing is recommended in the diagnostic workup of adrenal masses, even in the absence of overt clinical signs/symptoms of hormone excess.

  • Immunostaining for the highly sensitive and specific steroidogenic factor-1 is vital for accurate diagnosis.

  • Genomics can provide prognostic utility in management of adrenocortical carcinoma.