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Isabella Chiardi Thyroid Unit of Clinic for Endocrinology and Diabetology, Lugano Regional Hospital, Ente Ospedaliero Cantonale, Bellinzona, Switzerland
Faculty of Medicine and Surgery, Humanitas University, Rozzano, Milan, Italy

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Priska Gaffuri Istituto Cantonale di Patologia, Ente Ospedaliero Cantonale, Bellinzona, Switzerland

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Andrea Leoncini Servizio di Radiologia e Radiologia Interventistica, Istituto di Imaging Della Svizzera Italiana (IIMSI), Ente Ospedaliero Cantonale, Bellinzona, Switzerland

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Pierpaolo Trimboli Thyroid Unit of Clinic for Endocrinology and Diabetology, Lugano Regional Hospital, Ente Ospedaliero Cantonale, Bellinzona, Switzerland
Faculty of Biomedical Sciences, Università della Svizzera Italiana (USI), Lugano, Switzerland

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Summary

Thyroid metastases from nonthyroidal malignancies (NTMs) represent a diagnostic challenge, often displaying heterogeneous clinical manifestations. These metastases are rare but significant, accounting for approximately 2% of thyroid malignancies. Distinguishing them from primary thyroid malignancies is challenging due to the lack of specific ultrasound features, and the ultrasound-based risk stratification systems offer limited utility in such cases. Fine needle aspiration cytology is crucial for definitive diagnosis, yet it may not always provide accurate results. In this case report, we describe a unique instance of thyroid metastases originating from renal cell carcinoma, emphasizing the complexities in diagnosis and the importance of considering oncological conditions when assessing thyroid masses. Awareness of thyroid metastasis from NTMs, particularly in cases of diffuse thyroid hypoechogenicity and hypothyroidism, is essential for clinicians in their diagnostic approach.

Learning points

  • Thyroid metastases from nonthyroidal malignancies are diagnostic challenges due to their heterogeneous clinical presentations, often mimicking primary thyroid malignancies.

  • Thyroid metastases from nonthyroidal malignancies are relatively rare, but they still account for approximately 2% of thyroid malignancies.

  • It is fundamental to consider oncological conditions when assessing thyroid masses, especially in cases of diffuse thyroid hypoechogenicity, hypothyroidism, and history of other tumors.

  • Thyroid presentation is quite similar to that of autoimmune hypothyroidism, endocrinologists must be aware of the possibility of thyroid hypofunction due to the massive invasion of the parenchyma.

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Nele Van Roy Department of Diabetes and Endocrinology, Vitaz, Sint-Niklaas, Belgium

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Sylvester Heerwegh Department of Diabetes and Endocrinology, Vitaz, Sint-Niklaas, Belgium

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Dashty Husein Department of Diabetes and Endocrinology, Vitaz, Sint-Niklaas, Belgium

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Joke Ruys Department of Ophthalmology, Vitaz, Sint-Niklaas, Belgium

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Peter Coremans Department of Diabetes and Endocrinology, Vitaz, Sint-Niklaas, Belgium

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Summary

Bardet–Biedl syndrome (BBS) is a rare, autosomal recessive, multisystem non-motile ciliopathy of progressive onset. It is primarily characterised by rod–cone dystrophy, early-onset obesity and related complications, postaxial polydactyly, renal and genitourinary abnormalities, learning disabilities, and hypogonadism. The diagnosis is based on Beales’ modified diagnostic criteria. We present a case of two monozygotic female twins, 17 years of age at presentation, referred for obesity since childhood. The initial hormonal work-up was negative and no dysmorphic features were noted. They were diagnosed with exogenous obesity. However, after ophthalmologic problems became apparent, rod–cone dystrophy was observed and genetic testing was performed. A mutation in the BBS2 gene led to the diagnosis of BBS, although the full diagnostic criteria were not met. This case not only highlights the need to raise awareness for BBS but also exposes two limitations of the current diagnostic standard. The first limitation is the low sensitivity of the clinical diagnostic model, due to the progressive onset and the high variability of the syndrome. The second limitation is the unclear role of genetic testing. As genetic testing becomes more widely available, genetic diagnosis preceding clinical diagnosis will become more common, leading to a diagnostic conundrum. We propose an update of the diagnostic model. A less strict application in the presence of confirmed genetic mutations should be applied, as this could facilitate earlier diagnosis and intervention. This is important because therapeutic agents are being developed that could have a significant impact on quality of life and prognosis.

Learning points

  • Due to the low prevalence, the significant inter-and intrafamilial variation, and the slowly evolving phenotype, monogenic forms of obesity such as Bardet–Biedl syndrome are difficult to diagnose. Despite advances in the understanding of the presentation, pathophysiology and access to accurate genetic characterisation, a substantial number of diagnoses are still made by ophthalmology, as recognition of BBS in other departments of medicine, remains limited.

  • Clinical diagnosis of BBS is based on Beales’ modified diagnostic criteria which require the presence of four primary features or three primary features plus two secondary features. This model has its limitations. Due to the progressive onset of clinical symptoms, patients generally do not meet the diagnostic criteria early in life, leading to a delay in diagnosis. In addition, the role of genetic testing remains controversial. However, as it becomes more widely available, genetic diagnosis may precede a full clinical diagnosis.

  • BBS has an impact on the quality of life and prognosis of both the patient and the family. Obesity management strategies are an important part of the multidisciplinary approach, as there is no cure available. Setmelanotide has shown promising results in a phase 3 trial, but its effect in clinical practice remains unproven.

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Natalie Below Diabetes Centre, Gartnavel General Hospital, Glasgow, UK
University of Glasgow, Glasgow, UK

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Deborah Morrison Diabetes Centre, Gartnavel General Hospital, Glasgow, UK

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Ruth McGowan West of Scotland Centre for Genomic Medicine, Glasgow, UK

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Gregory C Jones Diabetes Centre, Gartnavel General Hospital, Glasgow, UK

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Summary

A 20-year-old South Asian male presented with polyuria, polydipsia, HbA1c 81 mmol/mol, BMI 28.8 and family history of both type 1 and type 2 diabetes mellitus. As autoantibody testing was negative and c-peptide level demonstrated significant endogenous insulin secretion, type 1 diabetes was excluded. Given his age and family history, the differential diagnosis included maturity-onset diabetes of the young (MODY), a rare form of diabetes caused by a single-gene variant. A high probability of MODY was calculated and he was subsequently referred for genetic testing. Although a useful tool, the pre-test probability calculator for MODY is only validated in White Europeans. A heterogenous variant of unknown clinical significance of the NEUROD1 gene was detected, leading to gliclazide use with poor response. The patient responded well to metformin. Type 2 diabetes was considered the most likely diagnosis. This case highlights the diagnostic challenges in young patients of Asian ethnicity and the importance of interpreting genetic results of unknown significance within the clinical context. Ethnicity-specific BMI thresholds should be used when classifying patients as overweight or obese.

Learning points

  • Variants of unknown significance detected by genetic sequencing should be interpreted within the context of the patient’s other clinical parameters.

  • It is important to use ethnicity-specific BMI thresholds for obesity.

  • Diagnosis of type 2 diabetes mellitus at younger ages is becoming increasingly common.

  • The pre-test probability calculator for MODY is only validated in White Europeans; although a useful guide, results should be interpreted with caution in patients of other ethnicities.

Open access
Vivi-Nelli Mäkinen Department of Endocrinology and Internal Medicine, Aarhus University Hospital, Aarhus, Denmark
Department of Internal Medicine, Regional Hospital, Horsens, Denmark

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Stine Horskær Madsen Department of Pathology, Aarhus University Hospital, Aarhus, Denmark

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Mette Ji Riis-Vestergaard Department of Endocrinology and Internal Medicine, Aarhus University Hospital, Aarhus, Denmark
Department of Internal Medicine, Gødstrup Hospital, Herning,Denmark

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Mette Bjerre Department of Clinical Medicine, Aarhus University, Aarhus University Hospital, Aarhus, Denmark

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Steen Bønløkke Pedersen Steno Diabetes Center Aarhus, Aarhus University Hospital, Aarhus, Denmark

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Sylvia L Asa Department of Pathology, University Health Network, Toronto,Canada

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Lars Rolighed Department of Otorhinolaryngology, Head and Neck Surgery, Aarhus University Hospital, Aarhus, Denmark

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Jens Otto Lunde Jørgensen Department of Endocrinology and Internal Medicine, Aarhus University Hospital, Aarhus, Denmark
Department of Clinical Medicine, Aarhus University, Aarhus University Hospital, Aarhus, Denmark

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Marie Juul Ornstrup Department of Endocrinology and Internal Medicine, Aarhus University Hospital, Aarhus, Denmark

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Summary

This case report describes a rare presentation of ectopic Cushing’s syndrome (CS) due to ectopic corticotropin-releasing hormone (CRH) production from a medullary thyroid carcinoma (MTC). The patient, a 69-year-old man, presented with symptoms of muscle weakness, facial plethora, and easy bruising. An inferior petrosal sinus sampling test (IPSS) demonstrated pituitary adrenocorticotrophic hormone (ACTH) secretion, but a whole-body somatostatin receptor scintigraphy (68Ga-DOTATOC PET/CT) revealed enhanced uptake in the right thyroid lobe which, in addition to a grossly elevated serum calcitonin level, was indicative of an MTC. A 18F-DOPA PET/CT scan supported the diagnosis, and histology confirmed the presence of MTC with perinodal growth and regional lymph node metastasis. On immunohistochemical analysis, the tumor cell stained positively for calcitonin and CRH but negatively for ACTH. Distinctly elevated plasma CRH levels were documented. The patient therefore underwent thyroidectomy and bilateral adrenalectomy. This case shows that CS caused by ectopic CRH secretion may masquerade as CS due to a false positive IPSS test. It also highlights the importance of considering rare causes of CS when diagnostic test results are ambiguous.

Learning points

  • Medullary thyroid carcinoma may secrete CRH and cause ectopic CS.

  • Ectopic CRH secretion entails a rare pitfall of inferior petrosal sinus sampling yielding a false positive test.

  • Plasma CRH measurements can be useful in selected cases.

Open access
Najoua Lassoued Endocrinology Department, Taher Sfar University Hospital, Mahdia, Tunisia

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Wafa Alaya Endocrinology Department, Taher Sfar University Hospital, Mahdia, Tunisia

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Senda Rebai Endocrinology Department, Taher Sfar University Hospital, Mahdia, Tunisia

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Sondos Arfa Internal Medecine Department, Taher Sfar University Hospital, Mahdia, Tunisia

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Baha Zantour Endocrinology Department, Taher Sfar University Hospital, Mahdia, Tunisia

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Mohamed Habib Sfar Endocrinology Department, Taher Sfar University Hospital, Mahdia, Tunisia

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Summary

Autoimmune polyglandular syndrome (APS) type 2 is characterized by the presence of Addison’s disease (AD) along with autoimmune thyroid disease and/or type 1 diabetes. APS type 2 is known as Schmidt’s syndrome when autoimmune adrenal insufficiency is associated with chronic lymphocytic thyroiditis. We report a very rare case of a 28-year-old female patient who had Schmidt’s syndrome revealed by a thyroid storm (TS) concomitant with an acute adrenal crisis. The onset of AD resulted in a surgical emergency. The patient presented with cardiogenic shock and an acute abdomen. The precipitation factor was Hashitoxicosis presented as TS. This life-threatening condition was successfully reversed with aggressive medical therapy based on antithyroid drugs and intravenous glucocorticoids. This hyperthyroid phase lasted for a period of 8 months. The patient eventually developed hypothyroidism, suggesting that Hashimoto's thyroiditis was the most likely diagnosis. She was started on levothyroxine replacement therapy and remained euthyroid on levothyroxine. The case we describe had several diagnostic pitfalls that are discussed both at the start as well as during the evolution.

Learning points

  • Autoimmune diseases can appear concomitantly or succeed each other over time. The clinician must be vigilant to detect these diseases in time in order to avoid a misdiagnosis of a life-threatening emergency such as adrenal insufficiency or thyroid storm.

  • Thyroid storm is an uncommon but life-threatening manifestation of hyperthyroidism. Diagnosis is dependent on clinical symptoms, and no specific laboratory tests are available.

  • Glucocorticoids should be used in the treatment of thyroid storm because they have an inhibitory effect on peripheral conversion of T4 to T3.

  • In patients who have severe thyrotoxicosis, especially in conjunction with hypotension, treatment with glucocorticoids has become standard practice because of the possibility of relative adrenal insufficiency or the possibility of undiagnosed Addison’s disease.

  • The differential diagnosis of hyperthyroidism can be challenging. Graves’ disease can be discussed in view of the severity of the clinical presentation and the prolonged duration of the hyperthyroid phase. Hashitoxicosis is the initial hyperthyroid phase in chronic autoimmune thyroiditis. The hyperthyroid phase is always followed by definitive resolution, with persistent euthyroidism and no hyperthyroid relapses.

  • Synthetic antithyroid drugs may be prescribed during the hyperthyroid phase of Hashimoto thyroiditis if the clinical presentation is severe and the duration of the hyperthyroid phase is prolonged.

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Laura González Fernández Endocrinology and Nutrition Department, Hospital General Universitario Gregorio Marañón, Madrid, Spain

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Alejandra Maricel Rivas Montenegro Endocrinology and Nutrition Department, Hospital General Universitario Gregorio Marañón, Madrid, Spain

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Noemí Brox Torrecilla Endocrinology and Nutrition Department, Hospital Ramón y Cajal, Madrid, Spain

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María Miguélez González Endocrinology and Nutrition Department, Hospital Fundación Jiménez Díaz, Madrid, Spain

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Jose Atencia Goñi Endocrinology and Nutrition Department, Hospital General Universitario Gregorio Marañón, Madrid, Spain

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Elisa Fernández Fernández Endocrinology and Nutrition Department, Hospital General Universitario Gregorio Marañón, Madrid, Spain

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Olga González Albarrán Endocrinology and Nutrition Department, Hospital General Universitario Gregorio Marañón, Madrid, Spain

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Juan Carlos Pércovich Hualpa Endocrinology and Nutrition Department, Hospital General Universitario Gregorio Marañón, Madrid, Spain

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Marcel Sambo Salas Endocrinology and Nutrition Department, Hospital General Universitario Gregorio Marañón, Madrid, Spain

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Rogelio García Centeno Endocrinology and Nutrition Department, Hospital General Universitario Gregorio Marañón, Madrid, Spain

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Summary

Ectopic Cushing’s syndrome (ECS) is a rare disease associated with significant comorbidity. Among the causes of Cushing's syndrome, adrenocorticotropic hormone-producing extrapituitary tumours are rarely reported. This low frequency makes it difficult for the physician to acquire experience in its management.

In this report, we aimed to describe the clinical presentation, diagnostic approach and treatment modalities of 12 patients with ECS treated in a single tertiarycentre over a 17-year period. Although they can appear in different locations through the neuroendocrine system, lung tumours are the most frequently reported, as it occurs in our series. They can show different levels of aggressiveness and mild to severe clinical course. Therefore, distinguishing Cushing's disease can be challenging and sometimes requires more specific techniques such as invasive tests or no conventional imaging. Treatment includes controlling both hypercortisolism and neoplastic disease, and multidisciplinary management is recommended.

Learning points

  • Ectopic Cushing's syndrome (ECS) accounts for 15% of endogenous Cushing's syndromes. Its infrequency implies that both diagnosis and treatment can be a challenge for clinicians without experience in its management.

  • The most common location is the lung. Although older series reported small cell lung carcinoma (SCLC) as the main ECS-producing tumour, currently most cases are attributed to lung carcinoids.

  • Low-grade tumours (lung carcinoids) present themselves with a more subtle and gradual hypercortisolism, and clinically this can be difficult to differentiate from hypercortisolism due to CD. In contrast, high-grade tumours (SCLC) show severe hypercortisolism with rapid evolution.

  • The diagnostic approach is complex especially when the tumour is not previously known and the clinical presentation is subtle. Functional tests are mandatory in these cases, and nuclear medicine imaging can help when conventional imaging tests fail to identify the tumour.

  • ECS treatment includes a wide variety of modalities oriented to treat both the excess of cortisol and the tumour itself. The tumour prognosis depends fundamentally on the type of adrenocorticotropic hormone-secreting tumour.

  • Expert and multidisciplinary team is essential for successfully treating these complex and ill patients.

Open access
Gabija Germanaitė Faculty of Medicine, Medical Academy, Lithuanian University of Health Sciences (LUHS), Kaunas, Lithuania

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Diana Šimonienė Department of Endocrinology, Lithuanian University of Health Sciences (LUHS), Kaunas, Lithuania

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Summary

Primary hyperparathyroidism (PHPT) is a common endocrinological pathology; nonetheless, it is rarely diagnosed in pregnancy. Primary hyperparathyroidism can present with clinically expressed hypercalcemia. High Ca levels in the blood may lead to a miscarriage. We present the case of a 39-year-old woman who visited our Endocrinology clinic in search of a cause of infertility. Blood work showed elevated Ca and parathyroid hormone (PTH) levels. Upper left parathyroid gland adenoma was found during a neck ultrasound. Parathyroid gland adenoma was likely the cause of PHPT and was treated with parathyroidectomy. Surgery was carried out, and the upper left parathyroid lobe adenoma was removed. High levels of Ca were found in all performed blood works since the first visit to the clinic, but following the surgery, Ca levels of the patient were in the normal range and the woman got pregnant for the third time, later delivering a healthy baby. In conclusion, we would like to put forward the idea that an evaluation of Ca level in the blood should be included in the protocol for treating patients with recurrent miscarriages. Early detection of hypercalcemia can improve the outcomes of disease that primary hyperparathyroidism can cause. Swift and accurate decrease of serum Ca correspondingly safeguards the woman from a possible pregnancy loss along with complications that arise from it.–

Learning points

  • Primary hyperparathyroidism (PHPT) is a common endocrinological pathology, nonetheless, it is rarely diagnosed in pregnancy.

  • Primary hyperparathyroidism can present with clinically expressed hypercalcemia, and high Ca levels in the blood may lead to a miscarriage.

  • Early detection of hypercalcemia can improve the outcomes of disease that primary hyperparathyroidism can cause.

  • Swift and accurate decrease of serum Ca correspondingly safeguards the woman from a possible pregnancy loss along with complications that arise from it.

  • All pregnant patients with hypercalcemia should be assessed for the presence of primary hyperparathyroidism as it is likely the cause.

Open access
Wenxin Zhang Department of Endocrinology and Metabolism, First Affiliated Hospital of Nanchang University, Nanchang, Jiangxi, People’s Republic of China

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Wenqiong Xu Department of Endocrinology and Metabolism, First Affiliated Hospital of Nanchang University, Nanchang, Jiangxi, People’s Republic of China

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Summary

Immune checkpoint inhibitors (ICPis) are novel immunotherapy drugs for a variety of cancers. Toripalimab is one of the ICPis that selectively blocks programmed death 1 (PD-1) and has been used for the treatment of malignant cancers in the hospitals of China. But with the widespread use of ICPis, some of the adverse reactions have gradually appeared. One of the most serious side effects is diabetes mellitus which is a relatively rare immune-related adverse event (irAEs) with life-threatening complications. We report a case of diabetes after the administration of toripalimab for the treatment of melanoma in southern China. To our knowledge, this is a rare case of diabetes occurring during toripalimab therapy, there is only one similar case reported in China so far. As China has a high morbidity of malignant cancer, a significant number of patients could be affected by the adverse reactions of using ICPis. Therefore, when ICPis are administrated, it is very important for clinicians to pay attention to one of the serious side effects – diabetes mellitus. Insulin therapy is often necessary after the diagnosis of ICPis-related diabetes, which has been proved as an effective method to prevent diabetic ketoacidosis (DKA) and other life-threatening complications in these patients.

Learning points

  • Toripalimab can cause the diabetes mellitus.

  • ICPis-related diabetes is treated primarily with insulin.

  • Immune checkpoint inhibitors cause diabetes by primarily destroying islet β cells.

  • There is not enough evidence to demonstrate that diabetic autoantibodies are related to diabetes caused by ICPis.

  • In addition to focusing on the efficacy of PD-1 inhibitor therapy, it is also necessary to pay attention to its adverse reactions, such as ICPis-related diabetes mellitus.

Open access
S M Constantinescu Department of Endocrinology, Cliniques Universitaires Saint-Luc, Brussels, Belgium

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G Wilms Department of Radiology, Cliniques Universitaires Saint-Luc, Brussels, Belgium

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R M Furnica Department of Endocrinology, Cliniques Universitaires Saint-Luc, Brussels, Belgium

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T Duprez Department of Radiology, Cliniques Universitaires Saint-Luc, Brussels, Belgium

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D Maiter Department of Endocrinology, Cliniques Universitaires Saint-Luc, Brussels, Belgium

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Summary

Complicated Rathke’s cleft cyst (RCC) is a rare occurrence of symptomatic bleeding or growth of a previously asymptomatic (and often undiagnosed) intrasellar cyst derived from remnants of Rathke’s pouch, situated on the midline between the adeno- and neurohypophysis. Symptoms may be identical to those of pituitary apoplexy: acute onset of headache, hypopituitarism, and neurological disturbances. Both syndromes may also exhibit a similar appearance of a large haemorrhagic sellar mass at initial radiological evaluation. We report on two patients who presented with headache and complete hypopituitarism. Based on the initial MRI, they were first diagnosed with pituitary apoplexy but managed conservatively with hormone therapy alone because of the absence of severe visual or neurological threat. Upon follow-up at 4 months, clinical evolution was good in both patients but their pituitary mass had not reduced in size and, after careful radiologic reviewing, was more indicative of a large midline complicated RCC. In conclusion, the diagnosis of complicated RCC is challenging because it can mimic pituitary apoplexy clinically, biologically, and radiologically. Clinicians should distinguish between the two entities using specific radiological signs or evolution of the mass at MRI if the patient does not undergo surgery. To our knowledge, we report conservative management of this rare condition for the first time, though it seems appropriate in the absence of neurological compromise or visual compression. Long-term follow-up is however mandatory.

Learning points

  • Complicated Rathke’s cleft cyst can mimic pituitary apoplexy, presenting with sudden onset of headache, hypopituitarism, and visual and neurological compromise in the most severe cases.

  • At diagnosis, pituitary MRI may not be able to differentiate between the two entities, showing a large haemorrhagic mass inside the sella, with little or no normal pituitary tissue visible. Patients are often diagnosed with apoplexy at this stage and may undergo pituitary surgery.

  • When surgery has not been performed initially in these patients, repeat imaging at 3–6 months is unchanged and does not show the expected involution usually seen after adenoma apoplexy.

  • Conservative management with hormonal replacement seems a valid option in the absence of visual or neurological deficits that would require trans-sphenoidal surgery.

Open access
N Viola Endocrinology Unit, Department of Clinical and Experimental Medicine

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C Urbani Endocrinology Unit, Department of Clinical and Experimental Medicine

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M Cosottini Neuroradiology, Department of Translational Research and New Technologies in Medicine and Surgery, University of Pisa, Pisa, Italy

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A Abruzzese Neuroradiology, Department of Translational Research and New Technologies in Medicine and Surgery, University of Pisa, Pisa, Italy

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L Manetti Endocrinology Unit, Department of Clinical and Experimental Medicine

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G Cosentino Endocrinology Unit, Department of Clinical and Experimental Medicine

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G Marconcini Endocrinology Unit, Department of Clinical and Experimental Medicine

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C Marcocci Endocrinology Unit, Department of Clinical and Experimental Medicine

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F Bogazzi Endocrinology Unit, Department of Clinical and Experimental Medicine

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I Lupi Endocrinology Unit, Department of Clinical and Experimental Medicine

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Summary

Pituitary apoplexy (PA) is a medical emergency with complex diagnosis and management. In this study, we describe a case of PA in a 63-year-old male treated with oral anticoagulant therapy for atrial fibrillation. In the patient, PA manifested itself with asthenia and severe headache not responsive to common analgesics. Despite the finding of a pituitary mass through CT, and in anticipation of the endocrinological evaluation and pituitary MRI, the patient’s clinical condition worsened with an escalation of headache and asthenia associated with deterioration of the visual field and impairment of consciousness level. The emergency assessments revealed an adrenal failure, whereas MRI showed a haemorrhagic pituitary macroadenoma with compression of the optic chiasm. Intravenous fluids repletion and high-dose hydrocortisone were started with a rapid improvement of the patient’s health and visual field abnormalities. Hydrocortisone was gradually reduced to a replacement dose. During the follow-up, panhypopituitarism was documented, and replacement therapies with l-thyroxine and testosterone were introduced. Three months later, a pituitary MRI showed a 50% reduction in the pituitary adenoma volume.

Learning points

  • Pituitary apoplexy (PA) is a medical emergency that can result in haemodynamic instability and abnormalities in the level of consciousness.

  • The management of PA requires a multidisciplinary team that includes endocrinologists, ophthalmologists, neuro-radiologists, and neuro-surgeons.

  • Pituitary MRI with gadolinium is the diagnostic gold standard for PA.

  • PA therapy aims to improve general conditions and treat compression symptoms, especially visual field abnormalities.

  • Adrenocorticotrophic hormone deficiency is a common and severe complication of PA. Thus, all patients with PA must be promptly treated with injective synthetic glucocorticoids (e.g. hydrocortisone 100 mg) and i.v. saline.

  • PA must be taken into consideration in case of sudden headache in patients with a pituitary macroadenoma, especially if other risk factors are recognized.

Open access