Endocrine Abstracts

Backgrounds: Preoperative diagnosis of parathyroid carcinoma (PC) is critical for the determination of the scope of surgical intervention. Nowadays, specific diagnostic markers for differentiation of PC and benign tumors are unknown, and less than half of patients with PC undergo necessary en bloc surgery.Aims: To develop the instrument for preoperative diagnosis of PC.Materials and methods: A multi-center retrospective study inclu...

Purpose: To establish the safety and efficacy of multikinase inhibitors (MKIs) treatment in real life.Methods: This retrospective observational descriptive study included patients with advanced differentiated thyroid cancer in treatment with MKIs as first and second line treatment. From November 2011 to May 2022. Clinical variables, efficacy and adverse events (AE) were collected. Variables are expressed as median and interquartile range.<p class="ab...

Introduction: Glucagonomas are rare functioning pancreatic neuroendocrine tumours that secrete glucagon. Gastrinomas are neuroendocrine tumours that secrete gastrin and are rarely located in the stomach. We report an extremely rare case of concomitant gastric gastrinoma and glucagonoma.Case presentation: A 64-year-old-man with a history of digestive haemorrhage and gastrectomy presented with weight loss, asthenia, anorexia and abdominal pain. Esophagogas...

Aim: Functional neuroendocrine tumors (NETs) represent a specific diagnostic and therapeutic challenge. Hormonal clinical syndrome can severely complicate clinical picture and affect treatment. Functional pancreatic NETs have been extensively studied. Our aim was to analyze functional lung NETs in terms of clinical characteristics and survival.Materials and methods: We retrospectively analyzed data from 230 patients with lung NETs treated at our departme...

Background: Parathyroid cancer (P?) in children is extremely rare, more often sporadic, but may be associated with a germline mutation. The only effective treatment is surgical approach due to the chemo-radio-resistance of PC.Clinical case: A 6-years-old girl presented with weakness, gait disturbance, pain in the limbs, an X-shaped curvature of the legs. At the age of 12, the patient’s well-being worsened sharply. The laboratory examination revealed...

Introduction: Over the last decades, there has been a significant increase in the incidence of thyroid cancer. Papillary thyroid cancer (PTC) is the most common histopathological type with often a favorable prognosis. Conventional therapies include surgical resection, adjuvant therapy with radioactive iodine, and suppressive therapy with thyroid hormones. However, the prognosis for iodine-resistant/refractory thyroid cancers or the advanced metastatic cancer is less favorable....

Multiple endocrine neoplasia (MEN) 1 is a rare hereditary autosomal dominant tumor syndrome characterized by two or more endocrine tumors. Non-endocrine neoplasms have been described as well. MEN1 is caused by inactivating mutations of the onco-suppressor gene MEN-1 (chromosome 11q13) which encodes the protein menin. Currently, 897 public variants of MEN-1 gene are reported. We present the case of a recently discovered pathogenic mutations of MEN-1 gene. A 32-year-old Italian ...

Background: MEN1-related primary hyperparathyroidism (PHPT) characterised by benign course, whereas malignant transformation is extremely rare. The diagnosis of parathyroid carcinoma (PC) within the MEN-1 syndrome is a challenge for a range of specialists.Clinical case: A 47-year Eastern European female. PHPT had been revealed during examination for multinodular goitre: PTH 614 nmol\l, calcium 3.06 mmol\l. Results of imaging methods were controversial (m...

Background: Adrenocortical carcinoma (ACC) is an uncommon endocrine malignancy, usually characterized by a late detection, aggressive clinical course, and poor outcome. The tumor microenvironment (TME) which includes infiltrating immune cells plays a critical role in tumor growth, survival, and prognosis in cancer patients. The presence of tumor-infiltrating immune cells (TIIC) affect the clinical benefit from novel strategies of immunological checkpoint blockade. Anti-immune ...

Introduction: Multiple endocrine neoplasia type 1 (MEN1) is an autosomal dominant disorder consisting in the development of primary hyperparathyroidism (PHPT), pancreatic neuroendocrine tumors, and pituitary adenomas. Occurrence of glucagonoma in MEN1 patients is rare (3%) and metastatic disease is present in 50% to 80% of patients at the time of diagnosis. The association of adrenal nodules/tumors and MEN1 is also rare.Case report: We present the case o...

Background: Alpelisib is an α-selective PI3K inhibitor indicated for the treatment of postmenopausal women and men with hormone receptor-positive (HR+), human epidermal growth receptor 2-negative (HER2–), PIK3CA-mutated locally advanced or metastatic breast cancer following disease progression or after endocrine therapy. Hyperglycemia is the most common adverse event (up to 60%) associated with its use. It occurs more frequently and lasts longer in patients with pred...

Introduction: Multiple endocrine neoplasia type 2A is an autosomal dominant disorder which is characterized by the occurrence of medullary thyroid carcinoma in association with pheochromocytoma and parathyroid tumors.Case report: We present the case of a 64-year-old woman, with familial history of thyroid carcinoma, renal lithiasis and personal history of right nephrectomy for renal lithiasis, who presented for the investigation of a left lateral cervica...

Introduction: Medullary thyroid carcinoma (MTC) is a neuroendocrine tumor that represents the most aggressive form of thyroid carcinoma, usually diagnosed at advanced stages. Serum calcitonin (CT) is the sensitive and specific marker of sporadic and hereditary CMT. It is a diagnostic, prognostic and follow-up marker.Purpose of the study: To investigate the correlation between the risk of metastasis and CT levels at the time of diagnosis of MTC.<p cla...

Background: The role of ferroptosis – a regulated form of cell death – in the pathophysiology of aldosterone-producing adenomas (APA) is unclear.Objective: To identify mechanisms of ferroptosis in human adrenal cells and translate these findings to APA pathophysiology.Methods: Cell death detection, lipid ROS generation and mRNA sequencing were performed on HAC15 cells treated with the specific ferroptosis inducer RSL3 (1,...

Background: Immune checkpoint inhibitors (ICIs) (anti-CTLA-4, PD-1 inhibitors, PD-L1 inhibitors) are considered a widely used therapeutic choice for several cancer types. However ICI therapy is frequently complicated by adverse events; endocrine toxicities are the most common ones.Methods: To assess in a retrospective observational study in a tertiary hospital the incidence and the characteristics of the immunotherapy-related endocrine adverse events (ir...

Introduction: Cushing syndrome (CS) is a rare and serious disease with high mortality. Diagnosis and management of the disease are difficult, as CS is typically characterized by the presence of multiple symptoms hypertension, diabetes, weight gain, or osteoporosis, the psychiatric and neurocognitive consequences are just as important by a set of anxio-depressive disorders resounding on the quality of life but also on the management of the disease.Objecti...

Background: Immune checkpoint inhibitors (ICI) are an innovative oncologic therapy used in several types of solid and hematological neoplasms. This treatment enables the antitumoral immune system response and results in significant improvement of clinical outcomes and survival rate. However, endocrine related adverse effects (ERAE) have been related, with thyroid and pituitary dysfunction being the most common endocrine diseases described. An association between the surge of e...

Neuroendocrine neoplasms (NENs) arise from specialized cells called neuroendocrine cells spread through the body, mainly in the gastrointestinal tract, pancreas and lung. Pathological classification of Lung NENs include well differentiated NENs, that can be classified as typical or atypical carcinoids, and poorly differentiated neuroendocrine carcinomas (NECs), classified as small-cell lung carcinoma (SC-NEC) or large-cell neuroendocrine carcinoma (LC-NEC). We present a case o...

Introduction: Neuroendocrine neoplasms (NENs) are rare heterogenous tumors which prevalence is increasing. Functional pancreatic neuroendocrine neoplasms (p-NENs) develop characteristic clinical syndromes in contrast to non-functional tumors that are diagnosed accidentally or at an advanced stage due to the lack of sensitive and specific biomarkers that could predict their clinical course.Aim: This study aimed to assess tumor markers (TMs) in patients wi...

Introduction: : Adrenocortical carcinoma (ACC) is a rare tumor, often with an unfavorable prognosis. The management of ACC is challenging due to its low frequency and limited experience. The objective of this study was to describe the adverse events (AE) in patients diagnosed with ACC treated in the Divisions of Endocrinology and Oncology at the Ramón y Cajal University Hospital (Madrid, Spain) in the last seven years.Methods: Records of patients wi...

Introduction: 177Lu-Oxodotreotide(PRRT-Lu) has the approval by EMA and FDA since 2017 for the treatment of patients with gastro-entero-pancreatic neuroendocrine tumors(GEP-NET). However, in daily clinical practice it has also been used in other NET tumors such as paragangliomas, lung NET, medullary thyroid cancer, and others. The aim of this study is to describe the characteristics and follow-up of patients treated with PRRT-Lu in a third level hospital in Spain fro...

Introduction: Paragangliomas are rare tumors originated from extraadrenals chromaffin cells. Most are sporadic, but between 30% and 50% are associated with hereditary syndromes. Mutations in the succinate gene dehydrogenase (SDH) have been identified as a cause of the hereditary paraganglioma-pheochromocytoma syndrome.Material and methods: Descriptive study of case series of patients belonging to the Southern Health Area of Seville with mutations in the ...

Case: A 59-year-old female with a past medical history of surgically treated primary hyperparathyroidism (PHPT) and breast cancer presented with irregular periods, post-menstrual bleeding, hot flushes, fatigue, and left loin pain. Furthermore, she reported weight gain of approximately 8 lb over 6 months and easy bruising in the absence of antiplatelet and anticoagulant therapy. There were no central, respiratory, or gastrointestinal symptoms. She was normotensive, and the clin...

Neuroendocrine tumors (NETs) of the breast are very rare. They represent less than 1% of breast carcinomas. It is important to differentiate metastatic neuroendocrine neoplasia from primary neuroendocrine neoplasia of the breast. Histopathological misinterpretation of a breast NET is common. In a study of 18 NET cases that metastasized to the breast, it was found that 62% of these tumors originated from the gastrointestinal tract and 28% from the lung. Forty-four percent of th...

Introduction: Insulinoma is the most common functional pancreatic neuroendocrine tumor (PanNET), accounting for more than 50% of all cases. Diagnosis of insulinomas may be challenging. In this study, we aim to discuss the clinicopathological features and long-term follow-up results of insulinoma cases followed up in our clinic.Material and Methods: It is a descriptive and retrospective study of 13 insulinoma cases diagnosed over 10 years. All data of the...

Introduction: Parathyroid carcinoma represents a very rare cause of primary hyperparathyroidism. It can be sporadic (most of cases), but also can occur in association with certain syndromes (multiple endocrine neoplasia, hyperparathyroidism-jaw tumor syndrome or isolated familial hyperparathyroidism).Case report: An 18-year-old woman was referred to the endocrine clinic because of a severe hypercalcemia, detected in the orthopedic surgery department, whe...

Introduction.: Parathyroid carcinoma (PC) is the one of the rarest endocrine malignances and occurs in 0.5–5% of cases among patients with primary hyperparathyroidism (PHPT). Diagnosis of PC can be challenging and often made after surgery or while local or distant metastases detection. We present a case of a patient with a large intrathyroid PC, which was initially identified as a malignant thyroid nodule.Case report.: 59-old caucasian women was dia...

Background: Progressive hirsutism and moderate to severe male-pattern balding in women requires exclusion of an adrenal or ovarian tumor.Case Presentation: A 51-year-old lady presented with excessive hair on her face and lower abdomen of 1 year duration which affected her quality of life. Her menopause started 3 years ago. Her body mass index was 28.6 kg/m2. She had hair on her upper lip, chin, and lower abdomen; she had a Ferriman–Gallwe...

Adrenocortical carcinoma (ACC) is a rare malignant tumour arising from the adrenal cortex, with an estimated annual incidence of one to two patients per million. Ectopic ACCs are extremely rare. They are believed to arise from cortical fragments arrested during embryologic migration and have been found close to the adrenal gland or along the path of gonadal descent. The majority of ACCs are sporadic, however, ACC has been linked with genetic disease processes, including multip...

Mesenteric paragangliomas are a rare entity; consequently, 20 cases have been reported to date. Although often found incidentally and considered benign, they have potential to metastasize. We present a case of 63-year old man with a 11-year history of the mesenteric paraganglioma. The lesion for the first time had been described on CT scan of the abdomen in 2010. However, the patient was lost to follow-up. In 2021 CT scan, followed by MRI of the abdomen, perfomed due to reccur...

IntroductionMixed neuroendocrine-non-neuroendocrine neoplasms (MiNEN) of the pancreas are composed of two morphologically different components, each representing at least 30% of the tumor: ductal or acinar adenocarcinoma and neuroendocrine neoplasm (NEN). They are extremely rare tumors whose etiopathogenesis and biological behavior are not fully understood. Given its rarity, we describe a case of insulinoma in the context of MiNEN....

Introduction: The incidence of thyroid cancers is increasing; they are considered to have a very good prognosis with good survival The frequency of metastases is 5 to 10%. The most common locations are lung and bone. The morbidity of metastatic disease is related to the initial histological type, tumour mass, location of metastases, age, possible loss of iodine uptake and 18F-FDG uptakeMaterials and methods: We collected 25 patients treated for hormone-d...

Introduction: An increased Body Mass Index (BMI) has been associated with higher prevalence of many cancers, including differentiated thyroid cancer. However, no data have been reported about potential impact of BMI on aggressiveness of presentation and clinical outcome of medullary thyroid cancer (MTC).Aim: To evaluate the potential influence of BMI on clinical presentation and outcome of MTC.Methods: We reviewed anthropometric an...

Multiple endocrine neoplasia type 1 (MEN1) is an autosomal dominant disorder caused by germline mutations of MEN1 gene, without genotype–phenotype correlation. It is defined as the occurrence of two or more primary neuroendocrine tumors (parathyroid, enteropancreatic, pituitary), or the occurrence of one of the MEN1-associated tumors in family members of a patient with a clinical diagnosis of MEN1. Multiple parathyroid tumors with hyperparathyroidism are the most common m...

Introduction: Although ectopic ACTH syndrome (EAS) is most commonly seen in cancers with neuroendocrine differentiation, it is not a common condition in germ cell tumors. Here we report a rare case of EAS due to metastatic germ cell tumor.Case report: A 53-year-old male patient presented with fatigue, weakness, swelling in both legs and weight loss for 3 months. He had type 2 diabetes and no history of smoking or alcohol. In the physical examination, the...

Background: Neuroendocrine tumors (NETs) and Adrenocortical carcinomas (ACCs) are rare and heterogeneous tumors entities. Advanced NETs and ACC own poor prognosis with less than 40% of five-year survival. There is a need for improved biomarkers to predict disease prognosis and treatment sensitivity. A broad overview of NETs and ACC genetics has never been explored.Aim: To investigate genotype–phenotype correlations on a pan-endocrine cancer level wi...

Multiple endocrine neoplasia type 1 (MEN-1) is characterized by an increased predisposition to the development of tumors of the endocrine tissues, such as parathyroid glands, anterior pituitary, and duodenopancreatic neuroendocrine tumors. It is an autosomal dominant disorder due to germline mutations in the MEN-1 tumor suppressor gene. This gene encodes the menin protein, which is involved in cell growth and differentiation, and in sensing or repairing DNA damage. We present ...

Introduction: PRES it’s a neurologic syndrome with multiple manifestations, that includes high blood pressure with a variable range. It usually develops in the context of medications or an underlying condition. The treatment is usually symptomatic and determined by said disease.Objective: We present the case of a patient with PRES linked to the diagnostic of a metastatic PHEO.Case-report: A 72-year-old male underwent left adre...

Background: Adrenocortical cancer (ACC) therapy is characterized by insufficient effectiveness. Currently, mitotane, an adrenolytic drug, is the only drug approved for treatment of ACC and is used in the adjuvant setting and in case of metastatic or advanced disease. However, the administration of mitotane to certain groups of patients remains controversial due to the low response rates, high toxicity and limited data on the benefit of treatment. Expression levels of the large...

Introduction: Merkel cell carcinoma is a rare and aggressive neuroendocrine skin cancer. It is characterized by a high rate of local recurrence after surgical resection as well as the occurrence of distant metastases. It mostly occurs in the elderly and the exact etiology is unknown. The most significant risk factors for its occurrence are exposure to ultraviolet light and the oncogenic effect of the Merkel cell polyoma virus.Aim: Demographic and clinica...

Introduction: Malignant struma ovari is a rare ovarian mature teratoma. The diagnosis can be done histopathologically. There are no specific diagnostic findings in terms of imaging, clinical and laboratory. We present two cases of malignant struma ovarii in papillary follicular variant subtypes in this report.Case report: Case 1; The patient was a 44 year old female, she had complaints of pelvic pain and a palpable lower abdominal mass. Pelvic MRI examin...

Introduction: Poorly differentiated thyroid cancer (PDTC) is a rare subtype of thyroid carcinomas, that diagnosis is difficult and often missed in daily routine. It is believed that most of them arise in well-differentiated thyroid carcinoma, although a subset of these lesions apparently also arise de novo.Case report: In 2007, a 55-year-old man had a total thyroidectomy and received radioactive iodine treatment post-surgery due to well-differentiated pa...

78-year old previously healthy man was reffered to Outpatient endocrinology clinic due to painful spine and shoulders. His lumbar and thoracic X-ray showed decreased lumbar and thoracic vertebrae (L1, L2 and Th 5) and densitometry indicated slightly decreased bone density with T score -1,0 in left femoral neck and L1 vertebra. Since patient mentioned recent unintentionally 10 kg weight loss he was processed to further work-up and additional blood tests were done. Secondary ost...

Liver neuroendocrine carcinoma is a relatively rare disease. There is lack of description due to prognostic information of the disease. Liver serves as an visceral organ, taking part in several endocrine functions, according to an investigations has produced specific proteins and activate thyroid hormones, glucocorticoids. This article describes clinical case of 54 years man with clinical diagnosis of Grave’s disease. Liver examination showed normal liver hormones, radiol...

Craniopharyngiomas (CP) are congenital, benign, embryonic malformations which arise from ectoblastic remnants of Rathke’s pouch and are located in the (supra-)sellar region. Many patients show a reduced growth rate and an increased BMI early before the diagnosis of CP. However, it is unknown whether patients with CP present with increased head circumference before diagnosis. A cohort of 90 patients was screened for medical records of their developmental monitoring visits....

Background: Pheochromocytomas and paragangliomas (PPGLs) are a group of rare neuroendocrine tumors. Dysglycemia has been observed in patients with PPGLs in some small case series. However, few large studies, and none in China, have described the outcomes of dysglycemia after resection, and the factors associated with the development and resolution of dysglycemia in patients with PPGLs.Methods: We retrospectively analyzed the clinical data of consecutive ...

Background: Insulinoma is a rare variety of endocrine neoplasm and is usually benign, solitary, and small in size. It is responsible of endogenous insulin secretion resulting in development of symptoms of hypoglycemia.Case presentation: We report 4 cases of insulinoma that were diagnosed and managed in the year 2022. Three women and one man aged respectively 76, 68, 60 and 42 years-old. All patients presented a long history of various adrenergic symptoms...

Large adrenal masses are strongly suggestive of malignancy. Nevertheless, bilateral adrenal masses are less common (around 20% of incidental adrenal lesions). Metastases and lymphoma are more frequent (especially when aging), and not so easy to differentiate on CT or MRI. Simple biological markers, such as plasma LDH or beta2microglobulin, could help.Case report: A 69-year-old patient was admitted for abdominal pain with progressive lethargy. Abdominal C...

Introduction: Biermer’s disease is a chronic inflammatory disease due to antibodies targeting parietal cells and intrinsic factor compromising vitamin B12 absorption leading to pernicious anemia, and gastric acid secretion leading to achlorhydria. Neuroendocrine tumors are rare neoplasms that have been reported during the evolution of chronic gastritis. We here describe a case of a type 1 gastric neuroendocrine tumor discovered alongside with Biermer’s disease.<p...

Medullary thyroid carcinoma (MTC) accounts for 1%–2% of thyroid cancers and has a variable clinical course. MTCs present with locoregional metastasis in 50% of patients and distant metastasis in 10% to 15% at the time of initial diagnosis. The lungs, liver, and bone are common metastatic sites, and distant metastasis is known to be a poor prognostic factor for long-term oncologic outcomes. We present the long course case, under somatostatin analogues (SSA) therapy, of a 7...

Thyroid cancer is the most common endocrine malignancy, and accounts for between 5 and 10% of thyroid nodules biopsied in endocrine services. As per the Global Cancer Observatory, the incidence of thyroid cancer in Bahrain is 2.12 cases per 100 000 people, which is less than the neighbouring countries of Saudi Arabia and Kuwait. The purpose of the study is to investigate the relationship between thyroid cancer and obesity in Bahrain, where 28.9% of males and 38.2% of females a...

Introduction: Angiosarcomas are aggressive malignant tumors but very rare especially when they are located in the thyroid, characterized histologically by their vascular differentiation and their polymorphic appearance.Observation: We report the case of a 68-years-old female patient, from a mountainous region who has a history of cholecystectomy in 2018, she underwent a total thyroidectomy for a compressive goiter, the postoperative period was marked by ...

Introdutcion: In recent years, thyroid microcarcinoma (TMC) predominates in the context of diagnosis and treatment of thyroid cancer. TMC has an excellent prognosis, its management is changed in the last few years, reducing surgical procedure and role of radioiodine ablation (RAI). Therefore, the question is what tests have a diagnostic value in patients with thyroid microcarcinoma after thyroidectomyThe aim of this study was to evaluate the role of 131 ...

Introduction: Primary thyroid non-Hodgkin’s lymphoma (PT-NHL), is a quite rare entity, accounting for 1.3–1.5% of thyroid neoplasm, and 0.5% of lymphoma. The extranodal marginal zone B-cell lymphoma of MALT, occurs in 23% of PTL. The aim of our work is to evaluate the clinicopathological features and treatment outcomes of patients with MALT primary thyroid lymphoma. We report two cases of MALT PTL taken care of in our department.Observation 1: ...

Objective: To study the carcinological factors associated with adrenal metastases (AMT) in bronchopulmonary cancer (PBC).Patients and Methods Case–control study analysing oncological data of 40 patients with PBC monitored by 18DFG PET/CT at the Nuclear Medicine Centre of Sfax, Tunisia. We compare the characteristics of 2 subgroups:G1 (n=17): patients with PBC and META (cases)G2 (n=23): ...

Craniopharyngiomas are rare, embryonic, malformational tumors of the (supra-)sellar region. Due to tumor and/or treatment-related hypothalamic lesions, patients develop morbid obesity. As a major vascular risk factor, obesity leads to reduced 20-year overall survival and a 3- to 19-fold higher cardiovascular mortality after craniopharyngioma compared to the general population. This review studies craniopharyngioma-specific risk factors for vascular damages. Three databases (Pu...

Introduction: Multiple endocrine neoplasia type 1 (MEN1) is a rare, heritable syndrome characterized by a hyperparathyroidism, pituitary adenoma and pancreatic islet cells.Case: A 56-year-old male patient had a single parathyroid excised for primary hyperparathyroidism 10 years ago. He was further investigated due to hypoglycemia and a mass was found in the head of the pancreas on MRI. It was reported as neuroendocrine tumor and no recurrence was found i...

Introduction: Ectopic ACTH syndromes account for 5–10% of Cushing syndrome, with intrathoracic and gastrointestinal–pancreatic neuroendocrine tumors being the most common causes, followed by small cell lung cancer, adrenal paraganglioma and medullary thyroid carcinoma.Case report: A 50-year-old male hypertensive and diabetic patient was transferred to our clinic for emergency endocrine care for hypokalemia. He had mild hypercortisolism and live...

Primary vaginal malignancy is uncommon, accounting for about 2% of all gynecological tract cancers, with small-cell neuroendocrine carcinoma of the vagina being exceptionally rare and aggressive. They display similar histological and immunohistochemical features to small-cell carcinomas of different origin. Few cases are reported in the literature so far and therefore, there is no current treatment protocol consensus. We report the case of a 34-year-old nulliparous patient, wh...

Introduction: Multiple endocrine neoplasia (MEN) is a group of inherited syndromes of autosomal dominant transmission characterized by the development of endocrine tumors.Materials and methods: It is a retrospective study collecting data of 9 patients with MEN, during a 30-year period.Results: The mean age at diagnosis was 34.6 years with a clear female predominance. The majority of our patients were from Gabes (4 cases). Seven pat...

Introduction: Neuroendocrine tumors of head and neck region are rare and heterogeneous. it can arise in all the different organs of this region, including the nasal cavity, paranasal sinuses, larynx, thyroid, can be divided into two broad groups: those with epithelial differentiation, and neurally derived tumours, including paragangliomas and olfactory neuroblastomas. Their morphological and clinical features mainly depend on the degree of differentiation and on the site of or...

Parathyroid cancer is a rare cause of primary hyperparathyroidism (PPH). The objective of this work is to discuss clinical and therapeutic specificities of these tumors.Case n°151-year-old woman from a consanguineous marriage, with a family history of recurrent renal lithiasis, neoplasia, and a personal history of breast neoplasia and uterine fibroid, consulted for abdominal pain, vomiting and weight loss. The diagnosis of pri...

Introduction: Paragangliomas are a diverse group of neuroendocrine tumors arising from chromaffin cells within paraganglionic tissues of the autonomic nervous system.The aim of this study is to review our experience in the management of these tumors.Methods: The medical records of 12 patients with 13 paraganliomas was performed in a a single academic hospital over a period of 32 years from 1990 to 2022.Result...

Introduction: Olfactory neuroblastoma causing syndrome of inappropriate antidiuretic hormone (SIADH) as paraneoplastic syndrome is very rare. There have been only 10 cases with antidiuretic hormone (ADH) producing olfactory neuroblastoma in the literature. The syndrome is due to ectopic production of protein hormones, hormone precursors, or hormone-like substances by the tumor tissue. Here, we report the case of olfactory neuroblastoma associated with SIADH.<p class="abste...

The aim of this study is to report a case of an unusual presentation of anaplastic thyroid carcinoma and describe its clinical, radiological and management particularities. Observation: We report the case of a 60 year-old female patient who presented with a two-week history of progressive aphagia, associated with hemoptysis. She also described recent shortness of breath, and a painless cervical mass evolving for 10 months, rapidly enlarging during the last four weeks. Her medi...

Introduction: Regardless of the fact that the incidence of thyroid cancer has increased significantly in recent years, the simultaneous occurrence of multiple tumors in one thyroid gland is still a rare phenomenon. These tumors have the morphology, either of a single mass with mixed histological features, or of a single lesion consisting of two distinct cell-populations, or of two different tumors between which normal thyroid tissue is interposed. Various theories have been pr...

Introduction: Ectopic thyroid tissue is a rare developmental abnormality due to aberrant embryogenesis and/or migration of the thyroid gland. Although rare (<1%), papillary thyroid carcinoma is the most common malignant transformation.Aim: Report a case of Tall cell variant of papillary carcinoma occurring on ectopic thyroid tissue in the thyroglossal duct.Observation: A 56-year-old male patient consulted for a cervical mass ap...

Neuroendocrine neoplasms (NENs) are rare but diverse group of malignancies that can arise from gastroenteropancreatic tract and lungs. NENs can be clinically symptomatic (functioning) or silent (non-functioning). We present a case found to have multiple NENs in the absence of known clinical syndrome and discuss role of genetic testing versus clinical screening. 54 Female, referred to endocrine team for investigation of hypoglycaemic symptoms and suspected insulinoma. Routine b...

Background: Insulinoma is a rare neuroendocrine tumor that causes inappropriate secretion of insulin resulting in hypoglycemia. The diagnosis of insulinoma may be difficult. Recurrence after surgery is rare and it usually concerns tumors with high histological grade.Case presentation: We report a case of a 52-year-old woman with recurrent episodes of symptomatic hypoglycemia. The laboratory investigations showed high insulin (20 IU/ml) and C-peptide leve...

Introduction: The insulinoma is a rare neuroendocrine tumor derived from the beta islet cell of the pancreas. It is usually sporadic and benign in 90% of the cases. It occurs more often in women in their fifties.Observation: We report the case of 76-year-old women with a medical history of hypertension and dyslipidemia, who was transferred to our department from the emergency unit after a diagnosis of severe episode of hypoglycemia (glucose level at 0.2 ...

Background: While the classic presentation of pheochromocytoma includes a triad of episodic headaches (90%), diaphoresis (60–70%), and palpitations (70%), literature has suggested 10% to 17% of patients with pheochromocytoma are either asymptomatic or mildly symptomatic; thus, highlighting the importance of appropriate endocrine work up of adrenal incidentalomas.Case: We report a 37-year-old male with a long-standing history of nocturia and dysuria,...

Introduction: Insulinomas are the most common cause of hypoglycemia due to endogenous hyperinsulinism. Almost all insulinomas are benign, and long-term recovery with complete disappearance of preoperative symptoms can be expected after complete resection.Case report: A 33-year-old woman, who developed gestational diabetes during her second pregnancy, which persisted post-partum, stopped insulin due to recurrent severe hypoglycemia, but which persisted ev...

Introduction: Acromegaly is an unusual endocrinopathy due to hypersecretion of growth hormone (GH) and concomitant rise of IGF-1. It is associated with premature of mortality caused by cardiovascular, respiratory and metabolic diseases, as well as increased risk of developing boh benign and malignant tumours. We report the case of a patient with acromegaly associated with papillary thyroid microcarcinoma (PTC).CASEA 50-years -old m...

Introduction: RET proto-oncogene is situated on chromosome 10 and encodes a tyrosine kinase receptor. A germline mutation of RET leads to the appearance of MEN 2 syndrome or familial medullary thyroid carcinoma (MTC). One of the most common germline mutation occurs in codon 634. Pheochromocytoma penetrance for codon 634 mutation was demonstrated to increase with age up to 88% by age 77 years.Aim: To present two familial cases of RET mutation illustrating...

Background: Medullary thyroid carcinoma (MTC) is a rare neuroendocine tumor that that may be associated with paraneoplastic ACTH-dependent cushing syndrome. There are few case reports on the coexistence of medullary thyroid carcinoma and adrenal adenoma with ACTH-independent Cushing syndrome.Case presentation: We report the case of a 42-year-old woman, with no family history of endocrine malignancy or endocrine disorders. She underwent total thyroidectom...

Introduction: The incidence of cancer has increased in case of diabetes, as well as the metabolic abnormalities observed during diabetes could have a critical role on carcinogen, nevertheless one should not deny the implication of common factors to diabetes and cancer (obesity, smoking, advanced age, etc).Study objectives: Describe the biological and morphological screening methods for neoplasia in the event of weight loss concomitant with chronic glycem...

Background: Adrenocortical carcinoma is a rare malignancy with a poor prognosis. Local recurrence is affected by the pathologic features (tumor staging and mitotic index) and the complete surgical resection. Treatment of a recurrent adrenocortical carcinoma can be a real challenge.Case description: We report a case of 47-year-old man who was operated for an adrenal tumor with bone metastases. Left adrenalectomy and splenectomy were performed and histolog...

Introduction: Sinonasal neuroendocrine carcinoma SNECs are a rare group of neoplasms that account for only 5% of all sinonasal malignancies. SNECs are categorized by their differentiation grade into well-, moderately- and poorly differentiated.Case report: We describe a classical case of SNEC with secondary orbital involvement in a 34-year-old male patient presented of the occurrence of a right epistaxis. First of all, the patient is smoking. The beginni...

Introduction: Digestive neuroendocrine tumors (DNETs) represent a heterogeneous group of tumors. This heterogeneity is manifested by the variable localization of these tumors but also by a great clinical and biological diversity. Thus, they occupy an increasingly important place in digestive oncology.NETs are very rare and represent about 1% of all digestive tumors, due to their slow evolution. The majority of them develop in the digestive tract (67.5%) ...

Introduction: Clear cell renal carcinoma (ccRCC) is a subtype of renal cell carcinoma, the most frequent one (80%) and usually affecting only one kidney. It is twice as common in men than women between 50 to 70 years old. It can be associated with some genetic conditions that predispose patients to ccRCC, like Von Hipple-Lindau disease. Some clinical observations and molecular studies raised the hypothesis that RCC is a hormone−dependent tumor.Case...