Giant Cystic Pheochromocytoma Associated With Neurofibromatosis Type 1: A Case Report.

Pheochromocytomas are tumors that develop from the chromaffin cells of the adrenal medulla. More than 40% of cases of pheochromocytomas are associated with genetic conditions such as neurofibromatosis type 1 (NF1) or von Hippel-Lindau syndrome. Cystic pheochromocytomas are rare, generally asymptomatic, and thus of bigger size at the time of diagnosis. Surgical treatment is necessary to prevent cardiovascular morbidity and malignancy risk. We report the case of a 27-year-old patient admitted for further examination of a left adrenal mass that was discovered by an abdominal CT scan in the context of abdominal pain associated with hypertension evolving for three years. The clinical examination showed the presence of multiple café au lait spots, axillary and inguinal freckling with two dermal neurofibromas diagnosed clinically, as well as Lisch nodules on bilateral ophthalmic examination, thus meeting the clinical criteria for the diagnosis of NF1. The clinical laboratory investigation showed elevated urinary metanephrine and normetanephrine levels. CT scan examination showed a 10 cm left adrenal cystic mass on abdominal CT. This mass uptake of the radioligand in metaiodobenzylguanidine (MIBG) scintigraphy without secondary extra-adrenal localization allowed the diagnosis of a seemingly benign cystic pheochromocytoma to be made. The patient was put on presurgical drug preparation with volume expansion and then underwent left unilateral adrenalectomy. The histopathological study was in favor of a rather aggressive cystic pheochromocytoma with a pheochromocytoma of the adrenal gland scaled (PASS) score of 9. Blood pressure and urine catecholamines at seven days, three months, six months, and one year after surgery were normalized. Cystic pheochromocytoma is a rare tumor with a potentially poor prognosis. It is characterized by a more insidious evolution and a larger volume at diagnosis. It should be considered a diagnosis in patients with a cystic adrenal mass or an extra-adrenal mass with fluctuating blood pressure during surgery. This case illustrates the importance of both presurgical preparation and screening for pheochromocytoma in neurofibromatosis type 1.

Tamoxifen- and Triptorelin-Induced Major Hypertriglyceridemia: A Case Report.

Tamoxifen, a selective estrogen receptor modulator (SERM), can have harmful side effects, such as hypertriglyceridemia, which can lead to acute pancreatitis. Meanwhile, triptorelin is an analog of natural GnRH (GnRHa), which may cause a small but significant increase in cholesterol and triglyceride (TG) levels. We describe below the case of a patient with breast cancer treated with Patey's operation, chemo-radiotherapy, and then with tamoxifen and triptorelin. After an exposure period of three months, she presented major hypertriglyceridemia at 56 g/L, total cholesterol at 13 g/L, LDL-cholesterol (LDL-C) at 4 g/L, and HDL at 0.25 g/L. The patient's treatment was stopped by her oncologist. One month after starting an adapted diet and fenofibrate, her TG levels were reduced to 2 g/L. We could confirm from these results that tamoxifen and triptorelin certainly modify lipid metabolism, hence the interest in evaluating the benefit-risk balance and regularly monitoring the lipid profile in order to avoid any fatal complication.

Adrenocortical Sarcomatoid Carcinoma Revealed by an Adrenal Incidentaloma: A Case Report.

Sarcomatoid carcinoma of the adrenal gland represents an exceedingly unusual and highly aggressive form of adrenocortical carcinoma. Its diagnosis is challenging because of its dual histological components: epithelial and sarcomatoid. Most patients are diagnosed at a late stage and die within months of diagnosis. We report on a 51-year-old man who had adrenocortical sarcomatoid cancer. It was diagnosed as a unilateral left adrenal incidentaloma discovered on a CT scan carried out for abdominal pain. By means of this case, we will present the clinical, radiological, and histological profile of this tumor.

Pheochromocytoma in an Ectopic Adrenal Gland.

A pheochromocytoma is an uncommon tumor that originates from the chromaffin cells of the adrenal medulla. Also, adrenal tissue not located in its typical position is referred to as ectopic adrenal tissue. It is relatively uncommon in adults and is usually asymptomatic. Therefore, a pheochromocytoma arising from ectopic adrenal tissue is even a rarer finding and presents as a unique diagnostic challenge. A 20-year-old man presented with vague abdominal pain, and upon imaging, a mass located behind the liver was initially discovered. Subsequently, it was identified as a mass growing in an ectopic adrenal gland. He underwent exploratory laparotomy and resection of the mass. A pheochromocytoma in an ectopic adrenal gland was confirmed by histopathology.

Rare Cases of Hibernomas Associated With Bilateral Pheochromocytoma: A Report of Two Cases.

Hibernoma is an infrequent benign lipomatous tumor that shows differentiation to brown fat. It is a slowly growing tumor of variable consistency but often firmer than a classic lipoma, mobile, and rarely infiltrating. To date, there are only a few cases of adrenal hibernoma in the literature. Herein, we report two cases. The first one is that of a 24-year-old female presenting with a bilateral adrenal incidentaloma discovered in the setting of abdominal pain whose histological study individualized a bilateral pheochromocytoma associated with a hibernoma. The second case is that of a young male of 23 years old. He was operated on in adolescence (age 14) for a left pheochromocytoma. The outcome was marked by the recurrence of the Menards triad ("headache, palpitations, and sweating") and hypertension at the age of 23 years. It was due to the appearance of a contralateral right pheochromocytoma, whose histological study showed an association with a hibernoma, and the genetic study revealed Von Hippel-Lindau (VHL) disease.

Oncocytic Tumors: An Uncommon Benign Adrenal Gland Lesions With Features of Malignancy.

Oncocytic cell neoplasms are usually found in the thyroid or salivary glands and the kidneys. Adrenal oncocytoma (AO) is an extremely rare localization. It is often non-functional and the suspicion of malignancy is considered when the size of an adrenal incidentaloma is greater than 4 cm. These adrenal oncocytomas, however, are large, round, and encapsulated with a benign presentation and evolution. We report on a 40-year-old male patient. Upon suspicion of a SARS-CoV-2 infection, the general physician instructed a chest CT scan, which, fortuitously, revealed a suspicious left adrenal lesion measuring 62x45mm. A biochemical investigation was negative for either pheochromocytoma or Cushing's syndrome, allowing the recommended surgery to be performed. The anatomopathological analysis showed an uncommon benign adrenal lesion, an adrenal oncocytoma.

Prolactinomas in Moroccan Population: Clinical, Paraclinical, Therapeutic, and Evolutionary Aspects.

Prolactinomas are usually benign tumors and are the most common type of secretory adenomas. Their diagnosis is well coded, and their severity depends on the tumor size identified by magnetic resonance imaging. The aim of this work is to study the epidemiological, clinical, paraclinical, and therapeutic profile of prolactinomas through a retrospective and descriptive study of 95 cases of prolactinomas conducted at the Endocrinology, Diabetology and Nutrition Department of the Hassan II University Hospital of Fez between January 2015 and November 2020. The mean age of patients in our series was 33.8 years (14-63) with a clear female predominance of 88.4%. Based on adenoma size, 37 cases were microprolactinomas and 58 cases were macroprolactinomas representing 38.9% and 61.1% respectively. Menstrual disorders were present in 50.5% of the women, and 68.4% had galactorrhea, while 44.2% of patients complained about local mass effects, especially headaches. The average size of adenomas was 15.7 mm (4-70 mm). The mean initial plasma prolactin (PRL) level was 423.2 ng/mL (103-7,663 ng/mL). Of our patients, 97.9% received medical therapy with dopamine agonists, particularly with cabergoline which represents the mainstay of therapy (85.3%), while 2.1% of patients underwent surgery due to resistance to treatment. In terms of evolution after treatment, three cases of resistance to treatment were reported. Thirty patients with microprolactinomas had a normalization of prolactin level within an average of five months, and we saw a regression of the size of the adenoma in 43.2% of patients, stabilization in 24.3% and a progression of the process in 5.4% of cases. Although in macroprolactinomas we found a normalization of prolactin level in 43 patients within an average of one year, the tumor syndrome disappeared in 31.6% of cases. Radiological monitoring also revealed a favorable tumor response in 82.7% of patients.

A Clinical, Etiological, and Therapeutic Profile of Gynecomastia.

Background and objective Gynecomastia is defined as a benign proliferation of male breast glandular tissue, either unilateral or bilateral, resulting from an imbalance of testosterone and estrogen. In this study, we aimed to describe the clinical, etiological, and therapeutic aspects of gynecomastia. Materials and methods A retrospective, descriptive study was conducted in the Department of Endocrinology, Diabetology, and Nutrition at the Hassan II University Hospital in Fez, Morocco, over a period of 10 years. We included all patients admitted for exploration and treatment of gynecomastia. The data were analyzed using Microsoft Excel 2016 and SPSS Statistics version 18 (IBM, Armonk, NY). Results A total of 86 patients were included in this study; the mean age of the patients was 33 years (range: 15-86 years). A family history of gynecomastia was found in 4.6%. Isolated gynecomastia was the most frequent symptom (60.4% of cases). It was bilateral in 54% of cases, stage II in 63% of patients, stage I in 17%, and stage III in 20%. The first-line assessment (renal insufficiency, hepatic insufficiency/cirrhosis, dysthyroidism) was normal in the majority of cases. The etiologies were dominated by hypogonadism in 32.6% of cases, pubertal gynecomastia in 21%, and senile gynecomastia in 8.1%. Regarding treatment, 42% of patients received an etiological treatment, while surgical treatment was provided in 28% of cases, observation in 15% of cases, and androgen therapy in 15%. Pathological examination of all surgical specimens was benign. The follow-up was marked by 30.3% of static gynecomastia, 29% of regression, 17.5% of good response after surgery, and 24.4% of treatment refusal. Conclusions It is important to adopt a step-by-step approach in treating gynecomastia, starting with detailed questioning and clinical examination. The surgical treatment is currently the treatment of choice, the final goal of which is good aesthetic as well as psychological outcomes.

Adrenal Ganglioneuroma: A Case Report.

Adrenal ganglioneuroma (AGN) are sympathetic differentiated tumors that originate from neural crest cells. It is a rare benign tumor in children and young adults. These lesions are usually asymptomatic and tend to be hormonally silent. Their discovery is fortuitous in imaging examinations. Preoperative diagnosis remains difficult, and the gold standard treatment is adrenalectomy. There is a good prognosis after surgery without recurrence. We herein report a case of adrenal ganglioneuroma in a 40-year-old man who benefited from an abdominal CT scan in the face of a complaint of abdominal discomfort and as part of the extension assessment of his colonic adenocarcinoma. Abdominal CT scan with contrast showed a left retroperitoneal mass of triangular shape within the adrenal lodge of tissue density, containing some calcifications not enhanced after injection of contrast product, measuring 90 x 62 mm in diameter (AP x T) with a relative washout calculated at 30%. Biopsy and histological examination of the mass suggested an adrenal ganglioneuroma.

Noradrenergic Pheochromocytoma: A Case Report.

Pheochromocytomas and paragangliomas are rare neuroendocrine tumors developed from chromaffin cells. They are exceptional in children with an atypical symptomatology. We report here a case of a 17-year-old boy who presented with a left retroperitoneal mass discovered on thoracic-abdominal-pelvic computed tomography (CT) scan in the presence of diffuse abdominal pain, more pronounced in the left hypochondrium. The exploration had objectified an exclusive secretion of urinary normetanephrine over 24 hours. Metanephrine and 3 ortho methyldopamine were within normal limits. He had adrenalectomy after controlling his blood pressure with an alpha blocker. Histology had confirmed a pheochromocytoma of non-aggressive potential Pheochromocytoma of the Adrenal gland Scaled Score (PASS) 2. The evolution was favorable with normalization of blood pressure and urinary catecholamines at one week, three months, six months, and one year. He tested negative for hereditary syndromic including Von Hippel-Lindau (VHL), RET genes, subject to the succinate dehydrogenase complex B and D subunit genes (SDHB-D), which have been requested.

Reversible Gynecomastia in HIV-Infected Man Treated With Triple Antiretroviral Therapy Containing Efavirenz: A Case Report.

Gynecomastia is benign hypertrophy of male breast glandular tissue, either unilateral or bilateral, secondary to increased estrogen/testosterone ratio (elevated estrogen level, decreased testosterone levels, or both). The condition can be related to a medical disease or caused by some drugs. Since the introduction of triple antiretroviral therapy (TAT), we have seen an improvement in the prognosis of human immunodeficiency virus (HIV) infection. Here we report the case of a 53-year-old man receiving follow-up care in Internal Medicine for HIV infection receiving TAT (tenofovir/efavirenz/emtricitabine). After one year, the patient presented in the Department of Endocrinology, Diabetology, Metabolic Diseases, and Nutrition of Hassan II University Hospital Center, Fez, with bilateral gynecomastia. Hormonal exploration did not reveal any abnormality, so the gynecomastia was attributed to efavirenz use. The regimen was replaced by tenofovir, lamivudine, and dolutegravir. The gynecomastia was resolved within two months of discontinuing efavirenz. In summary, we think that secondary gynecomastia should be suspected and screened in HIV patients receiving efavirenz-containing regimens.

A Case of Acute Adrenal Insufficiency: A Rare but Possible Cause of Hypercalcemia.

A 30-year-old woman presented with a 15-day history of epigastric pain, nausea, vomiting, asthenia, and weight loss. On admission, hypercalcemia was reported with a negative etiologic workup (including no primary hyperparathyroidism, malignancy, or vitamin D toxicity). Fluid replacement did not improve her hypercalcemia. We performed a blood ionogram and assessed the adrenocortical function, which showed that her serum cortisol was decreased, her adrenocorticotropic hormone (ACTH) was elevated, and anti-21 hydroxylase antibodies were positive. We established the diagnosis of autoimmune primary acute adrenal insufficiency. The patient was treated with hydrocortisone. Shortly after initiating the treatment, her serum calcium levels returned to normal and her symptoms improved. This case report highlights the fact that even though adrenal insufficiency is an uncommon etiology of hypercalcemia, it should not be totally ignored, especially since hypercalcemia can sometimes be indicative of adrenal impairment.

The Etiological Profile of Adrenal Incidentalomas.

Introduction An adrenal incidentaloma (AI) is an unsuspected tumor in one or both adrenal glands, which is discovered incidentally on an imaging exam not prompted by adrenal exploration. The etiologies can be multiple; they condition therapeutic management. The objective of our study is to describe the etiological and therapeutic profiles of AI in our department. Materials and methods A retrospective study was carried out in the Endocrinology, Diabetology, and Nutrition Department of the Hassan II University Hospital of Fez on patients managed for AI from September 2009 until March 2022. We included all the patients who were followed and/or hospitalized for adrenal incidentalomas. Results There were 86, predominantly female, patients (67.85%). The mean age was 58.91+/-14.40 years. The clinical findings were a unilateral adrenal mass in 73.25% of patients, localized on the left in 39.53%, on the right in 33.72%, and a bilateral one in 26.75%. Its size varied from 12 to 196 mm, with an average of 35.5 mm. The most common etiologies found in our series were a non-functional adrenal adenoma in 54.56%, a subclinical cortisolic adenoma in 19.76%, an adrenocortical carcinoma in 5.81%, and a pheochromocytoma in 5.81%. Adrenalectomy was indicated in 19.76% of our patients, 17.44% were monitored closely, 20.94% were monitored for comorbidities, and 41.86% had been advised to abstain from treatment. Conclusion An adrenal incidentaloma has become more and more frequent. It constitutes an entity with various etiologies, which can be serious. The main etiology in our series was non-functioning adrenal adenoma, for which therapeutic abstention was indicated in 48% of cases.

Short stature Revealing a Pycnodysostosis: A Case Report.

INTRODUCTION: Pycnodysostosis is a rare genetic disease characterized by osteosclerosis and bone fragility. The clinical aspects are varied including short stature, acro-osteolysis of distal phalanges, and dysplasia of the clavicles. Oral and maxillofacial manifestations of this disease are very clear. The head is usually large, a beaked nose, obtuse mandibular angle, and both maxilla and mandible are hypoplastic. Dental abnormalities are common. We report a case with the typical clinical and radiological characteristics of the Pycnodysostosis associated with a conductive hearing loss, an association rarely reported. CASE PRESENTATION: A 12-year-female was admitted in our institute for short stature with a dysmorphic facies for evaluation. The patient reported a history of multiple fractures of the long bones after a trivial fall. On physical examination, she had the following features: short stature, limited mouth opening, short hands and feet with dysplastic nails; frontal and occipital bossing; and hypoplasia of the maxilla and mandible. Examination of the mouth: grooved palate, caries of the teeth, impacted and malposed teeth, persistent deciduous teeth and missing teeth. Laboratory investigations were normal. The radiographic examination showed a generalized increase in the bone density, slight condensation of the skull base and a very open mandibular angle. X-rays showed tapered phalanges with acro-osteolysis of the distal phalanges. A symptomatic treatment was proposed based on fracture prevention, oral hygiene, frequent dental visits and psychiatric support. CONCLUSION: The clinical and radiological features are the bases for the diagnosis of this disease. It is important to make the diagnosis as early as possible in order to plan the treatment and to provide a better life quality to the patients.