Clitoromegaly in female child is a rare condition. This is often associated with androgen excess in disorders of sexual differentiation usually congenital adrenal hyperplasia. Nonhormonal causes of clitoromegaly are rare and are commonly due to neurofibromatosis, epidermoid cyst, rhabdomyosarcoma, and very rarely hemangioma. Hemangioma is one of the common benign neoplasms of infancy, usually located in head, trunk, and extremities. We are reporting 4-month-old child with clitoromegaly with normal hormonal assay. Clitoromegaly was due to hemangioma. Histopathology of mass confirmed the diagnosis. Only five cases have been reported in literature till date.

Renal cell carcinoma (RCC) arises from the renal tubular epithelial cells and comprises a group of heterogenous renal tumors. Renal tumors can metastasize to involve almost any body organ, the common sites being the lung, liver, bone, brain, adrenal gland, head, neck, and rarely, inferior vena cava (IVC), leading to lethal outcomes. We present a case of RCC with IVC invasion in a patient who presented with right-sided flank pain and gross hematuria. His routine biochemical and hematological parameters were unremarkable, and an abdominal examination revealed a complex renal mass with mild hydronephrosis. The patient underwent contrast-enhanced magnetic resonance angiography with venography, which showed a right renal upper polar mass lesion extending into the right vein obliterating it up to its junction with the IVC. Integrating examination and imaging findings were suggestive of right renal RCC. Our case highlights the importance of standard preoperative MRI imaging to assess IVC invasion and its morphologic features including vessel breach or complete occlusion of the IVC.

Pheochromocytomas and paragangliomas (PGLs) are rare non-epithelial neuroendocrine neoplasms of the adrenal medulla and extra-adrenal paraganglia respectively. Duodenal PGL is quite rare and there are only two previous reports. Herein, we report a case of multiple catecholamines (CAs)-producing PGLs in the middle ear, retroperitoneum, and duodenum, and review the literature of duodenal PGLs. A 40-year-old man complained right-ear hearing loss, and an intracranial tumor was suspected. Magnetic resonance imaging of the head revealed a 3-cm mass at the right transvenous foramen, which was surgically resected following preoperative embolization. The pathological diagnosis was a sympathetic PGL of the right middle ear. Six years later, family history of PGL with germline mutation of succinate dehydrogenase complex iron sulfur subunit B, SDHB: c.268C>T (p.Arg90Ter) was clarified. The patient had elevated levels of plasma and urine CAs again. Abdominal computed tomography scanning revealed two retroperitoneal tumors measuring 30-mm at the anterior left renal vein and 13-mm at near the ligament of Treitz. The larger tumor was laparoscopically resected, but the smaller tumor was not identified by laparoscopy. After the operation, the patient remained hypertensive, and additional imaging tests suggested a tumor localized in the duodenum. The surgically resected tumor was confirmed to be a duodenal PGL. After that, the patient remained hypertension free, and urinary levels of noradrenaline and normetanephrine decreased to normal values. No recurrence or metastasis has been found at 1 year after the second operation. CAs secretion from PGLs in unexpected location, like the duodenum of our patient, may be overlooked and leads to a hypertensive crisis. In such cases, comprehensive evaluation including genetic testing, fluorodeoxyglucose-positron emission tomography scanning, and measurement of CAs will be useful for detecting PGLs. Most previous reports on duodenal PGL were gangliocytic PGL which has been renamed composite gangliocytoma/neuroma and neuroendocrine tumor, and defined the different tumor from duodenal PGL. We reviewed and discussed duodenal PGLs in addition to multiple PGLs associated with SDHB mutation.

A 39-year-old woman presented with cushingoid features was worked up and diagnosed to have ACTH-independent Cushing's syndrome. Computed tomography of the whole abdomen revealed a left adrenal mass. She was scheduled for elective laparoscopic left adrenalectomy, however, a few days prior to the surgery, the patient had hematochezia. Colonoscopy revealed multiple ulcers on the terminal ileum, to which biopsy revealed Mycobacterium tuberculosis infection. The patient underwent laparoscopic left adrenalectomy on the same admission, which revealed adrenal adenoma on histopathology.

Here we present a rare case of a large co-secreting adrenocortical oncocytoma undergoing laparoscopic retroperitoneal-to-open removal. Our case was referred for surgical opinion as a result of a left suprarenal mass being identified as part of a work-up for new onset hirsutism and post-menopausal bleeding. We provide example of our surgical approach as well as discussion on this unique adrenal tumour variant.

Hepatobiliary neuroendocrine neoplasms are rare cancers in humans and dogs. To date, no large-scale primary hepatobiliary neoplasm omics analyses exist in any species. This limits the development of diagnostic biomarkers and targeted therapeutics. Neuroendocrine cancers are a heterogenous group of neoplasms categorized by their tissue-of-origin. Because the anatomic niche of neuroendocrine neoplasms shapes tumor phenotype, we sought to compare the proteomes of 3 canine hepatobiliary neoplasms to normal hepatobiliary tissue and adrenal glands with the objective of identifying unique protein signatures. Protein was extracted from formalin-fixed paraffin-embedded samples and submitted for tandem mass spectroscopy. Thirty-two upregulated and 126 downregulated differentially expressed proteins were identified. Remarkably, 6 (19%) of the upregulated proteins are correlated to non-hepatobiliary neuroendocrine neoplasia and 16 (50%) are functionally annotated within the exosome cellular compartment key to neuroendocrine signaling. Twenty-six (21%) downregulated proteins are enriched in metabolic pathways consistent with alterations in cancer. These results suggests that characteristic neoplastic protein signatures can be gleaned from small data sets using a comparative proteomics approach.

Corticomedullary mixed tumor (CMT) is a single adrenal tumor mass composed histologically by an admixture of adrenal cortical and medullary cells. It is a rare condition, with approximately 20 cases reported to date. To our knowledge, the positron emission tomography (PET) imaging findings of this mostly benign tumor have not been reported in the literature. We present a case of CMT who was evaluated with both 18F-fluorodeoxyglucose (18F-FDG) and 68Ga-DOTATATE. The hypermetabolic tumor seen on 18F-FDG PET/computed tomography scan showed no abnormal uptake by 68Ga-DOTATATE.

The widespread use of cross-sectional imaging modalities, such as computed tomography (CT) and magnetic resonance imaging (MRI), in the evaluation of abdominal disorders has significantly increased the number of incidentally detected adrenal abnormalities, particularly adrenal masses [...].

A 57-year-old male was imaged with 123I mIBG for left sided suprarenal mass. The planar and SPECT-CT acquisitions revealed features consistent with a left sided pheochromocytoma. There was physiological and high uptake in the hypertrophied left lobe of the liver which was normal at both MRI and non-contrast CT. Asymmetrical increased 123I mIBG uptake in the left lobe of the liver as compared to the right lobe is a known physiological finding with no obvious cause or significance.

An adrenal incidentaloma is an adrenal mass ≥ 1 cm in size discovered on imaging performed for indications other than suspected adrenal disease. It has variable etiologies, which can be benign or malignant, including primary or metastatic disease. We present a rare case of metastatic lung adenocarcinoma with isolated unilateral adrenal metastases, presenting as an adrenal incidentaloma in an asymptomatic patient with no known history of malignancy. A 76-year-old man with a past medical history of chronic obstructive pulmonary disease (COPD) and heavy tobacco use was admitted for the evaluation and treatment of pneumonia. He was found to have an incidental 4.6 cm unilateral adrenal mass on his CT chest. He underwent a workup for the mass, including further imaging studies that were indeterminate and a hormonal workup that concluded that the mass was nonfunctional. Due to the patient's comorbidities, it was determined that he was not a surgical candidate. A multidisciplinary team recommended a biopsy, which revealed metastatic lung adenocarcinoma. The primary lung cancer was located using positron emission tomography with 2-deoxy-2-(fluorine-18) fluoro-D-glucose combined with computed tomography (F-FDG-PET/CT). The patient was evaluated by an oncology service and started on chemotherapy. In this case report, we discuss the approach for evaluating adrenal incidentalomas as well as the role the biopsy has in this process based on a literature review. In addition, we draw a comparison between our case and similar cases in the literature while highlighting the differences that make this case unique.

Pheochromocytomas and paragangliomas are rare neuroendocrine tumors. Pheochromocytomas are derived from chromaffin cells of the adrenal medulla, while paragangliomas arise from the extra-adrenal autonomic paraganglia. Paragangliomas can derive from either parasympathetic or sympathetic paraganglia. The majority of parasympathetic ganglia-derived paragangliomas are nonfunctional and symptoms arise from mass effect, while sympathetic paragangliomas are frequently functional and present with symptoms that result from catecholamine hypersecretion. Here, we present the case of a 19-year-old female with hypertension whose biochemical tests revealed elevated plasma and urinary levels of norepinephrine and normetanephrine. Imaging studies showed a left paravertebral mass which was surgically removed. Histopathology confirmed a paraganglioma. Total surgical resection remains the gold-standard treatment and a cure can be achieved; however, all tumors may harbor malignant potential, and a long-term biochemical and imaging follow-up is required in all patients. Screening for genetic germline mutations may be helpful in identifying patients with a higher risk of recurrence or of developing other primary tumors.

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.

Space-occupying lesions of the adrenal glands are one of the most frequent tumors; however, only a fraction of approximately 20% need further diagnostics and treatment. The diagnostic standard is native computed tomography (CT). For larger tumors and those that cannot be clearly classified as benign, the supplementary radiological modalities magnetic resonance imaging (MRI), contrast CT and 18F-fluorodeoxyglucose positron emission tomography (18F-FDG-PET) should be used. Adrenal gland neoplasms can be hormone-active or hormone-inactive. The most important hormone-active adrenal gland neoplasms are those with autonomous cortisol secretion and autonomous aldosterone secretion as well as pheochromocytomas. Several laboratory tests are available each for the respective hormone activity. The indications for surgery are dependent on the dignity, size and hormone activity. Minimally invasive surgical techniques have become more important due to advantages such as lower blood loss; nevertheless, open surgery is still indispensable for large suspected tumors. Care must be taken preoperatively and postoperatively, particularly in cases of hormone-active tumors.

Introduction The present study aimed to prove the metyrapone short test in a day clinic to be suitable for examining the integrity of the hypothalamic-pituitary-adrenal (HPA) axis in patients with suspected secondary and tertiary adrenal insufficiency and to identify novel effector molecules in acute stress response. Methods 44 patients were prospectively enrolled. Based on stimulated 11-deoxycortisol levels, patients were divided into a physiological (11-deoxycortisol ≥70 µg/l) and a pathological (11-deoxycortisol <70 µg/l) response group. Clinical follow-up examination was performed for validation. Ultra-performance-liquid-chromatography-tandem-mass-spectrometry and a Fourier-transform-ion-cyclotron-resonance-mass-spectrometry were used for targeted and untargeted steroid metabolomics. Results At baseline, lower levels of cortisone (42 vs. 50 nmol/l, p=0.048) and 17-OH-progesterone (0.6 vs. 1.2 nmol/l, p=0.041) were noted in the pathological response group. After metyrapone administration, the pathological response group exhibited significantly lower 11-deoxycortisol (39.0 vs. 94.2 µg/l, p<0.001) and ACTH (49 vs. 113 pg/ml, p<0.001) concentrations as well as altered upstream metabolites. Untargeted metabolomics identified a total of 76 metabolites to be significantly up- or downregulated by metyrapone. A significant increase of the bile acid glycochenodeoxycholic acid (GCDC, p<0.01) was detected in both groups with an even stronger increase in the physiological response group. After a mean follow-up of 17.2 months, an 11-deoxycortisol cut-off of 70 µg/l showed a high diagnostic performance (sensitivity 100%, specificity 96%). Conclusion The metyrapone short test is safe and feasible in a day clinic setting. The alterations of the bile acid GCDC indicate that the liver might be involved in the acute stress response of the HPA axis.

Background: Idiopathic granulomatous mastitis (IGM) is a chronic inflammatory condition of the mammary gland that presents as a painful mass, and it must be distinguished from both infectious mastitis and breast cancer. When diagnosed during lactation, it can result in significant distress and early weaning. Injection of triamcinolone has been used as a successful treatment method, but safety in breastfed infants has not been established. Methods: We present a case of a lactating patient who received a direct injection of triamcinolone (dosage 40 mg) in her breast to treat IGM after failure of oral corticosteroids. Breastmilk samples were expressed by the patient 0, 1, 4, and 24 hours after the procedure, and then daily for 1 week. All the samples were analyzed using liquid chromatography mass spectrometry. The patient was supported by a breastfeeding and lactation medicine clinic. Results: After injection of triamcinolone into the granulomatous mass, breast milk samples were collected and analyzed. No samples were found to contain triamcinolone. A temporary but significant decrease in milk production was noted after injection, though only a slight decrease had been noted with 6 weeks of systemic corticosteroids. With support, the patient rebuilt milk production and continued to breastfeed from both breasts. Conclusion: Triamcinolone was not found in any milk samples (≥0.78 ng/mL) following therapeutic injection of the affected breast. The patient was able to continue breastfeeding from the affected breast with intermittent symptoms.

We reported a case of pheochromocytoma with initial presentation of cardiac arrest. The patient underwent implantable cardioverter defibrillator for primary prevention but subsequently experienced repeated implantable cardioverter defibrillator shocks and syncopal episodes. A mass was found in the adrenal gland by CT, which was confirmed by anatomopathological analysis of the surgical specimen.