Adrenocortical neoplasm in a 2-year-old child: Clinical approach and diagnostic imaging.

Adrenocortical tumors in children and adolescents are rare and aggressive, accounting for only 0.2% of pediatric cancers, with most cases associated with Li-Fraumeni syndrome. The most common manifestation is virilization due to androgen excess. Imaging techniques are crucial in the diagnosis and management of pediatric adrenocortical carcinoma. CT and MRI are essential for differentiating between benign and malignant lesions and assessing tumor characteristics and extent. Correlating imaging findings with clinical and histopathological data is vital for optimal diagnosis and treatment, underscoring the need for a multidisciplinary approach to managing these rare but aggressive neoplasms. This report presents the case of a previously healthy 2-year-old boy who exhibited virilization symptoms and was diagnosed with adrenocortical carcinoma.

Metastatic Axillary Cutaneous Squamous Cell Carcinoma in an African-American Female: A Rare Case.

Cutaneous squamous cell carcinoma (cSCC) is a common skin cancer, typically affecting older White males in sun-exposed areas, and metastasis is rare. We present a unique case of a 46-year-old obese African-American woman with a recurrent, deep abscess in her left axilla. It was initially treated with several incision and drainage procedures and antibiotics. Despite multiple interventions, the abscess recurred with severe pain and drainage. Subsequent biopsies revealed a high-grade malignant neoplasm, later confirmed as poorly differentiated cSCC with primary metastases to the lungs and secondary metastases to the adrenal glands and periaortic lymph nodes. Immunohistochemical staining supported the diagnosis. The patient's atypical presentation, including her race, a non-sun-exposed site, and younger age, highlights the need for vigilance in diagnosing cSCC in atypical cases. This case underscores the importance of early consideration of cSCC in differential diagnoses for persistent or recurrent abscesses, which can facilitate timely treatment, potentially preventing extensive metastasis and improving patient outcomes.

Case Report: Adrenal schwannoma associated with ganglioneuroma.

Background: An adrenal collision tumor (ACT) denotes the presence of distinct tumors with diverse behavioral, genetic, and histological features independently co-existing within the adrenal tissue without intermingling, and occurrences of such cases are infrequent. The concurrent occurrence of adrenal schwannoma and adrenal ganglioneuroma is exceedingly rare, and the diagnosis of these ACTs has been notably challenging due to their atypical clinical manifestations and imaging characteristics. Case summary: A 37-year-old man presented to the hospital 3 weeks after a computed tomography (CT) examination that revealed a left adrenal mass. Physical examination findings were unremarkable. Both CT and magnetic resonance imaging scans indicated the presence of a left adrenal mass. Plasma cortisol, adrenocorticotropic hormone, and renin-angiotensin-aldosterone system tests yielded normal results. Preoperative imaging confirmed the diagnosis of left adrenal pheochromocytoma. After thorough surgical preparation, a laparoscopic partial left adrenalectomy was performed. Subsequent postoperative pathological analysis identified adrenal schwannoma in conjunction with adrenal ganglioneuroma. The patient recovered well and was discharged on postoperative day 4. A routine urology clinic visit was included in his postoperative care plan. During follow-up assessments, CT scans of the left adrenal gland revealed no abnormalities. Conclusion: Adrenal schwannoma combined with ganglioneuroma represents an exceptionally rare collision tumor characterized by the absence of typical clinical or imaging features, leading to potential misdiagnosis. Adrenal incidentalomas present as multifaceted conditions, and this case serves to heighten awareness of their intricate nature. Due to the challenges in preoperative differentiation of various adrenal mass types, postoperative pathological analysis is imperative for guiding the subsequent treatment course for the patient.

Cushing's Syndrome Masquerading as Fibromyalgia: A Case Series.

Three young female patients with a history of generalized body pain were diagnosed with fibromyalgia. They visited several specialities which related patients' symptoms to their previous diagnosis of fibromyalgia and were treated symptomatically. These patients developed a multitude of clinical features including fractures, hypertension, abnormal weight gain, proximal myopathic pain and bruising. They were seen by rheumatologists whose assessment was that their clinical features were not entirely due to fibromyalgia and suspected that patients have a possible underlying endocrine cause. Patients were referred to an endocrinologist for further tests with suspicion of Cushing's syndrome. Laboratory tests and imaging confirmed a diagnosis of Cushing's syndrome. Two of them had adrenal adenoma and one had iatrogenic corticosteroid use. These cases emphasize the need for thorough clinical evaluation for patients who are thought to have fibromyalgia. Fibromyalgia is a diagnosis of exclusion.

Successful laparotomic ethanol ablation for an adrenal tumour in a dog.

Adrenalectomy is the gold standard for canine adrenal tumours, but not always recommended due to patient age, underlying conditions and perioperative mortality. Ethanol ablation is an alternative in human medicine for poor surgical candidates. A 13-year-old neutered female toy-poodle with hypercortisolism presented with severe haematuria. Ultrasonography revealed left adrenal and right kidney tumours. Due to high surgical risk, simultaneous laparotomic right nephroureterectomy and ethanol ablation of the left adrenal tumour were performed. Post-ethanol injection complications included transient hypertension and arrhythmia, which resolved spontaneously. The adrenal tumour size decreased within 2.5 months, and cortisol levels normalised within 8 days, remaining stable for 12 months. No hypercortisolism signs were observed without trilostane until death from renal insufficiency. Autopsy showed that the ablated left adrenal gland was an adrenocortical tumour and had shrunk. Ethanol ablation may be a feasible alternative to adrenalectomy for high-risk canine patients.

Laparoscopic resection of giant adrenal myelolipoma: A case report with review of literature.

Giant adrenal myelolipoma (AML) may cause severe symptoms. In contrast to the previous reports, laparoscopy may play a pivotal role in the management of giant AML. This report aims to discuss a case of giant AML managed successfully by laparoscopy. A 63-year-old male was found to have a giant (12 × 10 × 8 cm) left AML during a workup for left lower chest pain on imaging. laparoscopic excision of a left adrenal gland with the lesion was performed under general anesthesia. The patient was discharged from the hospital after 3 days uneventfully. AML is a benign tumor that is characterized by the presence of adipose tissue and hematopoietic elements. Myelolipomas are typically asymptomatic. AML diagnosis is based on imaging and blood workup. Small asymptomatic AML is usually managed conservatively, while symptomatic AML is managed with surgery. Even though an open approach is the standard option, laparoscopy, as a minimally invasive technique, in some centers may replace laparotomy. Laparoscopy can be a successful method for managing AML, even when they are large in size.

AI-guided identification of risk variants for adrenocortical tumours in TP53 p.R337H carrier children: a genetic association study.

Background: Adrenocortical tumours (ACT) in children are part of the Li-Fraumeni cancer spectrum and are frequently associated with a germline TP53 pathogenic variant. TP53 p.R337H is highly prevalent in the south and southeast of Brazil and predisposes to ACT with low penetrance. Thus, we aimed to investigate whether genetic variants exist which are associated with an increased risk of developing ACT in TP53 p.R337H carrier children. Methods: A genetic association study was conducted in trios of children (14 girls, 7 boys) from southern Brazil carriers of TP53 p.R337H with (n = 18) or without (n = 3) ACT and their parents, one of whom also carries this pathogenic variant (discovery cohort). Results were confirmed in a validation cohort of TP53 p.R337H carriers with (n = 90; 68 girls, 22 boys) or without ACT (n = 302; 165 women, 137 men). Findings: We analysed genomic data from whole exome sequencing of blood DNA from the trios. Using deep learning algorithms, according to a model where the affected child inherits from the non-carrier parent variant(s) increasing the risk of developing ACT, we found a significantly enriched representation of non-coding variants in genes involved in the cyclic AMP (cAMP) pathway known to be involved in adrenocortical tumorigenesis. One among those variants (rs2278986 in the SCARB1 gene) was confirmed to be significantly enriched in the validation cohort of TP53 p.R337H carriers with ACT compared to carriers without ACT (OR 1.858; 95% CI 1.146, 3.042, p = 0.01). Interpretation: Profiling of the variant rs2278986 is a candidate for future confirmation and possible use as a tool for ACT risk stratification in TP53 p.R337H carriers. Funding: Centre National de la Recherche Scientifique (CNRS), Behring Foundation, Conselho Nacional de Desenvolvimento Científico e Tecnológico (CNPq).

Polish diagnostic and therapeutic recommendations for adrenocortical carcinoma.

Advances in the diagnosis and treatment of adrenocortical carcinoma (ACC), along with the development of new therapeutic and diagnostic methods, have prompted a team of experts to formulate the first Polish guidelines for managing ACC. This article presents the diagnostic and therapeutic recommendations resulting from the discussion of specialists from various medical specialities, who participated in a series of online meetings aimed at developing consistent and effective recommendations under the National Oncology Strategy. These guidelines aim to optimise ACC treatment in Poland through coordinated efforts of multidisciplinary specialist teams, ensuring an effective and modern approach.

Management of undifferentiated adrenal gland metastases from malignant melanoma: case report.

Adrenal gland metastases from malignant melanoma are a common but poorly characterised condition. Their lack of consistent clinical features and poor response to immune checkpoint inhibitors pose a significant diagnostic and therapeutic challenge to practitioners. This case report describes a 78-year-old male with no prior history of melanoma presenting with nonspecific abdominal symptoms and unintentional weight loss who was found to have undifferentiated bilateral adrenal gland metastases from malignant melanoma. Despite ongoing investigations, the primary site of the adrenal gland metastases remained unknown, prompting the consideration of primary adrenal melanoma as a diagnosis. The patient underwent four cycles of treatment with immune checkpoint inhibitors, nivolumab and ipilimumab, followed by maintenance therapy and subsequent adrenal metastasectomy. Despite therapeutic efforts, the patient's tumour was resistant to treatment and became undifferentiated. The patient continued with palliative care until his death, more than three years after the onset of symptoms. The clinical features, pathophysiology, diagnosis, treatment, and prognosis of this patient's disease are discussed in detail to help inform the management of similar cases.

Impact of Aging on the Morphology of the Adrenal Gland in Rats / Impacto del Envejecimiento en la Morfología de la Glándula Suprarrenal en Ratas

SUMMARY: Aging is an inevitable biological process that affects the function of all organs, including the adrenal gland, which is essential for producing steroid hormones that regulate metabolism, stress response, and immune activation. Understanding how aging affects the morphology of this gland is crucial to developing interventions to mitigate its adverse effects. Thus, this study aimed to describe the morphoquantitative alterations of the adrenal gland in senescent Sprague Dawley rats compared to adult rats. Twelve male rats were divided into 6 adult rats aged 6 months (group A) and 6 senescent rats aged 36 months (group S). Histopathological studies, quantification of collagen fibers types I and III, and stereological analysis were performed to determine the volume density (Vv), surface area (Sv), and number (Nv) of the nuclei of the zona fasciculata cells. Adrenal gland tissue from group S presented dysplasia, metaplasia, intracellular fat accumulation, fibrosis, blood vessel dilation, and increased presence of apoptotic cells. Capsule thickening and increased collagen type I were also observed. There was a significant decrease in Vv, Sv, and Nv of zona fasciculata nuclei in group S compared to group A. The results indicate that aging induces significant morphoquantitative changes in the adrenal gland, which could contribute to the decrease in glucocorticoid production and alterations in aldosterone and cortisol secretion observed in senescence. Understanding these alterations is crucial to developing interventions that mitigate the adverse effects of aging on the endocrine system.

El envejecimiento es un proceso biológico inevitable que afecta la función de todos los órganos, incluida la glándula suprarrenal, fundamental para la producción de hormonas esteroides que regulan el metabolismo, la respuesta al estrés y la activación inmunológica. Comprender cómo el envejecimiento afecta la morfología de esta glándula es crucial para desarrollar intervenciones que mitiguen sus efectos adversos. Así, el objetivo de este estudio fue describir las alteraciones morfocuantitativas de la glándula suprarrenal en ratas Sprague Dawley senescentes comparadas con ratas adultas. Se utilizaron 12 ratas macho, divididas en dos grupos: 6 ratas adultas de 6 meses de edad (grupo A) y 6 ratas senescentes de 36 meses de edad (grupo S). Se realizaron estudios histopatológicos, cuantificación de fibras de colágeno tipos I y III y análisis estereológicos para determinar la densidad de volumen (Vv), de superficie (Sv) y de número (Nv) de los núcleos de las células de la zona fasciculada. El tejido de la glándula suprarrenal del grupo S presentó displasia, metaplasia, acumulación de grasa intracelular, fibrosis, dilatación de vasos sanguíneos y mayor presencia de células apoptósicas. También se observó un engrosamiento de la cápsula y un incremento del colágeno tipo I. Hubo una disminución significativa en Vv, Sv y Nv de los núcleos de la zona fasciculada en el grupo S en comparación con el grupo A. Los resultados indican que el envejecimiento induce cambios morfocuantitativos significativos en la glándula suprarrenal, lo que podría contribuir a la disminución en la producción de glucocorticoides y alteraciones en la secreción de aldosterona y cortisol observadas en la senescencia. Comprender estas alteraciones es crucial para desarrollar intervenciones que mitiguen los efectos adversos del envejecimiento en el sistema endocrino.

Neuroendocrine carcinoma of the adrenal gland: A rare case report and literature review.

Neuroendocrine carcinoma (NEC) presence in the adrenal glands is rare. Neuroendocrine carcinoma manifests across a wide range of clinical presentations, from asymptomatic cases to those characterized by hormone overproduction or the tumor's mass effect. We report a 48-year-old male referred by a urology specialist with a chief complaint of right-sided back pain for the past 6 months accompanied by nausea, vomiting, and sharp stabbing headaches. The patient had a history of right adrenalectomy surgery. Elevated blood pressure of 150/110 mmHg, and no abnormalities found. The radiologist found a solid lesion and cyst at the lower pole of the right kidney and observed multiple recurrent tumors in the right adrenal on the MRI examination. The biopsy revealed poorly differentiated carcinoma and adrenocortical carcinoma tissue on the second biopsy 2 months later. The patient was diagnosed with neuroendocrine carcinoma; the patient underwent a biopsy guided by CT, followed by a pathological assessment (PA). The surgeon carried out the tumor removal surgery and performed an immunohistochemical (IHC) analysis. A 3-month follow-up is planned to evaluate the potential need for adjuvant chemotherapy. The case underscores the importance of accurate pathological diagnosis and multimodal management in recurrent adrenal tumors, particularly when considering NEC as a differential diagnosis.

Endocrine Disorders in Nephrotic Syndrome-A Comprehensive Review.

Nephrotic syndrome is a clinical syndrome characterized by massive proteinuria, called nephrotic range proteinuria (over 3.5 g per day in adults or 40 mg/m2 per hour in children), hypoalbuminemia, oncotic edema, and hyperlipidemia, with an increasing incidence over several years. Nephrotic syndrome carries severe morbidity and mortality risk. The main pathophysiological event in nephrotic syndrome is increased glomerular permeability due to immunological, paraneoplastic, genetic, or infective triggers. Because of the marked increase in the glomerular permeability to macromolecules and the associated urinary loss of albumins and hormone-binding proteins, many metabolic and endocrine abnormalities are present. Some of them are well known, such as overt or subclinical hypothyroidism, growth hormone depletion, lack of testosterone, vitamin D, and calcium deficiency. The exact prevalence of these disorders is unknown because of the complexity of the human endocrine system and the differences in their prevalence. This review aims to comprehensively analyze all potential endocrine and hormonal complications of nephrotic syndrome and, vice versa, possible kidney complications of endocrine diseases that might remain unrecognized in everyday clinical practice.

Surface functionalized nanomaterial systems for targeted therapy of endocrine related tumors: a review of recent advancements.

The application of multidisciplinary techniques in the management of endocrine-related cancers is crucial for harnessing the advantages of multiple disciplines and their coordinated efforts in eliminating tumors. Due to the malignant characteristics of cancer cells, they possess the capacity to develop resistance to traditional treatments such as chemotherapy and radiotherapy. Nevertheless, despite diligent endeavors to enhance the prediction of outcomes, the overall survival rate for individuals afflicted with endocrine-related malignancy remains quite miserable. Hence, it is imperative to investigate innovative therapy strategies. The latest advancements in therapeutic tactics have offered novel approaches for the therapy of various endocrine tumors. This paper examines the advancements in nano-drug delivery techniques and the utilization of nanomaterials for precise cancer cures through targeted therapy. This review provides a thorough analysis of the potential of combined drug delivery strategies in the treatment of thyroid cancer, adrenal gland tumors, and pancreatic cancer. The objective of this study is to gain a deeper understanding of current therapeutic approaches, stimulate the development of new drug DDS, and improve the effectiveness of treatment for patients with these diseases. The intracellular uptake of pharmaceuticals into cancer cells can be significantly improved through the implantation of synthetic or natural substances into nanoparticles, resulting in a substantial reduction in the development of endocrine malignancies.

The Great Impostor: A challenging case of small-cell melanoma with isolated adrenal metastasis.

Small-cell melanoma masquerading as an adrenal non-Hodgkin lymphoma. The index report illustrates the deceptive cytomorphologic features of a small cell type malignant melanoma metastatic to the adrenal gland. The diagnosis was confirmed by performing immunocytochemistry on the cell block sections. The key cytomorphologic mimics and their distinctive features have also been highlighted.

Dielectric characterisation of human adrenal glands and adrenal tumours for the development of microwave ablation technologies for hypertension treatment.

Adrenal gland-induced hypertension results from underlying adrenal gland disorders including Conn's syndrome, Cushing's syndrome, and Pheochromocytoma. These adrenal disorders are a risk for cardiovascular and renal morbidity and mortality. Clinically, treatment for adrenal gland-induced hypertension involves a pharmaceutical or surgical approach. The former presents very significant side effects whereas the latter can be ineffective in cases where the adrenal disorder reoccurs in the remaining contralateral adrenal gland. Due to the limitations of existing treatment methods, minimally invasive treatment options like microwave ablation (MWA) have received significant attention for treating adrenal gland disorders. A precise comprehension of the dielectric properties of human adrenal glands will help to tailor energy delivery for MWA therapy, thus offering the potential to optimise treatments and minimise damage to surrounding tissues. This study reports the ex vivo dielectric properties of human adrenal glands, including the cortex, medulla, capsule, and tumours, based on the data obtained from four patients (diagnosed with Conn's syndrome, Cushing's syndrome, and Pheochromocytoma) who underwent unilateral adrenalectomy at the University Hospital Galway, Ireland. An open-ended coaxial probe measurement technique was used to measure the dielectric properties for a frequency range of 0.5-8.5 GHz. The dielectric properties were fitted using a two-pole Debye model, and a weighted least squares method was employed to optimise the model parameters. Moreover, the dielectric properties of adrenal tissues and tumours were compared across frequencies commonly used in MWA, including 915 MHz, 2.45 GHz, and 5.8 GHz. The study found that the dielectric properties of adrenal tumours were influenced by the presence of lipid-rich adenomas, and the dielectric properties of Cushing's syndrome tumour were lowest in comparison to the tumours in patients diagnosed with Conn's syndrome and Pheochromocytoma. Furthermore, a notable difference was observed in the dielectric properties of the medulla and cortex among patients diagnosed with Conn's syndrome, Cushing's syndrome, and Pheochromocytoma. These findings have significant implications for the diagnosis and treatment of adrenal tumours, including the optimisation of MWA therapy for precise ablation of adrenal masses.

Recurrent epithelioid angiomyolipoma of the adrenal gland: a case report and literature review.

Background: Epithelioid angiomyolipoma (EAML), a subtype of angiomyolipoma, is distinct. It has a biologic behavior of borderline tumor, a malignant tendency, and a risk of metastasis and recurrence. Adrenal EAML is very rare. It is true that only six cases of adrenal EAML have been documented in the English-language literature. Case Description: A 65-year-old man who underwent a laparoscopic left adrenalectomy in July 2022 has adrenal EAML and this is a case report about it. The mass was surrounded by abundant blood vessels and adherence with surround-tissue. Postoperative pathology of the tumor analysis revealed adrenal epithelioid vascular smooth muscle lipoma. The patient underwent left upper abdomen and lumbar pain in July 2022. The enhanced computed tomography (CT) scan of the abdomen showed markedly enhanced masses in and around the left adrenal gland. A second left laparoscopic adrenalectomy was performed under general anesthesia. Postoperative pathology showed two taupe nodules of left adrenal, maximum diameter 0.9 to 1.1 cm. The postoperative pathological diagnosis in combination with immunohistochemistry was EAML. The patient was discharged 10 days later with symptomatic treatment with low molecular heparin. Conclusions: Adrenal EAML has a biologic behavior of borderline tumor with malignant potential and a risk of distant metastasis and recurrence. Therefore, radical surgical resection should be considered as its necessary treatment. Long-term postoperative follow-up is an important part of the treatment.

Laparoscopic adrenalectomy performed by a general surgeon on functioning adrenal tumors: Treatment outcomes and risk prediction of persistent hypertension.

Background: Functional adrenal tumors may contribute to poor hypertension control and electrolyte abnormalities, thus increasing the risk of cardiovascular mortality. Currently, laparoscopic adrenalectomy is an effective surgical option that contributes to improved treatment outcomes as compared to open surgery. The purpose of this study was to evaluate the outcomes of laparoscopic adrenalectomy performed by a general surgeon at a low-volume center and to identify clinicopathological risk factors for postoperative persistent hypertension. Methods: A retrospective study of patients with functional adrenal tumors who underwent laparoscopic adrenalectomy at Srinakharinwirot University, Thailand, between 2014 and 2022. Clinicopathologic and postoperative data were examined. Results: This study included twenty-five patients; the indications for laparoscopic adrenalectomy included primary aldosteronism in 19 (76%), pheochromocytoma in 4 (16%), and Cushing's syndrome in 2 (8%). The average time of surgery was 103.5 ± 19.7 min, and intraoperative complications occurred in three patients (12%), with one patient requiring conversion to open surgery (4%). The postoperative systolic (125 ± 15 vs. 158 ± 18 mmHg; p < 0.001) and diastolic (78.5 ± 6.7 vs. 95.3 ± 10 mmHg; p = 0.013) blood pressure significantly decreased compared to prior surgery, but only 19 patients (76%) achieved a cure for hypertension. Multivariate analysis revealed that the patient's physical status, as classified by the American Society of Anesthesiologists (odds ratio (OR) = 0.66, 95% confidence interval (CI) 0.43-1.32, p = 0.001), and the need for at least three antihypertensive medicines (OR = 0.7, 95% CI 0.36-1.2, p = 0.002), were independent predictive factors of persistent hypertension after surgery. Conclusion: Laparoscopic adrenalectomy is a safe and effective surgical treatment for functional adrenal tumors, even when performed in a low-volume center. According to the American Society of Anesthesiologists' physical categorization, the patient's physical condition and the necessity for at least three antihypertensive medications are predictors of postoperative hypertension. Trial registration: The study was registered with the Thai Clinical Registry Trials: TCTR20230707007.

A case of adrenal Castleman disease combined with liver cirrhosis and splenomegaly.

To describe a rare case of left adrenal Castleman disease (CD), splenomegaly, and cirrhosis. An examination revealed a left adrenal mass for more than three months, the patient, 44, was well-prepared for surgery after her left adrenal tumor was removed laparoscopically using a retroperitoneal approach, her postoperative pathology suggested that she had Castleman disease of the adrenal glands, and there had been no metastasis or recurrence during the six-month follow-up period. We have evaluated linked literature reports in this article, reporting relevant clinical knowledge regarding the disease and synthesizing previous research, in an effort to increase our understanding of it.