Near-fatal pheochromocytoma crisis after beta-blocker and tumour haemorrhage.

Pheochromocytomas are rare neuroendocrine tumors characterised by the secretion of catecholamines and their metabolites. While some patients may be asymptomatic, they can also present with various symptoms including hypertensive crisis, headaches, palpitations, diaphoresis or other signs of catecholamine toxicity. Adrenal haemorrhage, though rare, is a potentially fatal complication that is often diagnosed during autopsy. In all patients with suspected pheochromocytoma, regardless of whether haemorrhagic conversion has occurred, prompt diagnosis is imperative. Early identification allows for the timely initiation of treatment, preventing potentially life-threatening complications. This case report details the haemorrhagic conversion of an undiagnosed pheochromocytoma in a female patient in her 30s.

Adrenal Vein Sampling for Primary Aldosteronism: Recommendations From the Australian and New Zealand Working Group.

Adrenal vein sampling (AVS) is the current recommended procedure for identifying unilateral subtypes of primary aldosteronism (PA), which are amenable to surgery with the potential for cure. AVS is a technically challenging procedure usually undertaken by interventional radiologists at tertiary centres. However, there are numerous variations in AVS protocols relating to patient preparation, sampling techniques and interpretation which may impact the success of AVS and patient care. To reduce practice variations, improve the success rates of AVS and optimise patient outcomes, we established an Australian and New Zealand AVS Working Group and developed evidence-based expert consensus recommendations for the preparation, performance and interpretation of AVS. These recommendations can be used by all healthcare professionals in a multidisciplinary team who look after the diagnosis and management of PA.

Predicting the resolution of hypertension following adrenalectomy in primary aldosteronism: Controversies and unresolved issues a narrative review.

BACKGROUND: Hypertension resolution following adrenalectomy in patients with primary aldosteronism (PA) remains a critical clinical challenge. Identifying preoperatively which patients will become normotensive is both a priority and a point of contention. In this narrative review, we explore the controversies and unresolved issues surrounding the prediction of hypertension resolution after adrenalectomy in PA. METHODS: A comprehensive literature review was conducted, focusing on studies published between 1954 and 2024 that evaluated all studies that discussed predictive models for hypertension resolution post-adrenalectomy in PA patients. Databases searched included MEDLINE®, Ovid Embase, and Web of Science databases. RESULTS: The review identified several predictors and predictive models of hypertension resolution, including female sex, duration of hypertension, antihypertensive medication, and BMI. However, inconsistencies in study designs and patient populations led to varied conclusions. CONCLUSIONS: Although certain predictors and predictive models of hypertension resolution post-adrenalectomy in PA patients are supported by evidence, significant controversies and unresolved issues remain. While the current predictive models provide valuable insights, there is a clear need for further research in this area. Future studies should focus on validating and refining these models.

Identification of Lipomatous Metaplasia in a Cortisol-secreting Adrenocortical Adenoma Treated With Mifepristone.

Adrenal adenomas are benign tumors of the adrenal cortex that may secrete excess hormones, such as cortisol. They are most commonly discovered during imaging studies for unrelated problems. Lipomatous metaplasia is a rare degenerative change in adrenal adenomas, characterized by the presence of adipose tissue and hematopoietic elements within the tumor. In this report, we present a case of an adrenal adenoma with lipomatous metaplasia in a patient with hypertension, hyperlipidemia, and type II diabetes mellitus. The discovery of this adrenal mass was prompted by an evaluation of the patient's progressive hirsutism. The tumor was found to be secreting cortisol, leading to Cushing syndrome. The patient subsequently underwent surgical resection of the mass after being treated with mifepristone. The histopathological examination confirmed it to be an adrenal cortical neoplasm with lipomatous metaplasia, characterized by uncertain malignant potential. The patient did well postoperatively. Three months after left adrenalectomy, the patient's hirsutism, A1c, and hypertension improved, allowing a reduction in antihypertensives. Her body mass index stabilized, her triglyceride decreased, and her dehydroepiandrosterone sulfate level normalized. She continued to do well at follow-up visits. Overall, this was a rare case of a functioning adrenal adenoma with lipomatous metaplasia, presenting both diagnostic and therapeutic challenges.

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.

Incidental detection of purely cystic pheochromocytoma in a young adult presenting with lower urinary tract infection.

Pheochromocytoma is a rare neuroendocrine tumor arising from adrenal medulla. Patients usually show classic triad of headache, palpitations and diaphoresis along with persistent or paroxysmal hypertension. Majority of the tumors are solid or mixed solid and cystic. But purely cystic variant is extremely rare with few cases reports available in the published literature. We report a case of purely cystic pheochromocytoma in a male in 30s who presented to our hospital for unrelated symptom of recurrent burning micturition. He was evaluated for causes of recurrent UTI which revealed incidental right adrenal cyst. The patient's blood pressure was raised on clinical examination and imaging showed simple adrenal cyst without solid component or septations. Suspecting possibility of pheochromocytoma, biochemical analysis was done which revealed elevated 24-hour urinary metanephrine. The diagnosis of cystic pheochromocytoma was made. Adrenalectomy was performed showing cystic lesion in the right adrenal region. Histopathology and immunohistochemistry revealed pheochromocytoma with cystic degeneration. Patient's hypertension resolved during the follow up. In conclusion, purely cystic pheochromocytoma must be considered as differential for adrenal cystic lesions, especially when atypical features are present.

Diagnostic and therapeutic approaches to a case of pregnancy complicated by bilateral adrenocortical adenomas with primary aldosteronism and Cushing's syndrome.

Aldosterone/cortisol co-secreting adenomas (A/CPA) are a rare type of primary aldosteronism(PA), and cases of aldosterone/cortisol co-secreting adenomas during pregnancy are extremely rare, with no reported cases to date. The unique physiological state of pregnancy increases cortisol secretion through the hypothalamic-pituitary-adrenal (HPA) axis and leads to elevated levels of all components of the renin-angiotensin-aldosterone system (RAAS). This can cause overlapping symptoms with abnormal cortisol and aldosterone secretion, making diagnosis very challenging. This case involves a 29-year-old woman who developed hypercortisolism at 33 weeks of pregnancy. Despite receiving treatment for her symptoms and having a successful delivery, she continued to experience hypertension and hypokalaemia after giving birth. Eventually, she was diagnosed with ACTH-independent Cushing's syndrome and primary aldosteronism due to independent cortisol and aldosterone secretion from bilateral adrenal adenomas. Following a thorough diagnosis, classification, treatment, and follow-up, the patient achieved a clinical cure while preserving normal adrenal function. Further investigation revealed that both diseases were caused by KCNJ5 and PRKACA mutations found in the bilateral adrenal adenomas.

Severe hydronephrosis complicated with primary aldosteronism: a case report and review of the literature.

BACKGROUND: Primary aldosteronism is characterized by high plasma aldosterone and low renin. The plasma aldosterone-to-renin ratio is recommended for screening. Severe hydronephrosis leads to renal parenchymal ischemia, resulting in increased renin secretion. Since nonsuppression of renin may cause a negative result in the aldosterone-to-renin ratio test, severe hydronephrosis and primary aldosteronism occurring simultaneously in a patient are challenging to diagnose. CASE PRESENTATION: A 54-year-old Chinese man of Han ethnicity was diagnosed with hypertension and severe hypokalemia (minimum 1.57 mmol/L) 13 years prior, and was also diagnosed with severe hydronephrosis due to congenital ureteral stenosis on the left side. His clinical features suggested primary aldosteronism, but the aldosterone-to-renin ratio result of the patient was negative every time he underwent the primary aldosteronism screening test. No further treatment for primary aldosteronism was performed, which led the patient to suffer from severe hypokalemia, such that he was taking 12-15 g/day potassium chloride orally to keep his blood potassium between 3.0 and 3.5 mmol/L (reference value, 3.5-5.5 mmol/L) for 13 years, and the patient needed to be hospitalized in the intensive care unit for rescue several times. At admission, although the aldosterone-to-renin ratio result of the patient was negative, we still did the saline stress test and captopril inhibition test, and the results showed that the plasma aldosterone level was not lower after the test than before the test. Adrenal enhanced computed tomography suggested an adenoma in the left adrenal gland, and the results of adrenal vein sampling suggested that the left side was the dominant side. Therefore, laparoscopic total resection of the left adrenal gland was performed, and 2 weeks later, the patient developed short-term renal function impairment and hyperkalemia, but his renal function and blood potassium returned to normal after treatment that included fluid rehydration. The patient's biochemical test results and clinical symptoms were completely normal after 1 year. CONCLUSION: We suggest that for patients with a high suspicion of primary aldosteronism in the clinic, comprehensive analysis must be performed in combination with clinical characteristic assessments, such as severe hydronephrosis, if renin is within the normal range or if the aldosterone-to-renin ratio result is negative at screening and diagnostic tests, and adrenal vein sampling should be performed if necessary. It can help avoid misdiagnoses and contribute to the treatment of patients with severe hydronephrosis and primary aldosteronism.

A Case of Renal Artery Thrombosis With Concurrent Adrenal Hemorrhage in Polycythemia Vera.

Polycythemia vera (PV) is a rare myeloproliferative neoplasm characterized by the clonal proliferation of hematopoietic stem cells, leading to an elevated red blood cell mass. This hyperproliferative state increases blood viscosity and predisposes patients to thrombotic events, which are a significant cause of morbidity and mortality in PV. The diagnosis of PV is typically confirmed through elevated hemoglobin or hematocrit levels, low serum erythropoietin, and the presence of the Janus kinase 2 (JAK2) mutation. Common complications include venous and arterial thromboses, hemorrhage, and transformation to myelofibrosis or acute leukemia. A 68-year-old female with a history of PV and chronic kidney disease (CKD) presented with uremic symptoms in the form of malaise and nausea. Laboratory investigations indicated acute kidney injury (AKI) and hyperkalemia. Imaging evaluation of renal US Doppler revealed renal artery thrombosis and an incidental adrenal hemorrhage. The patient was managed with intravenous heparin and did not receive thrombolytics or thrombectomy. Her renal function did not improve, necessitating the initiation of hemodialysis (HD) during hospitalization. Over the course of the next few weeks, her renal parameters improved and she managed to be discharged from dialysis. The primary goal of this study was to highlight a rare presentation of renal artery thrombosis secondary to polycythemia vera (PV) and discuss the complexities involved in managing the underlying disease and its thrombotic complication, particularly in the presence of concomitant bleeding. Effective management of PV-related thrombosis requires a delicate balance between anticoagulation to prevent further thrombotic events while carefully addressing the risk of hemorrhage.

PRIMARY BILATERAL ADRENAL LYMPHOMA PRESENTING WITH IMPENDING ADRENAL CRISIS.

Primary bilateral adrenal lymphoma is a very rare cause of adrenal insufficiency. We report the case of a 63-year-old woman who presented with signs and symptoms of impending adrenal crisis when referred for evaluation of large bilateral adrenal masses diagnosed on a computed tomography scan two weeks prior. Based on a high clinical suspicion of adrenal insufficiency, patient was initiated on glucocorticoid and mineralocorticoid therapy prior to laboratory confirmation of adrenal insufficiency. After stabilizing the patient and excluding pheochromocytoma, we proceeded with adrenal biopsy that revealed a nongerminal center-type diffuse large B-cell lymphoma. Our patient was treated with R-CHOP chemotherapy, with good response after 3 cycles but eventually died after the fifth cycle from neurologic complications. This case highlights the notion that primary adrenal insufficiency should be considered in patients presenting with bilateral adrenal masses. Although primary adrenal lymphoma is a very rare adrenal malignancy it should be considered in patients presenting with bilateral rapidly growing adrenal tumors and primary adrenal insufficiency.

Cushing's Syndrome Missed in Pregnancy.

Cushing's syndrome (CS) is uncommon during pregnancy and often difficult to diagnose due to similar symptoms shared with normal pregnancy. This case report discusses a 26-year-old woman who developed CS while pregnant, underlining the importance of early detection and the diagnostic challenges involved. The patient presented with gestational diabetes and pre-eclampsia during pregnancy. Post delivery, she continued to experience hypertension and facial swelling, which led to a diagnosis of CS. The patient underwent a successful laparoscopic adrenalectomy, which normalized her blood pressure and improved her symptoms. This case highlights the need for heightened awareness of CS in pregnant women exhibiting both gestational diabetes and hypertension, as early diagnosis and treatment are essential to reduce maternofoetal complications.

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.

Adrenal insufficiency - causes and laboratory diagnosis.

Adrenal insufficiency (AI) manifests as a clinical syndrome arising from either the direct impairment of adrenal glands, leading to primary AI characterized by deficiencies in glucocorticoids and mineralocorticoids, or adrenal cortex atrophy due to diminished adrenocorticotropic hormone (ACTH) stimulation, a consequence of hypothalamic and/or pituitary damage, resulting in secondary AI. The diagnosis of AI is based on clinical assessment and biochemical tests, including basal hormone level measurements and stimulation tests. In evaluating the results of laboratory tests, it is necessary to consider factors that may influence both pre-analytical and analytical phases, as well as the chosen methodology. Correct diagnosis of adrenal insufficiency and timely initiation of suitable replacement therapy are paramount. These steps are crucial not only for managing the condition but also to avert potentially life-threatening adrenal crises.

Texture analysis can predict response to etoposide-doxorubicin-cisplatin in patients with adrenocortical carcinoma.

BACKGROUND: The adrenocortical carcinoma (ACC) is a rare and highly aggressive malignancy originating from the adrenal cortex. These patients usually undergo chemotherapy with etoposide, doxorubicin, cisplatin and mitotane (EDP-M) in case of locally advanced or metastatic ACC. Computed tomography (CT) radiomics showed to be useful in adrenal pathologies. The study aimed to analyze the association between response to EDP-M treatment and CT textural features at diagnosis in patients with locally advanced or metastatic ACCs. METHODS: We enrolled 17 patients with advanced or metastatic ACC who underwent CT before and after EDP-M therapy. The response to treatment was evaluated according to RECIST 1.1, Choi, and volumetric criteria. Based on the aforementioned criteria, the patients were classified as responders and not responders. Textural features were extracted from the biggest lesion in contrast-enhanced CT images with LifeX software. ROC curves were drawn for the variables that were significantly different (p < 0.05) between the two groups. RESULTS: Long-run high grey level emphasis (LRHGLE_GLRLM) and histogram kurtosis were significantly different between responder and not responder groups (p = 0.04) and the multivariate ROC curve combining the two features showed a very good AUC (0.900; 95%IC: 0.724-1.000) in discriminating responders from not responders. More heterogeneous tissue texture of initial staging CT in locally advanced or metastatic ACC could predict the positive response to EDP-M treatment. CONCLUSIONS: Adrenal texture is able to predict the response to EDP-M therapy in patients with advanced ACC.

Bulky Isolated Adrenal Metastasis as First Presentation of Occult Hepatocellular Carcinoma (HCC) in a Patient with a Synchronous Squamous Carcinoma of the Tongue.

BACKGROUND: The diagnostic workup of an adrenal mass should always rule out the possibility of an adrenal metastasis, especially in a patient followed-up for a known primitive cancer. Sometimes, however, the incidental finding of a bulky lesion in a cancer patient can lead to the unexpected diagnosis of metastasis from a second occult cancer. Here, we report the case of a voluminous, isolated left adrenal metastasis from unknown and persistently occult hepatocellular carcinoma (HCC), incidentally found during the follow-up for squamous carcinoma of the tongue. CASE DESCRIPTION: A 72-year-old HBV/HCV-negative male patient with a history of alcohol abuse was referred to our hospital for gastric bleeding. Some weeks before, the patient was operated on for a locally advanced squamous cell carcinoma of the tongue, which required cervical lymph node neck dissection, temporary tracheostomy, jejunostomy, and plastic reconstruction. Subsequent diagnostic imaging revealed a left adrenal mass sized 9x15 cm with suspicious features. The hormonal workout was negative for pheochromocytoma and a hyperfunctioning adrenal lesion. The patient underwent laparotomic left adrenalectomy. The exploration of the liver was compatible with alcoholic cirrhosis and did not reveal any other palpable lesion. The adrenal mass histologically turned out to be a poorly differentiated G3 HCC. Subsequent radiological exams were unable to identify the primary liver lesion or any other neoplasms. Conversely, α-FP levels were initially high but reduced after treatment with sorafenib. After 2 years of follow-up, the patient is alive and well, albeit with evidence of locoregional inter-aortocaval lymphadenopathy. The primary HCC has never been identified, thus suggesting the hypothesis of a diffuse cirrhosis-like HCC. CONCLUSION: The suspicion of an adrenal metastasis in a patient with primary cancer with a low potential for adrenal metastatic spreading must raise the diagnostic suspect for another synchronous occult cancer beyond that for primary adrenal cancer. HCC can rarely first manifest as a metastatic adrenal lesion.

The molecular genetics of adrenal cushing.

Adrenal Cushing represents 20% of cases of endogenous hypercorticism. Unilateral cortisol-producing adenoma (CPA), a benign tumor, and adrenocortical carcinoma (ACC), a malignant tumor, are more frequent than bilateral adrenal nodular diseases (primary bilateral macronodular adrenal hyperplasia (PBMAH) and primary pigmented nodular adrenal disease (PPNAD)).In cortisol-producing adrenal tumors, the signaling pathways mainly altered are the protein kinase A and Wnt/ß-catenin pathways. Studying components of these pathways and exploring syndromic and familial cases of these tumors has historically enabled identification of many of the predisposing genes. More recently, pangenomic sequencing revealed alterations in sporadic tumors.In ACC, mainly due to TP53 alterations causing Li-Fraumeni syndrome, germline predisposition is frequent in children, while it is rare in adults. Pathogenic variants in the DNA mismatch repair genes MLH1, MSH2, MSH6, and PMS2, which cause Lynch syndrome or alterations of IGF2 and CDKN1C (11p15 locus) in Beckwith-Wiedemann syndrome, can also cause ACC. Rarely, ACC is described in other hereditary tumor syndromes due to germline pathogenic variants in MEN1 or APC and, in very rare cases, NF1, SDH, PRKAR1A, or BRCA2. Concerning ACC somatic alterations, TP53 and genetic or epigenetic alterations at the 11p15 locus are also frequently described, as well as CTNNB1 and ZNRF3 pathogenic variants.CPAs mainly harbor somatic pathogenic variants in PRKACA and CTNNB1 and, less frequently, PRKAR1A, PRKACB, or GNAS1 pathogenic variants. Isolated PBMAH is due to ARMC5 inactivating pathogenic variants in 20 to 25% of cases and to KDM1A pathogenic variants in food-dependent Cushing. Syndromic PBMAH may be due to germline pathogenic variants in MEN1, APC, or FH, causing type 1 multiple endocrine neoplasia, familial adenomatous polyposis, or hereditary leiomyomatosis-kidney cancer syndrome, respectively. PRKAR1A germline pathogenic variants are the main alteration causing PPNAD (isolated or part of Carney complex).

Cryptococcal invasion: a comprehensive case of adrenal and systemic infection amid immunosuppression.

We report a case of disseminated cryptococcosis, an uncommon fungal infection predominantly affecting the lungs and central nervous system, with the rare involvement of adrenal cryptococcosis, compounded by meningitis and pneumonia. The patient, previously diagnosed with primary myelofibrosis and undergoing oral Ruxolitinib treatment, exhibited immunosuppression. Imaging via chest and abdominal CT scans revealed inflammation in the right lung's middle lobe, splenomegaly, a splenic lesion, and a left adrenal mass, initially prompting considerations of pheochromocytoma. However, unilateral adrenalectomy and subsequent pathological examination disclosed extensive infiltration by inflammatory and multinucleate giant cells, with Periodic acid-Schiff (PAS) staining confirming the diagnosis. The identification of adrenal cryptococcosis was further supported by positive adrenal pus culture and significantly elevated capsular antigens in both serum and cerebrospinal fluid, at titers of 1:2560. Following a month of oral antifungal treatment, marked reductions in capsular antigen levels were noted, to 1:640 and 1:160 in serum and cerebrospinal fluid, respectively. The patient was discharged on a regimen of oral amphotericin B, flucytosine, and fluconazole, with regular outpatient follow-ups showing no signs of recurrence or dissemination.

Role of radiologists in the diagnosis and management of adrenal disorders.

This study aimed to focus on the role of radiologists in the diagnosis and management of adrenal lesions, particularly primary aldosteronism (PA) and secondary hypertension. As hypertension affects more than one-third of the population in Japan, identifying secondary causes such as PA and adrenal lesions is crucial. Establishing a radiological differential diagnosis of adrenal lesions using advanced imaging techniques, such as computed tomography and magnetic resonance imaging, is crucial. Knowledge of the imaging findings of various benign and malignant adrenal lesions, such as adrenocortical adenomas, cortisol-producing lesions, pheochromocytomas, adrenocortical carcinoma, malignant lymphoma, and metastatic tumors, is necessary. Adrenal venous sampling (AVS) plays a crucial role in accurately localizing aldosterone hypersecretion in PA, especially when imaging fails to provide a clear diagnosis. This paper details the technical aspects of AVS, emphasizing catheterization techniques, anatomical considerations, and the importance of preprocedural imaging for successful sampling. Furthermore, we explore segmental adrenal venous sampling (SAVS), a more refined technique that samples specific adrenal tributary veins, offering enhanced diagnostic accuracy, particularly for microadenomas or challenging cases that may be missed with conventional AVS. The methodology for performing SAVS, along with the interpretation criteria for successful sampling and lateralization, is also outlined. Furthermore, radiologists have initiated treatments for unilateral PA, such as radiofrequency ablation, and play an integral role in the management of adrenal lesions. Collaborative approaches across clinical departments are required to enhance patient management in medical care involving the adrenal gland.

Update on clinical characteristics in the evaluation of phaeochromocytoma and paraganglioma.

Pheochromocytomas and sympathetic paragangliomas (PPGL) are rare neuroendocrine tumors originating from chromaffin tissue of the adrenal medulla and extra-adrenal sympathetic paraganglia. Historically, many of these tumors were diagnosed postmortem, earning pheochromocytomas the moniker "great mimic" due to their diverse clinical manifestations that can resemble various other conditions. Over time, the clinical presentation of PPGL has evolved, with a shift from symptomatic or postmortem diagnoses to more frequent incidental discoveries or diagnoses through screening, with postmortem identification now being rare. The development of a clinical scoring system has improved the identification of patients at increased risk for PPGL. Notably, the proportion of PPGL patients with normal blood pressure ranges from 15 % to 40 %, varying based on the clinical context. Despite the tumor's reputation, PPGL is an exceedingly rare cause of resistant hypertension. Management of a pheochromocytoma crisis has advanced, with several classes of drugs available for treatment. However, PPGL during pregnancy remains a significant concern, associated with substantial maternal and fetal mortality rates. Additionally, PPGL can present as rare disorders, including catecholamine-induced cardiomyopathy, Cushing syndrome, and urinary bladder PGL. Given these varied presentations, heightened awareness and prompt recognition of PPGL are crucial for timely diagnosis and treatment, ultimately improving patient outcomes. In this article, we offer an in-depth analysis of the diverse clinical presentations of PPGL, highlighting their complexity and the associated diagnostic and treatment strategies.

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.