Introduction: Adrenocortical carcinoma (ACC) is an aggressive malignancy with high rates of recurrence even after surgical resection. Surgery remains the mainstay of treatment, while adjuvant options are limited. Mitotane is the only approved systemic therapy. Current guidelines recommend stereotactic body radiotherapy (SBRT) alongside adjuvant mitotane therapy in Rx, R1, R2 resections and in locally advanced disease. Case: We present a case of a 40-year-old female who presented with abdominal pain and was found to have a large heterogeneous left adrenal mass measuring 8.2 × 8.5 × 9.5 cm (Hounsfield Unit 63) on computed tomography (CT) imaging. Clinically, she was obese with a body mass index of 33.7 kg/m². No discriminatory feature of Cushing’s was present. Hormonal evaluation demonstrated autonomous cortisol secretion with failure of suppression on both overnight and low-dose dexamethasone suppression tests at 301 nmol/L and 313.6 nmol/L, respectively. DHEA, testosterone, and urinary metanephrine were within range. Hemoglobin A1c was 6.6%. She underwent open left adrenalectomy. Intra-operatively, a 12 × 10 cm adrenal tumor was identified with multiple areas of tumor rupture and spillage during mobilization. HPE confirmed high-grade ACC with high Weiss score of 8, Ki-67 index 60–80%, and mitotic count 54/50 hpf (pT2Nx). Post-operative CT imaging demonstrated a residual soft tissue lesion in the left adrenal bed (largest diameter 3.8 cm) with fluorodeoxyglucose avidity. We commenced adjuvant mitotane therapy, titrated to 2 g TDS with supraphysiological hydrocortisone replacement. Mitotane level was within therapeutic range (16 mcg/mL). She was deemed unsuitable for repeat surgery due to the proximity of the residual mass to the adjacent vessel and was planned for SBRT therapy after a multidisciplinary team discussion. Conclusion High-risk ACC with suspected residual disease remains a therapeutic challenge. While mitotane remains the cornerstone of adjuvant therapy, SBRT may represent a promising adjunctive local treatment modality in carefully selected patients. Further studies are required to define its role in improving local control and outcomes in ACC.
Adrenocortical Carcinoma ; Mitotane

Introduction: Cushing’s syndrome is a multisystem disorder with significant cardiovascular morbidity, yet presentation as acute heart failure is uncommon. When driven by adrenocortical carcinoma (ACC), the clinical course is often aggressive and rapidly fatal, with particularly poor out-comes in resourcelimited settings where access to therapy is constrained. Case: A 39-year-old previously well female presented with acute decompensated heart failure, newly diagnosed hypertension, type 2 diabetes mellitus, and obesity, preceded by a 3-year history of secondary amenorrhea and progressive weight gain. On admission, she exhibited florid Cushingoid features. Biochemical evaluation confirmed severe adrenocorticotropic hormone (ACTH)-independent hypercortisolism: morning serum cortisol 1,950 nmol/L, failure of suppression on overnight dexamethasone suppression test (post-ODST cortisol 2022.9 nmol/L), and elevated 24-hour urinary free cortisol (2,069 nmol/24 hours, 2.56 × upper limit of normal). Androgen excess was evident, with elevated dehydroepiandrosterone sulfate (DHEAS more than 27 µmol/L) and testosterone (9.91 nmol/L). ACTH was suppressed, supporting an adrenal source, while aldosterone was normal. Contrast-enhanced computed tomography demonstrated a large left adrenal mass (12 cm) with tumor thrombus extending into the inferior vena cava and renal veins, with extensive hepatic and pulmonary metastases, consistent with advanced ACC. Management was limited by disease severity and resource constraints. Ketoconazole was contraindicated due to transaminitis, and alternative steroidogenesis inhibitors were unavailable, leaving metyrapone as the only feasible option. Oncological therapy was deferred due to sepsis and clinical instability. Her course was fulminant, complicated by recurrent heart failure, sepsis, and metabolic derangements, culminating in refractory cardiopulmonary failure. She died within 1 month of diagnosis, prior to definitive oncological intervention. Conclusion Fulminant cortisol-secreting ACC may present catastrophically as acute heart failure and progress rapidly. Early recognition and timely access to multimodal cortisollowering therapy are critical, particularly in resourcelimited settings. In fulminant hypercortisolism, the challenge is not diagnosis—but timing.
Adrenocortical Carcinoma ; Hydrocortisone ; Heart Failure

Introduction: Adrenocortical carcinoma (ACC) is a rare and aggressive malignancy with a predilection for metastasis to the liver, lungs, and lymph nodes. Bone involvement is less common and typically occurs alongside widespread disease. Isolated skeletal progression without visceral involvement is unusual and not well characterized. Case: A 60-year-old female underwent left adrenalectomy in 2019 for an incidentally detected adrenal mass, which was reported as a benign adrenal cortical adenoma (Ki67 <3%). In 2022, she presented with persistent low back pain. Imaging demonstrated fluorodeoxyglucoseavid lesions involving the T12 vertebra and right ilium, without evidence of local recurrence or visceral metastases. Histopathological evaluation of a bone biopsy initially suggested a neuroendocrine neoplasm based on synaptophysin positivity. Following multiple expert reviews and integration of clinical, radiological, and immunohistochemical findings, a consensus diagnosis of metastatic ACC was established. She received palliative radiotherapy to symptomatic skeletal sites and subsequently completed six cycles of etoposide, doxorubicin, and cisplatin chemotherapy in 2023, achieving disease stabilization. Surveillance imaging in May 2025 demonstrated progression confined to the axial and appendicular skeleton, with no involvement of the adrenal bed or visceral organs. Mitotane therapy was initiated in December 2025. Ongoing management focuses on systemic disease control, symptom palliation, and multidisciplinary supportive care. Conclusion This case illustrates an uncommon pattern of ACC progression characterized by bone-dominant metastases in the absence of visceral disease. It also highlights the importance of reconsidering the initial histopathological diagnosis when clinical behavior is discordant. Vigilance for atypical metastatic patterns is warranted, even years after resection of an adrenal lesion initially classified as benign.
Adrenocortical Carcinoma

Adrenal cortical carcinoma (ACC) is a rare malignancy, and oncocytic ACC (OAC) is a rare histopathologic variant of ACC, with <70 cases documented worldwide as of 2021. This is a case of a 64-year-old female initially presenting with epigastric-to-periumbilical pain, left flank pain, nausea, vomiting, and bloatedness. Computed tomography (CT) urogram revealed a 12.2 x 12.7 x 15 cm left adrenal mass, with pulmonary metastases on chest CT. The patient was clinically and biochemically unremarkable based on the hormonal panel. Left thoracoabdominal adrenalectomy was done, and the left adrenal mass was identified as a high-grade non-functioning OAC on histopathology and immunohistochemistry. The patient showed clinical improvement on chemotherapy with etoposide, cisplatin, doxorubicin (EDP), without mitotane, due to local unavailability. In conclusion, while EDP + mitotane is the standard therapy for metastatic cases, EDP alone showed promising outcomes in improving quality of life and resolving paraneoplastic neuropathy in the absence of mitotane. To date and to the best of our knowledge, this is the first documented case of non-functioning OAC in the Philippines. DS-related Kaposi sarcoma (KS) progresses rapidly with early mucosal and systemic involvement. Early diagnosis and treatment are crucial as they significantly impact the outcome. This is a case of disseminated cutaneous and gastrointestinal KS in a 24-year-old Filipino male living with HIV, presenting with hyperpigmented violaceous subcutaneous nodules, odynophagia, progressive dysphagia, and symptomatic anemia. Capsule endoscopy demonstrated utility in diagnosing gastrointestinal KS lesions, highlighting its crucial role when conventional upper endoscopy was challenging due to oropharyngeal involvement. Diagnostic limitations, mainly the unavailability of human herpesvirus-8 (HHV-8) immunohistochemical stain, were addressed through high clinical suspicion. Multidisciplinary approach including chemotherapy with liposomal doxorubicin, radiotherapy, and supportive care eventually yielded good clinical response. This case emphasizes the importance of early diagnosis, timely intervention, and long-term surveillance in achieving favorable outcomes for patients with KS despite some diagnostic limitations.
Human ; Adrenocortical Carcinoma ; Abdominal Pain

Adrenocortical carcinoma (ACC) is a rare malignant tumour originating from the adrenal cortex. Half of the cases are functional, with ACTH independent autonomous cortisol production being the most common. It is rare for ACC to present with markedly elevated metanephrine levels which is a typical characteristic of pheochromocytoma. We report a case of a large functioning adrenal tumour with overlapping biochemistry features of ACC and pheochromocytoma. Biopsy confirmed the histopathological diagnosis of metastatic ACC.
Adrenocortical Carcinoma ; Pheochromocytoma

Adrenocortical carcinoma (ACC) accounts for 0.05-2% of all malignant tumors. Forty-five percent of ACCs with secretory function have excess glucocorticoids alone and only less than 1% secrete aldosterone.

This is a case of a 44-year-old Filipino female with hypertension and a 12-year-history of an incidentaloma of the left adrenal gland, with recent-onset complaints of increasing abdominal girth, purple striae, amenorrhea, moon facies and a dorsocervical fat pad. Laboratory findings revealed low potassium levels, non-suppressed cortisol on dexamethasone test suggesting Cushing’s syndrome and elevated aldosterone-renin ratio and plasma aldosterone concentration pointing to primary hyperaldosteronism. A computed tomography scan revealed a left-sided adrenal mass measuring approximately 23 cm in largest diameter suggestive of carcinoma without metastasis or lymph node involvement. Complete resection via open adrenalectomy was performed and histopathologic assessment revealed Adrenocortical Carcinoma with Weiss score of 4. The Ki-67 proliferative index was found to be >20%. Radiotherapy was done as an adjuvant treatment.

Although rare, co-secretion of cortisol and aldosterone can occur in functional tumors of adrenocortical carcinoma. Malignancy should always be considered in patients who present with a history of a unilateral adrenal mass and/ or in those with signs and symptoms of adrenal hormone excess. Thus, a proper assessment derived from a thorough medical history, physical examination and laboratory work-up is warranted in patients with an adrenal mass to ascertain the diagnosis and provide adequate management.

Adrenocortical carcinoma (ACC) is a rare and aggressive neoplasm with poor prognosis. We report a case of a 30-year-old female who presented with profound classic features of an adrenocorticotrophic hormone (ACTH)-independent Cushing’s syndrome (CS) and a large adrenal mass with massive venous tumor thrombosis of the entire inferior vena cava (IVC), left renal and adrenal veins confirmed by imaging. Adrenal biopsy histopathology and immunohistochemistry confirmed ACC. Systemic palliative chemotherapy was administered. This rare case presents a unique and atypical presentation of an extensive tumor thrombosis of IVC. With the advanced stage at diagnosis, aggressive nature and poor prognosis of the disease, there is still a need to determine viable therapeutic options for metastatic ACC associated with venous invasion.
Adrenocortical Carcinoma

Adrenocortical carcinoma is a rare type of endocrine malignancy with an annual incidence of approximately 1–2 cases per million. The majority of these tumors secrete cortisol, and a few secrete aldosterone or androgen. Estrogen-secreting adrenocortical carcinomas are extremely rare, irrespective of the secretion status of other adrenocortical hormones. Here, we report the case of a 53-year-old man with a cortisol and estrogen-secreting adrenocortical carcinoma. The patient presented with gynecomastia and abdominal discomfort. Radiological assessment revealed a tumor measuring 21×15.3×12 cm localized to the retroperitoneum. A hormonal evaluation revealed increased levels of estradiol, dehydroepiandrosterone sulfate, and cortisol. The patient underwent a right adrenalectomy, and the pathological examination revealed an adrenocortical carcinoma with a Weiss' score of 6. After surgery, he was treated with adjuvant radiotherapy. Twenty-one months after treatment, the patient remains alive with no evidence of recurrence.
Adrenal Gland Neoplasms ; Adrenalectomy ; Adrenocortical Carcinoma ; Aldosterone ; Dehydroepiandrosterone Sulfate ; Estradiol ; Gynecomastia ; Humans ; Hydrocortisone ; Incidence ; Male ; Middle Aged ; Radiotherapy, Adjuvant ; Recurrence

OBJECTIVE: To investigate the clinical and functional imaging examination and pathological features of adrenocortical carcinoma (ACC), in order to improve the diagnosis and treatment of ACC. METHODS: The clinical data of 93 patients with ACC were analyzed retrospectively. Their diagnosis, surgical treatment and follow-up of mitotane medcine therapy were madeaccording to clinical manifestations, adrenal endocrine function determination, imaging examination characteristics and histopathological results. RESULTS: Among the 93 patients, the age ranged from 11 to 76 years, with a median age of 48 years. The ratio of male to female was 1:1.2. Twenty-four hours urinary free cortisol (UFC) elevated in 86 cases, adrenocorticotropic hormone (ACTH) decreased in 88 cases, blood F rhythm disappeared in 82 cases, and 31 cases of aldosterone increased. Thirty-six cases of sexual hormone increased. Neuron specific enolase (NSE) increased in 27 cases. Insulin-like growth factor-1 (IGF-1) increased in 26 cases. Seventy-six cases of high-dose dexamethasone suppression test (HDDST) and low-dose dexamethasone supression test (LDDST) were not suppressed separately. There were 62 cases of hypertension, and typical Cushing manifestations in 81 cases. Blood glucose elevated in 54 cases. Hypokalemia was in 21 cases and androgen secretion increased in 36 cases. The maximum diameter of the tumor was 3-17 cm, with 6 cases of adrenal central vein, renal vein and inferior vena cava tumor thrombus. The recurrence time was 1.2-5.0 years after operation. Metastasis and recurrence were in 56 cases during the follow-up, lung metastasis in 13 cases, liver metastasis in 17 cases, retroperitoneal lymph node metastasis in 9 cases, lumbar metastasis in 7 cases, ovarian metastasis in 3 cases, abdominal wall and incision implantation in 4 cases. Fifteen cases with distant metastasis. Seventy-seven patients were treated with radical adrenalectomy, and the other patients were treated with renal and adrenal resection on the same side of the kidney. There were 5 cases of adrenal tumor with vena cava tumor thrombus in the removal of the tumor, and the other for the partial resection of the vena cava in 3 cases. Regarding the clinical stage, stage I was in 39 cases, stage II in 28 cases, stage III in 16 cases and stage IV in 10 cases. The patients were followed up for 8-69 months, and 43 patients survived more than 5 years. CONCLUSION Function imaging combined with clinical features and endocrine hormone levels have important roles in ACC early diagnosis. Radical excision is the only effective treatment. Adjuvant or adjuvant mitotane drug therapy can brused for the treatment of recurrence and metastasis ACC patients,. ACC is a tumor with high malignancy and poor prognosis.
Adolescent ; Adrenal Gland Neoplasms ; Adrenalectomy ; Adrenocortical Carcinoma ; Adult ; Aged ; Child ; Female ; Humans ; Male ; Middle Aged ; Neoplasm Recurrence, Local ; Retrospective Studies ; Young Adult

PURPOSE: Previous studies on adrenal incidentalomas (AIs) are limited by their retrospective design, small numbers of patients, Western populations, or use of an outdated imaging technique. We investigated the characteristics of AIs in Korean patients and compared them with those reported in the largest retrospective study in Italy to discover the effects of improved imaging techniques and ethnicity differences. MATERIALS AND METHODS: This was a prospective, multicenter, cross-sectional observational study including 1005 Korean patients. Levels of plasma adrenocorticotrophic hormone, 24-h urinary free cortisol (UFC), serum cortisol after a 1 mg-dexamethasone suppression test, 24-h urinary fractionated metanephrine, and plasma aldosterone and plasma renin activity were measured. All AIs were characterized using computed tomography (CT). RESULTS: Compared with the results of the Italian study, AIs in Korean patients were observed more frequently in men and predominantly on the left side. Korean patients with AIs were slightly younger, and fewer patients underwent surgery. Most AIs were nonfunctional in both studies, while fewer subclinical hypercortisolism and more primary aldosteronism (PA) cases were detected in Korean patients. In our study, high UFC levels showed very low sensitivity, compared to those in the Italian study. In pheochromocytoma or PA cases, there were no hormonal differences between the studies. AIs in Korean patients were smaller, such that a lower cutoff size for detecting adrenocortical carcinoma (ACC) could be warranted. CONCLUSION: Recent advances in CT technology were leveraged to provide accurate characteristics of AIs and to detect smaller ACCs.
Adrenocortical Carcinoma ; Adrenocorticotropic Hormone ; Aldosterone ; Cushing Syndrome ; Humans ; Hydrocortisone ; Hyperaldosteronism ; Italy ; Korea* ; Male ; Metanephrine ; Observational Study ; Pheochromocytoma ; Plasma ; Prospective Studies* ; Renin ; Retrospective Studies