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Unusual Hormonal and Ectopic Presentations of ACC

Special Populations

Adrenocortical carcinoma (ACC) may rarely present through atypical steroidogenic syndromes or from adrenal cortical tissue located outside the normal adrenal glands. Within ACC care, these presentations are most often discussed as uncommon functional phenotypes—especially aldosterone-producing or deoxycorticosterone (DOC)-producing tumors—or as ectopic/extra-adrenal cortical malignancies arising from adrenal rest tissue.123 They remain biologically plausible manifestations of adrenal cortical malignancy, but they are distinctly uncommon relative to both conventional ACC and the far more prevalent benign adrenal disorders that enter the same diagnostic pathways.45

These presentations usually first appear within standard endocrine or anatomic workups rather than through a syndrome specific to ACC. Aldosterone-producing ACC may resemble routine primary aldosteronism, whereas DOC-producing tumors may cause a hypermineralocorticoid state despite low or normal aldosterone levels.647 Ectopic ACC, by contrast, may initially be classified radiographically as a renal, retroperitoneal, gastrointestinal, or gonadal mass until pathology demonstrates adrenal cortical origin.8910

The evidence base is limited and heterogeneous. Most published data consist of case reports, small retrospective series, and narrative reviews collected over decades during which endocrine assays, imaging, and pathologic criteria changed substantially.11112 As a result, reported frequencies, phenotype-specific prognostic claims, and proposed distinguishing features should be interpreted cautiously. Mechanistic studies using ACC cell lines help illustrate steroidogenic plasticity, but they do not establish diagnostic or treatment standards in patients.131415

Diagnostic context

Unusual hormonal ACC is rare within an already rare cancer. In patients undergoing evaluation for primary aldosteronism, benign aldosterone-producing adenoma and bilateral hyperplasia remain much more common than carcinoma, and adrenal CT is used partly to exclude malignancy rather than because ACC is a frequent cause of aldosterone excess.16517 Similarly, an extra-adrenal abdominal or pelvic mass is far more likely to represent a non-ACC neoplasm than ectopic cortical carcinoma.183

What is relatively reliable is that standard endocrine workflows can identify a mineralocorticoid-excess pattern and standard imaging can localize a lesion. What is not reliable is the inference that localization or biochemical lateralization distinguishes benign from malignant cortical disease. Clinically, unusual severity, discordant steroid profiles, large tumor size, or atypical anatomy may justify broader evaluation and more explicit attention to malignancy.1920

Mineralocorticoid-producing phenotypes

Aldosterone-producing ACC

Aldosterone-producing ACC is a very rare cause of primary aldosteronism and an uncommon functional phenotype within ACC overall.21212 Reported patients generally have hypertension, hypokalemia, metabolic alkalosis, and renin suppression, but the syndrome is heterogeneous; normotensive presentations and presentations dominated by weakness or paralysis from profound potassium depletion have also been described.222324

Across pooled reports and retrospective series, malignant cases tend to be associated with larger tumors, more severe biochemical disturbance, and more frequent mixed steroid secretion than typical aldosterone-producing adenoma.111925 However, some ACCs show apparently isolated aldosterone excess and may even resemble lipid-poor adenomas on imaging.262728 The dependable clinical lesson is that severe or atypical primary aldosteronism should not automatically be treated as benign; the less dependable assumption is that a “pure” aldosterone phenotype excludes carcinoma.

A smaller subset of ACC appears to produce DOC or related intermediates, causing hypertension, hypokalemia, and renin suppression despite low or normal aldosterone.6729 These cases broaden the concept of mineralocorticoid-producing ACC beyond classic aldosterone excess and may fall outside conventional primary aldosteronism algorithms.

This pattern is clinically important but supported mainly by rare reports. The practical implication is that low aldosterone does not rule out an adrenal cortical source of mineralocorticoid-type hypertension, particularly when the clinical syndrome is severe or imaging shows a suspicious adrenal mass; in selected cases, broader steroid profiling may be informative.3031

Mixed and shifting steroidogenesis

Steroid secretion in unusual functional ACC may be mixed at presentation or may change over time. Recurrent or metastatic disease may lose aldosterone production or evolve toward broader glucocorticoid or androgen excess, limiting the reliability of a single hormone as a long-term disease marker.323334 Molecular data also suggest that aldosterone-producing ACC is genetically closer to other ACC phenotypes than to benign aldosterone-producing adenoma, despite overlapping clinical presentation.15

What appears reasonably consistent is that steroidogenesis in ACC is often disorganized and biologically plastic. What remains uncertain is how often phenotype switching occurs and whether any specific hormonal pattern predicts outcome. In practice, biochemical follow-up may help but should not substitute for standard oncologic surveillance.3512

Ectopic and extra-adrenal ACC

Ectopic ACC refers to adrenal cortical malignancy arising outside the orthotopic adrenal gland, presumably from adrenal rest tissue. Reported sites include the renal hilum, retroperitoneum, mesentery, abdominal wall, paratesticular or gonadal tissue, and bowel wall.836910 Some reported tumors are nonfunctional, whereas others produce cortisol, androgens, or mixed steroids.373

The main diagnostic issue is anatomic misclassification. On imaging, these lesions may resemble renal, mesenchymal, hepatic, gastrointestinal, retroperitoneal, or gonadal tumors, and definitive diagnosis usually depends on pathology and immunohistochemistry rather than preoperative certainty.373 Benign literature on heterotopic adrenal tissue in the kidney, liver, and pelvis supports the biologic plausibility of adrenal-rest lesions in these locations, but does not imply that ectopic ACC is a common explanation for extra-adrenal masses.383940

The reliable conclusion is that ectopic cortical malignancy can occur and can be hormonally active. The unreliable area is any strong site-specific or phenotype-specific prognostic inference, because nearly all evidence comes from isolated reports. Clinically, concordance between steroid excess and a non-adrenal mass may raise suspicion, but pathology remains decisive.

Differential-diagnostic pitfalls

These uncommon ACC presentations overlap with much more common benign adrenal disorders and with non-ACC paraneoplastic endocrinopathies. In mineralocorticoid excess, the principal comparators are aldosterone-producing adenoma and bilateral hyperplasia; in hypercortisolism, ectopic ACTH syndrome and other non-adrenal causes remain important alternatives.54142 Historical and modern non-ACC literature consistently shows that severe hypokalemic alkalosis, hypercortisolism, and even adrenal enlargement may arise from extra-adrenal malignancies with secondary adrenal hyperplasia rather than primary ACC.434445

This caution is better supported as a general diagnostic principle than as a quantifiable risk estimate. Hormone excess alone does not localize the source to the adrenal cortex, and abnormal adrenal imaging in a patient with cancer does not by itself establish ACC.4647 Similarly, rare non-cortical adrenal tumors may produce unusual systemic syndromes and complicate early diagnostic framing.48 The practical implication is that endocrine interpretation should remain anchored to anatomy, full biochemical characterization, and pathology.

Role in management and research

Recognition of unusual hormonal or ectopic presentation mainly affects suspicion, staging, and surgical planning rather than creating a separate management paradigm. In a patient entering a primary aldosteronism workflow, large size, suspicious imaging features, severe biochemical excess, or mixed steroid secretion may increase concern for ACC and favor oncologic resection principles over approaches designed for presumed benign adenoma.1920 By contrast, standard localization tests such as adrenal venous sampling may lateralize hormone excess without establishing whether the lesion is benign or malignant.2820

Available data do not show that unusual hormonal or ectopic presentation confers a clearly better prognosis than conventional ACC. Aldosterone-producing ACC can recur or metastasize despite initial biochemical remission, and outcome in ectopic ACC appears to depend largely on stage and resectability, although comparative data are sparse.11136 A broader hereditary or syndromic context may occasionally be relevant, especially in very young patients or those with multiple endocrine neoplasms, but evidence remains limited to selected reports.495051

Research interest in these phenotypes centers on steroidogenic heterogeneity and adrenal-rest biology. At present, the most reliable conclusion is that unusual hormonal and ectopic ACC presentations expand the clinical spectrum of ACC without yet defining distinct evidence-based management pathways. Larger registries and more standardized endocrine, pathologic, and molecular characterization are likely required before these rare presentations can be classified with confidence.1512

Included Articles

  • PMID 1529666: This case report describes an unusual hormonally atypical scenario in which severe primary hyperaldosteronism coexisted with a contralateral nonsecreting adrenocortical carcinoma. In vitro steroidogenesis studies showed the carcinoma had minimal mineralocorticoid and glucocorticoid synthesis, while the opposite adrenal Conn’s adenoma was the source of aldosterone excess.52
  • PMID 1877141: This case report describes a rare nonfunctioning adrenocortical rest carcinoma arising ectopically in the renal hilum, where it mimicked a renal tumor on imaging and was recognized after nephrectomy. The report highlights aberrant adrenal tissue as an unusual site of ACC-like malignancy outside the orthotopic adrenal gland.8
  • PMID 1179589: This case series describes aldosterone-producing adrenocortical carcinoma as an uncommon presentation of ACC, often mimicking primary aldosteronism. Patients may show severe hypokalemia and marked hyperaldosteronuria without clear glucocorticoid or androgen excess, and symptomatic response to spironolactone does not exclude carcinoma.22
  • PMID 3476050: This case report describes an unusual aldosterone-producing adrenocortical carcinoma in a 27-year-old man presenting with severe primary hyperaldosteronism, malignant hypertension, and intracerebral hemorrhage, without cortisol, androgen, or estrogen excess. It highlights isolated mineralocorticoid-secreting ACC as a rare hormonally atypical presentation in a young patient.53
  • PMID 3751090: This case series highlights aldosterone-producing adrenocortical carcinoma as a rare hormonally unusual ACC variant presenting with severe hypertension, hypokalemia, suppressed renin, and adrenal masses, with postoperative normalization of aldosterone and potassium after tumor resection in the reported patients.54
  • PMID 4722250: This case report describes a rare hormonally unusual ACC presenting with isolated mineralocorticoid excess, causing hypertension, hypokalemia-related neuromuscular symptoms, polyuria, and polydipsia without clinical hypercortisolism. It also documents an exceptionally late local recurrence 14 years after initial resection, with symptom resolution after repeat tumor removal.55
  • PMID 5343398: This case report describes the rare phenotype of aldosterone-secreting adrenocortical carcinoma presenting with severe hypertension, hypokalemic alkalosis, elevated urinary aldosterone, and suppressed plasma renin activity. The review of prior cases suggests many such tumors also co-secrete other adrenal steroids despite limited overt clinical signs of cortisol or androgen excess.56
  • PMID 6087704: This case series describes the uncommon variant of aldosterone-producing ACC presenting as primary aldosteronism. Compared with benign aldosterone-producing adenoma, patients had more severe hypokalemia, higher aldosterone and deoxycorticosterone levels, absent aldosterone circadian rhythm, and later progression to broader glucocorticoid and androgen excess.57
  • PMID 6342747: This case report describes the rare variant of aldosterone-producing adrenocortical carcinoma presenting as isolated primary hyperaldosteronism, with hypertension, hypokalemia, suppressed renin, and elevated aldosterone but no excess of other measured steroids. The review notes that only a handful of similar cases had been reported, underscoring this unusual hormonal phenotype.58
  • PMID 7183438: This case report describes a rare hormonally unusual ACC presenting as apparently pure primary aldosteronism, with severe hypertension, hypokalemia, suppressed renin, and markedly elevated aldosterone but without clear cortisol or androgen excess. The tumor was large and pathologically malignant despite this atypical endocrine phenotype.59
  • PMID 7792813: This review notes that primary aldosteronism can rarely be caused by adrenocortical carcinoma and may occur within multiple endocrine neoplasia type I contexts, where adrenal hyperplasia, adenoma, and carcinoma can coexist. It frames aldosterone-producing carcinoma as part of a broader hereditary endocrine neoplasia spectrum rather than a typical ACC presentation.49
  • PMID 8574286: This case report describes a rare deoxycorticosterone-producing adrenocortical adenoma causing mineralocorticoid-type hypertension and hypokalemia with low renin and low aldosterone, while cortisol, androgen, and estrogen excess were absent. The report also notes that DOC-producing adrenocortical tumors are extremely rare and may be either benign or malignant.60
  • PMID 9523526: This series notes that aldosterone-producing adrenocortical carcinoma is a rare cause of primary aldosteronism, accounting for about 1% of cases in the article’s framing. In the cohort, one patient had an 11 cm cortical carcinoma without nodal or distant metastases and remained free of recurrent disease 36 months after adrenalectomy.61
  • PMID 9647521: This case report describes a rare aldosterone-producing adrenocortical carcinoma presenting as normotensive primary aldosteronism with severe hypokalemia and recurrent quadriparesis. It highlights an unusual hormonal phenotype in ACC in which mineralocorticoid excess may occur without the expected hypertension.23
  • PMID 9890267: This case report describes an uncommon aldosterone-producing ACC presenting with severe hyperaldosteronism, hypokalemia, edema, polyuria, and normal blood pressure, illustrating normotensive hyperaldosteronism as a rare hormonal phenotype. The tumor also retained steroidogenic enzyme expression and aldosterone production in a xenograft model.62
  • PMID 10364761: This case report describes a rare 11-deoxycorticosterone-producing adrenocortical carcinoma presenting with hypertension, severe hypokalemia, muscle weakness, and elevated DOC despite normal aldosterone. The report frames DOC-producing ACC as an uncommon hormonally unusual variant that can mimic primary aldosteronism-like hypermineralocorticoid states.6
  • PMID 10800765: This case report describes an extremely rare hormonally unusual ACC presenting as pure primary aldosteronism without biochemical evidence of cortisol or androgen excess. It also notes that larger size and calcification in an aldosterone-producing adrenal tumor may raise suspicion for malignancy.26
  • PMID 10943735: This case report describes an extremely rare renin-producing adrenocortical carcinoma presenting with hypertension, severe hypokalemia, hyperreninemia, and concurrent cortisol and sex steroid excess. Immunostaining of metastatic tissue supported cortical origin and renin production, highlighting an unusual hormonally mixed ACC phenotype.63
  • PMID 11224006: This case report highlights aldosterone-producing ACC as a rare hormonally unusual presentation that can mimic Conn’s syndrome, with severe hypertension, hypokalemia, and metabolic alkalosis. It also notes that metastatic progression may be accompanied by loss of aldosterone secretion, limiting aldosterone as a follow-up marker.32
  • PMID 12490848: This retrospective series describes aldosterone-secreting ACC as a rare hormonally unusual variant, occurring in 11% of ACC cases in this surgical cohort and presenting with universal hypertension and hypokalemia, frequent severe biochemical hyperaldosteronism, and occasional mixed cortisol or androgen secretion. Compared with non-aldosterone-secreting ACC, it was associated with higher perioperative mortality but a numerically longer overall survival.11
  • PMID 12852254: This review notes that mineralocorticoid hypertension and primary aldosteronism are usually caused by aldosterone-producing tumors or adrenal hyperplasia, and only rarely by adrenal carcinoma. It also outlines inherited steroidogenic disorders that can produce hypertension through excess mineralocorticoid pathway activation.30
  • PMID 13275491: This case report describes an adrenocortical carcinoma presenting with isolated mineralocorticoid excess, causing severe hypokalemia, hypernatremia, hypertension, thirst, polyuria, and muscular weakness without cushingoid, virilizing, or carbohydrate abnormalities. Symptoms and electrolyte disturbances improved with potassium replacement and normalized after adrenal tumor removal, then recurred with metastatic relapse.64
  • PMID 15251840: This case report describes a rare pure aldosterone-producing adrenocortical carcinoma presenting with hypertension, hypokalemia, alkalosis, and a high aldosterone-to-renin ratio without cortisol, catecholamine, or androgen excess. It highlights that aldosterone-secreting ACC can recur years later and may lose its original hormonal phenotype.33
  • PMID 15788646: This review characterizes aldosterone-producing adrenocortical carcinoma as a rare cause of Conn’s syndrome, usually presenting with hypertension and hypokalemia but with highly variable pathology and clinical behavior. Reported outcomes were often poor, with frequent later metastases and no reliable clinical or histologic predictor of survival.1
  • PMID 16358925: This case report describes an uncommon aldosterone-producing adrenocortical phenotype: bilateral functioning adrenocortical adenomas causing primary aldosteronism, with coexisting renal cell carcinoma. It highlights the diagnostic challenge of distinguishing bilateral adenomas from idiopathic hyperaldosteronism and the use of partial adrenal-sparing surgery to reduce long-term steroid dependence.65
  • PMID 18689909: This case report highlights aldosterone-producing ACC as a very rare hormonally unusual variant presenting with primary hyperaldosteronism, hypokalaemic hypertension, and a unilateral adrenal mass. It also shows that recurrent metastatic disease may shift to glucocorticoid and androgen excess without recurrent hyperaldosteronism.34
  • PMID 18941436: This case describes probable ectopic cortisol-producing ACC arising from bilateral testicular adrenal rest cells, presenting with severe ACTH-independent Cushing syndrome and widespread metastatic disease. It highlights a rare extra-adrenal, paratesticular presentation in which distinction from malignant Leydig cell tumor remained difficult.37
  • PMID 19961896: Using the NCI H295R adrenocortical carcinoma cell line alongside primary bovine adrenal glomerulosa cells, this study shows that angiotensin II activates protein kinase D via the AT1 receptor and that protein kinase D enhances acute aldosterone secretion. The ACC-specific relevance is as a mechanistic model for aldosterone-producing adrenocortical cells rather than direct clinical evidence in ACC patients.13
  • PMID 20482833: This review notes that aldosterone-producing adrenocortical carcinoma is a very rare surgically correctable cause of primary aldosteronism, presenting with unilateral aldosterone hypersecretion, renin suppression, hypertension, and sometimes hypokalemia. The article situates this phenotype within the broader diagnostic workup for primary aldosteronism rather than providing ACC-specific management details.4
  • PMID 21106862: This mechanistic study used the H295R adrenocortical carcinoma cell line as a model of aldosterone regulation and found that PPAR-gamma activation suppressed angiotensin II-induced CYP11B2 expression and aldosterone secretion. The findings are relevant to hormonally unusual aldosterone-producing ACC biology rather than general ACC management.14
  • PMID 22707896: This case report highlights the rare presentation of aldosterone-producing adrenocortical carcinoma without hypertension, showing that ACC can rarely manifest as normotensive primary aldosteronism with hypokalemia and suppressed renin despite typical expectations of hypertension.24
  • PMID 23033463: This veterinary case report describes an aldosterone- and corticosterone-secreting adrenocortical carcinoma with liver metastases in a dog, including endocrine findings, pathology, and prolonged survival after adrenalectomy followed by hepatic metastasectomy. It highlights an unusual nonhuman ACC presentation rather than informing routine human ACC care.66
  • PMID 24048685: This retrospective series addresses the rare aldosterone-producing variant of adrenocortical carcinoma, reporting that in primary aldosteronism, severe hypokalemia, markedly elevated aldosterone-renin ratio, and tumor size greater than 4 cm were associated with malignant cases. The authors recommend open adrenalectomy rather than an endoscopic approach when these features are present.19
  • PMID 24505527: This case report describes aldosterone-producing ACC in a 22-year-old woman, highlighting a rare mineralocorticoid-secreting presentation with severe hypertension, hypokalemia, and metabolic alkalosis. The course was complicated by pregnancy-related follow-up interruptions, delayed recognition, local recurrence, and liver metastasis.67
  • PMID 24682757: This review highlights aldosterone-secreting ACC as a rare clinical variant, noting pure hyperaldosteronism in about 2.5% of ACC cases. The reported patient presented with hypertension, hypokalemia, and elevated aldosterone with low renin, illustrating that ACC can rarely mimic a primary aldosteronoma.21
  • PMID 25949323: This case report describes a 20-year-old man with primary aldosteronism caused by aldosterone-producing adrenocortical carcinoma, highlighting an unusually young presentation of a hormonally rare ACC subtype. The report emphasizes that ACC can present with secondary hypertension and hypokalemia even outside the usual peak age ranges.68
  • PMID 26161274: This case report describes an exceptionally rare aldosterone-producing adrenocortical carcinoma occurring alongside primary hyperparathyroidism, highlighting possible multiple endocrine neoplasia context and the need to screen for syndromic endocrine tumors when ACC presents with unusual hormonal phenotypes.50
  • PMID 26273475: This case report describes pure aldosterone-secreting adrenocortical carcinoma as an exceptionally uncommon hormonally unusual ACC that can mimic a benign aldosterone-producing adenoma, lack typical malignant imaging features, and present with recurrent primary hyperaldosteronism after apparently curative adrenalectomy.27
  • PMID 28127190: This case report describes a very rare hormonally unusual ACC phenotype: bilateral aldosterone-producing adrenocortical carcinoma in a 22-year-old man presenting with resistant hypertension and persistent hypokalemia. It highlights bilateral disease and mineralocorticoid excess as uncommon manifestations that should be considered in selected patients.69
  • PMID 28259701: In a retrospective unilateral adrenalectomy series for primary aldosteronism, 2 of 43 resected lesions were adrenocortical carcinomas, indicating that aldosterone-producing ACC can occur as an uncommon pathologic substrate of hyperaldosteronism. The report otherwise focuses on blood pressure outcomes after surgery rather than ACC-specific management.70
  • PMID 29938936: This review notes that aldosterone-producing adrenocortical carcinoma is a very rare cause of primary hyperaldosteronism, in contrast to the far more common bilateral adrenal hyperplasia and aldosterone-producing adenoma. It places aldosterone-secreting ACC within the spectrum of hormonally unusual adrenal cortical tumors.2
  • PMID 30475217: This study characterizes a rare hormonally unusual ACC subset with primary aldosteronism or hyperreninemic hyperaldosteronism. Among 18 identified cases, autonomous aldosterone production was present in many, but common aldosterone-producing adenoma mutations were absent and the overall mutational landscape resembled other ACC hormonal phenotypes.15
  • PMID 31051469: This case report describes a deoxycorticosterone-secreting adrenal cortical neoplasm identified during the third trimester of pregnancy, presenting with longstanding resistant hypertension, hypokalaemia, low renin, and low or normal aldosterone. It highlights pregnancy-specific management constraints, including MRI-based evaluation, avoidance of mineralocorticoid antagonists, and deferral of adrenalectomy until after delivery.31
  • PMID 31423440: This case report describes a rare nonfunctional ectopic adrenocortical carcinoma arising on the anterior abdominal wall, presenting as a very large abdominal mass without steroid excess. It highlights aberrant adrenal tissue as a possible origin and documents complete surgical resection with short-term disease-free follow-up.36
  • PMID 31695023: This review identifies adrenocortical carcinoma as a rare cause of primary aldosteronism, placing aldosterone-producing ACC within hormonally unusual adrenal cortical tumors. It notes that CT imaging in primary aldosteronism is used to exclude malignancy, highlighting ACC in the differential diagnosis of hyperaldosteronism.16
  • PMID 32138431: This case report describes a rare deoxycorticosterone-secreting ACC presenting with hypermineralocorticoidism, including hypertension, hypokalemia, suppressed renin, markedly elevated DOC, and normal aldosterone. It highlights that mineralocorticoid-producing ACC can show mixed steroid excess and an aggressive clinical course.7
  • PMID 32158565: This case report describes primary aldosteronism arising from CYP11B2-positive adrenocortical micronodules rather than a functional adrenal tumor, with persistent concern for contralateral aldosterone overproduction after unilateral adrenalectomy. It highlights aldosterone-producing micronodular disease as an unusual adrenal cortical phenotype relevant to rare hormonally atypical adrenal neoplasia contexts.71
  • PMID 32912887: This case report highlights aldosterone-producing adrenocortical carcinoma as a very rare hormonally unusual ACC subtype that can present with hypertension, persistent hypokalemia, and periodic paralysis. It emphasizes considering ACC in the differential of hyperaldosteronism when adrenal imaging shows a large lipid-poor suspicious mass.72
  • PMID 32944927: Aldosterone-producing ACC is described as an exceptionally rare functional presentation that can mimic typical primary aldosteronism, including small adrenal tumors initially interpreted as lipid-poor adenomas. The report emphasizes careful evaluation of adrenal masses in hyperaldosteronism and notes that recurrent disease may rarely show a changed hormonal phenotype.28
  • PMID 33599784: This review notes that aldosterone-producing adrenocortical carcinoma is a very rare cause of primary hyperaldosteronism, accounting for less than 1% of cases, and that adrenal CT in the diagnostic workup is intended in part to exclude adrenal carcinoma.5
  • PMID 33953990: This case report describes a rare aldosterone-producing ACC with hepatic metastases presenting with resistant hypertension, hypokalemia, and subclinical cortisol excess. It highlights aldosterone-producing ACC as an unusual hormonal variant and notes that hepatic venous sampling did not detect aldosterone production from liver metastases despite pathology and steroidogenic immunostaining supporting metastatic ACC.73
  • PMID 34829937: This review notes that aldosterone-producing adrenocortical carcinoma is a rare cause of primary aldosteronism. It places ACC within the broader context of mineralocorticoid-excess disorders that can present with hypertension, hypokalemia, metabolic alkalosis, and an adrenal mass during workup.18
  • PMID 35261465: This case report describes an extremely rare ectopic adrenocortical carcinoma arising from the colonic mesentery, presenting with abdominal pain from mass effect and later developing hepatic metastases after resection. The report highlights mesenteric ectopic ACC as an unusual adult presentation distinct from typical adrenal primary disease.9
  • PMID 36583872: This letter synthesizes published cases of aldosterone-producing adrenocortical carcinoma, a rare hormonally unusual ACC variant presenting with primary aldosteronism. Compared with aldosterone-producing adenoma, APAC was associated with larger tumors, more severe aldosterone excess and hypokalemia, possible cortisol or androgen cosecretion, and poor outcomes.25
  • PMID 37312493: This case report describes aldosterone-producing adrenocortical carcinoma, a very rare hormonal variant that can mimic primary aldosteronism with hypertension, hypokalemia, elevated aldosterone-renin ratio, and lateralizing adrenal venous sampling. The review of reported cases highlights its rarity and poor outcomes, emphasizing that malignant imaging features should prompt surgical planning aimed at complete en bloc resection.20
  • PMID 37646544: This case report highlights ectopic adrenocortical carcinoma arising in retroperitoneal adrenal rest tissue as a rare ACC variant that can present with ACTH-independent Cushing syndrome and androgen excess. It emphasizes considering ectopic cortical tumors in the differential diagnosis of retroperitoneal masses with hormonal hypersecretion.3
  • PMID 38255973: This review notes that aldosterone-producing carcinoma is a rare cause of primary aldosteronism within the broader differential diagnosis of autonomous aldosterone excess. It places this entity alongside other uncommon causes such as unilateral hyperplasia, familial hyperaldosteronism, and ectopic aldosterone production.17
  • PMID 38344480: This case highlights aldosterone-producing or primary-aldosteronism-like ACC as a rare hormonally unusual variant that can mimic benign aldosterone-producing adenoma. Disorganized steroidogenesis with minimal CYP11B1/CYP11B2 staining may explain discordance between clinical hormone excess, tumor growth, and metastatic hormone output.35
  • PMID 38645425: This case report describes an exceptionally rare aldosterone-producing ACC occurring with papillary thyroid carcinoma and Graves disease, highlighting that hormonally unusual ACC phenotypes can coexist with other endocrine disorders and may prompt evaluation for hereditary predisposition even when identified variants are not clearly pathogenic.51
  • PMID 40533166: This case report describes an exceptionally rare sarcomatoid variant of ACC presenting with mineralocorticoid intermediate excess, marked by severe deoxycorticosterone elevation, hypokalemia, and hypertension. It highlights a monophasic spindle-cell sarcomatoid tumor with unusual steroidogenic activity and limited prior literature on this hormonally atypical subtype.29
  • PMID 41385120: This review summarizes aldosterone-producing ACC as a very rare functional variant, identified in 27 histologically confirmed cases. Reported presentations were dominated by hypertension and hypokalaemia, often with severe cardiovascular manifestations, while available data did not show clear morphological or molecular differences from other ACCs.12
  • PMID 13436879: A 1957 case report described marked adrenocortical hyperactivity in a patient with bronchial carcinoma, with severe steroid-related metabolic features but no adrenal cortical tumor found at necropsy. This indirectly reinforces that unusual steroid excess is not specific to ACC and can complicate endocrine differential diagnosis.43
  • PMID 14243867: An older lung carcinoma case reported elevated corticoids, hypokalemic alkalosis, and hypercalcemia without evidence of an adrenal cortical tumor. It reinforces the note’s caution that unusual steroid excess syndromes may reflect non-ACC paraneoplastic physiology rather than ACC itself.74
  • PMID 14289988: In a 1965 series of 100 patients with bronchogenic carcinoma, elevated plasma corticoid levels were frequent whereas overt Cushing syndrome was uncommon, highlighting that biochemical hypercortisolism in cancer is not specific for ACC. The report therefore supports the note’s caution about non-ACC endocrine mimics of unusual steroid excess.75
  • PMID 14323654: A 1965 case report of islet-cell carcinoma with Zollinger-Ellison syndrome described rapid adrenocortical hyperfunction, refractory hypokalemic alkalosis, and adrenal hyperplasia without ACC. It supports the note’s point that dramatic hypercorticoid or mineralocorticoid-like findings can arise as paraneoplastic endocrine phenomena in non-ACC malignancy.76
  • PMID 1483915: A 1992 veterinary case report described a ferret with adrenocortical carcinoma occurring alongside alopecia and mammary gland hyperplasia, interpreted as possibly hormone-related. Its relevance to human ACC is indirect, but it illustrates that adrenocortical carcinoma may be associated with unusual endocrine manifestations beyond classic presentations.77
  • PMID 15227962: A case report and review of ectopic ACTH syndrome described bronchial carcinoid causing ACTH-dependent Cushing syndrome with partly pituitary-like dynamic test responses, illustrating how non-ACC tumors can mimic more typical endocrine patterns. This indirectly strengthens the note’s caution that unusual hypercortisolism should not be considered specific for ACC.41
  • PMID 29772620: A 2018 prostate cancer case report adds another non-ACC example of ectopic ACTH syndrome with rapid-onset hypertension, hypokalemia, and hypercortisolism, ultimately traced at autopsy to ACTH-positive small-cell/neuroendocrine prostate carcinoma metastatic to the adrenal glands. This supports the note’s differential point that adrenal involvement and severe cortisol excess do not by themselves establish ACC.42
  • PMID 30568069: A 2019 case report described cyclic Cushing syndrome from an ACTH-secreting pulmonary carcinoid, with fluctuating biochemical hypercortisolism, misleading pituitary/adrenal findings, and remission after lung resection. This extends the note’s non-ACC differential by highlighting cyclic ectopic ACTH as another mimic of adrenal or pituitary causes of Cushing syndrome.78
  • PMID 37070449: A 2023 case report described nonfunctional ectopic ACC in the wall of the small bowel, broadening the reported extra-adrenal sites for ACC and underscoring the risk of anatomic misclassification when hormone excess is absent.10
  • PMID 4300297: A 1968 review of bronchial carcinoma described ectopic corticotrophin syndrome, inappropriate antidiuretic hormone secretion, hypercalcemia, and other paraneoplastic endocrine abnormalities. For this ACC note, its main relevance is confirmatory: severe cortisol excess with hypokalemic alkalosis may arise from non-ACC malignancy and mimic adrenal disease.79
  • PMID 5839222: An older five-case series of bronchogenic carcinoma described ectopic ACTH-type adrenocortical hyperfunction with hypokalemic alkalosis, high corticosteroid indices, poor dexamethasone suppression, and often minimal classic Cushingoid appearance, reinforcing that these findings are not specific for ACC.80
  • PMID 5975426: A 1966 bronchial carcinoma case report described concurrent hyponatremia and increased adrenal corticosteroid output without clinical Cushing syndrome, interpreted as dual paraneoplastic hormonal activity. This indirectly supports the ACC note’s caution that steroid-related abnormalities in cancer are not specific for adrenocortical carcinoma.81
  • PMID 10580910: A feline case report linked adrenocortical carcinoma to hypertrophic osteopathy that largely regressed after surgical excision, despite no detected pulmonary metastases. This provides indirect comparative evidence that ACC can be associated with unusual remote paraneoplastic manifestations outside classic endocrine syndromes.82
  • PMID 10590787: A canine case of presumed hepatic carcinoid with hypercortisolism, hypokalemia, and metabolic alkalosis adds indirect comparative support for the non-ACC differential of ectopic ACTH syndrome. Despite limited confirmation of the hormone source, it is consistent with the note’s existing emphasis that severe steroid-related biochemical disturbance is not specific for ACC.83
  • PMID 11206001: A veterinary case report of metastatic adrenocortical tumor in a cat described biochemically confirmed primary hyperaldosteronism with severe hypokalemia, weakness, hypertension, and a unilateral adrenal mass with venous invasion. Although nonhuman, it supports the note’s comparative point that malignant adrenocortical tumors can present with prominent mineralocorticoid-excess syndromes.84
  • PMID 12803238: A case report of virilizing adrenocortical adenoma with Cushing syndrome, papillary thyroid carcinoma, and hypergastrinemia indirectly informs ACC care by highlighting that mixed adrenal steroid excess can coexist with other endocrine or neoplastic processes. This supports cautious evaluation of possible syndromic context in unusual adrenal presentations, while not constituting evidence about ACC behavior itself.85
  • PMID 13367176: A 1956 case report described fulminant Cushing syndrome caused by pancreatic islet-cell carcinoma with bilateral adrenal hyperplasia, initially suspected to be adrenal carcinoma. It supports the existing differential point that severe hypercortisolism with hypokalemic alkalosis can arise from non-ACC malignancy and mimic primary adrenal cortical disease.86
  • PMID 13766951: A 1961 lung carcinoma case showed extreme bilateral adrenocortical hyperplasia and marked ACTH hyper-responsiveness without clinical Cushing syndrome or adrenal cortical neoplasia. It supports the existing differential point that unusual steroid testing abnormalities can reflect non-ACC paraneoplastic or secondary adrenal change rather than primary ACC.87
  • PMID 13810998: A 1960 case report described two patients with Cushing syndrome and bilateral adrenal hyperplasia in whom unusual bronchial adenomas were subsequently identified. Although not an ACC report and mechanistically limited by era, it provides early indirect support that intrathoracic tumors can mimic primary adrenal disease in hypercortisolism workups.88
  • PMID 13843339: A 1959 case report of metastatic prostate carcinoma documented severe hypokalemic alkalosis with increased urinary aldosterone and glucocorticoid excretion, with bilateral adrenocortical hyperplasia found at autopsy instead of ACC. It reinforces that striking steroid-related abnormalities in cancer can arise from non-ACC mechanisms and mimic adrenal cortical malignancy.89
  • PMID 13844016: A 1960 study of 22 men with lung carcinoma reported evidence of adrenal hyperactivity in some patients, with a highlighted case showing elevated steroid indices, exaggerated ACTH response, and bilateral adrenal cortical hyperplasia at autopsy despite no overt Cushing syndrome. This older non-ACC literature supports the differential point that cancer-associated steroid abnormalities may arise from secondary adrenal changes rather than ACC.90
  • PMID 13938341: A 1963 JAMA study in lung carcinoma described bilateral adrenal cortical hypertrophy without overt Cushing syndrome and proposed a tumor-derived corticotropin-like factor with adrenal weight-maintaining effects but limited steroid-releasing activity. This indirectly supports the ACC note’s caution that adrenal enlargement or steroid-related abnormalities in cancer are not specific for adrenocortical carcinoma.91
  • PMID 13999999: A 1963 lung carcinoma study found frequent biochemical evidence of adrenocortical activation and autopsy-confirmed adrenal hyperplasia without clinical Cushing syndrome or mineralocorticoid excess. This confirms the note’s existing caution that steroid abnormalities in patients with cancer may reflect non-ACC paraneoplastic or secondary adrenal physiology rather than primary adrenal cortical malignancy.92
  • PMID 14065884: A 1963 bronchogenic carcinoma case with increased adrenal steroid output but no adrenal cortical tumor features reinforces that severe steroid abnormalities in cancer can reflect ectopic or secondary adrenal activation rather than ACC. The report is indirectly relevant and mainly supports the note’s differential-diagnostic caution.93
  • PMID 14169497: A 1964 lung carcinoma case with adrenocortical hyperfunction showed profound hypokalemic alkalosis, elevated urinary steroid indices, and adrenal hypertrophy/hyperplasia rather than ACC at autopsy. It supports the note’s existing point that severe endocrine and electrolyte abnormalities may arise from non-ACC paraneoplastic or secondary adrenal activation.94
  • PMID 14283380: A 1965 case report documented esophageal squamous cell carcinoma with severe hypokalemic alkalosis and hypercorticism, with ACTH-like activity demonstrated in hepatic metastases. This non-ACC article supports the note’s differential caution that ectopic ACTH from another malignancy can mimic adrenal or mineralocorticoid-producing ACC.95
  • PMID 1474334: A rat medullary thyroid carcinoma model with ectopic CRH expression caused elevated CRH, ACTH, and corticosterone with marked adrenal cortical hyperplasia and ultrastructural evidence of steroidogenic activation. Although indirect to ACC, it confirms that non-adrenocortical tumors can drive profound secondary adrenal hyperfunction that may mimic an adrenal primary process.96
  • PMID 18388437: A canine case report described metastatic adrenocortical carcinoma with combined aldosterone, cortisol, and corticosterone secretion, producing hypokalemia and clinical features of mixed endocrine excess. Although indirect to human ACC, it usefully reinforces the concept of heterogeneous, multi-steroid secretion in malignant adrenocortical tumors.97
  • PMID 222422: A 1979 case report described ACTH-secreting metastatic breast carcinoma causing severe ectopic ACTH syndrome, with biochemical hypercortisolism, dexamethasone non-suppression, and adrenal cortical hyperplasia at autopsy rather than ACC. This supports the note’s existing differential caution that striking hypercortisolism and hypokalemic alkalosis may reflect non-ACC malignancy.46
  • PMID 2982721: A 1985 case report described urinary bladder oat cell carcinoma with ACTH immunoreactivity and bilateral adrenal cortical hyperplasia at autopsy. While not an ACC report, it supports the differential point that ectopic ACTH syndromes can arise from unexpected extra-adrenal small-cell malignancies and may not show the full classic biochemical picture.98
  • PMID 4287900: A 1966 case series of nine patients with extra-adrenal neoplasms, mainly oat-cell bronchogenic carcinoma, reported severe adrenocortical hyperactivity with hypokalemic alkalosis, high cortisol indices, and adrenal hyperplasia rather than ACC. It reinforces that marked steroid-related and mineralocorticoid-like abnormalities in cancer can reflect ectopic or secondary adrenal activation from another malignancy, not primary adrenocortical carcinoma.99
  • PMID 4293813: A 1967 bronchial carcinoma case with assay-demonstrated tumor “corticotrophin” confirms the note’s existing caution that severe ACTH-dependent hypercortisolism with hypokalemic alkalosis and bilateral adrenal hyperplasia can mimic adrenal or pituitary disease without representing ACC. The report also notes only partial biochemical improvement with metyrapone and discusses the limits of pituitary-adrenal function tests for localization.100
  • PMID 4303970: A 1969 study of three patients with ACTH-producing bronchogenic carcinoma found that ectopic ACTH-associated Cushing syndrome could produce a distinctive urinary 17-ketosteroid profile, with increased 11-oxy fractions and reduced androsterone/dehydroepiandrosterone, in the setting of adrenal hyperplasia rather than ACC. This confirms the note’s existing diagnostic caution that abnormal steroid biochemistry in cancer is not specific for adrenocortical carcinoma.101
  • PMID 4311547: A 1968 bronchial carcinoma case showed high ACTH, hypercortisolism with blunted diurnal variation, measurable ACTH activity in tumor tissue, and transient endocrine regression with tumor-directed therapy. This supports the non-ACC differential for severe steroid excess and also illustrates that ectopic ACTH syndromes may occur without hypokalemia.102
  • PMID 4314206: A 1970 case report described ACTH-active primary liver carcinoma causing paraneoplastic hypercortisolism, adrenal hypertrophy, hypokalemic alkalosis, diabetes, pigmentation, and rapidly progressive myopathy. While not an ACC case, it reinforces the differential point that marked steroid-excess syndromes with weakness or myopathy may reflect ectopic ACTH from a non-adrenocortical tumor rather than ACC.103
  • PMID 4327364: A 1971 case report described oat-cell lung carcinoma with ectopic ACTH syndrome presenting first to psychiatric care as severe depression and a suicide attempt, later found to involve marked hypercortisolism, hypokalemia, and adrenal hyperplasia rather than ACC. It reinforces the diagnostic caution that dramatic ACTH-dependent Cushing syndrome may come from a non-adrenocortical malignancy and may lack classic external features early on.104
  • PMID 4336515: A 1972 case report documented ectopic ACTH syndrome from metastatic colonic adenocarcinoma using elevated plasma ACTH/cortisol, dexamethasone non-suppression, tumor ACTH assay, and a positive arteriovenous ACTH gradient across the primary lesion. It confirms that profound hypercortisolism with hypokalemic alkalosis may arise from a non-ACC gastrointestinal malignancy and complicate localization of the endocrine source.105
  • PMID 5677692: A 1968 series of patients with lung carcinoma found subclinical steroid hormone abnormalities, including elevated 17-hydroxycorticosteroid excretion, loss of cortisol diurnal rhythm, and increased estrogen excretion in some groups despite no overt endocrine syndrome. This confirms the note’s diagnostic caution that abnormal steroid testing in cancer patients may reflect non-ACC paraneoplastic or secondary endocrine effects rather than adrenocortical carcinoma.106
  • PMID 6243246: A paranasal sinus neuroendocrine carcinoma series included one case with ectopic ACTH-related hypercortisolism and bilateral adrenocortical hyperplasia, illustrating a non-ACC mimic of adrenal steroid excess; another case had calcitonin-producing tumor activity, underscoring broader paraneoplastic endocrine complexity in non-adrenal malignancy.107
  • PMID 6268276: A 1981 case series in small-cell lung carcinoma reported clinically overt ectopic ACTH syndrome in 5 of 157 patients, usually arising after initial diagnosis and often after partial chemotherapy response. Although not an ACC study, it reinforces that severe hypokalemic hypercortisolism with bilateral adrenal hyperplasia can reflect evolving ectopic ACTH production from non-adrenocortical malignancy rather than ACC.108
  • PMID 6323789: A small-cell lung carcinoma case showed clinically manifest ectopic ACTH and ADH syndromes together, with nonsuppressible hypercortisolism, hypokalemia, hyponatremia, and bilateral adrenal hyperplasia rather than ACC. This extends the note’s non-ACC differential by illustrating that multiple ectopic hormones from one tumor can mimic or obscure unusual adrenal presentations.109
  • PMID 8073560: A 1994 pathology case report identified true intrarenal heterotopic adrenal cortical tissue with preserved zonation and P450scc expression, suggesting steroidogenic potential. Although not an ACC case, it extends the note’s ectopic ACC section by supporting adrenal-rest tissue as a plausible substrate for rare extra-adrenal cortical malignancy near the kidney.38
  • PMID 9441583: Case report of metastatic esophageal squamous-cell carcinoma presenting with ectopic ACTH syndrome, severe hypercortisolism, hypokalemic alkalosis, and bilateral adrenal enlargement. It supports the existing differential point that apparent adrenal-pattern endocrine disturbance can reflect a non-ACC malignancy, even when adrenal imaging is abnormal and tumor ACTH staining is not demonstrable.45
  • PMID 14278782: A 1964 report of bronchial oat-cell carcinoma described profound adrenocortical hyperfunction with hypokalemic alkalosis and bilateral adrenal hyperplasia attributed to ectopic corticotropin-like activity. For ACC discussions, it provides indirect but relevant historical support that severe steroid excess and adrenal enlargement can arise from non-ACC malignancy and mimic adrenal cortical disease.44
  • PMID 20046505: A case report of intrahepatic ectopic adrenal adenoma extends the note’s discussion of ectopic cortical tissue by showing how adrenal-rest lesions can mimic primary hepatic tumors on CT and MRI. Although not an ACC case, it supports the plausibility and diagnostic difficulty of intrahepatic ectopic cortical neoplasms.39
  • PMID 31897433: A case report of thymic carcinoid with ectopic ACTH-dependent Cushing syndrome illustrates a key non-ACC mimic: severe hypercortisolism, hypokalemia, and atypical imaging findings can arise from extra-adrenal neuroendocrine tumors rather than adrenocortical carcinoma.47
  • PMID 32039708: This published erratum to an ACC case series on renal thrombotic microangiopathy corrected an author name only and did not modify the reported clinical content. Its relevance here is therefore limited to confirming the case-report-based nature of the evidence base for unusual ACC presentations.110
  • PMID 33889374: A case report of VIP-producing pheochromocytoma described an 11 cm adrenal mass initially considered adrenocortical adenoma versus carcinoma, later shown to be pheochromocytoma after catecholamine and VIP testing. For the ACC note, it adds an indirect but useful reminder that rare non-cortical adrenal tumors can mimic unusual functional ACC presentations and complicate early diagnostic framing.48
  • PMID 38680768: A 2024 case report of incidental mesosalpinx ectopic adrenal cortical tissue in an older woman extends the note’s discussion of ectopic adrenal-rest biology by adding a rare adult pelvic site and highlighting a pathology differential with metastatic clear cell renal cell carcinoma. The report is indirectly relevant to ACC because it concerns benign ectopic adrenal tissue rather than carcinoma, but it helps explain how extra-adrenal cortical lesions may be misclassified.40
  • PMID 40927294: A 2025 case report described the first documented adrenal cortical adenoma causing ACTH-dependent Cushing syndrome through ectopic CRH production. Although not ACC, it adds a rare adrenal-based mimic showing that an adrenal mass with severe hypercortisolism does not necessarily indicate cortisol-producing ACC or other ACTH-independent cortical disease.111
  • PMID 5503599: A historical study of advanced non-adrenal cancers found elevated cortisol, impaired dexamethasone suppression, and an association with poor prognosis, arguing that adrenocortical overactivity in cancer is not specific to ACC. This indirectly broadens the differential for apparent hypercortisolism in oncology patients.112

References

Footnotes

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