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ORPHA:1501

Adrenocortical carcinoma

medium confidenceDisorder

Also known as: ACC

Publications

17,938

97.5th percentile

Trials

68

Interventional, condition-specific

Researchers

1,398

Distinct authors in sample

Gene link

—

Readiness

4/6

Stages with a signal

Clinical definition (Orphanet)

A rare malignant adrenal disease characterized by a tumor that arises from the adrenal cortex. The clinical presentation depends on the hormones secreted; in children virilization is a predominant feature; other manifestations can include symptoms of Cushing syndrome or symptoms related to tumor volume or metastases.

How rare: 1-9 / 1 000 000 — roughly one to nine people per million. In a city the size of Kolkata, perhaps a few dozen.

Orphanet entry

Cross-references

Joined from Mondo / Orphanet. MeSH labels may enter searches; UMLS / OMIM / NCIT are stored for reference.

Additional Mondo synonyms (12)

adenocarcinoma, adrenocortical, malignant · adrenal cortex adenocarcinoma · adrenal cortex carcinoma · adrenal cortical adenocarcinoma · adrenal cortical carcinoma · adrenal cortical carcinoma (morphologic abnormality) · adrenocortical carcinoma · adrenocortical carcinoma (disease) · carcinoma of adrenal cortex · carcinoma of the adrenal cortex · carcinoma, adrenocortical, malignant · cortical cell carcinoma

Research stages

Trial readiness signals

Where this condition sits on an open-data research pipeline — not how close a treatment is, and not medical advice. Empty stages often mean “not in these databases under this Mondo ID,” not “impossible.”

4/6 stages with a signal

An interventional trial matched this condition name on ClinicalTrials.gov — see trials below.

  1. Gene identifiedNot found

    No GenCC disease–gene assertion in this build

  2. LiteraturePresent

    17,938 matched papers (11,912 in last 10 years) Source

  3. Phenotype characterisedPresent

    31 HPO annotations (e.g. Adrenocortical carcinoma; Abnormality of reproductive system physiology; Hypertrichosis) Source

  4. Animal modelNot found

    No Alliance genotype “model of” associations via Monarch for these Mondo IDs

  5. Orphan designationPresent

    2 FDA · 4 EMA designations (2 FDA orphan-indication approvals) — e.g. linsitinib Source

  6. Interventional trialPresent

    68 matched on ClinicalTrials.gov (18 recruiting in sample)

Biology

Genes and phenotypes

Gene–disease validity from GenCC, plus phenotypes and animal models joined from Monarch Initiative via Mondo ID — not a clinical diagnosis aid.

Do we know what causes it?

Not yet — the cause hasn't been pinned down in GenCC.

No strong gene–disease assertion joined for this Orphanet entity.

Phenotypes (Monarch / HPO)

31

Associated phenotypes · MONDO:0006639

  • Adrenocortical carcinoma
  • Abnormality of reproductive system physiology
  • Hypertrichosis
  • Abnormality of metabolism/homeostasis
  • Panic attack

Showing 5 of 31 — open Monarch for the full list.

Animal models (Monarch / Alliance)

None returned for this Mondo ID. Empty here is not proof that no model organism work exists under another name or gene.

Monarch fetch 2026-07-29

Therapies

Designations, candidates, and chemicals

FDA OOPD and EMA orphan designations, Open Targets clinical candidates, and CTD chemical associations via MyDisease.info. These never change the interventional-trial headline.

Orphan designation (FDA · EMA)

6

Designations · 2 with FDA orphan-indication approval

  • FDA linsitinibAdrenocortical carcinoma · 2012-03-09 · Not FDA Approved for Orphan Indication
  • FDA Gossypolcancer of the adrenal cortex · 1990-10-22 · Not FDA Approved for Orphan Indication
  • EMA N-[2,6-bis(1-methylethyl)phenyl]-N'-[[1-[4-(dimethylamino) phenyl]cyclopentyl]methyl]urea, hydrochloride saltTreatment of adrenocortical carcinoma · 07/06/2013 · PositiveEMA designation
  • EMA mitotaneTreatment of adrenal cortical carcinoma · 12/06/2002 · ExpiredEMA designation
  • EMA mitotaneTreatment of adrenal cortical carcinoma · 11/09/2002 · WithdrawnEMA designation
  • EMA LinsitinibTreatment of adrenal cortical carcinoma · 02/04/2012 · WithdrawnEMA designation

Sources: FDA OOPD · EMA orphan designations

Open Targets candidates

47

Drugs / clinical candidates · MONDO_0006639

CTD chemicals (MyDisease.info)

No CTD chemical associations returned for this Mondo ID.

Literature

Is anyone studying this?

17,938

17,938 papers — among the better-studied rare conditions, though still a fraction of common-disease literature (breast cancer: over 700,000). Median papers in the last 10 years for a rare disease in this dataset (publications denominator n=3967) is 59.

17,938 papers since the earliest indexed year in this search — median last-10-year count for a rare disease in this dataset is 59 (publications denominator n=3967).

11,912 in the last 10 years · medium confidence · 97.5th percentile (publications denominator)

Phrase hits: 17,938 · MeSH hits: 0

Open Europe PMC search

Who's working on it?

1,398

Distinct author names in 200 sampled papers — named people below.

Who's working on it?

People publishing on this condition (sampled Europe PMC records). Affiliation is the most recent found in that sample.

  1. 01
    Berruti A9 papers · 2026

    Department of Medical and Surgical Specialties, Radiological Sciences and Public Health, University of Brescia, ASST-Spedali Civili, Brescia, Italy.

    Papers in Europe PMC
  2. 02
    Cosentini D9 papers · 2026

    Department of Medical and Surgical Specialties, Radiological Sciences and Public Health, University of Brescia, ASST-Spedali Civili, Brescia, Italy.

    Papers in Europe PMC
  3. 03
    Laganà M9 papers · 2026

    Medical Oncology Department, ASST Spedali Civili of Brescia, Brescia, Italy.

    Papers in Europe PMC
  4. 04
    Altieri B6 papers · 2026

    Department of Internal Medicine I, Division of Endocrinology and Diabetes, University Hospital, University of Wuerzburg, 97080 Wuerzburg, Germany.

    Papers in Europe PMC
  5. 05
    Fassnacht M6 papers · 2026

    Department of Internal Medicine I, Division of Endocrinology and Diabetes, University Hospital, University of Wuerzburg, 97080 Wuerzburg, Germany.

    Papers in Europe PMC
  6. 06
    Habra MA6 papers · 2026

    The University of Texas MD Anderson Cancer Center, Houston, TX, USA.

    Papers in Europe PMC
  7. 07
    Kimpel O6 papers · 2026

    Department of Internal Medicine I, Division of Endocrinology and Diabetes, University Hospital, University of Wuerzburg, 97080 Wuerzburg, Germany.

    Papers in Europe PMC
  8. 08
    Sigala S6 papers · 2026

    Department of Molecular and Translational Medicine, University of Brescia, Brescia, Italy.

    Papers in Europe PMC
  9. 09
    Tamburello M6 papers · 2026

    Department of Molecular and Translational Medicine, University of Brescia, Brescia, Italy.

    Papers in Europe PMC
  10. 10
    Abate A5 papers · 2026

    Department of Molecular and Translational Medicine, University of Brescia, Brescia, Italy.

    Papers in Europe PMC

Clinical research

Is a treatment being tested?

68

interventional trials for this specific condition

68 interventional trials matched this specific condition name; 18 currently recruiting in our sample.

Data as of 11 September 2026

68 interventional trials — more than 77.2% of diseases in the trials denominator have none at all (5501 of 7126; this disease is at the 98th percentile).

medium confidence · 98th percentile (trials denominator)

Recruiting interventional trials

From the matched ClinicalTrials.gov set

68 interventional trials matched after quoted-phrase search and title/condition post-filter.

Observational and natural-history studies

21 observational studies match this condition. These do not test a treatment and are not counted in the interventional-trial headline, but they are genuine research: natural-history work often defines the endpoints needed for a future rare-disease trial, and families may be able to enroll.

Recruiting or not-yet-recruiting

Open the complete matched search on ClinicalTrials.gov

Other registries (secondary)

Broader net from EU CTIS, ISRCTN, and ICTRP when available — deduped against ClinicalTrials.gov IDs already counted above. Dual-model LLM relevance gates what we keep. These rows are not added to the interventional headline.

raw 70 · after dedupe 70 · already on CT.gov 0 · kept 0 · parent 0 · uncertain 70 · dropped 0 · fetched 2026-07-29

Source notes: ictrp: Error: ICTRP public search unavailable (WHO portal is SPA-only; SOAP needs partnership). Tried: https://apps.who.int/tri

No secondary-registry studies passed dual-model relevance for this condition name (after dedupe).

Uncertain / not reviewed (70)

Where to find support

Condition-specific patient organisations, when Orphanet lists them, are on the disease’s Orphanet page. We also link umbrella groups that support undiagnosed and ultra-rare families.

Orphanet entry for Adrenocortical carcinoma — check Associations / patient organisations on that page.

India — NPRD

Last verified 2026-07-26

This ORPHAcode is not on our curated NPRD list (direct or Mondo-parent match). That does not decide clinical eligibility; families in India should ask a notified Centre of Excellence about current coverage.

Hand-curated for this project. ORPHAcode mappings are best-effort and may be incomplete or imprecise for umbrella categories. Parent (Mondo) matches mean the policy lists a broader category — confirm eligibility with a Centre of Excellence. Financial entitlements summarised from public policy statements and may change. This is not official government guidance.

How we counted this

Europe PMC query (preferred label + any corrected label + Orphanet and Mondo exact synonyms, stoplisted; unioned with resolved MeSH labels when available). UMLS / OMIM / NCIT cross-references are stored on the overview but are not added to the query string.

"Adrenocortical carcinoma" OR "adenocarcinoma, adrenocortical, malignant" OR "adrenal cortex adenocarcinoma" OR "adrenal cortex carcinoma" OR "adrenal cortical adenocarcinoma" OR "adrenal cortical carcinoma" OR "adrenal cortical carcinoma (morphologic abnormality)" OR "adrenocortical carcinoma (disease)" OR "carcinoma of adrenal cortex" OR "carcinoma of the adrenal cortex" OR "carcinoma, adrenocortical, malignant" OR "cortical cell carcinoma"

Run this search on Europe PMC

ClinicalTrials.gov query (quoted phrases + MeSH via query.cond, plus recall-expansion terms when used):

"Adrenocortical carcinoma" OR "adenocarcinoma, adrenocortical, malignant" OR "adrenal cortex adenocarcinoma" OR "adrenal cortex carcinoma" OR "adrenal cortical adenocarcinoma" OR "adrenal cortical carcinoma" OR "adrenal cortical carcinoma (morphologic abnormality)" OR "adrenocortical carcinoma (disease)" OR "carcinoma of adrenal cortex" OR "carcinoma of the adrenal cortex" OR "carcinoma, adrenocortical, malignant" OR "cortical cell carcinoma"

Interventional trials matched via: phrase (mesh = registered under a MeSH descriptor no name phrase would catch; recall-expansion = gene / selected parent terms used only for trials).

Study-type breakdown: 68 interventional · 21 observational · 0 expanded access. Only interventional studies enter the trial headline.

Query health: ok — strategies attempted: phrase; with hits: phrase

Run this search on ClinicalTrials.gov

Synonyms dropped by stoplist: ACC

Confidence reasoning

  • Preferred label is multi-word and distinctive
  • 1 synonym(s) dropped by stoplist (may under-count)
  • No label/synonym collisions with other diseases in this corpus

Ingested 2026-07-26T17:34:31.445Z