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malignant Sertoli-Leydig cell tumor

vr.tr.malignant-sertoli-leydig-cell-tumor · thing-q586009

Enable an agent to recognize a documented malignant Sertoli-Leydig cell tumor, assess its disease state and evidential uncertainty, and support specialist decisions about investigation, treatment and surveillance.

Thing Registry Cross-cutting context XCT.STA

Bundle → Layer → Finding → Questions Filled

6 bundles · 11 layers · 17 findings · 35 questions

Diagnostic identity Establish what makes this lesion a malignant member of the Sertoli-Leydig tumor family.

Sertoli-Leydig lineage

Record the integrated tissue evidence and competing diagnoses.

Integrated lineage evidence

Retain the morphology, ancillary results and specialist interpretation supporting the mixed tumor identity.

  1. Which sampled areas demonstrate Sertoli differentiation, Leydig differentiation and associated stroma? definition
  2. Which competing diagnosis remains plausible, and what morphology or ancillary result discriminates it? boundary
  3. Which specimen and reviewing pathologist support the final integrated diagnosis? provenance

Malignant designation

Separate the assigned malignant category from risk indicators and subsequently observed behavior.

Basis of malignancy

Record the exact justification for the malignant label without requiring metastasis or treating every adverse feature as sufficient.

  1. Which classification edition and diagnostic criteria justify calling this Sertoli-Leydig tumor malignant? definition
  2. Does the record establish malignancy, describe malignant potential only, or leave behavior uncertain? boundary
  3. What specialist review is needed if the behavior code and pathology narrative disagree? action
Histological composition Describe differentiation and variant components while preserving sampling limitations.

Differentiation and sampling

Connect the reported differentiation to the tissue actually examined.

Representative differentiation

Document the differentiation assessment, variation across the tumor and adequacy of representation.

  1. What differentiation category was assigned, and which architectural and cytological observations support it? definition
  2. Were morphologically distinct solid, cystic and suspicious areas sampled sufficiently to support that assessment? provenance

Retiform and heterologous elements

Preserve architectural variants and the identity of additional tissue components.

Component-specific interpretation

Record retiform architecture separately from heterologous epithelial or mesenchymal tissue and its interpreted behavior.

  1. Which retiform and heterologous components are present, and what proportion or distribution was reported? measurement
  2. Which heterologous components are interpreted as benign or malignant, and what supports each interpretation? boundary
  3. Could an overrepresented component have obscured Sertoli-Leydig differentiation in the original specimen? boundary
Extent and endocrine phenotype Represent the tumor's anatomical burden and functional manifestations as separate dimensions.

Ovarian extent

Reconstruct stage from operative, imaging and pathological evidence.

Stage and rupture evidence

Preserve primary site, extraovarian findings, capsule status and the completeness of staging.

  1. Which findings establish the ovarian primary, laterality and any pelvic, peritoneal, nodal or distant involvement? measurement
  2. Was rupture preoperative or intraoperative, and were surface involvement and washings assessed? provenance
  3. Which FIGO stage is supported, and which missing observations prevent a firmer assignment? boundary

Functional presentation

Relate endocrine symptoms and circulating measurements to this tumor episode.

Individual secretory phenotype

Record symptoms, hormone measurements and their timing without presuming that every tumor is clinically androgenic.

  1. What virilization, menstrual changes, pain or mass-related symptoms occurred, and when did they begin? measurement
  2. Which hormones or markers were abnormal before treatment, using which assays and reference intervals? measurement
  3. What evidence distinguishes tumor-associated androgen excess from another endocrine cause? boundary
DICER1 context Connect molecular findings to the tumor and to appropriate evaluation of constitutional predisposition.

Tumor molecular evidence

Record what was tested in tumor tissue and how it was interpreted.

Tumor variant interpretation

Preserve variant identity, laboratory interpretation and technical limitations without making a molecular result sufficient for diagnosis.

  1. Which DICER1 variants were detected in which tumor specimen, with what assay coverage and laboratory classification? provenance
  2. Does the molecular result support the integrated diagnosis, conflict with it or remain uninformative? boundary

Constitutional assessment

Link the tumor episode to genetics evaluation while leaving syndrome management to its own model.

Predisposition evidence and referral

Distinguish documented constitutional findings from tumor-only results, family history and unresolved testing.

  1. Has constitutional DICER1 testing been performed, and what specimen, result and interpretive limitations were reported? provenance
  2. What genetics review or additional evaluation is indicated under the applicable specialist guidance? action
  3. Which findings belong to this tumor episode and which should link to a separate predisposition-syndrome record? boundary
Management and disease course Connect specialist decisions and follow-up observations to the tumor's documented characteristics.

Treatment decision context

Capture why a particular surgical, systemic or observation strategy was selected.

Individualized management rationale

Record stage, histology, residual disease, fertility priorities and the evidence used by the treating team; pediatric guidance requires explicit assessment before application to adults. [NCI childhood ovarian treatment guidance](https://www.cancer.gov/types/ovarian/hp/child-ovarian-treatment-pdq)

  1. What operation was performed, what reproductive structures remain, and what macroscopic or microscopic disease remains? measurement
  2. How did stage, differentiation and any malignant heterologous component inform the specialist treatment decision? action
  3. Which guideline or study supports the selected approach for this patient's age and tumor subtype? provenance

Response and new lesions

Track disease state and investigate possible relapse using evidence appropriate to the original tumor.

Longitudinal disease attribution

Link serial imaging, pathology and informative markers to dated disease assessments; retain uncertainty when a new lesion could represent recurrence or another primary.

  1. Which clinical, imaging and individually informative marker findings support the current disease state? measurement
  2. What comparison with the original pathology and molecular findings supports calling a new lesion recurrent Sertoli-Leydig tumor? boundary
  3. What specialist surveillance plan and escalation triggers were agreed, including follow-up when baseline hormones were normal? action
Evidence and external alignment What the world already says about this thing, gathered so the model can be checked against it.

Reported evidence

Findings from the breadth pass, kept separate from the structural claims.

Check these first

Recalled without web access and unsourced; every item is a lead to verify.

  1. Which of these check these first hold for the sense of malignant Sertoli-Leydig cell tumor this model covers, and on what evidence? provenance

Kinds and varieties

Recalled without web access and unsourced; every item is a lead to verify.

  1. Which of these kinds and varieties hold for the sense of malignant Sertoli-Leydig cell tumor this model covers, and on what evidence? provenance

Identifiers and schemes

Recalled without web access and unsourced; every item is a lead to verify.

  1. Which of these identifiers and schemes hold for the sense of malignant Sertoli-Leydig cell tumor this model covers, and on what evidence? provenance

Standards and regulation

Recalled without web access and unsourced; every item is a lead to verify.

  1. Which of these standards and regulation hold for the sense of malignant Sertoli-Leydig cell tumor this model covers, and on what evidence? provenance

Real-world use

Recalled without web access and unsourced; every item is a lead to verify.

  1. Which of these real-world use hold for the sense of malignant Sertoli-Leydig cell tumor this model covers, and on what evidence? provenance

Failure modes and hazards

Recalled without web access and unsourced; every item is a lead to verify.

  1. Which of these failure modes and hazards hold for the sense of malignant Sertoli-Leydig cell tumor this model covers, and on what evidence? provenance

Neighbouring kinds and how to tell them apart

Recalled without web access and unsourced; every item is a lead to verify.

  1. Which of these neighbouring kinds and how to tell them apart hold for the sense of malignant Sertoli-Leydig cell tumor this model covers, and on what evidence? provenance

Classifiers Filled

Family
Thing Registry
Category
Cross-cutting context
Entry kind
thing
Plane
XCT
Domain
XCT.STA

What it is Filled

A malignant Sertoli-Leydig cell tumor is a sex cord-stromal neoplasm, usually arising in the ovary, that shows Sertoli-cell and Leydig-cell differentiation and has malignant biological potential or demonstrated invasive or metastatic behavior.

In scope

  • Evidence establishing Sertoli-Leydig differentiation and the basis for classifying the tumor as malignant
  • Histological differentiation, retiform architecture and separately characterized heterologous components
  • Primary site, surgical staging, rupture, dissemination and residual disease
  • Tumor-associated androgen production and individually informative circulating markers
  • Tumor and constitutional DICER1 findings relevant to this disease
  • Specialist management decisions, treatment response and recurrence assessment

Out of scope

  • Benign Sertoli-Leydig cell tumors and the full parent tumor family
  • Pure Sertoli cell tumors, pure Leydig cell tumors and other ovarian neoplasms
  • Non-neoplastic causes of androgen excess
  • DICER1-related tumor predisposition as a complete inherited syndrome
  • General ovarian anatomy, reproductive physiology and infertility care
  • Detailed surgical techniques, chemotherapy prescribing and drug pharmacology

Why it exists Filled

Enable an agent to recognize a documented malignant Sertoli-Leydig cell tumor, assess its disease state and evidential uncertainty, and support specialist decisions about investigation, treatment and surveillance.

Distinguishing features Filled

  • Require a pathology-supported Sertoli-Leydig diagnosis plus a separately documented malignant classification; the parent family includes both benign and malignant tumors. [NCI tumor definition](https://www.cancer.gov/publications/dictionaries/cancer-terms/def/sertoli-leydig-cell-tumor)
  • Assess the combined Sertoli, Leydig and stromal differentiation across sampled tissue; an isolated androgen-producing cell population does not establish this mixed tumor identity.
  • Resolve retiform areas through integrated morphology and immunohistochemistry because they can resemble epithelial tumors; retiform architecture alone does not establish malignant behavior. [Retiform tumor series](https://pubmed.ncbi.nlm.nih.gov/12147087/)
  • Identify heterologous tissue within the overall tumor architecture rather than automatically reclassifying the lesion as a separate epithelial, germ-cell or sarcomatous neoplasm. [Diagnostic pitfall case study](https://pmc.ncbi.nlm.nih.gov/articles/PMC6083536/)
  • Treat virilization as supporting clinical context, not a required diagnostic test: confirmed retiform tumors have also presented without virilization. [Retiform tumor series](https://pubmed.ncbi.nlm.nih.gov/12147087/)

What robots and AI may and may not do Filled

Must not

  • Diagnose or stage the tumour for a specific person.
  • Recommend or advise against surgery or chemotherapy for a person.
  • Disclose a person's diagnosis, fertility status or genetic results without consent.
  • Comment on changes in a patient's appearance in a demeaning way.

Only with a human decision

  • Any treatment, fertility or surgical decision.

May

  • Explain the tumour in general terms from reputable medical sources.
  • Direct a person with signs such as new body hair growth or pelvic pain to a qualified clinician.

Moral aspects Filled

  • Masculinising effects of the tumour can be distressing; dignity matters.
  • Young patients face hard choices between treatment and fertility.

Who is affected

  • Patients, often young women
  • Families
  • Relatives with inherited risk

Owners Filled

Steward

Nobody owns the disease; patients and their clinicians answer for care.

Master systems

  • Cancer registries

Links to other meta-models Filled

parent

  • Q18555257 - registry parent class
  • Q5474322 - registry parent class

What else AI and robots need to interact with it Filled

Identity and identifiers required Filled

  • Vercy registry: vr.tr.malignant-sertoli-leydig-cell-tumor
  • Wikidata: Q586009 (https://www.wikidata.org/wiki/Q586009)

Direct properties not applicable Not applicable

  • Diagnostic and malignant-classification status: Suspected, provisional, specialist-confirmed or disputed; classification authority, edition and stated malignant criteria
  • Primary anatomical site: Organ, laterality and evidence of primary origin; unresolved site explicitly recorded
  • Histological differentiation: Well, intermediate, poorly differentiated or unassessable, retaining the original report terminology
  • Retiform and heterologous composition: Retiform pattern present, absent or unassessed; heterologous tissue type, extent and component-specific benign or malignant interpretation
  • Tumor dimensions: Three dimensions in cm, with specimen or imaging method and date
  • Anatomical extent: FIGO stage and edition for ovarian disease, with site-level evidence and staging completeness
  • Capsule and rupture status: Intact, preoperative rupture, intraoperative spill or unknown; surface involvement and washings recorded separately
  • Endocrine and marker activity: Testosterone in nmol/L or ng/dL; other measured markers in reported units, with assay ranges and dates
  • DICER1 testing context: Tumor or constitutional specimen; variant, laboratory classification, assay coverage, date and untested status
  • Post-treatment disease state: No evidence of disease, residual disease, response, stable disease, progression, recurrence or indeterminate, with assessment date

Plane XCT: no invented physical properties.

Recognition not applicable Not applicable

Not applicable

This disease cannot be reliably identified by outward appearance and requires specialist assessment of clinical and pathological evidence.

Capabilities and actions required Filled

  • Assemble a specialist pathology review packet showing the evidence for both tumor lineage and malignant classification
  • Identify missing sampling, staging or rupture documentation that limits disease assessment
  • Compare hormone and imaging trajectories against the tumor's documented baseline phenotype
  • Prepare a multidisciplinary decision summary linking stage, histology, residual disease and reproductive priorities
  • Flag unresolved constitutional DICER1 assessment for genetics review
  • Compare new lesions with the original tumor to support assessment of recurrence, metastasis or another primary

Hazards and failure modes required Filled

  • Hormone excess can cause virilisation and other effects.
  • Some cases are linked to inherited DICER1 variants that affect relatives.

Standards and interfaces required Filled

  • WHO Classification of Tumours, Female Genital Tumours, International Agency for Research on Cancer.

Context of use required Filled

  • This diagnosis is encountered in specialist gynaecological oncology and pathology when evaluating a rare tumour with Sertoli-cell and Leydig-cell differentiation.
  • It is used in clinical records and multidisciplinary review to organise evidence about tumour classification, extent and behaviour.
  • Documented findings support specialist treatment planning and surveillance while preserving uncertainty about an individual tumour's course.

Sources Filled

  1. Wikidata item Q586009: malignant Sertoli-Leydig cell tumor - identity and sense of the item
  2. Wikipedia: Sertoli–Leydig cell tumour - general description of the item

Open questions

  • Which authoritative definition and behavior-classification edition should delimit this registry entry, particularly for intermediate differentiation and uncertain malignant potential?
  • Does the undefined registry entry intentionally include non-ovarian tumors, and which classifications and staging systems would apply to those instances?
  • How much independent prognostic information do retiform architecture and each heterologous component add after accounting for stage, differentiation and rupture?
  • Which patients benefit from adjuvant systemic treatment, and how should uncertainty from rare-tumor observational evidence be represented?
  • What surveillance duration, modalities and marker strategy best detect recurrence or a metachronous primary across age groups, secretory phenotypes and constitutional DICER1 states?

Machine files

Provenance

thing registry research (pass 2) · unreviewed

Built from: models/things/publications/thing-q586009/spec.json