ULTRA-RARE CANCEROvarian CancerWHO 5th Edition Classification

Small Cell Carcinoma of the Ovary, Hypercalcemic Type (SCCOHT)

Ultra-Rare Malignancy (< 1 case per 1,000,000 / year) • Clinical Staging, Genomic Targets & Vetted Specialists

A rare and aggressive ovarian tumour of young women, driven by inactivation of SMARCA4 (BRG1). Around two-thirds of patients have raised blood calcium at presentation, which is what gives the disease its name. Despite the name it is not related to small cell lung cancer.

Request Rare Cancer Tumor Board Find NCI Treatment CentersAsk Vani AI Navigator

Emergent Referral AdvisoryImmediate Action

Certain rare malignancies progress rapidly or carry acute risk of airway obstruction, acute spinal compression, hydrocephalus, or biopsy-seeding. Do not perform needle biopsy or surgery outside of an NCI-designated specialty sarcoma or neuro-oncology unit without multidisciplinary tumor board review.

Report immediately to an emergency department or tertiary oncologist upon:

  • Ovarian mass with hypercalcaemia in a woman under 40 - test SMARCA4
Who It Affects

Young women and adolescent girls; median age around 24 years

Annual Incidence

Fewer than 1 per million women per year

Clinical Evidence Review

Last audited: 2026-08-13 against NCCN, ESMO, and WHO 5th ed. guidelines.

Clinical Presentation & Hallmark Symptoms

Presenting signs most frequently observed across clinical case series

Abdominal or pelvic pain and a palpable mass
Abdominal distension
Symptoms of hypercalcaemia - thirst, confusion, constipation, nausea
Irregular menstrual bleeding
Unintended weight loss

Genomic Profiling & Defining Molecular Lesions

Critical diagnostic fusions, somatic mutations, and therapeutic targets

Rare malignancies frequently depend on distinct oncogenic drivers rather than conventional environmental carcinogens. Comprehensive Next-Generation Sequencing (NGS comprehensive panel) and FISH/IHC are mandatory to establish the true diagnosis and screen for basket trial agents.

SMARCA4 (BRG1) loss-of-functionGermline SMARCA4 in a subset

Standard-of-Care Treatment Protocol

Frontline and multimodal strategies established under international consensus guidelines

1

Surgical staging and cytoreduction

Administered in specialized high-volume oncology programs with subspecialty pathology and organ-preservation protocols.

2

Intensive multi-agent chemotherapy (platinum-based)

Administered in specialized high-volume oncology programs with subspecialty pathology and organ-preservation protocols.

3

High-dose chemotherapy with autologous stem cell rescue in selected patients

Administered in specialized high-volume oncology programs with subspecialty pathology and organ-preservation protocols.

4

Radiotherapy to the pelvis in some protocols

Administered in specialized high-volume oncology programs with subspecialty pathology and organ-preservation protocols.

5

Correction of hypercalcaemia as an urgent supportive measure

Administered in specialized high-volume oncology programs with subspecialty pathology and organ-preservation protocols.

Clinical Trial Advisory: For rare and ultra-rare malignancies, enrollment in an active clinical trial or expanded-access program is widely considered the preferred standard of care by ASCO and NCCN panels.

Prognosis & Disease Trajectory

Objective clinical outlook without false reassurance

Poor overall, with the majority of patients relapsing, but long-term remission is reported in early-stage disease treated with aggressive multimodal therapy including stem cell rescue.

Note: Statistics reflect cohort averages. Individual outcomes depend heavily on performance status, resectability, biomarker expression, and timely access to specialized tertiary care.

Active Research, Biomarkers & Clinical Trials

Novel investigational agents, phase I/II trials, and international rare disease consortia

EZH2 inhibition (tazemetostat) exploits the SMARCA4-deficient state and is the leading targeted strategy; immune checkpoint blockade has shown activity in case series and is under formal study.

Need Help Matching to an Active Rare Cancer Trial?

ByOnco scans ClinicalTrials.gov, NCI trial networks, and institutional registries for open patient cohorts.

Request Trial Match
Expert Clinicians

Verified Specialists for Small Cell Carcinoma of the Ovary, Hypercalcemic Type

Browse All Oncologists

Dr. Douglas A. Levine

Gynecologic Oncologist

25+ yrs exp

NYU Langone (formerly MSKCC)

New York, USA

Gynecologic OncologySCCOHTOvarian Cancers
Vetted DirectoryConsult Specialist

Dr. Jeffrey Low

Senior Consultant Gynecologic Oncologist

20+ yrs exp

National University Cancer Institute Singapore (NUH)

Singapore, Singapore

Gynecologic OncologyRare Ovarian CancersSCCOHT
Vetted DirectoryConsult Specialist

Prof. Susana Banerjee

Medical Oncologist

24+ yrs exp

Royal Marsden Hospital

London, United Kingdom

Gynecologic CancersRare Ovarian TumorsSCCOHT
Vetted DirectoryConsult Specialist

Dr. Amita Maheshwari

Professor & Head, Gyne-Oncology

30+ yrs exp

Tata Memorial Hospital

Mumbai, India

Gynecologic OncologyRare Ovarian CancersSCCOHT
Vetted DirectoryConsult Specialist
Hospital Network

Designated Cancer Centers with Dedicated Programs

Search All 72 NCI Centers

Memorial Sloan Kettering Cancer Center

NY

NCI-Designated Comprehensive Cancer Center

World-leading rare tumor board & pediatric solid tumor protocols

Proton TherapyCAR-T Cell TherapyRobotic Surgery
1,000+ Active Clinical TrialsView Center Profile

University of Texas MD Anderson Cancer Center

TX

NCI-Designated Comprehensive Cancer Center

Largest specialized rare cancer and sarcoma multidisciplinary program

Proton TherapyCAR-T Cell TherapyCyberKnife SBRT
1,200+ Active Clinical TrialsView Center Profile

Dana-Farber / Harvard Cancer Center

MA

NCI-Designated Comprehensive Cancer Center

Pioneering genomic molecular tumor boards & rare histologies

Proton TherapyCAR-T Cell TherapyPhase I Unit
1,100+ Active Clinical TrialsView Center Profile

Mayo Clinic Comprehensive Cancer Center

MN

NCI-Designated Comprehensive Cancer Center

High-volume surgical oncology & rare endocrine/neuroendocrine expertise

Proton TherapyCAR-T Cell TherapyCyberKnife SBRT
600+ Active Clinical TrialsView Center Profile

Frequently Asked Clinical Questions

Authoritative guidance on diagnosis, tumor boards, genomic markers, and care options

Related Rare Malignancies in Catalog

ultra-rare

Malignant Rhabdoid Tumor

ultra-rare

Hepatosplenic T-cell Lymphoma (HSTCL)

ultra-rare

T-cell Prolymphocytic Leukemia (T-PLL)

ultra-rare

Blastic Plasmacytoid Dendritic Cell Neoplasm (BPDCN)

Independent Tumor Board Review

Facing a Diagnosis of Small Cell Carcinoma of the Ovary, Hypercalcemic Type?

Rare cancers demand rare expertise. Connect your medical records to US NCI-fellowship trained oncologists, subspecialist pathologists, and active clinical trial investigators within 12 to 72 hours.

Upload Scans for Second Opinion Schedule Virtual Teleconsult