Rare epithelial tumor of small intestine

Last reviewed

🖨 Print for my doctorAdvocacy Hub →
ORPHA:425368
Who is this for?
Show terms as
1FDA treatments3Active trials4Specialists8Treatment centers1Financial resources

Where are you in your journey?

UniteRare data is sourced from FDA.gov, ClinicalTrials.gov, Orphanet, OMIM, and NORD.
Report missing data

Overview

Rare epithelial tumors of the small intestine are uncommon cancers or abnormal growths that start in the cells lining the inside of the small intestine — the part of the digestive tract between the stomach and the large intestine. The small intestine is responsible for absorbing nutrients from food, so tumors here can interfere with digestion and overall health. These tumors can be benign (non-cancerous) or malignant (cancerous), and they include types such as adenocarcinoma, carcinoid tumors (also called well-differentiated neuroendocrine tumors), and other rare epithelial growths. Because the small intestine is long and hard to examine, these tumors are often found late. Symptoms can be vague at first, which makes early diagnosis challenging. People may experience belly pain, unexplained weight loss, nausea, or signs of a blockage in the intestine. Some tumors bleed slowly, causing anemia and fatigue. Treatment depends on the type and stage of the tumor but usually involves surgery to remove the growth, sometimes combined with chemotherapy or targeted therapy. Because these tumors are so rare, treatment is often guided by specialists at major cancer centers with experience in rare gastrointestinal cancers.

Also known as:

Key symptoms:

Abdominal pain or crampingUnexplained weight lossNausea and vomitingFeeling full quickly after eatingFatigue and weakness from anemiaDark or tarry stools (sign of bleeding)Blood in the stoolBloating or swelling of the abdomenDiarrhea or changes in bowel habitsYellowing of the skin or eyes (jaundice) if the tumor is near the bile ductA lump or mass felt in the abdomen

Inheritance

Sporadic

Usually appears on its own, not inherited from a parent

Age of Onset

Adult

Begins in adulthood (age 18 or older)

Orphanet ↗NORD ↗

FDA & Trial Timeline

3 events
Feb 2021Fecal Microbiota Transplant and Re-introduction of Anti-PD-1 Therapy (Pembrolizumab or Nivolumab) for the Treatment of Metastatic Colorectal Cancer in Anti-PD-1 Non-responders

M.D. Anderson Cancer Center — PHASE2

TrialACTIVE NOT RECRUITING
Jun 2020Ramucirumab and Paclitaxel or FOLFIRI in Advanced Small Bowel Cancers

SWOG Cancer Research Network — PHASE2

TrialRECRUITING
Mar 2017Pembrolizumab in Treating Patients With Small Bowel Adenocarcinoma That is Metastatic or Locally Advanced and Cannot Be Removed by Surgery

Academic and Community Cancer Research United — PHASE2

TrialACTIVE NOT RECRUITING

Data sourced from FDA regulatory filings and ClinicalTrials.gov. Updated periodically.

Treatments

1 available

Lynparza

olaparib· AstraZeneca Pharmaceuticals LP
for the maintenance treatment of adult patients with deleterious or suspected deleterious germline or somatic BRCA-mutated advanced epithelial ovarian, fallopian tube or primary peritoneal cancer who

for the maintenance treatment of adult patients with deleterious or suspected deleterious germline or somatic BRCA-mutated advanced epithelial ovarian, fallopian tube or primary peritoneal cancer who are in complete or partial response to first-line platinum-based chemotherapy

Clinical Trials

3 recruitingView all trials with filters →
Phase 23 trials
Fecal Microbiota Transplant and Re-introduction of Anti-PD-1 Therapy (Pembrolizumab or Nivolumab) for the Treatment of Metastatic Colorectal Cancer in Anti-PD-1 Non-responders
Phase 2
Active
PI: Michael J Overman (M.D. Anderson Cancer Center) · Sites: Houston, Texas · Age: 1899 yrs
Pembrolizumab in Treating Patients With Small Bowel Adenocarcinoma That is Metastatic or Locally Advanced and Cannot Be Removed by Surgery
Phase 2
Active
PI: Robert McWilliams (Academic and Community Cancer Research United) · Sites: Phoenix, Arizona; Scottsdale, Arizona +6 more · Age: 1899 yrs
Ramucirumab and Paclitaxel or FOLFIRI in Advanced Small Bowel Cancers
Phase 2
Actively Recruiting
PI: Michael J Overman (SWOG Cancer Research Network) · Sites: Anchorage, Alaska; Anchorage, Alaska +534 more · Age: 1899 yrs

Specialists

4 foundView all specialists →
SP
Sandip P Patel
Birmingham, Alabama
Specialist

Rare Disease Specialist

PI on 1 active trial
CD
Camila MV Moniz, Doctor
SAN ANTONIO, TX
Specialist
PI on 1 active trial
KR
Kanwal P Raghav
HOUSTON, TX
Specialist
PI on 2 active trials6 Rare epithelial tumor of small intestine publications

Treatment Centers

8 centers
🏥 NORD

Baylor College of Medicine Rare Disease Center

Baylor College of Medicine

📍 Houston, TX

🏥 NORD

Stanford Medicine Rare Disease Center

Stanford Medicine

📍 Stanford, CA

🔬 UDN

NIH Clinical Center Undiagnosed Diseases Program

National Institutes of Health

📍 Bethesda, MD

🔬 UDN

UCLA UDN Clinical Site

UCLA Health

📍 Los Angeles, CA

🔬 UDN

Baylor College of Medicine UDN Clinical Site

Baylor College of Medicine

📍 Houston, TX

🔬 UDN

Harvard/MGH UDN Clinical Site

Massachusetts General Hospital

📍 Boston, MA

🏥 NORD

Mayo Clinic Center for Individualized Medicine

Mayo Clinic

📍 Rochester, MN

👤 Mayo Clinic Center for Individualized Medicine

🏥 NORD

UCLA Rare Disease Day Program

UCLA Health

📍 Los Angeles, CA

Financial Resources

1 resources
Lynparza(olaparib)AstraZeneca Pharmaceuticals LP

Travel Grants

No travel grants are currently matched to Rare epithelial tumor of small intestine.

Search all travel grants →NORD Financial Assistance ↗

Community

Open Rare epithelial tumor of small intestineForum →

No community posts yet. Be the first to share your experience with Rare epithelial tumor of small intestine.

Start the conversation →

Latest news about Rare epithelial tumor of small intestine

No recent news articles for Rare epithelial tumor of small intestine.

Follow this condition to be notified when news becomes available.

Caregiver Resources

NORD Caregiver Resources

Support, advocacy, and financial assistance for caregivers of rare disease patients.

Mental Health Support

Rare disease caregiving can be isolating. Connect with counseling and peer support.

Family & Caregiver Grants

Financial assistance programs specifically for caregivers of rare disease patients.

Social Security Disability

Learn how rare disease patients may qualify for SSDI/SSI benefits.

Questions for your doctor

Bring these to your next appointment

  • Q1.What exact type of epithelial tumor do I have, and how does that affect my treatment options?,Has the tumor spread, and if so, where?,Should I be tested for a hereditary cancer syndrome like Lynch syndrome or FAP?,What are the goals of treatment — cure, control, or symptom relief?,Are there any clinical trials I might be eligible for?,How will treatment affect my ability to eat and absorb nutrients?,What signs or symptoms should prompt me to seek emergency care?

Common questions about Rare epithelial tumor of small intestine

What is Rare epithelial tumor of small intestine?

Rare epithelial tumors of the small intestine are uncommon cancers or abnormal growths that start in the cells lining the inside of the small intestine — the part of the digestive tract between the stomach and the large intestine. The small intestine is responsible for absorbing nutrients from food, so tumors here can interfere with digestion and overall health. These tumors can be benign (non-cancerous) or malignant (cancerous), and they include types such as adenocarcinoma, carcinoid tumors (also called well-differentiated neuroendocrine tumors), and other rare epithelial growths. Because th

How is Rare epithelial tumor of small intestine inherited?

Rare epithelial tumor of small intestine follows a sporadic inheritance pattern. Genetic counseling can help families understand recurrence risk and testing options.

At what age does Rare epithelial tumor of small intestine typically begin?

Typical onset of Rare epithelial tumor of small intestine is adult. Age of onset can vary across affected individuals.

Are there clinical trials for Rare epithelial tumor of small intestine?

Yes — 3 recruiting clinical trials are currently listed for Rare epithelial tumor of small intestine on UniteRare. See the clinical trials section on this page for phase, sponsor, and site details sourced from ClinicalTrials.gov.

Which specialists treat Rare epithelial tumor of small intestine?

4 specialists and care centers treating Rare epithelial tumor of small intestine are listed on UniteRare, sourced from ClinicalTrials.gov principal investigators, published research, and the NPPES NPI registry.

What treatment and support options exist for Rare epithelial tumor of small intestine?

1 patient support program are currently tracked on UniteRare for Rare epithelial tumor of small intestine. See the treatments and support programs sections for copay assistance, eligibility, and contact details.