GIST Specialist for Manhattan Beach, CA
Fewer than one in ten gastrointestinal stromal tumors occur under the age of forty, and those that do are frequently a different disease wearing the same name. Joshua Ellenhorn, MD, FACS treats young onset GIST on its own terms.
- Recognition of SDH-deficient and syndromic GIST
- Clinical Professor of Surgery, Cedars-Sinai Medical Center
- Stomach preserving surgery for multifocal disease
- Referral for genetic counselling where indicated
Why Young Onset GIST Is Treated Differently
The typical GIST patient is around sixty, has a single tumor driven by a KIT mutation, responds well to imatinib, and is managed over a few years. Apply that template to a thirty five year old and you will frequently get it wrong.
Young onset GIST is disproportionately SDH-deficient, a subtype defined by loss of the SDHB protein rather than by a KIT mutation. These tumors are almost always gastric, often multifocal from the outset, and can involve lymph nodes, which conventional GIST hardly ever does. They also respond poorly to imatinib, so a patient started on it by default may spend months on a drug that was never going to work.
The behaviour is different too. SDH-deficient disease tends to be indolent but persistent, which means the horizon is decades rather than years. That changes the surgical calculus fundamentally: preserving stomach at each intervention matters far more when a patient may face several over a lifetime, and a total gastrectomy performed early forecloses options that would have been useful later.
None of this is exotic. It requires the SDHB stain to be ordered and the result to be acted on.
The Syndromes Worth Knowing About
Most GIST mutations arise by chance in the tumor and are not passed on. The exceptions cluster in younger patients, and they have consequences for relatives as well as for you.
| Syndrome | What it involves | Inherited? |
|---|---|---|
| Carney-Stratakis dyad | GIST together with paraganglioma, caused by germline mutations in SDHB, SDHC or SDHD | Yes. Family testing is appropriate |
| Carney triad | GIST, paraganglioma and pulmonary chondroma, with a strong female predominance | No. Arises sporadically despite the syndrome pattern |
| Neurofibromatosis type 1 | Multiple small bowel GIST, typically wild type and imatinib resistant | Yes, NF1 itself is inherited |
| Familial GIST | Germline KIT or PDGFRA mutation producing multiple GIST across a family, sometimes with skin pigmentation changes | Yes, and rare |
| Sporadic SDH-deficient GIST | Gastric, often multifocal, SDHB negative, no other syndromic features | Frequently not, but germline testing is still warranted |
Genetic counselling is worth discussing if you are young, if your tumor is wild type or multifocal, or if anyone in your family has had a GIST or a paraganglioma. That conversation is separate from your cancer treatment and does not delay it.
What GIST Is, and What SDH Loss Changes
All GIST arise from the interstitial cells of Cajal, the pacemaker cells inside the wall of the digestive tract that coordinate the contractions moving food along. They are sarcomas, they grow outward from the wall, and they can reach a reasonable size before producing symptoms.
In conventional GIST a mutation in KIT or PDGFRA jams a growth signal permanently on, and targeted tablets were designed to switch it off. That is why imatinib works so well in the usual case.
SDH-deficient tumors take a different route to the same destination. The succinate dehydrogenase complex is part of cellular energy metabolism, and its loss drives growth through a mechanism imatinib does not touch. Hence the poor drug response, and hence the greater weight placed on surgery, and on doing surgery conservatively enough that it can be repeated.
Priorities Over a Longer Horizon
When a patient is thirty rather than sixty, decisions are judged against decades rather than years.
Establish the Subtype
SDHB staining and mutation testing before treatment, not after. A wild type or SDH-deficient result changes both the drug plan and how aggressive the operation should be, and getting it late means months lost.
Preserve Stomach
Complete removal of what needs removing, with as much stomach left as possible. Multifocal disease is not a reason for total gastrectomy in an indolent tumor, because the same patient may need surgery again in ten years.
Sustained Surveillance
Young patients need follow up measured in decades, which makes continuity the practical challenge. A schedule with defined intervals, held by someone who will still be tracking it, matters as much as the operation.
The Operations, on Record
Recordings of real GIST resections narrated by the surgeon who performed them. Worth watching for how little stomach is actually removed in a well planned gastric case.
Laparoscopic Resection of a Gastric GIST
A gastric GIST removed through small incisions with the capsule intact and the stomach largely preserved.
Gastric GIST Resection: A Simplified Approach
A refined approach that shortens operating time and recovery.
Robotic Resection of a Duodenal GIST
Duodenal GIST removed robotically with the pancreas and bile duct preserved.
Was SDHB Tested?
If you are under forty with a gastric GIST and nobody has mentioned SDHB staining or wild type status, that is the first question to raise. It determines whether the drug you may be about to start has any chance of working.
Certifications and Appointments
Two American Board of Surgery certifications, a Cedars-Sinai teaching appointment, and membership of the societies that publish the subtype guidance described above.
- American Board of Surgery, certified in General Surgery
- American Board of Surgery, certified in Colorectal Surgery
- California State Medical License
- Florida State Medical License
Joshua Ellenhorn, MD, FACS
Dr. Ellenhorn is a surgical oncologist and Clinical Professor of Surgery at Cedars-Sinai Medical Center, and a collaborative member of the Samuel Oschin Comprehensive Cancer Institute.
Young onset GIST is where a narrowly focused practice earns its keep in an unglamorous way. These patients need the rare subtypes recognised at diagnosis, they need surgery restrained enough to be repeatable, and they need someone still tracking them in fifteen years. None of that is technically difficult. All of it depends on the same person seeing enough of these tumors to know that a thirty year old with a multifocal gastric GIST is not simply an early version of the usual patient.
He is certified by the American Board of Surgery in General Surgery and in Colorectal Surgery, a Fellow of the American College of Surgeons, and a member of the Society of Surgical Oncology and the American Society of Clinical Oncology. He practises with the Surgery Group of Los Angeles.
What Patients Say
Reviews left by patients treated by Dr. Ellenhorn at the Surgery Group of Los Angeles.
★★★★★The staff at the Surgery Group of Los Angeles are wonderful. They are kind, very attentive, the office is clean and I did not have to wait long at all. I cannot say enough great things about Dr. Ellenhorn. He is an excellent surgeon who is highly skilled and very knowledgeable. I cannot thank him enough for all he has done for my family and I.
Erika Frank
★★★★★Very kind stuff, great service. Dr Joshua Ellenhorn is a very calm doctor who takes time with his patient.
Anita Lukacevic
From the South Bay
The 405 north to the 10 east then north on La Cienega is the usual route from Manhattan Beach, and Sepulveda north to La Cienega is the alternative when the freeway is heavy. Lincoln up the coast is slower in distance terms but often more predictable at peak. Expect thirty to forty minutes depending on the hour.
The office is in the medical plaza attached to Cedars-Sinai Medical Center, so consultations, pathology review, and surgery are in one building. For younger patients facing follow up over decades rather than years, that consolidation reduces the number of places a record can go missing.
Patients travelling from outside the region, including Arizona and Nevada, can have imaging and pathology reviewed remotely.
8635 W 3rd St, Suite 880W
Los Angeles, CA 90048
Monday to Saturday, 11:00am to 8:00pm
Closed Sunday
Frequently Asked Questions
I am in my thirties. Is GIST not an older person disease?
What is SDH-deficient GIST?
Should I have genetic testing?
What syndromes are associated with GIST?
Does multifocal disease mean it has spread?
Will I need my whole stomach removed if there are several tumors?
How far is your office from Manhattan Beach?
Can I still have children after this?
Under Forty With a GIST? Ask About the Subtype.
Young onset gastrointestinal stromal tumors are frequently SDH-deficient or wild type, which changes the drug, the operation, and whether your family should be tested. It starts with a stain that has to be ordered.
