Prognosis and survival, honestly

What the published numbers say

We will not soften this: the survival figures in the literature are hard to read. We also think they need to be read carefully, because they come from very different groups of patients. Here they are, side by side.

Two things stand out. First, the range is enormous — from a few months to several years — and the difference is largely explained by who was counted. Second, the more recent and the more specialised the series, the better the numbers.²⁴

How to read a survival statistic

A median survival of 31 months means that half of the patients in that group lived longer than 31 months — some much longer. It says nothing about where you fall. A 5-year survival of 49% means about half of that group were alive five years later; it does not mean the other half died at year five, nor that you have a coin-flip.

Every one of these numbers describes a group that is not you: different ages, different tumour sites and sizes, different stages, different surgeons, different years. The honest answer to "what is my prognosis?" is a conversation with a sarcoma oncologist who knows your imaging, your surgery report and your molecular profile — not a number from a website, including this one.

Why the published numbers are probably too pessimistic for some people

Several biases push the literature towards the grim end:

• Misdiagnosis bias. Seven in ten patients are first treated for a blood clot.² Older series are full of people diagnosed only when the disease was advanced — or at autopsy. Those cases dominate the historical averages.
• Publication bias. Unusual, dramatic or fatal cases get written up; uneventful long survivors rarely do. A person who had surgery eight years ago and is fine does not generate a paper.
• Era effect. FDG-PET/CT, better CT, and specialised cardiac-tumour surgery are recent. Series from the last decade report roughly double the survival of the older summaries.¹²
• No registry. Nobody is systematically counting the people who did well. That is not a rhetorical point — it is the specific gap this initiative was founded to close.

None of this makes intimal sarcoma a mild disease. It means the true distribution of outcomes is wider and better than the averages suggest, and that where you land depends a great deal on the decisions made in the first weeks.

What changes the odds

Across the series, the same factors keep appearing:²⁴⁶

• Complete surgical removal (R0). The strongest single factor in every series. In the specialised surgical series, only 45% of resections achieved clear margins even in expert hands — which is why the surgeon's experience with this exact operation matters.⁴
• Stage at diagnosis. Disease confined to the vessel does better than disease that has already spread. Shortening the time from "clot that won't go away" to "sarcoma" is the one thing that moves this.
• Treatment at all, and multimodal treatment. Median survival was 17 months with treatment versus about 3 months without in one cohort;³ chemotherapy appears to add benefit in advanced stages;² retrospective data suggest that combining surgery with anthracycline-based chemotherapy and/or radiotherapy helps selected patients.⁶
• Being at a sarcoma center. Every recent review makes management in a specialised multidisciplinary center its central recommendation.⁶
• Possibly, the molecular profile. Whether specific amplification patterns (MDM2, CDK4, PDGFRA and others) predict behaviour or response is unknown — nobody has enough linked cases to test it. This is the question our case base is built to answer.

Recurrence: what to expect after surgery

Intimal sarcoma frequently returns — either locally, where it was removed, or as metastases in the lungs or elsewhere. In the World Sarcoma Network series, patients with localised disease who received anthracycline chemotherapy had a median of 14.6 months before recurrence.⁵ In a pulmonary endarterectomy series, recurrence or metastasis was common within the follow-up period.⁸

What this means practically: structured follow-up imaging (typically CT, sometimes PET/CT, every few months at first) is part of treatment, not an afterthought — and recurrence is not the end of the road. Re-operation, radiotherapy, further chemotherapy and, increasingly, molecularly guided drugs or trials are all used. Ask your team for the follow-up schedule in writing.

Long-term survivors exist — and they are the most valuable data in this disease

They are real: 16% of one surgical series were alive at 10 years;⁴ a patient with intimal sarcoma of a coronary artery was disease-free seven years after diagnosis following surgery, chemotherapy and radiotherapy for a recurrence;⁷ the pooled analysis contains survivors well beyond five years.² What is missing is any systematic record of what these people had in common — their surgery, their treatments, their molecular profiles.

If you, or someone in your family, is three or more years past an intimal sarcoma diagnosis, you hold information that exists nowhere else. We want to write it down, in a consistent format, and put it next to every other case. Tell us your course → No names are needed; what was done, where, and in what order is enough.

We started with ourselves: Case IS-001 is one completely resected pulmonary artery intimal sarcoma, published with its full molecular profile and clinical course.⁹

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