I am very glad to tell you that Jane Gutkovich at EHEFoundation announced today the publication on their site (fightEHE.org) of the EHE Encyclopedia. Thank you, Jane and all the volunteers and contributors!! This is essentially a patient-driven registry and contains the stories of almost 180 patients to date. It is a living document insofar as people will continue to add to and update their stories and new patients will be added over time. Assembling this is a monumental undertaking that required tremendous perseverance and a lot of hard work by Jane and volunteers. It will be very useful for a variety of reasons, not least that EHE patients can review the stories to find similarly affected individuals. Everyone is different, but not so much that we can’t learn from each others’ experiences.

This enterprise grows from the work by Cynthia Pollak who established the HEARD registry, a similarly robust and useful on-line, patient-driven registry. We analyzed that database several years ago and published our findings in the journal Chest (see below). I will cover our findings in detail in another blog post. Suffice to say that we made a few important observations. One is that there EHE appears in a number of reasonably well-defined patterns of clinical disease and, more importantly, the extent of metastasis and the number of organs involved doesn’t seem to have much bearing on clinical outcome. This is important since it distinguishes EHE from almost all other cancers. Since discovery of the underlying genetic alteration and resulting biology of EHE, this finding makes a lot more sense.

In summary, please take advantage of the encyclopedia and contribute your story if you haven’t already. Finally, if you have trouble spelling encyclopedia, just go here.

Clinical Patterns and Outcome in Epithelioid Hemangioendothelioma With or Without Pulmonary Involvement Insights From an Internet Registry in the Study of a Rare Cancer

Kenneth Lau , MD ; Malek Massad , MD , FCCP ; Cynthia Pollak ; Charles Rubin , MD ; Joannie Yeh, MD; Jing Wang, PhD; Guy Edelman, MD; Jenny Yeh, MD; Sunil Prasad, MD; and Guy Weinberg , MD

ABSTRACT. Background: Epithelioid hemangioendothelioma (EHE) is a rare vascular neoplasm of endothelial origin with clinical behavior intermediate between hemangioma and angiosarcoma. The natural history of EHE is highly variable. This study uses an Internet registry to identify clinical patterns with prognostic significance in EHE.

Methods: Cases from the International Hemangioendothioma, Epithelioid Hemangioendothelioma, and Related Vascular Disorders (HEARD) Support Group were evaluated based on demographics,organ involvement, disease progression, presence or absence of pleural effusion, and treatment. Survival among various cohorts was compared using log-rank analysis of Kaplan-Meier plots.

Results: Two hundred sixty-four patients were identifi ed from April 2004 to November 2009. Fifty-eight cases were excluded because of inadequate information or wrong diagnosis. EHE was more common in female patients (61%). Male gender and age _ 55 years were associated with decreased survival. The most commonly affected organs were liver, lung, and bone. No specific organ or combination of organ involvement differentially affected survival, and survival was no different between patients with multiple vs single organ involvement. However, pattern B, defined as lesions without distinct borders (eg, pulmonary infiltrates, pleural effusion, ascites), hemoptysis, or involvement of more than two bones adversely affected survival in all cohorts.

Conclusion: A novel staging system with prognostic value for EHE is proposed. Pleural effusion or other signs of uncontained tumor growth, hemoptysis, and osseous involvement of more than two bones implied worse survival than did localized and discrete tumors, regardless of number of organs involved. A lay registry can provide useful insights into the clinical behavior of a rare cancer. CHEST 2011; 140(5):1312–1318