Church, James M

Jarrar, Awad M

Kalady, Matthew F

LaGuardia, Lisa

O'Malley, Margaret

English

PubMed

Matthew F Kalady

Awad M Jarrar

Lisa LaGuardia

Margaret O'Malley

James M Church

Crossref

Hyperplastic polyposis syndrome: a call for broader diagnostic criteria

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POSTER PRESENTATION Open Access
Hyperplastic polyposis syndrome: a call for
broader diagnostic criteria
Matthew F Kalady*, Awad M Jarrar, Lisa LaGuardia, Margaret O’Malley, James M Church
From 13th Annual Meeting of the Collaborative Group of the Americas on Inherited Colorectal Cancer
Honolulu, Hawaii, USA. 16-17 October 2009
Background
Hyperplastic Polyposis Syndrome (HPS) is a rare disease
characterized by multiple or large hyperplastic polyps
and carries an approximately 40% lifetime colorectal
cancer risk. Although a genetic basis has not been estab-
lished, HPS is believed to be a heritable syndrome and is
diagnosed by clinical criteria as set forth by the World
Health Organization (WHO). Based on clinical experi-
ence, we hypothesized that WHO criteria may be
narrowly restrictive and misses some patients with an
increased malignancy risk.
Methods
For this study, HPS was defined by meeting at least one
of the following criteria: 1) ≥20 HPs anywhere in the
colon, 2) ≥5 HPs proximal to the sigmoid colon, 3) ≥2
HPs at least 10mm in size, 4) any HPs and a 1st degree
relative with HPS. Colonoscopy and pathology databases
were retrospectively reviewed for patients meeting
criteria. Patient demographics, colonoscopic findings,
and personal and family history of cancer were recorded.
Results
Sixty patients (38 males, 22 females) meeting at least
one of the above criteria were included. Only 19 of
these 60 patients (32%) also satisfied WHO criteria for
HPS. Results are summarized in Table 1. Importantly, of
the additional 41 patients only meeting the broader cri-
teria, 27% had a personal history and 44% had family
history of colorectal cancer. This group also had extra-
colonic malignancies including breast, lung, prostate,
and testicular cancer.
Conclusion
HPS is associated with a personal and familial risk of
colorectal cancer and other malignancies. Applying
broader definitions identifies an additional population of
patients with increased personal and familial cancer risk.
More inclusive criteria should be used until a genetic
basis of disease better defines cancer risk.
Published: 25 May 2010
doi:10.1186/1897-4287-8-S1-P8
Cite this article as: Kalady et al.: Hyperplastic polyposis syndrome: a call
for broader diagnostic criteria. Hereditary Cancer in Clinical Practice 2010
8(Suppl 1):P8.
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* Correspondence: kaladym@ccf.org
Department of Colorectal Surgery, The Sanford R. Weiss Center for
Hereditary Colorectal Neoplasia, Digestive Disease Institute, Cleveland Clinic,
Cleveland, Ohio, USA
Table 1
HPS Criteria N Colorectal Cancer
History
Other Cancer
History
Personal Family Personal Family
WHO 19 5 (26%) 4 (21%) 8 (42%) 7 (37%)
Only New Criteria 41 11 (27%) 18 (44%) 9 (22%) 11 (29%)
All Patients 60 16 (27%) 18 (37%) 12 (23%) 18 (30%)
Kalady et al. Hereditary Cancer in Clinical Practice 2010, 8(Suppl 1):P8
http://www.hccpjournal.com/content/8/S1/P8
© 2010 Kalady et al; licensee BioMed Central Ltd.


Hyperplastic polyposis syndrome: a call for broader diagnostic criteria

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