Book Review

Familial and Hereditary Tumors

N Engl J Med 1995; 333:808September 21, 1995

Article

Familial and Hereditary Tumors
(Recent Results in Cancer Research. [136].) By M. Ponz de Leon. 347 pp., illustrated. New York, Springer-Verlag, 1995. DM198. ISBN: 0-387-57663-9

Every week it seems that a new cancer gene is discovered, and every month, a suspected carcinogen. Who will summarize this avalanche of information and integrate it for wide use?

With Familial and Hereditary Tumors, Dr. Ponz de Leon, professor of internal medicine at the University of Modena in Italy, has made such an attempt. At the outset, he hypothesizes “that almost invariably cancer develops because of a close interaction between genes and exogenous agents or, in other words, that an inherited condition renders individuals highly susceptible to tumors, but that these usually occur owing to the effect of exogenous factors acting on a predisposed substrate.” Despite the title of the book, the chief environmental determinants of cancer in humans are not ignored.

The clear organization of the book and of each chapter is a welcome reminder of the great advantages of a single-authored work. The 22 chapters are mostly divided on the basis of organ systems. With the exception of two long chapters on colon cancer and polyposis syndromes, the average chapter is just 14 pages long, with 66 references. Each chapter has an epigraph (not always referenced); a splendid one-page summary; a page each on epidemiology, clinical features, pathology, and management; and longer sections on familial aggregation, biomolecular markers, oncogenes and tumor-suppressor genes, and cytogenetics. Obviously, much information is compressed. The preface is dated September 1994, but I found no references published in 1994. Many reports published in 1989 and even one from 1982 were referred to as “recent.” Not mentioned are the cloning of the genes for von Hippel–Lindau disease and neurofibromatosis type 2 and the mapping of genes for hereditary nonpolyposis colorectal cancer, but the presentation of the main facts does not suffer. The author's attempt to strike an “ecogenetic” balance sometimes works; when it does not, it is usually because the data are not yet at hand.

Especially good are the early chapters on biomarkers, oncogenes, and tumor-suppressor genes. A large number of traits governed by single genes are usefully mentioned, without reducing the book to a tedious inventory of such traits. The inclusion of some previously unpublished small pedigrees initially appears to be unnecessary, but a closer look reveals that they make a clear point, as real-world examples and not blatant outliers.

The author has not been well served by his technical and English-language editors. The generally smooth writing is marred by excessive typographic errors and some malapropisms that should have been caught: “monolaterality” for unilaterality, “autoptic” for autopsy, “plasmatic” for plasma, “aspecific” for nonspecific, and — most egregious in a book on heredity — “familiarity” for familiality. The illustrations and tables are infrequent; a few puerile drawings are pointless.

Who might use this synthesis of information? Ponz de Leon's pioneering summary on the etiology of cancer provides the clinician with a reasonably current checklist to use in addressing questions posed by patients and their families. His vigorous pursuit of an ecogenetic hypothesis of cancer origins is a timely reminder for cancer researchers that there are more routes to human cancer than the often-repeated linear model of accumulated genetic changes.

John J. Mulvihill, M.D.
University of Pittsburgh, Pittsburgh, PA 15261