From the Archives 0 f the AFIP Richard

Fibrous MarkJ.

Kransdorf

Lt Col,

W.

Skeletal

fibrous

marrow

is replaced

a single

bone,

The

Gilkey,

Lt

Col,

dysplasia

archives

related

fibrous

dysplasia

po!yostotic) from

bone

This

thereof,

Armed

MC,

which

present

the

USA

427

where

the

are

norma! be

skeleton

monostotic

to diffuse-

con-

and

74

findings, and

by

bone

localized

radiographically

tomography,

appropriate

USA

of Pathology

and

of radio!ogic

computed

augmented

or affect Institute

cases

MC,

in which may

proved

spectrum

Col,

process

Forces

of histologically

scintigraphy,

imaging,

Moser,Jr,

anomaly

segment

(of

We

.

#{149} RicbardP.

tissue.

of the

50 1 cases

nance

USAF

a small

contain

those

USAR

MC,

fibroosseous

or even

radiologic

MC,

is a developmental

by

(AFIP) are

Co!,

Jr,

Dysplasla1

Fredrick

ly.

P. Moser,

cases

including

magnetic

accompanying

resopathologic

material. U INTRODUCTION Fibrous dysp!asia is a relatively common, benign skeletal disorder, typically encountered in adolescents and young adults. Rather than a true neoplasm, fibrous dysp!asia is a developmental anomaly in which the normal medu!!ary space of the affected bone is replaced by fibroosseous tissue (1) The process may affect a sin.

g!e bone

(monostotic

fibrous

dysplasia)

or many

bones

(polyostotic

fibrous

dys-

p!asia) Yellowish or brownish none!evated patches of cutaneous pigmentation (cafe-au-lait spots), situated predominantly on the trunk of the patient’s body, coexistent findings in one-third to one-half of patients affected with po!yostotic brous dysp!asia (1 ,2). The borders of these dermatologic lesions are typically regular or serrated (they have been likened to the appearance of the coast of Maine) in contrast to the regular (“coast of California”) margins observed in the cafe-au-taft spots of patients afflicted with neurofibromatosis (1). .

Abbreviations: phonate, SE Index

AFIP

From

terms:

Bones,

dysostoses,

1990;

Milton The

Institute

of Pathology,

H-E

hematoxylin.eosin,

MDP

methylene

diphos-

the Departments of Pathology,

S. Hershey reprint opinions

cial or as reflecting sity of the Health C RSNA, 1990

20.85,

40.85

Bones,

osteochondrodysplasias,

#{149}

20.85,

40.85

10:519-537

ofRadiologic 6825

cine, Uniformed Services Walter Reed Army Medical dress

Forces

= spin echo.

RadioGraphics

Institute

Armed

are fiin-

Medical requests

16th

Pathology St, NW,

University Center,

Center,

(MJ.K., R.P.M.) and Washington, DC 20306-6000;

of the Health Sciences, Washington, DC (Mj.K.);

Hershey,

Pa (R.P.M.).

Orthopedic Department

Pathology (F.W.G.), of Radiology and

Bethesda, Md (MJ.K., R.P.M.); and Department of Radiology,

Received

Februray

14, 1990;

Armed Nuclear

Department Penn 5tate

accepted

Forces Mcdi-

of Radiology, University,

February

15. Ad-

to R.P.M.

or assertions the views Sciences.

contained

herein

of the

Departments

are

the of the

private Army,

views

of the

Air Force,

authors or Defense

and

are

or the

not

to be construed

Uniformed

Services

as offiUniver-

519

Because of the coexistence of skin and skeletal deformities in these two eases, fibrous dysplasia was confused neurofibromatosis for many years. In Lichtenstein (3) introduced into the

literature the term bone” and reported

lesions diswith 1938, medical

“fibrous dysplasia of eight cases. In 1942,

Lichtenstein and Jaffe reviewed the existing literature on 75 previously reported cases of fibrous dysp!asia and added 1 5 cases of their

own,

establishing

fibrous

dysp!asia

as a dis-

tinct entity (4 ,5) For these contributions, fibrous dysplasia is sometimes referred to as Lichtenstein-Jaffe disease (1). Multiple endocrine disorders have been described in association with fibrous dysplasia. Of these, the best known is Albnight syn.

drome,

which

consists

of the triad

of po!yos-

totic fibrous dysplasia (typically unilateral), cutaneous cafe-au-taft spots (usually ipsilatera! to the bone lesions) , and endocrine dysfunction (especially precocious puberty in girls) (6) Manifestation of the comp!ete tnad is rare, however (1) Fibrous dysplasia has also been reported in association with other

#{149}