Pathology of the Pancreas

347 Pages·1995·15.538 MB·English

by  Alan H. Cruickshank, Emyr W. Benbow (auth.)

A.H. Cruickshank and E.W. Benbow
Pathology
of the Pancreas
Second Edition
With 276 figures
Springer
London Berlin Heidelberg New York
Paris Tokyo Hong Kong
Barcelona Budapest
Alan H. Cruickshank
The Old House
28 North Road
Grassendale Park
Liverpool L19 OLR
Emyr W. Benbow
Department of Pathology
The Medical School, University of Manchester
Oxford Road
Manchester M13 9PT
Front cover illustration: see Fig. 6.6 (page 96)
ISBN-13: 978-1-4471-3007-9 2nd edition. Springer-Verlag Berlin Heidelberg New York
British Library Cataloguing in Publication Data
Cruickshank, Alan H.
Pathology of the Pancreas. - 2 Rev. ed
1. Title II. Benbow, Emyr W.
616.3707
ISBN-13: 978-1-4471-3007-9 e-ISBN-13: 978-1-4471-3005-5
DOT: 10.1007/978-1-4471-3005-5
Library of Congress Cataloging-in-Publication Data
Cruickshank, Alan H.
Pathology of the pancreas 1 A.H. Cruickshank and E. W. Benbow. - 2nd ed.
p. cm.
Includes bibliographical references and index.
ISBN 3-540-19923-3 (alk. paper)
1. Pancreas - Diseases. 2. Pancreas - Histopathology, 1. Benbow, E.W. (Emyr W.), 1952- II. Title.
[DNLM: 1. Pancreatic Diseases. 2. Pancreas - pathology. WI 800
C955p 1955]
RC857.4.C78 1995
616.3'7 - dc20
DNLMIDLC
for Library of Congress 95-33
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© Springer-Verlag London Limited 1995
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To our wives
Preface to the First Edition
The recent advances in the techniques for imaging the pancreas without surgical interven
tion have reduced the inaccessibility of the pancreas. However, although certain lesions of
the pancreas can now be recognised and localised without an operation, the pathology of
the pancreas remains to be more thoroughly investigated. Moreover, the almost unrelated
exocrine and endocrine functions of the pancreas have led to the management of different
pancreatic diseases by different groups of specialists, while the effects of primarily non
pancreatic diseases upon the pancreas have tended to escape recognition. Even in the
autopsy room the pancreas is often inadequately examined, and autolysis may make
microscopic examination unrewarding. This book is an attempt by a general histopatholo
gist to make available some of his experience of the various aspects of pancreatic disease
that he has encountered during his working career.
My interest in the pathology of the pancreas was aroused while working with Prof.
Arnold Rice Rich of the Johns Hopkins Hospital, Baltimore. Rich himself, in his earlier
work, had continued the tradition, begun in the same department of pathology by Opie, of
carrying out morbid anatomical and experimental studies on pancreatic disease. Rich later
became more involved in work on tuberculosis and on the collagen diseases but his inter
est in disease of the pancreas persisted and the work he allotted to me included an experi
mental study of chemically induced diabetes mellitus.
After my return to England I spent the next 30 years as a member of the Department of
Pathology at the University ofLiverpool. There much of my time was occupied with teach
ing, with routine surgical pathology and with many autopsies upon patients that had died
in the Liverpool Royal Infirmary and in Newsham General Hospital. I also had to perform
a share of the autopsies carried out for the Liverpool City Coroner. It was during these
years that most of the material for this book was collected: retirement provided an oppor
tunity to review and select from the accumulated material. It was Mr. Michael Jackson of
the Springer-Verlag organisation who suggested that the book should be written, and it
was he also who then provided the encouragement and stimulation without which the
book would never have been finished.
I am particularly grateful to Prof. Donald Heath of the Department of Pathology at the
University of Liverpool for allowing me, although no longer a member of his staff, to use
the facilities of his department, and for giving me access to the records and the material
stored in the department. The assistance of the department's photographic staff has been
invaluable, and I am indebted to Mr. Alan J. Williams for all but a few of the photographs
and photomicrographs that I have used. I am grateful also to Mr. John Massey who
willingly undertook the work of recutting and staining sections from old blocks of tissue.
viii Preface to the First Edition
Dr. John Bouton of the Department of Pathology at Alder Hey Children's Hospital,
Liverpool, allowed me to use his records and Miss Catherine D. M. McDougall of his
department provided sections and electron micrographs. Dr. D. R. Clark, now in the
Department of Pathology, Launceston General Hospital, Tasmania, carried out many of
the post-mortem pancreatograms and Dr. Mavis McConnell of the Liverpool Regional
Cancer Registry provided records and follow-up information about cases of pancreatic
cancer. Many colleagues provided specimens or case records and such material has been
acknowledged in the text or in the figure legends. Dr. Anne Clark of the Diabetes Research
Laboratories of the Nuffield Department of Clinical Medicine, the University of Oxford,
generously provided material and advice for the chapter on the pancreatic lesions associ
ated with diabetes and I am most grateful to her. I am obliged to Mrs. G. C. Pattinson,
Mrs. Alison Rimmer and to Miss Collette Youd for having typed the manuscript and also
to the staff of the library of the Liverpool Medical Institution for much assistance with the
literature.
Liverpool, February 1986 A. H. Cruickshank
Preface to the Second Edition
As the senior author of the second edition of Pathology of the Pancreas I must begin by
stating that I had expected the edition that appeared in 1986 to be the only edition and to
be my swan song. Thus it was with some dismay that I received an invitation from the late
Michael Jackson to begin work on a second edition, and it was only when Michael Jackson
suggested that I should find a co-author that my reluctance to undertake the job was
overcome.
It was at a meeting of the North West branch of the Association of Clinical Pathologists
that Emyr Benbow and I found we had a common interest in generalised cryptococcal
infections, a case of which he had presented at the meeting. Better acquaintance showed
that there were other topics in pathology that interested both of us and that pathology of
the pancreas was such a topic. Until then, Emyr Benbow's main interest had been in the
field of pulmonary and cardiovascular pathology but, as a lecturer in pathology in the
University of Manchester, he had been influenced by Dr. Joan Braganza's enthusiasm for
the investigation of pancreatic disease, and had been particularly intrigued when Dr.
Braganza's ideas on pathogenesis had helped explain an autopsy case where acute pancre
atitis had occurred simultaneously in both pancreases in a patient generously endowed
with two! We met Michael Jackson and, with his approval, we agreed to start work.
Initial progress was slow for each revised or re-written chapter was read by both of us
and our written comments on what we had read were nearly as long as the chapter that
they concerned. This method of working, however, allowed our meetings, when they could
be arranged, to be more social and convivial rather than scholastic. Time went by but
eventually we decided that we must finish and after some terminal haste our typescript
was submitted. In the meantime Michael Jackson had died, and the task of stimulating and
encouraging us had been taken over by Dr. Gerald Graham, but he retired about the time
we finished writing and Dr. Andrew Colborne was just taking over as Medical Editor when
our typescript was delivered.
The preliminary editing of our efforts was entrusted to Dr. Margaret Carver and we are
grateful to her for the correction of our many mistakes and omissions. We found her rig
orous requirements for detail in our references to be more exacting than anything either
of us had met before but we appreciate the hard work she did in clarifying and tightening
our sentences, and in making our choice of words more genteel.
As in the case of the first edition, we are much obliged to Mr. Alan Williams for his
photography, and the senior author wishes to thank Mrs. B. N. Ridley and Miss Gaynor
Southern for typing his manuscript.
Liverpool, March 1995 A. H. Cruickshank
Contents
1 The Normal Pancreas 1
The Duct System of the Pancreas 3
Pancreas Divisum ....... . 9
Interpretation of Pancreatograms 9
The Effects of Age in Relation to Pancreatography 10
The Weight of the Normal Pancreas and Pancreatic Fat. 11
References . . . . . . . . . . . . . . . . . . . . . . . . . . 12
2 Normal Microscopic Structure. 13
The Islets of Langerhans . . . . . . . . . . . . 15
The Development of the Pancreas . . . . . . . 18
Epithelial Metaplasia and Dysplasia in the Pancreas 21
Pancreatic Injury, Repair and Regeneration 24
References . . . . . . . . . . . . . . . . . . . . . . . . 26
3 Congenital Pancreatic Abnormalities. . . . . . . . . . . . . . . . .. 29
Congenital Absence of the Pancreas ...... 29
Congenital Absence of the Islets of Langerhans 29
Congenital Absence of Insulin-Secreting Cells. 30
Congenital Short Pancreas . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .. 30
Agenesis of the Dorsal Pancreatic Bud 30
Pancreas Divisum .................................... 30
Annular Pancreas .................................... 31
Pancreatic Fibrosis, Bilateral Renal Dysplasia, Intrahepatic Biliary Dysgenesis and
Situs Inversus Totalis . . 32
Aberrant Pancreatic Ducts . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 33
xii Contents
Heterotopia of Pancreatic Tissue 33
Pancreatic Tissue in Teratomas . 39
Accessory Spleens Within the Pancreas 39
Congenital Cysts of the Pancreas .., . 40
Pancreatic Cysts Associated with Renal Cystic Disease 43
Von Hippel-Lindau Disease. . . . 43
Dermoid Cysts ........... 44
Pancreatic Lymphoepithelial Cysts 44
Pancreatic Atrophy ........ 45
Congenital Pancreatic Hypoplasia with Haematological Abnormalities
(Shwachman-Diamond Syndrome) ................... 48
Juvenile Nephronophthisis with Calcification of Basal Ganglia and Pancreatic
Insufficiency. . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 50
Refractory Sideroblastic Anaemia with Vacuolisation of Marrow Precursors and
Exocrine Pancreatic Dysfunction . 50
Beckwith-Wiedman Syndrome 50
References ........... 50
4 Effects upon the Pancreas of Disease in Various Organs and
Systems ....................................... 55
Cystic Fibrosis (Fibrocystic Disease of the Pancreas, Pancreatic Fibrosis,
Mucoviscidosis) ...................... . 56
Haemosiderosis of the Pancreas .............. . 64
Other Pancreatic Lesions Associated with Hepatic Disease 67
Chronic Venous Congestion . . . . . . . . . . . . . . . . . . 69
Pancreatic Lesions Associated with Chronic Ulcerative Colitis 69
Pancreatic Lesions Associated with Uraemia and Dehydration 69
The Pancreas in Acute Graft Versus Host Disease . . . . . . . . 71
Pancreatic Lesions in Toxaemia of Pregnancy ........ . 72
Pancreatic Lesions Associated with Death from Lightning Stroke 72
Pancreatic Lesions in Fatal Cases of Burning .., 72
Amyloidosis .................... . 72
Wilson's Disease (Hepatolenticular Degeneration) 74
Systemic Lupus Erythematosus 74
Progressive Systemic Sclerosis 74
Alpha! Antitrypsin Deficiency. 74
Nephropathic Cystinosis. 74
References . . . . . . . . . . . . 75
5 The Effects of Arterial Disease upon the Pancreas 77
Anatomy of the Arteries of the Pancreas . . . . .. . 77
Aneurysms of the Pancreatic Arteries .... ... . 78
Effects of Arteriosclerosis of the Pancreatic Arteries 81
Arterial Lesions in Relation to Acute Haemorrhagic Pancreatitis 81
Pancreatic Infarction . . . . . . . . . . 82
Injury to the Arteries of the Pancreas . 86
References .............. . 88
Contents xiii
6 The Pancreas in Diabetes Mellitus and Haemochromatosis 91
Insulin-Dependent Diabetes Mellitus (Type I Diabetes). . . . . . . . . . . . 92
Insulin-Independent Diabetes Mellitus (Maturity-Onset, Type II Diabetes) 103
Tropical Diabetes or Nutrition-Related Diabetes Mellitus .......... 108
The Pancreas in Haemochromatosis . . . . . . . . . . . . . . . . . . . . . . 109
The Pancreas in Diabetes Mellitus Associated with Other Endocrine Diseases 111
Insulin-Resistant Diabetes Mellitus. 112
References 112
7 Pancreatic Transplantation 117
Rejection ....... . 117
Recurrence of Diabetes . 118
Transplantation of Islets 118
References . . . . . . . . 119
8 Cystic Pancreatic Neoplasms. 121
Incidence ................... 121
Serous Cystadenoma, Microcystic Adenoma (Glycogen-Rich Cystadenoma) . 122
Mucinous Cystic Neoplasm with Latent Malignancy, Mucinous Cystadenoma,
Large Locular Cystadenoma .......................... 125
Cystadenocarcinoma . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 129
"Ductectatic" Cystadenoma and Cystadenocarcinoma (Intraductal Papillary
Neoplasms of the Pancreas) . . . . . . . . . . . . . . . . . . . . . . . . . . . 134
Solid and Papillary, Papillary and Cystic Epithelial Neoplasms of the Pancreas
(Frantz's Tumour) . . . . . . . 135
Cavernous Lymphangioma. . . 139
References . . . . . . . . . . . . . 140
9 Solid Carcinomas of the Exocrine Pancreas 143
Increasing Incidence 143
Aetiology ...... 144
Causes of Symptoms 147
Abnormal Immunity and Pancreatic Carcinoma 160
Neurofibromatosis and Pancreatic Carcinoma . 160
Lipomatous Pseudohypertrophy and Pancreatic Carcinoma 160
Skin Changes . . . . . . . . . . . . . . . . . . . . . . . . . . 160
Ectopic Hormone Production . . . . . . . . . . . . . . . . . 161
Hy percakaemia . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .. 161
Macroscopic Features ............................. 161
Microscopic Features ............................. 165
Carcinoma of the Ampulla of Vater, Common Bile Duct and Duodenum . 175
Microscopic Confirmation of a Diagnosis of Carcinoma of the Pancreas 176
Use of Tumour Markers in Diagnosis. . . . . . . . . . . . . . . . . . . . . 179