Protocol
applies to all carcinomas of the small intestine, including those with focal
endocrine differentiation. Excludes carcinoid tumors,
lymphomas,
and stromal tumors (sarcomas).
Procedures
• Cytology
This protocol is intended to assist pathologists
in providing clinically useful and relevant information as a result of the
examination of surgical specimens. Use of this protocol is intended to be
entirely voluntary. If equally valid protocols or similar documents are
applicable, the pathologist is, of course, free to follow those authorities.
Indeed, the ultimate judgment regarding the propriety of any specific procedure
must be made by the physician in light of the individual circumstances
presented by a specific patient or specimen.
It should be understood that adherence to this
protocol will not guarantee a successful result. Nevertheless, pathologists are
urged to familiarize themselves with the document. Where a physician chooses to
deviate from the protocol based on the circumstances of a particular patient or
specimen, the physician is advised to make a contemporaneous written notation
of the reason for the procedure followed.
The College recognizes that this document may be
used by hospitals, attorneys, managed care organizations, insurance carriers,
and other payers. However, the document was developed solely as a tool to
assist pathologists in the diagnostic process by providing information that
reflects the state of relevant medical knowledge at the time the protocol was
first published. It was not developed for credentialing, litigation, or
reimbursement purposes. The College cautions that any uses of the protocol for
these purposes involve considerations that are beyond the scope of this
document.
A. CLINICAL INFORMATION
1. Patient
identification
a. Name
b. Identification
number
c. Age (birth date)
d. Gender
2. Responsible
physician(s)
3. Date of procedure
4. Other clinical
information
a. Relevant history (Note A)
b. Relevant findings
(e.g. endoscopic/ imaging studies)
c. Clinical diagnosis
d. Procedure (e.g.
FNA, scraping, brushing)
e. Operative findings
f. Anatomic sites
(e.g. duodenum, jejunum, ileum: endoscopic
distance)
B. MACROSCOPIC EXAMINATION
1. Specimen
a. Description
b. Type (cell
block/slides/cytospins/fluids/other)
c. Unfixed/fixed
(specify fixative)
d. Number of slides
received
e. Quantity and
appearance of fluid specimen
f. Other (e.g.
tissue received for cytologic preparation)
g. Results of
intraprocedural consultation (Note B)
2. Material submitted
for microscopic evaluation (e.g. smear, cytocentrifuge, touch or filter preparation;
cell block)
3. Special
studies (specify) (Note C)
C. MICROSCOPIC EVALUATION
1. Adequacy of
specimen (if unsatisfactory for evaluation, specify reason)
2. Tumor, if present
a. Histologic type,
if possible (Note D)
b. Histologic grade, if possible (Note E)
c. Other descriptive
information (e.g. hemorrhage, necrosis)
3. Additional
pathologic findings, if present
4. Special studies (Note C)
5. Comments
a. Correlation with
intraprocedural consultation, as appropriate
b. Correlation with
other specimens, as appropriate
c. Correlation with
clinical information, as appropriate
A. CLINICAL INFORMATION
1. Patient
identification
a. Name
b. Identification
number
c. Age (birth date)
d. Gender
2. Responsible
physician(s)
3. Date of procedure
4. Other clinical
information
a. Relevant history (Note A)
b. Relevant findings
(e.g. endoscopic/imaging studies)
c. Clinical diagnosis
d. Procedure (e.g.
endoscopic biopsy)
e. Operative findings
f. Anatomic sites (e.g. duodenum, jejunum, ileum:
endoscopic distance)
B. MACROSCOPIC EXAMINATION
1. Specimen(s)
a. Tissues submitted
b. Unfixed/fixed
(specify fixative)
c. Number of pieces
d. Dimensions
e. Descriptive
features (e.g. color/consistency/configuration)
f. Layers of bowel
(if discernible)
g. Results of
intraoperative consultation
2. Tissues submitted
for microscopic evaluation
a. All biopsy
material
b. Frozen section
tissue fragment(s)
3. Special studies
(specify) (e.g. histochemistry, immunohistochemistry [designate each antibody],
morphometry, DNA analysis [specify type], electron microscopy, cytogenetic
analysis) (Note C)
C. MICROSCOPIC EVALUATION
1. Tumor
a. Histologic type (Note D)
b. Histologic grade (Note E)
c. Extent of invasion
d. Blood/lymphatic
vessel invasion
2. Additional
pathologic findings, if present
a. Benign neoplasms
b. Dysplasia
c. Crohn’s disease
d. Celiac disease
e. Other(s)
2. Results/status of
special studies (specify)
3. Comments
a. Correlation with
intraoperative consultation, as appropriate
b. Correlation with
other specimens, as appropriate
c. Correlation with
clinical information, as appropriate
A. CLINICAL INFORMATION
1. Patient
identification
a. Name
b. Identification
number
c. Age (birth date)
d. Gender
2. Responsible
physician(s)
3. Date of procedure
4. Other clinical
information
a. Relevant history (Note A)
b. Relevant findings
(e.g. endoscopic/imaging studies)
c. Clinical diagnosis
d. Procedure (e.g.
polypectomy)
e. Operative findings
f. Anatomic sites (e.g. duodenum, jejunum, ileum:
endoscopic distance)
B. MACROSCOPIC EXAMINATION
1. Specimen
a. Tissue(s)
submitted
b. Unfixed/fixed
(specify fixative)
c. Number of pieces
d. Dimensions
e. Descriptive
features (e.g. color, consistency, configuration)
f. Orientation (if
designated by surgeon)
g. Results of intraoperative consultation
2. Tissue submitted
for microscopic evaluation
a. Coronal section of
polyp(s) through resection margin or stalk (if applicable)
b. All other tissue
from polypectomy specimen(s)
c. Frozen section
tissue fragment(s)
3. Special studies
(specify, e.g., histochemistry, immunohistochemistry, morphometry, DNA analysis
[specify type], cytogenetic analysis) (Note C)
C. MICROSCOPIC EVALUATION
1. Tumor
a. Histologic type (Note D)
b. Histologic grade (Note E)
c. Depth of invasion,
as appropriate
d. Blood/lymphatic
vessel invasion
e. Interface with
adjacent normal mucosa
f. Distance
(millimeters) between tumor and closest margin(s)
2. Additional
pathologic findings, if present
a. Benign neoplasms
b. Dysplasia
c. Crohn’s disease
d. Celiac spruce
e. Other(s)
3. Other
tissue(s)/organ(s)
4. Results/status of
special studies (specify) (Note C)
5. Comments
a. Correlation with
intraoperative consultation, as appropriate
b. Correlation with
other specimens, as appropriate
c. Correlation with
clinical information, as appropriate
IV. Segmental resection back Top Main Page
A. CLINICAL INFORMATION
1. Patient
identification
a. Name
b. Identification
number
c. Age (birth date)
d. Gender
2. Responsible
physician(s)
3. Date of procedure
4. Other clinical
information
a. Relevant history (Note A)
b. Relevant findings
(e.g. endoscopic/imaging studies)
c. Clinical diagnosis
d. Procedure (e.g.
distal ileal resection)
e. Operative findings
f. Anatomic sites
(e.g. duodenum, jejunum, ileum)
B. MACROSCOPIC EXAMINATION
1. Specimen
a. Organ(s)/tissue(s)
submitted
b. Previously opened
c. Unfixed/fixed
(specify fixative)
d. Number of pieces
e. Dimensions (length/circumference)
f. Descriptive
characteristics (e.g. thickness of bowel wall in abnormal areas)
g. Orientation (if
designated by surgeon)
h. Results of intraoperative consultation
2. Tumor
a. Location
b. Configuration (Note G)
c. Size (3
dimensions)
d. Descriptive
features (e.g. color/consistency/hemorrhage)
e. Relationship to
mesenteric border
f. Ulceration
g. Obstruction/perforation
h. Proximal
dilatation
i. Depth of invasion
(layers of bowel present at lesion site, if discernible)
j. Status of
overlying serosa
k. Extension to other
organ(s)/structure(s)
3. Margins (Note H)
a. Proximal
b. Distal
c. Mesenteric
(radial), if applicable
4. Regional lymph
nodes (Note B)
5. Additional
pathologic findings, if present
a. Adenomatous polyps
(polyposis syndrome)
b. Hamartomatous
polyps (polyposis syndrome)
c. Crohn’s disease
d. Celiac disease
e. Other
6. Metastasis to other organ(s) or structure(s)
(specify)
7. Tissues submitted
for microscopic evaluation
a. Tumor
(1) point of deepest
penetration
(2) overlying serosa
(3) interface with
adjacent tissue
(4) interface with
uninvolved adjacent bowel
b. Margins (as
appropriate) (Note H)
c. All lymph nodes
d. Other lesions
(e.g., polyps/ulcers/fistulas)
e. Section(s) of
bowel uninvolved by tumor
f. Other
tissue(s)/organ(s)
g. Frozen section
tissue fragment(s)
7. Special studies
(specify) (e.g., histochemistry, immunohistochemistry, morphometry, DNA
analysis [specify type], cytogenetic analysis) (Note C)
C. MICROSCOPIC EVALUATION
1. Tumor
a. Histologic type (Note D)
b. Histologic grade (Note E)
c. Depth of invasion
d. Blood/lymphatic
vessel invasion
2. Margins (Note H)
a. Proximal
b. Distal
c. Mesenteric
(radial), as indicated
3. Additional
pathologic findings, if present
a. Adenoma(s)
b. Other types of
polyps
c. Dysplasia
d. Crohn’s disease
e. Celiac disease
f. Other
4. Regional lymph
nodes
a. Number
b. Number with
metastases
5. Metastasis to
other organ(s) or structure(s) (specify sites)
6. Other
tissue(s)/organ(s)
7. Results/status of
special studies (specify) (Note C)
8. Comments
a. Correlation with
intraoperative consultation, as appropriate
b. Correlation with
other specimens, as appropriate
c. Correlation with
clinical information, as appropriate
EXPLANATORY NOTES
A. Relevant History back
Top Main
Page
Conditions that predispose to small bowel
malignancy include Crohn’s disease, celiac disease, inherited polyposis
syndromes (including Familial Adenomatous Polyposis, Hereditary Non-Polyposis
Colon Cancer and Peutz-Jeghers syndromes). Prior surgery, especially for benign
or malignant tumors, clinical diagnosis, weight change, or change in body
habitus are also relevant.
B. Intraoperative Consultation back Top Main Page
Evaluation of specimens during the performance
of a procedure such as immediate evaluation of a cytologic aspirate or the
intraoperative gross or microscopic examination should be documented. The
sampling of the tissue should be documented in the macroscopic evaluation and
the findings of such examination should be documented in the final report, including
correlation with the final pathologic diagnosis or impression. Discrepancies,
if any, should be explained in the report.
C. Special
Procedures back Top Main Page
Special procedures may include:
immunohistochemical stains, histochemical stains, electron microscopy, flow
cytometry, cytogenetic studies etc. If such studies are performed in different
laboratories, either inter-institutional or intra-institutional, the
responsible laboratory should be stated.
D.
Histologic
Type back Top Main Page
For tumors of the small intestine, the protocol
recommends the histologic classification published by the World Health
Organization (WHO).(1)
WHO Classification of Small Intestinal Carcinoma
• Adenocarcinoma in situ / high grade dysplasia
• Adenocarcinoma
• Mucinous (colloid) adenocarcinoma (>50% mucinous)
• Signet-ring cell carcinoma (>50% signet-ring cells)*
• Squamous cell carcinoma
• Adenosquamous carcinoma
• Small-cell carcinoma**
• Undifferentiated carcinoma**
• Other (specify)
* By convention, signet ring cell carcinoma is
always assigned grade 3 (see below).
** By convention, small cell carcinoma and
undifferentiated carcinoma are assigned grade 4 (see below).
The term carcinoma, NOS (not otherwise
specified) is not part of the WHO classification. This protocol does not apply
to carcinoid tumors, lymphoma, or stromal tumors (sarcomas) of the small
intestine.
E. Histologic Grade back
Top Main
Page
A histologic grading system for adenocarcinomas
based on the extent of glandular formation in the tumor is recommended as shown
below.
Grade X Grade
cannot be assessed
Grade 1 Well
differentiated (>95% of tumor forms glands)
Grade 2 Moderately
differentiated (50-95% of tumor forms glands)
Grade 3 Poorly
differentiated (5-49% of tumor forms glands)
Grade 4 Undifferentiated*
(<5% of tumor forms glands)
The specific definitions of the above histologic
grades are as follows:
Grade
1: Well differentiated adenocarcinomas
are composed entirely of glands or have less than 5% of solid or cord-like
growth patterns.
Grade 2: Carcinomas
that are moderately differentiated have from 6% to 50% solid or cord-like
growth patterns.
Grade 3: Poorly
differentiated carcinomas have 49% to 90% of solid or cord-like growth
patterns.
Grade 4: Undifferentiated
carcinomas contain less than 5% of glands and consist primarily of pleomorphic
spindle and giant cells or small cells with considerable nuclear atypia, small
amounts of mucin and no endocrine differentiation.
F. TNM
and Stage Groupings back Top Main Page
Surgical
resection is the most effective therapy for small intestinal carcinoma, and the
best estimation of prognosis is related to the anatomic extent (stage) of
disease at the time of resection.
The protocol
recommends the TNM Staging System of the American Joint Committee on Cancer
(AJCC) and the International Union Against Cancer (UICC) but does not preclude
the use of other staging systems.(2,3)
By AJCC/UICC
convention, the designation "T" refers to a primary tumor that has
not been previously treated. The symbol "p" refers to the pathologic
classification of the TNM, as opposed to the clinical classification and is
based on gross and
therapy (e.g.,
surgical resection for cure) is categorized by a system known as R
classification, shown below.
RX Presence of residual tumor
cannot be assessed
R0 No residual tumor
R1 Microscopic residual tumor
R2 Macroscopic residual tumor.
For the surgeon,
the R classification may be useful to indicate the known or assumed status of
the completeness of a surgical excision. For the pathologist, the R
classification is relevant to the status of the margins of a surgical resection
specimen. That is, tumor involving the resection margin on pathologic
examination may be assumed to correspond to residual tumor in the patient and
may be classified as macroscopic or microscopic according to the findings at
the specimen margin(s).
In contrast,
tumor remaining in a resection specimen from a patient who has undergone
previous (neoadjuvant) treatment of any type (radiation therapy alone,
chemotherapy therapy alone, or any combined modality treatment) is codified by
the TNM using a prescript "y" (e.g., ypT1). Thus, yTNM indicates the
post-treatment status of the tumor. For many neoadjuvant therapies, the
classification of residual disease may be a strong predictor of postoperative
outcome. In addition, the ypTNM classification provides a standardized
framework for the collection of data needed to accurately evaluate new
neoadjuvant therapies.
In contrast to
"residual" tumor, classification of a tumor as "recurrent"
requires a documented disease-free interval after definitive therapy. Recurrent
tumor may also be classified according to the TNM categories, but the prefix
"r" (e.g., rpT1) is used to indicate the recurrent status of the
tumor.
Primary Tumor
(T)
TX Primary tumor cannot be
assessed
T0 No evidence of primary tumor
Tis Carcinoma in situ
T1 Tumor invades lamina propria
or submucosa
T2 Tumor invades the muscularis
propria
T3 Tumor
invades through the muscularis propria
into the subserosa or into the nonperitonealized perimuscular tissue (mesentery
or retroperitoneum*) with extension 2 cm or less
T4 Tumor
perforates the visceral peritoneum or directly invades other organs or
structures (includes other loops of small intestine, mesentery, or
retroperitoneum more than 2 cm, and the abdominal wall by way of the serosa;
for the duodenum only includes invasion of the pancreas)
*The
non-peritonealized perimuscular tissue is, for the jejunum and ileum, part of
the mesenetery and, for the duodenum, in areas where serosa is lacking, part of
the retroperitoneum.
Regional Lymph
Nodes (N)
NX Regional lymph nodes cannot be
assessed
N0 No regional lymph nodes
metastasis
N1 Regional lymph nodes
metastasis
Distant
Metastasis (M)
MX Distant metastasis cannot be
assessed
M0 No distant metastasis
M1 Distant metastasis
Stage Groupings
Stage 0 Tis N0 M0
Stage I T1 N0 M0
T2 N0 M0
Stage II T3 N0 M0
T4 N0 M0
Stage III Any T N1 M0
Stage IV Any T Any N M1
G. Configuration back Top Main Page
Configuration types include exophytic,
endophytic, diffusely infiltrative (linitis plastica), or annular. Exophytic type may be pedunculated or
sessile.
Margins include the proximal, distal and radial
margins of resection. For all small bowel segments, except the duodenum, the
mesenteric resection margin is the only pertinent radial margin. For the
duodenum, the non-pertionealized surface constitutes the pertinent radial
margin.
1. Jass JR, Sobin LH. Histological Typing of Intestinal Tumours.
2nd Ed. New York, Springer-Verlag, 1989.
2. Fleming ID, Cooper JS, Henson DE, et al. eds. AJCC Manual for
Staging of Cancer. 5th ed. Lippincott Raven, Philadelphia, 1997.
3. Fielding LP, Arsenault PA, Chapuis PH, et al.
Clinicopathological staging for colorectal cancer: an International
Documentation System (IDS) and an International Comprehensive Terminology
(ICAT). J Gastroenterol Hepatol. 1991;6:325-344.
BIBLIOGRAPHY
• Botsford TW, Crowe P, Crocker DW. Tumors of the Small
Intestine. Am J Surg. 1962;103:358-365.
• Galandiuk S, Hermann RE, Jagelman DG, Fazio VW, Sivak MV.
Villous Tumors of the Duodenum. Ann Surg. 1988;207(3):234-239.
• Holmes GKT, Dunn GI, Cockel R, Brookes VS. Adenocarcinoma of
the upper small bowel complicating coeliac disease. Gut. 1980; 21:1010-1016.
• Perzin KH, Bridge MF. Adenomas of the Small Intestine: A
Clinicopathologic Review of 51 Cases and a Study of Their Relationship to
Carcinoma. Cancer. 1981;48:799-819.
• Perzin KH, Bridge MF. Adenomatous and Carcinomatous Changes in
Hamartomatous Polyps of the Small Intestine (Peutz-Jeghers Syndrome). Cancer.
1982;49:971-983.
• Spira IA, Ghazi A, Wolff WI. Primary Adenocarcinoma of the
Duodenum. Cancer. 1977;39:1721-1726.
Author
Stephen
G. Ruby, MD
©2000.
College of American Pathologists (CAP). All rights reserved. None of the
contents of this publication may be reproduced, stored in a retrieval system or
transmitted in any form or by any means (electronic, mechanical, photocopying,
recording, or otherwise) without prior written permission of the publisher.
Contributors: back Top Main Page
CAP Cancer Committee; Gregorio Chejfec,
MD; John A. Payne, MD; Jerome B. Taxy, MD; Kay Washington, MD; Christopher Willett,
MD; James Williams, MD