Carcinoid tumor of the colon in a child

Carcinoid Tumor of the Colon in a Child By Gerschon

l This is a case with

a

primary

transverse

report

Suster,

Arthur

of a 9 yr old

carcinoid

tumor

of

G. Weinberg,

INDEX

boy

and

Leonard

WORDS:

Graivier

Carcinoid

tumor.

the

colon.

Carcinoid tumors are uncommon. and in the pediatric age group rarely occur at sites other than the vermiform appendix. Instances of rectal and small intestinal carcinoid have been recorded in children, but Involvement of the colon has not been described in this age group.

CASE

REPORT

A 9 yr old Caucasian boy was admitted to the hospital because of rectal bleeding. He was well until I mo prior to admission when he complained of IntermIttent. colicky abdominal pain. rect,jl bleeding, and poor appetite. Past medical history and family history were unremarkable. Phvsxdl examination was negative. but bright red blood was noted in his stool. Proctoscopy did not reveal any lesions. Stoola for ova and parasites were negative. A gastrointestmal series, including the terminal ileum. was normal. A barium enema revealed a 4 cm sessile mass in the proximal limb of the splenic flexure of the colon (Fig. I). Exploratory laparotomy was performed and a tumor was found approximately I5 cm proximal to the splenic flexure in the wall of the transverse colon. A left transverse colectomy with colocolostomy was performed. A segment of the mesentery Including the vessels draining this area was resected. The liver, terminal ileum, and appendix were normal to palpation. Postoperative studies including a bone marrow. bone scan. and liver and spleen scan did not reveal any abnormality. A 24hr urine i-hydroxyindole acetic acrd (5-HIAA, was 2 mg (normal O-10 mg). The resected specimen contained a pale gray, firm. sessile tumor 3.5 cm in diameter in the mesenteric aspect of the bowel wall. The tumor extended transmurally and measured 2 cm in depth. The overlying mucosa was centrally ulcerated. Several small tumor nodules were present on the serosal surface in contiguity with the main mass. There were no palpable mesenteric nodes. MIcroscopically. the tumor consisted of sheets. nests. and cords of relatively uniform cells ernbedded in a dense scirrhous stroma (Fig. 2). The cells had round to oval nuclei with little ple’omorphism and a moderate amount of amphophilic cytoplasm. In some areas the cells and nuclei were enlarged, with more disllnct cell margins. The histopathologic features were considered typical of carclnoid tumor. Argentatiin stains of the tumor cells were negatice. while adjacent normal mucosal enterochromaffin cells were strongly positive. The tumor cells were argyrophillc. The serosal nodules were interpreted as direct invasion. No lymph node metastases were identlfied. Electron microscopic studies were performed on material that had been fixed In IO”,, formalin. This resulted in suboptimal preservation, but small numbers of dense-cored neurosecretory granules were identitied in the tumor cells. The cell membranes showed frequent small desmosomes. some of which were associated with tonoflaments.

DISCUSSION

Gastrointestinal carcinoid tumor most common11 arises in the vermiform appendlw. followjed in decreasing frequency by the small intestine, rectum. and stomach. Colonic involvement is illfrequent,

but has been described in adults. In a review of 3,672 cases

From

the Departments

of Pediatric.r.

The Univer.vit~ of Texas Health Address Center,

reprint

requests

I935 Amelia

c 1977

Street,

Pathology,

Sciences Center,

to: A. G. Weinberg. Dallas.

by Grune & Stratton,

and Surgery.

Dallas. M.D..

of gastrointestinal

Children’s

Medical

carcinoid

Center.

and

Texas. Department

of Patholog>,.

Chiidren’s

Medicui

Te.uas 75235.

Inc. ISSN

0022

3468

Journal of Pediatric Surgery, Vol. 12, No. 5 (October),

1977

739

SUSTER,

WEINBERG,

Fig.

1.

large

Cheek

appcndiceal have not.

tumors

Forces

tumors The

of I9 pediatric

tumors,

in this age group.

coupled

appendiceal carcinoid

precludes

are frequently carcinoid carcinoid

tumor

between

in other

a 33”,, incidence.”

lesions

died

vived.’

Thus.

silent site

is most

from

their

increased likely

but

Colonic

rarity

are found

Hajdu

found

that

while

four

mortality

of of

I5 yr of age listed

at the

large

delayed

diagnosis

prognosis

complicating

of

adults

with in

only

in

of

Wilson.

deeply

superticial arising

and

is made

of metastasis invasive

large

invasion

sur-

cecal in

of

colonrc

distinctton

and

in

cccum

exceeds that

and

When with

extra-

tumors

the

tumors

general

tumors

rarity

extra-appendiceal

arise

Cheek

of 60”,,.

of

The

of carcinotd

involvement. The

to have a 71”,, incidence

bowael lesions

appendiceal.

in the colon.4

often

series

two of four patients

were

of extra-appendices1

most

of

20 yr.2

of colonic

course with a mortality

disease

a reflection

the

in a review

of the low inctdence

disease

tumors

tumors

than

The mean size of colontc

sites. the former

bowel

originating

is excellent.3

sites.2 In the collected

had the most maltgnant

and the latter

none

extra-

carcinoid

younger

all of which

a reflection

Metastatic

patients.

evaluation.’

cecal and other colonic

particular

is less clear.

less than

low frequency

by the time of diagnosis.

arising

the

noted

is likely

Although

Berardi.

no patient

26 tumors.

in childhood

in pediatric

statistical

massive

tumors

carcinoid

in childhood

has been reported

children

in children

found

with the overall

of appendiccal

tumors

tumor

colonic

age group. found

flex m

It

‘se colon.

involvement.’

in childhood,

of the rectum,

They

patients

colonic

in the pediatric

exclusive

of Pathology.’ tn pediatric

of

may occur

been described

involvement

prognosis

incidence

carcinoid,

all cases of carcinoid

Institute

Field et al., rn a review of colonic

a 2.5”,,

rectal

of the colon

et al. reviewed

Armed

found

including

to our knowledge,

1 I8 carcinoid Ryden

and Wilson

tumors.

ene

splenic

radiolucer

in transvel

tumor,

Barium tes

demonstra with

AND GRAIVIER

this

lesions

in

relatively

CARCINOID

Fig.

731

TUMOR

2.

Photomicrograph

Note

of colonic

underwent

nodules

anterior

specimrn. anatomosls

and

negative

;t urine

of the literature

been assoclatcd

were

was Identical

was present

wedge

diaphragmatic

does not suggest that otfcr

hepatic

were

patient

of the hepatic

lesions

Nere

in

rectal

tumors. Increxc

carcinoids

tumor<.

role

Dctermlncltion

;L detcctuble

In colon~c

chemothernpj

a sl_eniticant

of choice.

disc;w

with colonlc

The

previour

..\n

postoperatively

The to the

of

dr:llnapr.

was normal.

within

biopsied.

at the site

lymphatic

resection

the

The

palpated

tumor

the

E)

2 mm nodules

These

in

8

negn-

wx\

rc-

mctaqtases

performed.

The

\\cII.

the treatment

reaction

the

were

(H

he had

of the diaphragm.

liver.

level

-here

doe& \-rak

and do not produce

argentattin

of the tumor

recurrent

tumor nor

i-HIAA

of

remains

mctastatic

the

and C ,4T scan performed

removal

nor

of

of the

No

to surgery

patient

tumors.

lobe

of carcinoid

In the interim

up to 1.7 cm in diameter

right

original

turned

on

ctppearance

colonic t~ve and

in delinlng

resection

operation.

the undersurface

were found

arteriogram

remain.\

One year after

a “second-look”

typical

liver and bone scan. Several

the

excision

a pattern

been well with a negative

histologic

.A review

showing

of tumor metastatic

carcinoid

in proof:

added

patient

tumor

of urine

as hindyut drtined

reaction

v;tlue

trtxtment.

5-Hl.4A

tumclrs

111trhptophan

I\ Iehs well

The argcntatlin

k 01 much

in their

111dc:llin@ v.lth

Adequate

excretion are

generally

metabolites.x although

W:I\ nepatlve

.trgsn~atiin-

The

carclnold

\urglcal

ih nor hclpl’ul statu\ s)ndromc

of the ha\

in the present caw

REFERENCES I.

Curr

Cheek

KC.

Wilson

Prob in Surg Nov.

7. Herardl

H: (‘arcinoid 1970. pp

KS: Carcinoid

tumors.

I 30

tumors

01’the colon

(eucluhive

of the rectum ). 1~15 Cal

Kect

15-381.

I971 3. Ryden

Slz.

Drake

KM.

i-rancl~l\l

IL\:

SUSTER,

742

Carcinoid Cancer

tumors

in children.

JF, Adamson

carcinoids

in

LF.

Stockle

children.

HE:

Re-

Pediatrics

29:

953, 1962 5. Barker

DH.

Michener

argentathnoma~report

WM:

Malignant

of a case of an

II

RJ, Axtel

HK:

Carcinoids

7. Hajdu Carcinoid Clin

yr

tract.

SI.

Surg

Path 61:521,

8. Black

of the

vest 19:473,

Winawer

tumors

WL

and corresponding

old boy. Cleve Clin Q 32: 143. 1965 6. Sanders

gastrointestinal

AND GRAIVIER

Gynec

Obstet

119:

369, I964

36: 1538. 1975

4. Field view of

of the appendix

WEINBERG,

1968

a study

SJ.

Myers

WPL:

of 204 cases. Am

J

1974 Ill:

Enterchromaflin carcinoid

tumors.

cell types Lab

In-