T H E AMERICAN JOURNAL or CLINICAL PATHOLOGY
Vol. 44, No. 4
Copyright © 1905 by The Williams & Wilkins Co.
Printed in
MULTIPLE GRANULAR CELL MYOBLASTOMAS OF THE
U.S.A.
STOMACH
DAVID T. SCHWARTZ, M.D., AND HAROLD P. GAETZ, M.D.
Surgical Service and Department of Pathology, St. Luke's Hospital, New York, New York
Since Abrikossoff1 first described the
so-called granular-cell myoblastoma, only 3
cases of granular cell myoblastoma of the
stomach have been reported.11'1G The following are the fourth and fifth such cases to be
reported, and the first case of multiple granular cell myoblastomas to be found in the
stomach.
R E P O R T O F CASES
Case 1
A 39-year-old Negro man was seen at St.
Luke's Hospital, complaining of mild epigastric pain which was relieved by the ingestion of food, and vomiting small amounts of
gastric material for 4 months. Physical examination on admission was negative.
Laboratory studies were noncontributory.
An upper gastrointestinal barium roentgenogram showed multiple filling defects,
3 cm. in diameter, in the fundus and pars
media of the stomach. To the radiologist
these suggested multiple gastric polyps or
lymphosarcoma. The duodenal bulb was
normal. A gastric aspirate contained only 5
degrees of free acid 30 min. after histamine
stimulation.
At operation the stomach was found to
contain 5 mural masses, and a 90 per cent
gastrectomy and gastroduoclenostomy were
performed. The patient's postoperative
course was uncomplicated. When last seen,
56 days after operation, he was gaining
weight and feeling well.
Pathology. The most striking gross alteration of the subtotally resected stomach was
confined to the mucosal aspect of the greater
curvature. There were 5 discrete, nonencapsulated, white, whorled, firm submucosal
nodules, measuring up to 2.5 cm., fixed to
overlying mucosa (Fig. 1). The mucosa over
4 of these nodules was hyperplastic but flat,
whereas the fifth nodule was surmounted by
a 1.5-cm. pedunculated polyp.
Received, February 15, 1965.
The polyp (Fig. 2) had a fibrovascular
stalk, and impinging on the muscularis mucosa at the base were clusters of polygonal
and spindle-shaped granular cells (Fig. 3).
Other broad-based elevations had hyperplastic mucosa, with granular cells crowding
out mucosal glands (Fig. 4). There was
superficial ulceration in 1 of these mucosal
elevations.
The heterogeneous granules stained positive with periodic acid-Schiff and trichrome
sarcoplasmic stains. Most of the granularcell myoblastomas were situated in the submucosa, but extension into the mucosa
was present.
Case 2
A 44-year-old Negro man was first admitted to the Columbia Presbyterian Medical Center on September 9, 1960, with a
2-months' history of anorexia, cramping midepigastric pain, vomiting, and a 20-lb.
weight loss. Except for minimal midepigastric tenderness, his admission physical examination was normal. At the time of admission, the urine, complete blood count, and
roentgenogram of the chest were negative.
An upper gastrointestinal barium roentgenogram showed multiple filling defects on
the greater curvature of the stomach. On the
third hospital day a sleeve resection of the
middle third of the stomach, pyloromyotomy,
and gastrogastrostomy were performed. Five
gastric polyps were found. In addition, a
polyp, 1 cm. in diameter, proximal to the
incision in the wall of the lesser curvature,
was dissected from the submucosa and excised, and the mucosal defect was sewn
with catgut. This was the site of postoperative bleeding, and led to reoperation and
resection of the distal cardia and the remaining antrum of the stomach, with a
Hofmeister gastrojejunostomy. Thereafter
his postoperative course was complicated
by fever, which responded to Achromycin,
and coughing. He was discharged on Sep-
453
454
SCHWARTZ A
AND GAETZ
tember 20, 1960, with a 6-feeding ulcer
diet. When readmitted 10 months later for
an unrelated problem, he complained only
of mild epigastric pain. At that time the
hemoglobin was 13.8, but the stool guaiac
was recorded as positive. An upper gastrointestinal series revealed a normal pouch
and anastomosis without evidence of ulceration. The guaiac-positive stools were not
investigated further, and he was lost to
follow-up thereafter.
Pathology. The mucosa of the resected
stomach was the site of multiple, stalked,
broad-based, and sessile adenomatous
polyps, ranging in size from 0.8 cm. for
the latter, and up to 2.5 by 1.5 cm. for the
former. The lesser curvature gave rise to a
firm, white, submucosal nodule, measuring
1.8 by 0.8 cm., which fixed the overlying
mucosa. It was discrete but not encapsulated, and not related to the mucosal polyps.
The submucosal granular cell myoblastoma
and the multiple adenomatous polyps were
microscopically similar to those described
in the first case.
DISCUSSION
Although the embryologic origin of the
granular cell myoblastoma has been the
chief object of discussion in the literature,
agreement has not yet been reached as to
its nature. Abrikossoff1 thought that the
skeletal muscle origin of the tumor was
evident in its histologic appearance, and
Murray's 13 tissue cultures of cells from such
tumors confirmed Abrikossoff's impression.
Striations, however, have rarely been seen.
Fust and Custer10 first reported the appearance of granular cell tumors within
nerve sheaths, with neural histologic characteristics, and proposed the name "granular cell neurofibroma."
Although most recent authors favor the
neural theory of origin,8 neoplastic,11 degenerative, and traumatic origins have also
been suggested. Recent histochemical14 and
electron microscopic evidence support either
a neural or histiocytic origin. Fisher9 has
described virus-like particles in electron
micrographs of granular cell myoblastomas
from different locations, but has not evaluated their significance.
Vol.
44
The granular cell myoblastoma occurs
in the tongue in 38 per cent of reported
cases,7 and in the skin, skeletal, muscle,
and subcutaneous tissues in another 29
per cent. Abrikossoff2 was the first to describe the tumor in the gastrointestinal
tract, reporting the case of a patient with a
pea-sized granular cell myoblastoma in the
upper esophagus. Since that time, 8 additional cases of granular cell myoblastoma
of the gastrointestinal tract have been
described. 3,4 ' 6i 8 ' u ' 1 2 , 1 5 There have been
a total of 3 cases in the esophagus,2, s ' 1 6
5 in the stomach, 3 ' "• 1 5 (including the cases
reported here), 3 from the colon, 6 ' ll - 12 and
1 in the common bile duct.4 It is interesting
to note that Goodman's11 second patient,
Azzopardi's3 patient, and our patients had
symptoms of peptic disease. An association
between gastric granular cell myoblastoma
and peptic symptoms has not been noted
previously in the literature.
By 1962 there were 18 reported cases of
multiple granular cell myoblastomas, 5 and
another has been reported this year.17
Most of these occurred in subcutaneous
sites, and none of them in the gastrointestinal tract. The case reported here is the first
such example.
Although Goodman11 has noted the association of granular cell myoblastoma of
the gastrointestinal tract with epithelial
hyperplasia of the mucosa overlying it,
the second case reported here represents
the first documented association of the
tumor with frank multiple adenomatous
polyps. Histologic contiguity between the 2
abnormalities was found in the first case
(Fig. 2) but not in the second case. Although
the simultaneous occurrence of multiple
granular cell myoblastomas and histologically contiguous hyperplasia or adenomatous
epithelial change might suggest a single
etiologic agent, such an agent is not known.
SUMMARY
Two cases of granular cell myoblastoma
of the stomach (one multiple), associated
with benign gastric polyps, have been reported.
Acknowledgment.
Dr. P . Bohnslay and D r . R.
L a t t e s gave permission to report the second case.
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F I G . 1 (upper). Formalin-fixed, cross sections of 3 of the granular cell myoblastomas of Case 1. Note
tag of hyperplastic mucosa atop granular cell myoblastoma at left. Each bar on the ruler is equal to 1
mm.
F I G . 2 (lower). Base of polyp. A small amount of the stalked, convoluted gastric mucosa, replete
with normal glands, is present. Granular cells may be seen in the submucosa, within the muscularis
mucosae and mucosa. Hematoxylin and eosin. X 25.
455
r
F i o . 3 (upper). Section from a central area of the tumor, depicting the " o r g a n o i d " p a t t e r n of spindled
and polygonal cells. Note cytoplasmic granularity. Hematoxylin and eosin. X 400.
F I G . 4 (lower). Section demonstrating mucosal involvement by the granular cell myoblastoma. Note
" c r o w d e d " gastric glands amid "proliferating" granular cells. Periodic acid-SchifT. X 400.
456
Oct. 1965
M U L T I P L E GRANULAR CELL
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MYOBLASTOMAS
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