Since Abrikossoffs report,5 many instancs of tumors of this type

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MYOBLASTOMA
(GRANuLAR CELL MYOBLASTOXA OR MYOBLASTIC MYoMA)*
A. R. CRANE,M1D., and R. G. TBLAY, MD.
(From the Pathological Laboratories, Norfolk General Hospita, Norfolk, Va,
and St. John's Hospt, Brooklyn, N.Y.)
Tumors presumably composed of immature skeletal muscle elements
and variously described as myoblastic myoma, myoblastoma, granular
cell myoblastoma, rhabdomyoma, rhabdyome granulo-cellulaire, etc.,
are now recognized as a definite pathologic entity. Credit for the first
clear description of these tumors under the name "'myoblastic myoma"
is given to Abrlkossoff 5 who reported 5 cases in 1926. However, in
1854, Weber ' reported a case of hypertrophy of the tongue in a male,
2 Yyears old, who developed new growths after exesion of the portion
protruding beyond the teeth. These new growths prested micro-
scopic features identical with the tuors now known as myoblastomas.
Other earlier reports of identcal lesions were made by Heurtaux2
(I88I), Pendl3 (1I897) and Moschcowitz4 (1922). The case reported
byHeurtauxwas thatof a sa nodule occurring on the dorsum of the
tongue in a female, i9 years old, and was interpreted by him as granu-
lar-protein degenerato of muscle fibers. Pendl dei his neo-
plasm, occurring on the tongue of amale, 8 weeks of age, as a rhabdo-
myoma and explained it on the basis of misplacd embryonic tissue.
Moschcowitz labelledhis tumor, occurrig on the dorsum of the tongue
of a female, 30years old,asa "xanthoma," describing itasamyogenet-
ic form of thona. Abrikossoff,' writing m 1926, described 5 cases
(3 of the tongue and one each of the lip and gastrocnemius muscle),
dearly stating their relationship to striated muscle but regarding the
lesion as a degenerative process following tiauma or inflammation.
Later,however, inthe light of cases reportedby Klinge 10and of subse-
quent cases of his own, he felt that some of the tumors might have de-
veloped fromprimitive myoblasts of the embryo.
Since Abrikossoffs report,5 many instancs of tumors of this type
have been reported and we have been able to collect the details of I62
cases (Table I),including 5 of ourownreportedhere.
REPORT OF CASES
Case i
The ptit was a newborn white female (S. J. S37-657), delivered of a 23-
year-oldprmipa Physicalexmintion was negative save for the occurrence of
three distinct growths on the gingival ridge of the maxilla at about the location of
the right upper lateral insor, the lagest measuri 2 by I.5 cm. The largest
*Received for ption, May 12, 1944.
357
358 CRANE AND TREMBLAY
growth was smooth, egg-shaped and pedunculated and partially covered by the
smaller ones which were also pedunculated. All were covered by mucous mem-
brane. Ihere were no abnormal laboratory findings and roentgenograms of the
bony skeleton were negative. The tumor was excised and there was no recurrence.
Grossly, the specimen consisted of four smallpieces of tissue 0.7 cm.
in diameter and one piece 2 by I.5 cm. These were covered by smooth
mucosa and presented firm, white to light brown cut surfaces. Micro-
scopically (Fig. i), the sections showed the tissue to be covered by a
normal squamous epithelium and to be composed of large (30 to 70p.),
round, polyhedral, or spindle-shaped cells with acidophilic cytoplasm.
The cytoplasm contained dense, coarse, acidophilic to neutrophilic
granules giving ita granular appearance. Thenucleiwere small, round,
central and vesicular, though many pyknotic forms were also present.
No irregular, lobulated, or atypical tumor nuclei were noted nor were
mitoses seen. No lipoidal material was identified with fat stains. The
stromawas looseandedematous in someportions, but for themostpart
the cells were closely packed with scant supporting stroma.
Case 2
The patient was a newborn colored female (S. J. S43-643), born of a 27-year-old
woman whohad one previous normalpregnancy with the child living and well. The
physical examination was negative save for the occurrence of a smooth red tumor
covered by gingival membrane and located at the gingival margin of the mandible
just to the left of the midline. The tumor was excised and the child discharged.
She was returned 3 days later with a history of feeding poorly and slight bleeding
from the gum. At this time the physical examination showed marked listlessness
and dehydration with depression of the fontanelles. The wound in the mouth was
well healed. The heart and lungs were negative. The abdomen was distended and
an enlarged liver could be palpated. The extremities were negative. The child was
in extremis and died 4 hours after admission.
Grossly, the tumor consisted of a nodule of tissue I.5 by o.8 by o.8
cm. and covered over one surface by a smooth membrane. The cut
surfaces were soft, golden-yellow and fatty in appearance. Microscopi-
cally, the tissues was composed of cords and masses of large, round,
polyhedral, or spindle-shaped cells separated by a loose, edematous,
fibroblastic tissue (Fig. 2). The cells had a coarsely granular, eosino-
philic cytoplasm and showed small, central, round, vesicular nuclei
(Fig. 3). Some of the cells were elongated, cylindrical or teardrop
in shape, the longer ones showing one or two points of narrowing
and containing two or three nuclei in different portions of the cell.
With phosphotungstic acid and hematoxylin stain many cells showed
fine, fibrillary, cross and longitudinal, purple fibrils which did not
traverse the entire length of the cells (Fig. 4). The cells also showed
a purple to blue, granular appearance of portions of the cell membrane.
MYOBLASTOMA 359
Mallory's aniline blue stains showed similar fibrils and granular struc-
tures staining red and in a linear arrangement, and very coarse granu-
lations staining dark purple (Fig. 3). The connective tissue extended
around individual cells as fine strands of collagen but at some points
the cls were in direct apposition. No mitoses were noted. Spedal
stains for fat were negative.
The autopsy showed no evidence of tumor at the site of the original
lesion. The only positive findings were in the liver and kidneys. The
liver was enlarged (150 gm.) and presented a smooth yelow surface
and a firm yellow cut surface. Microscopically, the liver cells were
swollen and distorted by large, clear, fat droplets. The portal zones
showed an apparent increase in bile ducts as seen in biliary cirrhosis.
The kidneys were grossly normal but showed many fat droplets in the
convoluted tubules with a special stain, but no other abnormalities.
The anatomic diagnoses were: imbalance of fat metabolism with fatty
metamorphosis of the liver and lipoid nephrosis, biliary cirrhosis of the
liver, cerebral edema.
Case 3
The patient was a Negro, 26 years of age (S. V. i8566). A tumor the size of
a walnut was removed from the anal region. Further dinical details are not
available.
Grosly, the tumor measured 4 by 3 by 2.2 cm., was covered by skin
and showed a red-gray, firm cut surface. Microscopically, the tumor
was composed of strands and masses of cells which were polygonal to
rounded and often elongated. The cells were acidophilic, finely granu-
lar, andoccurred singlyandin solidmasses. Thestroma was dense and
fibrous. Special stains for fat were negative. The overlying squamous
epithelium showed some downgrowth of the rete pegs and melanotic
pigment had been mobilized, being present in macrophages in the
papillary layer.
Case 4
Awhitefemale, 29years old (S. V. 10324), whohad injuredherleft knee 8 years
previously, noted a mass the size of apea on the inner side of the thigh just above
theknee. lhis grewto the size of a small walnuL Ihere were a few longhairs over
the nodule. There was somepainin the region of the knee but the lesionproper was
not tender. Physical mination was negative save for the local lesion, which was
attached to the sirin but not to the bone or musde. The lesion was excised locally
and there has been no recurrence.
Grossly, the tumor was i.5 cm. in diameter, sharply circumscribed,
and presented a firm, gray-white cut surface. Microscopically, the
tumor was composed of groups and cords of polyhedral to spindle-
shaped eosinophilic cells separated by a dense fibrous tissue. The cells
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CRANE AND TREMlC3LAY
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MYODIASTOMA 36I
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MYOBLASTOMA 363
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were finely granular and had small vesicular nuclei. Mitoses were not
noted. A special stain for fat was negative.
Case 5
The patient was a white male, 47 years old (S. V. I3400), who complained of
a hard spot on his tongue of 2 months' duration. There was no history of injury.
The physical examination was negative save for the occurrence of a firm, bean-like
nodule at the lower right margin of the tongue. It was not ulcerated. The lesion
was excised locally without recurrence.
The gross findings are not known. Microscopically, the lesion was
composed of masses of large, granular, polyhedral to ribbon-like cells
intermingledwith skeletal muscle. The nuclei weresmall andvesicular.
No mitoses were seen. At some points adult skeletal muscle cells ended
inbulbous granular swellings similar in appearance to the cells compos-
ingthemajorportion of thelesion.
MORPHOLOGIC FEATURES
Gross Characteristics. The tumors reported as myoblastomas aver-
age I to 2 cm. indiameter, thelargestonebeing io cm. in diameter, and
the majority are sharply circumscribed or truly encapsulated. They
may, however, be poorly demarcated and irregularly penetrate the
underlying tissues. Those located under the mucous membrane are
usually covered by a smooth, intact epithelium, although some reported
cases have shown ulceration of the epithellum and others hyperkera-
totic, or even malignant proliferation of the epithelium. They may be
nodular or lobulated but usually present a solitary, distinct, round
mass. Frequently they are pedunculated, polypoid, or sessile. On sec-
tion they have firm, gray to yellowish-gray or tan surfaces.
Microscopic Characteristics. Thetumors are composedof largepoly-
hedral cells, the majority being irregularly rounded, but transitional
formshavingatear-drop, spindle, or cylindrical shape may alsobe pres-
ent. The rounded forms vary from 20 to 6o ,u in diameter, while the
spindle or cylindrical forms are much longer, measuring up to 300
IL
in our own cases. Under low magnification the cells appear to occur
singly or in groups, but with higher magnifications and special stains
for connective tissue, such as Mallory's aniline blue, Masson's tri-
chrome or van Gieson's, delicate strands of connective tissue are found
to penetrate among and surround the majority of the cells, but small
clusters of cells do occur in direct apposition to one another. In some
portions the collagen framework is abundant and edematous in poly-
poid lesions.
The most characteristic feature is the cytoplasm of the cells which
is abundant, acidophilic and granular. The granules vary from the fine
acidophilic to large, coarse forms that are neutrophilic. It must be em-
MYOBLASTOMA 365
phasized thatthecellsaregranular and not vacuolated and that speial
sim for lipoid are negative. Some authors 273 have suggested that
the cellsmaycontain gycogen andmucn-likesubstances but adate
sti with prope: fixation have not been done to warrant any state-
ment in regard to these substances. Longitulinal and cross striations
have been demonstrated but not constantly, and lemperer has de-
scribedanarrangementofthegranulesin rows resemblingfibris. With
im proper fiion, and staining (Zenkeres and Mallory's phos-
photungstic-add-hematoxylin and aniline blue stains) our own cases
show dear-cut, fine, longitudinal andcrss fibrils.
The nuclei are sml (7 to io ) and vesicular, but prkotic forms
may be numerous. They are round to oval and centrally located. In
our own cases some of the longer, cylndrical forms contain 2 or 3
nuclei. Mitoses are usually absent although Ceelen,17 Derman and
Golbert1 andMorpurgo31 reported their occurrence.
An additional as finding is the proliferation of the overlying
epithelium first mentioned by Keynes, noted by many others and em-
phasized by KSlemperer.Y Some cases of hyperkeratosis of the epithe-
liumhavebeenreported andcases reported by Schirmer,n Morpurgo31
and by Eichoff showed squamous cell carnomas of ffie overlying
s
epithelium,whileLerouxandDelaruex reported acase associatedwith
a basal cell carcnoma.
Differential Diagnosis. These tumors are most likely to be inter-
preted as thomas, the first two cases reported here having been so
diagnoed originally and case I so reported in the literature.74 The
total absence of fat differentiates them from the true thomas.
Geschickter I' reported 2 cases that were misintepreted as chordomas.
Scalled congenital epulis of the newbon, as described by Massim,s
Kleinee and Jorge, Nudelman and Gringauz, presents features iden-
tical with the myoblastomas and we believe that they should be so re-
garded. The tumors are also to be differentiated from rhabdomyomas
and rhabdomyosarcomas which are composed of adult skeletal muscle
elements.Cmi F s
CLCeAL FFATURES
Incilence as to Age and Sex. These tumors occur at all ages and
with equal frequency among males and females, as shown in Table II;
they are not more common in males as previously supposed. Forty-
seven per cent of the cases have occurred in persons under the age of
50years, with the greatest incdence occurring in the third, fourth and
fifth decades. Lesions of the alveolar processes occur chiefly in chil-
dren. All of the maxilla were in newborn infants, but two of three
tumors of the mandible occurred in the third and fifth decades.
Distribution. The location of the tumors in various portions of the
366
CRANE AND TREMBLAY
body is indicated also inTable II. The tongue is by far the most com-
monsite, 38 percentofreported cases occurring inthis locationandthe
vast majority being located on the dorsal surface. Skin, subcutaneous
tissue, muscle, alveolar processes, respiratory tract, breast, ear, andlip,
in decreasing order of frequency, account for the majority of the other
cases. The skin lesions are reported from thepelvic region, extremities
and breast. Isolated instances have also occurred in the anus, bladder,
TABLE II
Location, Age and Sex Distribution of z62 Cases of Myoblastoma
Ageindecades 1-9 10-19 20-29 30-39 40-49 0-S9 6o-69 70-79 Totals
Age and
Sex M FM FM FM FM FM FM FM FM F sexnot Final
given
Alveolarprocess 2 2
Mandible 4 I 4 6
Maxila I 6 I 6 47 I7
Breast I I 2 6 8
Ear zI I 3 3
Larynx and
vocalcord 2 I 2 2 I 5 3 8
Lip I I 3 4
Muscle 1 2 I 4 9 1.3
Skin I II1 1 3 II1 I 55s 7 17
Subcutis I I 2 IS 17
Tongue 2 I 3 6 I 8 6 7 2 3 3 2 I 29 I6 i6 6i
Trachea and
bronchi I I 2 I 3
Other* I 2 I II B2 4 4 I 9
AEU V LS BP M
Totals 3 II I 410 gII gIl 8 7 5 4 4 I47 5I 64 I62
*A=anus, B=bladder, E=upper esophagus, L=lacrimal sac, M=floor of mouth,
P=pharynx, S=spermatic cord, U= urethra, V=vulva.
This table includes our own cases and those collected from the literature, as presented in
Table I.
esophagus, lacrimal gland, floor of themouth, pharynx, spermatic cord,
urethra and vulva. Stout75 has recently encountered 2 examples in the
uterus. While morethanonenodule mayoccur at one site, there areno
instances of tumors occurring at more than one place in one patient.
Lesions. The tumors occur as firm, small (I to 2 cm.) nodules that
may be polypoid or sessile. They may increase slowly in size. The
overlying epithelium is usually intact but may be ulcerated. They are
nontender.
Course. The lesion is a benign one and local excision will effect a
complete cure. However, if incompletely removed they may recur lo-
cally, as reported by Horn and Stout; 51 and Geschickter 25 reported
a tumor starting in the sacral region which showed metastases to the
groin. The metastases were treated with radium and the patient was