GRANULAR-CELL
MYOBLASTOMA
OF THE VULVA
Review of the Literature and Report of Four Cases ALAN
(From the Gynecologic
Department Research
of Obstetrics of the School
and
Gynecology and and Hospital
of Medicine
myoblastoma
is a tumor
M.D.,
KITBIN,
the
PHILADELPHIA,
Gynecean Hospital Institute University of Pennsylvania)
PA.
for
of the
which
was first described in
RANULAR-CELL G 1926 by Abrikossoff,l hence the eponym, Abrikossoff’s tumor. It charact,eristicallg occurs as a small, solitary, painless, poorly encapsulated mass resembling a fibroma. ICxoscopically it is composed of irregular st,rands of fascicles of large, elongated cells resembling myoblasts, and containing coarse, granular, eosinophilic cytoplasm. The cells may have intlistinc,t borders, giving the appearance of a multinucleated granular cytoplxsmic mass. Varying amount,s of fibrous tissue are present,. When the tumors arise directly beneath squamous epithelium, as in the skin, vulva, oropharyns. or tongue, the overlying epithelium may show abnormal proliferation sugg:rc>stiveof early squamouscc~llcarcinoma. The histogenesis of the tumor is obscure. Indeed, four principal tissues of origin have been suggested : striated muscle, histiocytes, fibroblasts, and peripheral nerves.’ Evidence in favor of a neural origih is increasing.? Only 19 cases of its occurrence in the vulva. have been reported.“-lR They are summarized in Table I. Sixteen of the reports have been published within the last 10 years. This, combined with the fact that 4 cases which have been discovered in 011~’ institution are herein reported, suggests that the tumor may not be as rare as was previously thought, and that JI~‘C cases will be diagnosed as awareness of this lesion grows. CASE I.--E. M., a 30.year-old white para ii, during the course of examination because of intermenstrual bleeding, was discovered to have a 1 cm., firm, movable, nontender mass in the region of the left Bartholin’s gland. A dilatation and curettage, biopsy of the cervix, and excision of the mass were performed, At operation it was noted that no definite cleavage plane was present between the mass and the surrounding vulvar tissues. The tumor was excised with but little sui-roundihg tissue. The cut, surfare was pale gray and smooth. Microscopic sections (Fig. 1) showed it to be composed of fairly large pink-staining cells, closely packed together, with small nuclei. The cells tended to be spindle shaped in some areas, and were arranged in bundles or whorls. The cell borders were distinct in some areas, indistinct in others. The bundles of cells wore separated by varying amounts of fibrous tissue. The epithelium overlying the tumor was not removed. The patient has been free of evidence of recurrence for 3 years.
292
Volume 77 Nlrmber 2
MYOBLASTOMA
GRANULAR-CELL
OF
VULVA
293
CASE 2.*-A. D., a &?-year-old white woman, had noticed a slowly increasing lump in the skin of the left labium majus for 3 years. Examination showed a solid 1.5 by 1 em. mass in the left Iabium majus. It appeared to lie just beneath the skin and to be attached to it. It was thought to be a calcified sebaceous cyst. The mass was excised under local anesthesia. On cut surface it was grayish brown and homogeneous. Microscopic examination showed the The tumor (Fig. 2) was composed of largc cells conoverlying epidermis to be normal. taining coarsely granular cosinophilic cytoplasm. The supporting stroma consisted of dense,
Fig.
L-Case
1.
Note the typical large and frequently indistinct
Fig.
2.-Case
2. Section multinucleated
*Patient
of Dr.
William
illustrates granular T.
Fitts.
cells cell
with coarse, granular cytoplasm, borders. (X250; reduced ‘/4.)
the indistinct cytoplasmic Jr..
with
cell borders, (x250: mass. whose
kind
small
nuclei,
giving the appearance reduced $4.)
permission
this
case
is reported.
of
a
I.
19. Winkelstein Friedman18 20. Rubin (this Rubin (this ii: Rubin (this 23. Rubin (this
18.
17.
16.
1958
1958 1958 1958 1958
and
report) report) report) report)
1957
1956
1956
1956
30 48 45 37
50
22
42
50
39
1.0 1.5 0.5
Unknown 3 years 1 year 6 years
Multiple, 0.5 to 2.0
3
1.5 years
\Vhite
Whit c W’hitr Negro Negro
3.0
1.5
2.5
0 weeks
2 years
ci nlonths
Wide
23
excision
excision excision
F excision
excision
Local Wide Wide Wide
Local
\Vide
then
excision
excision local excision local excision local excision
excision
excision
Enucleation
Local excision, tomy Not stated
Local Local
Local Excision
Local
1.3 2. 8
2.5
deep
Wide excision Not stated
4 years 6 weeks
2.0
3.0 stated
Excision
1!-ide,
Local excision ‘ * Removal ’’ Excision
OF THE
rulvec-
then wide recurrence
TREATMENT
MYOBLASTOXA
I,ocal excision. excision after
2.0 3.0
Not
: 1
4 years 22 vcars
2 years
2 years Not stated
3.5
Negro
White
Segro
Negro
1956
14.
15.
33 14
1954 1956
34
Negro Not stated White
3” 23
1953 1954
Hanson and Beecham Hanson and Beecham Hanson and Beecham Mitchell and Kaplanl~ Chiodi et a.l.17
White Negro
stated 54
1953
61
1951 1950
2 years
Negro
2. 6 4.0 3.5
3 years 13 years Unknown 4.5
0.5
7.5 years
SIZE
DIAMETER INCM.)
TUMOR
OF GRA~WLAR-CELL
Unknown
Negro
White Negro Negro
White
ItACE
CASES
1)l:RATION OF SYHPTOMS
REPCRTED
9. Valentine and Thomas10 10. Watson and Taylor11 11. Sullivan, Malfetano, and Marsh12 12. Reich et al.14 13. Clarke13
23
1951
I
OF ALL
White Not stated Negro
L’5
1950
Not
39 26 72
1941 1946 1949
2. Tamis and Kowlesc 3. Powell5 4. Murphy, Dockerty, and Broderse 5. Kern, Kaufman, and CornbesT 6. Irgang and Ultmanns 7. Wrights 8. Bangle2
32
1937
AGE
S~SIMARY
Seathm&rys
1. von
AUTHOR
-
YEAR REPORTEII
TABLE
I RESULTS
~~~___
stated
recurrence
No No So So
recurrence recurrence recurrence recurrence
recurrence mately one Not. stated
recurrence
No So
stated
recurrence
recurrence recurrence
recurrence recurrencr
reeurrencc
Not
No
No No
No So
No
No recurrence Not stated
Not
No
3 months
11,‘2 years ll/i years
1?6
years
in 3 years in 8 years in 1 year in 6 month?
in approxiyear
in
in ti months
in in
in one yea, in .5 months
in 3 months
in one year
in
Recurrence in 3 months. then reoperation. Not stated for second operation No recurrence in 6 months No recurrence in 1% years Not stated
VULVA
# c E z
,a i= Pb
Volume 77 Number 2
GRANULAR-CELL
collagenous connective tissue. tissue. Accordingly, 3 weeks of residual tumor was found. S years
Fig.
the
is.
3.-Case
3.
The
large,
MYOBLASTOMA
The later The
granular
Fig. 4.-Case 4. Here there tumor shows some increased (X250; reduced x.)
tumor a wide patient
cells (X250:
is more activity,
OF
was found to local excision has remained
set in reduced
fibrous although
a
295
extend to the edge of the excised was carried out. A small amount free of evidence of recurrence for
dense %.)
stroma. this
VULVA
is
flbrous
stroma
are
clearly
Note that the epithelium not as prominent as it
seen.
overlying
sometimes
CASE 3 .-E. a 0.5 cm. mass of the right A., a 45-year-old Negro para iv, had noted labium majus for one year. It was thought to be a sebaceous cyst and excision was performed as an outpatient procedure under local anesthesia. The lesion was found to be a Microscopic examination solid tumor which on cut section grossly resembled a fibroma.
296 showed the overlying skin to be normal. The tumor consisted of typical granular blastoma cells in a fibrous stroma (Fig. 3). Lt extended to the edge of the specimen. patient was then admitted and a wide excision performed under general anesthesia. specimen removed Phowed residual tumor. Thcrc has been no evidence of recurrence one year.
myoThe Thcs in
CASE 4-G. B. was a 37-year-old Negro para i. In 1949 she received x-ray and radium for a squamous-cell epithelioma of the cervix, Stage II (International). In 1952 she note{1 It was nontender. It a tiny lesion described as a “pimple” on the right labium majus. Three other discrete gradually increased over the next, 6 years to the size of a 5 cent piece. One was excised elsewhere under local nodules appeared, and gradually increased in size. anesthesia in 1955. It is not known whether that tissue was submitted to microscopic examination. Another nodule was cbxcised under local HWSThe lesion gradually reappeared. thesia in the outpatient clinic of the Hospital of the University of Pennsylvania. Microscopic examination of the tissue removed showed a typical granular-cell myoblastoma which had been incompletely removed (Fig. 4). The paGent was advised to be admitted to the She procrastinated for 4 months. During hospital for more complete excision of the lesions. this time the nodule which had been excised recurred. When she finally consented to operaThey were firm tion the four lesions were widely excised, including the overlying skin. nodules ranging in size from 2 cm. to 0.5 cm. Each was in thr subcutaneous tissue, and freely movable. All on gross examination rfasembled fibromas. Mic.roscopic examination of the tumors showed islands and strands of large, elongated cells containing granular pinkThe epidermis appeared normal. staining eptoplasm and small oval nuclei within thr dermis. There has been no recurrence in 6 months.
Comment Granular-cell myoblastomas appear to have no part,icular predilection as to age or race. The clinical story is usually that of a slow-growing, firm, nonpainful nodule. When they occur in the vulva they are usually confused with sebaceous cysts, fibromas, hidradenomas, or lesions of Bart,holin’s gland. If the overlying skin ulcerates, they can be misdiagnosed as basal-cell or squamous-cell carcinomas. The tumor itself, since it is not encapsulated, may be incompletely excised. Such a practice might be dangerous, since, although the great majority of these tumors are benign. the; may recur if incompletely removed. Indeed, they have been reported to have metastasized in rare instances.” The tumors are said to be radioresistant,lQ hence wide local excision is the trcntment of choice.‘“, Ii
Summary Four cases of granular-cell myoblastoma of the vulva arc added to the 19 previously reported in the literature. In 3 of the 4 the tumors were diagnosed preopcratively as sebaceous cysts and excised under local anesthesia. In each instance, microscopic examination of the tissue obtained indicated incomplete removal of the tumor. This was confirmed when the patients were reoperated upon and wider excision performed. In one patient, such inadequate excision was followed by local recurrence on two occasions. Thus, while the great, majority of these tumors are benign, wide local excision is important to avoid recurrence. Very rarely, granular-cell myoblastomas have been noted to metastasize.
Volume 77 Number 2
GRANULAR-CELL
MYOBLASTOMA
OF
VULVA
297
References 1. 2. 3. 4. 5. 6. 7. 8. 9. 10. 11. 12. 13. 14. 75. 16. 17. 18. 19.
Abrikossoff, A. : Virchows Arch. path. 260:.215, 1926. Bangle, R., Jr.: Cancer 5: 950, 1952. von Szathmirv. Z.: Ztschr. Geburtsh. u. Gvnak. 114: 371. 1937. Tamis, A. B.,*&d Kowles, J. J.: Abr. J. &ST. & GYNE~. 42: 543, 1941. Powell, E. B.: Arch. Path. 42: 517, 1946. Murphy, G. H., Dockerty, M. B., and Broders, A. C.: Am. J. Path. 25: 1157, 1949. Kern, A. B., Kaufman, J. J., and Combes, F. C.: Arch. Dermat. & Syph. 62: 109, 1950. Irgang, S., and Ultmann, R.: Harlem Hosp. Bull. 4: 31, 1951. Wright, J. L.: New Zealand M. J. 50: 560, 1951. Valentine, F. M.. and Thomas. J. R.: AM. J. OBST. & GYNEC. 65: 213, 1953. Watson, H. B., and Taylor, C: W.: J. Obst. & Gynaec. Brit. Emp. SO: 917, 1953. Sullivan, T. F., Malfetano, J., and Marsh, M. R.: AM. J. OBST. & GYNEC. 67: 687, 1954. Clarke, J. Y.: Personal communication to Hanson and Beecham. Reich, W. J., Doswald, A. M., Williams, P. C., and Nechtow, M. J.: J. A. M. A. 156: 714, 1954. Hanson, 8. M., and Beecham, C. T.: Ax J. OBST. & GYNEC. 71: 190, 1956. Mitchell, J. A., and Kaplan, D.: A>r. J. OBST. & GYNEC. 71: 901, 1956. Chiodi, N. E., Siegel, I. A., Guerin, P. F., and McCaughan, D.: Obst. & Gynec. 9: 472, 1957. Winkelstein, L. B., and Friedman, S.: hhf. J. OBST. & GYNEC. 75: 325, 1958. Surg. Gynec. & Obst. 76: 315, 1943. Horn, R. C., Jr., and Stout, A. P.: