Granular-cell myoblastoma of the vulva

Granular-cell myoblastoma of the vulva

GRANULAR-CELL MYOBLASTOMA OF THE VULVA Review of the Literature and Report of Four Cases ALAN (From the Gynecologic Department Research of Obste...

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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.: