Electron Microscopy Of Rhabdomyosarcoma Of The Orbit A Study of Two Cases

15 Pages·2012·1.83 MB·English

December 1963 Volume 2, Number 6
INVESTIGATIVE OPHTHALMOLOGY
Electron microscopy of rhabdomyosarcoma
of the orbit
A study of two cases
Arnold ]. Kroll, Toichiro Kuwabara, and George M. Howard
A
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;habdomyosarcoma is believed to be Examination of the right eye demonstrated
the most common primary malignant or- proptosis of approximately 4 mm. with displace-
ment of the globe inferiorly and temporally. Limi-
bital tumor of childhood. A recent review
tation of voluntary motion and diplopia were
of 55 cases disclosed three histologic types: greatest on attempted gaze upward and inward.
embryonal (73 per cent), differentiated A firm, oval, nontender mass approximately 2 cm.
(11 per cent), and alveolar (16 per cent).1 in diameter was palpable in the superonasal orbit.
The fine structure characteristics of or- The vision was 20/25+ and the cornea, anterior
chamber, iris, and fundus were unremarkable.
bital rhabdomyosarcoma have not yet been
Confrontation field was full. On examination, the
reported. left eye was entirely normal.
There was no significant past medical history
Case reports, materials and methods and no family history of orbital tumor. The pa-
Case 1. K. L., a 7-year-old white boy, com- tient had six normal siblings. The general physical
plained of diplopia and was noted to have progres- examination was unremarkable, and x-ray studies
sive protrusion of the right eye of 6 weeks' dura- of the orbits, nasal sinuses, chest, and long bones
tion. were negative.
Biopsy of the orbital mass demonstrated a
malignancy and the orbit was exenterated. At
operation the mass was grayish white, firm, smooth
From the Howe Laboratory of Ophthalmology, in some areas, tabulated in others, and measured
Harvard University Medical School, Massachu- 2 by 2 cm. It was firmly attached to the supero-
setts Eye and Ear Infirmary, 243 Charles St., nasal aspect of the superior rectus muscle, but did
Boston, Mass. not invade the levator palpebrae or orbital bone.
This investigation was supported by Training Case 2. M. B., a 5%-year-old white girl, was
Grant No. 2B-5142 and Research Grant No. admitted to the hospital with proptosis of the
NB02698-04 from the National Institute of right eye of 6 weeks' duration.
Neurological Diseases and Blindness, United Eleven months prior to admission, a bimedial
States Public Health Service. rectus muscle recession was performed for eso-
523
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521 Kroll, Kuwalxira, and Howard
Drnwbrr lU(i3
Fij;. 1. Case 1. Low-power light micrograph of differentiated rhabdomyosarcoma. Large cells
with abundant cytoplasm are scattered among smaller spindle-shaped cells. (Hematowlin and
rosin. Ol>j. magnification • 16, ocular magnification -10.)
Fig. 2. Case 1. High-power (oil immersion) light micrograph ol a dillerentiated tumor cell
showing cross striations. (Wildcr's reticuluin stain. Ol)j. magnification -100, ocular magnifica-
tion >10.)
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Volume 2 Rhabdomyosarcoma of orbit 525
Number (i
tropia. Six weeks prior to admission, prominence of Sections were cut with a Servall ultramicrotome
tlie right upper lid was noted and an orbital mass with glass or diamond knives, mounted on bare
was palpated superonasally. The mass rapidly en- copper grids, stained with uranyl acetate, and
larged, and \xk weeks prior to admission a biopsy counterstained with lead at high pH.' The speci-
of the mass demonstrated a malignancy. mens were viewed in a JEM—5Y electron micro-
Two siblings aged 13 and 9 were living and scope. The unused portion of each tumor was
well. A maternal grandfather died of "cancer of fixed in formalin for light microscopy.
the kidney."
Examination of the right eye revealed a 3 mm. Light microscopy
displacement of the globe temporally and in-
Case 1. There was an irregular arrange-
feriorly. A well-localized soft 2 cm. mass was
palpated superiorly and nasally. Examination of ment of large cells with abundant eosino-
the left eye was within normal limits. X-ray studies philic cytoplasm scattered among smaller
of the right orbit revealed a soft tissue density spindle-shaped cells (Fig. 1). A moderate
but no invasion of bone. The hemoglobin, white
number of the larger cells demonstrated
blood cell count, and urinalvsis were within nor-
cross striations with Wilder's stain (Fig.
mal limits.
2). The diagnosis was rhabdomyosarcoma,
The orbit was exenteratcd and the tumor mass
was found to measure 2 by IVi cm. It was soft differentiated type.
and yellowish tan and was infiltrating into the Case 2. There was a loose arrangement
orbital soft tissue.
of stellate and spindle cells with large non-
Immediately after exenteration, each specimen
nucleolated nuclei and scanty, poorly stain-
was opened and 2 mm. square pieces of tumor
ing cytoplasm (Fig. 3). In the tumor
wen; quickly excised from areas remote from
extraocular muscle. Fixation was in cold (4° C.) periphery were seen several large, bizarre,
buffered 2 per cent osmium tetroxide (containing multinucleated tumor cells. No cells were
45 mg. per milliliter of sucrose and 0.002 per found to contain cross striations or longitu-
cent calcium chloride) for 2 hours. The tumor
dinal myofibrils. The diagnosis was rhabdo-
fragments were then dehydrated in a graded
myosarcoma, embryonal type.
series of ethanol, and embedded in Epon 812.-
Fig. 3. Uase 2. Low-power light micrograph of embryonal rhabdomyosarcoma. Stellate and
spindle cells are loosely arranged and have scanty cytoplasm. (Hematoxylin and rosin. Obj.
magnification *6.3, ocular magnification xl().)
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Inri'stinatwr Ophthalmology
526 Kroll, Kuwabara, and Howard
December 1963
4
Fig. 4. For legi-nd sec opposite papo
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Volume 2 Rhabdomijosarcoma of orbit 527
Number 6
Fig. 5. Case I. High-power electron micrograph of an area of cytoplasm of a less well-
dilfcrentiated tumor cell. Actomvosin filaments (f), on close inspection are noted to lie of two
types: "thick," 150 A wide, and "thin, ' 60 A wide filaments. RNP particles (r) are seen in
very close relationship to some filaments, giving the appearance of filament synthesis, HI.
mitochondrion. (*21,000, one micron mark.)
Fig. 4. Case; 1. Low-power electron micrograph of a less well-dillerentiated tumor cell. Note
the large cell size and cytoplasmie aggregates of actomyosin filaments (/), among which are
collections of HNP particles (r). The filaments are cut in longitudinal, oblique, and cross
section and do not have cross striations. Mitochondria (m) are plentiful. Extensive deposits
of glycogen (g) are present. The nucleus (n) is indented by large cytoplasmie processes (p)
which are seen in both longitudinal and cross section.
In the extracellular space are many collagen fibrils (c), alongside of which is a thin portion
of a stromal cell (v). (* 11,400, one micron mark.)
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528 Kroll, Kuwabara, and Howard Investigative Ophthalmology
Deeember 1963
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7.
Fig. 6. Case 1. Low-power electron micrograph of a well-differentiated tumor cell. The
aggregates of actomyosin filaments are relatively well ordered, and pathognomonic banding is
seen. The dark Z hands arc easily visible. Mitochondria (m) are plentiful and the nucleus
(n) contains a large dense nucleolus. ("11,700, one micron mark.)
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Volume 2 Rhabdomtjosarcoma of orbit 529
Number H
Fig. 7. Case 1. High-power electron micrograph of an area of a well-differentiated tumor
ei'll. The fine structure is similar to that in embryonal skeletal muscle. A, A band; '/,, L band:
/, 1 band; //, II band; M, M band; r, RNP particles; HI, mitochondrion. ( • 27,900, one micron
mark.)
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530 Kroll, Kuwabara, and Howard Investigative Ophthalmology
December 1963
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Fig. 8. CiiSd 1. Stromul cell of the tumor. Although fine filaments are visible within the
cytoplasm, these do not have the configuration or dimensions of actomvasin.
UNP particles (r) are both free and bound to membranes. In the latter ease they are said
to be part of the "rough-surfaced" endoplasmic retieulum (er). Mitochondria (m), vesicles
(i>). glycogen bodies (g), and lvsosomes (I) are seen.
Cytoplasmic processes (;)) project from the cell surface. Collagen fibers (c) are seen in the
extracellular space. ( • 15,0()(), one micron mark.)
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Voluntr 2 Rhabdomijosarcoma of orbit 531
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Fig. 9. Case 2. Low-power electron micrograph of embryonal rhalxlomvosarcoma. The plane
of section includes two portions of the nucleus in). Filaments within the cytoplasm do not
have the configuration of actomyosin and must lie regarded as nonspecific. A large vesicle
tv) is seen at the top of the micrograph, in, mitochondria; {,'<', Colgi complex. (• 14,500, one
micron mark.)
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532 Kroll, Kuwahani, and Howard Imc.stifiativc Ophthalmology
December 1963
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Fig. 10. Case 2. Portion ot a tumor coll. Clusters of RNP particles (r) and nonspecific filaments
are. scattered within the cytoplasm. Large mitochondria (m) are cut in longitudinal and
transverse section. Fluid-filled vesicles (v) have a rough, participate border. (x22,6(X), one
micron mark. )
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