Recurrent Multifocal Adult
Rhabdomyoma Diagnosed by
Fine-Needle Aspiration Cytology:
Report of a Case and Review of the
Literature
William P. Walker, M.D., a n d Michael J. L a s z e w s k i , M.D.
The fine needle aspiration (FNA) cytology of a recurrent
multifocal extracardiac adult rhabdomyoma is described, and
the literature is reviewed. The patient presented with dysphagia
and bilateral palpable neck masses 21 y r after resection of a
rhabdomyoma of the tongue. The clinical diflerential diagnoses
included ptotic submandibular glands and lymphadenopathy.
The aspiration smears and cytospin preparations contained
large polygonal cells with abundant granular cytoplasm with
indistinct borders and uniform, peripherally located nuclei.
Cross-striations were identifed within the cytoplasm of some
cells on Papanicolaou and rnodijied Wright-Giemsa stains.
This case represents only the fourth description of the cytology
of this entity and the jirst reported case of a recurrence
diagnosed by FNA. The characteristic cytomorphologic fea-
tures enabled a definitive diagnosis to be made 21 y r after the
original resection, sparing a poor-risk patient a debilitating
surgical procedure for a benign, slow-growing neoplasm. Diagn
Cytopathol 1990;6:3 54-3 5 8 .
Key Words: Adult rhabdomyoma; Fine-needle aspiration cytol-
ogy
Adult rhabdomyomas are rare, benign tumors of skeletal
muscle that are found predominantly in the head and
neck region, an area frequently biopsied by fine-needle
aspiration (FNA). The tumors mainly occur in men in the
sixth decade of life and are usually singly occurring;
however, 10 cases of multifocal tumors have been
described.’-6 Only recently has the cytomorphology of
adult rhabdomyomas been described with several features
felt to be distinctive of this entity.’-’ This case emphasizes
the characteristic cytomorphology of this rare neoplasm
and the usefulness of FNA in establishing a definitive
Received June 13, 1989. Accepted August 21, 1989.
From the Department of Pathology, University of Iowa, Iowa City,
IA.
Address correspondence to William P. Walker, M.D., Department of
Pathology, 110 MRC, University of Iowa Hospitals and Clinics, Iowa
City, IA 52242.
354 Diagnostic Cytopathology, Vol6, No 5
diagnosis. These benign, slow-growing tumors often occur
in areas adjacent to vital oropharyngeal structures, where
local excision may require a debilitating surgical proce-
dure. In the present case, a definitive diagnosis by FNA
spared a poor-risk patient further surgical intervention.
W e report the cytologic, histologic, immunohistochemi-
cal, and ultrastructural features of a multifocal adult
extracardiac rhabdomyoma with a recurrence diagnosed
by FNA 21 yr after the original excision.
Report of a Case
A 76-yr-old white male presented to the otolaryngology
clinic with complaints of difficulty swallowing and a
stricture-like feeling in his neck. He had noted bilateral
lumps in his neck for several years that were occasionally
painful. The patient had a 90 pack-yr history of smoking
and a history of ethanol abuse. His past medical history
was remarkable for excision of a rhabdomyoma of the
tongue 21 yr earlier; however, since that time he had been
well. On physical examination, the patient’s tongue devi-
ated to the right, and bilateral neck masses, 2.0 x 2.0 cm
on the right and 1.5 x 1.O cm on the left, were palpable.
Clinically, these masses were felt to be ptotic submandib-
ular glands or jugulodigastric lymph nodes. Endoscopic
examination of the nasopharynx, hypopharynx, and lar-
ynx was unremarkable. A magnetic resonance imaging
(MRI) scan revealed soft-tissue masses involving the left
parapharyngeal space, left lateral oropharyngeal region,
and the right side of the neck. The left lateral oropharyn-
geal mass extended to the base of the tongue. The
right-sided mass measured 4.0 x 2.5 cm in diameter and
was immediately adjacent to the right piriform sinus.
A percutaneous FNA biopsy of the right-sided mass
was performed using a 22-gauge needle. Smears were
air-dried and stained with a modified Wright-Giemsa
stain and also fixed in 95% ethanol for Papanicolaou
0 1990 WILEY-LISS, INC.
 MULTIFOCAL ADULT RHABDOMYOMA

staining. Cytospin preparations made from the material

obtained by rinsing the needle and syringe were similarly
stained after either air-drying or ethanol fixation. The
aspiration smears were sparsely cellular with a relatively
clean background, devoid of cellular or necrotic debris.
Lymphoid and salivary gland tissue was not present. The
smears were dominated by scattered, large, polygonal to
round cells with abundant, eosinophilic, granular cyto-
plasm. The cells occurred singly and in small clusters,
varying in size from 30 to 120 pm in greatest diameter.
The cytoplasmic borders were indistinct, and the cells
contained from one to five uniform nuclei with finely
granular, evenly distributed chromatin and a central
nucleolus (Fig. 1). The nuclei in many cells were located
at the periphery of the cytoplasm (Figs. 2 and 3). A few
scattered cells contained cross-striations in the cytoplasm
that were identified on both the modified Wright-Giemsa
and Papanicolaou stains and accentuated by a phospho-
tungstic acid-hematoxylin (PTAH) stain. Mitoses were
not identified. A diagnosis of recurrent multifocal adult
rhabdomyoma was made based on the characteristic
cytomorphology.
The partial glossectomy specimen from 21 yr earlier
was reviewed. The specimen consisted of a lobulated,
encapsulated mass of firm, pale, red-brown tissue without
hemorrhage or necrosis. The tumor fragments measured
2.5 x 1.5 x 1.0 cm; 3.0 x 1.5 x 1.5 cm; and 4.5 x 3.0 x
2.0 cm. Microscopically, the tumor consisted of closely Fig. 2. Alcohol-fixed cytospin preparation showing a cellular cluster
with cytoplasmic granularity and peripherally located nuclei. The nuclei
packed polygonal cells varying in size from 20 to 170 pm. are round to oval, each containing a distinct nucleolus (Papanicolaou,
Delicate fibrovascular septae containing scattered lympho- x 1,000).
cytes and neutrophils divided the tumor into lobules. The
cells had abundant, granular, eosinophilic cytoplasm with round nuclei were present either at the periphery of the
prominent peripheral vacuolization. Multiple oval to cells or clustered centrally (Fig. 4). The nuclei contained
single, oval, basophilic nucleoli. No mitoses were present.
Rare cells showed round, 2-4-pm eosinophilic intranu-
clear inclusions which were periodic acid-Schiff (PAS)-
positive and diastase-resistant. Crystalline, eosinophilic,
intracytoplasmic inclusions were present. Cross-striations
were occasionally seen in the cytoplasm and were visual-
ized with hematoxylin-eosin (H&E), trichrome, and
PTAH stains (Fig. 5). The cytoplasm demonstrated
strong PAS staining, which was removed by diastase
digestion.
Immunoperoxidase studies performed retrospectively
on formalin-fixed, paraffin-embedded tissue showed dif-
fuse, strong cytoplasmic positivity for vimentin, desmin,
muscle-specific actin (MSA), and myoglobin. The tumor
cells were negative for keratin, S-100, and chromogranin.
Electron microscopic studies showed the cells to have
numerous, variably sized mitochondria, often with irregu-
lar granular inclusions in the intracristal spaces. Abun-
dant glycogen was present. Thin filaments and rod-like
Fig. 1. Alcohol-fixed cytospin preparation showing a cellular cluster
with indistinct cytoplasmic borders and multinucleation (Papanicolaou, Z-band material with a tetragonal arrangement were also
x 400). present. The nuclei contained distinctive intranuclear

Diagnostic Cytopathology, Vol6, No 5 355

WALKER AND LASZEWSKI

plete resection may become evident after many years. No

Fig. 3. Air-dried cytospin preparation showing a cellular clustrx within
a bloody background. The cells have a low nuclear/cytoplasrnic ratio;
bland, regular nuclear chromatin; and granular cytoplasm (modified
Wright-Giemsa, x 400).
aggressive or metastasizing adult rhabdomyomas have
been described. Various pathogenetic mechanisms have
been advanced for these tumors, including a neoplastic
proce~s,'.'~ hamartomatous g r ~ w t hmaturation
,~ arrest or
defect, failure of orderly myofibril organization, and
tra~ma.~
Adult rhabdomyomas frequently are encapsulated or
well-circumscribed and are firm, lobulated, and homoge-
nously tan-gray. The tumor cells vary greatly in size and
contain abundant, granular, eosinophilic cytoplasm cen-
trally. Multiple peripheral vacuoles are present. The cells
contain multiple, oval, vesicular nuclei either peripherally
located or clustered centrally within the cytoplasm. Sin-
gle, prominent, basophilic nucleoli are often present.
Mitotic figures are not present. Intracytoplasmic inclu-
sions' and intranuclear inclusions have been described.
Occasional cells have prominent cross-striations, which
are best visualized with the PTAH stain. The cytoplasm
contains abundant PAS-positive material. The FNA
smears and cytospin preparations typically contain scat-
tered neoplastic cells, as previously described. The back-
ground may be bloody; however, necrosis and cellular or
proteinaceous debris are not identified. hIWIohistochem-
ical studies have shown myoglobin positivity in the tumor

inclusions with a granular core surrounded by a thick

filamentous cortex.
Discussion
Adult rhabdomyomas are rare, benign, slow-growing
neoplasms. Approximately 80 cases of adult rhabdomy-
oma, 10 of which were multicentric/multifocal,'d have
been described in the world's literature since the first case
report by Pendl in 1897." Over 90% of adult rhabdomyo-
mas have been described in the head and neck region, with
the most common locations being the pharyn~,*.~."-'~
l a r y n ~ , ~t'~~n. g' ~u e , ' ' - ' ~submandibular
*'~ region,'.*^'' floor
of m o ~ t h , ~ - ' ,and ~ ~ ,n' e~ ~ k . ~Other ' ~ common sites of
. ~ ~ less
occurrence include the sublingual region,*' orbit," buccal
m u ~ o s a , 'lip,19
~ soft palate,I5 mediastinum," stomach,"
and finger.lg No association exists between skeletal mus-
cle rhabdomyomas and cardiac rhabdomyomas occurring
in patients with tuberous sclerosis or other phakomatoses."
The male to female incidence ratio has been reported to
be 2: 1 to 4: 1 . l 5 , l 9 The mean age of patients at the time of
diagnosis is 52 yrI9; affected patients have ranged in age
from 5 wk to 82 yr.9,'3,20Symptomatology depend:s on the
location and size of the tumor, with size ranges of several
millimeters up to 13 cm having been described in the
literature.' Because of the slow growth rate of these
Fig. 4. Histologic section from the original neoplasm excised 21 yr
tumors, symptomatology may have been present for earlier. Note the similarity of the cytologic features to those of the
decades in some patient^,^ and recurrences after incom- material obtained by FNA on the recurrence (H&E, x250).

356 Diagnostic Cytopathology,Vo16, No 5

 MULTIFOCAL ADULT RHABDOMYOMA

Fig. 5. Histologic section from the original neoplasm demonstrating
disorganized cross-striations (PTAH, x 400).
~ e 1 l s . lElectron
~ microscopic studies have demonstrated
cells with intact basement membranes, which contain
myofibrils arranged into sarcomeres in various stages of
organization as well as clusters of irregular rods in the
cytoplasm resembling aberrant Z-line material.’”’~’6*’7
Intramit~chondrial~ and intranuclear inciusionsl6 may be
present, and glycogen and mitochondria frequently are
abundant.16
The differential diagnosis of adult rhabdomyomas in-
cludes paraganglioma, granular-cell tumor, oncocytoma,
acinic-cell carcinoma, rhabdomyosarcoma, fetal rhabdo-
myoma, hhbernoma, and normal skeletal muscle. Table I
illustrates the important cytomorphologic differentiating
features of each of these entities.
This articles describes only the fourth report of a
fine-needle aspiration cytologic diagnosis of an adult
rhabdomyoma and the first cytologic diagnosis of a
recurrent, multifocal, adult rhabdomyoma. The patient
was reluctant to undergo a second surgical procedure.
Based on the size, location, multifocal nature of the lesion,
the patient’s age and general poor health, and the benign
natural history of rhabdomyomas, it was decided that he
would be observed and followed with interval MRI scans.
The patient currently is alive and well 24 yr after his
original resection and 3 yr after the diagnosis of his
recurrence by FNA, with minimal change in the size of

Table I. Cvtologic Differential Diagnosis of Adult Rhabdomvoma

~~

Cross-
Aspirate Smear Nuclei Cytoplasm striations PAS Mitoses Other
Adult rhabdomyoma Variably sized cells Multiple, round to Eosinophilic; granular; Variable + - Occasional intranu-
(up to 120 pm); sin- oval, uniform chro- frequent vacuoliza- clear or intracyto-
gle cells or small matin; peripheral or tion plasmic crystals
clusters central
Paraganglioma Small groups of vari- Central, pleomorphic Eosinophilic; granular; - - Infrequent
ably pleomorphic variable vacuoles
polyhedral cells;
may be bloody
Granular-cell tumor Uniform, small, round Oval to round; central Abundant; eosino- - + - Histologic overlying
to polygonal cells or eccentric philic; granular; no pseudoepithelioma-
vacuoles tous hyperplasia
Oncoytoma/acinic- Highly cellular Central to eccentric; Abundant; eosinophilic - + Infrequent
cell carcinoma smears, often with variable pleomor- or clear; no vacuoles
naked nuclei phism; variable nu-
cleoli
Rhabdomyosarcoma Polymorphic cells, Pleomorphic; atypical Granular; eosinophilic; Variable + +
poorly differenti- no vacuoles
ated, round to spin-
dled
Fetal rhabdomyoma Admixture of undiffer- * No vacuoles; * * * *
entiated mesen-
chyme, myxoid
stroma, and skeletal
muscle cells
Hibernoma Uniform large cells Single, central Abundant, vacuolated - - - Lipid-rich
Normal skeletal Paucicellular, large- Uniform; multiple; Eosinophilic; granular; +t + -

muscle cell clusters peripheral no vacuoles

Key: -, negative; +, positive; *,depends on stage of skeletal muscle cell maturation; ?regular spacing; prominent.

Diagnostic Cytopathology, Vol6, No 5 357

WALKER AND LASZEWSKI
the lesion or severity of his symptoms. This case empha-
sizes the utility of FNA in making a definitive diagnosis of
adult rhabdomyoma.
Acknowledgment
T h e authors thank Ms. Linda Schneekloth for typing the
manuscript, Ms. Chris Bromley for performing t h e immunohis-
tochemical studies, a n d Mr. Joel Carl for his photographic
assistance.
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