CASE REPORTS
J Oral Maxillofac Surg
46:1090-1096.1088
Rhabdomyosarcoma of the
Maxillary Sinus:
Review of the Literature and Report of a Case
RI.CK B. WUEMAN, DMD,* NECKF. KATSIKERIS, DDS, DR DENT,t AND
JOHN M. SYM%NGTON, BDS, MSc, PHD, FDSRCSS
Introduction
Rhabdomyosarcoma (RMS) is a malignant tumor
of striated muscle that accounts for 8% to 19% of all
soft tissue sarcomas and 35% to 45% of those oc-
curring in the head and neck. The nose and parana-
sal sinuses, in a review of 771 cases, accounted for
8.1%.’ A review of the literature reveals few cases
originating in the maxillary antrum (Table l).*-“j
Most of these cases have been reported within the
larger context of the paranasal sinuses. This article
describes a case of rhabdomyosarcoma of the max-
illary sinus.
Report of a Case
A 21-year-old white man was seen on 25 January 1978
complaining of intermittent pain in the maxillary left pos-
terior region of 2 weeks duration. The upper left first
molar was diagnosed as having acute pulpitis secondary
to caries (Fig 1). A temporary restoration was placed with
plans for subsequent root canal therapy. The patient re-
turned 8 February 1978 with generalized pain in the right
maxilla. A Waters’ view film and periapical radiographs
were taken and the diagnosis of sinusitis was made. The
patient was placed on penicillin V 300 mg, four times a
day for 10 days.
The patient was next seen on 28 March 1978, at which
time he presented with a thin, brown mucous discharge
from the left nostril with occasional blood streaks. Pain
was present with mastication and palpation of the left
maxillary sinus. A draining tistula was in the vestibule
opposite the left first molar. There was no infraorbital
Received from the Department of Oral and Maxillofacial Sur-
gery, Faculty of Dentistry, University of Toronto, Ontario, Can-
ada.
* Formerly, Senior Resident; presently, in Private Practice,
Regina, Saskatchewan, Canada.
t Assistant Professor.
$ Professor and Chairman.
Address correspondence and reprint requests to Dr. Grieman:
Suite 300,255O 15 Avenue, Regina, Saskatchewan, Canada, S4P-
1N8.
0 1988 American Association of Oral and Maxillofacial Sur-
geons
0278-2391/88/4612-0011$3.00/O
1090
paresthesia. A firm, tender left submandibular node 2 cm
in diameter was palpable. A Waters’ view showed a gen-
eralized radiopacity of the left antrum (Fig 2). The patient
was placed on cloxacillin 500 mg four times a day and
referred to the Faculty of Dentistry.
On 7 April 1978 further periapical radiographs were
taken of the left maxillary area and a pulpectomy of the
first molar in the area was performed (Fig 3). The patient
was referred to the Oral Surgery department, where
epiphora and a prominent swelling in the upper left ves-
tibule were noted. An incisional biopsy of the swelling
was performed on 19 April 1978 (Fig 4). A diagnosis of
esthesioneuroblastoma was made and the patient was re-
ferred to a head and neck surgeon.
He was admitted to the hospital on 14 May 1978 for
further investigation and treatment. The medical history
was noncontributory. Clinical examination revealed bulg-
ing of his left cheek. The left nostril was practically oblit-
erated by tumor tissue that deviated his septum to the
right. Intraorahy a firm, reddish bulge 1 cm in diameter
was on the upper gingiva close to the midline. There was
constant epiphora of the left eye but no ophthalmoplegia,
exopthalmos, or impairment of vision. A number of large,
freely mobile nodes were in the neck. The hemoglobin on
admission was 8.8. The electrolytes, urinalysis, and liver
profile were normal. Radiologic studies included parotid
angiogram, brain scan, tomograms, and plain skull films.
There was evidence of a massive tumor in the antrum
extending into the nostril, with destruction of the poste-
rior medial wall of the antrum, with extensive destruction
of the floor and medial wall of the orbit and involvement
of the ethmoid air cells as well. The cribriform plate was
involved without evidence of any further extension into
the cranial cavity.
At this point the patient was tranferred to a local ra-
diotherapy hospital where multiple metastases were de-
tected. He received radiotherapy to multiple body sites
(head, neck, left hip, lower half body) from June to No-
vember 1978. He also received multi-agent chemotherapy
(cyclophosphamide, adriamycin, vincristine, and pred-
nisone) started in July 1978. In August 1978 a biopsy of
the left femur was performed. The tumor showed small,
pleomorphic cells, some of them bizarre in shape. No
differentiation pattern could be seen. Special stains failed
to reveal the presence of neural axons. A diagnosis of
anaplastic metastatic carcinoma was made.
The tumor progressed rapidly despite the radiotherapy
and chemotherapy he received. He developed a number
of complications, among which were spinal cord com-
pression, pancytopenia with leukoerythroblastic anemia,
GRIEMAN, KATSIKERIS, AND SYMINGTON 1091

Table 1.
Site of Initial Signs
Author Age (yr)/Sex Primary Tumor and Symptoms Microscopy

Pastore’ 46/M Maxillary sinus Headache, swelling, pain of upper jaw Pleomorphic
cross-striations
McCuaig3 12/M Maxillary sinus Hyperemia of eye, toothache
Riopelle4 22/M Maxillary sinus and - Alveolar
orbit cross-striations
Horn’ l/M Maxillary sinus - Botryoid
Allen’ 46/F Maxillary sinus and Swelling of nose, epiphora Embryonal
nose cross-striations
Dito’ Three cases Maxillary sinus - -
Taguchia 29/M Maxillary sinus and Nasal discharge and obstruction Pleomorphic, partly
nose alveolar,
cross-striations
Masson’ Seven cases Maxillary sinus - -
Williams’o 21/M Maxillary sinus and Nasal obstruction, toothache, proptosis Alveolar
nose cross-striations
Debezies” 26/M Maxillary sinus and Decreased vision, headache, nasal Alveolar
orbit discharge
Bailey” 30/F Maxillary sinus Blood tinged rhinorrhea, nasal -
obstruction
Donaldson” 12 Maxillary sinus T,No -
3 Maxillary sinus T,No
5 Maxillary sinus T,No -
Fu14 Maxillary sinus, - -
ethmoid, nasal
cavity
Maxillary sinus and - -
nasal cavity
Kanegaonkar” 21/F Maxillary sinus Pain, proptosis, infraorbital numbness, Embryonal and
diplopia alveolar
LaValIe Bundtzen16 46/F Maxillary sinus - -

FIGURE 1. Periapical radio-

graph of the posterior maxilla
showing carious involvement
of the distal aspect of the sec-
ond molar. There is evidence
of periapical involvement of
the mesiobuccal root.

FIGURE 2. Waters’ view of the skull. The left sinus is mostly
obliterated.
and hypercalcemia. He was admitted for the last time 18
days prior to death. His condition steadily deteriorated
and profound dyspnea developed. He died on 30 Novem-
ber 1978.
Autopsy revealed widespread metastases of which
chest, lymph node, and bone metastases were the most
extensive. The lung, pleura, and diaphragmatic me-
tastases were associated with massive pleural effusions.
All bones examined at autopsy (sternum, rib, and verte-
bral column) were involved by metastatic tumor. The left
antrum contained 30 to 40 mL of necrotic debris and pus.
Microscopic recurrent tumor was seen in the inflamed left
antral mucosa. No tumor was seen in the skull, orbits, or
nasopharynx .
The tumor was composed mainly of small undifferen-
tiated malignant cells. In a few areas the tumor cells had
a small amount of eosinophilic cytoplasm resembling em-
bryo& rhabdomyoblasts. In the pancreas the metastatic
tumor cells were elongated and cross striations were
clearly identified in their cytoplasm. The autopsy speci-
men from the sinus revealed large eosinophilic cells with
eccentrically placed nuclei (Fig 5). Immunoperoxidase
staining for neuron specific enolase and S-100 were neg-
ative whereas staining for myosin and desmin was posi-
tive. A final diagnosis of embryo& rhabdomyosarcoma
was made.
Discussion
Rhabdomyosarcoma is uncommon in the parana-
sal sinuses,3 perhaps because no cross-striated
muscle tissue exists in the area. The origin of these
RHABDOMYOSARCOMA OF THE MAXILLARY SINUS
tumors arising in the paranasal sinuses is not clear.
It has been suggested that they probably originate
from misplaced embryonic muscle cells. It has also
been suggested that there is teratomatous growth of
heterologous tissues, mesenchymal derivatives, in
addition to skeletal muscle.17
From histomorphologic considerations, rhabdo-
myosarcoma is the neoplastic analogue of the em-
bryogenic skeletal muscle. In accordance with the
stage of development, embryologically the malig-
nant myoblast may assume several forms18: a small
round mesenchymal cell, a mesenchymal syncy-
tium-like cell, a tubular form, “racquet” shaped
cell, “strap” shaped cell, and “spider” shaped cell.
Cross striations, although helpful in establishing the
diagnosis, are not essential.” The tumor is easily
diagnosed when cells contain cross striations, but
many tumors appear undifferentiated under light
microscopy. In the past, except for those tumors
demonstrating the most obvious features of a myo-
genie tumor, these neoplasms were often confused
with malignant lymphoma, neuroblastoma, retino-
blastoma, hemangioendothelioma, amelanotic mel-
anoma, myosarcoma, tibrosarcoma, or have simply
been referred to as small-cell malignant neoplasms.’
The original histologic criteria for the diagnosis of
RMS were established by Stout.20 Horne and
Enterline’ proposed four subtypes of RMS-
pleomorphic, embryonal, alveolar, and botryoid.
Stout and Lattes21 classified RMS into adult (pleo-
morphic) and juvenile (alveolar, embryonal, and
botryoid) types. The World Health Organization or-
ganizes RMS into predominantly pleomorphic, pre-
dominantly alveolar, predominantly embryonal,
and mixed.22 Of all RMSs occurring in the head and
neck, approximately 80% to 85% are embryonal (in-
cluding the botryoid type), 10% to 15% alveolar,
and 5% pleomorphic.’
Embryonal RMSs are usually found in children
from birth to 10 years of age. One fourth, however
are found in individuals over the age of 20 years.
Spindle shaped cells are arranged in interlacing and
parallel bundles and at times resemble syncytial
masses. Usually the nucleus is centrally located,
containing large masses of clumped chromatin, and
is surrounded by an acidophilic cytoplasm. Occa-
sional “tadpole” cells are seen, and there may be
areas of round cells with highly eosinophilic cyto-
plasm. The stroma is scant, with little collagen. Stri-
ations are difficult to find and not essential to the
diagnosis.23
The botryoid type has been described as differing
from the embryonal type only by gross form and
location. Most patients are between 2 and 5 years of
age at the time of diagnosis.’ The botryoid RMS
most commonly arises from mucous membranes of
a hollow viscus or body cavity. The tumors are pol-
GRIEMAN, KATSIKERIS, AND SYMINGTON 1093

FIGURE 3. Periapical radio-

graph of the left posterior
maxilla almost 2 months after
the initial presentation. The al-
veolar bone is obviously being
invaded by the tumor. Note
the loss of the lamina dura
around the premolars.

ypoid and often so edematous that they appear
myxoid. The presence of a layer of neoplastic
growth made up of short, spindle, embryonal cells
lying parallel to the surface allows for easy recog-
nition. Striations are rarely found and are not es-
sential for diagnosis.23
The pleomorphic RMS, originally described by
Stout,20 is primarily a tumor found in older individ-
uals, usually between 40 and 60 years of age.
“Strap” cells, cells with nuclei arranged in tandem,
and rhabdomyoblasts with longitudinal or vertical
striations are usually found with little difficulty.
Multinuclear syncytial cells with deeply acidophilic
cytoplasm are also seen. 23 The neoplasm may be
confused, microscopically, with malignant fibrous
histiocytoma and pleomorphic liposarcoma.’ The
extremities are by far the most common site, with
just 7% occurring in the head and neck.24
The alveolar variety, originally described by Rio-
pelle and Theriault,4 occurs chiefly in the 15 to 20-
year age group. Microscopically, it is characterized
by nests of cells separated by delicate or coarse

FIGURE 4. Section from the

original biopsy specimen
showing large cells with clear
cytoplasm and large deeply
stained nuclei. (Hematoxylin
and eosin. Original magnifica-
tion, X280.1
1094
fibrous trabeculae, imparting to the tumor a super-
ficial resemblance to an epithelial neoplasm.23 Cen-
trally, the noncohesive cells may float freely within
the alveolar spaces, whereas peripherally they are
attached to the fibrous septae by thin tapering cy-
toplasmic processes without an intervening base-
ment membrane. The nuclei are hyperchromatic
with inconspicuous nucleoli. The cytoplasm is more
abundant than that of a lymphocyte and tends to be
either amphophilic or acidophilic. Histologic differ-
ential diagnosis includes adenocarcinoma, malig-
nant lymphoma, granulocytic sarcoma, melanoma,
synovial sarcoma, and extraskeletal Ewing’s sar-
coma. This variant exhibits the highest proportion
of distant metastases and the lowest occurence of
local progression from the primary site.25
Masson’s trichrome stains the cytoplasm of dif-
ferentiated rhabdomyoblasts deep red whereas
phosphotungstic acid-hemotoxylin (PTAH) stains it
deep blue. The Masson’s stain is useful in scanning
for rhabdomyoblasts and in identifying “ribbon”
and “strap” cells. The PTAH assists in studying
myofibrils and cross striations. Periodic acid Schiff
(PAS), used with or without diastase, is useful in
identifying the glycogen content of cells. This helps
to differentiate RMS from tumors that do not con-
tain glycogen, such as neuroblastomas.26
Electron microscopic examination shows fea-
tures characteristic of developing muscle cells and
aid in the diagnosis of the tumor. Examples of spe-
cific features include thick and thin filaments in a
hexagonal array, an unambiguous Z line and A
bands with H and M bands. The presence of thick
and thin filaments without additional features of the
RHABDOMYOSARCOMA OF THE MAXILLARY SINUS
sarcomere indicate a myogenous origin, either leio-
myosarcoma or RMS. Other ultrastructural features
observed in well documented cases of RMS include
large irregularly shaped nuclei with projections and
invaginations, free ribosomes closely associated
with filaments, rough endoplasmic reticulum that is
often dilated, glycogen, an external lamina, promi-
nent nucleoli, and pleomorphic mitochondria.16
The orbit is the most common site of occurrence
of RMS in the head and neck, accounting for 36% of
all cases, with the nasopharynx accounting for
15.4%, the middle ear-mastoid for 13.8%, and the
nose and paranasal sinuses for 8.1%. Patients with
sinonasal RMS present with nasal obstruction, rhi-
norrhea epistaxis, sinusitis, local pain, otalgia,
headache, and toothache, and in advanced cases
they may have proptosis, visual disturbances, and
cranial nerve deficits. On physical examination, a
nodular or polypoid mass is found that is often con-
fused with an ordinary nasal polyp.’ As in the na-
sopharynx about a quarter of the tumors in this site
are of the botryoid type. l4 Radiologically, the abil-
ity to discriminate soft tissue densities more accu-
rately allows computerized tomography to appreci-
ate better the integrity of the bone margins such as
the thin walls of the sinuses.27
A review of the literature reveals that lesions of
the nose, nasopharynx, and paranasal sinuses are
often considered together. As they are contiguous
structures, tumors may originate in one area and
extend to adjacent locations. This makes precise
statements as to their origin rather difficult. In the
larger reviews, many authors were not specific as to
which of the paranasal sinuses were involved. Of
FIGURE 5. Autopsy speci-
I men from the sinus showing a
necrotic tumor with large eo-
sinophilic cells having eccen-
trically placed nuclei, resem-
bling embryonal rhabdomyo-
blasts. (Hematoxylin and
eosin. Original magnification,
X280.)
GRIEMAN, KATSIKERIS, AND SYMINGTON
the cases that were reported as arising in the max-
illary sinus (Table I), the average age (n = 16) was
23 years (range, 1 to 46 years). The predominant sex
(n = 13) was male (61%). Only three other cases
presented with dental complaints: two with tooth-
ache and one with generalized pain of the maxi:lla.
Definitive histologic diagnosis may be difficult as
is iIlustrated by this case. The antral mass was in-
terpreted as esthesioneuroblastoma (olfactory neu-
roblastoma) by light microscopy. A biopsy of the
left femur was reported as anaplastic carcinoma.
Special stains failed to demonstrate neural axons.
Muscle striations were seen only in autopsy tissue
from splenic metastasis. Feldman26 reported that
the diagnosis of RMS is often discarded because of
the absence of cross striations, the “hallmark” of
RMS. These are helpful in making the diagnosis,
but because they are rare in the juvenile variants
their absence should not cause abandonment of
RMS as a consideration. A good eosin counterstain
must be secured initially, because without this
stain, the acidophilic quality of the cytoplasm of
certain cells will not be apparent and non-striated
rhabdomyoblasts will not be readily distinguish-
able. Lawrence2’ points out that there seems to be
a general lack of awareness of the existence of the
embryonal RMS, the most common form of RMS.
The use of enzyme histochemistry and examination
of ultrastructure are desirable in difficult cases.
Except for the orbit, peripheral RMSs appear to
have a somewhat better prognosis than those found
in the head and neck. This could partly be explained
by the fact that adequate radical surgical resections
can be performed less readily in the head and neck
region, with the exeption of the orbit. The nasal,
paranasal sinus, and nasopharyngeal RMS have a
poorer prognosis because extensive local disease is
usually found at the time of diagnosis making sur-
gical extirpation of the entire tumor difficult or im-
possible, and because these tumors have already
metastasized when treatment is initiated.‘3~‘4
Most sarcomas of the head and neck metastasize
through venous channels usually not involving
lymph nodes, except occasionally by direct exten-
sion. In contrast, RMS frequently metastasizes to
lymph nodes.14 The lung is the most common site of
metastasis at death, followed by the regional lymph
nodes, bone, liver, and brain. Regional node in-
volvement is observed more commonly in patients
with primary lesions in the lower extemities and
with the alveolar subtype.25 Direct meningeal ex-
tension has been observed in 40% of patients when
the primary sites are adjacent to the meninges.29
Following treatment of tumors in the paranasal si-
nuses, distant metastasis is a greater threat to sur-
vival than local recurrence.3o
Prior to modem multimodality therapy, a 5-year
1095
survival of 8% to 21% could be expe.cted.9*18P28 The
last decade has witnessed remarkable advances in
the treatment of RMS. This has come about largely
through the efforts of the Intergroup Rhabdomyo-
sarcoma Study (IRS) that developed a clinicopath-
ologic staging system and established the superior-
ity of triple (surgery, radiotherapy, and chemother-
apy) over single method therapy. 13,21*26*31*32 For
tumors arising in the middle ear, mastoid, nasal cav-
ity, paranasal sinuses, and nasopharynx the 3-year
relapse-free interval is 45% as opposed to 75% in
other areas of t-he head and neck (scalp, neck, pa-
rotid, oral cavity, larynx).‘l
Summary
A case of rhabdomyosarcoma of the maxillary
sinus is reported. A brief review of the origin, his-
tomorphology, classification, clinical presentation,
behavior, prognosis, and treatment is given relative
to occurrence in the maxillary antrum. Misinterpre-
tation of the microscopic findings can delay the his-
tologic diagnosis. An error in tissue diagnosis may
be minimized by awareness of a chance encounter
with this lesion.
References
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New York, Marcel Dekker, 1985, p 787
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3. McCuaig DR: Rhabdomyosarcoma of the maxillary sinus.
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4. Riopele JL, Theriault JP: Sur une forme meconnue de sar-
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J Oral Maxillofac Surg

46:109&1098,1988

Oral Squamous Cell Carcinoma in

lden tical Twins:
Report of a Case
PHILIP B. BHASKAR, DMD,* R. GREGORY SMITH, DDS, MD,t AND
RONALD A. BAUGHMAN, DDS, MSDS

Oral cancer represents 3% of all human neo-
plasms reported each year, with approximately
30,000 new cases detected annually.’ Carcinomas
of the oral cavity comprise approximately 96% of
*Former Chief Resident, Department of Oral and Maxillofacial
Surgery, University of Florida Health Science Center, Jackson-
ville; currently a Fellow in Oral and Maxillofacial Surgery, Uni-
versity of Florida Health Science Center, Gainesville.
tAssistant Professor and Director, Oral and Maxillofacial Sur-
gery Advanced Training Programs, University of Florida Health
Science Center, Jacksonville.
#Professor and Director of Oral Pathology, and American Can-
cer Society Professor of Clinical Oncology, University of Florida
College of Dentistry/University of Florida Health Science Cen-
ter, Gainesville.
Address correspondence and reprint requests to Dr. Smith:
Department of Oral and Maxillofacial Surgery, University Hos-
pital of Jacksonville and University of Florida Health Science
Center Jacksonville, 655 W. Eighth Street, Jacksonville, Florida
32209.
this group and sarcomas comprise the remaining
4%.* Although the etiology of oral cancer is un-
known, many predisposing factors have been sug-
gested, including heredity. Genetic studies of hu-
man cancer have suggested that neoplasms of spe-
cific sites (breast, ovary, skin, stomach, rectum,
and lung) and specific types of neoplasms (leuke-
mia, malignant melanoma, and adenocarcinoma)
occur within families.3” Although no evidence of
familial grouping for oral cancer has been estab-
lished, reports of oral cancer within families are re-
corded in the literature,&’ including one report by
Cade’ of squamous cell carcinoma occurring in
twins. The purpose of this article is to report a sim-
ilar occurrence in twins.
Report of Two Cases

0 1988 American Association of Oral and Maxillofacial Sur-

Case 1
geons A 40-year-old black woman was seen at the University
0278-2391 I881461 2-0012$3.00/0 Hospital of Jacksonville Oral and Maxillofacial Surgery