Otolaryngology–Head and Neck Surgery (2005) 133, 42-50

Rhabdomyosarcomas of the Nose and Paranasal

Sinuses: Treatment Results in 15 Cases
Jochen Wurm, MD,a Jannis Constantinidis, MD, PhD,a
Gerhard G. Grabenbauer, MD, PhD,b Heinrich Iro, MD, PhDa

a
From the Department of Otorhinolaryngology, Head and Neck Surgery and
b
Department of Radiotherapy, University of Erlangen–Nuremberg.

HISTOLOGY AND EPIDEMIOLOGY

OBJECTIVE: Primary rhabdomyosarcomas (RMS) of the nose
and the paranasal sinuses occur very rarely. Treatment of these
tumors usually is conducted according to standardized therapy
protocols like the German Cooperative Soft Tissue Sarcoma Study
(CWS) or the Intergroup Rhabdomyosarcoma Study (IRS). The
role of surgery still remains controversial.
STUDY DESIGN AND SETTING: A retrospective analysis of
15 patients with an RMS of the nose or paranasal sinuses treated
between 1979 and 2000 is presented. Patients’ age ranged from 2
to 60 years, with an average of 22 years. Histologic subtypes
encompassed 9 embryonal (e) and 6 alveolar (a) RMS. Resection
of the tumor with subsequent radiochemotherapy (RCT) was per-
formed in 6 cases, and primary RCT, in 9 cases. The average
duration of follow-up was 4 years and 10 months.
RESULTS: Overall 5-year survival was 40%. In the patient
group subjected to tumor resection with subsequent RCT, 5-year
survival was 66%, compared with 33% after exclusive primary
RCT. With respect to histologic subtype, 5-year survival was 55%
for eRMS, as compared with 33% for aRMS. Moreover, infiltra-
tion of the skull base and the presence of a residual tumor after
primary therapy constituted factors associated with an unfavorable
clinical course.
CONCLUSIONS: Surgical removal of tumor with subsequent
radiochemotherapy can be recommended if a complete resection
and functionally and cosmetically satisfactory results appear pos-
sible. Patients with eRMS showed an overall more favorable clin-
ical course than patients with aRMS.
© 2005 American Academy of Otolaryngology–Head and Neck
Surgery Foundation, Inc. All rights reserved.
Rhabdomyosarcomas (RMS) are highly malignant tumors
that originate from the embryonal mesenchyma and have
the potential of differentiating to striated muscle tissue.
They can occur in nearly all parts of the body, exhibit
locally invasive growth, and frequently disseminate at an
early clinical stage. Histologically, four different subtypes
can be distinguished: embryonal, alveolar, pleomorphic,
and mixed-type RMS. Soft-tissue sarcomas account for
about 4%-8% of all malignant disorders in the age group
younger than 15 years. About 50% of these belong to the
RMS category. Incidence is 4.4 per 1 million children in the
white population. About 70% of new cases appear in chil-
dren below the age of 10 years, with a peak between the age
of 2 and 5 years.1 One-third of RMS cases in children
originate from the head and neck region. In contrast, RMS
in adults account for only about 10% of all soft tissue
sarcomas, and ⬍1% occur in the head and neck.2
STAGING
In the head and neck area, a distinction is drawn between
parameningeal RMS and RMS of other localizations. Para-
meningeal RMS include sarcomas of the nasopharynx, the
nasal cavity, paranasal sinuses, middle ear, and the mastoid
region as well as those from the infratemporal fossa and the
pterygopalatine fossa. Tumors that invade only the orbit are

Reprint requests: Jochen Wurm, MD, Department of Otorhinolaryn-

gology, Head and Neck Surgery, University of Erlangen-Nuremberg,
Waldstra␤e 1, 91054 Erlangen, Germany.

0194-5998/$30.00 © 2005 American Academy of Otolaryngology–Head and Neck Surgery Foundation, Inc. All rights reserved.
doi:10.1016/j.otohns.2005.03.023
Wurm et al Rhabdomyosarcomas of the Nose and Paranasal . . . 43

Table 1
Patients’ characteristics

Number, age (y) at ID TNM staging Localization Metastases Histologic type

1, 2 III NC, PNS, OB, SB eRMS

2, 8 II NC, NP eRMS
3, 5 III PNS, NP, SB eRMS
4, 14 II PNS, SB aRMS
5, 7 II NC, MS eRMS
6, 17 III NC, PNS, OB, SB LN aRMS
7, 40 III NC LN aRMS
8, 51 II NC eRMS
9, 6 III NP, SB eRMS
10, 3 III NC, PNS, SB eRMS
11, 55 II MS, ETH eRMS
12, 60 II NC eRMS
13, 18 II MS aRMS
14, 17 III NC, PNS, OB, SB LN aRMS
15, 34 II ETH aRMS
NC, nasal cavity; PNS, paranasal sinuses; NP, nasopharynx; OB, orbit; SB, skull base; ETH, ethmoid; MS, maxillary sinus; LN,
lymph node; eRMS, embryonal rhabdomyosarcoma; aRMS, alveolar rhabdomyosarcoma; ID, initial diagnosis

considered separately because of their good prognosis.3-5
The most common staging systems are the TNM-UICC
system and the system of the Intergroup Rhabdomyosar-
coma Study (IRS). The TNM-classification is a pretreat-
ment staging system. “T” describes the size of the tumor and
is divided into the subcategories T1 and T2. T1 means the
tumor is confined to the anatomic site of origin; T2 means
extended tumor growth. The suffixes a and b specify tumor
size ⱕ5 cm in diameter and ⬎5 cm in diameter, respec-
tively. “N” and “M” refer to regional lymph nodes and
distant metastases, whereas the digits 0 and 1 characterize
no clinical involvement or present metastases. Taking T, N,
and M category into account, the tumor is then grouped into
stages I to IV. Parameningeal RMS are automatically as-
signed to at least stage II of the TNM-system, independent
of their size. As a postoperative staging system, the clinical
groupings used by the IRS also include—apart from tumor
size, lymph node status and distant metastases—resectabil-
ity of the tumor. Expressed in very simple terms, the as-
signment to clinical groups is based on whether complete
resection was performed, tumor tissue had to be retained, or
only a biopsy for histologic identification was possible.
THERAPY PROTOCOLS
Since 1981, RMS have been treated in Germany in accor-
dance with the TNM-UICC classification and the histologic
subtype, as defined by the guidelines of the Cooperative
Soft Tissue Sarcoma Studies (CWS). In the years to follow,
a number of study protocols were established and modified
(CWS 81-96). These studies comprise treatment regimes for
primary or adjuvant polychemotherapy, if necessary in
combination with radiotherapy. Parameningeal RMS belong
to the high-risk group within the CWS.4,6
Overall, soft-tissue sarcomas rank among the more fre-
quent malignancies, especially in childhood, whereas pri-
mary RMS of the nose and paranasal sinuses are very rare.
Therefore, the observation periods of the few existing
retrospective studies in which the treatment results for these
tumors in particular were investigated extend over several
decades. But still, the number of patients remains low.2,7-9
Studies by the CWS or the IRS do contain larger case
figures, yet they present the treatment results of all para-
meningeal RMS put together, without further differentiation
of specific tumor localization.3,4,6,10 In the literature, how-
ever, individual case reports are more frequent.11-13 The
present work reports on our experience in the treatment of
RMS of the nose and paranasal sinuses in 15 patients.
Results of treatment, prognostic factors, and the biological
behavior of these tumors shall be elucidated.
MATERIAL AND METHODS
The medical reports of 15 patients treated for RMS of the
nose or paranasal sinuses between 1979 and 2000 were
retrospectively reviewed. At the time of initial diagnosis
(ID), patients’ age ranged from 2 to 60 years, with an
average of 22 years. Seven patients were younger than 15
years. Seven patients were male and 8 female. Histologic
subtypes included 9 embryonal (e) and 6 alveolar (a) RMS.
Staging was performed by means of computertomography
of the thorax, magnetic resonance imaging of the brain and
abdomen, bone marrow biopsies at 2 different sites, and
bone scintigraphy. According to the current TNM classifi-
cation, 8 patients belonged to stage II and 7 patients to stage
III (Table 1). Pretherapeutic imaging methods had estab-
lished extended RMS of the nose and paranasal sinuses with
infiltration of the skull base in 7 cases (Figs 1A and B). In
44 Otolaryngology–Head and Neck Surgery, Vol 133, No 1, July 2005
Figure 1 Rhabdomyosarcoma of the nose and paranasal sinuses with infiltration of the anterior skull base (A and B).
8 cases, the tumor remained locally confined to the nasal
cavity, the paranasal sinuses, and the nasopharynx. Re-
gional lymph node metastases were present in 3 patients at
initial diagnosis, whereas no distant metastases could be
identified in any of the other patients.
After histologic verification, primary radiochemotherapy
(RCT) was given to 9 patients according to the CWS pro-
tocols 91 and 96, with administration of 2 cycles of adria-
mycin–ifosfamide, or according to the VACA regime (vin-
cristine, actinomycin D, cyclophosphamide, adriamycin). In
the remaining 6 patients, tumor resection was carried out via
a combined endonasal–transfacial approach, with histolog-
ically clear margins in 4 patients and with histologically
residual tumor in 2 patients. Consistent with the CWS
protocols, primary surgery was performed if complete tu-
mor removal based on preoperative image data seemed most
likely and required reconstructions could be carried out with
reasonable morbidity. Surgery should not result in mutila-
tion of the patient. RCT according to the CWS-91, with 2
cycles of adriamycin–ifosfamide, or according to the SIOP
protocol (International Society of Pediatric Oncology), was
conducted as an adjuvant procedure here as well. Overall,
the doses applied during radiotherapy ranged from 43 to 66
Gy.
During follow-up, the primary tumor site was evaluated
by CT with contrast medium. Screening for distant metas-
tases was carried out by chest radiograph and bone scintig-
raphy. The duration of follow-up ranged from 7 months to
22 years, with an average of 4 years and 10 months (Tables
1 and 2).
Survival data of the patients, differentiating between the
life status at the end of follow-up (dead or censored), were
analyzed by using the Kaplan-Meier method. The Kaplan-
Meier estimates of the survival curves are visualized in
graphical displays showing the censored survival experi-
ence of patients by ticks in the curves. Because of the small
sample size of the study, the exact log rank test was used to
test for homogeneity of the 2 survival curves, and Fisher’s
exact test for evaluating differences in proportions.
Informed consent was obtained by each patient or pa-
tient’s relatives, respectively.
RESULTS
Overall Survival
The treatment results and clinical course of all patients are
summarized in Table 2. Overall 5-year survival was 40%,
and tumor-specific 5-year survival reached 46%. One pa-
tient died 6 years after initial diagnosis, not related to the
initial tumor.
In 11 cases, complete remission was initially achieved
after primary therapy. Of these, 5 patients developed a
local or regional recurrence or distant metastases be-
tween 5 and 14 months after primary therapy. All 5
patients died between 9 and 33 months after ID, despite
renewed surgical and radiation treatment. Residual tumor
was identified in 4 of the 15 patients after primary ther-
apy. Therapy was discontinued in 3 cases because of the
size of the residual tumor. These patients died between 7
and 11 months after ID. In 1 of the 4 cases, the residual
Wurm et al Rhabdomyosarcomas of the Nose and Paranasal . . . 45

Table 2
Therapy regimes and outcome

Primary therapy

Chemotherapy/ Outcome
No. Surgery radiotherapy (Gy) Result Clinical course Additional therapy (cause of death)

1 Biopsy P-CWS-96, RT Complete Local None Died 25 mo after ID

(49) remission recurrence (local recurrence)
2 Resection Post-CWS-91, RT Complete Disease-free Alive, 10 y disease-free
R0 (48) remission
3 Biopsy P-CWS-96, RT Residual Residual tumor None Died 11 mo after ID
(43) tumor (complications)
4 Biopsy P-CWS-96, RT Residual Residual tumor, None Died 8 mo after ID
(50) tumor meningiosis (meningiosis
sarcomatosa sarcomatosa)
5 Resection Post-ChT(V,A,C), Complete Disease-free Alive, 22 y disease-free
R2 RT (60) remission
6 Resection Post-ChT(SIOP), Residual Residual tumor None Died 7 mo after ID
R2 RT (60) tumor (residual tumor)
7 Biopsy P-ChT(I,A), RT Residual Residual tumor IRT Died 6 y, 1 mo after ID
(59) tumor (complications)
8 Resection Post-ChT(I,A)-RT Complete Distant Palliative RT Died 9 mo after ID
R0 (60) remission metastases (multiple
metastases)
9 Biopsy P-CWS-91, RT Complete Disease-free Alive, 9 y disease-free
(54) remission
10 Biopsy P-CWS-91, RT Complete Disease-free Alive, 7 y disease-free
(48) remission
11 Biopsy P-ChT-RT (48) Complete Regional Radical neck Died 25 mo after ID
remission recurrence dissection, RT (regional recurrence)
12 Resection Post-ChT(I,A), RT Complete Disease-free Alive, 3 y disease-free
R0 (66) remission
13 Biopsy P-VACA-Schema, Complete 2 local Tumor embolization, Died 2 y, 9 mo after ID
RT (50) remission recurrences, resection of local (bone metastases)
distant recurrence, RT
metastases distant metastases
14 Biopsy P-CWS-96, RT Complete Local and Resection of local Died 20 mo after ID
(54) remission regional recurrence, neck (multiple
recurrence, dissection, metastases)
distant thoracotomy, ChT,
metastases BMT,
laminectomy
15 Resection Post-CWS-91, RT Complete Disease-free Alive, 5 y disease-free
R0 (58) remission
P, primary; Post, postoperative; IRT, interstitial radiotherapy; ID, initial diagnosis; A, adriamycin, C, cyclophosphamide; I,
ifosfamide; V, vincristine; R0, resection with histologically clear margins; R2, resection with macroscopic tumor remnant; BMT, bone
marrow transplant; VACA, vincristine/actinomycin D/carboplatin/adriamycin; CWS, Cooperative Soft Tissue Sarcoma Study; SIOP,
International Society of Pediatric Oncology; ChT, chemotherapy; RT, radiotherapy; Gy, dose in Gray

mass was subjected to interstitial radiotherapy with Irid-
ium 192, low dose-rate. This patient survived tumor-free
for a period of more than 6 years but then died from
aspiration pneumonia.
Results by Treatment Methods
Tumor resection was performed in 6 patients. Of the 2
patients with histologically residual tumor, 1 patient had
subsequent adjuvant RCT and achieved complete remission.
This patient has been living free of tumor for 22 years. In
the 2nd patient, a residual tumor remained even after post-
operative RCT. This patient died 7 months after ID.
Despite postoperative histologically negative margins
and adjuvant RCT, distant metastases developed in 1 of 4
cases. This patient died 7 months after ID. The remaining 3
patients remained tumor-free after an observation period of
10 months. Primary RCT yielded complete remission in 6
cases (Figs 2A and B), and 3 cases showed a residual tumor.
46 Otolaryngology–Head and Neck Surgery, Vol 133, No 1, July 2005
Figure 2 Complete tumor remission after completed primary radiochemotherapy (A and B) (same patient as in Fig 1A and B).
In 2 patients with residual tumor, only minor reduction of
the original tumor mass was accomplished by primary RCT.
Subsequent surgery would have led to extensive mutilation
of the patients, with questionable outcome. Therefore, ther-
apy was discontinued. These patients died 8-11 months after
ID. The residual tumor became resectable in 1 case, but
surgery was refused by the patient. Primary RCT then was
followed by interstitial irradiation with Iridium 192. There-
after, a tumor-free survival of more than 6 years was at-
tained. Two of the 6 patients with complete remission re-
mained tumor free, 3 suffered a local recurrence or distant
metastases, and 1 patient suffered a regional recurrence.
Surgery was performed in 3 cases with resection of local
and regional recurrences after renewed irradiation and che-
motherapy. One patient received palliative radiotherapy be-
cause of multiple distant metastases. Despite additional
treatment efforts, all 4 patients died after a time interval of
20-33 months.
According to the Kaplan-Meier curve, the overall sur-
vival among those patients operated and subsequently
treated by RCT was 66%, whereas the overall survival of
those patients primarily treated by RCT amounted to 33%.
However, the difference showed no statistical significance
(P ⫽ 0.434; Fig 3).
Histologic Subtypes
The histologic subtypes comprised 9 patients with RMS and
6 cases with aRMS. Among the eRMS cases, there were 6
children between 2 and 8 years of age and 3 adults between
51 and 60 years. The 6 patients with aRMS were aged
between 14 and 40 years. Five-year survival of the patients
with eRMS was 55%; that of patients with aRMS only was
33% (P ⫽ 0.375; Fig 4). No significant differences with
respect to tumor stage or treatment methods were found in
either of the histologic subgroups.
Tumor Localization and Extent
On the basis of the applied pretherapeutic imaging methods,
extensive RMS of the nose and paranasal sinuses with
infiltration of the skull base were diagnosed in 7 of the 15
patients. In 3 of these cases, infiltration of the orbit was
established as well. Five patients died during the first 25
months after ID. Two patients have remained tumor free for
7-9 years. In 4 of 15 cases, the tumors were confined to the
nasal cavity and nasopharynx. Two patients died, 1 from
multiple distant metastases and 1 from non–tumor-related
causes 6 years after ID. Two patients are alive and disease-
free after 3-10 years’ follow-up. Four cases initially pre-
sented with RMS affecting the paranasal sinuses without
infiltration of the skull base. Two of them died from local
recurrences and distant metastases. The remaining 2 pa-
tients are alive and without evidence of recurrent tumor
after 5 and 22 years, respectively. In summary, RMS with
infiltration of the skull base showed a tumor-specific sur-
vival rate of only 28% compared with those tumors confined
to the nose and paranasal sinuses, with a survival rate of
62% (P ⫽ 0.132; Fig 5).
Regional formation of lymph node metastases before the
onset of therapy was detected in 3 patients. All of these
cases showed aRMS. The different incidence of lymph node
Wurm et al Rhabdomyosarcomas of the Nose and Paranasal . . .
1.0
0.9
0.8
Estimated survival function
0.7
0.6
0.5
0.4
0.3
0.2
0.1
0.0
0 20 40
Therapy
Figure 3 Kaplan-Meier curve showing the survival function of patients treated with primary RCT (dashed line) and patients treated with
tumor resection and subsequent RCT (solid line; P ⫽ 0.434).
metastases in eRMS and aRMS was statistically significant
(P ⫽ 0.04). Three patients developed distant metastases in
the course of the disease. In 1 case, distant metastases
developed after an interval of merely 5 months after ID,
despite reliable local tumor control. Histologically, 2 pa-
tients showed aRMS, and 1 showed eRMS.
DISCUSSION
RMS of the nose and paranasal sinuses often present with
unspecific symptoms. As a consequence, delays in estab-
lishing the correct diagnosis and initiating therapy occur in
many cases. At the time of definite histologic differentia-
tion, the tumors are mostly advanced and show a locally
invasive growth.
Therefore, RMS should be included in the differential
diagnosis of any destructively growing mass of the nose or
paranasal sinuses both in children and adults. An early
biopsy and histologic assessment by an experienced pathol-
ogist are of utmost importance.
Primary localization of these tumors represents one of
the most important prognostic factors in therapy of RMS.
Like all parameningeal RMS types, RMS of the nose and
paranasal sinuses are associated with a less favorable prog-
nosis than RMS with other localizations because of the risk
of intracerebral extension.2,9,14
Before the introduction of chemotherapy in the treatment
of RMS, the cure rates were only in the range of 10%-25%.
47
60 80 100 120 140 160 180 200 220 240 260 280
Time [month]
primary RCT
tumor resection with subsequent RCT
Five-year survival for these tumors has substantially
improved since the beginning of the 1970s, particularly
because of combined administration of several effective
cytostatics (especially adriamycin, actinomycin D, cyclo-
phosphamide, ifosfamide, vincristine) in combination with
selective and individually adapted radiotherapy. Cure rates
of partly ⬎90% have been reported in the studies of the IRS
and the CWS. Here too, particularly the histologic tumor
subtype and localization were identified as important prog-
nostic factors, next to tumor extension.3,4,6,10,14,15 Consis-
tent with these published data, we observed a worse out-
come among patients who intitially presented with tumors
infiltrating the anterior skull base compared with RMS con-
fined to the nose and paranasal sinuses, whereas further
evaluation did not indicate any difference between tumors
of the nasal cavity and tumors of the paranasal sinuses if the
skull base was not involved.
Overall the therapeutic results appear clearly less favor-
able, however, if RMS of the nose and paranasal sinuses are
viewed separately from those of other anatomic sites. Tu-
mor-specific survival rates ranged between 20% and 50% in
this patient group. The results of the present study, which
show a tumor-specific 5-year survival of 46% are well
within the range reported by the literature.2,5,6,8,9
Significance of Surgery
The significance of surgery has been the subject of critical
discussion in the literature. Anderson et al16 did not advo-
cate primary radical tumor resection if there is the risk of
sacrificing essential structures or producing an unsatisfac-
48
1.0
0.9
0.8
0.7
Estimated survival function
0.6
0.5
0.4
0.3
0.2
0.1
0.0
0 20
Histological subtypes of RMS
Figure 4 Kaplan-Meier curve showing the survival function of embryonal (dashed line) and alveolar (solid line) rhabdomyosarcomas
(P ⫽ 0.375).
tory functional outcome. Removal of the facial nerve or an
eye, for example, should be restricted to cases with recur-
rent tumors or residual tumors after primary radiochemo-
therapy. Also, according to Sercarz et al,9 surgery on pa-
tients with RMS of the nose and paranasal sinuses should be
restricted to a biopsy for histologic verification. According
to those investigators, surgical tumor removal leads to an
unacceptably high morbidity and to incomplete resection in
most cases. They saw an indication for surgery as given
only in patients with recurrent tumors or in cases in which
the radiation risks outweigh the risks of surgical removal.
In contrast to this, Healey et al17 recommended radical
tumor resection whenever complete locoregional removal
appears possible. Chemotherapy should be carried out sub-
sequently to control possible distant metastases. In such
cases, radiotherapy is still possible if local recurrences arise.
Because of progress in the fields of skull base surgery,
microsurgery, and plastic-reconstructive surgery, nowadays
the option of reconstructing important structures with a
functionally and cosmetically satisfactory result exists, if
these structures have to be sacrificed in the framework of
tumor resection.
Callender et al2 reported on a locoregional recurrence
rate of 75% after exclusive surgical therapy among their
patients with RMS of the nose and paranasal sinuses.
According to Treuner et al,6 patients with a tumor that
can be completely resected and subsequently treated by
RCT showed a cure rate of ⬎90%. Here it should be noted,
Otolaryngology–Head and Neck Surgery, Vol 133, No 1, July 2005
40 60 80 100 120 140 160 180 200 220 240 260 280
Time [month]
embryonal alveolar
though, that the results of this study are based on RMS in all
sites.
In those cases in which tumor resection with subsequent
RCT was performed, we were able to achieve a tumor-free
5-year survival of 66%. On the other hand, tumor-free
survival was only 33% in patients who had received RCT
exclusively. This group, however, represents patients with
extensive tumor growth primarily not amenable for surgery.
It appears undisputed that in many cases, RMS can be
reliably removed by surgery only at the expense of pro-
nounced functional restrictions. Moreover, the side effects
and serious long-term negative effects of RCT also have to
be considered in the discussion. Donaldson et al10 and
Paulino et al18 reported on long-term side effects in patients
after radiotherapy of the head and neck during childhood.
These included, in particular, growth disturbances in 60%,
facial asymmetry in 73%, unilateral hearing loss in 75%,
and visual impairment up to blindness in 82% of cases.
Rarer, but equally serious, long-term effects arising 10 years
or later after radiotherapy include osteoradionecrosis,
esophageal stenoses, malignant secondary tumors, and in-
tracerebral bleeding. Depending on the chemotherapy re-
gime, Crist et al,3 Koscielniak et al,4 and Ruymann and
Grovas15 reported on lethal complication in the context of
chemotherapy in 3.5%-12% of patients. These complica-
tions were caused mainly by septicemia, cardiotoxicity,
thrombocytopenic bleeding, and acute respiratory distress
syndrome.
Wurm et al Rhabdomyosarcomas of the Nose and Paranasal . . .
1.0
0.9
0.8
0.7
Estimated survival function
0.6
0.5
0.4
0.3
0.2
0.1
0.0
0 20
Infiltration of the skull base
Figure 5 Kaplan-Meier curve showing the survival function of rhabdomyosarcomas without (dashed line) and with (solid line)
infiltration of the skull base at initial diagnosis (P ⫽ 0.132).
We observed radiotherapy- and radiochemotherapy-in-
duced complications in 6 of 15 patients. These included
epilepsy, radiation necrosis of the brain and optic nerve,
growth disturbances, and polyneuropathy.
Moreover, a palatine fistula and a CSF fistula were ob-
served in 1 patient each after RMS resection. In our expe-
rience, surgical tumor resection with subsequent RCT is
recommended, independent of tumor stage, whenever com-
plete resection appears possible. But certain principles must
be adhered to. No effort should be spared to reconstruct
cosmetic and functional defects resulting from block resec-
tion so as to avoid mutilation of the patients. Precise pre-
operative planning with the selection of suitable removal
sites for reconstructive flap surgery is essential. Resected
tissue should be replaced by similar tissue, and nerve re-
construction should be performed at the time of primary
surgery. Next to the surgical reconstruction options, in
many cases, good cosmetic results can be attained by fitting
an epithesis, eg, after orbital exenteration.19 The descision
in choosing the optimal therapeutic strategy should be made
for each patient individually by a team of ENT surgeons,
neurosurgeons, maxillofacial surgeons, oncologists, and ra-
diotherapists.
Histologic Subtypes
In our patient group, we treated 9 cases of eRMS and 6
cases of aRMS. Five-year survival of patients with eRMS
was 55%, whereas 5-year survival of patients with aRMS
was only 33%. No significant differences as to tumor stage
49
40 60 80 100 120 140 160 180 200 220 240 260 280
Time [month]
no yes
or treatment methods were found for either subgroup. New-
man and Rice8 were unable to demonstrate any relationship
between different histologic subtypes and the clinical course
of their patients. Our experiences are in agreement with the
findings reported by most other investigators in that patients
with eRMS apparently have a better prognosis than those
with aRMS.2,4,6,14,20
Depending on histologic differentiation different patterns
of metastasis formation are observed. At the time of initial
diagnosis, lymph node metastases were clinically evident in
20% of our patients. In all these cases, patients were af-
flicted by aRMS. The different incidence of lymph node
metastases in eRMS and aRMS was statistically significant.
In contrast, a regional recurrence occurred in eRMS patients
in only 1 of 9 cases during the course of the disease.
In view of the low incidence of cervical lymph node
metastasis of merely 3% according to the IRS, Anderson et
al16 saw no indication for conducting elective neck dissec-
tion. However, both Callender et al2 as well as Newman and
Rice8 considered these figures to be clearly too low. No
other study has reported such a low incidence. These inves-
tigators themselves found regional metastasis in 15%-38%
of their patients. On the basis of their experience with
regional recurrences, the investigators inferred that these
patients obviously had benefited from elective neck dissec-
tion.
Taking the experience of our own group into account, we
recommend the implementation of elective neck dissection
in patients with aRMS.
50 Otolaryngology–Head and Neck Surgery, Vol 133, No 1, July 2005
CONCLUSIONS
Rhabdomyosarcoma of the nose and paranasal sinuses is
very rare and shows a nonspecific sympomatology. Early
CT and MR imaging procedures, as well as early histologic
assessment, are recommended. The tendency for a better
outcome can be observed in patients with an embryonal
rhabdomyosarcoma, in patients without evidence of skull
base infiltration, and among those cases treated by surgery
and subsequent radiochemotherapy. The results, however,
showed no statistical significance. Because of the statisti-
cally significant higher incidence of lymph node metastases
in patients with alveolar rhabdomyosarcomas, an elective
neck dissection should be considered if surgery of the pri-
mary tumor is performed.
REFERENCES
1. Halperin EC. In: Halperin EC, editor. Pediatric radiation oncology,
2nd ed. New York: Raven Press Inc.; 1994, p. 303– 40.
2. Callender TA, Weber RS, Janjan N, et al. Rhabdomyosarcoma of the
nose and paranasal sinuses in adults and children. Otolaryngol Head
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