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13]

Case Report

Complete response to radiation therapy for

nasopharyngeal sarcomatoid carcinoma
ABSTRACT Mi‑Jo Lee,
Nasopharyngeal sarcomatoid carcinoma (SaCa) is extremely rare, and concurrent chemoradiation is the standard treatment for Hyun‑Jin Son1
squamous cell‑based nasopharyngeal cancer (NPC).  This case report gives the first explanation of a nasopharyngeal SaCa patient
Departments of
treated with volumetric modulated arc therapy (VMAT) with simultaneous integrated boost (SIB), which is an excellent treatment Radiation Oncology
modality that leads to complete response for locally advanced NPC. A 70‑year‑old male presented with nasal obstruction, epistaxis, and 1Pathology, Eulji
and right neck node enlargements. Examination revealed an extensive tumor of nasopharyngeal tumor extending into the nasal cavity University Hospital,
and right parapharyngeal space with bilateral lymphadenopathy on positron emission tomography (PET)–computed tomography Daejeon, Korea
images of focal hypermetabolic bone lesion in C4 body (stage T3N2M1). An excisional biopsy of nasopharyngeal wall mass showed
For correspondence:
a SaCa. He received concurrent chemoradiation which was VMAT and systemic chemotherapy (cisplatin 60 mg). A dose of 70 Gy Dr. Mi‑Jo Lee,
was delivered to the planning target volume (PTV70) (gross tumor volume plus margin 3–5 mm) and PTV59.4 (a wider margin around Department of
high‑risk clinical target volume, including the clivus and neck nodes) all given in 33 fractions. Radiological examination such as Radiation Oncology,
magnetic resonance imaging (MRI) and PET images at the completion of external beam therapy revealed questionable residual Eulji University
Hospital, 95,
disease. Follow‑up MRI scans 4 weeks after radiotherapy revealed a complete tumor response. VMAT with SIB can be an effective
Dunsanseo‑ro, Seogu,
treatment option for SaCa of the advanced nasopharynx. Daejeon 35233, Korea.
E‑mail: minsk7144@
hanmail.net
KEY WORDS: Nasopharyngeal cancer, radiation therapy, sarcomatoid carcinoma, simultaneous integrated boost, volumetric arc therapy

INTRODUCTION precision and accuracy dose painting to target

Sarcomatoid carcinoma (SaCa) (spindle cell) of the
nasopharynx is a malignant tumor accomplished
biphasic feature of malignant epithelial and
connective tissues. SaCa is a very rare tumor,
Western report not existed, just a few studies
reported accounting for <0.3% of upper digestive
tract SaCas in Taiwan. [1] The most common
sites of SaCa are the larynx, oral, and sinus.[2]
SaCa is an aggressive malignant head‑and‑neck
tumor; it has worse 5‑year overall survival than
squamous cell nasopharyngeal carcinoma (NPC)
(38% vs. 72%).[1,3] The treatment of SaCa of the
nasopharynx is not standardized because of
its rarity; concurrent chemoradiation reported
curable treatment option as like squamous cell
origin nasopharyngeal carcinoma. In 1980 area,
three‑dimensional conformal radiation therapy
has been used in radiotherapy (RT) for decades,
but in recent years, volumetric arc therapy (VMAT)
has become the standard of radiation therapy
for head‑and‑neck malignancies. VMAT with
tumor volumes such that normal organs receive
a low dose per fraction.
This case study presents a patient with SaCa of the
nasopharynx who showed a short‑term complete
tumor response to VMAT with SIB and without Submitted: 15-Apr-2019
severe toxicity in time treatment. Revised: 09-Jun-2019
Accepted: 20-Oct-2019
CASE REPORT Published: 18-Jul-2020
A 70‑year‑old patient visited our facility with a
chief complaint of nasal obstruction and neck mass
for several months. Obstruction symptom was
associated with ear fullness and epistaxis. There
was no hoarseness, dysphagia, or anorexia. There
was no family history of malignancy or previously
radiation history. He quit smoking 10 years ago and
had a smoking capacity of 40.
Access this article online
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DOI: 10.4103/jcrt.JCRT_254_19
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simultaneous integrated boost (SIB) is one of the identical terms.

most delicate forms of RT. VMAT with SIB uses For reprints contact: WKHLRPMedknow_reprints@wolterskluwer.com

Cite this article as: Lee MJ, Son HJ. Complete response to radiation therapy for nasopharyngeal sarcomatoid carcinoma.
J Can Res Ther 2020;16:653-6.

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Lee and Son: Complete response to radiation therapy for nasopharyngeal sarcomatoid carcinoma
On laryngoscopy examination, there was a mass‑occupying
posterior right nasal cavity. His vocal cords appeared equal
and fully mobile, and there was no obvious mass. Both the
external auditory canal and tympanic membranes appeared
normal. Both neck nodes were palpable, especially the right
Level II–III lymph node sized 1.5 cm.
Histopathology indicated that the mass was consistent
with SaCa. The carcinoma component of the malignant cells
was positive for Epstein–Barr virus by in situ hybridization,
indicating a nasopharyngeal origin. The sarcomatous elements
were positive for cytokeratin (Pan‑CK, CK7, CK20, CK19, HM‑CK,
and CK5/6), EMA, and CD5 and negative for LCA, CD3, CD20,
CD30, and NK cell markers (CD56, TIA‑1, and granzyme B),
which excluded a diagnosis of lymphoma. Figure 1 shows the
pathological diagnosis.
Magnetic resonance imaging (MRI) of the nasopharynx
showed a relatively homogeneous, T2‑intermediate,
T1‑isosignal‑enhancing mass or mass‑like lesion from the
posterior wall mass extension of the nasopharynx to the
right nasal cavity and right ethmoid sinus with clivus
invasion. Multiple enlarged lymph nodes with necrosis
were observed in the right retropharyngeal area and both
Level II and III cervical lymph node chains bilaterally
(largest measuring 1.5 cm × 0.9 cm). Positron emission
tomography–computed tomography (PET–CT) indicated
a hypermetabolic bone lesion at the C4 spinal and skull
bases [Figure 2].
a b
c d
e tomography–computed tomography before treatment. (c and d) Near
Figure 1: (a) H and E, ×200, (b) Pan‑CK, (c) Vimentin, (d) CD5, (e)
Epstein–Barr virus in situ hybridization
654
The patient underwent systemic chemotherapy (cisplatin)
and referred to the radiation oncology department, where
he underwent 70 Gy of radiation therapy. Normal organs
prescription dose is demonstrated in Table 1.
During the simulation, the patient was immobilized in
the supine position with his arms above his head using
aqua plaster. CT was conducted using a 16‑slice CT scanner
(Somatom Sensation 16; Siemens Healthcare, Germany).
Treatment planning was performed with Monaco 5.0
(Elekta AB, Stockholm, Sweden) using the X‑ray voxel
Monte Carlo dose algorithm for a Synergy linear accelerator
(Elekta, England). The entire isodose curve is demonstrated
in Figure 3.
MRI and PET–CT 1 month after the completion of RT showed
that the nasopharyngeal mass had almost disappeared. In
addition, there were no palpable neck nodes. Patients are dead
after 2‑month complete radiation treatment. Cause death is
the dissemination of bony metastasis.
DISCUSSION
This male patient presented with no known additional risk
factors.[2,6-7] He denied any history of alcohol consumption or
a previous history of radiation exposure. He presented to us
only with a 3‑week history of nasal obstruction/bleeding, and
he was already noted to have posterior nasal mass sensation
with neck metastasis. VMAT with SIB uses high‑precision dose
painting to target tumors such that normal organs receive a low
dose per fraction. This case report presents a patient with SaCa
a b
c d
Figure 2: (a) Magnetic resonance imaging and (b) positron emission
complete response 4 weeks after treatment (c: magnetic resonance
imaging, d: positron emission tomography–computed tomography)
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Lee and Son: Complete response to radiation therapy for nasopharyngeal sarcomatoid carcinoma
Table 1: Normal organ prescription (late effect and QUANTEC in Perez and Brady’s Principle and practice of radiation
oncology 7th edition p360-1)
OAR
Spinal cord
Brain
Optic chiasm
Optic nerve
Lens
Eyeball
Salivary gland (each)
External auditory membrane
Tympanic membrane
GTV primary, gloss lymph nodes
PTV70: GTV + 3‑5 mm (based on comfort level)
CTV elective, CTV area including
CTV59.4: At least 1‑cm margin and positive neck node positive (Level I‑V)
OAR=Organ at risk, GTV=Gross tumor volume, PTV=Planning target volume, CTV=Clinical target volume, QUANTEC=Quantitative analyses of normal tissue
effects in the clinic
Figure 3: Volumetric modulated arc therapy plan with the planning
target volume. Dose distribution with isodose lines, from 100% to 30%
of the nasopharynx who showed a complete tumor response to
VMAT with SIB, and there was no in‑field recurrence or severe
toxicity except mild respiration system side effect.
NPC is the most common tumor of the nasopharynx. SaCas
include synovium spindle cell carcinoma, pseudosarcoma,
carcinosarcoma, and unusual variants of squamous carcinoma.[4]
SaCa has previously been reported in the upper aerodigestive
tract, prostate, and small intestine.[1,2,4,5]
Although the head‑and‑neck area is a common site for SaCa
development, very little has been written on this disease.[1,2]
SaCa is a male‑predominant disease, with older age, alcohol
consumption, tobacco use, and previous RT as additional
risk factors.[2,6,7] The mortality rate is higher than that in
squamous cell carcinoma, with better outcomes associated
with early‑stage and extraoral tumors.[2,8] The high mortality
rate of SaCa in the head‑and‑neck area is believed to be due to
lower resistance to tumor spreading along tissue planes and
the extensive lymph drainage system, with resultant lymphatic
and distant metastases.[6,9,10]
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Constraints
Max <50 Gy
Dmax <60 Gy
1% <60 Gy
1% <60 Gy
1% <10 Gy
Max <50 Gy, mean 35 Gy
V50% <30 Gy
Mean <50 G
V1% <75 Gy
212 cGy ×33 fraction
180 cGy ×33 fraction
In a series of 78 Taiwan cases collected from an extensive
head‑and‑neck cancer database from 1978 to 2008, only three
were nasopharyngeal SaCa.[1] These patients were treated with RT,
but that study included no description about RT administration.
Among the carcinosarcoma of the head and neck, laryngeal
sarcomatoid tumors seem to have a better prognosis compared
to extralaryngeal tumors. They also reported a high tendency
for local recurrence, distant metastasis, and poor outcome. In
patients who received concurrent chemoradiotherapy, only
36% (four of 11) underwent complete remission; the other seven
patients died within 6 months from the uncontrollable disease.
The current concept for the treatment of SaCa is RT when the
tumor is in the nasal cavity, nasopharynx, or hypopharynx,
where RT remains the primary treatment modality.
CONCLUSIONS
SaCa of the nasopharynx, although rare, is not to be taken
lightly by health professionals. It is known to be very aggressive
and has a poor prognosis. Therefore, early diagnosis and prompt
treatment are essential to improve the outcome and survival
rates of these patients. More research regarding the treatment
modalities for nasopharyngeal SaCa is still needed.
Declaration of patient consent
The authors certify that they have obtained all appropriate
patient consent forms. In the form the patient(s) has/have
given his/her/their consent for his/her/their images and other
clinical information to be reported in the journal. The patients
understand that their names and initials will not be published
and due efforts will be made to conceal their identity, but
anonymity cannot be guaranteed.
Financial support and sponsorship
Nil.
Conflicts of interest
There are no conflicts of interest.
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Lee and Son: Complete response to radiation therapy for nasopharyngeal sarcomatoid carcinoma

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