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Journal of the EgyptianNational Cancer Institute
Biswas et al. Journal of the Egyptian National Cancer Institute
(2020) 32:26 https://doi.org/10.1186/s43046-020-00038-0
CASE REPORTS Open Access
Inflammatory myofibroblastic tumor of
maxillary sinus successfully treated withradiotherapy and
corticosteroid: report of arare case
Rituparna Biswas1, Anirban Halder1* , Mimi Gangopadhyay2 and
Dipanwita Biswas3
Abstract
Background: Inflammatory myofibroblastic tumor (IMT) is a
mesenchymal tumor mainly found in lung orretroperitoneum and rarely
affects head and neck region. Extensive English literature search
reveals that less thanfifty cases of head and neck IMT have been
reported so far, maxillary sinus being fewer.
Case presentation: We present a case of IMT involving maxillary
sinus in a 48-year-old gentleman who attainedcomplete
clinico-radiologic response after treatment with radiotherapy (RT)
and concurrent oral prednisolone.
Conclusions: This is the first report where such magnificent
response was attained in primary setting treated withRT and
steroids as opposed to surgery which used to be considered as
standard of care till now.
Keywords: Inflammatory myofibroblastic tumor, Maxillary sinus,
Prednisolone
BackgroundInflammatory myofibroblastic tumor (IMT) is a rare
en-tity with controversial biological behavior. It is now
con-sidered as a benign neoplasm with locally aggressivebehavior,
having rare metastatic potential [1]. TheWorld Health Organization
(WHO) defined IMT as anintermediate soft tissue tumor that is
composed of myo-fibroblastic and fibroblastic spindle cells
accompanied byinfiltrate of numerous inflammatory cells, plasma
cells,eosinophils, and lymphocytes [1]. IMTs are usuallyfound in
the lung, abdomen, retroperitoneum, and ex-tremities, rarely
affecting head and neck region [2].Whether IMT is a neoplastic or a
reactive process hadbeen a matter of controversy, but
identification of ana-plastic lymphoma kinase (ALK) gene
rearrangement sug-gests more of a neoplastic etiology than a
reactiveinflammatory process [3]. The exact etiology and
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* Correspondence: [email protected] of Radiation
Oncology, Medica Cancer Hospital, Rangapani,Siliguri, IndiaFull
list of author information is available at the end of the
article
pathogenesis are not clearly understood due to paucityof cases;
however, reports indicating infection or an ab-normal immunological
reaction are considered as possi-bilities [1, 4]. Similarly, no
standardized treatmentguidelines are available yet. Herein, we
present a case ofIMT involving maxillary sinus in a 48-year-old
gentle-man who attained complete response after treatmentwith
radiotherapy and oral prednisolone.
Case presentationAn otherwise well, a 48-year-old male patient
presentedwith pain and swelling over right maxillary region for
4months associated with inability to open mouth fully.Clinically,
he was having fullness over right maxillary re-gion and severe
trismus (15 mm oral opening). Contrast-enhanced computed tomography
(CECT) scan of faceand neck region revealed a soft tissue mass in
right max-illary antrum with bony destructions—intraorbital
extra-conal extension abutting the inferior rectus muscle
withpossible involvement of the infraorbital nerve, medial
ex-tension to middle meatus, inferior meatus and involving
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Biswas et al. Journal of the Egyptian National Cancer Institute
(2020) 32:26 Page 2 of 5
nasolacrimal duct, anterior extension to premaxillary
space,inferior extension to alveolar process and posterolateral
ex-tension to infratemporal fossa, and pterygoid fossa withpossible
involvement of temporalis and lateral pterygoidmuscles (Fig. 1). No
evidence of lymph nodal or distant me-tastases was found on
imaging. Biopsy from tumor wastaken for histopathologic examination
(HPE). HPE and sub-sequent immunohistochemistry (IHC) suggested it
to be in-flammatory myofibroblastic tumor (Fig. 2).
Morphologicallytissue consisted of plasma cells, eosinophils, and
lympho-cytes in a variably collagenized spindle cell stroma.
Thespindle cells had bland nuclear morphology. On IHC,tumor cells
were positive for CD3, CD20, CD 138, CD19,kappa, lambda
(kappa:lambda was 2:1), SMA, and calponinand negative for CD56, CK,
Alk-1, desmin, h-Caldesmon,and Ki-67 was < 1%.In view of
extensive disease, he was deemed unresect-
able. Hence, he was treated with definitive radiotherapy(RT)
with dose 60 Gy in conventional fractionation over6 weeks
concurrent with oral steroids. For simulationand treatment, he was
immobilized with thermoplasticmold in supine position with head
extended using headrest. Intraoral stent was inserted to depress
tongue out-side radiation field as much as possible. CT
simulationusing 3 mm thickness with IV contrast was performed.Wet
cotton bolus was used during treatment. For targetvolume
delineation, all visible tumor in CECT scan wastaken into account
as gross tumor volume (GTV); forclinical target volume (CTV), 1 cm
margin was added toGTV all around and then carved out respecting
anatom-ical boundaries. Ipsilateral nasal cavity medially till
nasal
Fig. 1 CECT scan showing right maxillary tumor with a extension
to orbit (c meatus involvement and intraorbital invasion (axial
view)
septum, entire maxillary sinus, anteriorly and laterally
tillskin, ipsilateral ethmoid sinus, posteriorly
infratemporalfossa, and pterygopalatine fossa were encompassed
in-side CTV. Superiorly intraorbital margins of CTV werereduced to
exclude eye. Finally, 5-mm margin to CTVwas given to produce
planning target volume (PTV);however, margin was reduced to 1mm
beside ipsilateraleye and optic nerve. Radiation planning was done
by for-ward planning IMRT (intensity-modulated radiationtherapy)
(Fig. 3). Prednisolone was given in taperingdoses with starting
dose 60mg and was tapered by 10mg every subsequent week. He
responded well. After 1month of RT completion, clinically no
swelling was ap-preciated and trismus resolved. Contrast-enhanced
mag-netic resonance imaging (CEMRI) was done after 2months of RT
completion which revealed post-RTchanges with complete resolution
of tumor (Fig. 4). Heis doing fine till 6 months follow-up now.
DiscussionAs IMT of the maxillary sinus is a rare entity,
knowledgepertaining to this is availed from case reports and
seriesdescribed in English literature. It usually presents aslocal
pain or swelling as is found in index case. UnlikeIMTs at other
locations, systemic manifestations such asanorexia, weight loss,
and fever have not been observedin cases of the maxillary sinus
IMTs [5]. CT scan orMRI of IMTs in the paranasal sinus often
suggests infil-trative growth with aggressive malignant potential
[5]. Itusually appears as homogeneous soft tissue masses fillingthe
maxillary sinuses with no evidence of calcification or
sagittal view), b anterior and posterolateral extension (axial
view), and
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Fig. 2 a Hematoxylin and eosin staining, b cells staining with
lambda, c CD138 staining, and d cells positive for CD19
Biswas et al. Journal of the Egyptian National Cancer Institute
(2020) 32:26 Page 3 of 5
central necrosis. Bone destruction is frequently found
inadvanced cases as also evident in our case. Histologi-cally, IMT
is composed of variable admixture of fasciclesof myofibroblastic
spindle cells with a prominent poly-clonal infiltrate of numerous
plasma cells, lymphocytes,
Fig. 3 a Table showing dose received by OAR. b and c IMRT plan
showing dose c
and acute inflammatory cells, in a loose myxoid oredematous
stroma [5]. IHC is considered gold standardin obtaining diagnosis.
Till date, many cellular markershave been identified including
desmin, vimentin, smoothmuscle actin, cytokeratin, and ALK-1 that
aid in the
olor wash of 90% isodose coverage of PTV. d DVH of RT plan (red
line = PTV)
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Fig. 4 CEMRI showing post RT changes in a sagittal view and b, c
axial view showing diffuse mucosal thickening without any evidence
ofenhancing solid tumor
Biswas et al. Journal of the Egyptian National Cancer Institute
(2020) 32:26 Page 4 of 5
pathologic diagnosis of IMT [2, 6]. As of now, surgicalresection
with negative margins is considered mainstayof management [2, 6,
7]. Where partial resection wasonly feasible, adjuvant radiotherapy
or steroids wereemployed with promising results. In recurrent or
refrac-tory cases, radiotherapy and pharmacotherapies such
asNSAIDs, COX inhibitors, corticosteroids, and kinase in-hibitors
were used alone or in combination [7]. Reportsin favor of
chemotherapy are scarce [8]. To date, nostandardized
chemotherapeutic regimens had been in-troduced. Radiotherapy in
dose range 20–60 Gy has beenadministered in various reports [5, 6].
Our presentedcase attained complete clinico-radiological response
withradiation therapy (RT) dose 60 Gy along with concurrentoral
high-dose prednisolone in tapering dose. Extensiveliterature search
reveals this is the first report wheresuch overwhelming response
was attained in primarysetting treated with RT and steroids. Hence,
our reportsuggests that RT with steroids is equally effective
alter-native to surgery in treating a patient with maxillaryIMT.
However, further studies with large sample sizewill be needed to
corroborate our finding.
ConclusionWe report the first known case of IMT of
maxillarysinus being treated by radiotherapy and steroid
whoattained complete response, hence leading to advance-ment in
management and care of individuals affected byIMT. Our evidence
establishes radiotherapy with steroidas alternative to surgery
which used to be considered asstandard of care till now and this
paves way for futurestudies.
AbbreviationsIMT: Inflammatory myofibroblastic tumor; RT:
Radiotherapy; ALK: Anaplasticlymphoma kinase; CECT:
Contrast-enhanced computed tomography;HPE: Histopathologic
examination; IHC: Immunohistochemistry;IMRT: Intensity-modulated
radiation therapy; DVH: Dose-volume histogram;OAR: Organs at risk;
CEMRI: Contrast-enhanced magnetic resonance imaging
AcknowledgementsNot applicable
Authors’ contributionsRB and AH contributed to the conception of
the study. MG and AH wereresponsible for the acquisition. RB and DB
drafted the work. RB, AH, MG, andDB substantively revised it. All
authors have read and approved themanuscript.
FundingNot applicable
Availability of data and materialsNot applicable
Ethics approval and consent to participateNot applicable
Consent for publicationWritten informed consent has been taken
from patient.
Competing interestsThe authors declare that they have no
competing interests
Author details1Department of Radiation Oncology, Medica Cancer
Hospital, Rangapani,Siliguri, India. 2Department of Pathology,
Medica North Bengal Clinic, Siliguri,India. 3R G Kar Medical
College, Kolkata, India.
Received: 24 March 2020 Accepted: 13 May 2020
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https://doi.org/10.1155/2018/7928241
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