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: dranirban666@gmail.com1Department 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

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)

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

AbstractBackgroundCase presentationConclusions

BackgroundCase presentationDiscussionConclusionAbbreviationsAcknowledgementsAuthors’ contributionsFundingAvailability of data and materialsEthics approval and consent to participateConsent for publicationCompeting interestsAuthor detailsReferencesPublisher’s Note