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 Table of Contents  
CASE REPORT
Year : 2020  |  Volume : 32  |  Issue : 1  |  Page : 86-89

Offbeat phenomenon in antrum of highmore – Inflammatory myofibroblastic tumor


Oral Medicine and Maxillofacial Radiology, Ragas Dental College and Hospital, Uthandi, Chennai, Tamil Nadu, India

Date of Submission09-Dec-2019
Date of Decision09-Mar-2020
Date of Acceptance24-Mar-2020
Date of Web Publication17-Apr-2020

Correspondence Address:
Dr. G Jayashree
Oral Medicine and Maxillofacial Radiology, Ragas Dental College and Hospital, Uthandi, Chennai, Tamil Nadu
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/jiaomr.jiaomr_203_19

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   Abstract 


The Inflammatory Myofibroblastic Tumor (IMT) is a rare benign pathology of the head and neck region that tends to mimic a malignancy on account of its aggressive clinical and radiographic features. There are various descriptive terms for this lesion due to the uncertainty of the biologic nature of the lesion, namely: Fibrous Xanthoma, Plasma Cell Granuloma, Pesudosarcoma, Lymphoid Hamartoma, Myoxid Hamartoma, Inflammatory Pseudotumor and Benign Myofibroblastoma. Inflammatory Myofibroblastic Pseudotumor occurs throughout the body, most frequently in the peritoneal cavity, retroperitoneal space and lungs, among extra pulmonary lesions 15% accounts for the head and neck region. Most commonly, they are located in the region of the orbit. Inflammatory Myofibroblastic tumor presentation in maxillary sinus is rare and has an invasive potential. We have presented a case report of Inflammatory Myofibroblastic tumor of maxillary sinus, correlating Clinical, radiographic, and histopathologic along with immunohistochemical profile to arrive at a diagnosis, with special emphasis on the disputed nosology of this lesion and the latest therapeutic update.

Keywords: Benign, immunohistochemical, nosology, uncertainty


How to cite this article:
Sangeetha R, Jayashree G, Narmatha N, Kailasam S. Offbeat phenomenon in antrum of highmore – Inflammatory myofibroblastic tumor. J Indian Acad Oral Med Radiol 2020;32:86-9

How to cite this URL:
Sangeetha R, Jayashree G, Narmatha N, Kailasam S. Offbeat phenomenon in antrum of highmore – Inflammatory myofibroblastic tumor. J Indian Acad Oral Med Radiol [serial online] 2020 [cited 2020 May 30];32:86-9. Available from: http://www.jiaomr.in/text.asp?2020/32/1/86/282616




   Introduction Top


The Inflammatory Myofibroblastic Tumor (IMT) is a space-occupying lesion of unknown etiology, which clinically behaves as an aggressive lesion with extensive destruction but considered to be benign reactive process.[1] It was recognized as an intermediate mesenchymal tumor by the World Health Organization (WHO).[2] Inflammatory Myofibroblastic Pseudotumor occurs throughout the body, most frequently in the lungs. It rarely occurred in maxillary sinus as a non-specific mass growing over a period of months or years.[3],[4] The diagnosis was difficult in this scenario and was based on exclusion, where multiple biopsies were often required. The histopathological diagnosis played a key role in definitive diagnosis, characterized by myofibroblastic and fibroblastic spindle cells with diverse presentations, so various descriptive terms were given, namely: Fibrous Xanthoma, Plasma cell Granuloma, Pesudosarcoma, Lymphoid Hamartoma, and Myxoid Hamartoma.[5] Complementary radiographic investigations have helped to know the extent of the lesion. The treatment of choice was complete surgical resection and corticosteroid therapy. In this article, we have reported a case of Inflammatory Pseudotumor of right maxillary sinus with discussion aimed at better understanding the diagnosis, treatment, and prognosis of head and neck IMTs.


   Case Report Top


A 40-year-old man was presented to the outpatient department with pain in the upper right back tooth region for past 2 days, accompanied by swelling on the right side of the face. History of his presenting illness elaborated the pain was sudden in onset; sharp and continuous in nature after which the swelling developed, causing discomfort during mastication and he was esthetically compromised. Patient's dental history revealed that he had underwent extraction one month before, which was uneventful. On extra oral examination, a diffuse swelling was evident extending from the right ala of the nose to 1 cm away from tragus anteroposteriorly, superoinferiorly it extended from the infraorbital margin till corner of the mouth [Figure 1]. On palpation, the swelling was soft in consistency, tender and skin over the swelling was normal. On intra oral examination there was obliteration of buccal sulcus in relation to 15, 16, 17, 18 region with a sinus opening tract of 4 mm in diameter was evident with pus discharge mixed with blood in relation to 16, 17 regions [Figure 2]. There was no tenderness evident on palpation. Grade 1 mobility was present in 15, 16 tooth region. On correlating the history of previous dental extraction in 17 region and the clinical examination, it was provisionally diagnosed as oro-antral fistula. The clinical differential diagnosis was osteomyelitis, salivary gland neoplasms, carcinoma of maxillary sinus and odontogenic tumors.
Figure 1: Extra oral examination revealing facial asymmetry

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Figure 2: Intra oral examination showing a sinus opening in relation to 16, 17 region

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Later investigations were advised. The Orthopantomograph (OPG) revealed haziness of right maxillary sinus [Figure 3], which is suggestive of inflammation of maxillary sinus due to infection. Computed tomography (CT) showed hyper dense mass in relation to right maxillary sinus with erosion of the right posterolateral wall extending into buccal and infratemporal space [Figure 4](a) and (b)]. The radiological differential diagnosis was based on the sinus opacification with bone erosion and adjacent soft tissue infiltration of ill-defined mass this mimics that of a malignant process, so squamous cell carcinoma of maxillary sinus, adenoid cystic carcinoma of salivary gland and deep fungal infections like Mucormycosis can be considered.
Figure 3: Orthopantomograph showing haziness of right maxillary sinus

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Figure 4: (a) Computed tomography – Axial section showing hyper dense mass in relation to right maxillary sinus with erosion of the right posterolateral wall. (b) Computed tomography – Coronal section showing lesion extending into buccal and infratemporal space

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Incisional biopsy was done in relation to right buccal vestibule, showing moderately cellular tumor with interlacing dense bundles of spindle cells interspread by clusters of adipose cells and branching capillaries. The vascularity was increased with scattered arterioles. The spindle cells were strap like or thin with plump to oval nuclei. Mitosis was low, these were suggestive of low grade spindle tumor with features of inflammatory pseudotumor [Figure 5], then immunohistochemistry was done for markers CD 31 [Figure 6](a)], S 100 [Figure 6](b)], SMA [Figure 6](c)], Vimentin [Figure 6](d)], those features were compatible with inflammatory myofibroblastic tumor.
Figure 5: Histopathology section showing moderately cellular tumor with interlacing dense bundles of spindle cells with adipose cells and branching capillaries

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Figure 6: (a) Immunohistochemistry of CD 31, (b) Immunohistochemistry of S100, (c) Immunohistochemistry of SMA, (d) Immunohistochemistry of Vimentin

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On correlating the clinical features, radiographic investigation and histopathological investigation, final diagnosis of inflammatory myofibroblastic tumor of right maxillary sinus was given. The lesion was surgically excised along with partial maxillectomy [Figure 7] and obturator was advised. Patient was reviewed after 6 months for Post Prosthetic management [Figure 8].
Figure 7: Surgically excised specimen

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Figure 8: Post-Operative, corrected facial deformity

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   Discussion Top


Inflammatory Myofibroblastic Tumor was first described by Brunn in 1937, which represents a spectrum of myofibroblastic proliferation that includes reactive benign to malignant lesions.[5] They have been detected most commonly in the lung, they also occur in the head and neck region with an incidence of 15%.[6] The most commonly involved head and neck area are the orbit, oral cavity, pharynx, larynx, and esophagus. The maxillary sinus was very rarely involved. Pathologically, it consisted of multitude of cell types like myofibroblastic cells, fibroblastic cells, and inflammatory cells, which include plasma cells and eosinophils. Due to this nature of the lesion, a variety of names were used to describe this disease in the literature.[2],[5]

The Etiology of Inflammatory Myofibroblastic Tumor was not clear. Multiple theories have been postulated for the development of this tumor. Perrone et al. considered that adjacent necrosis or infection could be the inciting foci.[5] Even the role of viral infectious agents such as Epstein–Barr virus and human herpes virus-8 have been discussed in the literature. Autoimmunity has also been considered as an etiological factor. Chronic infection or inflammation leading to differentiation of plasma cells was another theory.[6] In our case, chronic infection due to the improper healing of the extraction site may be considered as the foci of Inflammatory Myofibroblastic Tumor.

Clinically, it was easily misinterpreted as malignant epithelial or mesenchymal spindle cell neoplasms because of its complicated pathologic characteristics like Bony destruction and invasion to adjacent site, so the patient was subjected to multiple biopsies to arrive at this diagnosis. In maxillary sinus involvement, it presented with loco regional features such as nasal obstruction, epistaxis, cheek swelling and facial deformity, similar to the clinical presentation of the case reported here. Numbness due to infraorbital nerve involvement, proptosis and diplopia due to orbital involvement and decreased vision due to optic nerve were the late complications.[4],[5],[7]

CT, MRI, and PET-CT were not Definitive diagnostic tool, they only aid in identifying the extent of the lesion. Histological analysis of IMT tumor was based on myofibroblasts and inflammatory cells.[5],[6] Fuji et al. have classified the tumor into three subsets based on the varied quantity of myofibroblasts and the inflammatory cells: Dominant lymphoplasmocytic infiltrate; dominant lymphohistiocytic infiltrate, young and active myofibroblastic process and predominately collagenized process with lymphocytic infiltrate.[8] Immunohistochemically, the tumor expresses Vimentin and SMA. Antiactin antibody positivity denotes its origin in the muscle. It was immunohistochemically negative to CD68, keratin, desmin, S-100, and caldesmon.[7]

Differential diagnosis of rhinoscleroma, Wegener's granulomatosis, fibrous histiocytoma fibromatosis, and soft tissue malignancies such as spindle cell sarcoma and myxofibrosarcoma needed to be considered due to the erosive properties of the lesion.[7],[8],[9] Occasionally, the histological appearance of the tumor may resemble plasmacytoma due to the presence plasma cells.[5]

The treatment options depended on the anatomical site, tumor morphology and histopathogical features. The mainstay of treatment modality was complete surgical excision and even medical management can be another treatment option for tumors which are difficult to access.[10] Medical treatment includes corticosteroid and chemotherapeutic agents like paclitaxel. If the tumor component was mainly of fibrotic tissue, it is more refractory to steroid treatment, and increase of inflammatory component makes the tumor more sensitive to corticosteroid. Recurrence was reported to be rare with a rate of less than 20%.[9]

In conclusion, Inflammatory Myofibroblastic Tumor of the maxillary sinus was a benign and extremely rare lesion that had been treated successfully in the majority of the cases. The main problem was that they can be easily confused clinically and radio graphically with a malignant process, so a poignant observation and investigation is required. We had described a patient with IMT of right maxillary sinus which was very rare occurrence, showed histological features that correlated with IMT and strong positivity for Vimentin and SMA, while focal faint positivity for ALK -1, which confirmed the diagnosis. The lesion was surgically excised and there was no sign of recurrence after 5 months of follow up. Though the case reported had features similar to literature description, the knowledge about this rare lesion was very limited which made the diagnosis part a hectic one, therefore this case report was aimed at the awareness of this lesion to avoid mishap in the future.

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.

Acknowledgements

The authors thank Dr. Meera Govindarajan, R and D Histopathology Lab, who provided insight, expertise, and histopathogical pictures that greatly assisted this article.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.



 
   References Top

1.
Karakök M, Ozer E, Sari I, Mumbuç S, Aydin A, Kanlikama M, et al. Inflammatory myofibroblastic tumor (inflammatory pseudotumor) of the maxillary sinus mimicking malignancy: A case report of an unusual location (is that a true neoplasm?). Auris Nasus Larynx 2002;29:383-6.  Back to cited text no. 1
    
2.
Kichenaradjou A, Barrett AW, Norris P, Rowell N, Newman L. Inflammatory pseudotumor of the maxilla. Oral Maxillofac Surg 2016;20:309-12.  Back to cited text no. 2
    
3.
Sultana J, Bashar A, Kamal M, Morol S. Inflammatory myofibroblastic tumor, an unusual presentation in maxilla and paranasal sinuses: Review of literature and a case report. J Dent Health Oral Disord Ther 2017;8:00277.  Back to cited text no. 3
    
4.
Thomas L, Uppal HS, Kaur S, David VC. Inflammatory pseudotumor of the maxillary sinus presenting as a sino-nasal malignancy. Eur Arch Otorhinolaryngol 2005;262:61-3.  Back to cited text no. 4
    
5.
Andrade NN, Mathai PC, Kamil R, Aggarwal N. Inflammatory myofibroblastic tumor: A case report and a clinical update. J Oral Biol Craniofac Res 2017;7:219-22.  Back to cited text no. 5
    
6.
Lazaridou M, Dimitrakopoulos I, Tilaveridis I, Iordanidis F, Kontos K. Inflammatory myofibroblastic tumor of the maxillary sinus and the oral cavity. Oral Maxillofac Surg 2014;18:111-4.  Back to cited text no. 6
    
7.
Palaskar S, Koshti S, Maralingannavar. Inflammatory myofibroblastic tumor. Contemp Clinc Dent 2011;2:274-7.  Back to cited text no. 7
    
8.
Surbhi, Metgud R, Naik S, Patel S. Spindle cell lesions: A review on immunohistochemical markers. J Can Res Ther 2017;13:412-8.  Back to cited text no. 8
    
9.
Ghosh A, Bandopadhyay A, Sarkar R. Low-grade myofibroblastic sarcoma of maxillary sinus and buccal mucosa: Two rare cases and review of the literature. Indian J Pathol Microbiol 2019;62:119-21.  Back to cited text no. 9
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10.
Kim JS, Hong KH, Kim JS, Song JH. Medical therapy of maxillary sinus inflammatory myofibroblastic tumors. Am J Otolaryngol 2016;37:376-8.  Back to cited text no. 10
    


    Figures

  [Figure 1], [Figure 2], [Figure 3], [Figure 4], [Figure 5], [Figure 6], [Figure 7], [Figure 8]



 

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