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Inflammatory myofibroblastic tumour of the jaw: A rare presentation
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JORMAS-659; No. of Pages 5 J Stomatol Oral Maxillofac Surg xxx (2018) xxx–xxx
Available online at
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Case Report
Inflammatory myofibroblastic tumour of the jaw: A rare presentation Luis Monteiro a, Manar Alhassani b,*, Tiago Resende a, Rui Albuquerque b a
Department of Medicine and Oral Surgery and Institute of Research and Advanced Training in Health Sciences and Technologies (IINFACTS), University Institute of Health Sciences (IUCS-N), CESPU, 4585-116 Paredes, Portugal b Department of Oral Medicine, Dental Institute, Guy’s and St Thomas’ NHS Foundation Trust/King’s College London, London, United Kingdom
A R T I C L E I N F O
A B S T R A C T
Historique de l’article : Received 12 February 2019 Accepted 21 February 2019
Inflammatory Myofibroblastic Tumour (IMT) is a rare entity of unknown aetiology and pathogenesis. It was initially described in the lung, although there have been reported cases affecting extra-pulmonary sites. The aetiology of IMT remains unclear, with current evidence suggesting that IMTs are neoplastic processes resulting from chromosomal translocations that often cause an overexpression of ALK tyrosine kinase. Histologically, a variably cellular myxoid morphology is common in these tumours, with the presence of inflammatory cell infiltrate. Clinically, symptoms are non-specific, although the presence of a swelling is frequently reported. Imaging studies are not very useful in the diagnosis, as they only describe the presence of a mass, and do not provide a definitive diagnosis. This article presents a case, of Inflammatory Myofibroblastic Tumour of the jaw, in a male patient aged 16 years.
C 2019 Elsevier Masson SAS. All rights reserved.
1. Introduction Inflammatory Myofibroblastic Tumour (IMT) was first observed in 1939, by Brunn [1], a number of terms have been given to this entity, including Inflammatory Myofibroblastic Sarcoma, Inflammatory Fibrosarcoma and Inflammatory Pseudotmour [2]. The term Myofibroblastic Inflammatory Tumour is now the most recent and commonly used. In 2013, the World Health Organization classification of soft tissue and bone defined IMT as a distinctive neoplasm composed of myofibroblastic and fibroblastic spindle cells accompanied by an inflammatory infiltrate of plasma cells, lymphocytes, and/or eosinophils [3]. Its aetiology remains unclear, although various pathogeneses have been suggested. Factors such as trauma, association with the Epstein–Barr virus, chromosomal translocation (2p23), and the ALK gene have been identified as possible causes [4,5,6]. Exposure to radiotherapy may also be related to the onset of the development of this type of pathology [7]. With regard to epidemiology, its incidence is rare. It affects a wide range of adults, with children and young adults more frequently affected. There is also thought to be a slight female * Corresponding author at: Dental Core Trainee in Oral Medicine, Department of Oral Medicine, Floor 22, Guy’s Tower, London, United Kingdom. E-mail address: [email protected] (M. Alhassani).
predominance. The lung and liver are the most common sites for IMT [1,8,9]. It rarely presents in the head and neck [10,11] however it can occur in the aero-digestive tract, major salivary glands and soft tissues of the neck. IMT is a rare neoplasm composed of a variable number of inflammatory and myofibroblastic cells. The myofibroblastic cells are usually spindled, ovate or stellate. Inflammatory cell infiltrate is commonly seen, with lymphocytes and plasma cells predominating [12]. The clinical presentation of IMT is non-specific and very diverse; it typically presents as a well-defined swelling that slowly progresses over a period of months or years [13]. Features of IMT include a tendency for local recurrence, and a small risk of distant metastasis [14]. IMT may mimic a malignant process both clinically and radiographically; therefore, accurate diagnosis is essential in order to deliver the most appropriate treatment. It should be noted that IMT has a potential for aggressive progression, vascular invasion and local recurrence [15,16]. Although the incidence of IMT in the maxillofacial region is rare, accurate diagnosis is valuable in distinguishing these lesions from their more serious competing differential diagnoses, as well as providing appropriate management. This article aims to present a case of IMT in the oral cavity and provide an updated literature review of current cases of IMT in the oral cavity.
https://doi.org/10.1016/j.jormas.2019.02.017 C 2019 Elsevier Masson SAS. All rights reserved. 2468-7855/
Please cite this article in press as: Monteiro L, et al. Inflammatory myofibroblastic tumour of the jaw: A rare presentation. J Stomatol Oral Maxillofac Surg (2019), https://doi.org/10.1016/j.jormas.2019.02.017
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JORMAS-659; No. of Pages 5 L. Monteiro et al. / J Stomatol Oral Maxillofac Surg xxx (2018) xxx–xxx
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Case report A 16-year-old male patient was referred by his dentist to the Oral Medicine department at the Valongo Hospital, Portugal, regarding a two-month history of a swelling in the upper left quadrant. The patient reported feeling "a mass in the jaw", which reduced after taking ibuprofen prescribed by his dentist. Medically, he was asthmatic and had no known drug allergies. He was a nonsmoker and reported no alcohol consumption. On examination, a 2 cm by 2 cm, mobile, sessile swelling was noted on the buccal mucosa adjacent to the upper left first molar (Fig. 1). The overlying mucosa was normal and there was no associated bleeding or suppuration. The upper left six was previously root treated. Extraoral examination was unremarkable with no facial swellings or asymmetry (Fig. 2). The initial differential diagnoses were fibroma,
peripheral ossifying fibroma, peripheral giant cell granuloma, haemangioma, lipoma and IMT. In the first instance, an incisional biopsy of the swelling was carried out. The biopsy revealed a well-defined, nodular lesion composed of fusiform cells with histiocyte and lymphoplasmic inflammatory infiltrate; no mitoses or necroses being identified. These characteristics were in keeping with the diagnosis of IMT. Given the biopsy findings, a decision was made to excise the lesion. The lesion was excised using peripheral osteotomy, and the
Fig. 4. Excised specimen, measuring 3 1.2 0.7 cm.
Fig. 1. Intra-oral view of the swelling in the buccal sulcus, adjacent to the upper first molar.
Fig. 2. Extra-oral view, no swelling can be seen and the overlying skin is normal.
Fig. 3. A full thickness muco-periosteal incision into the buccal sulcus revealed the mass, before excision.
Fig. 5. A. Hematoxylin and Eosin (H&E) stain, original magnification 40, lowpower microscopic view. Showing a chronic inflammatory infiltrate with spindle and polygonal cells in a fibrous connective tissue. B. H&E stain, original magnification 100, high-power microscopic view. Showing a chronic inflammatory infiltrate with spindle and polygonal cells in a fibrous connective tissue.
Please cite this article in press as: Monteiro L, et al. Inflammatory myofibroblastic tumour of the jaw: A rare presentation. J Stomatol Oral Maxillofac Surg (2019), https://doi.org/10.1016/j.jormas.2019.02.017
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JORMAS-659; No. of Pages 5 L. Monteiro et al. / J Stomatol Oral Maxillofac Surg xxx (2018) xxx–xxx
Fig. 6. Complete healing and no evidence of recurrence, 6 months post-op.
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upper left six was also extracted (Fig. 3). Co-Amoxiclav, lepicortinol and ibuprofen were prescribed postoperatively. Gross examination of the specimen revealed a 3 1.2 0.7 cm tissue mass with an irregular surface and brownish color weighing one gram (Fig. 4). This was sent for histopathological analysis. Histological examination revealed the presence of fibrous connective tissue and granulation tissue, indicative of an inflammatory process; bone trabeculae with a reactive aspect were also identified. In the periphery, striated muscle tissue with areas of necrosis were seen (Fig. 5). Immunohistochemical analysis was positive for the expression of CD34 and vimentin, and did not reveal expression of ALK, S100, Actin or CD1A. These features were compatible with, and supportive of the initial diagnosis of IMT. Therefore, definitive diagnosis of IMT was made.
Table 1 Author and year
Age (years)
Gender
Location
Size (cm)
Follow-up (with no signs of recurrence)
Listonet al., 1981 [5]
4 2 6 44 77 20 36 68 43 29 27 – 23 23 82 42 4 2 6 44 77 20 36 68 43 27 29 23 23 82 33 20 52 23 29 14 14 31 31 60 40 24 14 11 75 36 70 27 22 11 16
F F M M F M F F F F M – M M F F F F M M M F M F F F M F – M M F M F M F F F F M F M M F F M M F M F M
Buccal mucosa Buccal mucosa Buccal mucosa Buccal mucosa Buccal Mucosa Buccal mucosa Maxilla Buccal mucosa Retro-molar gingivae Hard palate Tongue Tongue Mandible Retro-molar gingivae Mandible Mandible Buccal mucosa Buccal mucosa Buccal mucosa Mandible Buccal mucosa Buccal mucosa Maxilla Buccal mucosa Retro-molar gingivae Tongue Hard Palate Mandible Mandible Mandible Mandible Mandible Neck Floor of mouth Maxilla Gingivae Mandible Tongue Gingivae Buccal mucosa Maxilla Maxilla Tongue Tongue Buccal mucosa Maxilla Retro-molar Mandible Mandible Mandible Buccal Mucosa
45 35 45 Not specified 1.5 2 Not specified 0.5 0.6 1.0 2.3 1.8 1.8 1.7 Not specified 1 2.5 4 5 55 3.0 45 35 45 Not specified 1.5 2 Not specified 56 1 2.3 1.7 1.8 1.8 1 2.5 4 5 55 322 – 433 3 5 32 32 Not specified Not specified Not specified 1.5 1.1 0.7 Not specified Not specified Not specified Not specified 7 4.5 3 34 543 Not specified Not specified 3 1.2 0.7
6 months 10 months Not specified 2 years Not specified 13 months 13 months Not specified 1 year Not specified Not specified Not specified Not specified 6 months 18 months 6 months 6 months 10 months 1 month 2 years 28 months 13 months 13 months Not specified 1 year Not specified Not specified Not specified 6 months 18 months 28 months 22 months 4 months 2 years 6 years 10 years Not specified 18-48 months
Earlet al., 1993 [14] Ramachandraet al., 1995 [5] Shecket al., 1995 [2] Ide et al., 1998 [4] Cable et al., 2000 [17] Ide et al., 2000 [18] Pankajet al., 2001 [19] Jordanet al., 2003 [20] Fanget al., 2004 [21] Brookset al., 2004 [8] Poh et al., 2004 [22] Brokkset al., 2005
Johann et al., 2008 [23] Oh et al., 2008 [24] Ma et al., 2008 Xavier et al., 2009 [25] Eleyet al., 2010 [26] Satomiet al., 2010 [27] Salgueiro et al., 2011
Binmandiet al., 2011 Kim et al., 2011 Lourenc¸o et al., 2012 Rautavaet al., 2013 Lazaridouet al., 2013 Rahmanet al., 2014 Niteshet al., 2015 Alkindiet al., 2016 Korlepara et al, 2017 Tateishi et al, 2016 Present Case
4 months 15 months 1 year Not specified 1 year 6 months Not specified Not specified Not specified 18 months 6 months
Please cite this article in press as: Monteiro L, et al. Inflammatory myofibroblastic tumour of the jaw: A rare presentation. J Stomatol Oral Maxillofac Surg (2019), https://doi.org/10.1016/j.jormas.2019.02.017
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There was no evidence of recurrence after 6 months of follow up and the patient remains asymptomatic (Fig. 6).
collected from the literature review show that intra-oral IMT occurs most frequently in the buccal mucosa (15) followed by the mandible (13) and maxilla (6). None of the cases showed any signs of recurrence at follow up.
2. Discussion IMT is a soft tissue mesenchymal tumour with low malignant potential. IMT has been described by various names, including inflammatory pseudotumour, plasma cell granuloma or benign myofibroblastoma, thus describing its heterogenic nature [8,9]. IMT rarely occurs outside of the lung or liver, with very few such cases described in the literature [8]. Occurrence in the oral cavity is extremely rare, with only few cases described [17]. Intra-orally, it presents as a firm, hard lesion, with rare intra – osseous presentation. It does not cause systemic symptoms. IMTs located in the oral cavity are generally found in children and young adults, as in the case presented here [12]. Obtaining a final diagnosis represents a challenge, in which the use of imaging studies (ultrasound or tomography) is limited, since they only describe the presence of a tumour, its shape, dimensions and neighboring structures. The definitive diagnosis is only achieved through histopathological study and immunohistochemistry. Immunohistochemistry may reveal positivity for cytokeratin, vimentin, anaplastic lymphoma kinase (ALK) and actin [5]. Although this case revealed a negative result for alpha-actin, there have been have been other reported cases of IMT which were actin negative [18,19]. From the histological point of view, IMT is composed of a variable mixture of myofibroblastic spindle cells with chronic inflammatory cells. The presence of atypical mitoses is rare. The stroma is mostly myxoid or collagenous, with prominent vascularization. The differential diagnoses of IMT at histologic level is extensive, and includes benign or malignant spindle cell tumours, such as nodular fasciitis, solitary fibrous tumour, benign fibrous histocystoma, amongst many others [8]. The pathogenesis of IMT is unknown and presents some controversy. Despite this, some hypotheses are raised, suggesting that the lesion is of infectious, autoimmune or traumatic origin. Scientific reports suggest that the presence of IMT in the liver may be associated with inflammatory bowel disease, Papillon-Lefevre syndrome, severe congenital neutropenia or leukemia [1,20,21,22,23]. HIV infection has also been associated with IMT in several organs [17,24]. Evidence of the role of Epstein-barr virus has also been noted in some cases of IMT in the liver, spleen and lymph nodes [17,24,25]. IMTs exhibit unpredictable clinical behaviour, although there is no evidence of malignant transformation [1]. Evidence of recurrence of these tumours is reported in 3% to 24% of cases [26], therefore complete surgical excision is essential, as is close monitoring after exicison [1]. However, there is no evidence of recurrence, malignant transformation or metastasis reported with oral IMTs [8]. The treatment of choice for IMTs is usually surgical excision. Besides surgical excision, the use of radiotherapy has been reported in cases of non-resectable lesions, non-responsive IMTs and as an adjuvant in cases of IMT with expression of ALK-1 and Ki67. However, radiotherapy with low dose intervals has been associated with the risk of sarcomatous transformation or may lead to a secondary malignant tumour [27]. Medical management, including local administration of corticosteroids has also been referred to as a treatment option, with the proposed mechanism of action being the reduction of inflammatory cell infiltrate present in these type of lesions, however this has not been shown to be effective in IMT of the head and neck [1,8]. Table 1 provides a review of 51 known cases of IMT in the oral cavity, including our case. The majority of patients with IMT were female (27 F, 22 M) with an average age of 33 (2-82). Data
3. Conclusion IMT in the oral cavity is a rare condition, with little information regarding its aetiology and pathogenesis. Complete excision of the lesion is the standard procedure when treating these cases, as has been demonstrated in this case. Post-operative follow-up of patients after surgical excision is fundamental, and to date no recurrence, malignant transformation or metastasis of IMTs in the oral cavity has been reported [8]. Albeit rare, the clinician should be aware of these lesions in order to differentiate them from their more serious, differential diagnoses. Disclosure of interest The authors declare that they have no competing interest.
References [1] Narla LD, Newman B, Spottswood SS, Narla S, Koll IR. Inflamatory pseudotumor. Radiographics 2003;23:719–29. [2] Matthew RL. Diagnostic Pathology; Soft Tissue Tumors. 2nd Ed. Philadelphia. Elsevier 2016;1:88–193. [3] Fletcher CDM, Bridge JA, Hogendoorn PCW, Mertens F. World Health Organization classification of tumours soft tissue and bone, 4th ed, Lyon: IARC Press; 2013. p. 83–4. [4] Alaani A, Hogg H, Warfield AT, et al. Air bag injury as a cause of inflammatory myofibroblastic pseudotumor of the subglottic larynx progressing to myositis ossificans. Acta Otolaryngol 2005;125(6):674–7. [5] Guilemany JM, Alos L, Alobid I, BernalSprekelsen M, Cardesa A. Inflammatory myofibroblastic tumor in the larynx: clinicopathologic features and histogenesis. Acta Otolaryngol 2005;125(2):215–9. [6] Rodrigues M, Taylor RJ, Sun CC, Wolf JS. Inflammatory myofibroblastc tumor of the larynx in a 2 year old male. ORL J Otorhinolaryngol Relat Spec 2005;67(2):101–5. [7] Flavio C, Limp Boa Vida M, Bernardo Duarte B, Akira Sakae F, Antonio Monteiro Marone S. Inflammatory myofibroblastic tumor: a rare laryngeal case. Braz J Otorhinolaryngol 2010;76(2):271. [8] Shek AW, Wu PC, Samman N. Inflammatory pseudotumour of the mouth and maxilla. J Clin Pathol 1996;49:164–7. [9] Van Weert S, Manni JJ, Driessen A. Inflammatory myofibroblastic tumor of the parotid gland: a case report and review of the literature. Acta Otolaryngol 2005;125:433–7. [10] Wenig BM, Devaney K, Bisceglia M. Inflammatory myofibroblastic tumor of the larynx. A clinicopathologic study of eight cases simulating a malignant spindle cell neoplasm. Cancer 1995;76(11):2217–29. [11] Sivanandan S, Lodha R, Agarwala S, Sharma M, Kabra SK. Inflammatory myofibroblastic tumor of the trachea. Pediatr Pulmonol 2007;42(9):847–50. [12] Theunisse HJ, van den Hoogen FJ. Airway obstruction due to inflammatory myofibroblastic tumor of the posterior pharyngeal wall. J Laryngol Otol 2011;125(6):655–9. [13] Constance A, Griffin AL, Hawkins CD, et al. Recurrent involvement of 2p23 in inflammatory myofibroblastic tumors. Cancer Res 1999;59:2776–80. [14] Gleason BC, Hornick JL. Inflammatory myofibroblastic tumours: where are we now? J Clin Pathol 2008;61(4):428–37. [15] Makhlouf HR, Sobin L. Inflammatory myofibroblastic tumors of the gastrointestinal tract: how closely are they related to inflammatory fibroid polyps? Hum Pathol 2002;33:307–15. [16] Avinash K, Vinod CH, Faram D. Inflammatory pseudotumor of ascending colon presenting as PUO: a case report. Indian J Surg 2004;66:234–6. [17] Binmadi NO, Packman H, Papadimitriou JC, Scheper M. Oral inflammatory myofibroblastic tumor: case report and review of literature. Open Dent J 2011;5:66–70. [18] Qui X, Montgomory E, Sun B. Inflammatory myofibroblastic tumor and lowgrade myofibroblastic sarcoma: a comparative study of clinicopathologic features and further observations on the immunohistochemical profile of myofibroblasts. Hum Pathol J 2008;39(6):846–56. [19] Yang P, Hirose T, Seki K, et al. Myofibroblastic tumor of soft tissue displaying desmin-positive and actin-negative immunophenotypes. Pathol Int J 1996;46(9):696–703. [20] Brooks JK, Nikitakis NG, Frankel BF, et al. Oral inflammatory myofibroblastic tumor demonstrating ALK, p53, MDM2, CDK4, pRb, and Ki-67 immunoreactivity in an elderly patient. Oral Surg Oral Med Oral Pathol Oral Radiol Endod 2005;99:716–26.
Please cite this article in press as: Monteiro L, et al. Inflammatory myofibroblastic tumour of the jaw: A rare presentation. J Stomatol Oral Maxillofac Surg (2019), https://doi.org/10.1016/j.jormas.2019.02.017
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JORMAS-659; No. of Pages 5 L. Monteiro et al. / J Stomatol Oral Maxillofac Surg xxx (2018) xxx–xxx [21] Tajima K, Kosugi S, Fuyama S, et al. Multiple inflammatory pseudotumors of the liver associated with acute myeloblastic leukemia. Intern Med 1998;37:542–5. [22] Hsiao CC, Chen CL, Eng HL. Inflammatory pseudotumor of the liver in Kostmann’s disease. Pediatr Surg Int 1999;15:266–9. [23] Czauderna P, Sznurkowska K, Korzon M, et al. Association of inflammatory pseudotumor of the liver and Papillon-Lefevre syndrome-case report. Eur J Pediatr Surg 1999;9:343–6. [24] Braun B, Cazorla A, Rivas C, et al. Inflammatory pseudotumor of the spleen in a patient with human immunodeficiency virus infection: a case report and review of the literature. Ann Hematol 2003;82:511–4.
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[25] Kutol JL, Pinkus GS, Dorfman DM, et al. Inflammatory pseudotumor of lymph node and spleen: an entity biologically distinct from inflammatory myofibroblastic tumor. Hum Pathol 2001;32:1382–7. [26] Pettinato G, Manivel JC, De Rosa N, Dehner LP. Inflammatory myofibroblastic tumor. Clinicopathologic study of 20 cases with immunohistochemical and ultratructural observations. Am J Clin Pathol 1990;94(5):538–46. [27] Ong HS, Ji T, Zhang CP, et al. Head and neck inflammatory myofibroblastic tumor: evaluation of clinicopathologic and prognostic features. Oral Oncol 2012;48:141–8.
Please cite this article in press as: Monteiro L, et al. Inflammatory myofibroblastic tumour of the jaw: A rare presentation. J Stomatol Oral Maxillofac Surg (2019), https://doi.org/10.1016/j.jormas.2019.02.017
JORMAS-659; No. of Pages 5 J Stomatol Oral Maxillofac Surg xxx (2018) xxx–xxx
Available online at
ScienceDirect www.sciencedirect.com
Case Report
Inflammatory myofibroblastic tumour of the jaw: A rare presentation Luis Monteiro a, Manar Alhassani b,*, Tiago Resende a, Rui Albuquerque b a
Department of Medicine and Oral Surgery and Institute of Research and Advanced Training in Health Sciences and Technologies (IINFACTS), University Institute of Health Sciences (IUCS-N), CESPU, 4585-116 Paredes, Portugal b Department of Oral Medicine, Dental Institute, Guy’s and St Thomas’ NHS Foundation Trust/King’s College London, London, United Kingdom
A R T I C L E I N F O
A B S T R A C T
Historique de l’article : Received 12 February 2019 Accepted 21 February 2019
Inflammatory Myofibroblastic Tumour (IMT) is a rare entity of unknown aetiology and pathogenesis. It was initially described in the lung, although there have been reported cases affecting extra-pulmonary sites. The aetiology of IMT remains unclear, with current evidence suggesting that IMTs are neoplastic processes resulting from chromosomal translocations that often cause an overexpression of ALK tyrosine kinase. Histologically, a variably cellular myxoid morphology is common in these tumours, with the presence of inflammatory cell infiltrate. Clinically, symptoms are non-specific, although the presence of a swelling is frequently reported. Imaging studies are not very useful in the diagnosis, as they only describe the presence of a mass, and do not provide a definitive diagnosis. This article presents a case, of Inflammatory Myofibroblastic Tumour of the jaw, in a male patient aged 16 years.
C 2019 Elsevier Masson SAS. All rights reserved.
1. Introduction Inflammatory Myofibroblastic Tumour (IMT) was first observed in 1939, by Brunn [1], a number of terms have been given to this entity, including Inflammatory Myofibroblastic Sarcoma, Inflammatory Fibrosarcoma and Inflammatory Pseudotmour [2]. The term Myofibroblastic Inflammatory Tumour is now the most recent and commonly used. In 2013, the World Health Organization classification of soft tissue and bone defined IMT as a distinctive neoplasm composed of myofibroblastic and fibroblastic spindle cells accompanied by an inflammatory infiltrate of plasma cells, lymphocytes, and/or eosinophils [3]. Its aetiology remains unclear, although various pathogeneses have been suggested. Factors such as trauma, association with the Epstein–Barr virus, chromosomal translocation (2p23), and the ALK gene have been identified as possible causes [4,5,6]. Exposure to radiotherapy may also be related to the onset of the development of this type of pathology [7]. With regard to epidemiology, its incidence is rare. It affects a wide range of adults, with children and young adults more frequently affected. There is also thought to be a slight female * Corresponding author at: Dental Core Trainee in Oral Medicine, Department of Oral Medicine, Floor 22, Guy’s Tower, London, United Kingdom. E-mail address: [email protected] (M. Alhassani).
predominance. The lung and liver are the most common sites for IMT [1,8,9]. It rarely presents in the head and neck [10,11] however it can occur in the aero-digestive tract, major salivary glands and soft tissues of the neck. IMT is a rare neoplasm composed of a variable number of inflammatory and myofibroblastic cells. The myofibroblastic cells are usually spindled, ovate or stellate. Inflammatory cell infiltrate is commonly seen, with lymphocytes and plasma cells predominating [12]. The clinical presentation of IMT is non-specific and very diverse; it typically presents as a well-defined swelling that slowly progresses over a period of months or years [13]. Features of IMT include a tendency for local recurrence, and a small risk of distant metastasis [14]. IMT may mimic a malignant process both clinically and radiographically; therefore, accurate diagnosis is essential in order to deliver the most appropriate treatment. It should be noted that IMT has a potential for aggressive progression, vascular invasion and local recurrence [15,16]. Although the incidence of IMT in the maxillofacial region is rare, accurate diagnosis is valuable in distinguishing these lesions from their more serious competing differential diagnoses, as well as providing appropriate management. This article aims to present a case of IMT in the oral cavity and provide an updated literature review of current cases of IMT in the oral cavity.
https://doi.org/10.1016/j.jormas.2019.02.017 C 2019 Elsevier Masson SAS. All rights reserved. 2468-7855/
Please cite this article in press as: Monteiro L, et al. Inflammatory myofibroblastic tumour of the jaw: A rare presentation. J Stomatol Oral Maxillofac Surg (2019), https://doi.org/10.1016/j.jormas.2019.02.017
G Model
JORMAS-659; No. of Pages 5 L. Monteiro et al. / J Stomatol Oral Maxillofac Surg xxx (2018) xxx–xxx
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Case report A 16-year-old male patient was referred by his dentist to the Oral Medicine department at the Valongo Hospital, Portugal, regarding a two-month history of a swelling in the upper left quadrant. The patient reported feeling "a mass in the jaw", which reduced after taking ibuprofen prescribed by his dentist. Medically, he was asthmatic and had no known drug allergies. He was a nonsmoker and reported no alcohol consumption. On examination, a 2 cm by 2 cm, mobile, sessile swelling was noted on the buccal mucosa adjacent to the upper left first molar (Fig. 1). The overlying mucosa was normal and there was no associated bleeding or suppuration. The upper left six was previously root treated. Extraoral examination was unremarkable with no facial swellings or asymmetry (Fig. 2). The initial differential diagnoses were fibroma,
peripheral ossifying fibroma, peripheral giant cell granuloma, haemangioma, lipoma and IMT. In the first instance, an incisional biopsy of the swelling was carried out. The biopsy revealed a well-defined, nodular lesion composed of fusiform cells with histiocyte and lymphoplasmic inflammatory infiltrate; no mitoses or necroses being identified. These characteristics were in keeping with the diagnosis of IMT. Given the biopsy findings, a decision was made to excise the lesion. The lesion was excised using peripheral osteotomy, and the
Fig. 4. Excised specimen, measuring 3 1.2 0.7 cm.
Fig. 1. Intra-oral view of the swelling in the buccal sulcus, adjacent to the upper first molar.
Fig. 2. Extra-oral view, no swelling can be seen and the overlying skin is normal.
Fig. 3. A full thickness muco-periosteal incision into the buccal sulcus revealed the mass, before excision.
Fig. 5. A. Hematoxylin and Eosin (H&E) stain, original magnification 40, lowpower microscopic view. Showing a chronic inflammatory infiltrate with spindle and polygonal cells in a fibrous connective tissue. B. H&E stain, original magnification 100, high-power microscopic view. Showing a chronic inflammatory infiltrate with spindle and polygonal cells in a fibrous connective tissue.
Please cite this article in press as: Monteiro L, et al. Inflammatory myofibroblastic tumour of the jaw: A rare presentation. J Stomatol Oral Maxillofac Surg (2019), https://doi.org/10.1016/j.jormas.2019.02.017
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JORMAS-659; No. of Pages 5 L. Monteiro et al. / J Stomatol Oral Maxillofac Surg xxx (2018) xxx–xxx
Fig. 6. Complete healing and no evidence of recurrence, 6 months post-op.
3
upper left six was also extracted (Fig. 3). Co-Amoxiclav, lepicortinol and ibuprofen were prescribed postoperatively. Gross examination of the specimen revealed a 3 1.2 0.7 cm tissue mass with an irregular surface and brownish color weighing one gram (Fig. 4). This was sent for histopathological analysis. Histological examination revealed the presence of fibrous connective tissue and granulation tissue, indicative of an inflammatory process; bone trabeculae with a reactive aspect were also identified. In the periphery, striated muscle tissue with areas of necrosis were seen (Fig. 5). Immunohistochemical analysis was positive for the expression of CD34 and vimentin, and did not reveal expression of ALK, S100, Actin or CD1A. These features were compatible with, and supportive of the initial diagnosis of IMT. Therefore, definitive diagnosis of IMT was made.
Table 1 Author and year
Age (years)
Gender
Location
Size (cm)
Follow-up (with no signs of recurrence)
Listonet al., 1981 [5]
4 2 6 44 77 20 36 68 43 29 27 – 23 23 82 42 4 2 6 44 77 20 36 68 43 27 29 23 23 82 33 20 52 23 29 14 14 31 31 60 40 24 14 11 75 36 70 27 22 11 16
F F M M F M F F F F M – M M F F F F M M M F M F F F M F – M M F M F M F F F F M F M M F F M M F M F M
Buccal mucosa Buccal mucosa Buccal mucosa Buccal mucosa Buccal Mucosa Buccal mucosa Maxilla Buccal mucosa Retro-molar gingivae Hard palate Tongue Tongue Mandible Retro-molar gingivae Mandible Mandible Buccal mucosa Buccal mucosa Buccal mucosa Mandible Buccal mucosa Buccal mucosa Maxilla Buccal mucosa Retro-molar gingivae Tongue Hard Palate Mandible Mandible Mandible Mandible Mandible Neck Floor of mouth Maxilla Gingivae Mandible Tongue Gingivae Buccal mucosa Maxilla Maxilla Tongue Tongue Buccal mucosa Maxilla Retro-molar Mandible Mandible Mandible Buccal Mucosa
45 35 45 Not specified 1.5 2 Not specified 0.5 0.6 1.0 2.3 1.8 1.8 1.7 Not specified 1 2.5 4 5 55 3.0 45 35 45 Not specified 1.5 2 Not specified 56 1 2.3 1.7 1.8 1.8 1 2.5 4 5 55 322 – 433 3 5 32 32 Not specified Not specified Not specified 1.5 1.1 0.7 Not specified Not specified Not specified Not specified 7 4.5 3 34 543 Not specified Not specified 3 1.2 0.7
6 months 10 months Not specified 2 years Not specified 13 months 13 months Not specified 1 year Not specified Not specified Not specified Not specified 6 months 18 months 6 months 6 months 10 months 1 month 2 years 28 months 13 months 13 months Not specified 1 year Not specified Not specified Not specified 6 months 18 months 28 months 22 months 4 months 2 years 6 years 10 years Not specified 18-48 months
Earlet al., 1993 [14] Ramachandraet al., 1995 [5] Shecket al., 1995 [2] Ide et al., 1998 [4] Cable et al., 2000 [17] Ide et al., 2000 [18] Pankajet al., 2001 [19] Jordanet al., 2003 [20] Fanget al., 2004 [21] Brookset al., 2004 [8] Poh et al., 2004 [22] Brokkset al., 2005
Johann et al., 2008 [23] Oh et al., 2008 [24] Ma et al., 2008 Xavier et al., 2009 [25] Eleyet al., 2010 [26] Satomiet al., 2010 [27] Salgueiro et al., 2011
Binmandiet al., 2011 Kim et al., 2011 Lourenc¸o et al., 2012 Rautavaet al., 2013 Lazaridouet al., 2013 Rahmanet al., 2014 Niteshet al., 2015 Alkindiet al., 2016 Korlepara et al, 2017 Tateishi et al, 2016 Present Case
4 months 15 months 1 year Not specified 1 year 6 months Not specified Not specified Not specified 18 months 6 months
Please cite this article in press as: Monteiro L, et al. Inflammatory myofibroblastic tumour of the jaw: A rare presentation. J Stomatol Oral Maxillofac Surg (2019), https://doi.org/10.1016/j.jormas.2019.02.017
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There was no evidence of recurrence after 6 months of follow up and the patient remains asymptomatic (Fig. 6).
collected from the literature review show that intra-oral IMT occurs most frequently in the buccal mucosa (15) followed by the mandible (13) and maxilla (6). None of the cases showed any signs of recurrence at follow up.
2. Discussion IMT is a soft tissue mesenchymal tumour with low malignant potential. IMT has been described by various names, including inflammatory pseudotumour, plasma cell granuloma or benign myofibroblastoma, thus describing its heterogenic nature [8,9]. IMT rarely occurs outside of the lung or liver, with very few such cases described in the literature [8]. Occurrence in the oral cavity is extremely rare, with only few cases described [17]. Intra-orally, it presents as a firm, hard lesion, with rare intra – osseous presentation. It does not cause systemic symptoms. IMTs located in the oral cavity are generally found in children and young adults, as in the case presented here [12]. Obtaining a final diagnosis represents a challenge, in which the use of imaging studies (ultrasound or tomography) is limited, since they only describe the presence of a tumour, its shape, dimensions and neighboring structures. The definitive diagnosis is only achieved through histopathological study and immunohistochemistry. Immunohistochemistry may reveal positivity for cytokeratin, vimentin, anaplastic lymphoma kinase (ALK) and actin [5]. Although this case revealed a negative result for alpha-actin, there have been have been other reported cases of IMT which were actin negative [18,19]. From the histological point of view, IMT is composed of a variable mixture of myofibroblastic spindle cells with chronic inflammatory cells. The presence of atypical mitoses is rare. The stroma is mostly myxoid or collagenous, with prominent vascularization. The differential diagnoses of IMT at histologic level is extensive, and includes benign or malignant spindle cell tumours, such as nodular fasciitis, solitary fibrous tumour, benign fibrous histocystoma, amongst many others [8]. The pathogenesis of IMT is unknown and presents some controversy. Despite this, some hypotheses are raised, suggesting that the lesion is of infectious, autoimmune or traumatic origin. Scientific reports suggest that the presence of IMT in the liver may be associated with inflammatory bowel disease, Papillon-Lefevre syndrome, severe congenital neutropenia or leukemia [1,20,21,22,23]. HIV infection has also been associated with IMT in several organs [17,24]. Evidence of the role of Epstein-barr virus has also been noted in some cases of IMT in the liver, spleen and lymph nodes [17,24,25]. IMTs exhibit unpredictable clinical behaviour, although there is no evidence of malignant transformation [1]. Evidence of recurrence of these tumours is reported in 3% to 24% of cases [26], therefore complete surgical excision is essential, as is close monitoring after exicison [1]. However, there is no evidence of recurrence, malignant transformation or metastasis reported with oral IMTs [8]. The treatment of choice for IMTs is usually surgical excision. Besides surgical excision, the use of radiotherapy has been reported in cases of non-resectable lesions, non-responsive IMTs and as an adjuvant in cases of IMT with expression of ALK-1 and Ki67. However, radiotherapy with low dose intervals has been associated with the risk of sarcomatous transformation or may lead to a secondary malignant tumour [27]. Medical management, including local administration of corticosteroids has also been referred to as a treatment option, with the proposed mechanism of action being the reduction of inflammatory cell infiltrate present in these type of lesions, however this has not been shown to be effective in IMT of the head and neck [1,8]. Table 1 provides a review of 51 known cases of IMT in the oral cavity, including our case. The majority of patients with IMT were female (27 F, 22 M) with an average age of 33 (2-82). Data
3. Conclusion IMT in the oral cavity is a rare condition, with little information regarding its aetiology and pathogenesis. Complete excision of the lesion is the standard procedure when treating these cases, as has been demonstrated in this case. Post-operative follow-up of patients after surgical excision is fundamental, and to date no recurrence, malignant transformation or metastasis of IMTs in the oral cavity has been reported [8]. Albeit rare, the clinician should be aware of these lesions in order to differentiate them from their more serious, differential diagnoses. Disclosure of interest The authors declare that they have no competing interest.
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Please cite this article in press as: Monteiro L, et al. Inflammatory myofibroblastic tumour of the jaw: A rare presentation. J Stomatol Oral Maxillofac Surg (2019), https://doi.org/10.1016/j.jormas.2019.02.017