Inflammatory myofibroblastic tumor involving ear lobule

Inflammatory myofibroblastic tumor involving ear lobule

Auris Nasus Larynx 39 (2012) 631–633 Contents lists available at SciVerse ScienceDirect Auris Nasus Larynx journal homepage: www.elsevier.com/locate...

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Auris Nasus Larynx 39 (2012) 631–633

Contents lists available at SciVerse ScienceDirect

Auris Nasus Larynx journal homepage: www.elsevier.com/locate/anl

Inflammatory myofibroblastic tumor involving ear lobule Kyu Hwan Jung a, Yong-Wan Kim a, Yoon Kyoung So a, Soo-Im Choi b, Moo Jin Baek a,* a

Department of Otorhinolaryngology – Head and Neck Surgery, Haeundae Paik Hospital, Inje University College of Medicine, 1435, Jwa dong, Haeudae-gu, Busan 612-030, Republic of Korea b Department of Pathology, Haeundae Paik Hospital, Inje University College of Medicine, 1435, Jwa dong, Haeudae-gu, Busan 612-030, Republic of Korea

A R T I C L E I N F O

A B S T R A C T

Article history: Received 12 July 2011 Accepted 20 January 2012 Available online 15 February 2012

We present herein an extremely rare case of an inflammatory myofibroblastic tumor (IMT) of the ear lobule with its management. A 50-year-old woman presented with a wart-like mass between the ear lobule and the facial skin. She had been accidentally lacerated her left ear lobule and visited our clinic. The mass had been incidentally found by the patient 1 year before the trauma and growing slowly without pain. Surgical excision and primary closure was performed. Histopathologic examination demonstrated ill-defined margined nodular proliferation of spindle cells in deep dermis with focal stromal hyalinization and lymphoplasmacytic infiltration compatible with the IMT. The patient showed no evidence of recurrence 6 months after surgery. To our knowledge, this is the first report of an IMT occurred in the external ear. Auricular IMT of our case was not aggressive in clinical nature and treated optimally with surgical excision. ß 2012 Elsevier Ireland Ltd. All rights reserved.

Keywords: Inflammatory pseudotumor Ear auricle

1. Introduction Inflammatory myofibroblastic tumor (IMT) is an uncommon fibro-inflammatory lesion, interchangeably used as inflammatory pseudotumor, that occurs most frequently in the lung, abdominal cavity, retroperitoneum, and extremities, and its occurrence in the head and neck region is less common [1]. IMT is diagnosed on the basis of the pathology and described as distinctive lesion composed of myofibroblastic spindle cells accompanied by an inflammatory infiltrate of plasma cells, lymphocytes, and eosinophils. IMT has been recognized under variety of names, such as inflammatory pseudotumor, plasma cell granuloma, pseudosarcomatous myofibroblastic proliferation, myofibroblastoma, xanthomatous pseudotumor, and atypical fibromyxoid nodule. IMT involving the ear have been reported to be relatively rare and mostly in the temporal bone with or without extension to the skull base [2]. To our knowledge, this is the first report of IMT involving the auricle. We described here the clinical feature, histopathologic finding, and treatment outcome. 2. Case report The patient was a 50-year-old woman who had accidentally slipped down and bumped into the door with her left ear. She was

* Corresponding author. Tel.: +82 51 797 0636/2291; fax: +82 51 797 2304. E-mail address: [email protected] (M.J. Baek). 0385-8146/$ – see front matter ß 2012 Elsevier Ireland Ltd. All rights reserved. doi:10.1016/j.anl.2012.01.008

lacerated the ear lobule by the accident and visited nearby local clinic. The patient also presented with a solitary poor-circumscribed wart-like cutaneous mass at the laceration site and referred to our clinic the next day. The mass was 1 cm  1 cm firm tender mobile tumorous lesion with subtle inflammation sign of redness and exudation crust around the lesion (Fig. 1). The mass had been incidentally found by the patient between the ear lobule and the facial skin a year before the trauma and growing slowly without pain. External ear canal and middle ear was normal and no lymph node was palpable in the head and neck region. Other general otorhinolaryngologic examination discovered nothing abnormal. CT or MRI was not conducted because she refused and the mass was clinically considered to be localized benign mass. Her blood and urine test results were within normal ranges and plain chest X-ray revealed no abnormal findings. Serologic screening tests including hepatitis B and C, HIV, and syphilis were normal. Surgical excisional biopsy and primary closure of her ear lobule and facial skin was performed under local anesthesia. The mass was excised completely without difficulty. Histopathologic examination demonstrated ill-defined margined nodular proliferation of spindle cells without significant nuclear atypia in deep dermis with focal stromal hyalinization and lymphoplasmacytic infiltration (Fig. 2). Occasional mitotic figures are found but without atypical mitosis. Immunohistochemical stains resulted in diffuse strong positive reaction of spindled tumor cells to smooth muscle actin along with vimentin and negative reaction to desmin, S-100, CD34 and c-kit. A diagnosis of IMT was confirmed based on these histopathologic findings. The patient showed no evidence of recurrence 6 months after surgery (Fig. 3).

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K.H. Jung et al. / Auris Nasus Larynx 39 (2012) 631–633

Fig. 1. Gross findings of the auricular lesion. Anatomical location and size of the auricular mass before surgery (A), a wart-like inflammatory cutaneous mass at the laceration site between ear lobule and preauricular skin (B).

Fig. 2. Microscopic examination revealed an ill-defined margined proliferation of spindle cells in dermis (A, H&E, 40). Area of collagen deposit (black star) (B, H&E, 100), and lymphoplasmacytic infiltration are noted among spindle cells without nuclear atypia or atypical mitotic figures (C, H&E, 200). The spindled tumor cells are positive for smooth muscle actins (D, IHC for SMA).

3. Discussion Various patterns of myofibroblastic proliferation with a prominent infiltration of inflammatory cells determine the histopathologic characteristic of the IMT [1]. IMT was established as a distinct tumor entity in 1994 by the WHO classification [3]. Some pathologists insisted that IMT is a definite tumor that occurs in the lung, upper aerodigestive airways, abdomen, pelvis, and retroperitoneum of children and adolescents, despite its morphological and immunohistochemical overlapping with Inflammatory pseudotumor (IPT) [4]. However, IPT is still used alternatively to the IMT. The term IPT is generally accepted to be used for a wide range of fibro-myofibroblastic lesions including xanthomatous pseudotumor (xanthogranuloma), plasma cell granuloma or pseudotumor, histiocytoma, inflammatory fibromyxoid tumor, pseudosarcomatous myofibroblastic proliferation, and inflammatory myofibroblastic proliferation [5].

IMT was reported to occur throughout the body. Pulmonary IMT was the first reported case and most commonly involved site of the lesion. Although IMT occurs at diverse extrapulmonary locations, lesions in the head and neck are extremely rare. Orbit was the most frequently reported head and neck site of involvement. The remaining sites in the head and neck were paranasal sinuses, infratemporal fossa, facial soft tissues, parotid gland, nasopharynx, pterygopalatine fossa, larynx, cervical esophagus, and in the skull base with dominantly involving meninges [6,7]. There were not many reports of IMT involving the ear. Almost reported cases were temporal bone IMT or IPT with or without skull base extension [2]. Although two cases were confined to the middle ear, most cases were involving mastoid and middle ear cavity with local aggressiveness of neural and inner ear destruction [2,7,8]. However, there was no report of IMT confined to the external ear. To our knowledge, this is the first case report of the IMT involving the auricle.

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Surgery is primarily considered for many cases depending on tumor location and behavior. High dose oral steroid can be used for single modality or combined with surgical resection. Some lesions, such as orbital IMTs, showed good response for primary radiotherapy, with up to 75% showing a reduction of mass [14]. Tumors with good response to corticosteroids are thought to be the most appropriate for radiotherapy. However, there are documented reports of local recurrence up to 15%, an incident that can be reflecting inadequate resection of the lesion or tumors with behavior nearest to inflammatory fibrosarcoma [15]. Coffin et al. also showed that IMT can be a cause of death because of uncontrolled local growth [1]. Auricular IMT of our case was not aggressive in clinical nature and treated optimally with surgical excision only. 4. Conclusion We present the first IMT case involving the ear lobule. Complete surgical excision can be considered for the initial treatment for the similar cases. If resection is not possible, corticosteroid or radiotherapy could be considered. Fig. 3. There was no evidence of recurrence at postoperative 6 months.

The mass of our case was located at the junctional skin crease of the ear lobule and the facial skin. The mass was reviewed by two specialized pathologists and found separated from the parotid tissue and definitely involving the ear lobule. Histologically, IMTs are composed of myofibroblastic spindle cells that are mixed with a prominent infiltrate of lymphocytes, plasma cells, and acute inflammatory cells [1]. There are three histologic types as follows; (1) a myxoid, vascular and inflammatory proliferation resembling granulation tissue or nodular fasciitis; (2) compact spindle cells with inflammation resembling a fibrous histiocytoma or a fibromatosis; and (3) dense plate-like collagen which resembles a desmoid tumor or a scar. In our case, an ill-defined margined proliferation of spindle cells was found with mixed infiltration of lymphoplasma cells and collagen deposit. The etiology of IMT still remains unknown. Some are considering IMT to be an inflammatory, reactive lesion because of the histologic findings as mixed inflammatory cells, the lack of atypia in the spindle cells, the polyclonality of the plasma cells, and the feature that the stromal cells consist predominantly of myofibroblasts [9]. Others insist that IMT is a true neoplasm, because most IMTs were reported to occur spontaneously and found recurrent rearrangement of ALK (anaplastic lymphoma kinase) locus on chromosome 2p23 and monoclonality in some cases [10,11]. Sethi et al. reported a case of postaural IMT similar to our case [12]. The patient was 12-year-old boy with postauricular trauma. Cancerous condition was initially suspected because the mass had grown rapidly during 2 weeks without pain. However, surgical excision revealed the mass to be an IMT. The mass of our case was not caused by a trauma but accidentally presented to the physician by a trauma. Clinical presentation of soft tissue IMT generally includes growing mass with pain and weight loss and mostly depends on the site of involvement. Presenting symptoms of temporal bone IMT were otalgia, otorrhea, headache, facial palsy, hearing loss, vertigo, and rarely visual changes. Our case of auricle IMT was a solitary growing mass without any symptom. There are controversies over the optimal treatment modality of IMT including surgery, radiotherapy and corticosteroids [13].

Conflict of interest None. Acknowledgment Dr. Moo Jin Baek takes responsibility for the integrity of the content of the paper. References [1] Coffin CM, Watterson J, Priest JR, Dehner LP. Extrapulmonary inflammatory myofibroblastic tumor (inflammatory pseudotumor). A clinicopathologic and immunohistochemical study of 84 cases. Am J Surg Pathol 1995;19:859–72. [2] Ajibade DV, Tanaka IK, Paghdal KV, Mirani N, Lee HJ, Jyung RW. Inflammatory pseudotumor (plasma cell granuloma) of the temporal bone. Ear Nose Throat J 2010;89:E1–3. [3] Coindre JM. Histologic classification of soft tissue tumors (WHO, 1994). Ann Pathol 1994;14:426–7. [4] Vecchio GM, Amico P, Grasso G, Vasquez E, La Greca G, Magro G. Posttraumatic inflammatory pseudotumor of the breast with atypical morphological features: a potential diagnostic pitfall. Report of a case and a critical review of the literature. Pathol Res Pract 2011;207:322–6. [5] Ryan GB, Cliff WJ, Gabbiani G, Irle C, Montandon D, Statkov PR, et al. Myofibroblasts in human granulation tissue. Hum Pathol 1974;5:55–67. [6] Chen YF, Zhang WD, Wu MW, Ou-Yang D, Zhang Q. Inflammatory myofibroblastic tumor of the head and neck. Med Oncol 2011;28(Suppl 1):S349–53. [7] Lee D-H, Shin O-R, Cho K-J, Kim J-H. Inflammatory pseudotumor in the middle ear cavity. Int J Pediat Otorhinolaryngol 2008;72:1569–72. [8] Lee R, Weber D, Ness A, Wasman J, Megerian C. Inflammatory pseudotumor of the middle ear masquerading as Bell’s palsy. Am J Otolaryngol 2007;28:423–6. [9] Cho K, Lee D, Jung S, Kim J. A case of an inflammatory myofibroblastic tumor of the mastoid presenting with chronic suppurative otitis media. Auris Nasus Larynx 2007;34:523–6. [10] Coffin CM, Patel A, Perkins S, Elenitoba-Johnson KS, Perlman E, Griffin CA. ALK1 and p80 expression and chromosomal rearrangements involving 2p23 in inflammatory myofibroblastic tumor. Mod Pathol 2001;14:569–76. [11] Su LD, Atayde-Perez A, Sheldon S, Fletcher JA, Weiss SW. Inflammatory myofibroblastic tumor: cytogenetic evidence supporting clonal origin. Mod Pathol 1998;11:364–8. [12] Sethi A, Malhotra V, Sethi D, Nigam S. Postaural inflammatory pseudotumor: an extremely unusual complication of trauma in a child. Ear Nose Throat J 2011;90:108–11. [13] Williamson RA, Paueksakon P, Coker NJ. Inflammatory pseudotumor of the temporal bone. Otol Neurotol 2003;24:818–22. [14] Orcutt JC, Garner A, Henk JM, Wright JE. Treatment of idiopathic inflammatory orbital pseudotumours by radiotherapy. Br J Ophthalmol 1983;67:570–4. [15] Dimitrakopoulos I, Psomaderis K, Iordanidis F, Karakasis D. Inflammatory myofibroblastic tumor of the maxillary sinus: a case report. J Oral Maxillofacial Surg 2007;65:323–6.