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Annals of DIAGNOSTIC PATHOLOGY VOL 5, NO 4 AUGUST 2001 ORIGINAL ARTICLES Nodular Fasciitis of the External Ear Region: A Clinicopathologic Study of 50 Cases Lester D.R. Thompson, MD, Julie C. Fanburg-Smith, MD, and Bruce M. Wenig, MD Nodular fasciitis (NF), uncommon in the auricular area, is a benign reactive myofibroblastic proliferation that may be mistaken for a neoplastic proliferation. Fifty cases of NF of the auricular region were identified in the files of the Otorhinolaryngic-Head and Neck Tumor Registry of the Armed Forces Institute of Pathology. The patients included 22 females and 28 males, aged 1 to 76 years (mean, .(49 ؍ years). The patients usually presented clinically with a mass lesion (n 27.4 or (28 ؍ Five patients recalled antecedent trauma. The lesions were dermal (n ,in those cases where histologic determination was possible (11 ؍ subcutaneous (n ؍ measuring 1.9 cm on average. The majority of the lesions were circumscribed (n 38), composed of spindle-shaped to stellate myofibroblasts arranged in a storiform growth pattern, juxtaposed to hypocellular myxoid tissue-culture-like areas with extravasation of erythrocytes. Dense, keloid-like collagen and occasional giant cells .Mitotic figures (without atypical forms) were readily identifiable .(18 ؍ were seen (n By immunohistochemical staining, myofibroblasts were reactive with vimentin, ac- tins, and CD68. All patients had surgical excision. Four patients (9.3%) developed local recurrence and were alive and disease free at last follow-up. All patients with were alive or had died of unrelated causes, without evidence of (43 ؍ follow-up (n disease an average 13.4 years after diagnosis. Nodular fasciitis of the auricular area occurs most often in young patients. Because NF is more often dermally situated than extremity NF, it may present with superficial ulceration and/or bleeding. Local recurrence is more frequent because of the difficulty in obtaining complete surgical excision around the ear. Ann Diagn Pathol 5: 191-198, 2001. This is a US government work. There are no restrictions on its use. Index Words: Nodular fasciitis, ear, auricular region, differential diagnosis, immu- nohistochemistry, myofibroblasts From the Departments of Endocrine and Otorhinolaryngic-Head & Neck Pathology and Soft Tissue Pathology, Armed Forces Institute of Pathology, Washington, DC. Presented at the 87th Annual Meeting of the United States and Canadian Academy of Pathology, Boston, MA, February 28 – March 7, 1998. The opinions or assertions contained herein are the private views of the authors and are not to be construed as official or as reflecting the views of the Department of Defense. Address reprints requests to Lester D.R. Thompson, MD, Department of Endocrine & Otorhinolaryngic-Head & Neck Pathology, Building 54, Room G066-11, Armed Forces Institute of Pathology, 6825 16th St, NW, Washington, DC 20306-6000. This is a US government work. There are no restrictions on its use. 1092-9134/01/0504-0001$0.00/0 doi:10.1053/adpa.2001.26969 Annals of Diagnostic Pathology, Vol 5, No 4 (August), 2001: pp 191-198 191 192 Thompson, Fanburg-Smith, and Wenig Table 1. Auricular Nodular Fasciitis Immunohistochemical Panel Antibody Primary Antibody Company Dilution Cellular Conditioning Keratin cocktail (AE1/AE3 mm Boehringer Mannheim 1:50 Protease treatment and CK1) Biochemicals, Indianapolis, 1:200 IN, and Dako, Carpinteria, CA Smooth muscle actin mm Sigma Immuno Chemicals, 1:8,000 None St Louis, MO S-100 protein rp Dako 1:800 None Glial fibrillary acidic protein rp Dako 1:2,000 Protease treatment XIIIA rp Calbiochem, San Diego, CA 1:1,000 Protease treatment Vimentin mm Biogenex, San Ramon, CA 1:800 Microwave pretreatment Muscle specific actin mm Enzo, Farmingdale, NY 1:80 None Desmin mm Dako 1:100 Protease treatment HMB-45 mm Dako 1:100 None CD34 mm Dako 1:40 Microwave pretreatment KP-1 mm Dako 1:200 Protease treatment Abbreviations: mm, mouse monoclonal; rp, rabbit polyplonal. ODULAR fasciitis (NF) is generally regarded status and was available for 43 patients. This clinical Nas a benign, reactive, tumor-like proliferation investigation was conducted in accordance and compli- of myofibroblasts that typically occurs in the ex- ance with all statutes, directives, and guidelines of the tremities. The head and neck region is a common Code of Federal Regulations Title 45, Part 46, and the location for NF, particularly in children, but NF of Department of Defense Directive 3216.2 relating to hu- the auricular region is rare. Therefore, we under- man subjects in research. Hematoxylin-eosin–stained slides from all patients took this study in order to more completely define were reviewed for morphologic assessment to meet the the clinical, histologic, and immunophenotypic established diagnostic criteria for NF.1 Further, all cases features of NF of the ear region and to compare had to primarily involve the auricular region, specifically these findings with NF occurring in more usual defined for this study as ear, not further specified, im- locations. mediately preauricular, postauricular, infraauricular, au- ricular (pinna), or the external auditory canal (lateral to Materials and Methods the tympanic membrane). Immunophenotypic analysis Fifty patients with a diagnosis of auricular NF were was performed in 32 cases (64%) with suitable material identified in the files of the Otorhinolaryngic-Head & by using the standardized avidin-biotin method of Hsu et 2 Neck Pathology Registry at the Armed Forces Institute of al using 4 m-thick, formalin fixed, paraffin embedded Pathology from the years 1970 to 1990. These 50 cases sections. Table 1 documents the pertinent, commercially were identified in a review of 2,930 (1.9%) benign and available immunohistochemical antibody panel used. malignant auricular neoplasms and soft tissue reactive The analysis was performed on a single representative conditions seen in consultation during the same refer- block in each case. When required for cellular condition- ence period. Forty-four cases were obtained from civilian ing, proteolytic antigen retrieval was performed by pre- sources, five from military hospitals, and one from a digestion for 3 minutes with 0.05% protease VIII (Sigma Veterans Administration medical center. Chemical Co, St Louis, MO) in 0.1-mol/L concentration Materials within the Institute’s files were supple- of phosphate buffer, pH of 7.8, at 37°C. Antigen en- mented by a review of the patient demographics (gen- hancement (recovery) was performed as required by der, age), symptoms at presentation (including dura- using formalin fixed, paraffin embedded tissue that was tion), and past medical history (specifically, a history of treated with a buffered citric acid solution and heated for trauma). In addition, we reviewed surgical pathology and 20 minutes in a calibrated microwave oven. Afterwards, operative reports and obtained follow-up information the sections were allowed to cool at room temperature in from oncology data services by written questionnaires or a citric acid buffer solution for 45 minutes before con- direct communication with the treating physician or the tinuing the procedure. Standard positive controls were patient. Follow-up data included the exact location, size, used throughout, with serum used as the negative con- treatment modalities, and current patient and disease trol. While we anticipated positivity for vimentin, for the Nodular Fasciitis of the External Ear 193 Table 2. Auricular Nodular Fasciitis: Mean Size and Three of these patients identified the use of a Duration of Symptoms by Location cotton tipped applicator in the ear canal before the Duration of development of an external auditory canal lesion, Symptoms whereas an additional patient was struck by a belt Anatomic Site Number Size (cm) (mos) buckle in the preauricular region. The remaining Ear, not otherwise specified 5 2.0 2.2 patient described an injury of uncertain nature to External auditory canal 6 1.3 1.6 the preauricular region. The duration of symptoms Pinna 2 2.3 4.0 ranged from a few days to 36 months, with a mean Preauricular 18 1.8 4.4 of 5 months. While, on average, there was a shorter Posterior auricular 19 2.1 7.7 duration of symptoms for patients with external Total 50 1.9 5.0 auditory canal lesions (1.6 months) when com- pared with the average for all locations combined (5.0 months) (Table 2), this difference was not statistically significant. actins and KP-1 we chose S-100 protein and HMB-45 to rule out desmoplastic melanomas, glial fibrillary acidic Contributing Diagnoses protein to rule out neural and adnexal tumors, cytoker- atin to rule out epithelial lesions, CD34 to rule out The most frequent misdiagnoses for the ear NF dermatofibrosarcoma protuberans and Kaposi’s sarcoma cases were (in order of frequency) dermatofibro- (because of the similar extravasated red blood cells), and sarcoma protuberans, fibrosarcoma, leiomyosar- factor XIIIa for the reactivity of passenger cells in many coma, malignant fibrous histiocytoma, proliferative fibrohistiocytic lesions in the differential diagnosis. fasciitis, inflammatory pseudotumor, fibromatosis, Categorical variables were analyzed using chi-square fibrous histiocytoma, and benign peripheral nerve tests to compare observed and expected frequency dis- sheath tumors. Clinical impressions included ne- tributions, except when small sample size (less than five) crotizing fasciitis, perichondritis, and squamous indicated the use of Fisher’s exact test. Comparison of means between groups were made with unpaired t-tests cell carcinoma with ulceration. or one-way analysis of variance, depending on whether there were two or more groups. Multiple comparisons Treatment and Follow-up were analyzed using the Tukey method. Confidence in- All patients were treated surgically, with or with- tervals of 95% were generated for all positive findings. out complete removal of the lesion. Follow-up data The alpha level was set at P Ͻ .05. All statistical analysis was performed with the computer software package, was available in 43 patients. Of these 43 patients, 41 Statistical Package for the Social Sciences 8.0 for PC were alive and two had died of unrelated causes (SPSS; Chicago, IL).