Ear, Nose & Throat Journal2023, Vol. 102(6) 409–411© The Author(s) 2021Article reuse guidelines:sagepub.com/journals-permissionsDOI: 10.1177/01455613211006006journals.sagepub.com/home/ear
AbstractNodular fasciitis (NF) has a low incidence, many classification subtypes and large histological variations. We describe a 17-month-old child who presented with a mass in the right cavity of auricular concha that was successfully cured by 2 procedures. In pediatric patients with fibroproliferative lesions, the possibility of NF should be excluded to avoid misdiagnosis and overtreatment.
Keywordsnodular fasciitis, ear, pathology, children
A 17-month-old infant was found to have a mass that was approximately the size of a rice grain in the right cavity of the auricular concha. The patient did not have pain, bleeding, or other discomfort, but the mass gradually increased in size more than 4 months prior. On September 18, 2018, the patient underwent resection of the external ear lesion and free flap transplantation in the right ear under general anesthesia in a local hospital. Pathology showed spindle cell lesions. The combined clinical and immunohistochemical findings were consistent with nodular fasciitis (NF).
However, the mass gradually increased to approximately 1.0 cm * 1.5 cm in size more than 1 month after the operation. On November 11, 2018, the patient was seen in the Department of Traditional Chinese Medicine at another hospital and received an external application of Musk for 1 day, without obvious effects.
The patient visited the outpatient department of our hospital on November 12, 2018. The physical examination showed a dark red mass with a size of 1.0 cm by 1.0 cm in the cavity of the auricular concha of the right ear (Figure 1A). At this time, immunohistochemistry was performed using the pathological section from the first operation at another hospital to consider the possibility of fibrosarcoma. A hematology consultant evaluated the disease condition (Figure 1B), and the patient was administered AVCP chemotherapy; however, no significant improvement in the mass size was observed after 1 week of chemotherapy (Figure 1C). The patient was transferred to our department for surgical treatment after communicating the disease condition to the family.
On December 10, 2018, the patient underwent right auricular mass resection and free flap implantation under general anesthesia. During the operation, the mass was found to posteriorly invade the cartilage, and the cartilage and the mass in the cavity of the auricular concha were completely removed (Figure 2A). The mass tissue, upper resection margin, lower resection margin, anterior resection margin, posterior resection margin, and bottom resection margin were sent for pathology. The skin was harvested from the anteromedial thigh and then grafted on the right concha defect site, aligned, sutured, and compressed with a Vaseline gauze. The postoperative pathological results included a fascicular or woven arrangement of spindle cells, occasional mitoses, stromal vascular proliferation, and local collagen fiber proliferation (Figure 3). Immunohistochemical staining was positive for Vim, SMA, and actin and negative for Des, S100, ALK, and CD34. In addition, the Ki-67 proliferation index was 8%. At the same time, it was reported that a small amount of the cartilage and fibrous tissue were observed under the microscope in the marginal tissue, but no tumor tissue was observed. These pathological findings were consistent with NF. After the surgery, the patient was administered Rocephin intravenously to prevent infection, and wound dressing changes were performed to facilitate wound healing. The Vaseline gauze was removed from the right concha 2 weeks after surgery, and the skin graft showed a ruddy complexion. There was no recurrence after 1 year of follow-up (Figure 2B).
Nodular fasciitis was first described in 1955 by Konwaler et al.1 Nodular fasciitis is a self-limiting, benign, fibroblastic, and myofibroblastic rapid proliferative disorder that can occur at any age but is predominantly found in young adults, aged 20 to 40 years, and less commonly in pediatric patients. These lesions are widely distributed and can be observed in the head and neck, trunk, and extremities.2-4 Nodular fasciitis clinically manifests as a rapidly growing nodular mass, which may be associated with pain, may have a red, pink, or mixed color and is generally less than 4.0 cm.5 In this case, the main symptom of the child was a rapid enlargement of the mass in the auricular cavity. Thompson et al6 found auricular NF in 1.5% of all NF cases and in 1.9% of all auricular lesions (including benign and malignant lesions). Magnetic resonance imaging (MRI) examination showed a round mass of approximately 0.93 cm × 2.6 cm × 2.8 cm in size in the right ear, with an abnormal signal that appeared iso-intense on the T1-weighted sequence and hyperintense on the T2-weighted sequence (Figure 4A and B). The MRI findings were consistent with those reported in the literature.2,7
Nodular fasciitis lesions can be classified as subcutaneous, fascial, or intramuscular depending on the site of the occurrence.8 In this case, the patient presented with a subcutaneous mass. According to microscopic findings, these lesions can be divided into myxoma, granuloma, and fibroma types, the morphology of which can change during the course of the disease.9 In the present case, the histopathological findings were consistent with previous reports.5,7,8,10 The etiology of NF is not clear, and current reports have found a rearrangement in the USP6 gene on chromosome 17, implying the neoplastic nature of the disease.11-13 Some scholars have suggested that in the appropriate clinical context, the detection of USP6 gene rearrangements is very useful for the diagnosis of NF and can reduce the rate of misdiagnosis.12
Nodular fasciitis should be differentiated from fibromatosis, fibrosarcoma, malignant fibrous histiocytoma, and other diseases. Misdiagnosis rate of this disease has been reported in the literature to be approximately 75%, and NF is often misdiagnosed as a malignant disease.4,14 The main reasons are as follows: (1) the course of the disease is fast, and the pathological features are difficult to distinguish; (2) there are many histological variations in the classified subtypes; (3) the disease is rare, and the number of cases is low. Furthermore, clinicians, imaging experts, and pathologists have insufficient experience in diagnosing this disorder, which easily leads to misdiagnosis. Both pathological findings and clinical features must be considered in the diagnosis.
Surgical resection is the main treatment for this disease. The main reason for NF recurrence is incomplete surgical resection, which can be corrected by reoperation. In pediatric patients, the differential diagnosis of a malignancy, such as through the presence of spindle cells in the tissue, active cell proliferation, a large number of mitotic figures, and inflammatory cell infiltration, is not preferred. In pediatric patients with fibroproliferative lesions, the possibility of NF should be excluded to avoid misdiagnosis and overtreatment.
The author(s) declared no potential conflicts of interest with respect to the research, authorship, and/or publication of this article.
The author(s) received no financial support for the research, authorship, and/or publication of this article.
Xiaojun Liu https://orcid.org/0000-0002-2958-6290
Xiaoyan Li https://orcid.org/0000-0003-1342-1640
1 Department of Otolaryngology-Head and Neck Surgery, Shanghai Children’s Hospital, Shanghai Jiao Tong University, Shanghai, China
Received: February 23, 2021; revised: February 23, 2021; accepted: March 08, 2021
Corresponding Author:Xiaoyan Li, MD, PhD, Department of Otolaryngology-Head and Neck Surgery, Shanghai Children’s Hospital, Shanghai Jiao Tong University, Shanghai, 200062, China.Email: submissionent@163.com
