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Submental nodular fasciitis: report of an unusual case.

Abstract

A 30-year-old woman presented for evaluation of a hard, enlarging, submental mass that was fixed to the underlying mandible. Fine-needle aspiration cytology initially led to a diagnosis of pleomorphic adenoma, which was unusual given the superficial location of the lesion on computed tomography. The lesion was a well-defined rim-enhancing mass, and it had displaced adjacent structures medially. Intraoperatively, the mass was found to abut against and erode the mandibular cortex. A diagnosis of nodular fasciitis was established by histologic analysis. Such a location for this tumor is unusual. The prognosis for patients with this benign condition is excellent following complete surgical excision. We discuss the clinical presentation and the cytologic, histologic, and radiologic features of this uncommon entity.

Introduction

Nodular fasciitis is a benign proliferation of fibroblasts. It is an interesting condition as the lesion is often mistaken for a malignancy clinically and for a pleomorphic adenoma cytologically. The prognosis for patients with this condition is excellent following complete surgical excision of the mass.

We describe a case of nodular fasciitis of the submental area, and we discus the clinical presentation and cytologic, histologic, and radiologic features of this uncommon condition.

Case report

A 30-year-old woman presented to our ENT Department with a 1-month history of an enlarging submental mass. The mass was initially associated with pain, but it became asymptomatic thereafter. The patient denied any other symptoms. She reported no history of preceding trauma and no significant medical history at all.

On examination, a hard, 3-cm mass was palpated in the right submental area. The mass was fixed to the underlying mandible but not to the overlying skin. No other palpable neck mass or cervical lymphadenopathy was noted. Findings on the remainder of the ENT examination were normal.

The results of fine-needle aspiration cytology (FNAC) were suggestive of a pleomorphic adenoma. Computed tomography (CT) demonstrated a rim-enhancing subcutaneous spherical lesion with central hypodensity (figure 1). The mass abutted the inferior margin of the mandible in the anterior aspect of the right submental area. Medially, it abutted and displaced the adjacent anterior belly of the digastric and mylohyoid muscles.

The patient underwent an excisional biopsy under general anesthesia. Intraoperatively, a firm, well-circumscribed, 3.0 x 2.5-cm mass was found to be abutting and causing superficial erosion of the mandibular cortex (figure 2). The surgery was uneventful, and the patient recovered well. Histologic analysis established a diagnosis of nodular fasciitis (figure 3).

Discussion

Nodular fasciitis is a benign proliferation of fibroblasts with pseudosarcomatoid features. It was first described as a distinct entity by Konwaler et al in 1955; they called it pseudosarcomatous fibromatosis. (1) The benign nature of this entity is well established, but its pathogenesis remains uncertain. Proposed mechanisms include a reactive or inflammatory process, and antecedent trauma has been suggested as a possible inciting factor. (2-4) However, many patients do not report a history of local injury or trauma, as was the case with our patient.

[FIGURE 1 OMITTED]

The most common clinical presentation is an acute, rapidly enlarging solitary subcutaneous mass that may be associated with discomfort or tenderness. The presence of multiple nodules at presentation has been reported. (5) There are three subtypes of nodular fasciitis: subcutaneous, intramuscular, and intermuscular (fascial). The subcutaneous subtype is the most common. (2,6)

Clinically, it is easy to misdiagnose these lesions as a malignant process because of their rapid growth and often firm to hard consistency. In our case, the lesions fixation to the underlying mandible was a worrisome but ultimately misleading feature.

Most nodular fasciitis masses range in size from 1 to 3 cm; rarely do they exceed 4 cm. (2,6,7) The peak incidence of nodular fasciitis occurs between 20 and 40 years of age (range: 6 mo to 79 yr). (2,7) Its incidence in males and females is similar. (2) In adults, the most common location is the upper extremities, followed in decreasing order by the trunk, head and neck, and lower extremities. (2) In children, the head and neck region is the most common site. (8)

Diagnostic FNAC is often performed, especially for lesions in the head and neck. Cytologically, nodular fasciitis can be easily mistaken for a malignant neoplasm of spindle cells or a tumor of salivary gland origin. (9) In fact, nodular fasciitis is frequently thought to represent a pleomorphic adenoma, as occurred in our case.

Pleomorphic adenoma exhibits a wide spectrum of morphologic patterns, and thus it is susceptible to errors in cytologic interpretation. Features that are common to both nodular fasciitis and pleomorphic adenoma are the presence of dissociated spindle and plasmacytoid cells with central to eccentric nuclei, clumps of intercellular stromal material, and a myxoid background. (9)

[FIGURE 2 OMITTED]

Similarities notwithstanding, Jain et al reported subtle differences between nodular fasciitis and pleomorphic adenoma on FNAC. (9) In nodular fasciitis, epithelial cells are absent, cytoplasmic borders are indistinct, and nucleoli appear prominent. In pleomorphic adenoma, epithelial cells are present, cytoplasmic borders are well demarcated, and nucleoli are indistinct. Also, mitotic activity is frequent in nodular fasciitis and usually absent in pleomorphic adenoma (unless there is malignancy).

Macroscopically, nodular fasciitis manifests as a well-circumscribed, unencapsulated, spherical, tan to gray-white lesion. A pseudocapsule of connective tissue maybe present. (6) Microscopically, it appears as a spindle cell lesion composed of a proliferation of fibroblastic and myofibroblastic cells; sometimes the cells are closely packed, but usually they are loosely packed with surrounding myxoid or collagenous stroma. (6) Finger-like projections that invade into the surrounding soft tissue may be present. Variable numbers of inflammatory cells may also be present. Extravasation of red blood cells is a prominent feature of the histologic pattern. Depending on the amount and type of extracellular matrix present, nodular fasciitis can be classified into three subtypes: myxoid, cellular, and fibrous.

[FIGURE 3 OMITTED]

Nodular fasciitis is a distinct entity. It differs from other fibrous conditions such as fibromatosis, juvenile aponeurotic fibroma, subepidermal nodular fibrosis, and dermatofibrosarcoma protuberans. (6) The histologic differential diagnosis includes benign tumors such as fibrous histiocytomas, pyogenic granulomas, peripheral nerve sheath tumors, and cutaneous smooth-muscle tumors. Malignant tumors in the differential diagnosis include fibrosarcomas and spindle cell carcinomas. The presence of the vascular component (extravasation of red blood cells), mucinous changes, and inflammatory infiltrate helps differentiate nodular fasciitis from fibrosarcomas and other soft-tissue tumors. (10)

Radiologically, nodular fasciitis generally appears as a relatively well-defined superficial soft-tissue mass. (2) One exception is seen in the intramuscular subtype, in which tumors can be large, deep-seated, and ill-defined. Nodular fasciitis can demonstrate aggressive behavior, such as invasion and destruction of adjacent structures, including bone. (2) Lesions demonstrate moderate to strong enhancement with contrast and, depending on the predominant stromal components, they may appear as a solid or cystic mass with peripheral, nodular, or rim-like enhancement. (2) The cystic area within a lesion represents a fluid-filled mucoid space. Our patients lesion demonstrated rim enhancement; although bony erosion was not obvious on CT, it was evident intraoperatively.

On magnetic resonance imaging, lesions can demonstrate variable signal intensities; this is related to the variability in tumor cellularity and vascularity. (2,4) In general, lesions with myxoid or cellular histologic features exhibit signal intensities greater than those of muscle on T2-weighted imaging, and lesions with fibrous histology are markedly hypointense. Signal intensities of the cystic portion of the lesion are also variable due to differences in water and protein content, as well as the presence or absence of hemorrhage. (2)

Radiologically, the differential diagnosis includes neurogenic tumors, minor salivary gland tumors, dermoid or epidermoid tumors, hemangiomas, sarcoidosis, aggressive fibromatosis, dermatofibromas, fibrosarcomas, and malignant histiocytomas. (2,4)

Because nodular fasciitis is a benign lesion, successful treatment can be achieved by complete local excision, which usually results in permanent resolution. Recurrences are rare after adequate excision. (6,8)

In summary, we have presented an uncommon case of nodular fasciitis to raise awareness of this condition. This case is of interest not only to ENT surgeons, but also to pathologists and radiologists. We have highlighted the pitfalls encountered in making a cytologic diagnosis of this condition, and we have discussed the histologic findings.

Although there are no pathognomonic features radiologically, nodular fasciitis should be included in the differential diagnosis of a well-defined enhancing mass in a superficial and unusual location, especially in a young patient with a rapidly enlarging mass and a history of recent trauma to the area.

References

(1.) Konwaler BE, Keasbey L, Kaplan L. Subcutaneous pseudosarcomatous fibromatosis (fasciitis). Am J Clin Pathol 1955;25(3):241-52.

(2.) Kim ST, Kim HJ, Park SW, et al. Nodular fasciitis in the head and neck: CT and MR imaging findings. A JNR Am J Neuroradiol 2005;26 (10):2617-23.

(3.) Dahl I, Jarlstedt J. Nodular fasciitis in the head and neck. A clinicopathological study of 18 cases. Acta Otolaryngol 1980;90 (1-2): 152-9.

(4.) Shin JH, Lee HK, Cho KJ, et al. Nodular fasciitis of the head and neck: Radiographic findings. Clin Imaging 2003;27(l):31-7.

(5.) Hutter RV, Stewart FW, Foote FW Jr. Fasciitis. A report of 70 cases with follow-up proving the benignity of the lesion. Cancer 1962; 15: 992-1003.

(6.) Kleinstiver BJ, Rodriguez HA. Nodular fasciitis. A study of forty-five cases and review of the literature. J Bone Joint Surg Am 1968;50(6): 1204-12.

(7.) Bernstein KE, Lattes R. Nodular (pseudosarcomatous) fasciitis, a non-recurrent lesion: Clinicopathologic study of 134 cases. Cancer 1982;49(8):1668-78.

(8.) Vyas T, Bullock MJ, Hart RD, et al. Nodular fasciitis of the zygoma: A case report. Can J Plast Surg 2008;16(4):241-3.

(9.) Jain D, Khurana N, Jain S. Nodular fasciitis of the external ear masquerading as pleomorphic adenoma: A potential diagnostic pitfall in fine needle aspiration cytology. Cytojournal 2008;5:14.

(10.) Kamiya H, Araki M, Kitajima Y. Nodular fasciitis of the cheek. Eur J Dermatol 2003;13(2):189-91.

Jiun Fong Thong, MRCS(Eng); Tiong Yong Tan, FRCR(UK); Shi Wang, FRCPA(UK); Paul Mok, FRCS(Glasg); David Loke, FRCS(Edin)

From the Department of Otorhinolaryngology, Singapore General Hospital (Dr. Thong); the Department of Radiology, Changi General Hospital, Singapore (Dr. Tan); the Department of Pathology, National University Hospital, Singapore (Dr. Wang); and the Department of Otorhinolaryngology-Head and Neck Surgery, Khoo Teck Puat Hospital, Singapore (Dr. Mok and Dr. Loke). The case described in this article occurred at Khoo Teck Puat Hospital.

Corresponding author: Dr. Jiun Fong Thong, Department of Otorhinolaryngology, Singapore General Hospital, Outram Rd., Singapore 169608. Email: thong.jiun.fong@sgh.com.sg
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Title Annotation:ORIGINAL ARTICLE
Author:Thong, Jiun Fong; Tan, Tiong Yong; Wang, Shi; Mok, Paul; Loke, David
Publication:Ear, Nose and Throat Journal
Article Type:Case study
Date:Oct 1, 2014
Words:1744
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