Nodular fasciitis of the cheek

ARTICLE

Nodular fasciitis is a benign, tumefactive, pseudoneoplastic proliferation of myofibroblasts. Its clinical course is characterized by sudden appearance and rapid enlargement. Although nodular fasciitis may be found anywhere on the body, it is located on the upper extremity in approximately fifty percent of cases. Nodular fasciitis usually arises in multiple fascial layers but can also appear in non-fascial areas. Because of the proliferation of fibroblasts and myofibroblasts, these benign lesions are occasionally misdiagnosed as fibrosarcoma. The following case report of nodular fasciitis suggests that this tumor can also occur on the face.

Case report

The patient is a 29-year-old woman who developed a rapidly enlarging painful subcutaneous nodule on her left cheek. She denied any history of trauma. Approximately 1 month after the development of this lesion, she was referred to a local surgery department with the diagnosis of an epidermal inclusion cyst. An attempt was made to remove it surgically. At surgery, the lesion was found to be a solid tumor rather than a cyst, and it was excised completely in order to establish a diagnosis. The specimen was identified initially as an "atypical fibrohistiocytic lesion" with the histologic differential diagnosis including leiomyosarcoma, malignant fibrous histiocytoma, fibrosarcoma, and nodular fasciitis (Fig. 1). Slides were sent to our hospital where pathologic diagnosis excluded nodular fasciitis. Because of the rather high degree of cellularity and the fact that several of specimen's protrusions extended to the matrix, a complete but conservative reexcision was recommended.

Only two weeks after the initial excision, the tumor had apparently regrown to near its original size (Fig. 2a). The operative procedure was performed with the patient under general anesthesia, and gross examination revealed a well-circumscribed, grayish-yellow mass measuring 1.5 cm in diameter. The mass was not fixed to the underlying masseter muscle and the overlying skin was freely movable. The lesion was dissected without difficulty and did not appear to be deeply infiltrated into the muscle (Fig. 2b). After one year of clinical follow up, the patient is disease-free.

Histologic examination of the lesion revealed non-encapsulated, spindle-shaped or stellate myofibroblastic cells in a loose "feathery tissue culture" arrangement with rare mitotic figures and abundant fibrillar cytoplasm in a matrix of stromal mucin (Fig. 3a). A lymphocytic infiltrate was present at the periphery of the mass. Margins were negative for tumor and, although the lesion was predominantly centered in the dermis, spindle cells were noted in adjacent fat and muscle. Other characteristics included lack of significant nuclear pleomorphism and increased vascularity with slit-like capillaries and extravasated red blood cells (Fig. 3b). We performed immunohistochemical analysis using the following antibodies; The results showed as follows; S-100: alpha: weak positive, beta: negative, vimentin: positive, desmin: negative, CD34: negative, NSE: negative, smooth muscle actin: weak positive, CD68(KP-1): positive.

Discussion

The entity now known as nodular fasciitis was first described by Konwalar et al. [1] in 1954, when it was termed "subcutaneous pseudosarcomatous fibromatosis (fasciitis)". In 1966, Mehregan [2] published one of the largest reviews ever in dermatologic literature, describing the characteristic clinical appearance and histology. Analyzing data from a total of 331 cases, Mehregan defined the typical appearance, epidemiology, and histologic findings. Nodular fasciitis can be divided into three subtypes based on their relationship with the fascia: subcutaneous, intramuscular, and fascial [3]. But uncommon clinical and pathological variants of nodular fasciitis, such as intravascular, cranial, ossifying, and proliferative fasciitis, have been described [4-6]. Additionally, intradermal nodular fasciitis have been reported by Goodlad and Fletcher [7, 8] in 1990. Classic nodular fasciitis as a rapidly growing nodule, most common in young adults between the ages 20 and 40 years with males and females equally affected [3].

Although nodular fasciitis may occur virtually anywhere on the body, the most common locations include the upper extremities (paticularly the volar aspect of the forearm), followed by the trunk (particularly the chest wall and back). Nodular fasciitis of the head-and-neck is next in frequency, and is the most common site in infants and children [3]. Werning [9] and Allen [10] reported that head-and-neck nodular fasciitis rates of up to 10-20 %. DiNardo et al. [11], who focused on head-and-neck nodular fasciitis in children, found cases that arose in areas devoid of fascia, which could easily be confused with fibrous histiocytoma. Indeed, nodular fasciitis usually arises in multiple fascial layers, although it can also appear in non-fascial areas. More than half of the cases of Ôintradermal' nodular fasciitis were in the head and neck region [12]. It has been postulated that the high percentage of dermal nodular fasciitis located on the head-and-neck may be due to the fact that the muscles in that location are attached to the overlying thin fascial skin by the superficial musculoaponeurotic system [13].

Typical histologic findings include haphazardly arranged pleomorphic arranged spindle cells in a myxoid stroma. The differential diagnosis of the present case at the histologic level includes a variety of benign and malignant entities. The benign tumors with spindle cells include fibrous histiocytoma, pyogenic granuloma, postoperative/post-traumatic spindle cell nodule [14], myofibroma, peripheral nerve tumor, cutaneous smooth muscle tumors. It is our experience that fibroblastic proliferations are often confused histologically with these benign tumors. We should check out the criteria of these tumors respectively and carried out immunohistochemistry which is also useful to comfirm the diagnosis [15]. Aside from clinical data, such as rapid growth and tenderness, the small size of the lesion is a helpful diagnostic feature as a benign tumor. Furthermore, nodular fasciitis must be distinguished from several malignant neoplasms with spindle cell proliferation. The main differential diagnosis of this tumor is fibrosarcoma, whereas other malignant spindle cell tumors may pose difficulties in the histological and immunohistochemical analysis, including kaposi sarcoma, spindle cell carcinoma [16], and spindle cell melanoma [17, 18]. The presence of the vascular component, the mucinous changes, and the inflammatory infiltrate help to differentiate nodular fasciitis from fibrosarcoma or soft tissue tumors. We should remember that, although it is generally easy to distinguish among typical examples of nodular fasciitis, proliferative myositis, myositis ossificans, and granulation tissue in healing wounds, there are some confounding aspects [19, 20]. Indeed, nodular fasciitis bears a close resemblance to organizing granulation tissue, and myofibroblastic proliferation may be initiated by a local injury or local inflammatory process, which supports a reactive proliferation triggered by trauma. However, only a small number of patients had trauma histories in one large series. In a large study, there were 18 "recurrences" out of 134 cases that were diagnosed initially as nodular fasciitis. Bernstein and Latters [21] elucidated four microscopic features crucial to the analysis of the recurrent lesions:

i) The nuclei are never hyperchromatic, and the chromatin remains diffuse and highly staining.

ii) The lesion has never been observed extending to the epidermis.

iii) The presence of even moderate numbers of plasma cells has repeatedly been associated with a true malignant lesion.

iv) There is a limit on the number of mitoses seen in a benign lesion, even one that is actively proliferating.

In our case, the initial diagnosis of nodular fasciitis recurring status post-excision was made based on the correlation of histological features. Because facial muscles tend to be difficult to identify, and because there has never been a documented case of nodular fasciitis arising in the cheek, we could not rule out fibrosarcoma at the initial examination. This case underscores the utility of the Bernstein and Latters' criteria for diagnosis of recurrent lesions.

CONCLUSION

In conclusion, although this case of nodular fasciitis of the cheek was clinically and histologically classic, the location was somewhat unusual. This case serves to illustrate both the potential head-and-neck presentation of this tumor, as well as its benign nature. Recommended treatment is usually complete consevative excision. However, for large and deep lesions there is management controversy, i.e., some authors recommended excision to ensure complete removal, whereas others note that even with incomplete excision, spontanous resolution usually occurs [22].

Article accepted on 23/12/2002

REFERENCES

1 - Konwaler BE, Weiss SW. Subcutaneous pseudosarcomatous fibromatosis(fasciitis). Am J Clic Pathol 1955; 25: 241-52.

2 - Mehregan A. Nodular fasciitis. Arch Dermatol 1966; 93: 204-10.

3 - Enzinger FM, Weiss S. Soft tissue tumor, 3nd ed. St Louis, MO: CV Mosby; 1995, p.167-76.

4 - Soule EH. Proliferative (nodular)fasciitis. Arch Pathol 1962; 73: 437-44.

5 - Chung EB, Enzinger FM.Proliferative fasciitis. Cancer 1975; 36: 1450-8.

6 - Yoshihide et al. A case of Proliferative fasciitis in the abdominal region. J Dermatol 2001; 28: 753-8.

7 - Goodland JR, Fletcher CDM. Intradermal variant of nodular Ôfasciitis'. Histopathology 1990; 17: 569-71

8 - Kang SK et al. Intradermal nodular fasciitis of the face. J Dermatol 2002; 29: 310-4.

9 - Werning JT. Nodular fasciitis of the facial region. Oral Surg Oral Med Oral Pathol 1979: 48: 441-6.

10 - Allen PW. Nodular Fasciitis. Pathology 1972; 4; 147-51.

11 - DiNardio LJ, Wetmore RF, Potsic WP. Nodular fasciitis of the head- and -neck in children. Arch Otolaryngol Head Neck Surg 1991; 117: 1001-2.

12 - Meffert J, Kennard C, Davis T, Quinn B. Intradermal nodular fasciitis presenting as an eyelid mass. Int J Dermatol 1996; 35: 548-52.

13 - Gelfand JM et al. Nodular fasciitis. Arch Dermatol. 2001; 137: 719-21.

14 - Proppe KH, Scully RE, Rosai J. Postoperative spindle cell nodules of genitourinary tract resembling sarcomas: A report of eight cases. Am J Sug Pathol 1984; 8: 101-8.

15 - Kayaselcuk F et al. Vimentin, smooth muscle actin, desmin, S-100 protein, p53, estrogen receptor expression in elastofibroma and nodular fasciitis. Ann Diagn Pathol 2002; 6: 94-9.

16 - Al-Bozom IA, Abrams J. Spindle cell carcinoma of the breast. Arch Pathol Lab Med 1996; 120: 1066-8

17 - Midili G et al. Differential diagnostic problems between nodular fasciitis and soft tissue sarcoma. A review of literature and a case report. G Chir 2000; 21: 49-52.

18 - Rimani M et al. Nodular fasciitis or pseudosarcoma. Pathol Biol 49; 808-11

19 - Has AF. Nodular fasciitis of the forehead. Dermatol Surg 1999; 25: 140-2.

20 - Stanley MW, Skoog L, Tani EM, Horwitz CA. Nodular fasciitis: spontaneous resolution following diagnosis by fine-needle aspiration. Diagn Cytopathol 1993; 9: 322-4.

21 - Bernstein KE, Latters R. Nodular(pseudosarcomatous) fasciitis, a nonrecurrent lesion: clinicopathologic study of 134 cases. Cancer 1982; 49: 1668-78.

22 - Katz MA et al. Nodular fasciitis of the hand. Clin Orthop 2001; 382: 108-11.