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Year : 2013  |  Volume : 56  |  Issue : 3  |  Page : 288-290
Nodular fasciitis: A diagnostic challenge

1 Department of General Surgery, Government Medical College, Amritsar, Punjab, India
2 Department of Pathology, Government Medical College, Amritsar, Punjab, India

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Date of Web Publication24-Oct-2013


Nodular fasciitis is a rare benign soft tissue tumor. The similarity in the cytological appearance of nodular fasciitis to that of pleomorphic adenoma makes it difficult to diagnose preoperatively that adds to its peculiarity. We report here a case of a middle-aged female with swelling in the neck that was diagnosed as pleomorphic adenoma on cytology. Surgical excision of the swelling was done and histopathological examination revealed it to be nodular fasciitis. This case is presented to highlight the challenge of correctly diagnosing nodular fasciitis.

Keywords: Cytology, nodular fasciitis, pleomorphic adenoma

How to cite this article:
Singh S, Paul S, Dhall K, Khichy S. Nodular fasciitis: A diagnostic challenge . Indian J Pathol Microbiol 2013;56:288-90

How to cite this URL:
Singh S, Paul S, Dhall K, Khichy S. Nodular fasciitis: A diagnostic challenge . Indian J Pathol Microbiol [serial online] 2013 [cited 2020 Nov 30];56:288-90. Available from: https://www.ijpmonline.org/text.asp?2013/56/3/288/120401

   Introduction Top

Nodular fasciitis is a rare benign neoplasm most commonly afflicting the soft tissues of upper extremity followed by trunk, head, and neck. [1] It typically involves subcutaneous tissue or fascia; however, intramuscular location is rarely seen. [2] It is commonly seen in fourth decade with no gender predilection. [3] It almost always poses a considerable diagnostic challenge due to a similar cytological picture to that of pleomorphic adenoma. [4] It should be surgically excised as recurrences are rare thereby having excellent prognosis. [5] We report a rare case of nodular fasciitis of neck in a 45-year-old lady.

   Case History Top

A 45-year-old female presented with a painless swelling on the right side of the neck since 2 months. It was initially small in size and gradually increased in size to about 2.5 × 2.5 cm. There was no history of trauma, fever, loss of appetite, and weight.

On examination, a nontender, nonfluctuant, firm swelling of 2.5 × 2.5 cm in size was present on the right side of neck at the junction of the two heads of sternocleidomastoid and at the level of the thyroid cartilage. The overlying skin was normal and swelling was movable in transverse plane with restricted movement in the vertical plane. Trans-illumination was negative. There was no generalized lymphadenopathy and systemic examination was within normal limits.

Fine-needle aspiration cytology (FNAC) showed hyper-cellular smear containing round-to-oval-to-spindle cells with chondromyxoid stromal fragments. Bi-nucleate cells and plasmacytoid cells were also seen in the myxoid background [Figure 1]. With these findings, diagnosis of pleomorphic adenoma was made. The swelling was excised which was embedded in the sternocleidomastoid muscle. Histopathology showed benign spindle cells arranged in sheets and fascicles containing moderate-to-abundant fuzzy cytoplasm with hyalinization at places and rare mitosis [Figure 2]a and b. Extravasated RBCs (red blood cells) and muscular invasion were seen at occasional places with no significant lymphocytic infiltrate [Figure 3]. Based on these findings, the diagnosis of nodular fasciitis was made.
Figure 1: Hyper-cellular smear having round-to-oval cells in chondromyxoid background (H&E, ×10)

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Figure 2: (a) Spindle-shaped cells arranged in sheets and fascicles with hyalinisation at places and rare mitosis (H&E, ×10), (b) Spindle-shaped cells arranged in sheets and fascicles with hyalinisation at rare places (H&E, ×40)

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Figure 3: Nodular fasciitis infiltrating muscular tissue with extravasation of red blood cells (H&E, ×10)

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On immunohistochemistry (IHC), there was widespread immunoreactivity for vimentin and smooth muscle actin along with focal immunoreactivity for muscle-specific actin thereby strongly favoring the diagnosis of nodular fasciitis [Figure 4], [Figure 5] and [Figure 6]. Immunonegativity for myogenin/ALK-1 ruled out rhabdomyosarcoma. The Ki-67 Proliferative Index was only 6% proving it to be a benign lesion. On follow-up after 6 months, no recurrence was seen.
Figure 4: Widespread positivity for vimentin (20)

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Figure 5: Widespread positivity for smooth muscle action (×40)

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Figure 6: Focal positivity for muscle specific action (40)

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   Discussion Top

Nodular fasciitis, first described by Konwaler and Weiss in 1955 [6] is also known as pseudosarcomatous fasciitis, pseudosarcomatous fibromatosis and infiltrative fasciitis. It is one of the most under-diagnosed lesion and can be confused with spindle cell sarcoma, fibromatosis, fibrous histiocytoma, proliferative fasciitis, benign nerve sheath tumors, and pleomorphic adenoma because of features such as short history, rapid growth, marked infiltration, and somewhat similar histopathological picture. It mostly presents as a solitary, painless, and rapidly growing nodule usually of less than 3 months duration. [7] The etiology is still unknown after a good number of cases that are reported worldwide. It is considered to occur due to unusual proliferation of myofibroblasts triggered by local injury or inflammatory process. [5] Although antecedent history of trauma has been implicated but its strong correlation with the disease occurrence has not been proven till date. It is commonly seen in the 40-50-year age group with almost equal distribution amongst both genders. [3] The most commonly affected sites are upper extremity and trunk followed in the decreasing order by head and neck and lower extremity. [1] Rarely involved sites include breast, [8] intra-vascular, intra-articular, nerve sheaths, and retroperitoneum.

Nodular fasciitis has to be clearly distinguished from spindle-cell sarcoma on the basis of degree of cytologic atypia which is marked in the spindle-cell sarcoma. [9] It should also be distinguished from fibromatosis (desmoid tumor) that shows dense collagenous stroma usually lacking myxoid areas along the with absence of extravasated RBCs. [10] The fibrous histiocytoma (dermatofibroma) also forms an important differential diagnosis of nodular fasciitis showing spindle cell proliferation admixed with epithelioid histiocytes but it also lacks prominent vasculature and extravasated RBCs. An ill-defined tumor growing along the fibrous septa (interlobular distribution) with large myofibroblasts admixed with immature fibroblast like spindle cells in a myxoid or collagenous background stroma but devoid of extravasated RBCs points toward the diagnosis of proliferative fasciitis. Benign nerve sheath tumors such as schwannoma and neurofibroma are also important differential diagnosis of nodular fasciitis that can be diagnosed on histology by the presence of Antoni A and Antoni B patterns of growth in schwannoma and abundance of collagen and little myxoid material in neurofibroma. Also neurofibroma lacks extravasated RBCs, frequently seen in nodular fasciitis. [10]

Fine-needle aspiration cytology poses a considerable element of uncertainty in the diagnosis of nodular fasciitis. Its features of nodular fasciitis may mimic that of pleomorphic adenoma due to the presence in both of spindle and plasmacytoid cells, clumping of inter-cellular stromal material, and myxoid background. [4] However, fuzzy cytoplasmic borders, multiple fragile cytoplasmic processes, and prominent nucleoli favor a diagnosis of nodular fasciitis whereas well-demarcated cytoplasmic borders and indistinct nucleoli are seen in cells of pleomorphic adenoma. [4] Nodular fasciitis predominantly contains spindle cells and mitotic cells whereas, pleomorphic adenoma contains abundance of plasmacytoid cells. On IHC, cytokeratin and S-100 positivity favor the diagnosis of pleomorphic adenoma. On the other hand, positivity for vimentin, smooth muscle actin, and muscle-specific actin is seen in nodular fasciitis.

A nodular fasciitis should be surgically excised to obtain complete cure as the chances of local recurrence are almost nil (<1%). [11]

To conclude, one should bear a high index of suspicion to diagnose this benign tumor whenever a rapidly growing small nodule gives a pleomorphic picture on cytology. There is an immense necessity to clearly differentiate it from pleomorphic adenoma.

   References Top

1.Meister P, Buckman FW, Konrad E. Nodular fasciitis (analysis of 100 cases and review of the literature). Pathol Res Pract 1978:162:133-65.  Back to cited text no. 1
2.Saad RS, Takei H, Lipscomb J, Ruiz B. Nodular fasciitis of parotid region: A pitfall in the diagnosis of pleomorphic adenomas on fine-needle aspiration cytology. Diagn Cytopathol 2005;33:191-4.  Back to cited text no. 2
3.Reitzen SD, Dogan S, Har-El G. Nodular fasciitis: A case series. J Laryngol Otol 2009;123:541-4.  Back to cited text no. 3
4.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.  Back to cited text no. 4
[PUBMED]  Medknow Journal  
5.Cyriac MJ, Celine MI, Kurien G, Puthiode U. Nodular fasciitis. Indian J Dermatol Venereol Leprol 2004;70:239-41.  Back to cited text no. 5
[PUBMED]  Medknow Journal  
6.Konwaler BE, Keasbey L, Kaplan L. Subcutaneous pseudosarcomatous fibromatosis (fasciitis). Am J Clin Pathol 1955;25:241-52.  Back to cited text no. 6
7.Bernstein KE, Lattes R. Nodular (pseudosarcomatous) fasciitis, a nonrecurrent lesion: Clinicopathologic study of 134 cases. Cancer 1982;49:1668-78.  Back to cited text no. 7
8.Dahlstrom J, Buckingham J, Bell S, Jain S. Nodular fasciitis of the breast simulating breast cancer on imaging. Australas Radiol 2001;45:67-70.  Back to cited text no. 8
9.Mahon JH, Folpe AW, Ferlic RJ. Intraneural nodular fasciitis: Case report and literature review. J Hand Surg Am 2004;29:148-53.  Back to cited text no. 9
10.Dayan D, Nasrallah V, Vered M. Clinico-pathologic correlations of myofibroblastic tumors of the oral cavity: 1. Nodular fasciitis. J Oral Pathol Med 2005;34:426-35.  Back to cited text no. 10
11.Allen PW. Nodular fasciitis. Pathology 1972;4:9-26.  Back to cited text no. 11

Correspondence Address:
Sumitoj Singh
Department of General Surgery, Government Medical College, Amritsar, Punjab
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/0377-4929.120401

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  [Figure 1], [Figure 2], [Figure 3], [Figure 4], [Figure 5], [Figure 6]


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