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nodular fasciitis

Nodular fasciitis

Nodular fasciitis also known as pseudosarcomatous fasciitis, infiltrative fasciitis, proliferative fasciitis or subcutaneous pseudosarcomatous fibromatosis, is a rapidly growing benign soft tissue tumor of fibroblastic or myofibroblastic proliferation that is frequently located in the deep subcutaneous tissue or in the fascia 1. Nodular fasciitis is included in the World Health Organization (WHO) classification of soft tissue tumors under “fibroblastic or myofibroblastic tumors” 2. Most nodular fasciitis are solitary and occur in young adults aged between 20 and 40 years, but children also may be affected 3. Nodular fasciitis affects both men and women with equal frequency. The most common locations for nodular fasciitis are in subcutaneous tissues of the volar aspect of the forearm (27%–29%) 4, the lower extremity, back or chest wall (15%–18%) 5, upper arm (12%) 6, neck 7 and head 8. Nodular fasciitis may also arise in the skeletal muscle 9, dermis 10 or in blood vessels 11, although it is rarely reported within joints, leading to misdiagnosis. A few cases of breast nodular fasciitis were described in the scientific literature; the World Health Organization categorized it as one of the benign mesenchymal breast tumors 12. The size of the lesions is generally small (i.e., <3 cm); however, sporadically they can be >10 cm 13.

Three general subtypes of nodular fasciitis may be identified on the basis of the lesion location 14:

  • subcutaneous
  • intramuscular
  • fascial

Other less common subtypes include dermal, intravascular, cranial, and ossifying. Most occurrences of nodular fasciitis are subcutaneous, fascia based, well circumscribed, non-encapsulated, and less than 2 cm in diameter. The intramuscular lesions tend to be larger, poorly circumscribed, and invade surrounding soft tissue. These lesions may be amenable to biopsy or excision without any need for imaging evaluation.

Nodular fasciitis typically manifests as a rapidly growing mass, often painless and palpable 15. Nodular fasciitis often comes to clinical attention when the patient presents with a rapidly growing, occasionally painful, palpable soft tissue mass 16. Patients may develop paresthesia if the mass exerts pressure on a peripheral nerve 1. Although nodular fasciitis is well accepted as a self-limited reactive process and not a true neoplasm, its precise cause is unknown 17.

Making a correct diagnosis of nodular fasciitis is important because nodular fasciitis is often misdiagnosed as a soft tissue sarcoma, which is the most important pathological differential diagnosis of this condition. Plaza et al. 18 reported that 2/3rd of their cases had been misdiagnosed as sarcoma. Differential diagnosis of nodular fasciitis includes, fibrosarcoma, fibroma, fibrous histiocytoma, and desmoids and histopathology and immunohistochemistry play a key role in identifying the condition. Magnetic resonance imaging (MRI) and histopathological examination, along with the clinical features, are the key to diagnosis.

Nodular fasciitis treatment options include, observation, as spontaneous regression has been reported 19, intra-lesional steroid or wide local excision. Recurrence after surgical excision is very rare (0.4-1%) 20. There is a report of cranial fasciitis, a rare variant of nodular fasciitis in children, in which rapid tumor growth occurred after fine‐needle biopsy 21.

Nodular fasciitis causes

The cause and pathophysiology of nodular fasciitis is poorly understood. Some authors describe nodular fasciitis as a reactive lesion related to trauma 15. However, the history of trauma was elicited in no more than 10–15% of patients 22. Others have described chromosomal abnormalities that are suggestive of a neoplastic origin. At this point there is no definitive evidence stating whether nodular fasciitis is a reactive or a self-limiting, neoplastic process.

Nodular fasciitis symptoms

In many cases (~45%), nodular fasciitis is localized to the upper extremity, particularly the volar aspect of the forearm (27%–29%) or upper arm (12%). Other common sites are:

  • back or chest wall (15%–18%)
  • head and neck (18%)
  • lower extremities (16%)

Symptoms of tenderness and pain are frequently described at presentation.

On physical examination, the nodule appears well circumscribed, often round or oval in shape, and is thought to lie in the subcutaneous tissue. The size is small, ranging from 0.5 to 5 cm with an average of 1.5-2 cm. Rare cases of larger lesions measuring up to 10 cm have been documented. It is usually freely moveable without attachment to the overlying skin. Nodules are located most commonly on the upper extremity, specifically the volar aspect of the forearm, with other common places being the trunk, head and neck, and lower extremities. Rare sites include the hand and foot. The intradermal variant can present with additional physical features of superficial ulceration and bleeding and they are often associated with increased amounts of pain.

Nodular fasciitis diagnosis

Histopathological examination and magnetic resonance imaging (MRI) is the key to diagnosis of nodular fasciitis. The imaging-based diagnosis should be verified with an excisional biopsy. On MRI, nodular fasciitis has been described as inhomogeneous, hypointense to muscle on T1-weighted images, and hyperintense to fat on T2-weighted images, with variable enhancement 16.

Pathologically nodular fasciitis is an unencapsulated lesion that is typically well demarcated from the surrounding uninvolved tissue but may be focally infiltrative 23. Although most cases of nodular fasciitis are centered in the fascia, they can also be dermal, subcutaneous, deep-fascial, intermuscular, intramuscular, and intravascular 24.

Histopathology of nodular fasciitis

Cytological diagnosis of nodular fasciitis can be very challenging, but immunohistochemistry helps in giving an accurate diagnosis. Microscopically, nodular fasciitis with its benign proliferation of fibroblasts and myofibroblasts, is typically mistaken for a sarcomatous lesion because of its rapid growth, abundant haphazardly arranged mass of plump spindle-shaped cells, and mitotic activity, but are differentiated by plump, immature looking fibroblasts, in short irregular bundles and fascicles 25. The spindle cells were arranged within a variably loose myxoid to collagenous stroma in the form of short intersecting bundles. The loose myxoid to collagenous stroma contained scattered lymphocytes and red blood cells. Proliferative fasciitis is a well recognized variant and is composed of epithelioid cells which resemble ganglion cells. Low power examination of nodular fasciitis shows a well-circumscribed discrete mass in the subcutaneous adipose tissue. Positive staining with alpha-smooth muscle actin (SMA) is a feature of nodular fasciitis, suggesting a myofibroblastic differentiation and less possibility of a sarcoma. Desmin, which is positive in various soft tissue sarcomas is often negative in nodular fasciitis 1.

Nodular fasciitis treatment

Nodular fasciitis is a benign self-limited lesion that is often surgically excised for diagnosis with subsequent complete resolution. Excisional biopsy results in immediate complete resolution of the lesion. In some cases, complete resolution is seen even after partial excision. In cases of spontaneous resolution, the time lag between the diagnosis and the confirmed resolution ranges from 1 month to 2 years 15. Some clinicians might regard complete excision as over-treatment and favor incisional or fine‐needle biopsy, because these procedures may lead to tumor regression, less complication and morbidity 26. Recurrences after surgical excision for nodular fasciitis have been described 27, 22, 28, 29. Bleeding is the only potential risk with excisional biopsy. Several weeks of observation after a diagnosis based on the results of percutaneous fine needle aspiration cytology (FNAC) also have been advocated given the self-limited course of the disease 30. It seems that resolution does not depend on the size of the lesion; Brown and Carty 22 described a 5.5 cm lesion spontaneously and rapidly regressed. Nevertheless, it is difficult to establish the appropriate criteria for a correct decision between excisional biopsy and conservative management.

Conservative management or excisional biopsy can be considered in typical cases with clinical, imaging and biopsy features 31. If the criteria for such an approach are not met, ultrasound-guided vacuum or surgical excision should be provided to establish definitive diagnosis and to cure. A conservative approach is appropriate if the lesion has a typical clinical appearance, with the core biopsy findings consistent with those of nodular fasciitis 22. In two cases in the literature the lesions were treated successfully with conservative management and disappeared spontaneously. If the criteria for conservative management are not met, however, excisional biopsy is effective, with no further treatment necessary. For example, the 20 cases of breast nodular fasciitis, an excisional biopsy was performed in 14 of 20 (70%) patients, while a “wait and see” approach was adopted in 4 of 20 (20%) individuals in which spontaneous resolution was observed 15.

Spontaneous regression and involution of nodular fasciitis in response to steroid injections have been reported 19.

Observation

Several studies have shown that an accurate diagnosis of nodular fasciitis can be made with a good clinical history and ctyology from FNAC. These studies have demonstrated spontaneous resolution of nodular fasciitis with a median period of 2 weeks and up to 16 weeks following FNAC. When there is an atypical clinical course, long duration of symptoms, lack of resolution within 4 weeks of diagnosis, or irregular cytologic results with FNAC, excisional biopsy is necessary to rule out other diseases.

The risk of incorrect diagnosis and the existence of a potentially more life-threatening lesion may encourage excisional biopsy over observation alone as the diagnostic and therapeutic modality. While FNAC-based diagnosis is quite recent, histologic diagnosis of nodular fasciitis has been the mainstay since it was first reported.

Intralesional corticosteroids

One case report of a 5-cm, ill-defined, subcutaneous nodule located on the volar aspect of the right forearm of a 37-year-old female was biopsied for diagnosis. Complete excision of the nodule was too difficult based on its size and infiltration into surrounding soft tissue. Conservative therapy with intralesional injection of corticosteroids demonstrated complete resolution of the lesion within 36 hours of follow-up 32. Other case studies have also shown regression and eventual resolution with the use of intralesional corticosteroid injections.

References
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