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Background
This soft tissue tumor is a benign and reactive proliferation of myofibroblasts, creating a spindle cell appearance under the microscope. It most commonly presents as a painful, rapidly growing mass in the head and neck region, usually in children or young adults. The upper extremities is also a common location. Most are solitary and are about 2 cm or less in overall dimension.
OUTLINE
EPIDEMIOLOGY CHARACTERIZATION SYNONYMS Subcutaneous pseudosarcomatous fibromatosis AGE RANGE-MEDIAN 20-40 years =
CHARACTERIZATION RADIOLOGY
Nodular fasciitis of the head and neck: radiographic findings.Shin JH, Lee HK, Cho KJ, Han MH, Na DG, Choi CG, Suh DC.
Department of Radiology, Asan Medical Center, University of Ulsan College of Medicine, Seoul, South Korea.
Clin Imaging 2003 Jan-Feb;27(1):31-7 Abstract quote Nodular fasciitis is an unusual benign reactive process of the soft tissues related to the fascia and characterized by fibroblastic proliferation.
Clinical findings and radiologic aspects of six cases are described [four computed tomography (CT), two ultrasonography (US), one magnetic resonance (MR)]. They were located in the infrahyoid anterior triangle (n=3), prevertebral space (n=2), and masticator space (n=1). Most of these lesions showed moderate to strong enhancement on CT or MR imaging, and were partially embedded in the adjacent muscle with preservation of smooth margin of the muscle.
Most cases showed a benign nature, except one case in the prevertebral space which showed aggressive nature with jugular fossa destruction and intracranial extension.
Nodular fasciitis: correlation of MRI findings and histopathology.Wang XL, De Schepper AM, Vanhoenacker F, De Raeve H, Gielen J, Aparisi F, Rausin L, Somville J.
Department of Radiology, University Hospital Antwerp, Wilrijkstraat 10, 2650 Edegem, Belgium.
Skeletal Radiol 2002 Mar;31(3):155-61 Abstract quote OBJECTIVE: To compare the histopathology of nodular fasciitis (NF) with the magnetic resonance imaging (MRI) findings in order to evaluate the basis of the MR signal characteristics.
DESIGN AND PATIENTS: Ten patients with NF, nine females and 1 male, with an age ranging from 13 to 58 years (mean 26.8 years) were studied. MRI findings, available in all 10 patients, were compared with the histopathology in nine patients, and an area-to-area comparative study of the whole specimen section histopathology and MRI was performed in two patients.
RESULTS: On the basis of an excisional biopsy or resection specimen, the nine lesions were classified into myxoid ( n=4), cellular ( n=3) and fibrous ( n=2) subtypes. Four myxoid lesions with a subcutaneous location showed a homogeneous SI comparable with muscle on T1-weighted images, high SI on T2-weighted images, and had homogeneous enhancement. One cellular lesion presented with homogeneous, slightly higher SI than muscle on T1-weighted images and inhomogeneous, high SI on T2-weighted images. Alcian blue stain of the whole specimen section revealed the lesion had two parts corresponding to different enhancement patterns on MRI. The blue-stained myxoid part showed markedly diffuse enhancement, while the non-stained cystic space had only peripheral enhancement. Two other cellular lesions had the same appearance on both T1- and T2-weighted images and showed inhomogeneous, diffuse enhancement. One fibrous subtype lesion presented with inhomogeneous, overall slightly higher SI than muscle on T1-weighted images, lower SI at the periphery and high SI in the center on STIR images and only peripheral enhancement. Microscopy and CD-31 staining of the lesion showed more extracellular matrix, with poor vascularity in the center and more collagenous matrix with higher vascularity at the periphery.
CONCLUSION: Although similar findings were found in some lesions, the large histologic variability of NF hampers the definition of a prototype of NF on MRI. However, the MRI appearance of the myxoid subtype is rather characteristic. Histologic findings reflect the different SI characteristics and enhancement pattern on MRI.
Flow Cytometry Diploid DNA content
GROSS APPEARANCE/
CLINICAL VARIANTSCHARACTERIZATION VARIANTS DERMAL
- Dermal nodular fasciitis: three case reports of the head and neck and literature review.
Nishi SP, Brey NV, Sanchez RL.
Department of Internal Medicine, University of Texas Medical Branch, Galveston, TX, USA.
J Cutan Pathol. 2006 May;33(5):378-82. Abstract quote
Nodular fasciitis is a benign reactive myofibroblastic proliferative process of unknown etiology. It presents as a solitary painless, rapidly growing nodule over several weeks' duration. The condition is self-limited, and proper diagnosis is essential to avoid unnecessary aggressive treatment. Diagnosis is often a challenge because it may be confused with a malignant tumor due to its aggressive clinical behavior and histological features. Immunohistochemical staining can be a useful tool to aid in the diagnosis.
Although most commonly located on the extremities and then the trunk, it is estimated that the head and neck region represents only 10 to 20%. The majority of cases arise in the soft tissue, i.e. fascia, muscle, or subcutaneous tissue. Interestingly, cases in the head and neck region often involve dermal tissue. There have been five separate reports documenting rare cases of dermal nodular fasciitis in the dermatopathology literature and one case series involving 28 of 50 dermal variants from the external ear region.
We report three additional cases of dermal nodular fasciitis occurring on the left cheek, base of the scalp, and right medial canthus.EAR Nodular fasciitis of the external ear region: A clinicopathologic study of 50 cases
Lester D.R. Thompson, MD
Julie C. Fanburg-Smith, MD
Bruce M. Wenig, MDAnn Diagn Pathol 5: 191-198, 2001 Abstract quote
Nodular fasciitis (NF), uncommon in the auricular area, is a benign reactive myofibroblastic proliferation that may be mistaken for a neoplastic proliferation.
Fifty cases of NF of the auricular region were identified in the files of the Otorhinolaryngic-Head and Neck Tumor Registry of the Armed Forces Institute of Pathology.
The patients included 22 females and 28 males, aged 1 to 76 years (mean, 27.4 years). The patients usually presented clinically with a mass lesion (n = 49). Five patients recalled antecedent trauma. The lesions were dermal (n = 28) or subcutaneous (n = 11) in those cases where histologic determination was possible, measuring 1.9 cm on average. The majority of the lesions were circumscribed (n = 38), composed of spindle-shaped to stellate myofibroblasts arranged in a storiform growth pattern, juxtaposed to hypocellular myxoid tissue-culture-like areas with extravasation of erythrocytes. Dense, keloid-like collagen and occasional giant cells were seen (n = 18). Mitotic figures (without atypical forms) were readily identifiable.
By immunohistochemical staining, myofibroblasts were reactive with vimentin, actins, and CD68. All patients had surgical excision. Four patients (9.3%) developed local recurrence and were alive and disease free at last follow-up. All patients with follow-up (n = 43) were alive or had died of unrelated causes, without evidence of disease an average 13.4 years after diagnosis.
Nodular fasciitis of the auricular area occurs most often in young patients. Because NF is more often dermally situated than extremity NF, it may present with superficial ulceration and/or bleeding. Local recurrence is more frequent because of the difficulty in obtaining complete surgical excision around the ear.
HAND
- Nodular fasciitis of the hand: a potential diagnostic pitfall in fine-needle aspiration cytopathology.
Plaza JA, Mayerson J, Wakely Jr PE.
Department of Pathology, The Ohio State University College of Medicine, Columbus.
Am J Clin Pathol. 2005 Mar;123(3):388-93. Abstract quote
Fine-needle aspiration biopsy (FNAB) is applied very uncommonly to soft tissue masses and even more infrequently to lesions of the hand. Nodular fasciitis, an uncommon pseudosarcomatous lesion of soft tissue, rarely occurs in the hand and, because of this, is not often considered in the differential diagnosis of hand masses.
We report 3 cases (2 men and 1 woman; mean age, 44.3 years) of soft tissue masses of the hand, which, after clinical and radiologic evaluation, were strongly suspected by an experienced orthopedic oncologist as harboring a soft tissue sarcoma. Each patient underwent FNAB, which showed markedly hypercellular smears with overlapping, relatively isomorphic spindle cells that were mistaken cytologically as possible sarcoma in 2 cases; 1 case was considered probable nodular fasciitis. All lesions eventually were diagnosed as nodular fasciitis after thorough histologic and immunohistologic evaluation.
Nodular fasciitis remains a difficult diagnosis by FNAB, particularly when it occurs in locations such as the hand.HEAD AND NECK
Nodular fasciitis of the head and neck region: a clinicopathologic description in a series of 30 cases.
Weinreb I, Shaw AJ, Perez-Ordoñez B, Goldblum JR, Rubin BP.Department of Pathology, University of Toronto, Ontario, Canada.
J Cutan Pathol. 2009 Nov;36(11):1168-73. Abstract quote
Nodular fasciitis (NF) is a reactive lesion composed of fibroblasts/myofibroblasts and most commonly found in extremities and trunk. NF has been described in the head and neck region (HNR) in 13-20% of cases. It is our impression based on consultation experience that many pathologists do not consider NF in the differential diagnosis of soft tissue masses arising in the HNR. Moreover, it is common for these lesions to be incompletely excised, leading to additional challenges in diagnosis.
We describe 30 cases of NF of the HNR in order to focus attention on this frequently overlooked diagnosis. While they had the typical histologic features of NF, the lesions had a tendency for smaller size, increased skeletal muscle involvement (30%) compared to fasciitis elsewhere in the body and diffuse and strong actin expression.
Follow up demonstrated one recurrence (7.1%) higher than reported elsewhere in the body. These latter features may add to the challenge in diagnosing NF in these locations.INTRAARTICULAR
- Intraarticular Nodular Fasciitis-A Rare Lesion: Clinicopathologic Analysis of a Series.
Hornick JL, Fletcher CD.
From the Department of Pathology, Brigham and Women's Hospital and Harvard Medical School, Boston, MA.
Am J Surg Pathol. 2006 Feb;30(2):237-241. Abstract quote
Nodular fasciitis is a benign myofibroblastic proliferation with a predilection for the subcutaneous tissues of the upper extremities, trunk, and head and neck of young adults. Nodular fasciitis is not generally recognized to arise within joints.
In this study, the clinicopathologic and immunohistochemical features of 10 cases of intraarticular nodular fasciitis are described. Six patients were female and 4 were male, with a median age of 33 years (range, 9-50 years). Lesional size ranged from 2 to 4 cm (median, 2.6 cm). Seven tumors arose in the knee, 2 in the hand, and 1 in the ankle. Most patients complained of joint pain; 4 presented with a palpable mass. Only 1 patient reported antecedent trauma. The duration of symptoms prior to surgery ranged from 2 months to 1 year (median, 6 months). The clinical differential diagnoses included giant cell tumor of tendon sheath, pigmented villonodular synovitis, synovial chondromatosis, inflammatory arthritis, and lymphoma. Grossly, the lesions were solid, nodular, rubbery, or firm masses.
Histologically, all tumors were circumscribed but unencapsulated and showed typical features of nodular fasciitis, being composed of cytologically bland plump spindle cells arranged in short, intersecting bundles within a variably loose myxoid to collagenous stroma, containing extravasated red blood cells and scattered lymphocytes. Five lesions showed prominent stromal hyalinization, in 2 cases keloidal in appearance. In 4 cases, the tissue at the periphery of the lesion showed hemosiderin deposition. By immunohistochemistry, all tumors examined were positive for SMA, 1 was positive for desmin, and all were negative for caldesmon and S-100 protein; none showed nuclear staining for beta-catenin.
Clinical follow-up information was available for 5 patients, ranging from 2 to 86 months. No lesion recurred. In summary, intraarticular nodular fasciitis occurs most commonly in the knees of young adults, and often appears to have a somewhat longer preoperative duration than typical subcutaneous or intramuscular nodular fasciitis.
Intraarticular lesions show morphologic features similar to other cases of nodular fasciitis, with the exception that stromal hyalinization and adjacent hemosiderin deposition are common, likely attributable to frictional trauma in this location.
PROGNOSIS AND TREATMENT CHARACTERIZATION Recurrence May have local recurrence 1% TREATMENT Local excision Henry JB. Clinical Diagnosis and Management by Laboratory Methods. Twentieth Edition. WB Saunders. 2001.
Rosai J. Ackerman's Surgical Pathology. Ninth Edition. Mosby 2004.
Sternberg S. Diagnostic Surgical Pathology. Fourth Edition. Lipincott Williams and Wilkins 2004.
Robbins Pathologic Basis of Disease. Seventh Edition. WB Saunders 2005.
DeMay RM. The Art and Science of Cytopathology. Volume 1 and 2. ASCP Press. 1996.
Weedon D. Weedon's Skin Pathology Second Edition. Churchill Livingstone. 2002
Fitzpatrick's Dermatology in General Medicine. 6th Edition. McGraw-Hill. 2003.
Weiss SW and Goldblum JR. Enzinger and Weiss's Soft Tissue Tumors. Fourth Edition. Mosby 2001.
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Last Updated November 2, 2009
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