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.
Epidemiology Pathogenesis Laboratory/Radiologic/Other Diagnostic Testing Gross Appearance and Clinical Variants Histopathological Features and Variants Differential Diagnosis Prognosis Treatment Commonly Used Terms Internet Links
EPIDEMIOLOGY CHARACTERIZATION SYNONYMS Subcutaneous pseudosarcomatous fibromatosis AGE RANGE-MEDIAN 20-40 years SEX (M:F) =
PATHOGENESIS CHARACTERIZATION CHROMOSOMAL ALTERATIONS
Clonal rearrangement of 15p11.2, 16p11.2, and 16p13.3 in a case of nodular fasciitis: additional evidence favoring nodular fasciitis as a benign neoplasm and not a reactive tumefaction.
Donner LR, Silva T, Dobin SM.
Department of Pathology, Scott & White Clinic and Memorial Hospital, Scott, Sherwood and Brindley Foundation, The Texas A&M University System Health Science Center College of Medicine, 2401 South 31st Street, Temple, TX 76508, USA.
Cancer Genet Cytogenet 2002 Dec;139(2):138-40 Abstract quote
This article describes a case of nodular fasciitis with the karyotype 47,XY,+4/46,XY,add(15)(p11.2), t(16;16)(p13.3;p11.2).
The presence of clonal chromosomal abnormalities in this case, as well as in three previously reported cases, indicates
TRAUMA A history is often elicited
Generally understood to be a reactive process
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
CHARACTERIZATION VARIANTS DERMAL
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.
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, MD
Ann 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.
- 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.
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.
Plump fibroblasts resembling fibroblasts found in tissue culture
Oval pale nuclei with prominent nucleoli
Mitotic figures common
Atypical mitotic figures should not be seen
Microhemorrhages between bundles of fibroblasts
Intervening matrix rich in mucopolysaccharides staining positive for alcian blue and digested with hyaluronidase-this matrix imparts a feathery pattern to the fibroblasts
Lesions of shorter duration have more myxoid changes
Longer duration may have fusion of smaller microcysts forming a central cystic space
Nodular fasciitis a correlative cytologic and histologic study of 13 cases.
Dahl I, Akerman M.
Acta Cytol 1981 May-Jun;25(3):215-23 Abstract quote
Nodular fasciitis, the most common pseudosarcomatous proliferative lesion of the soft tissues, is well defined histologically but not cytomorphologically. A histologic and cytologic study of 13 cases of nodular fasciitis is presented.
Nodular fasciitis is characterized histologically by proliferating fibroblastlike cells that vary from thin, elongated and spindle shaped with elongated nuclei to plump with one or more large, oval nuclei. The appearance of nodular fasciitis in fine needle aspirations is characterized by the presence of tumor cells varying widely from spindle shaped with long cytoplasmic processes to more plump and cytoplasmatic with one or more nuclei with nucleoli. An attempt to correlate the histologic and cytologic appearances is made.
It is stressed that all the cells in nodular fasciitis lack histologic and cytologic signs of malignancy. The differential diagnosis is discussed. The cytomorphology of nodular fasciitis is fairly characteristic, making it possible to recognize these lesions in fine needle aspirates.
Subcutaneous Most common form with well circumscribed spherical nodule along the fascia Intramuscular Superficially attached to the fascia Fascial Less circumscribed growing along the interlobular septa of the subcutaneous fat VARIANTS
Postoperative/posttraumatic spindle cell nodule of the skin: the dermal analogue of nodular fasciitis.
Wick MR, Mills SE, Ritter JH, Lind AC.
Section of Dermatopathology, Washington University Medical Center, St. Louis, Missouri 63110-1094, USA.
Am J Dermatopathol 1999 Jun;21(3):220-4 Abstract quote
Spindle cell proliferations of the skin are diverse, both morphologically and mechanistically.
The authors have encountered four examples of a distinctive reactive/reparative cutaneous spindle cell lesion that shows homology with ones seen in the genitourinary tract and oral cavity and that is known as "postoperative/posttraumatic spindle cell nodule" (PSCN).
These occurred in the skin of the face and scalp (2 cases), arm (1 case), and vulvar skin (one case), and were clearly related historically to prior episodes of trauma. The proliferations were characterized by variably-apposed and cytologically-bland spindle cells with numerous mitotic figures, set in a highly vascular stroma containing extravasated erythrocytes and inflammatory cells. All lesions were immunoreactive for vimentin, actin, and desmin, with no labeling for keratin.
Postoperative/posttraumatic spindle cell nodule of the skin is a significant pseudoneoplastic lesion that is related (and perhaps identical pathogenetically) to nodular fasciitis; as such, it may be mistaken for a sarcoma or a spindle cell carcinoma. Careful attention to clinicopathologic and histologic details should result in its accurate recognition.
DIFFERENTIAL DIAGNOSIS KEY DIFFERENTIATING FEATURES GENERAL
Fibrosarcoma versus fibromatoses and cellular nodular fasciitis. A comparative study of their proliferative activity using proliferating cell nuclear antigen, DNA flow cytometry, and p53.
Oshiro Y, Fukuda T, Tsuneyoshi M.
Second Department of Pathology, Faculty of Medicine, Kyushu University, Fukuoka, Japan.
Am J Surg Pathol 1994 Jul;18(7):712-9 Abstract quote
We analyzed the proliferative activities, immunoreactivity of the p53 protein, and aneuploidy in patients with benign and malignant fibrous lesions, including 19 with nodular fasciitis (cellular type) (6-88 years old, mean 42.9), 11 with abdominal fibromatoses (22-74 years old, mean 37.9), 13 with extraabdominal fibromatoses (2-38 years old, mean 19.5), and 23 with fibrosarcomas (adult type: 16-71 years old, mean 47.3; infantile type: 3 months to 9 years, mean 2.9) using immunohistochemistry to determine proliferating cell nuclear antigen (PC10) and p53 protein (CM1) as well as performing DNA flow cytometry.
The proliferating cell nuclear antigen (PCNA) score was measured as the ratio of PCNA-positive nuclear size/total nuclear size determined by an image analysis computer system. The distribution pattern of the PCNA-positive cells was uneven in each instance of nodular fasciitis, in contrast to the distribution in abdominal fibromatosis, extraabdominal fibromatosis, and fibrosarcoma. Both fibrosarcoma (28.4 +/- 20.0) and nodular fasciitis (33.6 +/- 20.9) exhibited a larger value and a greater variation in the PCNA score than did either abdominal (13.5 +/- 14.5) or extraabdominal fibromatosis (19.9 +/- 21.5). Abdominal fibromatosis exhibited a smaller value and less variation in the score. In short, the PCNA score did not correlate with the malignant potential. The proliferative index (S + G2 + M fraction) in fibrosarcoma was significantly higher than in either nodular fasciitis or abdominal fibromatosis. Aneuploidy was detected in five cases (26%) of fibrosarcoma, while six (26%) fibrosarcomas showed p53 positivity. Furthermore, p53-positive patients had a worse survival (0.01 < p < 0.05), and p53 positivity correlated with the proliferative index (p < 0.01).
In conclusion, the PCNA score simply indicates the proliferative activity independent of malignant potential. On the other hand, p53 positivity, proliferative index, and aneuploidy are all indicators of malignant potential in fibroblastic lesions, and p53 positivity may reflect a poor prognosis.
PROGNOSIS AND TREATMENT CHARACTERIZATION Recurrence May have local recurrence 1% TREATMENT Local excision
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