Copyright # Blackwell Munksgaard 2006
J Cutan Pathol 2006: 33: 378–382 Blackwell Munksgaard. Printed in Singapore
Journal of
Cutaneous Pathology
Dermal nodular fasciitis: three case reports of the head and neck and literature review 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. Nishi SPE, Brey NV, Sanchez RL. Dermal nodular fasciitis: three case reports of the head and neck and literature review. J Cutan Pathol 2006; 33: 378–382. # Blackwell Munksgaard, 2006.
Introduction Nodular fasciitis was initially described in 1955 by Konwaller et al. as ‘subcutaneous pseudosarcomatous fibromatosis (fasciitis)’ due to the possible clinical and histological confusion with a sarcoma. It is a benign, self-limited, fibroproliferative disease thought to be a reactive process rather than a true neoplasm.1–5 The etiology remains unknown, yet some authors suggest trauma as the inciting event, although there are few reports to substantiate this claim.6–8 Classically, nodular fasciitis presents as a freely mobile, well-circumscribed and tender nodule of a few weeks’ duration, seldom larger than 2 cm, between the ages of 20–40 years with males and females equally affected.9 These lesions spare the dermis and do not recur after local excision. Nodular fasciitis typically occurs on the upper and
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Shawn P. E. Nishi1, Nicole Vessels Brey2 and Ramon L. Sanchez3 1
Department of Internal Medicine, University of Texas Medical Branch, Galveston, TX, USA, 2 Department of Dermatology, University of Louisville, Louisville, KY, USA, and 3 Department of Dermatology, University of Texas Medical Branch Galveston, TX, USA
Nicole Vessels Brey, Department of Dermatology, University of Louisville, Louisville, KY 40292, USA Tel: þ 1 502 852 7287 Fax: þ 1 530 752 9766 e-mail:
[email protected] Accepted for publication May 10, 2005
lower extremities of adults, particularly the volar aspect of the forearm, and only 10–20% are estimated to occur in the head and neck region.1 In children, however, the head and neck region is, in fact, the most common site and recurrence of the lesion is more commonly seen than in adults.2,3,5,9 The lesion is usually located in the subcutaneous, fascial, or intramuscular regions, but uncommon variants such as intravascular, cranial, ossifying, proliferative, and recently documented, intradermal nodular fasciitis exist.2,4,8 Most cases of intradermal nodular fasciitis were in the head and neck region.7,10 Goodlad and Fletcher documented the first case of dermal nodular fasciitis in 1990, seven cases have since been documented in the English dermatopathologic literature, and 28 additional cases of dermal nodular fasciitis in the otorhinolaryngic literature involving the external ear region.7,9–12
Dermal nodular fasciitis Case reports Case 1 A 15-year-old female complained of a 1-year history of a painless nodule on the left cheek. Initial examination revealed a 0.6 0.7-cm nodule suspicious for a hidrocystoma or other cystic lesion. The nodule was excised, and histologic examination showed an incompletely excised nodular fasciitis. At subsequent follow-up, the nodule had grown to approximately 1.5 cm in size. The patient was then treated with intralesional steroid injection with reduction in size of the lesion and eventual resolution. Case 2 A 40-year-old female was consulted for a 1.0-cm tumor nodule on the base of the scalp, which had grown rapidly during the previous month. An excisional biopsy and histologic examination showed nodular fasciitis. This was followed by a re-excision of the base of the tumor. There was no recurrence at 1-year follow-up. Case 3 A 39-year-old male had a 5–6 weeks’ history of an 8 5-mm painless nodule on the right medial canthus that grew quickly (Fig.1). The patient was treated originally with doxycycline without improvement. An incisional biopsy was performed and histologic examination showed nodular fasciitis. The patient’s lesion resolved on follow-up.
Histologic findings Cases 1 and 3 were partial excisions while in case 2 the whole lesion had been removed. Routine hematoxylin- and eosin-stained sections in all three instances demonstrated involvement of the deeper half of the dermis with extension into the subcutaneous tissue. Low magnification showed well-circumscribed, unencapsulated tumor nodules composed of spindle cells. The tumor cells were arranged in bundles, which in some areas exhibited storiform pattern. In between the tumor cells there was loose, myxoid stroma that stained positively for mesenchymal mucin with colloidal iron stain. The individual tumor cells display plump, spindle to oval nuclei with small, eosinophilic nucleoli and indistinct cytoplasmic borders. Mitoses were infrequent in all of the cases. Scattered collections of lymphocytes and extravasated red blood cells were present. Immunohistochemical stain with smooth muscle actin was strongly positive in the tumor cells while S-100 staining was negative.
Discussion Nodular fasciitis has been classically described as a non-recurring, tender, solitary, mobile, rapidly developing subcutaneous nodule occurring over 1– 12 weeks and reaching between 1 and 5 cm in size.1,2 The dermal variant, however, can be present with additional symptoms of pain, superficial ulceration, and bleeding, located in less common areas, and possible recurrence.7,9,11 Without excision, spontaneous regression is known to occur. In some cases, there has been a history of preceding trauma, although this association remains uncertain.1,6 Clinically, fibromatosis, dermatofibroma, lipoma, fibrosarcoma, neuroma, neurofibroma, pyogenic granuloma, and benign cyst are the most common differential diagnoses considered.2,11 Our 15-year-old patient’s clinical presentation was unusual because the nodule had been present for 1 year. Upon initial excision, the nodule did not regress but recurred and had increased in size within 1 month of the initial presentation. This is consistent with several studies that show the majority of recurrences are in children, and of these recurrences, the location is usually involving the auricle.7,10,13 Although it is still unknown why the majority of dermal nodular fasciitis cases occur in the head
Fig. 1. Clinical appearance of nodular fasciitis in Case 3. This 39year-old male had a 5–6-week history of an 8 5-mm painless nodule of the right medial canthus that grew quickly.
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Fig. 2. Nodular fasciitis low magnification showing a well-circumscribed tumor nodule involving dermis and subcutaneous tissue. H&E 4.
Fig. 3. The tumor cells were spindle shaped and arranged in bundles with occasional storiform pattern. H&E 200.
and neck region, Meffert postulated that this could be explained by the more direct attachment of the fascial musculature to relatively thinner skin via the superficial musculoaponeurotic system in this location.11 On macroscopic examination, these lesions present as unencapsulated round-to-oval nodules that can be irregular or well-circumscribed, gray or white in color, and vary between gelatinous to firm in consistency (Fig.2).2,4,9,10 Histologically, they are highly cellular and characterized by spindle-shaped, immature-appearing myofibroblasts and fibroblasts haphazardly arranged in a mucoid matrix (Figs 3,4,6).2,4,9,10,14 A capillary network with red bloodcell extravasation is also apparent. The presence of ganglion cells has also been described.15,16 Mitoses are abundant and have been documented to vary between one to 10 per 10 high-power fields without atypical mitoses seen.2 Yoskovitch and colleagues4 note that the presence of clefts throughout the tumor is also characteristic. Nodular fasciitis is commonly misdiagnosed due to its sarcoma-like features. The histologic differential diagnosis of dermal nodular fasciitis is listed in Table 1. The more worrisome tumors in the histological differential diagnosis of nodular fasciitis are able to be excluded microscopically or immunohistologically.
Immunohistochemical studies confirm the myofibroblastic nature of the tumor cells with characteristic reactivity for smooth muscle actin and vimentin and lack of reactivity to S-100 (Fig.5) protein, cytokeratin, HMB45, keratin antigens, desmin, CD34, or factor-VIII-related antigens.1,7–10,13,15 Matusik and colleagues16 reported a positive CD68 staining. Fine needle aspiration (FNA) has also been used for diagnosis of cases in the head and neck region. Dahl and Akerman17 first documented this correlation between FNA findings and histologically confirmed nodular fasciitis. Aydin and colleagues15 give further support for FNA because they reported 90–95% reliability for distinguishing between benign vs. malignant mesenchymal lesions. Both studies recommend a non-operative follow-up with clinical observation in the face region. Surgical excision is only recommended for unclear cytological diagnosis or inability of the
Table 1. Histological differential diagnosis of intradermal nodular fasciitis Spindle cell carcinoma Spindle cell melanoma Proliferative fasciitis Solitary fibrous tumor Dermatofibroma Dermatofibrosarcoma protuberans Fibromatosis Peripheral nerve sheath tumors Cutaneous smooth muscle tumors
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Myxoid liposarcoma Fibrosarcoma Leiomyosarcoma malignant fibrous Histiocytoma benign fibrous Histiocytoma Atypical fibroxanthoma Pyogenic granuloma Inflammatory pseudotumor
Fig. 4. The stroma between the tumor cells showed mucinous material that stained positive with colloidal iron. Lymphocytes were admixed among the tumor cells. H&E 400.
Dermal nodular fasciitis
Fig. 5. The tumor cells were strongly positive for smooth muscle actin. They were negative for S100, cytokeratin, and dermis. SMA 400.
suggest that recurrence is simply continued growth of residual tumor after incomplete excision.9 This most likely accounts for the case of the 15-year-old patient’s recurrence. In summary, we presented three cases of dermal nodular fasciitis involving the head and neck region of three patients, two of them children. Histologically, the fibrillar character of the tumor cells with positive smooth muscle antigen (SMA), and abundant dermal mucin was characteristic of nodular fasciitis, unlike the findings seen in dermatofibroma, dermatofibrosarcoma protuberans, or atypical fibroxanthoma. One of our cases recurred after incomplete excision. However, further treatment with intralesional steroid injection resulted in reduction of size and resolution. Acknowledgements
patient to clinically follow up. Regardless of diagnostic modality, tissue must be examined given the differential diagnosis in the head and neck region. Local marginal excision of the benign tumor is the preferred treatment although partial excision may be adequate; it has been suggested that any residual tissue probably undergoes spontaneous regression by scarring, and even diagnostic FNA has been documented to initiate spontaneous regression.14,18 Recurrence of previously excised cases has been documented; recurrence is so uncommon, however, that it has been suggested that recurrence of the lesion originally classified as a nodular fasciitis should lead to a careful review and reassessment of the diagnosis.6 A definitive explanation for a true recurrence of nodular fasciitis cannot be given, but it is thought that the myofibroblasts that undergo injury during the excision process caused indigenous fibroblasts to transform to myofibroblasts augment the population of existing myofibroblasts. Others
Fig. 6. Colloidal iron stain showing greatly increased mucin material in the tumor. Colloidal iron, 150.
We thank Dr William R. Holder, Dr Kevin St. Clair, and Dr Joseph R. Terracina for their cases contributed in this manuscript.
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