Intravascular Fasciitis: Report of an Intraoral Case and Review of the Literature

Division of Oral Pathology, College of Dental Medicine, Medical University of South Carolina, MSC 507, 173 Ashley Ave, Charleston, SC 29425, USA.
Head and Neck Pathology 07/2011; 6(1):140-5. DOI: 10.1007/s12105-011-0284-9
Source: PubMed


Intravascular fasciitis (IF) is an unusual variant of nodular fasciitis. It is characterized by intraluminal, intramural, and extramural involvement of small to large arteries or veins. Only three cases involving the oral cavity have been reported previously in the literature. Here we present an additional case of oral IF arising in the submucosa of the upper lip of a 20-year old female. Microscopic examination showed a well-circumscribed, nodular proliferation of spindle cells arranged in intersecting fascicles. Occasional multinucleated giants cells also were noted. The tumor was present within the lumen of an intermediate-sized artery and extended into adjacent smaller vessels, thereby creating a multinodular appearance. Extramural extension into the surrounding connective tissue also was observed. Among the 31 cases of IF reported thus far (including the present case), the majority (n = 23) arose in individuals in the 1st through 3rd decades, with a 1.4:1 male:female ratio. The most common sites of involvement were the head and neck (n = 11) and upper extremity (n = 11), followed by the lower extremity (n = 6) and trunk (n = 3). Conservative excision is standard treatment, although local recurrence has been reported in three cases. It is important for the pathologist to be aware of this lesion in order to avoid misdiagnosis as a sarcoma with angioinvasion.

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    • "The background stroma varied from a dense hyalinized to edematous, myxoid appearance. Scattered multinucleated giant cells were noted in more than one third of cases including the current one [1,2,4,5,7-9,13]. Lymphocytes and red blood cells were often seen as well. "
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    ABSTRACT: Intravascular fasciitis is an uncommon variant of nodular fasciitis, which is a reactive proliferative lesion of myofibroblasts. Since its identification in 1981, only 32 cases of intravascular fasciitis have been reported in the English literature. The lesion is commonly located in the head, neck, and extremities, with only three cases arising from the trunk. Here we report the fourth case involving the trunk (the flank area). A 21-year-old African-American female presented with a subcutaneous mass on her flank. Grossly, the mass was red-tan, oval, and well-demarcated, measuring approximately 0.5 cm in diameter. Microscopically, the mass was composed of spindle cells arranged in a swirling and intersecting pattern inside the lumens of two blood vessels. It extended through the vascular walls into the surrounding fibroadipose tissue; in some sections, the spindle cells were intermixed with the perivascular fibrous tissue. Elastin stain revealed remnants of elastic lamina partially surrounding the lesion. The nuclei of the spindle cells were relatively uniform with tapered ends and prominent nucleoli. No significant mitotic activity was observed. Multinucleated giant cells were scattered among the spindle cells, along with infiltrating lymphocytes and extravasated red blood cells. Immunohistochemical stains showed the spindle cells were positive for smooth muscle actin, focally positive for muscle specific actin, and negative for S-100, confirming their myofibroblastic differentiation. The overall morphological and immunohistochemical features are consistent with intravascular fasciitis. By reporting this rare case, we would like to raise the awareness of this non-neoplastic lesion to avoid misdiagnosing it as a sarcoma with vascular invasion. Previously reported similar cases were also reviewed and compared with this case.
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    ABSTRACT: Intravascular fasciitis is a very unusual variant of nodular fasciitis. A unique case of this lesion occurring in the proximal portion of the superficial vein of the forearm in an otherwise healthy 26-year-old man is reported. The intravascular polypoid lesion grew longitudinally along the vascular lumen, was loosely attached to the intimal layer, and was partly anchored beyond the internal elastic lamina into the medial smooth muscle layer. However, extravascular involvement was absent. The histological features were identical to those observed in ordinary cellular nodular fasciitis. Because of its myofibroblastic phenotype exhibited by highly proliferative spindle cells, certain intimomedial myofibroblasts are thought to be the indigenous source of this unique fibroproliferative lesion. Unless the diagnosis of intravascular fasciitis is considered and appropriate differential markers examined, it may be confused with other intravascular lesions, such as intravascular leiomyoma, intravenous pyogenic granuloma, organized thrombus and, even, fibromuscular dysplasia if it arises in the arteries. A simple excision is considered curable. Even so, two recurrent cases have been documented to date.
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