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Intravascular fasciitis involving the flank of a 21-year-old female: a case report and review of the literature.
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PMID:  24581345     Owner:  NLM     Status:  Publisher    
Abstract/OtherAbstract:
BACKGROUND: 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).
CASE PRESENTATION: 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.
CONCLUSION: 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.
Authors:
Yan Zheng; Mary George; Frank Chen
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Publication Detail:
Type:  JOURNAL ARTICLE     Date:  2014-2-28
Journal Detail:
Title:  BMC research notes     Volume:  7     ISSN:  1756-0500     ISO Abbreviation:  BMC Res Notes     Publication Date:  2014 Feb 
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Created Date:  2014-3-3     Completed Date:  -     Revised Date:  -    
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Nlm Unique ID:  101462768     Medline TA:  BMC Res Notes     Country:  -    
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Languages:  ENG     Pagination:  118     Citation Subset:  -    
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Journal ID (nlm-ta): BMC Res Notes
Journal ID (iso-abbrev): BMC Res Notes
ISSN: 1756-0500
Publisher: BioMed Central
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Copyright © 2014 Zheng et al.; licensee BioMed Central Ltd.
open-access:
Received Day: 29 Month: 10 Year: 2013
Accepted Day: 18 Month: 2 Year: 2014
collection publication date: Year: 2014
Electronic publication date: Day: 28 Month: 2 Year: 2014
Volume: 7First Page: 118 Last Page: 118
PubMed Id: 24581345
ID: 3943986
Publisher Id: 1756-0500-7-118
DOI: 10.1186/1756-0500-7-118

Intravascular fasciitis involving the flank of a 21-year-old female: a case report and review of the literature
Yan Zheng1 Email: Zheng418@hotmail.com
Mary George1 Email: MGeorge@KaleidaHealth.Org
Frank Chen1 Email: FChen@KaleidaHealth.Org
1Department of Pathology, Buffalo General Medical Center, 100 High Street, Buffalo, NY 14203, USA

Background

Intravascular fasciitis is a rare benign lesion characterized by reactive proliferation of myofibroblasts in the superficial or deep fascia with involvement of arteries and/or veins. Intravascular fasciitis is a variant of the more common condition of nodular fasciitis, which does not show vascular invasion. Intravascular fasciitis was originally described by Patchefsky and Enzinger in 1981 [1]. Since then, a total of 32 cases have been reported in the English literature. The lesion is commonly located in the upper extremities, and head and neck, and the upper extremities, with only three cases arising from the trunk [1,2]. Here we report the fourth case involving the trunk area.


Case presentation

The patient was a 21-year-old African-American female who presented with a single nodule on the flank area. Grossly, the subcutaneous nodule 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 (Figure 1A). It extended through the vascular walls into the surrounding fibroadipose tissue; in some sections, the spindle cells were intermixed with the perivascular fibrous tissue (Figure 1B). Elastin stain revealed remnants of elastic lamina partially surrounding the lesion (Figure 1C). The nuclei of the spindle cells were relatively uniform with tapered ends and prominent nucleoli (Figure 1D). No significant mitotic activity was observed. Multinucleated giant cells were scattered among the spindle cells (Figure 1E), along with infiltrating lymphocytes and extravasated red blood cells (Figure 1F). Immunohistochemical stains showed the spindle cells were positive for smooth muscle actin (SMA; Figure 1G), focally positive for muscle specific actin (Figure 1H), and negative for S-100 (Figure 1I), confirming their myofibroblastic differentiation. The overall morphological and immunohistochemical features are consistent with intravascular fasciitis.


Discussion

Thirty-two previously reported cases and the current case of intravascular fasciitis are summarized in Table 1[1-14]. Intravascular fasciitis commonly occurs in adolescent and young adult patients with an average age of 24 years old (ranging from 6 months to 66 years old). Of the 33 cases, only six patients were over age of 30. Females and males were equally affected with a female to male ratio close to 1:1. The patients were generally healthy prior to the onset of the lesion. Possible predisposing factors including preceding trauma, thrombosis, and pregnancy-related hormonal changes were noted in a few cases [1,9,10]. The most common locations of the lesion are the upper extremities, head and neck, followed by the lower extremities and trunk. In the majority of cases, the lesion presented as a solitary nodule located subcutaneously or within muscular tissue, although one patient was reported to have a multi-nodular lesion [12]. The size of the lesions ranged from 0.6 to 5 cm in greatest dimension with duration from 2 weeks to 8 years. Other features such as pain/tenderness, mobility, and demarcation of the lesion varied among cases. Intravascular fasciitis is a benign condition, and usually cured by a simple local excision. Of the 16 cases that were followed for 6 months to 20 years, local recurrence was found in three patients [1,7].

Microscopically, intravascular fasciitis was characterized by a spindle cell proliferation inside the lumens or associated with the walls of arteries or veins of all sizes. Depending on the numbers of blood vessels involved and the longitudinal extension of the lesion, the mass exhibited a single or multi-nodular appearance. In two cases, organizing thrombi were found within the lesion [1,10]. One feature that easily mimics a sarcoma is the infiltrating growth. Indeed, it was not uncommon to find that the lesion extended through the vascular walls into the surrounding connective tissue and neighboring blood vessels, but the overlying epidermis was usually intact. In the original report by Patchefsky and Enzinger, they compared the sizes of intravascular and soft tissue components, and concluded that the soft tissue component was dominant; this feature was not reported in the remainder of the cases [1]. The spindle cells were arranged in a storiform pattern or haphazard manner, with plump vesicular nuclei, and in some cases with prominent nucleoli. Mitotic activity ranged from absent to prominent. However, unlike a soft tissue sarcoma, significant cytologic pleomorphism and abnormal mitotic figures were absent. 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.

Immunohistochemistry studies showed that the spindle cells were positive for vimentin and SMA, negative for keratin, S100 protein, desmin, CD31, CD34, and c-kit, confirming their myofibroblastic differentiation. The multinuclear giant cells were CD68 positive, suggesting that they are cells of histiocytic origin.

The pathogenesis of intravascular fasciitis has yet to be understood. The immunohistochemical characteristics of the spindle cells confirm their myofibroblastic origin. However, the factors initiating myofibroblast proliferation have not been clearly identified. Possible risk factors that have been proposed include preceding trauma, thrombosis, and high levels of estrogen. Specifically, two patients developed the lesion following trauma [1,5]. Organizing thrombi were found in two cases [1,10]. The occurrence of intravascular fasciitis in a 16-week pregnant woman, and a previous report showing weak expression of estrogen receptor in nodular fasciitis, led the authors to propose that pregnancy-related estrogen changes might be a predisposing factor [9]. However these conditions were associated with only a minority of cases. More studies are required to clarify their roles in the development of intravascular fasciitis.

Intravascular fasciitis can be misdiagnosed as a sarcoma with vascular invasion because of its intravascular proliferation. However, the cytologic features of intravascular fasciitis, including the absence of large atypical hyperchromatic nuclei and abnormal mitotic figures, are helpful in distinguishing these two conditions [15].


Conclusions

Intravascular fasciitis is a rare variant of nodular fasciitis associated with blood vessels. By reporting this rare case, we would like to raise the awareness of this non-neoplastic lesion to avoid misdiagnosis.


Consent

Written informed consent was obtained from the patient for publication of this case report and accompanying images. A copy of the written consent is available for review by the Editor-in-Chief of this journal.


Abbreviations

SMA: Smooth muscle actin; LMWK: Low molecular weight keratin; EMA: Epithelial membrane antigen.


Competing interests

The authors declare that they have no competing interests.


Authors’ contributions

MG collected and interpreted data, and made the diagnosis. YZ and FC were the major contributors in writing and revising the manuscript. All authors read and approved the final manuscript.


Acknowledgement

We thank all the histology technologists at Buffalo General Medical Center for their technical support.


References
Patchefsky AS,Enzinger FM,Intravascular fasciitis: a report of 17 casesAm J Surg PatholYear: 198171293610.1097/00000478-198101000-000067246849
Samaratunga H,Searle J,O'Loughlin B,Intravascular fasciitis: a case report and review of the literaturePathologyYear: 19967181110.1080/003130296001694138714261
Freedman PD,Lumerman H,Intravascular fasciitis: report of two cases and review of the literatureOral Surg Oral Med Oral PatholYear: 19867554955410.1016/0030-4220(86)90319-13466126
Kahn MA,Weathers DR,Johnson DM,Intravascular fasciitis: a case report of an intraoral locationJ Oral PatholYear: 19877630330610.1111/j.1600-0714.1987.tb00698.x3119803
Price SK,Kahn LB,Saxe N,Dermal and intravascular fasciitis. Unusual variants of nodular fasciitisAm J DermatopatholYear: 19937653954310.1097/00000372-199312000-000048311183
Beer K,Katz S,Medenica M,Intravascular fasciitisInt J DermatolYear: 19967214714810.1111/j.1365-4362.1996.tb03286.x8850051
Sticha RS,Deacon JS,Wertheimer SJ,Danforth RD Jr,Intravascular fasciitis in the footJ Foot Ankle SurgYear: 199772959910.1016/S1067-2516(97)80052-X9127210
Ito M,Matsunaga K,Sano K,Sakaguchi N,Hotchi M,Intravascular fasciitis of the forearm vein: a case report with immunohistochemical characterizationPathol IntYear: 19997217517910.1046/j.1440-1827.1999.00842.x10355974
Anand A,Tsapakis EM,Narvani AA,Alhakim A,Cannon SR,Tsiridis E,"Pseudosarcoma" in a pregnant womanWorld J Surg OncolYear: 20077710.1186/1477-7819-5-717229326
Sugaya M,Tamaki K,Does thrombosis cause intravascular fasciitis?Acta Derm VenereolYear: 20077436937010.2340/00015555-026817598046
Pantanowitz L,Duke WH,Intravascular lesions of the handDiagn PatholYear: 200872410.1186/1746-1596-3-2418513426
Wang L,Wang G,Gao T,Myxoid intravascular fasciitisJ Cutan PatholYear: 2009716366
Chi A,Dunlap W,Richardson M,Neville B,Intravascular fasciitis: report of an intraoral case and review of the literatureHead Neck PatholYear: 20127(1)140145 doi:10.1007/s12105-011-0284-9 LA - English. 21779880
Reiser V,Alterman M,Shlomi B,Issakov J,Dagan Y,Kleinman S,Shuster A,Kaplan I,Oral intravascular fasciitis: a rare maxillofacial lesionOral Surg Oral Med Oral Pathol Oral RadiolYear: 201272e40e4410.1016/j.oooo.2012.03.02722769420
Rosenberg AE,Pseudosarcomas of soft tissueArch Pathol Lab MedYear: 20087457958618384209

Figures

[Figure ID: F1]
Figure 1 

Intravascular fasciitis. (A) The mass is composed of spindle cells arranged in an intersecting pattern. (B) The mass extends through the vascular walls into the surrounding connective tissue. (C) Elastin stain reveals remnants of elastic lamina partially surrounding the lesion. (D) The nuclei of the spindle cells are uniform with tapered ends and prominent nucleoli. (E) Multinucleated giant cells are scattered within the mass. (F) Infiltrating lymphocytes and extravasated red blood cells are present. (G) The spindle cells are positive for SMA. (H) The spindle cells are focally positive for muscle specific actin. (I) The spindle cells are negative for S-100.



Tables
[TableWrap ID: T1] Table 1 

Clinical and pathologic features of the reported cases of intravascular fasciitis


References # Age (years) and sex Location Gross features Microscopic features IHC features of spindle cells
Patchefsky et al. 1981 [1]
17
20.5 (range from 0.5 to 57), 8 F and 9 M
Head and neck (n = 5)
1.5 cm* (range from 0.6 to 5), single, firm, non-tender, immobile mass
Feathery, edematous, myxoid, and hyalinized background, giant cells present in 1/3 cases
N/A
Upper extremity (n = 7)
Trunk (n = 2)
Lower extremity (n = 3)
Freedman et al. 1986 [3]
2
19, M
Right posterior mucobuccal fold
2.5 cm, single, firm mass with ulcer
Myxoid and highly vascular background with rare mitotic figures
N/A
53, M
Left buccal mucosa
2.0 cm, single, firm, immobile mass
Myxoid and locally hyalinized background, no mitotic figures present
N/A
Kahn et al. 1987 [4]
1
20, F
Left lower labial mucosa
1.5 cm, single, firm, immobile mass
Myxoid and highly vascular background, giant cells and mitotic figures present
N/A
Price et al. 1993 [5]
2
17, M
Outer canthus of right eye
2.0 cm, single mass
Myxoid background, giant cells and mitotic figures (Less than 1/HPF) present
N/A
20, M
Subcutis adjacent to eye and beneath orbicularis oculi
1.0 cm, single, firm, non-tender, mobile mass
Myxoid background, giant cells and mitotic figures (Less than 1/HPF) present
N/A
Samaratunga et al. 1996 [2]
1
49, M
Left inguinal region
3.0 cm, single, firm, non-tender mass
Myxoid background with cleft-like spaces, giant cells and mitotic figures (2/10 HPF) present
(+) Vimentin, SMA
(-) LMWK, S100
Beer et al. 1996 [6]
1
18, F
Lateral thigh
2.0 cm, single, tender, mobile mass
Focally myxoid and highly vascular background, mitosis present
(-) SMA, S100
Sticha et al. 1997 [7]
1
4, M
Plantar aspect of right foot
3.0 cm, single, firm, tender, immobile mass
Myxomatous and hyalinized background, giant cell and mitosis present
N/A
Ito et al. 1999 [8]
1
26, M
Flexor side of right forearm
Single, tender mass
Fibrous and vascular background, giant cells and mitotic figures (up to 1/10 HPF) present
(+) Vimentin, SMA
(-) Desmin
Anand et al. 2007 [9]
1
20, F
Hypothenar eminence of right hand
3.0 cm, single, firm, non-tender, mobile mass
Fibrous background, giant cells and mitotic figures present
(+) SMA
(-) S100, desmin
Sugaya et al. 2007 [10]
1
66, M
Medial border of right foot
0.3 cm, single, non-tender, mobile mass
Myxoid background, no giant cells present, rare mitotic figures
(+) Vimentin
(-) SMA, cytokeratin, S100, desmin, CD31, CD34, c-kit
Pantanowitz et al. 2008 [11]
1
17, M
Wrist
1.2 cm, single mass
N/A
N/A
Wang et al. 2009 [12]
1
28, F
Left leg
Multiple, firm, non-tender masses
Myxoid background, no giant cells or mitotic figures present
(+) Vimentin, SMA
(-) Ketatin, S100, desmin
Chi et al. 2012 [13]
1
20, F
Upper lip
0.5 cm, single, firm, non-tender, mobile mass
Giant cells and mitotic figures (11/10 HPF) present
(+) SMA
(-) S100
Reiser et al. 2012 [14]
1
58, F
Right cheek
1.7 cm, single mass
Focally myxoid and highly vascular background, no giant cells present, rare mitotic figures
(+) SMA
Focal (+) Bcl-2
(-) Pankeratin, S100, desmin, EMA
Current case, 2013
1
21, F
Flank
0.5 cm, single mass
Fibrous background, giant cells present with no mitotic figures
(+) SMA
Focal (+) muscle specific actin
            (-) S100

*Size in greatest dimension.

EMA: epithelial membrane antigen.

HPF: high power field.

LMWK: low molecular weight keratin.

N/A: not applicable.

SMA: smooth muscle actin.



Article Categories:
  • Case Report

Keywords: Intravascular fasciitis, Flank, Nodular fasciitis.

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