Ischemic Fasciitis: An Unusual Vulvovaginal Spindle Cell Lesion

Scanlon, Robert; Kelehan, P.; Flannelly, Grainne; McDonald, Douglas J.; McCluggage, W. Glenn

doi:10.1097/01.pgp.0000101150.01933.21

Cited by 16 publications

(7 citation statements)

References 7 publications

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“…Similar cases have been described involving the upper and lower extremities of obese patients 57 . There has been a single report of another vulval lesion in a paraplegic patient 58 . This was an ulcerated lesion with fibrinoid necrosis and high cellularity, which was considered to be histologically identical to ischaemic fasciitis or atypical decubital fibroplasia 58 .…”

Section: Massive Vulval Oedemamentioning

confidence: 99%

“…There has been a single report of another vulval lesion in a paraplegic patient 58 . This was an ulcerated lesion with fibrinoid necrosis and high cellularity, which was considered to be histologically identical to ischaemic fasciitis or atypical decubital fibroplasia 58 . The authors speculated that this lesion was secondary to prolonged immobilization and might, with massive oedema, form part of a spectrum of vulval lesions in immobilized patients.…”

Section: Massive Vulval Oedemamentioning

confidence: 99%

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Recent developments in vulvovaginal pathology

2009

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This review discusses recent developments in vulvovaginal pathology. A variety of morphologically bland mesenchymal lesions occur at this site with considerable histological and immunohistochemical overlap. Aggressive angiomyxoma exhibits HMGA2 immunoreactivity in approximately 50% of cases, and this nuclear transcription factor is emerging as a useful and relatively specific marker for aggressive angiomyxoma, although occasional vulvovaginal smooth muscle neoplasms are positive. HMGA2 is useful in the diagnosis of aggressive angiomyxoma and its distinction from mimics, in the evaluation of resection margins and in the assessment of the presence or absence of residual disease in re-excisions. Aggressive angiomyxoma is almost invariably positive with oestrogen and progesterone receptors, and there have been several reports of a dramatic reduction in size following gonadotropin releasing hormone agonist therapy. Recent series of the relatively newly described entities cellular angiofibroma and superficial myofibroblastoma of the lower female genital tract have expanded upon the morphological spectrum of these neoplasms. Recently described mesenchymal lesions at this site include massive oedema and prepubertal vulval fibroma. Gastrointestinal stromal tumours have been described as primary neoplasms in the vagina, and rectovaginal septum and extragastrointestinal stromal tumour should be added to the differential diagnosis of a vulvovaginal mesenchymal lesion. Many mesenchymal lesions in the vulvovaginal region exhibit immunoreactivity with both CD34 and desmin, a somewhat unusual immunophenotype in mesenchymal lesions at other sites. It is now established that there are two distinct types of vulval intraepithelial neoplasia (VIN), most commonly termed classic and differentiated VIN, the former associated with human papillomavirus (HPV) infection. There are two corresponding types of vulval squamous carcinoma with HPV-associated and non-HPV-associated variants, the latter often arising in a vulval dystrophy and associated with p53 mutation. However, in some cases there is clinicopathological overlap between HPV-associated and non-HPV-associated squamous carcinomas, and immunohistochemistry with p16 is more reliable than morphology in predicting the presence of HPV. There have been new developments regarding Paget's disease of the vulva with the identification of markers that are useful in diagnosis and evidence that the neoplastic cells represent a proliferation of adnexal stem cells residing in sebaceous units. The newly described entity vaginal tubulo-squamous polyp typically exhibits immunopositivity with prostatic markers, possibly indicating derivation from displaced periurethral Skene's glands.

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Section: Massive Vulval Oedemamentioning

confidence: 99%

Section: Massive Vulval Oedemamentioning

confidence: 99%

Recent developments in vulvovaginal pathology

2009

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“…14,17 A single case of ischemic fasciitis has been reported in the vulva in a paraplegic. 19 As discussed, perineal nodules have been reported in male cyclists but there has been only limited documentation of the morphologic features. The histologic features in 1 case were of central pseudocyst formation surrounded by dense fibrous tissue, which in turn was surrounded by edematous fibrous tissue containing fibroblast-like spindle cells together with degenerate elastic fibers and hemorrhagic foci.…”

Section: Discussionmentioning

confidence: 97%

Reactive Fibroblastic and Myofibroblastic Proliferation of the Vulva (Cyclist's Nodule)

McCluggage

Smith

2011

American Journal of Surgical Pathology

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Perineal nodules occurring in male cyclists are reported in the literature, although the histologic features are not extensively documented. There has been little description of similar lesions in the female population. We describe 4 cases in which a vulval nodule or swelling developed in competitive female cyclists aged 15 to 45 years. The lesions were unilateral and occurred on the right or left labium majus (2 cases each). The histologic features were similar in all cases and consisted of a haphazard admixture of adipose tissue, variably cellular hyalinized tissue containing bland spindle-shaped fibroblasts, blood vessels, and nerve fibers. In some areas, thick cords of fibrous tissue imparted a keloid-like appearance. Other histologic features included plump mesenchymal cells with round or ovoid nuclei and abundant eosinophilic cytoplasm resulting in an epithelioid, plasmacytoid, or ganglion-like appearance (2 cases), a lymphocytic infiltrate around blood vessels (3 cases), foci of fat necrosis (1 case), and collections of elastic fibers (2 cases). One case recurred, the histologic features of the recurrent lesion being identical to the original. The overall morphologic appearances, especially in the cases with plump mesenchymal cells, bore some resemblance to proliferative fasciitis. Immunohistochemically, the cells were estrogen receptor positive and the plump mesenchymal cells were smooth muscle actin positive, in keeping with myofibroblasts. Desmin, S100, CD34, and HMGA2 were negative. Pathologists should be aware of this pseudoneoplastic lesion occurring on the vulva, which arises in a specific clinical setting and has the potential to be misdiagnosed as a variety of other mesenchymal lesions. We term this lesion as reactive fibroblastic and myofibroblastic proliferation of the vulva or "cyclist's nodule."

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“…There has been a single report of another vulval lesion in a paraplegic patient (21). This was an ulcerated lesion with fibrinoid necrosis and high cellularity and which was considered to be histologically identical to the lesion known as ischemic fasciitis or atypical decubital fibroplasia (22).…”

Section: Discussionmentioning

confidence: 98%

Massive Vulval Edema Secondary to Obesity and Immobilization: A Potential Mimic of Aggressive Angiomyxoma

McCluggage

Nielsen

Young

2008

International Journal of Gynecological Pathology

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We report 2 cases of surgically resected vulval masses in women aged 27 and 40 years. One patient was wheelchair bound and the other was obese, both presented with bilateral vulvar swelling. One specimen measured 45 cm in maximum dimension and the other 5 cm and were described as grossly edematous or gelatinous. Histologically, in both cases, there was edema of the skin overlying the lesion. The lesion itself consisted of markedly edematous connective tissue with widely separated bland spindle-shaped cells and numerous dilated vascular channels, sometimes surrounded by cuffs of lymphocytes and plasma cells. In the larger of the 2 specimens, underlying edematous adipose tissue was present. To some extent, the appearances, especially the morphological features, mimicked aggressive angiomyxoma because of the presence of a mass, the lack of circumscription, the hypocellular, edematous appearance, and the presence of numerous vascular channels. However, a combination of clinical and pathological features, including bilateralism, lack of a true myxoid stroma, the presence of perivascular cuffs of lymphoid cells, and lack of staining with estrogen receptor, is against aggressive angiomyxoma. The appearances were interpreted as those of massive edema. In one case, there was recurrence of the mass after surgery. There has been a single previous report of a similar vulvar case in a quadriplegic female patient and of similar cases involving the upper and lower extremities of obese patients. Clinicians and pathologists should be aware of the existence of this lesion, which is likely due to lymphatic obstruction and lymphedema secondary to immobilization and obesity.

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Ischemic Fasciitis: An Unusual Vulvovaginal Spindle Cell Lesion

Cited by 16 publications

References 7 publications

Recent developments in vulvovaginal pathology

Recent developments in vulvovaginal pathology

Reactive Fibroblastic and Myofibroblastic Proliferation of the Vulva (Cyclist's Nodule)

Massive Vulval Edema Secondary to Obesity and Immobilization: A Potential Mimic of Aggressive Angiomyxoma

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