Reactive Fibroblastic and Myofibroblastic Proliferation of the Vulva (Cyclist's Nodule): A Hitherto Poorly Described Vulval Lesion Occurring in Cyclists
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."
- [Show abstract] [Hide abstract] ABSTRACT: Perineal nodular induration (PNI) is a fibroblastic pseudotumor that presents almost exclusively in male cyclists. It develops in the soft tissues of the perineum immediately posterior to the scrotum, as a bilateral or single, central or lateralized mass. Although well known to sport medicine specialists, it is a scarcely documented entity in the pathology literature. We present 2 cases of PNI with fine-needle aspiration cytology and immunohistochemistry. They consisted of a paucicellular fibroblastic proliferation containing CD34-reactive spindle and epithelioid cells, small foci of fibrinoid degeneration, numerous blood vessels, and entrapped groups of mature fat cells. Our cases show that the histopathological features of PNI are more varied than those previously described and its immunohistochemical profile is wider. A central cystic focus and a zonal pattern are not consistent features of this entity. The lesional cells can express CD34, a hitherto unreported immunohistochemical finding.0Comments 3Citations
- [Show abstract] [Hide abstract] ABSTRACT: The wide variety of mesenchymal lesions that involve the vulvovaginal region can result in diagnostic difficulties for pathologists, in part because of their relative rarity but also because of overlapping morphological features. They can be divided into those lesions which are specific to or characteristic of this site and those which can occur at any site with no predilection for the vulvovaginal region. Many of the site-specific or characteristic lesions are thought to arise from the specialized subepithelial stroma of the lower female genital tract that extends from the cervix to the vulva; the stromal cells of this region are hormone responsive and exhibit positive immunohistochemical staining with estrogen receptor (ER) and progesterone receptor (PR). As a consequence, most of the site-specific mesenchymal lesions are positive with ER and PR. Immunoreactivity with both desmin and CD34 is also common; this constitutes an unusual immunophenotype since mesenchymal lesions at other sites are uncommonly positive with both markers. The best known of the site-specific lesions is aggressive angiomyxoma, an infiltrative neoplasm with a marked propensity for local recurrence following excision. Recent developments with regard to aggressive angiomyxoma include the description of occasional metastasizing cases, the potential value of gonadotropin-releasing hormone agonists in management and the emergence of HMGA2 as a valuable diagnostic marker. Most of the other site-specific mesenchymal lesions are well circumscribed and exhibit little tendency for local recurrence. These include angiomyofibroblastoma, fibroepithelial stromal polyp, superficial myofibroblastoma of the lower female genital tract, and cellular angiofibroma. Smooth muscle tumors also occur in this region and are more likely than their uterine counterparts to have an epithelioid or myxoid appearance. Vulvovaginal smooth muscle neoplasms exhibit a propensity for local recurrence and the morphological features which predict malignant behavior differ from uterine smooth muscle neoplasms. Relatively, recently described mesenchymal lesions in the vulvovaginal region include massive vulval edema, prepubertal vulval fibroma, and reactive fibroblastic and myofibroblastic proliferation of the vulva (cyclist’s nodule). Gastrointestinal stromal tumors have been described as primary neoplasms in the rectovaginal septum and vagina (termed extragastrointestinal stromal tumors). A wide range of other mesenchymal lesions potentially occur in this region, and it is stressed that when dealing with a vulvovaginal mesenchymal lesion, as well as considering the site-specific lesions, pathologists should consider a wide range of diagnoses since many mesenchymal lesions potentially occur in this region.0Comments 0Citations
- [Show abstract] [Hide abstract] ABSTRACT: To define the clinical features, imaging results, histopathologic patterns, and clinical outcomes seen in patients who develop vulval swelling as a result of intensive cycling. The case notes of 8 female cyclists were retrospectively reviewed. The mean age of the patients was 45 years, and all were cycling long distances each week (range, 125-450 km; median, 210 km). All patients had a unilateral swelling of the labium majus. Five patients had magnetic resonance imaging showing asymmetry of the vulva with no enhanced signaling associated with inflammation. Histologic examination in 3 cases revealed dermal fibrosis and dermal edema associated with dilatation of lymphatic vessels. In 1 case, a perifollicular inflammatory infiltrate containing epithelioid granulomas was seen but Crohn's disease was excluded. In another case, fibromuscular hyperplasia was seen. Six patients had surgical excision of the swollen area, and 4 patients were able to resume cycling without problems up to 5 years of follow-up. The cosmetic appearance was initially considered satisfactory by 5 patients. However, 2 patients required a second surgical procedure to further improve the cosmetic appearance. In 1 case, there was a postoperative hematoma followed by an immediate recurrence of the swelling, which persisted. Unilateral swelling of the labium majus occurring in cyclists can be related to dermal fibroedema associated with lymphatic dilatation or to an increase in adipose tissue. Surgical results were satisfactory in 5 of the 6 patients who underwent excision.0Comments 1Citation