Vulvovaginal Region Research Articles

Aggressive Angiomyxoma of the Female Genital Tract

Aggressive angiomyxoma (AAM), a distinctive neoplasm occurring in the female perineum and pelvis, was initially described in 1983. Subsequently reported to occur in the inguinoscrotal region in men, this uncommon specialized genital stromal tumor is characterized by a paucicellular spindle cell proliferation separated by loose myxoid stroma with a prominent thick-walled, hyalinized vascular component. Despite the bland histologic appearance, AAM is an infiltrative tumor with a propensity for aggressive local and regional recurrences, especially when incompletely excised. Due to involvement of the deep pelvic soft tissues and retroperitoneal regions, these tumors often attain a large size and may not be amenable to complete surgical excision. While AAM can be very difficult to distinguish histologically from other myxoid soft tissue lesions of the vulvovaginal region, especially from the so-called benign angiomyofibroblastoma, differences in the clinical behavior between these various neoplasms require accurate diagnosis to ensure proper treatment. A variety of immunohistologic strategies have been proposed to be of value in differentiating these various tumors, but in many instances their relative contribution is overemphasized. In problematic cases, careful evaluation of a few key histologic features, in concert with attention to a series of practical considerations, including patient age, determination of adequacy of biopsy and tumor sectioning, tumor size, borders, and location (superficial versus deep), should permit accurate classification.

Cellular angiofibroma: clinicopathologic and immunohistochemical analysis of 51 cases.

Cellular angiofibroma is a recently described histologically distinctive benign mesenchymal neoplasm composed of 2 principal components, the cellular spindle cell component and prominent stromal blood vessels. Cases in males have sometimes been called "angiomyofibroblastoma-like tumor." We describe a series of 51 cases of cellular angiofibroma to further characterize its clinicopathologic and immunohistochemical features. There were 26 women and 25 men, ranging in age from 22 to 78 years (mean 53.5, median 52 years). Men tended to be older than women. Tumor size ranged from 0.6 to 25.0 cm (overall median size 3.9 cm, median in women 2.7 cm, median in men 6.7 cm). Most common sites were the vulvovaginal region (22 cases) and the inguinoscrotal region (19 cases). Preoperative duration (known for 25 patients) ranged from 1 week to 5 years, with presentation as a painless mass, except for 1 case each with intermittent genital bleeding and a painful mass. Most lesions were located primarily in subcutaneous tissue. Most cases were grossly well marginated. Two cases showed foci of hemorrhage and 1 case showed foci of necrosis. Microscopically, 41 tumors were well circumscribed, and 2 tumors infiltrated into the surrounding tissue. All tumors consisted of bland, spindle-shaped cells, short bundles of wispy collagen and numerous small- to medium-sized thick-walled vessels. Intralesional fat was present in 12 cases (6 female and 6 male cases). Mild cytologic atypia (5 cases) and frequent mitoses (3 cases) were infrequent; significant nuclear atypia and abnormal mitoses were absent. By immunohistochemistry, 29 of 48 tumors (60%) expressed CD34, 10 of 48 (21%) SMA, 4 of 48 (8%) desmin, but none expressed S-100 protein. Follow-up information was available for 40 patients (range 4-168 months; mean 31.2 months) and no patient has developed recurrence or metastasis to date.

A Histologic, Immunohistochemical and Ultrastructural Study of Fibroma, Myofibroblastoma, Leiomyoma and Hemangiopericytoma in Cattle

The immunohistochemical features of tumors from 7 Holstein cows showing fibroblastic, myofibroblastic, smooth muscle or pericytic differentiation are described. In cases 1 and 2, the tumors were characterized by neoplastic fibroblasts immunostained with vimentin alone, but some cells appeared to transiently express alpha smooth muscle actin (SMA) during tumor development. Cutaneous (case 3) and nasal (case 4) myofibroblastomas, which were characterized by SMA and fibronectin positivity, with a minority of cells showing positive reactivity for desmin, were readily distinguishable from tumors of fibroblasts, smooth muscle cells and pericytes by fibronectin staining. The same staining pattern was seen in a vulvar myofibroblastoma (case 5), which differed from a vaginal leiomyoma (case 6) coexpressing SMA and desmin in nearly all neoplastic cells. Both neoplasms, however, had estrogen receptor (ER), and may be derived from immature stromal cells distributed in the vulvovaginal region. A hemangiopericytoma (case 7) resembled myofibroblastomas in cytoskeletal immunophenotypes, but lacked fibronectin. Benign or locally invasive soft tissue tumors with spindle cell morphology and expression of SMA could be divided into several types by immunostaining for SMA, desmin, fibronectin and BR.

Aggressive Angiomyxoma: An Ultrastructural Study of Four Cases

The histogenesis of the aggressive angiomyxoma of the vulvo-vaginal region was studied. Four cases of aggressive angiomyxoma were examined by using light microscopy, electron microscopy, and immunohistochemistry. Myofibroblastic from smooth muscle cell differentiation was distinguished by paying close attention to the structures of the neoplastic cell membrane. Aggressive angiomyxomas exhibit subtle features of smooth muscle differentiation, suggesting that the neoplastic cells differentiate from a multipotent perivascular cell.

Cellular pseudosarcomatous fibroepithelial stromal polyps of the lower female genital tract: an underrecognized lesion often misdiagnosed as sarcoma.

Fibroepithelial stromal polyps of the vulvovaginal region are benign lesions that, when bland or hypocellular, are readily recognized. However those that exhibit bizarre cytomorphology, atypical mitoses, or hypercellularity, raising the possibility of malignancy, continue to be underrecognized. The authors reviewed a series of fibroepithelial stromal polyps to characterize further the morphologic features that can lead to a misdiagnosis of sarcoma. A total of 33 of 65 consecutive cases of fibroepithelial stromal polyps retrieved from the authors' consultation files were remarkable for marked hypercellularity (33 of 33), marked cytologic pleomorphism (21 of 33), mitotic counts of more than 10 mitoses per 10 high-power fields (12 of 33), and the presence of atypical mitoses (14 of 33). A total of 16 of 33 lesions had three or more of these features. Important morphologic clues to the diagnosis (shared with usual polyps at this site) were lack of an identifiable lesional margin, extension of abnormal stromal tissue up to the mucosal-submucosal interface, and the frequent presence of individually scattered multinucleate stromal cells, most often located close to the surface epithelium. Immunohistochemically, seven of 12 cases were desmin positive and one of 11 cases were smooth muscle actin positive. The age range of patients was 16 to 75 years (median, 32 years), and 21 patients (64%) were premenopausal. Sites included the vagina (18 of 33), cervix (seven of 33), and vulva (eight of 33). A total of 14 of 33 patients were pregnant, three patients were taking Tamoxifen, and one patient was on oral progesterone. Eight of 33 patients had multiple lesions at the time of presentation, of whom five were pregnant. Clinical follow-up was available in 21 of 33 patients. Three of 21 patients with follow-up had local, nondestructive recurrence. Two of these patients had multiple recurrences. None of the patients followed developed metastases. Cytologic atypia has been a previously recognized feature in these lesions; however, the occurrence of marked stromal cellularity and a mitotic rate of more than 10 mitoses per 10 high-power fields have not been emphasized previously. Moreover, the combination of these features has only rarely been documented. Awareness of the spectrum of histologic features that these lesions can exhibit is crucial in their accurate recognition, thus avoiding potential overtreatment.

Most frequent scenario for recurrent Candida vaginitis is strain maintenance with "substrain shuffling": demonstration by sequential DNA fingerprinting with probes Ca3, C1, and CARE2.

The following three basic scenarios have emerged for the genetic relatedness of strains in recurrent vaginal candidiasis: strain maintenance without genetic variation, strain maintenance with minor genetic variation, and strain replacement. To test the frequency of each of the three scenarios, the genetic relatedness of Candida albicans isolates from each of 18 patients with recurrent infections was assessed by sequential DNA fingerprinting with the following three probes: the Ca3 probe; the C1 probe, a subfragment of the Ca3 probe which hybridizes to hypervariable genomic fragments; and the unrelated CARE2 probe. In each of the 18 patients with recurrent infections, the same strain was responsible for sequential infections, suggesting that the predominant scenario is strain maintenance. However, in 56% of these patients, the strain exhibited minor genetic variations in sequential infections. These changes were not found to be progressive. Rather, the changes suggest that substrains of an established infecting strain are shuffled in sequential infections. Results are also presented that in 45% of patients with recurrent infections, oral and vulvovaginal isolates were identical, in 35% they were highly related but not identical, and in 20% they were unrelated. These results differ markedly from those for commensal isolates simultaneously cultured from the oral cavity and vulvovaginal region of healthy individuals. Finally, it is demonstrated that in all eight cases in which C. albicans was isolated from both the male sexual partner of the patient with a recurrent infection and the patient, an isolate from the male partner was identical or highly related to the vulvovaginal strain. These results demonstrate that in patients with recurrent vulvovaginitis, a single strain usually dominates both in the different body locations of the patient and in the male partner and that it is maintained through sequential infections. However, in patients with recurrent infections, different substrains of the established clone dominate in an apparently random fashion, a process that we refer to as "substrain shuffling".

Genetic dissimilarity of commensal strains of Candida spp. carried in different anatomical locations of the same healthy women

Candida spp. carriage and strain relatedness were assessed in 52 healthy women at 17 anatomical locations by using an isolation procedure which assesses carriage intensity and by using a computer-assisted DNA fingerprinting system which computes genetic similarity between strains on the basis of the patterns of Southern blots probed with the moderately repetitive sequence Ca3. Candida spp. were cultured from 73% of the test individuals, most frequently from the oral (56%), vulvovaginal (40%), and anorectal (24%) regions. Half of the test individuals with Candida spp. carried the organism simultaneously in more than one of the three general areas of carriage. Isolates from different body locations of the same individual were either completely unrelated, identical, or highly similar but nonidentical. In 11 cases in which Candida spp. were simultaneously isolated from the oral cavity and vaginal canal, seven pairs of isolates were genetically unrelated and four pairs were similar but nonidentical. In the latter cases, the isolate pairs each appear to have arisen by genetic divergence from a single progenitor. A comparison of the genetic relatedness of isolates from different individuals further uncovered a single strain which was vaginospecific in the Iowa City, Iowa area and reduced genetic diversity among vulvovaginal strains compared with those isolated from other body locations. These results suggest that strains adapt to different anatomical locations and, conversely, that in a healthy individual there is anatomical selection of vaginotropic, anotropic and orotropic strains of Candida spp.

Rhabdomyoma of the cricopharyngeus in an infant

Rhabdomyoma is a rare benign tumor characterized histologically by striated skeletal muscle. Rhabdomyomas can be classified into adult and fetal variants. The majority of adult variants arise in the head and neck region and are derived from the skeletal muscles of the pharyngeal (branchial) arches. The majority of cases of fetal variant rhabdomyomas have been described in the postauricular region of young children and the vulvovaginal region in middle-aged women. We present a case of a 15-month-old infant with a circumferential rhabdomyoma of the cricopharyngeus muscle requiring excision of the esophageal inlet and reconstruction with a free microvascular jejunal interposition graft. This case represents the youngest reported case of adult variant rhabdomyoma. The clinical, radiographic, and histologic findings of this unusual case and a review of reported cases of rhabdomyoma in the pediatric population will be presented.

Intracranial trigeminal nerve rhabdomyoma/choristoma in a child: A case report and discussion of possible histogenesis

Rhabdomyomas are rare tumors that usually arise within the heart, orocervical, or vulvovaginal regions. The cardiac tumors have a characteristic immature morphology, occur often in association with tuberous sclerosis, and are regarded as hamartomas rather than true neoplasms. The histogenesis of the extracardiac tumors and their true neoplastic nature are matters of controversy. We report the first case of a rhabdomyoma located inside the cranium. The intimate association with the mandibular division of the trigeminal nerve, the normal embryogenesis of the craniofacial muscles, and animal homograft and xenograft experiments provide a framework for considering this tumor, and possibly other rhabdomyomas, as a choristoma/hamartoma rather than a true neoplasm.

Angiokeratoma vulvae

A healthy 25-year-old white woman presented with a 2-year history of vaginal pruritus, burning, and dyspareunia. Numerous grayish purple macular and papular lesions, ranging in size from 0.5 to 2.0 mm, were observed on both labia majora. Some were centrally eroded. Biopsy confirmed the diagnosis of angiokeratoma. All lesions were electrocauterized, with the patient under local anesthesia. No new lesions appeared during a 6-month follow-up period, and the patient remained asymptomatic. Vulval angiokeratomas probably occur more commonly than the literature suggests. Occasionally they may be confused with other lesions. Since they are easily treatable, clinicians should be alert for their presence when diagnosing pruritic, painful, or bleeding lesions of the vulvovaginal region.

Extracardiac rhabdomyoma: a clinicopathologic study and review of the literature.

Extracardiac rhabdomyomas are rare benign neoplasms that in the past have been divided by histologic criteria into adult and fetal types. In this series, 15 previously unpublished cases are presented and analyzed in combination with 51 acceptable cases from the literature. The adult type of rhabdomyoma occurs almost exclusively in the head and neck region (93% of all cases), particularly in the larynx and pharynx of adult males. The fetal type of rhabdomyoma may be subdivided on histologic grounds into myxoid and cellular variants. The fetal myxoid type usually occurs in the vulvovaginal region of middle aged women or in the postauricular region of male infants. The fetal cellular type has not been previously emphasized. This type tends to occur in the head and neck region of adult males. It may be mistaken for a sarcoma because of its high degree of cellularity. Local recurrence of a rhabdomyoma is rare and has only been occasionally reported in the adult type of rhabdomyoma. In no instance has a rhabdomyoma been shown to possess aggressive local growth or metastatic potential. Therefore, it is essential that these neoplasms be recognized histologically and that they be treated conservatively by local excision.