A common tumor in an uncommon site: epithelioid Leiomyoma arising from the seminal vesicle-a case report.

BACKGROUND: Leiomyoma of the seminal vesicle is a rare leiomyoma characterized by the formation of benign leiomyomatous tissue within the seminal vesicle. Although histologically benign, excessive size can lead to urinary system disease if left untreated. Herein, we report a case of a seminal vesicle epithelioid leiomyoma. CASE PRESENTATION: A 36-year-old Chinese man sought medical attention at our hospital for urination pain and hemospermia. CT showed a 5.3 cm × 5.0 cm seminal vesicle mass with a mixed density in the right seminal vesicle. The gross specimen showed light yellow, gray, and white tissues, with softness and hemorrhage in some places. Histologically, it showed classic spindle cell proliferation, with spindle cells arranged in fascicles, and mitosis was rare. Immunohistochemistry showed frequent expression of smooth muscle markers, such as calponin, SMA, and desmin. A diagnosis of epithelioid leiomyoma was proposed according to the immunohistochemical findings and morphology. The patient did not receive adjuvant therapy. There was no evidence of tumor recurrence in the 10 months after surgery. CONCLUSIONS: We report the first case of epithelioid leiomyoma in the seminal vesicle. This disease should be included in the differential diagnostic list of seminal vesicle tumors with epithelioid morphology.

Vulvar epithelioid leiomyoma with myxoid change: A case report and literature review.

RATIONALE: Smooth muscle tumors of the vulva are infrequent neoplasms with diverse histologic features and unclear biologic behavior. Herein, we report a very rare case of vulvar epithelioid leiomyoma and review of previous reported cases of these tumors. In addition, we have discussed the representative diagnostic criteria of vulvar smooth muscle tumors and prognostic significance of epithelioid morphology. PATIENT CONCERNS: We recently met a 45-year-old woman with complaint of painful vulvar mass. INTERVENTIONS: Excisional biopsy was performed. DIAGNOSES: Pathologic examination revealed a vulvar epithelioid leiomyoma with multinodular growth pattern. Mitotic activity was rare and cellular atypia was not identified. Based on histology and immunohistochemical staining results, the case was diagnosed as vulvar epithelioid leiomyoma. OUTCOMES: After mass excision, the patient was discharged with no complication and there was no evidence recurrence for 6 months. LESSONS: After reviewing previous papers and diagnostic criterion, we thought that vulvar smooth muscle tumors with predominant epithelioid morphology may be associated with unfavorable prognosis, Therefore, pathologists should examine the epithelioid component in vulvar smooth muscle tumors carefully.

Review of the pathophysiology, diagnosis, and therapy of vulvar leiomyoma, a rare gynecological tumor.

The objective of this article is to discuss the pathophysiology, diagnosis, differential diagnosis, and therapy of vulvar leiomyoma. We performed a review of all English-language reports of vulvar leiomyoma published in PubMed from 1978 to 2015 using the following search terms: "vulval leiomyoma," "vulvar leiomyoma," "vulval smooth muscle tumor," and "external genitalia smooth muscle tumor." Vulvar leiomyomas, which are rare benign monoclonal tumors, most commonly occur in the fourth and fifth decades of life. The genetics of vulvar leiomyoma remain undefined. Three principal histological patterns have been identified: spindled, epithelioid, and myxoid. Imaging tests such as ultrasound, endoscopic ultrasound, and magnetic resonance imaging are used in diagnosis. Surgical excision is the only curative treatment for vulvar leiomyomas. Establishment of a full differential diagnosis list and correct final diagnosis before surgery are essential for optimal clinical management. Although recurrence of vulvar leiomyoma is extremely rare, long-term follow-up of all cases is advisable.

Fertility after uterine artery embolization for symptomatic multiple fibroids with no other infertility factors.

OBJECTIVES: To evaluate the fertility of women eligible for surgical multiple myomectomy, but who carefully elected a fertility-sparing uterine artery embolization (UAE). METHODS: Non-comparative open-label trial, on women ≤40 years, presenting with multiple symptomatic fibroids (at least 3, ≥3 cm), immediate pregnancy wish, and no associated infertility factor. Women had a bilateral limited UAE using tris-acryl gelatin microspheres ≥500 µm. Fertility, ovarian reserve, uterus and fibroid sizes, and quality of life questionnaires (UFS-QoL) were prospectively followed. RESULTS: Fifteen patients, aged 34.8 years (95%CI 32.2-37.5, median 36.0, q1-q3 29.4-39.5) were included from November 2008 to May 2012. During the year following UAE, 9 women actively attempting to conceive experienced 5 live-births (intention-to-treat fertility rate 33.3%, 95%CI 11.8%-61.6%). Markers of ovarian reserve remained stable. The symptoms score was reduced by 66% (95%CI 48%-85%) and the quality of life score was improved by 112% (95%CI 21%-204%). Uterine volume was reduced by 38% (95%CI 24%-52%). Women were followed for 43.1 months (95%CI 32.4-53.9), 10 live-births occurred in 8 patients, and 5 patients required secondary surgeries for fibroids. CONCLUSION: Women without associated infertility factors demonstrated an encouraging capacity to deliver after UAE. Further randomized controlled trials comparing UAE and myomectomy are warranted. KEY POINTS: • Women without infertility factors showed an encouraging delivery rate after UAE. • For women choosing UAE over abdominal myomectomy, childbearing may not be impaired. • Data are insufficient to definitively recommend UAE as comparable to myomectomy. • Further randomized trials comparing fertility after UAE or myomectomy are warranted.

Possible involvement of inflammatory/reparative processes in the development of uterine fibroids.

Uterine leiomyomas are benign tumors in the smooth muscle layer of the uterus. The most common histological type is the "usual leiomyoma", characterized by overexpression of ECM proteins, whereas the "cellular type" has higher cellular content. Our objective is to investigate the involvement of inflammatory and reparative processes in leiomyoma pathobiology. Using a morphological approach, we investigate the presence of inflammatory cells. Next, we determine the localization of the ECM, the presence/absence of fibrotic cells via α-sma and desmin and the immunohistochemical profile of the mesenchymal cells with respect to CD34. Finally, we explore the effect of inflammatory mediators (TNF-α, IL-1ß, IL-6, IL-15, GM-CSF and IFN-γ) on pro-fibrotic factor activin A mRNA expression in vitro. Higher numbers of macrophages were found inside and close to leiomyomas as compared to the more distant myometrium. Cellular leiomyomas showed more macrophages and mast cells than the "usual type". Inside the fibroid tissue, we found cells positive for α-sma, but negative for desmin and a large amount of collagen surrounding the nodule, suggestive of myofibroblasts producing ECM. In the myometrium and leiomyomas of the "usual type", we identified numerous CD34+ fibroblasts, which are known to give rise to myofibroblasts upon loss of CD34 expression. In leiomyomas of the "cellular type", stromal fibroblasts were CD34-negative. Finally, we found that TNF-α increased activin A mRNA in myometrial and leiomyoma cells. In conclusion, this study demonstrates the presence of inflammatory cells in uterine leiomyomas, which may contribute to excessive ECM production, tissue remodeling and leiomyoma growth.

Ligation-assisted endoscopic enucleation for treatment of esophageal subepithelial lesions originating from the muscularis propria: a preliminary study.

An innovative ligation-assisted endoscopic enucleation (EE-L) technique was developed for the diagnosis and treatment of esophageal subepithelial lesions (smaller than 12 mm) originating from muscularis propria by combining endoscopic band ligation and endoscopic enucleation techniques. The aim of the study was to evaluate efficacy and safety of EE-L technique in the treatment of esophageal subepithelial lesions (smaller than 12 mm) originating from muscularis propria. Forty-seven esophageal subepithelial lesions (smaller than 12 mm) originating from the muscularis propria in 44 patients were treated with EE-L between September 2010 and September 2012. The lesion was first aspirated into the transparent cap attached to the tip of endoscope. The elastic band was then released around its base. The purpose of ligation was to force the lesion to assume a polypoid form with a pseudostalk. Endoscopic enucleation was then performed until the tumor was completely enucleated from muscularis propria using a hook knife and forceps. All tumors (median diameter: 8.2 ± 2.3 mm, range: 4-12 mm) were enucleated completely. Histopathology identified 45 tumors (95.7%) as leiomyoma, 2 (4.3%) tumors as gastrointestinal stromal tumor with very low risk. The mean time of the EE-L procedure was 12.5 ± 4.6 minutes (range: 6-23 minutes). Two patients experienced self-limiting, non-life-threatening hemorrhage after EE-L. No perforation and massive hemorrhage requiring further endoscopic or surgical intervention occurred. There were no recurrences during the 6-24 months follow-up period. EE-L offers the option of localized treatment of small esophageal muscularis propria tumors (smaller than 12 mm) with relatively few complications and low mortality, and provides the advantage of allowing a histopathological diagnosis. All the resected lesions in this study had a benign pathology.

Spontaneous leiomyomas of the gastroesophageal junction in a chimpanzee (Pan troglodytes).

A 49-y-old, female chimpanzee presented with a history of cardiac failure. Postmortem examination revealed lesions consistent with congestive heart failure and 2 incidental, round, firm, pale-tan intramural nodules (diameter, 2 cm) in the stomach at the gastroesophageal junction (GEJ). Histologically, the GEJ nodules were diagnosed as benign spindle-cell tumors. Immunohistochemical evaluation revealed neoplastic cells diffusely labeled with α-smooth muscle actin and vimentin, multifocally labeled for desmin, and were negative for c-kit (CD117). Electron microscopy revealed intracytoplasmic bundles of myofilaments with dense bodies, basal lamina, and few pinocytic vesicles in the neoplastic cells. According to these findings, leiomyomas of the GEJ were diagnosed. Gastrointestinal stromal tumors have been documented to occur in chimpanzees, but there are no reports of GEJ leiomyomas. To our knowledge, this report is the first description of spontaneous leiomyomas of the GEJ in a chimpanzee.

Primary non-clear-cell adenocarcinoma of the vagina in a diethylstilbestrol exposed woman.

A 54-year-old woman with a history of in-utero diethylstilbestrol (DES) exposure, who had a prior hysterectomy for symptomatic leiomyomata and dysmenorrhea, presented for vaginal bleeding. Vaginal biopsies showed a non-clear-cell adenocarcinoma, and the patient was subsequently treated with radiation therapy. We present a case of primary vaginal non-clear-cell adenocarcinoma in a patient with in-utero DES exposure. Continued monitoring of older DES-exposed women for vaginal lesions is warranted because of reported cases of non-clear-cell adenocarcinoma and persistent risk of clear cell adenocarcinoma.

Unilateral ovarian agenesis and clear cell type epithelioid leiomyoma of uterus mimicking ovarian malignancy.

A case of unilateral absent ovary together with clear cell type epithelioid leiomyoma of uterus mimicking ovarian malignancy discovered during laparotomy is presented. Unilateral absence of an ovary is an extremely rare finding. Although the exact pathophysiological mechanism is not known, it could result from a defect in embryological development or asymptomatic torsion of ovary. Clear cell type epithelioid leiomyoma of uterus is also a rare variant, composed of round or polygonal 'clear' cells rather than typical spindle-shaped cells and ultra structurally differs from non-uterine counterparts.

Differentiation of large (≥ 5 cm) gastrointestinal stromal tumors from benign subepithelial tumors in the stomach: radiologists' performance using CT.

PURPOSE: To identify significant CT findings for the differentiation of large (≥ 5 cm) gastric gastrointestinal stromal tumors (GIST) from benign subepithelial tumors and to assess whether radiologists' performance in differentiation is improved with knowledge of significant CT criteria. MATERIALS AND METHODS: One-hundred twenty patients with pathologically proven large (≥ 5 cm) GISTs (n=99), schwannomas (n=16), and leiomyomas (n=5) who underwent CT were enrolled. Two radiologists (A and B) retrospectively reviewed their CT images in consensus for the location, size, degree and pattern of enhancement, contour, growth pattern and the presence of calcification, necrosis, surface ulceration, or enlarged lymph nodes. CT findings considered significant for differentiation were determined using uni- and multivariate statistical analyses. Thereafter, two successive review sessions for the differentiation of GIST from non-GIST were independently performed by two other reviewers (C and D) with different expertise of 2 and 9 years using a 5-point confidence scale. At the first session, reviewers interpreted CT images without knowledge of significant CT findings. At the second session, the results of statistical analyses were provided to the reviewers. To assess improvement in radiologists' performance, a pairwise comparison of receiver operating curves (ROC) was performed. RESULTS: Heterogeneous enhancement, presence of necrosis, absence of lymph nodes, and mean size of ≥ 6 cm were found to be significant for differentiating GIST from schwannoma (P<0.05). Non-cardial location, heterogeneous enhancement, and presence of necrosis were differential CT features of GIST from leiomyoma (P<0.05). Multivariate analyses indicated that absence of enlarged LNs was the only statistically significant variable for GIST differentiating from schwannoma. The area under the curve of both reviewers obtained using ROC significantly increased from 0.682 and 0.613 to 0.903 and 0.904, respectively, with information of the significant CT findings differentiating GISTs from non-GISTs (P<0.001). CONCLUSION: Non-cardial location, heterogeneous enhancement, presence of necrosis, larger lesion size, and absence of lymphadenopathy are highly suggestive CT findings for large GISTs in differentiation from schwannomas or leiomyomas. Regardless of radiologists' expertise, diagnostic performance in differentiation can be significantly improved with knowledge of these CT findings.

Adrenal perivascular epithelioid cell tumor: a case report with discussion of differential diagnoses.

A 50-year-old man presented with nausea and abdominal bloating sensation of a few months' duration and was found to have a large adrenal mass that was resected after computed tomography scan evaluation. The histopathologic examination showed epithelioid and spindle cells having clear to granular eosinophilic cytoplasm, and small, central, round to oval nuclei with small nucleoli. The tumor cells demonstrated reactivity for smooth muscle actin, HMB-45, Melan-A, and vimentin, with focal positivity for desmin and S100 protein and negative staining for inhibin, synaptophysin, and chromogranin. A diagnosis of perivascular epithelioid cell tumor was made. Perivascular epithelioid cell tumors can arise in a variety of locations, including omentum/mesentery, female genital tract, pelvic soft tissues, skin, and falciform ligament, but an adrenal primary tumor has not been previously reported, to the best of our knowledge. The differential diagnosis is broad and site-dependant and in our case includes several neoplasms with clear cells that can involve the adrenal gland, including a primary adrenocortical neoplasm. A review of the related literature and discussion of the differential diagnoses are presented.