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.

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.

Esophageal leiomyosarcoma diagnosed by endoscopic ultrasound-guided fine-needle aspiration.

Endoscopic ultrasound-guided fine-needle aspiration (EUS-FNA) has proven itself to be a reliable method for the diagnosis of gastrointestinal stromal tumors and other gastrointestinal mesenchymal neoplasms. The diagnosis and distinction of these entities remain important, as these neoplasms have different prognoses and treatment options. We present a case of a 14.5-cm esophageal mass in a 67-yr-old woman sampled by EUS-FNA. Smears showed a cellular, spindle cell lesion with marked cytologic and nuclear pleomorphism. A cell block showed neoplastic cells reacting strongly with antibodies to smooth muscle antigens and not with antibodies to keratin, CD34 and CD117. Resection confirmed the diagnosis of esophageal leiomyosarcoma. To the best of our knowledge, this represents the first reported case of an esophageal leiomyosarcoma diagnosed by EUS-FNA. Clinical, imaging, and cytologic features as well as differential diagnosis are discussed.

Renal angiomyolipoma with contrast-enhancing elements mimicking renal malignancy: radiographic and pathologic evaluation.

The diagnosis of angiomyolipoma can typically be made on the basis of characteristic computed tomography findings. Varying tissue compositions within an angiomyolipoma can create difficulty in pathologically differentiating benign from malignant lesions. Epithelioid angiomyolipoma is a variant of angiomyolipoma characterized by the presence of epithelioid cells. We report a case of two discrete contrast-enhancing lesions within an enlarging angiomyolipoma that radiographically mimicked malignant elements. This finding presented a diagnostic challenge in terms of selecting medical versus surgical intervention. Surgical excision of the lesions was performed and the pathologic examination revealed a benign angiomyolipoma with epithelioid features.

[Imaging features of gastric stromal tumors: radiologic-pathologic correlation. Report of 4 cases]. / Imagerie des tumeurs stromales gastriques: corrélations radio-anatomopathologiques. A propos de quatre cas.

GOAL: This study concerns the new anatomo-pathologic semantics of the ancient gastric leiomyoblastoma that become gastric stromal tumors (GST) and identified as stemming of "pace-maker" cells of Cajal related to the immunohistochemical characterization of the phenotype. MATERIAL: and methods. We limited the study to the mesenchymatous tumors to "pacemaker" cells. For this purpose, we report four documented observations of gastric stromal tumors correlated to the histology and to the immunohistochemical study. RESULTS: Although some signs are often described (exogastric development, heterogeneity with cystic and necrotic component, predominating peripheral enhancement...), radiological aspects of these tumors are not specific because, analogues to the other mesenchymal tumors (leiomyoma or schwannoma). The topographic diagnosis is difficult, realized by echoendoscopy, CT scan and MRI. The irregularity of contours evokes the malignancy as hemoperitoneum. GST do not show lymphophilic behavior that differentiates them from the adenocarcinomas and gastric lymphomas. The surgical treatment is the best treatment, allowing the immuno-histological diagnosis of certainty from the complete operative specimen. CONCLUSION: Some tumors are difficult in classifying despite classic histology necessitating immunohistochemical tests for the identification of muscular, nervous, autonomous nervous system flexion of mesenchymatous tumors with epithelioid or spindle cells of the gastrointestinal tract. Then a radio-clinical follow-up is therefore indicated: the evolution is the alone real marker of malignancy.

Endosonographic features predictive of benign and malignant gastrointestinal stromal cell tumours.

BACKGROUND/AIM: Some endoscopic ultrasonographic (EUS) features have been reported to be suggestive of malignancy in gastrointestinal stromal cell tumours (SCTs). The aim of this study was to assess the predictive value of these features for malignancy. METHODS: A total of 56 histologically proven cases of SCT studied by EUS between 1989 and 1996 were reviewed. There were 42 gastric tumours, 12 oesophageal tumours, and two rectal tumours. The tumours were divided into two groups: (a) benign SCT, comprising benign leiomyoma (n = 34); (b) malignant or borderline SCT (n = 22), comprising leiomyosarcoma (n = 9), leiomyoblastoma (n = 9), and leiomyoma of uncertain malignant potential (n = 4). The main EUS features recorded were tumour size, ulceration, echo pattern, cystic spaces, extraluminal margins, and lymph nodes with a malignant pattern. The two groups were compared by univariate and multivariate analysis. RESULTS: Irregular extraluminal margins, cystic spaces, and lymph nodes with a malignant pattern were most predictive of malignant or borderline SCT. Pairwise combinations of the three features had a specificity and positive predictive value of 100% for malignant or borderline SCT, but a sensitivity of only 23%. The presence of at least one of these three criteria had 91% sensitivity, 88% specificity, and 83% predictive positive value. In multivariate analysis, cystic spaces and irregular margins were the only two features independently predictive of malignant potential. The features most predictive of benign SCTs were regular margins, tumour size < or = 30 mm, and a homogeneous echo pattern. When the three features were combined, histology confirmed a benign SCT in all cases. CONCLUSIONS: The combined presence of two out of three EUS features (irregular extraluminal margins, cystic spaces, and lymph nodes with a malignant pattern) had a positive predictive value of 100% for malignant or borderline gastrointestinal SCT. Tumours less than 30 mm in diameter with regular margins and a homogeneous echo pattern are usually benign.

Sonographic and color Doppler findings of gastrointestinal myogenic tumors with a marked extraluminal growth.

We present five cases with gastro (four cases) and intestinal (one case) myogenic tumors with a marked extraluminal growth. In all cases, incidental discovery of an asymptomatic mass prompted further examination. One of three cases with a pedunculated growth mimicked a gallbladder cancer. The mass of a jejunal leiomyoma case changed markedly in location under probe compression. Color Doppler sonography confirmed not only the hypervascular nature of the mass but also feeding and draining vessels.

[Gastric leiomyoblastoma. Its preoperative diagnosis by echo-guided needle aspiration. A report of 2 clinical cases]. / Il leiomioblastoma gastrico. Diagnosi preoperatoria con agoaspirato ecoguidato. Descrizione di due casi clinici.

The authors report two cases of gastric leiomyoblastoma, one benign and one malignant, come to clinical observation for digestive hemorrhage; in both cases a preoperating diagnosis of benignity or malignity was made and in one case even of histotype, thanks to fine needle biopsy. After a short introduction regarding some historical notes, the predictive parameters of malignity, the classification of this rare myoid tumor, the authors discuss the distribution of the pathology in the population, the symptomatology, the diagnostic "iter" with particular regard to the needle biopsy, the surgical treatment, and the indispensable parameters for formulating a correct prognosis.

Epithelioid leiomyoma of the prostate.

We report a case of an unusual mesenchymal tumor of the prostate. Rectal-digital palpation and transrectal ultrasound showed a circumscribed and inhomogeneous tumor within the right prostatic lobe. Surgical treatment consisted of transurethral resection of the right prostatic lobe. On the basis of morphology and immunohistochemical detection of desmin and smooth-muscle-type actin, the diagnosis of an epithelioid leiomyoma was made. This is the first report of this entity in the prostate. A definite prediction of the biological potential is currently not yet possible.

[Gastric leiomyoblastoma. Histomorphologic and immunologic analysis of 3 cases]. / Leiomioblastoma gástrico. Análisis histomorfológico e inmunológico de tres casos.

We present three cases of Leiomioblastomas of stomach, diagnosed at the unit of Pathology of the Gastrointestinal Cancer Center "Dr Luis E. Anderson" -San Cristóbal-Táchira State Venezuela. The lesions are analyzed from the clinical, morphological and immunohistological points of view, utilizing Queratine and Vimentin techniques. This neoplasm is not frequent but it is interesting because its biologic behavior not always correspond to its histology-pattern.