Consideraciones del Leiomioma y su Variante Intraósea en Mandíbula, Algoritmo de Trabajo Según Análisis de Literatura / Considerations of Leiomyoma and its Intraosseous Variant in the Jaw, Working Algorithm According to Literature Analysis

RESUMEN: El Leiomioma es una neoplasia benigna originada en el tejido muscular liso por lo que puede manifestarse en cualquier región del cuerpo humano que contenga músculo liso, siendo el sitio más común el útero, la piel y en el tracto gastrointestinal. La escasa cantidad de tejido muscular liso en boca hace que su manifestación oral sea infrecuente, representando solo 0.06 % del total de este tipo de tumores. Los sitios más afectados suelen ser los labios, las mejillas, el paladar, la lengua y encías. Histológicamente se distinguen tres tipos: el Leiomioma Sólido, Angioleiomioma (Vascular) y Leiomioma Epitelioide (Leiomioblastoma). El origen de esta neoplasia en boca suele ser la túnica media de los vasos sanguíneos. El Leiomioma intraóseo suele ser aún menos frecuente, y con un diagnóstico diferencial complejo, con histopatología que en varias ocasiones no suele ser fácil de clasificar. Si bien es definida como un tumor benigno, su manifestación intraósea puede llegar a ser localmente agresiva y con un diagnóstico controversial, debiendo abordarse muchas veces como una neoplasia maligna. El objetivo de este artículo es presentar una revisión de la literatura de esta variante intraósea de Leiomioma situada en mandíbula, sus consideraciones clínicas y un algoritmo de tratamiento.

ABSTRACT: Leiomyoma is a benign neoplasm, the origin is the smooth muscle tissue that can be found in any area of the human body, which contains smooth muscle tissue. The most common regions it can be located, are the uterus, the skin and the gastrointestinal tract. The low quantity of muscle tissue in the mouth leads to infrequent oral manifestation, representing only 0.06 % of these tumors. The most affected regions are the lips, cheeks, palate, tongue and gums. The Histologic classification is: Solid Leiomyoma, Angioleiomyoma (vascular) and Epithelioid Leiomyoma (Leiomyoblastoma). The origin of this tumor in the mouth is the tunica media of the blood vessels. Nevertheless, and in spite of being defined as a benign tumor, it can be extremely aggressive, be subject to controversial diagnosis, and must often be treated as a malign neoplasm. The Intraosseous Leiomyoma is infrequent and presents a complicated differential diagnosis, with a histopathology that many times cannot be easily classified. The aim of this article is to present a review of intraosseous variant Leiomyoma in the mandible, the clinicians´ considerations and a treatment algorithm.

Nódulo en la mama tras radioterapia / A Nodule on the Breast after Radiation Therapy

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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.

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.

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.

Epithelioid smooth muscle tumors of the uterus do not express CD1a: a potential immunohistochemical adjunct in their distinction from uterine perivascular epithelioid cell tumors.

Uterine epithelioid smooth muscle tumors and uterine perivascular epithelioid cell tumors (PEComas) are known to display such a substantial overlap in morphologic and immunophenotypic characteristics that the existence of the latter as a distinct clinicopathologic entity at this location has been called into question. Recent research suggests that the constituent entities of the PEComa family at all anatomical locations, including lymphangioleiomyomatosis of the uterus, uniformly display immunoreactivity for CD1a. The purpose of this study is to determine the proportion of uterine epithelioid smooth muscle tumors that may similarly be CD1a-positive. Representative sections from 18 archived epithelioid smooth muscle tumors of the uterine corpus (6 epithelioid leiomyosarcomas and 12 epithelioid leiomyomas), diagnosed and classified as such based on World Health Organization criteria, were subjected to immunohistochemical stains for CD1a and HMB-45. The epithelioid component of the tissue sections evaluated ranged from 10% to 100% (mean, 70%). Two cases were composed predominantly of cells with overtly clear cytoplasm. All cases were entirely negative for CD1a. Of 18 cases, 1 (5.5%) (an epithelioid leiomyosarcoma) displayed immunoreactivity for HMB-45 in scattered lesional cells that constituted approximately 5% of the overall tumoral volume for the case. All others were HMB-45-negative. Given their rarity, future studies are required to confirm that all PEComas of the uterus are indeed uniformly positive for CD1a. However, if the latter staining pattern is confirmed, our findings herein suggest that CD1a may be a useful immunohistochemical adjunct in distinguishing uterine epithelioid smooth muscle tumors from uterine PEComas.

Epithelioid vascular leiomyoma of the uterus mimicking glomangiomyoma.

CASE REPORT: An unusual case of epithelioid vascular leiomyoma mimicking glomangiomyoma arising in the uterine corpus of a 55-year-old Japanese woman is presented. The surgically resected uterine mass, measuring 4.0x3.5 cm2, demonstrated a dark red well-circumscribed tumor. Histologically, the rounded epithelioid cells around the dilated vessels showed gradual transition to spindled smooth muscle cells. Immunohistochemistry was positive for smooth muscle actin, but negative for collagen IV. CONCLUSION: The experience in this case emphasizes that glomangiomyoma-like feature is interesting and might indeed be a new histological variant of uterine leiomyomas.

Gastrointestinal stromal tumors express ras oncogene: a potential role for diagnosis and treatment.

BACKGROUND: Gastrointestinal stromal tumors (GISTs) constitute the largest category of nonepithelial neoplasms of the gastrointestinal tract. Histologically, they have a spindle cell appearance but stain by immunohistochemistry for the proto-oncogene, c-kit (CD117). There is some evidence that phosphorylation of these receptors leads to a cascade that may activate the ras/mitogen-activated protein kinase pathway, which may, in turn, allow other oncogenes to become active. HYPOTHESIS: Immunohistochemical staining pattern of GISTs will aid in their differentiation from other spindle cell tumors and predict clinical outcome in patients. DESIGN AND SETTING: Retrospective review of patient records and paraffin block specimens of spindle cell tumors. PATIENTS: We have identified 65 patients with spindle cell tumors of the gastrointestinal tract at our institution in the past 10 years. Tumors were diagnosed by their morphology as leiomyomas, leiomyoblastomas, or leiomyosarcomas. MAIN OUTCOME MEASURES: CD117 and ras p21 were stained by immunohistochemistry on formalin-fixed, paraffin-embedded sections of normal and tumor tissues. RESULTS: Of the 65 patients, there were 23 patients diagnosed as having GIST confirmed by CD117 expression and 42 patients without GIST. Gastrointestinal stromal tumor samples of 17 (77%) of 22 patients stained positive for ras protein compared with 0 of 27 patients with leiomyomas (P<.001). CONCLUSIONS: To our knowledge, this study is the first to demonstrate that GISTs stain positive for ras p21. This molecular trait may be a useful diagnostic tool in addition to the c-kit (CD117) to separate GISTs from leiomyomas and leiomyosarcomas. In the future, ras inhibitors may potentially be a therapeutic to treat GISTs.

Clinicopathologic study of primary malignant gastrointestinal stromal tumor of the stomach, with special reference to prognostic factors: analysis of results in 140 surgically resected patients.

BACKGROUND: Malignant gastrointestinal stromal tumors (GISTs), previously termed leiomyosarcomas or epithelioid leiomyosarcomas, are known to show wide variability in their malignancy. We evaluated the clinicopathological features of a large number of primary malignant gastric GISTs to clarify which features were independent prognostic factors. METHODS: Clinicopathologic features (age, sex, tumor location, mode of growth and size, surgical method, ulceration, cell type, nuclear atypia, cellularity, mitotic index, growth pattern, necrosis, hemorrhage, direct tumor invasion, peripheral lymphoid cuffing, expression of alpha-smooth muscle actin [alpha-SMA], desmin, caldesmon, vimentin, CD34, c-kit protein and s-100 protein, and MIB-1 index) were evaluated by multivariate analysis in 140 patients with resected primary malignant gastric GISTs identify independent prognostic factors. RESULTS: Univariate analysis showed that each of the following factors had a significant deleterious influence on prognosis: male sex, tumor size 10 cm or more, presence of ulceration, an epithelioid cell component, severe nuclear atypia, high cellularity, a mitotic index of more than 10, an exogastric or invasive growth pattern, necrosis, hemorrhage, direct tumor invasion of surrounding tissue, negative caldesmon immunoreactivity, positive S-100 protein immunoreactivity, and a MIB-1 antigen labeling index of more than 10%. multivariate analysis showed that male sex, tumor size 10 cm or more, presence of an epithelioid cell component, and a mitotic index of more than 10 were statistically significant indicators of a poor prognosis ( P = 0.013, 0.001, 0.014, and <0.001, respectively). Multivariate analysis using the MIB-1 index instead of a mitotic count showed that male sex, tumor size 10 cm or more, presence of necrosis, and a MIB-1 antigen labeling index of more than 10% were independent predictors of a poor prognosis ( P = 0.009, 0.001, 0.043, and <0.001, respectively). CONCLUSION: Male sex, tumor size 10 cm or more, and cell proliferation as estimated by the mitotic index or MIB-1 index are independent indicators of a poor prognosis in primary malignant gastric GIST.

Tumor estromal gástrico maligno de crecimiento omental simulando un angioma / Gastrointestinal stromal tumor with omental enlargement mimicking an angioma

Los tumores del estroma gastrointestinal son raros y difieren histológicamente, inmunohistoquímicamente y genéticamente de los típicos leiomiomas, leiomiosarcomas y leiomioblastoma. En el caso que se presenta la evaluación radiológica mediante tomografía computarizada y resonancia magnética no pudo precisar su localización exacta de origen (tumor gástrico con crecimiento principal en el epiplón menor) ni detectar las metástasis a distancia (implantes peritoneales y mesentéricos). Su comportamiento radiológico era de angioma exofítico hepático o epiploico. Estas características se debieron al crecimiento en el epiplón y al importante componente angiomatoso presente en su constitución histológica. El tamaño tumoral y los implantes metastásicos lo clasificaron como maligno. El conocimiento de este tipo de tumores es importante para sospecharlos con las pruebas diagnósticas y planificar correctamente el tratamiento tras obtener muestras histológicas (AU)

Pedunculated exogastric leiomyoblastoma presenting as a wandering abdominal mass.

We report the imaging findings in a case of pedunculated exogastric leiomyoblastoma presenting as a wandering abdominal mass. Ultrasound and computed tomography showed a large, mixed solid and cystic mass in the peritoneal cavity. Computed tomography clearly showed that the mass was connected to the stomach by a narrow stalk. Small bowel follow-through showed subtle extrinsic indentation of the gastric body. The mass wandered from the right to the left side of the peritoneal cavity during various radiologic examinations.

[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.

Dermatofibroma con células granulares: a propósito de un caso y revisión de la literatura / Granular cell dermatofibroma: a case report and review of the literature

PResentamos un caso de dermatofibroma epitelioide rico en células granulares. Solamente se han descrito 11 casos de dermatofibroma de células granulares, de los cuales sólo tres tienen rasgos epitelioides. Es importante reconocer este subtipo de dermatofibroma, ya que puede confundirse con un gran número de procesos benignos o malignos, de diferente aplicación pronóstica y terapeútica (AU)

[Gastric leiomyoblastomas: a clinical case report]. / Leiomioblastomi gastrici: contributo clinico.

Leiomyoblastomas are about the 10% of non epithelial gastric neoplasms. They are interesting for clinical, therapeutic and prognostic findings very different in comparison commonest epithelial tumours. The authors, after discussing aetiology, pathogenesis, clinical outline, diagnosis, histological and pathological features as well as therapeutic treatment of these neoplasms, report a case describing diagnostic and therapeutic management.

[A case of epithelioid leiomyoma (leiomyoblastoma) of the urethra].

A 25-year-old woman was admitted to our hospital because of a painless, slow growing mass arising from the external genitalia. The mass had been developing for a few years. She did not have difficulty voiding nor was there hematuria. Magnetic resonance imaging revealed a well-circumscribed, 4 cm, solid tumor between the urethra and the anterior vaginal wall. After needle biopsy confirmed a benign tumor arising from the smooth muscle, the tumor was removed by a transvaginal approach. The histopathological diagnosis was epithelioid leiomyoma (leiomyoblastoma), which often occurs in the stomach or uterus, but seldom around the urethra. The patient has remained well without recurrence for 20 months after surgery.

Leiomioblastoma primario de epiplón. Aportación de un caso y revisión bibliográfica / Primary leiomyoblastoma of the omentum. A case report and a review of the literature

Presentamos un caso de leiomioblastoma primario de epiplón intervernido en nuestro servicio. Estos tumores son neoplasias que derivan del músculo liso y afectan fundamentalmente al estómago. La aparición como tumores primarios de epiplón es extremadamente rara. En la bibliografía médica mundial solamente se han publicado 14 casos. Aportamos un nuevo caso y realizamos una revisión bibliográfica discutiendo su clínica, diagnóstico, criterios histológicos de malignidad y tratamiento (AU)

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