Tumor nasal de músculo liso de comportamiento maligno incierto: informe de un caso / Nasal smooth muscle tumor of uncertain malignant potential: A case report

Los tumores de músculo liso que no pueden ser clasificados según su histología como leiomiomas o leiomiosarcomas se denominan tumores de músculo liso de comportamiento maligno incierto. La localización nasal de estos tumores es muy infrecuente y la extensión adecuada de la cirugía para tratar estas neoplasias no está bien definida. Se describe el caso clínico de una adolescente de 16 años, que consultó por padecer un tumor de aspecto vascular en la cavidad nasal derecha y que fue tratada con éxito mediante cirugía intranasal. El diagnóstico histológico fue tumor de músculo liso de comportamiento maligno incierto. Por la rareza de estas neoplasias, su infrecuente localización nasal y la falta de evidencia que soporte cuál debe ser la extensión de la cirugía, es relevante la descripción y discusión del caso clínico.

Smooth muscle tumors that cannot be histologically classified as leiomyomas or leiomyosarcomas are defined as smooth muscle tumors of uncertain malignant potential. The location of these tumors in the nose is very rare, and the appropriate surgical extent to manage these neoplasms has not been adequately defined. Here we describe the case of a 16-year-old female adolescent who consulted due to a vascular-like tumor in the right nasal cavity who was successfully treated with intranasal surgery. The histological diagnosis was smooth muscle tumor of uncertain malignant potential. Given that these neoplasms are rare, the uncommon location in the nose, and the lack of evidence indicating the extent of surgery, it is relevant to describe and discuss this clinical case.

Fumarate hydratase deficient uterine leiomyoma: a clinicopathological and molecular analysis of 80 cases / 中华病理学杂志

Objective: To investigate the clinicopathologic and molecular characteristics of fumarate hydratase (FH) deficient uterine leiomyoma. Methods: Eighty cases of FH deficient uterine leiomyoma were diagnosed from April 2018 to September 2022 in Department of Pathology, Peking University Third Hospital. Sanger sequencing of FH gene exons (exon 1-10) were performed on tumor tissues and matched non-tumor tissues/peripheral blood for all cases. FH immunohistochemistry were performed in 74 cases; S-(2-succino)-cysteine (2SC) were also detected by immunohistochemistry in five cases. Results: Patients' age ranged from 18 to 54 (36.0±7.5) years, with more than 60% exhibiting clinical symptoms of multiple and large leiomyomas (the median diameter was 70 mm). More than four histologic features, including staghorn vasculature, alveolar-pattern edema, bizarre nuclei, oval nuclei arranged in chains, prominent eosinophilic nucleoli with perinucleolar haloes and eosinophilic intracytoplasmic globules were observed in 98.5% (67/68) patients. The immunohistochemical sensitivity of FH and 2SC were 97.3% and 100%, respectively. Based on the Sanger sequencing results, the cases were divided into germline variant group (31 cases), somatic variant group (29 cases) and no variant group (20 cases). Sixty-nine percent (20/29) of the patients with FH germline variation had clear family history. Conclusions: Clinical features, histological morphology, FH and 2SC immunohistochemistry and Sanger sequencing have their own significance and limitations in differential diagnosis of FH deficient uterine leiomyoma. In clinical practice, the above information should be fully integrated and studied for accurate pathologic diagnosis and selection of patients with FH germline variation.

Mammary myofibroblastoma: a clinicopathological analysis of fifteen cases / 中华病理学杂志

Objective: To investigate the clinicopathological features, diagnosis and differential diagnosis of breast myofibroblastoma. Methods: The clinicopathological data and prognostic information of 15 patients with breast myofibroblastoma diagnosed at the Department of Pathology of the First Affiliated Hospital of Zhengzhou University, Zhengzhou, China from 2014 to 2022 were collected. Their clinical characteristics, histological subtypes, immunophenotypes and molecular characteristics were analyzed. Results: There were 12 female and 3 male patients, ranging in age from 18 to 78 years, with a median and average age of 52 years. There were 6 cases in the left breast and 9 cases in the right breast, including 12 cases in outer upper quadrant, 2 cases in inner upper quadrant and 1 case in outer lower quadrant. Most of the cases showed a well-defined nodule grossly, including pushing growth under the microscope in 13 cases, being completely separated from the surrounding breast tissue in 1 case, and infiltrating growth in 1 case. Among them, 12 cases were classic subtype and composed of occasional spindle cells with varying intervals of collagen fiber bundles; eight cases had a small amount of fat; one case had focal cartilage differentiation; one case was epithelioid subtype, in which epithelioid tumor cells were scattered in single filing or small clusters; one case was schwannoma-like subtype, and the tumor cells were arranged in a significant palisade shape, resembling schwannoma, and one case was invasive leiomyoma-like subtype, in which the tumor cells had eosinophilic cytoplasm and were arranged in bundles, and infiltrating into the surrounding mammary lobules like leiomyoma. Immunohistochemical studies showed that the tumor cells expressed desmin (14/15) and CD34 (14/15), as well as ER (15/15) and PR (15/15). Three cases with histologic subtypes of epithelioid subtype, schwannoma-like subtype and infiltrating leiomyoma-like subtype showed RB1 negative immunohistochemistry. Then FISH was performed to detect RB1/13q14 gene deletion, and identified RB1 gene deletion in all three cases. Fifteen cases were followed up for 2-100 months, and no recurrence was noted. Conclusions: Myofibroblastoma is a rare benign mesenchymal tumor of the breast. In addition to the classic type, there are many histological variants, among which the epithelioid subtype is easily confused with invasive lobular carcinoma. The schwannoma-like subtype is similar to schwannoma, while the invasive subtype is easily misdiagnosed as fibromatosis-like or spindle cell metaplastic carcinoma. Therefore, it is important to recognize the various histological subtypes and clinicopathological features of the tumor for making correct pathological diagnosis and rational clinical treatment.

Accuracy of Transvaginal Ultrasound in the Diagnosis of Intrauterine Lesions / Acurácia da ultrasonografia tranvaginal no diagnóstico de lesões intrauterinas

Abstract Objective To evaluate the accuracy of transvaginal ultrasound in the diagnosis of intrauterine lesions, using hysteroscopy as the gold standard. Methods This was a prospective observational study with 307 patients. All patients underwent hysteroscopy after a previous transvaginal ultrasound to compare the results. The hysteroscopy was performed by experienced examiners, and transvaginal ultrasounds were performed in various public and private services, which is reflective of routine healthcare practices in obstetrics and gynecology. The sensitivity, specificity, and accuracy of the transvaginal ultrasound were calculated using hysteroscopy as the gold standard. The level of agreement between the two exams was calculated using the Kappa test. Results Themean age was 56.55±12.3 years. For endometrial polyps, we observed a sensitivity of 39.8%, specificity of 72.7%, accuracy of 52.8%, and Kappa index of 0.11 (p=0.025). For fibroids, the sensitivity was 46.7%, specificity was 95.0%, accuracy was 87.9%, and Kappa index was 0.46 (p<0.001). For endometrial thickening, the sensitivity was 68.7%, specificity was 41.7%, accuracy was 47.6%, and Kappa index was 0.06 (p=0.126). For endometrial atrophy, we found a sensitivity of 6.7%, specificity of 99.3%, accuracy of 90.2%, and Kappa index of 0.10 (p=0.006). For the other findings, the sensitivity was 15.6%, specificity was 99.6%, accuracy was 87.3%, and Kappa index was 0.23 (P<0.001). Conclusion Our study demonstrated a low level of accuracy of transvaginal ultrasound for the diagnosis of endometrial lesions, when performed by a non-experienced professional. Thus, it is important to consider the use of hysteroscopy to avoid unnecessary and inappropriate treatments.

Resumo Objetivo Avaliar a acurácia do ultrassom transvaginal para o diagnóstico de lesões intrauterinas, tendo a histeroscopia como padrão de referência. Métodos Foi realizado um estudo observacional prospectivo em 307 pacientes, submetidas à histeroscopia após ultrassonografia prévia para comparação dos resultados. A histeroscopia foi realizada por duas médicas com experiência, e os exames de ultrassom foram realizados em diversas fontes, públicas ou privadas, como ocorre no cotidiano da assistência à saúde em nosso meio. Foram avaliados sensibilidade, especificidade e acurácia, tendo a histeroscopia como padrão-ouro. O nível de concordância foi avaliado pelo teste de Kappa. Resultados A idade média foi de 56,55±12,3 anos. Os resultados para pólipo endometrial foram: sensibilidade 39.8%, especificidade 72,7%, acurácia de 52,8%, e índice Kappa 0,11 (p=0,025). Para mioma, sensibilidade 46,7%, especificidade 95,0%, acurácia 87,9%, e índice Kappa 0,46 (p<0,001). Para espessamento endometrial, sensibilidade 68,7%, especificidade 41,7%, acurácia 47,6%, e índice Kappa de 0,06 (p=0,126). Para atrofia, sensibilidade 6,7%, especificidade 99,3%, acurácia 90,2%, e índice Kappa 0,10 (p=0,006). Para outros achados, sensibilidade 15,6%, especificidade 99,6%, acurácia 87,3%, e índice Kappa 0,23 (p<0,001). Conclusão Nosso estudo demonstrou baixo nível de acurácia da ultrassonografia transvaginal para o diagnóstico de lesões endometriais, quando realizada por profissional não experiente. Assim, é importante considerar o uso da histeroscopia para evitar tratamentos desnecessários e inadequados.

Does big mean evil? Giant, but benign uterine leiomyoma: case report and review of the literature / Será que grande significa maligno? Leiomioma gigante, porém benigno: Relato de caso e revisão de literatura

Abstract Uterine leiomyoma is themost prevalent benign type of gynecological tumor. It affects more than 80% of women worldwide and, within this group, more than 50% may be asymptomatic. However, large fibroid volumes may be associated with symptoms of extrinsic compression, and most of the cases do not present atypical cells. We present the case of a 49-year-old woman who underwent a total abdominal hysterectomy of a 13.5-kg uterine leiomyoma with no malignancies at histopathology and review the literature about giant uterine leiomyomas and their clinical repercussion. We concluded that large volumes do not always pose a threat regarding malignancy; however, future molecular studies are needed to investigate giant uterine fibroids.

Resumo Leiomioma uterino é o tumor benigno ginecológico de maior prevalência. Ele afeta mais de 80% das mulheres no mundo e, deste grupo, mais de 50% podem ser assintomáticas. Contudo, miomas de grande volume podem ser associados com sintomas de compressão extrínseca, e a maioria dos casos não apresentam células atípicas. Nós apresentamos o caso de uma mulher de 49 anos que foi submetida a histerectomia total abdominal de um espécime de 13,5 quilos sem malignidades ao exame histopatológico. Também revisamos a literatura sobre leiomiomas uterinos de grande volume e sua repercussão clínica. Concluímos que grandes volumes nem sempre representam um risco relacionado à malignidade; contudo, estudos moleculares futuros são necessários para investigar leiomiomas uterinos gigantes.

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.

Evaluation of KI-67 expression in uterine leiomyoma and in healthy myometrium: a pilot study

SUMMARY OBJECTIVE Evaluate the expression of KI-67 in uterine leiomyomas and adjacent myometrial tissue and verify the existence of a correlation between clinical parameters and KI-67 expression in tumors. METHODS This is a cross-sectional, controlled, analytical study. Samples of leiomyomas and myometrium were obtained from patients who underwent hysterectomy. The samples were processed by immunohistochemistry using KI-67 antibody, and the expression was evaluated by two blinded observers. Student›s T-test was used for comparison of means, and Pearson›s P test for correlation with clinical parameters. RESULTS A total of 9 patients were included in the study. The mean age was 40.7 years, ranging from 35 to 44 years. The mean expression of KI-67 in myometrium was 1.63%, and, in leiomyomas, 5.96% (p <0.001). The highest expression of KI-67 was moderately related to the severity of anemia, bleeding, and pain level. CONCLUSION The expression of KI-67 in normal myometrium was significantly lower than in leiomyomas. The highest expression of KI-67 was moderately related to the severity of anemia, bleeding, and pain level in the patients of this study.

RESUMO OBJETIVO Avaliar a expressão do KI-67 em leiomiomas uterinos e tecido miometrial adjacente e verificar a existência de correlação entre parâmetros clínicos e expressão do KI-67 em tumores. MÉTODOS Estudo transversal, controlado e analítico. Amostras de leiomiomas e miométrio foram obtidas de pacientes que realizaram histerectomia. As amostras foram processadas por imuno-histoquímica utilizando anticorpo para KI-67 e a expressão avaliada por dois observadores cegos. O teste t de Student foi utilizado para comparação de médias e o teste P de Pearson para correlação com parâmetros clínicos. RESULTADOS Um total de 9 pacientes foi incluído no estudo. A idade média foi de 40,7 anos, variando de 35 a 44 anos. A expressão média do KI-67 no miométrio foi de 1,63% e nos leiomiomas de 5,96% (p <0,001). A maior expressão do KI-67 foi moderadamente relacionada com a gravidade da anemia, sangramento e nível de dor. CONCLUSÃO A expressão do KI-67 no miométrio normal foi significativamente menor que nos leiomiomas. A maior expressão do KI-67 foi moderadamente relacionada à gravidade da anemia, sangramento e nível de dor nos pacientes deste estudo.

Leiomioma como simulador de neoplasia maligna uterina: reporte de un caso / Leiomyoma as a simulator of uterine malignant neoplasms: a case report

RESUMEN El cáncer de mama puede originar de forma muy excepcional metástasis en órganos genitales. Estas suelen presentarse de forma asintomática siendo su diagnóstico generalmente tardío. En el estudio de la enfermedad diseminada la tomografía de emisión de positrones con fluordeoxiglucosa asociada a la tomografía computada (FDG PET/CT) juega un rol importante, sin embargo presenta limitantes al momento de diferenciar entre lesiones secundarias, primarias y neoplasias benignas que pueden tener elevada captación de fluordeoxiglucosa (1). Por lo expuesto anteriormente el diagnóstico de certeza solo puede realizarse mediante el estudio anatomopatológico. El caso presentado a continuación abarca uno de los posibles diagnósticos diferenciales con FDG PET/CT que evidencia un incremento en la captación de fluordeoxiglucosa.

ABSTRACT Breast cancer rarely metastasize on pelvic organs and its diagnosis is often delayed due to the asymptomatic nature of this disease. Fluorodeoxyglucose Positron Emission Tomography/Computed Tomography (FDG PET/CT) is a useful method for early diagnosis. However, may present a false positive diagnosis from benign lesions that have an increased uptake of glucose. We present a case in which a patient who had breast cancer had an increase glucose uptake in FDG PET/CT from a benign lesion.

Dermatomiofibroma lineal: reporte de un caso / Linear dermatomyofibroma: report of a case

El dermatomiofibroma es un tumor benigno infre-cuente de origen mesenquimático. Generalmente, se presenta como un tumor rosado nodular, espe-cialmente en mujeres en la década de los 30, en hombros y axila. Clínica e histológicamente puede simular otros tu-mores mesenquimáticos como el dermatofibroma , leiomioma y el dermatofibrosarcoma protuberans. Se presenta caso de dermatomiofibroma lineal, para describir sus características clínicas e histo-lógicas, necesarias para su correcto diagnóstico y enfrentamiento terapéutico.

Dermatomyofibroma is an uncommon mesen-chymal benign tumor. It usually presents in wo-men at their thirties, as a pink nodular tumor on the shoulder and axilla. Clinically and histologically it may mimic other mesenchymal tumors such as dermatofibroma, leiomyoma and dermatofibrosarcoma protube-rans. We present the case of a linear dermatomyofi-broma and describe its clinical and histological characteristics, which may help the clinician in the correct diagnosis and therapeutic approach of this rare but benign tumor.

Differential expression, diagnostic and prognostic significance of stathmin-1 and CD147 in various uterine smooth muscle tumors / Expresión diferencial, diagnóstico y significado de pronóstico de stathmin-1 y CD147 en diferentes tumores del músculo liso uterino

SUMMARY: Uterine smooth muscle tumors (USMT) are common, behavior-distinct gynecological tumors; including: leiomyoma (ULM), leiomyosarcoma (ULMS), and smooth muscle tumors of undetermined malignant potential (STUMP). Pre-operative distinction is difficult, thus diagnosis relies on histopathology. Immunohistochemistry (IHC) had been used to help in distinction. We studied two markers (stathmin-1 and CD147) to demonstrate whether they have diagnostic/ prognostic assist. Sixty seven USMT are studied. Age, follow up, and recurrence/metastasis data were collected. Representative slides were stained and Histologic score (HS) calculated as stain intensity (SI) X percentage of positive tumor cells (PP). Results were grouped as low expression (LE) and high expression (HE); then correlated to tumor types, and risk of recurrence/ metastasis. Statistical analysis (P < 0.05); Sensitivity, specificity, positive and negative predictive values and confidence intervals in diagnosing ULMS were calculated. Stathmin-1 HS (p= 0.000) and HE (p=0.002) were different among groups. Same as for CD147 HS and HE (both p=0.000), with a gradient increase from LM to STUMP to ULMS. Sensitivity, specificity, positive and negative predictive values and confidence intervals in diagnosing ULMS were as following: For stathmin-1 HS: 92 %; 20 %; 42 %; and 80 % (CI= 44-96 %). For Stathmin-1 HE: 80 %; 66 %; 60 %; and 84 % (CI=66-94 %). For CD147 HS: 85 %; 22 %; 41 %; and 69 %. For CD147 HE: 58 %; 49 %; 42 %; and 65 % (CI= 45-80 %), respectively. Recurrence / metastasis were documented in 6 cases (4 ULMS; 2 STUMP) with follow up ranging from 6 months to 102 months. 5 tumors had stathmin-1 HE (p=0.099); 2 had CD147 HE (p=0.393) in the primary tumors. STMN1 and CD147 are helpful diagnostic tests for USMT sub-typing, especially for ULMS. Gradient increase of expression from LM, to STUMP, to ULMS may indicate a role in malignant transformation in USMT, and in increased risk of recurrences/metastasis.

RESUMEN: Los tumores del músculo liso uterino (USMT, por sus siglas en inglés) son tumores ginecológicos comunes y de comportamiento distinto; incluyendo: leiomioma (ULM), leiomiosarcoma (ULMS) y tumores de músculo liso de potencial maligno indeterminado (STUMP). La distinción preoperatoria es difícil, por lo que el diagnóstico se basa en la histopatología. La inmunohistoquímica (IHQ) se había utilizado para ayudar en la distinción. Estudiamos dos marcadores (stathmin-1 y CD147) para demostrar si había efecto diagnóstico / pronóstico. Se estudiaron 67 USMT. Se recopilaron los datos de edad, seguimiento y recurrencia / metástasis. Las muestras representativas se tiñeron y la puntuación histológica (HS) se calculó como la intensidad de la tinción (IS) x porcentaje de células tumorales positivas (PP). Los resultados se agruparon como expresión baja (EB) y expresión alta (EA); luego se correlacionaeon con los tipos de tumores y el riesgo de recurrencia / metástasis. Análisis estadístico (P <0,05); se calcularon la sensibilidad, la especificidad, los valores predictivos positivos y negativos y los intervalos de confianza en el diagnóstico de ULMS. Stathmin-1 HS (p = 0,000) y HE (p = 0,002) fueron diferentes entre los grupos. Igual que para CD147 HS y HE (ambos p = 0,000), con un aumento de gradiente de LM a STUMP a ULMS. La sensibilidad, la especificidad, los valores predictivos positivos y negativos y los intervalos de confianza en el diagnóstico de ULMS fueron los siguientes: Para stathmin-1 HS: 92 %; 20 %; 42 %; y 80 % (IC = 44-96 %). Para Stathmin-1 HE: 80 %; 66 %; 60 %; y 84 % (IC = 66-94 %). Para CD147 HS: 85 %; 22 %; 41 %; y el 69 %. Para CD147 HE: 58 %; 49 %; 42 %; y 65 % (IC = 45-80 %), respectivamente. La recurrencia / metástasis se documentaron en 6 casos (4 ULMS; 2 STUMP) con un seguimiento que osciló entre 6 meses y 102 meses. Cinco tumores tenían stathmin-1 HE (p = 0,099); dos tenían CD147 HE (p = 0,393) en los tumores primarios. STMN1 y CD147 son pruebas de diagnóstico útiles para la subclasificación de USMT, especialmente para ULMS. El aumento en el gradiente de la expresión de LM, a STUMP, a ULMS puede indicar un papel en la transformación maligna en USMT y en un mayor riesgo de recurrencias / metástasis.

Endoscopic full-thickness resection for gastric gastrointestinal stromal tumor originating from the muscularis propria

SUMMARY OBJECTIVE: This study retrospectively reviewed 46 cases of gastric gastrointestinal stromal tumors treated by endoluminal endoscopic full-thickness resection (EFR) microsurgery in our gastrointestinal endoscopy center. We aimed to evaluate the EFR for the treatment of gastric gastrointestinal stromal tumors originating from the muscularis propria. METHODS: A total of 46 patients with gastric gastrointestinal stromal tumors originated from the muscularis propria layer from January 2012 to June 2015 were treated with EFR. The patients were followed up with gastroscope and computed tomography (CT) for evaluation of therapeutic effect and safety. RESULTS: EFR was successfully accomplished to remove all tumors in 46 patients. The mean procedure time was 82.5±39.8min (56-188min). Except in 3 leiomyomas, pathological examination confirmed gastrointestinal stromal tumor (GIST) in 43 cases. None of the patients had occurred bleeding, peritonitis and other complications after EFR. Thereafter, all patients were followed up with gastro-scope after 1, 6,12 months. CONCLUSIONS: EFR is effective and safe for patients with gastric gastrointestinal stromal tumors originated from muscularis propria layer and has the advantage of less invasive treatment and higher tumor resection rate. It should be considered for further application.

RESUMO OBJETIVO: Este estudo revisou retrospectivamente 46 casos de tumores gástricos estromáticos gastrointestinais tratados por microcirurgia endoluminal endoscópica de ressecção completa (EFR) em nosso centro de endoscopia gastrointestinal. Pretendemos avaliar a EFR para o tratamento de tumores gastrointestinais estromáticos originários da muscularis própria. MÉTODOS: Um total de 46 pacientes com tumores gástricos estromáticos gastrointestinais originários da camada muscular própria, de janeiro de 2012 a junho de 2015, foi tratado com EFR. Os pacientes foram acompanhados com gastroscópio e tomografia computadorizada (TC) para avaliação de efeitos terapêuticos e segurança. RESULTADOS: A EFR foi realizada com sucesso para remover todos os tumores em 46 pacientes. O tempo médio de procedimento foi de 82,5±39,8 min (56-188 min). Exceto em três leiomiomas, exame patológico confirmou tumor estromal gastrointestinal (Gist) em 43 casos. Em nenhum paciente ocorreu sangramento, peritonite e outras complicações após EFR. Posteriormente, todos os pacientes foram acompanhados com gastroscópio após um, seis e 12 meses. CONCLUSÕES: A EFR é eficaz e segura para pacientes com tumores gastrointestinais originários da camada muscular própria e tem a vantagem de ser um tratamento menos invasivo e com maior taxa de ressecção tumoral. Deve ser considerada para posterior aplicação.

Corangioleiomioma placentario / Placentary chorangio-leiomyoma

Los corangiomas son los tumores placentarios no trofoblásticos más frecuentes; generalmente aparecen como hallazgos al momento del alumbramiento. La presencia de leiomiomas en las placentas también ha sido descripta esporádicamente, siendo por lo general tumores maternos uterinos incorporados a la placenta durante la gestación. En este trabajo se informa un caso muy inusual de lesión mesenquimática, localizada en la cara fetal placentaria, caracterizada por la combinación de rasgos histopatológicos e inmunohistoquímicos de corangioma y leiomioma. Se encontró un solo caso informado en la bibliografía científica internacional con estas características, denominado "corangioleiomioma". En nuestro conocimiento, el caso aquí expuesto es el primero comunicado en nuestro país.

Chorangiomas are the most common non-trophoblastic tumors, generally appearing as incidental findings at the moment of delivery. The presence of leiomyomas inside placental parenchyma has been also described sporadically. In these cases, leiomyomas were primary maternal uterine neoplasms incorporated into the placenta during pregnancy. This case report presents a very unusual case of mesenchymal lesion, located in the fetal surface of the placenta, characterized by combined histopathologic and immunohistochemical features of chorangioma and leiomyoma. A single case reported with these characteristics was found in the international scientific literature, named as "chorangioleiomyoma". To our knowledge, this is the first case reported in our country.

Atypical Leiomyomas of the Uterus: A Clinicopathologic Study of 54 Cases and an Immunohistochemical Analysis of Ki-67 and p53 / Leiomiomas atípicos del útero: un estudio clínico-patológico de 54 casos y un análisis inmunohistoquímico de Ki-67 y p53

ABSTRACT Objective: Uterine atypical leiomyomas (ALs) are extremely rare and occur in an age group almost one decade earlier than that for leiomyosarcomas. According to the literature, extensive clinicopathologic studies on ALs were limited to only two studies (2, 8). Atypical leiomyomas of the uterus are well-defined neoplasms with smooth muscle cells. The aim of this study was to investigate clinicopathologic findings in 54 cases diagnosed with ALs as well as Ki-67 and p53 expressions immunohistochemically. Methods: Fifty-four cases diagnosed between 2000 and 2013 were included. The histological and clinical features of the cases were reviewed, and their medical records were examined. Ki-67 and p53 were performed on all cases immunohistochemically. Results: The average age of the patients was 41.8 years. The average clinical follow-up period was 57 months. Hysterectomy was performed in 31 patients, and myomectomy in 21 patients, while resection of the mass was performed in two patients due to the intraligamentary mass. The average size of the neoplasms was 6.2 cm. Severe cellular atypia was noticed in 25 patients. While the number of mitoses was 1/10 high power field in 30 patients, it was 4/10 high power field in six patients. Ki67 was found to be positive in 50 patients at the rate of 1-5% immunohistochemically, while p53 demonstrated staining at the ratio of 10-15% staining in four patients. Conclusion: The differentiation of ALs from leiomyosarcomas is crucial. The recurrence rate after follow-up is 2%. In our opinion, the patients diagnosed with 'AL with limited experience' before should be correctly diagnosed as AL. We recommend that Ki-67 and p53 can be used as adjuvant markers immunohistochemically in the patients where a problem in differential diagnosis from leiomyosarcoma exists.

RESUMEN Objetivo: Los leiomiomas atípicos uterinos (LA) son extremadamente raros y se presentan en un grupo de edad casi una década antes que los leiomiosarcomas. De acuerdo con la literatura, los extensos estudios clínico-patológicos en los LA se limitaron a sólo dos estudios (2, 8). Los leiomiomas atípicos del útero son neoplasmas bien definidos con células de músculo liso. El objetivo de este estudio fue investigar los hallazgos clínico-patológicos en 54 casos diagnosticados con LA, así como las expresiones Ki-67 y p53, de forma inmunohistoquímica. Métodos: Se incluyeron cincuenta y cuatro casos diagnosticados entre 2000 y 2013. Se revisaron las características histológicas y clínicas de los casos y se examinaron sus historias clínicas. Ki-67 y p53 se realizaron en todos los casos de forma inmunohistoquímica. Resultados: La edad promedio de los pacientes fue de 41.8 años. El período promedio de seguimiento clínico fue de 57 meses. Se realizaron histerectomías a 31 pacientes, y miomectomías a 21 pacientes, en tanto que a dos pacientes se les realizó resección de la masa debido a la situación intraligamentosa. El tamaño promedio de los neoplasmas fue de 6.2 cm. Se observó atipia celular severa en 25 pacientes. El número de mitosis fue de 1/10 campos de gran aumento en 30 pacientes, en contraste con el número de mitosis de 4/10 campos de gran aumento en seis pacientes. Se encontró que Ki67 fue positivo en 50 pacientes a razón de 1-5% inmunohistoquímicamente, mientras que p53 mostró tinción a razón de 10-15% de tinción en cuatro pacientes. Conclusión: La diferenciación de LA entre los leiomiosarcomas es crucial. La tasa de recurrencia después del seguimiento es de 2%. En nuestra opinión, los pacientes diagnosticados con 'LA con experiencia limitada ' antes, deben ser diagnosticados correctamente como LA. Recomendamos que Ki-67 y p53 sean usados como marcadores adyuvantes inmunohistoquímicamente en los pacientes con un diagnóstico diferencial de leiomiosarcoma.

Leiomyoma of the breast parenchyma: a case report and review of the literature

ABSTRACT INTRODUCTION: Benign tumors are often seen in breast screening examinations. However, the differential diagnosis is not always simple because of radiological similarity between the different benign lesions. CASE REPORT: We present a rare case report of leiomyoma of the breast parenchyma in a 68-year-old asymptomatic patient. The mammographic and ultrasonographic findings were similar to those observed in benign lesions. CONCLUSION: The histopathological diagnosis requires careful differentiation from lesions that have smooth muscle proliferation, especially leiomyosarcoma. The most commonly performed treatment is resection of the lesion with free margins. Although breast leiomyoma is rare, it should be considered among the differential diagnoses for breast nodules of benign appearance. Resection with safety margins proved to be the only treatment needed.

Review of magnetic resonance-guided focused ultrasound in the treatment of uterine fibroids

Uterine leiomyoma is the most frequently occurring solid pelvic tumor in women during the reproductive period. Magnetic resonance-guided high-intensity focused ultrasound is a promising technique for decreasing menorrhagia and dysmenorrhea in symptomatic women. The aim of this study is to review the role of Magnetic resonance-guided high-intensity focused ultrasound in the treatment of uterine fibroids in symptomatic patients. We performed a review of the MEDLINE and Cochrane databases up to April 2016. The analysis and data collection were performed using the following keywords: Leiomyoma, High-Intensity Focused Ultrasound Ablation, Ultrasonography, Magnetic Resonance Imaging, Menorrhagia. Two reviewers independently performed a quality assessment; when there was a disagreement, a third reviewer was consulted. Nineteen studies of Magnetic resonance-guided high-intensity focused ultrasound-treated fibroid patients were selected. The data indicated that tumor size was reduced and that symptoms were improved after treatment. There were few adverse effects, and they were not severe. Some studies have reported that in some cases, additional sessions of Magnetic resonance-guided high-intensity focused ultrasound or other interventions, such as myomectomy, uterine artery embolization or even hysterectomy, were necessary. This review suggests that Magnetic resonance-guided high-intensity focused ultrasound is a safe and effective technique. However, additional evidence from future studies will be required before the technique can be recommended as an alternative treatment for fibroids.

Cotyledonoid Dissecting Leiomyoma with Symplastic Features: Case Report / Leiomioma dissecante cotiledonoide com aspectos simplásticos: relato de caso

Abstract Purpose Cotyledonoid dissecting leiomyoma is a leiomyoma variant exhibiting unusual growth patterns. We aimed to demonstrate this, as well as to point out another feature that has not been previously reported. Case Report A congested, multinodular myomectomy specimen was resected. Histologically, smoothmuscle fascicles with marked vascularity and extensive hydropic degeneration were detected. A total of 2 mitoses per 10 high power fields were counted, and the Ki-67 index was of 2-3%. We encountered atypical bizarre cells that have not been previously reported. Coagulative necrosis was not present. The patient was alive and well 36 months after surgery, with no evidence of recurrence. Conclusions Albeit the gross aggressive appearance, cotyledonoid dissecting leiomyomas are benign in nature. To this day, atypical cells have not been reported in this type of tumor. Despite the presence of symplastic features, cotyledonoid dissecting leiomyomas are clinically benign entities. Surgeons and pathologists should be acquainted with this variant.

Resumo Introdução O leiomioma dissecante na forma cotiledonoide é uma variante de leiomioma com padrões raros de crescimento. Além de demonstrá-los, vamos apontar outro aspecto anteriormente não relatado. Relato de Caso Uma amostra congestionada, multinodular de miomectomia foi excisada. Histologicamente, detectaram-se fascículos de músculos lisos com marcada vascularidade e extensa degeneração hidrópica. Contaram-se 2mitoses por 10 campos de alta potência, e o índice Ki-67 foi de 2-3%. Encontramos células atípicas, bizarras, que não haviam sido relatadas anteriormente. Não foi observada necrose coagulativa. A paciente encontrava-se saudável e sem evidências de recorrência 36 meses após a cirurgia. Conclusão De aparência bruta e grosseira, os leiomiomas dissecantes na forma cotiledonoide têm natureza benigna. Até hoje, células atípicas não haviam sido relatadas nesse tipo de tumor. Apesar dos aspectos simplásticos, os leiomiomas dissecantes na forma cotiledonoide são entidades clínicas benignas. Cirurgiões e patologistas devem estar familiarizados com essa variante.

Differential Diagnosis between Bartholin Cyst and Vulvar Leiomyoma: Case Report / Diagnóstico diferencial entre cisto de Bartholin e leiomioma vulvar: relato de caso

Abstract Genital leiomyomas are rare tumors that can often be misdiagnosed as Bartholin cyst. We report a case of a 32-year-old patient who had a cystic nodulation in the left labium majus that was suggestive of Bartholin cyst. A resection surgery was performed, and the definitive histopathology diagnosis was vulvar leiomyoma. The macroscopic features of cystic lesions difficult the differential diagnosis between leiomyoma and Bartholin cyst; therefore, a histopathologic examination is often recommended.

Resumo Os leiomiomas genitais são tumores raros, e frequentemente são diagnosticados como cisto de Bartholin. Relatamos o caso de uma paciente de 32 anos em que ocorreu nodulação cística no grande lábio esquerdo sugestiva de cisto de Bartholin. Uma cirurgia de ressecção foi realizada, e o diagnóstico histopatológico definitivo foi leiomioma vulvar. As características macroscópicas das lesões císticas dificultam o diagnóstico diferencial entre o leiomioma e o cisto de Bartholin, demodo que o exame histopatológico é frequentemente recomendado.

Endoscopic Ultrasonography in the Diagnosis and Treatment Strategy Choice of Esophageal Leiomyoma

OBJECTIVES: Esophageal leiomyoma is the most common benign tumor of the esophagus, and it originates from mesenchymal tissue. This study analyzed the clinicopathological characteristics of esophageal leiomyoma and aimed to evaluate the role of endoscopic ultrasonography in the diagnosis and treatment selection for these lesions. METHODS: Two hundred and twenty-five patients who had suspected esophageal leiomyomas in endoscopic ultrasonography were enrolled at the Endoscopy Center of The First Affiliated Hospital, Zhejiang University from January 1st, 2009 to May 31th, 2015. The main outcomes included the demographic and morphological characteristics, symptoms, comparisons of diagnosis and treatment methods, adverse events, and prognosis. RESULTS: One hundred and sixty-seven patients were diagnosed as having an esophageal leiomyoma by pathological examination. The mean patient age was 50.57±9.983 years. In total, 62.9% of the lesions originated from the muscularis mucosa, and the others originated from the muscularis propria. The median distance to the incisors was 30±12 cm. The median diameter was 0.72±0.99 cm. As determined by endoscopic ultrasonography, most existing leiomyomas were homogeneous, endophytic, and spherical. The leiomyomas from the muscularis mucosa were smaller than those from the muscularis propria and much closer to the incisors (p<0.05). SMA (smooth muscle antibody) (97.2%) and desmin (94.5%) were positive in the majority of patients. In terms of treatments, patients preferred endoscopic therapies, which led to less adverse events (e.g., intraoperative bleeding, local infection, pleural effusion) than surgical operations (p<0.05). The superficial leiomyomas presented less adverse events and better recovery (p<0.05) than deep leiomyomas. CONCLUSION: Endoscopic ultrasonography has demonstrated high accuracy in the diagnosis of esophageal leiomyomas and provides great support in selecting treatments; however, EUS cannot completely avoid misdiagnosis, so combining it with other examinations may be a good strategy to solve this problem.

Leiomiomatosis benigna metastásica: reporte de caso clínico / Benign intraperitoneal metastatic leiomyomatosis: a case report

Benign intraperitoneal metastatic leiomyomatosis is a rare benign disease that is observed when a leiomyoma is present in the peritoneal surface. Women who have undergone hysterectomy for leiomyomas are most commonly affected. Patients are usually asymptomatic at presentation, being frequently an incidental finding in imaging studies. Ultrasound and CT play an important role in the diagnosis. The lesions are histologically identical to their uterine counterparts. There are different theories about the pathogenesis of the disease, including peritoneal seeding after laparoscopic hysterectomy. Others support the hypothesis of multiple independent foci of smooth muscle proliferation. Treatment, as in uterine leiomyomatosis, is generally conservative. We report a 53-year-old hysterectomized woman with intraperitoneal leiomyomas detected in a routine physical examination as mobile abdominal masses who underwent successful laparoscopic resection.