Leiomiosarcoma de la vena cava superior con extensión a las cavidades cardíacas derechas / Leiomyosarcoma of the superior vena cava with extension to the right heart cavities

Fundamento: el leiomiosarcoma de la vena cava superior es una enfermedad rara y más cuando se extiende a las cavidades cardíacas. Objetivo: presentar una paciente con leiomiosarcoma de la vena cava superior que se extendió de forma intraluminal a las cavidades derechas del corazón lo que provocó obstrucción de las mismas y choque cardiogénico.Caso clínico: paciente de 45 años de edad, blanca, femenina sin antecedentes mórbidos de salud, la cual presentó enrojecimiento del rostro y agotamiento de las extremidades al peinarse. Se le realiza tomografía axial computarizada donde se identificó una lesión sugestiva de trombosis, se decide su traslado hacia un centro especializado, se llega a la conclusión de que la paciente era portadora de una lesión tumoral no trombótica, la cual resultó ser benigna en una biopsia realizada por lo cual se decide seguimiento. La paciente reingresa en estado de shock cardiogénico por progresión de la enfermedad y se decide su tratamiento quirúrgico de emergencia, durante la cirugía fallece. Conclusiones: el leiomiosarcoma de la vena cava superior es una enfermedad rara, existen pocos reportes en la literatura sobre este padecimiento. Se presentó un caso en el cual el tumor se extendía de forma intraluminal a las cavidades cardíacas derechas (aurícula y ventrículo)(AU)

Background: the leiomyosarcoma of the superior cava vein is a rare illness and more when it extends to the cardiac cavities. Objective: to present to the medical community a female patient with leiomyosarcoma of the superior cava vein that extended intraluminal form to the right cavities of the heart causing obstruction and cardiogenic shock. Clinical case: a 45-year-old white female patient with no morbid health history, who presented redness of the face and exhaustion of the extremities when combing. A computerized axial tomography was performed where a lesion suggestive of thrombosis was identified. It was decided to transfer her to a specialized center, in which it is concluded that the patient was a carrier of a non-thrombotic tumor lesion, which turned out to be benign in a biopsy done for which follow-up is decided. The patient reenters in a state of cardiogenic shock due to progression of the disease, which is why her emergency surgical treatment is decided and die during surgery.Conclusions: the leiomyosarcoma of the superior vena cava is a rare illness, with few reports in the literature. A case in which the tumor extended intraluminally to the right cardiac cavities (atrium and ventricle) is presented(AU)

Leiomiosarcoma uterino de alto grado / High grade uterine leiomyosarcoma

Los sarcomas de útero son un grupo diverso de tumores que constituyen 1 por ciento de las neoplasias del aparato genital femenino. La incidencia mundial es de 0,5 a 3,3 casos por cada 100,000 mujeres por año. Se presenta un caso poco frecuente con diagnóstico de leiomiosarcoma uterino de alto grado y se hace una revisión de la literatura sobre este tema(AU)

Uterine sarcomas are a diverse group of tumors that make up 1 percent of the neoplasms of the female genital tract. The worldwide incidence is from 0.5 to 3.3 cases per 100,000 women per year. A rare case with a diagnosis of high-grade uterine leiomyosarcoma is presented and a review of the literature on this subject is made(AU)

Complexo hiperplasia endometrial cística canino associado á leiomiossarcoma: relato de caso

Dentre as alterações proliferativas não neoplásicas do útero, o complexo hiperplasia endometrial cística - piometra é a alteração mais comum. O leiomiossarcoma é uma neoplasia maligna de musculatura lisa e origem mesenquimal que provoca áreas de necrose, sendo mais comum em cadelas. O presente trabalho relata o caso de uma paciente canina de 12 anos com hiperplasia endometrial cística assintomática, e leiomiossarcoma uterino, descobertos a partir de uma ovariohisterectomia eletiva e avaliação histopatológica.

Complexo hiperplasia endometrial cística canino associado á leiomiossarcoma: relato de caso

Dentre as alterações proliferativas não neoplásicas do útero, o complexo hiperplasia endometrial cística - piometra é a alteração mais comum. O leiomiossarcoma é uma neoplasia maligna de musculatura lisa e origem mesenquimal que provoca áreas de necrose, sendo mais comum em cadelas. O presente trabalho relata o caso de uma paciente canina de 12 anos com hiperplasia endometrial cística assintomática, e leiomiossarcoma uterino, descobertos a partir de uma ovariohisterectomia eletiva e avaliação histopatológica.(AU)

Leiomyosarcoma of the great saphenous vein / Leiomiossarcoma de veia safena magna

A 56-year-old male patient presented with a complaint of two painful, hard, palpable nodules in the right lower limb. A Doppler ultrasound scan revealed the presence of nodules, likely to be neoplastic. Computed angiography showed two solid hypervascular nodules in the right great saphenous vein, fed by branches of the posterior tibial artery. Embolization of the nodules using surgical cyanoacrylate was performed, followed by an excisional biopsy. Anatomical pathology and immunohistochemical analysis identified the nodule as a high-grade leiomyosarcoma, characterized by ten mitotic figures per ten high-power fields, necrosis and cell pleomorphism. Immunohistochemical analysis results were positive for caldesmon and desmin labeling. A second surgical procedure was performed to enlarge the free margins.

Paciente do sexo masculino, 56 anos, com queixa de dois nódulos palpáveis, dolorosos e rígidos, em membro inferior direito. O eco-Doppler colorido evidenciou nódulos de provável natureza neoplásica na veia safena magna direita distal, com fluxo de baixa velocidade no seu interior. A angiotomografia evidenciou dois nódulos sólidos hipervascularizados no trajeto da veia safena magna direita, possuindo ramos nutridores provenientes da artéria tibial posterior. Para realizar a biópsia excisional dos nódulos, optou-se inicialmente pela embolização do tumor com cola cirúrgica de cianoacrilato, devido à sua alta vascularização. Os exames anatomopatológico e imuno-histoquímico evidenciaram leiomiossarcoma de alto grau, com dez mitoses por dez campos de grande aumento, necrose tumoral e pleomorfismo celular. A imuno-histoquímica demonstrou positividade para os marcadores Caldesmon (anticorpo hCD) e Desmina (anticorpo D33). O paciente realizou uma nova cirurgia para ampliação de margens comprometidas e está em acompanhamento clínico com a Oncologia.

[Colonic intussusception by leiomyosarcoma]. / Intususcepción colónica por leiomiosarcoma.

We report the case of female patient, 34 years old, occupation Secretary. BACKGROUND: Polycystic ovary and chronic anemia. No family history of cancer. The patient reported three weeks abdominal pain, fever, bloody loose stools, nausea and vomiting. Abdominal pain is located in flank and right lower quadrant, is colicky, intensity 5 / 10, associated with abdominal distension. On physical examination, vital functions indicated T 38.6 ° FC 98 x min, FR 18 x min, BP 120/80, was awake, she looked pale, in generally fair condition, lucid and oriented. The abdomen was distended, the increased bowel sounds, tympanic to percussion, with tenderness in the right abdomen (more intense in the right iliac fossa), palpable mass is not defined edges lower right quadrant of about 6cm. CLINICAL DIAGNOSIS: abdominal pain syndrome (appendicular mass, intestinal obstruction, intussusceptions). In examinations auxiliars highlighted in 9.1 g of hemoglobin with decreased corpuscular constants. Reviewed by history "rectal bleeding", DRE: yellow stool, no trace of blood, so that colonoscopy was deferred. Reassessed at 24 hours, we decide surgery with a presumptive diagnosis of intestinal obstruction, intussusception. In surgery, we identified a transverse colon tumor (with colo-colonic intussusception) and 10cm of colon was resected and meso tumor: TT anastomosis was performed in transverse colon. The lesion was a tumor of the middle region of the transverse colon, proliferative fibroid appearance, which almost completely obstructed the intestinal lumen, measuring about 7x5cm. The study of pathology with immunohistochemistry indicated that the tumor corresponded to leiomyosarcoma of the colon. Was discharged in good condition. The rarity of this type of malignancy and this type of presentation led us to make this report.

Intususcepción colónica por leiomiosarcoma / Colonic intussusception by leiomyosarcoma

Reportamos el caso de una paciente mujer de 34 años, secretaria, con historia de anemia crónica y ovario poliquístico; sin historia familiar de cáncer. La paciente refería desde tres semanas antes del ingreso: náusea, vómito, dolor abdominal, fiebre y deposiciones sueltas con sangre. El dolor abdominal estaba localizado en flanco y fosa iliaca derecha, era tipo cólico, de intensidad 5/10 y asociado a distensión abdominal. En el examen clínico las funciones vitales indicaban T 38,6 °, FC 98 x min, FR 18 x min y PA 120/80; estaba despierta, lucida, pálida, en regular estado general. El abdomen estaba distendido, los ruidos hidroaéreos aumentados, timpánico a la percusión y con dolor a la palpación en hemiabdomen derecho (más intenso y con rebote positivo en fosa iliaca derecha); se palpaba masa de bordes no definidos en cuadrante inferior derecho, de aproximadamente 6cm. Diagnóstico clínico: Síndrome doloroso abdominal (¿plastrón apendicular, obstrucción intestinal: intususcepción?). En los exámenes auxiliares resaltaba la hemoglobina en 9,1 gr, con las constantes corpusculares disminuidas. El tacto rectal fue negativo, por lo que la colonoscopia fue diferida. Reevaluada a las 24 horas se decide cirugía. Se identificó una tumoración de colon transverso (con intususcepción colo-colónica), se resecó 10cm de colon y meso de tumoración; se realizó anastomosis T-T de colon transverso. La lesión era una tumoración proliferativa de 7x5 cm, que obstruía la luz del intestino casi totalmente. El estudio de anatomía patológica con inmunohistoquímica indicó que la lesión invaginada correspondía a un leiomiosarcoma de colon. Salió de alta en buenas condiciones. La rareza de éste tipo de neoplasia maligna de colon y lo esporádico de este tipo de presentación, nos indujo a realizar el presente reporte.

We report the case of female patient, 34 years old, occupation Secretary. Background: Polycystic ovary and chronic anemia. No family history of cancer. The patient reported three weeks abdominal pain, fever, bloody loose stools, nausea and vomiting. Abdominal pain is located in flank and right lower quadrant, is colicky, intensity 5 / 10, associated with abdominal distension. On physical examination, vital functions indicated T 38.6 ° FC 98 x min, FR 18 x min, BP 120/80, was awake, she looked pale, in generally fair condition, lucid and oriented. The abdomen was distended, the increased bowel sounds, tympanic to percussion, with tenderness in the right abdomen (more intense in the right iliac fossa), palpable mass is not defined edges lower right quadrant of about 6cm. Clinical diagnosis: abdominal pain syndrome (appendicular mass, intestinal obstruction, intussusceptions). In examinations auxiliars highlighted in 9.1 g of hemoglobin with decreased corpuscular constants. Reviewed by history “rectal bleeding”, DRE: yellow stool, no trace of blood, so that colonoscopy was deferred. Reassessed at 24 hours, we decide surgery with a presumptive diagnosis of intestinal obstruction, intussusception. In surgery, we identified a transverse colon tumor (with colo-colonic intussusception) and 10cm of colon was resected and meso tumor: TT anastomosis was performed in transverse colon. The lesion was a tumor of the middle region of the transverse colon, proliferative fibroid appearance, which almost completely obstructed the intestinal lumen, measuring about 7x5cm. The study of pathology with immunohistochemistry indicated that the tumor corresponded to leiomyosarcoma of the colon. Was discharged in good condition. The rarity of this type of malignancy and this type of presentation led us to make this report.

Leiomiosarcoma primario de mediastino que compromete el sistema de la vena cava superior: resección quirúrgica asociada a reconstrucción venosa / Primary leiomyosarcoma of the mediastinum involving the superior vena cava system: surgical resection and venous reconstruction

Mediastinal tumors have multiple presentation forms and various pathological origins. Sometimes they reach a large size and can involve the system of superior vena cava by compression or infiltration. We present a 48 year old man admited for dyspnea, headache and palpitations in decubitus, for the last two months. The X-ray and chest computed tomography showed an anterior mediastinal tumor and the tru-cut pathological study concluded leiomyosarcoma. He underwent surgical treatment; the tumor compromised the system of the superior vena cava and was resected with ligation and resection the left brachiocephalic trunk. Part of the superior vena cava was reconstructed with autologous pericardium. The right phrenic nerve was surrounded by the tumor mass and was resected in block. The patient recovered satisfactorily and was discharged on the eleventh day post surgery. The histopathologic study of the surgical specimen confirmed a leiomyosarcoma G2 (3,100 gr). Full adjuvant radiotherapy was done. At 15 months after surgery the patient is in good condition without evidence of recurrence or superior vena cava syndrome.

Los tumores del mediastino pueden tener múltiples formas de presentación y diversos orígenes anato-mopatológicos. A veces alcanzan gran tamaño y pueden comprometer por compresión o infiltración el sistema de la vena cava superior. Presentamos el caso de un hombre de 48 años que consultó por disnea, cefalea y palpitaciones en decúbito de dos meses de evolución. En la radiografía y en la tomografía computada de tórax se demostró un tumor de mediastino anterior, el estudio anatomopatológico mediante biopsia trucut concluyó leiomiosarcoma. Se realizó tratamiento quirúrgico, se resecó la masa tumoral que comprometía el sistema de la vena cava superior, se ligó y resecó el tronco venoso braquiocefálico izquierdo o innominado y se reconstruyó parte de la vena cava superior con parche de pericardio autólogo. El nervio frénico derecho se encontraba rodeado por la masa tumoral y se resecó junto con el tumor. El paciente evolucionó satisfactoriamente y fue dado de alta al décimo primer día post operado. El estudio de anatomía patológica de la pieza operatoria confirmó un leiomiosarcoma G2 de 3.100 gramos. Se completó tratamiento con radioterapia post operatoria. A 15 meses post operado el paciente se encuentra en buenas condiciones generales, sin evidencias de recidiva y sin síndrome de vena cava superior.

Derivação arterial extra-anatômica associada à leiomiossarcoma de coxa / Extra-anatomical arterial bypass with leiomyosarcoma of the thigh

As derivações extra-anatômicas são opções técnicas em situações em que as vias convencionais de revascularização são de alto risco cirúrgico ou anatomicamente impraticáveis. Neste relato, apresentamos o caso de uma paciente portadora de sarcoma de partes moles do terço proximal da coxa esquerda, com invasão tumoral dos vasos femorais. A paciente foi submetida à revascularização do membro inferior com enxerto extra-anatômico ilíaco externo para artéria poplítea acima do joelho com PTFF. Posteriormente, foi submetida à ressecção em monobloco do tumor, músculo quadríceps e vasos femorais, com boa evolução pós-operatória, patência do enxerto e sem recidiva local no seguimento de 24 meses.

[Uterine leiomyosarcoma. A report of a case]. / Sarcoma uterino. Reporte de un caso.

A 33 years old male patient was admitted at the Ginecology/Obstetric emergency room with acute pain abdomen. The patient underwent an exploratory laparotomy where it was found 1,400 cc of blood in the peritoneal cavity. It was also found a 10 cm diameter tumor on the right lateral uterus side involving the uterine tube, the ovary and the ipsilateral parametrium. In the omentum there were found two 3 cm diameter tumors like the one found in the uterus. The bleeding was under control so it was made a hysterectomy, bilateral salpingooophorectomy and omentectomy. Histopathologic diagnosis was reported two weeks after. The final diagnosis was serosa infiltration, uterine leiomyosarcoma and implant on peritoneum.

[Mesenteric cyst as a cause of acute abdomen. Report of 3 cases]. / Quiste de mesenterio como causa de abdomen agudo. A propósito de tres casos.

Mesenteric cyst is a tumor of multiple origins that surely are found more frequently than the literature report, at any rate, this tumor is uncommon. Because of absence of characteristic clinical findings, diagnosis cumbersome, until these cysts are of such a size that palpation becomes possible or when they cause compression to nearby viscera. Occasionally, diagnosis is made during surgery, even when it was emergency surgery. The present paper reports on three patients with mesenteric cyst found during surgery as emergency treatment. Histopathologic reports showed lymphangioma in two cases and leiomyosarcoma in one case, quite uncommon in this kind of lesion.

[Intussusception in adults. Case report with uncommon etiology and review of the literature]. / Intususcepción en adultos: presentación de un caso con etiología poco común y revisión de la literatura.

OBJECTIVE: To present a case of adult intussusception with uncommon etiology. BACKGROUND: Intussusception is a relatively common cause of intestinal obstruction in children, but is a rare clinical entity in adults, representing less than one per cent of intestinal obstruction in this patient population. METHOD: We report on 72-year old female patient with intestinal obstruction due to ileocolic intussusception related to leiomyosarcoma of the terminal ileum. A synchronic adenocarcinoma in the cecum was identified. The patient underwent right hemicolectomy and terminal ileum resection with primary anastomosis. Her postoperative course was uneventful. The patient is free of all evidence of disease at 2 year of follow-up. A thorough review of the literature of adult intussusception was carried out. CONCLUSION: From this review, the recommendation is to resect all cases of adult intussusception, different from the recommended approach in pediatric population.

Leiomyosarcoma of the esophagus in a patient with chagasic megaesophagus: case report and literature review.

Leiomyosarcoma constitutes approximately 0.5% of the malignant neoplasias of the esophagus and its association with megaesophagus has not been described. We report on a case of a woman with dysphagia that was slowly progressive from the age of 19 due to chagasic megaesophagus. The woman was subjected to cardiomyotomy at the age of 49. She presented a rapid worsening of the dysphagia due to leiomyosarcoma at the age of 61, and was subjected to subtotal esophagectomy with cervical esophagogastroplasty. She developed pulmonary and hepatic metastases 14 months after surgery and died six months later.

Leiomiossarcoma de mediastino em paciente com bócio adenomatóide: relato de um caso com necropsia / Leiomyosarcoma of mediastinum in patient with goiter: case report with necropsy

Trata-se de relato de caso com verificaçäo necroscópica em paciente com bócio adenomatóide, no qual foi encontrado massa tumoral peritraqueal e adjacente à tireóide, sendo diagnosticado por microscopia óptica sarcoma de partes moles com morfologia de musculatura lisa, caracterizando leiomiossarcoma. Existem raros casos deste tumor localizado no mediastino, descritos na literatura, e em nenhum destes há associaçäo com bócio adenomatóide.

[Leiomyosarcoma associated with Epstein-Barr virus in an adult with renal transplant]. / Leiomiosarcoma asociado con el virus de Epstein-Barr en un adulto con trasplante renal.

Recently the association between the Epstein-Barr virus (EBV) and smooth muscle lesions has been described in immunosuppressed children but it is infrequent in adults. The role of EBV in the pathogenesis of these lesions is obscure. We presents a 28 year old man with end stage renal disease transplanted in 1994. Two years later he developed several nodular lesions that affected both lungs, liver, spleen, retroperitoneal ganglia and the left thigh; one year later he died. The surgical specimen from the thigh and a liver biopsy were diagnosed as leiomyosarcoma. Immunohistochemical reactions against vimentin and smooth muscle actin were positive. In situ hybridization disclosed positivity against EBV nuclear antigens (EBNA-2) in neoplasic cells. This is the first case of sarcoma in transplanted patients of our institution and represents a rare case of leiomyosarcoma associated with EBV in adults.