RESUMEN
Background: Primary ureteral neoplasms are extremely rare in dogs, and ureteral involvement usually occurs owing to the invasion of renal and bladder tumors. Case Description: This case report describes a 12-year-old intact male mixed-breed dog referred to a private clinic with a six-month history of abdominal distention. A physical examination revealed mild abdominal pain. Hematological tests detected normocytic-normochromic anemia (hematocrit 33.6% [reference interval-RI: 37%-55%], red blood cells 4.93 M/µl [RI: 5.5-8.5 M/µl], and hemoglobin 12.4 g/dl [RI: 12-18.0 g/dl]). The results from the leukogram, thrombogram, renal, and hepatic panels were within the reference intervals for dogs. Abdominal ultrasonography revealed a cavitary mass measuring approximately 12 cm in diameter as the largest tumor in the left abdominal region over the left hepatic lobe or mesenteric site. Chest radiography did not reveal any metastasis. Therefore, the patient underwent exploratory laparotomy, during which the left ureter was found to be affected by a 12-cm mass that adhered to the left kidney. A unilateral left ureteronephrectomy was performed, and histology and immunohistochemistry (IHC) confirmed well-differentiated primary ureteral leiomyosarcoma. The patient survived for 130 days but died of lung metastasis. Conclusion: Ureteral leiomyosarcoma should be investigated and included in the list of differential diagnoses for primary ureteral neoplasms. Regardless of the therapeutic modality, the prognosis of ureteral leiomyosarcoma may be unfavorable, as shown in this report.
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Enfermedades de los Perros , Leiomiosarcoma , Neoplasias Ureterales , Masculino , Animales , Enfermedades de los Perros/cirugía , Enfermedades de los Perros/diagnóstico , Enfermedades de los Perros/patología , Perros , Leiomiosarcoma/veterinaria , Leiomiosarcoma/cirugía , Leiomiosarcoma/patología , Leiomiosarcoma/diagnóstico , Neoplasias Ureterales/veterinaria , Neoplasias Ureterales/cirugía , Neoplasias Ureterales/patología , Neoplasias Ureterales/diagnóstico , Nefrectomía/veterinaria , Resultado Fatal , Uréter/cirugía , Uréter/patologíaRESUMEN
Presentamos el caso de un paciente masculino de 57 años con un leiomiosarcoma retroperitoneal encontrado de manera incidental. Es una patología poco común, la incidencia anual es 2.7 casos por millón de personas. De igual manera, es una enfermedad de evolución silenciosa, por lo que pasa inadvertida hasta que la masa tumoral alcanza un gran tamaño. Los leiomiosarcomas se evidencian como extensas áreas de heterogeneidad y realce heterogéneo, ocasionado por la presencia de necrosis y focos hemorrágicos. Normalmente no hay presencia de calcificaciones y hay ausencia de tejido adiposo, de esta manera que una gran masa retroperitoneal mayor a 10 cm sin contenido graso y necrosis interna variable debe sugerir la posibilidad de un leiomiosarcoma. La eliminación quirúrgica completa es el tratamiento de elección, ésta se debe realizar con márgenes negativos amplios, factor que repercute en el pronóstico, ya que estos tumores tienden a alcanzar un tamaño muy grande y, a menudo, la extirpación amplia es imposible, lo que afecta la supervivencia del paciente. Este tipo de neoplasias tienen un mal pronóstico, pues se sabe que la supervivencia a los 5 años es de aproximadamente 15%, debido a su alta tendencia a la invasión local, metástasis o diagnóstico tardío.
We present the case of a 57-year-old male patient with a retroperitoneal leiomyosarcoma found incidentally. It is a rare disease, with an annual incidence of 2.7 cases per million people. Likewise, it is a disease with a silent evolution, so it goes unnoticed until the tumor mass reaches a large size. Leiomyosarcomas appear as extensive areas of heterogeneity and heterogeneous enhancement, caused by the presence of necrosis and hemorrhagic foci. Normally, there is no presence of calcifications and there is an absence of adipose tissue, so a large retroperitoneal mass greater than 10 cm with no fat content and variable internal necrosis should suggest the possibility of a leiomyosarcoma. Complete surgical removal is the treatment of choice, and should be performed with wide negative margins, a factor that affects the prognosis, since these tumors tend to reach a very large size and wide excision is often impossible, which affects the patient's survival. This type of neoplasm has a poor prognosis, since it is known that the 5-year survival rate is approximately 15%, due to its high tendency to local invasion, metastasis or late diagnosis.
Apresentamos o caso de um paciente do sexo masculino, 57 anos, comleiomiossarcoma retroperitoneal encontrado incidentalmente. É umapatologia rara, a incidência anual é de 2,7 casos por milhão de pessoas. Da mesma forma, é umadoença de evolução silenciosa, por issopassadespercebida até que a massa tumoral atinja umtamanho grande. Os leiomiossarcomasaparecem como extensas áreas de heterogeneidade e realce heterogêneo, causadas pela presença de necrose e focos hemorrágicos. Normalmente nãohápresença de calcificações e háausência de tecido adiposo, portantouma grande massa retroperitoneal maior que 10 cm semconteúdo de gordura e necrose interna variáveldeve indicar a possibilidade de leiomiossarcoma. A remoçãocirúrgica completa é o tratamento de escolha, este deve ser realizado com amplas margens negativas, fator que afeta o prognóstico, poisesses tumores tendem a atingir tamanhomuito grande e, muitasvezes, a remoção ampla é impossível, o que prejudica a sobrevida do paciente. Esse tipo de neoplasia apresentamauprognóstico, pois sabe-se que a sobrevida em 5 anos é de aproximadamente 15%, devido à sua alta tendência à invasão local, metástaseou diagnóstico tardio.
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Humanos , Masculino , Persona de Mediana Edad , Neoplasias Retroperitoneales/cirugía , Tomografía Computarizada Espiral/métodos , Leiomiosarcoma/cirugía , Neoplasias Retroperitoneales/diagnóstico por imagen , Dolor Abdominal , Resultado del Tratamiento , Leiomiosarcoma/diagnóstico por imagenRESUMEN
INTRODUCTION AND BACKGROUND: Primary leiomyosarcoma of the seminal vesicle is an extremely rare and highly malignant disease with less than 15 cases reported. CASE DESCRIPTION: A 34-year-old man presented with acute urinary symptoms. Imagen studies showed an abdominal mass (80 mm × 65 mm × 50 mm) with contrast enhancement, compressing the right side of the bladder but with a clear cleavage level between surrounding organs. The patient underwent a transrectal US-guided biopsy which was informed as compatible with leiomyosarcoma by immunohistochemical characterization. We performed a cystoprostatectomy and pelvic lymphadenectomy plus radiotherapy. Pathology showed a 7.5 cm × 6 cm nodular para-vesical Leiomyosarcoma histological grade 2 with 0/22 lymph nodes involved. Twelve months after the surgery no recurrences have presented. CONCLUSION: A multi-disciplinary therapeutic approach, combined with close follow-up, is mandatory to obtain good outcomes in such rare and challenging cases.
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Neoplasias Abdominales , Neoplasias de los Genitales Masculinos , Leiomiosarcoma , Masculino , Humanos , Adulto , Leiomiosarcoma/cirugía , Leiomiosarcoma/diagnóstico , Leiomiosarcoma/patología , Vesículas Seminales/patología , Vesículas Seminales/cirugía , Neoplasias de los Genitales Masculinos/cirugía , Biopsia , Cistectomía , Neoplasias Abdominales/patologíaRESUMEN
Pelvic soft tissue sarcomas (PSTS) are a rare, heterogeneous group of tumors. They have been usually analyzed with retroperitoneal sarcomas (RPS), but actually have key differences. Due to their unique anatomic location, symptomatic presentation of PSTS may be more common than RPS. Adequate imaging approach is paramount for guiding differential diagnosis, while preoperative biopsy is mandatory, especially when preoperative treatment may be considered as initial approach. The most frequent histologic subtype is leiomyosarcoma, which is different as expected in the retroperitoneum where liposarcoma is the commonest histology. Also solitary fibrous tumor is commonly diagnosed in the pelvis. Surgical approach for PSTS differs from that for RPS mainly due to anatomic relations. Similarly, in the lack of definite evidence from specific trials about neoadjuvant and adjuvant treatments, the anatomic constraints to obtain wide margins in the pelvis as well as the expected functional outcome in case of organ resections should be factored into decision for individualized treatment offer. Vascular and genitourinary involvement are frequent, as well as herniation through pelvic foramina. For these reasons a multidisciplinary surgical team should always be considered. Early referral of these patients to high-volume centers is critical and may impact on survival, given that optimal initial resection is a major predictor of curative treatment. International consensus on PSTS treatment is advocated, similarly to the recent efforts realized for RPS.
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Leiomiosarcoma , Liposarcoma , Neoplasias Retroperitoneales , Sarcoma , Neoplasias de los Tejidos Blandos , Humanos , Sarcoma/diagnóstico , Sarcoma/terapia , Sarcoma/patología , Liposarcoma/patología , Leiomiosarcoma/diagnóstico , Leiomiosarcoma/cirugía , Pelvis/patología , Neoplasias Retroperitoneales/diagnóstico , Neoplasias Retroperitoneales/cirugía , Neoplasias de los Tejidos Blandos/diagnóstico , Neoplasias de los Tejidos Blandos/cirugía , Recurrencia Local de Neoplasia/patologíaRESUMEN
El leiomiosarcoma primario de vena cava es una neoplasia poco frecuente originada a nivel de las células musculares lisas de la túnica media. Representa el 2% de todos los leiomiosarcomas y el 60% de los tumores de vena cava. Presentamos el caso clínico de una paciente de 64 años que consulta por dolor lumbar derecho. La tomografía computada evidencia una gran masa retroperitoneal que engloba la vena cava inferior y contacta con el riñón derecho; el estudio histopatológico de la biopsia certifica un leiomiosarcoma. Se realiza tratamiento quirúrgico mediante resección tumoral con vena cava inferior e interposición de prótesis sintética y nefrectomía derecha. El estudio anatomopatológico de la pieza certifica un leiomiosarcoma de alto grado sin compromiso de la cápsula renal con bordes de resección libres. La resección radical con márgenes negativos actualmente ofrece la mejor tasa de supervivencia. Aún se encuentra en discusión la indicación de la anticoagulación en pacientes con colocación de prótesis.
Primary leiomyosarcoma of the vena cava is a very rare neoplasm that originates from the smooth muscle cells of the tunica media. It represents 2% of all the leiomyosarcomas and 60% of the vena cava tumors. We report a case of 64-year-old patient consulting for a lower right back pain. The computed tomography shows a large retroperitoneal mass which encompasses the inferior vena cava and involves of right kidney. The surgical treatment of tumor resection is performed with right nephrectomy and interposition of Dacron prosthesis. The anatomopathological study shows a high-grade leiomyosarcoma, not compromising the renal capsule, with resection free. Radical resection with negative margins currently offers the best survival rate. Anticoagulation treatment for patients with prosthesis placement is still under discussion.
O leiomiossarcoma primário da veia cava é uma neoplasia rara originada das células musculares lisas da túnica média. Representa 2% de todos os leiomiossarcomas e 60% dos tumores de veia cava. Apresentamos o caso clínico de um doente de 64 anos que consultou por lombalgia direita. A tomografia computadorizada revelou grande massa retroperitoneal que envolvia a veia cava inferior e contatava o rim direito; o estudo citológico da biópsia revelou um leiomiossarcoma. O tratamento cirúrgico foi realizado por excisão tumoral e de veia cava inferior com interposição de prótese sintética e nefrectomia direita. O diagnóstico anatomopatológico foi leiomiossarcoma de alto grau sem envolvimento da cápsula renal com bordas de ressecção livres de patologia. A ressecção radical com margens negativas atualmente oferece a melhor taxa de sobrevida. A indicaçãode anticoagulação em pacientes com colocação de prótese ainda está em discussão.
Asunto(s)
Prótesis e Implantes , Vena Cava Inferior/patología , Leiomiosarcoma/cirugíaRESUMEN
BACKGROUND: Leiomyosarcoma is a rare malignant tumor of smooth muscle origin and represents 10-20% of all soft tissue sarcomas. Primary colon and rectal sarcomas constitute < 0.1% of all large bowel malignancies. In Li-Fraumeni syndrome, sarcomas are the second most frequent cancer (25%). Li-Fraumeni syndrome is a genetic disease with a familial predisposition to multiple malignant neoplasms. This syndrome has an autosomal dominant pattern of inheritance and high penetrance characterized by germline TP53 mutations. Patients with a history of cancer who do not meet all the "classic" criteria for Li-Fraumeni syndrome are considered to have Li-Fraumeni-like syndrome. To the best of our knowledge, this article is the first report of a patient with rectal leiomyosarcoma as the initial phenotypic manifestation of Li-Fraumeni-like syndrome. The authors also present a literature review. CASE PRESENTATION: A 67-year-old Brazilian woman underwent anterior rectosigmoidectomy and panhysterectomy secondary to rectal leiomyosarcoma. She subsequently developed carcinomatosis and died 2 years after the operation. Her family medical history consisted of a daughter who died at 32 years of age from breast cancer, a granddaughter diagnosed with adrenocortical carcinoma at 6 years of age and two siblings who died from prostate cancer. A genetic study was carried out to identify a pathogenic variant of Li-Fraumeni syndrome. In the DNA extracted from the peripheral blood leukocyte, restriction fragment length polymorphism was analyzed to search for mutations in the TP53 gene. The DNA sequencing identified the germline pathogenic variant p. R337H heterozygous in exon 10 of TP53. The patient was classified as having Li-Fraumeni-like syndrome. CONCLUSION: In patients with rectal leiomyosarcoma, it is advisable to investigate the family history of cancer and perform genetic studies to screen for Li-Fraumeni syndrome.
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Neoplasias de la Corteza Suprarrenal , Carcinoma Corticosuprarrenal , Leiomiosarcoma , Síndrome de Li-Fraumeni , Neoplasias Pélvicas , Neoplasias del Recto , Masculino , Femenino , Humanos , Niño , Anciano , Síndrome de Li-Fraumeni/complicaciones , Síndrome de Li-Fraumeni/diagnóstico , Síndrome de Li-Fraumeni/genética , Leiomiosarcoma/diagnóstico , Leiomiosarcoma/genética , Leiomiosarcoma/cirugía , Proteína p53 Supresora de Tumor/genética , Neoplasias del Recto/diagnóstico , Neoplasias del Recto/genética , Neoplasias del Recto/cirugía , Neoplasias de la Corteza Suprarrenal/complicaciones , Neoplasias de la Corteza Suprarrenal/genética , Predisposición Genética a la EnfermedadRESUMEN
Introduction: Primary skin leiomyosarcomas are infrequent neoplasms. They correspond to 2-3% of skin sarcomas and are most frequently located on the lower extremities, trunk and genitals. Methods: We present a case of a 73-year-old man with a 4-month evolution of foreskin leiomyosarcoma. The lesion was biopsied for histopathological study with HE and immunohistochemistry with smooth muscle actin, specific muscle actin, CD34, p63 and S-100 (-). Results: We observed a leiomyosarcoma of high histological grade and mitotic count. It was positive by immunohistochemistry for smooth muscle actin, while the other markers were negative. Surgical limits were compromised so a reoperation with wide margins of healthy tissue was necessary. Conclusion: The skin lesions should be removed all, without exception, since they can be neoplasms of variable biological behavior. The histological study must be complemented with immunohistochemistry to differentiate them from other neoplasms. For the prognosis, the histological grade, size, location and the possibility of resection with wide margins must be taken into account.
Introducción: Los leiomiosarcomas primarios de piel son neoplasias infrecuentes. Corresponden al 2-3 % de los sarcomas cutáneos y se localizan con mayor frecuencia en las extremidades inferiores, tronco y genitales. Método: Presentamos un caso de un varón de 73 años con un leiomiosarcoma en prepucio de 4 meses de evolución. Se le practicó biopsia excisional de la lesión para estudio histopatológico con HE e inmunohistoquímica con actina de músculo liso, actina muscular específica, CD34, p63 y S-100 (-). Resultados: Observamos un leiomiosarcoma de alto grado histológico y recuento mitótico. Presentó positividad por inmunohistoquímica para actina de músculo liso, en tanto que los otros marcadores fueron negativos. Los límites quirúrgicos estuvieron comprometidos por lo que fue necesaria una reintervención con amplios márgenes de tejido sano. Conclusión: Las lesiones de piel deben extirparse todas, sin excepción, ya que pueden tratarse de neoplasias de conducta biológica variable. El estudio histológico debe complementarse con inmunohistoquímica para diferenciarlas de otras neoplasias. Para el pronóstico se debe tener en cuenta el grado histológico, el tamaño, la localización y la posibilidad de resección con amplios márgenes.
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Leiomiosarcoma , Actinas , Anciano , Prepucio/patología , Humanos , Inmunohistoquímica , Leiomiosarcoma/patología , Leiomiosarcoma/cirugía , Masculino , PronósticoRESUMEN
OBJECTIVE: Primary leiomyosarcoma of the inferior vena cava (IVC) is best managed with surgical resection when technically feasible. However, consensus is lacking regarding the best choice of conduit and reconstruction technique. The aim of the present multicenter study was to perform a comprehensive assessment through the VLFDC (Vascular Low Frequency Disease Consortium) to determine the most effective method for caval reconstruction after resection of primary leiomyosarcoma of the IVC. METHODS: A multicenter, standardized database review of patients who had undergone surgical resection and reconstruction of the IVC for primary leiomyosarcoma from 2007 to 2017 was performed. The demographics, periprocedural details, and postoperative outcomes were analyzed. RESULTS: A total of 92 patients (60 women and 32 men), with a mean age of 60.1 years (range, 30-88 years) were treated. Metastatic disease was present in 22%. The tumor location was below the renal veins in 49 (53%), between the renal and hepatic veins in 52 (57%), and above the hepatic veins in 13 patients (14%). The conduits used for reconstruction included ringed polytetrafluoroethylene (PTFE; n = 80), nonringed PTFE (n = 1), Dacron (n = 1), autogenous vein (n = 1), bovine pericardium (n = 4), and cryopreserved tissue (n = 5). Complete R0 resection was accomplished in 73 patients (79%). In-hospital mortality was 2%, with a median length of stay of 8 days. The primary patency of PTFE reconstructed IVCs was 97% and 92% at 1 and 5 years, respectively, compared with 73% at 1 and 5 years for the non-PTFE reconstructed IVCs. The overall 1-, 3-, and 5-year survival for the entire cohort were 94%, 86%, and 65%, respectively CONCLUSIONS: The findings from our multi-institutional study have demonstrated that complete en bloc resection of IVC leiomyosarcoma with vascular surgical reconstruction in selected patients results in low perioperative mortality and is associated with excellent long-term patency. A ringed PTFE graft was the most commonly used conduit for caval reconstruction, yielding excellent long-term primary patency.
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Implantación de Prótesis Vascular , Leiomiosarcoma , Animales , Bovinos , Femenino , Humanos , Leiomiosarcoma/diagnóstico por imagen , Leiomiosarcoma/cirugía , Masculino , Persona de Mediana Edad , Politetrafluoroetileno , Estudios Retrospectivos , Resultado del Tratamiento , Vena Cava Inferior/diagnóstico por imagen , Vena Cava Inferior/patología , Vena Cava Inferior/cirugíaRESUMEN
BACKGROUND: De novo neoplasms are one of the major causes of death in patients after the first year of liver transplantation. The occurrence of sarcomas is extremely rare and the survival is often poor. However, early diagnosis and radical surgical treatment, may benefit some select liver transplant patients. METHOD: We describe the case of a liver transplant patient who developed a locally advanced inferior vena cava (IVC) leiomyosarcoma, who underwent radical surgical treatment with resection of the IVC associated with duodenopancreatectomy, right nephrectomy, and IVC reconstruction. We address aspects of the diagnosis and surgical strategy. CONCLUSION: This case report illustrates that IVC and multivisceral resections may be feasible and safe in highly selected liver transplant recipients. Major surgery should not be excluded as treatment option in an immunosuppressed liver transplant patient.
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Leiomiosarcoma , Páncreas , Neoplasias Vasculares , Vena Cava Inferior , Humanos , Leiomiosarcoma/patología , Leiomiosarcoma/cirugía , Trasplante de Hígado , Páncreas/cirugía , Neoplasias Vasculares/patología , Neoplasias Vasculares/cirugía , Vena Cava Inferior/cirugíaRESUMEN
Leiomyosarcoma is a rare type of cancer that affects smooth muscle tissue. Leiomyosarcomas are exceedingly rare in the oral cavity, particularly in the buccal mucosa. The diagnosis is challenging due to non-specific clinical features and significant overlap of morphological findings with several spindle cell tumors. We reported the clinicopathological and immunohistochemical features of a rare recurrent case of leiomyosarcoma in a 73-year-old female presenting clinically as a painful nodule on the posterior right buccal mucosa. Microscopically, the lesion showed atypical spindle cells arranged in a fascicular pattern and frequent mitotic figures. Immunohistochemistry showed strong positivity for vimentin, α-SMA, HHF35, h-caldesmon, and focal positivity for desmin. CD34 highlighted numerous blood vessels distributed throughout all tumor stroma. S-100 protein, myogenin, and pan-cytokeratin (AE1/AE3) were negative. Surgical excision followed by chemotherapy was carried out, and no recurrence was observed after 1 year of follow-up. Careful histopathological and immunohistochemistry analysis of these lesions is essential to ensure a correct diagnosis. Early surgical excision with tumor-free margins and prolonged follow-up is strongly recommended.
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Leiomiosarcoma , Recurrencia Local de Neoplasia , Anciano , Femenino , Humanos , Inmunohistoquímica , Leiomiosarcoma/cirugía , Mucosa Bucal/patologíaAsunto(s)
Colposcopía/métodos , Preservación de la Fertilidad/métodos , Leiomiosarcoma/cirugía , Neoplasias Vaginales/cirugía , Adulto , Femenino , Humanos , Leiomiosarcoma/diagnóstico por imagen , Leiomiosarcoma/patología , Tomografía Computarizada por Tomografía de Emisión de Positrones , Neoplasias Vaginales/diagnóstico por imagen , Neoplasias Vaginales/patologíaRESUMEN
Los leiomiosarcomas de la cavidad nasal y senos paranasales son neoplasias malignas de baja frecuencia, localmente agresivas. Presentan una alta tendencia a la recurrencia de aproximadamente 55% en nariz y senos paranasales. Están conformados por células musculares lisas. Según diversos autores, aproximadamente el 50% de los pacientes mueren antes del primer año y la supervivencia a los 5 años es del 20%. Anteriormente se pensaba que no poseían potencial metastásico, sin embargo, en series actuales se ha visto que presentan un alto poder metastásico de hasta el 50%. Las metástasis se presentan de forma tardía. El tratamiento recomendado consiste en la resección radical del tumor primario con un amplio margen de tejido normal y la radioterapia es de uso controversial en el manejo.
Leiomyosarcoma of the nasal cavity and paranasal sinuses are malignant, low frequency, locally aggressive neoplasm. They present a high tendency to recurrence of approximately 55% in the nose and paranasal sinuses. They are made up of smooth muscle cells. According to different authors, approximately 50% of patients die before the first year and survival at 5 years is 20%. Previously it was thought that they did not possess metastatic potential, however in current series it has been seen that they have a high metastatic power of up to 50%. Metastases present late. The recommended treatment consists of radical resection of the primary tumor with a wide margin of normal tissue.
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Humanos , Femenino , Persona de Mediana Edad , Neoplasias Nasales/cirugía , Neoplasias Nasales/diagnóstico por imagen , Leiomiosarcoma/cirugía , Leiomiosarcoma/diagnóstico por imagen , Senos Paranasales , Imagen por Resonancia Magnética , Neoplasias Nasales/patología , Endoscopía , Leiomiosarcoma/patología , Cavidad Nasal/cirugía , Cavidad Nasal/diagnóstico por imagenRESUMEN
Resumen Introducción Los leiomiosarcomas de la vena cava inferior son tumores raros, sólo hay 300 casos descritos en la literatura. Su incidencia es mayor en mujeres, suelen aparecer entre los 50-60 años, y presentan una progresión lenta y mal pronóstico. Los síntomas son inespecíficos haciendo que el diagnóstico se realice de forma tardía, éste se realiza mediante pruebas de imagen y biopsia guiada. Caso clínico Se presenta el caso de un varón de 73 años con diagnóstico de leiomiosarcoma de la vena cava inferior, como hallazgo incidental en TC de control, tratado mediante radioterapia neoadyuvante, cirugía y radioterapia intraoperatoria. Discusión El único tratamiento que ha descrito modificaciones en la supervivencia es la cirugía. El papel de la adyuvancia y neoadyuvancia en estos tumores es muy controvertido. La elección de la actitud terapéutica dependerá de la localización del tumor, tamaño, la relación con estructuras adyacentes y la presencia de circulación colateral.
Introduction Leiomyosarcomas of the inferior vena cava are rare tumors, with fewer than 300 cases reported. Its incidence is higher in females, usually appear in the sixth decade and they have a slow-growing and poor prognosis. Symptoms are generally non-specific. Diagnosis is made with imaging studies and guided biopsy. Clinical Case We report a case of a 73-year-old male patient with leimyosarcoma of the inferior vena cava treated by neoadjuvant radiotherapy, surgery and intraoperative radiotherapy. Discussion Surgery is the only treatment that can improve the survival. The role of the adjuvancy and neoadjuvancy is very controversial. Surgical management is determined by the location of the tumour, the relationship with adjacent structures and the presence of collateral veins.
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Humanos , Masculino , Anciano , Vena Cava Inferior/patología , Neoplasias Vasculares/cirugía , Neoplasias Vasculares/diagnóstico por imagen , Leiomiosarcoma/cirugía , Leiomiosarcoma/diagnóstico por imagen , Grupo de Atención al Paciente , Periodo Posoperatorio , Vena Cava Inferior/cirugía , Tomografía Computarizada por Rayos X , Resultado del Tratamiento , Radioterapia Adyuvante/métodos , Hallazgos Incidentales , Leiomiosarcoma/radioterapiaRESUMEN
Introducción: Los tumores ano-rectales del musculo liso son raros, la relación respecto de los de recto es de 0.1%, presentándose con un rango entre 40-70 años. El objetivo es analizar una serie de pacientes, el tratamiento empleado y actualización bibliográfica. Material y Método: Sobre una base de datos retrospectiva entre enero de 1983 y diciembre de 2018, sobre 421 pacientes operados por cáncer recto-anal, fueron extraídos 6 que correspondieron a tumores del musculo liso. Resultados: Correspondieron al sexo femenino 4, con edades entre 49 y 75 años (57.5 años); 4 de localización rectal, de ellos 3 fueron leiomiosarcoma, y 2 anales (leiomioma). En 2 se obtuvo diagnostico preoperatorio de certeza por punción mediante Tru-Cut. De 3 pacientes con leiomiosarcoma, a 2 se les realizo cirugía radical y al restante biopsia. Los 2 resecados recidivaron a los 6 meses y al año. Los 3 fallecieron entre los 2 y 16 meses por progresión de la enfermedad. La paciente con diagnóstico de leiomioma rectal, operada mediante cirugía radical, se encuentra sin recidiva a 18 meses. Los 2 pacientes resecados localmente por leiomioma de ano, presentaron en el postoperatorio absceso y fistula extraesfinteriana, uno de ellos con incontinencia severa. Ambos fueron re-operados y se encuentran asintomáticos, libres de recidiva a los 36 y 60 meses. Discusión: Los tumores del músculo liso ano-rectal son infrecuentes y presentan síntomas inespecíficos. La biopsia preoperatoria es imperiosa a fin de establecer una adecuada estrategia quirúrgica. Los malignos tienen alto índice de recidiva y mortalidad. (AU)
Introduction: Smooth muscle ano-rectal tumors are rare; the relation with respect to the rectum is 0.1%, in a patient's age range between 40-70 years. The objective is the analysis of a series of patients, the treatment used and bibliographic update. Material and method: On a retrospective, database between January 1983 and December 2018. About 421 patients operated for rectum-anal cancer, of which 6 corresponded to smooth muscle tumors. Results: Four were female, with ages between 49 and 75 years (57.5 years average); 4 were of rectal location, of which 3 were leiomyosarcoma, and 2 anal (leiomyoma). In two, a preoperative diagnosis of certainty was obtained by Tru-Cut. Two out of 3 patients with leiomyosarcoma, underwent radical surgery and the remaining one a biopsy. The two resected relapsed at 6 months and at one year. All 3 died between 2 and 16 months due to disease progression. The patient diagnosed with rectal leiomyoma, operated by radical surgery, is without recurrence at 18 months. The 2 patients resected locally for anus leiomyoma showed abscess and extrasphincteric fistula in the postoperative period, one of them with severe incontinence. Both were re-operated and are asymptomatic, free of recurrence at 36 and 60 months. Discussion: Ano-rectal smooth muscle tumors are uncommon and have nonspecific symptoms. Preoperative biopsy is imperative in order to establish an appropriate surgical strategy. Malignant tumors have a high rate of recurrence and mortality. (AU)
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Humanos , Masculino , Femenino , Persona de Mediana Edad , Anciano , Canal Anal/patología , Neoplasias del Recto/diagnóstico , Neoplasias del Recto/patología , Recto/patología , Biopsia con Aguja , Leiomiosarcoma/diagnóstico , Leiomiosarcoma/patología , Cuidados Posoperatorios , Neoplasias del Recto/cirugía , Diagnóstico por Imagen , Inmunohistoquímica , Estudios Retrospectivos , Proctoscopía/métodos , Quimioterapia Combinada , Leiomiosarcoma/cirugíaRESUMEN
Ovarian tumours in mares represent 2.5% to 6% of the most frequent neoplasms found in the equine species, with a higher chance of benignity. This study aims to describe a case of two different tumours found in the same ovary of a mare that presented clinical signs of suppressed oestrous cycle during 5 years. After unilateral ovariectomy, the ovary was sent to the histopathology examination which determined a mixed tumour of granulosa cell and leiomyosarcoma. After treatment, the mare returned to oestrus and got pregnant in the next season.
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Tumor de Células de la Granulosa/veterinaria , Enfermedades de los Caballos/patología , Leiomiosarcoma/veterinaria , Neoplasias Ováricas/veterinaria , Animales , Femenino , Tumor de Células de la Granulosa/patología , Tumor de Células de la Granulosa/cirugía , Enfermedades de los Caballos/cirugía , Caballos , Leiomiosarcoma/patología , Leiomiosarcoma/cirugía , Neoplasias Ováricas/patología , Neoplasias Ováricas/cirugía , Ovariectomía/veterinariaRESUMEN
Introducción: Los leiomiosarcomas (LMS) son tumores raros del tubo digestivo, corresponden a neoplasias malignas originadas de células de músculo liso, representando sólo el 0,1% del total. La localización rectal se da en el 11% de los LMS, aunque representan menos del 1% de los tumores malignos colón- icos. En nuestro medio también es una patología de baja frecuencia. Objetivo: Exponer método diagnóstico, tratamiento, y resultado quirúrgico en paciente con LMS rectal. Caso Clínico: Paciente mujer, 53 años, atendida en el Hospital Padre Hurtado, gran tabáquica, que se presentó con un cuadro de 1 año de evolución de dolor perianal, defecación laboriosa y 3 episodios de rectorragia. Al examen físico presentaba al tacto rectal a 4 cm del margen anal una masa de ±2 cm de superficie irregular y de consistencia pétrea. Se realizó colonoscopía objetivando la masa tumoral y tomando biopsia la que se informó como LMS rectal, incluyendo resultados de inmunohistoquímica. Siguiendo con el estudio se realizó TAC y RNM. Se decide la intervención quirúrgica realizándose una resección anterior baja, sin incidentes durante la cirugía y con un buen postoperatorio. Conclusión: El leiomiosarcoma es de extrema baja frecuencia en nuestro país, con sólo pocos casos reportados. La confirmación diagnóstica es con biopsia e inmunohistoquímica. La cirugía sugerida en la literatura es la resección anterior baja, la que se realiza en este caso con buenos resultados. (AU)
Introduction: Leiomyosarcomas (LMS) are rare digestive tract tumors. It is a malignant tumor originated from smooth muscle cells, representing only 0.1% of the total. Rectal location is given in 11% of LMS, however, they represent less than 1% of all malignant colon tumors. It is a very infrequent tumor in our medium. Objective: Show the diagnostic method, treatment, and surgical result. Case Presentation: A 53-year-old female, treated in Padre Hurtado Hospital, heavy smoker, presented with a 1-year history of perianal pain, difficulty during defecation, and three episodes of rectal bleeding. Digital rectal examination showed a mass 4 cm from the anal margin of approximately 2 cm, of irregular surface, and very hard consistency. A colonoscopy was performed, objectivating the tumoral mass and taking a biopsy which was informed as a rectal LMS, including immunohistochemistry results. Continuing with the study, a CT-Scan and MRI were performed. Treatment of choice was surgery; a low anterior resection was carried out with no incidents during the surgery and a favorable post-operatory. LMS is extremely infrequent in our country, with only a few cases reported. Conclusion: Diagnostic confirmation is made through biopsy and immunohistochemistry. Surgery suggested by literature is low anterior resection, which was carried out in this case with good results. (AU)