Tratamiento quirúrgico del leiomiosarcoma de vena cava inferior

Introducción: La baja incidencia del leiomiosarcoma de la vena cava inferior dificulta tanto la estandarización del diagnóstico como el tratamiento. Objetivo: Presentar el manejo realizado en nuestro centro de un paciente que desarrolló un leiomiosarcoma de vena cava inferior, una patología de baja incidencia y que las posibilidades de realizar un rescate quirúrgico son muy bajas. Resultados: Se presenta el caso de un paciente de 54 años con una tumoración sólida en porción infrarrenal y yuxtarrenal de vena cava inferior de 71 × 76 × 117 mm compatible con leiomiosarcoma de vena cava, con infiltración de uréter derecho que ocasiona uropatía obstructiva derecha grado I-II sin alteración de la función renal, que fue resecada y reconstruida mediante prótesis sin complicaciones. Discusión: Se discute la fisiopatología, el diagnóstico y manejo en relación con el caso presentado. Conclusión: la baja incidencia de estos tumores dificulta tanto la estandarización del diagnóstico como del tratamiento, aunque la cirugía sigue siendo el tratamiento de elección.

Introduction: The low incidence of leiomyosarcoma of the inferior vena cava hinders both the standardization of diagnosis and treatment. Objective: To present the management carried out in our center of a patient who developed an inferior vena cava leiomyosarcoma, a low incidence pathology with uncertain surgical rescue. Results: 54-year-old patient with a solid tumor in the infrarenal and juxtarenal portions of the inferior vena cava of 71 × 76 × 117 mm compatible with leiomyosarcoma of the vena cava, with infiltration of the right ureter that causes right obstructive uropathy grade I-II without kidney function changes; tumour was resected and continuity reconstructed with a prosthesis without complications. Discussion: The pathophysiology, diagnosis and management are commented. Conclusion: the low incidence of these lesions makes it difficult to standardize both diagnosis and treatment, although surgery remains the treatment of choice.

Tumor de músculo liso uterino de potencial maligno incierto (STUMP): reporte de caso y revisión de la literatura / Uterine smooth muscle tumor of uncertain malignant potential (STUMP): case report and literature review

Myometrial smooth muscle neoplasms are the most common gynecologic tumors with a prevalence of 70-80% at age 50. Among women undergoing hysterectomy or myomectomy for a suspected diagnosis of leiomyoma, 0.01% receive a diagnosis of STUMP. Clinically, the average age of presentation is between 41-48 years. Tumors ranging from 3 to 30 cm have been described. Signs and symptoms are similar to leiomyomas, such as abnormal uterine bleeding, anemia, dysmenorrhea, pelvic pain, pelvic mass, infertility or other types of pain secondary to compression of adjacent organs. Due to the limited literature available, there is no definite management consensus, and treatment and follow-up options are limited to observational studies. The standard treatment is total hysterectomy with or without bilateral salpingo-oophorectomy; if fertility has already been completed, there is no role for adjuvant hormonal therapy or chemotherapy. The overall 5-year survival is 92-100%. A case of a 31-year-old woman with STUMP is presented.

Leiomiosarcoma de vena cava inferior, reconstrucción mediante interposición de prótesis y anticoagulación

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.

Leiomioma submucoso de gran tamaño: una presentación inusual. Reporte de Caso / Large submucosal leiomyoma: an unusual presentation

Se ha postulado que más del 70 % de las mujeres antes de la menopausia son diagnosticadas de leiomiomas; de estas un 25 % presentan sintomatología grave. La ecografía es la modalidad de imagen de primera elección para su estudio. Se tiene registros de progresión de leiomiomas a leiomiosarcomas, que conllevan a un mal pronóstico y son responsables de una cuarta parte de las muertes por neoplasias uterinas, con una sobrevida a 5 años que van desde 46-53 %, lo cual incentiva el diagnóstico precoz y eficaz de masas uterinas. El caso reportado es una presentación inusual de un leiomioma de gran tamaño, que por los hallazgos de imagen se reportó como una neoplasia maligna, su aspecto macroscópico totalmente atípico reforzaba la sospecha imagenológica. Solo se pudo determinar el diagnóstico definitivo mediante estudio histopatológico posterior a histerectomía. Las evaluaciones ginecológicas de rutina deben incluir exámenes especializados de imagen pélvica, la ausencia de presentación típica o síntomas característicos de una patología no debe ser motivo para obviar exámenes complementarios que permitan un diagnóstico oportuno y tratamiento eficiente de leiomiomas, incluso en casos de presentaciones inusuales y desafiantes para el diagnóstico, como el que se ha reportado en esta obra.

It has been postulated that more than 70% of premenopausal women are diagnosed with leiomyomas; Of these, 25% have severe symptoms. Ultrasound is the imaging modality of choice for its study. There are reports of progression from leiomyomas to leiomyosarcomas, which leads to a poor prognosis, and is responsible for a quarter of deaths from uterine neoplasms, with a 5-year survival ranging from 46-53%; this should encourage early and effective diagnosis of uterine masses. The reported case is an unusual presentation of a large leiomyoma, which due to its imaging characteristics, was reported as a malignant neoplasm. Its completely atypical macroscopic appearance reinforced the imaging diagnosis suspicion. The definitive diagnosis could only be determined by histopathological study after hysterectomy. Routine gynecological evaluations should include specialized pelvic imaging exams, the absence of a typical presentation or characteristic symptoms of a pathology should not be a reason to obviate ancillary testing that would allow a timely diagnosis and effective treatment of leiomyomas, even in unusual and challenging presentations, such as with the patient in this case report.

Foreskin leiomyosarcoma: a case report. / [Leiomiosarcoma de prepucio: reporte de un caso]

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.

Tratamiento quirúrgico de los leiomiosarcomas de vena cava. Serie de casos en un hospital de tercer nivel y revisión de la literatura / Surgical treatment of vena cava leiomyosarcomas: Series of cases in a referral center and literature review

Introducción Los tumores primarios de vena cava inferior son tumores raros de origen mesenquimal que surgen de la musculatura lisa de la pared. Debido a su escasa prevalencia, existen pocos datos definitivos sobre su tratamiento y pronóstico. Su tratamiento se basa en principios oncológicos generales. Métodos Se ha analizado una serie de 6 casos intervenidos desde 2010 a 2020, evaluando distintos parámetros relacionados con las características demográficas del tumor, del tratamiento recibido y de los resultados obtenidos en supervivencia y morbilidad. Además, se ha llevado a cabo una revisión bibliográfica de la evidencia disponible actualmente. Resultados En todos los pacientes se llevó a cabo una resección quirúrgica óptima con R0 en 4/6 y R1 en 2/6. La mayor morbilidad sucedió en un paciente fallecido en periodo intraoperatorio. Se realizó cavorrafia en un paciente y cavoplastia en 5/6 utilizando injerto criopreservado en 3/6 y prótesis en 2/6. Al final del seguimiento de nuestra serie (con una media de seguimiento de 10,7 meses), el 50% de los pacientes continúan vivos. La media de supervivencia fue de 11,3±9,07 meses. De los 6 pacientes, 3 presentaron recidivas hematógenas con un intervalo libre de enfermedad de 9±2 meses. Conclusión El diagnóstico y tratamiento del leiomiosarcoma de vena cava inferior continúa siendo un reto. Debido a su baja prevalencia, resultará difícil establecer un tratamiento totalmente estandarizado, y se recomienda su abordaje en centros especializados. Por otra parte, se deberían intentar aunar los casos intervenidos de cara a avanzar en el conocimiento del abordaje de esta enfermedad (AU)

Introduction Primary tumors of the inferior vena cava are rare tumors of mesenchymal origin. They arise from the smooth muscles of the vena cava wall. Due to its low prevalence, there are few definitive data on its treatment and prognosis. Its treatment is based on general oncological principles. Methods A series of six cases operated from 2010 to 2020 were analyzed. Different parameters related to the demographic characteristics, the tumor, the treatment received, and the results obtained in survival and morbidity were analyzed. In addition, a bibliographical review of the currently available evidence was carried out. Results Optimal surgical resection was accomplished in all patients with R0 in 4/6 and R1 in 2/6. The greatest morbidity occurred in a patient who died in the intraoperative period. Cavography was performed in one patient and cavoplasty in 5/6 using cryopreserved graft in 3/6 and prothesis in 2/6. The 50% were still alive at the end of the follow-up (with a mean follow-up of 10.7 months). The mean survival was 11.3±9.07 months. 3/6 patients presented hematogenous recurrences with a disease-free interval of 9±2 months. Conclusion The diagnosis and treatment of inferior vena cava leiomyosarcoma is still a challenge. Due to its low prevalence, it will be difficult to establish a totally standardized treatment and its approach is recommended in specialized centers. On the other hand, a multicentric study should be made to collect the most cases as possible in order to advance in the understanding of the approach to this disease (AU)

[Leiomyosarcoma of the nasal fossa. A case report]. / Leiomiosarcoma de fosa nasal. A propósito de un caso.

A 54-year-old female patient presented with a left nasal obstruction. On physical examination a pink delimited mass in the left nostril was observed. A cranial computed tomography scan revealed an expansive mass in the upper anterior third of the left nasal fossa, partially obstructing it. Endoscopic resection of the mass was performed. Histopathology revealed an atypical mesenchymal proliferation formed by cells disposed in disorganized and interconnected long bundles. Tumor cells had abundant eosinophilic cytoplasm and an oval, vesicular and hyperchromatic nucleus. Frequent mitotic figures were observed, many of them atypical. Necrosis was not observed. Immunohistochemistry showed tumor cells to be positive for calponin, muscle specific actin, caldesmon and smooth muscle specific myosin. Ki-67 index proliferation was 30%. A diagnosis of leiomyosarcoma of the nasal fossa was established.

Leiomiosarcoma de fosa nasal. A propósito de un caso / Leiomyosarcoma of the nasal fossa. A case report

Paciente mujer de 54 años que acude a consulta por un cuadro de obstrucción nasal. En la exploración física se observa una lesión rosada, bien delimitada, en la fosa nasal izquierda. Se realiza TAC de macizo facial en la que se observa una masa expansiva a nivel del tercio anterosuperior de la fosa nasal izquierda. Se realiza resección endoscópica. Histológicamente se observa una proliferación mesenquimal atípica constituida por células que forman haces largos desorganizados y entrecruzados. Las células tumorales presentan un citoplasma amplio eosinófilo y núcleo ovalado, vesiculoso e hipercromático. Se aprecian frecuentes figuras mitóticas, muchas de ellas atípicas. No se observa necrosis. En el estudio inmunohistoquímico se evidenció inmunorreactividad de las células tumorales frente a calponina, actina muscular específica, caldesmón y miosina específica de músculo liso. El índice de proliferación frente a KI-67 fue de un 30%. Con todos estos hallazgos se estableció el diagnóstico de leiomiosarcoma de fosa nasal.(AU)

A 54-year-old female patient presented with a left nasal obstruction. On physical examination a pink delimited mass in the left nostril was observed. A cranial computed tomography scan revealed an expansive mass in the upper anterior third of the left nasal fossa, partially obstructing it. Endoscopic resection of the mass was performed. Histopathology revealed an atypical mesenchymal proliferation formed by cells disposed in disorganized and interconnected long bundles. Tumor cells had abundant eosinophilic cytoplasm and an oval, vesicular and hyperchromatic nucleus. Frequent mitotic figures were observed, many of them atypical. Necrosis was not observed. Immunohistochemistry showed tumor cells to be positive for calponin, muscle specific actin, caldesmon and smooth muscle specific myosin. Ki-67 index proliferation was 30%. A diagnosis of leiomyosarcoma of the nasal fossa was established.(AU)

Surgical treatment of vena cava leiomyosarcomas: Series of cases in a referral center and literature review.

BACKGROUND: Primary tumors of the inferior vena cava are rare tumors of mesenchymal origin. They arise from the smooth muscles of the vena cava wall. Due to its low prevalence, there are few definitive data on its treatment and prognosis. Its treatment is based on general oncological principles. METHODS: A series of 6 cases operated from 2010 to 2020 were analyzed. Different parameters related to the demographic characteristics, the tumor, the treatment received, and the results obtained in survival and morbidity were analyzed. In addition, a bibliographical review of the currently available evidence was carried out. RESULTS: Optimal surgical resection was accomplished in all patients with R0 in 4/6 and R1 in 2/6. The greatest morbidity occurred in a patient who died in the intraoperative period. Cavorraphy was performed in one patient and cavoplasty in 5/6 using cryopreserved graft in 3/6 and prothesis in 2/6. The 50% were still alive at the end of the follow-up (with a mean follow-up of 10.7 months). The mean survival was 11.3 ± 9.07 months. 3/6 patients presented hematogenous recurrences with a disease-free interval of 9 ± 2 months. CONCLUSION: The diagnosis and treatment of inferior vena cava leiomyosarcoma is still a challenge. Due to its low prevalence, it will be difficult to establish a totally standardized treatment and its approach is recommended in specialized centers. On the other hand, a multicentric study should be made to collect the most cases as possible in order to advance in the understanding of the approach to this disease.

Caracterização anatomopatológica de leiomiossarcomas uterinos da microrregião de Catanduva-SP / Anatomopathological aspects of uterine leimyosarcomas from the microregion of Catanduva-SP / Caracterización anatomopatológica de los leiomiosarcomas uterinos de la microrregión de Catanduva-SP

Introdução: O leiomiossarcoma é uma neoplasia maligna rara que afeta o corpo uterino. O diagnóstico definitivo ocorre por meio do exame histopatológico, que revela sua histogênese mesenquimal, a qual pode ser validada por marcadores imuno-histoquímicos de musculatura lisa ou do ciclo celular. Objetivos: Levantar a incidência de leiomiossarcomas uterinos diagnosticados pelo Serviço de Patologia do Hospital Emílio Carlos, Catanduva-SP, e descrever as variáveis anatomopatológicas de cada um deles. Material e Método: Foram avaliados os casos de leiomiossarcomas uterinos no período de janeiro de 2018 a agosto de 2021 a partir dos relatórios de exames anatomopatológicos. As variáveis relatadas envolveram idade, tamanho da lesão, grau de diferenciação, invasão linfovascular ou perineural e positividade para marcadores imuno-histoquímicos. Resultados: Encontrou-se 01 caso de leiomiossarcoma, medindo 6,5 cm, grau de diferenciação moderado, 20 mitoses para cada 10 campos de grande aumento e ausência de invasão em vasos e filetes nervosos. A imunomarcação ocorreu para actina de músculo liso, caldesmon, desmina e p16. O índice de proliferação celular medido pelo Ki67 foi alto (>25%). Conclusão: O leiomiossarcoma, embora raro, envolve uma análise criteriosa para sua diferenciação de neoplasias benignas, sendo o exame histopatológico, complementado pela imuno-histoquímica, fundamental nessa etapa. (AU)

Introduction: Leiomyosarcoma is a rare malignant neoplasm that affects the uterine body. The definitive diagnosis occurs through histopathological examination, which reveals its mesenchymal histogenesis, which can be validated by immunohistochemical markers of smooth muscle or cell cycle. Objectives: To raise the incidence of uterine leiomyosarcomas diagnosed by the Pathology Service of the Emílio Carlos Hospital, Catanduva-SP, and describe the anatomopathological variables of each of them. Material and Method: Cases of uterine leiomyosarcomas were evaluated from January 2018 to August 2021 from the reports of anatomopathological examinations. The variables reported involved age, lesion size, degree of differentiation, lymphovascular or perineural invasion and positivity for immunohistochemical markers. Results: One case of leiomyosarcoma was found, measuring 6.5 cm, moderate degree of differentiation, 20 mitoses for every 10 fields of great increase and absence of invasion in vessels and nerve fillets. Immunostaining occurred for smooth muscle actin, caldesmon, desmin and P16. The rate of cell proliferation measured by Ki67 was high (>25%). Conclusion: The leiomyosarcoma, although rare, involves a careful analysis for its differentiation from benign neoplasms, and the histopathological examination, complemented by immunohistochemistry, is fundamental in this stage.(AU)

Introducción: El leiomiosarcoma es una neoplasia maligna rara que afecta al cuerpo uterino. El diagnóstico definitivo se realiza mediante el examen histopatológico, que revela su histogénesis mesenquimal, que puede ser validada por marcadores inmunohistoquímicos de la musculatura lisa o del ciclo celular. Objetivos: Levantar la probabilidad de leiomiosarcomas uterinos diagnosticados por el Servicio de Patología del Hospital Emílio Carlos, Catanduva-SP, y describir las variables anatomopatológicas de cada uno de ellos. Material y Método: Se evaluaron los casos de leiomiosarcomas uterinos en el período comprendido entre enero de 2018 y agosto de 2021 a partir de los info rmes de examen anatomopatológico. Las variables reportadas fueron la edad, el tamaño de la lesión, el grado de diferenciación, la invasión linfovascular o perineural y la positividad de los marcadores inmunohistoquímicos. Resultados: Encontramos 01 caso de leiomiosarcoma, con un tamaño de 6,5 cm, un grado de diferencia moderado, 20 mitosis por cada 10 campos de gran aumento y ausencia de invasión en vasos y filetes nerviosos. La inmunomarcación se dio para la actina del músculo liso, el caldesmón, la desmina y la p16...(AU)

Leiomiosarcoma primario de hueso en fémur distal metastásico a clavícula: reporte de un caso / Primary leiomyosarcoma of bone in the distal femur with clavicle metastasis: a case report

El leiomiosarcoma (LMS) es un tipo de tumor de células fusiformes de muy baja incidencia, que tiene un comportamiento agresivo, con alta tasa de mortalidad, por lo que el manejo debe ser quirúrgico, con una resección amplia de la lesión. No está claro el papel de la radio ni de la quimioterapia en su manejo. Presentamos a una paciente de 28 años que consultó por dolor de 2 meses en la rodilla derecha. Radiográficamente, se caracterizó como una lesión osteolítica pura en el fémur distal. La resonancia nuclear magnética (RNM) contrastada mostró áreas hipervasculares dentro del tumor. La gammagrafía mostró un marcado aumento en la captación de radiotrazadores. Se tomó una biopsia, con un informe de patología de LMS óseo bien diferenciado. Se trató con 3 ciclos de quimioterapia neoadyuvante preoperatoria con ifosfamida 1.000 mg/m2 en los días 1 a 3, además de doxorrubicina 70 mg/m2 , y resección quirúrgica de la lesión y salvamento de la extremidad con endoprótesis de rodilla. Una vez que se resecó la lesión, la paciente recibió quimioterapia adyuvante con 4 ciclos de gencitabina 1.000 mg/m2 entre los días 1 y 8, y doxetacel 70 mg/m2 el día 1. Durante los dos meses de seguimiento, la paciente presenóa una fractura en el tercio medio de la clavícula, compatible con una lesión patológica en radiografías y tomografía por emisión de positrones (TEP). La biopsia reveló una lesión metastásica de LMS óseo que fue tratada mediante resección quirúrgica de la clavícula. Este es un caso único, dado que, durante el seguimiento, recibió tratamiento adyuvante con quimioterapia y se evaluó con una TEP, con una evolución clínica satisfactoria y sin evidencia de nuevas lesiones

Leiomyosarcoma (LMS) is a type of spindle-cell tumor of very low incidence that tumor has an aggressive behavior, with high mortality rates; therefore, its management must be surgical, with a wide resection of the lesion. The role of radio and chemotherapy in its management is not clear. We present the case of a 28-year-old female patient who consulted for pain lasting 2 months in the right knee. Radiographically, it was characterized as a pure osteolytic lesion in the distal femur. Contrast magnetic resonance imaging (MRI) showed hypervascular areas within the tumor. The scintigraphy showed a marked increase in radiotracer uptake. A biopsy was taken, with a pathology report of well-differentiated osseous LMS. It was treated with 3 cycles of preoperative neoadjuvant chemotherapy with ifosfamide 1,000 mg/m2 in the first 3 days, as well as doxorubicin 70 mg/m2 , and surgical resection of the lesion and limb salvage with knee endoprosthesis. Once the lesion was resected, the patient underwent adjuvant chemotherapy, with 4 cycles of gencitabine 1,000 mg/m2 between days 1 and 8, and doxetacel 70 mg/m2 on day 1. During the 2-month follow-up, the patient presented a fracture in the middle third of the clavicle, which was compatible with a pathological lesion on radiographs and positron-emission tomography (PET) scans. The biopsy showed a metastatic lesion of bone LMS, which was treated by surgical resection of the clavicle. This is a unique case, given that, during the follow-up, the patient underwent adjuvant treatment with chemotherapy, and was evaluated with a PET scan, with a satisfactory clinical evolution and no evidence of new lesions.

Leiomiosarcoma de vena cava / Vena cava leiomyosarcoma

Caso clínico: se presenta el caso de un varón de 64 años con sospecha de leiomiosarcoma de vena cava inferior (VCI) tratado con radioterapia neoadyuvante previa a resección quirúrgica en bloque tumoral y de VCI con reconstrucción mediante injerto de PTFE anillado, seguido de quimioterapia. Discusión: el leiomiosarcoma de VCI es una patología maligna muy infrecuente, de mal pronóstico y que requiere un manejo multidisciplinar. Representan la localización más frecuente de los leiomiosarcomas venosos, tienen predilección por el sexo femenino y una edad media de presentación entre 50 y 60 años. El síntoma más frecuente es el dolor abdominal, aunque muchos son asintomáticos. La prueba diagnóstica de elección es la RM y el tratamiento se basa en la resección quirúrgica, no estando aún claramente definido el papel de la radio y quimioterapia.(AU)

Case report: we present the case of a 64-year-old man with suspected inferior vena cava leiomyosarcoma (IVC) treated with neoadjuvant radiotherapy, tumor and IVC overall surgical resection with a ringshaped PTFE graft reconstruction, followed by chemotherapy. Discussion: IVC leiomyosarcoma is a very infrequent malignant disease, its prognosis is dark and requires multidisciplinary management. They represent the most frequent location of venous leiomyosarcomas, have a predilection for the female sex and a mean age of presentation between 50 and 60 years. The most frequent symptom is abdominal pain, although many are asymptomatic. The diagnostic test of choice is MRI and treatment is based on surgical resection, the role of radio and chemotherapy is not clearly defined yet.(AU)

Surgical treatment of vena cava leiomyosarcomas: Series of cases in a referral center and literature review. / Tratamiento quirúrgico de los leiomiosarcomas de vena cava. Serie de casos en un hospital de tercer nivel y revisión de la literatura.

INTRODUCTION: Primary tumors of the inferior vena cava are rare tumors of mesenchymal origin. They arise from the smooth muscles of the vena cava wall. Due to its low prevalence, there are few definitive data on its treatment and prognosis. Its treatment is based on general oncological principles. METHODS: A series of six cases operated from 2010 to 2020 were analyzed. Different parameters related to the demographic characteristics, the tumor, the treatment received, and the results obtained in survival and morbidity were analyzed. In addition, a bibliographical review of the currently available evidence was carried out. RESULTS: Optimal surgical resection was accomplished in all patients with R0 in 4/6 and R1 in 2/6. The greatest morbidity occurred in a patient who died in the intraoperative period. Cavography was performed in one patient and cavoplasty in 5/6 using cryopreserved graft in 3/6 and prothesis in 2/6. The 50% were still alive at the end of the follow-up (with a mean follow-up of 10.7 months). The mean survival was 11.3±9.07 months. 3/6 patients presented hematogenous recurrences with a disease-free interval of 9±2 months. CONCLUSION: The diagnosis and treatment of inferior vena cava leiomyosarcoma is still a challenge. Due to its low prevalence, it will be difficult to establish a totally standardized treatment and its approach is recommended in specialized centers. On the other hand, a multicentric study should be made to collect the most cases as possible in order to advance in the understanding of the approach to this disease.

Primary orbital leiomyosarcoma / Leiomiosarcoma primario de órbita

Abstract Leiomyosarcoma is an aggressive mesenchymal malignant tumor which rarely presents in head and neck structures. There are few cases published in the literature, and clinical suspicion, diagnosis and early treatment are considered to be essential for dealing with the adverse outcomes derived from its poor prognosis. We report the first case of primary orbital leiomyosarcoma in Colombia, diagnosed in an older adult who consulted due to eight months of progressive growth of the left frontotemporal region along with dysarthria, vision loss and ipsilateral painful proptosis. Imaging studies revealed a large osteolytic lesion involving the orbit and central nervous system. An incisional biopsy was performed, reporting high-grade leiomyosarcoma. Once metastasis was ruled out, complete excision of the lesion was ordered, along with radiation therapy. After 10 months of close follow up, the patient had progressed well, with no local recurrences or distant metastases. (Acta Med Colomb 2021; 46. DOI: https://doi.org/10.36104/amc.2021.1937).

Resumen El leiomiosarcoma es un tumor maligno de origen mesenquimal de naturaleza agresiva que infrecuentemente se presenta en estructuras de cabeza y cuello. Son pocos los casos publicados en la literatura, siendo considerado su sospecha, diagnóstico y tratamiento temprano fundamentales para enfrentar los desenlaces adversos derivados de su mal pronóstico. Se reporta el primer caso en Colombia de leiomiosarcoma primario de órbita, diagnosticado en un paciente adulto mayor quien consultó por ocho meses de aumento progresivo de la zona frontotemporal izquierda acompañado de disartria, pérdida de visión y proptosis dolorosa ipsilateral. Los estudios imagenológicos eviden ciaron extensa lesión osteolítica con compromiso orbitario y de sistema nervioso central. Se realizó biopsia incisional, con reporte de leiomiosarcoma de alto grado. Tras descartar metástasis, se indicó exéresis completa de la lesión e inicio de radioterapia. A los diez meses de estrecho seguimiento, el paciente presentó una evolución favorable sin recurrencias locales ni metástasis a distancia. (Acta Med Colomb 2021; 46. DOI: https://doi.org/10.36104/amc.2021.1937).

Leiomiosarcoma retroperitoneal con resección de vena cava / Retroperitoneal leiomyosarcoma with resection of the vena cava

RESUMEN El leiomiosarcoma vascular es un tumor maligno de baja incidencia cuya localización más frecuente es la vena vava inferior (VCI). Se presenta habitualmente en la 6a década de la vida. Según su localización se describen 3 tipos de acuerdo con su relación con las venas suprahepáticas y renales. Los cuadros clínicos de presentación son inespecíficos, ya que suelen debutar como hallazgos o con síndromes de congestión venosa pélvica/miembros inferiores. Su tratamiento quirúrgico radical requiere un equipo multidisciplinario entrenado en cirugía retrope ritoneal y vascular.

ABSTRACT Vascular leiomyosarcomas are rare tumors and are usually localized in the inferior vena cava (IVC). They usually occur in the 6th decade of life. They are classified into 3 groups according to the relation with the hepatic and renal veins. The clinical presentation is unspecific, ranging from an incidental finding to symptoms of venous pelvis congestion of lower extremity edema. Radical resection is the treatment of choice and requires multidisciplinary team trained in retroperitoneal and vascular surgery.

Leiomiosarcoma primario de cornete nasal inferior: reporte de caso clínico y revisión de la literatura / Primary leiomyosarcoma of inferior nasal turbinate: clinical case report and literature review

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.

Tratamiento multidisciplinar del leimiosarcoma de vena cava inferior / Multidisciplinary treatment of leiomyosarcoma of inferior cava

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.

Uterine pleomorphic leiomyosarcoma with osteoclastic giant cells: Case report with peritoneal washing cytology and cell block study.

Uterine leiomyosarcoma (LMS) with osteoclastic giant cells (OGCs) is extremely rare. However, its morphological appearance and aggressive behavior may have resulted in its being diagnosed as so-called giant cell malignant fibrous histiocytoma (MFH) in the past. Effusions are not uncommon in LMS and may be indicative of an unfavorable prognosis. We report a case with the cytological appearance of a uterine LMS with OGCs metastatic to lower pelvic peritoneum. The pelvic washing specimen consisted of three-dimensional aggregates of atypical cells. The cytohistologic and immunohistochemical study obtained from the cell block and the tumor mass showed overlapping features such as bizarre pleomorphic spindle cells containing numerous evenly dispersed OGCs. The malignant tumor cells showed extensive positivity for desmin, h-caldesmon and multifocal positivity for smooth muscle actin (SMA) whereas OGCs stained with CD68. We stress the usefulness of performing cell block and subsequent immunohistochemistry in order to make an accurate cytohistologic correlation.

Sarcoma gástrico primario: reporte de caso y revisión de la literatura / Primary gastric sarcoma: Case report and literature review

Resumen El cáncer gástrico, patología neoplásica de innegable importancia, corresponde en el 90 % de los casos a un adenocarcinoma. Dentro del 10 % restante, los linfomas y los tumores estromales gastrointestinales (Gastrointestinal Stromal Tumor, GIST) constituyen la mayoría. Sin embargo, los sarcomas no GIST siguen siendo un diagnóstico diferencial posible para tener en cuenta y configuran una patología neoplásica de tratamiento fundamentalmente quirúrgico. En particular, el leiomiosarcoma representa menos del 1 % de los tumores malignos del estómago y la literatura disponible al respecto consiste en reportes de caso o serie de casos. Por su rareza, presentamos este caso clínico y revisamos la literatura relacionada.

Abstract Gastric cancer, a neoplastic pathology of undeniable importance, accounts for 90% of cases to adenocarcinoma. GIST lymphomas and gastrointestinal stromal tumors are the majority of the other 10%. However, non-GIST sarcomas remain a possible differential diagnosis to keep in mind and constitute a neoplastic pathology whose treatment is fundamentally surgical. Leiomyosarcoma represents less than 1% of malignant stomach tumors, and the available literature consists of case reports or case series. Because of its rarity, we present this clinical case and review the literature.

Leiomiosarcoma y colitis ulcerosa: reporte de caso / Leiomyosarcoma and ulcerative colitis: Case report

Resumen Los pacientes con colitis ulcerosa (CU) y enfermedad de Crohn (EC) presentan un mayor riesgo de cáncer colorrectal (CCR), debido a la inflamación crónica, la susceptibilidad genética y los factores de riesgo ambientales. Sin embargo, las neoplasias no epiteliales son infrecuentes. Presentamos el caso de un varón de 83 años con una CU de larga evolución, que presenta una lesión polipoide. Una vez resecada, se diagnosticó de un leiomiosarcoma de alto grado. En la literatura, únicamente se han publicado tres casos previos de leiomiosarcoma en pacientes con CU, por lo que este reporte representaría el cuarto caso. La asociación directa de la CU y el leiomiosarcoma no ha sido bien establecida. No obstante, se sugiere que la inmunosupresión y la inflamación crónica son factores de riesgo.

Abstract Ulcerative colitis (UC) and Crohn's disease (CD) patients present an increased risk of colorectal cancer (CRC) due to chronic inflammation, genetic susceptibility and environmental risk factors. In contrast, non-epithelial neoplasms are uncommon. We discuss the case of an 83-year-old male with a long-standing UC, presenting with a polypoid lesion. Once resected, the lesion was identified as a high-grade leiomyosarcoma. A review of the literature revealed that only three previous cases of leiomyosarcoma among patients with UC have been published. Thus, this one would represent the fourth case where this rare non-epithelial neoplasm was detected in a patient with UC. The direct association of UC and leiomyosarcoma has not been well established; however, immunosuppression is suggested to be a risk factor for leiomyosarcoma in the literature.