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.

Tumores ano-rectales del músculo liso / Smooth muscle ano-rectal tumors

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)

Leiomiosarcoma rectal: reporte de un caso / Rectal leiomyosarcoma: a case report

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)

Ligadura da Veia Renal Esquerda e Preservação da Função Renal pelas Colaterais nos Tumores Retroperitoneais / Left Renal Vein Ligation and Preservation of Renal Function Through the Collateral Circulation after Retroperitoneal Tumor Resection / Ligadura de la Vena Renal Izquierda y Preservación de la Función Renal por los Colaterales en los Tumores Retroperitoneales

Introdução: O retroperitônio pode apresentar diversas patologias e possui certa complexidade e mau prognóstico em virtude do íntimo contato e envolvimento de grandes vasos nas abordagens cirúrgicas. A cirurgia é a opção terapêutica associada à sobrevida prolongada. O estudo tem por objetivo demonstrar que é possível a preservação da função renal, pelas colaterais, após a ligadura ou ressecção da veia renal esquerda sem reconstrução vascular. Relato dos casos: Dois casos de pacientes com tumores retroperitoneais com invasão vascular local onde, para sua completa ressecção, fizeram-se necessárias a secção e a ligadura da veia renal esquerda. Evoluíram com preservação da função renal em razão da drenagem venosa pelas colaterais, não sendo necessária a reconstrução vascular. Além disso, foi realizada, para melhor discussão dos casos, revisão bibliográfica. Conclusão: A secção e ligadura da veia renal esquerda mostraram-se seguras nos dois pacientes tratados com ressecção de tumores retroperitoneais, não sendo a sua reconstrução obrigatória quando preservadas as colaterais para drenagem venosa.

Introduction: The retroperitoneum can present several pathologies that have a certain complexity and a poor prognosis due to their proximity to large vessels, the involvement of which complicates surgical approaches. Surgery is the therapeutic option associated with the longest survival. The objective of this study was to demonstrate that renal function can be preserved after ligation or resection of the left renal vein, without vascular reconstruction, if the collateral circulation remains functional. Case reports: We report two cases of patients with retroperitoneal tumors with local vascular invasion, the complete resection of which required division and ligation of the left renal vein. Postoperatively, both patients showed preserved renal function, because of collateral venous drainage, and vascular reconstruction was not necessary in either case. We also present a review of the literature on such cases. Conclusion: Division and ligation of the left renal vein proved to be safe in both of the patients undergoing retroperitoneal tumor resection, and reconstruction of the vein is not mandatory when the collateral circulation is preserved.

Introducción: El retroperitoneo puede presentar diversas patologías y posee cierta complejidad y mal pronóstico debido al íntimo contacto e implicación de grandes vasos en el abordaje quirúrgico. La cirugía es la opción terapéutica asociada a la supervivencia prolongada. El estudio tiene por objetivo demostrar que es posible la preservación de la función renal, por las colaterales, después de la ligadura o resección de la vena renal izquierda sin reconstrucción vascular. Relato de los casos: Dos casos de pacientes con tumores retroperitoneales con invasión vascular local donde, para su completa resección, se hizo necesaria la sección y ligadura de la vena renal izquierda. Evolucionaron con preservación de la función renal debido al drenaje venoso por las colaterales, no siendo necesaria la reconstrucción vascular. Además, se realizó, para una mejor discusión de los casos, revisión bibliográfica. Conclusión: La sección y ligadura de la vena renal izquierda se mostró segura en los dos pacientes tratados con resección de tumores retroperitoneales, no siendo su reconstrucción obligatoria cuando se preservaron las colaterales para drenaje venoso

Tríada de Carney. Una rara asociación de tumores infrecuentes: caso clínico / Carney triad: report of one case

Carney described a disorder characterized by the presence of several uncommon tumors which were pulmonary chondromas, gastric sarcomas and extra-adrenal paragangliomas. We report a 14 year-old girl in whom multiple gastric tumors were discovered during a study of an iron deficiency anemia and was subjected to a partial gastrectomy. At 25 years of age, she developed several pulmonary chondromas and at 33 years, a mediastinal tumor with features of an extra-adrenal paraganglioma was found. At 35 years of age, a total gastrectomy was performed to remove a gastrointestinal stromal tumor with excision of peritoneal and lymph node metastasis. One year later, the patient died due to liver failure secondary to liver metastases.

Periorbital leiomyosarcoma treated by means of conventional surgery and frontal myocutaneous flap

Abstract Cutaneous leiomyosarcoma is a rare neoplasia, and its periorbital presentation is rather uncommon. We present a case of a male patient who was surgically treated, with the reconstruction performed with a frontal myocutaneous flap. The patient has been followed up for one year with adequate oncologic control and good aesthetic outcome.

High-grade Primary Renal Leiomyosarcoma

Objective: To investigate the clinical characteristics, prognosis, survival and diagnosis of high-grade primary renal leiomyosarcoma. Materials and Methods: From January 2003 to April 2013, 10 cases of high-grade primary renal leiomyosarcoma were retrospectively reviewed. We analyzed clinical manifestations, treatment and prognosis of our group and correlated to the literature. Results: Ten cases (five male and five female patients; age range 43­77 years, mean=57±std d:12.3 ) were enrolled. The mean diameter of the tumor masses was 9.35±4.5 cm (range 3-18 cm). 40% of the patients were asymptomatic while the major symptom of 60% patients was lumbar pain. Nephrectomy was performed in 90% of patients. Partial nephrectomy surgery was preferred for only one patient. Pleomorphism and necrosis with high-grade, pink spindle cell cytoplasm were viewed in all patients. All patients were high-grade, pink spindle cell cytoplasm and pleomorfism and necrosis were observed in all. In an immunohistochemical examination, vimentin was seen in 100%, desmin in 90% and smooth muscle actin in 80% of the patients. CD117 was negative in all patients. All of the cases were followed-up, and the time of survival varied from 6 to 68 months (mean 23.9±std d:20.1). No patient received adjuvant CTx and/or RTx. Conclusion: High-grade primary renal leiomyosarcomas (LMSs) are rare and highly malignant and the prognosis is poor. Early diagnosis and radical nephrectomy can prolong the patient's life. Surgery is the main treatment modality for renal (leiomyosarcoma) LMS

Leiomiosarcoma: un raro tumor renal / Leiomyosarcoma: a rare renal tumor

Introducción: El leiomiosarcoma renal es un tumor raro que constituye el 0,12 por ciento de todas las neoplasias renales malignas; su presentación clínica es muy variable. Caso clínico: Se reporta el caso de un hombre de 68 años de edad con lumbalgia derecha, en una tomografía contrastada de abdomen y pelvis se detecta una gran masa tumoral dependiente del polo superior renal derecho. Se realizó una nefrectomía radical y la resección incidental de una lesión pulmonar basal derecha, el diagnóstico histopatológico y de inmuno-histoquímica fue de un leiomiosarcoma renal maligno de alto grado metastásico a pulmón.

Introduction: Renal leiomyosarcoma is a rare tumor constituting 0.12% of all malignant renal neoplasms; the clinical presentation is highly variable. Clinical case: We report the case of a 68 year old man who presented with right back pain; CT scan (computed tomography) showed a large mass located at upper pole of the right kidney. We performed a radical nephrectomy and resection of a right basal lung node incidentally found, the histopathological and immunohistochemical diagnosis was a renal high-grade leiomyosarcoma with metastases to the lung.

Leiomiossarcoma cutâneo no tronco / Cutaneous leiomyosarcoma on the trunk

O Leiomiossarcoma cutâneo é um tumor maligno que representa 7 por cento dos sarcomas dos tecidos moles, afetando, mais frequentemente, homens entre os 50-70 anos. A localização, no tronco, é atípica, constituindo 10 a 15 por cento dos casos. A radioterapia e o traumatismo prévio têm sido referidos como fatores de risco. Descrevemos um homem de 57 anos, com tumor eritemato-violáceo, indolor, de consistência dura, localizado na região pré-esternal, com cerca de oito meses de evolução. A histopatologia evidenciou tumor maligno de células fusiformes, com núcleos em forma de "charuto", pleomórficos, com alto índice mitótico, ocupando toda a espessura da derme. Estas células expressaram actina do músculo liso, desmina e vimentina e foram negativas para proteína S-100 e pancitoqueratina. Foi efetuada a exérese cirúrgica radical do tumor.

Cutaneous leiomyosarcoma is a relatively uncommon tumor that accounts for 7 percent of all soft tissue sarcomas. It occurs more frequently in males between 50-70 years and only 10-15 percent of cases are located on the trunk. Radiotherapy and previous trauma have been implied as risk factors. We report the case of a 57 year-old male with an eight-month history of a hard painless erythematous-violaceous tumor on the presternal region. Histopathology evidenced a malignant spindle cell tumor, "cigar" shaped, with pleomorphic nuclei and a high mitotic index that occupied the entire dermal thickness. Immunohistochemical staining of the tumor cells was positive for smooth muscle actin, desmin and vimentin and negative for S-100 protein and pan-cytokeratin, which supported the diagnosis of dermal leiomyosarcoma. Radical surgery was performed to remove the tumor.

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.

Leiomiosarcoma del estroma intestinal: presentación de un caso / Intestinal stromal Leiomyosarcoma: a case presentation

Se presenta un paciente de 51 años de edad, alcohólico, con molestias en flanco y fosa ilíaca derechos donde se nota un aumento de volumen. Al examen físico se constata una tumoración abdominal gigante que ocupa todo el flanco y fosa ilíaca derechos y se proyecta a mesogastrio e hipogastrio. Se realizan estudios imagenológicos, incluyendo Tomografía Axial Computarizada contrastada de abdomen, y se somete a laparotomía exploradora con diagnóstico preoperatorio de linfoma abdominal, para realizar exéresis tumoral y estudio histológico, concluyéndose como un tumor del estroma gastrointestinal, específicamente un leiomiosarcoma de mesenterio. Se pone tratamiento con quimioterapia y la evolución clínica es favorable.

A 51 years, alcoholic patient is presented, with right flank and iliac fossa annoyances where a volume increase is noticed. To the physical examination of the area it has been confirmed a giant abdominal tumor, and it is projected to mesogastrio and hypogastrium. Imagenologic studies are realized, including Computerized Axial Tomography of abdomen, and it is underwent exploratory laparotomy with preoperative abdominal linfoma diagnosis, to realize tumoral exeresis and histological study, concluding as a gastrointestinal stromal tumor, specifically a mesentery leiomyosarcoma. It was treated with chemotherapy and the clinical evolution is favorable.

Leiomiossarcoma primário da glândula tireoide / Thyroid gland primary leiomyosarcoma

Aproximadamente 15 por cento a 20 por cento dos sarcomas ocorrem na região da cabeça e pescoço, 80 por cento em adultos, sendo apenas 0,014 por cento leiomiossarcomas primários de tireoide. Existem apenas 16 casos relatados no mundo, dos quais não há nenhum em nosso meio. São tumores com diagnóstico citológico pré-operatório difícil e podem ser confundidos com outras lesões mais comuns da tireoide, como carcinomas anaplásicos e medulares. O tratamento ideal ainda não está bem definido, visto que o prognóstico é ruim e a cirurgia radical associada à quimioterapia e à radioterapia adjuvantes não demonstra melhora nas taxas de recorrência e sobrevida. Relatou-se um caso de leiomiossarcoma primário da glândula tireoide em um paciente jovem, submetido a tireoidectomia total e esvaziamento cervical, associado à radioterapia adjuvante e realizou-se uma extensa revisão da literatura existente sobre o tema. Houve boa evolução pós-operatória, sem sinais de recidiva após quatro anos de seguimento.

Despite the fact that 15 percent to 20 percent of sarcomas occur in the head and neck and 80 percent in adults, only 0.014 percent are primary thyroid leiomyosarcomas. To the best of our knowledge, only 16 cases have been reported around the world, none in South America. Cytologic diagnosis is challenging and these tumors may be mistaken by more common ones such as anaplastic or medullary carcinomas. The treatment of choice for thyroid leiomyosarcomas is not well established yet because of its poor prognosis. Radical surgery associated with chemoradiotherapy has not been effective and did not improve survival rates. The authors report a case of primary thyroid leiomyosarcoma in a young male, who has been submitted to total thyroidectomy and selective neck dissection. Extensive literature review was performed by the authors. The patient received adjuvant radiotherapy, presenting good postoperative course. After four years evolution, there was no local recurrence or distant metastasis.

Leiomyosarcoma of the kidney / El Leiomiosarcoma del riñón

The case of a 42- year old woman with leiomyosarcoma of the kidney, a very rare renal lesion, is presented. Leiomyosarcomas are the most common of the primary renal sarcomas which account for less than 1% of renal tumours in adults.

Se presenta el caso de una mujer de 43 años de edad con un leiomiosarcoma del riñón - una lesión renal muy rara. Los leiomiosarcomas son los más comunes de los sarcomas renales primarios, y representan menos del 1% de los tumores renales en adultos.

Retroperitoneal leiomyosarcoma of the inferior vena cava: a case report

Leiomyosarcoma of the inferior vena cava [IVC] is a rare sarcoma, but it is the most common primary malignancy of the IVC. It has an extremely gloomy prognosis. We describe a 40 year-old white female complaining of abdominal fullness for 7 weeks before she sought medical assistance. Initial work-up including sonography and computed tomography showed a huge tumoral mass in the abdominal cavity seemingly originating from the IVC with displacement of the right ureter and hydronephrosis. The patient underwent surgical resection of the tumor. Pathologic diagnosis was leiomyosarcoma. Postoperatively, she was placed on coumadin and adjuvant chemotherapy was started. Considering the aggressiveness of this tumor, early radical enblock resection with clear margins is still the only chance for long-term survival

Leiomiosarcoma renal: 2 casos clínicos / Renal leiomyosarcoma

El leiomiosarcoma de origen renal es una patología rara y agresiva, más frecuente en mujeres y con gran tendencia a la recurrencia local y a distancia. Describimos dos casos clínicos de leiomiosarcoma renal, el primero correspondiente a una mujer de 46 años con gran lesión tumoral retroperitoneal derecha que fue sometida a nefrectomía radical y recibió tratamiento complementario con radioterapiaal lecho renal. El segundo caso corresponde a un hombre de 71 años con hallazgo de lesión tumoral renal izquierda cuyo tratamiento consistió en nefrectomía radical. El informe anatomopatológico fue compatible con leiomiosarcoma renal con alto grado de malignidad en ambos casos. El tratamiento de elección es la nefrectomía radical, con tratamiento adyuvante con quimioterapia y/o radioterapia. Se realiza un análisis de la literatura.

Renal leiomyosarcoma is a rare and aggressive disease, with a higher incidence on women and atendency for local and distant recurrence. We report two cases. A 46 year old female with aretroperitoneal lesion that underwent radical nephrectomy and complementary radiotherapy of renal fossa. A 71 year old man with a left renal tumor that underwent radical nephrectomy. Pathology informed a high grade leiomyosarcoma in both cases. Radical nephrectomy is the treatment of choice along with adjuvant chemo and radiotherapy Literature is reviewed.

Leiomiossarcoma primário de adrenal / Primary adrenal leiomyosarcoma

O leiomiossarcoma da glândula adrenal é uma neoplasia extremamente rara, cuja origem provém da veia central da adrenal ou de suas tributárias. Estes tumores são vistos com incidência aumentada em pacientes infectados pelo vírus da imunodeficiência humana adquirida (HIV) e também pelo vírus Epstein-Barr (EBV). Relata-se o caso de uma paciente de 48 anos diagnosticada, por meio de exames de imagem, massa ocupando loja adrenal esquerda, próxima ao pâncreas. Foi submetida à ressecção cirúrgica com achados histopatológicos e imunohistoquímico compatíveis com leiomiossarcoma primário de adrenal. A paciente foi a óbito após 53 meses da adrenalectomia. Concluiu-se que, não obstante a raridade desta neoplasia, sabe-se que, bem como os demais sarcomas de partes moles, o leiomiossarcoma de adrenal se comporta de maneira agressiva e tem na adrenalectomia o tratamento de escolha, não sendo necessário o emprego de quimio ou radioterapias de rotina, exceto em doença metastática ou irressecável.

Leiomyosarcoma of adrenal gland is an extremely rare tumor originating from the central adrenal vein or its tributaries. Patients with human immunodeficiency virus (HIV) and Epstein-Barr infection have a higher incidence of these tumors, but even in those, the overall incidence is very low. In this paper we report a case of a 48 year-old woman whose diagnosis was made by image exams that had suggested a mass in adrenal area. This patient was submitted to surgery for resection of the mass. The histopathologic and immunohistochemistry exams were compatible with primary leiomyosarcoma of adrenal gland. Fifty-three months after adrenalectomy the patient died with recurrent disease. Despite of the rarity of this tumor, leiomyosarcoma of adrenal gland has an aggressive behavior, such as the others soft tissue sarcomas. The adrenalectomy is the primary treatment, while chemotherapy or radiotherapy is not prescribed except in metastatic or bulky and not operative disease.