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

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

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

Leiomiossarcoma paratesticular: relato de caso e revisão de literatura / Paratesticular leiomysarcoma: a case of report and review of the literature

Introdução: O leiomiossarcoma é um tumor maligno de células mesenquimais. Este estudo tem como objetivo apresentar um caso clínico de sarcoma paratesticular subtipo leiomiossarcoma, tendo em vista sua escassez na literatura. Relato de caso: Paciente, 40 anos, sexo masculino, negro, hígido, iniciou quadro de dor em região de bolsa escrotal. Foi submetido à ultrassonografia que evidenciou tumor testicular à esquerda. Foi realizada abordagem cirúrgica. Considerações finais: O leiomiossarcoma é um tumor maligno com envolvimento de músculo liso. Devido à raridade do leiomiossarcoma paratesticular, não existe protocolo estabelecido na literatura. A importância da equipe multidisciplinar e especializada com acompanhamento longitudinal tem o intuito de instituir a terapia padrão-ouro e evitar recorrência.

Introduction: Leiomyosarcoma is a malignant tumor of mesenchymal cells. This study aims to present a clinical case of paratesticular sarcoma subtype leiomyosarcoma given its scarcity in the literature. Case report: The patient, 40 years old, male, black, healthy, started with pain in the scrotal region. He was submitted to ultrasonography which showed a testicular tumor on the left. A surgical approach was performed. Final consideration: Leiomyosarcoma is a malignant tumor with smooth muscle involvement. Due to the rarity of paratesticular leiomyosarcoma, there is no established protocol in the literature. The importance of a multidisciplinary and specialized team with longitudinal follow-up to establish the gold standard therapy and avoid recurrence.

Tumor Rabdomioblástico Inflamatório Pulmonar: Relato de Caso / Pulmonary Inflammatory Rhabdomyoblastic Tumor: Case Report / Tumor Rabdomioblástico Inflamatorio Pulmonar: Informe de Caso

Introdução: Os sarcomas primários do pulmão são tumores malignos raros com incidência estimada em torno de 0,5% de todas as neoplasias pulmonares. De suas tipificações descritas na literatura, duas especialmente, os leiomiossarcomas e os rabdomiossarcomas, apresentam semelhanças em seu perfil genético, morfológico e imuno-histoquímico, o que os levou a receberem uma mesma classificação: tumor rabdomioblástico inflamatório. Esse tipo de tumor costuma acometer tecidos moles em extremidades e tronco, e predomina em meio a homens jovens e de meia-idade. Em razão do pequeno número de casos de tumores rabdomioblásticos relatados na literatura, tanto seu diagnóstico quanto seu tratamento são pouco descritos. Relato de caso: Paciente, sexo feminino, 19 anos, apresentou três episódios de infecção respiratória no lobo inferior do pulmão direito em um período de 18 meses. A tomografia computadorizada do tórax evidenciou obstrução do brônquio intermediário e áreas de bronquiectasias no lobo inferior direito. Diante disso, realizou-se bilobectomia inferior-média, conduzida por cirurgia robótica. A paciente recebeu alta hospitalar três dias depois do pós-operatório. A imuno-histoquímica revelou tumor rabdomioblástico inflamatório de baixo grau. Conclusão: Este relato apresentou um caso de tumor pulmonar raro, abordado por uma técnica cirúrgica ainda não relatada para esse tipo de patologia.

Introduction: Primary lung sarcomas are rare malignant tumors with an estimated incidence of around 0.5% of all lung neoplasms. Of their typifications described in the literature, two especially, leiomyosarcomas and rhabdomyosarcomas, have similarities in their genetic, morphological and immunohistochemical profile, which led them to be classified with the same name: inflammatory rhabdomyoblastic tumor. This type of tumor usually affects soft tissues in the extremities and trunk, and predominates among young and middle-aged men. Due to the small number of cases of rhabdomyoblastic tumors reported in the literature, both their diagnosis and treatment are poorly described. Case report: Female patient, 19 years old, who had three episodes of respiratory infection in the lower lobe of the right lung in a period of 18 months. Computed tomography of the chest showed obstruction of the intermediate bronchus and areas of bronchiectasis in the right lower lobe. In view of this, a lower middle bilobectomy was performed through robotic surgery. The patient was discharged from hospital three days after the operation. Immunohistochemistry revealed low-grade inflammatory rhabdomyoblastic tumor. Conclusion: This report described a case of a rare lung tumor, submitted to a surgical technique not yet reported for this type of pathology.

Introducción: Los sarcomas pulmonares primarios son tumores malignos raros con una incidencia estimada en torno al 0,5% de todas las neoplasias pulmonares. De sus tipificaciones descritas en la literatura, dos en especial, los leiomiosarcomas y los rabdomiosarcomas presentan similitudes en su perfil genético, morfológico e inmunohistoquímico, lo que los llevó a recibir la misma clasificación: tumor rabdomioblástico inflamatorio. Este tipo de tumor suele afectar a los tejidos blandos de las extremidades y el tronco, y predomina en hombres jóvenes y de mediana edad. Debido al escaso número de casos de tumores rabdomioblásticos reportados en la literatura, tanto su diagnóstico como su tratamiento están pobremente descritos. Informe del caso: Paciente de sexo femenino, 19 años que consultó por tres episodios de infección respiratoria en el lóbulo inferior del pulmón derecho en un período de 18 meses. La tomografía computarizada de tórax mostró obstrucción del bronquio intermedio y áreas de bronquiectasias en el lóbulo inferior derecho. Ante esto, se realizó una bilobectomía media baja mediante cirugía robótica. La paciente recibió el alta hospitalaria tres días después de la operación. Se realizó inmunohistoquímica y se definió el diagnóstico de tumor rabdomioblástico inflamatorio de bajo grado. Conclusión: Este informe presenta un caso de tumor pulmonar raro, tratado mediante una técnica quirúrgica aún no reportada para este tipo de patología.

Leiomiosarcoma de vena cava inferior, reconstrucción mediante interposición de prótesis y anticoagulación / Leiomyosarcoma of the inferior vena cava, reconstruction with interposition of prosthesis and anticoagulation / Leiomiossarcoma da veia cava inferior, reconstrução por interposição de prótese e anticoagulação

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.

Robot-assisted laparoscopic enucleation in the treatment of leiomyosarcoma of urinary bladder: A case report / 中南大学学报(医学版)

Leiomyosarcoma of urinary bladder (LMS-UB) is a highly malignant mesenchymal tumor, accounting for less than 0.5% of all bladder malignancies, with a predominant clinical presentation of hematuria. Here we report a case of low-grade LMS-UB. A 44-year-old male patient was admitted to the hospital with urodynia for 2 weeks. The patient's pelvis CT showed a mass on the right part of the bladder. For this reason, he was initially diagnosed with bladder cancer. We performed a robot-assisted laparoscopic enucleation of the bladder tumor and low-grade LMS-UB was diagnosed with the histopathological examination. He underwent 5 cycles of adjuvant chemotherapy after surgery. At 19months postoperative follow-up, the patient had no symptoms, recurrence, or distant metastasis. There is no report on the treatment of LMS-UB with minimally invasive enucleation worldwide. This case provides a new comprehensive treatment method of enucleation combined with adjuvant chemotherapy for early low-grade LMS-UB to reduce complications and improve patients' quality of life after surgery.

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.

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.

Leiomiosarcoma de ovario: A propósito de un caso / Ovarian leiomyosarcoma: About a case

Introducción: El leiomiosarcoma de ovario, es una neoplasia extremadamente rara, forma parte del subgrupo de tumores de músculo liso que constituye menos del 1% en cuanto a tumores de ovario; dentro del mismo se encuentran clasificados el carcinosarcoma, angiosarcoma, fibrosarcoma y leiomiosarcoma, de acuerdo con la literatura revisada se han reportado 63 casos de leiomiosarcoma hasta la actualidad. Caso clínico: Presentamos el caso de una paciente mujer de 53 años; ingresó al área de emergencia por dolor abdominal de gran intensidad, acompañado de distensión abdominal y ortopnea; a la exploración física se palpó una masa poco móvil. La tomografía evidenció la presencia de una masa abdomino-pélvica que se extendió entre el asa intestinal y la raíz del mesenterio; comprimía vejiga, útero y ovarios. Se le realizó una histerectomía, con salpingo ooforectomía derecha, ooforectomía izquierda por medio de laparotomía exploratoria. Los estudios histopatológicos determinaron el diagnóstico de leiomiosarcoma. Fue tratado con cirugía y quimioterapia adyuvante.

Introduction: Ovarian leiomyosarcomas is an extremely rare neoplasm and are part of the subgroup of smooth muscle tumors that constitutes less than 1% in terms of ovarian tumors; within it are classified: carcinosarcoma, angiosarcoma, fibrosarcoma, and leiomyosarcoma, according to the reviewed literature, 63 cases of leiomyosarcoma have been reported to date. Clinical Case: We present the case of a 53-year-old female patient; she was admitted to the emergency area due to severe abdominal pain, accompanied by abdominal distension and orthopnea; Physical examination revealed a large, slightly mobile mass. The tomography showed the presence of an abdominopelvic mass that extended between the intestinal loop and the root of the mesentery; compressed bladder, uterus, and ovaries. She underwent exeresis hysterectomy with right salpingo-oophorectomy and left oophorectomy through exploratory laparotomy. Histopathological studies yielded results of a malignant neoplasm compatible with leiomyosarcoma. She was treated with surgery and adjuvant chemotherapy.

Metastasizing leiomyoma during pregnancy

Uterine leiomyoma is a benign tumor of myometrial tissue which affects women of reproductive age. Its prevalence increases with age and has a peak incidence at the age of forty. The term "metastasizing leiomyoma" refers to a tumor of dense connective tissue and smooth myometrial muscle cells located outside the uterus. This group of tumors can metastasize to different organs, the lung being its main focus. We present the case report of a 33-year-old female gravida 3, para 1, abortus 1, at 11 weeks of pregnancy, with pelvic masses. The diagnosis was metastasizing leiomyoma during pregnancy.

Primary ovarian leiomyosarcoma in a postmenopausal woman diagnosed using immunohistochemistry / Philippine Journal of Obstetrics and Gynecology

@#Primary ovarian leiomyosarcoma is a very rare tumor which is most commonly seen in postmenopausal women. Primary ovarian leiomyosarcoma has a very poor prognosis, with less than 20% of patients being alive at 5 years. The case is a 51‑year‑old female who presented with a lower abdominal mass secondary to an ovarian new growth. An exploratory laparotomy, peritoneal fluid cytology, unilateral salpingo‑oophorectomy with malignant frozen section of affected ovary, which revealed malignant tumor, proceeded with surgical staging total abdominal hysterectomy contralateral salpingo‑oophorectomy, then proceeded to complete surgical staging with infracolic omentectomy, Jackson‑Pratt drain insertion was performed. Microscopic and immunohistochemical findings established the diagnosis of primary ovarian leiomyosarcoma. Surgery is the cornerstone of treatment, while the role of chemotherapy and radiotherapy is still not clear because substantial data are lacking. The prognosis of primary pure ovarian leiomyosarcomas is extremely poor and there is no established treatment modality for this rare type of tumor.

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.

Cutaneous leiomyosarcoma: a 20-year retrospective study and review of the literature

Abstract Background: Cutaneous leiomyosarcoma is a rare malignant neoplasm with muscular origin, representing 2%-3% of all cutaneous soft tissue sarcomas. Objectives: The aim of this study was to characterize clinicopathological features of patients diagnosed with cutaneous leiomyosarcoma in our center over the last 20-years. Methods: A retrospective study of patients with a histopathological diagnosis of leiomyosarcoma between 1999 and 2018 was conducted. Results: Eleven patients were diagnosed with cutaneous leiomyosarcoma during this period. Most cases occurred in men (n = 7). Age at presentation ranged from 47 to 92 years (mean 64.9 years). Head and neck were the most frequently involved locations (n = 5). Ten leiomyosarcoma were dermal, with one cutaneous metastasis. Immunohistochemical staining was available for 7 patients, demonstrating positivity for smooth muscle actin in all of them. All neoplasms were treated surgically. Mean survival was 32.2-months. Study limitations: This was a retrospective study based on medical and pathological records. Conclusions: Histopathology is essential for the diagnosis of leiomyosarcoma, usually revealing a dermal or subcutaneous lesion composed of intertwined fascicles of smooth muscle fibers. Immunohistochemistry is then used to adequately differentiate leiomyosarcoma from other spindle cell tumors. When dealing with cutaneous leiomyosarcoma, it is advisable to carefully evaluate the depth of subcutaneous extension, since even minimal subcutaneous involvement may be associated with a poorer prognosis.

Leiomiosarcoma de la vena cava inferior. ¿Qué buscar en las imágenes? Revisión a propósito de un caso / Leiomyosarcoma of the inferior vena cava. What to look for in the images? Presentation of a case

El leiomiosarcoma de la vena cava inferior (VCI) es un tumor mesenquimal maligno, poco frecuente, de bajo crecimiento, que surge en las células de músculo liso de la túnica media de la pared vascular y que tiene en general un mal pronóstico. Las imágenes juegan un papel crucial en el abordaje diagnóstico y en el planeamiento quirúrgico, por lo que reconocer los hallazgos más frecuentes es necesario en la práctica radiológica. A continuación, se describe un caso que ilustra los aspectos más representativos de esta enfermedad en diferentes modalidades diagnósticas, incluyendo tomografía, resonancia magnética y PET-CT, con el objetivo de hacer un reconocimiento de las características que puedan llevar a un diagnóstico temprano y, por lo tanto, a favorecer la sobrevida de los pacientes.

Inferior vena cava leiomyosarcoma (IVC) is a low-growing, malignant mesenchymal tumor that arises in the smooth muscle cells of the tunica media of the vascular wall and has a generally poor prognosis. Images play a crucial role in the diagnostic approach and in surgical planning, therefore, recognizing the most frequent findings is necessary in radiological practice. We describe a case that outlines the most typical findings in different diagnostic modalities including tomography, magnetic resonance imaging and PET-CT, with the aim of recognizing the characteristics that can lead to an earlier diagnosis and therefore to favor patient survival.

Manifestaciones paraneoplásicas a propósito de un caso con sarcoma de partes blandas y metástasis ósea / Paraneoplastic manifestations regarding a case with soft tissue sarcoma and bone metastases

Introducción: El sarcoma de tejidos blandos (STB) constituye un grupo variado de más de 60 neoplasias que solo suponen 1 por ciento del total de los tumores. Objetivo: Describir el caso de un paciente con sarcoma de partes blandas con metástasis ósea y manifestaciones paraneoplásicas. Caso clínico: Se presenta el caso de un paciente masculino de 55 años de edad con antecedentes de diabetes mellitus tipo 2 e hipertensión arterial que refiere que hace 50 días inició con decaimiento marcado, luego de permanecer 1 mes con este síntoma inició con dolor en la columna en la parte más baja de la espalda, conjuntamente iniciando con un cuadro febril. Además, tuvo pérdida de la fuerza principalmente en las piernas, y hablaba cosas incoherentes sin sentido, gran inapetencia, con pérdida de peso de 50 lb en 20 días. Ante cuadro de pérdida de la conciencia y de la fuerza principalmente en las piernas se decidió su remisión hacia el Hospital Clínico Quirúrgico Lucía Íñiguez Landín de Holguín donde se concluyó como sarcoma de partes blandas con manifestaciones paraneoplásicas y metástasis ósea. Conclusiones: El caso presentado de sarcoma de partes blandas pretende aportar información sobre esta enfermedad tan infrecuente en nuestro medio(AU)

Introduction: Soft tissue sarcoma (STB) constitutes a varied group of more than 60 malignancies that only accounts for 1% of all tumors. Objective: To report a case of a patient with soft tissue sarcoma with bone metastases and paraneoplastic manifestations. Clinical case report: We report the case of a 55-year-old male patient with a history of type 2 diabetes mellitus and hypertension, who stated that 50 days ago he began to feel markedly listless. And after a month he began to suffer pain in the spine, in the lower back, and have a fever. In addition he had loss of strength mainly in the legs, and spoke inconsistently, nonsense, great loss of appetite, with weight loss of 50 lbs in 20 days. Given the condition of loss of consciousness and strength, mainly in the legs, it was decided to refer him to Lucía Iñiguez Landín Surgical Clinical Hospital in Holguín, where a soft tissue sarcoma with paraneoplastic manifestations and bone metastases was diagnosed. Conclusions: The report of this case of soft tissue sarcoma aims to provide information about this disease, which is so rare in our setting(AU)

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.

Primary nonlymphoid gastrointestinal neoplasms in dogs in Rio Grande do Sul / Neoplasmas gastrointestinais primários não linfoides em cães no Rio Grande do Sul

Gastrointestinal neoplasms (GIN) are uncommon in dogs, but they mainly show malignant behavior and poor prognosis. The types of GIN in dogs and their frequency, as well as their epidemiological and histopathological characteristics were analyzed through a retrospective study of biopsies from 24.711 dogs from 2005 to 2017. Additionally, histological sections of neoplasms were subjected to immunohistochemistry (IHC) using antibodies against pancytokeratin, vimentin, smooth muscle actin, c-Kit, S-100, CD31, CD79αcy, and neuron-specific enolase. Of the total samples from dogs analyzed, 88 corresponded to GIN. Neoplasms occurred more frequently in purebred dogs (64.8%, 57/88), males (53.4%, 47/88), with a median age of 10 years. The intestine was affected by 84.1% (74/88) of the cases. Of these, the large intestine was the most affected (67.6%, 50/74). Most of the neoplasms had malignant behavior (88.6%, 78/88). Regarding the classification of neoplasms, 46.6% (41/88) of the diagnoses corresponded to epithelial, 46.6% (41/88) were mesenchymal, 5.7% (5/88) were hematopoietic, and 1.1% (1/88) was neuroendocrine. The most frequently diagnosed neoplasms were papillary adenocarcinoma (19.3%, 17/88), leiomyosarcoma (17.0%, 15/88), gastrointestinal stromal tumors (GISTs) (12.5%, 11/88), and leiomyoma (5.0%, 8/88). Adenocarcinomas were located mainly in the rectum, whereas leiomyosarcomas and GISTs developed mainly in the cecum. Epithelial neoplasms showed a greater potential for lymphatic invasion whereas mesenchymal neoplasms appeared to be more expansive with intratumoral necrosis and hemorrhage. Immunohistochemistry was found to be an important diagnostic technique for the identification of infiltrating cells in carcinomas and an indispensable technique for the definitive diagnosis of sarcomas.(AU)

Neoplasmas gastrointestinais (NGI) são pouco comuns em cães, mas possuem principalmente comportamento maligno e prognóstico reservado. Os tipos de NGI em cães e sua frequência, bem como características epidemiológicas e histopatológicas foram analisados por meio de um estudo retrospectivo dos exames de biópsias de 24.711 cães entre os anos de 2005 a 2017. Adicionalmente, cortes histológicos de NGI foram submetidos à técnica de imuno-histoquímica (IHQ), utilizando os anticorpos anti-pancitoqueratina, vimentina, actina de músculo liso, c-Kit, S-100, CD31, CD79αcy e enolase neurônio específica. Do total de cães analisados, 88 corresponderam a NGI não linfoides. Os neoplasmas ocorreram com maior frequência em cães de raça pura (64,8%, 57/88), machos (53,4%, 47/88), com mediana de idade de 10 anos. O intestino foi acometido em 84,1% dos casos (74/88). Destes, o intestino grosso foi o segmento mais afetado (67,6%, 50/74). A maior parte dos neoplasmas tinha comportamento maligno (88,6%, 78/88). Quanto à classificação, 46,6% (41/88) dos diagnósticos corresponderam a neoplasmas epiteliais, 46,6% (41/88) mesenquimais, 5,7% (5/88) hematopoiéticos e 1,1% (1/88), neuroendócrino. Os neoplasmas mais frequentemente diagnosticados foram adenocarcinoma papilar (19,3%, 17/88), leiomiossarcoma (17,0%, 15/88), tumor estromal gastrointestinal (GIST) (12,5%, 11/88) e leiomioma (12,5%, 8/88). Adenocarcinomas localizavam-se principalmente no reto, enquanto leiomiossarcoma e GISTs desenvolveram-se principalmente no ceco. Os neoplasmas epiteliais demonstraram um potencial maior de invasão linfática enquanto que os mesenquimais aparentaram ser mais expansivos, com necrose e hemorragia intratumorais. A imuno-histoquímica mostrou ser uma técnica diagnóstica importante para a identificação de células neoplásicas infiltravas no caso dos carcinomas e uma técnica indispensável para o diagnóstico definitivo de sarcomas.(AU)

An atypical presentation of leiomyosarcoma causing extremity compartment syndrome of the crural region in a Dutch Warmblood mare: a case report

Duodenal Leiomyosarcoma Presenting with Gastrointestinal Bleeding and Obstruction: A Case Report / 대한소화기학회지

Duodenal leiomyosarcoma is a rare condition with a poor prognosis. Early diagnosis of duodenal leiomyosarcoma is challenging because it presents with nonspecific symptoms and endoscopic biopsies usually do not enable a definitive diagnosis. Duodenal leiomyosarcomas are diagnosed on the basis of the histopathological identification of a mesenchymal lesion composed of malignant tumor cells that on immunohistochemical examination is positive for smooth muscle actin and desmin. We report the case of a 38-year-old man who presented with gastrointestinal bleeding and obstruction who was diagnosed with duodenal leiomyosarcoma after surgical resection.

Neoplasias orofaciais em crianças e adolescentes / Orofacial neoplasms in children and adolescents

Objetivo:Este trabalho analisou os casos de neoplasias orofaciais em crianças e adolescentes, diagnosticadas pelo Laboratório de Patologia Oral da Universidade Federal de Pernambuco no período de março de 2000 a março de 2019. Métodos: Tratou-se de um estudo retrospectivo das neoplasias mais prevalentes diagnosticada no serviço. Os dados foram tabulados e analisados através do programa Statistical Package for the Social Sciences(SPSS) na versão 24.0. Resultados:Dos 6.766 casos diagnosticados, 101 foram de neoplasias, destes 52,5% foram no sexo feminino. A média de idade entre os pacientes foi de 12,6 anos. Em relação ao tipo de biópsia, a excisional foi mais prevalente e em 71,3% dos casos os diagnósticos histopatológicos confirmaram as hipóteses diagnósticas clínicas. A neoplasia benigna não odontogênica mais comum foi o papiloma oral com 30 casos (29,7%). Entre os tumores benignos de origem odontogênica, os odontomas foram os mais prevalentes com 16 casos (15,8%). Foram diagnosticados apenas dois tipos de neoplasias malignas: o carcinoma adenoide cístico e o leiomiossarcoma. Conclusão:Levantamentos epidemiológicos de lesões orofaciais são importantes para determinar a sua prevalência, além de contribuir com a determinação das características na população estudada, fornecendo ao cirurgião-dentista uma base sólida para o diagnóstico e manejo clínico das neoplasias nesses indivíduos.

Aim:To analyze the cases of orofacial neoplasms in children and adolescents, diagnosed by the Oral Pathology Laboratory of the Federal University of Pernambuco from March 2000 to March 2019. Methods: Treatment of a retrospective study of the most prevalent neoplasms diagnosed at the laboratory. Data were tabulated and analyzed using the Statistical Package for Social Sciences (SPSS), version 24.0. Results:Of the 6,766 cases diagnosed, 101 were neoplasms, 52.5% of these were not female. The average age among the patients was 12.6 years. Regarding the type of biopsy, tooth extractions were more prevalent, and in 71.3% of the cases, the histopathological diagnoses confirmed the clinical diagnostic hypotheses. The most common non-odontogenic benign neoplasm was the oral papilloma, found in 30 cases (29.7%). Among the benign tumors of odontogenic origin, the odontomas were the most prevalent in 16 cases (15.8%). Only two types of malignant neoplasms were diagnosed: adenoid cystic carcinoma and leiomyosarcoma. Conclusion:Epidemiological surveys of orofacial lesions are important to determine their prevalence and aid in defining the characteristics in the studied population, providing the dentist with a continuous basis for the diagnosis and clinical treatment of neoplasms in these individuals.