RESUMO
El condrosarcoma forma parte de los tumores primarios malignos más frecuentes. Las localizaciones pélvicas y de raíz de muslo requerirán abordajes amplios con la consecuente dificultad de cobertura de partes blandas. La hemipelvectomía externa es habitualmente el procedimiento de elección para estas localizaciones. Implica la resección de la extremidad inferior en bloque asociada a la hemipelvis ipsilateral, dando como resultado un gran defecto de cobertura. En algunas circunstancias, la extensa resección de partes blandas hace imposible la utilización de colgajos rotacionales locales. La reconstrucción con colgajo tipo "Fillet flaps" ofrece a estos defectos de partes blandas la opción de lograr una adecuada cobertura. El objetivo del trabajo es mostrar la resolución de un caso poco convencional de condrosarcoma de fémur proximal y su reconstrucción de partes blandas con un método nunca antes utilizado en nuestro medio.
Chondrosarcoma is one of the most frequent malignant primary tumors. Pelvic and proximal femur locations require extensive approaches with the consequent difficulty of soft tissue reconstruction. External hemipelvectomy is usually the procedure for these locations. It involves total en bloc resection of the lower extremity associated with ipsilateral hemipelvis, resulting in a large coverage defect. In some cases, local rotational flaps are impossible. Reconstruction with fillet flaps offers an adequate coverage for soft tissue defects. The objective of this study is to show the resolution of an unconventional case of chondrosarcoma of the proximal femur and its soft tissue reconstruction with a method never before used in our country.
O condrossarcoma é um dos tumores primários malignos mais frequentes. As localizações das raízes pélvicas e da coxa exigirão amplas abordagens com a conseqüente dificuldade em cobrir os tecidos moles. A hemipelvectomia externa costuma ser o procedimento de escolha para esses locais. Envolve a ressecção do membro inferior em bloco associado à hemipelve ipsilateral, resultando em um grande defeito de cobertura. Em algumas circunstâncias, a ressecção extensa de partes moles impossibilita o uso de retalhos rotacionais locais. A reconstrução com retalhos de filé oferece a esses defeitos de tecidos moles a opção de obter uma cobertura adequada. O objetivo deste trabalho é mostrar a resolução de um caso não convencional de condrossarcoma do fêmur proximal e sua reconstrução de partes moles com método nunca antes utilizado em nosso meio.
Assuntos
Humanos , Masculino , Adulto , Retalhos Cirúrgicos/transplante , Condrossarcoma/cirurgia , Procedimentos de Cirurgia Plástica/métodos , Neoplasias Femorais/cirurgia , Hemipelvectomia/métodos , Condrossarcoma/diagnóstico por imagem , Neoplasias Femorais/diagnóstico por imagemRESUMO
Soft-tissue sarcomas constitute an uncommon and heterogeneous group of tumors of mesenchymal origin. Diagnosis, treatment, and management should be performed by an expert multidisciplinary team. MRI/CT of the primary tumor and biopsy is mandatory before any treatment. Wide surgical resection with tumor-free tissue margin is the mainstay for localized disease. Radiotherapy is indicated in large, deep, high-grade tumors, or after marginal resection not suitable for re-excision. Perioperative chemotherapy should be discussed for high-risk sarcomas of the extremities and trunk-wall. In the case of oligometastatic disease, patients should be considered for local therapies. First-line treatment with anthracyclines (or in combination with ifosfamide) is the treatment of choice. Other drugs have shown activity in second-line therapy and in specific histological subtypes but options are limited and thus, a clinical trial should always be discussed.
Assuntos
Sarcoma/diagnóstico , Sarcoma/terapia , Neoplasias de Tecidos Moles/diagnóstico , Neoplasias de Tecidos Moles/terapia , Antraciclinas/uso terapêutico , Antineoplásicos/uso terapêutico , Lista de Checagem , Quimioterapia Adjuvante/métodos , Dermatofibrossarcoma/terapia , Feminino , Fibromatose Agressiva/genética , Fibromatose Agressiva/terapia , Humanos , Imageamento por Ressonância Magnética , Masculino , Oncologia , Terapia Neoadjuvante/métodos , Radioterapia/métodos , Neoplasias Retroperitoneais/terapia , Sarcoma/diagnóstico por imagem , Sarcoma/patologia , Sociedades Médicas , Neoplasias de Tecidos Moles/diagnóstico por imagem , Neoplasias de Tecidos Moles/patologia , Tumores Fibrosos Solitários/tratamento farmacológico , Espanha , Tomografia Computadorizada por Raios X , Neoplasias Uterinas/terapiaRESUMO
OBJECTIVE. We undertook this study to determine the radiologic features of desmo-plastic fibroblastoma. MATERIALS AND METHODS. We reviewed available radiologic images for 29 pathologically confirmed desmoplastic fibroblastomas, including images from MRI, radiography, ultrasound (US), and CT. RESULTS. The patient population included 14 women and 15 men (mean age, 60 years; range, 23-96 years). Typically, lesions were oval or lobulated and relatively small (mean, 5.6 cm). In 14 of the 22 cases that included patient histories, lesions had grown slowly, with two eventually causing pain. The remaining eight were discovered incidentally. All lesions involved or were below the deep fascia. Lesions were well-defined and associated with muscle (45%), deep fascia (28%), joint (21%), or tendon (7%). MR images were available in 26 cases; 14 included unenhanced and contrast-enhanced studies. On MRI imaging all lesions were well-defined and adjacent to dense connective tissue. On T1-weighted images, lesions showed varying amounts of low and intermediate signal intensity similar to that of tendon and skeletal muscle, respectively. On fluid-sensitive images, lesions were more heterogeneous, generally showing a wider spectrum of decreased to intermediate signal intensity. On contrast-enhanced MR images, enhancement was characteristically peripheral and septal with patchy areas of homogeneity. In the 10 cases with radiographs, images showed negative findings or a nonmineralized mass. The 10 available ultrasound studies showed mixed echogenicity. In eight patients, unenhanced CT showed lesions having attenuation similar to that of skeletal muscle. CONCLUSION. Desmoplastic fibroblastoma is an uncommon neoplasm with a relatively characteristic MRI appearance.
Assuntos
Fibroma Desmoplásico/diagnóstico por imagem , Imageamento por Ressonância Magnética/métodos , Neoplasias de Tecidos Moles/diagnóstico por imagem , Adulto , Idoso , Idoso de 80 Anos ou mais , Meios de Contraste , Diagnóstico Diferencial , Feminino , Fibroma Desmoplásico/patologia , Humanos , Masculino , Pessoa de Meia-Idade , Neoplasias de Tecidos Moles/patologia , Tomografia Computadorizada por Raios XAssuntos
Angiomioma , Doenças da Unha , Doenças Raras , Neoplasias Cutâneas , Idoso , Angiomioma/metabolismo , Angiomioma/patologia , Humanos , Masculino , Doenças da Unha/metabolismo , Doenças da Unha/patologia , Doenças Raras/metabolismo , Doenças Raras/patologia , Neoplasias Cutâneas/metabolismo , Neoplasias Cutâneas/patologiaRESUMO
ABSTRACT The glomus tumor is a rare benign soft tissue tumor. We report a case of a 45-year-old male patient who presented with a painful mass in the abdominal wall. The patient underwent total tumor resection and the anatomical pathology diagnosis was of glomus tumor. The glomus tumor presents differential diagnosis of carcinoid tumor, hemangiopericytoma and vascular leiomyoma.
RESUMEN El tumor glómico es un tumor raro y benigno de tejidos blandos. Se presenta el caso de un paciente de 45 anos, con el hallazgo de un nódulo doloroso en la pared abdominal. Se realizó una resección completa de la lesión y el diagnóstico por anatomia patológica fue de tumor glómico. El diagnóstico diferencial del tumor glómico se plantea con tumor carcinoide, hemangiopericitoma y leiomioma vascular.
RESUMO O tumor glômico é um tumor raro e benigno de tecidos moles. Relatamos o caso de um paciente do sexo masculino, 45 anos, que apresentava nódulo doloroso na parede abdominal. Foi realizada a ressecção total do tumor; o diagnóstico anatomopatológico foi de tumor glômico. Este apresenta diagnóstico diferencial, com tumor carcinoide, hemangiopericitoma e leiomioma vascular.
RESUMO
Soft-tissue sarcomas are uncommon and heterogeneous tumors of mesenchymal origin. A soft-tissue mass that is increasing in size, greater than 5 cm, or located under deep fascia are criteria for suspicion of sarcoma. Diagnosis, treatment, and management should preferably be performed by a multidisciplinary team in reference centers. MRI and lung CT scan are mandatory for local and distant assessment. A biopsy indicating histological type and grade is needed previous to the treatment. Wide surgical resection with tumor-free tissue margin is the primary treatment for localized disease. Radiotherapy is indicated in large, deep, high-grade tumors, or after marginal resection not likely of being improved with reexcision. Neoadjuvant and adjuvant chemotherapy improve survival in selected cases, usually in high-grade sarcomas of the extremities. In the case of metastatic disease, patients with exclusive lung metastasis could be considered for surgery. First-line treatment with anthracyclines (or in combination with ifosfamide) is the treatment of choice. New drugs have shown activity in second-line therapy and in specific histological subtypes.
Assuntos
Guias de Prática Clínica como Assunto , Sarcoma/diagnóstico , Sarcoma/terapia , Neoplasias de Tecidos Moles/diagnóstico , Neoplasias de Tecidos Moles/terapia , Humanos , Gradação de Tumores , Metástase Neoplásica , EspanhaRESUMO
Los lipomas son los tumores mesenquimatosos benignos más comunes. Están compuestos de células adiposas maduras, generalmente asintomáticos y de crecimiento lento. Se identifican con mayor frecuencia en individuos obesos y del sexo femenino. La localización en la vulva es rara. Presentamos un caso clínico atendido en el Centro de Diagnóstico Integral “Dr. Alan Delfín¨, Estado Trujillo, Venezuela. La paciente de 19 años acudió con un tumor de partes blandas lobulado, pediculado, no doloroso en región vulvar, de 23 cm de longitud. Se realizó tratamiento quirúrgico con exéresis y biopsia. El diagnóstico de lipoma fue confirmado por examen anatomopatológico.
Lipomas are the most common benign mesenchymal tumor. They are componed of mature fat cells, usually are asymptomatic and slow-growing. They are more frequently identified in obese and female individuals. Vulva localizations are rare. A clinical case treated at ¨Dr. Alan Delfín¨ Integral Diagnosis Center, Trujillo State, Venezuela. The patient came to consultation presenting a pedunculated, lobulated, painless soft tissue tumor, in vulvar region, 23 cm long. The patient underwent surgical treatment; exeresis and biopsy of lesion were performed. The anatomopathological exam confirmed the diagnosis of lipoma.
RESUMO
El mixofibrosarcoma es una neoplasia maligna infrecuente, que puede originarse en los tejidos blandos, se lo ha estadificado como de alto o bajo grado y la localización más frecuente son los miembros inferiores. El reconocimiento clínico del mismo es dificultoso, en primer lugar porque la clínica de otros sarcomas de partes blandas es similar y además semejan lipomas o aún quistes. La extirpación quirúrgica amplia es el tratamiento de elección, por la propensión de este sarcoma a la recidiva local. Los catalogados como de alto grado en un 30 % de los casos, pueden originar metástasis a distancia, especialmente a hueso, pulmón y ganglios linfáticos. La histopatología asegura el diagnóstico en la mayoría de los casos. Comunicamos el caso de un mixofibrosarcoma de bajo grado, en un hombre de 43 años, que a los tres años de control evolutivo, luego de la resección quirúrgica, no presenta recaída local ni distante.
Myxofibrosarcoma is an uncommon soft tissue sarcoma that is grading as low or high malignancy. The principal sites of involvement are the lower limbs. Clinical recognition is difficult since it resembles a cyst, a lipoma, other soft tissue sarcoma or even benign conditions as panniculitis. The wide surgical excision is the main therapeutic approach because local recurrence is frequent. The high grade variant is associated in 30 % of the cases with distant metastasis, especially to the lung, bone and lymph nodes. The histopathological features allow an accurate diagnosis in most cases. A 43 year-old man with a myxofibrosarcoma of the chest wall is reported with a follow-up of two years without recurrence.
RESUMO
Os tumores de partes moles da mão, principalmente os benignos como os cistos sinoviais, são queixas comuns, principalmente em consultas a cirurgiões da mão. Este trabalho tem por objetivo revisar os principais tipos de tumores de partes moles da mão, desde a sua apresentação clínica até uma revisão objetiva sobre seus diagnósticos e melhores opções terapêuticas. Visa auxiliar, também, na decisão de referência do paciente com uma lesão nodular ou cística da mão a um médico especialista após uma consulta ao médico generalista. As particularidades anatômicas e funcionais da mão tornam o estudo e o conhecimento das suas patologias fundamentais para um adequado manejo dos pacientes.
The soft tissue tumors of the hand, especially the benign ones such as synovial cysts, are common complaints, particularly in hand surgeons consultations. This work aims to review the main types of soft tissue tumors of the hand, from their clinical presentation to an objective review of their diagnoses and the best therapeutic options. It also aims to help in the decision to refer the patient with a nodular or cystic lesion of the hand to a medical specialist after consultation with the general practitioner. The anatomical and functional particularities of the hand make the study and knowledge of its conditions crucial for proper management of patients.
Assuntos
Humanos , Mãos , Neoplasias de Tecidos MolesRESUMO
O lipoblastoma é um tumor mesenquimal raro, composto de lipoblastos que continuam sua proliferação após o período pós-natal e que acometem, predominantemente, a população pediátrica. Apresenta prognóstico excelente, apesar do potencial de invasão local e do crescimento rápido. Os autores relatam o caso de uma paciente pediátrica portadora de volumosa lesão em antebraço direito, ocasionando importante comprometimento funcional do membro acometido. Aspectos relevantes no diagnóstico diferencial e manejo são discutidos neste trabalho, visto tratar-se de lesão com potencial risco incapacitante futuro, caso não manejada corretamente.
Lipoblastoma is a rare mesenchymal tumor occurring primarily in pediatric patients and formed by lipoblasts that proliferate after the postnatal period. Despite its potential for local invasion and rapid growth, its prognosis is excellent. In this study, we report the case of a pediatric patient with a ponderous lesion in the right forearm that caused considerable functional impairment of the affected limb. We also discuss the relevant aspects concerning the differential diagnosis and management of the disease, as it has the potential to cause incapacity without proper treatment.
Assuntos
Humanos , Feminino , Lactente , História do Século XXI , Neoplasias de Tecidos Moles , Cirurgia Plástica , Ferimentos e Lesões , Literatura de Revisão como Assunto , Tecido Adiposo , Adipócitos , Estudo de Avaliação , Lipoma , Neoplasias de Tecidos Moles/cirurgia , Neoplasias de Tecidos Moles/patologia , Cirurgia Plástica/métodos , Ferimentos e Lesões/cirurgia , Tecido Adiposo/embriologia , Tecido Adiposo/metabolismo , Adipócitos/fisiologia , Adipócitos/metabolismo , Lipoblastoma , Lipoblastoma/cirurgia , Lipoblastoma/patologia , Lipoma/cirurgia , Lipoma/patologiaRESUMO
Introducción: El hamartoma fibroso de la infancia es una proliferación benigna de los tejidos blandos, de presentación infrecuente; 91 % de los casos ocurre durante el primer año de edad y afecta más frecuentemente al sexo masculino. Se caracteriza por ser una lesión subcutánea de morfología característica con patrón de crecimiento organoide trifásico. Caso clínico: Lactante masculino de seis meses de edad, con tumor en la región plantar medial del pie izquierdo, el cual fue resecado completamente. En el estudio histopatológico se informó como hamartoma fibroso de la infancia. Conclusiones: Puede localizarse en cualquier sitio anatómico, aunque 5 a 10 % afecta las extremidades inferiores existen pocos casos informados en el pie. En biopsias pequeñas es importante su diagnóstico diferencial con otras lesiones fibroadiposas para el adecuado tratamiento. La resección quirúrgica amplia con márgenes libres de lesión confiere un buen pronóstico a los pacientes.
BACKGROUND: Fibrous hamartoma of infancy (FHI) is an infrequent benign proliferation of the soft tissues. Ninety one percent of cases occur during the first year of life. FHI is characterized as a subcutaneous lesion with characteristic morphology with a triphasic organoid growth pattern. CLINICAL CASE: We present the case of a 6-month-old male infant with a tumor in the medial plantar region of the left foot, which was completely withered. Histopathological study reported a fibrous hamartoma of infancy. CONCLUSIONS: Fibrous hamartoma of infancy most frequently affects males. It may be localized at any anatomic site, although 5-10% of cases affect the lower limbs. Few cases are reported in the foot. This is a lesion with a characteristic morphological pattern; however, in small biopsies, its differential diagnosis is important with other fibroadipose lesions for appropriate treatment. Ample surgical resection with lesion-free borders confers a good prognosis for these patients.