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Se presenta a un paciente con liposarcoma mediastinal gigante con dolor torácico, disnea, cuyos estudios por imágenes revelaban la presencia de una gran tumoración de 42 cm en su diámetro mayor que abarcaba todo el mediastino, comprometía ambas cavidades torácicas, rechazaba los pulmones, corazón y grandes vasos. La biopsia con aguja cortante bajo guía ecográfica fue informada como liposarcoma. El paciente tuvo resección completa del tumor mediante la incisión Clamshell. En el post operatorio inmediato, presentó shock circulatorio más disfunción multiorgánica (DOMS): plaquetopenia, insuficiencia renal aguda con necesidad de soporte dialítico, injuria hepática. El soporte y monitoreo especializado en la Unidad de Cuidados Intensivos (UCI) permitió mejoría clínica y buena evolución. Salió de alta en buenas condiciones.

We present a patient with giant mediastinal liposarcoma with chest pain, dyspnea, whose imaging studies revealed the presence of a large tumor measuring 42 cm in its greatest diameter that covered the entire mediastinum, involved both thoracic cavities, rejected the lungs, heart and big glasses. The sharp needle biopsy under ultrasound guidance was reported as liposarcoma. The patient had complete resection of the tumor through the Clamshell incision. In the immediate postoperative period, he presented circulatory shock plus multiple organ dysfunction (DOMS): plateletopenia, acute renal failure with the need for dialytic support, liver injury. Specialized support and monitoring in the Intensive Care Unit (ICU) allowed clinical improvement and good evolution. He was discharged in good condition.

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INTRODUCTION: conventional parosteal osteosarcoma is an uncommon malignant bone tumor, comprising 4% of all osteosarcomas. Although rare, parosteal osteosarcoma is the most common type of osteosarcoma of the bone surface. We present the clinical, histological and imaging characteristics of a rare histologic variant of a parosteal osteosarcoma, review the literature and emphasize the importance of radio-pathological correlation as well as the interpretation of a representative biopsy in order to obtain the correct diagnosis. CASE REPORT: a 36-year old woman began her condition one year prior to admission to the hospital with increased volume in the left knee and pain. Image studies showed a juxtacortical heterogeneous tumor localized on the posterior surface of the distal femoral metaphysis. An incisional biopsy was performed, with the diagnosis of a Parosteal Osteosarcoma and a wide surgical resection was undertaken. According to the findings of the surgical specimen, the diagnosis of a Parosteal Osteosarcoma with low grade chondrosarcoma and liposarcoma components was made. The knowledge of this rare parosteal osteosarcoma variant can lead the orthopedic oncologists to avoid overlooking the adipose component and provide adequate surgical margins. CONCLUSION: we present the clinical, histological and imaging characteristics of a Parosteal Osteosarcoma with low grade liposarcoma and chondrosarcoma components.

INTRODUCCIÓN: el osteosarcoma parosteal convencional es un tumor óseo maligno poco común, que comprende el 4% de todos los osteosarcomas. Aunque es poco común, el osteosarcoma parosteal es el tipo más común de osteosarcoma de la superficie ósea. Presentamos las características clínicas, histológicas y de imagen de una variante histológica rara de un osteosarcoma parosteal, revisamos la literatura y enfatizamos la importancia de la correlación radio-patológica, así como la interpretación de una biopsia representativa para obtener el diagnóstico correcto. REPORTE DE CASO: mujer de 36 años inició su cuadro un año antes de su ingreso al hospital con aumento de volumen en rodilla izquierda y dolor. Los estudios de imagen mostraron una tumoración heterogénea yuxtacortical localizada en la superficie posterior de la metáfisis femoral distal. Se realizó biopsia incisional, con diagnóstico de osteosarcoma parosteal y se realizó resección quirúrgica amplia. De acuerdo con los hallazgos de la pieza quirúrgica se realizó el diagnóstico de osteosarcoma parosteal con componentes de condrosarcoma y liposarcoma de bajo grado. El conocimiento de esta rara variante de osteosarcoma parosteal puede llevar a los ortopedistas oncólogos a considerar otros componentes y proporcionar márgenes quirúrgicos adecuados. CONCLUSIÓN: presentamos las características clínicas, histológicas y de imagen de un osteosarcoma parosteal con componentes de liposarcoma y condrosarcoma de bajo grado.

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PURPOSE: Soft tissue sarcomas are rare malignant tumors. Liposarcoma constitutes the most frequent histological subtype of retroperitoneal sarcoma. The prognosis of soft tissue sarcomas depends on clinical and histologic characteristics. OBJECTIVE: Evaluate variables that may be related to the overall and local recurrence-free survival in patients with retroperitoneal liposarcoma and discuss the need for visceral resection en-bloc for tumors. METHODS: A retrospective analysis was conducted of the medical records of 60 patients seen between 1997 and 2017 who underwent surgical resection of retroperitoneal liposarcoma. RESULTS: The overall survival rate at 5 years of follow-up was 75.22% (95% confidence interval [CI] 0.58-0.86). The probability of a local recurrence-free survival at 5 years of follow-up was 26.04% (95% CI 0.11-0.44). The multivariate analysis showed that dedifferentiated or pleomorphic tumors and R2/fragmented resection were associated with a shorter time to recurrence. No other characteristics markedly influenced the overall survival (P > 0.05). CONCLUSION: Patients with dedifferentiated or pleomorphic tumors and incomplete resection were associated with higher local recurrence rates than others. This study reinforces the need for complete and en-bloc resection with organs when there is clear involvement or technical surgical difficulty to maintain the tumor integrity.

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BACKGROUND: The addition of radiation therapy to surgery for retroperitoneal sarcoma remains controversial. Improved patient selection may help identify optimal candidates for multimodality treatment. The aim of this analysis was to define prognostic factors among patients who receive radiation therapy and surgery to aid in patient selection for multimodal therapy. METHODS: Patients who received radiation therapy and underwent curative-intent resection for retroperitoneal sarcoma between 2004 and 2016 were identified from a national cohort in the United States (National Cancer Database). A machine-based classification and regression tree model was used to generate similar groups of patients relative to overall survival based on preoperative factors. RESULTS: A total of 1,443 patients received radiation therapy in addition to surgery. Median age was 61 years old and 55.0% were female. Most patients (66%) received care at an academic or integrated network cancer program. With a median follow-up of 84 months, receipt of radiation therapy was not associated with improved overall survival (P = .81). Classification and regression tree analysis revealed a significant association between overall survival and American Joint Committee on Cancer stage group, age, tumor histology, and Charlson comorbidity score. Application of these parameters via machine learning stratified patients into 5 cohorts with distinct survival outcomes. In the most favorable cohort (Cohort 1: American Joint Committee on Cancer stage group ≤II, age ≤61, histology including fibrosarcoma, well differentiated liposarcoma, myxoid liposarcoma, and leiomyosarcoma), the 5-year overall survival was 81.7% and median overall survival was not reached; in the least favorable cohort (Cohort 6: American Joint Committee on Cancer stage group >II, age >68) where the 5-year survival was 41.3% and median overall survival was 45.2 months (P < .001 versus Cohort 1). CONCLUSION: In the absence of a defined survival benefit, patients with advanced American Joint Committee on Cancer stage group, older age, and medical comorbidities have relatively unfavorable overall survival after combined modality therapy and therefore stand the least to gain from the addition of radiation therapy to surgery. In contrast, younger patients with good performance status and retroperitoneal sarcoma histologies with a higher propensity for local recurrence may have the greatest opportunity to benefit from radiation therapy.

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Retroperitoneal liposarcomas are very infrequent retroperitoneal malignant tumours. Most patients complain of palpable abdominal mass and only half present with abdominal pain. With haematogenous spread they can reach, mainly, lungs and liver, but only in 10% of cases. Here we report the presentation and surgical treatment of a giant massive retroperitoneal liposarcoma in a 53-year-old man.

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Retroperitoneal liposarcomas are very infrequent retroperitoneal malignant tumours. Most patients complain of palpable abdominal mass and only half present with abdominal pain. With haematogenous spread they can reach, mainly, lungs and liver, but only in 10% of cases. Here we report the presentation and surgical treatment of a giant massive retroperitoneal liposarcoma in a 53-year-old man.

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OBJECTIVES: Misnaming low-grade lipomatous tumors poses a clinical and medicolegal challenge, potentially subjecting patients to expensive and unnecessary surgeries. The terms atypical lipomatous tumor (ALT) and "well-differentiated" liposarcoma (WDL) have been used interchangeably in pathology reports, scholarly works and consensus recommendations, creating vagaries between low-virulence extremity tumors and retroperitoneal disease with metastatic potential. METHODS: A systematic review was performed on all studies that reported on the local recurrence rate and metastasis of ALTs and WDLs in living human subjects. Local recurrence and metastases were compared using Fisher's Exact Test. RESULTS: In total, 20 studies evaluated ALTs (n=936), whereas 13 studied WDLs (n=626). Mean follow-up was 6.6±2.0 years (median, 7.0 y). No metastatic disease was observed among ALTs, whereas 15 patients with WDLs (2.7%, P<0.0001) had metastases. The local recurrence rate of ALTs was significantly lower than WDLs after both marginal (15.1%, 141/936 vs. 46.0%, 288/626, P<0.0001) and wide excisions (3.3%, 2/59 in ALT vs. 17.4%, 19/109, P=0.007). CONCLUSIONS: ALT should be reserved for extremity lesions meeting appropriate histopathologic criteria that represent nonmetastatic disease, reducing over-diagnosis, over-treatment, and patient risk.

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INTRODUCCIÓN: Los tumores paratesticulares representan del 7%-10% de las masas intraescrotales. Los sarcomas abarcan el 90% de las lesiones malignas del cordón espermático y de éstas, aproximadamente, el 3%-7% son liposarcomas. CASO CLÍNICO: Presentamos el caso de un varón de 45 años, que consultó en urgencias por una masa inguinoescrotal derecha no reductible, diagnosticándose de hernia inguinal incarcerada. Se realizó cirugía urgente evidenciando una gran tumoración de aspecto lipomatoso, dependiente de cordón espermático. Se realizó orquiectomía y hernioplastía inguinal. La anatomía patológica, reveló un liposarcoma bien diferenciado de cordón espermático. Posteriormente, se realizó estudio de extensión, sin afectación a distancia y no precisó tratamiento adyuvante. Actualmente, tras dos años de seguimiento no ha presentado recidiva. DISCUSIÓN: Sólo alrededor de 200 casos han sido comunicados previamente en la literatura y sólo 61 de éstos se presentaron simulando una hernia inguinal incarcerada. Debido a la baja incidencia de esta patología es difícil de conocer la historia natural y llegar a conclusiones sobre los resultados del tratamiento, el cual hasta el momento sigue siendo la orquiectomía radical, con escisión amplia de los tejidos locales. El papel de la radio y quimioterapia aun es controvertido. CONCLUSIONES: Los sarcomas del cordón espermático son neoplasias raras con alta tasa de recurrencia local. Su manejo inicial es quirúrgico. Se requiere de un alto índice de sospecha clínica para el diagnóstico ya que las implicaciones oncológicas varían en función del tratamiento que, en ocasiones, es llevado a cabo por cirujanos generales al simular una hernia inguinal.

INTRODUCTION: Paratesticular tumors represent 7%-10% of intraescrotal masses. Sarcomas account for 90% of malignant lesions of the spermatic cord and of these approximately 3%-7% are liposarcomas. CLINICAL CASE: This is the case of a 45 year old male who consulted in the emergency department for a non-reducible right inguino-scrotal mass and was diagnosed with an incarcerated inguinal hernia. Emergency surgery was performed which revealed a large lipomatous tumor, originating from the spermatic cord. Orchiectomy and hernioplasty were performed. Histopathology revealed a well-differentiated liposarcoma of the spermatic cord. Later extension study was conducted, without distant affectation, and did not require adjuvant treatment. Today, after two years of monitoring has been no recurrence. DISCUSSION: Only about 200 cases have been previously reported in the literature and only 61 of these were presented mimicking an incarcerated inguinal hernia. Due to the low incidence of this disease it is difficult to know the natural history and draw conclusions on the results of treatment, which so far remains the radical orchiectomy with wide local excision of the tissue. The role of radiotherapy and chemotherapy is still controversial

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O lipossarcoma de laringe é uma neoplasia extremamente rara, acomete principalmente o sexo masculino, principalmente na quinta década de vida. Existindo apenas cerca de 40 casos descritos na literatura, desses nenhum em língua portuguesa. O presente caso relata o diagnóstico em um paciente do sexo masculino, 57 anos, ex-tabagista, apresentando alteração de voz e obstrução de via área. Foi optado por ressecção cirúrgica completa com achados sugestivos de lipossarcoma bem diferenciado. Foi optado por manter seguimento, não tendo sido indicado quimioterapia e radioterapia adjuvantes.(AU)

Laryngeal liposarcoma is an extremely rare neoplasm that affects especially men in the fifth decade of life. There are only about 40 cases described in the literature, none of them in the Portuguese language. We report the case of a 57-year-old, former smoker man presenting with voice disorders and airway obstruction. We opted for complete surgical resection with findings suggestive of well-differentiated liposarcoma. We chose to keep following the patient, and no adjuvant chemotherapy and radiotherapy were indicated. (AU)

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