Characterization and predictive value of volume changes of extremity and pelvis soft tissue sarcomas during radiation therapy prior to definitive wide excision

PURPOSE: The purpose of this study was to characterize and evaluate the clinical significance of volume changes of soft tissue sarcomas during radiation therapy (RT), prior to definitive surgical resection. MATERIALS AND METHODS: Patients with extremity or pelvis soft tissue sarcomas treated at our institution from 2013 to 2016 with RT prior to resection were identified retrospectively. Tumor volumes were measured using cone-beam computed tomography obtained daily during RT. Linear regression evaluated the linearity of volume changes. Kruskal-Wallis tests, Mann-Whitney U tests, and linear regression evaluated predictors of volume change. Logistic and Cox regression evaluated volume change as a predictor of resection margin status, histologic treatment response, and tumor recurrence. RESULTS: Thirty-three patients were evaluated. Twenty-nine tumors were high grade. Prior to RT, median tumor volume was 189 mL (range, 7.2 to 4,885 mL). Sixteen tumors demonstrated significant linear volume changes during RT. Of these, 5 tumors increased and 11 decreased in volume. Myxoid liposarcoma (n = 5, 15%) predicted decreasing tumor volume (p = 0.0002). Sequential chemoradiation (n = 4, 12%) predicted increasing tumor volume (p = 0.008) and corresponded to longer times from diagnosis to RT (p = 0.01). Resection margins were positive in three cases. Five patients experienced local recurrence, and 7 experienced distant recurrence, at median 8.9 and 6.9 months post-resection, respectively. Volume changes did not predict resection margin status, local recurrence, or distant recurrence. CONCLUSION: Volume changes of pelvis and extremity soft tissue sarcomas followed linear trends during RT. Volume changes reflected histologic subtype and treatment characteristics but did not predict margin status or recurrence after resection.

Liposarcoma paratesticular gigante / Giant Paratesticular Liposarcom

Introducción Los liposarcomas son lesiones que se originan en el tejido mesodérmico, su localización paratesticular es muy rara. Se divide en 4 subtipos histológicos, siendo el mixoide el más infrecuente. Tiene una recurrencia del 70%, Por lo que su tratamiento inicial debe ser agresivo. Objetivo resaltar su gran tamaño y que sea considerado como diagnóstico diferencial de masa inguinoescrotal. Presentación del caso Paciente masculino de 87 años con masa dolorosa inguinoescrotal derecha con diagnóstico de hernia inguinal unilateral, se realiza orquiectomía inguinal derecha. En patología se recibe pieza quirúrgica de 2440 g; al estudio histológico se observa lesión tumoral mesenquimal maligna, rodeado por estroma nodular mixoide prominente. Se diagnostica liposarcoma mixoide de cordón espermático. (Figura 2). Conclusiones Son neoplasias raras mal diagnosticadas como hernia inguinal, escrotal o lipoma de la médula espermática. Tiene un alto riesgo de metástasis, siendo la orquiectomía inguinal con resección del cordón espermático el manejo quirúrgico ideal.

Introduction Liposarcomas are lesions originated in the mesodermal tissue, paratesticular location is very rare. It is divided into 4 histological subtypes, being the most uncommon the myxoid. It has a recurrence of 70%, so the treatment should be aggressive. Objective to emphasize the large size and the differential diagnosis of inguinoescrotal mass. Case Presentation 87-year-old male patient with right inguinoescrotal pain with diagnosis of unilateral inguinal hernia, right inguinal orchiectomy is performed. A surgical specimen of 2440 g is received in pathology. Histological study shows malignant mesenchymal tumor lesion, surrounded by prominent myxoid nodular stroma. Myxoid liposarcoma of the spermatic cord is diagnosed. (Fig. 2). Conclusions they are rare neoplasms and are poorly diagnosed as inguinal hernia, scrotal or lipoma of the spermal cord. It has a high risk of metastasis, the ideal surgical management is an inguinal orchiectomy with resection of the spermatic cord.

Myxoid Liposarcoma: A Single Institute Experience / 대한정형외과학회잡지

PURPOSE: This study assessed the treatment outcomes of myxoid liposarcoma in the extremities and investigate the prognostic factors. MATERIALS AND METHODS: A total of 91 patients with myxoid liposarcoma (83 primary, 8 recurrent) between 2001 and 2015 were reviewed retrospectively. The local recurrence and metastasis after treatment were examined. The survival rates and prognostic factors affecting the survival were investigated. The mean follow-up was 84 months (range, 5–196 months). RESULTS: The overall survival rates at 5-yr and 10-yr were 82% and 74%, respectively. The tumor size (p=0.04), round cell component (p<0.0001), grade (p=0.0002), and local recurrence (p=0.006) affected survival in primary patients. Extrapulmonary metastases were observed in 75.0% (18/24) of metastatic patients and the mean post metastasis survival was 26 months (range, 2–72 months). CONCLUSION: Myxoid liposarcoma developed mainly at the lower extremities. The tumor size, grade, component of round cells, and local recurrence were associated with the prognosis. The unique feature of extrapulmonary metastasis in myxoid liposarcoma should be noted in the treatment and follow-up.

Myxoid Liposarcoma of Spermatic Cord Misdiagnosed for Hemangioma

Myxoid liposarcoma arose from the spermatic cord is very rare. Also, it is difficult to diagnose by clinical findings or radiologic study. Even if it was a malignant tumor, scrotal malignancy is often misdiagnosed in many cases. A 55-year-old man presented with a mass in the left scrotum. Tumor markers showed all normal range. A scrotal magnetic resonance imaging scan revealed a vascular tumor such as a hemangioma. But, we performed radical orchiectomy. The mass was diagnosed as paratesticular myxoid liposarcoma. It is important to keep in mind the differential diagnosis of paratesticular liposarcoma in the case of a painless scrotal tumor.

The Magnetic Resonance (MR) Imaging Features of Myxoid Liposarcoma Arising from the Mesentery: a Case Report

Primary mesenteric liposarcoma is rare. It is difficult to make an accurate preoperative diagnosis of the myxoid type of liposarcoma by using imaging such as ultrasound or computed tomography (CT) due to the very small amount of fat that is located in the tumor. We report a case of primary myxoid liposarcoma of the mesentery which was difficult to differentiate from other solid mesenteric tumors with a myxoid component such as low grade fibromyxoid sarcoma, myxoid leiomyosarcoma or myxoma. Use of chemical shift magnetic resonance (MR) imaging to detect small fat components and its cystic appearance with solid components on the MR images can be useful to differentiate myxoid liposarcoma from the other mesenteric tumors with a myxoid component.

Liposarcoma mixoide retroperitoneal gigante: A propósito de un caso / Giant Retroperitoneal Myxoid Liposarcoma: A case report

El Liposarcoma es el sarcoma retroperitoneal más frecuente. En el Perú sólo se conoce la frecuencia en el Instituto Nacional de Enfermedades Neoplásicas, donde representan el 33,7% de los casos de sarcoma retroperitoneal. Su crecimiento es lento y su sintomatología más frecuente es dolor abdominal inespecífico y aumento del perímetro abdominal. La tomografía computarizada (TC) brinda información vital en el diagnóstico. Reportamos el caso de un varón de 34 años que presentó un cuadro de 8 meses de duración caracterizado por sensación de tumoración abdominal, dolor abdominal y constipación. La TC de abdomen mostró signos de tumor retroperitoneal con pequeñas áreas de densidad grasa (-53UH). El diagnóstico anatomo-patológico fue Liposarcoma mixoide con áreas de desdiferenciación, lo que es infrecuente en la región retroperitoneal. Se analizan la localización retroperitoneal y sus características tomográficas. (AU)

Liposarcoma is the most common retroperitoneal sarcoma. In Peru is only known the frequency of the Instituto Nacional de Enfermedades Neoplásicas, where it accounts for 33.7% of retroperitoneal sarcomas. The growth of this tumor is low, and it is associated with non-specific symptoms, including abdominal pain and increase in abdominal waist. CT-scan provides vital information for diagnosis. We report the case of a 34 year-old male patient who presented with a 8-month history of abdmominal pain, constipation and feeling of an intra-abdominal mass. The CT-scan revealed signs of a retroperitoneal mass with fat tissue densities (-53UH). Pathological diagnosis was dedifferentiated myxoid liposarcoma, which is infrequent in the retroperitoneum. We analyzed the retroperitoneal location and CT-scan features of this tumor. (AU)

Myxoid liposarcoma in a dog / 대한수의학회지

The pathological features of a mass in the back skin region of an 8-year-old castrated male dog are described herein. The cut section of the tumor was white to tan with a soft multilobulated mass containing hemorrhagic and necrotic foci and a mucinous-like composition. Microscopically, the tumor was composed of a mixture of lipocytes, lipoblasts, spindle cells and stellate cells and had a myxoid background. Oil red O staining revealed that the cytoplasm of neoplastic cells contained large numbers of lipid droplets. Immunohistochemically, tumor cells were positive for vimentin and S-100 protein. The skin mass was diagnosed as myxoid liposarcoma.

successful resection of cardiac metastasis of round cell liposarcoma

Myxoid is a common type of liposarcoma. Cardiac metastasis of sarcoma is a rare diagnosis. Transthoracic echocardiography is a useful tool for beginning the diagnosis. Curative resection of metastasis and then combination systemic chemotherapy for advanced disease is recommended. We report a 50 year old woman who presented with a cardiac metastasis of high grade round cell liposarcoma to right atrium and right ventricle. The tumor was diagnosed with computed tomography and transthoracic echocardiography. The tumor size was 10*10 cm. This is the second report of successful resection of a large intracardiac metastasis of liposarcoma

Myxoid Liposarcoma with Cartilaginous Differentiation: A Case Study with Cytogenetical Analysis

Myxoid liposarcoma is a subtype of liposarcoma. This specific subtype can be identified based on its characteristic histological and cytogenetical features. The tumor has a fusion transcript of the CHOP and TLS genes, which is caused by t(12;16)(q13;p11). Most of the fusion transcripts that have been identified fall into three categories, specifically type I (exons 7-2), type II (exons 5-2), and type III (exons 8-2). A total of seven myxoid liposarcomas associated with the rare phenomenon of cartilaginous differentiation have been documented in the literature. Currently, only one of these cases has been cytogenetically analyzed, and the analysis indicated that it was a type II TLS-CHOP fusion transcript in both the typical myxoid liposarcoma and cartilaginous areas. This study presents a second report of myxoid liposarcoma with cartilaginous differentiation, and includes a cytogenetical analysis of both the myxoid and cartilaginous areas.

Diagnostic value of ultrasound-guided core needle biopsy for soft tissue tumors / 中华病理学杂志

<p><b>OBJECTIVE</b>To study the diagnostic value and pitfalls of ultrasound-guided core needle biopsy (CNB) of soft tissue tumors.</p><p><b>METHODS</b>One hundred and six cases of CNB specimens encountered during the period from 2007 to 2012 were enrolled into the study. The pathologic diagnosis using CNB was compared with that using surgical specimens. Diagnostic accuracy was analyzed using Chi-square test, with respect to the histologic pattern (such as spindle cell and myxoid), biologic behavior (benign versus malignant) and immunohistochemical results. The 59 cases of sarcoma were subdivided into three grades according to FNCLCC grading system.</p><p><b>RESULTS</b>Histologic diagnosis could be made in 84.0% (89/106) cases. Thirteen cases were non-diagnostic on CNB. There were 4 cases on CNB showing diagnostic discrepancy with surgical specimens. Four cases of "benign lesions" on CNB found to be myxoid liposarcoma and lipoma-like liposarcoma upon resection. In general, myxoid pattern (9/17) seen on CNB showed less diagnostic correlation with surgical specimens, as compared to spindle cell and other histologic patterns (P < 0.01). The rate of diagnostic correlation was 79.7% (49/59) for the 59 cases of sarcoma studied, with grade 2 and grade 3 sarcoma showing better correlation (in contrast to 7/17 for grade 1 sarcoma) (P < 0.01). Comparative analysis showed no significant difference between benign/borderline tumors and sarcomas. The application of immunohistochemical study did not result in significant improvement in diagnostic accuracy on CNB.</p><p><b>CONCLUSIONS</b>Ultrasound-guided CNB is a reliable tool in pathologic diagnosis of soft tissue tumors and shows a high accuracy rate especially for high-grade sarcoma. Tumors with myxoid pattern, lipomatous tumors and grade 1 sarcomas are associated with lower diagnostic accuracy on CNB. Correlation with clinicoradiologic findings would also be helpful in diagnostic evaluation and surgical planning.</p>

Diagnostic Value of MDM2 and DDIT3 Fluorescence In Situ Hybridization in Liposarcoma Classification: A Single-Institution Experience

BACKGROUND: The amplification of murine double minutes (MDM2) is the primary feature of well-differentiated liposarcomas (WDLPS) and dedifferentiated liposarcomas (DDLPS), while DDIT3 rearrangement is the main one of myxoid liposarcomas (MLPS). Our aim was to evaluate the added value of MDM2 amplification and DDIT3 rearrangement in making a diagnosis and classifying lipogenic tumors. METHODS: Eighty-two cases of liposarcoma and 60 lipomas diagnosed between 1995 and 2010 were analysed for MDM2 amplification and DDIT3 rearrangement using a fluorescence in situ hybridization (FISH). The subtypes of liposarcoma were reclassified according to the molecular results, whose results were reviewed with an analysis of the relevant histologic and immunohistochemical findings. RESULTS: One case of lipoma (1.67%) was reclassified as a WDLPS. Of the liposarcomas, 13.4% (16/82) were reclassified after the molecular testing. Five cases of MLPS were reclassified as four cases of DDLPS and one case of myxoid lipoma. Two cases of WDLPS were reclassified as one case of spindle cell lipoma and another case of myxofibrosarcoma. Four cases of DDLPS were reclassified as two cases of leiomyosarcoma, one case of angiomyolipoma and another case of fibroinflammatory lesion. Of the six cases of pleomorphic liposarcoma, five were reclassified as DDLPS. CONCLUSIONS: In our series, a critical revision of diagnosis was found at a rate of 3.5% (5/142) after a review of the lipomatous lesions. The uses of molecular testing by MDM2 and DDIT3 FISH were valuable to make an accurate subtyping of liposarcomas as well as to differentiate WDLPS from benign lipomatous tumor.

Primary Pulmonary Myxoid Liposarcoma with Translocation t(12;16)(q13;p11) in a Young Female Patient: A Brief Case Report

Primary liposarcoma of the lung is an extremely rare disease. To date, only 14 cases have been reported in the literature. We experienced a case of myxoid liposarcoma of the lung treated by surgery. The tumor was well-defined, solid, lobulated mass measuring 3.5x2 cm, involving the bronchus of the left lower lobe. Microscopically, myxoid liposarcoma was identified. The fluorescence in situ hybridization confirmed the presence of a reciprocal translocation involving DNA damage-inducible transcript 3 (DDIT3) and fused in sarcoma (FUS) genes. The patient is still alive with no recurrence or metastasis at the time of writing this report (on 20 months postoperatively). To our knowledge, this is the first cytogenetic case report of pulmonary myxoid liposarcoma.

A Case of Myxoid Liposarcoma of the Cheek

PURPOSE: Liposarcoma is the most common soft tissue sarcoma, and usually occurs on the thigh or in the retroperitoneal space, but rarely in the oral region. This report presents a case of liposarcoma of the cheek and includes a review of the literatures. METHODS: A 21-year-old woman was admitted with a palpable mass in her cheek of about two years duration, which increased in size gradually initially, but had increased rapidly over the three months. There was no particular pain or tenderness. MRI showed a well-enhanced, well-defined mass, which suspected to be hemangioma. RESULTS: The spherical, well-encapsulated mass was surgically excised. Biopsy results revealed myxoid liposarcoma. FDG PET-CT on the seventh postoperative day, revealed a minimal to mild FDG-uptake soft tissue lesion around the mass defect area without evidence of distant metastasis. The patient is being observed and undergoing radiation therapy. CONCLUSION: Liposarcoma in the head and neck region is a rare disease, and can be overlooked as a benign tumor without a pathologic diagnosis. Therefore, proper treatment and follow-up are required based on an understanding of this disease.

Report of the first case of myxoid liposarcoma in Colombia: a rare tumor / Informe del primer caso de liposarcoma mixoide en Colombia: un tumor raro

Introduction: Primary esophageal myxoid liposarcoma is exceedingly rare. Sarcomas make up 1% of esophageal malignant tumors. There are only five (5) cases reported with this histological variant (myxoid) in previous literature. In Colombia, this is the first case reported and the sixth in the world. Objective: To report the first case in Colombia of myxoid liposarcoma of the esophagus with clinical, radiographic images, histology, surgical and to describe difficulties in the diagnosis. Methodology: We reviewed the clinical history of a 28-year old male patient. He was admitted to Hospital Universitario del Valle in Cali, Colombia with a clinical history of dysphagia, weight loss, and excessive salivation. The initial examination (esophagogram, cervical CAT scan and endoscopy) demonstrated a mass that was reported as a fibrovascular polyp. The finding of the pathological diagnosis was myxoid liposarcoma. Conclusions: The rarity of this condition recommends report of its detailed description. The myxoid liposarcoma of the esophagus can be diagnosed if a patient has a history of a slow-growing esophageal mass with a low tumor density in computed tomography in combination with surgical resection and histological examination.

Introducción: El liposarcoma mixoide es una neoplasia maligna del mesénquima con una presentación muy rara en esófago. Los sarcomas representan 1% de los tumores esofágicos malignos, y este tipo histológico es el menos frecuente. En la actualidad, se encuentran informes en la literatura de cinco (5) casos de esta variante histológica en el esófago. En Colombia, es el primer caso encontrado y el sexto (6º) a nivel mundial. Objetivo: Presentar el primer caso en Colombia de un liposarcoma mixoide en el esófago, sus características clínicas, imagenológicas, histología, manejo quirúrgico y las dificultades en su diagnóstico. Metodología: Se revisó la historia clínica de un paciente masculino de 28 años que ingresa por urgencias al Hospital Universitario del Valle en Cali, Colombia, con historia de disfagia, pérdida de peso y sialorrea. Los estudios imagenológicos como esofagograma, escanografía cervical y endoscopia de vías digestivas altas son consistentes con un pólipo esofágico y el manejo quirúrgico consistió en la resección parcial y luego la resección completa de la lesión. El informe histopatológico de la resección parcial comunicó un pólipo fibrovascular y el informe de la resección completa fue de liposarcoma mixoide. Las dificultades diagnósticas que surgieron en este caso se relacionan con otras encontradas en la literatura. Conclusiones: El liposarcoma mixoide del esófago es una entidad que presenta dificultades en su diagnóstico debido a que la presentación clínica no es específica. Aunque en estos casos las biopsias iniciales pueden suponer lesiones benignas, sólo hasta el procesamiento histológico de todo el espécimen, es posible realizar el diagnóstico de la entidad.

Liposarcoma retroperitoneal recidivante / Recidivant retroperitoneal liposarcoma

Los liposarcomas retroperitoneales son tumores infrecuentes. Sus peculiaridades biológicas y clínicas les confieren algunos aspectos diferenciales respecto al resto de los sarcomas retroperitoneales. Se presenta el caso de una paciente de 59 años con el diagnóstico de liposarcoma retroperitoneal, variedad mixoide, con recidiva en cuatro oportunidades, realizándose en todas exéresis completa del tumor, sin extirpación de órganos vecinos, con radioterapia como tratamiento adyuvante. La supervivencia de esta paciente fue de cuatro años. La elevada tendencia a la recidiva de estos tumores determina un pronóstico global malo.

Retroperitoneal liposarcomas are infrequent tumors. Their biological and clinical peculiarities confer them some differential aspects regarding the rest of retroperitoneal sarcomas. The case of a 59 year-old patient is presented with the diagnosis of retroperitoneal liposarcoma, myxoid variety, with recidivation in four opportunities, being carried out in all complete exeresis of the tumor, without extirpation of neighbor organs, with radiotherapy as adjuvant treatment. This patient's survival was of four years. The high tendency to recidivation of these tumors determines a bad global prognosis.

Intradural Involvement of Multicentric Myxoid Liposarcoma

Liposarcomas are malignant tumors of the soft tissue, with myxoid liposarcoma being the second most common subtype, tending to occur in the limbs, particularly in the thighs. Myxoid liposarcomas have an intermediate prognosis between well-differentiated and pleomorphic tumors. Spinal metastasis is usual but intradural involvement is extremely rare. We present an unusual case of a multicentric myxoid liposarcoma with intradural involvement. A 41-year-old woman complained of tingling sensation on her left arm. Radiological evaluation revealed multiple masses in her cervical spine, abdominal wall, liver, heart and right thigh, all of which were resected. She was histologically diagnosed with small round cell myxoid sarcoma and underwent adjuvant chemotherapy. However, magnetic resonance imaging analysis after 1 year revealed a large metastatic mass with bony invasion at the C6-T1 level. This mass consisted of extradural and intradural components causing severe compression of the spinal cord. She underwent resection via a posterior facetectomy of C6-7 and an anterior C7 corpectomy. However, the patient died of multiple metastases 18 months after the first diagnosis.

Myxoid Liposarcoma of the Spermatic Cord with Multiple Distant Metastasis

Myxoid liposarcoma of the spermatic cord (MLSC) is a rare variant of spermatic sarcoma. Typically, it presents during the sixth decade of life as a painless scrotal or inguinal mass. Although local recurrences are not uncommon, prognosis following complete tumor removal is good because metastases are rare. Although myxoid liposarcoma is common elsewhere in the body, less than 20 cases have been described in the spermatic cord. Distant metastasis with MLSC (e.g., bone, liver, lung) is extremely rare, and no cases have been reported in the English literature. We report one case of MLSC with multiple bone, liver and peritoneal metastasis, incidentally found during inguinal hernia repair.

A Case of Multiple Angiomyxolipoma / 대한피부과학회지

Angiomyxolipoma is a rare variant of lipoma and seven cases have been reported in the English literature. The reported cases mostly presented with a well-demarcated, asymptomatic, solitary, subcutaneous mass on the scalp or extremities. Histopathologic features of this tumor are characteristic and consist of the paucicellular myxoid areas and the mature fat tissue, with numerous thin, dilated blood vessels. The important histopathological differential diagnoses include myxoid spindle cell lipoma, myxoid lipoma, angiolipoma, angiomyolipoma, superficial angiomyxoma, myxoid liposarcoma and low-grade myxofibrosarcoma. We describe a 38-year-old man with multiple angiomyxolipoma on the gluteal area and the upper and lower extremities.