Analysis of influence factors for short-term recurrence of retroperitoneal liposarcoma after complete resection / 中华外科杂志

Objective: To examine the influence factors of short-term recurrence after complete surgical resection of retroperitoneal liposarcoma. Methods: The clinicopathological data of retroperitoneal liposarcoma at Department of General Surgery, the First Medical Center, People's Liberation Army General Hospital from January 2000 to January 2020 were retrospectively analyzed. There were 60 males and 31 females, aged (52.1±9.9) years (range: 30 to 84 years). Tumor recurrence within 12 months after complete resection was defined as short-term recurrence, and tumor recurrence more than 12 months was defined as non-short-term recurrence. The t test, rank-sum test, χ2 test and Fisher exact test were conducted for inter-group comparison. Logistic regression analysis was used to analyze the independent influence factors for the short-term recurrence of retroperitoneal liposarcoma after complete resection. The Kaplan-Meier curve was used to calculate the recurrence-free survival, and the Log-rank test was adopted for the comparison between the groups. Results: The univariate analysis results showed that irregular tumor morphology, multiple pathological subtypes, pathological scores>3, and multiple primary tumors are influence factors for short-term recurrence after complete resection of retroperitoneal liposarcoma (χ2: 4.422 to 7.773, all P<0.05). Regression analysis of the above risk factors showed that multiple primary tumors was the independent risk factor (OR=2.918, 95%CI: 1.127 to 7.556, P=0.027). In the short-term recurrence group, Kaplan-Meier curve analysis showed that patients with multiple primary tumors had a shorter median recurrence time than patients with unifocal tumor (6 months vs. 9 months, P=0.028). Conclusions: Multiple primary tumor is an independent risk factor for short-term recurrence after complete resection of retroperitoneal liposarcoma. It suggests that the frequency of follow-up after surgery should be increased for such patients.

Paratesticular liposarcoma: the clinicopathological features of nineteen cases / 中华病理学杂志

Objective: To investigate the clinicopathological features and differential diagnoses of paratesticular liposarcoma. Methods: The cases were collected from 2012-2020, from the archives of the Department of Pathology, Peking University Third Hospital, with diagnosis confirmed by histology, immunostaining and FISH tests. Results: Totally 19 patients were enrolled (including 11 in-hospital patients and 8 consultant cases). The patients aged 37-84 years (mean 57 years). The preoperative clinical diagnoses were spermatic cord/inguinal masses (nine patients), scrotal masses (seven patients), and inguinal hernia (three patients). Six lesions recurred after local resection, including one case extending from pelvic liposarcoma. Histologically, there were 10 cases of well-differentiated liposarcoma (WDLPS) and nine cases of dedifferentiated liposarcoma (DDLPS). WDLPSs mostly showed the combined features of lipoma-like, inflammatory and sclerosing subtypes (six patients); the other four WDLPSs had pure lipoma-like subtype features. DDLPSs were low-grade (three patients) or high-grade (six patients), with the morphology resembling myxofibrosarcoma, inflammatory myofibroblastoma, spindle cell sarcoma, pleomorphic undifferentiated sarcoma and pleomorphic liposarcoma. Intense inflammatory cells infiltration was commonly observed in five WDLPSs and two DDLPSs. Ossification was observed in three tumors. Immunohistochemically, the tumors were positive for MDM2 (8/10) and CDK4 (10/10), which were expressed in lipo-differentiating cells, spindle cells in WDLPS, and in dediffferentiated components. S-100 was only expressed by lipocytes (10/10). CD34 expression was positive and diffuse in the stromal cells of WDLPSs and focal or diffuse in dedifferentiated areas (10/10). FISH tests with an MDM2 gene probe were positive (12/12). Conclusions: Paratesticular liposarcoma may be overlooked by both clinicians and pathologists. WDLPS and DDLPS predominate, showing various histologic divergences. The presence of amplification of the 12q14-q15 region (containing the MDM2 and CDK4 genes) is helpful for making the correct diagnosis.

Liposarcoma del cordón espermático simulando una hernia inguinal incarcerada / Liposarcoma of the spermatic cord mimicking an incarcerated inguinal hernia

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

Lipossarcoma de laringe: relato de caso / Laryngeal liposarcoma: a case report

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)

Liposarcoma retroperitoneal gigante: a propósito de un caso / Giant retroperitoneal liposarcoma:a case report

Paciente masculino de 48 años con antecedente de hipertensión, que consulta por dolor abdominal difuso continuo, anorexia y aumento progresivo del perímetro abdominal. Al examen por TC se observa importante formación sólida heterogénea de densidad predominantemente grasa, que ocupa la casi totalidad del abdomen con desplazamiento de estructuras del abdomen superior; luego de la administración de medio de contraste formación presenta además componente de densidad de partes blandas de aspecto vegetante con realce heterogéneo que infiltra el retroperitoneo. Luego de su recesión completa, se concluye con el diagnostico de liposarcoma.

48 years old male patient with a history of hypertension, that consultation by diffuse abdominal pain continued, anorexia and progressive increase in the abdominal circumference. To review by TC is important training solid heterogeneous density predominantly fat that occupies almost the whole of the abdomen with displacement of structures in the upper abdomen, after the administration of contrast medium training is also a component of soft tissue density of vegetative aspect with heterogeneous enhancement that infiltrates the retroperitoneum, after his complete recession, it is concluded with the diagnosis of liposarcoma.

Liposarcoma retroperitoneal complicado: a propósito de un caso / Complicated retroperitoneal liposarcoma: a case report

Resumen Las neoplasias retroperitoneales suponen un reto para el profesional sanitario, tanto en el diagnóstico como en el tratamiento. Caso clínico: Varón de 56 años diabético tipo 2. Acude por edema en extremidad inferior derecha y parestesias. Se realiza ecografía doppler con resultados de trombosis venosa profunda y conglomerado adenopático. En TAC abdominal se observa gran masa retroperitoneal que engloba paquete vasculonervioso iliaco derecho. Se realiza vaciamiento ganglionar con resultado inicial de hamartoma. Se practica cirugía de resección de la masa y ligadura de la vena iliaca externa trombosada preservando la arteria iliaca. Anatomía patológica intraoperatoria sugestiva de sarcoma y definitiva de liposarcoma. Reingresa por shock hipovolémico y rotura de la arteria iliaca derecha. Se realiza ligadura y bypass femoro-femoral izquierdo-derecho. Al alta es derivado a oncología, falleciendo a los 6 meses. Discusión: El sarcoma es el tumor primario más frecuente a nivel retroperitoneal (15%). De difícil diagnóstico y tardío tratamiento, suelen ser asintomáticos hasta que debutan como una masa y clínica de compresión local o afectación de estructuras vasculonerviosas. El TAC es la prueba diagnóstica de elección. El tratamiento del tumor localizado es la cirugía; la radioterapia y quimioterapia se reservan para tumores irresecables o de alto grado.

Abstract Retroperitoneal neoplasias may be a diagnostic and therapeutic challenge for the clinician. Case report: A 56 year old male with prior history of type 2 diabetes consults with right inferior limb edema and paresthesias. He was diagnosed of deep vein thrombosis and lymph node conglomerate by ultrasound. A CT scan was performed, observing great retro peritoneal mass enclosing vascular and nervous structures. An initial histological diagnosis of hamartoma was made. Surgery was scheduled to remove the tumour, ligation of thrombosed external iliac vein and the external iliac artery was preserved. Intraoperative histological findings suggested sarcoma, definite studies showed presence of high grade liposarcoma. Two weeks after the intervention, the patient presents in hypovolemic shock due to rupture of the right external iliac artery. Emergent ligation of the artery and femorofemoral bypass was performed, with adequate postoperatory recovery. The patient was discharged and continued adjuvant oncology treatment and was exitus six months later. Discussion: Sarcomas are the most frequent primary retroperitoneal tumours. They are difficult to diagnose and often have untimely treatment. Sarcomas tend to be asymptomatic, or present with local compression symptoms, affecting vascular and nervous structures. CT scan is the gold standard for diagnostic imaging. Treatment requires a multidisciplinary approach, surgical resection as the main therapy; radio and chemotherapy represents a solution for irresectable or high grade malignancies.

Giant atypical lipoma

Abstract: Liposarcomas correspond to the most common histological subtype of soft tissue sarcomas. They can be subdivided into: well differentiated or atypical lipoma, undifferentiated, myxoid, round, and pleomorphic cells. Atypical lipomas are the most prevalent and usually appear as asymptomatic softened tumors. They are locally aggressive but rarely lead to distant metastases. The diagnosis of this tumor is based on the imaging and histopathologic findings. Treatment consists of excision surgery with complete tumor removal. It has a good prognosis due to the low percentage of distant metastases. We report a rare case of giant atypical lipoma as well as the adopted therapy and evolution.

Liposarcoma retroperitoneal gigante: reporte de caso y revisión de la literatura / Great retroperitoneal liposarcoma: case report and literature review

Introducción: El liposarcoma es el tumor retroperitoneal más frecuente. Es una enfermedad de evolución silenciosa cuyo tratamiento es el manejo quirúrgico. Caso clínico: Mujer de 42 años de edad, sin antecedentes de importancia, quien acude con gran liposarcoma. Se procede a realizar cirugía R0 y nefrectomía derecha. El resultado de patología reporta lipoma mixoide. Conclusiones: Los liposarcomas son tumores los cuales tienen dificultades en el manejo debido a un índice alto de irresecabilidad, patrón de recurrencia elevado, ineficacia en el empleo de tratamientos adyuvantes que impacten en la sobrevida y el control local.

Introduction: The liposarcoma is the most common retroperitoneal tumor. It is a silent disease evolution whose treatment is surgical management. Case report: 42 years old female with no history of importance who comes with great liposarcoma, we proceed to perform R0 surgery and right nephrectomy, the result of pathology reports myxoid lipoma. Conclusions: Liposarcomas are tumors which have difficulties in handling due to a high rate of unresectable, high recurrence pattern, inefficiency in the use of adjuvant therapies that impact on survival and local control.

Giant gastric lipossarcoma: case report and review of the literature / Lipossarcoma gástrico gigante: relato de caso e revisão da literatura

ABSTRACT Liposarcoma is one of the most common soft tissue sarcomas in adults, occurring in 15 to 20% of all patients with sarcoma. Primary liposarcoma of the stomach is rare. We report a case of patient with giant gastric liposarcoma who underwent surgery after a gastrointestinal bleeding. Preoperative hystopathological diagnosis was not established, even after three biopsy attempts. We discuss differential diagnosis, genetic causes, diagnosis strategies and treatment.

RESUMO O lipossarcoma é um tipo comum de sarcomas em adultos, com incidência entre 15 e 20% entre os sarcomas. No entanto, o acometimento do estômago é raro. Relatamos um caso de um lipossarcoma primário gástrico gigante com apresentação clínica de hemorragia digestiva. Foi submetido a tratamento cirúrgico sem diagnóstico definitivo, apesar de três biópsias realizadas. Revisamos diagnósticos diferenciais, influência genética e estratégias diagnósticas e terapêuticas.

Liposarcoma de células redondas de colon transverso y retroperitoneo: presentación de caso / Round cell liposarcoma of transverse colon and retroperitoneum: case report

Introducción: El liposarcoma representa la variedad más frecuente de los sarcomas de partes blandas del adulto. El subtipo células redondas representa el 15%. Por lo general compromete extremidades. La ubicación en colon es extremadamente rara. Objetivo: Evaluar el comportamiento de una patología infrecuente del colon y retroperitoneo, a partir de un caso clínico. Lugar de aplicación: Institución privada. Paciente y método: Paciente masculino de 45 años, operado con diagnóstico anatomopatológico de liposarcoma de células redondas por tumor colónico y retroperitoneal. Resultados: Se realizó colectomía derecha y resección de tumor abdominal. Conclusiones: Es una entidad poco frecuente, que por lo general se la diagnostica tardíamente. El tratamiento de elección es la cirugía. La quimioterapia y radioterapia, no han demostrado mejorar la supervivencia. Presenta un pronóstico malo, con alta tasa de recidiva.

Background: liposarcoma represents the most common kind of soft tissue sarcomas in adults. The round cell subtype represents 15%. Usually undertakes extremities. Colon location is extremely rare. Objective: To evaluate the behavior of a rare disease of the colon and retroperitoneum, from a clinical case. Place of application: Private institution. Patient and Methods: Male patient, 45 years, with a colonic and retroperitoneal tumor and pathological diagnosis of round cell liposarcoma. Results: Colectomy and abdominal tumor resection was performed. Conclusions: It is a rare condition, and diagnosis is usually late. Treatment of choice is surgery. Chemotherapy and radiotherapy have not been shown improvement in survival. Prognosis is poor with a high rate of recurrence.

Primary pleomorphic liposarcoma of breast: A rare case report.

Sarcoma of the breast constitutes less than 1% of all malignant breast tumors and liposarcoma of the breast has an incidence of 0.3% of all the mammary sarcomas. A 47-year-old woman presented with a mass in the right breast and mammography was suggestive of a malignant lesion. Modified radical mastectomy was performed. The tumor was diagnosed histologically as a pleomorphic liposarcoma. The patient was discharged and her postoperative recovery was uneventful.

Primary orbital liposarcoma: histopathologic report of two cases

Liposarcoma is a malignant tumor of adipose tissue. Considered the most common soft tissue sarcoma in adults, orbital liposarcoma is extremely rare. Most cases of orbital liposarcoma are primary and rarely metastatic. We report two cases of primary orbital liposarcoma with clinical presentation, radiologic studies [available for one case], and detailed histopathologic features. A brief review of primary orbital liposarcoma is also presented

Un cas de liposarcome de la cuisse observe chez l'enfant

Les liposarcomes sont rares chez l'enfant. Nous en rapportons un cas localise au niveau de la cuisse chez une fille de 12 ans. Il s'agit du premier cas observe chez l'enfant au Centre Hospitalier Universitaire Joseph Ravoahangy Andrianavalona (CHU-JRA) d'Antananarivo. Le diagnostic etait tardif car la tumeur avait envahi toutes les structures de la cuisse. Le traitement associait chimiotherapie et chirurgie. L'evolution etait defavorable avec dissemination neoplasique amenant le deces de la patiente apres trois mois. Les particularites epidemiologiques; diagnostiques et therapeutiques des liposarcomes de l'enfant sont discutees

A propósito de un caso de liposarcoma retroperitoneal / Apropos of a case of retroperitoneal liposarcoma

El liposarcoma retroperitoneal es un tumor maligno de origen mesenquimatoso, y el más frecuente de los sarcomas de partes blandas de localización retroperitoneal. Su crecimiento es lento e insidioso, lo que le permite adquirir grandes dimensiones permaneciendo asintomático. Se detecta en el examen físico o mediantes exámenes complementarios. Se presenta el caso de una paciente con una gran masa irregular hacia la mitad derecha y superior del abdomen y se muestran los resultados de los estudios complementarios que sugieren la presencia de un tumor retroperitoneal. Se encontró un tumor bien encapsulado que se extirpó totalmente. El estudio histológico confirmó un liposarcoma retroperitoneal bien diferenciado. La paciente ha tenido una evolución satisfactoria durante 2 años de seguimiento(AU)

The retroperitoneal liposarcoma is a malignant tumor of mesenchymatous origin, and it is the most frequent of the sarcomas of soft tissues of retroperitoneal localization. Its growth is slow and insidious, which allows it to acquire large dimensions in an asymptomatic way. It is detected in the physical examination or by complementary tests. The case of a patient with a large irregular mass on the upper right half of the abdomen is presented. The results of the complementary studies suggesting the presence of a retroperitoneal tumor are showed. A well encapsulated tumor that was totally removed was found. The histological study confirmed a well differentiated retroperitoneal liposarcoma. The patient has had a satisfactory evolution during 2 years of follow-up(AU)

Liposarcoma retroperitoneal como etiología de dolor abdominal: presentación de un caso y revisión de la literatura / Retroperitoneal liposarcoma as etiology of abdominal pain: case report and literature review

Soft tissue sarcomas are very uncommon types of tumors, with their embryological origin in the mesoderm and in nerve structures of the neuroectodermic layer. They represent only 1.5% of cases in the National Registry of Malignant Tumors in Mexico. They can be encountered anywhere connective soft tissue is found. Because of their specialized localization, retroperitoneal soft tissue sarcomas have a propensity to remain asymptomatic for long periods of time and reach a large size before being diagnosed. The only accepted treatment is wide surgical excision with clear margins, without a clear benefit for adjuvant treatment. The very uncommon nature of these tumors and their varied histopathology, site and behavior classify them as a difficult entity in terms of treatment. We present here the case of a 66-year-old female with a left-side retroperitoneal tumor, complaining only of vague abdominal pain as the presenting symptom. A CT-guided needle biopsy reported a sarcoma and the patient was subjected to laparatomy with complete resection of the tumor (30 x 13 x 10 cm). Histopathological report demonstrated a low-grade retroperitoneal sarcoma and free macroscopic and microscopic borders, without obvious invasion except for left kidney and ureter. The patient refused adjuvant treatment, and she is disease-free 7 years after treatment. Retroperitoneal sarcomas can cause pain and reach very large sizes. The best treatment available is wide surgical resection with clear margins. The most important prognostic factors are free margins, type of resection, age of patient and tumor histology.

Renal sparing surgery for perirenal liposarcoma: 24 months recurrence free follow up

Liposarcoma is the most common pathology seen in the soft tissue sarcoma of retroperitoneum. These tumors have been traditionally treated with radical surgery sacrificing adjacent organs to achieve clear margins. We have reported our experience of renal sparing surgery for perirenal liposarcoma in two patients with more than 24 month disease free survival.

Diagnostic laparoscopy and resection of a rare case of large mesenteric liposarcoma

Liposarcoma arising from the mesentery of the bowel is a rare lesion. Some of the most common presenting symptoms of primary mesenteric liposarcoma is increasing abdominal girth, weight loss, abdominal pain, abdominal discomfort with meals and the presence of a freely movable abdominal mass or masses. Our patient presented with a large intra-abdominal mass. Diagnostic laparoscopy revealed tumor confined to the mesentery of the ileum. Laparoscopy was attempted, though conversion was necessary to achieve negative margins. Laparotomy was required to resect the tumour with wide margins. Myxoid and well-differentiated types of liposarcoma are by far the most common histological type. Tumor size greater than 20cm predict significantly poorer prognosis. The treatment of choice for primary mesenteric liposarcoma is surgical resection with clear margins. Radiotherapy or systemic chemotherapy has no benefit in increasing long-term survival. Laparoscopy is of limited value