Liposarcoma gigante mediastinal. Cirugía radical con abordaje Clamshell y manejo en la Unidad de Cuidados Críticos / Giant Mediastinal Liposarcoma. Radical Surgery with Clamshell Approach and Management in Critical Care Unit

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.

Parosteal osteosarcoma with low grade chondrosarcoma and liposarcoma components. A rare histologic variant. Case report and literature review.

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.

Primary hepatic pleomorphic liposarcoma: Case report and literature review.

Primary hepatic liposarcoma is an extremely rare malignant tumour derived from adipocytes and is part of the group of mesenchymal tumours. We present the case of a 43-year-old Hispanic male patient with a pleomorphic hepatic liposarcoma and absence of MDM2 gene amplification. Two years and six months after surgery, the patient is asymptomatic. The present case is the first report of this entity with positive immunohistochemical testing for p16, p53, S100, vimentin and absence of MDM2 gene amplification.

Multivisceral resection for retroperitoneal liposarcoma-is it worth it? A 20-year single-center experience.

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.

Prognostic factors in patients receiving surgery and radiation therapy for retroperitoneal sarcoma: A machine-learning analysis.

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.

Pelvic soft tissue sarcomas.

Pelvic soft tissue sarcomas (PSTS) are a rare, heterogeneous group of tumors. They have been usually analyzed with retroperitoneal sarcomas (RPS), but actually have key differences. Due to their unique anatomic location, symptomatic presentation of PSTS may be more common than RPS. Adequate imaging approach is paramount for guiding differential diagnosis, while preoperative biopsy is mandatory, especially when preoperative treatment may be considered as initial approach. The most frequent histologic subtype is leiomyosarcoma, which is different as expected in the retroperitoneum where liposarcoma is the commonest histology. Also solitary fibrous tumor is commonly diagnosed in the pelvis. Surgical approach for PSTS differs from that for RPS mainly due to anatomic relations. Similarly, in the lack of definite evidence from specific trials about neoadjuvant and adjuvant treatments, the anatomic constraints to obtain wide margins in the pelvis as well as the expected functional outcome in case of organ resections should be factored into decision for individualized treatment offer. Vascular and genitourinary involvement are frequent, as well as herniation through pelvic foramina. For these reasons a multidisciplinary surgical team should always be considered. Early referral of these patients to high-volume centers is critical and may impact on survival, given that optimal initial resection is a major predictor of curative treatment. International consensus on PSTS treatment is advocated, similarly to the recent efforts realized for RPS.

Lipossarcoma Retroperitoneal Gigante: Relato de Caso / Giant Retroperitoneal Liposarcoma: Case Report / Liposarcoma Retroperitoneal Gigante: Informe de Caso

Introdução: Os lipossarcomas são tumores malignos raros de origem mesenquimal, a partir de precursores de adipócitos, podendo ocorrer no retroperitônio. Os lipossarcomas retroperitoneais representam de 0,3% a 0,6% das neoplasias malignas. Em virtude do crescimento lento e assintomático do tumor, tendo como principal manifestação clínica o aumento do volume abdominal, o quadro é comumente acompanhado de um diagnóstico tardio e curso indolente. Relato do caso: Homem, 70 anos de idade, com aumento progressivo abdominal há cerca de dois anos. A ressonância magnética demonstrou uma formação em topografia retroperitoneal à direita, promovendo importante desvio das estruturas da linha média, com presença de conteúdo sólido acima da estrutura supradescrita. Foi submetido à laparotomia xifopúbica, com apresentação de massa gigante retroperitoneal que ocupava todo abdome, com deslocamento das alças intestinais e rim direito. Foram realizadas ressecção do tumor em bloco, rafia diafragmática e colecistectomia. Após três meses do primeiro procedimento cirúrgico, o paciente apresentou novamente aumento do volume abdominal, com confirmação de recidiva tumoral após realização de exames de imagem. Um novo procedimento cirúrgico foi realizado, revelando tumor com cápsula rompida e múltiplos coágulos. Após alta hospitalar, foi submetido a sessões de radioterapia complementares à cirurgia. Conclusão: A rápida reapresentação do paciente após o surgimento da recidiva, comum nesses casos, foi essencial para a redução de resíduos na segunda cirurgia, evidenciando a importância de exames periódicos para o reconhecimento precoce da recorrência local. No presente caso, também foram realizadas sessões de radioterapia, com a finalidade de evitar a recidiva, sem sucesso.

Introduction: Liposarcomas are rare malignant tumors of mesenchymal origin, from adipocyte precursors, which may occur in the retroperitoneum. Retroperitoneal liposarcomas account for 0.3% to 0.6% of malignant neoplasms and the main symptom is increased abdominal volume. Due to the slow and asymptomatic growth of the tumor, the condition is commonly associated with late diagnosis and indolent course. Case report: 70-year-old man with progressive abdominal enlargement for about two years. Magnetic resonance imaging demonstrated a formation in the right retroperitoneal topography, promoting significant deviation of the midline structures. Furthermore, there was a solid content above the structure described above. A xiphopubic laparotomy was performed with presentation of giant retroperitoneal mass occupying the entire abdomen, displacing the intestinal loops and the right kidney. En bloc tumor resection, diaphragmatic raffia and cholecystectomy were performed. Three months after the first surgical procedure, the patient presented a novel increase of the abdominal volume which imaging tests showed tumor recurrence. A new surgical procedure was performed, revealing a tumor with a ruptured capsule and multiple clots. After hospital discharge, the patient was submitted to complementary radiotherapy Conclusion: The fast return of the patient after the recurrence, common in these cases, was essential to reduce the residues of the second surgery, highlighting the importance of periodic examinations for the early recognition of local recurrence. In this case, radiotherapy sessions were also performed to prevent recurrence, however, unsuccessful.

Introducción: Los liposarcomas son tumores malignos raros de origen mesenquimatoso, a partir de precursores de los adipocitos, y pueden presentarse en el retroperitoneo. Los liposarcomas retroperitoneales representan del 0,3% al 0,6% de las neoplasias malignas, siendo el síntoma principal el aumento de volumen abdominal. Debido al crecimiento lento y asintomático del tumor, la condición se acompaña comúnmente de un diagnóstico tardío y un curso indolente. Informe del caso: Varón, 70 años, con agrandamiento abdominal progresivo de unos 2 años de evolución. La resonancia magnética nuclear mostró una formación en la topografía retroperitoneal hacia la derecha, promoviendo una desviación significativa de las estructuras de la línea media. Además, se observó un contenido sólido por encima de la estructura descrita anteriormente. Considerando la principal hipótesis diagnóstica, liposarcoma de retroperitoneo, se realizó laparotomía, evidenciándose una masa retroperitoneal gigante que ocupaba todo el abdomen, que desplazaba las asas intestinales y el riñón derecho. Se realizó disección y resección del tumor en bloque para extirpar la masa tumoral, así como rafia diafragmática y colecistectomía. A los tres meses del primer acto quirúrgico, el paciente volvió a presentar aumento de volumen abdominal, en el que las pruebas de imagen mostraron recidiva tumoral. Se realizó un nuevo procedimiento quirúrgico que reveló un tumor con una cápsula rota y múltiples coágulos. Conclusión: El rápido retorno del paciente tras el inicio de la recidiva, frecuente en estos casos, fue fundamental para reducir los residuos en la segunda cirugía, destacando la importancia de las exploraciones periódicas para el reconocimiento precoz de la recidiva local. En este caso también se realizaron sesiones de radioterapia, con el objetivo de prevenir la recurrencia, sin éxito.

Lipossarcoma Bem Diferenciado do Retroperitônio com Desdiferenciação e Múltiplas Recidivas: Relato de Caso / Well-Differentiated Liposarcoma of the Retroperitoneum with Dedifferentiation and Multiple Recurrences: Case Report / Liposarcoma Bien Diferenciado de Retroperitoneo con Desdiferenciación y Múltiples Recurrencias: Informe de Caso

Introdução: Os lipossarcomas retroperitoneais são neoplasias mesenquimais raras, sendo mais comuns os bem diferenciados e os desdiferenciados. O subtipo bem diferenciado pode sofrer desdiferenciação para tumores de maior grau. São neoplasias difíceis de tratar cirurgicamente, pois apresentam altas taxas de recorrência local, alguns subtipos podem metastizar e são pouco sensíveis à radioterapia e à quimioterapia. Relato do caso: Paciente feminina, 45 anos, apresentou dor abdominal e massa abdominal palpável em 2017. Foi submetida à ressecção de lipossarcoma bem diferenciado de retroperitônio, sem intercorrências. Em 2020, manifestou dor abdominal e perda ponderal. A tomografia mostrou múltiplas massas volumosas abdominais, com biópsia sugestiva de lipossarcoma desdiferenciado. Foi submetida à radioterapia neoadjuvante e, em seguida, à ressecção cirúrgica das massas e ileocolectomia direita. Em 2022, apresentou quadro sugestivo de obstrução intestinal, sendo submetida à laparotomia que evidenciou intenso bloqueio de alças intestinais, fístula duodenal, tumor retroperitonial e peritonite fecal. Procedeu-se à ressecção de neoplasia retroperitoneal, ileostomia e rafia de fístula. O histopatológico mostrou lipossarcoma desdiferenciado recidivado. A paciente evoluiu com complicações operatórias e infecciosas, necessitando de cuidados intensivos e antibioticoterapia. Após melhora clínica, recebeu alta com dieta enteral e segue em acompanhamento ambulatorial. Conclusão: O lipossarcoma de retroperitônio pode sofrer desdiferenciação, recidivas multifocais e múltiplas recorrências, necessitando de várias abordagens cirúrgicas, o que aumenta a morbidade e o risco de complicações. A cirurgia com margens amplas continua sendo a principal modalidade terapêutica.

ABSTRACT Introduction: Retroperitoneal liposarcomas are rare mesenchymal neoplasms, with well-differentiated and dedifferentiated liposarcomas being most common. The well differentiated subtype can undergo dedifferentiation to higher grade tumors. These are difficult neoplasms to treat surgically because they have high rates of local recurrence, some subtypes can metastasize, and are poorly responsive to radiotherapy and chemotherapy. Case report: Female patient, 45 years old, presented abdominal pain and palpable abdominal mass in 2017. She underwent resection of well-differentiated liposarcoma of the retroperitoneum, without intercurrences. In 2020, she manifested abdominal pain and weight loss. Tomography showed multiple voluminous abdominal masses, with biopsy suggestive of dedifferentiated liposarcoma. The patient was submitted to neoadjuvant radiotherapy, followed by surgical resection of the masses and right ileocolectomy. In 2022, she presented symptoms suggestive of intestinal obstruction, and underwent laparotomy that revealed intense blockage of intestinal loops, duodenal fistula, retroperitoneal tumor, and fecal peritonitis. Retroperitoneal neoplasm resection, ileostomy and fistula closure were performed. Histopathology showed relapsed dedifferentiated liposarcoma. The patient evolved with operative and infectious complications, requiring intensive care and antibiotic therapy. After clinical improvement, the patient was discharged with enteral diet and continues under outpatient follow-up. Conclusion: Retroperitoneal liposarcoma may undergo multifocal dedifferentiation and recurrence, requiring several surgical approaches, increasing morbidity and the risk of complications. Wide margin surgery remains the main therapeutic modality.

Introducción: Los liposarcomas retroperitoneales son neoplasias mesenquimatosas raras, siendo los más comunes los liposarcomas bien diferenciados y desdiferenciados. El subtipo bien diferenciado puede sufrir desdiferenciación hacia tumores de mayor grado. Estas neoplasias son difíciles de tratar quirúrgicamente porque presentan altas tasas de recidiva local, algunos subtipos pueden hacer metástasis y responden mal a la radioterapia y la quimioterapia. Informe del caso: Mujer de 45 años, en 2017 presenta dolor abdominal y masa abdominal palpable. Fue sometida a la resección de un liposarcoma bien diferenciado del retroperitoneo, sin intercurrencias. En 2020, manifestó dolor abdominal y pérdida de peso. La tomografía mostró múltiples masas abdominales voluminosas, con biopsia sugestiva de liposarcoma desdiferenciado. Fue sometida a radioterapia neoadyuvante y luego a resección quirúrgica de las masas y a ileocolectomía derecha. En 2022, presentó síntomas de obstrucción intestinal y fue sometida a una laparotomía que reveló obstrucción de las asas intestinales, fístula duodenal, tumor retroperitoneal y peritonitis fecal. Se realizó la resección de la neoplasia retroperitoneal, la ileostomía y la fistulización. La histopatología mostró un liposarcoma desdiferenciado. La paciente evolucionó con complicaciones operatorias e infecciosas, requiriendo cuidados intensivos y terapia antibiótica. Tras la mejora clínica, la paciente fue dada de alta con dieta enteral y está en seguimiento. Conclusión: El liposarcoma retroperitoneal puede sufrir desdiferenciación multifocal y recurrencia, requiriendo varios a tratamientos quirúrgicos, aumentando la morbilidad y el riesgo de complicaciones. La cirugía con márgenes amplios sigue siendo la terapia principal.

Giant retroperitoneal liposarcoma surgical management.

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.

Giant retroperitoneal liposarcoma surgical management.

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.

Lipossarcoma pleomórfico esplênico primário em uma cadela / Primary splenic pleomorphic liposarcoma in a bitch

Background: Liposarcoma is a malignant neoplasm of lipoblasts with low incidence in dogs, representing 1.7% of tumorsdiagnosed in the spleen. In veterinary medicine, this neoplasm is classified morphologically into the myxoid, well-differentiated, undifferentiated and pleomorphic subtypes, the latter being one of the most aggressive forms, mainly in cavityorgans. This report refers to a primary splenic pleomorphic liposarcoma in a female dog, addressing anatomopathologicaland immunohistochemical aspects.Case: A 14-year-old, 35 kg bitch mongrel with history of absence of defecation, progressive weight loss, difficulty walking, sensitivity to abdominal palpation, prostration, pale mucous membranes, tachypnea and abdominal distention waspresented to diagnosis. The condition evolved to death and, on necroscopy, there was an increase in splenic volume withneoformation of whitish and reddish color, measuring 32 × 27 cm in its largest axes and weighing 8.9 kg. The neoformationexhibited areas of firm and soft consistency, and sectioning revealed focal areas of extensive necrosis and cavity collectionsof different diameters that allowed the flow of liquid serous contents with a brownish red color. Microscopy showed cellsof neoplastic morphology infiltrating the splenic parenchyma, mostly with slightly acidophilic cytoplasm and few intracytoplasmic lipid vacuoles, which varied in size and distribution. The nucleus of the cells was large, eccentric and irregular,with round to oval morphology, grossly lacy chromatin and single or multiple evident nucleoli. These cells exhibited markedanisocytosis, anisokaryosis and pleomorphism, with more than one mitotic figure per high magnification field visible. Mildinflammatory infiltrate, predominantly lymphocytic, permeated the neoplastic cells, and marked depletion of lymphoidfollicles and atrophy of the red pulp were found in the remaining splenic parenchyma. Immunohistochemical tests...(AU)

Clinical Application of Chromosome Microarray Analysis in the Diagnosis of Lipomatous Tumors.

Well-differentiated liposarcoma/atypical lipomatous tumor (WDLS/ALT) and dedifferentiated liposarcoma (DDLS) have characteristic supernumerary ring and giant marker chromosomes involving the chromosomal region 12q13-15 which contains MDM2 (12q15), CDK4 (12q14.1), HMGA2 (12q14.3), YEATS4 (12q15), CPM (12q15), and FRS2 (12q15). Detecting MDM2 amplification by fluorescence in situ hybridization (FISH) is considered to be the gold standard for the diagnosis of WDLS/ALT and DDLS. In this study, formalin fixed paraffin embedded clinical specimens (16 liposarcomas and 19 benign lipomatous tumors) were used to detect MDM2 amplification and other chromosomal alterations in WDLS/ALT and DDLS by single nucleotide polymorphism-based chromosome microarray (CMA). All 16 liposarcomas showed MDM2 amplification with a MDM2/cep12 ratio from 2.4 to 8.4 by CMA. Ten (62.5%) of these cases had CDK4/cep12 ratio ≥2.0. All the cases without CDK4 amplification were from the thigh. The MDM2/cep12 ratio of all the benign lipomatous tumors (19/19) was within the normal limits. Twenty-one of the 35 benign lipomatous tumors and liposarcomas were also tested for MDM2 amplification by FISH. All the FISH results were consistent with the CMA results (100%). Along with MDM2 amplification, all 16 liposarcomas (100%) also showed amplification of YEATS4, CPM and FRS2. Only 11 of 16 (69%) cases showed HMGA2 amplification. In conclusion, this study demonstrated that CMA on routine formalin fixed paraffin embedded tissue is a sensitive and specific clinical test for detection of MDM2 gene amplification. Moreover, CMA allows simultaneous detection of genomic changes of interest including CDK4 and others, which provides enriched information for diagnosing lipomatous tumors.

Long-Term Survival of a Patient with Recurrent Dedifferentiated High-Grade Liposarcoma of the Retroperitoneum Under Adjuvant Treatment with Viscum album L. Extract: A Case Report.

CASE PRESENTATION: A 68-year-old male patient underwent a resection of a DDL (T2 N0 M0, FNCLCC grade 2, stage IIIA) in the retroperitoneum. Three months after this first surgery, a recurrence occurred, and was treated with neoadjuvant and adjuvant doxorubicin plus ifosfamide and surgery (resection). A second recurrence-11 months after the second surgery-was treated with surgery and radiotherapy. The patient then began to undergo VAE treatment (0.2 mg-2 mg, subcutaneously, thrice a week). After the VAE treatment was initiated, the patient reported improved quality of life. A third recurrence-12 months after the third surgery-was treated with surgery, radiotherapy, and with an increased dose of VAE (20 mg). Sixty-nine months (5.8 years) after the fourth surgery a fourth recurrence occurred. It was again treated with surgery, along with a month of intravenous VAE infusions and subsequent subcutaneous VAE (20 mg) treatment. Finally, a fifth recurrence-5 months after the fifth surgery-was treated with subcutaneous and intravenous VAE applications and eribulin. The patient died 11 months after the last recurrence; he received a total of 103 months (8.6 years) of VAE treatment and achieved 10.5 years of survival. CONCLUSION: The case presented herein shows a long-time survival and a prolonged recurrence-free interval in a patient with retroperitoneal DDL treated with surgery, chemotherapy, radiotherapy, and VAE injections. On the basis of the antitumoral and immunomodulating effects of VAE and on the reported prolonged survival of VAE-treated patients with other types of tumors, the adjunct VAE treatment is presumed to have contributed to the favorable outcome. Regarding the clinical relevance of VAE treatment, further investigations are needed.

Resection of a giant retroperitoneal lipoma herniating through the inguinal canal.

Retroperitoneal lipomas are extremely rare with few cases reported so far in the literature. They can reach different sizes and present with a variety of symptoms. The differential diagnosis is mainly with well-differentiated liposarcoma (WDLPS). We present a 34-year-old woman with a retroperitoneal lipoma herniating through the inguinal canal into the proximal thigh. The patient underwent complete oncological resection using a Karakousis's abdominoinguinal incision. Retroperitoneal lipomas are a very rare condition and sometimes require resections technically challenging. MDM2 amplification is critical for its differential diagnosis with WDLPS.

Lipossarcoma pleomórfico esplênico primário em uma cadela / Primary splenic pleomorphic liposarcoma in a bitch

Background: Liposarcoma is a malignant neoplasm of lipoblasts with low incidence in dogs, representing 1.7% of tumorsdiagnosed in the spleen. In veterinary medicine, this neoplasm is classified morphologically into the myxoid, well-differentiated, undifferentiated and pleomorphic subtypes, the latter being one of the most aggressive forms, mainly in cavityorgans. This report refers to a primary splenic pleomorphic liposarcoma in a female dog, addressing anatomopathologicaland immunohistochemical aspects.Case: A 14-year-old, 35 kg bitch mongrel with history of absence of defecation, progressive weight loss, difficulty walking, sensitivity to abdominal palpation, prostration, pale mucous membranes, tachypnea and abdominal distention waspresented to diagnosis. The condition evolved to death and, on necroscopy, there was an increase in splenic volume withneoformation of whitish and reddish color, measuring 32 × 27 cm in its largest axes and weighing 8.9 kg. The neoformationexhibited areas of firm and soft consistency, and sectioning revealed focal areas of extensive necrosis and cavity collectionsof different diameters that allowed the flow of liquid serous contents with a brownish red color. Microscopy showed cellsof neoplastic morphology infiltrating the splenic parenchyma, mostly with slightly acidophilic cytoplasm and few intracytoplasmic lipid vacuoles, which varied in size and distribution. The nucleus of the cells was large, eccentric and irregular,with round to oval morphology, grossly lacy chromatin and single or multiple evident nucleoli. These cells exhibited markedanisocytosis, anisokaryosis and pleomorphism, with more than one mitotic figure per high magnification field visible. Mildinflammatory infiltrate, predominantly lymphocytic, permeated the neoplastic cells, and marked depletion of lymphoidfollicles and atrophy of the red pulp were found in the remaining splenic parenchyma. Immunohistochemical tests...

Inflammatory Variant of Atypical Lipomatous Tumor/Well-Differentiated Liposarcoma of the Buccal Mucosa: An Overview and Case Report with a 10-Year Follow-Up.

Liposarcomas of the oral cavity are rare. Those originating in the buccal mucosa cause challenging diagnostic and therapeutic issues since less than 40 cases of liposarcomas of the buccal mucosa and cheek have been reported in the worldwide literature. Herein, we present a case of atypical lipomatous tumor/well-differentiated liposarcoma affecting a 45-year-old female patient. Ultrasonography and magnetic resonance imaging confirmed a well-defined mass located in the right buccal mucosa, extending to the submucosal layers of the cheek. Histopathologically, a well-differentiated fatty neoplasm with presence of prominent stromal inflammatory cells was observed. Multifocally scattered bizarre hyperchromatic stromal cells, some of which multinucleated, were also observed. An immunohistochemical panel comprising vimentin, S-100, CD10, CD34, CD20, CD3, CD68, CD138, MDM2, Ki-67, and P53 was employed to better characterize the lesion. A local recurrence event occurred during a 10-year follow-up period. Surgical resection was performed during both episodes. We also provided an overview of demographic and clinicopathological characteristics, immunohistochemical features, imaging findings, and the differential diagnosis of liposarcoma of the oral cavity. Knowledge of the etiopathological and clinical aspects of this rare neoplasm is fundamental in order to rule out other conditions, including lipomatous lesions that affect the buccal mucosa.

Retroperitoneal liposarcoma in a nonagenarian

Retroperitoneal liposarcomas are rare tumors arising from the soft tissue of the retroperitoneum and are of mesenchymal cell origin. They can reach a large size prior to causing symptoms and generally have a poor prognosis. We present the case of a 93-year-old lady presenting with a large retroperitoneal liposarcoma at the site of a previous colonic anastomosis for the adenocarcinoma treatment. It caused minimal symptoms initially, but surgical resection was undertaken when the tumor was found to be growing significantly in size. However, due to the tumor's location and its invasion into surrounding structures, the resection was not feasible and subsequently abandoned. A retroperitoneal liposarcoma arising from the site of a previous colonic resection has not been previously described. A review of the diagnosis and current management of these lesions is also given.

Primary retroperitoneal tumor during pregnancy: Case report and review of the literature / Tumor retroperitoneal primario durante el embarazo: reporte de un caso y revisión de la literatura

OBJECTIVE: To report the case of a pregnant patient diagnosed with a primary retroperitoneal tumor and to conduct a review of the literature pertaining to the diagnosis, treatment and maternal-fetal prognosis of this disease condition during pregnancy. METHODS: A 19-year-old patient who presented with a retroperitoneal tumor identified on ultrasound. The results of the percutaneous biopsy showed a benign tumor. A healthy neonate was delivered by cesarean section. Surgical resection was performed four months later, and histopathology showed a mature cystic teratoma. A search was conduced in the Medline via PubMed, Lilacs, SciELO and ScienceDirect databases using the terms "pregnancy," "neoplasms," and "retroperitoneal neoplasms," including case reports and case series of retroperitoneal tumors during pregnancy. Diagnosis, treatment and maternal-fetal prognosis were identified. RESULTS: Overall, 1658 titles were identified. Of these, 34 case reports and 1 case series met the inclusion criteria. Of the cases, 62.9 % were benign. Diagnosis was made as an incidental finding in 63 % of cases, and 77 % of the masses were identified on ultrasound. Percutaneous biopsy was used in 8 % of cases, including the case reported here. Surgical treatment was used in 88 % of cases usually after delivery. Maternal mortality occurred in 8.5 % of cases. Fetal prognosis was good in 65 % of the pregnancies. CONCLUSIONS: Despite being frequently benign, retroperitoneal tumors during gestation have a reserved maternal and fetal prognosis in a substantial number of cases. There is a need to assess the risks and benefits of percutaneous biopsy.

TITULO: TUMOR RETROPERITONEAL PRIMARIO DURANTE EL EMBARAZO: REPORTE DE UN CASO Y REVISIÓN DE LA LITERATURA. OBJETIVO: Reportar el caso de una paciente gestante con diagnóstico de tumor retroperitoneal primario y hacer revisión de la literatura del diagnóstico, tratamiento y pronóstico materno-fetal de esta entidad durante el embarazo. METODOS: Gestante de 19 años que consulta por tumor retroperitoneal identificado por ecografía, se realizó biopsia percutánea con resultado de tumor benigno; parto por cesárea con recién nacido sano. A los 4 meses se realizó resección quirúrgica, la histopatología mostró un teratoma quístico maduro. Se realizó búsqueda en las bases de datos de: Medline vía PubMed, Lilacs, SciELO y ScienceDirect, con los términos: "embarazo", "neoplasias" y "neoplasias retroperitoneales", incluyendo reportes y series de caso de tumores retroperitoneales en el embarazo. Se identificó el diagnóstico, tratamiento y pronóstico materno-fetal. RESULTADOS: Se identificaron 1.658 títulos, de los cuales 34 reportes de casos y una serie de casos cumplieron con los criterios de inclusión. El 62,9 % eran tumores benignos. El diagnóstico se hizo como hallazgo incidental en el 63 % de los casos, el ultrasonido identificó la masa en el 77 %, la biopsia percutánea se utilizó en el 8 % de los casos incluyendo el reportado. El tratamiento quirúrgico fue utilizado en el 88 % de los casos, generalmente después del parto. Hubo mortalidad materna en el 8,5 % de los casos. El pronóstico fetal fue bueno en el 65 % de las gestaciones. CONCLUSIONES: Los tumores retroperitoneales en la gestación, a pesar de ser principalmente benignos, tienen un pronóstico materno y fetal reservado en un importante número de casos. Se requiere evaluar los beneficios y riesgo de la biopsia percutánea.

Liposarcoma of oral cavity: Systematic review of cases reported to date and analysis of prognostic factors.

The aim of this study was to integrate the available data published on Liposarcomas (LPSs) of the oral cavity into an analysis of its clinical features, treatment modalities, and prognostic factors. An electronic search was undertaken in January 2019. The eligibility criteria included publications that had enough clinical and histological information to confirm the diagnosis. Forty-five publications (104 LPSs) were included. The lesion was more prevalent in males from the fifth to seventh decades of life. Treatment (P = .03) and distant metastasis (P = .0001) were independently associated with survival. A lower possibility of recurrence was statistically associated with age (younger patients) (P = .03), tumor size (smaller than 2.8 cm) (P = .001), and treatment (radical surgery) (P = .04). LPS presents a good survival for patients after 5 years of follow-up (66.4%). Patients who were treated with conservative surgery and presented with distant metastasis showed poor prognosis.