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.

Clinical value of fluorescence in situ hybridization with MDM2 and DDIT3 probe in diagnosis of liposarcoma / 北京大学学报(医学版)

OBJECTIVE@#To investigate the value of using MDM2 amplification probe and DDIT3 dual-color, break-apart rearrangement probe fluorescence in situ hybridization (FISH) technique in the diagnosis of liposarcoma.@*METHODS@#In the study, 62 cases of liposarcoma diagnosed in Peking University First Hospital from January 2015 to December 2019 were analysed for clinicopathological information. Of these 62 cases of liposarcoma, all were analysed for MDM2 amplification and 48 cases were analysed for DDIT3 rearrangement using a FISH technique. Our study aimed to evaluate the status of MDM2 and DDIT3 by FISH in liposarcoma and correlate it with diagnosis of different subtypes of liposarcoma. The subtypes of liposarcoma were classified according to the FISH results, combined with the relevant clinicopathological features.@*RESULTS@#The patients aged 31-89 years (mean: 59 years) with a 1.75:1 male to female ratio. Histologically, there were 20 cases of atypical lipomatous tumour/well-differentiated liposarcoma (ALT/WDLPS), 26 cases of dedifferentiated liposarcoma (DDLPS), 13 myxoid liposarcoma (MLPS) and 3 pleomorphic liposarcoma (PLPS). Tumors with DDLPS (23/26) and WDLPS (8/20) were localized retroperitoneally, while both tumours of MLPS and PLPS were localized extra-retroperitoneally, and the difference of sites among the four subtypes of liposarcoma was statistically significant (P < 0.05). Histologically, varied mucoid matrix could be observed in the four subtypes of liposarcoma, and the difference was statistically significant (P < 0.05). MDM2 gene amplification was demonstrated in all cases of ALT/WDLPS and DDLPS (100%, 20/20 and 26/26 respectively); DDIT3 gene rearrangement was noted only in MLPS (100%, 13/13); most cases of DDLPS (96.2%, 25/26) and ALT/WDLPS (83.3%, 5/6, 6 cases selected for detection) demonstrated the picture of amplification of the DDIT3 telomeric tag. According to the instructions of DDIT3 break-apart rearrangement probe, the 5' telomere probe and 3' centromere probe spanned but did not cover the DDIT3 gene itself, on the contrary, the 5' telomere probe covered the CDK4 gene, while the DDIT3 and CDK4 gene were located adjacent to each other on chromosome, therefore, when the amplification signal appeared on the telomeric tag of the DDIT3 rearrangement probe, it indeed indicated the CDK4 gene amplification rather than the DDIT3 gene rearrangement. Then the 10 cases with DDIT3 telomeric tag amplification were selected for CDK4 and DDIT3 gene amplification probe FISH tests, and all the cases showed CDK4 gene amplification (100%, 10/10) and two of the 10 cases demonstrated co-amplification of CDK4 and DDIT3 (20%, 2/10); DDIT3 polysomy detected by DDIT3 gene rearrangement probe was found in 1 case of DDLPS and 2 cases of PLPS (66.7%, 2/3) with morphology of high-grade malignant tumour and poor prognosis.@*CONCLUSION@#Our results indicate that a diagnosis of different subtype liposarcoma could be confirmed based on the application of MDM2 and DDIT3 FISH, combined with clinicopathological findings. It is also noteworthy that atypical signals should be correctly interpreted to guide correct treatment of liposarcomas.

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.

Diagnostic value of MDM2 RNA in situ hybridization in atypical lipomatous tumor/well-differentiated liposarcoma and dedifferentiated liposarcoma / 中华病理学杂志

Objective: To investigate the value of MDM2 RNA in situ hybridization (RNA-ISH) in diagnosing atypical lipomatous tumor/well-differentiated liposarcoma (ALT/WDL) and dedifferentiated liposarcoma (DDL). Methods: A total of 26 ALT/WDL/DDLs diagnosed from March 2017 to May 2019 in West China Hospital, Sichuan University, Chengdu, China and 18 control cases were included. MDM2 RNA-ISH was performed on all samples and compared with the fluorescence in situ hybridization (FISH) and immunohistochemistry (IHC) regarding their performance in detecting MDM2. Results: All samples were detected successfully using the three methods. Among 26 ALT/WDL/DDLs, all cases showed MDM2 amplification and positivity for MDM2 RNA-ISH (26/26, 100%). Twenty-four (24/26, 92.3%) of the 26 tested cases were positive for MDM2 IHC while two of them were negative. Eighteen control cases were all negative for MDM2 FISH and RNA-ISH, and 15 (15/18) cases were negative for MDM2 IHC. The sensitivity and specificity of RNA-ISH were both 100%, and those of MDM2 IHC were 92.3% and 83.3%, respectively. Diffuse staining was identified in all MDM2 RNA-ISH positive ALT/WDL/DDLs, but identified in only 8/24 (33.3%) of the MDM2 IHC positive cases. Among the 11 ALT/WDL/DDL samples evaluated on tissue microarray, the positive rate of MDM2 RNA-ISH was 100% with diffuse staining in all cases. The positive rate of MDM2 IHC was 9/11 while only 1 of the 9 cases showed diffuse staining. The result of MDM2 RNA-ISH was identical to that of MDM2 FISH and was overall consistent with that of MDM2 IHC (Kappa=0.763, P<0.001). Conclusions: In ALT/WDL/DDLs, results of MDM2 RNA-ISH are highly consistent with those of FISH. MDM2 RNA-ISH is more sensitive and more specific and has more diffuse positive signals than the IHC. The findings indicate that MDM2 RNA-ISH is highly valuable for the diagnosis and differential diagnosis of ALT/WDL/DDLs.

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.

Decoded: A case report on dedifferentiated Liposarcoma on the Gluteal area

@#Dedifferentiated liposarcoma is a soft tissue sarcoma of adipocytic lineage. Histopathology and immunohistochemistry are essential for diagnosis. A 51-year-old Filipino woman presented with a rapidly enlarging left gluteal tumor. Histopathology revealed a multilobulated tumor having prominent myxoid stroma with numerous stellate-shaped, atypical cells bearing atypical mitotic figures. Other lobules were composed of sheets of pleomorphic cells, with atypical mitotic figures. The tumor stained positively with alcian blue, vimentin, MDM2 and p16 stains. Other immunohistochemical (IHC) studies done (pancytokeratin, CK7, CK 20, CD 34, CEA, desmin, EMA, SMA, S100) showed negative results. After a 2 cm wide excision of the sarcoma, patient was free from local tumor recurrence for 2 months, after which she was lost to follow-up. We report this case and a brief review of the current literature on dedifferentiated liposarcoma.

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.

Abdominal Actinomycosis misdiagnosed as liposarcoma

Actinomycosis is an uncommon, endogenous, and chronic infection with varied and nonspecific clinical features such as abdominal, pelvic or cervical masses, ulcerative lesions, abscesses, draining fistula, fibrosis, and constitutional symptoms. The disease ensues when the bacteria disrupt the mucosal barrier, invade, and spread throughout interfascial planes. Currently, the diagnosis of actinomycosis is challenging because of its very low frequency and depending on the clinical presentation it may masquerade malignancies. Therapy consists initially in intravenous penicillin, followed by an oral regimen that may be extended until a year of treatment. A timely diagnosis is crucial to avoid extensive therapeutic attempt as surgery. However, a biopsy or drainage of abscesses and fistula's tract may be required not only as a diagnostic procedure as part of the therapy. We report the case of a 72-year-old woman with an abdominal mass initially misdiagnosed as a liposarcoma. A second biopsy of a skin lesion of the abdominal wall made the diagnosis of actinomycosis, avoiding a major surgical procedure. The patient was treated with a long-term course of antibiotics with favorable outcome. Liposarcoma was ruled out after the patient's full recovery with antibiotics and the misdiagnosis was credit to the overconfidence on the immunohistochemical positivity to MDM2.

Liposarcoma retroperitoneal gigante de novo multifocal / Novo multifocal giant regression return liposarcoma

Los sarcomas de tejidos blandos son raros y representan menos del 1% de las neoplasias malignas recién diagnosticadas. La rareza de los tumores retroperitoneales, combinada con las variedades de subtipos histológicos, ha complicado los conocimientos sobre estos e impedido el desarrollo de terapia efectivas. Los liposarcomas retroperitoneales crecen lenta y silenciosamente, su pronóstico es malo y el tratamiento de elección es la resección quirúrgica. Se expone el caso de paciente femenino de 50 años de edad con el diagnóstico de ingreso de tumor abdominal, operada hace 39 meses por la misma causa, refiriendo liposarcoma tipo mixoide grado II, se realizó una laparotomía y se identificó masa tumoral retroperitoneal gigante con un peso de 6 100 gr. y se establece el diagnostico de tumor lipomatoso atipico/ liposarcoma bien diferenciado. Se informa este caso por la rareza de su conformación y la ausencia de reportes en la literatura nacional, con escasas patologías de similares características de presentación a nivel mundial.(AU)

Soft tissue sarcomas are rare and represent less than 1% of newly diagnosed malignancies. The rarity of retroperitoneal tumors, combined with the varieties of histological subtypes, has complicated the knowledge about these and impeded the development of effective therapy. Retroperitoneal liposarcomas grow slowly and silently, their prognosis is bad and the treatment of choice is surgical resection. We present the case of a female patient of 50 year old with a diagnosis of admission of an abdominal tumor, operated 39 months ago for the same cause, referring grade II myxoid-type liposarcoma, a laparotomy was performed and a giant retroperitoneal tumor was identified a weight of 6 100 gr. and the diagnosis of atypical lipomatous tumor / well differentiated liposarcoma is established. This case is reported due to the rarity of its conformation, the absence of reports in the national literature with few pathologies of similar presentation characteristics worldwide.(AU)

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

Primary colonic liposarcomatosis: report of a case with review of literature

The colon is a rare site of occurrence of liposarcoma, as either the primary site or by secondary involvement from a retroperitoneal liposarcoma. Liposarcomatosis denotes simultaneous occurrence of multiple liposarcomas. There are only 17 cases of primary colonic liposarcoma reported in the English literature­one of which was primary colonic liposarcomatosis. We depict the second case of primary colonic liposarcomatosis in a 57-year-old female who presented with abdominal swelling and pain. On exploratory laparotomy, two large masses were seen arising from the wall of the right colon along with multiple smaller masses attached to the colon. Right hemicolectomy with en bloc excision of the masses was performed along with hysterectomy and pelvic floor repair. Macroscopically, multiple exophytic masses and one endophytic mass were identified. The exophytic masses were of variable size and were found to hang from the colon by a thin pedicle simulating variable-sized appendices epiploicae. Histopathologically, the lesions showed the morphology of well-differentiated liposarcoma. This appears to be a case of primary colonic liposarcomatosis. There is only one other similar case reported in the English literature, to the best of our knowledge.

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)

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.

PET/CT imaging manifestations of different pathological subtypes of liposarcoma / 浙江大学学报·医学版

OBJECTIVE@#To analyze PET/CT imaging manifestations of different pathological subtypes of liposarcoma.@*METHODS@#The F-fluorodeoxyglucose(FDG) PET/CT features of 13 patients pathologically confirmed as liposarcoma were retrospectively reviewed. The metabolism degree and distribution of different subtypes of liposarcoma were compared.@*RESULTS@#The well-differentiated liposarcoma showed fat density mass with septa and irregular strip with mild FDG uptake. The myxoid liposarcoma showed cystic or cystic solid mass, single or multiple with mild-moderate FDG uptake heterogeneously or homogeneously. The dedifferentiated liposarcoma showed mixed soft tissue mass with high FDG uptake heterogeneously, larger lesion with necrosis centrally. The mixed type contained well differentiated type and dedifferentiated type, and showed multiple lesion with combined imaging manifestations. There were local invasions in 12 cases, no lymph node matastasis, and the recurrence of dedifferentiated liposarcoma with lung metastasis in 1 case. The maximum standard values (SUVmax) of FNCCLE grade G1, G2 and G3 liposarcoma were 3.00, 5.67 and 10.33, respectively; there was significant difference between G1 and G3 groups, G2 and G3 groups (all <0.05).@*CONCLUSIONS@#PET/CT manifestations of liposarcoma of various pathological subtypes are different. Preoperative PET/CT examination can clarify the pathological types, scope of tumor invasion and metastasis of liposarcoma, which provides more information for clinical decision-making and is helpful for the preparation of surgical plan.

A case of neck lipoblastoma in a pediatric patient / 대한두경부종양학회지

Lipoblastoma is a rare benign tumor with 80–90% occurring in children less than 3 years of age and 40% occurring in children less than 1 year of age. The most common site of incidence is limb, and then trunk. Neck is the rare site of incidence. The main symptom that the patient complains about is a rapidly growing neck mass without pain. When the size of mass increases, it can cause dyspnea, Horner's syndrome. Lipoblastoma is usually diagnosed as a lipoma in the fine needle aspiration. Since it is not differentiated from lipoma, liposarcoma, and hibernating adenoma in CT and MRI, the definitive diagnosis is histologic diagnosis through surgical resection. The treatment is complete surgical resection. And recurrence rate is 9–25% due to incomplete resection. Authors report this case with a review of literatures since we experienced a case of lipoblastoma diagnosed histopathologically after surgical treatment of neck mass.

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.