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.

Liposarcoma pleomórfico de Vulva - Reporte de Caso

El liposarcoma pleomórfico de vulva es extremadamente raro y hay pocos casos reportados en la literatura. Presentamos el primer caso del Instituto Oncológico Nacional en Panamá. Se trata de una mujer de 52 años con una tumoración vulvar de un año de evolución y de crecimiento lento. Se le toma una biopsia y es referida a nuestro Instituto. Se le realiza hemivulvectomia. La patología confirma liposarcoma pleomórfico con márgenes negativos. Tuvo una evolución postoperatoria favorable. El tratamiento de elección es quirúrgico. El tratamiento adyuvante es individualizado. (provisto por Infomedic International)

Pleomorphic liposarcoma of the vulva is extremely rare and there are few cases reported in the literature. We present the first case from the National Oncologic Institute in Panama. It is a 52-year-old woman with a slow-growing vulvar tumor of one year of evolution. A biopsy was taken, and she was referred to our Institute. A hemivulvectomy was performed. Pathology confirms pleomorphic liposarcoma with negative margins. Postoperative evolution was favorable. The treatment of choice is surgical. Adjuvant treatment is individualized. (provided by Infomedic International)

Bloqueo interescalénico en desarticulación de miembro torácico derecho por liposarcoma pleomórfico, para prevención de dolor de miembro fantasma. Reporte de caso / Interscalene block in right thoracic limb disarticulation due to pleomorphic liposarcoma, for the prevention of phantom limb pain. Case report

Resumen: El liposarcoma pleomórfico es una neoformación de tejidos blandos, que afecta principalmente las extremidades. Los pacientes que los presentan son candidatos a resección amplia o incluso amputación del miembro afectado. Como parte de una técnica anestésica integral y multimodal, destaca la utilización de bloqueos ecoguiados que disminuirán los requerimientos de opioides, teniendo un impacto importante en la preservación de la inmunidad del paciente oncológico, lo que le permitirá aumentar la tasa de supervivencia y su período libre de enfermedad; adicionando además un alfa 2 agonista, el cual nos brinda una adecuada analgesia postoperatoria, y realizando los bloqueos previos a la amputación, tenemos mayor tasa de éxito en la prevención del dolor y síndrome de miembro fantasma.

Abstract: Pleomorphic liposarcoma is a neoformation, which mainly affects the extremities. Being the patient candidate to perform a wide resection or even amputation of the affected limb. As part of a multimodal anesthetic technique, the use of ultrasound-guided blocks are useful, reducing opioid requirements, having an important impact on the preservation of the cancer patient's immunity, also adding an alpha 2 agonist, which provides adequate postoperative analgesia, and performing the blocks prior to amputation, we have a higher success rate in the prevention of pain and phantom limb syndrome.

Liposarcomas paratesticulares simultáneos con diferencias histopatológicas. Reporte de un caso

El liposarcoma es un tumor de origen mesenquimal que se presenta comúnmente en el retroperitoneo; es de crecimiento lento y su capacidad de producir metástasis depende de su tipo histológico. Este trabajo tiene como propósito presentar un caso de liposarcomas paratesticulares simultáneos con diferentes tipos histológicos, en un paciente masculino, de 80 años con antecedentes de salud y nivel socioeconómico bajo que venía presentando desde hacía dos años aumento de volumen en la región inguinal izquierda y testículo ipsilateral, al inicio fue indoloro; luego, a medida que aumentó de tamaño se tornó doloroso, abarcando la región inguinoescrotal derecha en su totalidad. El examen físico, incluido el signo de la transiluminación fue negativo. El estudio analítico mostró solamente velocidad de sedimentación globular en 95 mm/h y la LDH en 650 U/l; la ecografía testicular mostró una masa heterogénea, solida e hiperecoica, de aspecto graso inguinoescrotal y de más de 30 cm, sin llegarse a observar el testículo. Se realizó exéresis tumoral encontrándose tres tumores liposarcomatosos, dos de ellos con características histopatológicas similares y el otro con patrón histológico diferente. Luego del tratamiento quirúrgico el paciente se recuperó satisfactoriamente y no ha presentado recidiva según los controles periódicos realizados. Se consideró la publicación del caso pues los liposarcomas pueden llegar a confundirse a la exploración clínica con la presencia de una hernia inguinal de contenido graso con sintomatología similar a estas; por lo que debe hacerse una exploración clínica exhaustiva para realizar un correcto diagnóstico. El estudio histopatológico confirmó el diagnóstico de la enfermedad que, además, reveló la presencia de tres tumores liposarcomatosos y dos variantes histopatológicas del mismo, de lo cual no se encontraron reportes en la literatura revisada.

Liposarcoma is a mesenchymal origin tumor that commonly occurs in the retroperitoneum; It is slow growing and its ability to produce metastasis depends on its histological type. The purpose of this work is to present a case of simultaneous paratesticular liposarcomas with different histological types, in an 80-years-old male patient with a health history and low socioeconomic level; For two years he had been experiencing an increase in volume in the left inguinal region and ipsilateral testicle, which at first was painless; Then, as it increased in size, it became painful, covering the entire right inguinoscrotal region. Physical examination, including transillumination sign, was negative. The analytical study only showed erythrocyte sedimentation rate at 95 mm/h and LDH at 650 U/l; The testicular ultrasound showed heterogeneous, solid and hyperechoic mass, with a fatty inguinoscrotal appearance and measuring more tan 30 cm, without the testicle being observed. Tumor excision was performed and three liposarcomatous tumors were found, two of them with similar histopathological characteristics and the other with a different histological pattern. After the surgical treatment, the patient recovered satisfactorily and has not had a recurrence according to the periodic controls carried out. The publication of the case was considered because liposarcomas can be confused on clinical examination with the presence of an inguinal hernia with fatty content with symptoms similar to these; Therefore, a thorough clinical examination must be performed to make a correct diagnosis. The histopathological study confirmed the diagnosis of the disease; which, in addition, revealed the presence of three liposarcomatous tumors and two histopathological variants of the same, from which no reports were found in the reviewed literature.

Lamin B2 promotes migration of retroperitoneal liposarcoma cells by regulating AKT signaling pathway / 中华普通外科杂志

Objective:To investigate the effect of Lamins B2 (LMNB2) on the migration of human retroperitoneal liposarcoma (RPLS) cells SW872.Methods:Immunohistochemistry was used to analyze the the differential expression levels of LMNB2 in 33 RPLS tissue samples . The correlation between LMNB2 expression and clinical prognosis and clinicopathological features was analyzed. siRNA was used to lower the expression level of LMNB2 in tumor cells, and the effect of LMNB2 on the scratch healing ability and migration ability of SW872 cells was examined by using wound-healing assay and transwell migration assay. The expression levels of p-AKT and AKT in each group cells were detected by Western blot.Results:Patients with high LMNB2 expression had a lower recurrence-free survival and overall survival compared to those with low LMNB2 expression, and were more likely to experience recurrence, ( χ2=4.872, P=0.027; χ2=4.180, P=0.041; χ2=7.127, P=0.008). The migration ability of cells was significantly reduced following the silencing of LMNB2 expression ( t=11.240, P<0.01; t=7.445, P<0.01). The expression level of p-AKT in the silencing group was significantly lower than that in the control group, while there was no significant difference in the expression level of AKT between the two groups ( t=9.784, P<0.01). Conclusion:LMNB2 may promote the migration of human retroperitoneal liposarcoma cells SW872 by regulating AKT signaling pathway.

Recurrent dedifferentiated retroperitoneal liposarcoma complicating pregnancy / Philippine Journal of Obstetrics and Gynecology

@#Retroperitoneal liposarcoma is an uncommon tumor both in the pregnant and nonpregnant state. Its exact incidence is unknown because of its rarity. Due to its propensity for local recurrence, prognosis depends on histologic subtype and negative margins of resection. Surgery remains the mainstay of treatment, and timing is critical in terms of finding a surgically resectable lesion while balancing fetal risks. This report describes tumor recurrence in a pregnancy with a prior history of surgery for an atypical lipomatous tumor presenting as a retroperitoneal mass. After discussion with the multidisciplinary team, as well as with the patient’s consent, the plan was to defer surgery until 34 weeks, followed by scheduled tumor removal 2 weeks postpartum. She delivered abdominally to a baby girl with a good outcome. Histopathology showed dedifferentiated liposarcoma with a positive tumor margin. The patient has survived at 6-month follow-up and completed four cycles of chemotherapy with doxorubicin, ifosfamide, and mesna. However, surveillance imaging again showed tumor recurrence.

Diferenciación imagenológica/lipoma/liposarcoma pobremente diferenciado y liposarcoma bien diferenciado / Imaging differentiation/lipoma/dedifferentiated liposarcoma and well-differentiated liposarcoma

Resumen Los tumores de estirpe lipomatosa son lesiones frecuentes en los tejidos blandos, principalmente en el tejido celular subcutáneo y ocasionalmente en zonas más profundas. Los lipomas, tumores de estirpe benigna, son los más frecuentes y representan el 50% de todos los tumores de partes blandas. Se componen de tejido adiposo maduro y presentan características de imagen típicas en resonancia magnética (RM). Sin embargo, cuando estos tumores mesenquimales presentan elementos desdiferenciados y la apariencia de estos no presenta hallazgos típicos de un lipoma ni tampoco de un liposarcoma, se consideran dentro del grupo de tumores lipomatosos "atípicos", neoplasias que suelen presentar un curso relativamente benigno cuando se presentan en las extremidades en comparación con sus homólogos retroperitoneales. Estos tipos de tumores no suelen metastatizar, sin embargo, tienen altas tasas de recurrencia local y un potencial de desdiferenciación tardía en sarcomas de mayor grado con potencial de metástasis. Por otro lado, los liposarcomas, menos frecuentes, se distinguen por su localización en planos más profundos y presentan una apariencia diferente en la RM, con masas que muestran intensidad grasa, septos irregulares y áreas no adiposas. Destacaremos, mediante revisión de la bibliografía, la utilidad de la RM en la identificación y diferenciación de las lesiones de estirpe lipomatosa.

Abstract Lipomatous lineage tumors are common lesions in soft tissues, primarily in subcutaneous tissue and occasionally in deeper areas. Lipomas, benign tumors, are the most common and represent 50% of all soft tissue tumors. They consist of mature adipose tissue and exhibit typical imaging characteristics on magnetic resonance imaging (MRI). However, when these mesenchymal tumors present dedifferentiated elements and their appearance does not show typical findings of either a lipoma or a liposarcoma, they are considered within the group of "atypical" lipomatous tumors, neoplasms that often have a relatively benign course when occurring in the extremities compared to their retroperitoneal counterparts. These types of tumors do not usually metastasize; however, they have high rates of local recurrence and a potential for late dedifferentiation into higher-grade sarcomas with metastatic potential. On the other hand, liposarcomas, less common, are distinguished by their location in deeper planes and have a different appearance on MRI, with masses showing fatty intensity, irregular septa, and non-adipose areas. We will highlight, through literature review, the utility of MRI in the identification and differentiation of lipomatous lineage lesions.

Informe de un caso de liposarcoma paratesticular

Los liposarcomas son neoplasias que se originan de las células mesenquimales, y su localización paratesticular es infrecuente. Se dividen en cuatro subtipos histológicos, siendo el mixoide el más raro. Se muestra el informe de un caso de liposarcoma paratesticular mixoide y una revisión de la literatura, con el objetivo de brindar información sobre esta rara enfermedad. El caso es un paciente masculino de 70 años de edad, que acude a consulta por tumefacción escrotal derecha e indolora de 13 meses de evolución, diagnosticado inicialmente como una hernia inguinal unilateral. Se realizó orquiectomía inguinal radical derecha y ligadura de cordón alto. En anatomía patológica se recibe la pieza quirúrgica de 2500 g. Al estudio histológico se evidencia liposarcoma mixoide. La tomografía computarizada de tórax, abdomen y pelvis no mostró metástasis. La presencia de un liposarcoma debe tenerse en cuenta durante el estudio diagnóstico de masas escrotales, para minimizar la tasa de diagnóstico erróneo y manejo inadecuado.

Liposarcoma are neoplasms that originate from mesenchymal cells, and their paratesticular location is infrequent. They are divided into four histological subtypes, the myxoid being the rarest. The report of a case of myxoid paratesticular liposarcoma and a review of the literature are shown, with the aim of providing information on this rare disease. The case is a 70-years-old male patient who assists the clinic due to painless right scrotal swelling of 13 months evolution, who was initially diagnosed with a unilateral inguinal hernia. Right radical inguinal orchiectomy and high cord ligation were performed. The pathological piece of 2500g is received in pathological anatomy. Myxoid liposarcoma is evidenced at histological study. Chest, abdomen and pelvis computed tomography showed no metastasis. The presence of liposarcoma should be taken into account during the diagnostic study of scrotal masses to minimize the rate of misdiagnosis and inadequate management.

A rare case of primary giant myxoid liposarcoma of the pericardium

Primary pericardial neoplasms account for 6.7–12.8% of all primary tumors arising in the cardiac region. Pericardial tumors are most likely to be metastatic and are an extension of the primary tumors from the surrounding structures. Sarcomas of the pericardium are rare. Myxoid liposarcoma (ML) represents about 5% of all the soft-tissue sarcomas in adults. They are usually located in the deep soft tissues of the extremities. There have been less than 20 cases of pericardial liposarcomas reported on PubMed since 1973. Here, we present a rare case of primary giant pericardial myxoid liposarcoma (ML) in a 46-year-old female diagnosed on frozen section and later was confirmed histopathologically.

Liposarcoma retroperitoneal gigante bien diferenciado, un reporte de caso

Los liposarcomas son neoplasias malignas raras que se originan en el tejido adiposo, con mayor frecuencia en el retroperitoneo. De todas las variantes histológicas, el diferenciado es el más común y el de mejor pronóstico. El diagnóstico se establece con pruebas imagenológicas con o sin ayuda de biopsia. El tratamiento consiste en la resección completa del tumor, que puede o no acompañarse de terapia adyuvante. Se presenta el caso de un paciente de 41 años con distensión abdominal y pérdida de peso, en el que se confirma un liposarcoma retroperitoneal mediante una TC abdominal, por lo que fue sometido a una resección quirúrgica completa que resultó exitosa. El paciente se recuperó satisfactoriamente y se mantuvo sin recidivas durante el seguimiento. Se concluye que el diagnóstico y terapéutico varían de acuerdo con las características del tumor y su riesgo de recidiva; por lo que hasta la actualidad continúa siendo un desafío multidisciplinario.

SUMMARY Liposarcomas are rare malignant neoplasms that originate in adipose tissue, most frequently in the retroperitoneum. Of all histological variants, differentiated liposarcoma is the most common and has the best prognosis. Diagnosis is established by imaging tests with or without the aid of biopsy. Treatment consists of complete resection of the tumour, which may or may not be accompanied by adjuvant therapy. We present the case of a 41-year-old patient with abdominal distension and weight loss, who was confirmed to have retroperitoneal liposarcoma by abdominal CT scan and underwent successful complete surgical resection. The patient recovered satisfactorily and remained free of recurrence during follow-up. It is concluded that diagnosis and therapy vary according to the characteristics of the tumour and its risk of recurrence, which is why it remains a multidisciplinary challenge to this day.

A rare case of para-testicular dedifferentiated liposarcoma: a diagnostic dilemma

Paratesticular dedifferentiated liposarcoma (DD-LPS) are rare neoplasms. It has been estimated that 12% of DD-LPS occur in the paratesticular region, while the retroperitoneum is the most common site for this tumor. Herein, we presented a case of a 66-year-old male with a history of pedal edema and right scrotal swelling, CT scan shows a solid mass arising from the right side of the scrotum. Following this, a radical orchiectomy of the right testis with mass was performed, and after thorough microscopic and immunohistochemistry positive for MDM2 and CDK4, the diagnosis of low-grade dedifferentiated liposarcoma was given. This case report expands the morphologic spectrum of dedifferentiated liposarcoma and emphasizes the need to consider these tumours in the differential diagnosis of other neoplasms.

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.

CT and MRI manifestations of retroperitoneal dedifferentiated liposarcoma / 中国肿瘤临床

Objective:To investigate the computed tomography(CT)and magnetic resonance imaging(MRI)features of retroperitoneal ded-ifferentiated liposarcoma(DDL),and improve the understanding of DDL and the accuracy of preoperative diagnosis.Methods:Clinical and imaging features of 25 patients with retroperitoneal DDL from Tianjin Medical University Cancer Institute&Hospital,confirmed by patho-logy from January 2012 to June 2022,were retrospectively analyzed.Results:Among 25 cases of retroperitoneal DDL,19 and 6 had single and multiple tumors,respectively and 10 and 15 were oval and irregular shaped tumors,respectively.Most lesions had unclear boundaries,with 15 cases invading the surrounding tissues and organs.Small vessel shadows were visible in 15 cases,while calcifications or ossifications were observed in 7 cases,and cystic necrosis was observed in only 3 cases.Enhanced scanning exhibits a centripetal and progressive con-tinuous augmentation characteristic defined as"slow in and slow out."According to its manifestations in CT and MRI,it can be divided into two types:type I(soft tissue mass type),where the tumor has a soft tissue component with no fat content(14 cases);and type Ⅱ(fat con-taining),where the tumors exhibit both soft tissue and adipose components,most of which are clearly defined and rarely present in a mosa-ic shape.In abnormal fat areas,cord-like fibrous septa can be seen.Among them,the intratumoral fat composition<50%was Ⅱa type(10 cases).Intratumor fat composition≥50%was type Ⅱb(1 case).Conclusions:Combined with imaging classification,a comprehensive ana-lysis of the CT and MRI imaging characteristics of retroperitoneal DDL is of great value for its preoperative qualitative diagnosis.

Diagnosis of a Primary Pleomorphic Liposarcoma in a Tree Shrew（Tupaia belangeri subsp. yaoshanensis） / 实验动物与比较医学

A large spontaneous mass on the dorsal abdomen near the hip joint was found in an aged female Tupaia belangeri subsp. yaoshanensis after 5 years of routine feeding. The tumor in the diseased tree shrew was huge, with an intact surface and no ulceration; however, it caused inconvenience in movement while maintaining a good mental state. After inhalation anesthesia with isoflurane (2%-4%), the tree shrew was euthanized after the tree shrew entered deep anesthesia. Anatomical dissection of the tumor, the tumor boundary was unclear and infiltrated into surrounding tissues. HE staining showed that small focal pleomorphism and large areas of adipocytes were seen in the tumor tissue. The pleomorphic sarcoma area was mainly composed of atypical epithelioid cells with easily visible nuclear divisions. The size of the adipocytes was significantly different, and more pleomorphic adipocytes were seen. The cell volume was large, the nucleus was deeply stained and deformed, the edge was impressed, and the cytoplasm was seen with multiple vesicular lipid droplets. Immunohistochemical results showed that the tumor cells were positive for Vimentin, the small focal polymorphic adipocyte nucleus was positive for S-100, and Ki-67 exhibited a higher proportion of positivity. Combined with HE staining and immunohistochemical results, the spontaneous tumor in this tree shrew was comprehensively diagnosed as pleomorphic liposarcoma.

Intraoral lipoma with degenerative changes mimicking atypical lipomatous tumor: an immunohistochemical study

ABSTRACT Lipomas are mesenchymal neoplasms relatively uncommon in the oral cavity. Lipomas can exhibit histopathological features mimicking atypical lipomatous tumors (ALT) or dysplastic lipoma (DL) in the presence of degenerative changes. Relevantly, immunohistochemistry assists in the correct diagnosis. Herein, we present the case of a 54-year-old male with a sessile nodule located on the dorsum of the tongue. The histopathological analysis showed a diffuse, non-circumscribed adipocytic proliferation constituted by cells of variable size containing cytoplasmic vacuoles and displaced nuclei, some resembling lipoblasts supported by fibrous connective tissue stroma. By immunohistochemistry, tumor cells were positive for vimentin, S100, FASN, CD10, and p16. Rb expression was intact. Moreover, CD34, p53, MDM2, and CDK4 were negative. After 2-year of follow-up, no alteration or recurrence was observed. In conclusion, MDM2, CDK4, p53, and Rb immunomarkers can be used reliably to differentiate benign lipoma with degenerative changes from ALT and DL.

Histopathological Spectrum of Myxoid Soft‑Tissue Neoplasms in a Tertiary Care Center with a Special Focus on Vascular Patterns: A 13‑Year Compilation

Introduction: Myxoid soft‑tissue tumors are a diverse group of tumors which have similar histomorphology but have varied geneticsequence and clinical outcome, hence differentiating and diagnosing them is a challenge for any pathologist. This study describes the varioushistomorphological spectrum and vascular pattern of various myxoid soft‑tissue tumors. Materials and Methods: This was a retrospective and prospective observational study of myxoid soft‑tissue tumors over a period of 13 years. A total of 224 cases with myxoid morphology were included and were examined morphologically with a special focus on the vascular pattern. SPSS v 24 was used for statistical analysis. Results: The predominant lesions were benign in 164 (73.21%) cases, followed by malignant lesions in 43 (19.19%) cases and intermediate lesions 17 (7.58%) cases. Both benign and malignant lesions showed a male preponderance and were seen to arise predominantly from the extremities. The most common benign myxoid lesions in this study were of neural origin with myxoid neurofibroma constituting 65 (29. 01%) cases, followed by schwannoma 38 (16.9%) cases. Myxoid dermatofibrosarcoma protuberans was the most common intermediatelesion. Tumors with adipocytic differentiation were the predominant lesions among the malignant group, i.e myxoid liposarcoma seen in 17 (7.5%) cases. Conclusions: Vascular pattern in the myxoid lesions are subtle yet crucial in arriving at a histo‑morphological diagnosis. Further studies correlating the vascular pattern with the genetic profile of these tumours can help arriving at a histo‑morphological diagnosisof myxoid lesions.

Intraperitoneal dissemination of primary dedifferentiated liposarcoma of omentum simulating an ovarian cancer – A case report

Dedifferentiated Liposarcoma (DDLPS) is a rare subtype of liposarcoma with a high preponderance of local recurrence and distant metastasis. The frequent site of DDLPS is the retroperitoneum, followed by the limbs and trunk. The primary omental DDLPS is very rare and only a few cases have been reported in the literature. Here we report a rare case of primary giant DDLPS of omentum with intraperitoneal metastasis in a 61year old woman. The present case report adds to our knowledge, that a case of intraperitoneal DDLPS can mimic ovarian cancer clinically and should be considered in the differential diagnosis of a pelvic mass in postmenopausal women.

Lipossarcoma Pleomórfico de Mama em Paciente Jovem ­ Relato de Caso / Pleomorphic Breast Liposarcoma in a Young Patient ­ Case Report

O Sarcoma de partes moles é um tumor maligno raro, correspondendo a aproximadamente 1% dos tumores malignos em adultos. Objetivamos descrever um raro caso de Lipossarcoma pleomórfico em paciente jovem, sem história familiar, que representa 0,3% dos sarcomas da mama. Relatamos o caso de uma paciente do sexo feminino, 39 anos, com nodulação em prolongamento axilar de mama esquerda, cujas mamografia e ecografia caracterizaram nodulação regular, de limites definidos, sem fluxo central ou periférico e ecotextura heterogênea. A Core-Biopsy diagnosticou neoplasia maligna fusocelular. Realizada ressecção segmentar, cujos laudos histopatológico e de imuno-histoquímica concluíram como sendo Lipossarcoma pleomórfico de alto grau. O sarcoma pleomórfico é um subtipo raro e agressivo entre os lipossarcomas. O seu tratamento, assim como dos sarcomas de mama, é a ressecção cirúrgica com margens de segurança; a terapia adjuvante com radioterapia é indicada devido à taxa de recorrência deste tumor. A quimioterapia ainda tem papel indefinido nesses tipos de tumores. Concluímos que, por ser o Lipossarcoma pleomórfico uma neoplasia maligna rara, o diagnóstico e a conduta adequados se fazem necessários para prover terapêutica precisa e conter metastatização e recidiva local.

Soft tissue sarcoma is a rare malignant tumor, accounting for approximately 1% of malignant tumors in adults. We aim to describe a rare case of pleomorphic liposarcoma in a young patient with no family history, representing 0.3% of breast of breast sarcomas. We report the case of a 39-year-old female patient with nodulation in the axillary extension of the left breast, whose mammogram and ultrasonography characterized a regular nodulation, with defined limits, without central or peripheral flow, and heterogeneous echotexture. The Core-Biopsy diagnosed malignant fusocellular neoplasm. Segmental resection was performed. The histopathological and immunohistochemistry reports concluded that it was a high grade pleomorphic liposarcoma. Pleomorphic sarcoma is a rare and aggressive subtype among the liposarcomas. Its treatment, as with breast sarcomas, is surgical resection with safety margins; adjuvant therapy with radiotherapy is indicated due to the recurrence rate of this tumor. Chemotherapy still has an undefined role in these types of tumors. We conclude that, because pleomorphic liposarcoma is a rare malignant neoplasm, the diagnosis and management are necessary to provide an accurate therapy and contain metastasis and local recurrence.

A giant retroperitoneal liposarcoma with hemoperitoneum: A rare tumor with unusual presentation

Retroperitoneal liposarcoma (RPLS) is a rare mesenchymal tumor which accounts for 0.02–0.7% of all malignancy. The presentations of these tumors are non-specific and early diagnosis is often missed. We are reporting a rare case of 17-year-old girl who came to us with complaints of abdominal distension and other non-specific symptoms but after few hours of admission, she had acute abdomen with hemodynamic instability. She underwent emergency laparotomy and found to have a giant 25 × 20 × 10 cm ruptured retroperitoneum tumor causing hemoperitoneum. The tumor involved left ovary and fallopian tube as well. Complete resection of tumor with left salpingooophorectomy was done. The patient was kept in intensive care unit and discharged in good health after 15 days. This presentation of RPLS has not been reported yet and may demand early diagnosis and management of these tumors. The histopathology revealed dedifferentiated liposarcoma with rhabdomyosarcomatous differentiation. The patient is completely fine at 16 months of follow-up while writing this report. Again, heterologous dedifferentiation of liposarcoma with spindle cell component is a rare morphologic spectrum. Liposarcomas can have various histologic types. In dedifferentiated types, the line of differentiation needs to be identified to decide further line of treatment. Therefore, thorough histopathological analysis and immunostaining is needed. Treatment includes complete surgical resection. Role of radiotherapy and chemotherapy is debatable and is still under trial. Dedifferentiated RPLS with rhabdomyosarcomatous differentiation is a rare tumor and its presentation as acute abdomen and hemoperitoneum has never been reported previously

Detection of MDM2 gene amplification on tissue microarray-based Fluorescence In-Situ Hybridization (FISH) in well-differentiated and dedifferentiated liposarcomas, displaying a wide morphological spectrum: A validation study at a tertiary cancer referral centre

Background: Liposarcomas including atypical lipomatous tumors (ALT)/well-differentiated liposarcomas (WDLPS) and dedifferentiated liposarcomas (DDLPSs) display a histomorphological spectrum with their several diagnostic mimics. Murine double minute 2(MDM2) gene amplification characterizes ALT/WDLPS and DDLPS. Presently, there is no documented study from our subcontinent on the validation of MDM2 gene testing in these tumors. Material and Methods: Twenty-eight cases, diagnosed as ALT/WDLPS (n = 5) and DDLPSs (n = 23), along with 10 other tumors were tested for MDM2 gene amplification, using fluorescence in situ hybridization (FISH) on tissue microarrays (TMAs). Fourteen cases, diagnosed as ALT/WDLPS and DDLPS, along with 49 other tumors were tested for MDM2 immunostaining. Twenty tumors were tested for p16INK4a immunostaining. Results: FISH was interpretable in 25 (89.2%) cases. Among the 20 cases diagnosed as DDLPSs, 19 displayed MDM2 gene amplification. Among the 5 cases diagnosed as ALT/WDLPS, four showed MDM2 gene amplification. Finally, 19 cases were confirmed as DDLPS and 4 as ALT/WDLPS. Furthermore, 7/19 cases confirmed as DDLPS and all 4 cases as ALT/WDLPS tested for MDM2 immunostaining, displayed its diffuse immunoexpression, while a single case of DDLPS showed its focal immunostaining. None of the 49 control cases displayed diffuse MDM2 immunoexpression. ALL 16 DDLPSs and 4 cases of ALT/WDLPS displayed p16INK4a immunostaining. The sensitivity for diffuse MDM2 immunostaining was 87.5% in cases of DDLPS, 100% in ALT/WDLPS, and specificity was 100%. The sensitivity for MDM2 gene amplification was 94.7% in cases of DDLPS and 100% in cases of ALT/WDLPS. The sensitivity for p16INK4a was 100%. Conclusion: This constitutes the first sizable study on MDM2 testing in ALT/WDLPS and DDLPS from our subcontinent using TMAs. MDM2 gene amplification testing continues as the diagnostic gold standard for ALTs/WDLPSs and DDLPSs and is useful in cases of diagnostic dilemmas. Diffuse MDM2 (IF2 clone) and p16INK4a immunostaining, together seem useful for triaging cases for FISH.