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ABSTRACT A 41-year-old male patient refers to a painful tumor on the lateral side of the right leg. Ultrasonography visualized a predominantly hypoechoic well-delineated large heterogeneous mass in the subcutaneous tissue, showing an internal hyperechoic area. Magnetic resonance imaging (MRI) demonstrated an oval and lobulated tumor with well-defined margins at the anterolateral part of the leg, with contrast enhancement. The anatomopathological evaluation revealed a neoplasm with an extensive chondromyxoid matrix. The immunohistochemical study demonstrated positive vimentin (V9 clone), positive S-100 protein and negative desmin (D33 clone), smooth muscle actin (1A4), CD68 (KP1 clone) and caldesmon. The set of findings is compatible with myxoid liposarcoma.
RESUMEN Paciente masculino de 41 años de edad, reporta tumor doloroso en la parte lateral de la pierna derecha. La ultrasonografía demostró una masa voluminosa, hipoecogénica, heterogénea y bien delimitada en el tejido subcutáneo, con área hiperecogénica en su interior. La resonancia magnética (RM) reveló un tumor ovalado y lobulado, con márgenes bien definidas en la parte anterolateral de la pierna, presentando realce de contraste. El estudio anatomopatológico constató neoplasia con abundante matriz condromixoide. La inmunohistoquímica presentó vimentina (clon V9) y proteína S-100 positivas, y desmina (clon D33), actina de músculo liso (1A4), CD68 (clon KP1) y caldesmón negativos. El conjunto de hallazgos es compatible con liposarcoma mixoide.
RESUMO Paciente do sexo masculino, 41 anos de idade, relata tumor doloroso na face lateral da perna direita. A ultrassonografia demonstrou massa volumosa, hipoecogênica, heterogênea e bem delineada no tecido subcutâneo, com área hiperecogênica em seu interior. A ressonância magnética (RM) revelou um tumor ovalado e lobulado, com margens bem definidas na parte anterolateral da perna, apresentando realce pelo contraste. O estudo anatomopatológico constatou neoplasia com extensa matriz condromixoide. O estudo imuno-histoquímico apresentou vimentina (clone V9) e proteína S-100 positivas e desmina (clone D33), actina de músculo liso (1A4), CD68 (clone KP1) e caldesmon negativos. O conjunto de achados é compatível com lipossarcoma mixoide.
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Objective@#To detect the expression of New York esophageal squamous cell carcinoma antigen 1 (NY-ESO-1) in common types of mesenchymal myxoid tumors, and to investigate its significance in the diagnosis and differential diagnosis of myxoid liposarcoma.@*Methods@#A total of 43 formalin-fixed paraffin-embedded samples of mesenchymal myxoid tumors from the Affiliated Hospital of Qingdao University and Qingdao Municipal Hospital ranging between 2010 and 2017 were selected. NY-ESO-1 expression was detected by immunohistochemical staining. DDIT3 gene status was detected by fluorescence in situ hybridization (FISH). NY-ESO-1 mRNA was detected by reverse transcription-PCR (RT-PCR).@*Results@#Histopathology and FISH results confirmed that there were 11 cases of myxoid liposarcoma and 32 other types (including 7 cases of well-differentiated liposarcoma, 1 dedifferentiated liposarcoma, 3 lipomas, 2 lipoblastomas and 19 non-adipocytic tumors). Immunohistochemical staining showed that the positive expression propotion of NY-ESO-1 in myxoid liposarcoma was 11/11, and the positive location was the cytoplasm and nucleus of lipoblast cells. The expression intensity is higher in regions with round cell differentiation. Among the 32 cases of other mesenchymal myxoid tumors, only one well-differentiated liposarcoma showed positive immunoreactivity for NY-ESO-1. RT-PCR confirmed that 7 cases of myxoid liposarcoma (7/11) and one well-differentiated liposarcoma (1/7) had NY-ESO-1 mRNA expression.@*Conclusions@#NY-ESO-1 is positively expressed in myxoid liposarcoma. It can be served as a useful marker for the diagnosis and differential diagnosis of myxoid liposarcoma.
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Fundamento: los tumores malignos de partes blandas no son lesiones frecuentes, sin embargo, el liposarcoma es que el incide con mayor frecuencia entre estas enfermedades. Objetivo: mostrar un paciente con un lipoma mixoide del muslo. Caso clínico: paciente de 49 años de edad, blanco, masculino con antecedentes de salud anterior, el cual hace alrededor seis años comenzó con una bolita en la cara interna del muslo derecho, en los últimos seis meses ha crecido muy rápido. El paciente en todo momento refirió ausencia de dolor y justifica la demora en asistir al médico por tener miedo. A la exploración física del muslo derecho se detectó una masa de bordes irregulares de consistencia mixta, adherida a planos profundos por debajo de la fascia, fija pero no adherida al hueso no móvil, aunque no en relación a estar adherida al hueso, de 30 centímetros de largo, por 20 centímetros de ancho y 15 centímetros de profundidad, localizada en la cara interna del muslo derecho. Mediante la palpación se constató aumento de volumen y de la temperatura local del muslo. Se realizaron exámenes imaginológicos dentro de los que se incluyeron, ultrasonido diagnóstico de alta resolución, tomografía axial computarizada, imagen de resonancia magnética y arteriografía. Al tener en cuenta todos los elementos anteriores el paciente es llevado al salón de operaciones donde un equipo multidisciplinario compuesto por ortopédicos, angiólogos, cirujanos generales y anestesiólogos realizó la exceresis completa de la tumoración, a través de un margen de seguridad. Conclusiones: el liposarcoma mixoide es el tumor maligno de partes blandas más frecuente, así como su localización en el esqueleto apendicular, en particular en el muslo.
Background: malignant soft tissue tumors are not common, but among them liposarcoma has the higher incidence. Objective: to show a patient with a myxoid liposarcoma of the thigh. Case report: a 49-year-old white man, with record number 207009. The patient was taken to the Outpatient Orthopedic Department because of a soft tissue mass found in the right thigh for about six years, which in the last six months grew quickly. There was no pain and the patient refused medical assistance because of fear. On palpation a big soft tissue mass was detected with irregular margins, mix consistency, location under the fascia, deep-seated tumor although not fixed to the bone, and 30 centimeters long, 20 centimeters width and 15 centimeters deep found on the right thigh. Imaging exams like ultrasound, computed axial tomography, magnetic resonance imaging and arteriography were performed. The patient underwent surgery and a wide resection was carried out by a team of orthopedics, angiologists, general surgeons and anesthesiologists. Tumor resection was done by a safe margin. Conclusions: myxoid liposarcoma is the most common soft tumor as well as its location at the appendicular skeleton, like the thigh.
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Objective To investigate the diagnosis and differential diagnosis of dermatofibrosarcoma protuberans (DFSP). Methods Totally, 50 patients with DFSP visiting the Institute of Dermatology, Chinese Academy of Medical Sciences from 1998 to 2014 were enrolled. The clinical manifestations, histopathological and immunohistochemical features, treatment and prognosis of DFSP were retrospectively reviewed. Results The average age at onset of DFSP was (29.5 ± 15.9)years in the 50 patients, with a mean disease duration of 9.57 years. Skin lesions most frequently occurred on the trunk(n = 33, 66.0%), followed by the extremities, head and neck. DFSP was characterized by atrophic patches or plaques in 13 cases (26.0%), multiple nodules varying in size and arising on atrophic plaques or patches in 30 cases (60.0%), single or multiple nodules arising on normal skin in 7 cases (14.0%). Histologically, the tumor consisted of uniform infiltrative spindle cells arranged in a storiform or cartwheel pattern. In addition, the tumor cells expressed CD34 and vimentin. Twenty patients experienced recurrence at the primary site after resection of skin lesions with a recurrence rate of 43.5%. No distant metastasis or death occurred in these patients. Conclusions DFSP usually has various skin manifestations, is easily misdiagnosed, and can be confirmed based on histopathological and immunohistochemical findings. Local recurrence of DFSP is common, and may occur for many times after surgical excision, but lymphatic and distant metastases are rare.
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Myxoid liposarcoma is a subtype of liposarcoma. This specific subtype can be identified based on its characteristic histological and cytogenetical features. The tumor has a fusion transcript of the CHOP and TLS genes, which is caused by t(12;16)(q13;p11). Most of the fusion transcripts that have been identified fall into three categories, specifically type I (exons 7-2), type II (exons 5-2), and type III (exons 8-2). A total of seven myxoid liposarcomas associated with the rare phenomenon of cartilaginous differentiation have been documented in the literature. Currently, only one of these cases has been cytogenetically analyzed, and the analysis indicated that it was a type II TLS-CHOP fusion transcript in both the typical myxoid liposarcoma and cartilaginous areas. This study presents a second report of myxoid liposarcoma with cartilaginous differentiation, and includes a cytogenetical analysis of both the myxoid and cartilaginous areas.
Sujet(s)
Cartilage , Liposarcome , Liposarcome myxoïdeRÉSUMÉ
O lipossarcoma mixóide é um tumor lipogênico originado de tecidos moles profundos e raramente de lipomas benignos. Corresponde a cerca de 50% dos lipossarcomas e sua incidência predominante dá-se em torno dos 50 anos de idade. Pode afetar as extremidades, o retroperitônio e, menos comumente, a região da cabeça e pescoço, cursando com massa de crescimento lento e indolor. Tumores primários da glândula tireóide são extremamente raros, geralmente se apresentando com sintomas compressivos ao diagnóstico e simulação de um bócio retroesternal, representando uma armadilha para os cirurgiões. O diagnóstico e tratamento de escolha incluem a exérese radical com laudo histopatológico ou imunoistoquímico, com indicação de radioterapia adjuvante. Os autores descrevem um caso de lipossarcoma mixóide originado de tireóide em uma paciente de 55 anos de idade e discutem a possível simulação de bócios retroesternais e a importância do diagnóstico precoce.
Myxoid liposarcoma is a lipogenic tumor originated from deep soft tissues and rarely from benign lipomas, corresponding to around 50% of liposarcomas. Its predominant incidence is about 50 years old. They can affect the extremities, retroperitoneum and less commonly the region of head and neck coursing with slow growth and painless mass. Primary tumors from the thyroid gland are extremely rare, generally with compressive symptoms and simulation of a retrosternal goiter. Diagnosis and treatment include radical resection, histopathologic study and adjuvant radiotherapy. The authors report a case of a thyroid myxoid liposarcoma in a 50 year-old woman and discuss the simulation of retrosternal goiters and the importance of early detection.
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Primary ovarian myxoid tumor such as myxoma, myxoid liposarcoma and myxoid leiomyosarcoma is extremely rare neoplasm. We experienced a case of unusual myxoid tumor of the ovary in a 25 year-old woman. She was admitted for an incidentally found ovarian mass during antenatal check. Radiologic studies revealed a 5.5x5 cm-sized solid mass in left ovary and she was undertaken left oophorectomy. Grossly, the round ovarian mass was measuring 8x6x5 cm, and the cut surface was predominantly solid with myxoid appearance. Microscopically, the tumor was surrounded by thick collagenous capsule and had moderate cellularity and rich vascularity. The tumor cells were stellate-shaped with abundant extracellular myxoid material without atypia. We initially thought this lesion as myxoma, but the cellularity was too high as an ordinary myxoma. Myxoid liposarcoma could also be considered as the differential diagnosis, however there was no convincing lipoblast. So, we diagnosed that tumor as myxoma with uncertain malignant potential.
Sujet(s)
Adulte , Femelle , Humains , Collagène , Diagnostic différentiel , Léiomyosarcome , Liposarcome myxoïde , Myxome , Tumeurs de l'ovaire , Ovariectomie , OvaireRÉSUMÉ
Objective To explore the clinical and MR imaging features of myxoid liposarcoma.Methods Clinical and MR imaging data of 7 patients with histologically confirmed myxoid liposarcomas on extremities were retrospectively analyzed.The age of the patients ranged from 41 to 59 years with a median age of 51 years.Results Three tumors occurred in thigh,two in calf,one in foot and one in shoulder.Six tumors were situated deeply,and one was superficial.On T_1-weighted images,all 7 tumors showed predominant isointense or slightly hypointense signals relative to muscle,with 6 having lacy,linear or amorphous loci of high signal intensity.The major portion of each tumor displayed hyperintense signals compared with fat on T_2-weighted images.Following the injection of Gd-DTPA,all tumors showed inhomogenous and strong enhancement.All tumors had septa and were well defined without obvious surrounding edema and invasion of the adjacent bones.Conclusion Myxoid liposarcomas usually show predominant isointense or slightly hypointense signals relative to muscle on T_1-weighted images and hyperintense signals relative to fat on T_2-weighted images.The fat components within the tumors may be identified as linear,lacy or amorphous foci of high signal intensity on T_1-weighted images.The contrast enhancement of the mvxoid liposarcomas is usually pronounced and heterogeneous.