We report the unusual cardiac localization of a primary low-grade fibromyxoid sarcoma of the right ventricle in a 57-year-old woman. Histological examination revealed a prevalent myxoid appearance with whorling growth pattern of small or spindle cells with bland features alternating with rare more collagenous hypocellular areas with rare atypical cells. Genomic polymerase chain reaction of geno...

Hibernomas are extremely rare benign tumors of adipose tissue characterized by an admixture of brown fat cells with granular, multivacuolated cytoplasm and white fat cells. Hibernomas account for 1.6% of benign adipose tissue tumors and approximately 1.1% of all adipocytic tumors. Around 10% of these cases are intramuscular. It was initially described in the early 1900s as being composed of bro...

BACKGROUND To report on the activity of high-dose prolonged-infusion ifosfamide (HDIFX) chemotherapy in a retrospective series of patients affected by myxoid liposarcoma treated at Fondazione IRCCS Istituto Nazionale dei Tumori in Milan, Italy. PATIENTS AND METHODS Patients with an advanced myxoid liposarcoma treated with HDIFX (14 g/sqm, i.v., prolonged infusion of 14 days every 28 days) as ...

We report five cases of tumors occurring in three children and in two adults. The tumors had unusual histomorphology and a mixture of ependymal and piloid-like astrocytic features and a myxoid stroma similar to myxopapillary ependymomas. MR imaging in three of the cases showed aggressive, intensely enhancing partially cystic hypothalamic-suprasellar masses near midline and near the floor of the...

Tumors of the central nervous system are common in the pediatric population and constitute the second most prevalent tumor type in children. Within this group, spinal cord tumors are relatively rare and account for 1 to 10% of all pediatric central nervous system tumors. We describe a very rare case of an intradural extramedullary spinal cord tumor with a subcutaneous mass and discuss its clini...

Background Liposarcomas (LPS) are rare tumors accounting approximately for 19% of adult soft-tissue tumors [1]. The most recent classification of the WHO divides liposarcomas into 3 main clinicopatological and genetic subtypes: myxoid/round cells liposarcoma, well differentiated / dedifferentiated liposarcoma and pleomorphic liposarcoma [2]. The importance of a total macroscopical resection to ...

Intrathoracic lipomas are rare benign tumors; their behavior is not completely clear and their surgical removal may be challenging. We report a case of a giant right intrathoracic myxoid fusocellular lipoma compressing the lung, tracheobronchial tree, and esophagus which was removed through a posterolateral thoracotomy. Complete removal resulted in resolution of the chest pain and improvement o...

NY-ESO-1 (CTAG 1B) is highly expressed in the majority of synovial sarcomas and myxoid/round cell liposarcomas as well as in a subset of melanomas, but only rarely in other mesenchymal tumors. This points to a potential for using NY-ESO-1 in the differential diagnosis of these lesions. Furthermore, promising results have been obtained in clinical trials testing NY-ESO-1-targeted immunotherapy i...

Fusion genes: Ewing sarcoma: t(11;22)(q24;q12) EWS-FLI1 t(21;22)(q22;q12) EWS-ERG t(7;22)(p22;q12) EWS-ETV1 others FUS-ERG, EWS-FEV, Desmoplastic SRCT: t(11;22)(p13;q12) EWS-WT1 Extraskel myxoid chondrosarc: t(19;22)(q22;q12) EWS-TEC(CHN) Malignant mesenchymoma: t(11;22)(q24;q12) EWS-FLI1 Alveolar rhabdomyosarcoma: t(2;13)(q35;q14) PAX3-FKHR t(1;13)(p36;q14) PAX7-FKHR Myxoid round cell liposarc...