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Soft Tissue Sarcomas
Published in Pat Price, Karol Sikora, Treatment of Cancer, 2020
Thomas F. DeLaney, David C. Harmon, Karol Sikora, Francis J. Hornicek
Although the foregoing generalizations apply to most STSs, different histologic subtypes of sarcomas display their own patterns of chemosensitivity. Myxoid/round cell liposarcomas appear sensitive to doxorubicin and to trabectedin. Synovial sarcoma has a strong dose response to ifosfamide. Non-uterine leiomyosarcomas appear to have lower response rates to doxorubicin and ifosfamide, but may respond to trabectedin. Angiosarcomas are almost unique in being sensitive to paclitaxel. Rhabdomyosarcoma, desmoplastic small round cell tumors, and peripheral neuroectodermal tumors respond to combinations that include ifosfamide, etoposide, vincristine, doxorubicin, dactinomycin cyclophosphamide, and topotecan/irinotecan. Sunitinib has proven useful against solitary fibrous tumor/hemangiopericytoma, alveolar soft part sarcoma, and clear cell sarcoma. Everolimus has activity against tumors with perivascular epithelioid cell differentiation. Imatinib can help in metastatic dermatofibroma protuberans. Cediranib is effective against alveolar soft part sarcoma. Crizotinib is active against ALK translocated inflammatory myofibroblastic tumor. Bevacizumab with temozolomide has been employed for solitary fibrous tumor.
Primary tumours of the liver and biliary tract
Published in Anju Sahdev, Sarah J. Vinnicombe, Husband & Reznek's Imaging in Oncology, 2020
Hero K Hussain, Mohammad Abu Shattal
Imaging features of rare liver tumours, and uncommon entities such as inflammatory myofibroblastic tumour and solitary fibrous tumour can be suggestive but often non-specific, and tissue sampling is needed to establish a diagnosis.
Pleuropulmonary Blastoma
Published in Dongyou Liu, Handbook of Tumor Syndromes, 2020
Raúl Barrera-Rodríguez, Carlos Pérez-Malagón
Inflammatory myofibroblastic tumor originates in the lung, typically in children age >3–4 years, as a well-circumscribed, lobar-based mass. The tumor comprises myofibroblasts that can be demonstrated by immunostaining for smooth muscle actin (SMA). About 40%–50% of inflammatory myofibroblastic tumors have translocations involving ALK (the gene encoding anaplastic lymphoma kinase) and show immunostaining for the ALK protein.
Infantile Inflammatory Myofibroblastic Tumor of Spleen
Published in Fetal and Pediatric Pathology, 2022
Balamurugan Thirunavukkarasu, Pritam Singha Roy, Kirti Gupta, Aravind Sekar, Deepak Bansal
Inflammatory myofibroblastic tumor (IMT) is a mesenchymal neoplasm with unknown etiology. They are commonly reported in children and young adults [1]. The tumor exhibits wide spectrum of morphology and immunophenotypes. Morphological spectrum includes an inflammation predominant “pseudotumour” with scant lesional spindle cells to a highly cellular myofibroblastic tumor lacking inflammatory and stromal components mimicking a sarcoma [2]. Thus the variation in morphology and immunophenotype has led to this entity to be a diagnosis of exclusion. Nearly half of IMT show rearrangement in anaplastic lymphoma kinase (ALK) gene and overexpression of ALK protein making it a reliable diagnostic marker and application in targeted therapy using tyrosine kinase inhibitors [3]. Currently, a subset of ALK negative cases have shown abnormalities in other genes such as ROS1, ETS variant 6 gene (ETV6), neurotropic receptor tyrosine kinase 3 gene (NTRK3), and RET [3, 4]. We present a case of splenic IMT affecting an 18-month old infant with abdominal distention and failure to thrive.
Neonatal Gastrointestinal Stromal Tumor of the Sigmoid Colon: A Case Report and Review of Literature
Published in Fetal and Pediatric Pathology, 2020
Mostafa Kotb, Marwa Abdelaziz, Marwa Beyaly, Mohamed Mekawy, Hayssam Rashwan, Nagwa Mashali
Other spindle cell neoplasms that can be seen in the GIT include smooth muscle tumors, inflammatory myofibroblastic tumor and tumors of neural origin- namely schwannoma. [14] Tumors of smooth muscle origin (leiomyoma/leiomyosarcoma) are expected to be diffusely and strongly positive for SMA; thus, were excluded based on the focal expression of SMA in the present case. Inflammatory myofibroblastic tumor (IMT) was excluded since the tumor did not show the classic histological picture of IMT which entails the presence of an inflammatory infiltrate rich in lymphocytes and plasma cells along with a spindled population composed of plump myofibroblasts disposed against a loose myxoid stroma or less commonly a hyalinized stroma. Additionally, the neoplastic cells were negative for ALK1. [15] Moreover, intrabdominal IMTs patients often have systemic manifestations as fever and night sweats [14] which were absent in our case. Schwannoma was also excluded based on the negative staining of tumor cells for S100 which is known to be strong and diffusely positive in schwannoma. None of the aforementioned tumors is known to express CD117. [15]
Intrapericardial inflammatory myofibroblastic tumour in a 3-month-old infant associated with Coronavirus OC43 presenting with pericardial tamponade
Published in Paediatrics and International Child Health, 2020
Yasemin Nuran Donmez, Hayrettin Hakan Aykan, Diclehan Orhan, Recep Oktay Peker, Mustafa Yilmaz, Tevfik Karagoz
Inflammatory myofibroblastic tumour is a rare neoplasm, characterised by spindle cells proliferation and inflammatory cell infiltration [3]. IMT can occur in various organs or other systems, primarily in soft tissue and viscera such as different parts of the head and neck, lung, liver, gastrointestinal or genitourinary tract [4,5]. It is very rare in the heart [6,7]. About 60 cardiac IMT cases have been reported in the literature [4]. Although there are reports of a benign clinical course, it can be locally aggressive and has the potential to recur and metastasize [1]. The 2015 WHO classification of tumours of the heart and pericardium defines it as being of uncertain biological behaviour [7].