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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.
Delayed Metastasis of Clear Cell Sarcoma of Kidney to Bladder After 7 Disease-Free Years
Published in Fetal and Pediatric Pathology, 2018
After more than a 7-year disease-free interval, the patient presented with gross hematuria and passage of blood clots, which began on the day preceding clinical presentation. No other symptoms existed at that time. Imaging revealed a 5.2 × 3.9 × 3.5 cm lobulated mass arising from the right anterior bladder wall. Inflammatory myofibroblastic tumor and rhabdomyosarcoma were the impressions based upon the imaging findings. Both superficial and deep biopsies of the mass were performed. Extension of the tumor into the muscularis propria was noted on biopsy. The biopsy was interpreted as favoring metastatic CCSK. A partial cystectomy and right pelvic lymphadenectomy were performed. At the time of recurrence, the bone marrow was free of tumor and imaging studies did not demonstrate additional foci of metastatic disease such as in lungs or bone.
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].
Recurrent Giant Cell Fibroblastoma in an Infant: A Diagnostic Challenge
Published in Fetal and Pediatric Pathology, 2022
Priyanka Maity, Uttara Chatterjee, Mou Das, Sabita Patra
The cytological differential diagnoses of GCF include nodular fasciitis, proliferative fasciitis, proliferative myositis, inflammatory myofibroblastic tumor, benign fibrous histiocytoma, neurofibroma and DFSP. The presence of giant cells helps in distinguishing GCF from the aforementioned entities. Nodular fasciitis, in addition to spindle cells, may show stellate cells, focal atypia, mitotic figures and a tissue culture pattern. Inflammatory myofibroblastic tumor, unlike GCF, shows inflammatory cells. Benign fibrous histiocytoma shows foamy macrophages and pigment laden macrophages. DFSP exhibits a storiform pattern and lacks giant cells.