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Tumors of Fibrous Tissue
Published in Omar P. Sangueza, Sara Moradi Tuchayi, Parisa Mansoori, Saleha A. Aldawsari, Amir Al-Dabagh, Amany A. Fathaddin, Steven R. Feldman, Dermatopathology Primer of Cutaneous Tumors, 2015
Other features: Mitotic figuresHistologic variants: sclerosing epithelioid fibrosarcoma, fibrosarcoma with palisaded granuloma-like bodies (giant rosettes), and inflammatory fibrosarcoma
Fibrosarcoma
Published in Dongyou Liu, Tumors and Cancers, 2017
Fibrosarcoma of soft tissue is distinct from other fibroblastic or myofibroblastic tumors, such as myxofibrosarcoma (superficial multiple myxoid nodules or a single deep mass with infiltrative margins; multinodular tumor composed of pleomorphic spindle cells in myxoid background; curvilinear vessels [thick walled with broad arc] with characteristic condensation of round to spindled cells around vessels, incomplete fibrous septa, myxoid stroma [in 10% of cases], and infiltrating immature dendritic cells; high-grade tumors showing more cellular with atypical mitotic figures, hemorrhage, necrosis, and possibly bizarre multinucleated giant cells; positivity for muscle markers [HHF35, α-smooth muscle actin, calponin, desmin, and vimentin]), low-grade fibromyxoid sarcoma (also called Evans tumor, hyalinizing spindle cell tumor with giant rosettes; a well-circumscribed, slow-growing, painless mass of 6 cm [range 1–18 cm] with a fibromyxoid cut surface and gross infiltration; low cellularity, bland fibroblastic cells, and curvilinear or arcuate vessels; recurrence in 64%, metastasis in 45%, death in 42%; t(7;16)(q33;p11) in 66%, supernumerary ring chromosome in 25%), and sclerosing epithelioid fibrosarcoma (a grossly well-circumscribed, lobulated, bosselated, or multinodular, painful mass of 9 cm [range 4–22 cm] in deep soft tissue of muscle associated with fascia or periosteum, firm, gray-white cut surface; nests and cords of small to medium size, round to ovoid, relatively uniform epithelioid cells; hemangiopericytoma-like vasculature, vascular invasion, inconspicuous mitoses 4/10 high-power field [HPF]; histologically low grade but clinically aggressive with local recurrence in 50% and distal metastases in 43%; negativity for CD34, CD45 [leukocyte common antigen (LCA)], HMB45, desmin, α-smooth muscle actin, and CD68).
Determinants of Treatment Toxicity in Patients with Soft Tissue Sarcomas
Published in Nutrition and Cancer, 2023
Katja A. Schönenberger, Emilie Reber, Karin Schläppi, Annic Baumgartner, Zeno Stanga, Attila Kollár
The three most common soft tissue sarcomas (UPS, leiomyo- and liposarcoma) represented 70.7% of the population. Other sarcoma subtypes included were angiosarcoma, pleomorphic rhabdomyosarcoma, malignant peripheral nerve sheet tumor, solitary fibrous tumor and sclerosing epithelioid fibrosarcoma. The primary origin of the sarcoma was located at the extremities in 25% of the patients. Most patients (70.8%) suffered from a high-grade disease (grade 2 and 3 according to the Fédération Nationale des Centers de Lutte Contre le Cancer (18)). Cancer was locally advanced in 12% of the patients and metastatic in 88% of the patients (n = 21). Pulmonary metastases were observed in 76% of patients (n = 16), as were nonpulmonary metastases (n = 16, 76%). Liver metastases were observed in 48% of the patients (n = 10). The presence of liver metastases was not significantly associated with toxicity in the study population.
Sclerosing epithelioid fibrosarcoma of the lumbar spine: a case report and review of the literature
Published in British Journal of Neurosurgery, 2023
Hai-Tao Liu, Yue-Hui Zhang, Jia Song, Jiang Shao
Sclerosing epithelioid fibrosarcoma (SEF) is a rare subtype of carcinoma. It was first reported by Meis-Kindblom et al. in 1995 as a distinct soft tissue tumour occurring primarily in deep musculature and frequently associated with the adjacent fascia or periosteum.1–3 Histologically, SEF is characterized by proliferation of neoplastic oval or round cells arranged in cords or nest-like distribution against a collagen background with prominent features of sclerosis.4 It was believed to be a low-grade tumour with an indolent course, but many reports confirm that SEF has malignant potential, with a high rate of recurrence, metastasis, and poor clinical prognosis.4 Mortality with SEF can range from 25 to 57%.5