Soft Tissues
Joseph Kovi, Hung Dinh Duong in Frozen Section In Surgical Pathology: An Atlas, 2019
DIFFERENTIAL DIAGNOSIS: ClinicalA soft tissue sarcoma of the thigh in a female between ages 30 to 40 is probably an alveolar soft part sarcoma.MicroscopicAlveolar soft part sarcoma is characterized by a distinct alveolar pattern. The cells lining the alveolar structures are large and possess vesicular nuclei with prominent nucleoli. The cytoplasm is faintly granular. In benign granular cell tumor the nuclei are small and the nucleoli are not conspicuous. Cytoplasmic granularity is more pronounced in granular cell tumor than in alveolar soft part sarcoma. In comparison to pleomorphic rhabdomyosarcoma the neoplastic cells in alveolar soft part sarcoma exhibit very faint eosinophilia. Nuclear pleomorphism is a major cytologic characteristic of rhabdomyosarcoma. The nuclei are rather regular, although relatively large in alveolar soft part sarcoma. Metastatic renal cell carcinoma may be extremely difficult to distinguish from alveolar soft part sarcoma. However, renal cell carcinoma occurs in the middle or late years of adult life and approximately two thirds of the cases are found in males.
Immune RNA and Tumor Immunity*
Edward P. Cohen, A. Arthur Gottlieb in Immune RNA, 2020
Two types of patients were studied: patients with grossly detectable and measureable metastatic disease and patients with “minimum residual disease.” Patients in the latter category had no clinically detectable disease following surgical resection of all gross tumor, but had a greater than 50% likelihood of developing recurrent and/or metastatic disease within 24 months. The histologic types of tumors treated included malignant melanoma, hypernephroma, sarcoma, breast carcinoma, cholangiocarcinoma, and carcinoma of the stomach. The number of patients within each disease category and of each tumor type is given in Table 2. Of the three sarcoma patients, one had an alveolar soft part sarcoma, one a liposarcoma, and one an osteogenic sarcoma. Patients with gross disease were accepted for treatment only if: They had failed all standard therapy (including standard chemotherapy).No standard therapy of proven efficacy existed.They had relatively stable disease and standard therapy could be interrupted or withheld for 8 weeks (the minimum duration of the study).
Soft tissue sarcomas
Pat Price, Karol Sikora in Treatment of Cancer, 2014
Although the foregoing generalizations apply to most STSs, different histologic subtypes of sarcomas display their own patterns of chemosensitivity.450 Myxoid/round cell liposarcomas appear sensitive to doxorubicin and to trabectedin.451 Synovial sarcoma has a strong dose response to ifosfamide.440 Non-uterine leiomyosarcomas appear to have lower response rates to doxorubicin and ifosfamide, but may respond to Trabectedin.452 Angiosarcomas are almost unique in being sensitive to paclitaxel.453 Rhabdomyosarcoma, desmoplastic small round cell tumors and peripheral neuroectodermal tumours respond to combinations that include ifosfamide, etoposide, vincristine, doxorubicin, dactinomycin cyclophosphamide and topotecan/irinotecan.454,455 Sunitinib has proven useful against solitary fibrous tumor/haemangiopericytoma, alveolar soft part sarcoma, and clear cell sarcoma.456 Sirolimus has activity against tumors with perivascular epithelioid cell differentiation.457 Imatinib can help in metastatic dermatofibroma protuberans.458 Cediranib is effective against alveolar soft part sarcoma.459 Crizotinib is active against ALK translocated inflammatory myofibroblastic tumour.460 Bevacizumab with temozolamide has been employed for solitary fibrous tumour.461
Malignant Perivascular Epitheloid Cell Tumor with an Unusual Immunophenotype in a Ten-Year-Old Child
Published in Fetal and Pediatric Pathology, 2019
Nives Jonjic, Jelena Roganovic, Nedeljka Glavan, Ita Hadzisejdic, Irena Seili-Bekafigo
The major differential diagnosis in the current case includes clear cell sarcoma since it occurs mainly in the soft tissues and dermis, predominantly in adolescents and young adults, and histologically is characterized by nested growth pattern and a mixture of spindled, epitheloid cells. However, clear cell sarcoma shows consistent positivity for S-100, HMB-45, and MITF, and negativity for myoid markers. Alveolar soft part sarcoma, characterized by an organoid pattern and a sinusoidal-type vasculature, as well as melanoma, may be confused with PEComa. Alveolar soft part sarcoma is negative for specific melanocytic markers while melanoma, similar to clear cell sarcoma, shows positivity for S-100, HMB-45 and MITF. Therefore, S-100 negativity in PECOMA can be useful in the differential diagnosis of malignant melanoma, even though up to 11% of PEComas express S-100 as well [7]. For the diagnosis of PEComa it is very important to include the negative history for melanoma, perivascular arrangement of tumor cells, immunoreactivity for myoid markers and absence of specific EWSR1/ATF1 fusion transcript resulting from t(12;22) (q13;q12) in clear cell sarcoma.
Metastatic TFE3-overexpressing renal clear cell carcinoma with dense granules: a histological, immunohistochemical, and ultrastructural study
Published in Ultrastructural Pathology, 2018
Shoujun Chen, Elba A. Turbat-Herrera, Guillermo A. Herrera, Meghna Chadha, Rodney E. Shackelford, Eric X. Wei
TFE3 is a member of MiTF family, which is a key regulator in melanocyte development. Gain of function of the gene has been associated with malignant melanoma, clear cell sarcoma, RCC, alveolar soft part sarcoma, and PEComa. In this reported case, HMB45 and MART-1 are negative and therefore help rule out PEComas and melanoma. Translocation of XP11.2 resulting in ASPL-TFE3 gene fusion is identical to what is seen in alveolar soft part sarcoma. In alveolar soft part sarcoma, there are PAS-D-positive cytoplasmic granules, which are not seen in this case. In addition, the Golgi bodies in the tumor cells are typically extensive and contain granules and dense structures which often merge with crystals with a linear periodicity, which may be due to the activation of ASP sarcoma L gene.1,7,8 Such structures if found in a renal cell neoplasm strongly suggest XP11.2 translocation RCC.9 Moreover, neurosecretory type of granules, intracisternal microtules similar to those seen in melanomas and extraskeletal myxoid chondrosarcomas is also reported.1 Interestingly, similar crystalline-like structures are also observed in this tumor, which supports our diagnosis.9 While the lattice is not as well defined as in the classical inclusions that can be seen in alveolar soft part sarcoma, the overall internal substructure is consistent with what has been described in association with cytoplasmic crystalline material. These atypical inclusions most likely reflect aberrant crystal formation in a neoplasm with a molecular signature similar to alveolar soft part sarcoma.
What’s the latest with investigational drugs for soft tissue sarcoma?
Published in Expert Opinion on Investigational Drugs, 2022
Elena Cojocaru, Andrea Napolitano, Cyril Fisher, Paul Huang, Robin L Jones, Khin Thway
The phase 3 trial APROMISS compared dacarbazine vs anlotinib in patients with synovial sarcoma, randomized in a 1:2 ratio [49]. Patients had the option of crossing over on the progression of their disease and the results were presented at the ASCO meeting in 2021. A total of 79 participants received treatment with either dacarbazine or anlotinib and the PFS was statistically significant in the anlotinib arm, at 2.89 months compared to 1.64 months in the control arm with dacarbazine. Although the duration of PFS was not remarkably longer, the number of patients with a prolonged PFS was significantly higher in the anlotinib arm at 4, 6 and 12 months compared to dacarbazine (48.1%, 42.3% and 26.0% compared to 14.85%, 11.1% and 3.7%). The study has completed accrual for synovial sarcoma and leiomyosarcoma and is currently recruiting patients with alveolar soft part sarcoma. More ongoing trials are currently studying the combination of anlotinib with other active drugs in the treatment of sarcomas such as nivolumab, (NCT04165330), pegylated liposomal doxorubicin (NCT 04765228) or toripalimab (NCT04172805).
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