Primary pulmonary hyalinising spindle cell tumour with giant rosettes

Whale, Karen; Bennett, Genevieve

doi:10.1097/pat.0000000000000128

Cited by 2 publications

(3 citation statements)

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“…The morphologic results showed that 9 (9/26, 34.6%) cases were classic LGFMS and 13 (13/26, 50.0%) cases were LGFMS with a collagen rosette structure. In addition, 4 (4/26, 15.4%) cases had unusual features, consisting of 1 case with collagen rosettes and epithelioid component, 1 case with collagen rosettes and SEF-like areas, 1 lesion with round and epithelioid cell areas, and 1 case with classic morphology in the primary tumor and pleomorphic areas in the recurrent tumor ( 22 , 29 , 37 , 42 ). The frequency of these unusual features in the intrathoracic LGFMS (15.4%) was slightly higher than that of the overall LGFMS cases (<10%) ( 1 ).…”

Section: Discussionmentioning

confidence: 99%

“…Eleven historical intrathoracic cases had molecular results, including 3 cases with FUS rearrangement and 5 cases with FUS::CREB3L2 fusion gene. The genetic analysis of 2 cases was performed for differential diagnosis, and another 1 case was proved to be negative for FUS rearrangement but positive for MUC4 immunostaining ( 24 , 28 , 29 , 31 , 37 , 40 – 42 ). MUC4 could also be positive in other lesions, such as in alveolar rhabdomyosarcomas ( 44 ).…”

Section: Discussionmentioning

confidence: 99%

“…Additionally, LGFMSs with SEF-like areas and round-cell morphologic changes were reported to have more aggressive behavior ( 2 ). Four previous intrathoracic LGFMS cases had such morphologic change, and three of them had available follow-up information: one case was alive without disease, one case died of cardiac failure, and one case died of pericardial effusion and tamponade ( 22 , 29 , 37 , 42 ). Hence, more cases are needed to verify the relation between the SEF-like and round-cell components with the behavior and prognosis of LGFMS cases.…”

Section: Discussionmentioning

confidence: 99%

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Case report: Primary pleural low-grade fibromyxoid sarcoma in a 4-year-old boy with molecular confirmation

He,

Jing,

et al. 2023

Front. Oncol.

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Low-grade fibromyxoid sarcoma (LGFMS) is a rare malignant fibroblastic tumor, principally affecting the deep tissues of the proximal trunk and extremities in young adults. However, primary pleural LGFMS is extremely rare, and only three cases have been reported in the previous English literature without genetic confirmation. Furthermore, the historical pleural LGFMS cases were all adults, and the primary pleural LGFMS in children has never been reported to date. Here, we presented a primary pleural LGFMS in a 4-year-old boy with detailed clinical, pathological, and molecular results. Histologically, the current tumor showed typical alternating collagenous and myxoid areas, containing spindled or oval tumor cells arranged in a whorled and short fascicular pattern. In some areas, the tumor cells exhibited moderate atypia, and mitotic figures were identified but without the identification of giant collagen rosettes. Immunohistochemically, all the neoplastic cells showed strong and diffuse positivity for MUC4. Genetically, FUS gene rearrangement was revealed by fluorescence in-situ hybridization (FISH), and subsequently, next-generation sequencing (NGS) and polymerase chain reaction (PCR) further demonstrated the FUS::CREB3L2 fusion transcript. To the best of our knowledge, this is the first case of primary pleural LGFMS with the identification of FUS gene rearrangement and FUS::CREB3L2 fusion in a 4-year-old child. Our study expands the age range of pleural LGFMS and highlights the combination of morphological, immunohistochemical, and molecular analyses in such challenging cases.

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