Epithelioid inflammatory myofibroblastic sarcoma with VCL–ALK fusion of central nervous system: case report and brief review of the literature

Chopra, Shefali; Maloney, Nolan; Wang, Wei Lien

doi:10.1007/s10014-021-00416-z

Cited by 13 publications

(21 citation statements)

References 29 publications

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“…In 2021, Chopra et al reported the first case of EIMS with a VCL-ALK fusion [ 79 ]. VCL–ALK fusion has been described in renal cell carcinomas, a single case of high-grade glioma and a subset of epithelioid fibrous histiocytoma.…”

Section: Available Clinical Data On Epithelioid Inflammatory Myofibro...mentioning

confidence: 99%

Inflammatory Myofibroblastic Tumour: State of the Art

Gros

Tos

Jones

et al. 2022

Cancers

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An inflammatory myofibroblastic tumor (IMT) is a neoplasm composed of myofibroblastic and fibroblastic spindle cells accompanied by inflammatory cells, including lymphocytes and eosinophils. It is an ultra-rare tumor, the optimal management of which remains to be defined. Surgery is the treatment of choice for localized tumors. The treatment of advanced disease is not precisely defined. Chemotherapy regimens result in an overall response rate of approximately 50% based on retrospective data. The latest pathophysiological data highlight the role played by tyrosine kinase fusion genes in IMT proliferation. Anaplast lymphoma kinase (ALK) oncogenic activation mechanisms have been characterized in approximately 80% of IMTs. In this context, data regarding targeted therapies are most important. The aims of this article are to review the latest published data on the use of systematic therapy, particularly the use of molecular targeted therapy, and to publish an additional case of an IMT with Ran-binding protein 2 (RANPB2)-ALK fusion showing a long response to a tyrosine kinase inhibitor.

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Section: Available Clinical Data On Epithelioid Inflammatory Myofibro...mentioning

confidence: 99%

Inflammatory Myofibroblastic Tumour: State of the Art

Gros

Tos

Jones

et al. 2022

Cancers

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“…EIMS is first named in 2011 ( 2 ) and is a more aggressive subtype of IMT and characterized by epithelioid-to-round cell morphology and prominent inflammatory infiltrate. EIMSs can occur across a wide age range (4 months to 76 years), with a male and intra-abdominal predominance ( 15 ). Recently, IMTs associated with EML4-ALK have been classified as EIMS ( 5 ).…”

Section: Discussionmentioning

confidence: 99%

“…Even with auxiliary detection, it is difficult to make a definitive diagnosis of EIMS on a small biopsy due to its genetic overlap with other ALK-positive tumors. To date, more than 40 cases of EIMS have been reported, among which 20 cases ( 2 , 5 , 7 , 11 , 15 , 17 – 28 ) as well as present case with the complete clinicopathological, immunohistochemical and genetic characteristics were summarized in Table 1 , 2 . These 21 cases were composed of 14 (67%) adults (21-72 years old) and 6 (29%) children or adolescents.…”

Section: Discussionmentioning

confidence: 99%

“…Comparison of computed tomography images before and after treatment with ensartinib. abdominal predominance (15). Recently, IMTs associated with EML4-ALK have been classified as EIMS (5).…”

Section: Discussionmentioning

confidence: 99%

“…Among 11 cases, all tumors were located in the abdomen and most originated in the mesentery or omentum ( 2 ). In addition to abdomen, several cases with extra-abdominal sites of EIMS have been reported, including liver ( 8 ), lung ( 7 , 9 ), pericardium ( 10 ), ovary ( 11 ), cutaneous ( 12 ), stomach ( 13 ), groin ( 14 ) and central nervous system ( 15 ). A variety of gene partners have been observed in IMT including NPM, TMP3/4, CARS, CLTC, EML4, DCTN1, SEC31L1, ATIC and FN1 ( 5 ), with more prevalent fusions of RANBP2 ( 2 ), RRBP1 ( 16 ) and EML4 ( 5 ) in EIMS.…”

Section: Introductionmentioning

confidence: 99%

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Case report: Epithelioid inflammatory myofibroblastic sarcoma treated with an ALK TKI ensartinib

Xing

Huang

et al. 2023

Front. Oncol.

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Epithelioid inflammatory myofibroblastic sarcoma (EIMS) is an aggressive variant of inflammatory myofibroblastic tumor (IMT) and has a poor prognosis. EIMS is characterized by epithelioid morphology, neutrophilic infiltrate and specific fusion partners of anaplastic lymphoma kinase (ALK). Despite no standard therapy for EIMS, ALK tyrosine kinase inhibitors (TKIs) are recommended for these tumors. The present case describes an abdominal mass that presented in a 31-year-old male. The patient suffered from recurrence and multiple metastases 2 months after surgery. Ensartinib was administered and RANBP2-ALK fusion was detected. A partial response has been observed for 4 months and there has been no recurrence. This study provided a successful case with sustained response of targeted therapy.

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