CPT-11 chemotherapy rescued a patient with atypical sclerosing epithelioid fibrosarcoma from emergent condition

Pan, Chun-Hua; Han, Xiaoyan; Li, Jiansheng

doi:10.1007/s11670-012-0253-1

Cited by 7 publications

(2 citation statements)

References 10 publications

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“…Surgery appears to play an essential role in the management of this disease. The use of irinotecan19 as a rescue measure has been reported previously in SEF in a case report. Six patients (35.3%) were reported to have passed away.…”

Section: Discussionmentioning

confidence: 61%

Sclerosing epithelioid fibrosarcoma of the kidney

et al. 2023

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A woman in her 20s presented with headache and back pain and was found to have a left renal mass with bony metastases. She underwent nephrectomy, and on histopathology was initially diagnosed with stage 4 clear cell sarcoma of the kidney. She underwent palliative radiation and chemotherapy; however, the disease progressed and she came to our centre. We started her on second-line chemotherapy and submitted her tissue blocks for review. Due to her age and lack of sclerotic stroma in the tissue, we had our doubts about the diagnosis and hence, tissue sample was submitted for next-generation sequencing (NGS). NGS detected anEWSR1::CREBL1fusion, clinching the final diagnosis of sclerosing epithelioid fibrosarcoma of the kidney, a singular diagnosis rarely reported in the literature. Currently, the patient is post her third line of chemotherapy, is on maintenance, and is doing well and has resumed her daily activities.

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Section: Discussionmentioning

confidence: 61%

Sclerosing epithelioid fibrosarcoma of the kidney

et al. 2023

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“…Clinically, SEF demonstrates aggressive behavior with a mortality rate of up to 57%. Although Pan et al11 reported a case with brilliant response to Irinotecan, SEF is known as a chemo-resistant tumor.…”

Section: Discussionmentioning

confidence: 99%

Fibrosarcoma: a challenging diagnosis

Ferreira¹,

Fonseca²,

Piotto³

et al. 2013

ACR

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Fibrosarcoma represent a rare group of soft tissue malignancies derived from fibrous connective tissue and immature proliferating fibroblasts or undifferentiated anaplastic spindle cells. It affects patients in the fourth and fifth decade of life. Fibrosarcomas can be classified in subtypes such as low-grade fibromyxoid sarcoma (LGFMS) and sclerosing epithelioid fibrosarcoma (SEF), and others. Histological features that overlap between types of fibrosarcomas is well known and reported in the literature. We report the case of a 53-year-old patient who presented a tumor in the axillary fossa, which was initially diagnosed as a solitary fibrous tumor. Due to recurrence of the lesion, as well as the presence of distant metastases, the histological revision considered the diagnosis of breast metaplastic carcinoma, since the tumor expressed the p63 antigen and estrogen and progesterone receptors. Unexpected resistance to chemotherapy motivated the diagnosis re-evaluation, which was due to MUC4 expression and morphological characteristics concluded by a hybrid LGFMS-SEF tumor. The authors call attention to the difficult diagnosis in cases of soft tissue tumors. A broad panel of immunohistochemical research is required as the clinical course is essential to the final diagnosis.

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