ifosfamide

This multicentre study confirms that anthracycline-based regimens show activity in MCS, with responses more in line with soft tissue sarcoma than Ewing sarcoma. Their benefit in localized disease remains uncertain, but (neo)adjuvant chemotherapy with Ewing-like regimens should be considered for patients eligible for surgery. In advanced disease, trabectedin may provide prolonged disease control after anthracyclines.

P1, N=6, Active, not recruiting, Washington University School of Medicine | Phase classification: P1/2 --> P1 | N=35 --> 6 | Trial completion date: Jan 2029 --> Mar 2028 | Trial primary completion date: Jan 2027 --> Mar 2026 | Recruiting --> Active, not recruiting

P1/2, N=65, Recruiting, Hoffmann-La Roche | Trial completion date: Sep 2029 --> Nov 2032 | Trial primary completion date: May 2029 --> Nov 2029

The most promising salvage options for patients responding insufficiently to treatment are the chemotherapy combinations, topotecan/vincristine/doxorubicin (TVD), topotecan/cyclophosphamide/etoposide (TCE), ifosfamide/carboplatin/etoposide (ICE) or topotecan/cyclophosphamide (TopoCy), or [131I]-mIBG therapy. Early-phase clinical trials are also a possible option in this setting.

P=N/A, N=70, Not yet recruiting, The First Affiliated Hospital with Nanjing Medical University

P2, N=80, Recruiting, Mayo Clinic | Trial completion date: Mar 2026 --> Nov 2033 | Trial primary completion date: Dec 2025 --> Feb 2031

Postoperatively, the patient received adjuvant chemotherapy with the AIM (doxorubicin, ifosfamide, and mesna) regimen. Given the extreme rarity of PRSS, we discuss the diagnostic challenges, molecular characteristics, and treatment approach adopted at our institution, contributing to the limited but growing body of knowledge on this rare entity.

P2, N=34, Not yet recruiting, Shandong First Medical University Affiliated Cancer Hospital; Shandong First Medical University Affiliated Cancer Hospital

P2, N=58, Not yet recruiting, West China Hospital, Sichuan University; West China Hospital, Sichuan University

P1, N=29, Active, not recruiting, Roswell Park Cancer Institute | Trial completion date: Dec 2025 --> Dec 2026

He was treated with nine cycles of VDC-IE(vincristine, doxorubicin, and cyclophosphamide alternating with ifosfamide and etoposide) chemotherapy given at 3-week intervals, complicated by transient iron deficiency anaemia, followed by surgical excision with neck dissection and fibular flap reconstruction. Maintenance therapy with the histone deacetylase inhibitor vorinostat was initiated. This case highlights the importance of early recognition, multimodal therapy and emerging targeted treatment in improving outcomes for NUT carcinoma.

The patient underwent subtotal maxillectomy with intraoperative margin assessment, achieving complete surgical excision with histologically negative margins, followed by adjuvant chemotherapy with epirubicin, cisplatin, and ifosfamide, as well as localized radiotherapy. At 18 months of clinical and radiologic follow-up, there was no evidence of local recurrence, with preserved oral function and satisfactory aesthetic outcomes on clinical evaluation. This report highlights the importance of considering malignant small round cell tumors in rapidly enlarging maxillofacial lesions and emphasizes the role of multidisciplinary management in achieving sustained local disease control while preserving postoperative function.