Infantile fibrosarcoma (IFS) is a malignant tumor, commonly presenting in long bones and seldom encountered after 2 years of age. It is extremely rare in the faciomaxillary region. The condition is often mistaken for teratomas, and histopathology/immunohistochemistry is confirmatory. Treatment involves surgical resection as primary modality. Prognosis is much better compared to the adult variant with even scope for spontaneous regression following "conservative" resection. We present a case of IFS of the nasomaxillary complex in a 3-month-old female child. We managed our case with surgical resection, and the patient has remained disease free for over 2 years.
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http://dx.doi.org/10.4103/ams.ams_281_18 | DOI Listing |
J Clin Oncol
January 2025
Children's Hospital of Philadelphia/University of Pennsylvania, Philadelphia, PA.
Larotrectinib is a highly selective tropomyosin receptor kinase (TRK) inhibitor with efficacy in children with TRK fusion tumors. We evaluated patient outcomes after elective discontinuation of larotrectinib in the absence of disease progression in a protocol-defined wait-and-see subset analysis of eligible patients where treatment resumption with larotrectinib was allowed if disease progressed. We also assessed the safety and efficacy of larotrectinib in all pediatric patients with sarcoma.
View Article and Find Full Text PDFZhonghua Bing Li Xue Za Zhi
February 2025
Department of Pathology, Foshan Traditional Chinese Medicine Hospital, Foshan 528000, China.
To investigate the clinicopathological and genetic features of infantile rhabdomyofibrosarcoma (IRFS) with EGFR kinase domain duplication (EGFR-KDD). The clinical, morphological and immunohistochemical features of three IRFS with EGFR-KDD diagnosed from January 2022 to January 2024 at Department of Pathology, Foshan Traditional Chinese Medicine Hospital, Foshan, China were retrospectively analyzed using PCR or next generation sequencing technique; and related literature was reviewed. There were 1 male and 2 females, aged at presentation ranging from 1 to 4 years.
View Article and Find Full Text PDFHead Neck
December 2024
Department of Pediatric Hematology & Oncology, Klinik für Kinder- Und Jugendmedizin, Universitätsmedizin Rostock, Rostock, Germany.
Background: Infantile fibrosarcoma (IFS) is a rare pediatric tumor of intermediate malignancy with high local aggressiveness that typically presents in young infants. Its occurrence in the head and neck region is rare. Complete non-mutilating surgical resection is often not possible, requiring multimodal treatment.
View Article and Find Full Text PDFCytopathology
December 2024
Pathology Department, Union Hospital, Tongji Medical College, Huazhong University of Science and Technology, Wuhan, China.
NTRK (neurotropic tropomyosin receptor kinase)-rearranged spindle cell tumours represent a rare group of molecularly defined soft tissue neoplasms. These tumours, excluding infantile fibrosarcomas, are characterised by NTRK gene rearrangements and exhibit a range of histomorphologies, including spindle, epithelioid or rhabdoid cells with invasive growth. Their prognosis correlates with histological grade, and surgical resection is the primary treatment.
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