136 results match your criteria: "Infantile Digital Fibromatosis"

Real-World Performance of Integrative Clinical Genomics in Pediatric Precision Oncology.

Lab Invest

December 2024

Central European Institute of Technology, Masaryk University, Brno, Czech Republic; Department of Biology, Faculty of Medicine, Masaryk University, Brno, Czech Republic; Center for Precision Medicine, University Hospital Brno, Brno, Czech Republic; Department of Pathology, University Hospital Brno and Faculty of Medicine, Masaryk University, Brno, Czech Republic. Electronic address:

Despite significant improvement in the survival of pediatric patients with cancer, treatment outcomes for high-risk, relapsed, and refractory cancers remain unsatisfactory. Moreover, prolonged survival is frequently associated with long-term adverse effects due to intensive multimodal treatments. Accelerating the progress of pediatric oncology requires both therapeutic advances and strategies to mitigate the long-term cytotoxic side effects, potentially through targeting specific molecular drivers of pediatric malignancies.

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Pediatric Fibromatosis Lacks the Internal Tandem Duplication of EGFR Seen in Congenital Mesoblastic Nephroma.

Genes Chromosomes Cancer

September 2024

Department of Laboratory Medicine and Pathobiology, University of Toronto, Toronto, Ontario, Canada.

Article Synopsis
  • Classical and mixed congenital mesoblastic nephroma (CMN) show an internal tandem duplication (ITD) of the EGFR gene, while cellular CMN typically has an ETV6::NTRK3 gene fusion, which is also found in infantile fibrosarcoma.
  • A study on pediatric fibromatosis revealed that none of the cases exhibited EGFR ITD, suggesting that fibromatosis is not characterized by this genetic alteration.
  • The findings indicate that soft tissue tumors with EGFR ITD may resemble infantile fibrosarcoma or mixed CMN, but further research is needed to understand their biological behavior in comparison to fibrosarcoma and other related tumors.
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  • The study reports a rare case of a borderline phyllodes tumor in a 33-year-old woman, highlighting the unprecedented presence of eosinophilic inclusion bodies in the breast.
  • The research includes comprehensive diagnostic imaging and surgical intervention, leading to the identification of these inclusion bodies through molecular testing for the first time.
  • Although these inclusion bodies are uncommon in breast tumors and their significance remains unclear, they do not appear to influence the prognosis, suggesting further investigation could reveal potential mutations.
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Article Synopsis
  • Infantile digital fibromatosis (IDF) is a rare, benign tumor seen in infants and children, usually appearing as a single nodule made up of spindle cells in the skin of the fingers and toes.
  • Diagnosis often involves a biopsy, and treatment options generally include observation, corticosteroid injections, or surgical removal of the tumor.
  • Due to its rarity, many healthcare providers have limited experience with IDF, prompting a detailed review of its presentation, diagnosis, and treatment methods.
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Infantile Digital Fibromatosis.

Indian Pediatr

November 2021

Department of Pediatrics, Division of Pediatric Hematology/Oncology, Eskiºehir Osmangazi University Faculty of Medicine, 26480, Meºelik Campus, Eskiºehir, Turkey.

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Article Synopsis
  • Infantile digital fibromatosis (IDF) is a rare benign tumor primarily found in the fingers and toes of young children, often present at birth.
  • Diagnosis involves identifying specific inclusions in tissue samples, and while surgery is common, there is a high chance of the tumor returning.
  • This case study highlights a 5-month-old in Nigeria with IDF who had surgery, emphasizing the need for awareness of this condition in younger patients to guide proper treatment.
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Article Synopsis
  • Infantile digital fibromatosis is a rare, benign tumor primarily affecting infants, typically located on fingers and toes, and often shows spontaneous regression despite a high recurrence rate following surgery.
  • Although there has been some speculation about a link between this condition and human papillomavirus (HPV), reports are limited and inconsistent.
  • This case study highlights a recurrent instance of infantile digital fibromatosis with identified HPV infection after surgical removal, suggesting a potential connection that warrants further investigation.
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Article Synopsis
  • - Fibroblastic/myofibroblastic tumors represent 12% of pediatric soft tissue tumors, mostly benign or intermediate, but can be misdiagnosed due to diverse symptoms and unique microscopic characteristics.
  • - The study analyzed 7 cases of benign and intermediate tumors in children aged 0-12 years diagnosed through Fine Needle Aspiration Cytology (FNAC) over 3½ years, with conditions like Infantile Digital Fibromatosis and Fibrous Hamartoma of Infancy involved.
  • - FNAC smears of these lesions often showed limited cells with benign spindle-shaped cells in a collagen-rich background, highlighting the importance of proper cytological assessment for accurate diagnosis in pediatric soft tissue tumors.
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Article Synopsis
  • Fibromatoses are benign tumors made up of fibroblasts that grow aggressively and often return after treatment; they can be categorized into superficial and deep types, with superficial fibromatoses generally growing more slowly.
  • Superficial fibromatoses include conditions like palmar and plantar fibromatosis, and they typically present as nodules or cords under the skin, which can limit movement in the affected areas.
  • Treatment options range from monitoring to non-invasive techniques, and radical surgery; early intervention is crucial to prevent progression and maintain function.
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  • Terminal osseous dysplasia is a rare genetic syndrome linked to the X chromosome, believed to be lethal before birth for males.
  • The syndrome presents unique skin characteristics and has been associated with specific inclusion bodies in cells called fibroblasts.
  • This report is the first to link these inclusion bodies in digital fibromas with terminal osseous dysplasia, suggesting a new understanding of the condition.
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Article Synopsis
  • Infantile digital fibromatosis is a rare, benign tumor in children, primarily affecting the fingers and toes, characterized by specific histological features.
  • The article reviews diagnosis and treatment options by analyzing 20 publications from the PubMed/MEDLINE database and creating a treatment algorithm.
  • A successful case study involved excising a symptomatic nodule from a 9-month-old child, with no complications or recurrence noted after two years, while asymptomatic nodules may be monitored or treated nonsurgically.
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Article Synopsis
  • Infantile Digital Fibromatosis (IDF) is a rare non-cancerous growth that commonly affects fingers, typically seen at birth or in early childhood.
  • Treatment options are debated, partly because the condition often goes away on its own after the first year of life, but surgery has a high chance of recurrence.
  • The case study highlights a patient with IDF who experienced joint contracture and functional issues, successfully treated with cryotherapy and occupational therapy, leading to excellent outcomes.
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Infantile digital fibromatosis-like tumor of adult.

G Ital Dermatol Venereol

February 2018

Unit of Dermatology, Department of Experimental, Diagnostic and Specialty Medicine, University of Bologna, Bologna, Italy.

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Surgical approaches, anaesthetic management and outcome in pediatric superior mediastinal tumors.

J Egypt Natl Canc Inst

September 2017

Surgical Oncology Department, Children's Cancer Hospital (CCHE), Egypt; Department of Surgery, National Cancer Institute, Cairo University, Egypt.

Article Synopsis
  • Pediatric superior mediastinal tumors are diverse in type and growth, and this study assessed various surgical methods used for their removal in children's cases at a specific hospital in Egypt from 2008 to 2015.
  • The study involved 20 patients with different tumor types—including germ cell tumors and neuroblastoma—and categorized them based on how far the tumors extended in the body, with various surgical techniques utilized for removal.
  • No patients died during surgery, with a 20% rate of complications; by the end of 2016, most patients were in remission, but more research is needed due to the small sample size in this descriptive study.
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Congenital Infantile Fibrosarcoma Associated With a Lipofibromatosis-Like Component: One Train May Be Hiding Another.

Am J Dermatopathol

June 2017

Departments of *Maxillofacial and Plastic Surgery, and †Pathology, Necker Children Hospital, APHP, Paris, France; ‡Paris Descartes University, Paris, France; Departments of §Pediatric Radiology, and ¶Dermatology, Necker Children Hospital, APHP, Paris, France; ‖Laboratory of Solid Tumors Genetics and Central Laboratory of Pathology, Nice University Hospital, Nice, France; **Microenvironment, Signaling and Cancer, INSERM, Université Nice Sophia-Antipolis, Nice, France; ††Molecular Oral Pathology, INSERM, Centre de Recherche de Cordeliers, Paris, France; and ‡‡Adolescent and Pediatric Oncology Department, Institut Curie, Paris, France.

Article Synopsis
  • Congenital infantile fibrosarcoma (CIFS) is a type of soft tissue cancer commonly found in infants, primarily affecting the lower limbs and typically appearing in the first year of life.
  • The study highlights a unique case where CIFS appeared in conjunction with lipofibromatosis, a rare benign soft tissue tumor that impacts children.
  • The surrounding benign lipofibromatosis made it difficult to diagnose CIFS in a timely manner, causing confusion for healthcare providers.
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Infantile Digital Fibroma: A Rare Fibromatosis.

Arch Pathol Lab Med

October 2016

From the Department of Pathology, Montefiore Medical Center/Albert Einstein College of Medicine, Bronx, New York.

Article Synopsis
  • Infantile digital fibroma is a rare benign tumor found primarily in infants during their first 2 years, often appearing as a single lesion but can sometimes be multiple.
  • These fibromas can grow large enough to cause joint deformities or hinder daily activities, and they typically show distinctive cells under a microscope.
  • Treatment usually involves monitoring since many lesions disappear on their own, but if necessary, excision or newer methods like fluorouracil or steroids may be used to safely encourage regression.
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Article Synopsis
  • A 16-month-old boy was initially diagnosed with infantile digital fibromatosis, but after 9 months of growth, the mass was surgically removed.
  • The histopathological examination revealed it was actually a benign nerve sheath myxoma, not fibromatosis.
  • This case highlights the importance of considering nerve sheath myxomas in the differential diagnosis of dermal nodules in infants.
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