Jove
Visualize
Contact Us
JoVE
x logofacebook logolinkedin logoyoutube logo
ABOUT JoVE
OverviewLeadershipBlogJoVE Help Center
AUTHORS
Publishing ProcessEditorial BoardScope & PoliciesPeer ReviewFAQSubmit
LIBRARIANS
TestimonialsSubscriptionsAccessResourcesLibrary Advisory BoardFAQ
RESEARCH
JoVE JournalMethods CollectionsJoVE Encyclopedia of ExperimentsArchive
EDUCATION
JoVE CoreJoVE BusinessJoVE Science EducationJoVE Lab ManualFaculty Resource CenterFaculty Site
Terms & Conditions of Use
Privacy Policy
Policies

Related Experiment Videos

Infantile myofibromatosis

E B Chung, F M Enzinger

    Cancer
    |October 15, 1981
    PubMed
    Summary
    This summary is machine-generated.

    Congenital and infantile myofibromatosis is a rare tumor primarily affecting infants and young children. This study details 61 cases, distinguishing solitary and multicentric forms with varied clinical presentations and outcomes.

    Related Concept Videos

    You might also read

    Related Articles

    Articles linked to this work by shared authors, journal, and citation graph.

    Sort by
    Same author

    A clinicopathologic study of 45 pediatric soft tissue tumors with an admixture of adipose tissue and fibroblastic elements, and a proposal for classification as lipofibromatosis.

    The American journal of surgical pathology·2000
    Same author

    Epithelioid variant of pleomorphic liposarcoma: a study of 12 cases of a distinctive variant of high-grade liposarcoma.

    Modern pathology : an official journal of the United States and Canadian Academy of Pathology, Inc·1999
    Same author

    Sclerosing epithelioid fibrosarcoma. A variant of fibrosarcoma simulating carcinoma.

    The American journal of surgical pathology·1995
    Same author

    Variants of liposarcoma.

    The American journal of surgical pathology·1995
    Same author

    Plexiform malignant peripheral nerve sheath tumor of infancy and childhood.

    The American journal of surgical pathology·1994
    Same author

    Chondroid lipoma. A unique tumor simulating liposarcoma and myxoid chondrosarcoma.

    The American journal of surgical pathology·1993

    Area of Science:

    • Pediatric Pathology
    • Oncology
    • Dermatopathology

    Background:

    • Congenital and infantile myofibromatosis is a rare mesenchymal tumor.
    • It predominantly affects infants and young children, with most cases presenting at or shortly after birth.

    Purpose of the Study:

    • To present the clinical and pathological features of 61 cases of congenital and infantile myofibromatosis.
    • To distinguish between solitary and multicentric forms and analyze their characteristics.

    Main Methods:

    • Retrospective review of 61 cases of congenital and infantile myofibromatosis.
    • Analysis of clinical presentation, pathological findings, and patient outcomes.

    Main Results:

    • The tumor occurred in 88% of cases before age two, with 60% noted at birth.

    Related Experiment Videos

  • Solitary tumors (45 cases) were more common in males and affected head-neck/trunk soft tissues.
  • Multicentric tumors (16 cases) predominated in females, affecting soft tissues, bones, and viscera; 14/16 were congenital, 4 with visceral involvement.
  • Microscopically, tumors showed well-circumscribed nodules of spindle cells with intermediate staining characteristics.
  • Of 43 patients with follow-up, 36 were alive and well; 3 solitary lesions recurred.
  • Five of 15 multicentric lesions showed spontaneous regression; 4 patients died, 3 from visceral involvement.

    • Conclusions:

    • Congenital and infantile myofibromatosis exhibits distinct solitary and multicentric patterns with different demographic and site predilections.
    • While many cases have favorable outcomes, multicentric forms, especially with visceral involvement, carry a higher risk of mortality.
    • Spontaneous regression can occur in multicentric lesions, suggesting varied biological behavior.