Search our Database of Scientific Publications and Authors

I’m looking for a
    Congenital myofibromatosis in two siblings.
    Eur J Dermatol 2006 Mar-Apr;16(2):181-3
    Department of Dermatology, M. Bufalini Hospital, Viale Ghirotti, 286, 47023 Cesena, Italy.
    Infantile myofibromatosis (IM) is a rare mesenchymal disorder characterized by solitary or multiple nodules involving the skin, striated muscles, bones and, sometimes, viscera. Although most cases are sporadic, there have been a few reports of a familial pattern of inheritance. In most cases, diagnosing IM is not difficult. However, IM should be differentiated from neurofibromatosis, paediatric sarcomas, nodular fasciitis, fibrous hamartoma of infancy, and hyaline juvenile fibromatosis. The prognosis for IM depends on the degree of visceral involvement. Since spontaneous tumoral regression is usual, in cases of limited involvement therapeutic abstention and patient observation are recommended. Surgery, chemotherapy or radiotherapy are reserved for patients with multiple visceral localizations, particularly in the lung and gastrointestinal tract, which may have a worse prognosis and potentially fatal outcome. We report on a family in which two siblings, born from non-consanguineous parents, were affected by congenital myofibromatosis. In both patients, tumors were present at birth, with multicentric subcutaneous, muscular, skeletal and visceral involvement. The growing subcutaneous myofibromas were surgically removed, while the smallest ones disappeared spontaneously over the course of 24 months.

    Similar Publications

    [Familial infantile myofibromatosis].
    Ann Dermatol Venereol 2009 Apr 26;136(4):346-9. Epub 2009 Feb 26.
    Service de dermatologie, CHU Saint-Jacques, 2, place Saint-Jacques, 25000 Besançon cedex, France.
    Background: Infantile myofibromatosis (IM) is the most common fibrous disorder of infancy and childhood. It is characterized by congenital tumours of the skin, striated muscle, bones and viscera. Most cases are sporadic and few familial cases have been reported. Read More
    Imaging findings in seven cases of congenital infantile myofibromatosis with cerebral, spinal, or head and neck involvement.
    Neuroradiology 2012 Dec 16;54(12):1389-98. Epub 2012 Nov 16.
    Department of Radiology, Medical University Vienna, University Hospital Vienna, Waehringer Guertel 18-20, 1090, Vienna, Austria.
    Introduction: Congenital infantile myofibromatosis (IM) is a rare mesenchymal disease, presenting with tumors in the skin, muscle, viscera, bone, and subcutaneous tissue. It can present as (a) a solitary form with subcutaneous, erythematous nodules, (b) a multicentric form with subcutaneous, muscle, and/or bony lesions, and (c) a multicentric form with visceral involvement. Cerebral or spinal involvement in myofibromatosis has been reported rarely. Read More
    Multicentric infantile myofibromatosis: two perinatal cases.
    Eur J Pediatr 2007 Oct 21;166(10):997-1001. Epub 2006 Dec 21.
    Unité de Foetopathologie, Service d'Anatomie et Cytologie Pathologique, Centre Hospitalo-Universitaire de Bordeaux, Place Amélie Raba Léon, 33076, Bordeaux Cedex, France.
    Infantile myofibromatosis, the most common fibrous tumor of infancy, occurs in solitary, multiple, and generalized forms, with similar histology but different clinicopathologic and prognostic implications. This entity is a mesenchymal disorder characterized by the proliferation of fibrous tumors in the skin, muscles, viscera, bones, and subcutaneous tissues. Visceral lesions are associated with significant morbidity and mortality, generally within the first few months of life. Read More
    Multicentric infantile myofibromatosis.
    Int J Pediatr Otorhinolaryngol 1998 Oct;45(3):249-54
    Department of Otolaryngology-Head and Neck Surgery, Emory University, Atlanta, GA 30322, USA.
    Infantile myofibromatosis (IM) is a rare tumor of infancy and childhood, typically presenting as a firm, nodular mass involving soft tissue, bone or viscera. Approximately one-third of cases involve the head and neck. These tumors can be solitary or multicentric. Read More