Document Detail

Congenital generalized myofibromatosis: a disseminated angiocentric myofibromatosis.
MedLine Citation:
PMID:  8597844     Owner:  NLM     Status:  MEDLINE    
Infantile myofibromatosis occurs in solitary, multiple, and generalized forms, with similar histology but different clinicopathologic and prognostic implications. We report the findings in two male infants with fatal congenital generalized myofibromatosis (CGMF) who presented with multiple dermal and subcutaneous nodules at birth. Imaging studies revealed bony and visceral lesions, which progressed despite chemotherapy. One infant had severe hypercalcemia associated with extensive lytic bone lesions. Both infants died in respiratory failure and had a combination of pulmonary CGMF and diffuse alveolar damage. Involvement of skin, soft tissue, bone, heart, lungs, liver, gastrointestinal tract, and endocrine organs was confirmed at autopsy in each case. A consistent histologic pattern of interlacing fascicles of myofibroblasts with abundant eosinophilic cytoplasm was noted, with variable necrosis and calcifications in some sites. The myofibroblasts displayed vimentin and smooth muscle actin immunoreactivity. The lungs in each case had the presumably early lesions of CGMF with an angiocentric and perivascular growth of myofibroblasts. A similar vascular pattern was present in all affected organs. These two cases demonstrate the extraordinary presentation of CGMF, which suggests its multifocal origin from vascular subintimal mesenchymal or smooth muscle cells whose phenotype is that of myofibroblasts.
C M Coffin; K A Neilson; S Ingels; R Frank-Gerszberg; L P Dehner
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Publication Detail:
Type:  Case Reports; Journal Article    
Journal Detail:
Title:  Pediatric pathology & laboratory medicine : journal of the Society for Pediatric Pathology, affiliated with the International Paediatric Pathology Association     Volume:  15     ISSN:  1077-1042     ISO Abbreviation:  Pediatr Pathol Lab Med     Publication Date:    1995 Jul-Aug
Date Detail:
Created Date:  1996-04-22     Completed Date:  1996-04-22     Revised Date:  2004-11-17    
Medline Journal Info:
Nlm Unique ID:  9518033     Medline TA:  Pediatr Pathol Lab Med     Country:  UNITED STATES    
Other Details:
Languages:  eng     Pagination:  571-87     Citation Subset:  IM    
Lauren V. Ackerman Division of Surgical Pathology, Barnes Children's Hospital, St. Louis, Missouri, USA.
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MeSH Terms
Adrenal Gland Neoplasms / blood supply
Hemangiopericytoma / congenital,  pathology
Infant, Newborn
Lung Neoplasms / blood supply
Myofibromatosis / congenital*,  pathology*
Neoplasms, Vascular Tissue / congenital*,  pathology*

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