Multiple granular cell tumors in a child with Noonan syndrome.
Granular cell tumors are benign neurally derived neoplasms, involving cutaneous and subcutaneous tissues; and typically occur as solitary lesions. Multiple granular cell tumors occur in 10% of affected individuals, but are in children. Children with underlying somatic and genetic syndromes, including neurofibromatosis and Noonan syndrome, appear to be at higher risk of developing multiple granular cell tumors. Skin biopsy assists in diagnosis, since granular cell tumors have a similar appearance to other cutaneous nodules. Painful or rapidly growing granular cell tumors should be excised and asymptomatic non-growing granular cell tumors may be observed. Children with multiple granular cell tumors should have a complete physical examination to rule out an underlying genetic syndrome.
Ramaswamy
Preethi V
PV
Dartmouth Medical School, Dartmouth Hitchcock Medical Center, One Medical Center Drive, Lebanon, New Hampshire 03756, USA.
Storm
Craig A
CA
Filiano
James J
JJ
Dinulos
James G H
JG
D002648:Child; D005260:Female; D016586:Granular Cell Tumor / Q000175:diagnosis* / Q000235:genetics / Q000473:pathology / Q000601:surgery; D006801:Humans; D009378:Neoplasms, Multiple Primary / Q000175:diagnosis* / Q000235:genetics / Q000473:pathology / Q000601:surgery; D009634:Noonan Syndrome / Q000175:diagnosis* / Q000235:genetics; D054592:Protein Tyrosine Phosphatase, Non-Receptor Type 11 / Q000235:genetics; D012878:Skin Neoplasms / Q000175:diagnosis* / Q000235:genetics / Q000473:pathology / Q000601:surgery; D016896:Treatment Outcome
C516724:PTPN11 protein, human; D054592:Protein Tyrosine Phosphatase, Non-Receptor Type 11