Desmoplastic fibroma of the mandible in an infant

We report the CT and MR imaging findings of a case of a desmoplastic fibroma of the mandible in a 6-month-old girl who presented with a rapidly enlarging facial mass and who represents one of the youngest reported children with this rare tumor. Although imaging is non-specific and these lesions are best distinguished histologically, the desmoplastic fibroma should be considered when there is an occurrence of any bubbly or cystic expansile mandibular lesion presenting in childhood.The opinions and assertions contained herein are the private views of the authors and are not to be construed as official or as reflecting the views of the the United States Department of Defense.     

Juvenile (calcifying) aponeurotic fibroma of the neck

A case of juvenile (calcifying) aponeurotic fibroma (JAF) arising in the neck of a 5-year-old female is presented. The lesion was misdiagnosed as fibrous hamartoma of infancy (FHI) on a superficial biopsy prior to total excision of the mass. Only one instance of JAF of the neck has previously been reported; the tumor is usually located in the distal extremities and often recurs following excision. FHI occurs most frequently at and about the axilla and shoulder, and does not recur in most instances. Accepted: 14 January 1997     

MR imaging of calcifying aponeurotic fibroma of the thigh

Calcifying aponeurotic fibroma is a rare, benign soft-tissue proliferation that occurs in the distal extremities in children. Because this lesion has a tendency to recur after surgical resection, MR imaging to determine the extent of the tumor for surgical planning is optimal. We report the MR findings in a 4-year-old boy with a calcifying aponeurotic fibroma of the thigh.     

Successful excision of an atrial fibroma in a four-month-old male infant

Summary This report presents an infant who was initially admitted with a pericardial effusion. Subsequent evaluation led to the diagnosis of a primary cardiac tumor. Excision of the tumor which arose from the atrial septum was possible only by removing most of the free right atrial wall and a portion of the septal leaflet of the tricuspid valve. Reconstruction of the tricuspid valve leaflet by reattachment of chordae and a partial annuloplasty was done with the right atrial wall being replaced using pericardial tissue. The histology of the tumor was compatible with a fibroma, a type not previously reported in this location in the pediatric age group. Twenty-one months following operation, the child was restudied and the catheterization revealed mild to moderate tricuspid regurgitation and no recurrence of the tumor. The child is now 4 years of age and remains asymptomatic with normal growth and development and no evidence of fibromatosis.     

"Pedunculated tumor" of the right ventricle in an infant

A infant is reported who had pulmonary valve stenosis with a pedunculated throbus in the right ventricle. On angiocardiography this appeared as a tumour. The origin of the thrombus and the diagnostic problems interpreting the angiocardiographic appearances are discussed.     

Juvenile aponeurotic fibroma (Keasbey tumor) with metastatic course. Apropos of a case

Juvenile aponeurotic fibroma, isolated in 1953 by Louisa Keasbey is a rare tumour which is characterized by its recurrence and infiltration, but which has no tendency to metastasis. The case we report differs in its long-term outcome. The tumour diagnosed in a seven-year-old child has recurred locally for seven years, necessitated hand amputation, and finally developed a fibroblastic process extending to lung and pleura which caused the child's death. This new finding in Keasbey's disease led us to reconsider the nosological situation in juvenile fibromatoses and its reputation for benignity.     

Cardiovocal syndrome in an infant with a double outlet of the right ventricle

A 9-month-old boy with cardiovocal syndrome-paralysis of the left recurrent laryngeal nerve, and congenital heart disease — a double outlet of the right ventricle and pulmonary arterial hypertension is described. He had a hoarse cry due to left recurrent laryngeal nerve paralysis, which was diagnosed by indirect laryngoscopy. Paralysis was caused by traction of the enlarged great arteries on the left recurrent laryngeal nerve. After heart surgery the infant's voice returned to normal. It is important to recognize this rare syndrome at an early stage, as it may cause difficulties in post-operative respiratory management.