Proliferative Fasciitis in Childhood: A Review of Clinical Data Apropos of a Case.

Proliferative Fasciitis (PF) is a benign lesion with histologic and clinical features overlapping with those of malignant soft tissue tumours. Its occurrence in children is considered very rare. We present a case of PF appearing as a painful, red, gradually increasing in size lesion, during a period of a few weeks, on the finger of a five-year-old boy compromising the dermis and subcutaneous tissue. We were able to locate literature on 20 paediatric PF cases, which we review. Only five of these focus on the clinical data, the rest describing mainly histological findings. It is the first reported paediatric case appearing on the finger.

Imperforate Anus with Fistula Exiting at the Penile Skin.

We present the case of a male neonate with imperforate anus and a fistula exiting on the penile skin. Anorectal malformations in boys often present themselves with an entero-perineal or entero-urinary tract fistula, the type of which is a key feature for the classification and the treatment plan. A fistula exiting in front of the scrotum, such as described in our case, is very rare and is not incorporated in the current classification and treatment algorithms. Scarce reports on misjudgment concerning the position of the blind rectal pouch in similar cases, led us to perform a colostomy instead of a one-stage correction. A posterior sagittal anorectoplasty was performed eight months later and the rectal pouch was found inside the levator sling, justifying the cautious approach. The colostomy was closed three months later and after six months the distal part of the fistula was excised. We believe that in cases with a rare fistula presentation, the position of the rectal pouch is not predictable and the surgeon should proceed with caution.

Recurrent giant cell fibroblastoma: Malignancy predisposition in Kabuki syndrome revisited.

Kabuki syndrome is a genetic condition characterized by distinctive facial phenotype, mental retardation, and internal organ malformations. Mutations of the epigenetic genes KMT2D and KDM6A cause dysregulation of certain developmental genes and account for the multiple congenital anomalies of the syndrome. Eight cases of malignancies have been reported in young patients with Kabuki syndrome although a causative association to the syndrome has not been established. We report a case of a 12-year-old girl with Kabuki syndrome who developed a tumor on the right side of her neck. A relapsing tumor 19 months after initial excision, proved to be giant cell fibroblastoma. Τhis is the first report of giant cell fibroblastoma -a rare tumor of childhood- in a patient with Kabuki syndrome.