Infantile myofibromatosis (IM) is a rare benign tumor in children. It was first described as congenital fibrosarcoma. Later, sporadic cases were discussed, and in 1981, 61 cases were examined and named as IM. Three different forms were described: solitary, multicentric and visceral IM.1 Three quarters of soft tissue tumours in children and adolescents are benign, 95 % of them are fibromatosis, ...

Spectrum of Fibroblastic and Myofibroblastic Tumors When one considers soft tissue tumors in pediatrics, tumors of vascular (29%), neurogenic (15%), and myogenic (striated muscle, 14%) origin occur more often than fibroblastic-myofibroblastic tumors (12%). The spectrum of fibroblastic/myofibroblastic tumors is quite divergent from both clinical and histopathologic viewpoints (Table 1). These tu...

BACKGROUND Treatment of recurrent aggressive fibromatosis (AF) following surgical resection is a clinical challenge. Non-steroidal anti-inflammatory drugs (NSAIDs) have been reported to be an effective option for controlling the disease. However, long-term NSAID use can result in unfavorable complications. This study was a trial of the use of intralesional steroid injection (ILSI) including inv...

Desmoid tumour/aggressive fibromatosis (DT/AF) is a rare soft-tissue neoplasm that is locally aggressive but does not metastasize. There is no standard systemic treatment for symptomatic patients, although a number of agents are used. Tyrosine kinase inhibitors have recently been reported to show useful activity. We reviewed our bi-institutional (Royal Marsden Hospital, Cambridge University Hos...

Aside from the CTNNB1 and adenomatous polyposis coli (APC) mutations, the genetic profile of pediatric aggressive fibromatosis (AF) has remained poorly characterized. The aim of this study was to shed more light on the mutational spectrum of pediatric AF, comparing it with its adult counterpart, with a view to identifying biomarkers for use as prognostic factors or new potential therapeutic tar...

INTRODUCTION Imatinib, a tyrosine kinase inhibitor, is a major therapeutic option for the management of unresectable aggressive fibromatosis. Unfortunately, for most patients of low or very low average income countries, surgery often is the first treatment option. This is related to unavailability of chemotherapy or targeted therapy, and to a lack of financial resources or surgeons' lack of kno...

Gingival hyperplasia is a rare condition but it is important for cosmetic and mechanic reasons and because of its potential as an indicator of systemic disease. Gingival fibromatosis may exist as an isolated abnormality or as part of a syndrome. Aggressive periodontitis, another genetically transmitted disorder of the periodontium, typically results in severe, rapid destruction of the toothsupp...

Desmoid-type fibromatosis is a locally aggressive fibroblastic neoplasm with a tendency for local recurrence, despite adequate surgical resection. Its clinical presentation, biological behavior, and natural history can vary considerably. We present a unique case of desmoid-type fibromatosis arising in the left teres major muscle of a 62-year-old female. Physical examination showed a 7-cm, elast...

  Gardner's syndrome is an autosomal dominant inherited disorder. Familial polyposis of the colon, osteomas, hypertrophy of the retinal-pigmented layer and a multitude of soft tissue tumors are characteristic features. The syndrome may be presented with colonic or extracolonic symptoms. A 75-year-old male patient presented to Al-zahra Clinic with diffuse abdominal pain. An abdominal surgery wa...