Despite their classification as benign, desmoid tumours are difficult to diagnose and manage. They are prone to recurrence and resection can be debilitating. Rarely, synchronous or metachronous multicentric desmoid tumours occur and may require further excision. Therefore, early detection of recurrence and multicentric tumours is vital. We present a case of metachronous desmoid tumours, and rev...

Hyaline fibromatosis syndrome is a rare autosomal recessive disease characterized by the presence of contracture and joint pain, hyperpigmented plaques and nodules and gingival hypertrophy. These findings are the result of the accumulation of a hyaline amorphous material similar to collagen type VI in different tissues. This syndrome includes systemic hyalinosis and juvenile hyaline fibromatosi...

Pachydermodactyly (PDD) is a rare and benign form of digital soft tissues fibromatosis, which affects the skin of the fingers. The disorder is characterized by asymptomatic, symmetric, progressive soft tissue swelling of the proximal interphalangeal (PIP) joints of the fingers. The etiology of disease remains unknown. It is usually acquired, even though there are some publications that document...

Fibrous or desmoid tumors are grouped under the term fibromatosis; there are several groups; the pediatric aggressive form is rare, especially the pelvis and/or hip presentation. This causes a variable degree of destruction of the surrounding tissues. It is a diagnosis by exclusion and histopathologic testing; treatment is always surgical and conservative treatment is indicated only for special...

A 6-year-old girl was referred to Eppendorf University Hospital because of a suspected malignant mandibular tumour. An osteolytic lesion was depicted on panoramic radiograph in the premolar region. The tumour was resected with covering skin and the defect was closed by primary intention. Histological investigation revealed fibromatosis. No local recurrence of infantile fibromatosis occurred dur...

Plantar fibromatosis, or Ledderhose disease, is a rare hyperproliferative disorder of the plantar aponeurosis. It may occur at any age, with the greatest prevalence at middle age and beyond. This disorder is more common in men than woman and it is sometimes associated with other forms of fibromatosis. Diagnosis is based on clinical examination. Ultrasound (US) and magnetic resonance imaging (MR...

Idiopathic gingival fibromatosis is a rare heriditary condition characterized by slowly progressive, nonhemorrhagic, fibrous enlargement of maxillary and mandibular keratinized gingiva caused by increase in submucosal connective tissue elements. This case report gives an overview of gingival fibromatosis in a 11-year-old female patient who presented with generalized gingival enlargement. Based ...

Desmoid tumor (aggressive fibromatosis), as a member of a group of borderline neoplasms, is a rare tumor of fibroblastic origin that remains difficult to treat. Treatments with surgery, radiotherapy and different medical protocols including interferon (IFN)-alpha, hormonal agents such as tamoxifen (anti-estrogen) as well as non-steroidal anti-inflammatory drugs and low-dose antineoplastic agent...

Gingival fibromatosis (GF) is a heterogenous group of disorders characterized by progressive enlargement of the gingiva caused by an increase in submucosal connective tissue elements. This article presents a case report of a 14-year-old female patient with idiopathic gingival fibromatosis in the maxillary region with radiographic feature of congenitally missing maxillary permanent left lateral ...

An unusual case of fibromatosis of the dominant left flexor pollicus longus (FPL) in a thirteen year old schoolboy. Initially presenting with pain in the thenar eminence and difficulty flexing the metacarpal phalangeal joint (MPJ), other symptoms include locking, triggering and difficulty writing. MRI showed a 4cm segment of thickened abnormal tendon. Intra-operatively three 1cm nodules were ex...