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Maffucci's Syndrome
A subungual exostosis is a bony prominence
that can occur under the toenail. They generally are a result of some
form of trauma to the toe that results in the formation of bony
irregularity or prominence. When they are symptomatic, removal of the
spur is the treatment of choice. Additionally other small tumors called
osteochondromas and enchondroma can also form in the bone beneath the
toenail as well as in other bones in the body. An
osteochondroma is a benign bone tumor that accounts for 50% of all
benign bone tumors. They have a predilection for the long tubular bones
of adolescents and young adults. Its peak incidence is in the second
decade of life with a male to female ratio of approximately 2:1. They
are generally painless or minimally painful unless they cause
irritation to the surrounding tissue. When they are in the bone beneath
the toenail they can deform the toenail and cause an ingrown toenail.
The treatment of symptomatic osteochondromas is surgical excision. The
final diagnosis is made after the bone tumor is removed and examined by
a pathologist. Recurrence of the tumor is possible following their
removal. Hereditary multiple exostosis (osteochondromatosis) have a
prominent hereditary incidence which affects males more often then
females. The disease is characterized by the presence of multiple
exostosis, which are frequently bilateral and somewhat symmetrical and
usually make their appearance during childhood or adolescence. Enchondroma is a fairly common benign cartilaginous tumor, which is the most common bone tumor of the hands and feet.
They affect patients in a wide age range with no sex predilection. When
they occur within the small tubular bones the tumor can involve large
portions of these bones, causing thinning of the cortex of the bone.
This can weaken the bone and cause it to break spontaneously. When they
occur in the small bone in the end of the toe they can cause pain that
may mimic the pain of ingrown toenails.
Ollier's disease, also known as enchondromatosis, shows a strong
predilection for the small bones in the hands and toes (phalanges) and
the long bones behind the phalanges called metatarsals. It is often
shows an asymmetric involvement, tending to affect one side of the body
more than the other and has a propensity to transform into a malignant
sarcoma. Maffucci's syndrome is a very rare form of enchondromatosis
associated with multiple soft tissue hemangiomas. This tumor has a
greater predilection for the hands and feet, and has a greater toward
malignant transformation than Ollier's disease.
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