Moffitt’s Neurofibromatosis Clinic Celebrates
First Anniversary With Prestigious Designation
Within its first year of operation, the Neurofibromatosis Clinic
at Moffitt Cancer Center earned clinic network designation
by the Children’s Tumor Foundation (CTF). Under the
direction of Xia Wang, MD, PhD and Sepideh Mokhtari, MD,
Moffitt’s Neurofibromatosis Clinic became the only adult
neurofibromatosis clinic in the state with 98% of its patients
over the age of 18.
Neurofibromatosis (NF) is usually diagnosed in childhood or
early adulthood with health risks persisting throughout life.
NF is actually a group of genetic conditions that can either
be inherited or occur for the first the time in the family.
Traditionally, comprehensive specialty care has been provided
to children in pediatric hospitals. This leaves a great number
of adults affected with NF without access to comprehensive
specialty care. NF mainly causes tumors to develop anywhere
in the nervous system including the brain, spinal cord and
nerves. Most tumors are not cancerous, but complications
from NF can compromise multiple organs and systems and
lead to hearing loss, blindness, paralysis and severe pain.
Some tumors can be cancerous. Very often, each individual
can have multiple health conditions as a result from NF.
There are three types of Neurofibromatosis:
NF1 affects 1 in 3,000 people and is the most common form
of NF. Besides tumors on the nerve system, there is an
increased risk for cancerous tumors in multiple organs.
Individuals with NF1 may have typical features such as
small, light brown patches on the skin (often referred to
as café-au-lait spots) and small lumps on the skin as dermal
neurofibromas. The trademark, café-au-lait spots, often
appear shortly after birth, while dermal neurofibromas
usually appear later in childhood or in young adulthood.
Tumors on the optic nerve, responsible for vision, can lead
to blindness in childhood. Large tumors next to the spine
may lead to paralysis. A type of soft tissue cancer, MPNST,
can occur in 10% of the individuals with NF1. Increased risk
for additional cancers, such as breast cancer and gastrointestinal
stromal tumor (GIST) can also be life-threatening.
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NF2 affects about 1 in 25,000 people worldwide. The disorder
is characterized by the development of non-cancerous tumors
on the nerves that carry sound and balance information from
the inner ear to the brain (the eighth cranial nerve). These
are called vestibular schwannomas (formerly called acoustic
neuromas). These tumors usually affect both ears, often
leading to partial or complete hearing loss. Schwannomas
develop when Schwann cells, which form the insulating cover
around nerve fibers, grow abnormally. Schwannomas may
also develop on other nerves throughout the body. Additional
non-cancerous but devastating tumors can also develop in
the brain and spinal cord, such as meningiomas
and ependymomas.
Schwannomatosis is a rare form of neurofibromatosis
that has only recently been identified. It affects less
than 1 in 40,000 people, and causes the development
of multiple schwannomas.
Xia Wang, MD, PhD Sepideh Mokhtari, MD
Moffitt takes a comprehensive, multispecialty approach to care
and treatment of individuals with neurofibromatosis.
• Our surgical oncologists and neurosurgeons use advanced,
minimally invasive techniques to remove neurofibromas with
the highest level of precision – even from difficult-to-access
locations near nerve pathways.
• Our medical oncologists and radiation oncologists design
individualized nonsurgical treatment plans that may include
chemotherapy, radiation therapy, as well as novel options
such as targeted therapy.
• Our genetic specialists determine the genetic causes in
the blood and tumor and provide care and support for
the entire family.
• Our radiologists provide comprehensive evaluation of the
tumors, pathologists provide precise histological information,
and personalized medicine team identify targets for cuttingedge
therapies and clinical trial options.
• Our supportive care specialists provide medications and other
therapies to ensure the greatest degree of comfort for our
patients. For a younger patient, our team can also help his or
her parents develop action plans with teachers, psychologists
and other key professionals.
“Moffitt is home to so many talents,” says Dr. Wang. ”We have great
potential to become not only the center providing comprehensive
screening, prevention and treatment in the entire state, but also
accelerating in research to contribute to the cure.”
From the very beginning, Neuro-oncology program Chair Dr. Peter
Forsyth, and the Chair of the Department of Individualized Cancer
Management, Dr. Howard McLeod, have offered strong support for
this clinic.
Moffitt’s multi-disciplinary approach to treating NF extends outside
the walls of the cancer center and brings medical professionals
together across Tampa Bay and beyond. Currently, Moffitt’s team
collaborates with Tampa Bay Hearing and Balance Center and
Dr. Nicholas Avgeropoulos of Orlando Health to treat progressive
vestibular schwannoma with Avastin.
“I am very excited to get this clinic up and running,” says
Dr. Wang. “Team work is key and I am so happy we can
make it happen for patients.”
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6 NEURON NEWS | 2018 ISSUE MOFFITT.org
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