Clinical Briefing: Nonsurgical Treatment of Desmoid Tumors
May/June 2008
Desmoid tumors are classified as benign fibromatous neoplasms,
but are extremely aggressive, locally invasive, destructive
and often very painful. Eradication can be achieved by radical
surgery with high dose (>50 Gy) external
radiation, but this combination often results in disfigurement and considerable
morbidity.
Given the disparity between a nonlethal condition and a
treatment typically acceptable only in the context of cancer,
most patients choose to have conservative surgery. Because
microscopic residua at the tumor margin is highly regenerative,
however, desmoid tumors recur in up to half of patients who
choose not to have negative-margin surgery.
Two
views of a desmoid tumor in the
left lateral deltoid before (Figure 1)
and after (Figure 2) treatment with
vinblastine/methotrexate.
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To eliminate recurrence and preclude the
effects of surgery and radiation, the Penn Department of
Orthopaedic Surgery employs low-dose combination vinblastine/
methotrexate chemotherapy to treat desmoids tumors. Developed
in the late 1980s by current department Chair Richard
Lackman, MD, this highly efficacious nonsurgical treatment offers
significant advantages over surgery/radiation for the treatment
of progressive, recurrent and inoperable desmoid tumors.
At
10 years, 70 percent of patients with desmoids demonstrate
no tumor progression following low-dose vinblastine/ methotrexate
therapy.* Toxicity with the combination is low, and side
effects are generally transient.
* Source: Source: Azzarelli A, et al. Cancer.
2001;92:1259-1264.
"To date, vinblastine/methotrexate
therapy is the best option for the
eradication of desmoid tumors
because the combination offers
high efficacy while avoiding the
morbidity and complications
associated with negative margin
surgery and radiation."
– Richard D. Lackman, MD, FACS
Chair, Department of
Orthopaedic Surgery |
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Case Study
Mr. H, a 37-year-old male, was referred to the Penn Department
of Orthopaedic Surgery for evaluation of a suspicious mass
in his right shoulder. An avid sportsman, Mr. H discovered
the mass during a golf game, and treated it with over-the-counter
ointments for several weeks before visiting his physician.
At Penn, his physical
examination revealed restricted motion and a painful mass.
A core needle biopsy of the mass was performed, and demonstrated
the colloid tissue and spindle cells typical of desmoid tumors.
A subsequent MRI (Figure 1, above) found a large (5 cc) fibrotic
tumor within the lateral deltoid muscle with numerous adhesions
and extensions into the pectoralis major.
Mr. H was offered two options for treatment:
total excision of the tumor with removal of the anterior
deltoid muscle followed by irradiation, or combination
vinblastine/ methotrexate chemotherapy. To avoid the potential
disfigurement and debility resulting from surgery, Mr.
H chose to begin weekly intravenous injections of methotrexate
30 mg/m2 and vinblastine 6 mg/m2.
Within four weeks, the
mass had diminished by approximately 50 percent; an MRI
at eight weeks noted substantial continued shrinkage (Figure
2, above). Nausea, the sole side effect of the treatment,
was transient and minor. Mr. H continued his regimen for
six months. An MRI at his five year follow-up noted no recurrence
of the tumor. Mr. H has since resumed his normal activities,
including golf.
Our Team of Faculty
The department of Orthopaedic Surgery at Penn is a national
leader in the surgical treatment of the musculoskeletal system. The
spectrum of care provided by the department’s surgeons embraces hand,
elbow, joint and spine surgery, and trauma and reconstructive surgery.
In
addition, the department offers advanced therapies for
destructive lesions and diseases affecting the skeletal system,
and is at the forefront of pioneering clinical research to
develop new orthopaedic treatments and therapies. The department
also administers an orthopaedic training program
that attracts residents and fellows from the nation’s
leading medical schools.
Orthopaedic Surgery
Richard
D. Lackman, MD, FACS
Paul B. Magnuson Professor
and Chair, Department of Orthopaedic Surgery
Richard Lackman, MD, FACS, specializes in invasive and
noninvasive treatment of benign and malignant tumors of
the bone and soft tissue,
joint recontructive surgery and limb salvage surgery. In
the late 1980s,
Dr. Lackman developed the combination vinblastine/methotrexate
regimen
with his then collaborator, Dr. Arthur Weiss. This regimen
has since become
the leading nonsurgical treatment for desmoid tumors.
Christian
Ogilvie, MD
Assistant Professor of Orthopaedic Surgery
Christian Ogilvie, MD, has co-authored
numerous journal articles focusing upon orthopaedic surgery
and oncology. His interests include approaches to the management
of musculoskeletal neoplasms, osteoid osteoma and
bone metastases.
Hematology-Oncology
Arthur
Staddon, MD
Director, Joan Karnell Cancer Center
Director,
Sarcome Program
Arthur Staddon, MD, is a clinician, researcher, professor
and author. In addition to these pursuits, Dr. Staddon
contributes his expertise to
the managment of desmoid tumors and other orthopaedic soft
tissue
neoplasms at Penn.
Access
Patient appointments are available at:
Penn Orthopaedic Institute at
Pennsylvania Hospital
Garfield Duncan Building, Suite 2C
301 South 8th Street
Philadelphia, PA 19106
Pennsylvania Oncology Hematology Associates, Inc.
230 West Washington Square
Philadelphia, PA 19106
To refer a patient and/or consult with
a doctor call 800-789-PENN (7366) or you can also refer
a patient online.
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Referring Physicians: To speak with a Penn physician
or refer a patient, contact PennHealth through the secure online
referral form or by calling
1-800-789-PENN
(7366). |
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