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Dermatology > BENIGN NEOPLASMS
Plantar Fibromatosis
Article Last Updated: Jan 26, 2007
AUTHOR AND EDITOR INFORMATION
Section 1 of 10  
Author: Firas G Hougeir, MD, Staff Physician, Department of Dermatology, Mayo Clinic Scottsdale
Firas G Hougeir is a member of the following medical societies: American Academy of Dermatology
Coauthor(s):
Jose M Mascaro, MD, MS, Chairman, Professor, Department of Dermatology, Hospital Clinic, University of Barcelona, Spain
Editors: Neil Shear, MD, Professor and Chief of Dermatology, Professor of Medicine, Pediatrics and Pharmacology, University of Toronto Faculty of Medicine; Head of Dermatology, Sunnybrook Women's College Health Sciences Center and Women's College Hospital, Canada; Michael J Wells, MD, Associate Professor, Department of Dermatology, Texas Tech University Health Sciences Center; Jeffrey P Callen, MD, Professor of Medicine, Chief, Division of Dermatology, University of Louisville School of Medicine; Joel M Gelfand, MD, MSCE, Medical Director, Clinical Studies Unit, Assistant Professor, Department of Dermatology, Associate Scholar, Center for Clinical Epidemiology and Biostatistics, University of Pennsylvania; Dirk M Elston, MD, Director, Department of Dermatology, Geisinger Medical Center
Author and Editor Disclosure
Synonyms and related keywords:
PF, juvenile fibromatosis, JF, Dupuytren palmar contracture, DC, Ledderhose disease, LD, juvenile aponeurotic fibroma, JAF, infantile fibromatosis, aggressive infantile fibromatosis, AIF, superficial fibromatosis, SF, cerebriform mesodermic hamartomas, Proteus syndrome, PS, fibromas, desmoid tumors, Gardner syndrome, GAF, Gardner syndrome–associated fibromatosis, deep fibromatosis, DF, hamartomatous fibromatosis
Background
The fibromatoses represents a wide spectrum of locally infiltrative clinicopathologic processes characterized by the proliferation of generally mature fibroblasts associated with mature collagen. Some of these entities are present at birth or develop in early childhood (eg, juvenile fibromatosis [JF]). Others may appear in adulthood.
The term plantar fibromatosis (PF) is used for different conditions, as follows: (1) a relatively common plantar equivalent of Dupuytren palmar contracture named Ledderhose disease (LD); (2) a more uncommon plantar superficial fibromatosis that, unlike deep fibromatosis (eg, abdominal, extra-abdominal, and visceral fibromatosis) generally has a less aggressive and recurrent tendency; and (3) an extremely rare, benign cerebriform mesodermal hamartomatous proliferation that, in a plantar location, appears to be a clinicopathologic marker of Proteus syndrome (PS).
Juvenile aponeurotic fibroma (JAF) and aggressive infantile fibromatosis (AIF) can also be considered to be in the PF group when lesions are present on the sole of the foot.
Pathophysiology
PF represents not a single entity, but rather, a heterogeneous group of conditions with the common characteristics of plantar location and histologic features of mature collagen and fibroblasts with no malignant cytologic features.
In LD (described in 1897), as in Dupuytren contracture (DC) (first reported in 1831), repeated trauma, long-term alcohol consumption, chronic liver disease, diabetes, and epilepsy have been reported in association with the development of the lesions in middle-aged or elderly people. Often, patients with LD also have other fibrosing conditions such as DC, knuckle pads, or induratio penis plastica (ie, Peyronie disease, first reported in 1743 by François de la Peyronie, physician of Louis XV of France). Heredity is also a clear factor in many patients.
Superficial fibromatosis (SF) in a plantar location includes a variety of soft-tissue tumoral proliferations of fibroblasts. However, it has been shown that some forms are not due to fibroblast overgrowth but to myofibroblast proliferation; SF is more common in children and young adults than in older people.
Cerebriform mesodermic hamartomas on the soles represent a kind of mesodermal nevus and are usually associated with PS. This syndrome was named after the Greek god Proteus, the "Old Man of the Sea" and son of Poseidon who was able to change his shape to protect himself. PS is a complex malformative or asymmetric hypertrophic syndrome associated with multiple cutaneous and musculoskeletal manifestations such as epidermal verrucous nevus, vascular hamartomas, and exophytic cerebriform fibrolipomata and scoliosis, kyphosis, and exostosis, respectively. Hamartomatous cerebriform PF may develop on the soles before other manifestations of PS appear, and it is considered a marker for PS.
Fibromas and desmoid tumors (eg, intestinal polyps, osteomas, soft-tissue tumors, epidermal cysts) are common in Gardner syndrome, which was described in 1950. These tumors often arise over previous surgical scars. By means of direct DNA sequencing, recent studies show that somatic beta-catenin or adenomatous polyposis coli (APC) gene mutations are present in virtually 100% of cases of Gardner syndrome–associated fibromatosis (GAF), as well as most cases of deep fibromatosis (DF). On the other hand, no somatic mutations were identified in beta-catenin or APC genes in SF. Therefore, the divergent behaviors of SF in relation to DF and GAF, despite their similar clinical and histologic morphologic features, are based on genetic differences.
Frequency
United States
LD is relatively common, and plantar contracture develops in approximately 25% of middle-aged or elderly individuals (1 of every 4 with DC). Superficial plantar fibromatosis (SPF) is uncommon, and the hamartomatous form associated with PS is rare. The exact incidences of SPF and the hamartomatous form associated with PS are unknown.
Mortality/Morbidity
- The different varieties of PF may be asymptomatic. However, the feeling of a mass in the foot, difficulty fitting in shoes, and pain with weight bearing often affect patients' ability to stand or walk.
- Only AIF has an invasive course, as does fibrosarcoma; however, it does not metastasize.
- Recurrent forms are not uncommon, although plantar fasciectomy has a relatively reduced rate of recurrence in LD.
Race
Whites are affected more often than other groups.
Sex
- LD affects men approximately 10 times more often than it affects women.
- JAF is more common in boys than in girls.
- No sex predilection is evident for the other forms of PF.
Age
- LD is seen in middle-aged and elderly people.
- SPF and JAF are most common in children and youths than in adults.
- The exceptional AIF begins in an infant's first year of life. The rare hamartomatous variety also develops in infants.
History
- Patients with LD are not often aware of their disease because it is usually not painful.
- Likewise, patients with other forms of PF may not be aware of their disease; however, they may notice difficulty in standing, walking, or wearing shoes when nodules or bumps become big enough.
- LD is typically bilateral and progresses slowly but not indefinitely.
- SPF may grow gradually, and, in most cases, self-involution occurs. In some cases, SPF lesions enlarge and persist; if excised, they recur iteratively.
- JAF can spontaneously regress or persist. Recurrences after excision are common.
Physical
- LD consists of one or more small, asymptomatic, round or flattened, hard nodules that are generally located on the medial side of the sole. Flexion deformities usually do not occur in opposition to DC.
- SPF appears as one or more asymptomatic, bad limited, flat nodules of fibrous consistency and variable size. They are most commonly located on the plantar aspect of the anteromedial portion of the heel pad (see Image 1).
- JAF may appear as a localized form affecting adults or a diffuse variety observed in children. It is more common in males than in females, and the hard nodules grow slowly and adhere to deep structures (see Image 2).
- AIF is rare and ordinarily begins in the patient's first year of life. It grows rapidly and infiltrates the subcutaneous tissue, aponeurosis, and muscles with an expansive or infiltrative course like a fibrosarcoma. However, metastasis does not occur.
- Hamartomatous PF lesions look like raised cerebriform soft-to-firm exophytic masses on any plantar area, where they are covered by pink, lightly dark, or normal-colored skin (see Image 3). They can become large enough to cause disability.
- In patients with LD, the presence of other fibrosing conditions (eg, DC, knuckle pads, Peyronie disease in men) must be checked.
- In SPF, Gardner syndrome must be ruled out.
- In the presence of cerebriform fibrous exophytic plantar lesions, PS must be considered.
Causes
As with many tumors, the causes are not known.
CHILD Syndrome
Other Problems to be Considered
Scar and keloid
Sarcoma
Fasciitis
Neurofibroma
Achromic neuroid nevus and malignant melanoma
Serous tendinous cyst
Mucoid cyst
Calcinosis
Osteoma and calcaneus spur
Gout
Clinically, LD can be confused with posttraumatic neuroma. However, LD is usually bilateral, although one side may be more prominent than the other. Clear cell sarcoma must be considered when MRI findings lead to the diagnosis of LD.
SPF must be distinguished from the other types of fibromatoses (eg, JAF, AIF), but, even histologically, this identification is often not easy. Other conditions that may mimic SPF are deep granuloma annulare, calcinosis, mucoid cyst, and Darier-Ferrand dermatofibrosarcoma (dermatofibrosarcoma protuberans). Granuloma annulare usually involves more than one element. Calcinosis and osteoma feel harder than SPF and are easily visible on radiographs. Mucoid cysts are renitent. They grow relatively slowly, and their sizes vary; however, the differences in size are seen on a weekly, not necessarily daily, basis. Darier-Ferrand dermatofibrosarcoma usually involves pigmented overlying skin and has a slower evolution. In any case, biopsy is necessary to confirm the diagnosis.
Hamartomatous PF must be distinguished from neurofibroma, neurofibromalike melanocytic nevus, and melanoma arising in a neurofibromalike nevus. The correct diagnosis can be obtained only with microscopic examination.
Imaging Studies
- MRI is useful in detecting PF. Images show some signal intensity heterogeneity and, usually, infiltrative margins. MRI can show the degree of deep invasion of the PF, which often reaches the aponeurosis.
- The MRI signal intensity and the consistency of the clinical location of LD enable the diagnosis to be made with reasonable confidence. However, one must consider the diagnosis of clear cell sarcoma in the differential. In effect, the 2 entities can have similar MRI findings and clinical locations.
Histologic Findings
All types of PF have a dense fibrocellular tissue with mature collagen and fibrocytes in various stages of maturation, but they do not have prominent atypical features or abnormal mitotic activity. The overlying epidermis and superficial dermal tissue are usually normal, but the neoformation, which grows upward and downward, generally replaces the adipose tissue. The proliferation often involves many cellular foci surrounded by fibrous scarlike connective tracts.
In SPF, the limits are usually undefined. Some areas may be almost acellular, with a scarlike appearance. In other areas, or in the most active early cases, the fibrocytic component can be dense, with cells closely packed together; the differential diagnosis with fibrosarcoma can be difficult in these cases. The reticulin network is often prominent. Usually, inflammation-associated infiltrate is not present. The connective stroma may involve various aspects. The stroma may be dense and fibrous; less commonly it is loose. Sometimes, myxoid or chondroid areas can be seen. Vascularization is not a prominent feature. Local nerves and Vater-Pacini corpuscles can seem to be increased in number or size.
In JAF, the cells are round or oat-shaped, and the stroma is chondroid (cartilage analogue fibromatosis). Aggressive forms are usually more cellular and have increased mitotic activity. Peripherally, the proliferation is poorly limited and penetrates the neighboring structures. Hamartomatous cerebriform PF has a variable fibromalike, lipomatous, angiolipomatous, or combined structure.
The myofibroblast is the key proliferative cell in some so-called fibromatoses; therefore, these might be better named myofibromatoses. In these fibromatoses, results of tests for cytologic markers in muscle cells are positive.
In recurrent PF, as in aggressive fibromatosis, tumoral cells express platelet-derived growth factor-B (PDGF-B) proto-oncogene. This proto-oncogene encodes the B chain of PDGF-B, a mitogen for fibrocytes, whereas normal plantar fascia, nonrecurrent PF, and scars do not. Thus, the detection of PDGF-B may be a useful adjunct to the pathologic evaluation of invasive PF for prognostic purposes.
Osseous metaplasia and distinct multinucleate giant cells have been reported in LD.
Medical Care
- No medical care is effective in PF, and reported success probably is due to the possible spontaneous involution of SPF. Early treatments have included, anti-inflammatory medication, orthotics, and physical therapy. Other modalities have included methotrexate and radiation after surgery.
- For LD, the intralesional injection of corticosteroids has been tried, but its usefulness is doubtful, and these injections may have some utility at only the initial stage.
Surgical Care
- For LD, fasciectomy and excision of the fibrous tissue are the only possible treatments, if needed. Fasciectomy has been shown to reduce the rate of recurrences.
- For the other forms of PF, surgery is the only therapeutic alternative. However, in infantile forms, physicians should evaluate the need for surgery before performing it.
- Many JFs spontaneously regress, and biopsy may be performed to induce their involution.
- Some lesions can grow, and others can recur after excision that appears complete.
- Because tumor growth characteristics may be relatively important before surgery, physicians should consider the possibility of an expectant control.
- Hamartomatous PF does not regress spontaneously; therefore, surgical removal is appropriate.
Activity
Modification of activity, the use of orthotics, and physical therapy have been used as therapeutic modalities for the treatment of PF.
Prognosis
- LD has a favorable prognosis, although slow progression is not uncommon. Patients who undergo plantar fasciectomy have been shown to have a lower recurrence rate.
- SPF is usually benign and may regress spontaneously.
- Rare cases that are relatively progressive and recurrent occur.
- The hamartomatous form is also benign, and problems are related to difficulties in standing or walking or to associated PS.
- JAF is benign, but recurrences are common.
- AIF must be considered a locally aggressive fibrosarcoma.
Medical/Legal Pitfalls
- Because SPF is often self-regressive, a failure to evaluate the need for surgery may occur.
- Surgical scars can cause disability if they are in a plantar location.
- Failure to diagnose a sarcoma based on MRI findings similar to LD may lead to physician liability.
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Plantar Fibromatosis excerpt Article Last Updated: Jan 26, 2007
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