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Hand, Tumours: Malignant
Last Updated: April 24, 2003 |
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| Synonyms and related keywords:
malignant hand tumour, hand tumuor, hand malignancy, skin cancer,
hand carcinoma, skin carcinoma, hand mass, tumor of the hand, hand
lesion, malignant hand lesion, cancerous hand lesion, cancerous
hand tumor, primary malignant hand tumor, metastatic hand tumor,
sarcoma, squamous cell carcinoma, basal cell carcinoma,
basosquamous cell carcinoma, melanoma, malignant skin lesion,
dermatofibrosarcoma protuberans, Kaposi sarcoma, Kaposi’s
sarcoma, sweat gland tumor, Merkel cell carcinoma, carcinoma in
situ, CIS, subungual melanoma, chondrosarcoma, osteogenic sarcoma,
osteosarcoma, Ewing sarcoma, Ewing’s sarcoma, epithelioid
sarcoma, synovial sarcoma, liposarcoma, fibrosarcoma, malignant
fibrous histiocytoma, malignant schwannoma, rhabdomyosarcoma,
leiomyosarcoma, vascular leiomyosarcoma, angiosarcoma,
lymphangiosarcoma, clear cell sarcoma |
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AUTHOR
INFORMATION |
Section
1 of 11 
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| Author: Benjamin
W Beckert, MD, Staff Physician, Department of
Surgery, University of
Missouri Hospitals and Clinics
Coauthor(s): Matthew
J Concannon, MD, FACS, Director of Hand and
Microsurgery, Associate Professor, Department of Surgery, Division
of Plastic and Reconstructive Surgery, University of Missouri
Hospitals and Clinics
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| Benjamin W Beckert, MD, is a member of the following medical
societies: Alpha Omega
Alpha, American Medical
Association, Phi Beta Kappa,
and Southern Medical Association
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| Editor(s): Anthony E Sudekum, MD, Consulting
Staff, Department of Plastic Surgery, St John's Mercy Health
Center of Saint Louis; Francisco Talavera, PharmD, PhD,
Senior Pharmacy Editor, Pharmacy, eMedicine; David Chang,
MD, Associate Professor, Department of Plastic Surgery,
The University of Texas/Anderson Cancer Center; Nick
Slenkovich, MD, Staff Physician, Division of Plastic
Surgery, University of Alabama at Birmingham; and Susan E
Downey, MD, Associate Professor, Department of Surgery,
Division of Plastic Surgery, University of Southern California |
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INTRODUCTION |
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Although tumors of the hand are rather common, most are benign and only
1-2% are malignant. When evaluating a hand lesion, be judicious and
maintain a high index of suspicion for malignancy. Malignant hand tumors
represent a complex and challenging entity for the hand surgeon.
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CLASSIFICATION |
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Malignant tumors of the hand can be divided into 2 principal categories,
primary and metastatic. Primary malignant tumors are subclassified further
as skin tumors, musculoskeletal tumors, and/or soft tissue tumors. While
malignant hand tumors are relatively uncommon, the incidence of metastatic
tumors is exceedingly rare. In a review of patients with primary malignant
tumors evaluated at the Mayo Clinic from 1940-1983 by Amadio et al, only
18 patients were found to have metastatic disease to the hand (Amadio,
1987). Friedman reported an incidence rate of 0.0016-0.5% of neoplastic
metastasis to the skin of the hand (Friedman, 1991).
Tumor staging is important to characterize the degree of progression of
a malignant tumor. Histologic grading is also important, particularly when
examining soft tissue sarcomas.
Numerous staging methods exist; however, the TNM classification system
is the most widely used. This classification scheme incorporates the idea
that tumors spread in an orderly fashion. The localized tumor (T) first
spreads to adjacent lymph nodes (N) and, subsequently, to distant sites of
metastasis (M). Numbers added to the classification scheme further define
the tumor size or extent of spread, with larger numbers indicating more
advanced disease. Based on the TNM classification, the appropriate stage
can be identified. Grading classifications used most commonly are G0
(benign), G1 (low grade), and G2 (high grade). Low-grade lesions are
typically less aggressive and less likely to metastasize than high-grade
lesions. The appropriate intervention can be determined based on the stage
and histologic grade.
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PRIMARY
MALIGNANT TUMORS - CUTANEOUS |
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Of the primary sites for malignant tumors of the hand, the skin is by far
the most likely tissue to contain malignant cells. In order of decreasing
incidence, squamous cell carcinoma, basal cell carcinoma, basosquamous
cell carcinoma, and melanoma account for 90% of primary malignant tumors
of the hand. Other malignant skin lesions include dermatofibrosarcoma
protuberans, Kaposi sarcoma, sweat gland tumors, and Merkel cell
carcinoma.
Squamous cell carcinoma
Squamous cell carcinoma is the most common primary malignant tumor of
the hand, accounting for 75-90% of hand malignancies. The male-to-female
ratio is 2-5:1.
Risk factors include sun exposure, x-ray exposure, chronic ulcers,
immunosuppression, xeroderma pigmentosa, and Bowen disease. Solar
radiation is the most modifiable risk factor for squamous cell carcinoma,
especially for light-skinned, light-haired individuals. Dentists, x-ray
technicians, and others exposed to high doses of radiation to the hand are
at risk of developing squamous cell carcinoma. Ulcers that develop within
old burn or traumatic scars may undergo malignant change (Marjolin
ulcers), representing a more aggressive squamous cell carcinoma.
Transplant patients on immunosuppression therapy have a 4-fold increased
risk of developing skin cancer. Patients with xeroderma pigmentosum have a
1000-fold increase in the development of nonmelanotic skin cancers. Bowen
disease is carcinoma in situ.
Tumors often manifest as small, firm nodules with indistinct margins or
plaques. The surface may have various irregularities ranging from smooth
to verruciform to ulcerated. Skin coloration is often brown to tan.
Scaling, bleeding, and crusting also may occur. Typically, squamous cell
carcinoma is locally invasive; however, metastatic rates of up to 20% have
been reported in radiation beds and burn scars.
Treatment options include curettage and electrodesiccation, cryotherapy,
radiotherapy, and others. Standard treatment is excision with 1-cm
margins.
Basal cell carcinoma
Basal cell carcinoma accounts for 3-12% of hand malignancies. Risk
factors are similar to those for squamous cell carcinoma and include
chronic sun exposure, light complexion, immunosuppression, inorganic
arsenic exposure, and Gorlin syndrome.
Many types of basal cell carcinoma have been described, including
nodular, superficial, infiltrating, morpheic, pigmented, basal-squamous,
adenoid, cystic, keratotic, and fibroepithelioma. The classic description
is of a small, well-defined nodule with a translucent, pearly border with
overlying telangiectasias. Coloration varies with melanin content and the
presence of areas of necrosis. Metastasis occurs at a rate of 0.0028-0.1%.
Treatment options for basal cell carcinoma include curettage with
electrodesiccation, cryosurgery, and radiation, but the standard therapy
is surgical excision with 5- to 10-mm margins. Mohs microdermographic
surgery is often used around the mouth, nose, and periorbital regions, but
it is rarely indicated for lesions of the hand.
Melanoma
Melanoma accounts for approximately 3% of primary malignant hand
tumors, although this incidence is rising. Risk factors include long-term
sun exposure, previous dysplastic nevi, fair complexion, family history of
melanoma, and congenital nevi.
Various subtypes exist, including lentigo maligna melanoma,
intraepidermal malignant melanoma, superficial spreading malignant
melanoma, acral lentiginous malignant melanoma, nodular malignant
melanoma, and desmoplastic melanoma. Lesions with increased growth, change
in color or shape, irregular borders, and greater than 0.5 cm growth are
suggestive of melanoma. Aggressively pursue pigmented lesions under a nail
bed with no previous history of trauma with biopsy because these may be a
subungual melanoma.
Survival is related to the thickness of the lesion (in mm) (Breslow
thickness; also see Skin
Malignancies, Melanoma) in malignant melanoma without metastasis.
Measure this thickness from the granular layer of the epidermis or the
base of the ulcer to the greatest depth of the tumor. Breslow thickness is
classified into 4 groups. Tumors in group 1 have less than 0.76-mm depth
of invasion. Group 2 tumors have 0.76- to 1.5-mm depth of invasion. Those
in group 3 have 1.51- to 3-mm depth of invasion. Group 4 tumors have a
depth of invasion greater than 3 mm.
The principal treatment is surgical excision or amputation, with 1-cm
margins for every millimeter of invasion. Remove clinically palpable
nodes. Isolated limb perfusion may offer hope for salvage therapy.
Dermatofibroma protuberans
Dermatofibroma protuberans is a low-grade dermal sarcoma. This lesion
is rare on the hand. Typically, the lesion occurs as a painless
subcutaneous mass after a history of trauma. The lesion grows slowly;
however, if left unattended, it can become quite large and have multiple
nodules. This malignancy typically has lateral spread, but it can also
invade deeper structures and may ulcerate. It is generally reddish blue.
Metastasis is unlikely but has been reported.
Treatment consists of surgical excision with 3-cm margins. Radiation
therapy has been indicated for positive margins, but most authors
recommend reexcision to minimize the chance of local recurrence. Mohs
surgery has been indicated because of the extensions of cells from the
tumor mass, which account for the high recurrence rate.
Kaposi sarcoma
Kaposi sarcoma is a vascular malignancy of lymphatic endothelial cells.
The lesion manifests as a painless, violet nodule or plaque. Often, edema
is associated with this lesion. The classic patient population is of
Eastern European Jewish descent; however, with the rise in HIV infection,
these lesions are encountered in many other groups. The HIV-positive
population often has a more aggressive clinical disease with widespread
lesions and internal organ involvement. Therapy consists of radiotherapy,
cryosurgery, laser surgery, or primary excision.
Eccrine sweat gland malignant tumors
Eccrine sweat gland malignant tumors in the hand are rare. They
manifest as slow-growing, painless lesions. Typically, the patient is
elderly. The lesion is usually locally invasive, and, because of its
rarity, the rate of metastasis is unknown. Treatment consists of wide
local excision with a margin of normal tissue. These tumors are not
responsive to radiation or chemotherapy.
Merkel cell carcinoma
Merkel cell carcinoma is an aggressive cutaneous neoplasm observed in
sun-exposed areas. Older patients with long-term solar exposure are at
greatest risk. The tumor originates in the dermis and appears as a pinkish
nodule that enlarges by invading deeper structures. Surgical excision is
indicated for primary therapy; however, 40% of patients have local
recurrence, 55% with regional lymph node involvement and 46-49% with
distant metastases. Treatment options include salvage therapy with
reexcision, reexcision using Mohs surgery, chemotherapy, or radiotherapy.
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PRIMARY
MALIGNANT TUMORS - BONE AND SOFT TISSUE |
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Bone and soft tissue malignancies of the hand are much less common than
cutaneous lesions. Malignant bone tumors that occur in the hand include
chondrosarcomas, osteogenic sarcomas, and Ewing sarcoma. Additional soft
tissue malignancies, excluding skin, include epithelioid sarcoma, synovial
sarcoma, liposarcoma, fibrosarcoma, malignant fibrous histiocytoma,
malignant schwannoma, rhabdomyosarcoma, leiomyosarcoma, vascular
leiomyosarcoma, angiosarcoma, lymphangiosarcoma, and clear cell sarcoma.
Chondrosarcoma
Chondrosarcoma is the most common primary malignant bone tumor of the
hand. This tumor originates from the cartilage cells in joints and may
arise in a previously benign lesion, such as enchondroma. Men are affected
more often than women (male-to-female ratio of 2:1), and it is observed
most frequently in individuals older than 50 years.
The metacarpals and proximal phalanges are affected most commonly.
Clinically, chondrosarcoma manifests as a slow-growing mass. Expansion of
the lesion can cause local symptoms. Radiographically, radiopacities are
found within the cartilage. Most chondrosarcomas are locally invasive, but
they may metastasize in up to 10% of patients. The lungs are the most
common site of metastasis. After biopsy confirmation of the diagnosis,
patients must be staged appropriately to determine if adjunctive therapy
is indicated. Treatment consists of surgical excision. Chemotherapy or
radiation therapy does not appear to benefit the patient.
Osteogenic sarcoma
Osteogenic sarcoma (osteosarcoma) is a highly malignant bone tumor
characterized by the formation of neoplastic bone tissue. It is the most
common childhood malignant bone tumor but is rarely observed in the hand.
Osteogenic sarcoma in the hand often manifests in patients older than 40
years, and, typically, the proximal phalanges and metacarpals are
involved.
These lesions may manifest as masses with mild or intermittent pain. As
the lesion progresses, the pain becomes more severe and limits the range
of motion of surrounding joints. Plain radiographs illustrate lytic
lesions with surrounding new bone formation. The periosteum may be
elevated because of reactive bone adjacent to the tumor. After
confirmation of the tissue diagnosis, treatment consists of wide local
excision or amputation. Adjuvant chemotherapy, which improves survival,
can be performed prior to or following surgical treatment.
Ewing sarcoma
Ewing sarcoma is an uncommon aggressive malignant bone tumor composed
of small, uniform cells of uncertain origin; it may arise from primitive
marrow elements or immature mesenchymal cells. The tumor often arises in
long bones and is rarely found in the hand. The most common areas of
involvement in the hand are the metacarpals or phalanges. The tumor often
manifests with pain and swelling of the affected area, not unlike an
infection (it is often misdiagnosed as such). Systemic symptoms may soon
follow, with fever and leukocytosis. Radiographs reveal an "onion
skin" pattern, representing a circumferential layer of reactive
periosteal bone with an associated lytic lesion of the cortex. Magnetic
resonance imaging (MRI) studies can assist in defining bone and soft
tissue involvement.
After the diagnosis is made, the best treatment option remains
controversial, with choices of adjunctive chemotherapy, radiation therapy,
and surgery. This tumor may metastasize to many organs, including the
lungs, brain, and bones. The prognosis is grim for patients with local
recurrence. A multidisciplinary approach to treatment is often indicated.
Epithelioid sarcoma
Epithelioid sarcoma is the most common soft tissue sarcoma in the hand.
This lesion manifests as an ulceration on the palm or volar aspect of the
phalanges. It is often misdiagnosed. Epithelioid sarcoma tends to spread
proximally from its origin and carries a high risk of nodal metastases.
Some authors have suggested lymph node dissection or biopsy even without
clinical nodal disease. Wide local excision or amputation followed by
radiation therapy offers the best chance for cure.
Synovial sarcoma
Synovial sarcoma is a highly malignant soft tissue tumor that arises in
the region of a joint, tendon, or bursae. The tumor typically manifests as
a painful or tender mass near a joint, often in the carpus. The lesion may
have a long latent period with slow growth or may have a rapid growth
phase. Plain radiographs often demonstrate soft tissue calcifications.
This tumor metastasizes via lymphatic spread, which occurs in 25% of
patients. Treat synovial sarcoma with wide local excision or amputation
followed by radiation therapy, which decreases local recurrence.
Liposarcoma
Liposarcoma is a soft tissue sarcoma that is rarely observed in the
hand. Histologic subtypes include well-differentiated, myxoid, pleomorphic,
round cell, and dedifferentiated subtypes. These lesions can be confused
clinically with a benign lipoma because they often display slow growth.
After tissue biopsy, wide excision or amputation followed by adjuvant
radiation is recommended.
Fibrosarcoma
Fibrosarcoma is a malignant tumor of fibroblasts. These arise from
connective tissue such as fascia, periosteum, or tendons. Clinically, this
lesion appears as a soft tissue mass that exerts local effects by
compression of adjacent structures. Treatment for fibrosarcomas is similar
to that for other soft tissue malignancies of the hand and includes wide
excision and radiation therapy.
Malignant fibrous histiocytoma
Malignant fibrous histiocytoma is a malignant soft tissue tumor with
foci of macrophage differentiation. Few reports exist of this lesion
occurring in the hand. Treatment is similar to that for the above tumors,
with wide excision or amputation and adjuvant radiation therapy.
Malignant peripheral nerve sheath tumors
Malignant peripheral nerve sheath tumors include those tumors of neural
origin. Of these tumors, 50% occur in individuals with neurofibromatosis.
This tumor grows along and within the peripheral nerve. The clinical
manifestation is a soft tissue mass that often is painful to palpation.
Treatment consists of wide excision. A high incidence of local recurrence
is typical.
Rhabdomyosarcoma
Rhabdomyosarcoma is rare in the upper extremities, but it is the most
common soft tissue sarcoma in children. Rhabdomyosarcoma arises from
skeletal muscle and is divided into 4 subtypes, embryonal, botryoid,
alveolar, and pleomorphic. Alveolar rhabdomyosarcoma is the most common in
the hand. Clinically, this manifests as an enlarging, painful mass.
Associated paresthesias can occur secondary to local nerve compression.
Often, the patient has a history of associated trauma. Treatment has
improved in recent years, although survival rates have been reported to be
6% in 4 years. Local and regional lymphatic spread is common, and
metastasis to the heart, skeletal bone, lungs, pancreas, liver, and
kidneys can occur. Early diagnosis with appropriate surgical resection or
amputation followed by radiation and chemotherapy improves outcome.
Leiomyosarcoma
Leiomyosarcoma is a malignant tumor arising from smooth muscle cells.
These lesions are rarely observed in the extremities. They reportedly
appear as a solitary soft tissue mass, although they may be lobulated.
Epidermal changes with discoloration or ulceration may be present. After
appropriate staging, treatment involves excision with adjuvant
chemotherapy and radiation therapy. Reportedly, 5-year survival rates are
70-80%, with metastatic disease observed most commonly in the lungs.
Vascular leiomyosarcoma
Vascular leiomyosarcoma arises from large arteries or veins and has
been reported in the hand. This lesion manifests as an edematous mass, and
mass effect can cause local symptoms. Treatment consists of wide local
excision. Reportedly, 50% of patients have metastatic disease at the time
of surgery.
Angiosarcoma
Angiosarcoma is an aggressive, highly malignant tumor composed of
neoplastic endothelial cells. No sex predominance is recognized, and they
can occur in persons of any age. Most tumors undergo slow growth with
central necrosis, which reveals overlying skin changes. Treatment consists
of wide local excision.
Lymphangiosarcoma
Lymphangiosarcoma is a rare malignancy that reportedly develops in 0.1%
of patients with lymphedema of the arm following radical mastectomy.
Clinically, this manifests as multiple purplish nodules in the affected
brawny skin. Wide local excision is the best treatment, but this tumor is
associated with a poor prognosis.
Clear cell sarcoma
Clear cell sarcoma is an uncommon soft tissue sarcoma that reportedly
occurs near tendons and aponeurosis. It has been characterized as
"the malignant melanoma of soft parts" and may be misdiagnosed
as metastatic melanoma. This tumor is aggressive, with regional lymphatic
invasion commonly observed. For this reason, perform lymph node biopsies
on these patients. Treatment, as with most of these sarcomas, consists of
wide local excision.
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EVALUATION
- CUTANEOUS LESION |
Section
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The first step in treating the patient is to assess the cutaneous lesion
on the upper extremity. Remember that the lesion may represent a
malignancy.
History
Obtain a thorough history, including changes in color, size, pain at
the site, bleeding, scaling, or anything else the patient may have
noticed. Question the patient about habits in regard to solar exposure,
the use of sunscreens, and exposure to ionizing radiation. Importantly,
determine if the patient has had other cutaneous malignancies excised.
Also obtain a family history of malignancies.
Physical
Focus the physical examination on the lesion. Determine the size,
color, and any border irregularity, and assess adjacent skin and/or
structures. Magnification of the lesion under excellent lighting can
assist in the evaluation. Palpate adjacent lymph nodes to assess for
lymphadenopathy, possibly indicating spread of the malignancy.
Workup
Beyond a careful history and physical examination, evaluating cutaneous
lesions may include radiographic studies of the chest, computed tomography
(CT) or MRI scans, and evaluation of other anatomic structures. Laboratory
studies may include alkaline phosphatase, calcium, and liver function
tests.
Biopsy
Tissue biopsy is the next step in diagnosis. Individualize the type of
biopsy specimen obtained (eg, incisional, excisional, shave, punch) for
each lesion. The goal is to deliver to the pathologist a sample of tissue
that represents the pathology of the lesion. When possible, pursue
complete excisional biopsy.
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TREATMENT
- CUTANEOUS LESION |
Section
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Adequate resection is the key for treatment of malignant skin neoplasms.
Clearly, minimizing the deformity created is important, but the goal must
be removal of the malignant tissue, which may require later stages of
reconstruction. Achieve complete resection of the lesion with clear
histologic margins in normal surrounding tissue.
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MALIGNANT
SOFT TISSUE TUMORS |
Section
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Malignant soft tissue tumors of the hand (excluding skin) are exceedingly
rare; however, maintain clinical awareness when evaluating a soft tissue
mass in a hand.
History and physical
As with the evaluation of a cutaneous lesion, obtain a thorough history
when evaluating a soft tissue mass. Pertinent issues to pursue when taking
the history include time of onset, change in the lesion, skin changes,
pain, bleeding, and a history of trauma.
A complete and thorough physical examination is imperative for the
evaluation and diagnosis of a malignant hand lesion. Certainly, most soft
tissue lesions in the hand are benign, but use a systematic approach when
evaluating a soft tissue mass. First, inspect the lesion under an adequate
light source. Consider the anatomic structures surrounding the lesion and
possible etiologies for the mass. Next, palpate the lesion. Determine the
size, shape, and contour. Determine if the lesion is fixed or mobile, ie,
whether it is attached to surrounding structures. Ask the patient to flex
and extend the fingers and/or wrist to determine if the mass is arising
from a tendon or joint space. Also, do not ignore the remainder of the
hand. Inspect the hand for lesions other than the one in question.
Following a focused examination of the hand, complete the evaluation of
the extremity for possible lymphadenopathy or other suggestive lesions.
Workup
Laboratory studies may include measuring serum calcium, phosphorous,
and alkaline phosphatase levels to assess for possible bony metastasis.
Other helpful laboratory studies include a hematology profile if
considering an infectious etiology, a rheumatoid panel if an arthritide is
suggested, and an erythrocyte sedimentation rate to assess for
inflammation.
Multiple modalities are available for diagnostic imaging of potentially
malignant hand lesions. Images from plain radiographic studies offer a
good degree of bony definition with possible bony involvement of a lesion.
CT scan images have better overall and bony resolution than plain x-ray
images. If findings of bony invasion are equivocal on plain radiograph
images, CT is the next logical step in assessing the lesion.
MRI has a distinct advantage over CT scanning, with improved resolution
in soft tissue imaging. MRI can be used to determine the area of gross
involvement when assessed in various views (eg, coronal, axial, sagittal)
to better define lesions prior to operative intervention.
Bone scans can be useful in finding hot spots or areas of increased
radioisotope uptake. This technique is quite sensitive to pathology;
however, the findings are nonspecific. Bone scans are currently used more
often when assessing for bony metastasis of a primary malignancy.
At times, ultrasound can be helpful when assessing a soft tissue
lesion. Ultrasound is inexpensive, quite readily available, and effective
in determining if a lesion is solid or cystic. It can assist in defining
the type of biopsy that may be pursued on a lesion.
Biopsy
The next step in assessing a soft tissue mass is biopsy. The type of
biopsy performed depends on the outcome of previous diagnostic studies.
Plan carefully prior to biopsy because an excisional biopsy may lead to a
cure. Excisional biopsy is the goal; however, various other biopsies may
be implemented. Needle biopsy can be performed on a lesion, although it is
suited best to a lesion with a cystic component. Incisional biopsy may be
performed on a lesion that is too large to be excised without compromising
vital surrounding structures.
Remember that the goal is to deliver a representative sample of the
lesion to the pathologist. Whenever possible, pursue complete excision;
however, weigh the benefits of complete excision (with possible loss of
function and cosmetic result) against an incomplete excision. This is
often a function of experience and an index of suspicion that the lesion
is malignant. All biopsies should be performed by an experienced hand
surgeon because the type of incision should be based on sound techniques
that minimize contamination of surrounding tissue and allow for additional
resections to be performed without undue functional loss.
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METASTATIC
LESIONS |
Section
9 of 11
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Metastatic tumors to the hand are rare entities, with only a handful
reported in the literature, often as case reports. The lungs, kidneys,
head, and neck are the most common sites of metastatic source to the hand,
with the lungs accounting for 40% of reported primary tumors metastatic to
the hand. Presentation of a metastatic lesion to the hand can occur in an
individual with an unknown primary tumor or with a known source of
metastasis. Often, the diagnosis is delayed because the presentation (ie,
erythema, pain, swelling) can be confused with infection.
Typically, the prognosis is grim; however, long disease-free intervals
after appropriate excision are reported. Treatment entails therapy for the
primary tumor along with adjuvant appropriate resection of the area of
metastasis with clear margins. Exercise caution and treat each patient on
an individual basis according to the degree of metastatic disease.
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PICTURES |
Section
10 of 11
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BIBLIOGRAPHY |
Section
11 of 11
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