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4 - Malignant Tumors of The Hand and Wrist-Plate
4 - Malignant Tumors of The Hand and Wrist-Plate
4 - Malignant Tumors of The Hand and Wrist-Plate
ithelioid sarcomas, clear-cell sarco- ber wrap because of the potential go malignant transformation. Mar-
mas, and synovial sarcomas.1 risk of forcing malignant cells into ginal excisions for lipomas are ap-
Conventional radiographs provide adjacent tumor-free tissues. (Al- propriate, even though these tumors
important information. In addition though this possibility has not been frequently exceed 8 cm in diameter,
to routine posteroanterior and later- proved to occur, taking this precau- provided they appear to be clinically
al radiographic views, we recom- tion is prudent.) Intravenous region- benign. When there is any doubt, an
mend pronation and supination ob- al anesthesia (Bier block) is therefore incisional biopsy should be per-
lique views. For lesions in or avoided. Elevating the arm for sever- formed and excision of the tumor de-
adjacent to the pisiform and hook of al minutes before inflating the tour- ferred until the permanent sections
the hamate, a carpal tunnel view is niquet will provide a relatively are reviewed. Frozen sections may
useful. A more definitive carpal tun- bloodless surgical field. The surgical provide important information, but
nel view is obtained using computed approach to the lesion should be some pathologists avoid making a
tomography. Other imaging studies planned to permit excision of the bi- definitive diagnosis based solely on
that may aid in the diagnosis and opsy scar and surrounding tissues if these biopsy specimens.
surgical planning are magnetic reso- additional surgery is required. Longi- Once the tumor is identified as
nance imaging (MRI), bone scans tudinal incisions are generally pref- malignant, a treatment plan is initi-
(scintigraphy), and positron emission erable to transverse incisions. ated. In most cases, it involves either
tomography. There are two types of biopsies: a wide excision or a radical resec-
Biopsy is the final step in the diag- intralesional or incisional and mar- tion. The terms “excision” and “re-
nostic workup. Generally, the sur- ginal or excisional. An intralesional section” are paired with “wide” and
geon performing the biopsy should (incisional) biopsy, as its name sug- “radical,” respectively, to emphasize
be capable of performing any subse- gests, consists of removal of tissue important differences between the
quent surgery that is required. For from within the tumor. A marginal two procedures. A wide excision, of-
skin lesions, the biopsy is performed (excisional) biopsy is through the ten referred to as an en bloc resec-
using a punch, shave, or excision pseudocapsule (zone of reactive tis- tion, is excision of the tumor togeth-
technique. The shave technique is sue) that surrounds the tumor and er with its pseudocapsule (reactive
most applicable for pedunculated le- often consists of excision of the en- zone) and at least a 2- to 3-cm sur-
sions and involves an intradermal in- tire lesion. An intralesional biopsy rounding margin of normal tissue.
jection of a local anesthetic that el- can be excisional when it is used to The dissection is entirely within the
evates the lesion and makes it more curet a presumably benign lesion in involved anatomic compartment; al-
accessible. The lesion is then shaved bone before packing the tumor cav- though adjacent muscle tissue and
off with a scalpel or razor blade. ity with a bone graft. Allografts and bone are excised, the excision does
For soft-tissue and bone tumors, commercially available bone substi- not include the entire length of the
the biopsy is performed using either tutes are being used with increasing involved muscle or the entire bone.
a closed or an open technique. A frequency instead of autogenous For tumors outside the hand, a wide
closed biopsy with a fine needle or grafts. When a malignant tumor is excision often can be performed
trephine has the advantage of mini- suspected, an incisional biopsy is without jeopardizing or injuring vi-
mizing tissue contamination; how- preferred to determine the diagnosis tal structures. In the hand, however,
ever, the disadvantage of this tech- before proceeding with definitive it may be impossible to obtain an ad-
nique is that the tissue sample may treatment. equate tumor-free margin without
be inadequate for the pathologist to Marginal excision is commonly sacrificing important nerves, ves-
arrive at a definitive diagnosis or referred to as an excisional biopsy sels, intrinsic muscles, and tendons.
may not be from a representative because the tumor is “shelled out” A radical resection involves remov-
area of the tumor, resulting in misdi- or excised from the surrounding soft al of the tumor, its pseudocapsule
agnosis. Needle biopsies are general- tissues. Although acceptable for a (reactive zone), and all involved
ly successful except for very small benign lesion (eg, a ganglion), such muscles and/or bone in their entire-
tumors. treatment of a malignant lesion is ty as a single block of tissue. In the
Most biopsies are performed as unacceptable because tumor cells hand, a radical resection is often a
open surgical procedures, adhering usually remain in the pseudocapsule digit or ray amputation.
to several important principles. The (reactive zone). Generally, tumors
operation is performed under pneu- >3 cm in diameter in the hand are
General Surgical
matic tourniquet control unless not marginally excised; rather, they
Principles
medically contraindicated. Prior to are treated by wide local excision.
tourniquet inflation, the arm is not Lipomas are a notable exception, Soft-tissue sarcomas of the upper ex-
exsanguinated with an elastic or rub- however, because they rarely under- tremity are uncommon. Of approxi-
mately 5,000 soft-tissue sarcomas superficialis tendon (midportion of sible to avoid tender neuromas.
reported each year in the United the bone) is necessary, amputation at Although malignant tumors that
States, only 15% occur in the upper the level of the proximal interpha- remain within the cortex of a meta-
extremity and most of these are langeal joint is generally performed carpal often can be treated by ray re-
proximal to the wrist.1 These figures because active flexion of the joint is section, treatment is more compli-
are imprecise because no data are no longer possible. Tumors involv- cated and extensive for bone tumors
available on the exact number of cas- ing the middle phalanx of a finger that have broken through the cortex
es or types of malignancies treated usually require amputation through into the surrounding soft tissues.
each year. However, useful data are the MCP joint or a ray resection. Re- The priority of surgery is to obtain a
provided by the Surveillance, Epide- gardless of the level of amputation, safe margin of normal tissue. An ag-
miology, and End Results Program the permanent tissue sections must gressive malignant tumor of the
of the National Cancer Institute, be carefully inspected to ensure that thumb metacarpal that has broken
which collects data from tumor reg- the margins are tumor-free. through the cortex requires removal
istries in the United States.2 The When amputation of an index fin- of the entire first ray, the intrinsic
overall incidence of upper extremity ger at the level of the MCP joint is muscles, and occasionally the sec-
sarcomas has remained relatively appropriate, a modified ray resection ond metacarpal. In such cases, it
constant since these data were first is preferable to a disarticulation that may be possible to safely retain the
collected in 1973, but changes have retains the metacarpal head. A mod- index finger distal to the second
occurred in some subtypes. The ified ray resection eliminates that metacarpal as a “floating” finger
great majority of patients are Cauca- bony prominence and, unlike a stan- that can then be pollicized onto an
sian (83.7%), with males being more dard ray resection that removes the autograft or allograft that is arthro-
at risk (55.3%) than females. The av- entire metacarpal, preserves most of desed to the trapezium.1
erage patient age is 54.5 years; how- the normal width of the palm. In a Aggressive malignant tumors of a
ever, age varies depending on the modified ray resection, an oblique border finger metacarpal (ie, second
type of tumor. osteotomy is made through the or fifth) require ray resection of the
When a soft-tissue sarcoma does head-neck area of the second meta- involved finger and occasionally the
appear in the hand and wrist, it often carpal. This provides a smooth slope adjacent finger. Aggressive tumors of
presents as a painless mass that de- of the web between the thumb and a central finger (ie, middle or ring)
ceptively appears to be benign. Prior middle finger that also enhances the may require resection of three rays—
to biopsy, the mass may be misdiag- postoperative appearance of the the affected finger and the finger on
nosed as a ganglion, lipoma, soft- hand. A similar oblique osteotomy either side. Resection of the ulnar
tissue giant cell tumor, or even an through the head-neck area of the three rays will retain the thumb and
infection. Regardless of cell type, the fifth metacarpal (but with the angle index finger, thus preserving reason-
objective of treatment is wide exci- of the osteotomy in the opposite di- ably good pinch. With resection of
sion of the tumor. When the tumor rection) is used for a modified ray re- the second, third, and fourth rays, a
is distal to the metacarpophalangeal section of the little finger. rotational osteotomy of the fifth
(MCP) joint, the only option is com- When amputation of a thumb, metacarpal will improve tip-to-tip
plete or partial amputation. A soft- middle finger, or ring finger is neces- pinch between the remaining little
tissue or bone sarcoma confined to sary, the base of the metacarpal of finger and thumb. In some cases, all
the distal segment is often treated by that digit is preserved to permit sec- four finger rays must be resected to
amputation of that segment through ondary reconstruction. The pre- achieve a safe margin, leaving only
the next proximal joint. The carti- ferred reconstruction following a the thumb. Although prehension is
lage over the end of the middle pha- thumb amputation is pollicization significantly impaired, a thumb that
lanx is excised and the bone con- of the index finger. Following ampu- is sensate and mobile is still more
toured to eliminate prominent tation of a middle or ring finger, functional than a total prosthesis.
condyles. The digital nerves are cut transposition of the adjacent border The patient can be fitted with a par-
back to avoid tender neuromas, and ray is recommended to close the tial hand prosthesis that permits
the wound is closed using dorsal and space. Generally, pollicizations are pinch with the thumb. With any sur-
volar skin flaps. Skin closure should performed as secondary procedures, gery requiring resection of one or
be loose; it is preferable to resect whereas digital ray transpositions more rays, soft-tissue coverage of the
more of the middle phalanx than to are performed in conjunction with surgical site can be problematic.
have a tight skin closure that results amputation of a central finger to Soft-tissue closure can often be ac-
in a painful stump. When resection close the gap in the palm. With any complished using the skin and sub-
of the middle phalanx proximal to amputation, the digital nerves must cutaneous tissues of the amputated
the insertion of the flexor digitorum be cut back as far proximally as pos- finger as a fillet flap.
Figure 5 Figure 6
Figure 7 the elderly. They are aggressive le- That statistic is decreasing with im-
sions that spare the epidermis while proved surgical planning, using MRI
infiltrating deeper structures. Surgi- to determine the extent of tumor
cal excision with a 3-cm margin is spread. Treatment is wide excision
recommended, together with pro- of the lesion with a 3-cm margin, in-
phylactic regional lymph node dis- cluding the underlying deep fas-
section and adjuvant radiation ther- cia.17 Although adjuvant radiation
apy. Local recurrences are common, therapy has been used in patients
as are distant metastases; the prog- with positive margins, the preferred
nosis is worse than that for melano- treatment is excision of additional
mas. tissue.
Epithelioid sarcoma is one of the
Merkel cell tumor appearing as a raised more common soft-tissue sarcomas
Treatment of
nodule. These tumors rarely ulcerate. in the hand.18 Although the overall
Soft-Tissue Tumors
(Case courtesy of David Polsky, MD, incidence of the tumor is low, the
PhD, NYU Department of Dermatology Dermatofibrosarcoma protuberans is hand is frequently involved. The
Photography Archives.) a low-grade malignant tumor that typical presentation is a painless
arises in the dermis. The lesion ap- mass on the volar aspects of fingers
pears as a painless nodule that in or in the palms of young males, be-
crine carcinoma, originally was time may ulcerate and take on the tween the ages of 10 and 35 years
thought to be a variant of squamous appearance of a pyogenic granulo- (Figure 8). In many cases, it presents
cell carcinoma (Figure 7). However, ma.16 Dermatofibrosarcoma protu- as an ulcerating nodule that is ini-
immunohistochemical markers have berans extends into the subcutane- tially misdiagnosed as an infec-
shown that the tumor is of neuroepi- ous tissues, where it tends to spread tion.19 Generally, epithelioid sarco-
thelial differentiation. As with other in a horizontal fashion. Consequent- mas at surgery deceptively appear to
skin malignancies, they have a predi- ly, the recurrence rate following ex- be innocent. They are solid, gray-
lection for sun-exposed areas. Merkel cision of these tumors historically white, and seem to be encapsulated.
cell carcinomas are usually seen in has been high, approximately 50%. However, they are extremely malig-
Figure 8
Epithelioid sarcoma of soft tissue. A, Posteroanterior radiograph of a ring finger demonstrating a soft-tissue mass that had been
present for approximately 1.5 years. The deformity of the phalanx is secondary to the soft-tissue tumor. B, MRI scan of the same
ring finger demonstrating a soft-tissue tumor causing secondary bone deformity. C, Photomicrograph demonstrating a dense
proliferation of malignant epithelioid cells with areas of central necrosis (arrow).
Figure 11
Chondrosarcoma of the index metacarpal. A, Posteroanterior radiograph demonstrating bone expansion with cortical irregularity
and multiple radiolucencies in the distal half of the second metacarpal. B, MRI scan demonstrating expansion and disruption
of the cortex, with tumor extension into the soft tissues. C, Photomicrograph demonstrating a moderately cellular chondro-
sarcoma with large cells lined within lacunae. Several multinucleated tumor cells are evident (hematoxylin-eosin, original
magnification ×120). (Panel A reproduced with permission from Cawte TG, Steiner GC, Beltran J, Dorfman HD:
Chondrosarcoma of the short tubular bones of the hands and feet. Skeletal Radiol 1998;27:625-632.)
ing from a preexisting solitary sarcomas are radioresistant and (Figure 12). The extent of extra-
enchondroma is exceedingly rare.33 chemoresistant; the sole treatment osseous soft-tissue extension gen-
Because enchondromas are the most is surgery. In the hand, a ray resec- erally corresponds to the size of the
common benign cartilage tumors in tion is usually required. Fortunately, intramedullary tumor and the extent
the hand, differentiating between be- chondrosarcomas are generally slow- of cortical destruction. Some inves-
nign and malignant lesions is criti- growing and have an excellent prog- tigators have suggested that osteo-
cally important. There are several nosis. Metastases occur in <10% of genic sarcomas in the hand are less
key differences. Chondrosarcomas cases based on tumor grade. Chon- aggressive than tumors at other sites
tend to be painful and generally ap- drosarcomas are associated with lo- and have a better response to treat-
pear in older individuals (ages 40 to cal recurrences following inadequate ment that combines surgery with
60 years) compared with benign en- intralesional curettages. neoadjuvant and adjuvant chemo-
chondromas. A dramatic increase in Although osteosarcoma is the therapy.34,35 Ray amputation is the
size of an enchondroma in a patient most common primary bone tumor procedure of choice for an osteosar-
with Ollier’s disease is an ominous in children and adolescents, fewer coma of a phalanx. An osteosarcoma
sign. Radiographs and MRI provide than 40 cases in the hand have been of a metacarpal requires more exten-
important information; marked cor- reported. Generally, patients are old- sive surgery that usually involves re-
tical expansion and destruction ac- er, with a median age of 50 years. moving at least two rays.
companied by tumor extension into Many lesions of osteosarcoma are Ewing’s sarcoma involves the up-
the soft tissues usually is indicative secondary to radiation, Paget’s dis- per extremity in approximately 20%
of a malignant lesion (Figure 11). Re- ease, or multicentric metastatic dis- of cases. Fewer than 30 cases have
currence following curettage of a ease. Radiographs show areas of scle- been reported in the hand; the young-
previously diagnosed enchondroma rosis and periosteal reaction as est patient was a 5-month-old infant
also is cause for concern. Chondro- well as areas of bone destruction with a tumor in a distal phalanx.36
Figure 12 Figure 13
Figure 14
Metastatic lesion in the hand. A, Posteroanterior radiographic view of the thumb demonstrating destructive lesion of nearly the
entire distal phalanx in a patient with metastatic carcinoma of the lung. B, Gross specimen demonstrating almost complete
destruction of the distal phalanx by tumor. Note the clear differences between the distal and middle phalanges. (Case courtesy
of Bruce Ragsdale, MD.)
50% of cases. When a metastasis ex- nign lesions, some malignancies are RN, Pederson WC (eds): Green’s Oper-
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