CHAPTER 86 HAND TUMORS
BENJAMIN LEVI AND KEVIN C. CHUNG
In this chapter, we will address the management of tumors of the upper extremity, including those of the soft tissues.
Surgical biopsy incisions should be oriented longitudinally, along the long axis of the limb (Figure 86.1A–C), facilitating subsequent extirpation if required. Incisions should not be oriented transversely (Figure 86.1D). Historic teachings recommend against exsanguination of the arm before tourniquet inflation to avoid spread of the tumor cells; however, no studies have confirmed that this actually occurs.
All tissue that is removed should be sent for pathologic evaluation. The biopsy should ideally include a portion of the tumor as well as some surrounding normal tissue, although this is not always practical. After the diagnosis has been established, the surgeon plans for excision with appropriate margins in a one-stage or multistaged procedure. Most wounds from large tumor excisions can be closed temporarily with a skin graft or skin substitute while awaiting pathologic examination of the margins. If tendon or bone is exposed after deep resection, more complex tissue rearrangements or free tissue transfers may be required, after clear margins are confirmed.
BONY AND SOFT TISSUE TUMORS
Malignant tumors of the hand are rare, often leading to delayed treatment.1,2 If a malignant diagnosis is suspected, a tissue biopsy is obtained to establish the diagnosis, to assess the grade and clinical behavior, and to stage the tumor.
FIGURE 86.1. Orientation of excisional biopsy. (A–C) Correct orientation of vertically fashioned excision of hand lesion.
Histologic Grade and Surgical Staging
Unlike skin cancers, staging of bone tumors of the hand takes into account histologic grade (G) (Table 86.1). The most commonly used classification is as follows:
G1: Low grade, few cells, much stroma, little necrosis, mature cells, fewer than five mitoses per high-power field
G2: High grade, many cells, little stroma, much necrosis, immature cells, more than 10 mitoses per high-power field
Benign Bony and Soft Tissue Tumors
General. Benign bone tumors of the hand and forearm are frequently asymptomatic, slow growing, and require no surgical intervention. If these lesions enlarge, become symptomatic, or appear to have an aggressive subtype, however, a biopsy is required.
Ganglions and Mucous Cysts. Ganglion cysts are the most common soft tissue masses in the upper extremity and are well-circumscribed, mucin-filled lesions that are attached to the underlying joint capsules or tendon sheaths. Ganglions are most commonly seen in patients 30 to 40 years old and are more common in women. A history of trauma is present in 10% of cases. The most common anatomic location is the dorsal wrist, which represents 60% to 70% of upper extremity ganglions. Sometimes referred to as retinacular cysts, these lesions are postulated to originate from the extensor retinaculum and are usually located over the scapholunate ligament between the second and fourth extensor compartments (Figure 86.2A–C). Dorsal ganglion cysts should be excised under tourniquet control. A transverse ellipse is excised around the tumor in an attempt to avoid its puncture. However, if the cyst is large, the cyst can be punctured when dissection becomes difficult to facilitate excision of the dorsal wrist capsule in continuity with the cyst stalk. During surgical excision of cysts, extensor tendons are visualized and retracted radially and ulnarly in order to avoid damaging them. Cysts over the radial border of the wrist should also be dissected with particular care to avoid the sensory branch of the radial nerve, which can course directly over the cyst (Figure 86.2C).
Ganglion cysts of the volar wrist are often located between the radial artery and the flexor carpi radialis, directly over the distal end of the radius or scaphoid tubercle. These cysts originate from the radiocarpal joint ligaments or scaphotrapezial joint. Radial and ulnar artery patency should be evaluated by an Allen test. The cyst will be attached intimately to the radial artery, which may be injured during dissection. The radial artery should be gently peeled off the cyst which can be punctured to facilitate removal. Volar ganglions are excised in a manner similar to dorsal ganglions; however, the incision is designed to allow for visualization of the neurovascular bundles. To prevent recurrence of all ganglions it is necessary to remove the stalk. Ganglions located in the distal interphalangeal (DIP) joint are called mucous cysts. Mucous cysts are usually seen in older patients and cause attenuation of the overlying skin or grooving in the nail from pressure exerted by the cyst (Figure 86.3A andB). Patients often say these lesions appeared suddenly or over the course of several months. The cysts may also subside or enlarge with activity and rupture. Osteoarthritis is always seen in the underlying joint in these patients. Aspiration may alleviate symptoms; however, this rarely provides a long-term solution.5 The cysts can be excised through a V-shaped incision centered over the lateral DIP joint with skin excision or rotation-advancement flaps.6 More recent studies have demonstrated similar outcomes with a transverse incision centered over the DIP joint. Osteophytes and affected portions of the joint capsule are excised to prevent recurrence.7
Giant Cell Tumor of Tendon Sheath. Giant cell tumors of the tendon sheath or benign fibrohistiocytomas represent the second most common soft tissue mass of the hand. These lesions are thought to represent a reactive process and thus do not have single cell type of origin.8 Clinically, giant cell tumors of the tendon sheath present as firm, painless nodules close or adherent to the tendon sheath. These benign tumors can also be found within the tendon sheath and can cause triggering. The most common locations for these lesions are the middle finger, index finger, and thumb, and these lesions grossly appear yellowish or tan (Figure 86.4A and B). Histologically, a large number of histiocytes with no mitotic figures are seen. Surgical excision of the tumor and its stalk is the recommended treatment. When present in phalanges, adequate exposure and visualization of the digital nerves is necessary. Despite complete excision, recurrence of these tumors has been shown to be 13% at 3 years.9
Epidermal Inclusion Cyst. Epidermal inclusion cysts are the third most common tumor of the hand. The palm and fingertips are the most commonly affected regions, and these masses are more common in individuals with occupations susceptible to common penetrating hand injuries. These lesions are thought to form after penetrating trauma causes implantation of epidermal cells in the dermis. Epidermal inclusion cysts are slow growing, firm, round, nontender masses attached to the overlying skin that are filled with protein, cholesterol, and fat. Complete excision is necessary to prevent recurrence.
Glomus Tumor. The glomus body is an arteriovenous anastomosis within the dermis that regulates vascular flow for temperature control at the fingertip. Glomus tumors are a benign proliferation of this glomus body.10 Patients present with painful subcutaneous and subungual nodules that are hypersensitive to cold.11 Glomus tumors can also appear as a subungual bluish discoloration with or without overlying nail plate changes. Radiologic diagnosis can be made by magnetic resonance imaging (MRI) on which the T2-weighted images are characteristic. Treatment of choice for glomus tumors is currently surgical excision through a transungual approach, which has been shown to provide a low recurrence rate and rare nail deformities.12-14
FIGURE 86.2. Dorsal wrist ganglion cyst removal. A. Dorsal ganglion cyst seen over the ulnar dorsal wrist. B. A transverse incision is made over the ganglion and the ganglion is freed from the surrounding tissue taking care not to injure the overlying superficial radial sensory nerve (arrow). C. After ganglion removal, the superficial radial sensory nerve can be seen on the blue background.
Osteoid Osteoma. Osteoid osteoma is a benign bone tumor found in the fingers, wrist, or distal radius. The classic presentation is nighttime pain, swelling, and tenderness that are relieved by aspirin. A small radiolucent lesion with a sclerotic margin is present on plain radiographs. If untreated, this tumor may resolve, though it can take several years. If nonsurgical management is employed, aspirin is prescribed for pain. Recently, radiofrequency ablation has become popular for osteoid osteomas proximal to the wrist. Due to the proximity of these tumors to neurovascular structures in the hand, radiofrequency ablation is not recommended distal to the wrist.15 Surgical treatment includes marginal resection, or curettage and bone grafting. Clinical history differentiates this tumor from an osteoblastoma which is histologically identical. Furthermore, osteoid osteomas are more commonly cortical, medullary, or subperiosteal, whereas osteoblastomas are usually medullary in origin.
Osteochondroma. Although a common tumor of the general skeleton, osteochondromas are rare within the upper extremity. Osteochondromas originate in the physis or regions of tendon insertion.16-18Though rare in the hand, they are most commonly seen in the distal aspect of the proximal phalanx in the second to third decade of life.19 Clinically, osteochondromas cause the overlying skin to protrude and may mechanically inhibit range of motion, as well as cause tendon snapping, irritation, and rupture.20,21 Radiographically, they appear as bony exostoses, and histologically they demonstrate a benign cartilage cap similar to that seen in the epiphysis. These lesions can be left undisturbed, but resection is recommended and curative if functionally debilitating. Though the potential for malignant transformation exists in the general skeleton, such degeneration has not been reported in the hand.
FIGURE 86.3. Mucous cyst. A. A mucous cyst is seen over the middle finger DIP joint. The surgical incision and subsequent rotational flap is drawn. B. Three weeks postoperative view of the hand after excision and rotational flap closure of the mucous cyst.
Giant Cell Tumor of Bone. Giant cell tumor of bone is a benign tumor with the potential to metastasize. It is most commonly found in the distal radial metaphysis. Though rare in the hand, the hamate is the most common carpal bone affected.22 Patients usually present in the third decade with pain and swelling. Radiographically, these lesions are radiolucent without bone formation or calcification within the lesion. These lesions also cause cortical bone thinning and can encroach on the articular surface. A more precise radiographic classification was described by Campanacci et al.23:
Grade I: Well-marginated, intact cortex
Grade II: Well-defined, thinned expanded cortex
Grade III: Nondiscrete borders bulging into soft tissues and lacking a shell of reactive bone
Histologically, these tumors demonstrate mononuclear stromal cells that can be seen invading local muscle (Figure 86.5A). Hallmark giant cells have nuclei similar to stromal cells (Figure 86.5B). Though benign in nature, chest computed tomography (CT) evaluation is recommended due to the potential for metastasis to the lung. Biopsies of suspected tumors are designed in the direction of future incisions if a larger resection is required (Figure 86.6A and B). Exact treatment protocols are difficult to compare given the rare occurrence of the disease. In general, smaller isolated tumors are treated with curettage and bone grafting. More aggressive treatment including wide excision or amputation is reserved for large giant cell tumors of the hand. In the distal radius, curettage with resection and allograft is recommended over curettage and cementation.24 We advocate en bloc resection followed by reconstruction using a fibula free flap. If present in the distal carpal row, treatment includes wide excision with limited carpal fusion, whereas proximal row lesions require proximal row carpectomy. Giant cell tumors of the radial shaft should be resected en bloc accompanied with nonvascularized or vascularized bone graft reconstruction. A free fibula osteocutaneous flap offers an ideal bone and soft tissue composite flap to reconstruct such a defect (Figure 86.7A–H).
FIGURE 86.4. Excision of giant cell tumor of the tendon sheath. A. Giant cell tumor of the tendon sheath over the middle finger flexor tendons. B. Excision of giant cell tumor showing its yellow, tan appearance.
FIGURE 86.5. Giant cell tumor histology. A. Giant cell tumor abutting normal muscle. Black arrow points to tumor. Blue arrow points to muscle. B. High-power views of giant cells with black arrow pointing to the giant cell nuclei.
Enchondroma. Enchondromas are the most common primary bone tumor arising from the bones in the hand.25 Studies have shown that 35% of enchondromas present within the hand, representing up to 90% of bony tumors of the hand. Such lesions occur across all ages; however, they are most commonly seen in the fourth decade of life. Enchondromas most commonly arise in the proximal phalanx, followed by the metacarpal, middle phalanx, and carpal bones. Patients who demonstrate multiple enchondromas should be suspected of having Ollier disease or multiple enchondromatosis. This is an uncommon, nonhereditary bone disorder in which afflicted patients have multiple enchondromas in the metaphysis and diaphysis of long bones or short tubular bones of the hands. Though enchondromas can present bilaterally, Ollier disease usually presents unilaterally.
Enchondromas are considered benign; however, the risk of malignant transformation into a chondrosarcoma or osteosarcoma is said to be as high as 30%. Patients are followed with serial X-rays. Radiographically, these tumors appear as an intramedullary radiolucency, with a lytic pattern that may include rings and arcs of chondroid calcifications (Figure 86.8). Histologically, enchondromas possess lamellar bone, hyaline cartilage, and matrix calcifications.26 Patients present with a painless mass, swelling, or a pathologic fracture.
If a fracture is found at the time of diagnosis, the fracture is managed by immobilization and after the fracture has healed, the tumor is removed. Complete resection of enchondromas is necessary to avoid recurrence. Figure 86.9 demonstrates wide local excision of a metacarpal enchondroma. Intraoperative radiography can be used to ensure complete excision of the tumor. The resulting defect is reconstructed with an autogenous bone graft or allograft material, though some surgeons avoid autograft to decrease the risk of contamination of the donor field (Figure 86.9D). We have found that demineralized bone matrix can be used to reconstruct the bony defect and allows for complete bony healing of the defect (Figure 86.10).
FIGURE 86.6. Giant cell tumor biopsy. A. Preoperative photo of a wrist with a giant cell tumor. B. Vertically oriented biopsy incision of a giant cell tumor.
FIGURE 86.7. Giant cell tumor extirpation and reconstruction using a free fibula osteocutaneous flap. A. Preoperative X-ray of the giant cell tumor. B. Surgical incision is fashioned over the previous biopsy incision and oriented transversely to allow for wide exposure. C.Isolation and mobilization of the tumor. Arrow points to tumor. D. Planned free tissue transfer of a fibula myocutaneous flap. E–F. Inset of the free fibula myocutaneous flap into the defect. G. Final closure of the defect after radical excision of the Giant Cell Tumor. H.Postoperative X-ray of free fibula reconstruction.
MALIGNANT HAND TUMORS
Since most tumors of the hand are benign, malignant tumors are rarely suspected, which may cause delay in diagnosis. Furthermore, the rare occurrence hampers research on this topic beyond small case series. In general, patients with suspected malignancies have laboratory studies including serum alkaline phosphatase, calcium, and phosphorus. In addition, blood urea nitrogen and creatinine are obtained to evaluate for metabolic bone disease. Diagnostic imaging is performed to assess the size and location of the tumor in addition to sites of possible metastases.
FIGURE 86.8. Preoperative radiograph of an enchondroma of the small finger metacarpal shaft.
Soft Tissue Sarcoma
Soft tissue sarcomas of the upper extremity are rare and represent only 15% of all sarcomas seen annually.27 Recent publications suggest that patients with sarcoma of the hand might have improved outcomes over those in other anatomic locations. This improved outcome may be because a mass in the hand is more obvious than deep in more muscular regions of the anatomy.28 Preoperative evaluation includes both X-ray and MRI of the hand, as well as a chest CT scan to evaluate for metastases. In addition, many centers also recommend a positron emission tomography to evaluate for extra-pulmonary sites of disease.29 Lymph node dissections are performed for regional nodal involvement. If nodal status is unknown, recent studies have used sentinel lymph node biopsies for rhabdomyosarcomas and pediatric sarcomas.30,31
Surgical excision remains the recommended treatment for soft tissue sarcomas, with the goal of achieving negative surgical margins. Ray amputations or wrist disarticulations may be required. Because of the functional requirements, critical components of the hand are preserved when possible, rather than hand amputation. Adjuvant therapy is employed to eradicate residual tumor that may be present when radical ablative procedures are not performed in the hand. Regardless of the secondary defect caused by tumor extirpation, attention is paid to soft tissue coverage to maximize functional recovery. A team approach is employed for soft tissue sarcomas including medical and radiation oncologists. Radiation has been shown to reduce local recurrence rates and is indicated for high-grade tumors with close resection margins. Preoperative radiation is added for tumors larger than 5 cm and those abutting important structures.2 Chemotherapeutic adjuvant therapy reduces local and distant recurrence rates and offers a slight survival advantage.32
Chondrosarcomas are the most common primary malignant tumor of the hand and are aggressive with a high rate of local recurrence.33-35 These tumors are most common in patients over 60 years old and are usually located in the proximal phalanx and metacarpal bones. Clinically, these lesions present as a slow growing, firm, painful mass. X-rays demonstrate stippled calcifications, cortical expansion, and a poorly defined border with frequent extension into the soft tissues. Histologically, cartilage cells with mitotic figures and hypercellularity are seen. Despite being malignant, these tumors are slow growing and metastasize in only 10% of cases.36 Diagnosis is made through a well-planned biopsy using an incision that can be used for subsequent resection. Subsequent staging includes a chest CT to evaluate for lung metastases. Current treatment recommendations take into account the tumor grade, risk of recurrence, and functional loss. In general, en bloc resection is recommended with ray amputation performed for digital involvement. While studies have demonstrated similar outcomes with curettage and local adjuvant treatment, we believe that radical surgical excision offers a more definitive removal and better prognosis.33,34,37,38
FIGURE 86.9. Enchondroma resection. A. An indentation over the small finger can be seen from the initial biopsy. B. Vertically oriented excision over the region of the tumor is performed allowing visualization of the intrinsic extensors. C. Resection of the metacarpal leaves a gap in the remaining metacarpal shaft. D. Rather than risk seeding a donor site, demineralized bone matrix is used to fill the dead space.
FIGURE 86.10. Postoperative X-ray after enchondroma resection and placement of demineralized bone matrix.
Osteogenic sarcomas of the hand are rare, comprise only 0.18% of all osteosarcomas, and usually occur in patients between 50 and 70 years old.2 Clinically, these tumors present as rapidly enlarging, firm, painful masses. Radiographs demonstrate a sclerotic or lytic lesion with soft tissue invasion. Histologic analysis reveals malignant osteoblasts with abnormal nuclei, increased mitoses, and immature bone with osteoid. Like chondrosarcomas, these lesions are most commonly found in the proximal phalanges and metacarpals. Treatment requires the combination of chemotherapy and wide resection with possible ray amputation.39,40 Preoperative chemotherapy shrinks the tumor, which may help preserve important structures during tumor resection. Regardless of whether preoperative chemotherapy is initiated, postoperative chemotherapy is started within 21 days after tumor resection.41,42
Ewing sarcoma is a common childhood sarcoma, but is rarely found in the hand. Patients with Ewing’s sarcoma often present with swelling, pain, and erythema that can be associated with leukocytosis and fever. Such constitutional symptoms can be initially confused with an infectious process. When involving the hand, Ewing sarcomas most commonly arise in the metacarpals or phalanges and invade surrounding soft tissues. Radiographs demonstrate large, lytic expansile lesions that are destructive to the bone and its surrounding tissues. MRI offers additional information regarding soft tissue involvement. As in other malignant bone tumors, biopsies are oriented to allow for subsequent resection. Treatment regimens include chemotherapy for systemic control, as recent studies have demonstrated a 78% survival when patients are treated with surgery and chemotherapy.43
Tumors of the hand are rare compared with other anatomic locations. The density of important anatomic structures and the limited local options for reconstruction make early diagnosis critical to ensure favorable outcomes. The first step is an appropriately oriented biopsy. Definitive extirpation is then performed with the recommended margin. The “reconstructive ladder” is applied to the defect, keeping in mind the limited utility of skin grafts to cover important, poorly vascularized structures such as cartilage and bone. As in skin and bone tumors elsewhere in the body, a multidisciplinary approach optimizes treatment for the uncommon malignant tumors of the hand and upper limb.
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