Hand Tumors

Question 1

What is the role of computed tomography in assessing upper extremity tumors?

Answer 1

Used to evaluate extent of bone destruction as well as calcified lesions.

Question 2

What is the role of magnetic resonance imaging in assessing upper extremity tumors?

Answer 2

Used for evaluation of lesions involving bone and soft tissue.

Question 3

How is clonality used to differentiate between neoplasms and benign tissue growths (ie, Dupuytren disease)?

Answer 3

Benign and inflammatory conditions are typically polyclonal, whereas neoplasms are monoclonal.

Question 4

In which direction should the incision be oriented when obtaining a biopsy of an upper extremity mass?

Answer 4

Longitudinal (rather than transverse or zigzag) so as to incorporate the biopsy site within the definitive excision or
amputation.

Question 5

When performing a biopsy on a lesion, should one dissect around muscle planes or split the muscle sharply?

Answer 5

Split sharply (to avoid seeding other muscle compartments).

Question 6

When performing an open biopsy, should drains be placed?

Answer 6

No, drains can seed tumor cells along their tracts. Simply obtain hemostasis.

Question 7

Should a tourniquet be used when operating on a neoplasm of the upper extremity?

Answer 7

Yes, but exsanguination of the arm should be done through elevation, not Esmarch bandage.

Question 8

What is the staging system for musculoskeletal tumors?

Answer 8

Stage Grade
IA Low (G1)
IB Low
IIA High (G2)
IIB High
Site
Intracompartmental Extracompartmental Intracompartmental Extracompartmental

Question 9

What is the usual etiology of epidermal inclusion cysts?

Answer 9

Trauma that causes epidermal cells to become embedded in the dermis.

Question 10

Where do epidermal inclusion cysts usually occur?

Answer 10

In the glabrous, non–hair-bearing skin of the palms and fingertips (especially perionychium).

Question 11

What is the treatment of epidermal inclusion cysts?

Answer 11

Complete excision along with the skin puncture wound (or punctum, if present

Question 12

What are the clinical findings seen with a subungual glomus tumor?

Answer 12

Severe pain, cold sensitivity, tenderness, nail deformity, or discoloration.

Question 13

What is the treatment of a subungual glomus tumor?

Answer 13

Removal of nail, excision of tumor.

Question 14

What is the likely diagnosis of a patient with a subcutaneous elevation just proximal to the eponychial fold and nail grooving?

Answer 14

A mucous cyst, or fluid-filled ganglion of the distal interphalangeal joint associated with bony spurs and nail grooving.

Question 15

What is the treatment of mucous cysts?

Answer 15

Cyst excision and removal of bone spurs.

Question 16

What is the name of a rapidly growing vascular, friable nodule frequently seen on fingertips in young adults, more common after pregnancy?

Answer 16

Pyogenic granuloma.

Question 17

What is the name of the lesion that is commonly found on the dorsal hand that is round, elevated, and usually has a central crater? This lesion usually resolves spontaneously, and only occasionally progresses to squamous cell carcinoma.

Answer 17

Keratoacanthoma.

Question 18

What is the natural history of keratoacanthomas?

Answer 18

They undergo three phases:
1. proliferation
2. maturation
3. involution
The clinical story will usually include the rapid growth of a preexisting lesion that then gradually gets smaller as the
central crater expels a keratin plug.

Question 19

What is the recommended treatment for keratoacanthomas?

Answer 19

Surgical excision or intralesional injection with 5-fluorouracil (5-FU) or methotrexate.

Question 20

What are the first and second most common tumors or masses of the hand?

Answer 20

Ganglions and giant cell tumors (GCTs), respectively.

Question 21

What is the predominant cell type in GCTs (aka localized nodular synovitis)?

Answer 21

Histiocytes.

Question 22

What is the usual site of origin of GCTs?

Answer 22

Flexor tendon sheath.

Question 23

What is the treatment of GCTs?

Answer 23

Excision along with stalk (if present).

Question 24

What is the difference between a neurofibroma and a neurilemmoma?

Answer 24

Neurofibroma: in the substance of the nerve. Neurilemmoma (also known as schwannoma): on the nerve surface.

Question 25

How does this difference impact treatment?

Answer 25

Neurilemmoma can be “shelled out” easily, while neurofibroma requires transection at the proximal and distal fascicles.

Question 26

Are these common tumors?

Answer 26

Neurilemmomas are the most common benign nerve tumors in the upper extremity.

Question 27

A patient presents with multiple neurofibromas of the upper extremity and cutaneous cafe ́-au-lait spots. What is your diagnosis?

Answer 27

Von Recklinghausen disease or neurofibromatosis type 1.

Question 28

A patient presents with bilateral acoustic schwannomas. What is the likely diagnosis?

Answer 28

Neurofibromatosis type 2. Note that these patients rarely have neurofibromas.

Question 29

What diagnostic studies can be used to differentiate between neurofibroma and neurilemmoma?

Answer 29

Magnetic resonance (MR) and nerve conduction studies.

Question 30

What is the treatment of fibromatosis and juvenile aponeurotic fibromas?

Answer 30

Wide excision with skin grafting or free tissue transfer, which still has a high potential for recurrence

Question 31

What is the chief problem seen with desmoid tumors?

Answer 31

High rate of recurrence (especially in female patients).

Question 32

In general, what is the treatment of upper extremity arteriovenous malformations?

Answer 32

Ligation of feeding vessels, complete excision.

Question 33

Are vascular malformations really neoplasms?

Answer 33

No, they are simply an abnormal collection of blood vessels.

Question 34

What is the most common benign bone tumor?

Answer 34

Enchondroma.

Question 35

What are the common locations of enchondromas?

Answer 35

Metacarpals (most common) and phalanges. Carpal bones are rarely involved.

Question 36

What is Ollier disease?

Answer 36

Multiple enchondromatosis.

Question 37

What is Maffucci syndrome?

Answer 37

Multiple enchondromas and hemangiomas.

Question 38

What is the lifetime chance of a solitary enchondroma undergoing malignant transformation?

Answer 38

10%.

Question 39

What do enchondromas degenerate into?

Answer 39

Chondrosarcomas.

Question 40

How do enchondromas frequently present?

Answer 40

Pathologic fracture.

Question 41

What is the treatment of enchondromas?

Answer 41

Curettage and bone grafting. In cases of pathologic fracture, it is optimal to allow fracture healing prior to definitive resection

Question 42

What benign cartilaginous tumor is similar to enchondromas and most commonly found at the metaphyseal–diaphyseal junction of the phalanges?

Answer 42

Periosteal chondroma.

Question 43

What is the peak age range of unicameral bone cysts (UBCs)?

Answer 43

5 to 10 years. In fact, it is seen almost exclusively in children.

Question 44

What is the typical presentation of UBC?

Answer 44

An incidental finding on X-ray or a pathologic fracture through the cyst.

Question 45

What nonsurgical treatment is used for UBCs?

Answer 45

Intralesional steroid injection.

Question 46

What is the name of a blood-filled cyst that typically occurs in the metaphysis of a metacarpal and then grows toward the physis?

Answer 46

Aneurysmal bone cyst (ABC).

Question 47

What is the peak age range of ABC?

Answer 47

Second decade of life.

Question 48

What is the typical presentation of ABC?

Answer 48

Swelling and pain, often following an injury.

Question 49

What is the treatment of ABCs?

Answer 49

Curettage and bone grafting. This is an erosive, although benign, lesion that has to be removed.

Question 50

What is the usual structure of an osteochondroma?

Answer 50

Bone stalk and cartilaginous cap growing from the metaphysis in skeletally immature patients.

Question 51

Is excision necessary for osteochondromas?

Answer 51

No, unless they are symptomatic. They rarely undergo malignant degeneration.

Question 52

What are the symptoms of an osteoid osteoma?

Answer 52

Pain at night relieved by nonsteroidal anti-inflammatory drugs.

Question 53

How do osteoid osteomas present on imaging studies?

Answer 53

Sclerotic nidus with a lucent halo, less than 1.0 cm in diameter (by definition).

Question 54

What is the histology of osteoid osteomas?

Answer 54

Very vascular nidus of osteoblasts with surrounding cortical reactive bone formation.

Question 55

What is the treatment of osteoid osteomas?

Answer 55

Curettage and bone grafting.

Question 56

What is an osteoblastoma?

Answer 56

Same as an osteoid osteoma, but greater than 1 cm in diameter. They have unlimited growth potential and all should be resected.

Question 57

What is the clinical presentation of a giant cell tumor (GCT) of bone ?

Answer 57

Gradual swelling, pain, sometimes with pathologic fracture, most often in the distal radius.

Question 58

Why do some classify GCTs as low-grade malignancies?

Answer 58

They can metastasize and cause death.

Question 59

Where do GCTs of bone typically metastasize?

Answer 59

The lungs (2%).

Question 60

How do GCTs look on radiographs?

Answer 60

Lytic lesion; no new bone formation; encroaches on, but does not penetrate joint surface.

Question 61

What is the surgical treatment of GCTs?

Answer 61

Wide excision, joint reconstruction if necessary.

Question 62

What is the pathophysiology of fibrous dysplasia?

Answer 62

Bone marrow of involved bone(s) filled with noncalcified collagen.

Question 63

What is the X-ray appearance of fibrous dysplasia?

Answer 63

Ground-glass opacity.

Question 64

Is treatment of fibrous dysplasia of the hands required?

Answer 64

Not usually. Surgical treatment is usually performed for pathologic fractures, or “impending” fractures.

Question 65

What are some other common soft tissue masses in the upper extremity?

Answer 65

Ganglia, lipomas, foreign body granulomas, retinacular cysts, palmar fibromatosis, or nodules (Dupuytren).

Question 66

What is the most common malignant tumor of the hand?

Answer 66

Squamous cell carcinoma.

Question 67

What is the most common type of melanoma among African Americans and Asians?

Answer 67

Acral lentiginous melanoma, usually presenting on hands and feet.

Question 68

When treating melanoma of the hand, how is the amputation level determined?

Answer 68

Amputate proximal to the nearest joint (ie, for a subungual melanoma, amputation would be through the middle phalanx).

Question 69

What role does sentinel node biopsy play in the treatment of subungual melanoma, and why?

Answer 69

Sentinel node biopsy is useful with subungual melanoma because depth of the tumor is difficult to determine in the nail bed.

Question 70

What is the name of a lesion arising in the dermis that presents as a purple-red plaque or nodule?

Answer 70

Dermatofibrosarcoma protuberans (DFSP).

Question 71

Has Mohs surgery been shown to be effective for DFSP?

Answer 71

Yes.

Question 72

What is a strong risk factor for malignant peripheral nerve sheath tumor?

Answer 72

Neurofibromatosis (Von Recklinghausen disease).

Question 73

What is a synovial cell sarcoma?

Answer 73

High-grade sarcoma that grows in proximity to (but not in) joints. Size of the lesion is proportional to the mortality.

Question 74

What is the treatment of synovial cell sarcoma?

Answer 74

Wide excision, lymph node sampling (and dissection if nodes involved), and consideration of adjuvant radiation therapy.

Question 75

What other sarcoma is similar to synovial cell sarcoma and usually arises from muscle?

Answer 75

Epithelioid sarcoma.

Question 76

What about the spread of epithelioid sarcoma makes it dangerous?

Answer 76

It moves proximally along fascial planes, tendons, and lymphatics.

Question 77

Where is malignant fibrohistiocytoma usually found in upper extremities?

Answer 77

Deep muscle mass of adductor pollicis or within muscles of the flexors.

Question 78

What is the most common malignant primary bone tumor of the hand seen in children and teens?

Answer 78

Osteogenic sarcoma.

Question 79

How does osteogenic sarcoma look on plain radiograph?

Answer 79

Bone growth outside normal skeletal boundaries with hazy “cloud-like” bone formation into soft tissues.

Question 80

Is there a role for external beam radiation in treatment of osteogenic sarcoma?

Answer 80

No, but chemotherapy has greatly improved survival and limb salvage.

Question 81

What is the most common malignant primary bone tumor of the hand in adults?

Answer 81

Chondrosarcoma.

Question 82

Is chondrosarcoma sensitive to chemotherapy or radiation?

Answer 82

No.

Question 83

What is the benign predecessor that can rarely degenerate into a chondrosarcoma?

Answer 83

Enchondroma.

Question 84

What is a rare bone tumor of the metacarpals and phalanges that consists of abnormal endothelial cells?

Answer 84

Angiosarcoma.

Question 85

What is a typical presentation of a Ewing sarcoma of the hand?

Answer 85

Pain, swelling, soft tissue mass; can also have fever, elevated WBC and/or ESR.

Question 86

What is the radiographic presentation of Ewing sarcoma?

Answer 86

Large lytic lesion of bone with a soft tissue component.

Question 87

What is the periosteal reaction on radiographs called in Ewing sarcoma?

Answer 87

“Onion skin” appearance or “sunburst” pattern.

Question 88

What are the common sites of Ewing sarcoma of the hand?

Answer 88

Metacarpals, phalanges.

Question 89

What is the treatment of Ewing sarcoma?

Answer 89

Surgical excision, systemic chemotherapy and/or external beam radiation.

Question 90

What percentage of patients with primary cancers in other parts of the body will develop a metastasis to the hands or feet?

Answer 90

0.3%.

Question 91

What is the most common primary carcinoma that metastasizes to the hand?

Answer 91

Bronchogenic carcinoma (followed by breast and kidney).

Question 92

When primary carcinoma metastasizes to the hand, where does it go?

Answer 92

The distal phalanx.