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Benign Soft Tissue Tumors of the Hand PDF Print E-mail
O. Ugwonali, MD
Columbia Presbyterian Medical Center
Columbia University
New York, New York

Vishal Sarwahi, MD

Melvin P. Rosenwasser, MD
Robert E. Carroll Professor of Hand Surgery
Chief of Orthopaedic Hand and Trauma Services
Director of Trauma Training Center
Columbia University Medical Center
New York, New York


Pretest


Introduction

More than 90% of hand and wrist masses are benign soft tissue tumors.1 Tumor size, location, mobility, color, and texture provide clues to diagnosis. Biopsy is the only means for a definitive diagnosis.


Ganglions

Ganglions comprise 33% to 69% of all benign soft tissue tumors of the hand and wrist.2-3 Ganglions have a male to female ratio of 1:3 and are common in the third to fourth decades of life.4 The dorsal wrist ganglion, which originates at the scapholunate ligament, accounts for the majority of ganglions.4 Ganglions can also originate from bone, tendons, ligaments, or joints. Pain, discomfort, weakness, restricted motion, and disfigurement are the typical presenting features but, at times, ganglions are painless, firm, and immobile masses that vary in size. Transillumination is a hallmark of ganglions.

Needle aspiration is both diagnostic and therapeutic, although the rate of recurrence is high after this procedure. The recurrence rate is significantly reduced when a dorsal wrist ganglion, along with its pedicle and a portion of the capsule to which it is attached (45%), is surgically excised compared to excision of the cyst alone (13% to 40%).5 Since the main cyst of the volar wrist ganglion (second most common) may be situated between the bifurcating branches of the radial artery, Allen’s test is essential prior to surgical excision to ascertain the patency of the radial and ulnar arteries. Digital massage or needle rupture may negate or delay the need for surgical excision of the volar retinacular ganglion of the hand’s proximal tendon sheath, which is the third most common ganglion of the wrist and hand. Treatment of mucous cysts, which are ganglions of the distal interphalangeal joint associated with osteoarthritic changes, entails excision of the cyst and osteophyte resection of fusion.


Giant Cell Tumor of Tendon Sheath

Also known as pigmented villonodular tenosynovitis or fibroxanthoma, giant cell tumor of tendon sheath is the second most common soft tissue tumor of the hand and wrist. It presents as a slowly enlarging, painless multilobulated mass and tends to occur on the palmar surfaces of fingers, commonly causing nerve compression and bone or joint invasion. The lesion does not transilluminate. There is a propensity for involvement of the radial three digits and the distal interphalangeal joint region. Plain radiograph may show a soft tissue mass or pressure erosion of the bone. Bone invasion is not typical and may indicate an aggressive neoplasm. Opening the joint or tendon sheath during excision minimizes the risk of recurrence, which otherwise ranges from 5% to 50%.6-9 Recurrence is believed to be due to the presence of a satellite lesion or as a result of incomplete excision. Osseous erosion and tumors of the distal interphalangeal joint are associated with a high recurrence rate.


Epidermal Inclusion Cyst

First described by Wernher in 1855,10 epidermal inclusion cysts are the third most common tumor of the hand.2,3 Several theories for its etiology exist but the current opinion is that an open injury or laceration, which allows the entry of epidermis into subcutaneous tissue, stimulates the development of epidermal inclusion cysts. It is usually a painless, mobile, and firm palmar mass mostly in the fingertip and is usually only of cosmetic concern unless it becomes large enough to impair function. It does not transilluminate. When involving the bone, the radiograph may show a well-demarcated lytic lesion or occasionally cortical perforation, which may raise the suspicion of malignancy or infection. Biopsy in such cases aids in diagnosis. Curative treatment entails surgical excision and curettage of the intraosseous portion, while painstakingly preserving the extensor mechanism. If the lesion is not resectable, amputation of the distal phalanx may become necessary.11 Recurrence is uncommon.


Vascular Lesions

Vascular tumors are the fourth most common tumor and account for approximately 8% of all hand tumors.1,2,12-15 The majority of these tumors are benign and can be either congenital or acquired. The congenital lesions include the hemangiomas and other vascular malformations, whereas the acquired lesions occur secondary to trauma or neoplastic growth.16 An important component of the physical examination that affects management is the Allen’s test, which confirms the patency of the deep and superficial palmar arches. Although angiography remains the gold standard for the evaluations of complex vascular tumors, magnetic resonance imaging (MRI), closed-system venography radionuclide and duplex color imaging, computed tomography (CT), and xeroradiography can add valuable information.17-26

Glomus tumors
Glomus tumors arise from the glomus or neuromyoarterial apparatus, which normally function as temperature regulators, and are located in large numbers under nails, in finger pads, and elsewhere in hands and feet. Glomus tumors are classified as solitary, multiple painful, or multiple painless tumors.22 Up to 75% of glomus tumors occur in the hand27 and 65% are located in the fingers. Initially, patients complain of intermittent severe lancinating pain around the distal finger and, later, patients complain of point-specific tenderness and cold insensitivity, which represent the classic triad of glomus tumors.28 Ridging of the nail may occur, as well as pressure-induced indentation of the distal phalanx, which is seen on radiographs. Well-formed vascular channels and nonmyelinated nerve endings are seen on histology. In 50% of cases, physical examination is unremarkable. Localized bluish discoloration in the nail bed is strongly suggestive. MRI can pinpoint lesions of more than 5 mm in the finger that appear bright on T2 radiographs and dark on T1 radiographs. Treatment of glomus tumors is surgical excision. Persistent symptoms after surgical excision have been attributed to the existence of multiple glomus tumors.24 There is a 20% rate of recurrence. Thus, persistence of symptoms for more than 3 months should prompt reexploration.

Pyogenic granulomas
A pyogenic granuloma is an inflammatory bed of granulation tissue with recurrent bleeding, usually associated with a previous history of puncture wound or retained foreign body. Pyogenic granulomas commonly occur at the end of a digit, involve the nailbed, and present as a pedunculated, red, and raised mass. Numerous vascular channels and endothelial cells are seen on histology. Cauterization with silver nitrate or excision is curative.

Hemangiomas
Hemangiomas are embryonic elements of mesodermal tissues that develop into independently growing blood channels of three types: cavernous, capillary, and mixed. Only 30% are present at birth but 70% to 90% are visible by 4 weeks of age. The first phase of growth is rapid and lasts for 10 to 12 months, during which time the tumor may change color. In the second phase, the lesion grows at the same rate as the child grows with some fading of its color. The third phase is characterized by involution such that by 5 years of age, 50% of hemangiomas have regressed and 70% have regressed by age 7.

Hemangiomas are also classified as involuting (i.e., present at birth, rapidly grows for 4 to 6 months, and disappears by age 7) or noninvoluting, which may be capillary (port wine) or cavernous. Hemangiomas are three times more common in females, which is the basis of a probable hormonal etiology. Pain or fullness is the most common symptom. Cutaneous lesions are reddish and subcutaneous lesions have a bluish hue. A hemangioma appears as a soft tissue mass with calcifications on radiograph. Ultrasonography helps in evaluating size and flow characteristics. CT with contrast shows a homogenous lesion with well-defined borders. MRI assesses its proximity to important structures. With the exception of capillary hemangiomas, noninvoluting lesions should be excised. Muscle or bone may need to be excised in the treatment of infiltrative or intramuscular lesions.

Lymphatic malformations
Lymphatic malformations are wart-like lesions that consist of dilated lymphatic sinuses resulting from a malformation of the lymphatic system.29,30 The malformations are present at birth, grow proportionally with the child, and have the potential to involve the entire upper extremity.29,31,32 Amputation may be required because excision can be difficult. Radiation may aid in curtailing recurrences.

Hemangioendothelioma
Hemangioendotheliomas are rare low-grade malignancies that may be confused with angiosarcomas. Patients may present with a lytic bone lesion. Surgical excision is the treatment.

Vascular malformations
Vascular malformations include arteriovenous (AV) fistulas and aneurysms (which are either congenital or acquired in etiology). Bruits and thrills may be observed on examination. Congenital malformations are classified according to the cell type (arterial, venous, capillary, or lymph) and flow (low or high). A malformation may appear as a calcified phlebolith with new bone formation or as erosion on radiographs. These uncommon lesions appear within the first 2 to 5 years of life and grow proportionately with the child. Disfigurement is the most common presenting complaint.33 Congenital AV malformations are difficult to treat and surgery should be the last resort. Ligation of feeding arteries and communication may be attempted; amputation of the digits or the hand may be necessary. An acquired vascular malformation is seen after penetrating injuries. Acquired vascular malformations cause discomfort and fullness, and may occasionally be associated with distal ischemia. Good results can be expected with single-stage ligation or reconstruction.

True aneurysms
True aneurysms contain all the elements of the arterial wall and are a result of weakness of the wall secondary to blunt trauma or other acquired causes.34-36 They occur most frequently in the ulnar artery in the palm. Treatment is resection with grafting if the artery is required for distal perfusion.

Pseudoaneurysms
Pseudoaneurysms occur secondary to penetrating trauma involving arterial puncture and are more common than true aneurysms. These lesions develop after an arterial hematoma forms into a cavity line with endothelium and collagen and slowly enlarge several weeks after the initial injury. A fibrous wall distinguishes pseudoaneurysms from true aneurysms.35 Treatment for pseudoaneurysms is resection or reconstruction.


Nerve Tumors

Tumors of peripheral nerves represent less than 5% of all tumors of the upper extremity.37 Nerve tumors are slow growing and minimally symptomatic; however, associated pain and neurologic deficit should raise concerns of possible malignancy. Diagnosis is rarely made preoperatively. True tumors of peripheral nerves must be shown to be of neuroectodermal origin. Preoperative evaluation may be aided by high-resolution ultrasonography, CT scanning, and MRI,38-40 but none of these modalities can reliably differentiate benign from malignant lesions.

Neurofibroma
A neurofibroma, the most common benign nerve tumor, is histologically composed of Schwann’s cells, axons, perineural cells, and fibroblasts. Unlike schwannomas, a neurofibroma can have fascicles within the lesion because of its more central location. Neurofibromas exist in three configurations: plexiform, diffuse, and localized. Centrally located within a nerve, the localized neurofibroma may be solitary or multicentric. The diffuse form, believed to arise from nerve endings within the skin and infiltrate the dermis and subcutaneous tissue, is typically found on the dorsum of the hands and fingers and forms plaque-like, ill-defined swellings. The plexiform growth, which is associated with Recklinghausen’s disease, produces an irregular thickening of small cutaneous nerves or larger deep nerves and has a high potential for malignant degeneration.41 For deep plexiform lesions, incisional biopsy is needed to confirm diagnosis. These tumors are more difficult to remove than neurilemomas. Nerve repair or grafting may be necessary when removal of the entire lesion is planned given the difficulty of microdissection. If malignant, en bloc excision of the entire compartment or amputation is necessary.

Schwannoma
A schwannoma usually presents as an asymptomatic round or oval soft tissue mass less than 3 cm in diameter along the course of a peripheral nerve. It is occasionally associated with Recklinghausen’s disease. A schwannoma is well encapsulated, eccentrically located on a nerve, and devoid of fascicles. It is the second most common benign nerve tumor and the most common in the upper extremity. It is frequently misdiagnosed as a ganglion and may have similar consistency. Up to 20% of schwannomas arise in association with the median, ulnar, or radial nerve. Since malignant degeneration and recurrence after surgical excision are rare, surgical management involves only excisional biopsy. Microsurgical removal from the nerve is possible because fascicles do not enter the lesion. It is easily "shelled out" from the surrounding nerve at surgery. The risk for postoperative neurological deficit is approximately 4%, which increases after recurrence or incisional biopsy. Malignancy must be considered if a schwannoma lacks encapsulation or is adherent to the adjacent soft tissue.

Granula cell tumor
Granula cell tumors, previously referred to as granular cell myoblastoma,42 are nontender subcutaneous lesions of schwann cell origin,43,44 75 % of which are solitary. More than 20% present in the upper extremity. Excisional biopsy is adequate surgical management but fascicular dissection may be necessary if the mass is involved with a nerve.

Lipofibromatous hamartoma
A lipofibromatous hamartoma consists of nerve bundles surrounded by adipose and fibrous tissue.45,46 It may be associated with macrodactyly (distal tissue overgrowth). This entity has been referred to as macrodystrophia lipomatosa, infiltrating lipoma of nerve, perineural lipoma, and intraneural lipoma of the median nerve. Usually seen within the median nerve at the wrist and its digital branches, it presents with symptoms of carpal tunnel syndrome or swelling at the wrist. Macrodactyly or distal tissue overgrowth may also be present. Since removal of fibrofatty tissue from between nerve fascicles is difficult and significantly causes risk of nerve injury, treatment usually consists of nerve release.37,47,48 Nerve resection and grafting are reserved for recurrent dysfunctional or painful lesions.49

Neurothekeoma
A neurothekeoma usually presents as a solitary, slow-growing, asymptomatic soft tissue mass less than 1 cm in diameter. Of Schwann or perineural cell origin, its histology shows epithelioid, stellate or spindle-shaped cells arranged in a fascicular pattern or in concentric whirls. Excisional biopsy is usually curative.


Lipoma

Usually occurring in middle-aged women, lipomas are encapsulated fat and are mobile, nontender, and commonly involve the thenar area. Because malignant degeneration is unlikely and recurrence rate is low, local excision is usually sufficient. Partial removal of musculature may be necessary in excision of lipomas that arise from intrinsic muscles.1


Fibrous_Lesions

Nodular fasciitis
Nodular fasciitis is an uncommon soft tissue tumor rarely seen in the hand. It presents with pain and tenderness overlying a firm, fixed subcutaneous mass. Immature fibroblasts with mitotic activity may be seen on histology. The natural history appears to be self-limited with recurrence not usually seen even after incomplete excision. A soft tissue sarcoma must be ruled out.

Infantile digital fibroma
Infantile digital fibroma usually appears before the age of 4 (80% appear before age 1) as a mass on the dorsal surface or side of the ulnar three digits. This tumor almost exclusively occurs in fingers and toes. Intracytoplasmic inclusion bodies may be seen. It is a benign but locally aggressive lesion. Observation is recommended unless deformity or contracture is present. After wide local excision, recurrence is still common (60% cases).

Juvenile aponeurotic fibroma
A juvenile aponeurotic fibroma or calcifying aponeurotic fibroma is a hard mass, which does not cause pain, over the thenar or hypothenar surface that may appear as stippled calcification on radiographs. Recurrence is also common even with wide local excision (in more than 50% cases).

Fibroma of tendon sheath
A fibroma of tendon sheath is a white or tan encapsulated lesion that may be periungal or may arise from a tendon or its sheath. They are painless and slow growing. The recurrence rate is low with excision.

Dermatofibroma
A dermatofibroma involves the skin and possibly deeper tissue. It is elevated, does not cause pain, and shows differentiated fibroblastic tissue and histiocytic cells with foreign body multinucleated giant cells. Recurrence is possible with excision.

Desmoplastic fibroma
Desmoplastic fibroma or desmoids are aggressive fibrous tumors that tend to occur in young men, rarely involving the hand. Since progressive growth with deep extension is common, treatment is wide local excision. Amputation may be required in recurrent cases.1


Keratoacanthoma

Occurring in middle-aged to elderly patients, keratoacanthomas with central umbilication present in the subungal area and on the hair-bearing areas on the dorsum of the hand. These nodular lesions may appear and grow rapidly and can resemble a squamous cell carcinoma or may be its malignant precursor. Hence, long-term follow-up is essential. Abundant keratin with squamous epithelium is present on histology. Treatment by excisional biopsy is usually curative.1


Nevi (Moles)

Nevi are sharply demarcated collections of melanocytes, which are tan to dark brown, and develop early in life, frequently on the dorsum of the hands and feet. They continue to appear throughout the aging process and are characterized by their clinical appearance and level of dermal invasion. Junctional nevi are flat, compound nevi are raised, and blue nevi are smooth and uniform in color. Unlike these lesions, senile lentigo and congenital melanocytic nevi have a significant risk for undergoing malignant degeneration into a melanoma. Bleeding nevi or a change in color or size indicate surgical excision. An atypical nevus (dysplastic nevus), which is large, irregular, and with mixed shades of color, should also be excised.1


Keratoses

Found in areas of sun exposure on the hands or fingers, actinic keratoses and cutaneous horns show hyperkeratosis and atypical-appearing keratinocytes on histology. However, cutaneous horns tend to be thick, firm, and raised, whereas actinic keratoses tend to be scaly and erythematous. Excisional biopsy is usually curative.1


References

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