Part VI • Hand, Wrist, And Upper Extremity

Chapter 96: Benign And Malignant Masses Of The Hand

Questions: 14
Answered: 0/14
Score: 0%
1 2 3 4 5 6 7 8 9 10 11 12 13 14
Question 1 of 14 GANGLION CYSTS
Your resident sees a child with a painless soft-tissue mass on the radial volar aspect of the wrist and diagnoses a ganglion cyst. Which one of the following is correct?
A
Ganglion cysts are the most common benign mass in the upper limb and have an equal sex distribution.
B
A biopsy will generally be required to confirm the diagnosis.
C
Wrist ganglia consistently indicate underlying degenerative change in the joint.
D
The volar wrist is the most common site for ganglia in children.
E
Prompt surgical excision is still recommended to exclude malignancy.
✓ Correct Answer: D. The volar wrist is the most common site for ganglia in children.
Clinical Explanation

The volar wrist is generally thought to be the most common site for ganglion cysts in children. Ganglion cysts are the most common benign mass in the hand and are two or three times more common in females. They are most common in the second to fourth decades. Diagnosis is based on history and clinical examination and may be enhanced by transillumination using a penlight. Radiographs should be obtained to exclude underlying joint pathology or soft-tissue calcification, but these do not usually help diagnose a ganglion. Ultrasonography may be of use when there is doubt about the nature and extent of a probable ganglion cyst and a solid rather than cystic mass may require biopsy or excision to exclude malignancy. Two-thirds of ganglia in children are reported to resolve spontaneously,1 but persistent lesions or those associated with pain or reduced function may require treatment. Aspiration may be sufficient, but patients should be warned there is a 50% recurrence rate. The addition of hyaluronidase or steroid does not offer any additional advantage and the recurrence rate following surgical excision in children is particularly low at 5%2 compared to 25% on average in adults.3 This may reflect the greater likelihood of underlying degenerative changes in adults.

References:
  • 1. Rosson JW, Walker G. The natural history of ganglia in children. J Bone Joint Surg Br 1989;71(4):707–708
  • 2. Simon Cypel TK, Mrad A, Somers G, Zuker RM. Ganglion cyst in children: reviewing treatment and recurrence rates. Can
  • J Plast Surg 2011;19(2):53–55
Question 2 of 14 GANGLION CYSTS OF THE WRIST
Which one of the following ligaments is most commonly associated with symptomatic ganglion cysts in adults?
A
Radiolunate
B
Scapholunate
C
Radiocapitate
D
Scaphotriquetral
E
Radioscaphoid
✓ Correct Answer: B. Scapholunate
Clinical Explanation

Dorsal wrist ganglia are the most common symptomatic ganglia in adults and are most frequently identified between the third and fourth extensor compartments. They are most often associated with the scapholunate ligament, which is a key structure in providing carpal stability. Volar wrist ganglion cysts are the most common asymptomatic wrist ganglia in adults and tend to occur between flexor carpi radialis (FCR) and the radial artery. They can arise from the FCR tendon sheath, radiocarpal or scaphotrapezial joints. Many ganglia, such as mucous cysts at the distal interphalangeal (DIP) joint, flexor sheath ganglia and some dorsal wrist ganglia, can comfortably be excised under local anesthesia. Volar wrist ganglia are more commonly excised under regional block or general anesthesia with a tourniquet to aid careful dissection around the radial artery.1–3

References:
  • 1. Nahra ME, Bucchieri JS. Ganglion cysts and other tumor related conditions of the hand and wrist. Hand Clin
  • 2004;20(3):249–260, v
  • 2. Lowden CM, Attiah M, Garvin G, Macdermid JC, Osman S, Faber KJ. The prevalence of wrist ganglia in an asymptomatic
  • population: magnetic resonance evaluation. J Hand Surg [Br] 2005;30(3):302–306
  • 3. Zhang A, Falkowski AL, Jacobson JA, Kim SM, Koh SH, Gaetke-Udager K. Sonography of wrist ganglion cysts: Which
  • location is most common? J Ultrasound Med 2019;38(8):2155–2160
  • GIANT CELL TUMORS OF THE TENDON SHEATH
  • 3. A patient is referred with a suspected giant cell tumor of the tendon sheath. Which one of the following
  • statements is correct?
  • D. Previous injury may be a predisposing factor.
  • Giant cell tumors of the tendon sheath are also called pigmented villonodular synovitis. They are yellow-brown–
  • colored subcutaneous masses that develop spontaneously and may be a reaction to injury. They are most common
  • between the third and fifth decades of life and may be clinically confused with ganglion cysts. The radial three
  • digits are most frequently affected, although other sites can be involved. Continued growth of the tumor will
  • usually interfere with hand function, particularly with range of motion. Treatment with marginal excision
  • is advocated to preserve function, but recurrence rates are high because of close margins or satellite lesions.
  • Adjunctive radiotherapy is sometimes used to treat recurrent tumors or following incomplete surgical excision.1–3
  • 1. Adams EL, Yoder EM, Kasdan ML. Giant cell tumor of the tendon sheath: experience with 65 cases. Eplasty 2012;12:e50
  • 2. Garg B, Kotwal PP. Giant cell tumour of the tendon sheath of the hand. J Orthop Surg (Hong Kong) 2011;19(2):218–220
  • 3. Errani C, Ruggieri P, Asenzio MA, et al. Giant cell tumor of the extremity: a review of 349 cases from a single institution.
  • Cancer Treat Rev 2010;36(1):1–7
Question 3 of 14 GIANT CELL TUMORS OF THE TENDON SHEATH
A patient is referred with a suspected giant cell tumor of the tendon sheath. Which one of the following statements is correct?
A
It is most likely to affect the little finger.
B
The patient is most likely to be younger than 20 years.
C
Hand function is unlikely to be affected.
D
Previous injury may be a predisposing factor.
E
Recurrence after surgical excision is unlikely.
✓ Correct Answer: D. Previous injury may be a predisposing factor.
Clinical Explanation

3. Head L, Gencarelli JR, Allen M, Boyd KU. Wrist ganglion treatment: systematic review and meta-analysis. J Hand Surg Am 2015;40(3):546–53.e8

Question 4 of 14 FINGERTIP LESIONS
A 28-year-old hairdresser presents with a nontender 5-mm soft-tissue mass in the right middle finger pulp. What is the most likely diagnosis?
A
Giant cell tumor
B
Epidermal inclusion cyst
C
Glomus tumor
D
Pyogenic granuloma
E
Melanoma
✓ Correct Answer: B. Epidermal inclusion cyst
Clinical Explanation

Epidermal inclusion cysts are commonly seen in the palms and fingertips of patients with occupations that predispose them to penetrating injuries, as in this scenario. The penetrating injury causes implantation of epidermal cells into the dermis. This can also occur secondary to surgery at any site if epidermal implantation occurs. For example, leaving epidermal tissue in the wound or dragging it in with sutures. Examination of a patient with an inclusion cyst will typically show evidence of a firm, spherical, painless mass with fixity to skin. Treatment is achieved with complete surgical excision to minimize recurrence.1 A giant cell tumor could also present as a nontender nodule, but these tend to be more proximally located in the digit rather than at the pulp. A glomus tumor is a benign tumor of the arteriovenous anastomosis involved in the regulation of cutaneous circulation in the distal part of the digit. Discoloration (often red/blue tinged) in the presence of exquisite tenderness over the finger or thumb nail is a strong indication for the presence of a glomus tumor. A pyogenic granuloma is another vascular tumor that can occur at the site of minor trauma. They usually present with a friable nodule that bleeds easily. They may be amenable to cauterization or excisional surgery. Melanoma can affect the digits and would tend to be observed as a cutaneous lesion or pigmented area within the nail rather than a mass in the pulp. Pigmentation of the nail is therefore concerning in the absence of trauma and may warrant urgent nail-bed biopsy to exclude a subungual melanoma.2,3

References:
  • 1. Lucas GL. Epidermoid inclusion cysts of the hand. J South Orthop Assoc 1999;8(3):188–192
  • 2. Athanasian EA. Bone and soft tissue tumors. In: Green DP, Hotchkiss RN, Pederson WC, eds. Green’s Operative Hand
  • Surgery. 4th ed. Philadelphia, PA: Churchill Livingstone; 1998
  • 3. Fleegler EJ. Skin tumors. In: Green DP, Hotchkiss RN, Pederson WC, eds. Green’s Operative Hand Surgery. 4th ed. Phila-
  • delphia, PA: Churchill Livingstone; 1998
Question 5 of 14 FINGERTIP LESIONS
A 78-year-old retired schoolteacher presents with a tender soft-tissue swelling between the dorsal nailfold and DIP joint of the right ring finger. It has developed over the past 12 months. She has a groove in the nail plate. What is the most likely diagnosis?
A
Neurofibroma
B
Enchondroma
C
Basal cell carcinoma (BCC)
D
Mucous cyst
E
Melanoma
✓ Correct Answer: D. Mucous cyst
Clinical Explanation

Mucous cysts are small ganglia that originate from the DIP joint and are associated with osteoarthritis. They may be associated with nail grooving and osteophytes. Treatment is with surgical excision of the cyst and adjacent osteophytes. This is usually performed under local anesthetic and some cases may need excision of skin and a graft or local advancement flap (such as a hatchet based on the middle phalanx). Occasionally joint fusion is required if the mucus cysts are recurrent and bothersome. Neurofibromas are common nerve tumors that can proliferate within and along nerve fibres. They can therefore be associated with functional abnormality such as paresthesia or pain, although for the most part they appear as benign, nodular soft-tissue growths within the skin on a background of neurofibromatosis. They may need surgical excision depending on site, size, and symptoms experienced by the patient. Enchondromas are benign bone tumors seen in the hand, most often in patients in their third or fourth decade of life. They are well-defined lytic lesions with relative lack of normal bone replaced by aberrant cartilage that can lead to weakness of the bone. Hence, most enchondromas are diagnosed when patients present with pathological fractures or as an incidental finding on X-ray for another reason. Treatment involves curettage and replacing the defect with bone graft or bone graft substitute. This is normally performed in the elective setting once any fractures have healed. BCCs are slow-growing malignant skin tumors that are relatively common on the hand, but less common on the individual digits themselves. They tend to occur in the age group as in this scenario (i.e., age 60 onward) but are slow growing with typical features including a pearly appearance, pink/red discoloration, telangiectasia, and ulceration. Excision of BCCs on the hand may require reconstruction with a full-thickness skin graft or local flap. Melanomas are malignant tumors of the skin that are relatively uncommon in the hand. When they do occur on the hand, volar or subungual locations are most common. Acral lentiginous, nodular, and superficial spreading are common subtypes seen. Wide excision may necessitate digital amputation at the next most proximal joint.1,2

References:
  • 1. Athanasian EA. Bone and soft tissue tumors. In: Green DP, Hotchkiss RN, Pederson WC, eds. Green’s Operative Hand
  • Surgery. 4th ed. Philadelphia, PA: Churchill Livingstone; 1998
  • 2. Fleegler EJ. Skin tumors. In: Green DP, Hotchkiss RN, Pederson WC, eds. Green’s Operative Hand Surgery. 4th ed. Phila-
  • delphia, PA: Churchill Livingstone; 1998
Question 6 of 14 TUMORS OF THE HAND AND WRIST
A 43-year-old right-hand–dominant carpenter presents with a small soft-tissue swelling over the volar aspect of the right wrist that has developed over 8 months. He complains of altered sensation in the little finger and pain in the ulnar digits while working, especially in cold weather. What is the most likely diagnosis?
A
Carpal boss
B
Ulnar artery aneurysm
C
Ganglion cyst
D
Chondrosarcoma
✓ Correct Answer: B. Ulnar artery aneurysm
Clinical Explanation

This patient has symptoms of ulnar nerve compression close to Guyon’s canal. This can occur as a result of lesions such as a lipoma or a ganglion cyst, although the latter tends to occur on the radial rather than the ulnar aspect. Furthermore, pain and cold sensitivity on working would fit with an ischemic component to the compression. Palpation of a pulsatile mass would strongly support the diagnosis of an aneurysm, especially in the setting of emboli and digital ischemic changes. This condition is commonly seen in manual laborers, such as builders and carpenters, where it is termed “hypothenar hammer syndrome.” It results from repetitive trauma to the ulnar artery at the wrist. Treatment involves resection, ligation, or interposition vein grafting.1 Osteoid osteomas are uncommon, benign tumors that typically affect the distal phalanx in young adult patients. They are particularly associated with night pain and this is often relieved by nonsteroidal anti-inflammatory drug use. Chondrosarcoma is a malignant tumor that can present with a mass (often close to the metacarpophalangeal joint) that is usually painful and tends to occur in older individuals. Imaging is important in further characterizing such bony lesions. Carpal bosses are often confused with ganglion cysts on the dorsal wrist, but some key differences are that bosses tend to be painful, do not fluctuate in size, are generally firmer and noncompressible, and are sited distal to the carpus over the metacarpal bases.2,3

References:
  • 1. Ferris BL, Taylor LM Jr, Oyama K, et al. Hypothenar hammer syndrome: proposed etiology. J Vasc Surg 2000;31(1, Pt
  • 1):104–113
  • 2. Athanasian EA. Bone and soft tissue tumors. In: Green DP, Hotchkiss RN, Pederson WC, eds. Green’s Operative Hand
  • Surgery. 4th ed. Philadelphia, PA: Churchill Livingstone; 1998
  • 3. Goiney C, Porrino J, Richardson ML, Mulcahy H, Chew FS. Characterization and epidemiology of the carpal boss utilizing
  • computed tomography. J Wrist Surg 2017;6(1):22–32
Question 7 of 14 GLOMUS TUMORS
A patient is referred with a suspected glomus tumor of the hand. Which one of the following is true?
A
It is the most commonly occurring benign hand tumor.
B
It carries a moderate risk of malignant transformation.
C
The main symptom is altered sensation in the affected digit.
D
Use of a tourniquet in clinic can help aid clinical diagnosis.
E
Radiographic imaging is key to confirming the diagnosis.
✓ Correct Answer: D. Use of a tourniquet in clinic can help aid clinical diagnosis.
Clinical Explanation

Glomus tumors are benign vascular tumors of the fingertip and are reasonably common but still represent less than 5% of all hand tumors. The key triad of symptoms are: 1. Stabbing pain in the fingertip 2. Pinpoint tenderness 3. Cold sensitivity They tend to occur most commonly in females and almost never undergo malignant change. Assessment of patients with suspected glomus tumors would normally show discoloration of the fingertip and pain on pinpoint pressure of the affected region of the finger tip. This is termed “Love’s maneuver.” The use of a tourniquet can be useful because inflation can mitigate the pain caused by this, with recurrence of the pain on deflation. This is termed “Hildreth’s sign.” Plain radiographs are not especially useful in identifying this condition, but are useful to rule out other diagnoses. Ultrasound with high-frequency transducer or magnetic resonance imaging (MRI) can be helpful. Treatment of glomus tumors involves complete excision through the sterile matrix. Recurrence rates are low provided complete excision is achieved.1,2

References:
  • 1. Lee W, Kwon SB, Cho SH, Eo SR, Kwon C. Glomus tumor of the hand. Arch Plast Surg 2015;42(3):295–301
  • 2. Morey VM, Garg B, Kotwal PP. Glomus tumours of the hand: review of literature. J Clin Orthop Trauma 2016;7(4):286–291
Question 8 of 14 SIGNS OF SOFT-TISSUE SARCOMA
You are working in the sarcoma clinic, and a 34-year-old patient is referred to you with a soft-tissue mass in the volar compartment of the proximal forearm. It has been present for 16 months, measures 4 cm in diameter, is fixed by resisted elbow flexion, and is nontender. Which one of the following represents the most significant concern regarding this lesion?
A
The size
B
The lack of pain
C
The duration of symptoms
D
The depth of the mass
E
The patient’s age
✓ Correct Answer: D. The depth of the mass
Clinical Explanation

This may well be a benign mass such as a lipoma; however, the most concerning feature of this lesion is the tissue depth. Because it is fixed on elbow flexion, this suggests it involves the volar muscle compartment. Being deep to the fascia is one of the key criteria of concern for soft-tissue sarcomas. The other red flags are size greater than 5 cm, pain, and rapid growth. The more of these features that coexist, the more likely it is that malignancy is present. In general, the single most important concern is rapid growth of the lesion (i.e., weeks to months).1–3

References:
  • 1. Grimer R, Judson I, Peake D, Seddon B. Guidelines for the management of soft tissue sarcomas. Sarcoma
  • 2010;2010:506182
  • 2. Stomeo D, Tulli A, Ziranu A, Mariotti F, Maccauro G. Chondrosarcoma of the hand: a literature review. J Cancer Ther
  • 2014;5:403–409
  • 3. Henderson M, Neumeister MW, Bueno RA Jr. Hand tumors: II. Benign and malignant bone tumors of the hand. Plast
  • Reconstr Surg 2014;133(6):814e–821e
Question 9 of 14 DIAGNOSING SOFT-TISSUE SARCOMA
A young adult patient is referred to the clinic with a deep, rapidly enlarging, painful soft-tissue mass in the left hand. Which one of the following is the preferred method for obtaining an initial tissue diagnosis in this case?
A
Fine-needle aspiration cytology
B
Incisional biopsy
C
Excisional biopsy
D
Core needle biopsy
E
Marginal excision
✓ Correct Answer: D. Core needle biopsy
Clinical Explanation

Patients with suspected soft-tissue sarcomas should undergo triple assessment in a specialist clinic. This involves a history, examination, imaging, and biopsy. Where timing allows, it can be helpful to obtain imaging before biopsy to preserve soft-tissue appearances (e.g., on MRI), or imaging may be used to guide biopsies of deeper lesions. The recommended approach to obtaining a tissue biopsy is to take several core biopsies, because this maximizes diagnostic yield.1 If the lesion is superficial or small, then incisional or excisional biopsies may occasionally be considered. When a biopsy is obtained, it must be planned in such a way that the biopsy tract can be completely removed en bloc during the subsequent tumor excision; hence, biopsies are generally better performed by the treating surgical team. Fine-needle aspiration cytology is not recommended as a primary diagnostic modality, although it may be useful in confirming disease recurrence. Furthermore, it only provides cells for analysis, rather than formal tissue subtyping.1,2

References:
  • 1. Grimer R, Judson I, Peake D, Seddon B. Guidelines for the management of soft tissue sarcomas. Sarcoma
  • 2010;2010:506182
  • 2. Henderson M, Neumeister MW, Bueno RA Jr. Hand tumors: II. Benign and malignant bone tumors of the hand. Plast
  • Reconstr Surg 2014;133(6):814e–821e
Question 10 of 14 IMAGING IN SOFT-TISSUE SARCOMA
A patient is referred to the clinic with a suspicious soft-tissue mass in the right upper limb. It has doubled in size over the past month. Which one of the following is the best imaging modality to use for this patient?
A
High-resolution CT
B
Isotope bone scan
C
MRI
D
Ultrasound
E
PET scan
✓ Correct Answer: C. MRI
Clinical Explanation

An MRI scan is the preferred imaging modality for patients with suspected soft-tissue sarcoma, both for identifying the nature and extent of the lesion and to aid surgical planning. MRI avoids high-dose radiation and provides clear images to help assess soft-tissue and bony changes. That said, it is usual to begin with plain radiographs in the assessment of most soft tissue or bone abnormality of the hand and this will often provide some useful initial information regarding the underlying diagnosis. CT may also be useful especially for the assessment of the bone. Ultrasound is a useful modality for soft-tissue lesions, as it is low cost, noninvasive, and yet can provide valuable information on the characteristics of the mass. It can provide information on whether, for example, the lesion is a lipoma versus a liposarcoma. It also avoids a radiation dose. Once the diagnosis of sarcoma has been confirmed histologically, a high-resolution staging CT scan should be performed to exclude metastatic spread. Isotope bone scans and PET scans are not currently recommended as routine diagnostic or staging investigations for soft-tissue sarcomas.1–3

References:
  • 1. Grimer R, Judson I, Peake D, Seddon B. Guidelines for the management of soft tissue sarcomas. Sarcoma
  • 2010;2010:506182
  • 2. Sobanko JF, Dagum AB, Davis IC, Kriegel DA. Soft tissue tumors of the hand. 2. Malignant. Dermatol Surg 2007;33(7):771–
  • 3. Patel DB, Matcuk GR Jr. Imaging of soft tissue sarcomas. Linchuang Zhongliuxue Zazhi 2018;7(4):35
Question 11 of 14 SARCOMA SUBTYPES
Which one of the following describes a soft-tissue sarcoma seen in patients with immune compromise presenting with dark blue to violaceous firm macules and plaques?
A
Kaposi’s sarcoma
B
Epithelioid sarcoma
C
Clear cell sarcoma
D
Fibrosarcoma
E
Synovial sarcoma
✓ Correct Answer: A. Kaposi’s sarcoma
Clinical Explanation

There are a number of different types of sarcoma. These include epithelioid, clear cell, fibrosarcoma, synovia, and Kaposi’s. Kaposi’s sarcoma is a sarcoma subtype which can be aggressive and is seen in immunocompromised patients such as those with AIDS or human herpes virus 8. It tends to present in the fourth or fifth decade of life with a male-to-female preponderance of 15:1. Clinical presentation involves dark blue to violaceous firm macules, which become replaced by infiltrative plaques measuring up to 3 cm in diameter. Treatment includes control of HIV load where applicable, radiotherapy, and chemotherapy. Kaposi’s sarcoma cannot be cured but can usually be controlled. Epithelioid sarcoma is the most common sarcoma subtype occurring in the hand and is insidious, slow growing, and typically originates on the volar surface of the palm or digits. It is treated with wide resection margins. Clear cell sarcoma is rare. It is a slow-growing tumor with a poor prognosis and tends to be a deep mass attached to tendons and aponeuroses or fascia. Fibrosarcoma can occur in the deep subcutaneous space, the facial septa, or muscle. It is insidious in onset and metastasizes by hematogenous spread. Synovial sarcoma originates in soft tissues close to joints (tendon, tendon sheath, bursa). This also has a poor prognosis and a high incidence of metastases. Treatment involves surgery and radiotherapy or chemotherapy.1–3

References:
  • 1. Schneider JW, Dittmer DP. Diagnosis and treatment of Kaposi sarcoma. Am J Clin Dermatol 2017;18(4):529–539
  • 2. Sobanko JF, Meijer L, Nigra TP. Epithelioid sarcoma: a review and update. J Clin Aesthet Dermatol 2009;2(5):49–54
  • 3. Cormier JN, Pollock RE. Soft tissue sarcomas. CA Cancer J Clin 2004;54(2):94–109
Question 12 of 14 MANAGEMENT OF SOFT-TISSUE SARCOMAS
When considering the treatment principles for sarcoma of the hand, which one of the following is correct?
A
Wide surgical excision is performed with 1- to 2-cm margins.
B
Surgical excision is unlikely to require amputation.
C
Any diagnostic biopsy tract must be excised en bloc with the resection.
D
All patients will require postsurgical radiotherapy (adjuvant).
E
All patients are likely to need systemic chemotherapy postsurgery.
✓ Correct Answer: B. Surgical excision is unlikely to require amputation.
Clinical Explanation

The treatment principles of sarcoma are wide resection with margins typically of 2 to 3 cm of normal tissue, or as an anatomical compartment, and the diagnostic biopsy tract must be excised en bloc with the main resection specimen. This means that in most centers, the biopsy and resection surgery are performed by the same team such that the placement of the core biopsy scar is kept within the planned confines of the final resection margins. Using margins of 2 to 3 cm in the hand means that the risk of needing amputation is high. However, limb- preserving surgery and reconstruction with free tissue transfer can be utilized. Local recurrence is rare provided adequate margins are achieved in the first instance. Many, but not all, patients will need adjuvant radiotherapy after sarcoma surgery. Chemotherapy is not generally indicated, although it depends on the tumor subtype. Adjuvant treatment is guided by the subtype of tumor, patient’s overall health, and the surgical resection margins achieved.1–3

References:
  • 1. Grimer R, Judson I, Peake D, Seddon B. Guidelines for the management of soft tissue sarcomas. Sarcoma
  • 2010;2010:506182
  • 2. Brien EW, Terek RM, Geer RJ, Caldwell G, Brennan MF, Healey JH. Treatment of soft-tissue sarcomas of the hand. J Bone
  • Joint Surg Am 1995;77(4):564–571
  • 3. Nicholson S, Milner RH, Ragbir M. Soft tissue sarcoma of the hand and wrist: epidemiology and management challenges.
  • J Hand Microsurg 2018;10(2):86–92
Question 13 of 14 BENIGN BONE LESIONS OF THE HAND
A 26-year-old woman presents with a fracture of the proximal phalanx of the left ring finger sustained while washing dishes. She is otherwise healthy. A plain radiograph shows a well-demarcated, round, lytic lesion with some areas of calcification within the proximal phalanx. What is the most likely diagnosis?
A
Nora’s lesion
B
Periosteal chondroma
C
Enchondroma
D
Maffucci’s syndrome
E
Ollier’s disease
✓ Correct Answer: C. Enchondroma
Clinical Explanation

The presence of a pathologic fracture and this radiologic appearance is suggestive of an enchondroma. Enchondromas are benign bone tumors that most often occur in the third of fourth decade of life. They result from an aberrant focus of cartilage in the metacarpals or phalanges. Patients often present with a pathological fracture at the enchondroma site and radiographs show a well-defined lytic lesion with stippled calcification. The presence of multiple enchondromas is called Ollier’s disease, and, when combined with vascular malformations, is termed Maffucci’s syndrome. Nora’s lesion was described by Nora and is also known as bizarre parosteal osteochondromatous proliferation.1 It is a rare lesion affecting the tubular bones of the hand (proximal and middle phalanges and metacarpals). It is thought to be a reactive lesion and develops as an exophytic outgrowth from the cortical surface. It contains bone, cartilage, and fibrous tissue. Typical presentation involves development of an obvious tender mass over a few months. Treatment involves excision using a high-speed burr (saucerization). Periosteal chondromas are also rare and represent surface lesions arising from the periosteum of the tubular bones. They typically present as painful but slow-growing masses.2,3

References:
  • 1. Nora FE, Dahlin DC, Beabout JW. Bizarre parosteal osteochondromatous proliferations of the hands and feet. Am J Surg
  • Pathol 1983;7(3):245–250
  • 2. Feldman F. Primary bone tumors of the hand and carpus. Hand Clin 1987;3(2):269–289
  • 3. Henderson M, Neumeister MW, Bueno RA Jr. Hand tumors: II. Benign and malignant bone tumors of the hand. Plast
  • Reconstr Surg 2014;133(6):814e–821e
Question 14 of 14 MALIGNANT BONE TUMORS OF THE HAND
Which one of the following is the most common primary malignant bone tumor in the hand and tends to occur in middle age patients?
A
Ewing’s sarcoma
B
Chondrosarcoma
C
Osteosarcoma
D
Malignant giant cell tumor
E
Osteoblastoma
✓ Correct Answer: B. Chondrosarcoma
Clinical Explanation

Chondrosarcoma is the most common primary malignant bone tumor in the hand and may develop de novo or within a benign lesion such as an osteochondroma. Chondrosarcomas represent about one-fifth of the total malignant bone tumors, but of course are not solely restricted to the hand. Overall, this is still a rare disease (1/1000,000) and tends to present in middle age (40–70). This is one area in which it differs from other bone tumors such as Ewing’s sarcoma and osteosarcoma which both occur most often in adolescents and young adults. Osteosarcoma can occur in the hand in middle-aged patients but is extremely rare. Early chondrosarcomas tend not to metastasize, and may be responsive to nonsurgical treatment. However, advanced stage tumors are not only more likely to metastasize more readily, they are more resistant to both chemo and radiation therapies. The most common site of metastasis is the lung, hence the reason for regular chest radiographs in these sarcoma patients. Chondrosarcoma of the hand usually occurs in the epiphyseal regions of the phalanges or metacarpals and presents as a destructive bony lesion which is often slow growing. However, the most common anatomic sites are the femur, humerus, and pelvis. Treatment for any site is wide surgical resection. Radiotherapy is sometimes indicated in either the adjunctive setting where margins are close, or in the palliative setting.1–4 Ewing’s sarcoma, osteosarcoma, and malignant giant cell tumors are less common in the hand. Ewing’s sarcoma is an aggressive subtype of sarcoma that occurs in adolescents and young adults. It most commonly affects the femur, although it was first described by Ewing in 1921 within the radius of a 14-year-old girl.5 Osteosarcoma management differs from that of Ewing’s and chondrosarcoma in that it tends to be very responsive to chemotherapy. Therefore, management usually involves neoadjuvant chemotherapy then surgery and further chemotherapy. Osteoblastoma is usually considered benign, although an aggressive variant has been described.1,2

References:
  • 1. Stomeo D, Tulli A, Ziranu A, Mariotti F, Maccauro G. Chondrosarcoma of the hand: a literature review. J Cancer Ther
  • 2014;5:403–409
  • 2. Sobanko JF, Dagum AB, Davis IC, Kriegel DA. Soft tissue tumors of the hand. 2. Malignant. Dermatol Surg 2007;33(7):771–
  • 3. Boehme KA, Schleicher SB, Traub F, Rolauffs B. Chondrosarcoma: a rare misfortune in aging human cartilage? The
  • role of stem and progenitor cells in proliferation, malignant degeneration and therapeutic resistance. Int J Mol Sci
  • 2018;19(1):311
  • 4. Riedel RF, Larrier N, Dodd L, Kirsch D, Martinez S, Brigman BE. The clinical management of chondrosarcoma. Curr Treat
  • Options Oncol 2009;10(1-2):94–106
  • 5. Ewing J. Diffuse endothelioma of bone. Proc NY Path Soc 1921;21:17–24

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