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Flashcards in Tumor Deck (10):

A 30-year-old woman comes to the office for consultation regarding a 2-year history of a slow-growing mass on the volar aspect of the left ring finger. The growth is adjacent to the distal interphalangeal (DIP) joint. Physical examination shows the mass is firm and cannot be transilluminated. The overlying skin is intact. Which of the following is the most likely diagnosis?
A) Desmoid tumor
B) Ganglion cyst
C) Giant cell tumor
D) Pyogenic granuloma
E) Vascular tumor

C) Giant cell tumor

The mass described in this scenario is a giant cell tumor of the tendon sheath. These growths are common in adults younger than 50 years of age. These masses are slow-growing, firm, lobulated, and painless. Giant cell tumors are the second most common tumor of the hand, after ganglion cysts. Unlike ganglion cysts, giant cell tumors cannot be transilluminated. Moreover, they usually occur on the volar aspect of the hand, and bone invasion is not commonly seen. These characteristics are not true of vascular tumors, desmoids, or pyogenic granulomas.


A 48-year-old right-hand–dominant man comes to the office for evaluation of a tender mass of the distal volar forearm. Tinel sign is positive at the site of the mass. MRI shows that the mass involves the median nerve. Biopsy of the mass confirms a malignant peripheral nerve sheath tumor. On the basis of these findings, this diagnosis is most likely associated with which of the following conditions?
A) Amyotrophic lateral sclerosis
B) Charcot-Marie-Tooth disease
C) Multiple sclerosis
D) Neurofibromatosis 1
E) Parkinson disease

D) Neurofibromatosis 1

The correct response is that neurofibromatosis 1 (NF1) is associated with malignant peripheral nerve sheath tumors (PNSTs).

The majority of “nerve tumors” are PNSTs. Schwannomas and neurofibromas are the most common.

Malignant soft-tissue tumors of the hand are very rare and constitute only 1 to 2% of hand tumors. Malignant PNSTs have a very low annual incidence of 0.001% in the general population, and between 2 and 5% in patients with NF1. The plexiform subtype of neurofibromas poses a substantial risk of malignant degeneration.

Amyotrophic lateral sclerosis, multiple sclerosis, Charcot-Marie-Tooth disease, and Parkinson disease are not commonly associated with malignant PNSTs.


A 25-year-old man is evaluated because of a deep 6-cm, palpable mass to the mid-volar forearm. MRI is performed, but the diagnosis remains inconclusive. An initial diagnostic biopsy is planned. Which of the following is most appropriate during this procedure?
A) Avoidance of cautery
B) Leaving the biopsy site open with compressive dressings
C) Performance of an excisional biopsy
D) Placement of a skin crease transverse incision
E) Use of a tourniquet without Esmarch bandage

E) Use of a tourniquet without Esmarch bandage

An open biopsy is done for forearm tumors that cannot be diagnosed as benign on clinical or radiographic grounds. For a large lesion, an incisional biopsy is performed to minimize the risk of surrounding surgical field tumor contamination. Wide undermining must be avoided.

The biopsy is done with a bloodless field and a tourniquet, but elevate the arm only for exsanguination without an Esmarch bandage. Obtain meticulous hemostasis before wound closure. Sutures are placed close to the wound edges to avoid additional tumor skin contamination.

While a transverse incision may provide a better cosmetic result, it may complicate limb-sparing surgery if pathology determines malignancy.


A 7-year-old girl is brought for evaluation because of a 2-month history of an asymptomatic 1-cm mass over the volar radial aspect of the wrist. The parents note no history of trauma. On physical examination, the mass is not adherent to the skin but affixed to the deeper tissues. She has full range of motion of the wrist without signs of carpal instability. X-ray studies of the wrist show no abnormalities. MRI is consistent with a ganglion. There is no evidence of carpal ligamentous injury. Which of the following is the most appropriate next step in management?
A) Observation
B) Sclerotherapy
C) Closed rupture
D) Wrist arthroscopy
E) Surgical excision

A) Observation

The next step in management is a period of observation. Although there is no general consensus regarding the best treatment for pediatric ganglions, most prefer observation and splinting for asymptomatic wrist ganglions. In the literature, the majority of pediatric wrist ganglions will spontaneously rupture and resolve without intervention. The diagnosis of volar wrist ganglion has been made by history, physical examination, and MRI, making needle biopsy unnecessary. This mass is asymptomatic and there is no sign of carpal ligamentous injury on the x-ray study or MRI, therefore there is no surgical indication for excision or wrist arthroscopy.

Closed rupture is a trauma and antiquated therapy for ganglions. Sclerotherapy does not treat wrist ganglia.


A 48-year-old woman comes for evaluation because of a 6-month history of an increasing mass on the right ring finger. She reports that a similar mass was removed from this location 2 years ago. Medical records show that the mass was solid and of a variegated tan-brown color. The pathology report identified foamy histiocytes and hemosiderin deposits. Physical examination today shows a firm, well-demarcated mass on the dorsal-ulnar aspect of the ring finger proximal phalanx. Skin is not adherent to the mass. A photograph is shown. This patient is at increased risk for which of the following?
A) Compromise of blood flow to the finger
B) Invasion of the underlying bone
C) Local recurrence or extension
D) Metastasis to the liver
E) Spread to the regional lymph nodes

C) Local recurrence or extension

Ganglion cysts are the most common tumor of the hand, but are cystic in character. Giant cell tumor of the tendon sheath (also called localized nodular synovitis, fibrous xanthoma, and pigmented villonodular tenosynovitis) is the second most common tumor, but it is the most common solid tumor affecting the hand. The hemosiderin deposits give the tumor its variable tan-brown appearance.

This tumor is noted to recur locally, particularly if incompletely excised. Giant cell tumors are not known to metastasize either distantly or to regional lymphatics. Whereas giant cell tumor of bone involves the bone itself, giant cell tumor of tendon sheath does not. This tumor is not known to invade or compromise the digital vessels and thus would not compromise blood flow to the digit.


A 55-year-old woman comes to the office because of a 2-year history of a painless mass within the thenar eminence. Physical examination shows a soft, mobile, nontender mass that does not transilluminate. Which of the following is the most likely diagnosis?
A) Epidermal cyst
B) Ganglion
C) Giant cell tumor
D) Glomus tumor
E) Lipoma

E) Lipoma

Lipomas are one of the most common tumors of the body with 10 to 20% occurring in the hand and wrist. Lipomas are most frequently located in the thenar eminence followed by dorsal or volar sides of the digits. The female-to-male ratio is 2:1 and age range is third to sixth decade.

Epidermal cysts have a 2:1 male-to-female ratio with a peak incidence at age 39. They are located in the distal phalanges, usually palmar index and long.

Ganglions account for 50 to 70% of all benign hand tumors with a female-to-male ratio of 3:1. They usually occur in the second to fourth decade with locations being dorsal carpal, volar carpal, volar retinacular, and mucous cyst of the distal interphalangeal joints. Some superficial ganglions will transilluminate.

Benign giant cell tumors of the tendon sheaths have no sex difference, occurring between the fourth and sixth decade and are located in the digits, mostly volar index and long.

Glomus tumors account for only 1 to 5% of hand tumors. There is a 2:1 female-to-male ratio, usually occurring between ages 30 to 50, and the tumors are subungual 50% of the time.


A 48-year-old man is evaluated for reconstruction after resection of a tumor of the distal radius. Physical examination shows a 10-cm defect of the metaphysis and shaft. Which of the following is the most appropriate source of bone for reconstruction?
A) Contralateral fibula
B) Contralateral radius
C) Humeral shaft
D) Medial femoral condyle
E) Osteodistraction of the ipsilateral radius

A) Contralateral fibula

The preferred source of bone for such a long piece of bone reconstruction is the fibula microsurgical vascularized transfer. Another viable option, which was not listed, could be the iliac crest.

Other sources listed would not yield as much bone stock, nor would they offer sufficient bicortical bone to yield a stable reconstruction with rigid fixation, such as:

Contralateral radius (presumably with the radial vascular supply)
Humeral shaft (presumably with the posterior radial collateral vessels)
Medial femoral condyle (based on descending genicular vessels)
Thus, they would not be the preferred source, although they could all be transferred microsurgically.

Osteodistraction would not be the first line of treatment for this defect because of the length of bone transport necessary.


A 3-year-old boy is brought to the office because of a 9 × 8 × 6-cm mass of the left side of the chest. His parents report that the mass has been present for 8 months and has grown rapidly for the past 3 months. MRI confirms that the mass is calcified, lobulated, originates from the fourth and fifth ribs, and encroaches into the chest cavity to lie adjacent to the left ventricle. Other smaller lesions are identified in the scapulae, clavicles, and ribs. CT scan is shown. Examination of the specimen obtained on biopsy of the largest legion shows osteochondroma. Which of the following is the most appropriate management at this time?
A ) Radiation therapy with adjuvant chemotherapy
B ) Radical resection with adjuvant chemotherapy
C ) Wide local excision, reconstruction, and adjuvant radiation therapy
D ) Wide local excision with reconstruction
E ) No intervention at this time

D ) Wide local excision with reconstruction

The lesion in the patient described is a giant calcified osteochondroma; more specifically, it is within the setting of multiple hereditary exostoses, given the multiple smaller lesions in other areas. The resected specimen is seen in the picture shown after wide local excision.

An osteochondroma is a cartilage-covered bony growth, or exostosis, that arises from a surface of a bone – in the scenario described, the fourth and fifth ribs. It is the most common bone tumor in children, may be solitary or multiple, and may arise spontaneously or as a result of previous trauma. An osteochondroma can arise in any bone that develops from endochondral ossification. Multiple osteochondromas may be hereditary with an autosomal dominant pattern (Bessel-Hagen syndrome or hereditary multiple exostoses), as in the scenario described. Malignant transformation of an osteochondroma occurs in 1 to 25% of patients, with the risk being greater in hereditary multiple exostoses.

Numerous complications are associated with osteochondromas, including mechanical effects and deformity, fracture, vascular compromise, neurologic sequelae, and overlying bursa formation. Treatment of solitary lesions should be individualized – patients with small asymptomatic or minimally symptomatic lesions, typical imaging findings, and no functional or mechanical impairment or progressive deformity should be observed regularly for the possibility of spontaneous regression or malignant transformation. Larger, or giant, symptomatic osteochondromas should be treated by wide bony resection and reconstruction of the defect.

There is no role for neoadjuvant or adjuvant chemotherapy or radiation therapy for this lesion.


A 26-year-old man comes to the office after jamming the index finger of his nondominant left hand while playing baseball 2 days ago. He says he has been in pain since the incident occurred. Physical examination shows a shortened, radially deviated left index finger. X-ray study shows a well-defined radiolucent lesion in the diaphysis of the proximal phalanx with a displaced apex-volar fracture. Which of the following is the most appropriate initial treatment?
A ) Amputation of the finger at the metacarpophalangeal (MCP) joint
B ) Closed reduction and percutaneous pin fixation only
C ) Curettage of the mass, bone grafting, and percutaneous pin fixation
D ) Serial x-ray studies every 3 months
E ) Wide resection of the mass and bone grafting

B ) Closed reduction and percutaneous pin fixation only

Enchondromas are benign, cartilaginous lesions that are the most common primary bone tumors arising from the bones in the hand. Approximately 35% of all enchondromas arise in the hand, and enchondromas account for as many as 90% of bone tumors seen in the hand. The proximal phalanx is the most common site of involvement, followed by the metacarpal and middle phalanx. Enchondromas may be diagnosed as an incidental finding on x-ray study. Pathologic fractures may occur. Initial treatment is to stabilize the fractures.

Amputation of the finger is not indicated with this benign lesion. Subsequent operative treatment involves curettage of the lesion with bone grafting and fixation. Small enchondromas found incidentally in x-ray study can be followed with serial x-ray. Wide resection is not indicated given the clinical course of the lesion.


A 36-year-old man comes to the office because of a 2-cm painless mass over the dorsum of the metacarpal of the index finger of the dominant right hand. A ganglion is suspected. The mass is excised with primary closure. Pathologic examination shows a high-grade epithelioid sarcoma with a single positive margin. After staging, which of the following is the most appropriate management?
A ) Aggressive curettage of the positive margin followed by high-dose adjuvant radiation therapy with no further treatment
B ) Amputation at the wrist
C ) High-dose adjuvant radiation therapy with close follow-up of the wound bed
D ) Wide local excision

C ) High-dose adjuvant radiation therapy with close follow-up of the wound bed

The most common subtypes of soft-tissue sarcoma found in the hand include epithelioid sarcoma, synovial sarcoma, and malignant fibrous histiocytoma. It is not uncommon for them to be misdiagnosed initially and treated as benign tumors of the hand.

The mainstay of treatment of extremity sarcomas is wide local excision. Although preoperative and postoperative radiation therapy is often used as an adjunct to wide local excision, adjuvant radiation therapy is not an acceptable replacement for margin-free resection. Curettage is also inadequate to obtain appropriate resection margins and would, in fact, put the patient at risk for seeding of the donor wound bed with malignant cells.

Local control and survival have been shown to be comparable in patients treated with either primary amputation or limb salvage when presenting with sarcomas of the hand. Therefore, primary amputation at the wrist is virtually never indicated.