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Introduction
  • A rare malignant slow-growing, nodular, soft tissue tumor
    • often mistaken for a benign granulomatous process.
  • Age & location
    • occurs in adolescents and young adults (ages 10-35 years)
    • 2:1 male to female ratio
    • most common soft tissue sarcoma of the hand and wrist
      • also occurs in forearm, buttock/thigh, knee, and foot
    • mass is deep or superficial
      • may ulcerate and mimic skin carcinoma, rheumatoid nodule, or granuloma when superficial
      • may be attached to tendons, tendon sheaths, or fascia when deep
  • Genetics
    • unknown
  • Malignancy
    • regional lymph node metastases common
    • systemic metastasis to lungs can occur
  • Prognosis
    • extremely poor
Symptoms
  • Symptoms
    • small, firm, painless, slow growing mass commonly occurring in the upper extremity
  • Physical exam
    • 3-6 cm firm, painless mass
      • may have skin ulceration
Imaging
  • Radiographs
    • calcification occurs within the lesion in 10-20%
    • erosion of adjacent bone is sometimes found
  • MRI
    • indeterminate in appearance
      • dark on T1
      • bright on T2
    • tendon sheath nodule may be visualized
Histology
  • Characteristics post 
    • nodular pattern with central necrosis within granulomatous areas
    • epithelial appearance with ovoid or polygonal cells with eosinophilic cytoplasm
    • dense, hyalinized collagen deposits intercellularly
    • cellular pleomorphism is rare
    • keratin-positive staining
Treatment
  • Operative
    • wide excision with adjuvant radiotherapy
      • indication
        • all operable tumors
      • technique
        • perform sentinel node biopsy to evaluate for regional lymph node metastasis
      • outcomes
        • high rate of multiple recurrences if mistaken for a benign lesion and inadequately excised
    • amputation
      • indications
        • may be necessary to prevent spread of disease in cases of multiple recurrences
 

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Questions (3)

(SBQ05.1) A 25-year-old man presents with a nodular mass on the palm of his hand that has been slowly enlarging over the past six months. The patient denies a history of injury, arthralgias, fevers, and weight loss. On examination, the mass is firm with minimal tenderness to palpation and no erythema. A clinical photograph and biopsy specimen are shown in Figures A and B. The lesion depicted here has a propensity to metastasize to what location? Review Topic

QID:1986
FIGURES:
1

Brain

5%

(10/199)

2

Bone

10%

(19/199)

3

Lung

44%

(88/199)

4

Lymph nodes

40%

(79/199)

5

Thyroid

1%

(2/199)

Select Answer to see Preferred Response

PREFERRED RESPONSE 4

Figure A reveals a small, nodular, slow-growing mass on the hand characteristic of epithelioid sarcoma. The histology depicted in Figure B confirms the diagnosis of epithelioid sarcoma given the epithelial appearance and nodular pattern with central necrosis. Epithelioid sarcoma is the most common soft tissue sarcoma of the hand and frequently metastasizes to regional lymph nodes. Epithelioid sarcoma is one of five high-grade, histologic sarcoma subtypes with a propensity for regional lymph node metastatic spread. The other subtypes include rhabdomyosarcoma, clear cell sarcoma, synovial sarcoma, and vascular sarcomas. Sentinel lymph node biopsy may be indicated for disease staging and in determining prognosis.

Andreou and Tunn, in their review of soft tissue sarcomas, present data on the application of sentinel node biopsy in patients with histologic sarcoma subtypes associated with a higher propensity for regional lymph node metastasis. Currently, there is no validated, noninvasive method to assess regional lymph node status. The authors suggest that lymphatic mapping with sentinel lymph node biopsy may prove to be a useful adjunct in the staging of soft tissue sarcomas.

Blazer et al., in a review on the management of sarcoma, present an argument for the selective use of sentinel node biopsy in the management of sarcomas with a propensity for regional lymph node metastasis. They suggest that the evaluation of the regional lymph node basin by lymphatic mapping and sentinel node biopsy may be an excellent strategy for selecting patients who might benefit from lymphadenectomy or early systemic therapy.

Maduekwe et al. performed a retrospective review of 29 patients with surgically resectable synovial, epithelioid, or clear cell sarcomas who underwent sentinel lymph node biopsy to identify occult lymph node metastasis. They found that only one patient had a positive sentinel node with micrometastatic disease at the time of the initial pathological examination. This finding suggests that the incidence of positive sentinel lymph node biopsy after complete radiological evaluation is lower than previously reported. They recommend that sentinel nodes should be subjected to more intense methods of analysis including serial step-sectioning and H&E and immunohistochemical staining to increase detection rates for micrometastases.

Incorrect Answers:
Answers 1, 2, 3, and 5: These locations are not common sites for epithelioid sarcoma metastasis.


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(SBQ05.2) A 25-year-old right hand dominant male presents for a second opinion with the painless mass seen in Figure A. He denies recent fevers, chills, or any other systemic symptoms. An incisional biopsy is performed and a histologic specimen of the lesion is seen in Figure B. Which of the following is the most likely diagnosis? Review Topic

QID:1987
FIGURES:
1

Rheumatoid nodule

5%

(125/2293)

2

Ganglion cyst

5%

(106/2293)

3

Epithelioid sarcoma

78%

(1781/2293)

4

Squamous cell carcinoma

10%

(224/2293)

5

Chondrosarcoma

2%

(45/2293)

Select Answer to see Preferred Response

PREFERRED RESPONSE 3

The clinical photograph and histologic section are consistent with epithelioid sarcoma.

Epithelioid sarcoma is the most common soft tissue sarcoma of the hand and frequently metastasizes to regional lymph nodes. The other sarcomas with nodal metastasis include rhabdomyosarcoma, clear cell sarcoma, synovial sarcoma, and vascular sarcomas. Sentinel lymph node biopsy may be indicated for disease staging and in determining prognosis.

Pradhan et al. describe the management of 63 patients treated for soft-tissue sarcoma of the hand. Clear cell sarcoma (17.4%), epitheloid sarcoma (17.4%), and synovial sarcoma (16%) were the most common types of tumors encountered. Radiotherapy use did not affect local recurrence rates in the setting of an inadequate surgical margin.

McPhee et al. describe the evaluation and treatment of 24 patients diagnosed with primary soft tissue sarcoma of the hand. Malignant fibrous histiocytoma (37.5%) and epithelioid sarcoma (25%) were the most common diagnoses and the authors found tumor grade and surgical margin most affected local recurrence and survival.

Figure A reveals a small, nodular, slow-growing mass on the hand characteristic of epithelioid sarcoma. The histology depicted in Figure B confirms the diagnosis of epithelioid sarcoma given the epithelial appearance and nodular pattern with central necrosis.

Incorrect Answers:

Answer 1: Rheumatoid nodule - while the clinical finding (firm nodule) overlaps, this is a granulomatous lesion with inflammatory, non-atypical cells
Answer 2: Ganglion cyst - this should be adjacent to a joint and composed of mucinous fluid with thin, bland capsule
Answer 4: Squamous cell carcinoma - again, clinical appearance could overlap somewhat, although SCC should be a little less firm, but the classic appearance of is pavement-type epithelial cells with deposits of keratin ("pearls")
Answer 5: Chondrosarcoma - this is a bone tumor


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(SBQ05.3) Figure A shows the clinical photo of a 28-year-old homeless man who presents with a left thumb mass which has been present for the last three years. He reports having a similar mass that was excised at an outside hospital 5 years earlier. A biopsy of the mass is taken, and the histopathology is shown in Figures B and C. What is the most likely diagnosis? Review Topic

QID:1988
FIGURES:
1

Rheumatoid nodule

2%

(4/221)

2

Squamous cell carcinoma

40%

(88/221)

3

Epithelioid sarcoma

48%

(106/221)

4

Angiosarcoma

6%

(13/221)

5

Epidermal inclusion cyst

4%

(8/221)

Select Answer to see Preferred Response

PREFERRED RESPONSE 3

Figure A shows an erythematous, ill defined, multi-nodular firm mass with areas of ulceration. Figures B and C shows the characteristic appearance of epitheliod sarcoma with a multi-nodular granulomatous pattern and areas of central necrosis.

Epitheliod sarcomas are the most common soft tissue sarcoma in the hand. They typically occur on the upper extremities of young adults. They have a tendency to spread locally by way of lymphatics or along fascial planes. Diagnosis is typically confirmed with biopsy that shows distinct nodular aggregates of epithelioid and spindle cells with zonal necrosis. It exhibits immunohistochemical reactivity for epithelial markers including keratin, vimentin and CD34. Treatment is wide surgical excision with adjuvant radiotherapy, and inadequate excision typically results in recurrence.

In this case, the clinical photo is quite concerning. It should be noted that initial appearance may often be benign and is frequently misdiagnosed. At time of presentation 30% of cases have metastasized, further reinforcing the importance of correct diagnosis upon initial presentation.

Halling et al. describe clinical, pathologic, treatment and outcome features of 55 cases of epitheliod sarcoma. 27% of patients died of the disease despite surgical management. They also found a decreasing rate of recurrence when more aggressive surgical margins were taken.

Illustration A shows a more benign appearing example of epithelioid sarcoma.

Incorrect answers:
Answer 1: While the histopathology may also be consistent with a rheumatoid nodule, his age and the location of the mass make epithelioid sarcoma the more correct answer.
Answer 2: Histopathology is not consistent with squamous cell carcinoma, as evidenced by lack of keratin "pearls."
Answer 4: Angiosarcoma biopsy would show well formed vascular channels.
Answer 5: Epidermal inclusion cysts typically have a more benign clinical appearance and a low risk of recurrence after excision.

ILLUSTRATIONS:

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