Epithelioid Sarcoma

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
- 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

TAG

(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? Topic

Review Topic

FIGURES: A

1. Brain
2. Bone
3. Lung
4. Lymph nodes
5. Thyroid

PREFERRED RESPONSE ▶

DISCUSSION: 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.

REFERENCES:

1. Andreou D, Tunn PU. Sentinel node biopsy in soft tissue sarcoma. Recent Results Cancer Res. 2009;179:25-36. Review. PMID:19230532 (Link to Abstract)

2. Blazer DG 3rd, Sabel MS, Sondak VK. Is there a role for sentinel lymph node biopsy in the management of sarcoma? Surg Oncol. 2003 Nov;12(3):201-6. Review. PMID:12957624 (Link to Abstract)

3. Maduekwe UN, Hornicek FJ, Springfield DS, Raskin KA, Harmon DC, Choy E, Rosenberg AE, Nielsen GP, DeLaney TF, Chen YL, Ott MJ, Yoon SS. Role of sentinel lymph node biopsy in the staging of synovial, epithelioid, and clear cell sarcomas. Ann Surg Oncol. 2009 May;16(5):1356-63. Epub 2009 Mar 4. PMID:19259743 (Link to Abstract)

Question Authors:

Deborah Allen MD, Patrick O'Donnell MD/PhD, Michael Hughes MD,

Please Rate Educational Value!

4.0

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TAG

(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? Topic

Review Topic

FIGURES: A

1. Rheumatoid nodule
2. Squamous cell carcinoma
3. Epithelioid sarcoma
4. Angiosarcoma
5. Epidermal inclusion cyst

PREFERRED RESPONSE ▶

DISCUSSION: 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: A

REFERENCES:

1. Enzinger FM, Weiss SW: Soft Tissue Tumors, ed 3. St Louis, MO, Mosby, 1995, p 1074.

2. Halling AC, Wollan PC, Pritchard DJ, et al: Epithelioid sarcoma: A clinicopathologic review of 55 cases. Mayo Clin Proc 1996;71:636-642. PMID:8656704 (Link to Abstract)

Question Authors:

Mark Karadsheh MD, Patrick O'Donnell MD/PhD, Michael Hughes MD,

Please Rate Educational Value!

5.0

Average 5.0 of 3 Ratings

Level of Evidence 3 (Retrospective Cohort Study or Case-Control Study)

Halling AC, Wollan PC, Pritchard DJ, et al: Epithelioid sarcoma: A clinicopathologic review of 55 cases. Mayo Clin Proc 1996;71:636-642. PMID:8656704 (Link to Abstract)

Level of Evidence 4 (Case Series)

Maduekwe UN, Hornicek FJ, Springfield DS, Raskin KA, Harmon DC, Choy E, Rosenberg AE, Nielsen GP, DeLaney TF, Chen YL, Ott MJ, Yoon SS. Role of sentinel lymph node biopsy in the staging of synovial, epithelioid, and clear cell sarcomas. Ann Surg Oncol. 2009 May;16(5):1356-63. Epub 2009 Mar 4. PMID:19259743 (Link to Abstract)

Textbooks

Review of Orthopaedics, 6th Edition, Mark D. Miller MD, Stephen R. Thompson MBBS MEd FRCSC, Jennifer Hart MPAS PA-C ATC, an imprint of Elsevier, Philadelphia, Copyright 2012
AAOS Comprehensive Orthopaedic Review, Jay R. Leiberman. Published by American Academy of Orthopaedic Surgeons, Rosemont IL. Copyright 2009
Orthopaedic Knowledge Update 10, John M Flyn. Published by American Academy of Orthopaedic Surgeons, Rosemont IL. Copyright 2011
Hoppenfeld SP. Surgical Exposures in Orthopaedics: The Anatomic Approach. Lipponcott, Williams, and Wilkins, Philadelphia, PA, Copyright 2009
Orthopaedic In-training Examination (OITE) Questions 2004-2012, American Academy of Orthopaedic Surgeons, Rosemont IL. Copyright 2004-2012
Self-Assessment Examination (SAE) Questions 2004-2012, American Academy of Orthopaedic Surgeons, Rosemont IL. Copyright 2004-2012

Undefined

Andreou D, Tunn PU. Sentinel node biopsy in soft tissue sarcoma. Recent Results Cancer Res. 2009;179:25-36. Review. PMID:19230532 (Link to Abstract)
Blazer DG 3rd, Sabel MS, Sondak VK. Is there a role for sentinel lymph node biopsy in the management of sarcoma? Surg Oncol. 2003 Nov;12(3):201-6. Review. PMID:12957624 (Link to Abstract)
Pradhan A, Cheung YC, Grimer RJ, Peake D, Al-Muderis OA, Thomas JM, Smith M. Soft-tissue sarcomas of the hand: oncological outcome and prognostic factors. J Bone Joint Surg Br. 2008 Feb;90(2):209-14. PMID:18256090 (Link to Abstract)
McPhee M, McGrath BE, Zhang P, Driscoll D, Gibbs J, Peimer C. Soft tissue sarcoma of the hand. J Hand Surg Am. 1999 Sep;24(5):1001-7. PMID:10509279 (Link to Abstract)
Enzinger FM, Weiss SW: Soft Tissue Tumors, ed 3. St Louis, MO, Mosby, 1995, p 1074.

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