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 re-excision of the tumor bed is recommended for inadequate initial resection amputation indications may be necessary to prevent spread of disease in cases of multiple recurrences
QUESTIONS 1 of 6 1 2 3 4 5 6 Previous Next Sorry, this question is for PEAK Premium Subscribers only Upgrade to PEAK (SBQ05PA.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: A B 1 Rheumatoid nodule 5% (171/3498) 2 Ganglion cyst 4% (145/3498) 3 Epithelioid sarcoma 79% (2756/3498) 4 Squamous cell carcinoma 10% (345/3498) 5 Chondrosarcoma 2% (63/3498) ML 2 Select Answer to see Preferred Response PREFERRED RESPONSE 3 (SBQ05PA.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: A B C 1 Rheumatoid nodule 2% (8/516) 2 Squamous cell carcinoma 39% (201/516) 3 Epithelioid sarcoma 50% (259/516) 4 Angiosarcoma 5% (27/516) 5 Epidermal inclusion cyst 3% (18/516) ML 4 Select Answer to see Preferred Response PREFERRED RESPONSE 3 (SBQ05PA.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: A B 1 Brain 4% (16/411) 2 Bone 6% (26/411) 3 Lung 38% (155/411) 4 Lymph nodes 50% (206/411) 5 Thyroid 1% (5/411) ML 4 Select Answer to see Preferred Response PREFERRED RESPONSE 4 Sorry, this question is for PEAK Premium Subscribers only Upgrade to PEAK Sorry, this question is for PEAK Premium Subscribers only Upgrade to PEAK
Upgrade to View Premium Videos Upgrade to View Premium Videos Epithelioid Sarcoma - Pathology Rounds Derek Moore General - Epithelioid Sarcoma 11/17/2012 683 views