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SPC

SPC. 骨科 住院醫師 楊碩文 骨科 李建和 主任. Basic Data. 劉 先生 64 y/o Chief Complain : Palpable a solid painless mass over right leg posterior aspect for 3 years and the mass rapid enlargement with pain for last 2 weeks. Present illness. Denied any systemic disease

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SPC

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  1. SPC 骨科 住院醫師 楊碩文 骨科 李建和 主任

  2. Basic Data • 劉 先生 64 y/o Chief Complain : Palpable a solid painless mass over right leg posterior aspect for 3 years and the mass rapid enlargement with pain for last 2 weeks

  3. Present illness • Denied any systemic disease • Old open fracture of right tibia and fibula s/p external fixation 40+ years ago (fracture healed well) • Sustained a solid painless mass about one egg size over right posterior leg for about 3 years • No traumatic history at lesion area or infection sign and discharge sinus • The mass rapidly enlarged with pain and tender for last 2 weeks

  4. Physical Examination • A solid, fixed, tumor mass with local tenderness over right calf muscle about 10x8x6 cm in size • Right ankle weakness of dorsiflexion ,no sensory disorder

  5. Lab Data

  6. Impression • r/o Soft tissue malignant tumor of right leg • Imaging studies • Plain radiographs of the affected area • MRI with contrast • Chest CT scan • Biopsy was arranged and done on 100/07/25

  7. Leiomyosarcoma of extremity

  8. Leiomyosarcoma of Soft Tissue • Arise directly from the smooth muscle cells lining small blood vessels • Extremely rare occurrence • Most malignant leiomyosarcomas arise independently, and are not associated with benign tumors

  9. Leiomyosarcoma of Cutaneous Origin • Man : women = 2:1 • First diagnosed (1-2 cm), and prognosis is generally good • Deeper lesions can metastasize in up to 30-40% of cases, usually hematogenously to the lungs

  10. American Joint Committee on Cancer(AJCC) Staging System

  11. Treatment • Surgery • Achieving wide surgical margins is important in preventing local recurrence • In this case: rescetable mass wild rescetion • Radiation Therapy • Pre-operatively (neoadjuvant) or post-operatively (adjuvant): • In this case: margin free, no consider R/T • Chemotherapy • Treatment of metastatic disease • In this case: no sign of metastasis, no consider C/T

  12. Prognosis: Soft Tissue Leiomyosarcoma • worse prognosis : • age >62 years, size >4cm, tumor necrosis, vascular invasion, or previous intralesional surgery • 50% 3-year survival to 64% 5-year survival

  13. Prognosis: Cutaneous Leiomyosarcoma • True intradermal leiomyosarcoma is thought not to metastatsize • Wide excision of truly intradermal tumors, if achievable, is curative

  14. Bone scan

  15. CT pre- & post-C

  16. STIR T1WI STIR T2WI

  17. AT PT GN Soleus EDL PL Gd-T1WI

  18. No pulmonary meta.

  19. case I 劉x慶 11806 xxx T11-9233, 9263 Frozen section and biopsy Soft tissue, calf, right, intra-operative biopsy, spindle cell tumor, favor sarcoma with myogenic differentiation T11-9983 Soft tissue, leg, right, wide excision, leiomyosarcoma

  20. Frozen section: one tissue fragment measuring 1.6 x 0.7 x 0.7 cm in size neoplastic spindle cells arranged in sheet and fascicular pattern with lymphocyte spindle cell tumor, favoring sarcoma

  21. neoplastic spindle cells arranged in sheet and fascicular pattern with areas of necrosis

  22. Neoplastic spindle cells with pleomorphic and blunt-ended nuclei

  23. A piece of skin 11.8 x 4.3 cm. One surgical scar measuring 2.5 cm in length One part of calf muscle: 12.0 x 7.7 x 4.8 cm. and 240 gm. with an intramuscular tumor: 4.9 x 4.7 x 3.5 cm

  24. On section, the tumor is heterogeneous brown to yellow and soft with focal hemorrhage and necrosis The tumor is located intramuscularly. The overlying skin is not involved. The tumor measures 0.4 cm, 3.8 cm, 4.0 cm, 3.9 cm and 2.2 cm away from the lateral, medial, deep, superior and inferior section margins of the specimen

  25. Necrosis: red area (left side), central tumor, normal muscle (right side)

  26. Hemorrhage and necrosis Tumor area

  27. Hemorrhage and necrosis

  28. Lymphoplasma cell Thick-walled vessel Tumor cells with eosinophilic or clear cytoplasm

  29. Tumor cells with eosinophilic or clear cytoplasm and pleomorphic and blunt-ended nuclei

  30. Tumor cells with eosinophilic or clear cytoplasm and pleomorphic nuclei Some lymphocyte and eosinophil infiltrate

  31. Mitotic figure: 3/10 hpf

  32. Atypical mitosis

  33. Small separated tumor nodule is noted Main tumor mass

  34. Skin is free from the tumor

  35. HE Actin (+), h-caldesmon (+) Desmin (+) Vimentin (+)

  36. CK (F+) CD31(F+), CD34 (-) Myogenin (-) S-100, HMB45 (-)

  37. Findings and Differential diagnosis Gross findings: heterogeneous brown to yellow and soft with focal hemorrhage and necrosis → malignancy (sarcoma) Microscopic findings: • Perpendicularly oriented fascicles of spindle cells • Brightly eopsinophilic cytoplasm • Blunt-ended nuclei • Nuclear atypia →myogenic differentiation

  38. Immunostudy: (+) Vimentin, Actin, Desmin, h-caldesmon Focal (+): CK, CD31 (-): myogenin, S-100, HMB45, CD34 Spindle cell sarcoma, with myogenic differenitation • h-Caldesmon  leiomyosarcoma (consultation with Dr. Hsuan-Ying Huang in Kaohsiung CGMH)

  39. MICROSCOPIC Histologic Type: Leiomyosarcoma FNCLCC (French Federation of Cancer Centers Sarcoma Group) grading system: Tumor differentiation: Score 2: sarcomas of definite histologic type Mitotic count: Score 3: 20 or more than 20 mitoses per 10 HPF (our case: 22/10 hpf) Tumor necrosis: Score 1: less than or equal to 50 % tumor necrosis Histological grade: Grade 3: total score 6 -8 Margins: Margins negative for sarcoma Distance of sarcoma from closest margin: 0.4 cm, lateral soft tissue margins Lymph-Vascular Invasion: Not identified

  40. Pathologic Staging (pTNM) (AJCC/UICC TNM, 7th edition)Primary Tumor (pT): pT1b: Tumor 5 cm or less in greatest dimension, deep tumorRegional Lymph Nodes (pN): pNX: Regional lymph nodes cannot be assessed (not sampled)Anatomic stage/prognostic groups: pStage IIA at least (pT1b NX MX G3)

  41. Leiomyosarcoma - 1 • Definition: malignant neoplasm composed of cells exhibiting smooth muscle differentiation • Etiology: EB virus associated in immunosuppressed patient or associated with radiation • Incidence: Rare: 10-15% of extremity sarcoma (but common if including the uterine and visceral lesions) 4. Age: middle-aged adults • Gender: no preference (women easily found in retroperitoneal and inferior vena cava areas)

  42. Leiomyosarcoma - 2 • Classification: a. leiomyosarcoma of soft tissue b. leiomyosarcoma of cutaneous origin c. leiomyosarcoma of vascular origin d. leiomyosarcoma in the immunocompromised host e. leiomyosarcoma of bone Variant and special forms: Myxoid leiomyosarcoma Inflammatory leiomyosarcoma Pleomorphic leiomyosarcoma Leiomyosarcoma with osteoclastic-like giant cells Epitheliod leiomyosarcoma

  43. Immunohistochemistry for leiomyosarcoma • Desmin + • Actin-sm + • Calponin + • Caldesmon + • CK-PAN + • ER - • CD34 - • PR - • S-100 - • HMB-45 -

  44. Leiomyosarcoma - 3 • S/S: deep soft tissue mass – often asymptomatic a. retroperitoneal – abdominal pain b. vena cava → Upper portion: Budd-Chari syndrome (hepatomegaly, jaundice, ascites) Mid-portion: Renal obstruction Lower portion: lower extremity edema • Treatment: *surgical excision, radiation, chemotherapy • Prognosis: depend on site and stages of lesions a. Restricted in cutis → essentialy never meta. As “atypical smooth muscle tumor” b. in subcutis: up to 1/3 meta. 10-20% die of diseasec. retroperitoneum: 80% die of disease, typical with metastasis d. bone: up to ½ meta. 5 y survival: 65% e. vena cava: 5y – 50%, 10y – 30% survival f. head and neck: over ½ metastasis

  45. 北醫附醫， 萬芳醫院， 雙和醫院(1995-2011/6) (1998-2011/6) (2008-2011/6) 1. leiomyoma: 2161 Uterus: 1693 age:10-97 (average: 51.4) 2. leiomyosarcoma:12 Uterus: 3 age: 42-63 (average:51.3) 3/1693: 0.18% Non-uterus: 9 age: 41-93 (mean: 58.2) M;F: 3:6 extremity-2, retroperitoneum-2, G-I-3, vagina-1, scrotum-1 1. leiomyoma: 423 Uterus: 355 age:17-82 (average: 47.6) 2. leiomyosarcoma: 3 Uterus: 0 Non-uterus: 3 age: 48-62 (mean:57.3) M:F: 2:1 extremity-1, retroperitoneum-1, G-I-1 1. leiomyoma: 8313 Uterus: 8033 age:10-96 (average: 47.3) 2. leiomyosarcoma: 39 Uterus: 20 age: 35-67 (average:52.1) 20/8033: 0.24% Non-Uterus:23 age: 39-92 (mean: 69) M:F:9:14 Intraabdomen-5, extremity-5, G-I-4, retroperitoneum-2, urinary bladder-2, back-1, pararectum-1, cervix-1, vagina-1, palate-1

  46. SPC 100.08.26 王樂明醫師/劉偉民主任

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