SL-28 CASE 3 Marco Gambarotti, MD Surgical Pathology Rizzoli - - PowerPoint PPT Presentation

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SL-28 CASE 3 Marco Gambarotti, MD Surgical Pathology Rizzoli - - PowerPoint PPT Presentation

Mar 07, 2023 391 likes •651 views

SL-28 CASE 3 Marco Gambarotti, MD Surgical Pathology Rizzoli Orthopaedic Institute SL-28 CASE 3 Female, 30-years-old Severe obesity Chronic low back pain for several months Worsening of back pain after minor spinal trauma 2

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SL-28 CASE 3

Marco Gambarotti, MD Surgical Pathology Rizzoli Orthopaedic Institute

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SL-28 CASE 3

  • Female, 30-years-old
  • Severe obesity
  • Chronic low back pain for several

months

  • Worsening of back pain after minor

spinal trauma 2 months before

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Bone lesion in L3 (5.5x5x3 cm)

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Soft tissue lesion in the left thigh (8x5x3.5 cm)

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Thigh

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L3

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Differential diagnosis:

  • High-grade osteosarcoma
  • Sclerosing rhabdomyosarcoma
  • Metastatic carcinoma
  • Lymphoma (sclerosing variant)
  • Myoepithelial carcinoma
  • Angiosarcoma
  • Sclerosing epithelioid fibrosarcoma
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MUC4 +

Molecular analysis: Pos t(11;22) EWSR1-CREB3L1

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  • Diagnosis: Sclerosing Epithelioid Fibrosarcoma
  • Treatment: Neoadjuvant CT (Epi+Ifo, 2 cycles) then

excision of the soft tissue mass. L3 vertebrectomy after two further cycles of CT (Etoposide and Cisplatinum)

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Thigh

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Thigh

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Thigh

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Thigh

Diagnosis: Sclerosing Epithelioid Fibrosarcoma with Low-Grade Fibromyxoid Sarcoma areas and metaplastic bone (necrosis 10%)

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L3

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L3 (necrosis 60%)

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Outcome: DOD after 2 years F.U. (lung mets, bone mets)

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Sclerosing Epithelioid Fibrosarcoma

  • Adults (median age; 45 years)
  • M=F
  • Deep soft tissue of the limbs (+++ lower limbs) and

limb girdles; rarely in bone

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  • Local recurrences (30-50%)
  • Metastasis (> 50%) to lungs, bone, pleura, and soft

tissues

  • About 35% of patients die of disease
  • Poor response to conventional chemotherapy

Sclerosing Epithelioid Fibrosarcoma

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Differential diagnosis:

  • Extraskeletal osteosarcoma
  • Spindle cell/sclerosing rhabdomyosarcoma
  • Metastatic carcinoma
  • Metastatic melanoma
  • Lymphoma (sclerosing variant)
  • Malignant mesothelioma
  • Paraganglioma
  • Hyalinizing plexiform leiomyoma
  • Ossifying fibromyxoid tumor
  • Epithelioid synovial sarcoma
  • Epithelioid sarcoma
  • Clear cell sarcoma
  • Epithelioid MPNST
  • Myoepithelial carcinoma
  • Angiosarcoma

Sclerosing Epithelioid Fibrosarcoma

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t(11;16)(p11;p11) FUS-CREB3L1 rare rare t(7;16)(q34;p11) FUS-CREB3L2 hybrid 95% t(11;22)(p11;q12) EWSR1-CREB3L1 most cases rare t(7;22)(q34;q12) EWSR1-CREB3L2 rare MUC4+ ~100% ~100% EMA+ 50% 80%

SEF LGFMS

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Codivilla-Putti Research Centre Codivilla-Putti Research Centre Rizzoli Orthopaedic Institute Rizzoli Orthopaedic Institute