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Leiomyosarcoma

DEFINITION AND PATHOGENESIS

  • A primary or secondary (metastatic) usually moderate grade tumor arising from smooth muscle origin

IMPORTANCE

  • Relatively rare, accounting for ~7% of all soft-tissue sarcomas

CLINICAL FEATURES

  • 9-80 years of age (mean = 52)
  • M:F = 1-2.4:1
  • 90% with pain averaging 11 months in duration (± pathologic fracture)
  • May have a history of a 'benign' uterine fibroid (malignant degeneration in up to 1%) resected previously---review the pathology slides (2-4 years previously)
  • Has been reported in a pt with a hx of retinoblastoma
  • Has been reported associated (probably incidentally) with the femoral interference screw after ACL reconstruction
    • (Biopsy of the lesion through the screw hole after screw removal)
  • GI sxs
    • Massive hematemesis reported in a gastric location

RADIOLOGIC FEATURES

  • Diaphyseal or metaphyseal
  • Average lesion is 7cm in size
  • Bones involved: distal femur, tibia, jaw, proximal humerus, fibula, pelvis, clavicle, ribs
  • Purely lytic lesion with geographic to moth eaten margins, without sclerotic margins
  • 10-15% with soft tissue mass
  • ± Codman's triangle
  • MRI
    • T1-weighted images isointense with bone
    • T2-weighted images heterogeneous
    • May be nearly isointense to fat on T2, hypointense to fluid
  • ± soft tissue mass (esp pelvic lesions)

GROSS PATHOLOGY

  • Pink to yellow, firm, ± hemorrhage/necrosis
  • May arise directly from blood vessels in the extremities
    • 11/16 from the femoral vein
    • 3/16 from the popliteal vein
    • 2/16 from the posterior tibial vein
    • 3-33 cm (mean 10.4 cm)

HISTOLOGIC AND MOLECULAR FEATURES

  • Both primary and metastatic lesions have the same histologic appearance
  • Low power: eosinophilic cell bundles with smooth muscle appearance, tending to cross at right angles
  • Thin-walled vessels between thick bundles of spindle cells
  • Intracytoplasmic filaments seen at high power H&E stain, on EM, with immunostaining for actomyosin, with polarizing lenses, and PTAH staining (intracellular myofilaments)
  • + reticulin staining in lower grade lesions
  • + to desmin, laminin, vimentin, actin, and ?-SMA staining to myoglobin staining (for rhabdomyosarcoma)
  • + PAS with/without diastase
  • Trichrome staining: red cytoplasm
  • Saffron- or phloxine-tartrazine hematoxyin: muscle pink, collagen yellow
  • ± artifactual intracytoplasmic pseudovacuaoles caused by fixation
  • Cigar-shaped nuclei
  • ± hyalinized areas
  • Mitotic activity
    • 1-3/hpf = grade I
    • 3-20/hpf = grade 2
    • >20/hpf = grade 3
    • >20 with nuclear pleomorphism = grade 4
  • EM:
    • Spindle cells with basal lamina and thin filaments with dense bodies indicating smooth muscle differentiation
  • Cytogenetics
    • Similar 32K BAC array and gene expression profiling to undifferentiated pleomorphic sarcoma

DIFFERENTIAL CLINICOPATHOLOGIC DIAGNOSIS

  • Fibrosarcoma
  • MFH
  • Metastatic leiomyosarcoma
    • Has been reported with extramedullary spinal cord compression from a missed dx of uterine leiomyosarcoma

DISEASE COURSE AND TREATMENT

  • Women require work up for a primary uterine leiomyosarcoma (review of previous slides, pelvic exam, CT of the pelvis, hysterectomy if warranted)
  • All pts warrant evaluation for a silent primary in the retroperitoneum or GI tract
  • Inferior vena cava lesions txd with neoadjuvant external-beam XRT (45-50 Gy) and then en bloc resection
    • No reconstruction of the inferior vena cava was performed
      • LE edema in 50%
  • Chemotherapy
  • Survival
    • Lung and skeletal metastases with 50% 5 year survival warrants wide resection in solitary lesions with adjunctive chemotherapy
    • Overall survival at 5 yrs of pts whose tumors arise directly from blood vessels is 25%
    • Predictors of metastases
  • Other areas of metastases

SPECIAL CONSIDERATION

  • PRIMARY DURAL LEIOMYOSARCOMA

    • Reported without uterine primary
  • SUPERFICIAL LEIOMYOSARCOMA

REFERENCES

Abed R, Abudu A, Grimer RJ, Tillman RM, Carter SR, Jeys L: Leiomyosarcomas of vascular origin in the extremity. Sarcoma. 2009;2009:385164. Pubmed

Alexander A, Rehders A, Raffel A, Poremba C, Knoefel WT, Eisenberger CF: Leiomyosarcoma of the inferior vena cava: radical surgery and vascular reconstruction. World J Surg Oncol Jun 26;7:56.

Antonescu CR, Erlandson RA, Huvos AG: Primary leiomyosarcoma of bone: a clincopathologic, immunohistochemical, and ultrastructural study of 33 patients and a literature review. Am J Surg Pathol 21:1281-94,1997.
Berlin O, Angervall L, Kindblom LG, et al: Primary leiomyosarcoma of bone. Clinical, radiographic, pathologic-anatomic, and prognositc study of 16 cases. Skeletal Radiol,16:364,1987.

Burke JP, Maguire D, Dillon J, Moriarty M, O'Toole GC: Whipple's procedure for an oligometastasis to the pancreas from a leiomyosarcoma of the thigh. Ir J Med Sci. 2009 Nov 17.

Carneiro A, Francis P, Bendahl PO, Fernebro J, Akerman M, Fletcher C, Rydholm A, Borg A, Nilbert M: Inidistinguishable genomic profiles and shared prognostic markers in undifferentiated pleomorphic sarcoma and leiomyosarcoma: different sides of a single coin? Lab Invest. 2009 Jun;89(6):668-75. Erratum in: Lab Invest. 2009 Jul;89(7):840.
Caron JJ, Pambuccian SE, Steen JT, Cheng EY: Leiomyosarcoma of the distal femur after anterior cruciate ligament reconstruction. Clin Orthop 419:214-7,2004.

Daylami R, Amiri A, Goldsmith B, Troppmann C, Schneider PD, Khatri VP: Inferior vena cava leiomyosarcoma: Is reconstruction necessary after resection? J Am Coll Surg. 2010 Feb;210(2):185-90. Pubmed
Guse TR, Weis LD: Leiomyosarcoma of the femur in a patient with a history of retinoblastoma. A case report. J Bone Joint Surg 76A:904,1994.
Kidooka M, Okada T, Takayama S, Nakasu S, Handa J: Primary leiomyosarcoma of the spinal dura mater. Neuroradiology 33:173-4,1991.
Mirra JM: Smooth muscle tumors. In Bone Tumors. Clinical, radiologic, and pathologic correlations. JM Mirra (Ed). Lea & Febiger, Philadelphia, 1989, pp. 869-891.
Nanassis K, Alexiadou-Rudolf C, Tsitsopoulos P: Spinal manifestation of metastasizing leiomyosarcoma. Spine 24:987-90,1999.

Palla L, Gentile P, Cannata C, Ascenzi P, Buonomo O, Cervelli V: A neglected giant subcutaneous leiomyosarcoma of the shoulder: a case report. Eur Rev Med Pharmacol Sci. 2009 Sep-Oct;13(5):389-92.

Salas S, Stoeckle E, Collin F, Bui B, Terrier P, Guillou L, Trassard M, Ranchere-Vince D, Gregoire F, Coindre JM: Superficial soft tissue sarcomas (S-STS): a study of 367 patients from the French Sarcoma Group (FSG) database. Eur J Cancer. 2009 Aug;45(12:2091-102. Pubmed

Sleijfer S, Ouali M, van Glabbeke M, Krarup-Hansen A, Rodenhuis S, Le Cesne A, Hogendoorn PC, Verweij J, Blay JY: Prognostic and predicitve factors for outcome to first-line ifosfamide-containing chemotherapy for adult patients with advanced soft tissue sarcomas: an exploratory, retrospective analysis on large series from the European Organization for Research and Treatment of Cancer-Soft Tissue and Bone Sarcoma Group (EORTC-STBSG). Eur J Cancer. 2010 Jan;46(1):72-83.

Soufi M, Errougani A, Chekkof RM: Primary gastric leiomyosarcoma in young revealed by a massive hematemesis. J Gastrointest Cancer. 2009;40(1-2):69-72.

Sundaram M, Akduman I, White LM, McDonald DJ, Kandel R, Janney C: Primary leiomyosarcoma of bone. AJR 172:771-6,1999.
Wirbel RJ, Verelst S, Hanselmann R, Remberger K, Kubale R, Mutschler WE: Primary leiomyosarcoma of bone: clinicopathologic, immunohistochemical, and molecular biologic aspects. Ann Surg Oncol 5:635-41,1998.

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