Latest Updates

Rare Fibroblastic and Myofibroblastic Tumors

Yu Liang, MD, PhD, Laleh Melstrom, MD, MS, FACS, Vasilena Zheleva, MD
    • Advances in cytogenetic analysis allowing more accurate diagnosis of these rare tumors, some of which have specific gene rearrangements
    • Advances in targeted therapies such as crizotinib for tumors with ALK gene rearrangements
    • Advanced in radiation therapy allowing dose escalation to the target with sparing of normal tissue (as in intensity modulated radiotherapy)

Current Opinion 

David S Knopman, MD

    Pediatric Tumors: Extraskeletal Ewing Sarcoma

    John Groundland, MD, MS, Sara Shaw, BS
      • Skeletally based Ewing sarcoma, extraskeletal Ewing sarcoma, primitive neuroectodermal tumor, and Askin tumor were once considered distinct entities due to their various presentations but are now understood to be part of a singular group, the Ewing sarcoma family of tumors (ESFT), due to their common cell of origin and characteristic chromosomal translocations, most commonly t(11;22)(q24;q12).
      • Despite identification of the characteristic chromosomal translocations and the resultant fusion proteins (e.g., EWS-FLI1), targeted therapies specific for ESFT have yet to be sufficiently developed for clinical practice
      • The current treatment regimen of ESFT includes neoadjuvant multiagent cytotoxic chemotherapy, followed by local control and completed with adjuvant, cytotoxic chemotherapy.

    Current Opinion 

    David S Knopman, MD

      Pediatric Tumors: Extraskeletal Ewing Sarcoma

      John Groundland, MD, MS, Sara Shaw, BS
        • Skeletally based Ewing sarcoma, extraskeletal Ewing sarcoma, primitive neuroectodermal tumor, and Askin tumor were once considered distinct entities due to their various presentations but are now understood to be part of a singular group, the Ewing sarcoma family of tumors (ESFT), due to their common cell of origin and characteristic chromosomal translocations, most commonly t(11;22)(q24;q12).
        • Despite identification of the characteristic chromosomal translocations and the resultant fusion proteins (e.g., EWS-FLI1), targeted therapies specific for ESFT have yet to be sufficiently developed for clinical practice
        • The current treatment regimen of ESFT includes neoadjuvant multiagent cytotoxic chemotherapy, followed by local control and completed with adjuvant, cytotoxic chemotherapy.

      Surgical Management of the Axilla in Breast Cancer

      Cory Donovan, MD, Armando E Giuliano, MD
        • Surgical axillary management for patients with early breast cancer has evolved from complete axillary dissection to sentinel lymph node biopsy (SLNB).
        • Today, the vast majority of breast cancer patients should undergo SLNB alone. This includes many patients with sentinel lymph node metastases.
        • Radiation may replace axillary lymph node dissection (ALND) for patients with early sentinel node–positive disease but may be unnecessary for most patients.
        • SLNB can adequately stage the axilla if three or more sentinel nodes are removed.
        • There has not been a demonstrable increase in axillary recurrence or a decrease in survival with the decreased use of ALND.

      Surgical Management of the Axilla in Breast Cancer

      Cory Donovan, MD, Armando E Giuliano, MD
        • Surgical axillary management for patients with early breast cancer has evolved from complete axillary dissection to sentinel lymph node biopsy (SLNB).
        • Today, the vast majority of breast cancer patients should undergo SLNB alone. This includes many patients with sentinel lymph node metastases.
        • Radiation may replace axillary lymph node dissection (ALND) for patients with early sentinel node–positive disease but may be unnecessary for most patients.
        • SLNB can adequately stage the axilla if three or more sentinel nodes are removed.
        • There has not been a demonstrable increase in axillary recurrence or a decrease in survival with the decreased use of ALND.

      Pediatric Tumors: Rhabdomyosarcoma

      John Groundland, MD, MS, R L Randall, MD, FACS, Sara Shaw, BS
        • Classification of rhabdomyosarcoma (RMS) into groups and variants has led to improved understanding of disease prognosis and the need for tailored intervention.
        • Multiagent chemotherapy with surgical excision and/or therapeutic radiation has led to an overall improvement in 5-year survival of RMS from 25 to 75% between 1970 and 2010.
        • Despite improvement in survival of low- and intermediate-risk RMS patients, patients with high-risk RMS or recurrent disease continue to respond inadequately to treatment and have poor survival outcomes.
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