TY - JOUR
T1 - Angiosarcomas arising in the viscera and soft tissue of children and young adults
T2 - A clinicopathologic study of 15 cases
AU - Deyrup, Andrea T.
AU - Miettinen, Markku
AU - North, Paula E.
AU - Khoury, Joseph D.
AU - Tighiouart, Mourad
AU - Spunt, Sheri L.
AU - Parham, David
AU - Weiss, Sharon W.
AU - Shehata, Bahig M.
PY - 2009/2
Y1 - 2009/2
N2 - Angiosarcomas are rare tumors that predominantly affect adult and elderly patients and pursue an aggressive clinical course with high mortality. Although angiosarcomas are well described in a variety of clinical settings, they have been incompletely characterized. We identified 15 high-grade angiosarcomas arising from the viscera and soft tissue of patients 21 years old and younger from institutional and consultation files. Both clinical (patient age, tumor site, tumor size, tumor focality) and histologic features including growth pattern (vasoformative vs. solid), nuclear grade (high vs. low), mitotic rate (mitotic figures/10high-power fields), necrosis (present vs. absent), and cell shape (epithelioid vs. nonepithelioid) were assessed. Tumors arose in both sexes (8 males; 7 females); age at diagnosis ranged from 3 months to 19 years (mean, 10y, 5mo; median, 11y). Tumors were located in the mediastinum (7), visceral organs (2 in liver, 1 in spleen), breast (2), mesentery (1), pelvis (1), and deep soft tissue of upper extremity (1). Tumor size was documented for 8 patients (range 3.5 to 13cm; mean 8.1cm). Eight cases showed epithelioid morphology and 7 cases were primarily spindled. Of 8 cases assessed for podoplanin expression by immunohistochemistry, 7 were negative and 1 was positive. Clinical follow-up was obtained for all patients: 10 (67%) died of disease (range, 27mo to 11y; mean, 6y 8mo) and 4 patients were alive without disease (range, 27 to 132mo; mean, 79mo). Although extremely rare, angiosarcomas do affect children and young adults and this diagnosis should be considered in atypical vascular tumors occurring in the mediastinum and those with brisk mitotic activity and/or necrosis.
AB - Angiosarcomas are rare tumors that predominantly affect adult and elderly patients and pursue an aggressive clinical course with high mortality. Although angiosarcomas are well described in a variety of clinical settings, they have been incompletely characterized. We identified 15 high-grade angiosarcomas arising from the viscera and soft tissue of patients 21 years old and younger from institutional and consultation files. Both clinical (patient age, tumor site, tumor size, tumor focality) and histologic features including growth pattern (vasoformative vs. solid), nuclear grade (high vs. low), mitotic rate (mitotic figures/10high-power fields), necrosis (present vs. absent), and cell shape (epithelioid vs. nonepithelioid) were assessed. Tumors arose in both sexes (8 males; 7 females); age at diagnosis ranged from 3 months to 19 years (mean, 10y, 5mo; median, 11y). Tumors were located in the mediastinum (7), visceral organs (2 in liver, 1 in spleen), breast (2), mesentery (1), pelvis (1), and deep soft tissue of upper extremity (1). Tumor size was documented for 8 patients (range 3.5 to 13cm; mean 8.1cm). Eight cases showed epithelioid morphology and 7 cases were primarily spindled. Of 8 cases assessed for podoplanin expression by immunohistochemistry, 7 were negative and 1 was positive. Clinical follow-up was obtained for all patients: 10 (67%) died of disease (range, 27mo to 11y; mean, 6y 8mo) and 4 patients were alive without disease (range, 27 to 132mo; mean, 79mo). Although extremely rare, angiosarcomas do affect children and young adults and this diagnosis should be considered in atypical vascular tumors occurring in the mediastinum and those with brisk mitotic activity and/or necrosis.
KW - Angiosarcoma
KW - Pediatric
KW - Podoplanin
KW - Soft tissue
KW - Viscera
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U2 - 10.1097/PAS.0b013e3181875a5f
DO - 10.1097/PAS.0b013e3181875a5f
M3 - Article
C2 - 18987547
AN - SCOPUS:60249102545
SN - 0147-5185
VL - 33
SP - 264
EP - 269
JO - American Journal of Surgical Pathology
JF - American Journal of Surgical Pathology
IS - 2
ER -