PMID- 3024596
OWN - NLM
STAT- MEDLINE
DCOM- 19870113
LR  - 20190514
IS  - 0003-4932 (Print)
IS  - 1528-1140 (Electronic)
IS  - 0003-4932 (Linking)
VI  - 204
IP  - 6
DP  - 1986 Dec
TI  - Intracardiac extension of Wilms' tumor. A report of the National Wilms' Tumor 
      Study.
PG  - 693-7
AB  - Extension of Wilms' tumor through the inferior vena cava into the heart presents 
      a formidable clinical challenge. Excision of such a tumor without provoking 
      emobilization may require cardiopulmonary bypass (CPB). The completeness of 
      excision and the likelihood of tumor embolization during operation guide 
      subsequent radiation therapy (RT) and chemotherapy. To help define these issues, 
      the clinical records of 15 patients enrolled in three National Wilms' Tumor 
      Studies (NWTS) who had intracardiac tumor extension (ICE) were reviewed. The 
      median age at diagnosis was 4 years. One patient had clear cell sarcoma (CCS); 
      the remainder had favorable histologic findings (FH). The clinicopathologic stage 
      was stage II in one patient, stage III in eight patients, and stage IV in six 
      patients. ICE was detected before operation in six patients, during operation in 
      five patients, and after operation in five patients. CPB was used in 10 patients. 
      Eleven patients (73%) had operative complications, with major intraoperative 
      hemorrhage occurring most often (six patients). Complications occurred less often 
      when ICE was recognized before operation (three of six patients) than when it was 
      not (eight of nine patients). Embolization occurred in only two patients. There 
      were no operative deaths. The patient with CCS died. Eleven of 14 patients with 
      FH survived, with an actuarial event-free, 2-year survival rate of 86%. There 
      were no patients in the first NWTS. Of the six patients in the second NWTS 
      (NWTS-2), four died (67%). All nine patients in the third NWTS (NWTS-3) survived, 
      but follow-up was shorter (median 4 years 9 months vs. 2 years 7 months). No 
      particular surgical procedure was associated with an increased death rate. This 
      review suggests Wilms' tumor with ICE presents a formidable surgical undertaking 
      but has a relatively good prognosis. Embolization is an uncommon event in ICE 
      (two patients, 13.3%), allowing a planned operative approach. Echocardiography 
      and ultrasonography provide accurate preoperative diagnosis. And ICE should be 
      suspected in patients with extensive vena cava thrombosis or who have hypotension 
      or heart failure during examination or surgery.
FAU - Nakayama, D K
AU  - Nakayama DK
FAU - Norkool, P
AU  - Norkool P
FAU - deLorimier, A A
AU  - deLorimier AA
FAU - O'Neill, J A Jr
AU  - O'Neill JA Jr
FAU - D'Angio, G J
AU  - D'Angio GJ
LA  - eng
GR  - R01 CA054498/CA/NCI NIH HHS/United States
GR  - CA-11722/CA/NCI NIH HHS/United States
PT  - Journal Article
PT  - Research Support, U.S. Gov't, P.H.S.
PL  - United States
TA  - Ann Surg
JT  - Annals of surgery
JID - 0372354
SB  - IM
MH  - Adolescent
MH  - Child
MH  - Child, Preschool
MH  - Combined Modality Therapy
MH  - Female
MH  - Heart Neoplasms/diagnosis/*secondary/surgery
MH  - Humans
MH  - Kidney Neoplasms/*pathology/surgery
MH  - Male
MH  - Neoplasm Invasiveness
MH  - Neoplasm Staging
MH  - Postoperative Complications
MH  - Prognosis
MH  - Vena Cava, Inferior
MH  - Wilms Tumor/diagnosis/*secondary/surgery
PMC - PMC1251427
EDAT- 1986/12/01 00:00
MHDA- 2001/03/28 10:01
PMCR- 1986/12/01
CRDT- 1986/12/01 00:00
PHST- 1986/12/01 00:00 [pubmed]
PHST- 2001/03/28 10:01 [medline]
PHST- 1986/12/01 00:00 [entrez]
PHST- 1986/12/01 00:00 [pmc-release]
AID - 10.1097/00000658-198612000-00013 [doi]
PST - ppublish
SO  - Ann Surg. 1986 Dec;204(6):693-7. doi: 10.1097/00000658-198612000-00013.