sudden death as the presenting symptom of Wilms’ tumor
Afshin MOHAMMADI
Urmiye Üniversitesi Tıp Fakültesi, Radyoloji Anabilim Dalı, Urmiye, Batı Azerbaycan, İran.
ÖZET
Wilms tümörünün başvuru semptomu olarak rekürren pulmoner tümor embolisi ve ani ölüm
Böbrek tümörlü çocukların sadece %4-6’sında başvuru sırasında inferior vena kava veya sağ atriyal infiltrasyon bulun- maktadır. Tümör embolisi daha nadirdir ve Wilms tümöründe başvuru semptomu olarak ani ölüm şimdiye kadar sadece sekiz olguda bildirilmiştir. Küçük pulmoner emboli ile başvuran ve sonrasında masif pulmoner emboliye bağlı ani ölüm gelişen nadir bir Wilms tümörlü olguyu sunuyoruz. Tanı sırasında, stabil hemodinami ve invaziv vasküler infiltrasyonlu hastalarda acil cerrahi gerekli olabilir gibi görünmektedir.
Anahtar Kelimeler:Wilms tümörü, pulmoner emboli, ani ölüm.
SUMMARY
Recurrent pulmonary tumoral embolism and sudden death as the presenting symptom of Wilms’ tumor
Afshin MOHAMMADI
Department of Radiology, Faculty of Medicine, Urmia University, Urmia, West Azerbaijan, Iran.
Only 4-6% of children with renal tumor show inferior vena caval or right atrial infiltration at presentation. Tumor emboli are even rarer, and so far, sudden death as the presenting symptom has only been described at presentation in Wilms’ tumor in eight cases. We report a unique case of Wilms’ tumor that presented with small pulmonary emboli and immediately after asthatwas diagnosed before deathby detection of small emboli and immediately after sudden death as massive pulmonary embolism. It seems that in cases of invasive vascular infiltration with a stable hemodynamic condition at diagnosis imme- diate surgery may be necessar
Key Words: Wilms’ tumor, pulmonary embolism, sudden death.
Yazışma Adresi (Address for Correspondence):
Dr. Afshin MOHAMMADI, Urmia, Modaress BLVD, Imam Hospital URMIA - IRAN
e-mail: mohamadi_afshin@yahoo.com
Wilms’ tumor is the most common urinary tract neop- lasm of childhood (1). Only 4-6% of children with re- nal tumor show inferior vena caval (IVC) or right atrial infiltration at presentation (2-4). However, up to 40%
of Wilms tumors have invaded the renal vein by the ti- me of diagnosis (3). Tumor emboli are even rare, and sudden death as presenting symptom has only been described at presentation in WT in 8 cases so far (2).
Although pulmonary metastases may already be pre- sent in up to one fourth of cases at the time of initial diagnosis, it is exceedingly rare for pulmonary emboli to develop as a first manifestation of WT (5,6). We re- port a unique case of recurrent pulmonary emboli that was diagnosed premortem by detection of small em- boli and after sudden death due to massive pulmonary embolism.
CASE REPORT
A 8-year-old boy was in his usual state of good health until the day of admission when after vomiting he be- came pale and complained of shortness of breath.
Both lungs were clear to auscultation but in inspecti- on mild intercostal retractions were obvious. Abdo- men was distended and a firm abdominal mass was palpable at left side of abdomen measuring 10 x 10 cm that did not across through the midline of abdo- men.
During first day of admission Arterial Blood Gas and ot- her Laboratory data such as CBC & ESR, BUN, Cr, Na, K & PH was normal but there was microscopic hema- turia in Urine analysis (8-10 RBC/hpf).
Chest X-ray was obtained at admission and showed mild right side pleural effusion without pulmonary con- solidation or nodular opacity Figure 1.
Abdominal ultrasonography revealed a large well mar- ginated hypoechoeic mass arising from lower pole of left kidney.
24 hours after admission the patient became tachype- nic and complains from exacerbation of shortness of breath and chest pain.
In physical examination there was respiratory distress and intercostal retractions on inspection and pulmo- nary rales in right lower lobe of right lung on ausculta- tion. Chest and abdominal computed tomography (CT) scan with intravenous and oral contrast was performed for further evaluation of abdominal mass and respira- tory problem.
Chest CT showed several pleural based pulmonary no- dules and a wedge shape confluence of perivascular pulmonary nodules in the right lower lobe of right lung and with lesser extend in left lower lobe (Figure 2).
Chest CT also showed a filing defect that extended from IVC to right atrium (Figure 3), and small filing de- fect in the right pulmonary artery (Figure 4).
Abdominal CT with intravenous and oral contrast sho- wed a huge left renal mass extending into left renal ve- in and IVC (Figure 5).
After 36 hours of admission the patient suddenly col- lapsed. Unfortunately, resuscitation was not successful.
Autopsy confirmed the diagnosis and showed a large left renal mass extending into left renal vein and inferi- or vena cava up to right atrium accompanying with a saddle mixed tumor and clot emboli in pulmonary ar- tery bifurcation (Figure 6).
Histology revealed nephroblastoma, with diffuse anap- lasia, stage 3.
Figure 1. Chest X-ray show right side blunting of costophe- renic angle (black arrow) due to pleural effusion.
Figure 2. Axial CT image show bilateral nodular opacity in both right and left lower lobe (black arrow).
DISCUSSION
WT is one of the most common tumors in infants and children. WT typically is manifested as an asymptoma- tic abdominal mass or abdominal pain, fever and he- maturia (7) .
WT is one of the successes of pediatric oncology, with an overall cure rate of over 85%, using relatively simp- le therapies. This excellent outcome has been the result of collaborative efforts among surgeons, pediatricians, pathologists and radiation oncologists. The results that have been achieved in children with WTs support the strong value of the multidisciplinary team approach to cancer (8).
The initial pulmonary tumor emboli causing sudden death as presenting symptom has only described at presentation in WT in 8 cases so far as Table 1 (2).
The low reported incidence of intravascular extention of tumor thrombosis (4-6%) may be due to imaging dif- ficulties in the past years (24). In adults, sudden deaths have been reported in 11 cases associated with malig-
nancies, like broncogenic carcinoma, pancreatic carci- noma, leukemia, gastric carcinoma, thyroid carcino- ma, urinary bladder carcinoma, astrocytoma, and only once with renal tumor (9).
Pulmonary embolism in children and infants is one of the difficult diagnoses to make on the basis of clinical finding.
As WT patients often present without any symptom, it is unlikely to prevent from sudden death due to pulmo- nary tumor embolism as presenting symptom.
Figure 3. Axial CT image show filling defect extending from IVC in to right atrium (black arrow).
Figure 4. Axial CT image show a filling defect (white arrow) in right lower lobe branch of right pulmonary artery.
Figure 5. A. Axial CT image show a large well defined hypo- dense mass arising from lower pole of left kidney (white ar- row) and low-attenuation filling defect within an enlarged left renal vein (white arrowhead) that represent direct tumor ex- tension. B. Axial CT image show low-attenuation filling de- fect within hepatic portion of IVC.
Figure 6. Autopsy photograph shows a serpiginous mixed tu- mor and clot embolus at the bifurcation of the pulmonary ar- tery.
A
B
Most tumor cells which reach the lungs become trap- ped within pulmonary capillaries, many malignant cell trigger the coagulation cascade, obstruction of pulmo- nary vessels usually result from the presence of both tumor cells and associated clot. This secondary chan- ge may produce complete and irreversible obstruction of pulmonary vessels.
Some authors believe that a presumptive diagnosis, based an atypical presentation and exclusion of throm-
boembolic disease is adequate. Massive tumor emboli are a rare cause of acute respiratory distress in child- ren. Five patients from case of WT embolization to the lung have been reported that occurred during intraope- rative manipulation.
Although, it has been shown that chemotherapy before surgery downstages the WT and reduces the risk of tu- mor ruptures, it is clear that treatment decisions should be made based on the characteristics and stability of the individual patients especially for those with cardiac involvement (10).
CT scan is a valuable method for examining renal ve- in, IVC and right atrial invasion by WT.
This case demonstrate that WT can embolize preope- ratively. We conclude that death due to pulmonary tu- mor emboli in pediatric renal tumors is rare and in the cases of invasive vascular infiltration at diagnosis with stable hemodynamic condition immediate start of sur- gery may be necessary.
CONFLICT of INTEREST None declared.
Table 1. Characteristics of reported pediatric renal tumor cases who presented with sudden death due to pul- monary embolism.
Tumor Stage at
Patient Reported Sex Age side RVI presentation Histology Outcome
1 Anselmi M 4 Y R Yes NFS WT Died
et al, 1970
2 Zakowski M 6 Y R Yes 4 WT Died
et al, 1990
3 Zakowski F 7 Y R Yes 4 WT Died
et al, 1990
4 Bulas F 6 Y R Yes 4 WT Died
et al, 1991
5 Ceelen F 3 Y L Yes 4 WT Alive
et al, 1997
6 Lampert F 4 Y R Yes NFS WT Died
et al, 2001
7 Van den heuvel- M 7 Y L Yes 4 WT Died
Eibrink, 2008
8 Van den heuvel- F 5 Y L Yes 3 SS Died
Eibrink, 2008
Figure 7. A 8-year-old boy with Wilms’ tumor. Pathology re- vealed nests and sheets of primitive blastema with interve- ning mesenchyme.
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