Journal of Pediatric Surgery 49 (2014) 1864–1866

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Pediatric Surgical Image

Intrarenal neuroblastoma with pulmonary metastases mimicking a Wilms tumor Shannon G. Farmakis a,⁎, Marilyn J. Siegel b,1 a b

Department of Radiology, Saint Louis University School of Medicine, 3635 Vista Ave. at Grand Blvd., St. Louis, MO 63110, United States Mallinckrodt Institute of Radiology, Washington University School of Medicine, 510 South Kingshighway Blvd., St. Louis, MO 63110, United States

a r t i c l e

i n f o

Article history: Received 25 August 2014 Received in revised form 1 October 2014 Accepted 7 October 2014

a b s t r a c t Primary intrarenal neuroblastoma is extremely rare and is a mimic of Wilms tumor. We present a case of a renal mass in a 14 month old male with lung metastases at the initial presentation. This was thought to represent a Wilms tumor. Histology of a biopsied lung nodule revealed neuroblastoma. © 2014 Elsevier Inc. All rights reserved.

Key words: Intrarenal Neuroblastoma Wilms tumor Renal tumors Pediatric

1. Case Report A previously healthy 14 month old boy presented with a two week history of fevers and more acute onset of decreased appetite, cough and vomiting. His mother had also noticed a firm bulge in the abdomen. Physical examination in the emergency department was remarkable for a palpable abdominal mass and hypertension (blood pressure 118/74). An abdominal radiograph was obtained which suggested a large left abdominal mass. Abdomen and pelvis CT showed a large heterogeneously enhancing mass arising from the left kidney (Fig. 1). The mass crossed the midline and displaced the aorta and its branches. No calcifications were seen within the mass. Chest CT showed multiple solid pulmonary nodules (Fig. 2). No lytic or sclerotic lesions were identified. Given the findings of a renal mass and presence of lung metastases, the favored diagnosis was Wilms tumor. After admission, the patient underwent a renal Doppler ultrasound to evaluate his hypertension which confirmed the intrarenal origin of the mass; the spectral waveform did not show evidence of renal artery stenosis (Fig. 3). Pertinent laboratory values were: hemoglobin 6.4 g/dl (normal 10.5–13.5), urine homovanillic acid (HVA) 54.7 mcg/mg Cr (normal b23.0), serum LDH N2500 units/L (normal 130–400), and serum uric acid 6.6 mg/dl (normal 2.0–6.0). The right lower lobe lung nodule was biopsied given the concern and risk for tumor rupture if nephrectomy was attempted (Fig. 2). Histology revealed metastatic

⁎ Corresponding author. Tel.: +1 314 268 5782. E-mail address: [email protected] (S.G. Farmakis). 1 Tel.: +1 314 454 6229. http://dx.doi.org/10.1016/j.jpedsurg.2014.10.043 0022-3468/© 2014 Elsevier Inc. All rights reserved.

neuroblastoma. Subsequently the patient underwent conventional studies for staging neuroblastoma and predicting prognosis. MYCN gene amplification was positive. An MIBG study revealed increased areas of activity in the pelvic bones, proximal femora, left humerus, and thoracolumbar spine consistent with osseous metastases and within the left kidney (Fig. 4). A bone marrow biopsy of the left iliac crest confirmed the presence of neuroblastoma. The patient received BuMed (busulfan and melphalan) followed by a stem cell transplant which resulted in tumor shrinkage and a reduction in the number of lung metastases documented on interim imaging studies. A left nephrectomy and adrenalectomy were performed. A normal left adrenal gland was identified embedded between residual tumor nodules. A 5-month follow-up CT showed further reduction in pulmonary metastases and no evidence of residual or recurrent abdominal tumor. 2. Discussion Neuroblastoma is the most common extracranial solid tumor of childhood. The prevalence is about one case per 7000 live births, and there are approximately 650 new cases of neuroblastoma annually in North America. Most children with neuroblastoma are between 1 and 5 years of age, with a median age at diagnosis of 19 months [1]. These tumors originate in neural crest cells and may occur anywhere along the sympathetic chain. Most arise in the adrenal medulla (40%) and abdomen (25%). The others may arise in the thorax (15%), cervical region (5%), and pelvis (5%) [2]. Intrarenal neuroblastoma is a very rare primary renal malignancy. In a series of 868 neuroblastoma cases in a 10-year span reported by Shamberger et al. [3], the incidence of renal neuroblastoma was 1% to

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Fig. 3. Longitudinal ultrasound image of the left kidney demonstrates the heterogeneous mass arising from within the renal parenchyma (arrows).

Fig. 1. Contrast-enhanced CT axial (a) and coronal (b) images of the abdomen pelvis. There is a large heterogeneously enhancing mass arising from within the left kidney. There was extracapsular extension. No intratumoral calcifications were seen. The mass crossed the midline and displaced the abdominal vessels rather than encasing them. The renal vein was not visible; however, no inferior vena cava invasion was present. The left adrenal gland was not able to be identified.

Fig. 2. Axial CT of the chest image displays one of the multiple bilateral solid pulmonary nodules (arrow) which were thought to represent Wilms tumor metastases. This nodule was the largest and was later biopsied revealing the diagnosis of neuroblastoma.

2%. It is hypothesized that these tumors originate from adrenal rests within the renal parenchyma or from intrarenal sympathetic ganglia [4,5]. The pathology classification system of neuroblastoma describes “favorable” and “unfavorable” histologies and the biologic behavior can be predicted by the International Neuroblastoma Pathology Committee (INPC, also called Shimada system). Intrarenal neuroblastomas are more likely to have unfavorable histology, characterized by increased anaplasia (32%) compared to those occurring in the adrenal glands [6]. Many intrarenal neuroblastomas have metastatic disease at presentation. Four out of five (80%) patients reported by Kessler et al. [4] had metastatic disease, either in bone and bone marrow or lymph nodes. Dickson et al. [7] reported that 3 out of 8 (37.5%) patients had bone marrow disease. This is compared to a reported metastases frequency of 60% in neuroblastoma to the bone, bone marrow, liver, lymph nodes, or skin [2]. Presenting findings of intrarenal neuroblastoma are not different from those of classic neuroblastoma. The tumors usually present as a firm, nontender, abdominal mass. Other findings include systemic manifestations such as fever, weight loss, anemia, bone pain. An important

Fig. 4. Posterior view from the MIBG study which shows increased activity in the periphery of the large left renal mass consistent with the primary neuroblastoma. Central decreased activity represents the intratumoral necrosis (*). Additional sites of abnormal activity are seen in the proximal left humerus, thoracolumbar spine, iliac bones, and proximal femora (arrows) consistent with osseous metastases. Normal activity is seen in the urinary bladder.

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clinical clue to the diagnosis is hypertension which has been reported in 66-100% of intrarenal neuroblastoma compared with 27% of extrarenal neuroblastoma [4,6]. The cause is thought to be tumor secretion of catecholamines and/or tumor compression of renal vessels and subsequent activation of the renin-angiotensin-aldosterone system [7–9]. However, this finding is not specific and hypertension also occurs in 20% of patients with Wilms tumors [2]. In patients with a renal mass and hypertension, assaying urine catecholamine levels (vanillylmandelic acid and homovanillic acid) may be helpful to assess the possibility of neuroblastoma as these levels have been have been reported to be increased in over 90% of neuroblastoma [7]. On imaging, intrarenal neuroblastoma mimics Wilms tumor. Both tumors appear as intrarenal soft tissue masses replacing most of the renal parenchyma. The presence of calcifications in intrarenal neuroblastoma is variable with Dickson et al. reporting calcifications in 67% of tumors (6 of 9 cases) and Kessler et al reporting calcifications in 40% (2 of 5 cases) [4,7]. Lung metastases in neuroblastoma at presentation are very rare (3.6%) and are more commonly seen in patients with MYCN amplified tumors, those with elevated LDH levels, or with an adrenal primary [10]. In conclusion, intrarenal neuroblastoma should be considered with the combination of a renal mass with hypertension, especially if calcifications are present. Distinction of this tumor from Wilms

tumor is crucial since both tumors have different prognostic and therapeutic responses. References [1] United States. National Cancer Institute. Neuroblastoma (PDQ®). http://www.cancer. gov/cancertopics/pdq/treatment/neuroblastoma/HealthProfessional/page1; 2014. [Accessed 5 June 2014]. [2] Wang LL, Yusten J, Louis C, et al. Solid tumors of childhood. In: DeVita, editor. DeVita, Hellman, and Rosenberg’s cancer: principles and practice of oncology. 9th ed. Wolters Kluwer Health/Lippincott Williams & Wilkins; 2011. p. 1760–92. [3] Shamberger RC, Smith EI, Joshi VV, et al. The risk of nephrectomy during local control in abdominal neuroblastoma. J Pediatr Surg 1998;33:161–4. [4] Kessler OJ, Siegel JF, Brock WA. Intrarenal neuroblastoma masquerading as Wilms' tumor. Urology 1998;51:313–6. [5] Sellaturay SV, Arya M, Banisadr S, et al. Primary intrarenal neuroblastoma: a rare, aggressive tumour of childhood mimicking Wilms' tumour. J Pediatr Urol 2006;2:522–4. [6] Shamsian BS, Kajizadi M, Rezaei N, et al. Primary intrarenal neuroblastoma with hypertension and disseminated intravascular coagulation. Case Rep Oncol Med 2013; 2013:1–4 [doi.org/10.1155]. [7] Dickson PV, Sims TL, Streck CJ, et al. Avoiding misdiagnosing neuroblastoma as Wilms tumor. J Pediatr Surg 2008;43:1159–63. [8] Lowe LH, Isuani BH, Heller RM, et al. Pediatric renal masses: Wilms tumor and beyond. Radiographics 2000;20:1585–603. [9] Garnier S, Maillet O, Haouy S, et al. Prenatal intrarenal neuroblastoma mimicking a mesoblastic nephroma: a case report. J Pediatr Surg 2012;47:E21–3. [10] DuBois SG, London WB, Zhang Y, et al. Lung metastases in neuroblastoma at initial diagnosis: a report from the international neuroblastoma risk group (INRG) project. Pediatr Blood Cancer 2008;51:589–92.

Intrarenal neuroblastoma with pulmonary metastases mimicking a Wilms tumor.

Primary intrarenal neuroblastoma is extremely rare and is a mimic of Wilms tumor. We present a case of a renal mass in a 14month old male with lung me...
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