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Year : 2016  |  Volume : 12  |  Issue : 1  |  Page : 430-431

Infantile rhabdoid tumor mimicking hepatic hemangioendothelioma

1 Department of Pediatric Oncology and Pediatric, Bone Marrow Transplantation Unit, Çukurova University, Adana, Turkey
2 Department of Neonatology, Faculty of Medicine, Çukurova University, Adana, Turkey
3 Department of Pathology, Faculty of Medicine, Çukurova University, Adana, Turkey
4 Department of Radiology, Faculty of Medicine, Çukurova University, Adana, Turkey

Date of Web Publication13-Apr-2016

Correspondence Address:
Serhan Kupeli
Department of Pediatric Oncology and Pediatric, Bone Marrow Transplantation Unit, Faculty of Medicine, Çukurova University, Adana
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/0973-1482.172138

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How to cite this article:
Kupeli S, Yöntem A, Narli N, Doran F, Soyupak S. Infantile rhabdoid tumor mimicking hepatic hemangioendothelioma. J Can Res Ther 2016;12:430-1

How to cite this URL:
Kupeli S, Yöntem A, Narli N, Doran F, Soyupak S. Infantile rhabdoid tumor mimicking hepatic hemangioendothelioma. J Can Res Ther [serial online] 2016 [cited 2021 Jan 24];12:430-1. Available from: https://www.cancerjournal.net/text.asp?2016/12/1/430/172138


Malignant rhabdoid tumors are aggressive and often fatal malignancies of childhood, mainly occurring in the kidney and brain.[1] They are high-grade neoplasms of the central nervous system (CNS), kidneys, and soft tissue.[2] Rhabdoid tumor of the kidney (RTK) is unique among childhood renal neoplasms in its frequent association with primary or metastatic CNS lesions.[3] Infantile hepatic hemangioendothelioma (IHH) is among the most common hepatic tumors in children.[4] Here, we report a newborn with an infantile rhabdoid tumor, mimicking hepatic hemangioendothelioma radiologically and causing diagnostic challenge.

A male infant of 38 weeks' gestational age was born to a 35-year-old primigravid woman by cesarean section. The abdomen was distended, and liver was palpated 6 cm subcostally at the midclavicular line with an extension to the left part of the abdomen. Ultrasonography of abdomen showed heterogeneous, predominantly cystic mass in the left lobe of the liver which was compatible with cavernous hemangioma or hemangioendothelioma. Propranolol 1 mg/kg/day in two doses was started for hemangiomatous lesions on day 8 postnatally. Progressive enlargement of the mass, an extension to the pelvis, and new lesions in the right lobe of the liver was noticed in follow-up. Contrast-enhanced computed tomography (CT) determined hepatic, perihepatic, and retroperitoneal spread without bone involvement on day 16 [Figure 1]a. The patient was evaluated to be unsuitable for surgery. Other system examinations were normal. Cranial magnetic resonance imaging showed a solid mass measured 22 mm × 16 mm in posterior fossa [Figure 1]b. There was a deterioration in follow-up. A Tru-Cut biopsy was performed, and pathologic diagnosis was consistent with rhabdoid tumor [Figure 2]. Immunohistochemical studies showed CK19, Pancreatin, EMA, S100, and Desmin positivity with INI-1 negativity. On the 40th day of hospitalization, chemotherapy protocol consisting of vincristine, actinomycin-D, and cyclophosphamide was started. Four days after the first cycle of chemotherapy, the patient died due to sepsis and multiorgan failure.
Figure 1: Hepatic, perihepatic, and retroperitoneal involvement in abdominal computerized tomography (a). Solid mass in posterior fossa in T2 weighted axial magnetic resonance imaging (b)

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Figure 2: Pathologic appearance of the tumor taken from liver by Tru-Cut biopsy

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IHH is the third most common hepatic tumor in children, the most common benign vascular tumor of the liver in infancy, and the most common symptomatic liver tumor during the first 6 months of life.[4] Because the prevalence of RTK is relatively low, and because the CT findings are not pathognomonic, a renal mass seen on CT in a child is unlikely to represent RTK regardless of its CT features. It has been proposed that if CT could accurately predict the correct diagnosis of RTK preoperatively, then imaging protocols should include imaging of the brain.[3]

Corticosteroids have long been the mainstay treatment for hemangiomatous lesions, but after successful use of propranolol on severe hemangiomas in 2008, propranolol has been the preferred choice of treatment in many centers.[5] Propranolol, a beta-blocking agent, was started with an intension to treat the giant lesion in liver, but no regression was seen on propranolol treatment. Only after biopsy and pathologic examination, the correct diagnosis could be made. In case of progressive hepatic mass in the perinatal period, clinicians must be aware of possible rhabdoid tumors and abdominal and cranial imaging should be done to prevent delay in diagnosis.

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Conflicts of interest

There are no conflicts of interest.

 > References Top

Isaacs H Jr. Fetal and neonatal rhabdoid tumor. J Pediatr Surg 2010;45:619-26.  Back to cited text no. 1
Sultan I, Qaddoumi I, Rodríguez-Galindo C, Nassan AA, Ghandour K, Al-Hussaini M. Age, stage, and radiotherapy, but not primary tumor site, affects the outcome of patients with malignant rhabdoid tumors. Pediatr Blood Cancer 2010;54:35-40.  Back to cited text no. 2
Agrons GA, Kingsman KD, Wagner BJ, Sotelo-Avila C. Rhabdoid tumor of the kidney in children: A comparative study of 21 cases. AJR Am J Roentgenol 1997;168:447-51.  Back to cited text no. 3
Avagyan S, Klein M, Kerkar N, Demattia A, Blei F, Lee S, et al. Propranolol as a first-line treatment for diffuse infantile hepatic hemangioendothelioma. J Pediatr Gastroenterol Nutr 2013;56:e17-20.  Back to cited text no. 4
Küpeli S. Use of propranolol for infantile hemangiomas. Pediatr Hematol Oncol 2012;29:293-8.  Back to cited text no. 5


  [Figure 1], [Figure 2]


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