A Case of Congenital Orbital Malignant Rhabdoid Tumor: Systemic Metastasis following Exenteration

Kook, Koung Hoon; Park, Moon Sung; Yim, Hyunee; Lee, Sang Yeul; Jang, Jae Woo; Grossniklaus, Hans E.

doi:10.1159/000213643

Cited by 18 publications

(13 citation statements)

References 18 publications

(10 reference statements)

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“…Ranging from parapharyngeal to orbital to paratesticular, extrarenal MRTs present a diagnostic challenge due to their widely variable presentation and morphological features. 9 A case study of a newborn with a thigh mass, found to be MRT, showed poor response to chemotherapy, with progressive dissemination and a survival of 70 days. 4 A small number of cases of extrarenal MRT diagnosed in utero have been reported.…”

Section: Discussionmentioning

confidence: 99%

Neonate With Paraplegia

Stolten

Betrand²,

Lotterman

et al. 2015

Clin Pediatr (Phila)

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A newborn female presented with bilateral lower limb paralysis and anuria. Physical exam was notable for a mass on the right flank as well as the previously mentioned paraplegia. No facial defects or evidence of limb deformities were present. Both the course of pregnancy and delivery were unremarkable. Ultrasound performed at 18 weeks was normal by history.Spinal magnetic resonance imaging (MRI) demonstrated a heterogeneous retroperitoneal mass. The mass was inferior to the right kidney, displacing it superiorly. Both the inferior vena cava and the aorta were displaced anteriorly. The mass extended into the right paraspinous region and spinal canal, entering at L2-3 and L3-4 right neural foramina. It extended superior to T4 and inferiorly to L5-S1. C6 enhancement may have represented skip epidural metastasis. The lesion displaced the thecal sac/spinal cord from T6-T10. T10-L2 vertebral body had no identifiable spinal cord. No other metastatic foci were detected. The newborn also had bilateral hydronephrosis, which was more extensive on the right side (see Figures 1 and 2).Bone marrow aspirate, urine vanillylmandelic acid and homovanillic acid, metaiodobenzylguanidine scan, and a bone scan were all negative. Needle biopsy of the mass was performed and neuroblastoma or other small cell tumors were considered. Slides from the needle biopsy were sent to the Mayo Clinic for review. Morphological and immunohistology studies at Mayo confirmed the diagnosis of malignant rhabdoid tumor.

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Section: Discussionmentioning

confidence: 99%

Neonate With Paraplegia

Stolten

Betrand²,

Lotterman

et al. 2015

Clin Pediatr (Phila)

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“…Since their initial description in the kidney, MRT occurring in extrarenal locations have been reported in the heart, 63 liver, 52 thymus, 42 brain, 7,61 prostate, 13 soft tissues, 51,66 pelvis, 18 vulva, 54 bladder, 25 skin 11 and orbit. 21,23,39,46,48,57,61,65,68 Composite tumors may be encountered in many mesenchymal soft tissue (Table 2). The vast majority of noncomposite orbital examples of MRT have been reported in children.…”

Section: Emergence Of Rhabdoid Phenotypementioning

confidence: 99%

“…The vast majority of noncomposite orbital examples of MRT have been reported in children. 21,23,39,57,65,68 The literature contains only two prior descriptions of noncomposite rhabdoid tumors in the adult orbit: one in the lacrimal gland 48 and the other in the intraconal space. 37 We know of no previous instance of a benign orbital tumor ultimately manifesting an emergent malignant rhabdoid clone (composite tumor).…”

Section: Emergence Of Rhabdoid Phenotypementioning

confidence: 99%

Malignant rhabdoid transformation of a longstanding, aggressive, and recurrent orbital angiomyxoma

Callahan

Stagner

Lee

et al. 2015

Survey of Ophthalmology

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“…Cerrahi tedavide negatif sınır sağlanabilmesi hedeflenir ancak enükleasyon ve egzanterasyon ilk planda tercih edilecek bir yöntem değildir. Literatürdeki olguların dördünde tümörün kontrol edilemeyen büyümesi, birinde ise hastanın daha önce retinoblastom tanısı ile enükleasyon geçirmiş olması nedeniyle egzanterasyon yapılmıştır (8,(10)(11)(12)14). Hastamızda ilk yapılan ameliyat ile negatif cerrahi sınır sağlanamamış, ameliyat sonrası iki haftalık dönemde lokal nüks saptanarak kemoterapi başlanmıştır.…”

Section: A B Cunclassified

Malignant rhabdoid tumor of the orbit

Karaman¹,

Çelkan²,

Dervışoğlu³

et al. 2013

TJPATH

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Malignant rhabdoid tumor is a rare and malignant tumor of childhood. Generally it originates from the kidney and central nervous system, but occasionally it may arise from the orbit. Differential diagnosis from other childhood soft tissue sarcomas should be done. We report here an 8-week-old female infant with malignant rhabdoid tumor of the orbit who was treated with chemotherapy and surgery. The 8 week-old girl was referred to our hospital with a history of right proptosis first noted at birth. Physical and laboratory evaluation of the patient was normal except for right proptosis. The mass was removed surgically. Histopathologic examination and immunohistochemical findings of the specimen were evaluated as malignant rhabdoid tumor. Chemotherapy was administered. While in clinical remission, she succumbed during a febrile episode. Malignant rhabdoid tumor can rarely originate from the orbit. Malignant rhabdoid tumor should be kept in mind in the differential diagnosis of orbital masses, and surgery, chemotherapy and local radiotherapy should be used as combined therapy due to the poor prognosis. Key Words: Rhabdoid tumor, Orbita, Child ÖZMalign rabdoid tümör, çocukluk döneminin nadir rastlanan malign bir tümörüdür. En sık yerleşim yeri santral sinir sistemi ve böbrektir. Ancak nadir olarak orbitadan da kaynaklanabilir. Çocukluk çağının diğer yumuşak doku sarkomları ile ayırıcı tanısı yapılmalıdır. Bu yazıda, perinatal dönemde orbital malign rabdoid tümör tanısı ile cerrahi ve kemoterapi uygulanan 8 haftalık bir kız olgu sunulmuştur. Sekiz haftalık olgu, doğumdan itibaren fark edilen sağ göz kapağında şişlik şikayeti ile hastanemize getirildi. Sağ proptozisi dışında fizik muayene ve laboratuvar değerlendirmesi normaldi. Kitle cerrahi olarak çıkartıldı. Örnek, histopatolojik ve immünohistokimyasal inceleme sonucu malign rabdoid tümör olarak değerlendirildi ve kemoterapi verildi. Olgu klinik olarak remisyonda iken, febril nötropeni atağı ile kaybedildi. Malign rabdoid tümör, nadir de olsa gözden kaynaklanabilir. Göz kitlelerinin ayırıcı tanısında düşünülmeli ve kötü gidişli bir tümör olduğundan cerrahi, kemoterapi ve lokal radyoterapi gibi tedavi seçenekleri kombine olarak kullanılmalıdır.

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A Case of Congenital Orbital Malignant Rhabdoid Tumor: Systemic Metastasis following Exenteration

Cited by 18 publications

References 18 publications

Neonate With Paraplegia

Neonate With Paraplegia

Malignant rhabdoid transformation of a longstanding, aggressive, and recurrent orbital angiomyxoma

Malignant rhabdoid tumor of the orbit

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