Harlequin syndrome associated with ganglioneuroblastoma-induced Horner syndrome
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CASE REPORT
Harlequin syndrome associated with ganglioneuroblastoma-induced Horner syndrome Kenji Miyata 1
&
Mitsuko Akaihata 1 & Yasuto Shimomura 1 & Toshinori Hori 1 & Kenitiro Kaneko 2 & Akihisa Okumura 1
Received: 16 September 2020 / Accepted: 26 October 2020 # Springer-Verlag GmbH Germany, part of Springer Nature 2020
Abstract A 1-year-old boy presented with a 4-month history of hypertension, ptosis of the right upper eyelid, left hemifacial sweating, and flushing. He was diagnosed with Harlequin syndrome associated with Horner syndrome. Computed tomography revealed a mass lesion in the right superior mediastinum. Therefore, the patient underwent total tumor resection. Histological examination demonstrated ganglioneuroblastoma. The MYCN oncogene was not amplified, and the mitosis–karyorrhexis index was low. Accordingly, radiation and chemotherapy were not performed. No recurrence was observed within 8 months after surgery, and the patient’s blood pressure was normalized. However, the ptosis, hemifacial sweating, and flushing persisted. Keywords Horner syndrome . Harlequin syndrome . Ganglioneuroblastoma . Neuroblastoma
Introduction Ganglioneuroblastoma (GNB) is a rare malignant tumor of the adrenal gland or sympathetic nervous system in children. GNB has histological features of both ganglioneuroma and neuroblastoma, along with the potential for malignant behavior [1]. Treatments for GNB include surgery, chemotherapy, and radiotherapy. Treatment approaches are determined on the basis of tumor stage, biological prognostic factors, and image-defined risk factors [1, 2]. Total resection with negative margins and without complications is often difficult, but residual tumor progression is rare in patients without advanced or metastatic GNB [3]. Thus, the prognosis is generally favorable in patients with GNB [4]. Lance et al. first reported Harlequin syndrome, reporting unilateral facial flushing and sweating due to dysfunctional vasomotor and sudomotor sympathetic innervations of the face, which arise from the Th2–3 and Th1 levels of the spinal cord, respectively [5]. Harlequin syndrome in children results primarily from surgical injury [6–8]. Horner syndrome is * Kenji Miyata [email protected] 1
Department of Pediatrics, Aichi Medical University, School of Medicine, 1-1 Yazakokarimata, Nagakute, Aichi 480-1195, Japan
2
Department of Gastrointestinal Surgery, Aichi Medical University, School of Medicine, Nagakute, Japan
characterized by the clinical triad of ptosis, miosis, and facial anhidrosis, which occur following interruption of the oculosympathetic pathway. Horner syndrome can be induced by injury to any part of the oculosympathetic pathway, most often by lesions in sympathetic nerves arising from the level of C8 to the level of Th2 in the spinal cord. Horner syndrome in children can arise from congenital or acquired causes. Notably, neoplasms can cause acquired Horner syndrome [9, 10]. The co-occurrence of Harlequin and Horner syndromes has rarely been reported in children. He
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