{"title":"孤立的霍纳综合征是儿童咽旁横纹肌肉瘤的唯一表现。","authors":"Molham Alahmad, Ahmad Alkheder, Arige Alassaf","doi":"10.1177/01455613251352732","DOIUrl":null,"url":null,"abstract":"<p><p>Isolated Horner syndrome (HS) in young children warrants thorough investigation for occult malignancy, despite its rarity as a presenting sign. This report details a unique case of a 30 month-old male presenting with sudden-onset, isolated left HS as the sole manifestation of rhabdomyosarcoma (RMS) in parapharyngeal space. Contrast-enhanced magnetic resonance imaging (MRI) revealed a 2.5 × 3.5 × 5 cm lesion within the left poststyloid parapharyngeal space, displacing the carotid artery anteriorly, and the internal jugular vein posteriorly. Gross total resection via a transcervical approach was performed; intraoperatively, the tumor involved the cervical sympathetic trunk, necessitating its sacrifice, resulting in persistent postoperative HS. Histopathological and immunohistochemical analysis (positive CK, PLAP, CD99) confirmed a diagnosis of embryonal RMS. The child received adjuvant chemoradiotherapy. One-year follow-up with positron emission tomography - computed tomography (PET-CT) showed no evidence of recurrence. This case underscores several critical points: (1) Isolated HS in young children necessitates urgent neuroimaging to exclude malignancy, even in the absence of palpable masses or neurological deficits; (2) The parapharyngeal space, though an exceptionally rare primary site for RMS (constituting a minor subset of head and neck cases), carries significant implications due to its proximity to the sympathetic chain, readily causing HS via mass effect; (3) MRI is pivotal for diagnosis and surgical planning in this anatomically complex region; (4) Multimodal therapy is essential for local control in embryonal RMS; (5) Sacrifice of the sympathetic trunk during resection likely leads to permanent HS, a factor for preoperative counseling. Vigilant long-term follow-up remains crucial.</p>","PeriodicalId":93984,"journal":{"name":"Ear, nose, & throat journal","volume":" ","pages":"1455613251352732"},"PeriodicalIF":0.0000,"publicationDate":"2025-06-25","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":"0","resultStr":"{\"title\":\"Isolated Horner Syndrome as the Sole Presentation of Pediatric Parapharyngeal Rhabdomyosarcoma.\",\"authors\":\"Molham Alahmad, Ahmad Alkheder, Arige Alassaf\",\"doi\":\"10.1177/01455613251352732\",\"DOIUrl\":null,\"url\":null,\"abstract\":\"<p><p>Isolated Horner syndrome (HS) in young children warrants thorough investigation for occult malignancy, despite its rarity as a presenting sign. This report details a unique case of a 30 month-old male presenting with sudden-onset, isolated left HS as the sole manifestation of rhabdomyosarcoma (RMS) in parapharyngeal space. Contrast-enhanced magnetic resonance imaging (MRI) revealed a 2.5 × 3.5 × 5 cm lesion within the left poststyloid parapharyngeal space, displacing the carotid artery anteriorly, and the internal jugular vein posteriorly. Gross total resection via a transcervical approach was performed; intraoperatively, the tumor involved the cervical sympathetic trunk, necessitating its sacrifice, resulting in persistent postoperative HS. Histopathological and immunohistochemical analysis (positive CK, PLAP, CD99) confirmed a diagnosis of embryonal RMS. The child received adjuvant chemoradiotherapy. One-year follow-up with positron emission tomography - computed tomography (PET-CT) showed no evidence of recurrence. This case underscores several critical points: (1) Isolated HS in young children necessitates urgent neuroimaging to exclude malignancy, even in the absence of palpable masses or neurological deficits; (2) The parapharyngeal space, though an exceptionally rare primary site for RMS (constituting a minor subset of head and neck cases), carries significant implications due to its proximity to the sympathetic chain, readily causing HS via mass effect; (3) MRI is pivotal for diagnosis and surgical planning in this anatomically complex region; (4) Multimodal therapy is essential for local control in embryonal RMS; (5) Sacrifice of the sympathetic trunk during resection likely leads to permanent HS, a factor for preoperative counseling. Vigilant long-term follow-up remains crucial.</p>\",\"PeriodicalId\":93984,\"journal\":{\"name\":\"Ear, nose, & throat journal\",\"volume\":\" \",\"pages\":\"1455613251352732\"},\"PeriodicalIF\":0.0000,\"publicationDate\":\"2025-06-25\",\"publicationTypes\":\"Journal Article\",\"fieldsOfStudy\":null,\"isOpenAccess\":false,\"openAccessPdf\":\"\",\"citationCount\":\"0\",\"resultStr\":null,\"platform\":\"Semanticscholar\",\"paperid\":null,\"PeriodicalName\":\"Ear, nose, & throat journal\",\"FirstCategoryId\":\"1085\",\"ListUrlMain\":\"https://doi.org/10.1177/01455613251352732\",\"RegionNum\":0,\"RegionCategory\":null,\"ArticlePicture\":[],\"TitleCN\":null,\"AbstractTextCN\":null,\"PMCID\":null,\"EPubDate\":\"\",\"PubModel\":\"\",\"JCR\":\"\",\"JCRName\":\"\",\"Score\":null,\"Total\":0}","platform":"Semanticscholar","paperid":null,"PeriodicalName":"Ear, nose, & throat journal","FirstCategoryId":"1085","ListUrlMain":"https://doi.org/10.1177/01455613251352732","RegionNum":0,"RegionCategory":null,"ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":null,"EPubDate":"","PubModel":"","JCR":"","JCRName":"","Score":null,"Total":0}
Isolated Horner Syndrome as the Sole Presentation of Pediatric Parapharyngeal Rhabdomyosarcoma.
Isolated Horner syndrome (HS) in young children warrants thorough investigation for occult malignancy, despite its rarity as a presenting sign. This report details a unique case of a 30 month-old male presenting with sudden-onset, isolated left HS as the sole manifestation of rhabdomyosarcoma (RMS) in parapharyngeal space. Contrast-enhanced magnetic resonance imaging (MRI) revealed a 2.5 × 3.5 × 5 cm lesion within the left poststyloid parapharyngeal space, displacing the carotid artery anteriorly, and the internal jugular vein posteriorly. Gross total resection via a transcervical approach was performed; intraoperatively, the tumor involved the cervical sympathetic trunk, necessitating its sacrifice, resulting in persistent postoperative HS. Histopathological and immunohistochemical analysis (positive CK, PLAP, CD99) confirmed a diagnosis of embryonal RMS. The child received adjuvant chemoradiotherapy. One-year follow-up with positron emission tomography - computed tomography (PET-CT) showed no evidence of recurrence. This case underscores several critical points: (1) Isolated HS in young children necessitates urgent neuroimaging to exclude malignancy, even in the absence of palpable masses or neurological deficits; (2) The parapharyngeal space, though an exceptionally rare primary site for RMS (constituting a minor subset of head and neck cases), carries significant implications due to its proximity to the sympathetic chain, readily causing HS via mass effect; (3) MRI is pivotal for diagnosis and surgical planning in this anatomically complex region; (4) Multimodal therapy is essential for local control in embryonal RMS; (5) Sacrifice of the sympathetic trunk during resection likely leads to permanent HS, a factor for preoperative counseling. Vigilant long-term follow-up remains crucial.