Surgical Treatment for Pediatric Mediastinal Neurogenic Tumors

Surgical Unit of the Institute of Child Health and Great Ormond Street Hospital for Children, London, United Kingdom.
The Annals of thoracic surgery (Impact Factor: 3.65). 08/2010; 90(2):413-8. DOI: 10.1016/j.athoracsur.2010.04.086
Source: PubMed

ABSTRACT Mediastinal neurogenic tumors are relatively rare in children, and controversies persist about the best surgical approach and long-term survival.
With ethics approval we reviewed the case notes of 43 consecutive children (22 boys) operated on for mediastinal neurogenic tumor in two different institutions from March 1998 to June 2009.
The average age at diagnosis was 3 years (range, 0.6 to 4.7 years). Of the 43 operated tumors, 20 were neuroblastomas (47%), 13 were ganglioneuroblastomas (30%), and 10 were ganglioneuromas (23%). Only 6 (14%) of these tumors were discovered incidentally; the remaining 37 (86%) presented symptomatically: cough (30%), dyspnea (21%), wheezing (11.6%), neurologic spinal compression (7%), dancing eyes syndrome (7%), and Horner syndrome (7%). Tumor resection was undertaken by open operation in 38 cases (88%) and by thoracoscopy in 5 cases (12%). The children operated on by means of thoracoscopy had significantly smaller tumors (p = 0.01) and shorter duration of thoracic drain (p = 0.011) and hospitalization (p = 0.016) than those who were operated on by thoracotomy. There was not any surgical death. Postoperative complications occurred in 11 children (25.6%). Tumor recurrence occurred in 4 children (9.3%) operated on by open surgery, and there were 2 deaths of children with neuroblastoma. The overall survival was 95.4% in an average follow-up of 3.5 years (range, 0.7 to 4.4 years).
Surgical resection of children with mediastinal neurogenic tumors treated at two international tertiary hospitals was safe, and the thoracoscopic approach was appropriate for small tumors. The long-term survival was higher when compared with those reported for other primary neurogenic tumor locations.

  • Source
    [Show abstract] [Hide abstract]
    ABSTRACT: Cold abscess neck is a common disease entity in Indian subcontinent, however its association with Horner’s syndrome is rare. Our patient was a girl presenting with neck mass with loss of sweating on same side of face. Her investigations revealed a multiloculated minimally enhancing lesion arising from posterior cervical space and extending into mediastinum, provisional diagnosis of tubercular cold abscess was made and Anti Tubercular Therapy (ATT) was started. She did not show much improvement, so she was operated via left lateral thoracotomy and the mass was excised, post operatively she recovered well. There are several causes of Horner’s syndrome but its association with tubercular cold abscess neck is unique and very few case reports exist in English literature.
    Indian Journal of Thoracic and Cardiovascular Surgery 09/2013; DOI:10.1007/s12055-013-0224-2
  • [Show abstract] [Hide abstract]
    ABSTRACT: Peripheral primitive neuroectodermal tumors (pPNET) are an extremely rare disease entity of malignant tumors belonging to the Ewing sarcoma family that usually occur in children and adolescents with a predilection for the truncal and axial soft tissue. Thoracopulmonary manifestation in children mostly involves the chest wall as a large tumorous mass or the posterior mediastinum. In adults these tumors are even more infrequent. We report about a 46-year-old woman with a pPNET of the thymus which infiltrated the pericardium. According to the English literature this is the first reported case both of a thymic pPNET and a pPNET arising in the anterior mediastinum. This illustrates that pPNETs belong to the differential diagnosis of neurogenic mediastinal malignancies not only in the pediatric age population but also in adults and even in so far unknown localizations like the anterior mediastinum.
    Interactive Cardiovascular and Thoracic Surgery 11/2010; 12(2):303-5. DOI:10.1510/icvts.2010.254052 · 1.11 Impact Factor
  • [Show abstract] [Hide abstract]
    ABSTRACT: Thoracic ganglioneuroma is sporadic and rarely reported. Pre-operative misdiagnosis often occurs in clinical practice. To improve diagnostic accuracy and facilitate differential diagnosis, we summarised the CT and MRI findings of thoracic ganglioneuroma. 22 cases of thoracic ganglioneuroma confirmed by surgery and pathology were retrospectively analysed in terms of CT (16 cases) and MRI data (6 cases). Of 22 lesions, 19 occurred in the posterior mediastinum, 2 in the lateral pleura and 1 in the right chest. The CT value of the plain scans ranged from 20 to 40 HU (mean 29.1 HU) in 16 cases. Punctate calcification was noted in four cases. Patchy fat density shadow was found in one case. Arterial-phase CT found nearly no enhancement (6 cases) or slight enhancement (10 cases) with a CT value of 0-12 HU (mean 5.8 HU). In the delayed phase, enhancement was strengthened progressively, and CT value of 10-20 HU (mean 13.6 HU) was achieved after 120 s. T(1) weighted images showed homogeneous hypointense signals in five cases and hypointense signals mixed with patchy hyperintense signal shadow in one case. T(2) weighted images demonstrated heterogeneous hyperintense signals in all six cases, of which the whorled appearance was noted in one case. Gadolinium-diethylenetriaminepentaacetic acid (Gd-DTPA)-enhanced MRI found mildly heterogeneous enhancement in the arterial phase, and progressive mild enhancement in the delayed phase. Thoracic ganglioneuroma shows hypodensity in plain CT. On CT and MRI, non-enhancement or slight enhancement in artery phase and progressive mild enhancement in delay phase are characteristic manifestations of ganglioneuroma in the thorax.
    The British journal of radiology 05/2012; 85(1016):e365-72. DOI:10.1259/bjr/53395088 · 2.02 Impact Factor
Show more