Ectopic cervical thymus associated with infant death: 2 case reports and literature review

Ishida, Toshiki; Kotani, Hirokazu; Miyao, Masashi; Abiru, Hitoshi; Kawai, Chihiro; Osamura, Toshio; Tamaki, Keiji

doi:10.1016/j.ijporl.2013.06.030

Cited by 19 publications

(20 citation statements)

References 33 publications

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“…There have been case reports of catastrophic complications attributed to ECT, including death due to asphyxiation in young infant . Central neck lesions lie in close proximity to the trachea, so it is possible that expansion of these lesions could lead to compressive symptoms.…”

Section: Discussionmentioning

confidence: 99%

Ectopic cervical thymus in children: Clinical and radiographic features

et al. 2019

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Objectives: Ectopic thymus is rare and can be a diagnostic challenge. This study evaluated the management of children radiographically diagnosed with ectopic cervical thymus.Methods: A retrospective review of 100 patients was performed. Data related to clinical presentation, radiological imaging, pathology, and management were collected. Changes in lesion volume were tracked over time. Clinical characteristics were compared based on lesion location in the neck using analysis of variance modelling.Results: There were 115 lesions with radiographic features of ectopic cervical thymus (15 children had bilateral lesions). Diagnosis was based on ultrasound in 98% of patients, magnetic resonance imaging in 18%, and computed tomography in 11%. Mean (SD) follow-up duration was 2 (2.2) years. Forty-four percent (51/115) of lesions involved the thyroid gland, 29% (33/115) were in the central neck but separate from the thyroid, 18% (21/115) had mediastinal extension, and 8% (9/115) involved the submandibular region. Location was unclear for two patients. Submandibular lesions were on average 12.4 cm 3 larger (95% CI, 8.2, 16.6) than mediastinal lesions at diagnosis, P ≤ .001. Volume of thymic tissue decreased over time, from a mean (standard deviation [SD]) volume of 4.3 cm 3 (9.2) at initial ultrasound to 2.7 cm 3 (6.1) at final ultrasound (paired t-test, P = .008). Only two patients required surgery: one for compressive symptoms, and the other to rule out malignancy.Conclusion: Ninety-eight percent of children with ectopic cervical thymus were managed conservatively without issues. We propose a classification system based on location to ease communication among clinicians and to help follow these lesions over time.

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Section: Discussionmentioning

confidence: 99%

Ectopic cervical thymus in children: Clinical and radiographic features

et al. 2019

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“…Moreover, paracrine signals emanating from the thymus have the potential to modulate the development of nearby organs, since morphogenic defects were observed not only for the thymus and parathyroid, but also the thyroid when the secreted ligand Wnt4 was overexpressed by TECs. Interestingly, ectopic thymus and thyroid tissue is occasionally observed in humans, and while typically benign, such ectopic tissue can in some instances be detrimental 55, 56 . Cervical thymus tissue has been detected in wild type mice 57 , with the frequency, position and number of ectopic thymic lobes differing among different strains, as well as between individuals within a given strain 57, 58 .…”

Section: Discussionmentioning

confidence: 99%

Elevated levels of Wnt signaling disrupt thymus morphogenesis and function

Swann

Happe

Boehm

2017

Sci Rep

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All vertebrates possess a thymus, whose epithelial microenvironment is essential for T cell development and maturation. Despite the importance of the thymus for cellular immune defense, many questions surrounding its morphogenesis remain unanswered. Here, we demonstrate that, in contrast to the situation in many other epithelial cell types, differentiation of thymic epithelial cells (TECs) proceeds normally in the absence of canonical Wnt signaling and the classical adhesion molecule E-cadherin. By contrast, TEC-intrinsic activation of β-catenin-dependent Wnt signaling blocks the morphogenesis of the thymus, and overexpression of a secreted Wnt ligand by TECs dominantly modifies the morphogenesis not only of the thymus, but also of the parathyroid and thyroid. These observations indicate that Wnt signaling activity in the thymus needs to be precisely controlled to support normal TEC differentiation, and suggest possible mechanisms underlying anatomical variations of the thymus, parathyroid and thyroid in humans.

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“…Ectopic thymus can be located anywhere along the developmental pathway of thymic descent through the neck to the upper mediastinum (1)(2)(3)(4). Although ectopic intrathyroidal thymus has recently been reported in children, it is thought to be uncommon and can be misdiagnosed as a thyroid tumor, resulting in unnecessary surgery (5)(6)(7)(8)(9)(10)(11).…”

Section: Introductionmentioning

confidence: 99%