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Órgano Oficial del Instituto Nacional de Pediatría
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2024, Number 3

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Acta Pediatr Mex 2024; 45 (3)

Giant congenital melanocytic nevus with cutaneous Ganglioneuroma with adipocytic metaplasia. Divergency in neural crest stem cell–derived tumors

Hernández AD, Díaz MR, Santiago MMÁ, Echeverria VL, Lara BM, Cruz GE, Pérez ME, Hernández PA, Rodríguez JR
Full text How to cite this article

Language: Spanish
References: 13
Page: 223-228
PDF size: 296.13 Kb.


Key words:

Melanocytic congenital nevus, tumor, ganglioneuroma, Neuroblastic tumors, Neural crest cells.

ABSTRACT

Background: Neuroblastic tumors derive from neural crest cells, their biological behavior is related to the degree of differentiation, of them, ganglioneuroma has a benign spectrum due to its complete differentiation, it occurs mainly in the retroperitoneum and mediastinum, very few have been reported. cases in the skin and less within a congenital melanocytic nevus.
Clinical case: 11-month-old female, who presented from birth dermatosis on the back, posterior neck and occipital region, dark brown, velvety and hypertrichosis, congenital melanocytic nevus; in addition to a tumor in the posterior neck, pedunculated, soft, mobile, spherical,11x8 cm in diameter, cutaneous ganglioneuroma. MRI with evidence of soft tissue dorsal cervical-thoracic tumor without compromise of the vertebral column or spinal canal, complete resection was performed with an integral capsule of the tumor, it evolves during the postoperative period without complications, being discharged after 24 hours, with a histopathological report of ganglioneuroma, currently under surveillance, with no evidence of lesion.
Conclusions: Ganglioneuroma is not usually expressed in the skin or in a substrate with dermal lesions, although it is rare, cases of ganglioneuroma associated with melanocytic nevi have been reported, complete resection is the main treatment, adequate follow-up coupled with histopathological diagnosis is the key to success. Schwann cells precursors of neural crest are responsible of divergent differentiation, including melanocytic cells and pigmentation disorders.


REFERENCES

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Acta Pediatr Mex. 2024;45