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A diffuse scalp neurofibroma in a 4-year-old child

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1. Title Title of document A diffuse scalp neurofibroma in a 4-year-old child
2. Creator Author's name, affiliation, country Iryna S. Kletskaya; N.I. Pirogov Russian National Research Medical University
; Россия
2. Creator Author's name, affiliation, country Polina A. Serebrennikova; N.I. Pirogov Russian National Research Medical University; Россия
2. Creator Author's name, affiliation, country Tatiana A. Gassan; N.I. Pirogov Russian National Research Medical University; Россия
2. Creator Author's name, affiliation, country Anton G. Narbutov; N.I. Pirogov Russian National Research Medical University
; Россия
2. Creator Author's name, affiliation, country Olga V. Makarova; N.I. Pirogov Russian National Research Medical University
; Россия
3. Subject Discipline(s)
3. Subject Keyword(s) pediatric surgery; dermatology; plastic surgery; case reports; diffuse neurofibroma; meissnerian neurofibroma; tactile corpuscles; tumors; endoexpander
4. Description Abstract

BACKGROUND: Structures resembling Meissner's tactile corpuscles have been revealed in various peripheral nerve tumors, including schwannomas and neurofibromas, as well as in congenital melanocytic nevi and neural crest hamartomas. As a rule, Meissner's tactile corpuscles are few in number, but in rare cases they represent the predominant component of the formation tissue.

CLINICAL CASE DESCRIPTION: The authors describe a case of their observation: a 4 y.o. patient with diffuse neurofibroma consisting of structures resembling tactile corpuscles. Clinically, this fibroma looked like a defect in the occipital soft tissue region with excessive folding and alopecia. At the histological examination, the dermis and subcutaneous adipose tissue were seen involved in the pathological process. The formation tissue consisted mostly of "neuroid" structures resembling tactile corpuscles located among fibroblast-like cells with mast cell admixture in the infiltrate. An immunohistochemical examination revealed expression of S100 in them, EMA, GLUT1 in the perineuria, and CD34 in the stroma. Melanocytic marker (MelanA, HMB45) expression was not detected.

CONCLUSION: The case presented by the authors is of interest to professionals due to the rarity of this morphological variant, difficulties of clinical and histological differential diagnostics with other tumors and tumor-like processes in children.

5. Publisher Organizing agency, location Eco-Vector
6. Contributor Sponsor(s)
7. Date (DD-MM-YYYY) 09.09.2024
8. Type Status & genre Peer-reviewed Article
8. Type Type Research Article
9. Format File format PDF (Rus)
10. Identifier Uniform Resource Identifier https://jps-nmp.ru/jour/article/view/706
10. Identifier Digital Object Identifier (DOI) 10.17816/ps706
10. Identifier Digital Object Identifier (DOI) (PDF (Rus)) 10.17816/ps706-625
11. Source Title; vol., no. (year) Russian Journal of Pediatric Surgery; Vol 28, No 4 (2024)
12. Language English=en ru
13. Relation Supp. Files Fig. 1. View of the patient at the age of 1 month. (558KB) doi: 10.17816/ps706-1101
Fig. 2. View of the patient at the age of 6 months. (555KB) doi: 10.17816/ps706-1102
Fig. 3. View of the patient after the first stage of surgical treatment — expander dermatension. (648KB) doi: 10.17816/ps706-1103
Fig. 4. Expression of pancytokeratin in the epidermis and skin appendages: a — skin area with numerous concentric structures in the dermis and subcutaneous fat (hematoxylin and eosin staining, magnification ×40); b — numerous structures resembling tactile Meissner corpuscles (hematoxylin and eosin staining, magnification ×200); c — expression of S100 in “neuroid” structures (immunohistochemical staining, magnification ×250); d — EMA expression in the perineurium surrounding the “neuroid” structures (immunohistochemical study, magnification ×350); e — expression of CD34 in the stroma with the formation of a pattern resembling a “fingerprint” (immunohistochemical staining, magnification ×250); f — expression of HMB45 in melanocytes of the basal layer of the epidermis, melanocytes number is not increased (immunohistochemical staining, magnification ×350). (1MB) doi: 10.17816/ps706-1104
Fig. 5. View of the patient on the 7th postoperative day after the second stage of treatment. (765KB) doi: 10.17816/ps706-1105
Fig. 6. Patient in 6 months after the complete course of treatment. (755KB) doi: 10.17816/ps706-1106
14. Coverage Geo-spatial location, chronological period, research sample (gender, age, etc.)
15. Rights Copyright and permissions Copyright (c) 2024 Kletskaya I.S., Serebrennikova P.A., Gassan T.A., Narbutov A.G., Makarova O.V.