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

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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.

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作者简介

Iryna Kletskaya

N.I. Pirogov Russian National Research Medical University

编辑信件的主要联系方式.
Email: ikletskaya@gmail.com
ORCID iD: 0000-0002-8552-7682
SPIN 代码: 1046-3870

MD

俄罗斯联邦, 117 Leninskiy avenue, 119571 Moscow

Polina Serebrennikova

N.I. Pirogov Russian National Research Medical University

Email: blackbutlers@yandex.ru
ORCID iD: 0000-0002-9317-1671
SPIN 代码: 7630-4720

MD

俄罗斯联邦, 117 Leninskiy avenue, 119571 Moscow

Tatiana Gassan

N.I. Pirogov Russian National Research Medical University

Email: tatgassan@mail.ru
ORCID iD: 0000-0001-7322-2892

MD, Dr. Sci. (Medicine), Professor

俄罗斯联邦, 117 Leninskiy avenue, 119571 Moscow

Anton Narbutov

N.I. Pirogov Russian National Research Medical University

Email: anarbutov@mail.ru
ORCID iD: 0000-0003-0168-8671

MD, Cand. Sci. (Medicine)

俄罗斯联邦, 117 Leninskiy avenue, 119571 Moscow

Olga Makarova

N.I. Pirogov Russian National Research Medical University

Email: makarovao77@mail.ru
ORCID iD: 0009-0002-7497-3502

MD

俄罗斯联邦, 117 Leninskiy avenue, 119571 Moscow

参考

  1. Elder D, Massi D, Scolyer R, Willemze R. WHO classification of skin tumours, 4th edition. WHO Press; 2018.
  2. Watabe K. Immunohistochemical and ultrastructural studies on tactile-like corpuscles in neurofibromas. No To Shinkei. 1985;37(8):799–807. (In Japan)
  3. Sode T, Kunzler E, Uzoma B, et al. A meissnerian neurofibroma: Case report of a rare neurofibroma variant. J Cutan Pathol. 2020;47(10):967–969. doi: 10.1111/cup.13759
  4. Hill RP. Neuroma of Wagner-Meissner tactile corpuscles. Cancer. 1951;4(4):879–882. doi: 10.1002/1097-0142(195107)4:4<879::aid-cncr2820040425>3.0.co;2-2
  5. Kaiserling E, Geerts ML. Tumour of Wagner-Meissner touch corpuscles. Wagner-Meissner neurilemmoma. Virchows Arch A Pathol Anat Histopathol. 1986;409(2):241–250. doi: 10.1007/BF00708331
  6. Smith KJ, Mezebish D, Williams J, et al. The spectrum of neurocristic cutaneous hamartoma: Clinicopathologic and immunohistochemical study of three cases. Ann Diagn Pathol. 1998;2(4):213–223. doi: 10.1016/s1092-9134(98)80010-5
  7. Stieler KM, Rowert-Huber J, Vogt A, et al. Cutaneous cephalic neurocristic hamartoma on the head with melanocytic, cartilage, blood vessel, neural, and bony tissue. Am J Dermatopathol. 2021;43(4):284–286. doi: 10.1097/DAD.0000000000001826
  8. Karamitopoulou-Diamantis E, Paredes B, Vajtai I. Cutaneous neurocristic hamartoma with blue naevus-like features and plexiform dermal hyperneury. Histopathology. 2006;49(3):326–328. doi: 10.1111/j.1365-2559.2006.02459.x
  9. Turel MK, Chacko G, Raja A, Scheithauer BW. Neurocristic cutaneous hamartoma of the scalp. J Pediatr Neurosci. 2012;7(3): 181–184. doi: 10.4103/1817-1745.106473
  10. Wong J, Roy SF, Kokta V. Neurocristic cutaneous hamartoma with perineuriomatous differentiation: Can it be distinguished from perineuriomatous melanocytic nevi? Am J Dermatopathol. 2021;43(10):757–758. doi: 10.1097/DAD.0000000000001934

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2. Fig. 1. View of the patient at the age of 1 month.

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3. Fig. 2. View of the patient at the age of 6 months.

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4. Fig. 3. View of the patient after the first stage of surgical treatment — expander dermatension.

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5. 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).

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6. Fig. 5. View of the patient on the 7th postoperative day after the second stage of treatment.

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7. Fig. 6. Patient in 6 months after the complete course of treatment.

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版权所有 © Kletskaya I.S., Serebrennikova P.A., Gassan T.A., Narbutov A.G., Makarova O.V., 2024

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