Intraosseous Neurothekeoma of the Hand in a 16-year-old Boy^[*]

Abstract

Soft-tissue tumors are rare in the pediatric population. First described in 1969 as myxoma of the nerve sheath, the neurothekeoma is a benign tumor lesion with presumable origin in the nerve sheath. It occurs mainly in female children and presents as a mass of slow, subcutaneous growth, asymptomatic and without alteration of the local pigmentation. It is predominantly located in the head, neck, and extremities of the upper limbs. This report presents the case of a 16-year-old male with a tumor mass originating from the nerve sheath in the 4^th left metacarpal, intraosseous, and relapsed after previous surgical resection 2 years before this observation. A marginal resection of the tumor mass was performed on the distal region of the fourth metacarpal, followed by curettage of the proximal phalanx and filling with structural autologous bone graft. The patient maintained a favorable postoperative clinical evolution, without local pain or range of motion limitation in his fingers. Radiologically, a progressive trabecular filling of the proximal phalanx of the fourth metacarpal was observed. At 17 months of follow-up, the patient is asymptomatic and shows no signs of relapse. The description of this case serves to increase the familiarity with this rare pathology, and aid its diagnosis and treatment.

Keywords

neurothekeoma - hand - soft tissue neoplasms - biopsy

^* Study developed at Pediatric Orthopedics Service, Pediatric Hospital, Hospital and University Center of Coimbra, Portugal. Originally Published by Elsevier Editora Ltda.

• Referências

• 1 Iacob G, Tene S, Iuga M, Simion G. A very rare, petro-clival, neurothekeoma tumor. Case Report. Romanian Neurosurg. 2015; 29 (03) 239-246
• 2 Gallager RL, Helwig EB. Neurothekeoma--a benign cutaneous tumor of neural origin. Am J Clin Pathol 1980; 74 (06) 759-764
• 3 Harkin JC, Reed RJ. Tumors of the peripheral nervous system. Atlas of Tumor Pathology. Washington, D. C: Armed Forces Institute of Pathology; 1969
• 4 Laskin WB, Fetsch JF, Miettinen M. The “neurothekeoma”: immunohistochemical analysis distinguishes the true nerve sheath myxoma from its mimics. Hum Pathol 2000; 31 (10) 1230-1241
• 5 Ding YI, Zou F, Peng W. , et al. A giant neurothekeoma of the left shoulder blade: A case report. Oncol Lett 2016; 11 (02) 1130-1134
• 6 Uygur S, Ayhan S, Kandal S, Gureli M, Uluoglu O. Giant neurothekeoma in the forearm of an infant. J Plast Reconstr Aesthet Surg 2009; 62 (08) e259-e262
• 7 Bhaskar AR, Kanvinde R. Neurothekeoma of the hand. J Hand Surg [Br] 1999; 24 (05) 631-633
• 8 Blumberg AK, Kay S, Adelaar RS. Nerve sheath myxoma of digital nerve. Cancer 1989; 63 (06) 1215-1218
• 9 Donofrio V, Passeretti U, Russo S, Boscaino A, De Rosa G. Neurothekeoma of the thumb. A case report. Tumori 1988; 74 (06) 751-754
• 10 Kaltman J, Best S, Kaltman S, Lopez E, Velez I. Intraosseous neurothekeoma of the mandible: a rare occurrence. Inter J of Oral Maxil Pathol. 2012; 3 (01) 13-17