Neurothekeoma in the Axilla Causing Persistent Shoulder Pain: Case Report / Neurotecoma na axila causando dor persistente no ombro: Relato de caso

Abstract Neurothekeomas, also known as neural sheath myxomas, are rare benign tumors of the neural sheath affecting most commonly the head, arms and shoulder of women in their 2nd and 3rd decades of life. Due to the low prevalence and undefined clinical picture, they are hardly considered in the initial differential diagnosis of skin tumors. We report the case of a 24 year-old woman who was seen in 2016 reporting > 1 year of moderate pain and limited mobility of her left shoulder. Clinical evaluation revealed restricted mobility of the affected shoulder and nuclear magnetic resonance imaging showed a T2-weighted contrast-enhanced multilobular mass in the quadrilateral area apparently invading the adjacent humeral cortical region. Histopathology of a needle sample material revealed loose fibroconnective tissue with no signs of invasion, mitosis or atypical figures. Successful surgical excision was performed and the diagnosis of neurothekeoma was confirmed after detailed histopathology, including immunohistochemistry. The patient was asymptomatic at 18 months of follow-up, with full recovery of shoulder movement and no signs of relapse.

Resumo Neurotecomas, também conhecidos como mixomas da bainha neural, são tumores benignos raros da bainha neural afetando mais comumente a cabeça, braços e ombros de mulheres entre 20 e 40 anos de idade. Devido à baixa prevalência e quadro clínico mal definido, essas lesões são raramente consideradas no diagnóstico diferencial de tumores cutâneos. Relatamos o caso de uma mulher de 24 anos de idade que procurou atendimento em 2016 relatando dor moderada por mais de um ano e limitação dos movimentos do ombro esquerdo. Ao exame, foi constatada restrição da mobilidade dessa articulação e uma ressonância magnética revelou imagem multilobular com aumento de sinal em T2 na região quadrilateral, aparentando invasão da região cortical do úmero subjacente. A histopatologia de uma biópsia incisional mostrou lesão composta por tecido conjuntivo frouxo, sem sinais de invasão, figuras de mitose ou atipias. Foi realizada excisão completa da lesão e o diagnóstico de neurotecoma foi confirmado após análise histopatológica que incluiu painel imunohistoquímico. À revisão de 18 meses, a paciente estava assintomática com recuperação completa do movimento e sem evidência de recidiva da lesão.

Intraosseous Neurothekeoma of the Hand in a 16-year-old Boy / Neurotequeoma intraósseo da mão de um jovem de 16 anos

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 4th 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.

Resumo Os tumores dos tecidos moles são raros em idade pediátrica. Descrito pela primeira vez 1969 como um mixoma da bainha nervosa, o neurotequeoma é uma lesão tumoral benigna com presumível origem na bainha nervosa. Ocorre maioritariamente em crianças do sexo feminino e apresenta-se como uma massa de crescimento lento, subcutânea, assintomática e sem alteração da pigmentação local. Localiza-se predominantemente na cabeça, no pescoço, e nas extremidades dos membros superiores. Os autores apresentam um caso clínico de um jovem de 16 anos do sexo masculino com massa tumoral com origem na bainha nervosa no 4º metacarpo esquerdo, intraóssea e recidivada após ressecção cirúrgica 2 anos antes do estudo. Foi feita ressecção marginal da massa tumoral localizada sobre a região distal do quarto metacarpo e curetagem da falange proximal e preenchimento com enxerto ósseo autólogo corticoesponjoso. O paciente apresentou uma evolução clínica pós-operatória favorável, sem queixas álgicas e sem limitações da mobilidade dos dedos da mão. Radiologicamente, foi observado preenchimento trabecular progressivo da falange proximal do quarto metacarpo. Aos 17 meses de seguimento, o paciente se encontra assintomático e não apresenta quaisquer sinais de recidiva. Com a descrição deste caso, os autores pretendem aumentar a familiaridade com essa rara patologia, seu diagnóstico e tratamento.

Rare Case of Nerve Sheath Myxoma of the Eyelid Misdiagnosed as Mucocele

No abstract available.

Cellular Neurothekeoma of the Scalp in the Elderly

Cellular neurothekeoma (CNT) is an uncommon variant of neurothekeoma that is composed of pithelioid to spindled cells with variable nuclear atypia or pleomorphism but no myxoid stroma. CNT occurs predominantly in the head and neck or upper trunk of children and young adults, with female predominance. The following case is different from typical CNTs. An 88-year-old female presented with a palpable mass on the scalp, which we excised. Histologically, the tumor was non-encapsulated and composed of spindled and epithelioid cells arranged in fascicles and nodules separated by a collagen-rich stroma. Immunohistochemical analysis showed that the epithelioid and spindle-shaped cells were focally positive for vimentin, neuron-specific enolase, smooth muscle actin, CD68, and CD10 but negative for S-100 protein, HMB-45, epithelial membrane antigen, and CD34. We report a new case of CNT that arose in the scalp of an older patient and that was associated with uncommon clinical, histological, and immunohistochemical profiles.

Cellular Neurothekeoma of the Scalp in the Elderly

Cellular neurothekeoma (CNT) is an uncommon variant of neurothekeoma that is composed of pithelioid to spindled cells with variable nuclear atypia or pleomorphism but no myxoid stroma. CNT occurs predominantly in the head and neck or upper trunk of children and young adults, with female predominance. The following case is different from typical CNTs. An 88-year-old female presented with a palpable mass on the scalp, which we excised. Histologically, the tumor was non-encapsulated and composed of spindled and epithelioid cells arranged in fascicles and nodules separated by a collagen-rich stroma. Immunohistochemical analysis showed that the epithelioid and spindle-shaped cells were focally positive for vimentin, neuron-specific enolase, smooth muscle actin, CD68, and CD10 but negative for S-100 protein, HMB-45, epithelial membrane antigen, and CD34. We report a new case of CNT that arose in the scalp of an older patient and that was associated with uncommon clinical, histological, and immunohistochemical profiles.

Cellular neurothekeoma: case report and its (un) relation with nerve sheath myxoma

Neurothekeoma is an uncommon, benign neoplasm presenting in young adults, primarily on the head and neck. It was initially related to nerve sheath myxoma but with the advent of immunohistochemistry, new insights into its cellular differentiation and origin have emerged, unlinking Neurothekeoma and nerve sheath myxoma. Herein we describe a 19-year-old male who had had a frontal, fl esh-colored, asymptomatic papule for 2 years. Histology showed a dermal fusocellular-spindle cell tumor, including an eosinophilic cytoplasm with mild cellular pleomorphism and moderately dense fi brous stroma. IHQ was positive for CD10 and negative for S100 and Claudin-1. These fi ndings were compatible with cellular Neurothekeoma. The lesion was completely extirpated and at the 6-month follow-up, the patient was asymptomatic and had experienced no recurrences.

A Case of the Cellular Neurothekeoma on Scalp / 대한피부과학회지

No abstract available.

Neurotecoma celular, localización lingual: presentación de caso clínico en el postgrado de cirugía plástica y reconstructiva del Instituto Autonómo Hospital Universitario de los Andés / Cell neurotecoma, location lingual: presentation of a clinical case in the postgraduate studies on plastic and recontructive surgery at the Instituto Autonómo Hospital Universitario de los Andés-Mérida-Venezuela

El Neurotecoma Celular es un raro tumor benigno de histopatogénesis incierta extremadamente atípico. Clínicamente la lesión es poco característica, generalmente es un nódulo solitario asintomático de crecimiento lento que se puede presentar en piel y mucosas. El diagnóstico se basa en los hallazgos histológicos y de inmunohistoquímica, se considera que los neurotecomas en sus distintas variantes: mixoide, celular o mixta forman parte de un espectro de tumores con un supuesto origen en la vaina nerviosa, sin embargo numerosos autores afirman que se trata de tumores diferentes que justifican una clasificación separada. A nivel mundial existen tan solo alrededor de 300 reportes de este tipo de tumoración, por ello el interés de presentar el caso de un paciente masculino de 13 años de edad quien ingreso y fue tratado por el servicio de cirugía plástica y reconstructiva del hospital universitario de los andes y quien curso con una neoformación de aproximadamente 30mm de diámetro, firme, de crecimiento lento, localizada en la porción del margen derecho de la lengua con 14 meses de evolución. El diagnóstico histopatológico confirmó un Neurotecoma Celular(AU)

The Cell Neurothekeoma is a rare benign tumor of uncertain histopatogenesis extremely atypical. Clinicallyncharacteristic lesion, usually an asymptomatic solitary nodule of slow growth. It can occur in the skin and mucous membranes. The diagnosis is based on histological and immunohistochemically findings, the neurotecomas are considered that in its different considered that in its different variants: myxoid, mixed cell or mixed, are a part of putative origin tumors in the nerve sheath, however many authors claim that this are different tumors that justify a separate classification. Worldwide there are only about 300 reports of this type of tumor so the interest of presenting the case of a male patient of 13 years old who income and was treated by the service of plastic and reconstructive surgery at the Andes University Hospital and he coursed with a new formation of approximately 30mm diameter, firm, slow-growing, located in the right lateral portion of the tongue with 14 months duration. Histopathological diagnosis confirmed a Cell Neurothekeoma(AU)

Myxoid Cellular Neurothekeoma: A New Entity of S100-Negative, CD68-Positive Myxoid Neurothekeoma

Neurothekeoma is a rare cutaneous neoplasm, occurring as a cutaneous papule or nodule on the face, shoulders, and upper extremities. Neurothekeoma has been subclassified as either the myxoid, cellular, or mixed type, depending on the amount of myxoid matrix and on immunohistochemical analysis. We observed a clinical case with conflicting histopathological and immunohistochemical findings. In this case, microscopic examination showed the typical presentation of myxoid neurothekeoma; however, immunohistochemical staining was negative for S100 protein and positive for CD68, which is the characteristic pattern of cellular neurothekeoma. We report a very rare form of myxoid cellular neurothekeoma of the face in a young woman.

A Case of Nerve Sheath Myxoma on the Fingertip

PURPOSE: Nerve sheath myxoma is a rare cutaneous neoplasm originating from the peripheral nerve sheath and divided into three groups : myxoid, cellular and mixed type. There is a controversy on it's origin whether schwannian cell or perineurial differentiation, or anything else. Myxoid nerve sheath myxoma is asymptomatic, soft, papule or nodule in middle-age adults. We report a case of myxoid nerve sheath myxoma on the fingertip. METHODS: A 53-year-old woman presented with a painful, 0.4 x 0.4 x 0.6 cm sized, corn shaped nodule on the left 3rd fingertip. We put into surgical excision and studied it by histopathologically and specific immnohistochemical stain. RESULTS: The tumor has well defined nodules separated by thin fibrous connective tissue with abundant myxoid stroma and were positively stainded for S-100 protein, NSE and GFAP. After surgical treatment it was healed without recurrence. CONCLUSION: Nerve sheath myxoma is rare neoplasm and located mainly on face, but very rarely on the fingertip. We report a case of painful myxoid nerve sheath myxoma located on the 3rd fingertip.

A Case of Myxoid Neurothekeoma on the Hand / 대한피부과학회지

Neurothekeoma is a benign dermal soft tissue tumor that is probably of nerve sheath origin. It usually presents as a slow-growing, solitary papule or nodule that is mainly located on the head and neck or upper extremities except for the hand. There are three histologic subtypes - myxoid, mixed and cellular type - depending on the amount of myxoid stroma. Immunohistochemical markers such as S-100, glial fibrillary acidic protein, nerve growth factor receptor, NK1C3 (CD57) and Ki-M1p can be applied to the tumor cells to distinguish among thesethree subtypes of neurothekeoma. Only one case has been previously reported on the hand in Korea. Herein, we report a rare case of myxoid neurothekeoma on the hand with a review of the related literature.

Neurothekeoma of the eyelid: a case report.

The purpose of this article is to describe a rare benign tumor of nerve sheath origin arising from the eyelid in an elderly male. Local excision was done and histopathological examination revealed a neurothekeoma. Six months later the patient was doing well with no recurrence. The case was unique in that the patient was an elderly male while neurothekeoma is commonly seen on the face of young adults, especially females.

A Case of Cellular Neurothekeoma / 대한피부과학회지

Cellular neurothekeoma is a rare, benign, cutaneous neoplasm of uncertain histogenesis and usually presents as a solitary papule or nodule on the head or neck area of children and young adults. Cellular neurothekeoma was thought to represent a cellular variant of nerve sheath myxoma but this neoplasm is histologically different from nerve sheath myxoma. Cellular neurothekeoma is characterized by a fasciculated, somewhat well-circumscribed proliferation of epithelioid and spindle cells. Herein, we report a case of cellular neurothekeoma with a review of related literature.

Neurothekeoma celular: Presentación de un caso / Cellular neurothekeoma. A one-case presentation

Se presenta un caso de neurothekeoma calular en una mujer de 38 años, cuyo asiento de la lesión tumoral es en el vestíbulo de la fosa nasal izquierda, el cual se resecó completamente. Se describe el caso por ser un tumor de reciente caracterización, raro, tanto en nuestro medio como en la literatura médica internacional revisada(AU)

A case of neurothekeoma calular is presented in a 38 year-old woman whose seat of the lesion tumoral is in the lobby of the grave nasal left, which was dried up completely. The case is described to be a tumor of recent characterization, strange, as much in our means as in the revised international medical literature(AU)

Neurotecoma celular atípico nasal / Atipical nasal cellular neurothecoma

El neurotecoma hace parte del raro y heterogéneo grupo de tumores mixoides de los tejidos blandos. Inicialmente se llamó mixoma dérmico de vaina nerviosa por la semejanza de sus células Inmorales con las células de Schwnann. Es un tumor benigno típicamente dérmico que afecta principalmente a los adultos jóvenes, con un predominio en las mujeres y de localización más frecuente en la cabeza y el cuello. Histológicamente se divide en neurotecoma mixoide, intermedio y celular. Ultraestructuralmente se han identificado cuatro tipos celulares y por inmunohistoquímica tiñen con marcadores de músculo liso y enolasa neuroespecífica. Se describe un neurotecoma celular nasal en un hombre de 25 años con múltiples biopsias que sugirieron los diagnósticos de tumor fusocelular, melanoma y xantofíbroma atípico

A Case of Myxoid Nerve Sheath Myxoma / 대한피부과학회지

Nerve sheath myxoma(NSM) is rare benign neoplasm of the dermis, divided into myxoid, cellular and mixed types. Myxoid NSM is asymptomatic, soft, skin colored or translucent papule or nodule in middle-aged adults and typically located on the face and the upper extremities. We, herein, reported a case of myxoid NSM in a 3 1-year -old male who had a painful, 1 X 1cm sized, dome-shaped nodule on the left sole.