ABSTRACT
Neurotecomas são neoplasias raras, benignas, de presumida linhagem fibro-histiocítica. Ao exame dermatológico e ao anatomopatológico, apresentam múltiplos diagnósticos diferenciais, o que torna sua identificação desafiadora. Relatamos o caso de paciente do sexo masculino, de 28 anos de idade, que apresentou crescimento de pápula endurecida na pálpebra superior direita, com histopatológico e imuno-histoquímica sugestivos de neurotecoma
Neurothekeomas are rare, benign dermal tumors of presumed fibrohistiocytic lineage. They present multiple differential diagnoses, making their identification challenging at the dermatological and anatomopathological examination. We report the case of a 28-year-old man who presented a hardened papule growth on the left upper eyelid with histopathology and immunohistochemistry suggestive of neurothekeoma.
ABSTRACT
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.
Subject(s)
Humans , Female , Adult , Arm , Recurrence , Skin Neoplasms , Axilla , Biopsy , Magnetic Resonance Spectroscopy , Neurothekeoma , Connective Tissue , Shoulder Pain , Diagnosis, Differential , Head , Joints , Mitosis , Myxoma , NeoplasmsABSTRACT
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.
Subject(s)
Humans , Male , Adolescent , Pain , Soft Tissue Neoplasms , Wounds and Injuries , Biopsy , Bone and Bones , Bone Transplantation , Neurothekeoma , HandABSTRACT
Neurothekeoma is a benign soft tissue tumour of neural origin. It is mostly seen in young females and commonly involves the head and neck region. We present a case of a 47 year old male, who presented with a nodule on his scalp. It was clinically diagnosed to be a case of sebaceous cyst however histologically proven to be a myxoid neurothekeoma. The histologic variants include myxoid, cellular and mixed tumors. Myxoid neurothekeoma is a cutaneous neoplasm of nerve sheath origin. This case report presents the rarity of the lesion that needs to be differentiated from lesions of similar histological features.
ABSTRACT
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.
Subject(s)
Aged , Aged, 80 and over , Child , Female , Humans , Young Adult , Actins , Epithelioid Cells , Head , Mucin-1 , Muscle, Smooth , Neck , Neurothekeoma , Phosphopyruvate Hydratase , S100 Proteins , Scalp , VimentinABSTRACT
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.
Subject(s)
Aged , Aged, 80 and over , Child , Female , Humans , Young Adult , Actins , Epithelioid Cells , Head , Mucin-1 , Muscle, Smooth , Neck , Neurothekeoma , Phosphopyruvate Hydratase , S100 Proteins , Scalp , VimentinABSTRACT
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.
.Subject(s)
Humans , Male , Young Adult , Nerve Sheath Neoplasms/pathology , Neurothekeoma/pathology , Skin Neoplasms/pathology , Diagnosis, Differential , ImmunohistochemistryABSTRACT
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)
Subject(s)
Humans , Male , Adolescent , Mouth Neoplasms , Tongue Neoplasms , Neurothekeoma/pathology , Surgery, Oral , Medical OncologyABSTRACT
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.
Subject(s)
Female , Humans , Neurothekeoma , Shoulder , Upper ExtremityABSTRACT
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.
Subject(s)
Glial Fibrillary Acidic Protein , Hand , Head , Korea , Neck , Nerve Growth Factor , Neurothekeoma , Upper ExtremityABSTRACT
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.
Subject(s)
Child , Humans , Young Adult , Head , Neck , NeurothekeomaABSTRACT
Neurothekeoma is a relatively uncommon tumor of nerve sheath origin. Most of these tumors are benign and malignant transformation was not reported. Usually, these tumors arise during childhood or early adulthood life with female preponderance. They have a predilection for the head, neck and shoulder region but any part of the body may be affected. Their usual size is between 0.5-3.0 cm. Rapid growth and ulceration are rare, and local recurrence tends to occur after incomplete excision. We report a case of neurothekeoma developed in the scalp of a 21-year-old woman. It was first noticed about 1year ago. This mass had been increasing in size slowly. On physical examination, 2 x 1.5 cm sized papule with localized alopecia was identified on the frontal scalp area. It was excised in 5.5 x 3.6 cm. The histologic finding of the lesion showed that lobules, fascicles or rests of spindle shaped or epitheloid cells in a myxoid of mucosubstance. The tumor cells were positive for S-100 and vimentin but showed negative for EMA, cytokeratin, desmin, actin and HMB45. After surgical excision of tumor, split-thickness skin graft was done. Three months after the operation, rectangular shape tissue expander (180 cc Sebbin(R)) was inserted on both sides of the previous wound. After removal of tissue expander, the wound closed with local flap. The patient did not have any evidence of recurrence for 6 months.
Subject(s)
Female , Humans , Young Adult , Actins , Alopecia , Desmin , Head , Keratins , Neck , Neurothekeoma , Physical Examination , Recurrence , Scalp , Shoulder , Skin , Tissue Expansion Devices , Transplants , Ulcer , Vimentin , Wounds and InjuriesABSTRACT
Nerve sheath myxoma(NSM) is a rare cutaneous neoplasm of uncertain histogenesis. NSM can be divided into two groups; NSM1 (a myxoid NSM) and NSM2 (a cellular neurothekeoma). NSMs are characterized histologically by well-defined, lobular or plexiform dermal proliferation of stellate, spindle-shaped cells embedded in abundant myxoid stroma. A variable amount of nerve fascicles are entrapped within and around the tumor. Two variants of NSM share the same features to a certain degree but differ in cellularity and mucin deposition. We report two cases of NSMs with different clinical and histopathologic features.
Subject(s)
Mucins , NeurothekeomaABSTRACT
A case of nerve sheath myxoma also called as neurothekeoma in a 33-year-old woman is described. The lesion appeared as a painful, elevated nodule on the scalp for several months, without an appreciable increase in size. Microscopically, it showed typical histologic characteristics of nerve sheath myxoma, and tumor cells revealed strong, positive reaction for S-100 protein and negativity for epithelial membrane antigen (EMA) on immunohistochemical staining. These immunohistochemical findings of this case support the view that the origin cells of this tumor may be schwann cells rather than perineurial cells. The histogenesis and differential diagnosis of this tumor are discussed.