RESUMO
Diffuse neurofibroma is an uncommon subtype of neurofibroma that occurs principally in children and young adults. It is most common in the head and neck region and appears clinically as a plaque-like elevation of the skin. A 3-year-old girl presented to our hospital with a complaint of skin-colored elevated plaques with hair on her lower back since birth. An ultrasonogram showed several 5~10 mm sized subcutaneous hypoechoic nodules with increased vascularity. The histopathologic examination revealed ill-defined, uncapsulated infiltrative, spindle shaped cells within the dermis and subcutaneous tissue. Immunohistochemical examination with antibodies to S-100 protein showed positive. We report a rare case of diffuse neurofibroma with hair.
Assuntos
Criança , Humanos , Adulto Jovem , Anticorpos , Derme , Cabelo , Cabeça , Pescoço , Neurofibroma , Parto , Pré-Escolar , Proteínas S100 , Pele , Tela SubcutâneaRESUMO
A neurofibroma is a benign tumor of the peripheral nerve sheath characterized by proliferation of Schwann cells, perineural cells, and endoneurial fibroblasts. Different types of neurofibromas can be identified, including localized, plexiform, and diffuse types. Neurofibromas can involve any site on the body skin. The diffuse variant is rare and occurs primarily in children and young adults. It involves the skin and subcutaneous tissue in a plaque-like fashion on the head and neck regions. We present a case of a 10-year-old boy who had a diffuse neurofibroma on the scalp.
Assuntos
Criança , Humanos , Adulto Jovem , Fibroblastos , Cabeça , Pescoço , Neurofibroma , Nervos Periféricos , Couro Cabeludo , Células de Schwann , Pele , Tela SubcutâneaRESUMO
Diffuse neurofibroma is an uncommon variant of neurofibroma that usually presents on the head and neck. It is common in children and young adults. Clinically, the lesion presents as large, poorly circumscribed swelling. A 25 year-old female presented with diffuse swelling on the occipital region of the scalp for several years. Magnetic resonance imaging showed 8x9 cm sized, ill defined infiltration on the occipital scalp. The histologic examination revealed unencapsulated loosely arranged spindle cells within the dermis and subcutaneous tissue. Meissnerian differentiation was observed within the tumor cells. The tumor cells were positive for S-100 protein. We report a case of diffuse neurofibroma on the occipital region of the scalp.
Assuntos
Criança , Feminino , Humanos , Adulto Jovem , Derme , Cabeça , Imageamento por Ressonância Magnética , Pescoço , Neurofibroma , Proteínas S100 , Couro Cabeludo , Tela SubcutâneaRESUMO
A 37-year-old man presented with a 1-year history of asymptomatic ill-defined subcutaneous thickening with overlying whitish hairs on right occipital scalp. Histopathologic examination from this subcutaneous mass revealed neurofibromatous tissue with a diffuse infiltrative growth. S-100 protein staining showed decreased melanocytes and melanin in hair follicles. He was diagnosed as a diffuse neurofibroma associated with poliosis. To our knowledge, only two cases of poliosis associated with neurofibroma have ever been reported in English.
Assuntos
Adulto , Humanos , Cabelo , Folículo Piloso , Melaninas , Melanócitos , Neurofibroma , Proteínas S100 , Couro CabeludoRESUMO
This report describes a case of diffuse neurofibroma(DNF) that presented with spontaneous intratumoral hemorrhage. A 31-year-old man with cutaneous manifestations typical of neurofibromatosis type 1(NF1) was referred to hospital with a progressively expanding scalp swelling. Magnetic resonance imaging(MRI) showed an acute hematoma surrounded by a dense mass on the parietooccipital scalp. Plain skull radiographs and a computed tomography(CT) scan revealed bony defects in the occipital bone around the lambdoid suture and the skull base, including the sphenoid and petrous bones. The tumor extended to the upper cervical region and infiltrated through the fascia of the musculature. The tumor and adjacent soft tissue were highly vascular, which made surgical resection difficult. We describe the clinical, radiological and pathological features of this patient and discuss the surgical methods used to avoid a life-threatening hemorrhage during surgery.
Assuntos
Adulto , Humanos , Fáscia , Hematoma , Hemorragia , Neurofibroma , Neurofibromatoses , Osso Occipital , Osso Petroso , Couro Cabeludo , Crânio , Base do Crânio , SuturasRESUMO
A 31-year-old man presented with an erythematous tumor within a giant pigmentation on the left arm, which was partially covered with hairs and had a few subcutaneous nodules within it. Also he had a bluish tumor on the left palm, multiple tumors and cafe au lait spots on the upper trunk. Histologic examination from the tumor, pigmentation and subcutaneous nodules on the left arm, and the tumors on the upper trunk and left palm all revealed neurofibromas. Interestingly, the rare types of the neurofibromas, diffuse and pigmented neurofibroma were included in these.
Assuntos
Adulto , Humanos , Braço , Manchas Café com Leite , Cabelo , Neurofibroma , Neurofibromatoses , PigmentaçãoRESUMO
A 13-year-old girl presented in our unit with progressive alopecia and a long history of edematous scalp in the occiput. Biopsy specimen of a lesion showed two-type pathologic abnormality consisting of diffuse neurofibroma in the lower portion and trichotillomania in the upper portion. Since the tumor spread diffusely through the lower dermis and subcutaneous fat tissue without destroying surrounding structures, we concluded that the trichotillomania might result from repetitive scratching or other traumatic behavior at the same site of underlying diffuse nerurofibroma. We reported a case of the concomitant occurrence of trichotillomania associated with diffuse neurofibroma.