RESUMO
Hydroa vacciniforme-like lymphoma is a recently recognized cutaneous T-cell lymphoma associated with Epstein-Barr virus. The disease is observed in children of Latin American or Asian ethnicity. The authors report the clinical, histopathological, and immunophenotypical features of 9 new Mexican patients (M:F = 2:1; mean age, 14.5 years; median age, 13.3 years; age range, 4-27 years), expanding on previous observations of this elusive disease. The most common clinical aspects were persistent facial edema with necroses and pitted scars. Histopathological analyses revealed variably dense lymphoid infiltrates with common angiodestructive features. Neoplastic cells expressed CD3 and cytotoxic markers in all cases and were constantly positive for Epstein-Barr virus (EBER-1). Expression of other markers was variable. Follow-up data revealed that all patients died within 6 months or less, thus showing a very aggressive course with poor prognosis.
Assuntos
Edema/patologia , Infecções por Vírus Epstein-Barr/complicações , Face/patologia , Neoplasias Faciais/patologia , Hidroa Vaciniforme/patologia , Linfoma Cutâneo de Células T/patologia , Adolescente , Adulto , Complexo CD3/análise , Criança , Pré-Escolar , Cicatriz/patologia , Cicatriz/virologia , Edema/virologia , Extremidades/patologia , Neoplasias Faciais/química , Neoplasias Faciais/virologia , Feminino , Humanos , Hidroa Vaciniforme/virologia , Imuno-Histoquímica , Linfoma Cutâneo de Células T/química , Linfoma Cutâneo de Células T/virologia , Masculino , México , Necrose/patologia , Necrose/virologia , Prognóstico , Tronco/patologia , Adulto JovemRESUMO
INTRODUCTION: Chondroid syringoma (CS) is a rare cutaneous tumor characterized by mixte epithelial and mesenchymal component. The confident histological diagnosis can be obtained by immuno-histochemistry study. Here we present 10 new cases with their clinico-hystological characteristics. METHODS: The 10 cases were observed between January 2000 and august 2013, in Fort-de-France and Louis-Mourier universitary hospitals. For all the cases a controlled histological study was performed by a dermatopathologist expert and immuno-histochemistry was added. Clinical and immuno-histological data were analyzed. RESULTS: The lesions were almost localized on the face (3/10) and the extremities (3/10). The size was about 1.2 to 5.2cm. Every case was treated by surgery, no malignant case was diagnosed. Histologically, all the 10 cases presented as a well-limited dermic tumor with a mixte epithelial and mesenchymal component. The stroma was myxo-chondroid, and the epithelial component consisted in epithelial cavities lined by one or two cell layers with eccrine (4/10) or apocrine (5/10) features. Immuno-chemistry study reveals positivity for EMA, ACE and CK7 for the internal cells, and positivity for S100 protein and vimentin of the extern cell layer. DISCUSSION: Chondroid syringoma is characterized by a mixte epithelial with eccrine and apocrine cells and a myxo-chondroid stroma. Our study has some clinical and histological particularities (lesions on the extremities, epidermic connecting ). The main differentials diagnoses are the other annexial tumors. The treatment is surgical. CONCLUSION: The histological diagnosis of CS is quite easy, but in case of doubt, immuno-chemistry will help, showing a double mesenchymal and epithelial differentiation.
Assuntos
Adenoma Pleomorfo/patologia , Neoplasias Cutâneas/patologia , Adenoma Pleomorfo/química , Adenoma Pleomorfo/cirurgia , Adulto , Idoso , Idoso de 80 Anos ou mais , Glândulas Apócrinas/patologia , Biomarcadores Tumorais , Glândulas Écrinas/patologia , Células Epiteliais/patologia , Extremidades/patologia , Neoplasias Faciais/química , Neoplasias Faciais/patologia , Neoplasias Faciais/cirurgia , Feminino , Humanos , Queratina-7/análise , Masculino , Mesoderma/patologia , Pessoa de Meia-Idade , Mucina-1/análise , Estudos Retrospectivos , Proteínas S100/análise , Neoplasias Cutâneas/química , Neoplasias Cutâneas/cirurgia , Células Estromais/patologia , Vimentina/análiseRESUMO
Atypical fibroxanthoma (AFX) is a low-grade, dermal, mesenchymal neoplasm, which lacks a specific lineage of differentiation. The classical histologic appearance of AFX is that of a pleomorphic and spindle cell neoplasm with marked nuclear pleomorphism, mitotic figures, and often prominent storiform pattern that superficially resembles a pleomorphic high-grade sarcoma ("malignant fibrous histiocytoma"). Many histologic variants have been described. We have reviewed 15 cases of AFX characterized by a pure spindle cell morphology that could be easily mistaken for other spindle cell dermal neoplasms. All of our cases were stained with CD68, CD163, CD10, S-100p, p63, wide-spectrum keratin, CD31, CD34, smooth muscle actin (SMA), desmin, calponin, and h-caldesmon. All 15 cases showed an immunoprofile consistent with AFX. In 9 cases, SMA was also strongly expressed; this finding, coupled with the malignant spindle cell histomorphology, can lead to an erroneous diagnosis of cutaneous leiomyosarcoma with potential clinical consequences. Awareness of this pattern of immunoreactivity in this unusual variant of AFX is of importance for avoiding diagnostic misinterpretation. This study intends to characterize the nature and frequency of SMA immunoreactivity in AFX and to discuss the potential diagnostic pitfalls of immunohistochemical markers in distinguishing this entity from other malignant spindle cell neoplasms.
Assuntos
Neoplasias Faciais/química , Neoplasias Faciais/patologia , Histiocitoma Fibroso Maligno/química , Histiocitoma Fibroso Maligno/patologia , Leiomiossarcoma/diagnóstico , Neoplasias Cutâneas/química , Neoplasias Cutâneas/patologia , Actinas/análise , Idoso , Idoso de 80 Anos ou mais , Antígenos CD/análise , Antígenos de Diferenciação Mielomonocítica/análise , Diferenciação Celular , Diagnóstico Diferencial , Feminino , Humanos , Masculino , Pessoa de Meia-Idade , Miofibroblastos/fisiologia , Proteínas de Neoplasias/análise , Neprilisina/análise , Receptores de Superfície Celular/análiseRESUMO
Desmoplastic melanoma tends to present as firm, amelanotic papules. Microscopically, it reveals a proliferation of fusiform cells in the dermis and variable collagen deposition, as well as intraepidermal melanocytic proliferation of lentiginous type in most cases. Biopsy in a 61-year-old white male patient, who had received a diagnosis of lentigo maligna on his face 10 years before, revealed a proliferation of dermal pigmented spindle cells and collagen deposition, reaching the deep reticular dermis, with a lentiginous component. Immunohistochemistry with S-100, Melan-A and WT1 showed positivity, but it was weak with HMB45. Desmoplastic melanoma associated with lentigo maligna was diagnosed. Several authors discuss whether desmoplastic melanoma represents a progression from the lentiginous component or arises "de novo". Desmoplastic melanoma represents a minority of cases of primary cutaneous melanoma (less than 4%). Identification of lentigo maligna indicates that desmoplastic melanoma should be carefully investigated.
Assuntos
Neoplasias Faciais/patologia , Melanoma/patologia , Neoplasias Cutâneas/patologia , Biópsia , Neoplasias Faciais/química , Humanos , Sarda Melanótica de Hutchinson/química , Sarda Melanótica de Hutchinson/patologia , Antígeno MART-1/análise , Masculino , Melanoma/química , Pessoa de Meia-Idade , Invasividade Neoplásica , Proteínas S100/análise , Neoplasias Cutâneas/química , Proteínas WT1/análiseRESUMO
Desmoplastic melanoma tends to present as firm, amelanotic papules. Microscopically, it reveals a proliferation of fusiform cells in the dermis and variable collagen deposition, as well as intraepidermal melanocytic proliferation of lentiginous type in most cases. Biopsy in a 61-year-old white male patient, who had received a diagnosis of lentigo maligna on his face 10 years before, revealed a proliferation of dermal pigmented spindle cells and collagen deposition, reaching the deep reticular dermis, with a lentiginous component. Immunohistochemistry with S-100, Melan-A and WT1 showed positivity, but it was weak with HMB45. Desmoplastic melanoma associated with lentigo maligna was diagnosed. Several authors discuss whether desmoplastic melanoma represents a progression from the lentiginous component or arises "de novo". Desmoplastic melanoma represents a minority of cases of primary cutaneous melanoma (less than 4%). Identification of lentigo maligna indicates that desmoplastic melanoma should be carefully investigated.
Os melanomas desmoplásicos apresentam-se como pápulas amelanóticas firmes; à microscopia exibem proliferação de células fusiformes na derme e variável deposição de colágeno, além de proliferação melanocítica lentiginosa, intraepidérmica, na maioria dos casos. Realizada biópsia de pele de paciente masculino, 61 anos, branco, com diagnóstico de lentigo maligno na face, há 10 anos. O exame histopatológico revela proliferação dérmica de células fusiformes pigmentadas e deposição de colágeno, invadindo até a profundidade da derme reticular, associado a componente lentiginoso; presença de positividade imuno-histoquímica com S-100, Melan-A e WT1, e marcação fraca com HMB45. Diagnóstico de melanoma desmoplásico, associado a lentigo maligno. Existe divergência quanto à origem do melanoma desmoplásico, a partir do componente lentiginoso ou "de novo", na ausência de lentigo associado. O melanoma desmoplásico representa uma minoria dos casos de melanoma cutâneo primário (menos de 4%). A presença de lentigo maligno pode servir de sinal de alerta para possível relação com melanoma desmoplásico.
Assuntos
Humanos , Masculino , Pessoa de Meia-Idade , Neoplasias Faciais/patologia , Melanoma/patologia , Neoplasias Cutâneas/patologia , Biópsia , Neoplasias Faciais/química , Sarda Melanótica de Hutchinson/química , Sarda Melanótica de Hutchinson/patologia , Antígeno MART-1/análise , Melanoma/química , Invasividade Neoplásica , /análise , Neoplasias Cutâneas/química , Proteínas WT1/análiseRESUMO
AIM: Tuberous sclerosis is a neurocutaneous syndrome characterized by affect multiple organs such as brain, kidneys, heart, eyes, lungs and skin. The aim of this study was to analyze the pattern of immunolocalization of markers MMP-1, MMP-10, TIMP-1, α-SMA and TGF-ß1 in oral and facial angiofibromas in individuals affected by tuberous sclerosis. METHODS: Microscopical analyses on hematoxilin-eosin and immunohistochemistry reactions were performed to analyze the previously cited biological markers pattern in orofacial angiofibromas. RESULTS: Reactivity was observed for MMP-1, MMP-10 and TGF-ß1, in addition to negative for TIMP-1 and α-SMA, except perivascular and epithelial staining for this. Concerning the intensity, a strong marking for MMP-1 in the basal layer of the epithelium, and a slight positivity in the suprabasal layers predominated. MMP-10 was slightly expressed in all epithelial layers. The connective tissue showed slight to moderate reactivity for MMP-1 and MMP-10. TIMP-1 demonstrated slight to moderate marking in the various layers of a single lesion and to TGF-ß1 expression showed varied in intensity staining both between lesions and between tissue layers. CONCLUSION: MMP-1, MMP-10 and TGF-ß1 exhibited reactivity in oral and cutaneous angiofibromas with heterogeneous distribution patterns among both tissue elements analyzed in the intensity of marking the same among the specimens. TIMP-1 showed reactivity predominantly negative in the specimens analyzed and α-SMA presented restricted to epithelial and perivascular regions of these lesions.
Assuntos
Actinas/análise , Angiofibroma/química , Biomarcadores Tumorais/análise , Neoplasias Faciais/química , Metaloproteinase 10 da Matriz/análise , Metaloproteinase 1 da Matriz/análise , Neoplasias Bucais/química , Proteínas de Neoplasias/análise , Neoplasias Primárias Múltiplas/química , Inibidor Tecidual de Metaloproteinase-1/análise , Fator de Crescimento Transformador beta1/análise , Esclerose Tuberosa/metabolismo , Adolescente , Adulto , Idoso , Angiofibroma/genética , Criança , Células Epiteliais/química , Células Epiteliais/ultraestrutura , Neoplasias Faciais/genética , Feminino , Fibroblastos/química , Fibroblastos/ultraestrutura , Neoplasias Gengivais/química , Neoplasias Gengivais/genética , Humanos , Técnicas Imunoenzimáticas , Neoplasias Labiais/química , Neoplasias Labiais/genética , Masculino , Pessoa de Meia-Idade , Neoplasias Bucais/genética , Neoplasias Primárias Múltiplas/genética , Pericitos/química , Pericitos/ultraestrutura , Neoplasias Cutâneas/química , Neoplasias Cutâneas/genéticaRESUMO
Cellular neurothekeomas are relative uncommon benign dermal tumors of uncertain histogenesis. Most commonly they arise as a solitary papule or nodule on the head and neck or upper trunk of young adults with a slight female predominance. There has been only 1 previous report of multiple neurothekeomas. The patient described herein was a 16-year-old otherwise healthy boy who presented with approximately 30 facial papules that arose over the course of 6 months and progressively enlarged. Histologically, all lesions were composed of spindled to epithelioid cells, but varied in the degree of cellularity and dermal sclerosis. Immunohistochemical staining showed that the cells of interest expressed S100A6, vimentin, CD63 (NKI/C3), PGP 9.5, and factor XIIIa and were negative for CD68, glial fibrillary acid protein (GFAP), S-100, HMB-45, epithelial membrane antigen, actin, and CD57 consistent with a diagnosis of multiple desmoplastic cellular neurothekeomas.
Assuntos
Neoplasias Faciais/patologia , Neurotecoma/patologia , Neoplasias Cutâneas/patologia , Pele/patologia , Adolescente , Biomarcadores Tumorais/análise , Biópsia , Neoplasias Faciais/química , Humanos , Imuno-Histoquímica , Masculino , Neurotecoma/química , Pele/química , Neoplasias Cutâneas/químicaRESUMO
We report the case of a male newborn infant with a pedunculated dermic tumor, located in the right malar region; who underwent a complete surgical resection of the tumor and had a satisfactory postoperative evolution. The histopathologic findings disclosed a subcutaneous tumor with a nodular aspect and a subendothelial intravascular growth, constituted by a dual population of small cells and spindle-shaped cells, distributed in a biphasic pattern. All tumor cells showed a strong pericellular reaction for PAS. The immunohistochemical studies revealed: diffuse cytoplasmic positivity for CD34 and Vimentin in all tumor cells, and only spindle-shaped tumor cells and less differentiated isolated neoplastic cells, presented cytoplasmic positivity for the smooth muscle alpha-actin. The electronic microscopy demonstrated a layer of basal membrane and in the citoplasm, numerous intermediate filaments with focal condensations. Based on all these findings, we conclude that this is a myofibroma, a "true hemangiopericytoma" with myofibroblastic differentiation. For this reason, we propose the term myofibropericytoma, in order to highlight its pericytic origin and its myofibroblastic differentiation. We emphasize the need to recognize this entity, in view of its low frequency and the possibility of a diagnostic mistake with other soft tissues tumors that display haemangiopericytoma-like features.