Aggressive Osteoblastoma of Temporal Bone Causing Facial Palsy in a 9-year-old Child: A Case Report Based on 2020 WHO Classification of Bone Tumors / Osteoblastoma agressivo do osso temporal causando paralisia facial em uma criança de 9 anos: Um relato de caso baseado na classificação da OMS de 2020 de tumores ósseos

Aggressive osteoblastoma (AO) is an uncommon bone tumor that represents a borderline lesion between osteoblastoma and osteosarcoma. The vertebral column, the sacrum, the pelvis, and jaw/craniofacial bones are primarily affected. Aggressive osteoblastoma does not metastasize and is treated by surgical resection. The authors report a case of AO in a 9-year-old female patient presenting with 5th and 7th cranial nerve palsy. Prior pathological history included resection of an expansile nodule in the left temporal bone. Conventional radiological examination and computed tomography (CT) of the skull revealed an osteoblastic lesion arising in the petrous portion of the left temporal bone, measuring 5.2 cm in the largest dimension. The patient was subjected to partial surgical resection of the process. Microscopy revealed a primary neoplastic bone composed of numerous epithelioid round osteoblasts disposed in solid sheets and with mild atypia, large eosinophilic cytoplasm, and an eccentric, ovoid nucleus. The process exhibited loose stroma, low mitotic index, osteoid formation, and a few osteoclast-like multinucleated giant cells. The diagnosis of AO was thus established. After 5 months of clinical follow-up, the patient is asymptomatic, without evidence of tumoral growth on CT scans.

O osteoblastoma agressivo (AO) é um tumor ósseo incomum que representa uma lesão limítrofe entre osteoblastoma e osteossarcoma. A coluna vertebral, o sacro, a pelve e os ossos maxilares/craniofaciais são afetados principalmente. O osteoblastoma agressivo não metastatiza sendo tratado por ressecção cirúrgica. Os autores relatam um caso de OA em paciente do sexo feminino, de 9 anos, com paralisia de V e VII pares cranianos. A história patológica prévia incluiu ressecção de nódulo expansivo no osso temporal esquerdo. O exame radiológico convencional e a tomografia computadorizada (TC) de crânio revelaram lesão osteoblástica surgindo na porção petrosa do osso temporal esquerdo, medindo 5,2 cm em sua maior dimensão. O paciente foi submetido à ressecção cirúrgica parcial do processo. A microscopia revelou osso neoplásico primário composto por numerosos osteoblastos epitelióides redondos dispostos em lâminas sólidas e com leve atipia, grande citoplasma eosinofílico e núcleo ovoide excêntrico. O processo exibiu estroma frouxo, baixo índice mitótico, formação de osteóide e algumas células gigantes multinucleadas semelhantes a osteoclastos. O diagnóstico de OA foi assim estabelecido. Após 5 meses de acompanhamento clínico, o paciente encontra-se assintomático, sem evidência de crescimento tumoral na tomografia computadorizada.

Bony Histiocytic Sarcoma of the Scapula: Case Report.

Case report of a pediatric patient with a diagnosis of bony histiocytic sarcoma of the scapula, submitted to oncologic resection (Tikhoff-Linberg type II surgery), who returned to previous activities and was disease-free after 24 months of follow-up.

Association between tumor-infiltrating lymphocytes and sentinel lymph node positivity in thin melanoma.

BACKGROUND: Sentinel lymph node biopsy in thin invasive primary cutaneous melanoma (up to 1mm thick) is a controversial subject. The presence of tumor-infiltrating lymphocytes could be a factor to be considered in the decision to perform this procedure. OBJECTIVE: To evaluate the association between the presence of tumor-infiltrating lymphocytes and lymph node metastases caused by thin primary cutaneous melanoma. METHODS: Cross-sectional study with 137 records of thin invasive primary cutaneous melanoma submitted to sentinel lymph node biopsy from 2003 to 2015. The clinical variables considered were age, sex and topography of the lesion. The histopathological variables assessed were: tumor-infiltrating lymphocytes, melanoma subtype, Breslow thickness, Clark levels, number of mitoses per mm2, ulceration, regression and satellitosis. Univariate analyzes and logistic regression tests were performed as well the odds ratio and statistical relevance was considered when p <0.05. RESULTS: Among the 137 cases of thin primary cutaneous melanoma submitted to sentinel lymph node biopsy, 10 (7.3%) had metastatic involvement. Ulceration on histopathology was positively associated with the presence of metastatic lymph node, with odds ratio =12.8 (2.77-59.4 95% CI, p=0.001). The presence of moderate/marked tumor-infiltrating lymphocytes was shown to be a protective factor for the presence of metastatic lymph node, with OR=0.20 (0.05-0.72 95% CI, p=0.014). The other variables - clinical and histopathological - were not associated with the outcome. STUDY LIMITATIONS: The relatively small number of positive sentinel lymph node biopsy may explain such an expressive association of ulceration with metastatization. CONCLUSIONS: In patients with thin invasive primary cutaneous melanoma, few or absent tumor-infiltrating lymphocytes, as well as ulceration, represent independent risk factors for lymph node metastasis.

Association between tumor-infiltrating lymphocytes and sentinel lymph node positivity in thin melanoma

Abstract: Background: Sentinel lymph node biopsy in thin invasive primary cutaneous melanoma (up to 1mm thick) is a controversial subject. The presence of tumor-infiltrating lymphocytes could be a factor to be considered in the decision to perform this procedure. Objective: To evaluate the association between the presence of tumor-infiltrating lymphocytes and lymph node metastases caused by thin primary cutaneous melanoma. Methods: Cross-sectional study with 137 records of thin invasive primary cutaneous melanoma submitted to sentinel lymph node biopsy from 2003 to 2015. The clinical variables considered were age, sex and topography of the lesion. The histopathological variables assessed were: tumor-infiltrating lymphocytes, melanoma subtype, Breslow thickness, Clark levels, number of mitoses per mm2, ulceration, regression and satellitosis. Univariate analyzes and logistic regression tests were performed as well the odds ratio and statistical relevance was considered when p <0.05. Results: Among the 137 cases of thin primary cutaneous melanoma submitted to sentinel lymph node biopsy, 10 (7.3%) had metastatic involvement. Ulceration on histopathology was positively associated with the presence of metastatic lymph node, with odds ratio =12.8 (2.77-59.4 95% CI, p=0.001). The presence of moderate/marked tumor-infiltrating lymphocytes was shown to be a protective factor for the presence of metastatic lymph node, with OR=0.20 (0.05-0.72 95% CI, p=0.014). The other variables - clinical and histopathological - were not associated with the outcome. Study limitations: The relatively small number of positive sentinel lymph node biopsy may explain such an expressive association of ulceration with metastatization. Conclusions: In patients with thin invasive primary cutaneous melanoma, few or absent tumor-infiltrating lymphocytes, as well as ulceration, represent independent risk factors for lymph node metastasis.

Bony Histiocytic Sarcoma of the Scapula: Case Report / Sarcoma histiocítico ósseo em escápula: relato de caso

Abstract Case report of a pediatric patient with a diagnosis of bony histiocytic sarcoma of the scapula, submitted to oncologic resection (Tikhoff-Linberg type II surgery), who returned to previous activities and was disease-free after 24 months of follow-up.

Resumo Relato de caso de paciente pediátrico com diagnóstico de sarcoma histiocítico ósseo em escápula, submetido à ressecção oncológica (cirurgia de Tikhoff-Linberg tipo II), com retorno às atividades prévias e livre de doença após 24 meses de seguimento.

Congenital and neonatal lupus erythematosus: two case reports.

Neonatal lupus erythematosus is an autoimmune disease produced by the passage of maternal antinuclear antibodies and extractable nuclear antigen antibodies through the placenta. At the moment of the diagnosis, the mothers are asymptomatic in 40 to 60% of cases. The most common manifestations are cutaneous lesions and congenital heart block. The cutaneous findings are variable and usually begin within the first weeks or months of life. Congenital lupus erythematosus is a congenital variant of neonatal lupus erythematosus. We present one case of congenital lupus erythematosus and one case of neonatal lupus erythematous, showing the variability of this disease.

Congenital and neonatal lupus erythematosus: two case reports / Lúpus eritematoso congênito e neonatal: relato de dois casos

Neonatal lupus erythematosus is an autoimmune disease produced by the passage of maternal antinuclear antibodies and extractable nuclear antigen antibodies through the placenta. At the moment of the diagnosis, the mothers are asymptomatic in 40 to 60% of cases. The most common manifestations are cutaneous lesions and congenital heart block. The cutaneous findings are variable and usually begin within the first weeks or months of life. Congenital lupus erythematosus is a congenital variant of neonatal lupus erythematosus. We present one case of congenital lupus erythematosus and one case of neonatal lupus erythematous, showing the variability of this disease.

Lúpus eritematoso neonatal é uma doença auto-imune produzida pela passagem de anticorpos maternos antinucleares e anticorpos contra antígenos extraíveis nucleares através da placenta. No momento do diagnóstico, as mães são assintomáticas em 40 a 60% dos casos. As manifestações mais comuns são lesões cutâneas e bloqueio cardíaco congênito. Os achados cutâneos são variáveis e geralmente começam nas primeiras semanas ou meses de vida. Lúpus eritematoso congênito é uma variante do lúpus eritematoso neonatal. Apresentaremos um caso de lúpus eritematoso congênito e um caso de lúpus eritematoso neonatal, mostrando a variabilidade da doença.

Early diagnosis of cutaneous melanoma: an observation in southern Brazil.

BACKGROUND: The incidence of melanoma cutaneous and the mortalities rates are rising in most countries worldwide. OBJECTIVE: to describe the histopathological characteristics of cutaneous melanoma, according to the criteria established by the Brazilian Group of Melanoma, and to evaluate early diagnosis in a cancer treatment referral center. METHODS: we performed a cross-sectional descriptive study of cases of primary cutaneous melanoma identified after excisional biopsy and processed at the pathology laboratory of Complexo Hospitalar Santa Casa between Jan 1st 2000 and Jan 15th 2005. The following variables were analyzed: age, gender, topography, histopathologic subtype, Breslow thickness, growth phase, Clark level, mitotic index, peritumoral and intratumoral lymphocytic inflammatory infiltrate, angiolymphatic invasion, ulceration, regression, type of regression, microscopic satellitosis, and surgical margins. RESULTS: 328 cases, 57% female and 43% male, were analyzed. Mean age was 55.6 years. For women, the most common tumor location was in inferior(29.26%) and superior limbs(23.94%), while for men melanoma was mainly found in the back(35%), followed by anterior chest/abdomen(14.29%) (p<0.05). Prevalence of histologic subtypes was the following: superficial spreading melanoma(62.8%), lentigo maligna(14.9%), nodular(14.6%), acral(7.3%), and desmoplastic(0.3%) types. Regarding Breslow, 26.2% were in situ, 36.9% had <1 mm, and only 15.9% were ? 4mm in depth. CONCLUSION: the distribution of histopathologic subtypes, as well as Breslow thickness, was in accordance with previous studies in outpatient populations. The profile of cases of cutaneous melanoma diagnosed in a tertiary hospital seems to be experiencing some changes over the last two decades, with a current trend for earlier diagnosis.

A precocidade diagnóstica do melanoma cutâneo: uma observação no sul do Brasil / Early diagnosis of cutaneous melanoma: an observation in southern Brazil

FUNDAMENTOS: A incidência do melanoma cutâneo e as taxas de mortalidade a ele associadas estão crescendo na maioria dos países do mundo. OBJETIVO: Descrever as características histopatológicas do melanoma cutâneo, segundo critérios do Grupo Brasileiro de Melanoma, e avaliar a precocidade diagnóstica em hospital de referência do sul do Brasil para o atendimento de melanoma. MÉTODOS: Estudo transversal com casos de melanoma cutâneo primário reconhecidos após biópsia excisional, processados no laboratório de Patologia do Complexo Hospitalar Santa Casa de Porto Alegre entre 1º/1/2000 e 15/1/2005. Outras variáveis analisadas: idade, sexo, topogra fia da lesão, subtipos histopatológicos, índice Breslow, fase de crescimento, índice de Clark, índice mitótico, infiltrado inflamatório linfocitário peritumoral e intratumoral, invasão angiolinfática e perineural, presença de úlcera e regressão, tipo de regressão, satelitose microscópica e margens cirúrgicas. RESULTADOS: Incluídos 328 casos, sendo 57 por cento mulheres e 43 por cento homens, com média de idade de 55,63 anos. A localização foi preferencialmente nos membros inferiores (29,26 por cento) e superiores (23,94 por cento) nas mulheres. Nos homens, predominou no dorso (35 por cento) e no tórax anterior/abdome (14,29 por cento) (p<0,05). Os subtipos histológicos se apresentaram com as seguintes frequências: espalhamento superficial (62,8 por cento), lentigo maligno (14,9 por cento), nodular (14,6 por cento), acral (7,3 por cento) e desmoplásico (0,3 por cento). Quanto ao Breslow: 26,2 por cento dos casos eram in situ, 36,9 por cento eram <1mm, enquanto apenas 15,9 por cento apresentavam mais de 4mm de profundidade. CONCLUSÃO: A distribuição dos subtipos histológicos e o nível de profundidade (Breslow) foram semelhantes aos encontrados em estudos anteriores em população de base não hospitalar. O perfil dos casos de melanoma cutâneo avaliados em hospital terciário parece estar se modificando nas últimas duas décadas, com tendência a diagnósticos mais precoces, atualmente.

BACKGROUND: The incidence of melanoma cutaneous and the mortalities rates are rising in most countries worldwide. OBJECTIVE: to describe the histopathological characteristics of cutaneous melanoma, according to the criteria established by the Brazilian Group of Melanoma, and to evaluate early diagnosis in a cancer treatment referral center. METHODS: we performed a cross-sectional descriptive study of cases of primary cutaneous melanoma identified after excisional biopsy and processed at the pathology laboratory of Complexo Hospitalar Santa Casa between Jan 1st 2000 and Jan 15th 2005. The following variables were analyzed: age, gender, topography, histopathologic subtype, Breslow thickness, growth phase, Clark level, mitotic index, peritumoral and intratumoral lymphocytic inflammatory infiltrate, angiolymphatic invasion, ulceration, regression, type of regression, microscopic satellitosis, and surgical margins. RESULTS: 328 cases, 57 percent female and 43 percent male, were analyzed. Mean age was 55.6 years. For women, the most common tumor location was in inferior(29.26 percent) and superior limbs(23.94 percent), while for men melanoma was mainly found in the back(35 percent), followed by anterior chest/abdomen(14.29 percent) (p<0.05). Prevalence of histologic subtypes was the following: superficial spreading melanoma(62.8 percent), lentigo maligna(14.9 percent), nodular(14.6 percent), acral(7.3 percent), and desmoplastic(0.3 percent) types. Regarding Breslow, 26.2 percent were in situ, 36.9 percent had <1 mm, and only 15.9 percent were ? 4mm in depth. CONCLUSION: the distribution of histopathologic subtypes, as well as Breslow thickness, was in accordance with previous studies in outpatient populations. The profile of cases of cutaneous melanoma diagnosed in a tertiary hospital seems to be experiencing some changes over the last two decades, with a current trend for earlier diagnosis.

Angiosarcoma in a 3-year-old child with congenital lymphedema

Angiosarcoma occurring in chronic lymphedema has been described in more than 200 cases, especially in the upper extremity following radical mastectomy (Stewart-Treves syndrome). However, angiosarcoma developing in congenital lymphedema is quite rare and the literature presents only 14 cases. Our patient is a girl with congenital lymphedema of the left lower limb that developed an angiosarcoma at 3 years of age, noted initially as a painful nodular lesion on the left thigh. This seems to be the earliest presentation of angiosarcoma associated with lymphedema. It claims attention to a careful evaluation of any lesions in a child with lymphedema to provide a better prognosis, which is allowed by precocious diagnosis and rapid interventions.

Histiocitoma fibroso maligno angiomatoide retroperitoneal: relato de caso / Retroperitoneal angiomated malignant fibrous histiocytoma: a case report

Apresenta-se o caso de uma paciente de sessenta anos com um volumoso histiocitoma fibroso maligno angiomatoide retroperitoneal. A presenca de massa abdominal palpavel era a unica queixa da paciente, que foi submetida a tratamento cirurgico. Discutem-se as caracteristicas histologicas, clinicas e biologicas, bem como o manejo e o prognostico deste tipo de neoplasia