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.

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.

Evaluation of Kindlin-1 and Ki-67 immunohistochemical expression in primary cutaneous malignant melanoma: a clinical series

Background: The capacity for prognostic prediction of cutaneous melanoma, one of the most aggressive cancers, is still difficult due to the tumor heterogeneity and lack of reliable tumor markers. The objective of this study is to correlate, through immunohistochemistry, a Ki-67 and Kindlin-1 staining in malignant melanomas with the prognosis of the disease. Methods: A historical cohort study. Immunohistochemistry, using mouse anti-human Kindlin-1 and Ki-67 monoclonal antibodies, was performed using tissue blocks from primary cutaneous melanoma patients treated between 2006 and 2014 at our institution. Information regarding pathological data and outcomes were retrieved from medical records. Statistical analyses were conducted in SPSS version 18.0. Results: Thirty patients were included. The median age was from 50.93 ± 15.31 years old. The expression of Ki-67 was detected in all patients with primary cutaneous melanoma, while Kindlin-1 was negative in two. Kindlin expression was not significantly correlated with Ki-67 expression by Spearman's rank correlation analysis (P = 0.46), as well as the expression of both markers and the clinical stage (P = 0.34 and 0.18, respectively). Breslow, Clark and mitotic rate were significantly correlated with AJCC stage (P = 0.001). Conclusion: Other studies investigating clinical evolution are needed to further test the potential of these markers as possible prognostic markers (AU)

Giant cell tumor of the tendon sheath: a rare periungual location simulating myxoid cyst

ABSTRACT Giant cell tumor of the tendon sheath is a benign soft tissue tumor most frequent between the third and fifth decades of life. It can mimic and make differential diagnoses with several hand tumors. Definitive diagnosis and the treatment of choice are reached with complete resection and histopathological examination. Here we describe a case with clinical presentation similar to that of a myxoid cyst.

Renal mixed epithelial and stromal tumor: case report / Tumor epitelial-estromal misto do rim: relato de caso

Mixed epithelial and stromal tumor (MEST) represents a recently described biphasic kidney neoplasm, which predominantly affects perimenopausal females. The authors report the case of a young male patient with a MEST exhibiting positivity for estrogen and progesterone receptors. Computed tomography/magnetic resonance imaging (CT/MRI) showed an expansive lesion affecting the right kidney. Grossly, a solid-cystic tumor was identified, which measured 5.7 × 3.5 × 2.4 cm. On microscopic examination, a biphasic tumor constituted by stromal and epithelial elements, without significant atypias, was identified. The stromal element was composed of spindle cells revealing positive immunoexpression for actin, desmin, vimentin, and estrogen receptors. The epithelial component exhibited a predominantly tubular pattern showing positive immunoreaction for cytokeratins. The diagnosis of MEST was then established.

O tumor epitelial e estromal misto (TESM) representa uma neoplasia renal bifásica descrita recentemente que afeta predominantemente mulheres na perimenopausa. Os autores relatam o caso de um paciente jovem, do sexo masculino, com TESM exibindo positividade para receptores de estrogênio e progesterona. A tomografia computadorizada/ressonância magnética (TC/RM) mostrou lesão expansiva no rim direito. Ao exame macroscópico, identificou-se tumor sólido-cístico medindo 5,7 × 3,5 × 2,4 cm. À microscopia, foi encontrado tumor bifásico constituído por elementos estromais e epiteliais, sem atipias significativas. O componente estromal era composto por células fusiformes, exibindo imunoexpressão positiva para actina, desmina, vimentina e receptores de estrogênio. Os elementos epiteliais mostraram padrão predominantemente tubular e exibiram imunorreação positiva para citoqueratinas. O diagnóstico de TESM foi então estabelecido.

Association between trochanteric bursitis, osteoarthrosis and total hip arthroplasty / Associação entre bursite trocantérica, osteoartrose e artroplastia total do quadril

OBJECTIVE: this was an epidemiological study on trochanteric bursitis at the time of performing total hip arthroplasty. METHODS: sixty-two sequential patients who underwent total hip arthroplasty due to osteoarthrosis, without any previous history of trochanteric bursitis, were evaluated. The bursas were collected and evaluated histologically. RESULTS: there were 35 female patients (56.5%) and 27 male patients (43.5%), with a mean age of 65 years (±11). Trochanteric bursitis was conformed histologically in nine patients (14.5%), of whom six were female (66.7%) and three were male (33.3%). CONCLUSIONS: 14.5% of the bursas analyzed presented inflammation at the time that the primary total hip arthroplasty due to osteoarthrosis was performed, and the majority of the cases of bursitis were detected in female patients...

OBJETIVO: fazer um estudo epidemiológico da bursite trocantérica no momento da feitura da artroplastia total do quadril (ATQ). MÉTODOS: foram avaliados 62 pacientes, sequenciais, submetidos à ATQ por osteoartrose, sem história prévia de bursite trocantérica. As bursas foram coletadas e avaliadas histologicamente. RESULTADOS: foram observados 35 pacientes do sexo feminino (56,5%) e 27 do masculino (43,5%), com média de 65 anos (+/-11). A bursite trocantérica foi confirmada histologicamente em nove (14,5%), seis do sexo feminino (66,7%) e três (33,3%) do masculino. CONCLUSÕES: das bursas analisadas, 14,5% apresentaram inflamação no momento da feitura da ATQ primária por osteoartrose. A maioria das bursites foi detectada nas pacientes femininas...

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.

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.

Piloleiomioma simulando morfea en un niño / Piloleiomyoma mimicking morphea in a child

Los piloleiomiomas son tumores cutáneos raros que tienen origen en el músculo erector del pelo. Presentamos el caso de un niño de tres años de edad con una placa indurada en el glúteo izquierdo, asintomática, que estuvo presente desde los tres meses de edad. El examen anatomopatológico mostró un piloleiomioma. Este tumor benigno es raro en niños y suele ser confundido con otras dermatosis. En este artículo revisamos los casos pediátricos publicados y los aspectos genéticos actuales de esta enfermedad. Además, llamamos la atención para el diagnóstico diferencial con otras patologías en la edad pediátrica (AU)

Piloleiomyomas are rare benign smooth muscle tumors arising from the aerector pili muscles in the skin.We describe a three year-old boy presenting with an asymptomatic hard plaque on the left gluteus, which was present since 3 months of age.The anatomopathological examination evidenced a piloleiomyoma.There are few previous reports of piloleiomyomas in children and so they are frequently confounded with other disorders. In this paper, we review the literature on the pediatric cases reported and the current genetics aspects of this disease. Moreover, we make the differential diagnosis with other entities in children (AU)

Pseudotumor inflamatório do fígado: diagnóstico diferencial de carcinoma hepatocelular / Inflammatory pseudotumor of the liver: differential diagnosis of hepatocellular carcinoma

The authors present a rare case of inflammatory pseudotumor of the liver whose differential diagnosis with hepatocellular carcinoma - in some cases - is only possible with the histological examination. In this case, a female patient, 38 years-old was suffering from abdominal pain, enlarged liver, thinning, tiredness and fever; alpha-fetoprotein, the liver function tests, amylase and lipase were normal. Abdominal ultrasound and computed tomography showed a node in the right liver lobe. The patient was submitted to a right hepatectomy for hepatocellular carcinoma. Histological examination showed an inflammatory pseudotumor of the liver. The patient was discharged from the hospital on the seventh postoperative day; she is asymptomatic for three years.

Neoplasia Endócrina cística näo-funcionante de pâncreas / Non-functioning endocrine cystic neoplasia of the pancreas

é descrito o caso de uma paciente de 45 anos portadora de neoplasia endócrina cística näo-funcionante de pâncreas. Apresentava dor abdominal, exames laboratoriais normais e métodos de imagem que demonstraram lesäo cística na cauda pancreática. O tratamento cpnsistiu em pancreateectomia caudal esplenectomia, sendo o pós-operatório sem complicaçöes. A paciente permanece assintomática após 2 anos de acompanhamento. Apesar da onfrequência dessa forma de apresentaçäo das neoplasias endócrinas pancreáticas, ela deve ser incluida no diagnóstico diferencial das neoplasias císticas do pâncreas

Linfoma Primário de Mama / Primary breast lymphoma

Os autores relatam o caso de uma mulher de 58 anos de idade apresentando um nódulo mamário no quadrante ínfero-lateral da mama esquerda. A punçäo aspirativa realizada previamente à cirurgia (serectomia com esvaziamento axilar ipsolateral) e a congelaçäo evidenciaram carcinoma. O estudo anátomo-patológico, entretanto, mostrou tratar-se de um linfoma-B maligno do tipo difuso de grandes células. Linfomas primários de mama säo entidades raras, e este relato de caso é mais uma contribuiçäo para o entendimento dessa doença

Poroma écrino: relato de caso e revisäo de literatura / Eccrine Poroma: a case report and literature review

Este relato apresenta um caso de poroma écrino, uma neoplasia benigna e incomum da glândula sudorípara, localizado no torax de um menino de treze anos. Como os tumores anexiais têm classificaçöes controversas, os autores também fazem uma revisäo da literatura sobre poroma écrino abordando os mais recentes achados no seu diagnóstico e tratamento. O tumor apresentado neste relato chama a atençäo näo apenas pelo seu tamanho e pela idade do paciente mas também pelo local encontrado

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

Acantosis nigricans: estudo retrospectivo em pacientes endocrinológicos / Acanthosis nigricans: retrospective study in endocrinologic patients

A Acalantosis Nigricans, uma lesäo cutânea que pode ser um marcador de outras patologias, foi avaliada numa populaçäo de 1245 pacientes endocrinológicos na qual mostoru uma prevalência de 4,5 por cento. Dos casos diagnosticados, 66,6 por cento das mulheres, 57,1 por cento dos homens e a totalidade das crianças, eram obseos. Dentre as manifestaçöes de hiperandrogenismo, a acne esteve presente em 61,2 por cento e o hirsutismo em 48,8 por cento das mulheres. O andrógeno mais frequentemente elevado foi a testosterona, seguido pelo sulfato de dehidroepiandrosterona. A expressäo clínica da Acantosis Nigricans foi, na maior parte das vezes, discreta ou moderada. Näo foi observada correlaçäo entre a intensidade de apresentaçäo clínica e histológica da lesäo. Neste trabalho discute-se a importância da detecçäo desta lesäo e o acompanhamento de seus portadores