Extensive renal sinus lipomatosis in xanthogranulomatous pyelonephritis simulating liposarcoma

ABSTRACT Renal replacement lipomatosis is a condition characterized by varying degrees of renal parenchymal atrophy and perirenal fibrofatty proliferation secondary to chronic inflammation such as xanthogranulomatous pyelonephritis. In severe cases, imaging findings can be misdiagnosed as retroperitoneal liposarcoma.

Nevus lipomatosus cutaneous superficialis

Abstract: Nevus lipomatosus cutaneous superficialis is a rare benign hamartomatous skin tumor characterized by dermal deposition of mature adipose tissue. Two clinical forms have been described (classical and solitary types). We describe a case of nevus lipomatosus cutaneous superficialis with a 13-year history of growth in a young woman who had a solitary skin-colored tumoral mass on the right buttock. Histopathological findings were typical and confirmed the diagnosis. In this case, the lesion was a skin-colored isolated mass, as described in the solitary type, but its localization and age of appearance were compatible with the classical type. The combination of simultaneous clinical findings of both types had not been published before.

Nevus lipomatosus cutaneous superficialis

Abstract We report a case of nevus lipomatosus cutaneous superficialis of Hoffman-Zurhelle (NCLS), with multiple lesions, in a ten-year-old child. The NLCS is considered rare. The classical clinical presentation is characterized by multiple skin-colored or yellowish papules and nodules, which can have a linear distribution. Histologically, it is characterized by the presence of mature ectopic adipocytes in the dermis. The main therapeutic option is surgical excision. The classical Nevus lipomatosus cutaneous superficialis is reported in this case.

Lipoma gigante do coxim adiposo bucal: relato de caso e revisão da literatura / Giant lipoma of the buccal fat pad: case report and literature review

INTRODUÇÃO: Lipomas são os tumores benignos mesenquimais mais comuns. Entretanto, são pouco frequentes na face, principalmente o tipo considerado gigante, derivado do coxim adiposo bucal. A literatura é escassa e provavelmente a sua incidência é subestimada. MÉTODO: Relato do caso de tratamento cirúrgico de lipoma gigante bucal e temporal recidivado, e revisão da literatura no Pubmed na língua inglesa e na Revista Brasileira de Cirurgia Plástica. RESULTADOS: O tumor, anatômica e morfologicamente, corresponde ao coxim adiposo bucal, foi totalmente retirado pelo acesso facial e temporal, sem sequelas ao nervo facial. Foram encontrados 31 casos relatados de lipoma de origem do coxim adiposo bucal, entretanto, vários casos foram encontrados e relatados sob outras denominações. DISCUSSÃO: O coxim adiposo bucal é maior e mais complexo que se conhecia, e várias patologias se derivam deste, sendo importante o diagnóstico diferencial do lipoma simples com o de células fusiformes e com o lipossarcoma, devido a sua extrema semelhança. CONCLUSÃO:Nas lesões lipomatosas da face, a possível origem no coxim adiposo bucal deve ser considerada. Um estudo amplo dessas lesões com a finalidade de uniformizar a terminologia e de determinar a sua real incidência deve ser realizado.

INTRODUCTION: Lipomas are the most common benign mesenchymal tumors. Nevertheless, they are infrequent in the face, particularly giant lipomas, which are derived from the buccal fat pad. The literature regarding these tumors is scarce and their incidence is likely underestimated. METHODS: We present a case report of surgical treatment of a relapsed giant buccal and temporal lipoma and review the related English literature in Pubmed and that in the Brazilian Journal of Plastic Surgery. RESULTS: The tumor, which anatomically and morphologically corresponded to the buccal fat pad, was completely excised by facial and temporal access without sequelae to the facial nerve. A total of 31 reported cases of lipoma originating from the buccal fat pad were found; however, several were found and reported under other names. DISCUSSION: The buccal fat pad is larger and more complex than assumed, and several pathologies are derived thereof, making the differential diagnosis of simple lipoma with fusiform cell lipoma and liposarcoma difficult due to their extreme similarities. CONCLUSION: In lipomatous lesions of the face, the possible origin in the buccal fat pad must be considered. An extensive study of these lesions with the purpose of standardizing the terminology and determining its real incidence must be performed.

Caso original de lipo-hipertrofia simétrica: relato de caso e revisão de literatura / An original case of symmetric lipohypertrophy: case report and review of the literature

Paciente do sexo feminino, saudável, meia-idade e com história prolongada de massas com crescimento lento localizadas bilateralmente e simetricamente nos membros superiores e inferiores, próximas às principais articulações. Exames de imagem e patológicos após excisão das massas revelaram massas constituídas por gordura subcutânea normal. Não houve evidências de lipomas bem-circunscritos. Na revisão de literatura, destacaram-se diversos distúrbios lipo-hipertróficos, com possível apresentação simétrica. As características das afecções encontradas não eram, porém, totalmente concordantes com as características de nossa paciente. Embora não de modo absoluto, a doença que mais estreitamente se assemelhou ao caso foi lipodistrofia simétrica rara, conhecida como doença de Madelung. Dos 150 casos relatados, em apenas 3 foi descrito comprometimento dos membros inferiores, como ocorreu em nosso caso. Este relato apresenta descrição detalhada do caso, seu manejo e seguimento no pós-operatório. Os tipos distintos de lipodistrofias simétricas também são discutidos.

A middle-aged healthy woman who presented with longstanding history of slow growing masses located bilaterally and symmetrically on the upper and lower extremities closed to major joints. Imaging and pathology tests following excision of masses revealed normal subcutaneous fat. There was no evidence of well-circumscribed lipomas. A review of the literature identified a number of lipohypertrophic disorders, which may be present in a symmetrical fashion. The characteristics of the disorders, however, did not fully agree with characteristics observed in our patient. Although not absolute, the closest disease found to fit our case is a rare symmetrical lipodystrophy known as Madelung's disease. Of 150 reported cases, only 3 described involvement of lower extremities as seen in our case. We report a detailed description of a case, its management and post-operative follow-up. Different types of symmetrical lipodytrophies are also discussed.

Lipomatose epidural idiopática familiar: relato de três casos e revisão da literatura / Familiar Idiopathic Spinal Epidural Lipomatosis: Report of Three Cases and Review of the Literature

Lipomatose epidural é a doença causada pelo aumento da quantidade de gordura no espaço epidural da coluna vertebral, levando à compressão medular ou radicular. A medula toracolombar é a mais comumente envolvida. É geralmente encontrada em homens. As principais causas são a obesidade, altos níveis de corticoides exógenos ou endógenos e hipotireoidismo. A forma idiopática é rara, correspondendo a 17% dos casos. Manifesta-se como mielopatia compressiva ou das raízes da cauda equina. A ressonância magnética é o exame de escolha; sugere-se o diagnóstico quando a espessura da camada de gordura epidural ultrapassa 6 mm. Nós adotamos neste caso a laminectomia com ressecção da gordura epidural, citada como uma das possíveis formas de tratamento. A maioria dos trabalhos demonstramelhora gradual, acentuada ou completa, do déficit neurológico nos primeiros 2 anos após a cirurgia.

Epidural lipomatosis is a disease caused by increased fat content in the epidural space of the spine, leading to root or spinal cord compression. The thoracolumbar spinal cord is themost commonly involved. It is usually found in men. Themain causes are obesity, high levels of exogenous or endogenous steroids and hypothyroidism. The idiopathic form is rare, accounting for 17% of cases. It manifests as compressive myelopathy or radiculopathy. MRI is the test of choice, suggests the diagnosis when the thickness of the epidural fat exceeds 6mm. We adopt in this case laminectomy with fat resection, cited as one of the possible forms of treatment. Most studies show a gradual improvement, marked or complete neurological recovery in the first two years after surgery.

Síndrome de Proteus: relato de casos / Proteus Syndrome: case reports

INTRODUÇÃO: A síndrome de Proteus é uma doença complexa e rara, classificada nos grupo das hamartoses. Foi primeiramente descrita em dois pacientes, em 1979, por Cohen e Hayden. Existe dificuldade no diagnóstico, sendo comum a confusão com síndromes de Klippel-Trenaunay-Weber, neurofibromatose ou Stuge-Weber. Apresentamos dois casos tratados no Serviço de Cirurgia Plástica e Reparadora da Universidade Federal do Paraná. MÉTODO: Paciente masculino (caso 1), que chegou ao serviço aos 6 anos de idade, tendo como principais apresentações lipomatoses e assimetrias. A segunda paciente (caso 2) deu entrada no serviço com 20 anos de idade e diagnóstico de síndrome de Klippel-Trenaunay-Weber, que posteriormente mostrou se tratar de síndrome de Proteus. CONCLUSÃO: A hipótese etiológica mais aceita para a doença é genética. Acredita-se que exista mosaicismo somático e que a doença seja letal no estado não mosaico. Morte prematura é bastante frequente. Entretanto, a sequela mais comum é a ocorrência de tumores incomuns. O cuidado dos pacientes portadores da síndrome é um desafio devido às suas consequências médicas e psicossociais.

INTRODUCTION: Proteus syndrome is a complex and rare disorder classified as a hamartomatous disease. It was first described in two patients in 1979, by Cohen and Hayden. Proteus syndrome is difficult to diagnose, and is often confused with Klippel-Trenaunay-Weber syndrome, neurofibromatosis, or Sturge-Weber syndrome. In this study we describe two patients who were treated at the Plastic and Reconstructive Surgery Service of the Federal University of Paraná. METHOD: A 6-year-old male patient (case 1) presented to the Service with lipomatosis and asymmetry, as the primary findings. A 20-year-old (case 2) was admitted to the Service with a diagnosis of Klippel-Trenaunay-Weber syndrome, which later was shown to be Proteus syndrome. CONCLUSION: The etiological hypothesis that is most accepted for this disease is genetic. It is believed that somatic mosaicism may occur during pathogenesis, which can be lethal in the mosaic state. Premature death is common. However, the most common sequelae are the occurrence of unusual tumors. The care of patients with this syndrome is a challenge due to medical and psychosocial consequences.

Lipomas gigantes: série de 14 casos / Giant lipomas: a 14-case series

INTRODUÇÃO: Os lipomas são os tumores mesenquimais benignos mais frequentes na população. Para ser considerado gigante, deve apresentar pelo menos 10 cm em uma de suas dimensões ou pesar ao menos 1.000g. O objetivo do trabalho é analisar uma série de casos de lipomas gigantes atendidos em nosso serviço. MÉTODO: Análise retrospectiva de prontuários e registros anatomopatológicos dos diagnósticos de lipomas submetidos a tratamento cirúrgico no período de janeiro 2003 a janeiro 2010. RESULTADOS: Foram avaliados 14 pacientes, com média de idade de 52,3 anos (33-72) e tempo médio de evolução até o tratamento de 4,4 anos (seis meses - 20 anos); 64% dos casos não apresentavam sintomas. Todos os pacientes foram submetidos à ressecção cirúrgica da neoplasia. O peso médio das peças foi de 3,8 kg (512 g - 22 kg) e o tamanho médio no maior eixo foi de 27,5 cm (15-66 cm). Foram observadas complicações pós-operatórias em 42% dos casos. CONCLUSÃO: Observamos períodos prolongados de evolução até o tratamento, mesmo em casos com lesões de grande volume. A exérese cirúrgica aberta deve ser considerada o tratamento de eleição para estes tumores.

INTRODUCTION: Lipomas are the most common benign mesenchymal tumors worldwide. To be considered giant, they must be at least 10 cm in one of its dimensions or weigh at least 1,000 g. The objective of this study is to analyze a series of cases of giant lipomas seen in our practice. METHOD: We conducted a retrospective analysis of the medical and anatomopathological records of patients diagnosed with lipomas who underwent surgery between January 2003 and January 2010. RESULTS: We evaluated 14 patients with a mean age of 52.3 years (range, 33-72 years) and a mean evolution time until treatment of 4.4 years (range, 6 months to 20 years); 64% of cases presented no symptoms. All patients underwent surgical tumor resection. The average weight of the pieces was 3.8 kg (range, 512 g to 22 kg), while the average length of the longest axis was 27.5 cm (range, 15-66 cm). Postoperative complications were observed in 42% of cases. CONCLUSION: We observed prolonged periods of evolution until treatment, even in cases of large-volume lesions. Open surgical excision should be considered the treatment of choice for these tumors.

Lipofibromatosis accompanied by several congenital anomalies, report of a rare case.

Lipofibromatosis is a rare tumor of infancy, which has been reported about 10 years ago, and since then very few reports have been published. This tumor has been reported in association with congenital anomalies such as macrosyndactyly, but there is no report of multiple congenital anomalies with lipofibromatosis in the English literature as far as our knowledge goes. Herein, we report a case of this tumor associated with syndactyly, bilateral complete cleft lip and palate, trigonocephaly, and atrial septal defect.

Intra-articular lipoma arborescens of the knee joint

Intra-articular lipoma arborescens [LA] is a rare entity that can present with monoarticular or polyarticular involvement of joints such as knees, ankles, hips, shoulders, and elbows. We describe a case in a 26-year-old man who presented with intermittent pain and swelling of the left knee joint for the previous 2 years. Physical examination showed only tenderness over the medial line of the left knee joint. MRI found only joint effusion; therefore, the patient was referred to the orthopedic clinic where he underwent arthroscopy, which revealed a diffuse yellow soft tissue synovial papillary growth involving both medial and lateral gutters of the suprapatellar pouch. A biopsy reported intra-articular LA. This is the first case of LA reported in Saudi Arabia. LA should be included in the clinical and radiologic differential diagnosis of cases with persistent knee joint effusion

Recurrent diffuse lipomatosis of the neck--a case report.

Diffuse lipomatosis is a rare disorder of adipose tissue occurring in young people . It has a predilection for trunk and proximal extremities where it presents as poorly circumscribed overgrowth of fatty tissue. Definite diagnosis is established by histological examination of tumor. Though it attains extensive size and has a high tendency to recur, the clinical course is benign.

Nevus lipomatosus cutaneous superficialis--a case report.

We report a case of nevus lipomatosus cutaneous superficialis, an uncommon hamartomatous disorder.