Paraneoplastic Sweet's syndrome

Abstract: Sweet's syndrome is a rare dermatosis with little-known pathogenesis, associated with some clinical conditions such as infections, autoimmune diseases, inflammatory bowel diseases, vaccination, medications and neoplasms. Hematologic malignancies are the diseases most related to paraneoplastic Sweet's syndrome, but this clinical entity can also be found occasionally in some solid tumors, including genitourinary tract tumors. We report a rare case of paraneoplastic Sweet's syndrome associated with the diagnosis of cervical cancer.

Oligoartritis, paniculitis y pioderma gangrenoso como manifestaciones de un Linfoma no Hodgkin / Oligoarthritis, panniculitis and pyoderma gangrenosum associated with non-Hodgkin lymphoma: report of one case

An assortment of clinical and laboratory abnormalities may occur as paraneoplastic syndromes in lymphomas. Rheumatological and dermatological manifestations such as paraneoplastic arthritis and pyoderma gangrenosum must be underscored. We report a 28 years old woman who developed pyoderma gangrenosum and two years later presented with arthritis of knees and ankles associated with panniculitis interpreted as erythema induratum that was pathologically confirmed. She developed a reactivation of pyoderma gangrenosum, that was refractory to treatment. Complementary studies showed a pulmonary nodule and a right paravertebral mass with involvement of the psoas muscle. Biopsies of both masses and a new pathological skin study demonstrated a large B-cell non-Hodgkin's lymphoma.

Heart failure as the first manifestation of renal cell carcinoma

We report the rare case of a patient with advanced renal cell carcinoma (RCC) who initially presented to the hospital with symptoms of cardiac failure. Preoperative cardiac studies did not reveal any underlying ischemia. After resection of a large 14-cm left renal tumor, cardiac function was noted to improve dramatically. We discuss this case of concomitant RCC and nonischemic cardiomyopathy.

A case of Paraneoplastic Pemphigus associated with Castleman's disease / Um caso de Penfigo Paraneoplastico (PNP) associado a doenca de Castleman

We present a case of PNP associated with Castleman's Disease. We have also reviewed the literature and described the characteristics of the two associated diseases. Gene clonal rearrangement was done to help diagnosis. We used, in addition, stereotactic radiosurgery which, as far as we know, has never before been employed to treat PNP associated with Castleman's Disease. This produced a good response, suggesting that it might be a good alternative treatment for PNP associated with Castleman's Disease when it is too difficult to operate.

Apresentamos um caso de PNP associada à doença de Castleman.Também revisamos a literatura, e referenciamos as características das duas doenças associadas. Um rearranjo genético clonal foi feito para ajudar o diagnóstico. Além disso, usamos a radiocirurgia que até então nunca havia sido utilizada para tratar PNP associada à doença de Castleman. Esta produziu uma boa resposta, sugerindo que pode ser uma boa alternativa para o tratamento de PNP associada com a doença de Castleman quando é muito difícil fazer uma cirugia convencional.

Manifestaciones cutáneas de enfermedades sistémicas / Cutaneous manifestations of systematic diseases

Un gran número de enfermedades sistémicas durante su evolución pueden presentar manifestaciones cutáneas, enocasiones propias de la dolencia, aunque en otras pueden ser el único indicador de la presencia de una enfermedadinterna asintomática, por ello es fundamental su reconocimiento. Su aparición debe alertar al médico sobre el probableinicio de ciertos desórdenes sistémicos, lo que permitirá realizar un diagnóstico precoz, con las implicanciasclínicas y terapéuticas de intervención a corto y largo plazo. El propósito de este trabajo es describir las lesionescutáneas que con mayor frecuencia se asocian a enfermedades internas, lo cual puede ser de gran utilidad para elejercicio de médicos generalistas, internistas y dermatólogos.

Skin signs of systemic diseases occur frequently, and sometimes constitute the first symptoms of an internal disease; furthermore,these manifestations may be the sole expressions of otherwise asymptomatic systemic disorders, so it is neecessary tobecome acquainted with them. Their appearance should alert us of the likely onset of a systemic disorder, which allows anearly diagnosis, with clinical and therapeutic implications both in the short and the long term. We describe the clinicalfeatures of skin lesions observed in several internal diseases which will be useful to general practitioners, internists anddermatologists in the diagnosis of systemic diseases.

Paraneoplastic cutaneous manifestations: concepts and updates* / Manifestações cutâneas paraneoplásicas: conceitos e atualizações

The skin often signals systemic changes. Some neoplastic diseases that affect internal organs may trigger several cutaneous manifestations. Although these dermatoses are relatively unusual, the recognition of some typical paraneoplastic dermatoses may lead to the early diagnosis of a neoplasm and determine a better prognosis. In this review article, we discuss the paraneoplastic cutaneous manifestations strongly associated with neoplasms, which include acanthosis nigricans maligna, tripe palms, erythema gyratum repens, Bazex syndrome, acquired hypertrichosis lanuginosa, necrolytic migratory erythema, Leser-Trélat sign and paraneoplastic pemphigus. We also review the clinical manifestations of each condition and include updated knowledge on disease pathogenesis.

A pele é, muitas vezes, reflexo de manifestações sistêmicas. Doenças neoplásicas que afetam órgãos internos podem exibir manifestações cutâneas diversas. Apesar de relativamente incomuns, o reconhecimento de dermatoses paraneoplásicas pode levar ao diagnóstico precoce da neoplasia e, consequentemente, determinar melhor prognóstico. Nesta revisão serão discutidas as manifestações cutâneas paraneoplásicas com maior força de associação a neoplasias, que incluem acantose nigricante maligna, paquidermatoglifia adquirida, erythema gyratum repens, síndrome de Bazex, hipertricose lanuginosa adquirida, eritema necrolítico migratório, sinal de Leser-Trélat e pênfigo paraneoplásico. Para cada condição serão revisadas e atualizadas as manifestações clínicas, principais neoplasias associadas e etiopatogenia.

Signo de Leser-Trélat asociado a adenocarcinoma gástrico: Caso clínico / Leser-Trélat sign associated with gastric cancer: Report of one case

We report a 66-year-old male presenting with malaise, heartburn and pruritic seborrheic keratoses in both feet of sudden onset, suggesting a Leser-Trélat sign. An upper gastrointestinal endoscopy disclosed a gastric cancer. The patient was subjected to a total gastrectomy and duringfollow up, the skin lesions had disappeared.

Dermatomiosite vesiculobolhosa como manifestação paraneoplásica de tumores sincrônicos de próstata e língua: relato de caso / Paraneoplastic vesiculobullous dermatomyositis with synchronic prostate and tongue tumors: case report

A dermatomiosite é uma miopatia inflamatória que cursa com manifestações cutâneas. No adulto acima de 50 anos, pode estar associada a neoplasias malignas, comportando-se como um grande sinalizador de malignidade. OBJETIVO: Demonstrar essa associação com aspectos peculiares: dermatomiosite de apresentação atípica paralela a dois tumores sincrônicos, em geral, não relacionados àquela. RELATO DO CASO: Descrevemos o caso de um paciente do gênero masculino de 72 anos, que desenvolveu dermatomiosite, inicialmente apenas com quadro cutâneo clássico, evoluindo com lesões vesiculobolhosas, e, meses após, com miopatia. Após extensa investigação, foi localizado um adenocarcinoma de próstata. Houve remissão da doença após tratamento do câncer e administração de glicocorticoide. Durante a retirada gradual do glicocorticoide, ocorreu reativação da dermatomiosite, sendo reiniciadas investigações, que revelaram a presença de carcinoma escamoso de língua. Tratada esta neoplasia, houve remissão completa, mesmo após a retirada total do corticoide. CONCLUSÃO: Trata-se de um caso raro por envolver as formas menos usuais de apresentação da dermatomiosite, tanto com relação ao quadro cutaneomuscular, quanto à sua associação a tumores de próstata e língua (tumores nunca antes relatados juntos). Este caso demonstra a importância da investigação minuciosa em busca de neoplasias na abordagem desses pacientes.

Dermatomyositis is an inflammatory myopathy with skin manifestations. In the adult over the age of 50 years, it can be associated with malignant neoplasias, being, thus, a signal of malignancy. OBJECTIVE: To show the association of dermatomyositis of atypical presentation with two synchronous tumors, usually not related to that. CASE REPORT: We report the case of a 72-year-old male, who developed dermatomyositis, initially with only classic skin findings, which progressed to vesiculobullous lesions, and, months later, to myopathy. After extensive investigation, prostate adenocarcinoma was diagnosed. After treatment of the cancer and administration of glucocorticoid, the disease went into remission. During gradual glucocorticoid withdrawal, dermatomyositis recurred, and the new investigation revealed the presence of squamous cell carcinoma of the tongue. After treating this neoplasia, complete remission occurred, even after total corticoid withdrawal. CONCLUSION: This is a rare case involving less usual dermatomyositis presentation forms, relating to the cutaneous-muscle findings and the association with prostate and tongue tumors (tumors never reported together). This case demonstrates the importance of a careful investigation, searching for neoplasias, when approaching such patients.

The Incidence and Clinical Significance of Paraneoplastic Syndromes in Patients with Hepatocellular Carcinoma / 대한간학회지

BACKGROUND/AIMS: Patients with hepatocellular carcinoma (HCC) may manifest paraneoplastic syndromes such as hypercholesterolemia, hypoglycemia, hypercalcemia and erythrocytosis. This study was aimed at evaluating the incidence and clinical significance of paraneoplastic syndromes in Korean HCC patients. METHODS: The medical records of 165 HCC patients who were diagnosed and died in the Kyung Hee University Hospital, were reviewed retrospectively. The following variables were analyzed: age, gender, hepatitis markers, platelet, liver function test, alpha-fetoprotein (AFP), Child-Pugh score, tumor features, and the duration of their survival. RESULTS: In total, paraneoplastic syndromes were presented in 43.6% of the HCC patients during the course of their disease. Hypercholesterolemia was solely presented in 14.5%, hypoglycemia in 12.7% and hypercalcemia in 7.8%. The patients who presented with more than 2 syndromes were 8.5%. While 80% of erythrocytosis (4/5) and 51.6% of hypercholesterolemia (16/31) was presented at the time of HCC diagnosis, hypoglycemia and hypercalcemia mainly occurred as terminal events. The patients with paraneoplastic syndromes were younger and had higher rates of portal vein thrombosis, bi-lobar tumor involvement and tumor more of more than 10 cm in diameter, compared to those patients without them. The proportion of patients with a serum AFP more than 400 ng/mL tended to be higher in the patients with paraneoplastic syndromes. The HCC patients with paraneoplastic syndromes, except for erythrocytosis, had a shorter survival than those patients without them. CONCLUSIONS: Paraneoplastic syndromes are not infrequently presented in HCC patients, especially at an advanced stage, and the survival of these patients is relatively shorter.

Manisfestações músculo-esqueléticas das síndromes paraneoplásicas / Rheumatic paraneoplastic syndromes

Tumores podem causar o aparecimento de síndromes clínicas não relacionadas aos seus efeitos locais ou metástases, conhecidas como paraneoplásicas. O seu reconhecimento é fundamental porque pode permitir o diagnóstico precoce de uma neoplasia. Os principais mecanismos e as características clínicas das síndromes paraneoplásicas músculo-esqueléticas são revisadas neste artigo.

A Case of Paraneoplastic Nephrotic Syndrome in a Patient with Ovarian Carcinoma

Nephrotic syndrome is a rare manifestation of malignancy associated with paraneoplastic syndrome. Paraneoplastic nephrotic syndrome has been reported in various malignancies: malignant lymphoma, colon cancer, lung cancer and prostate cancer. However, an ovarian carcinoma associated with nephrotic syndrome has rarely been reported. Only six cases of ovarian carcinoma associated paraneoplastic nephrotic syndrome has been reported worldwide, but no cases have been reported in Korea. Here, we report a case of paraneoplastic nephrotic syndrome in a patient with an ovarian carcinoma. The patient presented with ascites, proteinuria and hypoalbuminemia. An initial computed tomography (CT) scan and ultrasonography evaluations showed no specific findings suggestive of an ovarian tumor. Despite treatment for nephrotic syndrome, the symptoms became more aggravated. There after, follow up evaluation at Yonsei University Medical Center, including serum CA 125, pelvis MRI and peritoneal fluid examination were performed. On the pelvis MRI, a left ovarian mass was detected with an ascitic fluid collection. The serum CA 125 level was elevated to 2211 U/ml. The peritoneal fluid cytological examination showed malignant cells suggestive of an ovarian carcinoma. Combination chemotherapies including paclitaxel plus carboplatin, topotecan plus gemcitabine and oxaliplatin plus capecitabine were administered to the patient, and complete remission was achieved on image and tumor marker studies. There was complete recovery from the nephrotic syndrome with no evidence of ascites and proteinuria. These findings suggest that nephrotic syndrome caused by paraneoplastic syndrome can be resolved only after the complete control of the underlying malignancy.

Paraneoplastic limbic encephalitis associated with bronchogenic carcinoma: a case report.

Paraneoplastic limbic encephalitis is a rare clinical entity, associated most often with the oat cell carcinoma of the lung. Clinically, it presents with affective changes in personality, memory loss, confusional state, hallucinations, and seizures; with dementia being the common feature as the disorder progresses. Response to treatment is disappointingly poor.

Detecçäo de síndrome paraneoplásica pela cintilografia óssea / Detection of paraneoplastic syndrome using bone scintigraphy

O melanoma maligno é um dos poucos tumores que podem causar hipercalcemia como síndrome paraneoplásica. Este relato demonstra a utilidade da cintilografia com MDP-99mTC no diagnóstico desta síndrome. A captaçäo difusa e acentuada do radiofármaco em partes moles, associada à presença de múltiplas metástases ósseas, levou à hipótese de hipercalcemia por síndrome paraneoplásica e, posteriormente, ao diagnóstico final de melanoma maligno

Manifestaciones ungueales de tumores malignos internos / Nail manifestations of internal malignant tumors

Las uñas pueden ser de gran ayuda en la pesquisa de tumores malignos en otras zonas del organismo. En ellas pueden estar presentes alteraciones específicas desde el punto de vista histológico, ya que se producen por infiltración de células tumorales, como células de Langerhans en la histiocitosis X, células T atípicas en linfomas T y células metastásicas en algunos casos, como en cáncer pulmonar, mamario, colónico y de recto. Otras manifestaciones ungueales son inespecíficas más frecuentes que las primeras y pueden presentarse en concomitancia con la neoplasia o precederla por meses o años; generalmente regresan al mejorar el tumor, pero no es posible determinar una clara relación causa-efecto, ya que muchas de estas alteraciones tienen múltiples etiologías. La única alteración inespecífica que puede considerarse como paraneoplásica es la acroqueratosis de Basex. Las metástasis en las uñas son muy raras, y se ven en el cáncer pulmonar, mamario, colónico y renal

Encephaloradiculopathy: a non-metastatic complication of hepatocellular carcinoma.

We describe a patient with a silent hepatocellular carcinoma in whom a progressive dysfunction developed in the brainstem, cerebrum, and cerebellum. This clinical manifestation was also associated with polyradiculopathy. The changes were microscopically unlike those seen in hepatic encephalopathy, progressive multifocal leukoencephalopathy and other metabolic encephalopathy. The findings seem to have occurred as a paraneoplastic syndrome of the nervous system. Although in the present case, the cause-and-effect relationship between the hepatocellular carcinoma and the encephaloradiculopathy is only hypothetical, we suggest that the demyelinating process which involves both central and peripheral nervous system in our case resulted from interaction of tumor with the host-immunological mechanism. The neurological presentation in our case is unique and has never been reported before.

Síndrome de Trousseau y cáncer de pulmón: revisión de un caso

Es importante el conocimiento por los clínicos del síndrome de Trousseau (tromboflobitis migratoria) debido a su asociación a ciertas neoplasias malignas, por lo que el hallazgo casual de este síndrome paraneioplásico nos encausa a la búsqueda de una distasia oculta. En el caso que presentamos el síndrome aparecio 6 meses antes de que se diagnosticara el Ca pulmonar