Variante "no clásica" o "atípica" del síndrome de Stauffer en paciente con carcinoma renal de células claras: reporte de caso / "Non-classical" or "atypical" variant of Stauffer syndrome in a patient with clear cell renal carcinoma: a case report

La manifestación paraneoplásica conocida como síndrome de Stauffer tiene una presentación atípica, caracterizada por ictericia y colestasis intrahepática. Presentamos el caso de un paciente de 53 años de edad, con antecedente de una masa renal derecha en plan de resección quirúrgica programada, con cuadro de evolución de dolor abdominal en hipocondrio derecho e ictericia. A su ingreso se documentó hepatoesplenomegalia, elevación de bilirrubinas a expensas de la directa, y de fosfatasa alcalina junto con elevación de transaminasas. Se descartaron causas obstructivas a nivel de vía biliar intra y extrahepática. No se documentaron metástasis o lesiones focales a nivel de parénquima, ni lesiones de etiología vascular que explicaran el cuadro. También se descartó hepatitis B, C e infección por VIH, por lo cual se consideró un probable síndrome de Stauffer. Fue llevado a nefrectomía intrahospitalaria, con posterior diagnóstico patológico compatible con carcinoma de células claras. Luego del procedimiento se normalizó la bioquímica hepática y se corrigió la ictericia. Es importante reconocer que la afectación hepática en el contexto de neoplasias, no es solo atribuida a metástasis a distancia, sino también a la existencia de síndromes paraneoplásicos como condicionantes.

The paraneoplastic manifestation known as Stauffer syndrome has an atypical presentation, characterized by jaundice and intrahepatic cholestasis. We present the case of a 53-year-old patient, with a history of a right renal mass with a planned surgical resection, who developed abdominal pain in the right upper quadrant and jaundice. Upon admission, hepatosplenomegaly, elevated bilirubin, at the expense of direct bilirubin, alkaline phosphatase and elevated transaminases were documented. Intra- and extrahepatic bile ducts obstruction were ruled out. There were no documented metastases or focal lesions at the level of the parenchyma, or lesions of vascular etiology that could explain the condition. Hepatitis B, C and HIV infection were also ruled out, and a probable Stauffer syndrome was considered. In-hospital nephrectomy was performed, with subsequent pathology compatible with clear cell carcinoma. After the procedure, liver biochemistry was normalized and jaundice was corrected. It is important to recognize that liver involvement in the context of neoplasms is not only attributed to distant metastases but to the existence of paraneoplastic syndromes as determining factors.

La dermatomiositis paraneoplásica como una presentación inusual de cáncer de mama / Paraneoplastic dermatomyositis as an unusual presentation of breast cancer

Introducción: La dermatomiositis es una inflamación muscular autoinmune con presencia de rash, se manifiesta con debilidad muscular proximal, asociado a complicaciones cardiacas como miocarditis y/o trastornos de la conducción. En algunas ocasiones puede ser la única manifestación clínica de una neoplasia maligna oculta, por lo cual su detección temprana puede tener grandes repercusiones en el pronóstico del tumor oculto. Objetivo: Describir el caso de una paciente con un síndrome paraneoplásico dado por una dermatopolimiositis como presentación inusual de un cáncer de mama. Caso clínico: Se trata de una paciente en quinta década de la vida sin antecedentes personales de importancia, que debuta con una debilidad en miembros superiores de predominio proximal incapacitante, fiebre y elevación importante de la creatinquinasa (CPK), bajo un diagnóstico de una dermatopolimiositis como manifestación principal de un cáncer de mama infiltrante. Conclusiones: La dermatomiositis en mujeres de mediana edad debe hacer sospechar en una patología neoplásica oculta. Lo más importante es descastar el cáncer de mama(AU)

Introduction: Dermatomyositis is an autoimmune muscle inflammation exhibited rash, and proximal muscle weakness, associated with cardiac complications such as myocarditis and / or conduction disorders. In some cases, it can be the only clinical manifestation of hidden malignancy, so its early detection can have great repercussions on the prognosis of the hidden tumor. Objective: To describe the case of a patient with a paraneoplastic syndrome caused by dermatopolymyositis as an unusual presentation of breast cancer. Clinical case report: This is a patient in her fifties with no significant clinical personal history, who had disabling proximal weakness, predominantly in her upper limbs, fever and significant elevation of creatine kinase (CPK), under a diagnosis of dermatopolymyositis as the main manifestation of an infiltrating breast cancer. Conclusions: Dermatomyositis in middle-aged women should make us suspect an occult neoplastic pathology. The most important is to rule out breast cancer(AU)

Síndrome vascular acral secundária à neoplasia de pâncreas. Relato de caso e revisão de literatura / Acral vascular syndrome secondary to pancreatic cancer: case report and literature review

Paciente do sexo feminino, de 65 anos, foi admitida em nossa instituição com síndrome do dedo azul. Durante a investigação diagnóstica para as principais etiologias possíveis, foi evidenciado adenocarcinoma de cabeça de pâncreas, tendo sido elaborada associação como manifestação paraneoplásica. A investigação da etiologia da síndrome do dedo azul é de suma importância para a conduta terapêutica adequada, já que as causas podem ter evolução extremamente grave na ausência de intervenção, como é o caso dos pacientes que apresentam tal síndrome secundária à neoplasias.(AU)

A 65-year-old female patient was admitted to our institution with the blue toe syndrome. During the diagnostic investigation for possible etiologies, pancreatic head carcinoma was evidenced, and an association as a paraneoplastic manifestation was established. The investigation of the etiology of the blue toe syndrome is very important for the proper treatment, since the causes may have extremely serious development in the absence of intervention, as it is the case of patients with this syndrome secondary to cancer.(AU)

The coexistence of hypercalcemia and hypoglycemia in a patient with a renal tumor and B cell lymphoma

SUMMARY Paraneoplastic syndromes are a heterogeneous group of malignant diseases caused by events which involve endocrine, immune and metabolic aspects and whose symptoms vary according to the substance produced and the primary tumor. Hypercalcemia is a frequent complication in cancer patients. Prognosis of cancer patients with hypercalcemia is usually poor. A factor called parathyroid hormone related peptide, whose actions are similar to those of the parathyroid hormone, is thought to be the most common cause of malignancy associated hypercalcemia. Non-islet hypoglycemic cell tumor consists of a rare syndrome characterized by the presence of a solid tumor and severe fasting hypoglycemia determined by an insulin-independent pathway. We report a case of a 59-year-old-man with a renal tumor and a T-cell rich large B cell lymphoma who was hospitalized due to severe hypercalcemia and hypoglycemia. The laboratory examination reported hypercalcemia with inhibited PTH and hypoglycemia with inhibited insulin secretion, arriving to the conclusion of tumoral peptide production. He received denosumab and corticoid therapy. The patient died one month later despite initial improvement after medical treatment. While a single paraneoplastic manifestation may be expected in most tumors, the coexistence of two or more of them is rare, except in hepatocellular carcinomas, and it has not yet been described in renal tumors.

Small cell lung cancer associated with multiple paraneoplastic syndromes / Cáncer de pulmón de células pequeñas asociado a múltiples síndromes paraneoplásicos

Abstracts We report the case of a patient presenting with multiple severe electrolyte disturbances who was subsequently found to have small cell lung cancer. Upon further evaluation, she demonstrated three distinct paraneoplastic processes, including the syndrome of inappropriate antidiuretic hormone, Fanconi syndrome, and an inappropriate elevation in fibroblast growth factor-23 (FGF23). The patient underwent one round of chemotherapy, but she was found to have progressive disease. After 36 days of hospitalization, the patient made the decision to enter hospice care and later she expired.

Resumen Se reporta el caso de una paciente que ingresó al hospital para evaluación de múltiples trastornos electrolíticos y, posteriormente, se le hizo el diagnóstico de cáncer de pulmón de células pequeñas. Tras la evaluación médica, se detectaron tres síndromes paraneoplásicos: síndrome de secreción inadecuada de hormona antidiurética, síndrome de Fanconi y elevación inapropiada del factor 23 de crecimiento de fibroblastos. Se le administró quimioterapia sin éxito, por lo cual se decidió darle tratamiento paliativo y, un tiempo después, falleció.

Hipoglicemia inducida por tumor fibroso solitario pulmonar: síndrome de Doege-Potter / Hypoglycemia induced by a solitary fibrous tumor of the lung or Doege-Potter syndrome: report of one case

We report the case of a 75-year-old man who was admitted to the hospital with symptoms of severe hypoglycemia. He had a history of solitary fibrous tumor diagnosed by Pathology after its complete surgical resection eight years before. The laboratory examination reported hypoglycemia with inhibited Insulin secretion. A computed tomography of the thorax revealed a large solid heterogeneous mass in the left hemithorax. Solitary fibrous tumor is a rare neoplasm. The association of solitary fibrous tumor and paraneoplastic hypoglycemia is known as Doege-Potter syndrome and occurs in less than 5% of all solitary fibrous tumors.

Caso clínico: hipercalcemia refractaria asociada a linfoma b, una emergencia oncológica / Clinical case: Refractory hypercalcemia associated to B-cell lymphoma, an oncological emergency

La hipercalcemia asociada a procesos tumorales es un hallazgo poco frecuente en la edad pediátrica. El manejo terapéutico va encaminado a favorecer la calciuresis con diversos métodos farmacológicos e, incluso, técnicas de depuración extrarrenal. El objetivo de la exposición de este caso clínico es presentar a un paciente con hipercalcemia grave refractaria, que solo respondió a un tratamiento etiológico precoz con el empleo de quimioterapia, y se evitaron así las posibles complicaciones secundarias a dicha alteración electrolítica.

Hypercalcemia as a paraneoplastic syndrome is rare in children. Therapeutic management is aimed at promoting calciuresis with various pharmacological methods, even with extrarenal purification techniques. The aim of presenting this case is to highlight the importance of early etiologic treatment through chemotherapy as an urgent treatment in a refractory and severe hypercalcemia case, in order to avoid possible secondary complications due to this electrolyte disturbance.

Ataxia e coreia como manifestações de síndrome paraneoplásica / Ataxia and chorea related to paraneoplasic syndrome

Relatamos um caso de ataxia subaguda e coreia em uma paciente adulta. Após extensa investigação, foi estabelecido diagnóstico de neoplasia de mama em associação com síndrome paraneoplásica (SP). SP é rara e pode ser o primeiro sintoma de uma neoplasia oculta. Ataxia é o distúrbio de movimento mais comumente relacionado à SP, no entanto a apresentação de coreia é incomum. A paciente apresentou instalação subaguda de ataxia e coreia. Assim, a SP neurológica deve ser considerada no diagnóstico diferencial de ataxia associada à coreia esporádica a despeito de tal apresentação ser rara.

We report a case of subacute ataxia and chorea in a female adult patient. After an extensive investigation breast cancer was discovered. The final diagnosis was paraneoplastic syndrome (PS). PS is rare and it could be the first symptom of an occult neoplasia. Ataxia is one of the most common movement disorder related to PS although chorea is rare. The patient presented ataxia and chorea, which is not common. Therefore, neurologic PS is important to be considered in the differential diagnosis of sporadic ataxia, as well as chorea.

Remisión completa de síndrome nefrótico asociado a carcinoma cervicouterino luego de tratamiento del tumor: Comunicación de 2 casos / Complete remission of nephrotic syndrome associated with carcinoma of the cervix after treatment of the tumor: Report of two cases

Nephrotic syndrome secondary to paraneoplastic glomerulopathies is exceptional. We are aware of only three cases reported of cervical carcinomas associated with nephrotic syndrome. Two women, aged 40 and 79 years, presented with nephrotic syndrome. The first had a membranous nephropathy and the second was not biopsied. The first women had a metrorrhagia after 8 months of unsuccessful therapy with corticosteroids and immunosuppressive drugs. An advanced cervical carcinoma with lymph node metastases was found. In the second patient, a cervical carcinoma and hematometra was discovered two months after diagnosis ofa nephrotic syndrome. The syndrome subsided completely, nine months after radiotherapy and chemotherapy in the first patient and 10 months after hysterectomy in the second patient.

Poroceratose superficial disseminada num doente com colangiocarcinoma: manifestação paraneoplásica? / Disseminated superficial porokeratosis in a patient with cholangiocarcinoma: a paraneoplastic manifestation?

As poroceratoses compreendem um grupo de doenças da queratinização epidérmica, hereditárias ou adquiridas, caracterizadas histologicamente pela presença de lamela cornoide. A variante clínica designada por poroceratose superficial disseminada tem sido descrita na literatura, associada a estados de imunossupressão, como transplantação de órgãos, terapêuticas imunossupressoras e infecções. A sua associação a neoplasias sólidas foi raramente descrita na literatura, estando publicados apenas 5 casos. Descrevemos o caso clínico de um paciente que desenvolveu, subitamente, lesões de poroceratose superficial disseminada, concomitantemente ao diagnóstico de um colangiocarcinoma.

Porokeratosis refers to a group of hereditary or acquired disorders of epidermal keratinization and is characterized histologically by the presence of a cornoid lamella. The clinical variant referred to as disseminated superficial porokeratosis has been described in the literature in association with immunosuppressive conditions that include organ transplant, infections and immunosuppressive treatments. The association of disseminated superficial porokeratosis with solid organ malignancies has seldom been described, only 5 such cases having been published. The present report refers to a patient with lesions of disseminated superficial porokeratosis of sudden onset shortly before diagnosis of a cholangiocarcinoma.

Eritema necrolítico migratorio: a propósito de un caso / Necrolytic migratory erythema: a case report

Presentamos el caso de una paciente de 62 años con un cuadro de tres años de evolución, caracterizado por placas y pápulas eritematosas arciformes que comenzaron en cara, extendiéndose luego al resto del cuerpo, asociado a baja de peso y depresión. Después de un completo estudio y dos biopsias de piel se diagnóstica eritema necrolítico migratorio (ENM). El ENM junto con estomatitis/glositis, baja de peso, diarrea, diabetes y anemia forman el síndrome paraneoplásico asociado al tumor de células a pancreáticas, llamado síndrome del glucagonoma, El ENM corresponde a lesiones maculopapulares, coalescentes, de borde serpiginoso, acompañadas de una bula central que se erosiona y forma costras. La biopsia cutánea muestra hiperplasia psoriasiforme y espongiótica, paraqueratosis y separación de las capas superficiales de la epidermis. La resección del tumor conduce a la resolución del ENM. Presentamos este caso y revisión del tema por la baja frecuencia de esta enfermedad y para reforzar lo importante de su sospecha temprana.

We report the case of o 62 year old woman with a 3 year history of erythematous arciform plaques and papules that began in the face and spread to the rest of the body. These lesions were associated with depression and weight loss. After a comprehensive study and two skin biopsies, necrolytic migratory erythema (NME) was diagnosed together with glossitis/stomatitis, weight loss, diarrhea, diabetes and anemia, NME is part of the paraneoplastic syndrome associated with a cell pancreatic tumor, known as glucagonoma syndrome NME lesions are characterized by a coalescent maculopapular rash with a serpiginous edge and a central bulla that erodes and become crusted. Histological studies show a psoriasiform and spongiotic hyperplasia, porakeratosis, and detachment of the superficial layers of the epidermis. ENM usually resolves after tumor resection. We present this case and a review of the literature because of the low frequency of this disease and to reinforce the importance of its early suspicion.