[Thorns in hands, think in spiny keratoderma]. / Espinas en manos, piense en queratodermia espinosa.

Spiny keratoderma is a rare dermatological manifestation that occurs sporadically or hereditarily. These are millimetric hyperkeratotic lesions on the palms and/or soles, usually asymptomatic. Histopathologically, they consist of well-defined columns of parakeratosis on a thinned stratum corneum. Sporadic cases can be associated with chronic diseases or neoplasms. We present a case of palmar spiny keratoderma in a man in the seventh decade of life with cirrhosis due to primary sclerosing cholangitis, and hepatocellular carcinoma. He had remission of the skin lesions two months after performing a liver transplant. This behavior favors the interpretation of spiny keratoderma as a paraneoplastic manifestation of hepatocellular carcinoma. We have not found previous reports of spiny keratoderma from Argentina. We review the literature on this entity.

La queratodermia espinosa palmar es una manifestación dermatológica infrecuente que se presenta de forma esporádica o hereditaria. Se trata de lesiones hiperqueratósicas milimétricas en palmas y/o plantas, habitualmente asintomáticas. Histopatológicamente consisten en columnas de paraqueratosis bien delimitadas sobre un estrato córneo adelgazado. Los casos esporádicos se pueden asociar a enfermedades crónicas o neoplasias. Presentamos un caso de queratodermia espinosa palmar en un hombre en la séptima década de la vida con cirrosis secundaria a colangitis esclerosante primaria y hepatocarcinoma. Presentó remisión de las lesiones cutáneas a los dos meses de realizarse un trasplante hepático. Este comportamiento favorece la interpretación de la queratodermia espinosa como manifestación paraneoplásica del hepatocarcinoma. No hemos encontrado informes previos de queratodermia espinosa en Argentina. Realizamos una revisión de la literatura sobre esta entidad.

Doege-Potter syndrome due to a solitary fibrous tumor of the pleura: a case report.

BACKGROUND: Doege-Potter syndrome is a rare paraneoplastic phenomenon associated with solitary fibrous tumors of the pleura (SFTPs). It is characterized by the presence of severe, sustained, and treatment-refractory hypoglycemia. Hypoglycaemia, which may be the sole symptom at disease onset, is mediated by the secretion of high-molecular-weight insulin-like growth factor (IGF-2). Most tumors exhibit benign behavior, with a 100% survival rate at 5 years. However, 10% of these tumors may display aggressive behavior with local or metastatic recurrence. We present a clinical case of a patient with a benign solitary fibrous tumor of the pleura who presented with symptomatic hypoglycemia and required pulmonary and pleural surgical resection to control the paraneoplastic phenomenon. CASE PRESENTATION: A Hispanic 46-year-old man presented with a 15-day history of transient alterations in consciousness worsened by fasting. The relevant medical history included obstructive sleep apnea treated with continuous positive air pressure (CPAP) and previous smoking. In-hospital studies revealed noninsulinemic hypoglycemia and a benign SFTP. Complete surgical resection was performed while the patient received dextrose fluids and corticosteroids perioperatively for hypoglycemia. Subsequently, the hypoglycemia resolved, and the patient was followed-up without disease recurrence. CONCLUSION: Doege-Potter syndrome is challenging to recognize. However, effective treatment can be achieved with a high survival rate. Raising awareness among healthcare professionals about the recognition of this paraneoplasic syndrome patients will improve diagnostic suspicion, biochemical confirmation, the development of diagnostic and therapeutic guidelines, and the creation of predictive indices for aggressive presentations requiring closer monitoring.

Encephalitis associated with anti-mGluR5 antibodies.

A 30-year-old woman had 5 days of visual hallucinations, nystagmus, memory impairment and mutism. On examination, she was disorientated with reduced attention span, gaze-evoked nystagmus, paratonia and abnormal frontal reflexes. Cerebrospinal fluid (CSF) showed 80 cells, protein 0.41 g/L and glucose 3.2 mmol/L (plasma glucose 5.0 mmol/L). MR scan of the brain showed involvement of limbic and extra-limbic regions and brainstem. Commercial cell-based assays were negative, but tissue-based assays showed neuropil staining, and cell-based assays for anti-metabotropic glutamate receptor 5 (mGluR5) antibodies were positive in serum and CSF. Six months later, she was diagnosed with Hodgkin's lymphoma. This case emphasises the broader clinical spectrum of anti-mGluR5 encephalitis, challenging its initial characterisation as Ophelia syndrome. It underscores the significance of interpreting commercial cell-based assays and advocates for tissue-based assay testing followed by cell-based assay testing in serum and CSF for diagnosing rare autoimmune encephalitis.

[Bazex syndrome: a rare paraneoplastic disease]. / Síndrome de Bazex: una enfermedad paraneoplásica infrecuente.

Bazex syndrome is a paraneoplastic disorder most commonly linked to squamous cell carcinomas of the upper aerodigestive tract, followed by lung cancer and other malignancies. It manifests through three stages of skin involvement that mirror the tumor's progression. Remarkably, skin lesions precede tumor symptoms or diagnosis in two-thirds of cases, underscoring the crucial role of suspecting this condition as it can promptly reveal an underlying neoplasm. Treatment primarily focuses on addressing the root neoplasm, with recurrent skin lesions potentially indicating tumor relapse. In this context, we present a clinical case involving a male patient whose manifestation of this syndrome facilitated the timely diagnosis of lung adenocarcinoma. This case underscores the significance of understanding this uncommon syndrome and its link to cancer, enabling early and accurate oncological diagnosis.

El síndrome de Bazex es una enfermedad paraneoplásica que se asocia con mayor frecuencia a carcinomas de células escamosas del tracto aerodigestivo superior, seguido en frecuencia por el cáncer de pulmón y otras neoplasias. Afecta a la piel en tres etapas que tienen un comportamiento paralelo al crecimiento del tumor. En dos tercios de los casos, las lesiones cutáneas preceden a los síntomas o al diagnóstico del tumor. De ahí la importancia de la sospecha de esta entidad, que puede desenmascarar a la neoplasia asociada en una etapa temprana. Su tratamiento consiste en tratar la neoplasia subyacente. La recurrencia de las lesiones cutáneas puede revelar la recaída del tumor. Comunicamos el caso clínico de un paciente de sexo masculino en el cual el hallazgo de este síndrome permitió realizar el diagnóstico de un adenocarcinoma de pulmón, lo cual destaca la importancia de conocer a esta rara enfermedad y su asociación con cáncer, para poder realizar el diagnóstico oncológico de forma temprana y oportuna.

[Stiff person syndrome associated with thymoma]. / Síndrome de la persona rígida asociado a timoma.

Stiff-person syndrome is a rare neurological condition characterized by muscular rigidity of the trunk and extremities and muscle spasms triggered by sensory or emotional stimuli, which progresses towards prostration. It has a pathophysiogenic mechanism with an immunological basis, in which autoantibodies, such as antiGAD65, play a central role. Likewise, the detection of these antibodies corroborates the diagnosis in a patient with a suggestive clinical picture. Four to 6% of cases have underlying neoplasms. Treatment is based on symptomatic, immunomodulatory, and underlying disease management in paraneoplastic cases. We report a case of classic stiff person syndrome associated with thymoma and review the main characteristics of this entity.

El síndrome de persona rígida es un cuadro neurológico infrecuente caracterizado por rigidez muscular de tronco y extremidades y espasmos musculares gatillados por estímulos sensoriales o emocionales, que progresa hacia la postración. Cuenta con un mecanismo fisiopatogénico con base inmunológica, en el cual los autoanticuerpos, como el antiGAD65, cumplen un rol central. Asimismo, la detección de dichos anticuerpos corrobora el diagnóstico ante un paciente con cuadro clínico sugestivo. Un 4 a 6% de los casos tienen neoplasias subyacentes. El tratamiento se basa en el manejo sintomático, inmunomodulador y de la enfermedad de base en los casos paraneoplásicos. Reportamos un caso de síndrome de persona rígida clásico asociado a timoma y describimos las características principales de esta entidad.

Icteric Variant of Stauffer Syndrome as a Paraneoplastic Manifestation of Type 1 Papillary Renal Cell Carcinoma.

Intrahepatic cholestasis as a paraneoplastic manifestation was first described by Dr. Maurice H. Stauffer in 1961. This paraneoplastic manifestation was primarily associated with renal cell carcinoma characterized by abnormal liver enzymes without hepatic metastasis. Stauffer syndrome is classified into 2 types: classical and jaundice variants. Indeed, the jaundice variant is extremely rare and only described in 13 published cases. We report a case of intrahepatic cholestasis associated with a type 1 papillary renal cell carcinoma with complete resolution after surgical treatment.

Metastatic gastric cancer from breast carcinoma presenting with paraneoplastic rheumatic syndrome: A case report.

BACKGROUND: Breast cancer is the most frequently diagnosed cancer worldwide. It is the leading cause of death by malignant disease in women. CASE SUMMARY: A female patient, 73 years of age, sought care due to weakness, mild abdominal pain, arthralgia, and weight loss. She was taking anastrazole as maintenance therapy for localized breast cancer and had moderate anemia and elevated acute-phase markers. Upper digestive endoscopy showed isolated erosion in the gastric corpus. This lesion was compatible with signet-ring cell adenocarcinoma in anatomopathological study and was confirmed as metastasis of a breast carcinoma in immunohistochemistry, which was positive for estrogen antibody. Further imaging studies determined numerous proximal bone metastases. The patient was treated with prednisone for paraneoplastic syndrome, which improved the anemia and rheumatic disease, and with chemotherapy, which greatly improved the symptoms. She has been followed-up for 6 mo, and her anemia, arthralgias, and acute phase markers have normalized. CONCLUSION: Systemic treatment strategies seem to be the best choice for gastric metastasis from breast cancer, resulting in disease control and relapse-free survival. Prospective studies with longer follow-up are needed to better understand the biological, pathological, and clinicopathological characteristics and outcomes of the endoscopic features associated with metastatic gastric cancer from breast carcinoma.

Severe thyrotoxicosis as initial presentation of gastric choriocarcinoma: a case report.

BACKGROUND: Extragonadal choriocarcinoma is rare and can be associated with hyperthyroidism when producing very high levels of human chorionic gonadotropin. CASE PRESENTATION: A 62-year-old Hispanic female presented with a 3-week history of shortness of breath, palpitations, extreme weakness, new-onset hot flashes, and right flank pain. Her physical examination was remarkable for tachycardia, hepatomegaly, hyperreflexia, and tremor; goiter was absent. Laboratory studies revealed increased lactate dehydrogenase, alkaline phosphatase, suppressed thyroid stimulating hormone, very elevated T4, and absent thyroid stimulating immunoglobulin. 18F-fluorodeoxyglucose positron emission tomography-computed tomography exhibited hepatomegaly with multiple large fluorodeoxyglucose-avid liver masses and a focus of fluorodeoxyglucose avidity in the stomach with no structural correlate. A thyroid scan (99mTcO 4 - ) showed diffusely increased tracer uptake. She was started on propranolol and methimazole. Upon stabilization of severe thyrotoxicosis, upper endoscopy was performed, showing a ~ 5 cm bleeding lesion in the greater stomach curvature body; biopsy was consistent with choriocarcinoma; beta-human chorionic gonadotropin hormone was 2,408,171 mIU/mL. The patient received methotrexate followed by etoposide and cisplatin. Methimazole was titrated down, and upon liver failure the medication was stopped. The thyrotoxicosis was effectively controlled with antithyroid drug and concurrent chemotherapy. At ~ 1.5 months after initial diagnosis, the patient died due to bleeding/acute liver failure with coagulation defects followed by multiple organ failure. CONCLUSIONS: Severe thyrotoxicosis can represent an unusual initial presentation of metastatic choriocarcinoma in the setting of extreme elevation of beta-human chorionic gonadotropin. Primary gastric choriocarcinoma is an aggressive malignancy with very poor outcomes. The co-occurrence of severe thyrotoxicosis with advanced primary gastric choriocarcinoma and imminent liver failure complicates management options.

Enfermedad de cambios mínimos como glomerulopatía paraneoplásica en un paciente con tumor sólido / Minimal Change Disease as a paraneoplastic glomerulopathy in a patient with solid tumor

Resumen La enfermedad de cambios mínimos (ECM) es la principal causa de síndrome nefrótico en niños y una causa poco común en adultos, asociada usualmente a causas secundarias, predominantemente de origen hematológico. Es poco frecuente que este tipo de glomerulopatía se diagnostique de manera sincrónica en pacientes con tumores sólidos. Presentamos el caso de un paciente de 35 años con diagnostico reciente de cáncer de recto quien ingresa con un cuadro compatible con síndrome nefrótico, a quien se le diagnostica, mediante una biopsia renal, una enfermedad de cambios mínimos, con mejoría parcial del cuadro posterior al manejo quirúrgico de la patología neoplásica. Consideramos que este caso puede ser útil en el enfoque diagnóstico y el manejo de pacientes que presenten tumores sólidos de origen gastrointestinal con síndrome nefrótico concomitante.

Abstract Minimal change disease is the main cause of nephrotic syndrome in children and a rare cause in adults, usually associated with secondary causes, predominantly hematological. This type of glomerulopathy is rarely diagnosed synchronously in patients with solid tumors. We present the case of a 35-year-old patient with a recent diagnosis of rectal cancer who is admitted with a condition compatible with nephrotic syndrome, diagnosed with minimal change disease in the renal biopsy, and with partial improvement in the condition after surgical management of neoplastic pathology. We consider that this case may be useful in the diagnostic approach and the management of patients with solid tumors of gastrointestinal origin with concomitant nephrotic syndrome.

Cáncer de mama triple negativo asociado a dermatomiositis paraneoplásica: Reporte de caso y revisión de la literatura / Triple-negative breast cancer associated with paraneoplastic dermatomyositis: Case report and literature review

Resumen La dermatomiositis (DM) es un tipo de miopatía inflamatoria bien definida, inmunomediada, con afectación específica del músculo esquelético y con compromiso variable de piel y otros órganos. Se caracteriza por debilidad muscular proximal, lesiones cutáneas patognomónicas de dermatomiositis como el signo de Gottron, eritema violáceo o heliotropo, y evidencia de inflamación muscular por enzimas elevadas, cambios miopáticos en electromiografía y biopsia muscular anormal. Tiene una asociación bien establecida con diferentes tipos de cáncer pero es rara su asociación con cáncer de mama. Cuando se presentan de manera concomitante, su diagnóstico requiere un estudio multidisciplinario para orientar el origen paraneoplásico frente a una etiología propiamente autoinmune que requiera terapia inmunosupresora dirigida. Describimos el caso de una paciente con diagnóstico simultáneo de carcinoma infiltrante de mama triple negativo y criterios de dermatomiositis como manifestación paraneoplásica.

Abstract Dermatomyositis (DM) is a well-defined immune-mediated inflammatory myopathy, with specific involvement of skeletal muscle and variable involvement of skin and other organs. It is characterized by proximal muscle weakness, pathognomonic skin lesions of dermatomyositis such as Gottron's sign, violaceous or heliotrope rash, and evidence of muscle inflammation due to elevated enzymes, myopathic changes on electromyography, and abnormal muscle biopsy. It has a well-established association with different types of cancer, but its association with breast cancer is rare. When they occur concomitantly, their diagnosis requires a multidisciplinary study to confirm the paraneoplastic origin versus a primarily autoimmune etiology that may require targeted immunosuppressive therapy. We describe the case of a patient with a simultaneous diagnosis of triple-negative infiltrating breast carcinoma and criteria for dermatomyositis as a paraneoplastic manifestation.

Bilateral diffuse uveal melanocytic proliferation with multifocal diffuse integumentary melanocytic proliferation paraneoplastic syndrome: A case report.

Bilateral diffuse uveal melanocytic proliferation (B-DUMP) is a rare paraneoplastic syndrome typically presenting with bilateral visual loss. B-DUMP is associated with extraocular systemic malignancies with the most common being lung cancer in males and uro-gynaecological cancer in females (mainly ovarian cancer). Cutaneous and/or mucosal involvement in patients with B-DUMP has been reported but it is not well characterised. Herein, we present a female in her 70s with diagnosis of stage IV vaginal clear-cell carcinoma and metastatic melanoma of unknown primary that developed progressive bilateral loss of visual acuity compatible with 'B-DUMP'. Simultaneously, she developed multifocal bilateral bluish-greyish patches on the skin that were shown to have a proliferation of dermal melanocytes. We propose that the clinical and histopathologic cutaneous findings seen in patients with B-DUMP be termed 'diffuse integumentary melanocytic proliferation (DIMP)'.

Dermatomiositis y cáncer colorrectal: reporte de caso y revisión de la literatura / Dermatomyositis and colorectal cancer: Case report and literature review

Resumen Los pacientes con dermatomiositis presentan un mayor riesgo de asociación con patología maligna subyacente, sin conocerse una causa precisa de esta relación. Son múltiples las posibles localizaciones anatómicas, entre las que se incluyen los ovarios, mamas, zona gástrica, zona colorrectal, sangre, pulmones y próstata. Se presenta el caso de una mujer de 58 años que durante el estudio de pérdida anormal de peso manifestó debilidad muscular grave y alteraciones dermatológicas asociadas con el hallazgo de adenocarcinoma del colon transverso.

Abstract Patients with dermatomyositis are more likely to have an underlying malignancy, although the exact cause of this association is unknown. There are multiple possible anatomical sites, including ovaries, breasts, stomach, colorectum, blood, lungs, and prostate. We present the case of a 58-year-old woman who during abnormal weight loss study showed severe muscle weakness and skin alterations, associated with finding of adenocarcinoma of the transverse colon.

Pioderma gangrenoso ampollar como forma de presentación de leucemia mieloide agudo: informe de un caso / Acute myeloid leukemia presented in the form of bullous pyoderma gangrenosum: a case report

El pioderma gangrenoso ampollar es una variedad infrecuente de pioderma gangrenoso, que se asocia en el 50-70% de los casos con trastornos oncohematológicos. Se comunica el caso de una paciente de 59 años, que consultó por fiebre y ampollas purpúricas de rápida progresión, con compromiso cutáneo mucoso. Con sospecha de una enfermedad neutrofílica, ampollar, o infección por gérmenes oportunistas, se realizó biopsia de piel para estudio histopatológico, inmunofluorescencia directa y cultivo. Los cultivos y la inmunofluorescencia directa fueron negativos, y la anatomía patológica reveló un denso infiltrado inflamatorio con predominio neutrofílico en dermis. Ante el diagnóstico de pioderma gangrenoso ampollar, se realizó una punción-aspiración de médula ósea cuyo resultado fue compatible con leucemia mieloide aguda. Se instauró tratamiento con corticosteroides sistémicos, a pesar de lo cual la paciente evolucionó desfavorablemente y falleció a los 15 días de su ingreso hospitalario. Este caso ilustra la asociación de esta enfermedad cutánea con trastornos oncohematológicos y el mal pronóstico que esto implica a corto plazo. (AU)

Bullous pyoderma gangrenosum is an infrequent type of pyoderma gangrenosum, associated with onco hematological diseases in 50-70% of cases. We present the case of a 59-year-old patient with fever and mucocutaneous hemorrhagic bullous of rapid progression. A biopsy for histopathology, direct immunofluorescence (DIF) and skin culture was made, considering the possibility of neutrophilic dermatoses, bullous dermatosis or an opportunistic infection. The results of both the culture and the DIF were negative. The histopathological examination of the specimen revealed a dense dermal polymorphic infiltrate composed primarily of neutrophils. Considering bullous pyoderma gangrenosum as a potential diagnosis, a bone-marrow biopsy was performed. This study revealed an acute myeloid leukemia. Although systemic corticosteroid therapy was begun, the patient presented an unfavorable evolution that led to her death 15 days after her admission at the hospital. This case shows the association between bullous pyoderma gangrenosum and onco hematological diseases. In addition, it highlights the poor prognosis related to these diseases in the short term. (AU)

Neuropatia periférica focal associada ao linfoma em cães / Focal peripheral neuropathy associated with lymphoma in dogs

Background: Peripheral neuropathies result in sensory, motor or autonomic dysfunctions due to impairment of peripheral spinal or cranial nerves. Neoplasms such as lymphoma are cited as one of the many aetiological causes and it mayaffect the nerve directly, by compression, or indirectly (paraneoplastic) by remote action of the neoplasm located in anextra-neural site. This study aimed to report two cases of cranial nerve neuropathy (trigeminal and facial) associated withcanine lymphoma, contributing to a better understanding of its paraneoplastic effects on the nervous system, as well asthe diagnosis and treatment of these conditions.Cases: Two cases of canine lymphoma associated with possible signs of paraneoplastic peripheral neuropathy were attendedat the Veterinary Hospital from the Universidade Federal de Minas Gerais (HV UFMG). Case 1. A spayed mixed breedbitch, with lethargy and unilateral exophthalmos. Brain computed tomography revealed a retrobulbar mass and cytology wasdiagnostic for extranodal lymphoma. Subsequent to computed tomography, the dog was presented with hypotrophy of thefacial musculature and difficulty in grasping food, consistent with trigeminal nerve palsy, which resolved after institutionof the 19-week chemotherapy protocol from the University of Wisconsin. Nevertheless, disease reccurred and a rescueprotocol was initiated. Case 2. A female Dalmatian, spayed, was diagnosed with multicentric lymphoma, after cytologyof the left mandibular lymph node. Chemotherapy was initiated with the same protocol of the previous case. However,the disease progressed and it was observed facial asymmetry with ptosis of the left eyelid, pina and lips, in addition todifficulty in grasping food, suggesting facial and trigeminal cranial nerve palsy. Clinical signs resolved after institutionof a rescue chemotherapy protocol. However, in both cases, disease progression and poor clinical condition resulted in...(AU)

Neuropatia periférica focal associada ao linfoma em cães / Focal peripheral neuropathy associated with lymphoma in dogs

Background: Peripheral neuropathies result in sensory, motor or autonomic dysfunctions due to impairment of peripheral spinal or cranial nerves. Neoplasms such as lymphoma are cited as one of the many aetiological causes and it mayaffect the nerve directly, by compression, or indirectly (paraneoplastic) by remote action of the neoplasm located in anextra-neural site. This study aimed to report two cases of cranial nerve neuropathy (trigeminal and facial) associated withcanine lymphoma, contributing to a better understanding of its paraneoplastic effects on the nervous system, as well asthe diagnosis and treatment of these conditions.Cases: Two cases of canine lymphoma associated with possible signs of paraneoplastic peripheral neuropathy were attendedat the Veterinary Hospital from the Universidade Federal de Minas Gerais (HV UFMG). Case 1. A spayed mixed breedbitch, with lethargy and unilateral exophthalmos. Brain computed tomography revealed a retrobulbar mass and cytology wasdiagnostic for extranodal lymphoma. Subsequent to computed tomography, the dog was presented with hypotrophy of thefacial musculature and difficulty in grasping food, consistent with trigeminal nerve palsy, which resolved after institutionof the 19-week chemotherapy protocol from the University of Wisconsin. Nevertheless, disease reccurred and a rescueprotocol was initiated. Case 2. A female Dalmatian, spayed, was diagnosed with multicentric lymphoma, after cytologyof the left mandibular lymph node. Chemotherapy was initiated with the same protocol of the previous case. However,the disease progressed and it was observed facial asymmetry with ptosis of the left eyelid, pina and lips, in addition todifficulty in grasping food, suggesting facial and trigeminal cranial nerve palsy. Clinical signs resolved after institutionof a rescue chemotherapy protocol. However, in both cases, disease progression and poor clinical condition resulted in...

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.

Eletrocardiografia computadorizada em cadelas com tumores mamários / Electrocardiography in bitches with mammary tumors

The incidence of cancer has increased due to greater longevity of the animals as a consequence of better control of other diseases, improvement of nutrition and good practices in preventive medicine. Malignant tumors can cause paraneoplastic syndrome such as hypercalcemia, anemia, cachexia, among others and consequent cardiovascular disorders. The electrocardiography (ECG) is a complementary exam that can reveal with its traces these rhythmic disorders. Based on that, the objective of this study was to evaluate the ECG in bitches with mammary tumors in order to detect in which type of tumor would be found significant changes as in cardiac rhythm, ECG waves, segments or cardiac axis. Twenty-nine female dogs were used in this study, being 19 bitches with mammary tumor, and they were allocated in three groups: G1: control group (n = 10), G2: benign neoplasia group (n = 6) and G3: malignant neoplasia group (n = 13). The most prevalent type of neoplasia in G2 was the benign mixed tumor (83%), followed by adenoma (17%) whereas in G3: carcinoma in mixed tumor (61%); papillary carcinoma (23%). Regarding cardiac rhythm, it was found sinus arrhythmia (SA) and normal sinus rhythm (NS): G1: 50% SA and 50% NS; G2: 67% SA and 33% NS; G3: 54% SA and 46% NS. No ventricular or atrial arrhythmias were detected. For other parameters in G1, G2 and G3, respectively (mean ± Std error): FC (bpm): 110±9.2, 120 ± 8.5, 124±7,5; P (ms): 48 ± 1.6, 51 ± 1.8, 50 ± 1.2; P (mV) 0.19 ± 0.02, 0.2 ± 0.02, 0.19 ± 0.02; PR (ms): 94 ± 4.3, 93 ± 5.5, 89 ± 3.9; QRS (ms): 56 ± 1.54, 60 ± 4, 62 ± 1.2; R (mV): 1.1 ± 0.06, 1.2 ± 0.24, 0.9 ± 0.13; QT (ms): 203 ± 9.4; 204 ± 7.9; 182 ± 15.6; and cardiac axis (°): 66 ± 6.2, 61 ± 7.9, 70 ± 7.5. There were no significant differences for all cardiac parameters and also for ST interval and T wave morphology. All electrocardiographic parameters found are in accordance with other studies carried out in the canine species. The results regarding tumor types differ from what was found in another study, in which in 18 bitches with mammary tumors, 55% were benign, with prevalence of adenomas (38%) followed by benign mixed tumors, and 45% malignant, with adenocarcinoma prevailing (22%). In another study, it was verified in 63 bitches the predominance of tubular carcinoma (26.56%) and carcinoma in mixed tumors (23.44%). Diverging from the electrocardiographic parameters of this study, a significant difference was found in the R wave amplitude value in the research by Barros et al., (2015) who performed computerized electrocardiography in 50 dogs, not only with mammary neoplasms (55% mammary carcinomas), but also in mastocytomas, lymphomas, benign tumors and other sarcomas. This author found out that the R wave amplitude values of the neoplasia group were lower when compared to the control group. In addition, we suspect that other types of tumors could result in more paraneoplastic syndrome than the mammary neoplasms found in this research. Neoplasms as lymphomas (T cells), apocrine gland anal sac adenocarcinomas, multiple myelomas and thymomas are known as a cause of hypercalcemia of malignancy and histiocytic sarcomas, myelomas, leukemia and lymphomas causing anemia. In conclusion, ECG has no changes in benign or malignant mammary tumors in dogs. However, this fact does not exclude the importance of its performance in pre-anesthetic evaluations. Further studies with a larger sample including the clinical staging of these bitches with a balanced number of animals with low and high staging are suggested.(AU)

Sweet syndrome as a paraneoplastic manifestation of cholangiocarcinoma: A case report.

BACKGROUND: Sweet's syndrome, also known as acute febrile neutrophilic dermatosis, is a rare skin disorder that may be associated with cancer. CASE SUMMARY: A 58-year-old female presented with a cholestatic syndrome and significant weight loss three months before admission. Five months earlier, she had abruptly developed skin lesions with erythematous papules that evolved to erythematous blisters. Clinical evaluation and laboratory tests confirmed hepatic cholangiocarcinoma. Skin lesions histopathological findings showed neutrophilic dermatosis, massive edema, fibrin, necrosis, and elastosis. These results, in association with the macroscopic aspects of the findings, led to the diagnosis of paraneoplastic Sweet's syndrome due to cholangiocarcinoma. As staging was consistent with an advanced tumor without a cure perspective, we opted to perform percutaneous biliary drainage, and subsequently, palliative care. Eventually, after a few weeks, the patient died. CONCLUSION: In conclusion, the diagnosis of the underlying disease-causing Sweet's syndrome must be accurate, and patients need to be followed-up, as neoplasia such as cholangiocarcinoma may be a later manifestation.

Paraneoplastic Cerebellar Degeneration Secondary to BRAF Mutant Melanoma Metastasis from an Occult Primary Cancer.

Melanoma metastasis from an unknown primary cancer has an incidence of 3.2% among melanoma patients. Furthermore, paraneoplastic neurological syndromes (PNS) are rare, occurring in 1-3% of patients with malignancies. Paraneoplastic cerebellar degeneration (PCD) is one of the classic PNS and is characterized by acute or subacute onset of ataxia and/or presence of onconeural antibodies. A 61-year-old male with ataxia, vertigo, and headache later developed dysarthria, multidirectional nystagmus, hyperactive delirium, auditory hallucinations, psychomotor agitation, and myoclonus. Toxicological, metabolic, infectious, and autoimmune etiologies were assessed and reported negative. An osteolytic lesion was observed in the right iliac crest via computed tomography (CT). A positron emission tomography-CT reported increased fluorodeoxyglucose uptake of a right iliac and right inguinal ganglion. After biopsy of the right inguinal ganglion, a BRAF mutation-positive melanoma metastasis from an occult primary cancer was diagnosed. Dermatologic, ophthalmologic, and endoscopic gastrointestinal assessment did not reveal a primary malignant melanoma. The patient's movement disorders and neuropsychiatric symptoms improved with quetiapine, prednisone, azathioprine, and cyclophosphamide. Oncological management was conducted with MAPK pathway inhibitors (i.e., dabrafenib and trametinib). Movement disorders associated with neuropsychiatric symptoms are complex to diagnose. PNS are rare and often associated with antibodies against neural antigens expressed by the tumor. The case presented above describes a patient with a BRAF-positive malignant melanoma metastasis from an occult primary associated with PCD - to the best of our knowledge, the first reported in the literature.