ABSTRACT Merkel cell carcinoma is an aggressive malignancy that frequently recurs/disseminates, but metastases to the genitourinary tract are rare. Only eight cases of Merkel cell carcinoma metastatic to the testis are reported. We describe the ninth case of this event and provide a review of the literature. A 58-year-old man diagnosed with Merkel cell carcinoma of the wrist, presented, 37 months later, a recurrence in the form of a testicular metastasis. The tumor consisted of a monotonous proliferation of small, blue, round cells, with immunoexpression of neuroendocrine markers and the typical dot-like paranuclear immunostaining for cytokeratin 20, in the absence of immunostaining for cytokeratin 7. The patient is alive with no evidence of disease. Clinicians should be aware of the possibility of metastatic dissemination to the testis since genital examination/imaging is not part of routine follow-up for these patients, but timely orchiectomy may be curative.

Gemcitabine-associated thrombotic microangiopathy (gTMA) is a rare entity that is usually associated with a poor prognosis, with loss of kidney function and often death. The management of this syndrome includes discontinuation of the drug. Other approaches have been tried, with no proven efficacy and inconsistent results, such as glucocorticoids, intravenous immunoglobulin, plasma infusion and rituximab. Drug-induced hemolytic uremic syndrome, a form of thrombotic microangiopathy (TMA), has shown good response to the anti-C5 monoclonal antibody eculizumab and anecdotal cases have been reported where eculizumab improved gTMA. We present a case where a patient with gTMA on hemodialysis was treated with eculizumab, with full recovery of hematological disorders and kidney function. We suggest that clinicians be aware of gTMA as a potentially life-threatening condition and that eculizumab should be considered as a possible first-line agent.

Light chain deposition disease is a systemic disorder characterized by deposition of monoclonal light chains in various organs. We present a case of a 58#8209;year#8209;old woman who was referred for a nephrology consultation due to worsening renal function and nephrotic range proteinuria. The diagnosis work#8209;up mentioned a plasmatic creatinine of 1.5mg/dL (estimated glomerular filtration rate of 38 mL/min/1.73m2 by MDRD equation), a urinary sediment with microhematuria, a protein/creatinine ratio of 5, a seric and urinary immunoelectrophoresis compatible with a monoclonal gammopathy IgG/Kappa, hypocomplementemia, type II cryoglobulinemia and a pulmonary nodule of irregular shape 15mm in diameter. A renal biopsy was performed and showed a marked expansion of the mesangium with nodules of amorphous material and small outbreaks of tubular atrophy associated with interstitial fibrosis. The nodules were PAS positive and Congo red negative and stained for light chain kappa by immunocytochemistry. Immunofluorescence was negative for IgA, IgG, IgM, C1q and C3c. A diagnosis of light chain deposition disease was made and concomitant multiple myeloma excluded. Treatment was initiated with bortezomib, dexamethasone and thalidomide with complete hematological remission and improvement in renal function. She also showed normalization of the cryoglobulinemia and disappearance of the pulmonary nodule previously detected, despite worsening of cardiac function as a result of the chemotherapy implemented. This clinical case highlights the well-known renal involvement in light chain deposition disease, but also some atypical clinical associations, namely type II cryoglobulinemia and pulmonary nodule disease.

Background: End-stage kidney disease is associated with considerable morbidity and mortality, a feature that is shared with malignant neoplasms. Hence, patients with the cumulative effect of these two diseases frequently give rise to the question of whether dialysis should be implemented. The primary goal of this study was to characterize the clinical progression and evaluate the outcome of a group of oncology patients on chronic haemodialysis and also to identify the characteristics associated with prolonged survival. Methods: Retrospective analysis of all patients on the chronic haemodialysis programme in an oncology hospital-based haemodialysis centre between January 1991 and September 2014. Results: 141 patients were treated during this period. The main aetiologies for end-stage kidney disease were multiple myeloma (24.8%) and chronic interstitial disease (22.7%), while the most common tumours were genitourinary cancer (47.5%) and multiple myeloma (24.8%). Multiple tumours were present in 22.0% of patients and 19.2% harboured metastatic disease. Overall, 66.7% of patients died during this period; 7.8% were transferred to other centres as a result of clinical stability; 4.3% recovered renal function; 1.4% received a kidney transplant and 19.9% were still alive at the end of the study. Overall survival was 58.8% at 2 years and 34.8% at 5 years. Multiple myeloma (HR=5.950; 95% CI: 2.512-14.092) and gastrointestinal cancers (HR=3.277; 95% CI: 1.176-9.134) were associated with increased likelihood of death. Conclusions: Survival among patients with often locally advanced or metastatic oncological disease on chronic haemodialysis was unexpectedly high, with 1/3 still alive at 5 years. Accordingly, decision-making in the cancer

Os pólipos de prolapso mucoso (MPPs) são lesões inflamatórias raras enquadradas na síndrome de prolapso mucoso. Apresentamos o caso de um homem, 40 anos, com antecedentes de obstipação, referenciado à nossa instituição por suspeita de neoplasia do reto devido a hematoquésias e lesão palpável ao toque retal. A colonoscopia mostrou uma lesão com 25 mm, de aspeto subepitelial, com mucosa marcadamente eritematosa e erosionada. As biopsias bite-on-bite revelaram dilatação e distorção das criptas, infiltrado inflamatório misto e fibrose. Devido à suspeita inicial de neoplasia foi decidida resseção após discussão com o doente, que não foi possível devido à elevação inadequada da lesão. Efetuada resseção parcial, permitindo o diagnóstico seguro de MPP. As hematoquésias cessaram após tratamento da obstipação. Os MPPs podem mimetizar lesões neoplásicas anorrectais devendo ser incluídos no diagnóstico diferencial. A conjugação da história clínica com os aspetos endoscópicos e histológicos é fundamental para o diagnóstico correto e orientação adequada.

Mucosal prolapse polyps (MPPs) are rare inflammatory lesions that are part of the mucosal prolapse syndrome. We present the case of a 40-year-old male with history of constipation referred to our institution with suspected rectal malignancy due to hematochezia and a palpable rectal mass. Colonoscopy revealed a 25 mm wide lesion suggestive of subepithelial origin but with marked erythema and erosion in the mucosa. Crypt dilatation and distortion, mixed inflammatory infiltrate and fibrosis were apparent on histological evaluation after bite-on-bite biopsies. Due to the initial suspicion of malignancy, resection was decided after discussion with the patient. However, due to non-elevation partial resection was performed allowing the diagnosis of MPP. Hematochezia ceased after obstipation treatment and endoscopic follow-up showed maintenance of the lesion with the same characteristics except for reduced dimension. MPP may mimic neoplastic lesions and should be considered in the differential diagnosis of rectal masses. History, endoscopy and histological characteristics are all necessary and important in the diagnosis of MPP.

A doença de Whipple é uma doença rara, provocada por uma bactéria Gram-positiva intracelular Tropheryma whipplei. É um diagnóstico difícil de fazer e, apesar de se poder apresentar com uma clínica muito variada, a sintomatologia gastrointestinal está quase sempre presente. O diagnóstico é estabelecido pela identificação de inclusõesPAS-positivas em macrófagos da lâmina própria da mucosa duodenal. Os autores descrevem o caso de um doente que se apresenta com um quadro clínico muito sugestivo de doençalinfoproliferativa, na ausência de sintomas gastrointestinais, cujo diagnóstico foi estabelecido pela análise histológica de fragmento de umaadenopatia retroperitoneal obtida por punção guiada por tomografia computorizada

Whipple’s disease is a rare disease caused by Tropheryma whipplei, a Gram-positive intracellular bacterium. It’s a difficult diagnosis to do and although gastrointestinal manifestations are almost always present, it can be present with a great diversity of symptoms. The diagnosis is made by the identification of PAS-positive inclusions in the macrophages of duodenal mucosa. The authors describe a case of a patient presenting with a clinical picture suggestive of alymphoproliferative disease, in the absence of gastrointestinal symptoms, with diagnosis of Whipple’s disease being made by pathological analysis of a fragment obtained by computer tomography guided biopsy of a retroperitoneal adenopathy