Paraneoplastic cutaneous manifestations: concepts and updates
Paraneoplastic panuveitis
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Bone metastases from prostate cancer can have different behav- iours. Their osteoclastic or osteoblastic activities are associated with calcium metabolism disorders: hyper and hypocalcemia, respectively. Case 1: 88-year-old male with arterial hypertension (HTN) and bone metastases from adenocarcinoma of the prostate (Prostatic-Specific antigen—PSA 2416 ng/mL) under therapy with bicalutamide. He was admitted with refusal to eat, psychomotor agitation and worsening of chronic kidney disease. He had 16.7 mg/dL of ionized calcium. Pamidronate was initiated with consequent improvement of symptoms and renal function. Case 2: 80-year-old male, smoker 240 pack-year, was admitted with acute renal failure secondary to rhabdomyolysis after a non-observed fall. He presented sudden dyspnea, accompanied by malaise, upper abdominal pain and generalized muscle weakness in the context of severe hypocalcemia (4.6 mg/dL). Etiologic study found bone metastasis of prostate adenocarcinoma (PSA 94.90 ng/mL). Replacement began with calcium carbonate and bicalutamide, with progressive improvement of symptoms. Bone metastasis due to prostate cancer may acquire different behaviours that influence the treatment choice and prognosis. doi:10.1016/j.ejim.2013.08.400 ID: 887 Paraneoplastic polymyalgia rheumatica—A surgically treatable disease? J. Tavares, V. Romão, G. Eugénio, S. Calretas, R. Santos, J. Nascimento Costa Medicina Interna, Centro Hospitalar e Universitário de Coimbra, EPE, Coimbra, Portugal Introduction: Malignant neoplasm's are associated with a variety of paraneoplastic rheumatological syndromes. Polymyalgia rheumatica (PMR), a relatively common disease in the elderly, is characterized by stiffness and pain in the shoulders, neck, and hips. The association between PMR and cancer is controversial. Case presentation: A 82-year- old man was admitted to our hospital because of stiffness and pain in the shoulders with great limitation in his daily activities, for the last month. His past medical history included diabetes, osteoarthritis and hyperten- sion. On physical examination, the patient had pain with movement and had decreased active range of motion of the shoulders. His blood tests showed normochromic, normocytic anaemia and ESR 84 mm/h. The diagnosis of PMR was made and deflazacort was prescribed, without achieving a complete symptom relief and with ERS levels of 53–59 mm/h. After 5 months the patient developed macroscopic haematuria. Further study led to the diagnosis of an infiltrative bladder urothelial carcinoma and prostate adenocarcinoma. The patient underwent radical cystectomy and prostatectomy. Meanwhile deflazacort was suspended due to a postoperative infection. He was re-evaluated 1.5 months after discharge, and was completely asymptomatic with an ESR level of 38 mm/h. Conclusion: The association between cancer and rheumatic disorders is a matter of discussion. The paraneoplastic nature is often based in a time-relation and in specific signs: rapid-onset of symptoms, atypical age, poor response to corticosteroids or immunosuppressive therapy, atypical distribution of involved joints, and abnormal laboratory tests. In the case described the initial lack of response, with improvement/ resolution of complaints after surgery seems to establish a relationship between the two situations suggesting that it is a paraneoplastic polymyalgia rheumatic. Recognition of a paraneoplastic rheumatic disease may permit early diagnosis and treatment of the underlying cancer. doi:10.1016/j.ejim.2013.08.401 ID: 897 Splenic plasmablastic lymphoma A. Vilas-Boas, F. Teixeira-Andrade, N. Pereira, M. Honavar, R. Môço Internal Medicine, Hospital Pedro Hispano - Unidade Local de Saúde de Matosinhos, Matosinhos, Portugal B-cell lymphomas with plasmablastic differentiation are a heterogeneous group of rare lymphomas that share the same morphology and/or immunophenotype but have distinct clinico- pathologic and molecular features. This group includes diseases like anaplastic large cell lymphoma kinase (ALK) positive large B-cell lymphoma and human immunodeficiency virus (HIV) or human herpes virus-8 (HHV-8) associated lymphoma. A 63-year-old man with previously known arterial hypertension, dyslipidemia and coronary artery disease, was admitted to our hospital with symptomatic hypotension, fever and cutaneous erythema that started 2 months earlier. He had been previously treated for the rash with antihistamines and corticosteroids, topic and oral, without significant response. He also reported decreased appetite and non- quantified weight loss during the past year. On examination he had hypotension and fever and a pruriginous cutaneous erythema that spared only palms and plants. Blood tests revealed thrombocytope- nia (86,000/μL), elevated erythrocyte sedimentation rate (37 mm/1st hour) and lactate dehydrogenase (400 U/L). Thoraco-abdomino- pelvic computed tomography showed a homogeneously enlarged spleen. Further investigation showed decreased cholesterol levels and increased immunoglobulin E. Immunophenotypic analysis of peripheral blood and bone marrow showed no evidence of neoplastic disease. During hospitalization the cutaneous erythema progressive- ly disappeared and thrombocytopenia worsened to a minimum value of 8000/μL, without response to high-dose corticosteroids and platelet transfusion. Due to the refractory thrombocytopenia, the patient was submitted to laparoscopic splenectomy. After splenec- tomy the patient started to get better with normalization of platelet count. He also showed no more fever and systolic blood pressure raised to normal levels. Pathologic analysis of the spleen showed HHV-8 associated splenic plasmablastic lymphoma, stages I–E. The patient was also submitted to chemotherapy with cyclophospha- mide, doxorubicin, prednisolone and rituximad with good response. Plasmablastic lymphomas are very rare diseases particularly in patients without HIV infection. It also affects more frequently the digestive tract, making this one of the few reported cases in the literature of this kind of lymphoma. The recognition of this complex condition with challenging diagnosis is important to avoid delay of treatment. doi:10.1016/j.ejim.2013.08.402 ID: 902 Paraneoplastic panuveitis Liliana Cunha, Marta Amaral, Filipe Paula, Irina Mocanu Medicina IV, Hospital Prof. Dr. Fernando Fonseca, Amadora, Portugal Panuveitis is diffuse inflammation of the uveal tract of the entire eyeball that is often seen in association with systemic inflammatory diseases. We present a case report of a 63 year old men, active smoker, without any previous disease, that presented with reduced visual acuity in both eyes and blurred vision on the right, without any other symptom. On ophthalmologic observation it was found panuveitis in both eyes. Arteriography of the retinal vessels showed Abstracts e155
Transcript of Paraneoplastic panuveitis
Bone metastases from prostate cancer can have different behav-iours. Their osteoclastic or osteoblastic activities are associated withcalcium metabolism disorders: hyper and hypocalcemia, respectively.Case 1: 88-year-old male with arterial hypertension (HTN) and bonemetastases from adenocarcinoma of the prostate (Prostatic-Specificantigen—PSA 2416 ng/mL) under therapy with bicalutamide. He wasadmitted with refusal to eat, psychomotor agitation and worsening ofchronic kidney disease. He had 16.7 mg/dL of ionized calcium.Pamidronatewas initiatedwith consequent improvement of symptomsand renal function. Case 2: 80-year-old male, smoker 240 pack-year,was admitted with acute renal failure secondary to rhabdomyolysisafter a non-observed fall. He presented sudden dyspnea, accompaniedby malaise, upper abdominal pain and generalized muscle weakness inthe context of severe hypocalcemia (4.6 mg/dL). Etiologic study foundbone metastasis of prostate adenocarcinoma (PSA 94.90 ng/mL).Replacement began with calcium carbonate and bicalutamide, withprogressive improvement of symptoms. Bone metastasis due toprostate cancer may acquire different behaviours that influence thetreatment choice and prognosis.
doi:10.1016/j.ejim.2013.08.400
ID: 887Paraneoplastic polymyalgia rheumatica—A surgicallytreatable disease?J. Tavares, V. Romão, G. Eugénio, S. Calretas, R. Santos, J. Nascimento Costa
Medicina Interna, Centro Hospitalar e Universitário de Coimbra, EPE,Coimbra, Portugal
Introduction:Malignant neoplasm's are associated with a variety ofparaneoplastic rheumatological syndromes. Polymyalgia rheumatica(PMR), a relatively common disease in the elderly, is characterized bystiffness and pain in the shoulders, neck, and hips. The associationbetween PMR and cancer is controversial. Case presentation:A 82-year-oldmanwas admitted to our hospital because of stiffness and pain in theshoulders with great limitation in his daily activities, for the last month.His past medical history included diabetes, osteoarthritis and hyperten-sion. On physical examination, the patient had pain withmovement andhad decreased active range of motion of the shoulders. His blood testsshowed normochromic, normocytic anaemia and ESR 84 mm/h. Thediagnosis of PMR was made and deflazacort was prescribed, withoutachieving a complete symptom relief andwith ERS levels of 53–59 mm/h.After 5 months the patient developed macroscopic haematuria. Furtherstudy led to the diagnosis of an infiltrative bladder urothelial carcinomaand prostate adenocarcinoma. The patient underwent radical cystectomyand prostatectomy. Meanwhile deflazacort was suspended due to apostoperative infection. He was re-evaluated 1.5 months after discharge,and was completely asymptomatic with an ESR level of 38 mm/h.Conclusion: The association between cancer and rheumatic disorders isa matter of discussion. The paraneoplastic nature is often based in atime-relation and in specific signs: rapid-onset of symptoms, atypicalage, poor response to corticosteroids or immunosuppressive therapy,atypical distribution of involved joints, and abnormal laboratory tests.In the case described the initial lack of response, with improvement/resolution of complaints after surgery seems to establish a relationshipbetween the two situations suggesting that it is a paraneoplasticpolymyalgia rheumatic. Recognition of a paraneoplastic rheumaticdisease may permit early diagnosis and treatment of the underlyingcancer.
doi:10.1016/j.ejim.2013.08.401
ID: 897Splenic plasmablastic lymphomaA. Vilas-Boas, F. Teixeira-Andrade, N. Pereira, M. Honavar, R. Môço
Internal Medicine, Hospital Pedro Hispano - Unidade Local de Saúde deMatosinhos, Matosinhos, Portugal
B-cell lymphomas with plasmablastic differentiation are aheterogeneous group of rare lymphomas that share the samemorphology and/or immunophenotype but have distinct clinico-pathologic and molecular features. This group includes diseases likeanaplastic large cell lymphoma kinase (ALK) positive large B-celllymphoma and human immunodeficiency virus (HIV) or humanherpes virus-8 (HHV-8) associated lymphoma. A 63-year-old manwith previously known arterial hypertension, dyslipidemia andcoronary artery disease, was admitted to our hospital withsymptomatic hypotension, fever and cutaneous erythema thatstarted 2 months earlier. He had been previously treated for therash with antihistamines and corticosteroids, topic and oral, withoutsignificant response. He also reported decreased appetite and non-quantified weight loss during the past year. On examination he hadhypotension and fever and a pruriginous cutaneous erythema thatspared only palms and plants. Blood tests revealed thrombocytope-nia (86,000/μL), elevated erythrocyte sedimentation rate (37 mm/1sthour) and lactate dehydrogenase (400 U/L). Thoraco-abdomino-pelvic computed tomography showed a homogeneously enlargedspleen. Further investigation showed decreased cholesterol levelsand increased immunoglobulin E. Immunophenotypic analysis ofperipheral blood and bone marrow showed no evidence of neoplasticdisease. During hospitalization the cutaneous erythema progressive-ly disappeared and thrombocytopenia worsened to a minimum valueof 8000/μL, without response to high-dose corticosteroids andplatelet transfusion. Due to the refractory thrombocytopenia, thepatient was submitted to laparoscopic splenectomy. After splenec-tomy the patient started to get better with normalization of plateletcount. He also showed no more fever and systolic blood pressureraised to normal levels. Pathologic analysis of the spleen showedHHV-8 associated splenic plasmablastic lymphoma, stages I–E. Thepatient was also submitted to chemotherapy with cyclophospha-mide, doxorubicin, prednisolone and rituximad with good response.Plasmablastic lymphomas are very rare diseases particularly inpatients without HIV infection. It also affects more frequently thedigestive tract, making this one of the few reported cases in theliterature of this kind of lymphoma. The recognition of this complexcondition with challenging diagnosis is important to avoid delay oftreatment.
doi:10.1016/j.ejim.2013.08.402
ID: 902Paraneoplastic panuveitisLiliana Cunha, Marta Amaral, Filipe Paula, Irina Mocanu
Medicina IV, Hospital Prof. Dr. Fernando Fonseca, Amadora, Portugal
Panuveitis is diffuse inflammation of the uveal tract of the entireeyeball that is often seen in association with systemic inflammatorydiseases. We present a case report of a 63 year old men, activesmoker, without any previous disease, that presented with reducedvisual acuity in both eyes and blurred vision on the right, withoutany other symptom. On ophthalmologic observation it was foundpanuveitis in both eyes. Arteriography of the retinal vessels showed
Abstracts e155
vasculitis. In blood analysis there was a 10 hg macrocitic anemia,leukopenia with light monocytosis and mild thrombocytopenia. Weperform a myelogram and a bone biopsy that revealed chronicmyelomonocytic leukemia. We also carry out a thorax TV thatshowed a suspect pulmonary nodule that was biopsied and showedsquamous-cell carcinoma, that we assume to be the cause of thepanuveitis. We initiate empirically corticosteroids topical andsystemic with significant improvement. This case reveals thedifficulty of finding a cause for eye disease without any systemicmanifestation and presents both hematological changes that candivert the differential diagnosis of the symptom.
doi:10.1016/j.ejim.2013.08.403
ID: 919Foot pain to metastatic renal carcinomaE. Meira, C. Canha, E. Ferreira, R. Monteiro, F. Santos
Internal Medicine, University Hospital of Coimbra, Coimbra, Portugal
Introduction: Renal cell carcinoma has great avidity through thebone (25–50% renal cell carcinoma metastatize to the bone). Renal
neoplasm could be asymptomatic till advanced stage of the disease,where pain, related to bone metastasis, could be the first symptom.Case report: Male, 49 years old, referred intense pain on the left foot,for the last 2 months, without trauma history. He also complaineddyspnea during the past 2 weeks, associated with cough, asthenia andanorexia. He denied weight lost, fever or diaphoresis. The lung murmurwas diminished in the inferior half of the left side. The blood testshowed hypochromic microcytic anemia (hemoglobin 8.1 g/dL, MGV78.5 fl), leukocytosis (11.8 G/L), trombocitosis (634 G/L), hypercalce-mia (15.0 g/dL), elevated reactive C protein (11.48 mg/dl). Foot X-rayrevealed osteolytic lesion on the medial cuneiform of the left foot. CTScan showed right renal neoplasm with multiple metastasis (lung,pleura, mediastinum and intercostal muscles) and inferior cava veinand ipsilateral renal vein thrombosis. At this level, the patient didn'tgather clinical conditions to proceed to curative or palliative surgicaltreatment, neither to chemotherapy. By progressive deterioration, thepatient died after a month of diagnosis. Conclusion: Renal cell tumorcan grow without any local symptoms, being mostly diagnosed whensymptomatic metastasis appears. That explains why this carcinoma isalso known as internist tumor, due to its protean manifestations andability to mimic other malignancies or benign conditions.
doi:10.1016/j.ejim.2013.08.404
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