RESUMO
Extranodal NK/T-cell lymphoma (ENKTL), nasal type and aggressive NK cell leukemia are rare in Western World been less than 1% in USA to 8% in Asia among Non-Hodgkin's lymphomas. It is aggressive, with poor outcome and optimal treatment is unclear. A combination therapy that includes Peg-Asparaginase (SMILE) has been employed in young patients. An 85-year-old Puerto Rican male presented with anorexia, epistaxis, vertigo and involuntary facial movements. He was treated with injectable Onabotulinum toxin A due to suspicion of a hemifacial spasm. However, a CT scan demonstrated a left maxillary sinus lesion extending into the left middle turbinate with biopsy consistent with ENKTL. We adjusted therapy to patient's age and performance receiving Gemcitabine-Oxaliplatin (Gemox) with radiation obtaining a complete response with persistent negative Epstein Barr DNA titers. ENKTL is a rare disease initially misdiagnosed in our elderly patient, who demonstrated adequate response with a modified therapeutic regime.
Assuntos
Linfoma Extranodal de Células T-NK , Idoso de 80 Anos ou mais , Humanos , Masculino , Biópsia , Hispânico ou Latino , Linfoma Extranodal de Células T-NK/diagnóstico , Linfoma Extranodal de Células T-NK/patologia , Linfoma Extranodal de Células T-NK/terapia , Oxaliplatina/uso terapêuticoRESUMO
Mucosa-associated lymphoid tissue (MALT) lymphomas are B-cell neoplasms that commonly affect the gastrointestinal (GI) tract, usually the stomach. In most cases, extranodal marginal zone lymphoma (ENMZL) is an indolent disease. Bone marrow involvement is common with MALT lymphoma accompanied by paraproteinemia; such involvement impels disease progression. Here, we present the case of an 82-year-old Hispanic patient with long-standing ENMZL in whom the gastric site responded to antibiotic treatment and Helicobacter pylori eradication, but the disease progressed over the years, with a biclonal gammopathy and bone marrow involvement with marked plasmacytic differentiation. In view of this, we suggest the routine evaluation of paraprotein in patients with ENMZL.
Assuntos
Cromossomos Humanos Par 11 , Linfoma de Zona Marginal Tipo Células B , Paraproteinemias/diagnóstico , Neoplasias Gástricas , Idoso de 80 Anos ou mais , Medula Óssea/patologia , Doenças da Medula Óssea/patologia , Cromossomos Humanos Par 11/genética , Fadiga/diagnóstico , Infecções por Helicobacter/tratamento farmacológico , Helicobacter pylori , Humanos , Imunoglobulina G/sangue , Imunoglobulina M/sangue , Cadeias lambda de Imunoglobulina/sangue , Neoplasias Pulmonares/diagnóstico por imagem , Linfoma de Zona Marginal Tipo Células B/sangue , Linfoma de Zona Marginal Tipo Células B/tratamento farmacológico , Linfoma de Zona Marginal Tipo Células B/genética , Linfoma de Zona Marginal Tipo Células B/patologia , Masculino , Mieloma Múltiplo/diagnóstico , Proteínas de Fusão Oncogênica/genética , Neoplasias Gástricas/sangue , Neoplasias Gástricas/tratamento farmacológico , Neoplasias Gástricas/genética , Neoplasias Gástricas/patologia , Trissomia/genéticaRESUMO
Prolymphocytic leukemias (PLLs) are rare mature lymphoid disorders of B- and T-cell subtypes with distinct features and an aggressive clinical course. PLL represents only 2% of all mature lymphocytic leukemias in adults. T-PLL represents 20% of all PLLs cases. T-cell prolymphocytic leukemia (T-PLL) is more rare and more rapidly progressive and aggressive than B-PLL; it is generally resistant to conventional chemotherapy, and historically the median survival has been about 7 months. Clinicians will often only see a case of T-PLL once every 5 to 10 years, which makes recognition of the disorder difficult. The prognosis is poor and there is no curative therapy. We report a 77-year-old male patient with de novo T-PLL presenting with WBC count of 1,115,000. We will discuss the clinical, morphologic, immunophenotypic and cytogenetic features of this rare entity. A distinctive hematologic aspect of T-PLL is a rapidly rising white blood cell count with a doubling time of weeks to months. The key morphologic feature in the diagnosis of T-PLL is a population of more than 55% prolymphocytes in the peripheral blood. The diagnosis can be made on peripheral blood by flow cytometry where a monoclonal lymphocyte population will show positivity for T-cell markers. T-PLL is characterized by complex chromosomal abnormalities, which suggests that chromosomal aberrations might occur progressively during the course of the disease, thus explaining the aggressive nature of this condition. The main challenge as a clinician treating T-PLL is to deliver long-term disease-free survival. The most important predictor of outcome is response to alemtuzumab therapy (Campath). Knowledge of the disrupted pathways and mechanisms underlying activation and proliferation in T-PLL has raised the possibility of developing future and promising treatment approach that targets these pathways and signals by the use of future molecule inhibitors. T-PLL is a rare disease and careful attention should be given to correctly diagnose this T-cell leukemia. Physicians should be aware of this unusual entity. With the advent of alemtuzumab, although much progress has been made in the treatment of this disease, autologous or allogeneic hematologic stem cell transplant (HSCT) still remains the only hope for cure.
RESUMO
Splenic artery aneurysms (SAA) are a rare life threatening clinical diagnosis. We present a case of a young Hispanic woman with an aneurysm of the middle branch of the splenic artery and active leakage. The defect was embolized with complete resolution of the retroperitoneal bleeding. Physicians should be aware of this rare entity especially when female patients presents complainiing of severe epigastric pain with associated hypovolemic shock.
Assuntos
Aneurisma Roto/complicações , Aneurisma/complicações , Hemorragia/etiologia , Artéria Esplênica/patologia , Feminino , Hispânico ou Latino , Humanos , Espaço Retroperitoneal/patologia , Adulto JovemRESUMO
Paroxysmal nocturnal hemoglobinuria (PNH) is a non-malignant, acquired clonal hematopoietic stem cell disease that can present with bone marrow failure, hemolytic anemia, smooth muscle dystonias, and thrombosis. We present a case of a 32 year-old-female, G2P2A0 with no past medical history of any systemic illnesses who refers approximately 2 months of progressively worsening constant heartburn with associated abdominal discomfort. CBC showed leukopenia (WBC 2.9 x 103 /µL) with neutropenia (segmented neutrophils 48%), macrocytic anemia (Hgb 6.1 g/dL, hematocrit 20%, MCV,113 fL) and thrombocytopenia (platelet count 59 x 109/L). Abdomino-pelvic CT scan revealed a superior mesenterc vein thrombosis, which was treated initially with low-molecular-weight heparih for full anticoagulation. Peripheral blood flow cytometry assays revealed diminished expression of CD55 and CD59 on the erythrocytes, granulocytes and monocytes.' Paroxysmal nocturnal hemoglobinuria is a rare, clonal, hematopoietic stem-cell disorder whose manifestations are almost entirely explained by complement-mediated intravascular hemolysis. The natural history of PNH is highly variable, ranging from indolent to life-threatening. The median survival is 10 to 15 years, but with a wide range. Thrombosis is the leading cause of death, but others may die of complications of bone marrow failure, renal failure, myelodysplastic syndrome, and leukemia. Anticoagulation is only partially effective in preventing thrombosis in PNH; thus, thrombosis is an absolute indication for initiating treatment with Eculizumab. Nevertheless, bone marrow transplantation (BMT) is still the only curative therapy for PNH but is associated with significant morbidity and mortality.
Assuntos
Azia/etiologia , Hemoglobinúria Paroxística/diagnóstico , Dor Abdominal/etiologia , Adulto , Anticorpos Monoclonais Humanizados/uso terapêutico , Anticoagulantes/uso terapêutico , Medula Óssea/patologia , Fadiga/etiologia , Feminino , Glicosilfosfatidilinositóis/deficiência , Hemoglobinúria/etiologia , Hemoglobinúria Paroxística/complicações , Heparina de Baixo Peso Molecular/uso terapêutico , Humanos , Isquemia Mesentérica/diagnóstico por imagem , Isquemia Mesentérica/tratamento farmacológico , Isquemia Mesentérica/etiologia , Pancitopenia/etiologia , Tomografia Computadorizada por Raios X , Varfarina/uso terapêuticoRESUMO
Madelung's disease is an extremely rare disorder of unknown etiology characterized by multiple, non-encapsulated, infiltrative lipomas located symmetrically on the trunk, neck, and proximal parts of the limbs. Approximately 200 patients have been reported in the medical literature. In this case report we present an extremely unusual case of multiple symmetric lipomatosis compatible with Madelung's disease.
Assuntos
Lipomatose Simétrica Múltipla/diagnóstico , Humanos , Masculino , Pessoa de Meia-IdadeRESUMO
Kaposi's sarcoma is a rare malignancy requiring infection with human Herpes virus for development. We report a case of a 76-year-old immunocompetent male with recurrent leg cellulitis. The cellulitis eventually developed into a non-healing ulcer and a palpable nodule consistent with nodular Kaposi's sarcoma.
Assuntos
Sarcoma de Kaposi , Idoso , Humanos , Imunocompetência , Masculino , Sarcoma de Kaposi/patologia , Sarcoma de Kaposi/terapiaRESUMO
Neuroendocrine carcinomas of the colon and rectum comprise fewer than 1% of all colorectal cancers. These aggressive tumors generally have a poor prognosis compared to that associated with colorectal adenocarcinoma. We describe herein the case of a 68-year-old female presenting with a bleeding rectal mass involving the anal canal, which case was associated with hyponatremia due to inappropriate serum levels of antidiuretic hormone. The histopathological examination was consistent with a small-cell neuroendocrine tumor. She was treated with combination chemotherapy and radiation therapy. The Syndrome of Inappropriate Antidiuretic Hormone (SIADH) was managed with vasopressin antagonists. After the completion of therapy, endoscopic ultrasound revealed evidence of residual disease, for which she underwent an abdominoperineal resection (APR). The patient died 4 months later of disease progression. To our knowledge, this is the first report of a small-cell neuroendocrine tumor involving the rectum and anal canal that presented with the paraneoplastic syndrome, SIADH.
Assuntos
Carcinoma Neuroendócrino/complicações , Carcinoma de Células Pequenas/complicações , Síndromes Paraneoplásicas/etiologia , Neoplasias Retais/complicações , Idoso , Feminino , HumanosRESUMO
Choledochal cyst type II, is an extremely rare cause of portal hypertension, severe pancytopenia in an adult patient, and a complication of long-standing disease. We present an uncommon cause of portal hypertension in a young female patient secondary to an obstructive choledochal cyst type II provoking massive splenomegaly and severe pancytopenia. A high level of clinical suspicion is important due to its high mortality rate if it remains undiagnosed. To our knowledge there are few publications describing this rare type of choledochal cyst in a Hispanic adult.
Assuntos
Cisto do Colédoco/complicações , Hipertensão Portal/complicações , Cirrose Hepática Biliar/etiologia , Pancitopenia/complicações , Adulto , Feminino , HumanosRESUMO
A 64 year-old Hispanic male patient presented to our institution with a three-month history of frontal headaches, reduced vision, retroorbital pain, photophobia, sinus congestion, bloody nasal discharge, and decreased audition in the left ear. The diagnosis of metastatic lymphoepithelioma to the orbit was made based on clinical history, histopathological examination of an orbital biopsy, and imaging studies. Lymphoepithelioma rarely develops in Hispanic populations. However, it is endemic in certain areas, including North Africa, Southeast Asia. China and the far north hemisphere. Radiotherapy is th treatment of choice of localized lymphoepithelioma and concurrent chemotherapy-radiotherapy with neoadjuvant chemotherapy has been used in locally advanced metastatic settings.
Assuntos
Carcinoma/secundário , Neoplasias Nasofaríngeas/patologia , Neoplasias Orbitárias/secundário , Hispânico ou Latino , Humanos , Masculino , Pessoa de Meia-IdadeRESUMO
We report a case of a young female patient presenting with a high serum beta-HCG levels, amenorrhea, nausea and anemia which mimicked pregnancy followed by upper gastrointestinal bleeding. A gastric tumor was shown on endoscopy. Histopathologic evaluation revealed Primary Gastric Choriocarcinoma (PGC). The patient was treated with three cycles of standard nongestational choriocarcinoma chemotherapy. Tumor persistence was evidenced by CT Scans and high serum beta-HCG levels. The patient died approximately six months after diagnosis. Our case report suggest that PGC is a highly aggressive tumor that is often associated with liver and lungs metastasis without evidence of pelvic organ abnormality and is associated with some hormonal effects, such as amenorrhea, anemia, nausea and vomiting mimicking pregnancy in young adult female
Assuntos
Coriocarcinoma não Gestacional/diagnóstico , Erros de Diagnóstico , Neoplasias Gástricas/diagnóstico , Protocolos de Quimioterapia Combinada Antineoplásica/uso terapêutico , Biomarcadores Tumorais/sangue , Bleomicina/administração & dosagem , Coriocarcinoma não Gestacional/tratamento farmacológico , Coriocarcinoma não Gestacional/secundário , Gonadotropina Coriônica Humana Subunidade beta/sangue , Cisplatino/administração & dosagem , Etoposídeo/administração & dosagem , Evolução Fatal , Feminino , Humanos , Ifosfamida/administração & dosagem , Neoplasias Hepáticas/secundário , Neoplasias Pulmonares/secundário , Metotrexato/administração & dosagem , Paclitaxel/administração & dosagem , Gravidez , Gravidez Ectópica/diagnóstico , Terapia de Salvação , Neoplasias Gástricas/tratamento farmacológico , Adulto JovemRESUMO
This case report describes the clinical presentation, imaging findings and pathologic features of a rare aggressive breast tumor in a pre-menopausal woman, namely primary angiosarcoma. Recognition of this extremely rare entity is needed to make an early diagnosis, institute early therapy and eventually improve patient's survival.
Assuntos
Neoplasias da Mama/patologia , Hemangiossarcoma/patologia , Adulto , Feminino , Humanos , Pré-MenopausaRESUMO
We report the case of a female patient with an incidental finding at routine mammography evaluation which consisted of a benign spindle cell tumor, namely Breast Myofibroblastoma. It is arranged in fascicles with interspersed broad bands of hyalinized collagen with variable immunohistochemical reactivity to desmin, vimentin, smooth muscle actin and CD 34. It is usually not reactive to cytokeratins and S-100 as seen in the myoepitheliomas. Recurrence of the lesion after excisional surgical procedure is not documented at medical literature. It is important to recognize the benign nature of this neoplasm to prevent extensive mutilating surgical procedures.