RESUMEN
A 52-year-old man with consciousness disorder following a 2-day history of general fatigue, diarrhea, vomiting and excessive thirst was admitted to our hospital. Severe hyperglycemia (1,739 mg/dL) with a slightly elevated HbA1c level (6.9%), ketonuria and low C-peptide level (0.07 ng/mL) confirmed the diagnosis of fulminant type 1 diabetes mellitus (FT1DM). Following sudden unexplained cardiogenic shock shortly after the initiation of insulin therapy with no evidence of myocardial ischemia assessed by coronary angiography, the patient was supported with percutaneous venoarterial extracorporeal membrane oxygenation. Electron microscopic analysis of the myocardium revealed massive lipid droplets without the infiltration of inflammatory cells. His left ventricular function began to recover during the following days and returned to a normal level on day 14. Currently, the impact of FT1DM on intramyocardial lipid deposition is poorly understood. However, this case suggests that even short-term exposure to high concentrations of glucose can be responsible for lipotoxicity followed by severe cardiac dysfunction.
Asunto(s)
Diabetes Mellitus Tipo 1/complicaciones , Gotas Lipídicas , Miocitos Cardíacos/ultraestructura , Choque Cardiogénico/patología , Humanos , Masculino , Persona de Mediana Edad , Choque Cardiogénico/etiologíaRESUMEN
A 75-year-old man was admitted to our hospital for further examination of swollen lymph nodes and a possible gastric submucosal tumor. He had persistent fever and anorexia. Blood examination showed anemia, thrombocytopenia, and elevated lactate dehydrogenase and soluble interleukin 2 receptor levels. Swollen lymph nodes and splenomegaly were evident on computed tomography, and the submucosal tumor was revealed by esophagogastric endoscopy. Cervical lymph node biopsy and endoscopic biopsy were performed, which revealed a diagnosis of intravascular lymphoma. In Asian countries, patients with intravascular lymphoma often have hemophagocytic syndrome without lesions of the central nervous system or skin, which is called the Asian variant of intravascular lymphoma. In this case, the patient had no indicative lesions and had no evidence of the hemophagocytic syndrome. He also had lymph node swelling and a gastric submucosal tumor, which are rare in intravascular lymphoma. The patient was treated with chemotherapy (R-CHOP;rituximab-cyclophosphamide, doxorubicin, vincristine, and prednisolone), and complete response was demonstrated (based on the Response Evaluation Criteria for Solid Tumours [RECIST] guideline). In cases of possible intravascular lymphoma, gastrointestinal endoscopy and biopsy should be considered because they are a useful diagnostic strategy.
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Protocolos de Quimioterapia Combinada Antineoplásica/uso terapéutico , Mucosa Gástrica/patología , Linfoma/tratamiento farmacológico , Neoplasias Gástricas/tratamiento farmacológico , Anciano , Biopsia , Mucosa Gástrica/irrigación sanguínea , Mucosa Gástrica/diagnóstico por imagen , Humanos , Linfoma/diagnóstico por imagen , Masculino , Tomografía de Emisión de Positrones , Neoplasias Gástricas/irrigación sanguínea , Neoplasias Gástricas/diagnóstico por imagen , Neoplasias Gástricas/patología , Tomografía Computarizada por Rayos XRESUMEN
A 41-year-old woman was diagnosed with duodenal follicular lymphoma. She had no other lesions and was assigned to a "watch and wait" policy. Swelling of the inguinal lymph nodes appeared 45 months later, and rituximab monotherapy resulted in complete remission. However, follicular lymphoma recurred in the stomach, rectum and mesenteric and external iliac lymph nodes 81 months after the therapy. The patient received rituximab monotherapy again and has remained in complete remission in the fifteenth year after the initial diagnosis. This case suggests the usefulness of rituximab monotherapy in the long-term management of intestinal follicular lymphoma.
Asunto(s)
Antineoplásicos/uso terapéutico , Neoplasias Duodenales/tratamiento farmacológico , Linfoma Folicular/tratamiento farmacológico , Rituximab/uso terapéutico , Adulto , Neoplasias Duodenales/patología , Femenino , Estudios de Seguimiento , Humanos , Linfoma Folicular/patologíaRESUMEN
Micropapillary carcinoma (MPC) has been recently reported as a variant of invasive breast carcinoma. MPC is also known to be an aggressive variant of adenocarcinoma, and it is associated with poor prognosis and a high propensity for lymphovascular invasion and lymph node metastases. MPC of the breast, urinary bladder, and lung has been reported relatively frequently; however, there have been few reports on gastrointestinal MPC. Furthermore, MPC of the small bowel has not been reported yet. Here we report a case of MPC of the jejunum. A female septuagenarian was admitted because of anorexia and weight loss. We established a diagnosis of primary jejunal cancer and resected the duodenum and a part of the jejunum. Histologically, the tumor consisted of adenocarcinoma with moderate to poor differentiation. Among the carcinoma cells, approximately 10% formed small papillary neoplastic cell clusters surrounded by clear spaces, suggesting a diagnosis of MPC. We present the details of the case along with a review of relevant literature.
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Carcinoma Papilar/diagnóstico , Neoplasias del Yeyuno/patología , Anciano , Carcinoma Papilar/cirugía , Femenino , Humanos , Neoplasias del Yeyuno/cirugía , Metástasis Linfática , Estadificación de Neoplasias , Tomografía Computarizada por Rayos XRESUMEN
The purpose of this study was to reveal the diagnostic accuracy of initial pathologic assessment of biopsied samples in patients with gastrointestinal follicular lymphoma lesions. A total of 48 patients with follicular lymphoma (Lugano system stage I: n = 30; II1: n = 4; II2: n = 4; IV: n = 10) with gastrointestinal involvement who underwent endoscopic biopsy were enrolled and retrospectively reviewed. Nine (18.8%) of the 48 patients were not appropriately diagnosed as having follicular lymphoma at the initial biopsy. The initial pathological diagnosis included extranodal marginal zone lymphoma of mucosa-associated lymphoid tissue (n = 4), necrotic tissue (n = 2), duodenitis (n = 1), or suspected lymphoma of unspecified subtype (n = 2). The reasons for these inappropriate diagnoses were insufficient histopathologic analysis lacking CD10 and BCL2 staining (n = 7) and unsuitable biopsy samples taken from erosions or ulcers that contained scanty lymphoma cells or no lymphoid follicles (n = 2). In conclusion, incomplete histopathologic analysis and unsuitable biopsy samples are pitfalls in the diagnosis of gastrointestinal follicular lymphoma.
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Neoplasias Gastrointestinales/diagnóstico , Linfoma de Células B de la Zona Marginal/diagnóstico , Linfoma Folicular/diagnóstico , Adulto , Anciano , Anciano de 80 o más Años , Biopsia , Diagnóstico Diferencial , Neoplasias Duodenales/diagnóstico , Neoplasias Duodenales/patología , Femenino , Neoplasias Gastrointestinales/patología , Humanos , Linfoma de Células B/metabolismo , Linfoma de Células B/patología , Linfoma de Células B de la Zona Marginal/patología , Linfoma Folicular/patología , Masculino , Persona de Mediana Edad , Estudios RetrospectivosRESUMEN
PURPOSE: Lymph node enlargement in the neck is a common presentation of malignant lymphoma (ML) and requires tissue sampling for accurate diagnosis. Although delayed diagnosis may be critical for some patients, unnecessary biopsy should be avoided wherever possible. This study examined the process for determining the necessity to perform a biopsy and evaluated the value of an open biopsy as a diagnostic tool to enable definite subclassification of the disease. METHODS: The subjects included 20 patients with suspected ML who underwent cervical lymph node extirpation at Okayama Saiseikai general hospital between 2007 and 2010. The decision to perform a biopsy was made based on the results of sonographic evaluation, fine needle aspiration cytology (FNAC), and serum levels of lactate dehydrase (LDH) and soluble interleukin-2 receptor (sIL-2r). RESULTS: The diagnosis was ML in 15 patients (75%), Castleman's disease in 1 (5%), and benign lymphadenopathy in 4 (20%). CONCLUSIONS: A lymph node biopsy remains the gold standard for the diagnostic evaluation of ML. Sonographic evaluation combined with serum levels of LDH and sIL-2r is useful in determining the need for biopsy. Many of the cases of ML where it was difficult to determine whether a biopsy should be performed were relatively low grade and critical conditions could be avoided by close observation of the patient.
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Escisión del Ganglio Linfático , Linfoma/diagnóstico , Cuello , Biopsia del Ganglio Linfático Centinela , Adolescente , Adulto , Anciano , Anciano de 80 o más Años , Biomarcadores/sangre , Biopsia con Aguja Fina , Femenino , Humanos , Hidroliasas/sangre , Ganglios Linfáticos/diagnóstico por imagen , Linfoma/patología , Masculino , Persona de Mediana Edad , Receptores de Interleucina-2/sangre , Solubilidad , Ultrasonografía , Adulto JovenRESUMEN
BACKGROUND/AIMS: Von Hippel-Lindau disease is an autosomal dominant genetic disorder characterized by neoplasms developing in multiple organs. Although the pancreas is one of the most frequently involved organs, the frequency of pancreatic cysts, cystadenomas, neuroendocrine tumors and diabetes has not been sufficiently evaluated due to the low prevalence of this disease. In this paper, we review and retrospectively analyze 11 patients with von Hippel-Lindau disease. METHODOLOGY: Eleven patients (6 males, 5 females) who underwent CT or MRI scans at Okayama University Hospital between 2002 and 2009 were enrolled in this study. Their pancreatic CT scans, MRI scans, biochemical test results and clinical histories were retrospectively reviewed. RESULTS: All patients had one or more pancreatic involvements. Nine of the 11 patients had multiple pancreatic cysts, 2 had dilatation of the main pancreatic duct, 3 had a non-functioning pancreatic endocrine tumor (one patient required pancreatoduodenectomy due to the endocrine carcinoma) and 3 had diabetes mellitus. Pancreatic cystadenomas were not detected in this case series. CONCLUSIONS: The prevalence of pancreatic involvement was 100% in this study. Regular screening and scheduled follow-up for pancreatic lesions and diabetes should be performed on individuals predisposed to von Hippel-Lindau disease.
Asunto(s)
Diabetes Mellitus/epidemiología , Quiste Pancreático/epidemiología , Neoplasias Pancreáticas/epidemiología , Enfermedad de von Hippel-Lindau/epidemiología , Adolescente , Adulto , Anciano , Biomarcadores/sangre , Glucemia/análisis , Diabetes Mellitus/sangre , Diabetes Mellitus/diagnóstico , Diabetes Mellitus/terapia , Femenino , Prueba de Tolerancia a la Glucosa , Hospitales Universitarios , Humanos , Japón/epidemiología , Imagen por Resonancia Magnética , Masculino , Persona de Mediana Edad , Quiste Pancreático/diagnóstico , Quiste Pancreático/terapia , Neoplasias Pancreáticas/diagnóstico , Neoplasias Pancreáticas/terapia , Prevalencia , Pronóstico , Estudios Retrospectivos , Tomografía Computarizada por Rayos X , Adulto Joven , Enfermedad de von Hippel-Lindau/terapiaRESUMEN
An elderly frail lady with features of malnutrition was investigated by endoscopy because of nausea and vomiting. Candida esophagitis was found, and there was symptomatic and endoscopic resolution after treatment with amphotericin B.
RESUMEN
An 18-year-old woman with a 2-year history of hypertension and headache was diagnosed with noradrenalin-secreting bilateral adrenal pheochromocytomas with paragangliomas in the background of von Hippel-Lindau disease with family histories and a missense mutation, 712C to T (Arg167Trp) in the VHL gene. She had optic disc hemangioma in the left eye which gradually enlarged and caused serous retinal detachment on the macula in one year. Low-dose external beam radiation (20 Gy) was administered to the left eye using a lens-sparing single lateral technique. She underwent craniotomy for cerebellar hemangioblastoma at the age of 22 years and total pancreatectomy for multiple neuroendocrine tumors at the age of 24 years. In the 6-year follow-up period after the radiotherapy, the optic disc hemangioma gradually reduced in size and its activity remained low, allowing good central vision to be maintained. External beam radiation is recommended as a treatment option for the initial therapy for optic disc hemangioma.
Asunto(s)
Hemangioma/radioterapia , Disco Óptico , Neoplasias del Nervio Óptico/radioterapia , Enfermedad de von Hippel-Lindau/complicaciones , Adulto , Femenino , Hemangioma/complicaciones , Humanos , Neoplasias del Nervio Óptico/complicaciones , Factor A de Crecimiento Endotelial Vascular/antagonistas & inhibidoresRESUMEN
We describe herein 2 patients who developed Vogt-Koyanagi-Harada syndrome in the course of renal biopsy-proven immunoglobulin A (IgA) nephropathy. A 61-year-old man with an 11-year history of IgA nephropathy and a 16-year history of thyroiditis, and a 56-year-old man with a 5-year history of IgA nephropathy developed Vogt-Koyanagi-Harada syndrome. At the time of the eye disease presentation, IgA nephropathy was stable without corticosteroids in both patients. Vogt-Koyanagi-Harada syndrome was successfully treated with intravenous administration of prednisolone tapered from 200 mg daily. Vogt-Koyanagi-Harada syndrome is associated with IgA nephropathy, suggesting a similar autoimmune mechanism for both diseases.
Asunto(s)
Glomerulonefritis por IGA/complicaciones , Síndrome Uveomeningoencefálico/diagnóstico , Síndrome Uveomeningoencefálico/inmunología , Glucocorticoides/uso terapéutico , Humanos , Masculino , Persona de Mediana Edad , Prednisolona/uso terapéutico , Síndrome Uveomeningoencefálico/tratamiento farmacológicoRESUMEN
Objective To analyze the clinical characteristics and endoscopic features of patients with lanthanum deposition in the stomach. Patients We retrospectively reviewed seven patients with lanthanum deposition in the stomach who were diagnosed at Okayama Saiseikai General Hospital. We investigated the patient sex, age at diagnosis, medical and medication histories, gastrointestinal symptoms, complications, presence or absence of gastric atrophy, and outcome. We also investigated any changes in the endoscopic features if previous endoscopic images were available. Results Seven patients (six males and one female) had lanthanum deposition. The median age was 65 years (range, 50-79 years). All patients had been undergoing dialysis (continuous ambulatory peritoneal dialysis in one patient, hemodialysis in six patients). The dialysis period ranged from 16 to 73 months (median, 52 months). The patients had all been taking lanthanum carbonate for a period ranging from 5 to 45 months (median, 27 months). Gastric atrophy was noted in 6 patients (85.7%). One patient had difficulty swallowing, and 1 other patient had appetite loss. The other 5 patients were asymptomatic. Endoscopic features included annular whitish mucosa (n = 4), diffuse whitish mucosa (n = 3), and whitish spots (n = 2). Five patients underwent multiple esophagogastroduodenoscopy. The endoscopic features were unchanged in 2 patients, whereas the whitish mucosa became apparent and spread during the course in 3 patients. Conclusion We identified 7 patients with lanthanum deposition in the stomach. All patients showed whitish lesions macroscopically. Although the pathogenicity of gastric lanthanum deposition is uncertain, lanthanum-related lesions in the stomach progressed during continuous lanthanum phosphate intake in several patients.
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Hiperfosfatemia/tratamiento farmacológico , Hiperfosfatemia/etiología , Fallo Renal Crónico/complicaciones , Fallo Renal Crónico/terapia , Lantano/uso terapéutico , Diálisis Renal/efectos adversos , Estómago/química , Anciano , Femenino , Humanos , Hiperfosfatemia/diagnóstico por imagen , Hiperfosfatemia/patología , Japón , Lantano/análisis , Masculino , Persona de Mediana Edad , Fosfatos/análisis , Estudios RetrospectivosRESUMEN
A 67-year-old Japanese man with neurofibromatosis type 1 underwent right hemicolectomy owing to abscess formation around the cecum. A pathological analysis revealed diffuse intestinal ganglioneuromatosis in the cecum and colon. Colonoscopy performed eight months after hemicolectomy revealed multiple ulcers throughout the colon and rectum. The colorectal ulcers failed to respond to conservative treatment and ultimately required surgical resection. Diffuse ganglioneuromatosis was observed again in the resected specimen. This report illustrates a rare manifestation of diffuse intestinal ganglioneuromatosis in a patient with neurofibromatosis type 1.
Asunto(s)
Enfermedades del Colon/complicaciones , Ganglioneuroma/complicaciones , Neoplasias Intestinales/complicaciones , Neurofibromatosis 1/complicaciones , Úlcera/complicaciones , Anciano , Colectomía/métodos , Colon/patología , Enfermedades del Colon/patología , Enfermedades del Colon/cirugía , Colonoscopía , Ganglioneuroma/patología , Humanos , Neoplasias Intestinales/patología , Masculino , Neurofibromatosis 1/patología , Úlcera/patologíaRESUMEN
A 67-year-old man was admitted to our hospital due to esophageal cancer. Cancer existed at the lower esophagus and subtotal esophagectomy and lymphadenectomy was performed. The postoperative course was uneventful. Pathological findings revealed moderately differentiated squamous cell carcinoma that metastasized to the abdominal lymph nodes which include the paraaortic lymph nodes. He complained of anorexia three months after the operation and was found to have multiple liver and mediastinal lymph node metastases. He was admitted for chemotherapy. Before starting chemotherapy, he suddenly died without any sign of hemorrhage or respiratory disorder. Autopsy showed metastatic lesions to the heart and mediastinal lymph nodes, liver, thoracic vertebrae, kidney, adrenal gland and heart. Metastatic nodules in the heart were on the ventricular septum where the conducting system exists. No direct invasion from the pericardium was observed. Blockade of the conducting system of the heart was considered to have caused the severe arrhythmia and sudden cardiac arrest.
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Carcinoma de Células Escamosas/patología , Carcinoma de Células Escamosas/secundario , Neoplasias Esofágicas/patología , Neoplasias Cardíacas/secundario , Tabiques Cardíacos , Anciano , Carcinoma de Células Escamosas/complicaciones , Muerte Súbita , Paro Cardíaco/etiología , Neoplasias Cardíacas/complicaciones , Humanos , MasculinoRESUMEN
Metastatic tumors are rare in the pancreas, and some cases are difficult to distinguish from pancreatic cancer. However, distinguishing between them is very important to formulate a treatment plan. A case of a rare disease, called overlap cancer, involving metastatic tumors to the pancreas and right kidney from lung cancer, and duodenal papilla cancer, is described. Endoscopic ultrasound-guided fine needle aspiration (EUS-FNA) is useful for diagnosing metastatic pancreatic tumors, particularly in patients with multiple cancers.
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Carcinoma de Células Escamosas/patología , Carcinoma de Células Escamosas/secundario , Biopsia por Aspiración con Aguja Fina Guiada por Ultrasonido Endoscópico , Neoplasias Pulmonares/patología , Neoplasias Pancreáticas/patología , Neoplasias Pancreáticas/secundario , Anciano , Carcinoma de Células Escamosas/diagnóstico por imagen , Humanos , Masculino , Neoplasias Pancreáticas/diagnóstico por imagenRESUMEN
Many reports have described loss of neurons in the substantia nigra in Pick's disease (PiD). In those reports, however, "Pick's disease" includes PiD without Pick bodies (PB), and there is only limited data available on regional nigral pathology in PiD with PB. To elucidate the pathological changes of the substantia nigra in PiD with PB, we examined five cases and 12 age-matched controls by morphometry. The number and size of pigmented and nonpigmented neurons, as well as the area of the substantia nigra were examined. The area of the substantia nigra was significantly reduced in PiD with PB. The pigmented and nonpigmented neuron counts in PiD with PB were not statistically different from those in controls. There was a significant reduction in the size of pigmented neurons in PiD with PB to 82% with that in the controls. In addition, after reviewing 48 cases of PiD with PB reported in the literature, we found that none of the cases with typical frontotemporal lobe symptoms exhibited parkinsonism until the terminal stage. These data are useful for discriminating PiD with PB from other diseases showing frontotemporal characteristics, including the frontal lobe degeneration type and the motor neuron disease type of frontotemporal dementia.
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Neuronas/patología , Enfermedad de Pick/patología , Sustancia Negra/patología , Adulto , Anciano , Anciano de 80 o más Años , Recuento de Células , Tamaño de la Célula , Diagnóstico Diferencial , Femenino , Humanos , Inmunohistoquímica , Cuerpos de Inclusión/patología , Masculino , Persona de Mediana EdadRESUMEN
INTRODUCTION: Solid pseudopapillary neoplasm of the pancreas is a rare neoplasm that has been reported to account for between 0.17% and 2.7% of all non-endocrine tumors of the pancreas. It is usually seen in young women. Because solid pseudopapillary neoplasms are rarely aggressive and have low-grade malignant potential and an excellent prognosis after complete resection, it is an ideal pancreatic tumor for treatment by minimally invasive surgery. Therefore, making an accurate pre-operative diagnosis is very important. CASE PRESENTATION: A 24-year-old Japanese man who had been found to have mild transaminase elevations at a medical check-up visited our hospital for further examination. Abdominal computed tomography showed a 40mm-diameter tumor in the pancreatic tail and mild fatty liver. He was admitted to our hospital for additional examination. The abdominal contrast-enhanced computed tomography scan taken at our institution showed an increasingly enhanced mass of 40mm diameter in the pancreatic tail. Ultrasonography showed a low-level echoic mass of 35mm diameter in the pancreatic tail. T1-weighted magnetic resonance imaging showed low signal intensity in the tail of the pancreas. T2-weighted magnetic resonance imaging showed high signal intensity there. Diffusion magnetic resonance imaging showed high signal intensity. An endoscopic ultrasound yielded the same results as the abdominal ultrasonogram. In addition, [18F]-fluorodeoxyglucose positron emission tomography/computed tomography showed abnormal accumulation (maximum standardized uptake value, 6.53). This finding raised our suspicion of a pancreatic malignant tumor. However, the patient could not be confidently diagnosed solely on the basis of imaging. Endoscopic ultrasound-guided fine-needle aspiration was performed, which led us to a diagnosis of solid pseudopapillary neoplasm. On that basis, we performed minimally invasive surgery (spleen-preserving laparoscopic distal pancreatectomy). CONCLUSION: Atypical solid pseudopapillary neoplasm without cysts should be considered when diagnosing pancreatic tumors. A definitive pre-operative diagnosis of solid pseudopapillary neoplasm made on the basis of endoscopic ultrasound-guided fine-needle aspiration can guide the surgical approach used.
Asunto(s)
Neoplasias Glandulares y Epiteliales/diagnóstico , Neoplasias Pancreáticas/diagnóstico , Biopsia por Aspiración con Aguja Fina Guiada por Ultrasonido Endoscópico , Humanos , Masculino , Imagen Multimodal , Neoplasias Glandulares y Epiteliales/cirugía , Pancreatectomía , Neoplasias Pancreáticas/cirugía , Tomografía de Emisión de Positrones , Tomografía Computarizada por Rayos X , Adulto JovenRESUMEN
Follicular cholangitis is a sclerosing cholangitis with hilar biliary stricture that must be differentiated from both immunoglobulin G4-related sclerosing cholangitis and primary sclerosing cholangitis. This disorder is extremely rare and difficult to distinguish from hilar biliary cholangiocarcinoma. We report here a case of a Japanese female patient in her 60s with this disease. The patient visited a family doctor for itching and general fatigue. Blood examination showed elevated hepatobiliary enzyme levels. Various imaging studies showed dilation of the bilateral intrahepatic bile duct and wide stenosis from the proximal bile duct to the right and left hepatic duct. They also showed the enlargement of multiple lymph nodes in the hepatoduodenal ligament, periaorta, and mesocolon. Based on endoscopic retrograde cholangiopancreatography-directed brush cytology, we diagnosed this patient with hilar cholangiocarcinoma and performed left trisegmentectomy of the liver. The pathology results showed that the wall from the bilateral hepatic duct to the proximal bile duct had thickened irregularly with dense fibrosis and a marked formation of lymph follicles. The mucosal epithelia did not have malignant findings. The diagnosis was follicular cholangitis. This case indicates that follicular cholangitis should be considered as a differential diagnosis of hilar biliary stricture.
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Neoplasias de los Conductos Biliares/diagnóstico , Colangitis Esclerosante/diagnóstico , Tumor de Klatskin/diagnóstico , Diagnóstico Diferencial , Femenino , Humanos , Persona de Mediana EdadRESUMEN
A case of thyroidal mucosa-associated lymphoid tissue (MALT) lymphoma mimicking IgG4-related disease is described. A 54-year-old male presented with acute swelling of the anterior neck. Anaplastic thyroid carcinoma (ATC), malignant lymphoma (ML), or acute deterioration of Hashimoto's thyroiditis were initially suspected, and an emergent tracheostomy was required for progressive airway obstruction; a simultaneous biopsy from the thyroid tissue was performed. Histopathologically, the lesion consisted of sclerotic fibrosis and diffuse and dense infiltration by small lymphoid cells without atypia and plasma cells, many of which were IgG4-positive. Blood examination also revealed high serum IgG4 levels. Riedel's thyroiditis was suspected. However, despite medical treatments, a firm swelling of the thyroid still remained. In an in situ hybridization study, IgG4-negative plasma cells showed immunoglobulin light-chain restriction (κ-monotype), and immunoglobulin heavy (IgH) chain gene monoclonal re-arrangement was detected by polymerase chain reaction. The lesion was finally diagnosed as MALT lymphoma. When IgG4-related disease is suspected, it is important to thoroughly exclude other possibilities.
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Inmunoglobulina G/análisis , Linfoma de Células B de la Zona Marginal/patología , Células Plasmáticas/patología , Neoplasias de la Tiroides/patología , Humanos , Masculino , Persona de Mediana Edad , Células Plasmáticas/inmunología , Glándula Tiroides/patologíaRESUMEN
Peripheral T-cell lymphoma, not otherwise specified (PTCL-NOS) consists of a heterogeneous group of lymphomas. Patients generally show an aggressive clinical course and very poor outcome. Although the 2008 World Health Organization classification of PTCL-NOS includes 3 variants, low-grade lymphoma is not included. Of 277 PTCL-NOS cases recorded in our consultation files, we examined the clinicopathologic characteristics of 10 patients with T-cell lymphomas composed of small-sized cells with slight nuclear atypia. Eight patients showed extranodal involvement (5 patients, spleen; 3 patients, thyroid), and 5 patients were at clinical stage I or II. Histologically, all samples presented diffuse infiltrate of small lymphoid cells, with few mitotic figures. Immunohistologically, all samples were positive for CD3, and CD20 was detected in 5 samples. All samples showed a low Ki-67 labeling index (mean, 1.05%), and 7 samples were positive for central memory T-cell markers. Clonal T-cell receptor γ chain and/or α-ß chain gene rearrangements were detected in all 10 patients. Five patients received chemotherapy, whereas for 3 patients, treatment consisted only of observation following surgical resection of the spleen or thyroid. Nine patients were alive at a median follow-up time of 19.5 months, whereas 1 patient died of an unrelated disease. The present study strongly indicates that T-cell lymphoma with small-sized lymphoma cells and a low Ki-67 labeling index is a distinct variant. Recognition of this novel lymphoma subtype, which should not be defined merely as PTCL-NOS, should be seriously considered.
Asunto(s)
Linfoma de Células T Periférico/patología , Neoplasias del Bazo/patología , Neoplasias de la Tiroides/patología , Adulto , Anciano , Anciano de 80 o más Años , Biomarcadores de Tumor/genética , Biomarcadores de Tumor/metabolismo , Núcleo Celular/patología , Proliferación Celular , Quimioterapia Adyuvante , Células Clonales , Femenino , Reordenamiento Génico de la Cadena alfa de los Receptores de Antígenos de los Linfocitos T/genética , Reordenamiento Génico de la Cadena beta de los Receptores de Antígenos de los Linfocitos T/genética , Reordenamiento Génico de la Cadena gamma de los Receptores de Antígenos de los Linfocitos T/genética , Humanos , Antígeno Ki-67/metabolismo , Linfoma de Células T Periférico/genética , Linfoma de Células T Periférico/metabolismo , Masculino , Persona de Mediana Edad , Estadificación de Neoplasias , Neoplasias del Bazo/genética , Neoplasias del Bazo/metabolismo , Neoplasias de la Tiroides/genética , Neoplasias de la Tiroides/metabolismoRESUMEN
OBJECTIVE: The case of an 80-year-old man showing a metastatic cervical small cell neuroendocrine carcinoma is presented. RESULTS: The primary site could not be found at first; it took 8-10 months to detect the primary lesion in the larynx. CONCLUSION: (18)F-deoxyglucose positron emission tomography (FDG-PET) was useful to find the submucosal lesion. Despite surgical treatments and chemotherapy, the patient survived for only 21 months.