11/56. Interdigitating dendritic cell sarcoma: a report of four paediatric cases and review of the literature.AIMS: To report a series of four paediatric cases of interdigitating dendritic cell sarcoma (IDCS) and add to the known extranodal sites of occurrence for this tumour. neoplasms derived from interdigitating dendritic cells are rare, with only 33 cases being reported in the literature (medline search). These tumours usually occur in lymph nodes in the adult population. methods AND RESULTS: The patients were a 10-year-old girl with a large soft tissue mass bulging into the left chest, a 12-year-old girl with a right paraspinal mass, a 21-month-old boy with generalized lymphadenopathy and hepatosplenomegaly and a 6-year-old girl with a large bladder mass. paraffin blocks and haematoxylin and eosin slides were available in all cases. In addition, immunohistochemistry and electron microscopy were performed. A diagnosis of IDCS was made in all cases. CONCLUSION: The diagnosis of IDCS can rarely be entertained on clinical information alone. Microscopically, there is a wide spectrum of features. Thus, immunohistochemistry and electron microscopy are crucial in making the diagnosis. The differential diagnosis includes inflammatory pseudotumour, follicular dendritic cell sarcoma, true histiocytic lymphoma, malignant Langerhans cell histiocytosis, anaplastic large-cell lymphoma, melanoma, and a range of sarcomas. IDCS displays aggressive behaviour and approximately half of the patients die of the disease.- - - - - - - - - - ranking = 1keywords = chest (Clic here for more details about this article) |
12/56. Colonic obstruction caused by adhesive splenomegaly: report of a case.INTRODUCTION: splenomegaly caused by hematologic or infectious diseases is a well-known complication that may cause discomfort because of increased intra-abdominal pressure; however, complete obstruction of the large bowel usually never occurs. methods: We present a case of a 76-year-old male with polycythemia vera admitted with symptoms of complete colonic obstruction. At operation, the descending colon was found to be completely obstructed by a large adhesive spleen. Incision of the adhesions, complete colonoscopy, and mobilization of the colon was performed. RESULTS: Afterward, the patient had a normal colonic function; however, four days later he had a magnetic resonance-confirmed cerebral thrombosis followed by an episode of aspiration pneumonia, which he died of ten days after the operation. CONCLUSIONS: Colonic obstruction caused by splenomegaly is extremely rare, but may happen in patients with hematologic diseases.- - - - - - - - - - ranking = 62.518266091199keywords = discomfort (Clic here for more details about this article) |
13/56. Mediastinal germ cell tumor associated with histiocytic sarcoma of spleen: case report of an unusual association.We present an unusual association of mediastinal germ cell tumor containing seminoma and angiosarcoma components and splenic histiocytic sarcoma. A 15-year-old boy presented with chest pain. Histopathologically, an anterior mediastinal mass contained typical seminoma, immature teratoma, embryonal carcinoma, angiosarcoma, yolk sac tumor, and polyembryoma. An abdominal ultrasonogram revealed a huge splenomegaly with multiple ill-defined low echogenic nodules, 1 month after the second cycle of chemotherapy. Histopathologically, large, round-to-oval tumor cells with abundant eosinophilic cytoplasm often contained eccentrically placed nuclei with vesicular chromatin and an irregular nuclear membrane. The tumor cells were immunoreactive for CD68, CD31, and CD4. The cytogenetic results showed deletion of the long arm of chromosome 5 and trisomy 8. This lesion might have been on the pathway of multistep tumorigenesis toward a final leukemia.- - - - - - - - - - ranking = 1keywords = chest (Clic here for more details about this article) |
14/56. hydroxyurea and splenic irradiation-induced tumour lysis syndrome: a case report and review of the literature.Therapeutic agents for chronic myeloid leukaemia (CML) in the chronic phase include hydroxyurea, interferon alpha, allogeneic stem cell transplantation and the tyrosine kinase inhibitor imatinib (STI 571, Gleevec). For elderly patients, oral hydroxyurea is suitable for the relief of symptoms caused by hyperleukocytosis, and splenic irradiation would be considered if abdominal discomfort or fullness induced by splenomegaly were present. Tumour lysis syndrome (TLS) is seldom seen in the treatment for CML, and TLS caused by hydroxyurea or splenic irradiation is rarely observed. Herein, we report an elderly CML patient who received treatment with hydroxyurea, allopurinol, hydration and splenic irradiation. After 3 days, acute TLS developed. Aggressive supportive treatment, including haemodialysis, stabilized the condition.- - - - - - - - - - ranking = 62.518266091199keywords = discomfort (Clic here for more details about this article) |
15/56. Eosinophilic pleural effusion, peripheral eosinophilia, pleural thickening, and hepatosplenomegaly in sarcoidosis.In this atypical case of sarcoidosis with an unusual combination of clinical and laboratory findings, a 32-year-old male presented with a 3-month history of thoracic pain complicated with dyspnea. Laboratory tests, chest radiography, and CT scans of the chest and abdomen revealed eosinophilia of pleural effusion and blood, pleural thickening, hepatosplenomegaly, and bronchiolitis obliterans. In cases such as this, in which pleural fluid eosinophilia is accompanied by peripheral eosinophilia and splenohepatomegaly, underlying malignancies such as Hodgkin lymphoma should be ruled out. A biopsy of the mediastinal lymph nodes suggested noncaseating epithelioid granulomas, characteristic of sarcoidosis. The patient underwent prednisolone therapy for 1 year and is doing well 2 years after initial diagnosis.- - - - - - - - - - ranking = 2keywords = chest (Clic here for more details about this article) |
16/56. Primary abdominal lymphonodular cryptococcosis in children: 2 case reports and a literature review.BACKGROUND: cryptococcus neoformans is an important opportunistic fungal pathogen that is acquired via the respiratory tract. It causes several clinical syndromes and most commonly presents as meningitis. The establishment of C neoformans infections in immunocompetent individuals could be owing to increased virulence, dose of the organism, or genetically determined differences in the ability to generate a protective immune response against the organism. OBJECTIVES: The purpose of this study is to report on 2 case studies and a literature review and to discuss the diagnosis and management of primary abdominal lymphonodular cryptococcosis. methods: We conducted 2 case studies and a literature review. RESULTS: Two cases of primary abdominal lymphonodular cryptococcosis in childhood were reported herein. The patients suffered from C neoformans without immunoglobulin or lymphocyte abnormalities. The cause of hospitalization of the 2 cases was mainly the persisting fever with or without abdominal pain. Imaging findings indicated dramatic lymphadenopathy and light hepatosplenomegaly. Excision biopsy of the abnormal lymph node demonstrated reactive lymphoid hyperplasia and the presence of C neoformans. The results of nested polymerase chain reaction confirmed the diagnosis of C neoformans. CONCLUSION: This is the first report on C neoformans in the primary abdominal lymph node in children. This report suggests that C neoformans infection should be considered in the differential diagnosis of fever and marked lymphadenopathy, and empirical administration of antifungal agent may be necessary. C neoformans antibody test, polymerase chain reaction assay, fine-needle aspiration biopsy, or surgical excision biopsy is needed in early diagnosis.- - - - - - - - - - ranking = 89.005008444361keywords = abdominal pain (Clic here for more details about this article) |
17/56. Stauffer's syndrome variant with cholestatic jaundice: a case report.cholestasis is a common feature of several malignant diseases, including pancreatic, hepatic, gallbladder, and ampullary carcinomas. It is usually secondary to main bile duct obstruction or widespread hepatic metastasis, but it can also be a paraneoplastic syndrome of other underlying malignancies. Stauffer's syndrome is a rare paraneoplastic manifestation of renal cell carcinoma (RCC) that is characterized by elevated alkaline phosphatase, erythrocyte sedimentation rate, alpha-2-globulin, and gamma-glutamyl transferase, thrombocytosis, prolongation of prothrombin time, and hepatosplenomegaly, in the absence of hepatic metastasis and jaundice. A rare variant of this syndrome with jaundice has recently been described in 3 cases in the literature. We report a patient who presented with abdominal pain and cholestatic jaundice in whom RCC was incidentally found during initial workup. jaundice and liver dysfunction resolved completely after surgical resection of the tumor. This case illustrates the protean manifestations of RCC, and the importance of considering Stauffer's syndrome and its variant in the differential diagnosis of anicteric and icteric cholestasis, which may allow early recognition and treatment of an underlying malignancy.- - - - - - - - - - ranking = 89.005008444361keywords = abdominal pain (Clic here for more details about this article) |
18/56. Outcome of total splenectomy with portosystemic shunt for massive splenomegaly and variceal bleeding in cystic fibrosis.Multilobular biliary cirrhosis and portal hypertension are frequent complications of cystic fibrosis liver disease, leading to esophageal varices and splenomegaly. Therapy is focused on variceal bleeding control; however, reduction of spleen volume is also important to restore gastric volume and resolve invalidating abdominal discomfort. We report long-term follow up (median duration, 5.5 years; range, 14 months-21.5 years) of 6 patients with cystic fibrosis (4 men, 2 women; median age, 14 years; range, 8-18 years) who underwent splenectomy with a splenorenal shunt operation. Three patients received elective surgery for massive splenomegaly with important abdominal discomfort, recurrent variceal bleeding, and hypersplenism. Three were urgently treated to control variceal bleeding after several sessions of sclerotherapy. All but 2 received antipneumococcal vaccination before surgery. Four patients had a weight gain of 10% within 3 months of surgery, and 3 developed spontaneous puberty. lung function remained stable, and there was an overall reduction of respiratory tract infections. The youngest patient, however, died of overwhelming septicemia during treatment with steroids. Although total splenectomy has important risks, in well-selected cases, it can have benefits. Immuno- and chemoprophylaxis, combined with patient awareness of supplementary risk of infections is indispensable to minimize septic complications.- - - - - - - - - - ranking = 125.0365321824keywords = discomfort (Clic here for more details about this article) |
19/56. Acute portal vein thrombosis and massive necrosis of the liver. An unusual complication after stenting for chronic pancreatitis.CONTEXT: ERCP can provide information which is invaluable in managing chronic pancreatitis but it is associated with infrequent, although significant, complications and rare mortality. The complications uniquely associated with diagnostic ERCP include pancreatitis and sepsis (primary cholangitis). CASE REPORT: A 32-year-old man presented with severe upper abdominal pain radiating to the back, associated with vomiting and abdominal distension. The patient was diagnosed as having had chronic calcific pancreatitis recently and had undergone ERCP with pancreatic duct stenting elsewhere. Two days after the procedure, the patient developed severe abdominal pain, vomiting and abdominal distention, and patient was referred to our hospital 7 days after the procedure. Investigation revealed massive liver necrosis and portal vein thrombosis. This patient had a life-threatening complication following pancreatic duct stenting for chronic pancreatitis and was managed medically. CONCLUSION: Therapeutic pancreatic endoscopy procedures are technically demanding and should be restricted to high volume centers. There is a continuing need for evaluation and comparison with alternative strategies. In a good surgical candidate, it is better to avoid stenting.- - - - - - - - - - ranking = 180.47018765247keywords = abdominal pain, upper (Clic here for more details about this article) |
20/56. Case report: splenic infarction and acute splenic sequestration in adults with hemoglobin sc disease.While acute splenic sequestration and splenic infarction are commonly observed in infants and young children with sickle cell anemia, they are rarely experienced by adult hemoglobin S homozygotes because the recurrent splenic infarction that takes place during childhood is typically followed by scarring, atrophy, and splenic fibrosis. Both acute splenic sequestration and splenic infarction do remain relatively common in adults with the other sickle hemoglobinopathies. These episodes are almost certainly a consequence of the persistently enlarged and distensible spleens that often remain present in these conditions. In this report, the authors describe two adult patients with hemoglobin sc disease: one who developed acute splenic sequestration and one with splenic infarction. In neither case was there a history of recent air travel or exposure to altitude. The clinical course of these two syndromes is presented, and the hematologic, radiologic, and pathologic manifestations are discussed. Because they can sometimes be difficult to distinguish from one another, and because a failure to identify acute splenic sequestration can be catastrophic, these two entities must be included in the differential diagnosis for any hemoglobin SC patient who present with an unexplained fall in hemoglobin, left upper quadrant pain, unexplained fever, or symptomatic splenomegaly.- - - - - - - - - - ranking = 2.4601707637455keywords = upper (Clic here for more details about this article) |
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