1/18. Secondary cardiac tumor in children.We describe our clinical experience of eight cases of secondary cardiac tumor. The pathology of the tumors were lymphoma (three), Wilms' tumor (two), malignant teratoma (one), neuroblastoma (one), and pleuropulmonary blastoma (one). Metastatic sites were the right atrium in Wilms' tumor and neuroblastoma, the left atrium in pleuropulmonary blastoma and malignant teratoma, and multiple sites in lymphoma. Primary masses in the mediastinum extended directly to the heart (three lymphoma, malignant teratoma, pleuropulmonary blastoma). Wilms' tumor and neuroblastoma showed cardiac metastases through the inferior vena cava. Many cases revealed vague abnormal cardiovascular findings (symptoms in six; physical signs in five). In five cases surgery was performed to relieve the possible obstruction to flow and to identify the pathology (lymphoma in three, Wilms' tumor in one, and malignant teratoma in one). Chemotherapy prior to operation resulted in the disappearance of the intracardiac masses in each case of Wilms' tumor and pleuropulmonary blastoma. All three patients with lymphoma died immediately after operation. Four died of multiple metastases or pneumocystis pneumonia several months after operation. This study indicates that suspicion of a secondary cardiac tumor is crucial to early diagnosis. Because of the poor postoperative outcome, surgery for secondary cardiac tumors should be done cautiously only in cases with definite hemodynamic decompensation.- - - - - - - - - - ranking = 1keywords = physical (Clic here for more details about this article) |
2/18. cafe-au-lait spots and early onset colorectal neoplasia: a variant of HNPCC?BACKGROUND: cafe-au-lait spots (CALS) are classically found in neurocutaneous syndromes such as neurofibromatosis, but have not been associated with hereditary colorectal cancer. However, review of hereditary colorectal cancer case reports reveals occasional description of CALS on physical exam. methods: We describe the colonic and extracolonic phenotype in a family with CALS and early onset colorectal neoplasia (adenomas and/or cancer) and review 23 additional families reported in the literature. RESULTS: Among the 24 families, 32/59 (54.2%) individuals had colorectal adenomas diagnosed at a mean age of 15.7 /- 1.1 (SE) years (range 5-38 years). The majority (24/32, 75.0%) of persons at first colorectal examination had oligopolyposis (< 100 polyps) versus polyposis (> or = 100 polyps). Forty-two of 59 (71.2%) individuals were affected with colorectal cancer, diagnosed at a mean age of 31.9 /- 2.7 years (range 5-70 years). A brain tumor was found in 28/59 (47.5%) affected individuals (4 families with 2 or more cases) with an overall mean age of diagnosis of 16.5 /- 1.2. lymphoma and/or leukemia was found in 8/24 (33.3%) families (one family with 3 cases). Two families had mutation of the mismatch repair gene, hPMS2 (1 with homozygous germline mutation), while two carried homozygous germline mutations of another mismatch repair gene, hMLH1. CONCLUSIONS: cafe-au-lait spots with early onset colorectal neoplasia may identify families with a variant of HNPCC characterized by oligopolyposis, glioblastoma at young age, and lymphoma. This variant may be caused by homozygous mutation of the mismatch repair genes, such as hPMS2 or hMLH1.- - - - - - - - - - ranking = 1keywords = physical (Clic here for more details about this article) |
3/18. Lymphoepithelioma-like carcinoma of the breast: report of a case mimicking lymphoma.Only 10 cases of lymphoepithelioma-like carcinoma of the breast have been reported in the literature. This report adds one more case to the published literature. A 62-year-old woman presented with a mass in her left breast on physical examination. The mammographic images showed a 3.0 cm, poorly defined mass in the upper outer quadrant. A biopsy was recommended. The gross specimen consisted of a 5 cm portion of breast parenchyma with no discrete tumor. On microscopic examination, the tumor was composed of sheets of epithelioid cells arranged as single cells or in cords partially obscured by a dense lymphocytic infiltrate. The epitheliod cells extensively expressed cytokeratin stain, but did not express E-cadherin. The lymphoid cells expressed L26 stain in the germinal centers, and CD3 stain in the T lymphocytes surrounding the germinal centers and in between tumor cells. in situ hybridization showed no evidence of Epstein-Barr virus infection in the tumor cells. An overall review of 11 cases shows that the disease is usually seen in older patients. In situ and invasive lobular component was reported in 36% of the cases. Eight of 11 were negative for E-cadherin, 36% were estrogen receptor-positive, 18% were progesterone receptor-positive, and all of them were HER2/neu negative. None of the reported cases have been associated with Epstein-Barr virus infection. Only two of the cases showed lymph node metastasis, and long-term follow-up in one of them showed good prognosis. In summary, lymphoepithelioma-like carcinoma of the breast is a tumor with a good prognosis that should be considered as a possible diagnosis in breast tumors with an intense lymphocytic infiltrate.- - - - - - - - - - ranking = 4.9506038978167keywords = physical examination, physical (Clic here for more details about this article) |
4/18. Multifocal primary breast lymphoma.Primary breast lymphoma (PBL) is a rare condition, accounting for only 0.04 to 0.5% of breast malignancies and less than 2% of extranodal lymphomas. Clinical presentation and imaging may suggest a benign condition. Reports of treatment vary widely. Surgical therapy has been reported to include only biopsy or extend to partial mastectomy, total mastectomy, or even radical mastectomy. Chemotherapy with various agents is often used. radiotherapy has been used in the adjuvant setting or as primary local therapy. Immunotherapy and radioimmunotherapy have shown some promise in other lymphomas and may be useful here as well. There is no standard or consensus of treatment for PBL. This report describes a case of multifocal PBL in a 45-year-old female and discusses the physical findings, diagnosis, and treatment options for this condition.- - - - - - - - - - ranking = 1keywords = physical (Clic here for more details about this article) |
5/18. Concurrent FSGS and Hodgkin's lymphoma: case report and literature review on the link between nephrotic glomerulopathies and hematological malignancies.BACKGROUND: The link between the nephrotic syndrome (NS) and malignancy was first described in 1922. In solid tumors, the NS is most often due to membranous glomerulonephropathy, whereas in common hematological malignancies, minimal-change disease predominates. Focal segmental glomerulosclerosis (FSGS) is among the least frequently reported renal lesion associated with malignancy. methods: We report a case of the simultaneous diagnoses of FSGS and Hodgkin's lymphoma, and review the literature on various nephrotic glomerulonephropathies associated with common leukemia and lymphoma. RESULTS: Although nephrotic glomerulonephropathies rarely occur in association with acute leukemia, they have often been described in chronic lymphocytic leukemia (CLL). Membranoproliferative glomerulonephropathy and membranous glomerulonephropathy are the most common lesions observed in CLL. Nephrotic glomerulonephropathies have also been well documented among patients with lymphomas, in particular, Hodgkin's lymphoma. While minimal-change disease is most commonly found in association with Hodgkin's lymphoma, more diverse and complex renal lesions are associated with non-Hodgkin's lymphoma. FSGS remains a rare association with hematological malignancies. CONCLUSIONS: Nephrotic glomerulonephropathies are not only linked to solid-organ tumors, but also to hematological malignancies. A thorough evaluation, including a physical examination for lymphadenopathy and organomegaly, as well as a hematological evaluation, must be performed in all patients presenting with nephrotic glomerulonephropathies.- - - - - - - - - - ranking = 4.9506038978167keywords = physical examination, physical (Clic here for more details about this article) |
6/18. Tumour lysis syndrome and the anaesthesiologist: intensive care aspects of paediatric oncology.Children presenting with advanced leukaemia and non-Hodgkin's lymphoma may develop life-threatening complications in the early stages of management. Major metabolic disturbances with encephalopathy, septicaemic shock, pneumonitis, massive haemorrhage, or the physical effects of tumour masses may on occasion warrant intensive therapy. Close liaison between paediatric oncologists, oncological surgeons, and anesthesiologists is essential in establishing admission criteria for such cases and in defining therapeutic end points in the event of multisystem failure. This paper discusses the principles of intensive care management of patients with haematological malignancies by considering two cases who developed the tumour lysis syndrome with respiratory and renal failure. A case associated with metabolic encephalopathy is also described.- - - - - - - - - - ranking = 1keywords = physical (Clic here for more details about this article) |
7/18. One-stage decompression and stabilization in the treatment of spinal tumors.Nowadays, extradural vertebral neoplasms which compress the spinal cord are being diagnosed earlier and therefore laminectomy for decompression relieves many patients of their neurological deficits before permanent cord damage has set in. In addition, radiotherapy and oncological treatment have prolonged life expectancy as well as the quality of life in these patients. As the indications for surgery have grown, the problem of spinal instability resulting from direct involvement of the supporting structures of the spine or due to the surgical procedure per se, must be considered. Many of these patients would rapidly return to leading their normal activities if not confined to bed rest or uncomfortable orthopedic devices. The Authors present 9 patients with extradural vertebral lesions: 4 plasmacytomas, 3 metastatic carcinomas, 1 malignant lymphoma and 1 aggressive osteoblastoma treated by simultaneous decompression and stabilization of the spine. All patients showed remarkable improvement of neurological symptoms except in one case where massive pulmonary embolism caused death a few hours after surgery. This surgical treatment offers the advantages of performing wider and better decompressive maneuvers and, at the same time, assuring fast mobilization of the patient after surgery who is often in poor general conditions, thus reducing post-operative complications due to delayed physical therapy and bed confinement.- - - - - - - - - - ranking = 1keywords = physical (Clic here for more details about this article) |
8/18. Primary pulmonary lymphoma. A clinical and immunohistochemical study of six cases.Six patients with lymphomatous lesions primarily involving the pulmonary parenchyma were studied. In these patients, both the history and physical findings were vague and minimal. The laboratory findings also were nonspecific, although the findings of large multiple lesions in the lungs and pleural adhesion or effusion were more consistent with lymphoma. Histologic examination revealed lymphocytic infiltration of the pulmonary parenchyma in all six patients and presence of germinal or growth centers in some areas of the lesions in four. The hilar or mediastinal lymph nodes were not involved in five patients so examined. Immunocytochemical study of cytoplasmic immunoglobulin revealed monoclonal lymphocytic proliferation in five patients and negative staining in one patient. Clinical, histologic, or immunohistochemical studies alone may not be sufficient to detect all of the lymphomatous lesions. The combined use of all of these parameters is more advantageous for accurate diagnosis of these lesions. Treatment is surgical resection. radiotherapy or chemotherapy are used when residual disease is present after surgery. Three patients died of disseminated lymphoma 96, 42, and 8 months after diagnosis, respectively, and three patients are still alive at 18, 24, and 4 months, respectively.- - - - - - - - - - ranking = 1keywords = physical (Clic here for more details about this article) |
9/18. Myopathic carnitine deficiency associated with lymphocytic malignant non-Hodgkin lymphoma and monoclonal immunoglobulin g-K.A 47-year-old male patient suffered from recurrent myalgia, induced by fasting or physical exercise. Later, he developed progressive muscular weakness. serum levels of creatine phosphokinase (CPK) were elevated to approx. 400 U/1. Muscle biopsies showed lipid storage myopathy and signs of acute fiber necrosis, muscle carnitine was decreased to below 20% of controls, carnitine palmitoyl transferase (CPT) activity was normal. carnitine was also moderately decreased in a liver biopsy and in plasma. urine excretion of carnitine was low, no elevation of short-chain dicarboxylic acids could be found. The patient was also found to suffer from a lymphocytic malignant non-Hodgkin lymphoma, a monoclonal immunoglobulin (Ig) G-k in plasma and lymphocytic infiltration of bone marrow were demonstrated. At that time no evidence had been obtained to indicate that these two diseases could be related to each other. autoantibodies against skeletal muscle could not be demonstrated. absorption of L-carnitine p.o. was normal, however, plasma levels of carnitine fell again rapidly. Administration of 3 X 2 g L-carnitine per day normalized the patient's plasma carnitine levels and led to an increase of plasma short-chain acylcarnitine and ketone bodies, particularly beta-hydroxybutyrate (B-HOB). However, no significant clinical improvement could be seen. Additional application of prednisone led to normalization of CPK serum levels.- - - - - - - - - - ranking = 1keywords = physical (Clic here for more details about this article) |
10/18. Recurrent testicular infiltrates following radiation therapy for lymphoid malignancy.From a group of 28 patients with biopsy-proven testicular leukemia seen at our institution since 1979, we describe two boys with lymphoid malignancy who, subsequent to 1,800 rad and 2,400 rad testicular radiation, had second overt testicular relapses. This experience suggests that these patients need careful follow-up physical examinations, that higher doses may be needed for prolonged local control, and that there may be a role for follow-up testicular biopsies.- - - - - - - - - - ranking = 4.9506038978167keywords = physical examination, physical (Clic here for more details about this article) |
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