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[Study of neurological manifestations of chronic lymphatic leukemia (lymphosis). Role of associated globulin anomalies (apropos of 8 cases)].

The authors emphasize the relative frequency of neurological symptoms in lymphatic leukaemia. Clinically, these cause more or less diffuse encephalitic or multineuritic syndromes, generally a combination of the two. Their pathogenesis is usually connected with lymphoid tissue infiltration into either the meninges or the vascular sheaths of the central nervous system or the sheaths of the roots or of the peripheral nerves. The authors stress the possible function of immunoglobulin abnormalities of the C.S.F. indicative of the presence of the leukaemic process within the nervous system. This pathogenesis prompts the use of therapeutic methods directly attacking leukaemic infiltration of the nervous system (focal cobalt therapy, intrathecal chemotherapy) and the authors have found that these give favourable results.

Aged↗

MR of head and neck adenopathy in asymptomatic HIV-seropositive men.

PURPOSE: Adenopathy is a well-known component of AIDS-related complex. Our goal was to determine whether asymptomatic HIV-infected persons have characteristic findings of head and neck adenopathy on MR imaging and whether there is an association between the MR findings and the CD4 lymphocyte counts. METHODS: We blindly and retrospectively evaluated the distribution and size of lymphadenopathy seen on screening MR examinations that were performed on 50 asymptomatic HIV-positive male subjects and 50 age-matched HIV-negative control subjects. We also correlated the imaging findings in HIV-seropositive and -seronegative subjects with their CD4 counts. RESULTS: The HIV-positive subjects had a higher incidence of adenopathy compared with the controls. Statistically significant differences were found between the groups in size and number of neck lymph nodes, thickness of the adenoids, size of high, deep cervical-retropharyngeal lymph nodes, and presence of parotid abnormalities. We found a correlation between increasing lymph node size and decreasing CD4 levels. CONCLUSION: We conclude that HIV-positive asymptomatic patients have a high incidence of head and neck abnormalities including lymphadenopathy, and alteration in their CD4 counts not commonly seen in seronegative control subjects.

Adult↗

[Classification of vascular abnormalities and neoplasms].

Vascular malformations and neoplasms are very common skin disorders, found in up to 5% of newborns. However, a clear distinction has to be made between proliferating vascular lesions and permanent malformations. An exact classification is also extremely useful, since many new specific diagnostic and therapeutic measures have been developed in recent years. True proliferating tumors are, for example, childhood hemangiomas, glomus tumors, granuloma pyogenicum, tufted angiomas, senile angiomas, and malignant vascular lesions. Vascular malformations can affect capillaries, veins or arteries, as well as lymphatic vessels. Arteriovenous shunts and combined malformations may also exist (Hamburg classification). Nevi flammei, nevi anaemici, hematolymphangiomas, angiokeratotic nevi, circumscribed venous-arterious malformations, and the blue-rubber-blebnevus-syndrome may either be infiltrating or circumscribed and are characterised by a persistence of the primitive vessel network. In contrast, other malformations involve various vascular trunks, showing vessel dilation or obstruction, often combined with changes in bone or soft tissue. Significant large vessel malformations are the Bockenheimer syndrome, the Klippel-Trenaunay syndrome and the Parkes-Weber syndrome. Combinations involving both large trunks and extravascular space such as the Klippel-Trenaunay syndrome also occur.

Adolescent↗

Pathology of noncirrhotic portal hypertension: clinicopathologic study in pediatric patients.

From 1995-2002, 14 patients with predominantly prehepatic, noncirrhotic portal hypertension were evaluated. At presentation, the eight females and six males had a mean age of 9 years (range 2-18). Seven were admitted with gastrointestinal, mostly esophageal bleeding, three with splenomegaly, three with hepato-pulmonary syndrome, and one with hyperammonemia. Imaging studies showed portal vein obstruction in six patients and non-obstructed but frequently anomalous vascular patterns, including hypoplasia of the portal vein, in the remaining eight patients. At the onset, liver function was marginally abnormal in all patients, but thrombocytopenia of approximately 100 x 10(9)/L was consistently observed, probably reflecting chronic mild consumption coagulopathy and hypersplenism. The most striking and frequent histopathologic finding in 25 liver samples, was the presence of hypoplastic portal triads with collapsed portal vein radicles. In contrast, other triads showed markedly distended and misshapen portal vein radicles and likely lymphatics. These two patterns of collapse and distention presumably reflect areas of impaired versus overloaded intrahepatic portal venous flow. Some of the biopsies showed variable portal/sinusoidal fibrosis. Four patients (two with intestinal bleeding, two with hepatopulmonary syndrome) required liver transplants and are doing well. Eight patients are doing well clinically after surgical or spontaneous vascular shunting. Two patients with intestinal bleeding and hepato-pulmonary syndrome, respectively) who had congenital dyskeratosis, underwent bone marrow transplantation and died of nonhepatic-related complications. It is possible to suggest prehepatic causes of portal hypertension even in needle biopsies when collapsed portal vein radicles are present in portal triads, but more than one biopsy sample with larger bore bioptomes may be required to see the changes. Conversely, identifying these changes may suggest to the clinicians the need to work-up a patient for portal hypertension.

Adolescent↗

[Mediastinal lymph node metastasis of thyroid cancer occurring 15 years after the initial operation; report of a case].

A 71-year-old woman was found to have an abnormal shadow on a chest X-ray. Fifteen years earlier she had undergone a subtotal thyroidectomy for thyroid cancer without any lymph node metastasis. Chest computed tomography (CT) revealed a mediastinal tumor with full of blood stream. Since the positron emission tomography (PET) disclosed an increased uptake of fluoro-2-deoxy-D-glucose (FDG) in the tumor, a malignant lymphatic tumor was therefore suspected. An immunohistological examination of biopsy specimens taken by thoracoscopic procedure demonstrated tumor to be lymph node metastasis of the previous thyroid cancer. After a tumor resection by means of a thoracotomy and total thyroidectomy, the patient was scheduled to receive radioiodine therapy. The previously reported cases are also herein reviewed.

Aged↗

Solitary fibromastocytic tumor arising in an inguinal lymph node: the first description of a unique spindle cell tumor simulating mastocytosis.

A 77-yr-old man presented with a right inguinal mass. At herniotomy, an enlarged inguinal lymph node was also removed. Gross examination revealed a firm gray tumorous nodule measuring 3 cm in maximum diameter. On histological examination, the lymphatic tissue was found to have been almost completely replaced by spindle-shaped tumor cells arranged in whorls and bundles. In Giemsa-stained sections, about one-third of the tumor cells were seen to be strongly metachromatic tissue mast cells. The other tumor cells reacted only with a monoclonal antibody against vimentin out of a broad panel of antibodies directed against various antigens expressed by leukocytes, mesenchymal cells, and epithelial cells. A small fraction of the nonmetachromatic tumor cells reacted with the proliferation-associated antibody MIB1. Immunostaining with an antibody against p53 tumor-suppressor gene products was negative. Electron-microscopic examination revealed mast cells containing abundant granules, which were intermingled with fibroblasts that had bizarre, elongated nuclei. No myofibroblasts, desmosomes, or basement membrane material were detected. Clinical examination revealed no abnormalities, and blood and bone marrow findings were completely normal. A diagnosis of solitary (apparently benign) fibromastocytic tumor was established on the basis of the relative tumor cell numbers, the cytological features, and the clinical data. To the authors' knowledge, this is the first description of a solitary spindle cell tumor arising in a lymph node and simulating mastocytosis.

Aged↗

Angioimmunoblastic lymphadenopathy with dysproteinemia.

Three cases of angioimmunoblastic lymphadenopathy with dysproteinemia are reported which illustrate the characteristic clinico-pathological features. These cases also provide information relating to the ultrastructure of the interstitial acidophil material, the histopathological spectrum, where treatment has been attempted, and an incidence figure for an Australian community. Detailed immunological investigations in one case suggest that the disease is a primary B-lymphocyte abnormality.

Adult↗

Metastases to the uterine corpus from extragenital cancers. A clinicopathologic study of 63 cases.

This report is an analysis of 63 cases of metastatic cancers to the uterine corpus from extragenital neoplasms. The patients' ages ranged from 34-88 years (mean, 59.7 years). Twenty lesions were discovered in surgical specimens and 43 were detected at autopsy. The primary tumors arose in the breast (42.9%), colon (17.5%), stomach (11.1%), pancreas (11.1%), gallbladder (4.8%), lung (4.8%), cutaneous melanoma (3.2%), urinary bladder (3.2%), and thyroid (1.6%). In five (25%) of the surgical cases, uterine metastases were the first indication of the presence of an extragenital primary cancer. Metastases to leiomyomas were found in 13 instances. The myometrium was more often involved than the endometrium, but endometrial curettings contained the metastatic tumor in numerous cases. Metastases to the ovaries were detected in almost two thirds of cases. Although an infrequent event, abnormal uterine bleeding may result from secondary spread to the uterine corpus from an extragenital primary neoplasm.

Adult↗

Correlation of numerical chromosome 11 and 17 imbalance with metastasis of primary breast cancer to lymph nodes.

Abnormalities of chromosomes 11 and 17 have been widely reported in invasive carcinoma of the breast. Interphase cytogenetics using pericentromeric repeat probes allows the evaluation of numerical chromosomal aberrations in tumour cell populations. We have developed a method for interphase cytogenetics on fine needle aspirates taken from breast tumours and have applied it to the analysis of chromosomes 11 and 17 in 49 cases of invasive adenocarcinoma. Frequency distributions of signal number were generated for each case and no correlation was found between modal signal number and tumour size at presentation, nodal status or tumour differentiation. In 14 cases, two copies of each of chromosomes 11 and 17 were present, and in 14, the number of chromosomes 11 and 17 were equal but abnormal. In 14 cases, the chromosome 11 number was greater than chromosome 17 and in 7 cases, the chromosome 17 number was greater than chromosome 11. Chromosome inequality correlated with the presence of lymph node metastases or disseminated disease at presentation and the absence of in situ carcinoma. There was no relationship with the presence of vascular invasion. These data suggest that numerical chromosome 11 and 17 imbalance may indicate the ability of breast cancers to metastasize rather than invade vessels. The pattern of numerical chromosome abnormality described may define a subgroup of tumours with a greater tendency for metastasis.

Adenocarcinoma↗

Histoplasmosis and penicilliosis: comparison of clinical features, laboratory findings and outcome.

INTRODUCTION: Histoplasmosis and penicilliosis are infections caused by the dimorphic fungi, Histoplasma capsulatum and Penicillium marneffei, respectively. The aim of this study was to compare the clinical presentation, laboratory and radiologic findings and outcome of these infections at Srinagarind Hospital, Khon Kaen, Thailand. METHODS: The medical records of patients who had positive cultures for Histoplasma capsulatum and Penicillium marneffei during 1996-2002 were reviewed. The data were compared and analyzed by the Chi-square and Fisher's exact tests. RESULTS: There were 32 and 36 medical records of patients with H. capsulatum and P. marneffei infection, respectively, available for review. The most common underlying disease of patients with histoplasmosis and penicilliosis was acquired immunodeficiency syndrome (AIDS), which accounted for 90.6% and 91.7%, respectively. The most common clinical findings in both infections were fever, weight loss, cough, anemia, lymphadenopathy, hepatomegaly and splenomegaly. Frequencies of skin lesions were not statistically different between either group (P=0.20). Laboratory findings were similar between the two infections, except hyperbilirubinemia, which was more common in the penicilliosis group (P=0.02). There were similar abnormal X-ray findings in both groups with interstitial infiltration the most common abnormality. CONCLUSIONS: Histoplasmosis and penicilliosis had similar clinical presentations, laboratory findings and chest X-ray abnormalities. Itraconazole is recommended as secondary prophylaxis in HIV-infected patients who have histoplasmosis or penicilliosis.

AIDS-Related Opportunistic Infections↗

FDG-PET evaluation of vaginal carcinoma.

PURPOSE: To compare the results of CT and positron emission tomography (PET) and F-18 fluorodeoxyglucose (FDG) in the detection of the primary tumor and lymph node metastases in carcinoma of the vagina. METHODS AND MATERIALS: This was a prospective registry study of 23 consecutive patients with carcinoma of the vagina, in which we respectively compared the results of CT and whole-body FDG-PET. The tumor was clinical Stage II in 16 patients, Stage III in 6, and Stage IVa in 1 patient. The primary tumor ranged in size from 2 to 10 cm (mean 4.9), and 4 patients had palpable groin lymph nodes. All patients were treated with external beam radiotherapy and brachytherapy, 14 received concurrent chemotherapy, and 2 underwent primary tumor excision before the imaging evaluation. The median follow-up was 21 months in those patients alive without disease. Survival was estimated by the Kaplan-Meier method. RESULTS: Of the 21 patients with an intact primary tumor, CT visualized it in 9 (43%). CT also demonstrated abnormally enlarged groin lymph nodes in 3 patients and both groin and pelvic lymph nodes in 1 patient (4 of 23, 17%). FDG-PET identified abnormal uptake in all 21 intact primary tumors (100%). Abnormal uptake was found in the groin lymph nodes in 4 patients, pelvic lymph nodes in 2, and both groin and pelvic lymph nodes in 2 patients (8 of 23, 35%). The 3-year progression-free and overall survival estimate was 73% and 68%, respectively. CONCLUSION: The results of this study have demonstrated that FDG-PET detects the primary tumor and abnormal lymph nodes more often than does CT.

Carcinoma↗

An aggressive spindle cell squamous carcinoma arising in a patient with long-standing erythroderma.

Spindle cell squamous carcinoma (SCSC) of the left hand arising in a patient with long-standing erythroderma is reported. Histopathologically, spindle shaped atypical cells were observed neighboring the cells of well differentiated squamous cell carcinoma. These two types of tumor cells, spindle cells and well differentiated cells, were present side by side and merged into each other. The erythroderma had been present for over 20 years, and both clinical and histopathological findings suggested cutaneous T cell lymphoma, but were not diagnostic for mycosis fungoides, Sézary syndrome, or adult T cell lymphoma. Flow cytometry of peripheral blood cells showed a low CD4/CD8 ratio which suggested impaired T cell function. Multiple metastases of SCSC occurred in a short period and the patient died ten months after his first visit to us. The aggressive course of this case was unusual, and may be due to immunological abnormalities associated with the long standing erythroderma with impaired T cell function.

Aged↗

Lymphography in childhood rhabdomyosarcomas.

Forty-one children with subdiaphragmatic rhabdomyosarcoma underwent bipedal lymphography. Twenty-two (53.5%) of the lymphograms were interpreted as being positive. In our series, the lower limbs were the most common primary site of involvement, were more frequently involved by the alveolar histologic subtype which carries a poor prognosis, and were associated with a higher incidence of lymph node metastases. Positive lymphographic findings in this group of children were similar to those seen in both adults and children with other solid tumors, i.e., the presence of discrete lymph node filling defects. However, in 3 cases, abnormalities more characteristic of lymphoma were identified. Evaluation of lymph node metastases as demonstrated by lymphography has prognostic significance.

Abdominal Neoplasms↗

Utility of axillary views in postmastectomy patients.

This study was performed to determine the utility of routine axillary radiography in postmastectomy patients. The records of 185 postmastectomy patients who had undergone axillary radiography were retrospectively reviewed. Of 499 views obtained, only 10 (2%) were reported as abnormal. Only one of the 10 patients with abnormal studies had a demonstrated recurrence that corresponded to the mammographic finding. This patient also had positive physical examination results at that time. Eight of the 185 patients developed local recurrences, four of which were in the axilla of the affected side. These four cases were all detected at physical examination; two patients underwent relatively concurrent axillary radiography. One of these two patients with abnormal physical examination results had a positive axillary view obtained in the same month. The other of these two patients with abnormal physical examination results had a negative axillary view obtained 3 months earlier. The authors conclude that routine axillary radiography is not useful in postmastectomy patients.

Axilla↗

Short-time splenic irradiation for splenomegaly.

BACKGROUND: Splenic irradiation is routinely used in the supportive treatment of lymphoepithelial and hemopoietic diseases associated with splenomegaly. A new short-time irradiation schedule with conventional dosage (Group A: 2 Gy/fract.) was compared retrospectively with low-dose prolonged treatment schedules (Group B: < or = 1 Gy/fract.) to establish its tolerance and its efficacy in terms of relieving splenomegaly-associated symptoms. PATIENTS AND METHOD: Between 12/1996 and 3/2002 49 patients (6 with CLL, 14 with CML, 6 with NHL, 16 with MPD, 6 with OMF and 1 with AML) underwent 85 treatment courses (13 courses low-dose prolonged treatment and 72 courses short-time treatment).The spleens had been exposed to pretreatment doses of 3-70 Gy (mean 20.75 Gy). Splenic size was 12-35 cm (mean 22.76 cm). One treatment was done with 60Co. 9-20 MeV electron beams were used in 23 treatments and 6 MV photons in 61 treatments. Blood counts were monitored daily. Clinical improvement (pain relief, improvement of splenomegaly-associated symptoms), spleen volume reduction and effects on blood counts were evaluated and documented at the end of the treatment. RESULTS: Of the 85 treated patients, 55 (Group A: 47/72, Group B: 8/13) showed a > 10% reduction of spleen volume and 62 (Group A: 57/72, Group B: 5/13) clinical improvement. 15 of 49 patients underwent multiple irradiation (2-8 courses) without clinical or hematologic complications. The intervals between the courses were 2-19 months. Rapid response in terms of reduction in splenic size in 23 patients permitted field reduction during treatment. Significant hematologic abnormalities were absent throughout. CONCLUSION: The short-time treatment schedule ensured a rapid response with relief of pain and improvement of gastrointestinal and pulmonary symptoms at an overall treatment time shorter than that of low-dose prolonged treatment schedules. No differences in terms of hematologic abnormalities were found between single doses of 2 Gy and < or = 1 Gy.

Adult↗

[Cytogenetic findings in ductal carcinoma of the breast].

Breast cancer in women is an important medical problem with public health and social implications. In spite of its great clinical importance, little is known about the cytogenetic features of breast carcinomas. Chromosomal abnormalities in some malignant tumors have been used for diagnosis and prognosis or for localizing genes involved in the pathology malignancies. In this report, we present the chromosomal abnormalities found in 32 primary breast ductal carcinomas. The tumor samples were studied using the technique for short-term culturing and cytogenetic analysis with G-banding. Only one tumor with normal karyotype was observed. Thirty one (99%) of the tumors had chromosomal abnormalities including 21 (65.6%) in which chromosome 1 was involved (trisomy, monosomy or structural abnormalities of the type t(1q;2p) and del(1q42). Other recurrent anomalies such as del(12p); del 4(p); +7; +8; -7; -3; were observed. The significance of these findings and their role in tumorogenesis will become more evident with close follow-up of women who have tumors with an abnormal karyotype.

Adult↗

Human pancreatic ribonuclease 1: expression and distribution in pancreatic adenocarcinoma.

BACKGROUND: Human pancreatic ribonuclease (RNase 1) is a pancreatic enzyme that is present at high levels in the serum of most patients with pancreatic adenocarcinoma. For this reason, the authors studied its patterns of expression at the single-cell level in pancreatic adenocarcinoma tissues by immunohistochemical analysis and in situ hybridization (ISH). METHODS: Immunohistochemical analysis with polyclonal antibodies against RNase 1 and by ISH with digoxigenin-labeled RNase 1 probe were used to detect RNase 1 in the neoplastic cells of ductal type pancreatic adenocarcinomas. RESULTS: Fifteen of 18 carcinoma samples were positive for RNase 1, demonstrating that the expression of ribonuclease that the authors observed previously in human pancreatic adenocarcinoma cell lines was not an artifact of cell culture. The authors also found RNase 1 in some of the metaplastic ducts and atrophic islets in 4 of 6 chronic pancreatitis samples, and they observed RNase 1 immunostaining in hyperplastic ducts adjacent to one of the well-differentiated adenocarcinomas. CONCLUSIONS: The expression levels of RNase 1 by tumor cells from pancreatic adenocarcinomas are consistent with the high RNase 1 levels found in the serum of most patients with pancreatic adenocarcinoma. This expression of RNase 1, which is an acinar protein, demonstrates that the patterns of gene expression in pancreatic adenocarcinoma are distinct from those of normal pancreatic duct cells. Conversely, RNase 1 expression levels in altered ductal cells from some chronic pancreatitis tissues and hyperplastic ducts from carcinoma tissues suggest that abnormal expression levels may be an early event in pancreatic tumorigenesis.

Adenocarcinoma↗