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PubMed2004

Nasopharyngeal carcinoma in children: ten years' experience at the Tata Memorial Hospital, Mumbai.

PURPOSE: To evaluate the disease characteristics and outcome of children with nasopharyngeal carcinoma treated at the Tata Memorial Hospital, Mumbai. METHODS AND MATERIALS: Between 1990 and 2000, 81 pediatric patients with a diagnosis of nasopharyngeal carcinoma were treated at the Tata Memorial Hospital. The median age was 14 years. The male/female ratio was 2.8:1. Of the 81 patients, 32 (39%), 21 (26%), and 28 (35%) had T1-T2, T3, and T4 (TNM International Union Against Cancer staging system, 1997), respectively. Ninety-one percent presented with nodal metastasis. Thirty patients (37%) had lymph nodes >6 cm, and 45 (56%) had bilateral nodes at presentation. Histologically, 77 patients (95%) had undifferentiated carcinoma. Eighty-five percent received neoadjuvant multiagent chemotherapy containing bleomycin, methotrexate, and cisplatin, followed by radiotherapy (RT). RESULTS: After a median follow-up of 50 months, the disease-free survival (DFS) and overall survival (OS) rate for the entire group was 45% and 54%, respectively. Kaplan-Meier curves were used for evaluation of prognostic factors and were compared using the log-rank test. Nodal status had a significant impact on DFS (p = 0.021) and OS (p = 0.006). Complete responders to chemotherapy had superior DFS (p = 0.000) and OS (p = 0.000). RT doses >60 Gy resulted in better DFS (p = 0.020) and OS (p = 0.012). Combined chemotherapy plus RT resulted in improved DFS (p = 0.457) and OS (p = 0.296), although the difference was not statistically significant. CONCLUSION: Combined modality management using chemotherapy and RT resulted in satisfactory locoregional control and OS in pediatric patients with nasopharyngeal carcinoma. Nodal involvement, response to chemotherapy, and RT dose were important prognostic factors.

Adolescent↗

PubMed2007

Chemotherapy for metastatic melanoma: time for a change?

Melanoma is a neoplasm with a rising incidence. Early-stage melanoma is curable, but advanced, metastatic melanoma almost uniformly is fatal, and patients with such advanced disease have a short median survival. Systemic therapy remains unsatisfactory, inducing complete durable responses in a small minority of patients. For the current review, the authors focused on the current role of cytotoxic chemotherapy in the treatment of metastatic melanoma and the future prospects for improvements for multiagent chemotherapy and chemotherapy combined with immunomodulatory and/or molecularly targeted agents. They discuss roles of single-agent chemotherapy, combination chemotherapy, combinations of chemotherapy with immunomodulatory or hormone agents, biochemotherapy, and combination chemotherapy with targeted therapies.

Antineoplastic Agents↗

PubMed1986

Therapeutic efficacy of multiagent chemotherapy with drug delivery enhancement by blood-brain barrier modification in glioblastoma.

Reversible osmotic blood-brain barrier (BBB) modification was used in 38 patients with glioblastoma to enhance the delivery of chemotherapeutic agents. The patients ranged in age from 14 to 70 years (mean, 43), and all had prior surgery and radiation; 5 had also received systemic chemotherapy. Karnofsky Performance Status (KPS) scores ranged from 60 to 100% (mean, 79) on admission to the treatment program. Barrier modification was achieved by intracarotid or intravertebral artery infusion of mannitol, and a chemotherapy regimen of methotrexate, cytoxan, and procarbazine was given in conjunction with barrier modification. The 38 glioblastoma patients were compared to two control groups of patients with glioblastoma; these encompassed 14 patients treated with surgery and radiation and 8 treated with surgery, radiation, and systemic chemotherapy. Survival analysis using the Cox Proportional Hazards Regression Model (corrected for age, sex, presence or absence of necrosis, and functional status) showed that patients receiving chemotherapy with BBB modification had a statistically significant (P = 0.0006) longer expected survival (17.5 months) than the control groups (12.8 and 11.4 months, respectively). Presently 16 patients of the barrier-enhanced treatment group are alive at 5 to 42 months from diagnosis (median, 20) with KPS scores ranging from 40 to 90% (median, 65). The neurological complications seen included a stroke-like syndrome in 3 patients (1 with decreased motor movement in the hand, 1 with marked hemiparesis, and 1 with hemiplegia), transient exacerbation of preexisting neurological deficits lasting 2 to 3 days, and a 15% incidence of seizures during or within 24 hours of the BBB modification. In 2 of the 38 patients, radiographic documentation of central nervous system tumor regression concurrent with the development of new tumor nodule(s) in portions of the brain distant from the region of osmotic BBB opening was seen. These studies indicate that chemotherapeutic drug delivery to tumors (as well as surrounding brain) can be augmented by osmotic BBB modification and that such therapy can result in a prolongation of survival.

Adult↗

PubMed1996

Site-specific DNA cleavage within the MLL breakpoint cluster region induced by topoisomerase II inhibitors.

The MLL gene located at 11q23 is frequently disrupted by chromosomal translocation in a wide spectrum of newly diagnosed acute leukemias. Recently, it has become apparent that the MLL gene is very frequently disrupted by chromosomal translocations in patients with secondary leukemias associated with chemotherapeutic regimens incorporating topoisomerase II inhibitors. These secondary leukemias associated with topoisomerase II inhibitors (most commonly teniposide, etoposide, or doxorubicin) have distinct clinical and biologic features which have led to the speculation that they are induced by treatment with topoisomerase II inhibitors. We have identified a site within the MLL breakpoint cluster region (bcr) that is highly sensitive to double-strand DNA cleavage induced by topoisomerase II inhibitors. This finding is quite specific and highly reproducible. Although it was initially discovered in malignant lymphoblasts isolated from a patient receiving multiagent chemotherapy, this site-specific double-strand DNA cleavage can be induced in tissue culture using malignant cell lines as well as peripheral blood from normal individuals. Site-specific cleavage occurs in a significant fraction of cells using a variety of model systems, is both time and dose dependent, and can be induced with either doxorubicin or etoposide. This site-specific cleavage maps to the same region as a consensus topoisomerase II cleavage site within the MLL bcr. These results suggest that site specific cleavage within the MLL bcr induced by topoisomerase II inhibitors may be an early step leading to MLL translocations and secondary leukemia.

Base Sequence↗

PubMed2002

Atypical teratoid/rhabdoid tumors of the central nervous system: clinical, radiographic and pathologic features.

INTRODUCTION: Atypical teratoid/rhabdoid tumors (ATT/RT) of the central nervous system (CNS) are uncommon malignancies of childhood with an aggressive course and a uniformly fatal outcome. METHODS: The medical records, radiographic images and pathologic files at the Rainbow Babies and Childrens Hospital over the previous 6 years were retrospectively reviewed. RESULTS: Eight children underwent surgery for CNS ATT/RT at our institution since 1996. There were 6 boys and 2 girls. Median age at presentation was 21 months. Four tumors had multifocal disease at the time of diagnosis. Six patients received multiagent chemotherapy including 3 patients with autologous bone marrow transplantation, and 6 patients received radiation therapy. Median survival was 9 months from the time of diagnosis. CONCLUSIONS: In spite of aggressive therapy, the prognosis for ATT/RT remains dismal. The search for effective treatment strategies will require a better understanding of the biology and molecular genetics of this tumor.

Adolescent↗

PubMed1998

Multiagency outcome evaluation of children's services: a case study.

Outcome monitoring has become a focus of accountability for public and nonprofit human service agencies. Besides providing answers to funders' questions about the services' impact, outcome monitoring helps administrators improve program effectiveness. After a three-year development period and a one-year implementation experience, SumOne for Kids represents a technically advanced outcome-monitoring system for children's mental health and/or child welfare services. Initiated, designed, and tested by 31 children's service agencies throughout Pennsylvania, and with state bureaucrats' and policy makers' encouragement, SumOne for Kids represents an effort to create a bottom-up/top-down process for implementing a statewide outcome-monitoring system. This article describes the genesis of this outcome-monitoring system, primary design principles, use of social validation for outcome selection, resolution of methodological difficulties, and reasons for selecting functional over clinical outcomes. The article reviews lessons learned through the development experience instructive to children's service managers, program evaluators, and industry leaders interested in establishing outcome-monitoring systems.

Child↗

PubMed2005

Multilevel en bloc spondylectomy and chest wall excision via a simultaneous anterior and posterior approach for Ewing sarcoma.

STUDY DESIGN: A case study of a patient with Ewing sarcoma of T8 and T9 with paravertebral and chest wall involvement, who underwent neoadjuvant chemotherapy and subsequent multilevel en bloc spondylectomy and chest wall excision using a simultaneous anterior and posterior approach. OBJECTIVE: To show the feasibility of treating Ewing sarcoma of the thoracic spine with paravertebral and chest wall extension by multiagent chemotherapy followed by a multilevel en bloc spondylectomy and chest wall excision using a simultaneous anterior and posterior approach. SUMMARY OF BACKGROUND DATA: Ewing sarcoma is a primary malignant bone tumor that occasionally involves the spinal column. Most patients with Ewing sarcoma of the spine are treated with systemic chemotherapy followed by definitive local control. Radiation therapy is the usual mode of local control in these patients because the spinal column has historically been considered a surgically inaccessible site where wide surgical margins are difficult to obtain. However, en bloc spondylectomy techniques have been described that can probably further decrease the risk of local recurrence, thereby minimizing or even eliminating the need for radiation therapy. To our knowledge, a combined en bloc spondylectomy and chest wall excision in a patient with Ewing sarcoma in the spine has not been previously reported. METHODS: Neoadjuvant chemotherapy consisting of vincristine, doxorubicin, and cyclophosphamide was administered. After completion of the chemotherapy, an en bloc spondylectomy of T8 and T9 with removal of the chest wall was achieved using a simultaneous anterior and posterior approach to the spine. A stackable carbon fiber cage filled with autograft and allograft bone was inserted between T7 and T10. The spine was stabilized with anterior and posterior instrumentation. The chest wall was reconstructed with contoured polymethylmethacrylate and polypropylene (Marlex, Textile Development Associates, Inc., Franklin Square, NY) mesh. RESULTS: The patient maintained normal neurologic function, and pain was lessened. The margins were free of tumor, and tumor necrosis was 100%. After surgery, radiotherapy was not administered. No local tumor recurrence or distant metastases were evident at the last follow-up. Balance in the coronal and sagittal planes was maintained. The patient has returned to work and resumed normal activities of daily living. CONCLUSIONS: Multilevel en bloc spondylectomy and chest wall excision performed using a simultaneous anterior and posterior approach is a safe and effective technique that may be used to achieve adequate margins in select patients with malignant tumors involving the thoracic spine and chest wall. This technique can eliminate the need for radiation therapy in patients with Ewing sarcoma and probably decreases the risk of local recurrence compared with radiation therapy alone.

Adult↗

PubMed2004

Very late central nervous system relapse in a patient with B cell lymphoblastic lymphoma.

Very late relapse of lymphoblastic lymphoma (LBL) is very rare. We report a case of a patient who developed central nervous system (CNS) relapse of LBL 16 years after the onset of the primary disease. An 8-year-old girl was hospitalized with a skin tumor in the occipital region on November 27, 1984. Examination of a biopsy of the skin tumor showed typical features of non-Hodgkin's lymphoma (diffuse medium-sized cell type). She received multiagent chemotherapy and went into remission. On July 4, 2000, she was hospitalized with persistent headache. Cranial magnetic resonance imaging showed a cerebellar lesion, which was hypointense on T1-weighted images and of heterogeneous intensity on T2-weighted images. A midline suboccipital craniotomy was performed and pathological examination revealed a diffuse proliferation of lymphoid cells, which were positive for terminal deoxynucleotidyl transferase, but negative for CD45RO, CD3 and CD20. Tumor cells stained positively for CD10, CD22, CD38 and HLA-DR. Revised immunohistochemistry of the primary specimens of skin tumor obtained 16 years earlier revealed a phenotype similar to that of the CNS disease. Polymerase chain reaction products for the immunoglobulin gene from both the skin and cerebellar specimens were an identical size. Thus, the original diagnosis of diffuse medium-sized lymphoma was revised to B cell LBL. An isolated CNS relapse of LBL was apparent in the present case. After salvage chemotherapy, the patient underwent high-dose chemotherapy with autologous peripheral blood stem cell support and subsequent craniospinal irradiation. She went into a lasting complete remission.

Cell Proliferation↗

PubMed2004

Intravascular large B-cell lymphoma presenting with mass lesions in the central nervous system: a report of five cases.

We have encountered five cases of intravascular large B-cell lymphoma (IVL) presenting with central nervous system (CNS) mass lesions during their clinical course. The age of the patients ranged from 50 to 74 years and three patients were male. All of these cases histopathologically showed typical intravascular localization of the neoplastic cells in the initial biopsy specimens obtained from sites other than the CNS. Despite multiagent chemotherapy, patients suffered from single or multiple CNS mass lesions 5-44 months after the initial diagnosis of IVL, except for one case in which IVL and the CNS mass lesion were diagnosed at the same time. The subsequent biopsy and autopsy specimens obtained from the CNS mass lesions revealed diffuse infiltration of the tumor cells with perivascular spreading, but minimal or no intravascular components. Immunohistochemical analysis of intravascular tumor cells and CNS mass lesions revealed expression of CD20, CD79a, bcl-2 and negative for CD3e and Epstein-Barr virus encoded RNA. The overall features of the CNS mass lesions were very similar to or indistinguishable from those of the primary CNS lymphomas. This implies that CNS mass lesions in the IVL cases can be correctly diagnosed only by careful attention to clinical and pathological findings. Moreover, there is the possibility that some cases previously diagnosed as primary CNS lymphomas may have include IVL cases. Further investigation is needed to explore this unusual phenomenon.

Aged↗

PubMed1999

Child death reviews: a gold mine for injury prevention and control.

OBJECTIVES: The purpose of this study was to demonstrate how child death review teams can be used to prevent future deaths through retrospective, multiagency case analysis and recommendations for educational programs and policy change. METHODS: A listing of all deaths to persons ages 21 years and younger in Philadelphia that occurred in 1995 was compiled by the Philadelphia Interdisciplinary Youth Fatality Review Team (PIYFRT), a multiagency, multidisciplinary, community based group created in 1993 with the mission to prevent future deaths through review, analysis, and initiation of corrective actions. Data were collected on demographic variables, as well as the circumstantial variables on injuries such as weapon type, alcohol and drug use, and contact with the criminal justice system, among others. Each case was reviewed thoroughly to determine whether or not the death was preventable. Selected injury related death cases were analyzed further by demographic and circumstantial variables. RESULTS: In 1995, 607 children ages 21 years and younger died in Philadelphia from natural causes (61.6%), unintentional injuries (16.3%), homicide (18.6%), suicide (2.3%), and undetermined causes (1.2%). More than a third (37.2%) of all deaths were considered preventable. Of the injury deaths (n=224), 95% were judged to be preventable. Preventable fire/burn injury deaths (n=29) were associated with lack of a smoke detector, nonsupervision of children, and faulty home appliances. Violent deaths were associated with substance abuse, gang involvement, chronic truancy, academic failure, and access to weapons. CONCLUSIONS: Relevant policies for these preventable or intervenable deaths are discussed such as use of non-battery powered smoke detectors.

Adolescent↗

PubMed2000

Novel multiagent chemotherapy for bone marrow relapse of pediatric acute lymphoblastic leukemia.

BACKGROUND: Despite improvements in the treatment of pediatric acute lymphoblastic leukemia, approximately one in five patients will develop recurrent disease. The majority of these patients do not survive. This limited institution study sought to improve event-free survival (EFS) by intensification of chemotherapy. PROCEDURE: Twenty-one patients with either an isolated marrow (n = 16) or a combined marrow and central nervous system relapse (n = 5) received treatment according to Children's Hospital of Philadelphia protocol CHP-540. Six patients had an initial remission of <36 months, and five patients had relapsed within 1 year of completion of phase III therapy. Induction and reinduction therapy consisted of idarubicin, vincristine, dexamethasone, asparaginase, and triple intrathecal chemotherapy. Consolidation and reconsolidation therapy employed high-dose cytarabine, etoposide, and asparaginase given in a sequential manner. Maintenance therapy included courses of high- or low-dose cytarabine followed by sequential etoposide and asparaginase pulse, moderate-dose methotrexate with delayed leukovorin rescue, and vincristine/dexamethasone pulses. Therapy continued for 2 years from the start of interim maintenance in the 16 patients who did not receive a bone marrow transplant (BMT). Two patients underwent an HLA-identical sibling BMT specified by protocol. Four received a nonprotocol-prescribed alternative donor BMT. RESULTS: The complete remission induction rate was 95%. With a median follow-up from date of relapse of 49 months in survivors, the actuarial EFS based on intent to treat is 75%. There were three toxic deaths in patients in CR and two deaths from relapse. CONCLUSIONS: This regimen is toxic but effective and deserves study in a larger setting.

Adolescent↗

PubMed2003

Patterns of failure after the multimodality treatment of uterine papillary serous carcinoma.

PURPOSE: Uterine papillary serous carcinoma (UPSC) is an aggressive variant of endometrial carcinoma. The majority of patients with clinical Stage I UPSC are found to have extrauterine disease at the time of surgery. Most authors report survival rates of 35-50% for Stage I-II and 0-15% for Stage III and IV UPSC. Surgical treatment as the sole therapy for patients with Stage I-IV UPSC is unacceptable because of high recurrence rates. Chemotherapy, radiotherapy, or both have been added after surgery in an attempt to improve survival. However, the survival benefit to patients from such multimodality therapy remains uncertain. This study analyzes the patterns of failure in patients with FIGO Stages I-IV UPSC treated by multimodality therapy. METHODS AND MATERIALS: Forty-two women with FIGO Stages I-IV UPSC who were treated by multimodality therapy were analyzed retrospectively between 1988 and 1998. Data were obtained from tumor registry, hospital, and radiotherapy chart reviews, operative notes, pathology, and chemotherapy flow sheets. All the patients underwent staging laparotomy, peritoneal cytology, total abdominal hysterectomy and salpingo oophorectomy, pelvic and para-aortic lymph node sampling, omentectomy, and cytoreductive surgery, when indicated followed by radiotherapy and/or chemotherapy. Therapy consisted of external beam radiation therapy in 11 patients (26%), systemic chemotherapy in 20 (48%), and both radiotherapy and chemotherapy in 11 (26%). The treatments were not assigned in a randomized fashion. The dose of external beam radiation therapy ranged from 45-50.40 Gy (median 45). Of the 31 patients (74%) who received chemotherapy, 18 received single-agent (58%), whereas 13 received multiagent chemotherapy (42%). RESULTS: Median follow-up for all patients was 19 months (range 4-72). Median follow-up for the surviving patients was 36 months (range 21-72). Their median age was 65 years. Six patients (14%) had Stage I, 8 patients (19%) had Stage II, 10 (24%) had Stage III, and 18 (43%) had Stage IV disease. Twenty-nine patients (69%) had suffered recurrence at the time of last follow-up. The actuarial failure rate at 2 and 5 years was 58% and 67%, respectively. The majority of the patients (19/29) recurred in the abdomen, vagina, or pelvis (66%). Metastases outside the abdomen were much less common as the first site of failure (17%). Twenty-five patients (60%) had died at the time of reporting; the observed survival rate at 2 years and 5 years was 52% and 43%, respectively. CONCLUSIONS: Our data suggest that, after multimodality therapy of FIGO Stage I-IV UPSC, most patients developed abdominopelvic (locoregional) failure, and the great majority of the failures occurred in the abdomen, vagina, and pelvis (66%). Abdominopelvic failure as a component of distant failure occurred in an additional 5 patients (17%). Distant failure alone occurred in 17% of the patients.We propose that future studies should combine whole abdominal radiotherapy (WART) with pelvic and vaginal boosts, in addition to chemotherapy for FIGO Stage I-IV UPSC, especially in patients with minimal residual disease, to attempt to improve the dismal prognosis of patients with UPSC.

Aged↗

PubMed2001

Central nervous system involvement in gestational trophoblastic neoplasia.

OBJECTIVE: To evaluate characteristics of patients with central nervous system (CNS) lesions of gestational trophoblastic disease and determine prognostic and therapeutic implications applicable to management. METHODS: Nine patients among 56 cases of malignant gestational trophoblastic neoplasia (GTN) were analyzed prospectively in a single institution between the years 1990 and 1997 with at least two years of follow-up. Brain metastases were documented by physical exam and computed tomography scan or magnetic resonance imaging. In terms of therapy, all of the patients received an etoposide, methotraxate, actinomycin, cyclosphamide and vincristine (EMA-CO) regimen for 5 to 9 courses. To prevent unexpected intracranial hemorrhage, all patients received radiation therapy. No intrathecal chemotherapy was given. Remission was defined as three weekly beta-hCG levels below assay sensitivity (<5 mIU/ml). RESULTS: The mean age of the patients at diagnosis was 29.6 years. While two of the patients initially presented with symptoms related to cranial involvement, five were diagnosed during routine investigation for metastasis in malignant GTD and the remaining two developed cerebral metastases during the therapy. Besides central nervous system involvement, six had additional lung, two had hepatic and splenic and one had pelvic metastases. Overall survival was 66.6%. Two patients had a fulminant clinical course and were lost one month after initial diagnosis. CONCLUSION: Early diagnosis via computed tomography of the head and beta-hCG serum testing along with aggressive, multiagent intervention (EMA-CO) have greatly improved patient prognosis of this once highly fatal condition.

Adult↗

PubMed1977

The use of a mid-Manhattan hotel as a support system.

The mass media have focused recently on the failure of psychiatric institutions to provide adequate follow-up for its patients released into the inner city. Many expatients deteriorate and become part of the "revolving door": single-room occupancy--city hospitals--state hospital. This paper attempts to demonstrate the feasibility of utilizing existing community resources (for example, a large midtown Manhattan hotel and local service agencies) to cope with this problem. A description of the multiagency organized hotel programs as well as objective and subjective data is provided in order to substantiate our contention that this approach offers a viable alternative to long-term hospitalization.

Adult↗

PubMed1986

Large noncleaved follicular center cell lymphoma. Clinical features in 53 patients.

To clarify the clinical characteristics of large noncleaved lymphoma (LNC-FCC; intermediate grade, large cell, noncleaved, Working formulation), 53 patients were studied. Thirty-one were male and 22 female. Median age was 54 years. Initial symptoms included lymphadenopathy (40%), pain (34%), and B symptoms (21%). Stage I disease was present in 6, Stage II in 9, Stage III in 14, and Stage IV in 24 (72% Stage III or IV). Gastrointestinal (GI) tract involvement was present in 13. Central nervous system (CNS) disease was present at diagnosis in two patients, occurred during therapy in two, and was the sole site of relapse in two. Bone marrow involvement was found in 7 of 50 patients (14%). Complete remission was attained in 60% of all patients. Twenty-nine Stage III and IV patients received intensive multiagent chemotherapy; complete remission (CR) was attained in 69%. In contrast, zero of nine patients with Stage III or IV disease who did not receive an anthracycline-containing regimen, attained CR. Median survival for the entire group was 25 months. It was concluded that, in our patients with LNC-FCC, GI involvement was prominent (25%) and CNS disease was not uncommon (11%). Long-term disease-free survival may be achieved in patients with more advanced disease after the administration of anthracycline-containing combination chemotherapy.

Adolescent↗

PubMed2003

Natural killer cell neoplasms: a distinctive group of highly aggressive lymphomas/leukemias.

Natural killer (NK) cell neoplasms, which include extranodal NK/T-cell lymphoma (nasal and extranasal) and aggressive NK cell leukemia, are generally rare, but they are more common in people of Oriental, Mexican and South American descent. These neoplasms are highly aggressive, and show a strong association with Epstein-Barr virus. Extranodal NK/T-cell lymphoma most commonly affects the nasal cavity and other mucosal sites of the upper aerodigestive tract. Patients present with nasal obstruction or midfacial destruction. Despite the early stage of disease at presentation, overall survival is poor. Patients with the extranasal form of the lymphoma often present with high-stage disease, commonly involving the skin, gastrointestinal tract, testis, and soft tissue, and the prognosis is even worse. Histologically, the lymphoma can show a broad cytologic spectrum, but apoptosis, necrosis, and angioinvasion are common. The most common immunophenotype is CD2(+), surface CD3(-), cytoplasmic CD3(+), CD56(+). Based on currently available data, treatment of nasal NK/T-cell lymphoma should consist of radiotherapy, with or without multiagent chemotherapy. More research is required to ascertain the role of high-dose chemotherapy with stem cell rescue and that of non-multidrug resistance-related chemotherapeutic agents. Aggressive NK cell leukemia affects younger patients, who present with poor general condition, fever, and disseminated disease; they often die within a short time from systemic disease or complications such as multi-organ failure. The peripheral blood and bone marrow show atypical large granular lymphocytes, which exhibit an immunophenotype similar to that of extranodal NK/T-cell lymphoma. Aggressive NK cell leukemia must be distinguished from T-cell large granular lymphocyte leukemia and indolent NK cell lymphoproliferative disorder, both of which are indolent.

Diagnosis, Differential↗

PubMed2005

CD56/NCAM-positive Langerhans cell sarcoma: a clinicopathologic study of 4 cases.

This report concerns the clinicopathologic features of 4 patients with CD56/neural cell adhesion molecule (NCAM)-positive Langerhans cell sarcoma (LCS). Three of the patients were elderly, between 59 and 62 years of age at presentation, and the other was 35 years old. The presenting symptoms included fever, bone pain, and weakness. The patients shared some clinical findings, such as multiorgan involvement of lymph nodes, skin, lung, bone marrow, and spleen. LCS carries a poor prognosis, and 3 of the patients died of the disease within several years of presentation despite multiagent chemotherapy and radiotherapy. Of special interest is that all of the cases showed CD56 expression on the tumor cells in addition to expression of CD1a, S100beta, and langerin, the presence of which suggests derivation from Langerhans cells. For control, CD56 was also examined in 8 cases of Langerhans cell histiocytosis (LCH), a single-system unifocal or multifocal disease, and the results of staining of the tumor cells were negative. Our findings indicated that CD56 may be a clinically relevant biologic marker for predicting an intractable course of Langerhans cell neoplasms, although it is often difficult to draw a definite morphologically-based distinction between LCS and LCH.

Adult↗

PubMed1999

Cerebrospinal fluid asparagine concentrations after Escherichia coli asparaginase in children with acute lymphoblastic leukemia.

PURPOSE: The CNS is an important sanctuary site in childhood acute lymphoblastic leukemia (ALL). CSF asparagine concentration reflects asparaginase systemic pharmacodynamics. We evaluated the time course of CSF asparagine depletion in children with ALL during and after a course of Escherichia coli asparaginase. PATIENTS AND METHODS: Thirty-one children (24 newly diagnosed and seven at relapse) received E coli asparaginase 10,000 IU/m2 intramuscularly three times weekly for six and nine doses, respectively, as part of multiagent induction chemotherapy. CSF asparagine levels were measured before, during, and after asparaginase dosing. RESULTS: The percentage of patients with undetectable (< 0.04 micromol/L) CSF asparagine was 3.2% (one of 31 patients) at baseline, 73.9% (17 of 23) during asparaginase therapy, and 56.3% (nine of 16) 1 to 5 days, 43.8% (seven of 16) 6 to 10 days, 20.0% (two of 10) 11 to 30 days and 0% (zero of 21) more than 30 days after asparaginase therapy. The proportion of patients with depleted CSF asparagine was higher during asparaginase therapy than at baseline (P < .001), 11 to 30 days (P = .003), and more than 30 days after asparaginase therapy (P < .001). Median CSF asparagine concentrations were 4.42 micromol/L before, less than 0.04 micromol/L during, and less than 0.04 micromol/L at 1 to 5 days, 1.63 micromol/L at 6 to 10 days, 1.70 micromol/L at 11 to 30 days, and 5.70 micromol/L at more than 30 days after asparaginase therapy, respectively. CSF depletion was more common in patients with low baseline CSF asparagine concentrations (P = .003). CONCLUSION: CSF asparagine concentrations are depleted by conventional doses of E coli asparaginase in the majority of patients, but they rebound once asparaginase therapy is completed.

Adolescent↗