Primary lymphoma of bone in children.

1989 ◽  
Vol 7 (9) ◽  
pp. 1275-1280 ◽  
Author(s):  
W L Furman ◽  
S Fitch ◽  
H O Hustu ◽  
T Callihan ◽  
S B Murphy
Keyword(s):  
Progressive Disease ◽  
Large Cell ◽  
Initial Therapy ◽  
National Institutes Of Health ◽  
Primary Lymphoma ◽  
Histologic Subtype ◽  
Lymphoma Treatment ◽  
Local Radiation ◽  
Multiagent Chemotherapy ◽  
Cessation Of Treatment

Primary lymphoma of bone (PLB) occurs infrequently in children. Between January 1962 and November 1988, 395 children with non-Hodgkin's lymphoma (NHL) were treated at St. Jude Children's Research Hospital. Eleven of these patients (2.8%) presented with a bone primary, usually in the femur (eight of 11 patients). The median age of these seven boys and four girls at presentation was 13 years (range, 5.5 to 19 years). Seven patients had one or more additional bones involved. All patients had high-grade lymphomas based on the National Institutes of Health (NIH) Working Formulation. The histologic subtypes included six large-cell lymphomas, three lymphoblastic lymphomas, one small-noncleaved, non-Burkitt's lymphoma, and one unclassifiable lymphoma. Treatment consisted of multiagent chemotherapy combined with local radiation therapy in seven of 11 patients. Six of 10 children who received chemotherapy as a component of their initial therapy, including all who presented with localized tumor, are alive with no evidence of disease 2+ to 85+ months (median, 42.5 months) after cessation of treatment; one patient has just completed chemotherapy. Each of the four patients who died showed leukemic conversion 5 to 11 months after diagnosis, and three died of progressive disease. One patient died of sepsis during chemotherapy-induced neutropenia with no evidence of disease at necropsy. We conclude that optimal therapy for PLB, as with all other forms of NHL, should focus on the histologic subtype and stage of disease.

Stomach conservation in stages IE and IIE gastric non-Hodgkin's lymphoma.

1990 ◽  
Vol 8 (2) ◽  
pp. 266-271 ◽  
Author(s):  
M H Maor ◽  
W S Velasquez ◽  
L M Fuller ◽  
K B Silvermintz
Keyword(s):  
Progressive Disease ◽  
Treatment Plan ◽  
Gastric Lymphoma ◽  
Subtotal Gastrectomy ◽  
Large Cell ◽  
Endoscopic Biopsy ◽  
Tumor Dissemination ◽  
Abdominal Disease ◽  
Local Radiation ◽  
Well Differentiated

Thirty-four patients with stages IE and IIE gastric lymphoma were treated with chemotherapy and radiotherapy combinations without stomach resection. In 20 patients, the diagnosis was established by endoscopic biopsy only; the other 14 had laparotomy and biopsy. No patient had a gastrectomy before treatment. Nineteen patients had stage IE disease and 15 had stage IIE. Lymphoma diagnoses were: diffuse large-cell, 26; immunoblastic, three; diffuse well-differentiated, three; nodular mixed, one; and unclassified, one. The treatment plan was to deliver an initial four cycles of chemotherapy, followed by radiotherapy, and finally, more chemotherapy. Thirty-three patients received cyclophosphamide, doxorubicin, vincristine, prednisone, and bleomycin (CHOP-Bleo). Four patients with stage IIE disease received cyclophosphamide, methotrexate, etoposide, and dexamethasone (CMED). Twenty-three patients (68%) never had a relapse. Three patients had successful salvage therapy, one for local recurrence and two for tumor dissemination. Five patients died of recurrent abdominal disease, and one died of tumor dissemination. Two died of treatment-related complications, one of sepsis during treatment with CMED and one of bleomycin-induced lung fibrosis. No patient developed stomach perforation or bleeding as a result of chemotherapy or radiotherapy. Twenty-four of the 26 surviving patients were able to retain their stomachs. One patient required a gastrectomy for progressive disease during chemotherapy, and another required a subtotal gastrectomy for relief of an obstruction caused by cicatrization. These data show that surgery is not a necessary procedure in gastric lymphoma. Favorable results can be achieved by combining effective chemotherapy and local radiation.

Chiasmatic optic glioma treated with chemotherapy

1985 ◽  
Vol 63 (6) ◽  
pp. 862-866 ◽  
Author(s):  
Jeffrey G. Rosenstock ◽  
Roger J. Packer ◽  
Larissa Bilaniuk ◽  
Derek A. Bruce ◽  
Jerri-Lynne Radcliffe ◽  
...  
Keyword(s):  
Radiation Therapy ◽  
Young Children ◽  
Progressive Disease ◽  
Actinomycin D ◽  
Initial Therapy ◽  
Optic Glioma ◽  
Newly Diagnosed ◽  
Content Type ◽  
Novel Approach ◽  
Clinical Stabilization

✓ Chiasmatic optic glioma is a rare tumor with an erratic natural history, usually seen in young children. A prior study from this institution demonstrated that these lesions were frequently lethal, despite initial clinical stabilization following radiation therapy, and that visual, intellectual, and late endocrinological disabilities were prevalent. A novel approach was developed in 1977, when an initial clinical response to vincristine was recorded in a child with a recurrent optic glioma. Since then, all children with recurrent optic glioma and all children aged 6 years old and under with newly diagnosed optic glioma have been offered a program of initial therapy with vincristine and actinomycin D for six cycles over 18 months. The four children with recurrent tumor who were treated with that regimen remain clinically stable 13 to 115 months after chemotherapy. Twelve children (eight under 24 months old) with newly diagnosed optic glioma have been treated with this program, and three are still on therapy. Four developed progression while on therapy, and five remain stable from 1 to 60 months posttherapy. The four children who developed progressive disease have been treated with radiation therapy and remain stable. Six of the 12 children showed shrinkage of their tumor on computerized tomography while receiving chemotherapy. This program may serve as an alternative to initial radiation therapy in young children.

Primary Lymphoma of Bone: Long Term Results in Patients Treated with Vincristine - Adriamycin - Cyclophosphamide and Local Radiation

1991 ◽  
Vol 3 (3) ◽  
pp. 189-193 ◽  
Author(s):  
G. Bacci ◽  
A. Ferraro ◽  
R. Casadei ◽  
E. Barbieri ◽  
M. Avella ◽  
...  
Keyword(s):  
Long Term Results ◽  
Primary Lymphoma ◽  
Local Radiation

Prognostic Value of Squamous Cell Carcinoma Antigen in Patients with Uterine Cervical Carcinoma

1988 ◽  
Vol 74 (2) ◽  
pp. 221-225 ◽  
Author(s):  
Jyotsna M. Bhatavdekar ◽  
Nandita Ghosh ◽  
Minakshi K. Shukla ◽  
Damodar B. Balar ◽  
Anita Bhaduri ◽  
...  
Keyword(s):  
Squamous Cell Carcinoma ◽  
Cell Carcinoma ◽  
Cervical Carcinoma ◽  
Squamous Cell ◽  
Large Cell ◽  
Diagnostic Value ◽  
Squamous Cell Carcinoma Antigen ◽  
Histologic Subtype ◽  
Uterine Cervical Carcinoma ◽  
Highly Correlated

Squamous cell carcinoma antigen (SCC Ag) was estimated in 30 controls, in 16 patients with benign lesions of the uterine cervix, and in 51 patients with uterine cervical carcinoma. The rate of positivity of the antigen among the cancer patients was 87% (N = 49). SCC Ag estimations were of no diagnostic value, since 37% of patients with bening lesions had elevated levels compared to controls. SCC Ag was highly correlated to histologic subtype. The highest values were obtained in keratinizing tumors, followed by large cell nonkeratinizing and small cell nonkeratinizing types. Moreover, elevation of SCC Ag was statistically significant (p < 0.001) among all the three histologic subtypes compared to controls. However, SCC Ag levels were not consistently correlated to the stage of the disease. Patients with pretherapeutic SCC Ag levels above 30 ng/ml had a faster recurrence rate and shorter survival than those who exhibited antigen values below 2.0 ng/ml. From our results, it is suggested that SCC Ag has limited use as a parameter for early diagnosis of cervical carcinoma, but it seems to reflect advancement of the disease. These findings indicate that SCC Ag elevation may prove to be a valuable marker in predicting subclinical disease.

Primary lymphoma of bone in children: analysis of treatment results with adriamycin, prednisone, Oncovin (APO), and local radiation therapy.

1986 ◽  
Vol 4 (4) ◽  
pp. 496-501 ◽  
Author(s):  
J S Loeffler ◽  
N J Tarbell ◽  
H Kozakewich ◽  
J R Cassady ◽  
H J Weinstein
Keyword(s):  
Bone Tumors ◽  
Disease Free Survival ◽  
Clinical Staging ◽  
Primary Lymphoma ◽  
Actuarial Survival ◽  
Cell Counts ◽  
Local Radiation ◽  
Disease Free Survival Rate ◽  
Second Tumor ◽  
Age Range

Primary lymphoma of bone is an unusual extranodal presentation of pediatric non-Hodgkin's lymphoma (NHL). Treatment with radiotherapy alone has resulted in a disease-free survival rate of approximately 50% in most adult series. Between January 1973 and April 1985, 11 children with biopsy-proven NHL of bone were seen and treated at our institutions. The minimal clinical staging included chest and bone radiographs, a radionuclide bone scan, complete blood cell counts and serum chemistries, and a bone marrow aspirate and biopsy. The age range was 9 to 17 years with a median age of 14 years. Histology included diffuse lymphoblastic lymphoma in four patients and diffuse histiocytic lymphoma in seven. Each patient was treated with the Adriamycin/prednisone/Oncovin (APO) protocol and ten patients received concomitant radiation to the whole bone when possible and a boost to the primary lesion(s). The median tumor dose was 5,000 rad (range, 3,600 to 5,600). The median follow-up was 8 years. There have been no relapses, but two patients have developed second bone tumors 5 and 7 1/2 years after beginning therapy. Each second tumor arose directly in the radiation field. The overall 8-year actuarial survival is 83%. We conclude that APO and local radiation results in excellent overall survival for children with primary NHL of bone. The occurrence of two second bone tumors, however, raises questions regarding dose and/or the role of radiation for this disease.

scholarly journals NETest Liquid Biopsy Is Diagnostic of Lung Neuroendocrine Tumors and Identifies Progressive Disease

2019 ◽  
Vol 108 (3) ◽  
pp. 219-231 ◽  
Author(s):  
Anna Malczewska ◽  
Kjell Oberg ◽  
Lisa Bodei ◽  
Harry Aslanian ◽  
Anna Lewczuk ◽  
...  
Keyword(s):  
Liquid Biopsy ◽  
Progressive Disease ◽  
Tumor Tissue ◽  
Disease Status ◽  
Large Cell ◽  
Clinical Progression ◽  
Lung Cancers ◽  
Recist 1.1 ◽  
Blinded Study ◽  
Matched Tissue

Background: There are no effective biomarkers for the management of bronchopulmonary carcinoids (BPC). We examined the utility of a neuroendocrine multigene transcript “liquid biopsy” (NETest) in BPC for diagnosis and monitoring of the disease status. Aim: To independently validate the utility of the NETest in diagnosis and management of BPC in a multicenter, multinational, blinded study. Material and Methods: The study cohorts assessed were BPC (n = 99), healthy controls (n = 102), other lung neoplasia (n = 101) including adenocarcinomas (ACC) (n = 41), squamous cell carcinomas (SCC) (n = 37), small-cell lung cancer (SCLC) (n = 16), large-cell neuroendocrine carcinoma (LCNEC) (n = 7), and idiopathic pulmonary fibrosis (IPF) (n = 50). BPC were histologically classified as typical (TC) (n = 62) and atypical carcinoids (AC) (n = 37). BPC disease status determination was based on imaging and RECIST 1.1. NETest diagnostic metrics and disease status accuracy were evaluated. The upper limit of normal (NETest) was 20. Twenty matched tissue-blood pairs were also evaluated. Data are means ± SD. Results: NETest levels were significantly increased in BPC (45 ± 25) versus controls (9 ± 8; p < 0.0001). The area under the ROC curve was 0.96 ± 0.01. Accuracy, sensitivity, and specificity were: 92, 84, and 100%. NETest was also elevated in SCLC (42 ± 32) and LCNEC (28 ± 7). NETest accurately distinguished progressive (61 ± 26) from stable disease (35.5 ± 18; p < 0.0001). In BPC, NETest levels were elevated in metastatic disease irrespective of histology (AC: p < 0.02; TC: p = 0.0006). In nonendocrine lung cancers, ACC (18 ± 21) and SCC (12 ± 11) and benign disease (IPF) (18 ± 25) levels were significantly lower compared to BPC level (p < 0.001). Significant correlations were evident between paired tumor and blood samples for BPC (R: 0.83, p < 0.0001) and SCLC (R: 0.68) but not for SCC and ACC (R: 0.25–0.31). Conclusions: Elevated ­NETest levels are indicative of lung neuroendocrine neoplasia. NETest levels correlate with tumor tissue and imaging and accurately define clinical progression.

scholarly journals A Novel JAK1 Mutant Breast Implant-Associated Anaplastic Large Cell Lymphoma Patient-Derived Xenograft Fostering Pre-Clinical Discoveries

Cancers ◽  
2020 ◽  
Vol 12 (6) ◽  
pp. 1603 ◽  
Author(s):  
Danilo Fiore ◽  
Luca Vincenzo Cappelli ◽  
Paul Zumbo ◽  
Jude M. Phillips ◽  
Zhaoqi Liu ◽  
...  
Keyword(s):  
Cell Lymphoma ◽  
Breast Implant ◽  
Large Cell ◽  
Tumor Xenograft ◽  
Sensitivity Threshold ◽  
Primary Lymphoma ◽  
Molecular Features ◽  
Primary Sample

Breast implant-associated lymphoma (BIA-ALCL) has recently been recognized as an independent peripheral T-cell lymphoma (PTCL) entity. In this study, we generated the first BIA-ALCL patient-derived tumor xenograft (PDTX) model (IL89) and a matching continuous cell line (IL89_CL#3488) to discover potential vulnerabilities and druggable targets. We characterized IL89 and IL89_CL#3488, both phenotypically and genotypically, and demonstrated that they closely resemble the matching human primary lymphoma. The tumor content underwent significant enrichment along passages, as confirmed by the increased variant allele frequency (VAF) of mutations. Known aberrations (JAK1 and KMT2C) were identified, together with novel hits, including PDGFB, PDGFRA, and SETBP1. A deep sequencing approach allowed the detection of mutations below the Whole Exome Sequencing (WES) sensitivity threshold, including JAK1G1097D, in the primary sample. RNA sequencing confirmed the expression of a signature of differentially expressed genes in BIA-ALCL. Next, we tested IL89’s sensitivity to the JAK inhibitor ruxolitinib and observed a potent anti-tumor effect, both in vitro and in vivo. We also implemented a high-throughput drug screening approach to identify compounds associated with increased responses in the presence of ruxolitinib. In conclusion, these new IL89 BIA-ALCL models closely recapitulate the primary correspondent lymphoma and represent an informative platform for dissecting the molecular features of BIA-ALCL and performing pre-clinical drug discovery studies, fostering the development of new precision medicine approaches.

scholarly journals Large cell lymphoma complicating acute lymphoblastic leukemia

Blood ◽  
1984 ◽  
Vol 63 (4) ◽  
pp. 935-939 ◽  
Author(s):  
R Ellerbroek ◽  
K Foucar ◽  
A Kowal-Vern ◽  
JD Kemp ◽  
T Kisker ◽  
...  
Keyword(s):  
Acute Lymphoblastic Leukemia ◽  
Small Bowel ◽  
Complete Remission ◽  
Cell Lymphoma ◽  
Lymphoblastic Leukemia ◽  
Rare Complication ◽  
Large Cell ◽  
Terminal Deoxynucleotidyl Transferase ◽  
Large Cell Lymphoma ◽  
Multiagent Chemotherapy

Abstract Non-Hodgkin's lymphoma (NHL) is a very rare complication of acute lymphoblastic leukemia (ALL). We present the pathologic, clinical, immunologic, and ultrastructural features of the third reported example of NHL following successfully treated ALL. This white girl developed ALL with predominantly L1 cells at 3.5 yr of age. The lymphoblasts were terminal deoxynucleotidyl transferase (TdT) positive and were non-B, non-T cells. She achieved a complete remission with standard induction therapy and has remained in continuous complete remission. Four and one- third years after the onset of ALL, she developed multifocal, pleomorphic large cell lymphoma of the small bowel, which resulted in episodes of intussusception and obstruction. These pleomorphic and frequently multinucleated lymphoma cells lacked TdT, common ALL antigen, and all tested markers of B cell, T cell, and histiocyte differentiation. Following three small bowel resections, systemic multiagent chemotherapy, and abdominal irradiation, she is currently free of disease.

Frequent expression of follicular dendritic cell markers in Hodgkin lymphoma and anaplastic large cell lymphoma

2013 ◽  
Vol 66 (7) ◽  
pp. 589-596 ◽  
Author(s):  
Soo Hee Kim ◽  
Ji-young Choe ◽  
Yoonkyong Jeon ◽  
Jooryung Huh ◽  
Hye Ra Jung ◽  
...  
Keyword(s):  
Hodgkin Lymphoma ◽  
Anaplastic Large Cell Lymphoma ◽  
Cell Lymphoma ◽  
Large Cell ◽  
Class Iii ◽  
Diagnostic Efficacy ◽  
Histologic Subtype ◽  
The Mean ◽  
Large Cell Lymphoma ◽  
Follicular Dendritic

AimsAlthough the tumour cells of Hodgkin lymphoma (HL) are derived from mature B-cells, the lineage infidelity of Hodgkin/Reed–Sternberg cells (HRSs) often causes diagnostic problems. Recently introduced HRS markers are also positive for follicular dendritic cells (FDCs). We investigated the expression of several FDC markers in HL and anaplastic large cell lymphoma (ALCL) and evaluated their diagnostic efficacy.MethodsEighty-five cases of HL and 52 cases of ALCL were included in this study. Immunohistochemistry was performed for glioma-associated homologue (GLI) 3, class III β-tubulin (TUBB3), fascin, clusterin, γ-synuclein, podoplanin, syntenin, CD21, CD35 and EGFR.ResultsHRSs were diffusely positive for GLI3, fascin and TUBB3; the mean positivity rates per case were 94% for GLI3, 82% for fascin, 69% for TUBB3, 17% for clusterin, 17% for γ-synuclein and 14% for syntenin. Podoplanin, CD21, CD35 and EGFR were almost negative. However, the frequency of marker expression was not associated with the histologic subtype or the presence of Epstein–Barr virus (EBV). ALCL showed a similar pattern to HL, but the overall frequency of positivity was lower than that observed in HL. The mean positivity rates were 56% for GLI3, 62% for fascin, 58% for TUBB3 and 21% for clusterin. The other markers were nearly negative. Anaplastic large cell lymphoma kinase positivity did not affect the expression rates.ConclusionsThis study confirmed the frequent expression of FDC markers in HL and ALCL. Especially, GLI3, fascin and TUBB3 are the most sensitive markers. Further studies are required to evaluate the association between FDCs, HRSs and ALCL cells.

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