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1.
Front Oncol ; 14: 1375125, 2024.
Article in English | MEDLINE | ID: mdl-38567161

ABSTRACT

Purpose: The aim of this study was to translate, culturally adapt, and evaluate the psychometric properties of the Spanish Long-Term Quality of Life (LTQL) questionnaire. Methods: The LTQL was initially translated into Spanish and cross-culturally adapted based on established guidelines. The Spanish LTQL was administered to patients with breast cancer who had completed their initial treatment 5 years earlier, along with other self-report measures: Quality of Life in Adult Cancer Survivors (QLACS), Hospital Anxiety and Depression Scale (HADS) and EORT-QLQ-BR23. Reliability was evaluated using internal consistency and test-retest. Convergent and known-groups validity were examined. Structural validity as determined by confirmatory factor analysis (CFA) and Rasch analyses was used to assess the unidimensionality and item-functioning of the LTQL domains. Results: Cronbach's alpha were above 0.7 in all domains. Test-retest coefficients were between 0.72 to 0.96 for LTQL domains. LTQL total score was correlated with others total scores of other measures: QLACS (r=-0.39), HADS depression (r=-0.57), HADS anxiety (-0.45) and EORTC-QLQ-BR23 (r=-0.50). CFA provided satisfactory fit indices, with RMSEA value of 0.077 and TLI and CFI values of 0.901 and 0.909, respectively. All factor loadings were higher than 0.40 and statistically significant (P<0.001). Rasch analysis showed that Somatic Concerns domain had 4 misfitting items, and Philosophical/Spiritual View of Life and social Support domains only 1 misfit item. However, unidimensionality was supported for the four domains. Conclusion: The findings support the validity and reliability of the Spanish version of LTQL questionnaire to be used in long-term cancer female survivors.

2.
Eur J Cancer Care (Engl) ; 31(6): e13730, 2022 Nov.
Article in English | MEDLINE | ID: mdl-36226900

ABSTRACT

OBJECTIVE: The objective of this work is to identify unmet information needs of long-term-survivors of breast cancer (BC) and future research needs from the perspectives of patients and health care professionals. METHODS: Two online Delphi surveys were conducted. Participants in Survey 1 were patients. Participants in Survey 2 were health care professionals from both primary and secondary care involved in BC care. Both surveys included three successive rounds. The first round aimed to identify research and information needs; the second round aimed to rank the relative importance of those needs; the third round aimed to find consensus. RESULTS: The most important information needs were self-management recommendations of common health problems after treatment and complications of breast reconstruction after 5 years. The most important research priorities were related to interventions and tools to increase information provision by professionals about certain tests, diet, and coordinated action between primary and specialised care during follow-up, and indications and safety issues of pregnancy in survivors. CONCLUSIONS: Two fundamental ideas were identified: (1) Patients request information about self-management common health problems after treatment and breast reconstruction complications. (2) Health care professionals emphasise the need for a standardised approach based on protocols, recommendations, and coordinated actions in the provision of information. IMPLICATIONS FOR CANCER SURVIVORS: Given the increasing number of BC survivors, it is essential to identify information and research needs to improve their care and health outcomes.


Subject(s)
Breast Neoplasms , Pregnancy , Female , Humans , Breast Neoplasms/therapy , Breast Neoplasms/complications , Survivorship , Survivors , Health Personnel , Surveys and Questionnaires , Research
3.
Oncol Res Treat ; 41(12): 755-761, 2018.
Article in English | MEDLINE | ID: mdl-30419558

ABSTRACT

BACKGROUND: A high neutrophil to lymphocyte ratio (NLR) has been associated with adverse outcomes in non-small cell lung cancer (NSCLC). However, information on epidermal growth factor receptor (EGFR)-mutant advanced NSCLC is scarce, and most of the studies published have been conducted in Asian populations. We aimed to assess the influence of pretreatment NLR on progression-free survival (PFS) and overall survival (OS) in Western European patients treated with EGFR tyrosine kinase inhibitors (TKIs). METHODS: A retrospective evaluation of 41 patients with EGFR-mutant advanced NSCLC treated with EGFR TKIs between June 2010 and May 2016 was carried out. The association between several prognostic factors including pretreatment NLR and survival was analyzed. RESULTS: Median PFS and OS were 10.58 and 20.84 months, respectively. OS for patients with a high NLR was 7.4 months, compared to 24.6 months for patients with a low NLR (p = 0.0122). In multivariate analysis, poor performance status (ECOG PS ≥ 2) and presence of ≥ 3 metastatic locations were identified as significant independent prognostic factors for worse PFS. For OS, unfavorable prognostic factors were a high NLR and central nervous system metastasis at diagnosis. CONCLUSION: Pretreatment NLR is an independent prognostic factor for OS in Western European patients with EGFR-mutant NSCLC treated with EGFR TKIs.


Subject(s)
Carcinoma, Non-Small-Cell Lung/blood , Lung Neoplasms/blood , Lymphocytes , Neutrophils , Protein Kinase Inhibitors/therapeutic use , Aged , Carcinoma, Non-Small-Cell Lung/drug therapy , Carcinoma, Non-Small-Cell Lung/genetics , Carcinoma, Non-Small-Cell Lung/mortality , Disease Progression , ErbB Receptors/antagonists & inhibitors , ErbB Receptors/genetics , Erlotinib Hydrochloride/therapeutic use , Female , Follow-Up Studies , Gefitinib/therapeutic use , Humans , Lung Neoplasms/drug therapy , Lung Neoplasms/genetics , Lung Neoplasms/mortality , Lymphocyte Count , Male , Middle Aged , Prognosis , Progression-Free Survival , Retrospective Studies , Survival Analysis
4.
Med. clín (Ed. impr.) ; 148(8): 345-350, abr. 2017. tab, graf
Article in Spanish | IBECS | ID: ibc-161618

ABSTRACT

Antecedentes y objetivo: Se evaluaron los factores de riesgo asociados a supervivencia a largo plazo en pacientes con metástasis hepáticas de carcinoma colorrectal sometidos a tratamientos ablativos. Pacientes y métodos: Estudio de cohorte unicéntrico, retrospectivo, analizando los pacientes consecutiva y prospectivamente, presentando metástasis hepáticas no resecables de carcinoma colorrectal, siendo tratados con procedimientos ablativos entre 1996 y 2013. Los factores asociados a supervivencia fueron identificados utilizando el modelo de Cox de riesgo proporcional con covariables dependientes del tiempo. También se realizó una selección de variables con el criterio de información de Akaike. Se calcularon el riesgo relativo y los intervalos de confianza al 95% para cada factor. La significación estadística se estableció en p<0,05. Resultados: Setenta y cinco pacientes con metástasis hepáticas de cáncer colorrectal, con una edad media de 65,6 (10,3) años, fueron sometidos a 106 tratamientos. Las variables seleccionadas fueron: extensión tumoral (RR 3,070, IC 95% 1,776-5,308) y buena calidad de vida (RR 0,308, IC 95% 0,150-0,632). La mediana de supervivencia global fue de 18,5 meses (IC 95% 17,4-24,4) y la mediana de pronóstico de supervivencia, de 13,5 frente a 23,4 meses para pacientes con y sin extensión del tumor, y de 23 frente a 12,8 meses para pacientes con buena y regular o mala calidad de vida, respectivamente. Conclusiones: La extensión tumoral y la buena calidad de vida fueron los únicos factores predictivos estadísticamente significativos de supervivencia a largo plazo en pacientes con carcinoma colorrectal con metástasis hepáticas sometidos a tratamiento ablativo con control ultrasonográfico (AU)


Background and objective: The risk factors associated to long-term survival were assessed in patients with liver metastases of colorectal carcinoma undergoing ablative therapies. Patients and methods: Single-centre cohort study, retrospectively analysed and prospectively collected consecutive patients with unresectable metastatic liver disease of colorectal carcinoma treated with ablative therapies between 1996 and 2013. Factors associated with survival time were identified using Cox's proportional hazard model with time-dependent covariates. A forward variable selection based on Akaike information criterion was performed. Relative risk and 95% confidence intervals for each factor were calculated. Statistical significance was set as P<.05. Results: Seventy-five patients with liver metastases of colorectal cancer, with a mean age of 65.6 (10.3) underwent 106 treatments. Variables selected were good quality of life (RR 0.308, 95% CI 0.150-0.632) and tumour extension (RR 3.070, 95% CI 1.776-5.308). The median overall survival was 18.5 months (95% CI 17.4-24.4). The survival prognosis in median was 13.5 vs. 23.4 months for patients with and without tumour extension, and 23.0 vs. 12.8 months for patients with good and fair or poor quality of life, respectively. Conclusions: Good quality of life and tumour extension were the only statistically significant predictors of long-term survival in patients of colorectal carcinoma with liver metastatic disease undergoing ablative treatment with ultrasound (AU)


Subject(s)
Humans , Male , Female , Colorectal Neoplasms/complications , Colorectal Neoplasms , Survivorship , Catheter Ablation , Neoplasm Metastasis , Liver Neoplasms/complications , Liver Neoplasms , Ultrasonography, Interventional/instrumentation , Ultrasonography, Interventional/trends , Cohort Studies , Confidence Intervals , Radio Waves/therapeutic use
5.
Med Clin (Barc) ; 148(8): 345-350, 2017 Apr 21.
Article in English, Spanish | MEDLINE | ID: mdl-28073517

ABSTRACT

BACKGROUND AND OBJECTIVE: The risk factors associated to long-term survival were assessed in patients with liver metastases of colorectal carcinoma undergoing ablative therapies. PATIENTS AND METHODS: Single-centre cohort study, retrospectively analysed and prospectively collected consecutive patients with unresectable metastatic liver disease of colorectal carcinoma treated with ablative therapies between 1996 and 2013. Factors associated with survival time were identified using Cox's proportional hazard model with time-dependent covariates. A forward variable selection based on Akaike information criterion was performed. Relative risk and 95% confidence intervals for each factor were calculated. Statistical significance was set as P<.05. RESULTS: Seventy-five patients with liver metastases of colorectal cancer, with a mean age of 65.6 (10.3) underwent 106 treatments. Variables selected were good quality of life (RR 0.308, 95% CI 0.150-0.632) and tumour extension (RR 3.070, 95% CI 1.776-5.308). The median overall survival was 18.5 months (95% CI 17.4-24.4). The survival prognosis in median was 13.5 vs. 23.4 months for patients with and without tumour extension, and 23.0 vs. 12.8 months for patients with good and fair or poor quality of life, respectively. CONCLUSIONS: Good quality of life and tumour extension were the only statistically significant predictors of long-term survival in patients of colorectal carcinoma with liver metastatic disease undergoing ablative treatment with ultrasound.


Subject(s)
Colorectal Neoplasms/pathology , Hepatectomy/methods , Liver Neoplasms/secondary , Liver Neoplasms/surgery , Ultrasonography, Interventional , Adult , Aged , Aged, 80 and over , Colorectal Neoplasms/mortality , Female , Follow-Up Studies , Humans , Liver Neoplasms/diagnostic imaging , Liver Neoplasms/mortality , Male , Middle Aged , Quality of Life , Retrospective Studies , Risk Factors , Survival Analysis , Treatment Outcome
6.
Clin Lymphoma Myeloma Leuk ; 15(12): 761-5, 2015 Dec.
Article in English | MEDLINE | ID: mdl-26319620

ABSTRACT

BACKGROUND: Late-onset neutropenia (LON) is a known adverse effect to rituximab therapy. Information about its real incidence and clinical implications comes from case reports and few retrospective studies specifically designed to study LON. However, large prospective studies of LON are lacking in the literature. We aimed to determine the incidence of LON in a group of non-Hodgkin lymphoma patients treated with rituximab and to analyze the clinical course, complications, and risk factors associated with LON. PATIENTS AND METHODS: We retrospectively reviewed 183 patients with a diagnosis of non-Hodgkin lymphoma consecutively treated with rituximab alone or in combination with chemotherapy. RESULTS: We identified 11 patients with grade 3/4 LON (13 episodes) out of 183 patients (6%). The median time to onset of LON was 75 days, and the median time to recovery from neutropenia was 100 days. The median neutrophil count nadir was 0.55 × 10(9)/L (range, 0.06-0.9 × 10(9)/L). Two patients presented infectious complications, one with fatal outcome. CONCLUSION: In our experience, the incidence of recognized LON is low (6%), although its real incidence may be greater because of the asymptomatic course and quick recovery in most cases. Infectious complications are unusual, but life-threatening complications can emerge. A careful evaluation of all cases of LON is warranted.


Subject(s)
Antineoplastic Agents/adverse effects , Lymphoma, Non-Hodgkin/drug therapy , Neutropenia/chemically induced , Rituximab/adverse effects , Adult , Aged , Aged, 80 and over , Antineoplastic Agents/therapeutic use , Female , Humans , Male , Middle Aged , Retrospective Studies , Rituximab/therapeutic use
7.
Future Oncol ; 10(12): 1967-80, 2014 Oct.
Article in English | MEDLINE | ID: mdl-25386813

ABSTRACT

The prognosis of follicular lymphoma (FL) has significantly improved over the last decade, particularly following the introduction of the anti-CD20 monoclonal antibody rituximab, which has challenged the old concept of FL as an incurable disease. However, the decision whether to start treatment in a patient with advanced FL or adopt a watch-and-wait policy remains a subject of controversy. Furthermore, the optimal first-line treatment for FL remains a clinical challenge owing to the numerous different therapeutic options available. In this review, the authors focus on the initial management of patients with newly diagnosed FL, consider the different treatment options for every stage, paying special consideration to the therapeutic approaches for each clinical scenario, and discuss future directions.


Subject(s)
Lymphoma, Follicular/therapy , Clinical Trials as Topic , Humans
8.
Hematol Oncol ; 32(3): 139-44, 2014 Sep.
Article in English | MEDLINE | ID: mdl-24123108

ABSTRACT

There is no standard treatment for patients with gastric marginal zone B-cell lymphoma of the mucosa-associated lymphoid tissue (MALT lymphoma) who are resistant to, or ineligible for, anti-Helicobacter pylori (anti-HP) therapy. In this study, we investigated the activity of the rituximab, cyclophosphamide, vincristine and prednisone (R-CVP) regimen in patients with gastric MALT lymphoma. Patients were included provided they had untreated gastric MALT lymphoma (except for anti-HP therapy) and were resistant to, or ineligible for, anti-HP therapy. Treatment plan consisted of six to eight 21-day cycles of the R-CVP chemotherapy regimen. Toxicity, response, relapse and survival were evaluated. Twenty patients (12 women and 8 men) were included in the analyses with median age of 59 years. Thirteen patients (65%) had stage I tumours, and seven patients (35%) had stages II-IV tumours. The overall response rate was 100%, with 19 (95%) complete responses and one (5%) partial response. Regimen toxicity was mild and mainly hematological, and no cases of gastric bleeding or perforation occurred. After a median follow-up of 56.3 months, three patients had relapsed, and 19 patients remained alive (specific lymphoma survival 100%), of whom 17 had no evidence of disease. In our experience, the R-CVP regimen is a well-tolerated and effective treatment for patients with gastric MALT lymphoma who are resistant to, or ineligible for, anti-HP therapy.


Subject(s)
Antineoplastic Combined Chemotherapy Protocols/therapeutic use , Lymphoma, B-Cell, Marginal Zone/drug therapy , Stomach Neoplasms/drug therapy , Adult , Aged , Aged, 80 and over , Antibodies, Monoclonal, Murine-Derived/administration & dosage , Cyclophosphamide/administration & dosage , Female , Follow-Up Studies , Humans , Lymphoma, B-Cell, Marginal Zone/mortality , Lymphoma, B-Cell, Marginal Zone/pathology , Male , Middle Aged , Neoplasm Staging , Prednisone/administration & dosage , Retrospective Studies , Rituximab , Stomach Neoplasms/mortality , Stomach Neoplasms/pathology , Treatment Outcome , Vincristine/administration & dosage
9.
Clin Transl Oncol ; 11(9): 604-8, 2009 Sep.
Article in English | MEDLINE | ID: mdl-19776000

ABSTRACT

BACKGROUND: The results of CHOP-21 (cyclophosphamide, doxorubicin, vincristine and prednisone given every 21 days) for the treatment of aggressive B-cell lymphoma have recently been improved by the addition of rituximab and by increasing the dose density. R-CHOP-14 combines these two approaches. PATIENTS AND METHODS: We present our experience with R-CHOP-14 in a retrospective single-centre review of 50 patients consecutively treated for aggressive B-cell lymphoma. RESULTS: The median age was 59 years and 48% of patients were >60 years. Stage III-IV was present in 62% of the patients and international prognostic index was high-to-intermediate risk or high risk in 32% of the patients. Toxicity was mainly haematological, with grade 3-4 neutropenia observed in 32% and febrile neutropenia in 18%. Other relevant toxicities were peripheral neuropathy in 45% (grade 3 in 4%) and cardiac dysfunction grade 3 in 7.5%. After therapy, 82% of the patients achieved complete response or unproved complete response. With a median follow-up of 30 months, 3-year event-free survival and overall survival were 67% and 82% respectively. CONCLUSIONS: In our experience the combination of RCHOP- 14 is highly effective in patients with aggressive B-cell lymphoma. However special attention must be paid to the control of early and late toxicities.


Subject(s)
Antibodies, Monoclonal/administration & dosage , Antineoplastic Combined Chemotherapy Protocols/administration & dosage , Lymphoma, B-Cell/therapy , Adult , Aged , Antibodies, Monoclonal/adverse effects , Antibodies, Monoclonal, Murine-Derived , Antineoplastic Combined Chemotherapy Protocols/adverse effects , Combined Modality Therapy , Cyclophosphamide/administration & dosage , Cyclophosphamide/adverse effects , Dose-Response Relationship, Drug , Doxorubicin/administration & dosage , Doxorubicin/adverse effects , Drug Administration Schedule , Female , Humans , Immunotherapy/methods , Lymphoma, B-Cell/mortality , Lymphoma, B-Cell/pathology , Male , Middle Aged , Neoplasm Invasiveness , Prednisone/administration & dosage , Prednisone/adverse effects , Radiotherapy, Adjuvant , Retrospective Studies , Rituximab , Survival Analysis , Treatment Outcome , Vincristine/administration & dosage , Vincristine/adverse effects
10.
Breast J ; 13(2): 189-91, 2007.
Article in English | MEDLINE | ID: mdl-17319862

ABSTRACT

Nonislet cell tumor hypoglycemia (NICTH) is uncommon, and has been related to the production of insulin-like growth factor II or its high-molecular-weight precursor (big IGF-II) by the tumor. Cases of NICTH have been related to different tumors, mainly sarcomas. However, only three cases secondary to a phyllodes tumor of the breast (PTB) have been previously reported. We present the clinical course of a woman with a giant PTB, who developed an irreversible hypoglycemic coma secondary to big IGF-II production by the tumor. A brief review of the literature and possible mechanisms of this infrequent condition are also commented.


Subject(s)
Breast Neoplasms/metabolism , Coma/etiology , Hypoglycemia/etiology , Insulin-Like Growth Factor II/metabolism , Phyllodes Tumor/metabolism , Protein Precursors/metabolism , Fatal Outcome , Female , Humans , Middle Aged
12.
Clin Transl Oncol ; 8(6): 450-2, 2006 Jun.
Article in English | MEDLINE | ID: mdl-16790399

ABSTRACT

Extra-nodal Hodgkin's lymphoma (HL) represents 15% of all Hodgkin's lymphomas; the primary intestinal site accounts for 1% and with involvement of the ascending colon being rare. We present the case of a patient of 62 years of age diagnosed as having acute appendicitis. Anatomopathology on the excised appendectomy tissue indicated nodular lymphocytic predominant Hodgkin's lymphoma (NLPHL). The morphology indicated isolated L&H (lymphocytic or histiocytic) cells or in groups, surrounded by T lymphocytes, in an environment of germinal centres together with phenomena that would be interpreted as progressive transformation. Immunohistochemistry staining of the HL cells were positive for CD45, CD20, Bc16, EMA and MUM1 and negative for CD15 and CD30. No complementary treatment was administered. Following a literature search, the present case would appear to be the first of its kind.


Subject(s)
Appendicitis/diagnosis , Cecal Neoplasms/diagnosis , Diagnostic Errors , Hodgkin Disease/diagnosis , Appendectomy , Biomarkers, Tumor/analysis , Cecal Neoplasms/pathology , Cecal Neoplasms/surgery , Diagnosis, Differential , Germinal Center/pathology , Hodgkin Disease/pathology , Hodgkin Disease/surgery , Humans , Male , Middle Aged
13.
Eur J Dermatol ; 16(3): 290-2, 2006.
Article in English | MEDLINE | ID: mdl-16709497

ABSTRACT

Angiosarcoma is a rare malignant tumor, with a predilection for skin in the head and neck region, although it has been described in many other locations. Its association with chronic lymphedema is well known, mainly in the setting of postmastectomy lymphedema of the arm in breast cancer patients (termed Stewart-Treves syndrome). However, angiosarcoma can appear in lower limbs with chronic lymphedema and rarely in other locations such as the abdominal wall. Herein, we present a unique case of angiosarcoma developing in the abdominal wall of a morbidly obese patient after extensive dermolipectomy.


Subject(s)
Abdominal Wall/pathology , Dermatologic Surgical Procedures , Hemangiosarcoma/diagnosis , Lipectomy/methods , Obesity, Morbid/complications , Hemangiosarcoma/complications , Hemangiosarcoma/diagnostic imaging , Humans , Immunohistochemistry , Male , Middle Aged , Obesity, Morbid/diagnostic imaging , Obesity, Morbid/surgery , Tomography, X-Ray Computed
14.
J Cancer Res Clin Oncol ; 132(5): 332-8, 2006 May.
Article in English | MEDLINE | ID: mdl-16435143

ABSTRACT

PURPOSE: To assess the efficacy and the toxicity of a new combination of epirubicin, cyclophosphamide and paclitaxel as neoadjuvant chemotherapy for breast cancer. METHODS: Patients with stage II and III breast cancer received 3-4 cycles of epirubicin 75 mg/m(2) plus cyclophosphamide 600 mg/m(2) on day 1, and paclitaxel at a dose of 100 mg on days 1, 8, 15 and 22 on a 28-day cycle. RESULTS: Forty-nine patients were enrolled in the study. Stage II was present in 16 patients (32.7%) and stage III in 33 patients (67.3%). Relevant toxicities were nausea/vomiting grades III-IV in 6 patients (12.2%) and neutropenia grade III-IV in 33 patients (67.3%). The overall clinical response rate was 83.7%. Partial response was observed in 25 patients (51%), complete response in 16 patients (32.7%), stable disease in 7 patients (14.3%) and progression in 1 patient. Thirty-three non-inflammatory breast cancer patients underwent surgery, 29 with breast-conserving surgery (87.9%). Pathological complete response was found in 5 patients (15.1%). CONCLUSIONS: The combination of epirubicin, cyclophosphamide and weekly paclitaxel as given in this study is safe and shows good activity in the neoadjuvant setting of stage II and III breast cancer patients.


Subject(s)
Antineoplastic Combined Chemotherapy Protocols/therapeutic use , Breast Neoplasms/drug therapy , Carcinoma/drug therapy , Neoadjuvant Therapy , Adult , Aged , Antineoplastic Combined Chemotherapy Protocols/adverse effects , Breast Neoplasms/pathology , Breast Neoplasms/surgery , Carcinoma/pathology , Carcinoma/surgery , Cyclophosphamide/administration & dosage , Cyclophosphamide/adverse effects , Drug Administration Schedule , Epirubicin/administration & dosage , Epirubicin/adverse effects , Female , Humans , Infusions, Intravenous , Mastectomy, Segmental , Middle Aged , Paclitaxel/administration & dosage , Paclitaxel/adverse effects , Treatment Outcome
15.
Clin Transl Oncol ; 7(10): 464-7, 2005 Nov.
Article in English | MEDLINE | ID: mdl-16373056

ABSTRACT

Primitive neuroectodermal tumours (PNET) are aggressive neoplasias that are diagnosed, usually, in infancy. Their appearance in adulthood is rare and, exceptionally, in association with neurofibromatosis type I (NF-1). We present a case of a 37 year-old man with NF-1 combined with PNET in the intra-arachidial lumbar region. Diagnosis was by Nuclear Magnetic Resonance (NMR) and biopsy of soft tissue mass which showed a PNET with undifferentiated round cells and immunohistochemically positive for CD99, neurone-specific enolase, synaptophys in and LEU-7. Surgery was performed with spine decompression and resection of 80% of the tumour, with symptoms improvement. Radiotherapy was administered on the lumbosacral column, but only up to 30 Gy because of severe actinic enteritis and pan-cytopenia grade III. Six months later, the patient was hospitalized with deterioration in his overall clinical status with multi-organ involvement. The patient died and an autopsy was performed. The initial treatment of the PNET is surgery and, if possible, the radical extirpation of the tumour. Administration of radiotherapy and chemotherapy appears to increase survival. We comment on the clinical, histological, cytological and immunohistochemical aspects together with a review of the literature. To the best of our knowledge this is the first documentation of such a case.


Subject(s)
Neoplasms, Multiple Primary/diagnosis , Neuroectodermal Tumors, Primitive/diagnosis , Neurofibromatosis 1/diagnosis , Spinal Cord Neoplasms/diagnosis , Adult , Humans , Lumbar Vertebrae , Male
16.
Clin. transl. oncol. (Print) ; 7(10): 464-467, nov. 2005. ilus
Article in En | IBECS | ID: ibc-040806

ABSTRACT

Primitive neuroectodermal tumours (PNET) are aggressive neoplasias that are diagnosed, usually, in infancy. Their appearance in adulthood is rare and, exceptionally, in association with neurofibromatosis type I (NF-1). We present a case of a 37 year-old man with NF-1 combined with PNET in the intra-arachidial lumbar region. Diagnosis was by Nuclear Magnetic Resonance (NMR) and biopsy of soft tissue mass which showed a PNET with undifferentiated round cells and immunohistochemically positive for CD99, neurone-specific enolase, synaptophysin and LEU-7. Surgery was performed with spine decompression and resection of 80% of the tumour, with symptoms improvement. Radiotherapy was administered on the lumbosacral column, but only up to 30 Gy because of severe actinic enteritis and pan-cytopenia grade III. Six months later, the patient was hospitalized with deterioration in his overall clinical status with multi-organ involvement. The patient died and an autopsy was performed. The initial treatment of the PNET is surgery and, if possible, the radical extirpation of the tumour. Administration of radiotherapy and chemotherapy appears to increase survival. We comment on the clinical, histological, cytological and immunohistochemical aspects together with a review of the literature. To the best of our knowledge this is the first documentation of such a case


Subject(s)
Male , Adult , Humans , Neuroectodermal Tumors, Primitive/pathology , Neurofibromatosis 1/complications , Lumbosacral Region/pathology , Neuroectodermal Tumors, Primitive/therapy , Magnetic Resonance Spectroscopy , Biopsy , Phosphopyruvate Hydratase/analysis , Synaptophysin/analysis , Decompression, Surgical , Enteritis/complications , Pancytopenia/complications
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