Assuntos
Histiocitoma Fibroso Benigno , Histiocitoma Fibroso Maligno , Crizotinibe/uso terapêutico , Proteína de Ligação ao Elemento de Resposta ao AMP Cíclico/metabolismo , Histiocitoma Fibroso Maligno/tratamento farmacológico , Histiocitoma Fibroso Maligno/genética , Histiocitoma Fibroso Maligno/patologia , Humanos , Proteínas de Fusão Oncogênica/genética , Proteínas de Fusão Oncogênica/metabolismo , Proteína EWS de Ligação a RNA/genética , Receptores Proteína Tirosina QuinasesRESUMO
The quality of pathologic assessment of rectal cancer specimens is crucial for treatment efficiency and survival. The Royal College of Pathologists (RCP) recommends evaluating the quality of the pathology report in routine practice using three quality indicators (QIs): the number of lymph nodes (LNs) analyzed (≥ 12), the rate of venous invasion (VI ≥ 30%), and peritoneal involvement (pT4a ≥ 10%). In this study, we evaluated the three QIs of the French national pathology reports and compared them with British guidelines and assessed the influence of neoadjuvant radiochemotherapy on QIs. From January 1 to December 31, 2016, all pathology reports for rectal adenocarcinoma were collected from French departments. Neoadjuvant radiochemotherapy included long-course radiotherapy with concomitant 5-FU-based chemotherapy. A total of 983 rectal cancer pathology reports were evaluated. A median of 15 LNs were analyzed and 81% of centers had ≥ 12 LNs. The rate of VI was 30% and 41% of centers had ≥ 30% VI. The rate of pT4a was 4% and 18% of centers reported ≥ 10% pT4a. None of the centers reached the threshold for the three QIs. All three QIs were lower after radiochemotherapy compared to surgery alone. In conclusion, in French routine practice, the values of two of the three QIs (LNs analyzed and VI) were globally in line with RCP guidelines. However, the rate of pT4a was very low, particularly after radiochemotherapy, suggesting its low value in rectal cancer.
Assuntos
Linfonodos/patologia , Metástase Linfática/patologia , Neoplasias Retais/patologia , Neoplasias Retais/terapia , Idoso , Quimiorradioterapia/métodos , Feminino , França , Humanos , Excisão de Linfonodo/métodos , Masculino , Pessoa de Meia-Idade , Terapia Neoadjuvante/métodos , Estadiamento de Neoplasias , Radioterapia Adjuvante/métodos , Resultado do TratamentoRESUMO
AIM: The study aimed to evaluate the accuracy of imaging for measurement of the length of the ileocolic segment affected by Crohn's disease. METHOD: Fifty-four consecutive patients who underwent resection between 2011 and 2014 for ileocolic Crohn's disease were prospectively studied. All had preoperative MR or CT enterography. Two independent radiologists measured the length of the diseased intestinal segment. The measurements were compared with the length of disease assessed on pathology of the non-fixed surgical specimen. RESULTS: The median preoperative length of the Crohn's disease segment on imaging was 20.5 (2-73) cm and 20 (3-90) cm, as measured by the two radiologists. Interobserver agreement was substantial (κ = 0.69) with a correlation coefficient (r) of 0.82 (P < 0.001). The median length of the Crohn's disease segment on pathological examination was 16.5 (2-75) cm and was closely correlated with the radiological measurement (r = 0.76, P < 0.001). The length of the Crohn's disease segment on imaging was correct to within 5 cm of the value on pathology. It was correct in 30 (55%) patients and was underestimated and overestimated in 6 (11.1%) and 18 (33.3%). A length of disease of less than 20 cm found on imaging in 26 patients was confirmed in 25 (96%) on pathology, whereas a length of more than 20 cm found on imaging in 28 patients was confirmed in 18 (64%) on pathology. The sensitivity, specificity, positive predictive value, negative predictive value and overall accuracy of imaging for predicting a length of less than 20 cm were 71%, 95%, 96%, 64% and 79%. CONCLUSION: Imaging accurately identifies the length of the ileocolic segment of Crohn's disease when it is 20 cm or less on pathological examination. In patients with more extensive disease, imaging tends to overestimate the length and should be interpreted with caution.
Assuntos
Colo/diagnóstico por imagem , Doença de Crohn/diagnóstico por imagem , Íleo/diagnóstico por imagem , Imageamento por Ressonância Magnética/estatística & dados numéricos , Tomografia Computadorizada por Raios X/estatística & dados numéricos , Adulto , Idoso , Idoso de 80 Anos ou mais , Colo/patologia , Doença de Crohn/patologia , Feminino , Humanos , Íleo/patologia , Imageamento por Ressonância Magnética/métodos , Masculino , Pessoa de Meia-Idade , Variações Dependentes do Observador , Valor Preditivo dos Testes , Estudos Prospectivos , Sensibilidade e Especificidade , Tomografia Computadorizada por Raios X/métodos , Adulto JovemRESUMO
AIM: The study evaluated the outcome of severe acute antipsychotic (neuroleptic) drug related colitis requiring emergency surgery. METHOD: From 2009 to 2014, 20 patients underwent emergency surgery for acute and severe neuroleptic-related ischaemic colitis. Neuroleptic-induced colitis was defined as another cause besides inflammatory, infectious or ischaemic colitis with a relationship to treatment by antipsychotic drugs. RESULTS: The main drugs involved were cyamemazine (n = 9, 45%), loxapine (n = 5, 25%), haloperidol (n = 4, 20%) and alimemazine (n = 4, 20%). Most (n = 14, 70%) patients presented with haemodynamic instability requiring massive resuscitation and vasopressive drugs. CT signs of digestive impairment were found in 13 (65%) patients having emergency surgery. The lesions were pancolonic in 40%; transparietal necrosis was found in 45% and 15% had colonic perforation. Twelve (60%) patients had total or subtotal colectomy and eight (40%) a segmental colectomy with colostomy or ileostomy in all cases. The postoperative mortality was 15% and morbidity was 70%, necessitating surgical reintervention in two (10%) patients. Of the 17 surviving patients, 11 (64.7%) had restoration of intestinal continuity after a median delay of 103 days, with a postoperative morbidity rate of 36.3%. In the intent-to-treat population, the permanent stoma rate was 30%. CONCLUSION: The morbidity and mortality of surgery for neuroleptic-drug-induced colitis is higher than for colitis due to other causes. A better knowledge of this condition should lead to early diagnosis.
Assuntos
Antipsicóticos/efeitos adversos , Colite Isquêmica/cirurgia , Colostomia/estatística & dados numéricos , Tratamento de Emergência/métodos , Ileostomia/estatística & dados numéricos , Adulto , Idoso , Idoso de 80 Anos ou mais , Colectomia/métodos , Colectomia/estatística & dados numéricos , Colite Isquêmica/induzido quimicamente , Colite Isquêmica/mortalidade , Colostomia/métodos , Feminino , Humanos , Ileostomia/métodos , Análise de Intenção de Tratamento , Masculino , Pessoa de Meia-Idade , Complicações Pós-Operatórias/epidemiologia , Complicações Pós-Operatórias/etiologia , Estudos Prospectivos , Resultado do TratamentoRESUMO
AIM: Pathological response to chemotherapy without pelvic irradiation is not well defined in rectal cancer. This study aimed to evaluate the objective pathological response to preoperative chemotherapy without pelvic irradiation in middle or low locally advanced rectal cancer (LARC). METHODS: Between 2008 and 2013, 22 patients with middle or low LARC (T3/4 and/or N+ and circumferential resection margin < 2 mm) and synchronous metastatic disease or a contraindication to pelvic irradiation underwent rectal resection after preoperative chemotherapy. The pathological response of rectal tumour was analysed according to the Rödel tumour regression grading (TRG) system. Predictive factors of objective pathological response (TRG 2-4) were analysed. RESULTS: All patients underwent rectal surgery after a median of six cycles of preoperative chemotherapy. Of these, 20 (91%) had sphincter saving surgery and an R0 resection. Twelve (55%) patients had an objective pathological response (TRG 2-4), including one complete response. Poor response (TRG 0-1) to chemotherapy was noted in 10 (45%) patients. In univariate analyses, none of the factors examined was found to be predictive of an objective pathological response to chemotherapy. At a median follow-up of 37.2 months, none of the 22 patients experienced local recurrence. Of the 19 patients with Stage IV rectal cancer, 15 (79%) had liver surgery with curative intent. CONCLUSION: Preoperative chemotherapy without pelvic irradiation is associated with objective pathological response and adequate local control in selected patients with LARC. Further prospective controlled studies will address the question of whether it can be used as a valuable alternative to radiochemotherapy in LARC.
Assuntos
Protocolos de Quimioterapia Combinada Antineoplásica/uso terapêutico , Neoplasias Retais/tratamento farmacológico , Reto/cirurgia , Adulto , Canal Anal/cirurgia , Protocolos de Quimioterapia Combinada Antineoplásica/administração & dosagem , Bevacizumab/administração & dosagem , Camptotecina/administração & dosagem , Camptotecina/análogos & derivados , Quimiorradioterapia , Quimioterapia Adjuvante/métodos , Feminino , Fluoruracila/administração & dosagem , Humanos , Leucovorina/administração & dosagem , Masculino , Pessoa de Meia-Idade , Gradação de Tumores , Estadiamento de Neoplasias , Tratamentos com Preservação do Órgão , Compostos Organoplatínicos/administração & dosagem , Pelve , Neoplasias Retais/patologia , Neoplasias Retais/cirurgia , Estudos RetrospectivosRESUMO
BACKGROUND: Deletions at 13q14.3 are common in chronic lymphocytic leukemia and are also present in diffuse large B-cell lymphomas (DLBCL) but never in immunodeficiency-related DLBCL. To characterize DLBCL with 13q14.3 deletions, we combined genome-wide DNA profiling, gene expression and clinical data in a large DLBCL series treated with rituximab, cyclophosphamide, doxorubicine, vincristine and prednisone repeated every 21 days (R-CHOP21). PATIENTS AND METHODS: Affymetrix GeneChip Human Mapping 250K NspI and U133 plus 2.0 gene were used. MicroRNA (miRNA) expression was studied were by real-time PCR. Median follow-up of patients was 4.9 years. RESULTS: Deletions at 13q14.3, comprising DLEU2/MIR15A/MIR16, occurred in 22/166 (13%) cases. The deletion was wider, including also RB1, in 19/22 cases. Samples with del(13q14.3) had concomitant specific aberrations. No reduced MIR15A/MIR16 expression was observed, but 172 transcripts were significantly differential expressed. Among the deregulated genes, there were RB1 and FAS, both commonly deleted at genomic level. No differences in outcome were observed in patients treated with R-CHOP21. CONCLUSIONS: Cases with 13q14.3 deletions appear as group of DLBCL characterized by common genetic and biologic features. Deletions at 13q14.3 might contribute to DLBCL pathogenesis by two mechanisms: deregulating the cell cycle control mainly due RB1 loss and contributing to immune escape, due to FAS down-regulation.
Assuntos
Deleção Cromossômica , Cromossomos Humanos Par 13/genética , Perfilação da Expressão Gênica , Linfoma Difuso de Grandes Células B/genética , Adulto , Idoso , Idoso de 80 Anos ou mais , Protocolos de Quimioterapia Combinada Antineoplásica/uso terapêutico , Feminino , Humanos , Imuno-Histoquímica , Estimativa de Kaplan-Meier , Linfoma Difuso de Grandes Células B/tratamento farmacológico , Linfoma Difuso de Grandes Células B/mortalidade , Masculino , Pessoa de Meia-Idade , Análise de Sequência com Séries de Oligonucleotídeos , Reação em Cadeia da Polimerase em Tempo RealRESUMO
We report a case of a poorly differentiated endocrine large cell carcinoma of the extrahepatic bile ducts in a 73-year-old man, revealed by abdominal pain, jaundice and weight loss. Computed tomography and endoscopic retrograde cholangiography found tumoral stenosis of the main bile duct. Brush cytology detected tumor cells. Pathological examination of the resected bile duct disclosed a high-grade large cell carcinoma with morphological endocrine features and positivity for chromogranin A. This tumor was associated with a minor component of adenocarcinomatous cells. Despite polychemotherapy, the patient had widely metastatic disease a few months later. We discuss here the histogenesis of this tumor as well as its nosological position among the endocrine and mixed tumors of bile ducts.
Assuntos
Neoplasias dos Ductos Biliares , Ductos Biliares Extra-Hepáticos , Carcinoma , Idoso , Neoplasias dos Ductos Biliares/diagnóstico , Neoplasias dos Ductos Biliares/cirurgia , Carcinoma/diagnóstico , Carcinoma/cirurgia , Humanos , MasculinoRESUMO
We report a second observation of autoimmune myelofibrosis associated with an inflammatory myositis in a 30-year-old female. The links between myelofibrosis and autoimmunity are discussed.
Assuntos
Doenças Autoimunes , Dermatomiosite/complicações , Mielofibrose Primária/complicações , Transtornos Puerperais , Adulto , Anti-Inflamatórios/administração & dosagem , Anti-Inflamatórios/uso terapêutico , Biópsia , Fármacos Dermatológicos/administração & dosagem , Fármacos Dermatológicos/uso terapêutico , Dermatomiosite/diagnóstico , Dermatomiosite/tratamento farmacológico , Dermatomiosite/patologia , Quimioterapia Combinada , Feminino , Seguimentos , Glucocorticoides/administração & dosagem , Glucocorticoides/uso terapêutico , Humanos , Ácido Micofenólico/administração & dosagem , Ácido Micofenólico/análogos & derivados , Ácido Micofenólico/uso terapêutico , Prednisona/administração & dosagem , Prednisona/uso terapêutico , Mielofibrose Primária/imunologia , Fatores de TempoRESUMO
Symptomatic nervous system leukemic infiltration is rarely observed in CLL. Various clinical manifestations including headache, confusion, cranial nerve palsies, focal central deficits and peripheral neuropathies have been seldom reported, occurring in less than 1% of patients. We report herein 2 CLL patients with unusual clinical presentations of nervous system invasion. They presented multiple progressive peripheral deficits due to meningoradiculitis. In both, CSF immunophenotyping analysis identified a majority of T cells (>90%), and less than 10% of B-CLL cells expressing CD5, CD19 and CD20. Our analyses revealed the transformation of CLL into an aggressive B-cell lymphoma in one case (Richter's syndrome). A post mortem study showed massive infiltration of cranial nerves and spinal roots by large B lymphomatous cells. In the other case, CNS oriented chemotherapy led to remission and total neurological recovery. In practice, the etiological diagnosis of neurological deficits in CLL patients is difficult. CSF analysis may be useful, requiring viral PCR, repeated cytological studies and immunophenotyping analysis. Although rare, leptomeningeal leukemic localization has to be discussed, even in the absence of overt Richter syndrome, and may require an early therapeutic test.
Assuntos
Leucemia Linfocítica Crônica de Células B/complicações , Leucemia Linfocítica Crônica de Células B/patologia , Meningite/etiologia , Meningite/patologia , Radiculopatia/etiologia , Radiculopatia/patologia , Idoso , Idoso de 80 Anos ou mais , Nervos Cranianos/patologia , Evolução Fatal , Humanos , Infiltração Leucêmica/patologia , Masculino , Raízes Nervosas Espinhais/patologiaAssuntos
Neoplasias da Vesícula Biliar/diagnóstico , Tumores de Células Gigantes/diagnóstico , Neoplasias de Tecido Fibroso/diagnóstico , Idoso de 80 Anos ou mais , Colágeno/metabolismo , Feminino , Vesícula Biliar/metabolismo , Vesícula Biliar/patologia , Neoplasias da Vesícula Biliar/metabolismo , Neoplasias da Vesícula Biliar/patologia , Tumores de Células Gigantes/metabolismo , Tumores de Células Gigantes/patologia , Humanos , Neoplasias de Tecido Fibroso/metabolismo , Neoplasias de Tecido Fibroso/patologiaRESUMO
OBJECTIVES: To study risk factors for incident cervical intraepithelial neoplasia (CIN) among HIV-infected women. PATIENTS AND METHODS: Prospective study of a population of 97 HIV-infected women with normal Pap smear at inclusion. RESULTS: Fourteen CIN (diagnosed by colposcopy and confirmed with biopsy) were observed within a median follow-up of 38 months (13 CIN 1, one CIN 2). The incidence of cervical lesions was estimated to be 2%, 7% and 10% respectively at one year, two and three years after inclusion, The time to occurrence was very variable (ranging from 7 months to 6 years) among our patients. No known risk factors, in particular neither the CD4 cell count nor antiretroviral treatment, were identified to be associated with occurrence of CIN in our study population. CONCLUSION: Regardless of their immune status and HIV treatments, extensive and prolonged gynaecological follow up of HIV-infected women remains necessary.
Assuntos
Infecções por HIV/epidemiologia , Displasia do Colo do Útero/epidemiologia , Neoplasias do Colo do Útero/epidemiologia , Adulto , Feminino , Seguimentos , França/epidemiologia , Humanos , Pessoa de Meia-Idade , Estudos ProspectivosAssuntos
Adenocarcinoma Mucinoso/patologia , Carcinoma de Células Renais/patologia , Neoplasias Renais/patologia , Adenocarcinoma Mucinoso/metabolismo , Carcinoma de Células Renais/metabolismo , Feminino , Humanos , Imuno-Histoquímica , Queratina-7 , Queratinas/análise , Neoplasias Renais/metabolismo , Pessoa de Meia-Idade , Mucina-1/análise , Lectinas de Plantas/análise , Vimentina/análiseAssuntos
Neoplasias das Glândulas Suprarrenais/patologia , Sarcoma de Kaposi/patologia , Adolescente , Neoplasias das Glândulas Suprarrenais/diagnóstico por imagem , Neoplasias das Glândulas Suprarrenais/terapia , Humanos , Masculino , Sarcoma de Kaposi/diagnóstico por imagem , Sarcoma de Kaposi/terapia , Tomografia Computadorizada por Raios XRESUMO
Two genital cases of Ewing's sarcoma/peripheral neuroectodermal tumor in 14 and 15 year old teenagers are reported. They arose in uteri and vulva, as small round cell tumors with some rosette-like formations and intense membranous immunoreactivity for CD99. The EWS/FLI-1 transcript was present in the vulvar tumor. Under chemotherapy, uterine tumor outcome was fatal within 9 months. The vulvar lesion was treated by surgery and chemotherapy, without relapse after 7 months. Likewise to these 2 cases, the literature seems to indicate different prognosis for uterine and vulvar tumors.
Assuntos
Tumores Neuroectodérmicos Primitivos Periféricos/diagnóstico , Sarcoma de Ewing/diagnóstico , Neoplasias Uterinas/diagnóstico , Neoplasias Vulvares/diagnóstico , Antígeno 12E7 , Adolescente , Antígenos CD/análise , Protocolos de Quimioterapia Combinada Antineoplásica/uso terapêutico , Moléculas de Adesão Celular/análise , Evolução Fatal , Feminino , Humanos , Imageamento por Ressonância Magnética , Tumores Neuroectodérmicos Primitivos Periféricos/patologia , Tumores Neuroectodérmicos Primitivos Periféricos/terapia , Sarcoma de Ewing/patologia , Sarcoma de Ewing/terapia , Neoplasias Uterinas/tratamento farmacológico , Neoplasias Uterinas/patologia , Vulva/cirurgia , Neoplasias Vulvares/patologia , Neoplasias Vulvares/terapiaAssuntos
Rabdomiossarcoma/diagnóstico , Neoplasias Uterinas/diagnóstico , Idoso , Diagnóstico Diferencial , Feminino , Humanos , Rabdomiossarcoma/diagnóstico por imagem , Rabdomiossarcoma/patologia , Rabdomiossarcoma/radioterapia , Rabdomiossarcoma/cirurgia , Ultrassonografia , Neoplasias Uterinas/diagnóstico por imagem , Neoplasias Uterinas/patologia , Neoplasias Uterinas/radioterapia , Neoplasias Uterinas/cirurgiaRESUMO
We report the case of a 70-year-old man who presented a bulky gastric mass. The diagnosis of poorly differenciated adenocarcinoma was made on the biopsy. On surgical specimen, the final diagnosis of gastric angiosarcoma with secondary intestinal involvement was established. The patient did not respond to chemotherapy and died 8 months later. The diagnosis was difficult because of epithelioid areas which expressed both cytokeratin and vascular antigens. Human herpesvirus 8 previously detected in some soft tissue angiosarcoma was not found by polymerase chain reaction.