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BACKGROUND: Cellular myofibroma is a rare subtype of myofibroma that was first described in 2017. Its diagnosis is often challenging because of its relative rarity, lack of known genetic abnormalities, and expression of muscle markers that can be confused with sarcomas that have myogenic differentiation. Currently, scholars have limited knowledge of this disease, and published cases are few. Further accumulation of diagnostic and treatment experiences is required. CASE SUMMARY: A 16-year-old girl experienced left upper limb swelling for 3 years. She sought medical attention at a local hospital 10 months ago, where magnetic resonance imaging revealed a 5-cm soft tissue mass. Needle biopsy performed at a local hospital resulted in the diagnosis of a spindle cell soft tissue sarcoma. The patient was referred to our hospital for limb salvage surgery with endoprosthetic replacement. She was initially diagnosed with a synovial sarcoma. Consequently, clinical management with chemotherapy was continued for the malignant sarcoma. Our pathology department also performed fluorescence in situ hybridization for result validation, which returned negative for SS18 gene breaks, indicating that it was not a synovial sarcoma. Next-generation sequencing was used to identify the SRF-RELA rearrangement. The final pathological diagnosis was a cellular/myofibroblastic neoplasm with an SRF-RELA gene fusion. The patient had initially received two courses of chemotherapy; however, chemotherapy was discontinued after the final diagnosis. CONCLUSION: This case was misdiagnosed because of its rare occurrence, benign biological behavior, and pathological similarity to soft tissue sarcoma.
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BACKGROUND: Angiosarcoma (AS) is a rare and highly aggressive soft tissue disease that most commonly arises in deep soft tissues. There are only a few reported cases of AS involving the ovary and even fewer reports of the underlying molecular abnormalities. Here, we briefly review two cases of primary ovarian AS (oAS) with specific molecular events and immune checkpoints. The clinical features and prognosis of the disease, diagnosis, differential diagnosis, and new treatment approaches are discussed based on a literature review. CASE SUMMARY: Case 1: A 51-year-old female patient was admitted with right lower limb pain for 5 mo, and lower abdominal pain with hematuria for 1 mo. Partial removal of rectus abdominis muscle and fascia, partial hysterectomy, bilateral salpingo-oophorectomy, and inguinal and pelvic lymphadenectomy were performed. Pathology revealed primary oAS. Fluorescence in situ hybridization revealed c-MYC gene amplification. MESNA + ADM + IFO + DTIC (MAID) regimen was administered, but stable disease was achieved. The patient died 1 mo later. Case 2: A 41-year-old female patient presented with fatigue, nausea, decreased appetite, and diffuse abdominal pain. On physical examination, the abdomen was distended and a complex cystic mass was palpable in the right pelvic cavity. Pathology revealed primary oAS. MAID chemotherapy was administered and programmed death ligand 1 (PD-L1) staining was performed on the tumor samples. The patient benefited from anti-PD-1 immunotherapy and is alive without any evidence of disease 27 mo off therapy in follow-up. CONCLUSION: Long-term survival benefit for primary oAS can be achieved by alternative therapeutic strategies using pathological indicators to inform treatment.
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To describe the clincopathological features and evaluate risk factors of post-transplant lymphoproliferative disorder (PTLD) after allogeneic haematopoietic stem cell transplants (allo-HSCT), with comparison between paediatric and adult .Clinicopathological features of 81 cases of PTLD after allo-HSCT were analysed by histopatholgy, immunohistochemistry and in situ hybridisatioin.The cases included 58 males and 23 females with a median age of 26.7 years (range 6-55 years) and the PTLDs developed 1-60 months post-transplant (mean 5.9 months). The histological types indicated 10 cases of non-destructive PTLD, including 4 of plasmacytic hyperplasia, 5 of infectious mononucleosis and 1 of florid follicular hyperplasia. Fifty-six cases were polymorphic PTLD, and 15 were monomorphic PTLD, including thirteen of diffuse large B cell lymphoma, 1 of extranodal nasal type natural killer (NK)/T cell lymphoma and 1 of plasmablastic lymphoma. Foci and sheets of necrosis were observed in 31 cases. The infected ratio of Epstein-Barr virus (EBV) was 91.4%. Some cases were treated by reduction of immunosuppression, antiviral therapy, donor lymphocyte infusion or anti-CD20 monoclonal rituximab. Thirty-three cases died. Compared with that of adult, overall survival of paediatric recipient may be better.The first half year after allo-HSCT is very important for the development of PTLD. Type of PTLD, EBV infection and graft-versus-host disease are risk factors. The prognosis of PTLD is poor, and PTLD after allo-HSCT exhibits some features different from that after solid organ transplantation and some differences existing between adult and paediatric recipients.
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Infecciones por Virus de Epstein-Barr/complicaciones , Trasplante de Células Madre Hematopoyéticas/efectos adversos , Trastornos Linfoproliferativos , Trasplante Homólogo/efectos adversos , Adolescente , Adulto , Células Alogénicas/virología , Antígenos CD20 , Niño , Infecciones por Virus de Epstein-Barr/fisiopatología , Femenino , Herpesvirus Humano 4/aislamiento & purificación , Herpesvirus Humano 4/patogenicidad , Humanos , Trastornos Linfoproliferativos/complicaciones , Trastornos Linfoproliferativos/tratamiento farmacológico , Trastornos Linfoproliferativos/mortalidad , Trastornos Linfoproliferativos/patología , Masculino , Persona de Mediana Edad , Factores de Riesgo , Rituximab/uso terapéuticoRESUMEN
BACKGROUND: To study the effects of splenectomy on treatment and diagnosis of tumours of lymphoid tissue of the spleen. METHODS: Fifty-three cases were reviewed from Peking University People's Hospital from 2002 to 2017. According to WHO classification of tumours of haematopoietic and lymphoid tissues (2008) and classification updated (2016), the cases were studied by microscopy, immunohistochemistry and in situ hybridization, combined with the bone marrow biopsy and clinical examination. RESULTS: In 53 cases, the male to female ratio was 3.4:1, the mean age was 55.4 years old, the median survival time was 17.0 months, and all patients present with variable degree of splenomegaly. The elevated percentage of lymphocyte in peripheral blood can be seen in 22 cases, and elevated LDH level in 24 cases. Abnormal blood counts can be seen in 26 cases before operation, and 22 cases remission to normal level partly or completely after operation. The clinical symptoms included abdominal pain or distension, fatigue, fever, and weight loss, etc. Seventeen cases present with lymphoadenopathy of abdomen or other sites. Fourteen cases were stage I or II, whereas 6 were stage III, 28 were stage IV. Forty-three cases were splenic B-cell marginal zone lymphoma (SMZL)(48.8%,21/43), DLBCL(23.3%,10/43), splenic diffuse red pulp small B-cell lymphoma (SDRPSBL)(11.6%,5/43), mantle cell lymphoma (MCL)(9.3%,4/43), follicular lymphoma (FL)(4.7%,2/43), composite lymphoma (CL, DLBCL and classical Hodgkin lymphoma)(2.3%,1/43) in turn, and the remaining 10 cases were chronic lymphocytic leukaemia/small lymphocytic lymphoma (CLL/SLL) (nâ¯=â¯4), hairy cell leukaemia (HCL) (nâ¯=â¯1), hepatosplenic T-cell lymphoma (HSTL) (nâ¯=â¯5). The survival period of SMZL and DLBCL was 25.7, 18.6 months, respectively. Thirteen cases were dead (27.1%, 13/48). The chemotherapy protocol included Hyper-CVAD A/B with/without R (Rituximab), COP, CHOP with/without R etc. The prognosis of those with elevated LDH level, high clinical staging, B symptom, and older than 60â¯year old was obviously worse, and the prognosis of DLBCL was worse than that of SMZL. CONCLUSIONS: Most splenic lymphoid tumors present with splenomegaly and abnormal blood counts, and complete or part remission of blood counts can be seen after splenectomy, and splenectomy is also helpful for pathological diagnosis. The most common pathological types are SMZL and DLBCL. The definite diagnosis can be made by combining with clinical features, histopathology, immunophenotype, genetics, bone marrow biopsy and laboratory examination.
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Tejido Linfoide/patología , Linfoma de Células B de la Zona Marginal/patología , Linfoma Folicular/patología , Linfoma de Células B Grandes Difuso/patología , Neoplasias del Bazo/patología , Adulto , Anciano , Femenino , Humanos , Tejido Linfoide/cirugía , Linfoma de Células B de la Zona Marginal/mortalidad , Linfoma de Células B de la Zona Marginal/cirugía , Linfoma Folicular/mortalidad , Linfoma Folicular/cirugía , Linfoma de Células B Grandes Difuso/mortalidad , Linfoma de Células B Grandes Difuso/cirugía , Masculino , Persona de Mediana Edad , Estudios Retrospectivos , Esplenectomía , Neoplasias del Bazo/mortalidad , Neoplasias del Bazo/cirugía , Tasa de Supervivencia , Resultado del Tratamiento , Adulto JovenRESUMEN
BACKGROUND: Non-small-cell lung cancer (NSCLC) is characterized by abnormalities of numerous signaling proteins that play pivotal roles in cancer development and progression. Many of these proteins have been reported to be correlated with clinical outcomes of NSCLC. However, none of them could provide adequate accuracy of prognosis prediction in clinical application. METHODS: A total of 384 resected NSCLC specimens from two hospitals in Beijing (BJ) and Chongqing (CQ) were collected. Using immunohistochemistry (IHC) staining on stored formalin-fixed paraffin-embedded (FFPE) surgical samples, we examined the expression levels of 75 critical proteins on BJ samples. Random forest algorithm (RFA) and support vector machines (SVM) computation were applied to identify protein signatures on 2/3 randomly assigned BJ samples. The identified signatures were tested on the remaining BJ samples, and were further validated with CQ independent cohort. RESULTS: A 6-protein signature for adenocarcinoma (ADC) and a 5-protein signature for squamous cell carcinoma (SCC) were identified from training sets and tested in testing sets. In independent validation with CQ cohort, patients can also be divided into high- and low-risk groups with significantly different median overall survivals by Kaplan-Meier analysis, both in ADC (31 months vs. 87 months, HR 2.81; P < 0.001) and SCC patients (27 months vs. not reached, HR 9.97; P < 0.001). Cox regression analysis showed that both signatures are independent prognostic indicators and outperformed TNM staging (ADC: adjusted HR 3.07 vs. 2.43, SCC: adjusted HR 7.84 vs. 2.24). Particularly, we found that only the ADC patients in high-risk group significantly benefited from adjuvant chemotherapy (P = 0.018). CONCLUSIONS: Both ADC and SCC protein signatures could effectively stratify the prognosis of NSCLC patients, and may support patient selection for adjuvant chemotherapy.
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Adenocarcinoma/patología , Protocolos de Quimioterapia Combinada Antineoplásica/uso terapéutico , Biomarcadores de Tumor/metabolismo , Carcinoma de Pulmón de Células no Pequeñas/patología , Carcinoma de Células Escamosas/patología , Neoplasias Pulmonares/patología , Adenocarcinoma/tratamiento farmacológico , Adenocarcinoma/metabolismo , Carcinoma de Pulmón de Células no Pequeñas/tratamiento farmacológico , Carcinoma de Pulmón de Células no Pequeñas/metabolismo , Carcinoma de Células Escamosas/tratamiento farmacológico , Carcinoma de Células Escamosas/metabolismo , Femenino , Estudios de Seguimiento , Perfilación de la Expresión Génica , Regulación Neoplásica de la Expresión Génica , Humanos , Neoplasias Pulmonares/tratamiento farmacológico , Neoplasias Pulmonares/metabolismo , Masculino , Persona de Mediana Edad , Pronóstico , Estudios Prospectivos , Transducción de Señal , Tasa de Supervivencia , Análisis de Matrices TisularesAsunto(s)
Disgenesia Gonadal 46 XY/diagnóstico , Gonadoblastoma/diagnóstico , Neoplasias de Células Germinales y Embrionarias/diagnóstico , Neoplasias Ováricas/diagnóstico , Adolescente , Quimioterapia Adyuvante , Femenino , Disgenesia Gonadal 46 XY/tratamiento farmacológico , Disgenesia Gonadal 46 XY/metabolismo , Gonadoblastoma/tratamiento farmacológico , Gonadoblastoma/metabolismo , Humanos , Neoplasias de Células Germinales y Embrionarias/tratamiento farmacológico , Neoplasias de Células Germinales y Embrionarias/metabolismo , Neoplasias Ováricas/tratamiento farmacológico , Neoplasias Ováricas/metabolismoRESUMEN
OBJECTIVE: To explore the viral etiology of human breast cancer to determine whether there are novel molecular targets for gene therapy of breast cancer and provide evidence for the research of gene therapy and vaccine development for breast cancer. METHODS: PCR was used to screen HPV16 and HPV18 oncogenes E6 and E7 in the SKBR3 cell line and in 76 paraffin embedded breast cancer tissue samples. RNA interference was used to knock down the expression of HPV18 E6 and E7 in SKBR3 cells, then the changes in the expression of cell-cycle related proteins, cell viability, colony formation, metastasis, and cell cycle progression were determined. RESULTS: HPV18 oncogenes E6 and E7 were amplified and sequenced from the SKBR3 cells. Of the patient samples, 6.58% and 23.68% were tested to be positive for HPV18 E6 and HPV18 E7. In the cell culture models, the knockdown of HPV18 E6 and E7 inhibited the proliferation, metastasis, and cell cycle progression of SKBR3 cell. The knockdown also clearly affected the expression levels of cell cycle related proteins. CONCLUSION: HPV was a contributor to virus caused human breast cancer, suggesting that the oncogenes in HPV were potential targets for gene therapy of breast cancer.
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Neoplasias de la Mama/terapia , Terapia Genética/métodos , Proteínas Oncogénicas Virales/genética , Papillomaviridae/fisiología , Infecciones por Papillomavirus/terapia , Adulto , Anciano , Anciano de 80 o más Años , Secuencia de Bases , Neoplasias de la Mama/genética , Femenino , Humanos , Persona de Mediana Edad , Proteínas Oncogénicas Virales/metabolismo , Infecciones por Papillomavirus/genética , Alineación de SecuenciaAsunto(s)
Adenomiosis/patología , Hiperplasia/patología , Adenomiosis/metabolismo , Adulto , Antígeno Ca-125/metabolismo , Hiperplasia Endometrial/metabolismo , Hiperplasia Endometrial/patología , Neoplasias Endometriales/metabolismo , Neoplasias Endometriales/patología , Endometriosis/metabolismo , Endometriosis/patología , Femenino , Humanos , Hiperplasia/metabolismo , Persona de Mediana EdadRESUMEN
OBJECTIVE: To investigate the correlation between age and Gleason score (GS) in patients with prostate adenocarcinoma. METHODS: This study included 674 patients with pathologically confirmed prostate adenocarcinoma. We determined the GS, primary grade, and secondary grade of the cases, and compared them among different age groups. RESULTS: The mean age of the patients was (70.22 ± 8.26) yr, ranging from 25 to 96 years, (69.06 ± 8.35) yr in those with GS 6, (70.55 ± 8.16) yr in GS 7, (70.99 ± 6.54) yr in GS 8, (71.56 ± 9.18) yr in GS 9, and (72.79 ± 11.36) in GS 10. The mean GS, primary grade, and secondary grade were 7.08 ± 1.09, 3.54 ± 0.72, and 3.53 ± 0.66, respectively, and the mean GSs in the < 60 yr, 60-69 yr, 70-79 yr, and ≥ 80 yr groups were 6.86 ± 1.10, 6.99 ± 1.10, 7.08 ± 1.04, and 7.38 ± 1.23, respectively. Those with GS 6, 7, and ≥ 8 accounted for 37.7%, 34.3%, and 28.0%, respectively. The patients aged < 60, 60-69, 70-79, and ≥ 80 years constituted 10.5% (71/674), 30.6% (206/674), 47.6% (321/674), and 11.3% (76/674), respectively. The age of the patients was significantly correlated with GS (r2 = 0.013, P = 0.003) and the primary grade (r2 = 0.014, P = 0.002), but not the secondary grade (r2 = 0.005, P = 0.055). CONCLUSION: Of the prostate adenocarcinoma patients, those aged ≥ 70 years form a larger proportion, and those with GS ≥ 7 comprise a higher percentage. The age of the patient is correlated with Gleason score but has a limited value in its prediction.
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Adenocarcinoma/patología , Factores de Edad , Neoplasias de la Próstata/patología , Adulto , Anciano , Anciano de 80 o más Años , Humanos , Masculino , Persona de Mediana Edad , Clasificación del TumorRESUMEN
OBJECTIVE: To explore the basic features of the prostate carcinoma Gleason grading system of the International Society of Urological Pathology (ISUP). METHODS: We analyzed the means and proportions of the Gleason score (GS), primary grade (PG), secondary grade (SG) and third grade (TG) of 667 cases of prostate carcinoma. RESULTS: The means of GS, PG, SG and TG were 7.06 +/- 1.10, 3.53 +/- 0.66, 3.53 +/- 0.72 and 4.30 +/- 0.96, respectively. The cases with GS 5, 6, 7, 8, 9 and 10 accounted for 0.4% (3/677), 37.2% (252/677), 34.4% (233/677), 13.7% (93/677), 12.0% (81/677) and 2.2% (15/677), respectively; those with GS < or = 6 and > or = 7 constituted 37.7% (255/677) and 62.3% (422/677); those with GS3 + 3, 4 + 3 and 3 + 4 made up 37.2% (252/677), 19.2% (130/677) and 15.2% (103/677); and the TG cases held 10.3% (70/677), including 30.0% (21/70) of grade 3, 10% (7/70) of grade 4 and 60.0% (42/70) of grade 5. CONCLUSION: Our study showed a high proportion of GS, a low proportion of GS < or = 6, and a high proportion of GS > or = 7 in the ISUP prostate carcinoma Gleason grading system. TG of GS needs to be further understood.
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Clasificación del Tumor/métodos , Neoplasias de la Próstata/patología , Humanos , MasculinoAsunto(s)
Adenoma/patología , Carcinoma Basocelular/patología , Próstata/patología , Hiperplasia Prostática/patología , Neoplasias de la Próstata/patología , Adenoma/metabolismo , Biomarcadores/metabolismo , Biomarcadores de Tumor/metabolismo , Carcinoma Basocelular/metabolismo , Diagnóstico Diferencial , Humanos , Hiperplasia , Inmunohistoquímica , Masculino , Próstata/metabolismo , Hiperplasia Prostática/metabolismo , Neoplasias de la Próstata/metabolismoRESUMEN
The prognosis of gastric cancer (GC) is associated with Cdx2 and nuclear PTEN coexpression. This study aimed to determine the expression patterns of Cdx2 and PTEN in various GC tissues and cell lines to identify their relationship in GC. Immunohistochemistry was undertaken to assess the expression patterns of Cdx2 and PTEN in paraffin-embedded specimens of 228 GC patients who had undergone radical D2 gastrostomy with long-term follow-up. Cell growth and tumorigenicity were analyzed in the BGC823 cells with exogenous Cdx2 and any changes in the associated signaling pathways were interpreted in exogenous cdx2 expression and cdx2 knockdown. Cdx2 was found in the nuclei of GC cells in 43.4% (99/228) of the paraffin-embedded biopsies. A higher expression of nuclear PTEN was observed in 36.4% (83/228). Coexpression of Cdx2 and nuclear PTEN was detected in GC tumors (59/228, 25.9%) which correlated with the prognosis of advanced GC patients (p<0.001). The expression levels of Cdx2 and PTEN were variable in the different GC cell lines. However, the trends were similar between PTEN and Cdx2 in GC tissues and cell lines. High expression of Cdx2 and PTEN significantly reduced tumorigenicity in BGC823 cells compared with the empty vector control. Exogenous expression of Cdx2 triggered the upregulation of PTEN expression and decreased PI3K and pAkt expression and vice versa. The coexpression levels of PTEN and Cdx2 in GC tumors correlated with prognosis in GC patients. Cdx2 may play a role in the upregulation of PTEN by triggering PI3K/Akt inactivation in GC cells.
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Proteínas de Homeodominio/genética , Fosfohidrolasa PTEN/genética , Transducción de Señal/genética , Neoplasias Gástricas/genética , Adulto , Anciano , Anciano de 80 o más Años , Factor de Transcripción CDX2 , Proliferación Celular , Femenino , Estudios de Seguimiento , Gastrostomía , Regulación Neoplásica de la Expresión Génica , Proteínas de Homeodominio/metabolismo , Humanos , Masculino , Persona de Mediana Edad , Fosfohidrolasa PTEN/metabolismo , Fosfatidilinositol 3-Quinasas/metabolismo , Proteínas Proto-Oncogénicas c-akt/metabolismo , Neoplasias Gástricas/patologíaRESUMEN
OBJECTIVE: To study the feasibility of endometrial sampling device as a sampling tool during the follow-up visit for endometrial cancer patients undergone conservative treatment. METHODS: Before the hysteroscopy examination, endometrial sampling device was used to take the endometrium specimens 43 times in 19 patients who had been diagnosed as endometrial cancer or atypical hyperplasia, and were undergone conservative treatment during May 2012 to Mar. 2013. All cases accepted vaginal ultrasound screening before every sampling by endometrial sampling device. The histological results were compared with those done by hysteroscopy. RESULTS: The average age of those patients was (30 ± 6) years old. The mean thickness of the endometrium during the treatment was (0.81 ± 0.65) cm. The qualified rate for the sampling was 95% (41/43). Compared with the specimens undergone by hysteroscopy direct sampling, 32 samples got by the endometrial sampling device with thicker endometrium (0.93 ± 0.70) cm had the same histological results, while the other 9 patients with thinner endometrium (0.40 ± 0.14) cm were not (P = 0.031). CONCLUSION: The endometrial sampling device could be used during the follow-up visit for the conservative treatment patients with endometrial cancer or atypical hyperplasia, the vaginal ultrasound screening should be used together to figure out those with thinner endometrium.
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Biopsia/instrumentación , Biopsia/métodos , Neoplasias Endometriales/diagnóstico , Endometrio/patología , Adulto , Hiperplasia Endometrial/diagnóstico , Hiperplasia Endometrial/tratamiento farmacológico , Hiperplasia Endometrial/patología , Neoplasias Endometriales/tratamiento farmacológico , Neoplasias Endometriales/patología , Estudios de Factibilidad , Femenino , Estudios de Seguimiento , Humanos , Histeroscopía/métodos , Persona de Mediana Edad , Lesiones Precancerosas/diagnóstico , Lesiones Precancerosas/tratamiento farmacológico , Lesiones Precancerosas/patología , Progestinas/uso terapéutico , Sensibilidad y Especificidad , Adulto JovenRESUMEN
Post-transplant lymphoproliferative disorders (PTLDs) are serious, life-threatening complications of solid-organ transplantation (SOT) and bone marrow transplantation, and are associated with high mortality. PTLDs represent a heterogeneous group of lymphoproliferative diseases, which show a spectrum of clinical, morphologic, and molecular genetic features ranging from reactive polyclonal lesions to frank lymphomas. We describe clinicopathologic features of 17 cases of PTLD after allogeneic hematopoietic stem cell transplantation (allo-HSCT), which were analyzed by in situ hybridization for EBV and a panel of antibodies directed against numerous antigens, including CD20, PAX5, CD3, bcl-6, CD10, MUM-1/IRF4, CD138, Kappa, Lambda, CD30, CD15, and Ki67. The cases included 13 males and 4 females with a median age of 31 years (range 9-49 years) and the PTLDs developed 1.5-19 months post-transplant (mean 4.7 months). The histological types indicated five cases of early lesions, two of plasmacytic hyperplasia and three of infectious mononucleosis-like PTLD. Eight cases were polymorphic PTLD, and four were monomorphic PTLD, including three of diffuse large B cell lymphoma, and one of plasmablastic lymphoma. Foci and sheets of necrosis were observed in five cases. The infected ratio of EBV was 88.2 %. Some cases were treated by reduction of immunosuppression, antiviral therapy, donor lymphocyte infusion, or anti-CD20 monoclonal rituximab. Eight cases died. The first half year after allo-HSCT is very important for the development of PTLD. The diagnosis of PTLD relies on morphology and immunohistochemistry, and EBV plays an important role in the pathogenesis of PTLD. The prognosis of PTLD is poor, and, notably, PTLD after allo-HSCT exhibits some features different from those of PTLD after SOT.
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Infecciones por Virus de Epstein-Barr/complicaciones , Trasplante de Células Madre Hematopoyéticas/efectos adversos , Trastornos Linfoproliferativos/etiología , Trastornos Linfoproliferativos/patología , Adolescente , Adulto , Niño , Infecciones por Virus de Epstein-Barr/tratamiento farmacológico , Femenino , Estudios de Seguimiento , Humanos , Inmunohistoquímica , Hibridación in Situ , Ganglios Linfáticos/patología , Trastornos Linfoproliferativos/tratamiento farmacológico , Masculino , Persona de Mediana Edad , Membrana Mucosa/patología , Trasplante Homólogo , Adulto JovenRESUMEN
OBJECTIVE: To study the clinical and histopathologic features, diagnosis, pathogenesis and therapy of post-transplant lymphoproliferative disorders (PTLD). METHODS: The clinical and pathologic features of 15 cases of PTLD were retrospectively analyzed by light microscopy, immunohistochemistry and in-situ hybridization, according to the updated 2008 WHO classification of tumors of hematopoietic and lymphoid tissues. RESULTS: Amongst the 15 cases studied, 14 cases had received allogenic hematopoietic stem cell transplantation (AHSCT) and 1 case had received renal transplantation. There were altogether 12 males and 3 females. The male-to-female ratio was 4:1. The mean age was 30.4 years and the median age was 31 years (range from 9 to 60 years). PTLD developed 1.5 to 132 months after transplantation (median 13.0 months). The mean age of the 14 patients with AHSCT was 28.3 years (range from 9 to 45 years) and PTLD developed 1.5 to 19 months after transplantation (mean 4.5 months). Major clinical presentation included fever and lymphadenopathy. Twelve cases involved mainly lymph nodes and the remaining 3 cases involved tonsils, stomach and small intestine, respectively. The histologic types in 4 cases represented early lesions, including plasmacytic hyperplasia (n = 1) and infectious mononucleosis-like PTLD (n = 3). Seven cases were polymorphic PTLD, with 4 cases containing a predominance of large cells. Graft-versus-host disease was also seen in the case of small intestinal involvement. Four cases were monomorphic PTLD, 3 of which were diffuse large B-cell lymphoma, 1 was plasmablastic lymphoma and 1 was a mixture of monomorphic and polymorphic PTLD. Foci of necrosis were seen in 5 cases. The proliferating index of Ki-67 was high. The positive rate of EBV-encoded RNA in AHSCT was 92.9%. The duration of PTLD onset was shorter in EBV-positive cases (range from 1.5 to 7 months) than EBV-negative cases (range from 19 and 132 months). Some cases were treated by reduction of immunosuppression, antiviral agents or anti-CD20 monoclonal antibody Rituximab. The duration of follow-up in 14 patients ranged from 0 to 8 months. Five of the patients died of the disease. CONCLUSIONS: The diagnosis of PTLD relies on morphologic examination and immunohistochemistry. Most of them are of B-cell origin. EBV plays an important role in the pathogenesis of PTLD. The duration of disease onset is shorter in EBV-positive cases. PTLD in AHSCT cases occurs in younger age group, with shorter duration of onset, as compared to solid organ transplantation. The prognosis of PTLD is poor. The modalities of treatment include reduction of immunosuppression, antiviral agents or anti-CD20 monoclonal antibody Rituximab.
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Trasplante de Células Madre Hematopoyéticas/efectos adversos , Trastornos Linfoproliferativos/etiología , Trastornos Linfoproliferativos/patología , ADP-Ribosil Ciclasa 1/metabolismo , Adolescente , Adulto , Anticuerpos Monoclonales de Origen Murino/uso terapéutico , Antígenos CD20/metabolismo , Antineoplásicos/uso terapéutico , Niño , Infecciones por Virus de Epstein-Barr , Femenino , Estudios de Seguimiento , Herpesvirus Humano 4/aislamiento & purificación , Humanos , Inmunosupresores/uso terapéutico , Antígeno Ki-1/metabolismo , Trasplante de Riñón/efectos adversos , Leucemia/terapia , Linfoma de Células B Grandes Difuso/tratamiento farmacológico , Linfoma de Células B Grandes Difuso/etiología , Linfoma de Células B Grandes Difuso/patología , Linfoma de Células B Grandes Difuso/virología , Trastornos Linfoproliferativos/tratamiento farmacológico , Trastornos Linfoproliferativos/virología , Masculino , Persona de Mediana Edad , ARN Viral/metabolismo , Estudios Retrospectivos , Rituximab , Adulto JovenRESUMEN
OBJECTIVE: To study clinical and histopathological features, and diagnosis of mediastinal tumours of haematopoietic and lymphoid tissues (MTHL). METHODS: Forty cases of MTHL were analyzed for clinicopathology by microscopy and immunohistochemical staining and in situ hybridization, according to the updated 2008 WHO classification of tumours of haematopoietic and lymphoid tissues. RESULTS: In 40 cases of MTHL, there were 20 males and 20 females. The ratio of male/female was 1:1. The mean age was 31.8 years and median age was 29 years (range, 12 - 70 years).Superior vena cava syndrome was observed in 28 cases. The specimens of 4 cases were obtained by lumpectomy, whereas 36 cases by biopsy (25 cases by thoracoscopy, 1 by core needle aspiration). Twenty cases lay in anterior mediastinum, and 2 in posterior, 1 in superior, 8 in anterior and superior, 2 in posterior and superior, 2 in anterior and middle, 1 in middle and anterior mediastinum.Frozen section were performed in 28 cases, and 17 cases were diagnosed as tumours of haematopoietic and lymphoid tissues (consistency ratio was 60.7%). Twelve cases were classical Hodgkin lymphomas (cHL) (8 were nodular sclerosis subtype, and 3 were mixed cellarity, 1 was lymphocyte-rich subtype), and 10 were primary mediastinal (thymic) large B cell lymphoma (PMBCL), 10 were precursor lymphocyte neoplasm [8 were T lymphoblastic leukemia/lymphomas (T-LBL), 2 were B-LBL], 1 was MALT lymphoma, 1 was composite lymphoma (PMBCL and cHL), 2 were myeloid sarcomas, 4 were gray zone lymphomas (GZL) (3 had morphology reminiscent of cHL, and 1 of DLBCL, all cases were positive for CD20, PAX5, CD30 and CD15).EBER were detected in 11 cases by in situ hybridization, 2 of which were positive (18.2%), and the 2 positive cases were cHL. CONCLUSIONS: MTHLs occur predominantly in adolescents and young adults, mainly present as superior vena cava syndrome and anterior mediasinal masses. cHL, PMBCL, T-LBL were the most common MTHLs.GZLs mainly occur in young adults, those whose morphology reminiscent of cHL, immunohistochemistry reminiscent of PMBCL, and vice versa. Thoracoscopy, frozen section and a suitable panel of antibodies were practical approaches to MTHL.
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Enfermedad de Hodgkin/patología , Linfoma de Células B/patología , Neoplasias del Mediastino/patología , Leucemia-Linfoma Linfoblástico de Células Precursoras B/patología , Leucemia-Linfoma Linfoblástico de Células T Precursoras/patología , Adolescente , Adulto , Anciano , Antígenos CD20/metabolismo , Niño , Femenino , Estudios de Seguimiento , Enfermedad de Hodgkin/metabolismo , Humanos , Antígeno Ki-1/metabolismo , Antígeno Lewis X/metabolismo , Linfoma de Células B/metabolismo , Linfoma de Células B de la Zona Marginal/metabolismo , Linfoma de Células B de la Zona Marginal/patología , Masculino , Neoplasias del Mediastino/metabolismo , Persona de Mediana Edad , Factor de Transcripción PAX5/metabolismo , Leucemia-Linfoma Linfoblástico de Células Precursoras B/metabolismo , Leucemia-Linfoma Linfoblástico de Células T Precursoras/metabolismo , Estudios Retrospectivos , Síndrome de la Vena Cava Superior/metabolismo , Síndrome de la Vena Cava Superior/patología , Tasa de Supervivencia , Adulto JovenRESUMEN
OBJECTIVES: To study the morphologic changes of fallopian tubal epithelium in patients with ovarian serous epithelial tumors and to explore the relationship between the tubal epithelial changes and tumorigenesis of serous ovarian carcinoma. METHODS: The fallopian tubes in 79 cases of high-grade serous ovarian carcinoma, 12 cases of low-grade serous ovarian carcinoma, 16 cases of serous borderline ovarian tumor and 11 cases of non-ovarian benign tumors were serially examined under light microscope. Immunohistochemical study with EnVision method was used to detect the expression of p53 and bcl-2 protein in the fallopian tubal epithelium in all cases. The occurrences of secretory cell outgrowth (SCOUT), p53 signature, serous tubal intraepithelial carcinoma (STIC) and serous invasive carcinoma were analyzed. RESULTS: SCOUT in tubal epithelium was observed in 60.8% (48/79) of the high-grade serous carcinoma group, 4/12 of the low-grade serous carcinoma group, 3/16 of the serous borderline tumor group and 2/11 of the non-ovarian benign tumor group (P = 0.001). P53 signature, STIC and serous invasive carcinoma occurred only in the fallopian tubal epithelium of patients with high-grade serous ovarian carcinoma, with the positive rates being 29.1% (23/79), 15.2% (12/79) and 44.3% (35/79), respectively. Of the 23 cases with p53 signature, 17 cases had solitary lesion and 6 cases involved more than two sites. A total of 33 p53 signature positive foci were found, with 22 foci located at fimbria and 11 at ampulla. Bcl-2 expression was demonstrated in 90.9% of those foci (30/33). Of the 12 patients with STIC, 7 cases were solitary and 5 cases involved more than two sites. A total of 18 STIC foci were found, with 16 foci located at fimbria and 2 at ampulla. All of them were positive for bcl-2. CONCLUSIONS: SCOUT is found in fallopian tubal epithelium in patients with serous ovarian epithelial tumors, especially high-grade serious carcinoma. On the other hand, p53 signature, STIC and invasive serous carcinoma of tubal epithelium are observed only in patients with high-grade serous ovarian carcinoma, with a predilection of fimbrial involvement. Correlation exists between SCOUT, p53 signature, STIC and high-grade serous ovarian carcinomas. Bcl-2 and p53 immunostaining is helpful for demonstrating such lesions.
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Transformación Celular Neoplásica , Cistadenocarcinoma Seroso/patología , Neoplasias de las Trompas Uterinas/patología , Trompas Uterinas/patología , Neoplasias Ováricas/patología , Adulto , Anciano , Cistadenocarcinoma Seroso/metabolismo , Células Epiteliales/patología , Epitelio/patología , Neoplasias de las Trompas Uterinas/metabolismo , Femenino , Humanos , Inmunohistoquímica , Persona de Mediana Edad , Estadificación de Neoplasias , Neoplasias Ováricas/metabolismo , Lesiones Precancerosas/metabolismo , Lesiones Precancerosas/patología , Proteínas Proto-Oncogénicas c-bcl-2/metabolismo , Proteína p53 Supresora de Tumor/metabolismoRESUMEN
OBJECTIVE: To study the clinicopathologic features and differential diagnosis of perivascular epithelioid cell tumors (PEComas) occurring in the urinary system. METHODS: The clinicopathologic features of 21 cases of PEComa from September 2002 to September 2010 occurring in the urinary system were retrospectively reviewed. Immunohistochemical study for HMB 45, S-100 protein, smooth muscle actin, desmin, Melan A and Ki-67 was carried out. RESULTS: Amongst the 21 cases studied, there were 5 males and 16 females. The age of patients ranged from 16 to 76 years (median = 51.3 years). Twenty cases occurred in the kidney and 1 in the bladder. The predominant histopathologic subtype of renal PEComas was classic type (10/20), followed by epithelioid type (5/20), smooth muscle type (3/20), inflammatory type (1/20) and sclerosing type (1/20). Immunohistochemical study showed that HMB 45 and smooth muscle actin were positive in 95.2% (20/21) and 80.9% (17/21) cases, respectively. Melan A, desmin and S-100 protein were expressed in 71.4% (15/21), 61.9% (13/21) and 33.3% (7/21) cases, respectively. The mean proliferative index was 1.29% (range = 0 to 5%). HMB 45 and Melan A were expressed in all of the 5 cases of epithelioid PEComas, whereas smooth muscle actin and desmin were only expressed in one of them. There was no significant difference between epithelioid PEComas and non-epithelioid PEComas in the expression of HMB 45, Melan A, smooth muscle actin and desmin. Positive staining for HMB 45 and smooth muscle actin was demonstrated in the case of bladder PEComa. CONCLUSIONS: PEComas of the urinary system predominantly affect the kidney. Epithelioid renal PEComas and bladder PEComa are relatively rare and have unique pathologic features. It is necessary to distinguish PEComas from other malignant tumors. Immunohistochemical study for HMB 45, Melan A and smooth muscle actin is helpful for confirmation of diagnosis.
Asunto(s)
Actinas/metabolismo , Neoplasias Renales/patología , Antígeno MART-1/metabolismo , Antígenos Específicos del Melanoma/metabolismo , Neoplasias de Células Epitelioides Perivasculares/patología , Adolescente , Adulto , Anciano , Carcinoma de Células Renales/metabolismo , Carcinoma de Células Renales/patología , Desmina/metabolismo , Diagnóstico Diferencial , Femenino , Tumores del Estroma Gastrointestinal/metabolismo , Tumores del Estroma Gastrointestinal/patología , Humanos , Inmunohistoquímica , Neoplasias Renales/metabolismo , Leiomiosarcoma/metabolismo , Leiomiosarcoma/patología , Masculino , Melanoma/metabolismo , Melanoma/patología , Persona de Mediana Edad , Neoplasias de Células Epitelioides Perivasculares/metabolismo , Proteínas S100/metabolismo , Neoplasias de la Vejiga Urinaria/metabolismo , Neoplasias de la Vejiga Urinaria/patología , Adulto Joven , Antígeno gp100 del MelanomaRESUMEN
OBJECTIVE: To explore the impact of 2009 International Federation of Gynecology and Obstetrics (FIGO) staging system alteration for stage I endometrioid adenocarcinoma on its' prognosis assessing. METHODS: A retrospective study was carried out on 244 cases with endometrial carcinoma admitted in Peking University People's Hospital from Jan.1995 to Feb.2008. RESULTS: (1) All 244 patients were divided into FIGO 2009 Ia group (n = 200) and FIGO 2009 Ib group (n = 44) according to FIGO 2009 staging system, while they were divided into FIGO 1988 Ia group (n = 34), FIGO 1988 Ib group (n = 156) and FIGO 1988 Ic group (n = 29). The others 25 cases were stage IIa (n = 16) and stage IIIa with merely positive abdominal cytology (n = 9) according to FIGO 1988 staging system.(2) The higher percentage of low-grade in FIGO 1988 Ia group than that in FIGO 2009 Ia group (P = 0.003). Compared with FIGO 2009 Ia group, the age of the patients, surgery extent, the percentage of lymph node excision and received chemotherapy and radiotherapy, there were no difference in FIGO 1988 Ia and Ib group, respectively (P > 0.05). There were 5.9% (2/34) and 6.7% (10/150) found relapse among FIGO 1988 Ia group and FIGO 1988 Ib group, and there were 2.9% (1/34) and 2.7% (4/150) for the two groups died of carcinoma. Compared with FIGO 2009 Ia group, there were not significant difference [7.5% (13/200) vs. 3.0% (6/200); P > 0.05]. The 5 years and 10 years progression-free survival (PFS) of FIGO 1988 Ia group and Ib group were (97.0 ± 3.0)%, (90.9 ± 6.5)% and (95.3 ± 2.1)%, (90.2 ± 3.6)%, respectively, in which there were not significant difference compared with that in FIGO 2009 Ia group [(96.1 ± 1.6)%, (89.6 ± 3.2)%; P > 0.05]. The 5 years and 10 years overall survival (OS) in FIGO 1988 Ia group and Ib group were 100%, (93.8 ± 6.0)% and (96.9 ± 1.8)%, (95.2 ± 2.5)%, respectively, in which there were did not significant difference with that in FIGO 2009 Ia group [(97.9 ± 1.2)%, (93.4 ± 2.8)%; P > 0.05].(3) There were not significant difference between FIGO 1988 Ic group and FIGO 2009 Ib group (P > 0.05) for the age of the patients, grade, surgery extent, lymph node excision, the percentage of received chemotherapy and radiotherapy. Between FIGO 1988 Ic group and FIGO 2009 Ib group, there were 3.4% (1/29) and 6.8% (3/44) cases found relapse, respectively. And there were 0 and 2.3% (1/44) cases died of carcinoma in the two groups, in which there were not differ much either (P > 0.05). The 5 years and 10 years PFS in FIGO 1988 Ic group were all 100%, while they were 100% and (90.9 ± 6.2)% in FIGO 2009 Ib group. The 5 years and 10 years OS in FIGO 1988 Ic group were all 100%, but were 100% and (95.0 ± 4.9)% in FIGO 2009 Ib group, in which they all did not significantly differ much (P > 0.05). (4) The patients in FIGO 2009 Ia group were younger than those in FIGO 2009 Ib group (P < 0.01). The percentage of low grade in FIGO 2009 Ia group were higher than that in FIGO 2009 Ib group (P = 0.029). The percentages of received chemotherapy and radiotherapy in FIGO 2009 Ia group were lower than that in FIGO 2009 Ib group remarkably (P < 0.01). But there were not significant difference in the uterine excision extent and the percentage of lymph node excision between the two groups (P > 0.05). There were not significantly differ in the relapse rates and the death rates between the FIGO 2009 Ia group and FIGO 2009 Ib group (P > 0.05). There were also not significant difference in PFS and OS between the two groups (P > 0.05). CONCLUSIONS: There were not significant difference in the prognosis between FIGO 2009 stage Ia and FIGO 1988 stage Ia and Ib. There were also not significant difference in the prognosis between FIGO 2009 stage Ia and FIGO 2009 stage Ib, which may be due to received more chemotherapy and radiotherapy in FIGO 2009 stage Ib patients.