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1.
Cureus ; 16(5): e59684, 2024 May.
Artigo em Inglês | MEDLINE | ID: mdl-38836164

RESUMO

SMARCB1 is a gene known to cause carcinogenesis in many soft tissue tumors, including malignant rhabdoid tumors and epithelioid sarcoma. Since the first report of a subtype of sinonasal carcinoma characterized by a deficiency of the SMARCB1 gene in 2014 to date, fewer than 200 cases have been reported. We report a case of SMARCB1-deficient sinonasal carcinoma with clear cell morphology. In our case, there are no evident basaloid or plasmacytoid/rhabdoid tumor cells, which are typical histopathological features of SMARCB1-deficient sinonasal carcinoma. SMARCB1-deficient sinonasal carcinoma is prone to recurrence and has a very poor prognosis. As the development of molecularly targeted agents progresses, therapeutic efficacy is expected to improve. Simultaneously, the importance of early and accurate diagnosis of SMARCB1-deficient sinonasal carcinoma will increase. With the limited information provided by biopsy specimens, it is necessary to confirm the loss of SMARCB1 expression by immunohistochemistry and investigate the presence of SMARCB1 gene deletion by molecular genetics, considering the possibility of SMARCB1-deficient sinonasal carcinoma even in atypical cases without basaloid or plasmacytoid/rhabdoid cell morphology, as in our case.

2.
Hum Resour Health ; 22(1): 33, 2024 May 27.
Artigo em Inglês | MEDLINE | ID: mdl-38802943

RESUMO

BACKGROUND: Cancer was ranked as the second leading cause of global mortality in 2019, with an increasing incidence. An adequate workforce of healthcare professionals with special skills and knowledge in cancer care is vital for addressing the disparities in cancer prognosis. This study aimed to elucidate the trends in the advanced practice nursing workforce (APNW) in cancer care, which included certified nurse specialists (CNSs) and certified nurses (CNs) in each prefecture of Japan from the system's inception to the present. Further, it sought to analyze the regional disparities and compare these trends with other healthcare resources to identify contributing factors associated with the APNW in cancer care in each prefecture. METHODS: We performed a panel data analysis using publicly available data on the APNW in cancer care in each prefecture of Japan from 1996 to 2022. Gini coefficients were calculated to examine the trends in geographic equality. Univariate and multivariable fixed effect panel data regression models were used to examine regional factors associated with an APNW in cancer care. RESULTS: From 1996 to 2012, the APNW in cancer care increased from four to 6982 staff, while their Gini coefficients decreased from 0.79 to 0.43. However, from 2012 to 2022, the Gini coefficients decreased slightly from 0.43 to 0.41. The coefficient value was comparable to that for the disparity between hospital doctors (0.43) but more pronounced compared to those for other medical resources, such as hospitals (0.34), hospital nurses (0.37), and designated cancer care hospitals (0.29). The APNW in cancer care in each prefecture was significantly associated with a higher number of designed cancer care hospitals in the previous year (see first quartile, the coefficient for second quartile: 0.31, 95% confidence interval (CI) 0.21-0.40), and a fewer number of hospital doctors (- 1.89, 95%CI - 2.70 to - 1.09). CONCLUSIONS: The size of the APNW in cancer care has increased since the system was established in 1996 up till 2022. With the increase in numbers, geographic inequality narrowed until 2012 and has since then remained stagnant.


Assuntos
Prática Avançada de Enfermagem , Disparidades em Assistência à Saúde , Neoplasias , Humanos , Japão , Prática Avançada de Enfermagem/estatística & dados numéricos , Mão de Obra em Saúde/estatística & dados numéricos
3.
Urol Case Rep ; 54: 102738, 2024 May.
Artigo em Inglês | MEDLINE | ID: mdl-38633512

RESUMO

Leiomyomatosis and renal cell carcinoma (HLRCC) are rare autosomal dominant cancer syndromes characterized by cutaneous leiomyoma, uterine leiomyoma, and renal cell carcinoma (RCC). RCC in HLRCC is an aggressive metastatic tumor that develops at a young age. Here, we report the case of a patient with HLRCC who was diagnosed after the spontaneous rupture of a renal tumor. The patient underwent cytoreductive surgery, followed by combination therapy with the immune checkpoint inhibitor (ICI) nivolumab and cabozantinib, a tyrosine kinase inhibitor (TKI); however, no improvements were achieved.

4.
Cureus ; 16(1): e51970, 2024 Jan.
Artigo em Inglês | MEDLINE | ID: mdl-38196984

RESUMO

AIM: This study aimed to identify the modules of the End-of-Life Nursing Education Consortium-Japan Core Curriculum (ELNEC-J), which are particularly necessary for second- to fourth-year nurses. METHODOLOGY:  This cross-sectional study recruited certified nurse specialists in cancer nursing (CNSCNs) endorsed by the Japanese Nursing Association enrolled in Advanced Practice Registered Nurses (APRNs) in Japan. We asked individuals who were active members of the volunteer association of CNSCNs in the Tokai region to participate via email, and we collected data using Google Forms. The participants were asked about their background, including APRN experience and current position. Furthermore, we asked them to select three necessary modules for second- to fourth-year nurses' education from the 10 modules of the ELNEC-J. RESULTS:  The study recruited a total of 19 (89%) APRNs (response rate: 100%). Out of them, 14 (73.6%) had more than six years of clinical experience in APRNs, and 12 (63.1%) held managerial positions. Regarding the 10 modules of the ELNEC-J, the responses for the necessary modules were as follows: nursing care at end-of-life 13 (68.4%), pain management 12 (63.2%), symptom management 10 (52.6%), communication 10 (52.6%), and ethical issues in palliative care nursing five (31.6%). CONCLUSION: According to the perspective of APRNs responsible for palliative care education for incumbent nurses, nursing care at the end of life, pain management, symptom management, and communication are required for second- to fourth-year nurse education.

5.
Medicine (Baltimore) ; 102(51): e36747, 2023 Dec 22.
Artigo em Inglês | MEDLINE | ID: mdl-38134085

RESUMO

Recently, cone-beam computed tomography (CBCT)-guided surgeries have been developed for bone and soft tissue tumors. The present study aimed to evaluate the efficacy of CBCT-guided curettage for osteoid osteoma. Our study population included 13 patients who underwent primary curettage for osteoid osteoma using intraoperative CBCT in a hybrid operating room between April 2019 and November 2022. We collected the following data: sex, age, follow-up period, symptom onset to time of surgery, tumor size and location, length of skin incision, operating time, radiation dose, recurrence, postoperative complications, and visual analog scale for pain during the last follow-up. There were 10 male and 3 female patients, and the mean age was 25.0 years (range, 9-49 years). The mean follow-up period was 10.6 months (range, 0.4-24.0 months). The locations of the tumors were the proximal femur in 6 patients, the acetabular region in 2 patients, and the ilium, tibial shaft, calcaneus, cuboid, and talus in 1 patient each. The mean time of symptoms onset to surgery was 18.7 months (range, 2.3-69.9 months). The mean maximum diameter of the tumor was 5.9 mm (range, 3.5-10.0 mm). The mean length of the skin incision was 2.2 cm (range, 1.5-3.5 cm). The mean operating time was 96.9 minutes (range, 64-157 minutes). The mean dose of radiation was 193.2 mGy (range, 16.3-484.0 mGy). No recurrences, postoperative complications, and reoperation were observed in this study. All the patients reported 0 mm on the visual analogue scale for pain on the last follow-up. CBCT-guided curettage for osteoid osteoma was minimally invasive and reliable. This procedure can be effective for the treatment of lesions found in deep locations such as the pelvic bone and proximal femur or an invisible lesion that cannot be detected by regular fluoroscopy.


Assuntos
Neoplasias Ósseas , Calcâneo , Osteoma Osteoide , Tálus , Humanos , Masculino , Feminino , Adulto , Osteoma Osteoide/diagnóstico por imagem , Osteoma Osteoide/cirurgia , Osteoma Osteoide/patologia , Tomografia Computadorizada por Raios X/métodos , Radiografia Intervencionista/métodos , Neoplasias Ósseas/diagnóstico por imagem , Neoplasias Ósseas/cirurgia , Tomografia Computadorizada de Feixe Cônico/métodos , Dor , Tálus/patologia , Complicações Pós-Operatórias , Calcâneo/patologia , Resultado do Tratamento
6.
J Clin Med ; 12(21)2023 Oct 30.
Artigo em Inglês | MEDLINE | ID: mdl-37959327

RESUMO

p16 overexpression is often used as a surrogate marker for human papillomavirus (HPV) infection in oropharyngeal squamous cell carcinoma but remains an uncertain diagnostic tool for HPV-related sinonasal squamous cell carcinoma (SNSCC). Our study involved 79 consecutive SNSCC patients who were treated at a tertiary referral university hospital during 2006-2021. We retrospectively examined their clinical characteristics and conducted p16 immunohistochemistry and HPV detection. We found that 12.7% of the patients exhibited p16 overexpression, which was significantly more common in the nasal cavity and increased from 2015 onward. The HPV was a high-risk type and viral loads ranged from 4.2 to 1.6 × 106 copies/ng DNA with genome integration. Five-year overall survival (OS) and five-year relapse-free survival (RFS) rates were 74.6% and 69.9%, respectively. Our multivariate analysis showed that T category (T1-4a) and hemoglobin levels (≥13.7) were significant favorable prognostic factors for OS, while T category, performance status, and p16 overexpression were significantly associated with RFS. In patients with p16 overexpression, OS was 100% and RFS was 90%. Our findings suggest that p16 overexpression is a reliable surrogate marker for transcriptionally active HPV infection and predicts a favorable prognosis.

7.
Anticancer Res ; 43(9): 4221-4227, 2023 Sep.
Artigo em Inglês | MEDLINE | ID: mdl-37648319

RESUMO

BACKGROUND/AIM: 18F-fluorodeoxyglucose (FDG) is known to accumulate in the liver. We investigated whether accumulation of FDG was correlated with the degree of liver fibrosis and the grade of necro-inflammatory activity. PATIENTS AND METHODS: This retrospective study included 35 patients who underwent FDG-positron emission tomography (PET)/computed tomography (CT) before liver surgery. On fusion images of CT and PET, by placing regions of interest on the lateral, anterior and posterior segments of the liver and the aorta, the standardized uptake value (SUV) mean, and SUV normalized by lean body mass (SUL) mean of the liver were measured, and the ratio SUVmean liver/SUVmean aorta was calculated. According to the New Inuyama Classification, subjects were classified into three groups based on the grade of liver-fibrosis degree, i.e., F0, F1+F2 and F3+F4, and into three groups based on the grade of necro-inflammatory activity, i.e., A0, A1 and A2. Each of the above parameters was then compared among the groups using a Tukey test. RESULTS: Average SULmean liver values of the F0, F1+F2 and F3+F4 groups were 1.573±0.211, 1.845±0.220 and 1.716±0.119, respectively. The SULmean liver of the F1+F2 group was significantly higher than that of the F0 group (p=0.0296). No significant difference was observed for the other two parameters. None of the parameters exhibited significant difference among the A0, A1, and A2 groups. CONCLUSION: FDG accumulation in the liver may be increased in the early stage of liver fibrosis. SULmean liver could be used to determine the necessity for therapeutic intervention in chronic liver disease.


Assuntos
Fluordesoxiglucose F18 , Tomografia por Emissão de Pósitrons combinada à Tomografia Computadorizada , Humanos , Estudos Retrospectivos , Cirrose Hepática/diagnóstico por imagem
8.
Surg Case Rep ; 9(1): 133, 2023 Jul 21.
Artigo em Inglês | MEDLINE | ID: mdl-37477751

RESUMO

BACKGROUND: Myxoid liposarcoma (MLS), with its risk factors, tends to spread to the lungs and extraperitoneally, with intraperitoneal metastases occurring rarely. We present an unusual case of a myxoid liposarcoma that metastasized to the abdominal organs. CASE PRESENTATION: A 60-year-old female patient was referred to our hospital for the evaluation of a right upper limb tumor that had been growing for 7 years. The patient refused surgery, and during follow-up, tumor hemorrhage resulted in hemorrhagic shock. The patient's right upper limb was immediately amputated. MLS was diagnosed histopathologically. Subsequently, the patient underwent adjuvant chemotherapy. Computed tomography (CT) revealed a right buttock mass, a pelvic mass, and left cardiophrenic angle lymph nodes 3 years after the initial surgery. Contrast-enhanced abdominal CT revealed a relatively low-density, lobulated pelvic tumor. Contrast-enhanced pelvic magnetic resonance imaging (MRI) revealed a low-intensity, lobulated mass on T1-weighted images and a high-intensity mass on T2-weighted images. The pelvic mass showed no significant fluorodeoxyglucose (FDG) uptake on positron emission tomography (PET)-CT. On clinical examination, gynecological malignancies were ruled out as the origin of the pelvic lesions. After resection of the right buttock mass, pelvic mass, and left cardiophrenic angle lymph nodes, the patient underwent laparoscopic surgery for a preoperative diagnosis of small intestinal mesenteric metastasis of MLS. A tumor was found in the mesentery of the small intestine and removed with a margin of 5 cm on both the proximal and distal sides. The specimen measured 10 × 8 × 5 cm and contained a multifocal mass. The tumor was found in the mesentery of the small intestine, with no mucosal or submucosal invasion. The patient was diagnosed with MLS with small mesenteric intestinal metastases. On postoperative day 8, the patient was discharged after an uneventful postoperative course. Twelve months after the surgery, there was no evidence of local or distant recurrence. CONCLUSIONS: Small intestinal mesenteric metastases of MLSs are rare. Moreover, there are few reports on laparoscopic resection. In this case, the laparoscopic approach was useful in detecting the tumor location and determining the range of resection.

10.
Mod Pathol ; 36(8): 100169, 2023 08.
Artigo em Inglês | MEDLINE | ID: mdl-36997002

RESUMO

Adult T-cell leukemia/lymphoma (ATLL) is a mature T-cell tumor caused by human T-lymphotropic virus type 1 (HTLV-1). The typical ATLL immunophenotypes are described in the 2017 World Health Organization Classification of Tumours of Haematopoietic and Lymphoid Tissues (positive: CD2, CD3, CD5, CD4, and CD25; negative: CD7, CD8, and cytotoxic markers; and partially positive: CD30, CCR4, and FOXP3). However, limited studies are available on the expression of these markers, and their mutual relationship remains unknown. Furthermore, the expression status of novel markers associated with T-cell lymphomas, including Th1 markers (T-bet and CXCR3), Th2 markers (GATA3 and CCR4), T follicular helper markers (BCL6, PD1, and ICOS), and T-cell receptor (TCR) markers, and their clinicopathologic significance is unclear. In this study, we performed >20 immunohistochemical stains in 117 ATLL cases to determine the comprehensive immunophenotypic profile of ATLL, which were compared on the basis of clinicopathologic factors, including morphologic variants (pleomorphic vs anaplastic), biopsy locations, treatments, Shimoyama classification-based clinical subtype, and overall survival. CD3+/CD4+/CD25+/CCR4+ was considered a typical immunophenotype of ATLL, but approximately 20% of cases did not conform to this pattern. Simultaneously, the following new findings were obtained: (1) most cases were negative for TCR-ß and TCR-δ (104 cases, 88.9%), indicating the usefulness of negative conversion of TCR expression to provide differentiation from other T-cell tumors; (2) the positivity of CD30 and CD15 and the negativity of FOXP3 and CD3 were significantly associated with anaplastic morphology; and (3) atypical cases, such as T follicular helper marker-positive (12 cases, 10.3%) and cytotoxic molecule-positive cases (3 cases, 2.6%), were identified. No single markers could predict the overall survival among patients with acute/lymphoma subtypes of ATLL. The results of this study illustrate the diversity of ATLL phenotypes. In T-cell tumors occurring in HTLV-1 carriers, the possibility of ATLL should not be eliminated even when the tumor exhibits an atypical phenotype, and the confirmation of HTLV-1 in the tissue is recommended.


Assuntos
Vírus Linfotrópico T Tipo 1 Humano , Leucemia-Linfoma de Células T do Adulto , Linfoma de Células T , Linfoma , Adulto , Humanos , Vírus Linfotrópico T Tipo 1 Humano/genética , Fatores de Transcrição Forkhead
11.
Mol Clin Oncol ; 18(3): 23, 2023 Mar.
Artigo em Inglês | MEDLINE | ID: mdl-36844466

RESUMO

An 83-year-old woman presented with a 1-year history of a growing mass on the lateral surface of the right knee. Magnetic resonance imaging revealed a large soft tissue tumor in the subcutis of the right knee. The mass in the right knee rapidly increased, due to hemorrhage from the tumor. A needle biopsy revealed that the diagnosis was synovial sarcoma. The patient underwent wide excision and lateral collateral ligament reconstruction using the plantaris tendon. The patient had a Musculoskeletal Tumor Society Score of 86% at the lateset follow-up. In conclusion, reconstruction of the lateral collateral ligament using the plantaris tendon may be useful for preserving the function of the knee joint after resection of the soft tissue due to sarcoma of the knee.

12.
Medicine (Baltimore) ; 101(36): e30127, 2022 Sep 09.
Artigo em Inglês | MEDLINE | ID: mdl-36086737

RESUMO

The purpose of the present study was to clarify clinical outcomes of elderly patients with soft tissue sarcoma who underwent surgery neither with neoadjuvant nor adjuvant chemotherapy. The median follow-up period was 46.3 (range 6.7-99.0) months. All patients underwent surgical resections. R0 margins were achieved in 24 cases (92.3%) and R1 margins in 2 cases (7.7%). The 1-, 2-, and 5-year sarcoma-specific survival (SSS) rates were 92.3%, 88.5%, and 83.8%, respectively. Multivariate analysis showed no significant risk factors for SSS. No significant relationship of histological grades and local recurrences (P = .56) or distant metastases (P = .54) was shown. In the current study, we observed a comparable survival ratio, despite no neoadjuvant or adjuvant chemotherapies performed. Tumor resections with adequate margins might, at least in part, have contributed to the decent survival ratio regardless of histological grade. Twenty-six consecutive patients aged ≥ 70 years, who underwent surgical resections of soft tissue sarcoma between January 2013 and December 2019, were included. SSS were analyzed by the Kaplan-Meier method, and the relationships between SSS and clinical parameters were evaluated by Cox proportional hazards analysis.


Assuntos
Sarcoma , Neoplasias de Tecidos Moles , Idoso , Quimioterapia Adjuvante , Humanos , Margens de Excisão , Fatores de Risco , Sarcoma/patologia , Neoplasias de Tecidos Moles/patologia
13.
Urol Case Rep ; 41: 101968, 2022 Mar.
Artigo em Inglês | MEDLINE | ID: mdl-34950566

RESUMO

Large adrenocortical adenomas have rarely been reported. We describe a case of a 26-year-old man who underwent an adrenalectomy for a large adrenocortical adenoma (8.6 × 7.7 cm). Although the lesion had typical malignant features on imaging, histopathological examination revealed an adrenocortical adenoma. This highlights that imaging alone may not be able to distinguish adrenocortical carcinomas from adrenal masses. In most cases, a resection should be performed for early diagnosis and management of large adrenal masses with malignant features on imaging. To our knowledge, this is the first report of a large adrenocortical adenoma diagnosed with multiple imaging investigations.

14.
Int J Clin Oncol ; 24(4): 454-459, 2019 Apr.
Artigo em Inglês | MEDLINE | ID: mdl-30377853

RESUMO

BACKGROUND: Clinical efficacy of aggressive end-of-life (EOL) chemotherapy remains unclear. METHOD: Medical records of patients with advanced cancer between August 2011 and August 2016 were retrospectively analyzed. The primary endpoint was to identify prognostic factors at the last administration of chemotherapy. The secondary endpoint was to analyze the relationship between EOL symptoms and EOL treatment details. RESULTS: Among 300 evaluated patients, the number of patients who died within 14 and 30 days from the last administration of chemotherapy were 16 (5.3%) and 50 (16.7%), respectively. Multivariate analysis revealed that ECOG-PS (OR 3.698, p < 0.001) and GPS2 (OR 3.791, p = 0.028) were significant prognostic factors. The MST of patients with both PS 2-4 and GPS2 (+) was 38 days, while that in patients with both PS 0-1 and GPS2 (-) was 134.5 days. The prevalence rate of nausea and vomiting (25.0%) and the mean hydration volume (0.50 L/day) in patients who died within 30 days from the chemotherapy was significantly higher than others (7.4%) (0.20 L/day). CONCLUSION: ECOG-PS and GPS were significant prognostic factors for aggressive EOL chemotherapy. Information on these factors may aid clinical decision-making in terms of risk-benefit balance, particularly in patients with poor prognosis.


Assuntos
Neoplasias/tratamento farmacológico , Assistência Terminal/métodos , Idoso , Antineoplásicos/efeitos adversos , Antineoplásicos/uso terapêutico , Feminino , Humanos , Estimativa de Kaplan-Meier , Masculino , Pessoa de Meia-Idade , Análise Multivariada , Náusea/induzido quimicamente , Neoplasias/mortalidade , Neoplasias/terapia , Prognóstico , Estudos Retrospectivos , Resultado do Tratamento , Vômito/induzido quimicamente
15.
Case Rep Obstet Gynecol ; 2018: 7513287, 2018.
Artigo em Inglês | MEDLINE | ID: mdl-29707395

RESUMO

A 40-year-old pregnant woman presented with a fetal abdominal cyst and oligohydramnios. Color Doppler scan revealed a single blood vessel from the fetal aorta into a single umbilical artery. Severe oligohydramnios limited ultrasonographic evaluation of the fetal lower limbs, kidneys, or bladder. The pregnancy was terminated; the fetus showed fused lower limbs, bulging abdomen, and absent external genitalia and was diagnosed with type III sirenomelia. On autopsy, no normal bladder was observed, but duodenal atresia, anorectal atresia, and right renal agenesis were found. An intra-abdominal cyst, diagnosed histologically as a saccular cloaca, occupied the abdominal cavity. Ultrasonographic diagnosis of fetal sirenomelia is difficult due to poor depiction of the lower limbs. A vitelline artery leading to a single umbilical artery and a fetal abdominal cyst occupying most of the abdominal cavity are considered fetal sirenomelia associated with large defects of the gastrointestinal and genitourinary tracts.

16.
Int J Clin Oncol ; 23(3): 532-538, 2018 Jun.
Artigo em Inglês | MEDLINE | ID: mdl-29275449

RESUMO

BACKGROUND: Lymphovascular space involvement is reported to be an important risk factor in endometrial cancer. This study was conducted to evaluate the separate prognostic effects of lymphatic invasion and venous invasion on the outcomes of patients with endometrial cancer. METHODS: From 2006 to 2013, 189 histologically confirmed endometrial cancer patients were examined. To study the venous invasion (v) of the endometrial cancer, Victoria blue-H&E staining-which positively stains the elastic fibers of vessels-was performed. Immunohistochemical staining with D2-40 was used to study the lymphatic invasion (ly) of the endometrial cancer. RESULTS: The median age of the patients was 57 (range 25-84) years. ly(+) and/or v(+) patients were significantly more likely to present an advanced cancer stage, G3 tumor, and deep myometrial invasion than ly(-)/v(-) patients. The incidence of lymph node metastasis was high in ly(+) patients, and that of ovarian metastasis was high in v(+) patients. Lymphatic vessel invasion was significantly correlated with regional lymph node metastasis. We found a significantly higher incidence of distant metastasis in ly(+) patients. Most recurrences in ly(+)/v(-) patients occurred in lymph nodes, while those in ly(+)/v(+) patients occurred mainly at distant organs. Finally, the prognosis was significantly poorer for ly(+) patients, in whom lymphatic invasion was an independent prognostic factor along with distant metastasis. CONCLUSIONS: Our study suggests that by separately evaluating lymphatic invasion and blood vessel invasion in endometrial cancer cases, useful information for predicting lymph node metastasis and recurrence sites as well as prognostic information can be obtained.


Assuntos
Neoplasias do Endométrio/mortalidade , Neoplasias do Endométrio/patologia , Metástase Linfática/patologia , Adulto , Idoso , Idoso de 80 Anos ou mais , Neoplasias do Endométrio/cirurgia , Feminino , Seguimentos , Humanos , Imuno-Histoquímica , Linfonodos/patologia , Vasos Linfáticos/patologia , Pessoa de Meia-Idade , Miométrio/patologia , Recidiva Local de Neoplasia/patologia , Neoplasias Ovarianas/patologia , Neoplasias Ovarianas/secundário , Prognóstico , Fatores de Risco , Taxa de Sobrevida
17.
Am J Surg Pathol ; 41(12): 1713-1721, 2017 Dec.
Artigo em Inglês | MEDLINE | ID: mdl-28877060

RESUMO

Undifferentiated sarcoma harboring the BCOR-CCNB3 fusion is characterized by its predilection to affect skeletons of adolescent males, cellular small round/spindle cell morphology, and CCNB3 immunoreactivity. We analyzed 11 cases of BCOR-CCNB3 sarcoma, 10 of which were identified in a reverse transcription-polymerase chain reaction-based screen of 85 patient samples recorded in our database as unclassified small round or spindle cell sarcomas. BCOR rearrangements were confirmed by fluorescence in situ hybridization in 8 tumors. All patients were males aged between 6 and 31 years. In addition to 5 tumors in soft tissue and 4 in the axial or appendicular skeletons, which are typical locations, a tumor was located in the paranasal sinus and another in the lung. Microscopically, the tumors comprised proliferating atypical spindle and/or small round cells with diverse morphologic features such as small concentric whorls, myxoid stroma, a hemangiopericytomatous appearance, and/or hyalinized collagen resembling a solitary fibrous tumor, and angiomatous or slit-like spaces containing extravasated erythrocytes. Tumor cells were immunoreactive to CCNB3 (9/11), BCOR (10/10), TLE1 (6/10), bcl-2 (9/11), CD99 (8/10), CD56 (8/10), c-kit (4/10), and cyclin D1 (10/10). In an immunohistochemical analysis of an additional 412 small round or spindle cell tumors, CCNB3 was detected in 6 (1.5%) and BCOR in 18 (4.4%). Our analysis highlights the varying clinicopathologic features of this tumor, which partially overlap with other small round or spindle cell tumors, including solitary fibrous tumor and vascular tumors. Because CCNB3 and BCOR immunohistochemistry lacks adequate sensitivity and specificity, a molecular genetic approach remains essential for diagnosis.


Assuntos
Biomarcadores Tumorais/análise , Diferenciação Celular , Ciclina B/análise , Fusão Gênica , Imuno-Histoquímica , Proteínas Proto-Oncogênicas/análise , Proteínas Repressoras/análise , Sarcoma de Células Pequenas/química , Adolescente , Adulto , Biomarcadores Tumorais/genética , Criança , Ciclina B/genética , Predisposição Genética para Doença , Humanos , Hibridização in Situ Fluorescente , Masculino , Fenótipo , Valor Preditivo dos Testes , Proteínas Proto-Oncogênicas/genética , Proteínas Repressoras/genética , Reprodutibilidade dos Testes , Reação em Cadeia da Polimerase Via Transcriptase Reversa , Sarcoma de Células Pequenas/genética , Sarcoma de Células Pequenas/patologia , Adulto Jovem
18.
Case Rep Pathol ; 2017: 1765460, 2017.
Artigo em Inglês | MEDLINE | ID: mdl-28503335

RESUMO

Primary villoglandular mucinous adenocarcinoma of the vulva is rare tumor. We report a case of 68-year-old woman who developed this very uncommon malignant tumor. Immunohistochemical examination of this tumor revealed positive staining for Cytokeratin 20, Mucin 2, and CDX2, although Cytokeratin 7 and Mucin 6 were negative. This positive staining indicated the tumor enteric type characters. In order to exclude the possibility of the metastasis from another site, we thoroughly evaluated clinical data and extensively investigated the whole body. However, we could not detect any other tumors. The patient was treated by tumor resection. The patient remains free of disease 5 years after surgery.

19.
Case Rep Obstet Gynecol ; 2016: 3467849, 2016.
Artigo em Inglês | MEDLINE | ID: mdl-27239357

RESUMO

Background. A rare case of low-grade endometrial stromal sarcoma (LG-ESS) extending to inferior vena cava (IVC) and cardiac chambers. Case Report. A 40-year-old woman had IVC tumor, which was incidentally detected by abdominal ultrasonography during a routine medical checkup. CT scan revealed a tumor in IVC, right iliac and ovarian veins, which was derived from the uterus and extended into the right atrium and ventricle. The operation was performed, the heart and IVC were exposed, and cardiopulmonary bypass was initiated. A right atriotomy was performed, and the intracardiac mass was removed. Then the tumor in IVC and the right internal iliac vein were removed after longitudinal venotomies in the suprarenal and infrarenal vena cava, the right common iliac vein. Next the pelvis was explored. Tumors were found originating from the posterior wall of the uterus and continuing into both the right uterine and ovarian vein. The patient underwent total hysterectomy with bilateral salpingooophorectomy. Complete tumor resection was achieved. Histopathological analysis confirmed a diagnosis of LG-ESS. She showed no evidence of disease for 2 years and 3 months. Conclusions. Our case highlights the importance of a multidisciplinary approach in treating this rare cardiovascular pathological condition through preoperative assessment to final operation.

20.
Cancer Epidemiol ; 38(5): 490-5, 2014 Oct.
Artigo em Inglês | MEDLINE | ID: mdl-25155209

RESUMO

BACKGROUND: Despite the fact that rare cancer is a new target of cancer control in Japan, the incidence of rare cancers is unknown and there is no generally accepted definition of rare cancers in this country. With the aim of calculating incidences of rare cancers in Japan, we therefore adopted a definition and classification of rare cancers that had been published in the European Union (EU) in 2011. METHODS: Using incidence data between 1998 and 2007 submitted by 12 of population based cancer registries in Japan that met our quality criteria and drawing on the EU definition (incidence <6 per 100,000 per year), we estimated the incidences of 845 combinations of tumor sites and histological groups and thus identified the cancers that are rare in Japan. RESULTS: After identifying 193 combinations of tumor sites and histological groups that fit our criteria for rare cancers, we estimated their incidence to be about 75 per 100,000, which corresponds to about 94,800 new diagnoses in 2012 or approximately 15% of all cancer diagnoses. The categorization of rare and common cancers was almost the same in Japan as in EU. CONCLUSIONS: The present study provides an indication of the size of the rare cancer burden in Japan and epidemiological information to explore this. We are expecting further discussion based on our results with stakeholders in order to construct a Japanese definition of rare cancers.


Assuntos
Neoplasias/epidemiologia , Doenças Raras/epidemiologia , Efeitos Psicossociais da Doença , Humanos , Incidência , Japão/epidemiologia , Neoplasias/patologia , Doenças Raras/patologia
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