- Lymphoblastic Lymphoma: Guidelines From the International Lymphoma Radiation Oncology Group (ILROG). [Review]
- IJInt J Radiat Oncol Biol Phys 2018 Nov 01; 102(3):508-514
- Presentation with a large mediastinal mass is a hallmark of acute lymphoblastic lymphoma, a disease that is treated in the same way as acute lymphoblastic leukemia even in the absence of marrow invol...
Presentation with a large mediastinal mass is a hallmark of acute lymphoblastic lymphoma, a disease that is treated in the same way as acute lymphoblastic leukemia even in the absence of marrow involvement. The role of mediastinal radiation for patients who achieve complete remission after chemotherapy has been overlooked and controversial. This document presents current knowledge on the role of radiation for lymphoblastic lymphoma and best practices for addressing how to deliver mediastinal radiation with modern technology.
- Mediastinal extension of a pancreatic pseudocyst. [Journal Article]
- RCRadiol Case Rep 2018; 13(6):1192-1194
- A 50-year-old man with a history of chronic pancreatitis due to alcoholism presented with dyspnea, at which time he was diagnosed with pleural effusions, treated, and discharged. Two months later, he...
A 50-year-old man with a history of chronic pancreatitis due to alcoholism presented with dyspnea, at which time he was diagnosed with pleural effusions, treated, and discharged. Two months later, he was readmitted with hemoptysis and abdominal pain. CT and MRI of the chest demonstrated a mediastinal cystic mass that communicated with the retroperitoneum. Ultrasound-guided aspiration of the cystic mass revealed high levels of amylase, confirming that the mass was a rare pancreatic pseudocyst extending into the mediastinum.
- Unicentric Castleman disease presenting as a retroperitoneal peripancreatic mass: A report of two cases and review of literature. [Journal Article]
- WJWorld J Gastroenterol 2018 Sep 14; 24(34):3958-3964
- Castleman disease (CD) is a rare disorder of lymph nodes and related tissues. CD generally occurs in the mediastinum, as well as in cervical, retroperitoneal and axillary regions. The disease is clas...
Castleman disease (CD) is a rare disorder of lymph nodes and related tissues. CD generally occurs in the mediastinum, as well as in cervical, retroperitoneal and axillary regions. The disease is classified into two major types: unicentric CD (UCD) and multicentric CD. The occurrence of UCD in the retroperitoneal peripancreatic region is quite rare. We encountered two cases of retroperitoneal peripancreatic UCD in our hospital during the past three years. Following a series of medical examinations, including magnetic resonance imaging, computed tomography, ultrasonography and postoperative histopathological examination, these two patients were diagnosed with UCD, which presented as a retroperitoneal peripancreatic mass. The mass in each patient was completely excised, and no postoperative radiochemotherapy was administered. Both patients recovered well without recurrence during a follow-up period of 30 mo and 8 mo.
- Mediastinal Myeloid Sarcoma with TP53 Mutation Preceding Acute Myeloid Leukemia with a PICALM-MLLT10 Fusion Gene. [Case Reports]
- AHActa Haematol 2018 Sep 18; 140(2):97-104
- CONCLUSIONS: MS with a mediastinal localization is rare and often misdiagnosed as malignant lymphoma. Acute leukemia harboring a PICALM-MLLT10 fusion gene is characterized by a mixed T cell and myeloid phenotype. The rearrangement is a rare recurrent translocation associated with specific clinical features, as illustrated in this case report.
- Thymic epithelial neoplasms with Rhabdomyomatous component: a Clinicopathological and Immunohistochemical study of 7 cases. [Journal Article]
- HPHum Pathol 2018 Sep 11
- Seven primary thymic epithelial neoplasms, three thymomas, and four thymic carcinomas with rhabdomyomatous component are herein described. The patients are two women and 5 men between the ages of 42 ...
Seven primary thymic epithelial neoplasms, three thymomas, and four thymic carcinomas with rhabdomyomatous component are herein described. The patients are two women and 5 men between the ages of 42 and 62years (average: 52years). Clinically, the patients presented with non-specific symptoms of cough, chest pain, and dyspnea. None of the patients had history of myasthenia gravis or of previous malignancy. Diagnostic imaging revealed the presence of an anterior mediastinal mass in all the patients. Surgical resection was accomplished in all the cases. The three thymoma cases were encapsulated tumors: histologically two were lymphocyte rich (WHO type B1) and one was an atypical thymoma (WHO type B3). All the thymic carcinomas were ill-defined tumor masses with infiltrative borders and histologically were high grade carcinomas. In each tumor, in different proportion, there were easily identifiable areas with rhabdomyomatous component characterized by larger cells with eosinophilic cytoplasm and eccentrically placed nuclei. Immunohistochemical stains in all cases showed clearly demarcated presence of the epithelial component (keratin positive / desmin and myoglobin negative) and the rhabdomyomatous component (desmin and myoglobin positive / keratin negative). The three patients with thymoma are alive and without recurrence 3 and 5years after surgical resection, while three patients with carcinoma died between 2 and 3years post-surgical resection. One patient with thymic carcinoma was lost to follow-up. The current cases highlight the ubiquitous distribution of myoid cells in different types of thymic epithelial neoplasms.
- Successful cisplatin-etoposide chemotherapy-based treatment of a primary small cell neuroendocrine carcinoma of the tonsil with multiple metastases: a case report. [Journal Article]
- OTOnco Targets Ther 2018; 11:5391-5395
- Extrapulmonary small cell neuroendocrine carcinoma (SNEC) is an extremely rare and highly malignant tumor with a poor prognosis. Multiple metastases of SNEC are even more rare, and patients with loca...
Extrapulmonary small cell neuroendocrine carcinoma (SNEC) is an extremely rare and highly malignant tumor with a poor prognosis. Multiple metastases of SNEC are even more rare, and patients with locally advanced and metastatic disease generally face a poor outcome. To date, only a few cases of SNEC have been reported. Here, we describe a rare case of a 70-year-old female patient with SNEC of the tonsil who presented with multiple metastases and had achieved a complete response (CR) of the primary lesion and cervical lymph nodes for more than 1 year after receiving palliative chemotherapy. Initially, the patient presented with a 2-month history of throat pain. Magnetic resonance imaging and computed tomography revealed a soft mass with moderate enhancement on the left tonsil, which was confirmed by incisional aspiration biopsy. She was additionally sent for a positron emission tomography scan to evaluate small metastases in the left cervical lymph node, right lung, multiple mediastinal lymph nodes, and the fourth lumbar (L4) vertebra body metastases. Histopathological examination of the SNEC confirmed a nested, typical endocrine appearance with small round cells containing ovoid-shaped nuclei and high mitotic activity. Immunohistochemically, the tumor cells were positive for cytokeratin 8/18+, synaptophysin+, CD56+, and Ki-67 (<50%). The patient received 6 cycles of cisplatin combined with etoposide and was subsequently placed under close observation (<12 months). To date, she has achieved a CR of the primary lesion and cervical lymph nodes. In summary, we have described a case of successful treatment after chemotherapy for SNEC and have elucidated professional knowledge regarding the relevant aspects of SNEC.
- Surgical therapy and next-generation sequencing-based genetic alteration analysis of malignant solitary fibrous tumor of the pleura. [Journal Article]
- OTOnco Targets Ther 2018; 11:5227-5238
- CONCLUSIONS: The high frequency of NAB2-STAT6 fusion indicates its prominent role in SFTP, while somatic mutations such as FLT1-R593W and KDR-V297I may also contribute to the malignant angiogenic phenotype. The present study affirmed the heterogeneity of SFTP, and more sophisticated classification methods will be needed to explore its underlying mechanisms.
- Combined transbronchoscopic needle aspiration (TBNA) and rapid on-site cytological evaluation (ROSE) for diagnosis of tuberculous mediastinal lymphadenitis: A case report. [Journal Article]
- MMedicine (Baltimore) 2018; 97(37):e11724
- CONCLUSIONS: TBNA is an efficacious and safe approach, which is worth popularizing for the clinical diagnosis of mediastinal masses. Meanwhile, ROSE is useful to reduce the numbers of needle passes during TBNA. We aimed to emphasize 2 key points in this case report. Firstly, a rare symptom of Tuberculosis in adults was supported by the patient. Secondly, TBNA combined with ROSE is useful for the diagnosis of tuberculous mediastinal lymphadenitis.
- Cyclin D1-positive Mediastinal Large B-Cell Lymphoma With Copy Number Gains of CCND1 Gene: A Study of 3 Cases With Nonmediastinal Disease. [Journal Article]
- AJAm J Surg Pathol 2018 Sep 11
- Primary mediastinal large B-cell lymphoma (PMBL) is a mature large B-cell lymphoma of putative thymic B-cell origin involving the mediastinum with younger age distribution and better prognosis than d...
Primary mediastinal large B-cell lymphoma (PMBL) is a mature large B-cell lymphoma of putative thymic B-cell origin involving the mediastinum with younger age distribution and better prognosis than diffuse large B-cell lymphoma (DLBCL), not otherwise specified. Recently, based on gene expression profile analysis and morphologic findings, cases of PMBL without mediastinal involvement have been reported. In this study, we analyzed 3 cases of nodal DLBCL with morphologic features of PMBL presenting in submandibular or supraclavicular lymph nodes, in middle-aged to elderly patients, 2 of them without clinical or radiologic evidence of mediastinal involvement. The 3 patients presented with stage I/II disease and had excellent response to R-CHOP/R-EPOCH therapy. The 3 cases showed MAL expression and were positive for CD23 and/or CD30. All 3 cases expressed cyclin D1 with copy number gains of CCND1 gene but without rearrangement. There was no rearrangement of CIITA or PDL1/PDL2. Reverse transcriptase-multiplex ligation-dependent probe amplification, a mRNA-based gene expression profile analysis revealed high probability of PMBL (87.6%, 98.7%, and 99%) in these 3 cases. Targeted next-generation sequencing analysis showed SOCS1 mutations in the 3 cases, and TNFAIP3 and XPO1 mutations in one, further supporting the diagnosis of PMBL. In conclusion, we report 3 cases of nodal PMBL, 2 of them without mediastinal mass, and expression of cyclin D1 due to copy number gains of CCND1 gene, a diagnostic pitfall with mantle cell lymphoma and DLBCL, not otherwise specified.
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- Congenital cystic adenomatous malformation: a case report and a literature review. [Journal Article]
- AMActa Med Litu 2018; 25(2):95-100
- CONCLUSIONS: CCAM are often diagnosed by accident when performing routine pregnancy ultrasound examinations. CT is the most reliable X-ray-based examination method for confirming a diagnosis. When CCAM is suspected in the foetus, amniocentesis and cariotype identification are performed, but chromosomal anomalies related to CCAM are often not identified. Currently, the best treatment results have been achieved by applying combined prenatal therapy and early surgical treatment.