Thymic tumor is a relatively rare type of thoracic cancer, which is pathologically-classified as thymic epithelial tumors by World Health Organization (WHO), including thymoma and thymic carcinoma. The incidence of thymic epithelial tumors in China is about 4.09 per million, which is slightly higher than that in European and American countries. For surgically-resectable thymic epithelial tumors, complete resection with or without postoperative radiotherapy or chemotherapy is preferred. For advanced unresectable disease, conventional radiotherapy and chemotherapy is the mainstream treatment but lack of standard first-line and second-line regimens. In recent years, targeted drugs and the immune checkpoint inhibitors have gradually exhibited certain therapeutic prospect in this subset of tumor. Since there was no guideline regarding thymic epithelial tumor released in China, Multidisciplinary Committee on Oncology, Chinese Physicians Association analyze, summarize and discuss the relevant research data for the diagnosis, treatment and prognosis of thymic epithelial tumors, and formulate the "Chinese guidelines for clinical diagnosis and treatment of thymic epithelial tumors (2021 Edition)" for clinicians' reference.
China ; Humans ; Neoplasm Staging ; Neoplasms, Glandular and Epithelial/therapy* ; Thymoma/therapy* ; Thymus Neoplasms/therapy*

Objective To investigate the clinicopathological features,immunohistochemical characteristics,diagnosis,treatment and prognosis of intrathyroid thymic carcinoma.Methods Clinical data of 7 patients with intrathyroid thymic carcinoma were retrospectively reviewed.Histological examination and immunohistochemical staining were performed on the surgically resected tumors.The infection of Epstein-Barr virus(EBV)was detected by EBER in situ hybridization.Results The 7 patients included 5 males and 2 females.The age ranged from 40 to 71 years,with a median of 54 years.The tumors were located in the thyroid gland,with the maximum diameter ranging from 2.2 cm to 6.0 cm and the average maximum diameter of(4.0±1.2)cm.All the patients underwent thyroid gland resection and local lymph node dissection.After operation,all the cases were treated with radiotherapy and five of them additionally received chemotherapy.Six patients were followed up for 10-163 months,all of whom were still alive,including 2 patients with recurrence in situ,1 patient with homolateral cervical lymph node metastasis and the rest with no recurrence or metastasis.CK-pan,P63,CD5 and CD117 were expressed in all the cases,while TTF-1,TG,CT and PAX8 were negative.One case of them expressed SYN and CgA.Ki-67 proliferation index ranged from 10% to 90%.EBER in situ hybridization showed negative results in all 7 cases.Conclusions Intrathyroid thymic carcinoma is a relatively low-grade malignant tumor.The combination of immunohistochemical CD5,CD117 and monoclonal PAX8 is helpful in the diagnosis and differential diagnosis of intrathyroid thymic carcinoma.EBV may not be involved in the development of intrathyroid thymic carcinoma.Thyroid gland resection plus central lymph node dissection is an important treatment measure for intrathyroid thymic carcinoma.For patients with regional lymph node metastasis and obvious peripheral tissue invasion,postoperative radiotherapy with/without chemotherapy can effectively delay the disease progression.
Child, Preschool ; Epstein-Barr Virus Infections ; Female ; Herpesvirus 4, Human ; Humans ; Male ; Neoplasm Recurrence, Local ; Retrospective Studies ; Thymoma ; Thymus Neoplasms/therapy*

BackgroundThe co-existence of myasthenia gravis (MG) and thymoma makes the surgical treatment more complicated and adjuvant radiation more controversial. The aim of this study was to investigate adjuvant radiotherapy for thymoma with MG after extended thymectomy.

MethodsA total of 181 patients with both MG and thymoma were recruited between 2003 and 2014 at Tongren Hospital, China. Among all the patients, 157 patients received radiation therapy after surgery (Group A); whereas the other 24 patients did not receive radiation therapy (Group B). According to the time that patients started mediastinal radiation therapy, we subdivided the 157 patients in Group A into subgroups (1-month subgroup, n = 98; 2-month subgroup, n = 7; and 3-month subgroup, n = 52). We then compared the effect of the mediastinal radiation therapy across these different groups using the survival rate, the rate of postoperative myasthenic crisis, and the complete stable remission (CSR) rate as the primary endpoints.

ResultsThere was a significant difference in the occurrence of postoperative myasthenic crisis between 1-month subgroup and Group B (χ = 4.631, P = 0.031). The rates of reaching CSR were 32.6% in 1-month subgroup, 25% in 3-month subgroup, and 22.7% in Group B, respectively. The overall survival rates of 1-month subgroup, 3-month subgroup, and Group B were 88.8%, 83.3%, and 77.3%, respectively. Analysis on the Kaplan-Meier survival curves demonstrated that within 8 years after surgery, there was no significant difference in aspects of overall survival and disease-free survival between 1-month subgroup and Group B, and between 3-month subgroup and Group B; over 8 years after surgery, the disease-free survival rates in 1-month subgroup, 3-month subgroup and Group B were 79.4%, 70.6%, and 55.3%, respectively.

ConclusionsAdjuvant radiation within 1 month after extended thymectomy may be helpful in controlling postoperative MG, such as decreasing the possibility of postoperative myasthenic crisis, and raising cumulative probabilities of reaching CSR.

Adult ; Aged ; Aged, 80 and over ; Female ; Humans ; Kaplan-Meier Estimate ; Male ; Middle Aged ; Myasthenia Gravis ; surgery ; therapy ; Postoperative Period ; Radiotherapy, Adjuvant ; methods ; Thymectomy ; methods ; Thymoma ; surgery ; therapy ; Thymus Neoplasms ; surgery ; therapy ; Treatment Outcome

OBJECTIVETo confirm the anticancer effect of total annonaceous acetogenins (TAAs) abstracted from Annona squamosa Linn. on human hepatocarcinoma.

METHODSThe inhibitory effect of TAAs was demonstrated in H22-bearing mice. The potency of TAAs was confirmed as its 50% inhibiting concentration (IC50) on Bel-7402 cell under Sulfur Rhodamine B staining. Both underlying mechanisms were explored as cellular apoptosis and cell cycle under flow cytometry. Mitochondrial and recipient apoptotic pathways were differentiated as mitochondrial membrane potential under flow cytometry and caspases activities under fluorescence analysis.

RESULTSThe inhibitory rate of TAAs in mice was 50.98% at 4 mg/kg dose. The IC50 of TAAs on Bel-7402 was 20.06 µg/mL (15.13-26.61µg/mL). Effective mechanisms of TAAs were confirmed as both of arresting cell cycle at G1 phase and inducing apoptosis dose- and time-dependently. Mitochondrial and recipient pathways involved in apoptotic actions of TAAs.

CONCLUSIONTAAs is effective for hepatocarcinoma, via inhibiting proliferation and inducing apoptosis.

Acetogenins ; chemistry ; pharmacology ; therapeutic use ; Animals ; Annona ; chemistry ; Antineoplastic Agents, Phytogenic ; chemistry ; pharmacology ; therapeutic use ; Apoptosis ; drug effects ; Carcinoma, Hepatocellular ; drug therapy ; enzymology ; pathology ; Caspases ; metabolism ; Cell Cycle ; drug effects ; Cell Line, Tumor ; Cell Proliferation ; drug effects ; Chromatography, High Pressure Liquid ; Dose-Response Relationship, Drug ; Humans ; Liver Neoplasms ; drug therapy ; enzymology ; pathology ; Male ; Membrane Potential, Mitochondrial ; drug effects ; Mice ; Organ Specificity ; drug effects ; Spleen ; drug effects ; Thymus Gland ; drug effects ; Xenograft Model Antitumor Assays

OBJECTIVETo study the effect of spleen lymphocytes on the splenomegaly by hepatocellular carcinoma-bearing mouse model.

METHODSCell counts, cell cycle distribution, the percentage of lymphocytes subsets and the levels of IL-2 were measured, and two-dimensional gel electrophoresis (2-DE) was used to investigate the relationship between spleen lymphocytes and splenomegaly in hepatocellular carcinoma-bearing mice.

RESULTSCompared with the normal group, the thymus was obviously atrophied and the spleen was significantly enlarged in the tumor-bearing group. Correlation study showed that the number of whole spleen cells was positively correlated with the splenic index. The cell diameter and cell-cycle phase distribution of splenocytes in the tumor-bearing group showed no significant difference compared to the normal group. The percentage of CD3+ T lymphocytes and CD8+ T lymphocytes in spleen and peripheral blood of tumor-bearing mice were substantially higher than that in the normal mice. Meanwhile, the IL-2 level was also higher in the tumor-bearing group than in the normal group. Furthermore, two dysregulated protein, β-actin and S100-A9 were identified in spleen lymphocytes from H22-bearing mice, which were closely related to cellular motility.

CONCLUSIONIt is suggested that dysregulated β-actin and S100-A9 can result in recirculating T lymphocytes trapped in the spleen, which may explain the underlying cause of splenomegaly in H22-bearing mice.

Animals ; Carcinoma, Hepatocellular ; complications ; Cell Cycle ; Female ; Liver Neoplasms ; complications ; Lymphocytes ; physiology ; Mice ; Mice, Inbred ICR ; Neoplasms, Experimental ; therapy ; Spectrometry, Mass, Matrix-Assisted Laser Desorption-Ionization ; Spleen ; cytology ; pathology ; Splenomegaly ; etiology ; therapy ; Thymus Gland

Adult ; Aged ; Carcinoma, Squamous Cell ; diagnostic imaging ; pathology ; therapy ; Chemotherapy, Adjuvant ; Female ; Follow-Up Studies ; Humans ; Male ; Middle Aged ; Radiotherapy, Adjuvant ; Survival Rate ; Thymus Neoplasms ; diagnostic imaging ; pathology ; therapy ; Tomography, X-Ray Computed

OBJECTIVETo review the presentation, diagnosis, staging and treatment of thymoma.

DATA SOURCESData were obtained from papers on thymoma published in English within the last 30 years. No formal systematic review was conducted, but an effort was made to be comprehensive.

STUDY SELECTIONStudies were selected if they contained data relevant to the topic addressed in the particular section. In particular, standards adopted by the International Thymic Malignancies Interest Group through a formal process of achieving worldwide consensus are featured. Because of the limited length of this article, we have frequently referenced recent reviews that contain a comprehensive amalgamation of literature rather than the actual source papers.

RESULTSThymomas are rare malignant tumors. They account for about half (47%) of anterior mediastinal tumors. About one third of these are associated with myasthenia gravis. Computed tomography with intravenous contrast is the standard diagnostic modality. Thymomas appear as round or oval masses in early stages but irregular shapes with calcifications occurring in later stages. They can invade surrounding structures including mediastinal fat, pleura, major blood vessels and nerves. Fine needle aspiration, core needle biopsy or open biopsy is used to obtain tissue diagnosis. Masaoka-Koga classification is currently used to stage thymomas. All thymomas should be considered for resection due to their malignant potential. A complete resection is a major prognostic factor and every effort should be made to achieve this even if this means resection and reconstruction of a major thoracic structure. Median sternotomy is the standard approach for thymoma resection. A number of minimally invasive techniques are used in selective centers. While stage I and II tumors undergo primary surgery, preoperative chemotherapy appears to increase the chances of complete resection for stage III and IVa tumors. Postoperative radiation could be considered for patients with residual disease. Excellent 5 and 10-year survival rates are noted for completely resected early stage thymomas.

CONCLUSIONSThymic malignancies are rare tumors. Standards have recently been achieved to allow better communication and promote collaborative research. Surgical resection is the mainstay of treatment, but a multimodality approach is useful for many patients.

Humans ; Incidence ; Neoplasm Recurrence, Local ; therapy ; Neoplasm Staging ; Thymoma ; diagnosis ; pathology ; therapy ; Thymus Neoplasms ; diagnosis ; pathology ; therapy ; Tomography, X-Ray Computed

Adult ; Anemia, Aplastic ; pathology ; therapy ; Female ; Hematopoietic Stem Cell Transplantation ; Humans ; Remission Induction ; Thymoma ; pathology ; therapy ; Thymus Neoplasms ; pathology ; therapy ; Transplantation, Homologous

OBJECTIVETo study the clinicopathologic features of primary thymic extranodal marginal zone B-cell lymphoma of mucosa-associated lymphoid tissue (MALT).

METHODSThe clinical and pathologic findings were evaluated in 3 cases of biopsy confirmed thymic MALT lymphoma. The clincopathologic features, treatment and prognosis were discussed and literatures reviewed.

RESULTSOne male and two female patients presented with asymptomatic mediastinal masses with a history of Sjögren syndrome. They were aged 36, 35 and 41 years respectively, and only one patient had B symptoms. Grossly, all three tumors were encapsulated and had multiple variable-sized cysts on cut-surface. Histopathologically, the normal thymic lobular architecture was effaced by abnormal dense lymphoid infiltration. Prominent lymphoepithelial lesions were formed by centrocyte-like cells infiltrating and expanding Hassall's corpuscles and epithelial cyst lining. All cases showed apparent plasmacytic differentiation. Immunohistochemically, the tumor cells were positive for CD20, CD79a, bcl-2 and negative for CD3, CD5, cyclin D1, CD43, CD10, bcl-6, and CD23. The plasma cells showed kappa light chain restriction. Immunoglobulin heavy chain rearrangement in three cases was confirmed by PCR. All patients were at early stage and received routine chemotherapy with or without radiotherapy after surgical removal. All patients achieved complete remission with 24, 18 and 3 months follow-up, respectively.

CONCLUSIONSPrimary thymic MALT lymphoma may be a rare distinctive lymphoma. It can be diagnosed by HE and immunohistochemical study and should be differentiated from reactive lymphoid proliferation, other types of lymphoma and mediastinal thymoma.

Adult ; Antibodies, Monoclonal, Murine-Derived ; therapeutic use ; Antigens, CD20 ; metabolism ; Antineoplastic Combined Chemotherapy Protocols ; therapeutic use ; Cyclophosphamide ; therapeutic use ; Diagnosis, Differential ; Doxorubicin ; therapeutic use ; Female ; Follow-Up Studies ; Gene Rearrangement, B-Lymphocyte, Heavy Chain ; Humans ; Immunoglobulin Heavy Chains ; genetics ; Keratin-19 ; metabolism ; Lymphoma, B-Cell, Marginal Zone ; drug therapy ; genetics ; metabolism ; pathology ; surgery ; Male ; Prednisone ; therapeutic use ; Pseudolymphoma ; pathology ; Thymus Hyperplasia ; pathology ; Thymus Neoplasms ; drug therapy ; genetics ; metabolism ; pathology ; surgery ; Vincristine ; therapeutic use

OBJECTIVETo improve the understanding of thymic neuroendocrine carcinoma (TNC) by retrospective analysis of the clinical data of 21 cases.

METHODSThe clinical data of 21 patients with TNC treated in the Peking Union Medical College Hospital from 1998 to 2010 were retrospectively analyzed.

RESULTSThere were 12 males and 9 females, with onset age ranging from 13 to 67 years and the mean age of 43 years. The clinical manifestation was diverse, in which the malaise of chest and back accounted for 57.1% (12/21), Cushing's syndrome 33.3% (7/21), multiple endocrine neoplasia type 1 accounted for 4.8% (1/21), pharyngeal malaise 4.8%, superior vena cava syndrome 4.8% (1/21) and asymptomatic patients accounted for 4.8% (1/21). Eighteen of them demonstrated metastasis, in which 13 had metastasis to lymph nodes and local vascular invasion, 9 to lung or pleura, 5 to bone, 1 to liver and 1 was recurred in situ. Twenty of them were treated by surgical resection. Eleven of them received radiotherapy and 9 chemotherapy after surgery. One patient was too severe to endure surgery, and was a treated by chemotherapy only.

CONCLUSIONTNC is rare, and its clinical features are complex, with a high level of malignancy. The first choice of treatment is resection. Adjuvant radiotherapy and/or chemotherapy may be applied in patients with metastasis.

Adolescent ; Adrenocorticotropic Hormone ; metabolism ; Adult ; Aged ; Bone Neoplasms ; secondary ; Carcinoma, Neuroendocrine ; metabolism ; pathology ; secondary ; surgery ; therapy ; Chemotherapy, Adjuvant ; Cushing Syndrome ; metabolism ; pathology ; surgery ; therapy ; Female ; Humans ; Hydrocortisone ; metabolism ; Immunohistochemistry ; Lung Neoplasms ; secondary ; Lymphatic Metastasis ; Male ; Middle Aged ; Multiple Endocrine Neoplasia Type 1 ; metabolism ; pathology ; surgery ; therapy ; Radiotherapy, Adjuvant ; Retrospective Studies ; Thymectomy ; Thymus Neoplasms ; metabolism ; pathology ; surgery ; therapy ; Young Adult