-
Journal of Pediatric Surgery Nov 1990Malignant thymomas are extremely rare in children, with only 27 cases reported thus far in the pediatric surgical literature. We report four additional cases diagnosed...
Malignant thymomas are extremely rare in children, with only 27 cases reported thus far in the pediatric surgical literature. We report four additional cases diagnosed at this institution over the past 20 years (ages 3 to 14 years). Clinical presentations included superior vena cava syndrome, cough, dyspnea, cyanosis, enlarging mediastinal mass, spontaneous pneumothorax, and pleuropericardial effusion. Three patients underwent incomplete resection of the mass or biopsy because of "unresectability" and were treated with radiotherapy and adjunctive chemotherapy. One patient underwent near complete macroscopic resection as well as radiotherapy and chemotherapy. All patients died at intervals ranging from 6 months to 2 1/2 years after diagnosis. Three patients were found to have metastatic disease prior to death or at autopsy. In one case, the initial pathological diagnosis was lymphocytic thymoma. After ultrastructural studies were performed, the diagnosis was changed to thymic T-cell lymphoma. This patient subsequently developed acute lymphoblastic leukemia 3 months after surgical resection followed by radiotherapy. Malignant thymomas are highly aggressive tumors in children. A radical surgical approach with complete excision of the tumor and contiguous structures in continuity, with adjunctive radiotherapy and chemotherapy remains the only hope for survival in children with these rare lesions.
Topics: Adolescent; Child, Preschool; Diagnosis, Differential; Female; Humans; Male; Neoplasm Invasiveness; Thymoma; Thymus Neoplasms
PubMed: 2273428
DOI: 10.1016/0022-3468(90)90750-4 -
The Annals of Thoracic Surgery Nov 1997Primary thymomas arising in an intrapulmonary location without an associated mediastinal component are rare entities. The origin of thymomas in this unusual location...
Primary thymomas arising in an intrapulmonary location without an associated mediastinal component are rare entities. The origin of thymomas in this unusual location remains unknown. Knowledge of the natural history and the prognosis of these tumors is also limited because of their rarity.
Topics: Aged; Humans; Lung Neoplasms; Male; Thymoma
PubMed: 9386728
DOI: 10.1016/S0003-4975(97)00935-1 -
The Annals of Thoracic Surgery Aug 2004Most thymic neoplasms fall under the designation of thymoma, consisting of well-differentiated epithelial cells, resembling normal thymus. At the opposite spectrum are... (Review)
Review
BACKGROUND
Most thymic neoplasms fall under the designation of thymoma, consisting of well-differentiated epithelial cells, resembling normal thymus. At the opposite spectrum are thymic carcinomas; the cell of origin while similar is malignant. Recently a third category of thymic neoplasms, atypical thymomas, has been recognized representing thymic neoplasms manifesting atypia although without overt cytomorphologic criteria of malignancy.
METHODS
Seven patients with a diagnosis of atypical thymoma were encountered over a 6-year period from the patient files of the cardiothoracic division of The Ohio State Medical Center.
RESULTS
In all patients there was gross or light microscopic invasive disease with involvement of the capsule, phrenic nerve, diaphragm, chest wall, and lung. Surgical extirpation/de-bulking along with radiation therapy in six and chemotherapy in one led to complete disease regression. Intrathoracic recurrences developed in 4 involving lung, pleura, chest wall and diaphragm. All patients are well.
CONCLUSIONS
Atypical thymomas are locally aggressive tumors with a high incidence of intrathoracic recurrence; extrathoracic spread is not seen. Our study corroborates other reports that death attributable to atypical thymoma is uncommon.
Topics: Adult; Aged; Antineoplastic Combined Chemotherapy Protocols; Blood Vessels; Chemotherapy, Adjuvant; Cisplatin; Combined Modality Therapy; Cyclophosphamide; Diaphragm; Doxorubicin; Female; Humans; Lung; Male; Middle Aged; Neoplasm Invasiveness; Neoplasm Recurrence, Local; Pericardium; Phrenic Nerve; Pleura; Radiotherapy, Adjuvant; Remission Induction; Retrospective Studies; Thoracic Wall; Thymoma; Thymus Neoplasms; Treatment Outcome; Vincristine
PubMed: 15276487
DOI: 10.1016/j.athoracsur.2003.12.052 -
International Journal of Surgical... Aug 2023The 2021 "World Health Organization (WHO) Classification of Thoracic Tumours" classifies epithelial tumors of the thymus (thymomas) based on cytomorphology. Thymomas... (Review)
Review
The 2021 "World Health Organization (WHO) Classification of Thoracic Tumours" classifies epithelial tumors of the thymus (thymomas) based on cytomorphology. Thymomas with benign cytomorphology are classified as type A, AB, B1, B2, and B3, while those with malignant cytomorphology are classified as thymic carcinoma. Although all thymomas have malignant potential, extra-thoracic metastasis of thymomas is exceedingly rare and the exact incidence is not known. Literature review demonstrated 39 cases of thymoma with extra-thoracic metastases reported since the publication of the 1999 WHO Classification of Thoracic Tumours. Nine of these cases presented with metastasis to the liver in the setting of concurrent metastasis to other organs, while only three cases metastasized solely to the liver. We herein report a well-documented case of type B1 thymoma with relatively limited stage (IIb) with an isolated, single liver metastasis occurring seven years after primary resection in a patient with concomitant myasthenia gravis. The following report includes a review of the literature, a discussion of thymoma classification and its relevance to prognosis, and an overview of other extra-thoracic metastatic thymoma cases.
Topics: Humans; Thymoma; Thymus Neoplasms; Prognosis; Thymus Gland; Liver Neoplasms; Neoplasm Staging
PubMed: 36259324
DOI: 10.1177/10668969221115818 -
Journal of Thoracic Oncology : Official... Jan 2009Thymomas are rare intrathoracic malignant tumors. Commonly used staging system is the Masaoka classification, based on peroperative and histopathological findings.... (Review)
Review
Thymomas are rare intrathoracic malignant tumors. Commonly used staging system is the Masaoka classification, based on peroperative and histopathological findings. Surgery is the cornerstone of the management of thymomas, initially being useful for precise histopathological diagnosis and staging, and in most cases ensuring the first step of the therapeutics simultaneously. After tumor stage, complete resection is the most constant and significant prognostic factor for progression-free and overall survival. Postoperative radiotherapy is recommended in incompletely resected thymomas. Completely resected stage II and III tumors may also benefit from adjuvant radiotherapy to reduce local recurrence rates but without impact on survival. In primary unresectable thymomas, multimodal strategy nowadays includes neoadjuvant chemotherapy, extensive surgery, adjuvant radiotherapy, and in some cases, adjuvant chemotherapy. The most popular chemotherapy regimens combine cisplatin, adriamycin, etoposide, cyclophophamide, or ifosfamide.The management of thymomas is a paradigm of cooperation between clinicians, surgeons, and pathologists from establishing the diagnosis to organizing the therapeutic strategy and evaluating the prognosis. As a consequence of their rarity, no prospective randomized trials are available and collaborative studies are warranted to evaluate and improve current therapeutic standards, taking into account recent improvements in techniques, such as robotic surgery, radiotherapy, and supportive treatments.
Topics: Humans; Thymoma; Thymus Neoplasms
PubMed: 19096319
DOI: 10.1097/JTO.0b013e31818e105c -
Cancer Treatment Reviews Aug 2013Thymic malignancies are extremely rare although usually affect young adults and continue to remain an important health problem. Like other rare diseases, progress in... (Review)
Review
Thymic malignancies are extremely rare although usually affect young adults and continue to remain an important health problem. Like other rare diseases, progress in thymic malignancies has been slow and the treatment cornerstone still remains surgical resection. Next generation sequencing and other advances in molecular biology are shedding light onto the multiple genetic aberrations involved and have opened a new field for research with molecularly targeted therapies such as CKIT inhibitors or anti-EGFR therapies. In this review we will summarize the current knowledge in the pathophysiology, diagnosis, prognosis and latest advances in the management of thymomas and thymic carcinomas.
Topics: Humans; Molecular Targeted Therapy; Thymoma; Thymus Neoplasms
PubMed: 23261165
DOI: 10.1016/j.ctrv.2012.11.005 -
Thoracic Surgery Clinics Feb 2011Myasthenia gravis (MG) associated with thymomas differs from nonthymomatous MG, and thymomas associated with MG are also different from non-MG thymomas. According to the... (Review)
Review
Myasthenia gravis (MG) associated with thymomas differs from nonthymomatous MG, and thymomas associated with MG are also different from non-MG thymomas. According to the World Health Organization classification, the incidence of MG in thymomas was the highest in the subtypes B2, B1, and AB. Transsternal approach is still regarded as the gold standard for surgical treatment of thymomas. Less-invasive techniques of thymectomy are promising, but it is too early to estimate their real oncological value. In the series including more than 100 patients, the prognosis for survival is better in patients with thymomas associated with MG than in those with non-MG thymomas, and the prognosis for patients with MG associated with thymoma is worse than that for patients with nonthymomatous MG.
Topics: Autoimmune Diseases; Combined Modality Therapy; Humans; Immunosuppressive Agents; Myasthenia Gravis; Neoplasms, Second Primary; Plasmapheresis; Remission Induction; Thoracic Surgery, Video-Assisted; Thymectomy; Thymoma; Thymus Neoplasms
PubMed: 21070986
DOI: 10.1016/j.thorsurg.2010.08.009 -
Bulletin Du Cancer Apr 1999The "Standards, Options and Recommendations" (SOR) project, started in 1993, is a collaboration between the Federation of the French Cancer Centres (FNCLCC), the 20... (Review)
Review
CONTEXT
The "Standards, Options and Recommendations" (SOR) project, started in 1993, is a collaboration between the Federation of the French Cancer Centres (FNCLCC), the 20 French Cancer Centres and specialists from French Public Universities, General Hospitals and Private Clinics. The main objective is the development of clinical practice guidelines to improve the quality of health care and outcome for cancer patients. The methodology is based on literature review and critical appraisal by a multidisciplinary group of experts, with feedback from specialists in cancer care delivery.
OBJECTIVES
To develop clinical practice guidelines according to the definitions of Standards, Options and Recommendations for the clinical care of malignant thymoma in adult.
METHODS
Data have been identified by literature search using Medline (december 1998) and the expert groups personal reference lists. Once the guidelines were defined, the document was submitted for review to national and international independent reviewers, and to the medical committees of the 20 French Cancer Centres.
RESULTS
The main recommendations for malignant thymoma management are that: 1) the clinical diagnosis is based on appropriate clinical and radiological findings; 2) the final diagnosis is pathological and made from a biopsy, except in cases of well-encapsulated tumors which are completely resected. The biopsy, via anterior mediastinostomy, should be performed by the surgeon who will subsequently perform the definitive surgery; 3) surgical resection must be complete including thymus and perithymic fat and performed by an experienced surgeon; 4) the therapeutic strategy for malignant thymoma is based on the three current staging systems and involves surgery with radiotherapy given if the capsule is invaded or penetrated. Radiotherapy should be given in experienced centres. Inclusion of patients in prospective clinical trials is recommended in order to determine the usefulness of neoadjuvant chemotherapy and multimodality approaches; 5) treatment of metastatic malignant thymoma is based on chemotherapy. Secondary surgery may be performed with the aim of achieving complete resection. Inclusion in clinical trials is recommended; 6) at the present time, there are no clear data on which to base guidelines for timing and duration of follow-up studies in this condition. Because of late recurrence, follow-up should be long.
Topics: Adult; Humans; Neoplasm Recurrence, Local; Neoplasm Staging; Paraneoplastic Syndromes; Thymoma; Thymus Neoplasms
PubMed: 10341342
DOI: No ID Found -
Clinical Lung Cancer Nov 2015Thymomas, tumors that arise from the epithelial cells of the thymus gland, are the most common tumors of the anterior mediastinum despite their overall rarity. They are... (Review)
Review
Thymomas, tumors that arise from the epithelial cells of the thymus gland, are the most common tumors of the anterior mediastinum despite their overall rarity. They are not classified together with malignancies although it is recognized that they can be invasive and persistent even after attempted treatment. Because of their rarity, optimal treatment protocols remain a challenging topic. Although surgery is recognized as the cornerstone of management, the role and benefit of use of postoperative radiotherapy (PORT), remains questionable. Unequivocal evidence, although exclusively from retrospective studies, indicates that stage I thymoma is adequately treated with complete resection alone. As for stage II there is still a need to better determine the indications of PORT. For stage III and IV, existing data point to the fact that PORT plays a significant role in the management of thymoma. In patients for whom radiotherapy (RT) is indicated, 50 Gy appears to be adequate for microscopic disease and higher doses should be used for macroscopic tumor. With advances in RT delivery techniques, which allow for higher doses to be delivered to larger areas affected by tumor while sparing normal tissue, it is prudent to identify a place for this modality in the optimal management of thymoma patients.
Topics: Combined Modality Therapy; Epithelial Cells; Humans; Neoplasm Staging; Radiotherapy; Thymectomy; Thymoma; Thymus Neoplasms
PubMed: 26074303
DOI: 10.1016/j.cllc.2015.05.003 -
Annals of Medicine Oct 1999A WHO committee recently defined criteria for distinguishing between thymic epithelial tumours (TET) and classified them as type A, AB, B1-3 and C thymomas. As the... (Review)
Review
A WHO committee recently defined criteria for distinguishing between thymic epithelial tumours (TET) and classified them as type A, AB, B1-3 and C thymomas. As the terminology for each WHO type is still controversial, it is recommended to use also other names in addition to the WHO classification to allow comparability of future clinicopathological studies. We consider type A and AB thymomas (medullary and mixed thymomas) clinically benign, whereas type B1-3 thymomas (predominantly cortical and cortical thymomas and well-differentiated thymic carcinomas) are of low-grade malignant potential and most type C thymomas (category II malignant thymomas) are highly malignant. Not yet approved by the WHO are the recently described 'thymoma with pseudosarcomatous stroma' and the 'low-grade metaplastic carcinoma of the thymus', which are considered as benign or low-grade malignant tumours, respectively. Thymic pathology frequently occurs in myasthenia gravis (MG). Production of autoantibodies against the acetylcholine receptor results from an antigen-driven immune reaction that starts inside the thymus, is maintained there but spreads to extrathymic sites already during the early phase of MG. Paraneoplastic MG occurs only in type A, AB and B1-3 thymomas. Abnormal TET microenvironments trigger nontolerogenic T-cell selection by neoplastic epithelial cells. Only after export of substantial numbers of naive, potentially autoreactive T cells to extratumorous sites does T-cell activation outside the thymoma initiate the autoimmune process. Early surgery after onset of MG is essential in thymitis to prevent substantial export of autoreactive T cells from the inflamed thymus to extrathymic organs, and it usually alleviates MG symptoms. In thymoma, 'dissemination' of autoreactive T cells to extratumorous sites has already continued for many months or even years before emergence of symptoms of MG. Therefore, thymoma surgery is aimed against oncological and local cardiovascular complications and rarely succeeds in alleviating symptoms of MG.
Topics: Carcinoma; Humans; Myasthenia Gravis; Prognosis; Thymoma; Thymus Gland; Thymus Neoplasms; World Health Organization
PubMed: 10574149
DOI: No ID Found