. 1 Management of Unresectable Thymoma/Thymiccarcinoma Dr. Ajay Yadav Medical Oncology Epidemiology • Thymicepithelial tumors-1.3-3.2 per million annually • 90% of thymus tumors-thymoma • 10%-thymiccarcinoma,carcinoidtumor EnglesE et al. Epidemiology of thymoma and associated malignancies.J ThoracOncol.2010 Oct Epidemiology Journal of Thoracic Oncology 2014 Indian Data • 62 pts(2006-2011),Tata Memorial Hospital,Mumbai. • 36M, 26F; • 22-84yrs, median 51.5 years • Stage1-22,2-13,3-18,4-9 • Type A-7,AB-14,- B-30 (49%).C-11 • Sx,Sxwith adj .,NACT/CTRT,palliative • 3yrs survival-95%,89%,100%,71% RathodS et al.Thymoma:Firstlarge Indian experience.IndianJournal of Cancer.2014 Clinical Features • 1/3 rd -Mediastinalmass, local symptoms (chest pains, dyspnea, hemoptysis, dysphonia, Horner syndrome, and superior vena cava compression). • 1/3 rd- paraneoplasticsyndrome, such as myasthenia gravis (MG) or pure red cell aplasia (PRCA). • 1/3 rd -mediastinalmass incidentally discovered on radiographic imaging Paraneoplasticsyndrome • Myasthenia Gravis • 30%- 50% thymoma develop MG • 10% MG-thymoma • Pure Red Cell Aplasia PRCA: • 5%thymoma-PRCA • 10% PRCA-thymoma • Isolated anemia,low Reticcount,bonemarrow –absence of erythroid precursors. • Hypogammaglobulinemia– • In 5% to 1 0 % of patients with thymoma • 10% hypogammaglobulinemia-thymoma. • Recurrent sinusitis ( common) red cell hypoplasia. Devita.Principle and Practice of Oncology.9 th Edition
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Management of UnresectableThymoma/Thymic carcinoma
Dr. Ajay Yadav
Medical Oncology
Epidemiology
• Thymic epithelial tumors-1.3-3.2 per million annually
• 90% of thymus tumors-thymoma
• 10%-thymic carcinoma,carcinoid tumor
Engles E et al. Epidemiology of thymoma and associated malignancies.J Thorac Oncol.2010 Oct
Epidemiology
Journal of Thoracic Oncology 2014
Indian Data
• 62 pts(2006-2011),Tata Memorial Hospital,Mumbai.
• 36M, 26F;
• 22-84yrs, median 51.5 years
• Stage1-22,2-13,3-18,4-9
• Type A-7,AB-14,- B-30 (49%).C-11
• Sx,Sx with adj .,NACT/CTRT,palliative
• 3yrs survival-95%,89%,100%,71%
Rathod S et al.Thymoma:First large Indian experience.Indian Journal of Cancer.2014
Clinical Features
• 1/3rd -Mediastinal mass, local symptoms (chest pains, dyspnea, hemoptysis, dysphonia, Horner syndrome, and superior vena cava compression).
• 1/3rd- paraneoplastic syndrome, such as myasthenia gravis (MG) or pure red cell aplasia (PRCA).
• 1/3rd -mediastinal mass incidentally discovered on radiographic imaging
Paraneoplastic syndrome
• Myasthenia Gravis
• 30%- 50% thymoma develop MG
• 10% MG-thymoma
• Pure Red Cell Aplasia PRCA:
• 5%thymoma-PRCA
• 10% PRCA-thymoma
• Isolated anemia,low Retic count,bone marrow –absence of erythroid precursors.
• Hypogammaglobulinemia –
• In 5% to 1 0 % of patients with thymoma
• 10% hypogammaglobulinemia- thymoma.
• Recurrent sinusitis ( common) red cell hypoplasia.
Devita .Principle and Practice of Oncology.9 th Edition
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Second malignancies
• B cell NHL
• Soft tissue sarcoma
Paraneoplastic syndrome associated with thymoma
Upto Date 21.2
Anterior mediastinal anatomySuspecting anterior med.mass in CXR(Hilum overlay sign)
• Standard uptake higher in type B3 thymomasthymic carcinomas,thymic hyperplasia
• Optional in aggressive and advanced stage histology
FNAC or Biopsy
• FNAC-
• Difficult to distinguish by cytology
• Sensitivity< 60%.
• Biopsy:
• Larger samples can be obtained by incisional biopsy (e.g., anterior mediastinotomy or video-assisted thoracoscopy)
• Sensitivity >90% .
• Should not violate the pleural space.
Mehran R, Ghosh R, Maziak D, et al. Surgical treatment of thymoma. Can J Surg 2002;45:25-30.
Murakawa T et al. Results from surgical treatment for thymoma. 43 years of experience. Jpn J Thorac Cardiovasc Surg
IHC in anterior mediastinal masses
Saad et al. Utility of Immunohistochemistry in Separating Thymic Neoplasms From Germ Cell Tumors and Metastatic
Lung Cancer Involving the Anterior Mediastinum. Applied Immunohistochemistry & Molecular Morphology.June 2003
WHO histology
• A,B(morphology of neoplastic cells and their nuclei)
• A-spindle cells,
• B dendritic cells
• AB-Both
• B
• B1-cortex (normal thymus,large number of lymphocyctes),
• B2-less lymphocytes,
• B3 endothelial cell with round or polygonal cells with no mild atypia with minor lymphocytes
• C- Thymic carcinoma
Differences between thymoma and thymiccarcinoma
Thymoma Thymic carcinoma
Histology Organotypic tissue Non Organotypic tissue
Clinical course Less Aggressive More Aggressive
Metastasis Less distant metastasis Distant metastasis
Genomic alteration B1 –similar genomic
features
Thymic Squamous cell ca.- losses of chromosomes 1q,
6q, 13q, and gains of chromosome 1q, 17q, and
18.
Response to RT B1,B2(Lymphocytic)>B3
Recurrence Mixed ,medullay pattern
Survival B1 10 yrs-95% 5 yrs-30-50%
Onuka T et al.J Thor Oncol.2008,Wright CD et al.J Thorac Cardiov Surg 2005
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Histological type of Thymic Carcinoma Masaoka staging
a.Masaoka A et al. Cancer 1981,b.Okumara M.Cancer 2002
Staging Staging grouping
Management Management
• Locally advanced thymoma:
• Is it resectable?
• Does induction therapy make it resectable?
• What are the agents used and their results?CT,RT alone or CTRT
• How do we manage once it is resected?
• Chemotherapy combination used?Which one is superior?
• Thymic carcinoma:
• What are the multimodality approach used?Any role of targeted agents?
• Recurrent thymoma
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NCCN guidelines Resectability in thymoma(Indian data)
• 1989-2009,Retrospective study,
• 150 thymic tumors(5 NEC,145-thymoma)
• Stage1-34,2-39,3-56,4a-16
• 6 invasive thymoma-reconstruction of SVC,and /or brachiocephalic vein.
• All 6 pts adj.RT,CT(6 cycles cyclophosphamide,epirubicin,carboplatin)
• All alive at the end of f/u 18-24mnths
Arvind K et al.Resection and reconstruction of mediastinal great vessels in invasive thymoma.Indian Journal of Cancer.2010
Predicting unresectability in high risk thymoma
• Trial to study modalities for pre-operative treatment,
• Inclusion criteria predicting unresectability
• tumour size >8 cm, or
• size ranging from 5 to 8 cm with one or more of the following criteria: multifocal calcification, heterogeneous appearance, irregular or scalloped borders, great vessel invasion or encirclement.
• tumours <5 cm in greatest diameter, obvious great vessel invasion/encirclement in CT scan
Preoperative Treatment of Patients With High Risk Thymoma Clinicaltrial.gov NCT00387868
Multimodality tt thymoma:CT as induction
Shin DM et al..Annals of Internal Medicine.July 1998
Multimodality tt thymoma:CT as induction
• Median f/u-43mnths
• 9 complete resection DFS rate 7yrs-73%
• 2 pts incomplete resection-recurrence (OS at 7 yrs-100%)
• Major S/E-myelosuppression
• Ki-67 minimum if tumor necrosis >80%
Multimodality tt:CTRT as induction.
• MGH, 1997-2006,Retrospective
• 10 pts,stage3-7,stage4-3
• Cisplatin,etoposide/RT-Sx-cis,etoposie if incomplete resection and high risk of recurrence(close margin)