Version 0 Thymic Epithelial Tumours 2nd revision, published September 2017



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Version 2.0 Thymic Epithelial Tumours 2nd revision, published September 2017

Core/ Non-core

Element name

Values

Commentary

Implementation notes

Non-core

CLINICAL INFORMATION

Multi select value list (choose all that apply):
• Not provided
• Myasthenia gravis
• Pure red cell aplasia
• Rheumatoid arthritis
• Hypogammaglobulinemia (Good’s syndrome)
• Lupus
• Addison’s disease
• Cushing’s disease
• Previous neoplasm (specify)
• Preoperative therapy (specify)
• Other disorders (specify)

It is helpful to know whether the patient has myasthenia gravis or other conditions including neoplasms that can be associated with thymomas. Knowledge of any neoadjuvant treatment is also important as it may explain necrosis and scarring seen macroscopically and microscopically, and allows the pathologist to comment on histologic treatment response.
If clinical conditions other than those listed are provided, then these should be noted under ‘Other disorders’.

 

Non-core

OPERATIVE PROCEDURE

Single selection value list:
• Extended thymectomy
Radical thymectomy
• Partial thymectomy
• Total thymectomy
• Not specified
• Other (specify)

Documentation of the operative procedure is useful, as correlation of the type of procedure with the material received can be important for both pathological diagnosis and patient safety. Further, the type of surgical procedure is important in determining the assessment of surgical margins.1
The surgeon should inform the pathologist of the type of operation/procedure.
A thymectomy is an operation to remove the thymus. A partial thymectomy is the removal of less than the whole thymus. A total (standard) thymectomy is the removal of the thymus gland without surrounding fatty tissue. An extended thymectomy is the removal of the thymus gland including the fatty tissue of the mediastinum and neck. A radical (maximal) thymectomy is the removal of the thymus gland and wide resection of fatty tissue of the middle and anterior mediastinum and neck from the diaphragm to the thyroid gland and between both phrenic nerves; the technique includes visualization of recurrent laryngeal and phrenic nerves and wide opening of both pleural spaces.

References


1 Detterbeck FC, Moran C, Huang J, Suster S, Walsh G, Kaiser L and Wick M (2011). Which way is up? Policies and procedures for surgeons and pathologists regarding resection specimens of thymic malignancy. J Thorac Oncol 6:S1730-1738.

 

Core

SPECIMEN(S) SUBMITTED

Multi select value list (choose all that apply):
• Not specified
• Partial thymus
• Complete thymus
• Thymus plus surrounding tissue (radical thymectomy)
• Mediastinal pleura
Pericardium
• Lung
o Right
• Wedge
• Lobe
• Entire Lung
o Left
• Wedge
• Lobe
• Entire Lung
• Phrenic nerve
• Right
• Left
• Great vessels
• Brachiocephalic (innominate) vein
• Superior vena cava
• Extrapericardial pulmonary artery/veins
• Aorta (ascending, arch or descending)
• Arch vessels
• Intrapericardial pulmonary artery
• Myocardium
• Diaphragm
• Separate extrathymic tumour nodules
• Lymph nodes
• Other (specify)

Specimen type should indicate what was submitted.1 Specimen type varies according to the type of operation. If the specimen was obtained by a radical thymectomy, the specimen type is indicated as “Thymus plus surrounding tissue.”
Specimens obtained by combined resection with other organs or parts thereof, should be itemised, such as lung, pleura, pericardium, great vessels and myocardium. Other organs or tissues are reported as “Other” and details should be recorded.1-3
Separate extrathymic tumour nodules submitted should be recorded; these include pleural and pericardial seedings, pulmonary intraparenchymal nodules and distant organ metastases. The location, number and size of extrathymic nodules are described later in the dataset (see SEPARATE EXTRATHYMIC TUMOUR NODULES/METASTASES).
Submitted lymph nodes should also be recorded.4,5 These may be submitted separately or within a combined mediastinal specimen, so labelling or discussion with the surgeon may be required. Further details on lymph nodes are captured later in the dataset (see LYMPH NODE STATUS).
Orientation of the specimen is crucial given the prognostic importance of margin status and pathologic tumour stage in resected thymic epithelial tumours (TETs). Once the tumour is removed from the tumour bed, orientation becomes difficult. Furthermore, the fatty tissue can become easily disrupted. Therefore, orientation of the specimen ideally should be started in situ by the surgeon and areas of concern need to be clearly communicated to the pathologist. Orientating the specimen on a mediastinal board is encouraged (Figure 1).1 Anterior, posterior, right and left surfaces should be clearly distinguished (e.g. inked with different colours or with a detailed block key). Furthermore, the surgeon should mark areas of concern and also representative areas adjacent to the pericardium, the innominate (brachiocephalic) vein and superior vena cava (or mark these structures if resected) and right/left mediastinal pleural surfaces (if resected).

Figure 1: Mediastinal board that could be used to orient the specimen1 Mediastinal board. A diagram on a soft board is useful in maintaining proper dimensions and orientation of specimens. Printing this figure as a full page corresponds roughly to the normal mediastinal dimensions and can be placed directly on a standard soft specimen board that is generally available in surgical pathology departments.


(Reprinted from Detterbeck FC, Moran C, Huang J, Suster S, Walsh G, Kaiser L and Wick M (2011). Which way is up? Policies and procedures for surgeons and pathologists regarding resection specimens of thymic malignancy. J Thorac Oncol 6:S1730-1738 with permission from Elsevier)

References


1 Detterbeck FC, Moran C, Huang J, Suster S, Walsh G, Kaiser L and Wick M (2011). Which way is up? Policies and procedures for surgeons and pathologists regarding resection specimens of thymic malignancy. J Thorac Oncol 6:S1730-1738.
2 Detterbeck FC, Stratton K, Giroux D, Asamura H, Crowley J, Falkson C, Filosso PL, Frazier AA, Giaccone G, Huang J, Kim J, Kondo K, Lucchi M, Marino M, Marom EM, Nicholson AG, Okumura M, Ruffini E and Van Schil P (2014). The IASLC/ITMIG Thymic Epithelial Tumors Staging Project: proposal for an evidence-based stage classification system for the forthcoming (8th) edition of the TNM classification of malignant tumors. J Thorac Oncol 9(9 Suppl 2):S65-72.
3 Nicholson AG, Detterbeck FC, Marino M, Kim J, Stratton K, Giroux D, Asamura H, Crowley J, Falkson C, Filosso PL, Giaccone G, Huang J, Kondo K, Lucchi M, Marom EM, Okumura M, Ruffini E and Van Schil P (2014). The IASLC/ITMIG Thymic Epithelial Tumors Staging Project: proposals for the T Component for the forthcoming (8th) edition of the TNM classification of malignant tumors. J Thorac Oncol 9(9 Suppl 2):S73-80.
4 Kondo K, Van Schil P, Detterbeck FC, Okumura M, Stratton K, Giroux D, Asamura H, Crowley J, Falkson C, Filosso PL, Giaccone G, Huang J, Kim J, Lucchi M, Marino M, Marom EM, Nicholson AG and Ruffini E (2014). The IASLC/ITMIG Thymic Epithelial Tumors Staging Project: proposals for the N and M components for the forthcoming (8th) edition of the TNM classification of malignant tumors. J Thorac Oncol 9(9 Suppl 2):S81-87.
5 Bhora FY, Chen DJ, Detterbeck FC, Asamura H, Falkson C, Filosso PL, Giaccone G, Huang J, Kim J, Kondo K, Lucchi M, Marino M, Marom EM, Nicholson AG, Okumura M, Ruffini E and Van Schil P (2014). The ITMIG/IASLC Thymic Epithelial Tumors Staging Project: A Proposed Lymph Node Map for Thymic Epithelial Tumors in the Forthcoming 8th Edition of the TNM Classification of Malignant Tumors. J Thorac Oncol 9(9 Suppl 2):S88-96.

 

Non-core

SPECIMEN INTEGRITY

Single select value list:
• Intact specimen
• Surface disrupted
• Fragmented specimen

Although there are no studies specifically evaluating the prognosis of patients who underwent thymectomy where the capsule was disrupted intraoperatively or the lesion was resected in fragments, it is important to record these features because in these circumstances the pathologist cannot properly evaluate the presence of capsular invasion or completeness of resection. The latter are important prognostic features.
• ‘Intact specimen’ means that a TET is either completely surrounded by a fibrous capsule or is present in its entirety within the submitted specimen, without rupture of the tumour into surrounding tissues or on to the external surface of the specimen.
• ‘Surface disrupted’ means that a TET remains in one piece but shows exposure of the tumour onto the external surface of the specimen, secondary to disruption.
A fragmented specimen is when a TET is submitted in piecemeal form that precludes satisfactory identification of margins.

References


1 Detterbeck FC, Moran C, Huang J, Suster S, Walsh G, Kaiser L and Wick M (2011). Which way is up? Policies and procedures for surgeons and pathologists regarding resection specimens of thymic malignancy. J Thorac Oncol 6:S1730-1738.
2 Nicholson AG, Detterbeck FC, Marino M, Kim J, Stratton K, Giroux D, Asamura H, Crowley J, Falkson C, Filosso PL, Giaccone G, Huang J, Kondo K, Lucchi M, Marom EM, Okumura M, Ruffini E and Van Schil P (2014). The IASLC/ITMIG Thymic Epithelial Tumors Staging Project: proposals for the T Component for the forthcoming (8th) edition of the TNM classification of malignant tumors. J Thorac Oncol 9(9 Suppl 2):S73-80.

 

Non-core

MACROSCOPIC SITE OF PRIMARY TUMOUR

Single select value list:
• Not specified
• Thymic
o Single tumour
o >1 tumour
• Ectopic (specify site/s)

TETs usually arise as a single nodule or mass in the thymus in the anterior mediastinum. However, cases of multiple, synchronous TETs have been described.1-3 Although synchronous TETs generally occur in the thymus in the anterior mediastinum, these tumours can also occur at ectopic sites. Although rare, ectopic TETs have been described in the neck, posterior mediastinum, pretracheal fat, deep to phrenic nerves, posterior to brachiocephalic (innominate) vein, aortopulmonary window, aortocaval groove, anterior mediastinal fat, cardiophrenic fat and base of skull. Ectopic thymomas can also present in the lung, where they should be dealt with as primary pulmonary neoplasms. Importantly, ectopic TETs should be distinguished from pleural or pericardial implants and metastases because the latter will up-stage the tumour. Many reported synchronous TETs differ in tumour subtype and stage. In addition, a case of synchronous thymoma and thymic carcinoid tumour has been reported in a patient with multiple neuroendocrine neoplasia type I.4 Therefore, when synchronous TETs are identified, each tumour should be recorded, microscopically reviewed and staged.

References


1 Suzuki H, Yoshida S, Hiroshima K, Nakatani Y and Yoshino I (2010). Synchronous multiple thymoma: report of three cases. Surgery today 40:456-459.
2 Bernatz PE, Harrison EG and Clagett OT (1961). Thymoma: a clinicopathological study. J Thorac Cardiovasc Surg 42:424-444.
3 Leuzzi G, Marino M, Alessandrini G, Sciuto R, Pescarmona E and Facciolo F (2015). Synchronous triple thymoma and true thymic hyperplasia simultaneously detected by F FDG PET-CT. Rev Esp Med Nucl Imagen Mol 34(4):272-274.
4 Miller BS, Rusinko RY and Fowler L (2008). Synchronous thymoma and thymic carcinoid in a woman with multiple endocrine neoplasia type 1: case report and review. Endocr Pract 14:713-716.

 

Non-core

MAXIMUM DIMENSION OF PRIMARY TUMOUR

Numeric: __mm
OR
Cannot be assessed

A retrospective analysis of 5845 cases showed that size was not useful in predicting survival in relation to staging of TETs, so this is viewed as a non-core rather than as a core parameter.1
Identification of the primary tumour may be uncertain in cases with multiple foci and therefore the maximum dimension of the largest tumour should be recorded.
The maximum tumour size should still be recorded as the number of blocks sampled in a resected tumour is recommended to be 1 per centimetre of the maximum diameter. Inadequate sampling may lead to incorrect tumour classification.2

References


1 Nicholson AG, Detterbeck FC, Marino M, Kim J, Stratton K, Giroux D, Asamura H, Crowley J, Falkson C, Filosso PL, Giaccone G, Huang J, Kondo K, Lucchi M, Marom EM, Okumura M, Ruffini E and Van Schil P (2014). The IASLC/ITMIG Thymic Epithelial Tumors Staging Project: proposals for the T Component for the forthcoming (8th) edition of the TNM classification of malignant tumors. J Thorac Oncol 9(9 Suppl 2):S73-80.
2 Moran CA and Suster S (2000). On the histologic heterogeneity of thymic epithelial neoplasms. Impact of sampling in subtyping and classification of thymomas. Am J Clin Pathol 114(5):760-766.

 

Core

HISTOLOGICAL TUMOUR TYPE

 

Tumours should be classified according to the World Health Organisation (WHO) 2015 classification system for thymic tumours (see below).1-3
In cases of TETs showing more than one morphological subtype the following should be applied:
1) TETs showing more than one histological thymoma subtype: The diagnosis in such tumours should list all the histological WHO types, starting with the predominant component and then minor components. All should be quantified in 10% increments. This rule does not apply to AB thymoma which is a distinct entity (this should be documented as type AB 100%).2,4
2) TETs consisting of a thymic carcinoma component together with one or more thymoma component: Irrespective of the size/percentage of the thymic carcinoma component the diagnosis in such tumours should begin with the label “thymic carcinoma” (specifying the histological type and percentage) followed by the thymoma component(s) (quantified in 10% increments).1,2
3) TETs consisting of more than one thymic carcinoma component (with or without a thymoma component, and excluding thymic small cell carcinoma and thymic large cell neuroendocrine carcinoma, see below): the diagnosis in such tumours should begin with the predominant carcinoma; minor carcinoma components should be quantified next in 10% increments, eventually followed by the thymoma components, if present.1,2
4) Heterogeneous thymic tumours with a small cell or large cell neuroendocrine carcinoma component: These tumours are labelled ‘combined small cell carcinoma’ or ‘combined large cell neuroendocrine carcinoma’; the various components should be given and quantified in 10% increments.

References


1 WHO (World Health Organization) (2015). WHO Classification of Tumours of the Lung, Pleura, Thymus and Heart. Fourth edition Travis WD, Brambilla E, Burke AP, Marx A and Nicholson AG. IARC Press, Lyon, France.
2 Marx A, Ströbel P, Badve SS, Chalabreysse L, Chan J, Chen G, de Leval L, Detterbeck F, Girard N, Huang J, Kurrer MO, Lauriola L, Marino M, Matsuno Y, Molina TJ, Mukai K, Nicholson AG, Nonaka D, Rieker R, Rosai J, Ruffini E and Travis WD (2014). ITMIG Consensus Statement on the Use of the WHO Histological Classification of Thymoma and Thymic Carcinoma: Refined Definitions, Histological Criteria and Reporting. J Thor Oncol 9:596-611.
3 Marx A, Chan JK, Coindre JM, Detterbeck F, Girard N, Harris NL, Jaffe ES, Kurrer MO, Marom EM, Moreira AL, Mukai K, Orazi A and Strobel P (2015). The 2015 World Health Organization Classification of Tumors of the Thymus: Continuity and Changes. J Thorac Oncol 10(10):1383-1395.
4 Strobel P, Bauer A, Puppe B, Kraushaar T, Krein A, Toyka K, Gold R, Semik M, Kiefer R, Nix W, Schalke B, Muller-Hermelink HK and Marx A (2004). Tumor recurrence and survival in patients treated for thymomas and thymic squamous cell carcinomas: a retrospective analysis. J Clin Oncol 22(8):1501-1509.

Heading
Use the 2015 WHO classification. Where relevant, if more than one subtype, list in 10% increments

Core

Thymoma

Single selection value list:
Not identified
Present

 

if present, record predominant subtype and other subtypes

Core

Predominant subtype

List type (2015 WHO classification) and %

 

 

Core

Other subtype

List type (2015 WHO classification) and %

 

Repeat for each other subtype

Core

Thymic carcinoma

Single selection value list:
• Not identified
• Present

 

if present, record predominant subtypes and other thymic carcinoma patterns

Core

Predominant subtype

List type (2015 WHO classification) and %

 

 

Core

Other subtype

List type (2015 WHO classification) and %

 

Repeat for each other subtype

Core

Thymic neuroendocrine tumours

Single selection value list:
• Not identified
• Present

 

If present, record subtypes and percentage

Core

Typical carcinoid tumour

Numeric:___%

 

 

Core

Atypical carcinoid tumour

Numeric:___%

 

 

Core

Large cell neuroendocrine carcinoma

Numeric:___%

 

 

Core

Small cell carcinoma

Numeric:___%

 

 

Core

Final histological diagnosis

Text

 

Use 2015 WHO classification for combined tumours

Core

EXTENT OF DIRECT INVASION

 

The Masaoka-Koga staging system has been the most frequently used for staging,1,2 with refinement of definitions for anatomic staging parameters proposed in 2011,3 but this staging system has now been superseded by a TNM-based classification based on data from the ITMIG retrospective database of over 8000 patients analysed by an International Association for the Study of Lung Cancer (IASLC), thymic domain, committee.4,5 The T category is dependent on extent of direct local invasion. Use of an elastic stain is strongly recommended in assessing involvement of mediastinal structures in relation to elastic layers within mediastinal and visceral pleura, fibrous layer of the pericardium and the adventitia and media of the great vessels.
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