Treatment algorithm

Please note that formulations/routes and doses may differ between drug names and brands, drug formularies, or locations. Treatment recommendations are specific to patient groups: see disclaimer

ACUTE

resectable tumour

1st line – surgery

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ACUTE
|
resectable tumour
1st line – 

surgery

Resectability depends on tumour size, edge characteristics, apparent encapsulation, and symptoms (absence of symptoms favours an early-stage tumour).

Clinically encapsulated thymomas are resected for both pathological diagnosis and treatment.[43] Total thymectomy (resection of the entire thymus gland) is recommended to reduce risk of recurrence, although thymomectomy with wide margins may be sufficient. There is growing data indicating that, in specialised centres with experienced surgeons, a minimally-invasive approach for non-invasive thymoma is safe and can achieve the same oncological outcomes compared to open surgery.[51][52]​​[53][54][55][56][57][58][59]​​​​​​ Macroscopically tumour-free resection margins must be achieved. Operative morbidity and mortality are generally very low.

Consider – postoperative radiotherapy

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ACUTE
|
resectable tumour
Consider – 

postoperative radiotherapy

Additional treatment recommended for SOME patients in selected patient group

The pathology report together with the operating surgeon's impression determine the need for adjuvant mediastinal radiotherapy.

Masaoka-Koga stage I tumours (encapsulated tumour with no evidence of invasion) are almost invariably completely resected and do not require any adjuvant therapy (if negative margins are obtained).

Masaoka-Koga stage II tumours (microscopic or macroscopic invasion, but not invading through the mediastinal pleura or pericardium) are also almost always completely resected. Adjuvant radiotherapy can be considered for some Masaoka-Koga stage II tumours, particularly those with higher risk features such as high grade, close margins, or adherence to the pericardium.[62][63] The benefit of adjuvant radiation for Masaoka-Koga stage II tumours with a negative margin is controversial.

Masaoka-Koga stage III tumours (invasion into local structures) are more likely to be incompletely resected and generally have close resection margins, even when officially pathologically 'negative'. Most patients with Masaoka-Koga stage III tumours are, therefore, referred for adjuvant radiation regardless of the official margin status and whether or not they received neoadjuvant chemotherapy.[64]​ Randomised evidence investigating the benefit of radiotherapy is lacking. Standard doses range from 45-60 Gy in the postoperative setting and 60-66 Gy in the definitive setting.[65]​ Elective nodal radiation is not standard.

Plus – medical optimisation pre-surgery

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ACUTE
|
resectable tumour
|
with myasthenia gravis
Plus – 

medical optimisation pre-surgery

Treatment recommended for ALL patients in selected patient group

Patients with myasthenia gravis should have formal evaluation with neurology. Patients must be medically optimised before undergoing surgery because the stress of surgery can precipitate a myasthenic crisis, leading to respiratory failure.[85]

As thymoma surgery is always elective, there is sufficient time to medically optimise status. See Myasthenia gravis (Treatment approach).

locally advanced tumour

1st line – preoperative chemotherapy ± radiotherapy

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ACUTE
|
locally advanced tumour
1st line – 

preoperative chemotherapy ± radiotherapy

Once a tissue diagnosis of a locally advanced thymoma or thymic carcinoma is made, neoadjuvant chemotherapy is usually given if the tumour is thought to be resectable.[66][67][68][69]​ Definitive chemoradiotherapy may be used if the tumour is deemed unresectable from the start or after a trial of neoadjuvant chemotherapy. Occasionally, neoadjuvant chemotherapy plus radiotherapy is performed, largely on the basis of institutional preference.[71]

Commonly utilised neoadjuvant chemotherapy regimens include: cisplatin plus doxorubicin plus cyclophosphamide; cisplatin plus etoposide; cisplatin plus vincristine plus doxorubicin plus cyclophosphamide; and carboplatin plus paclitaxel (especially for patients who are not candidates for cisplatin because of baseline hearing loss, renal insufficiency, or other comorbidities).[63][69]​​​[70]​​​​ Response rates to neoadjuvant chemotherapy range from 40% to 100%, and pathological complete responses have been observed in 6% to 40%. Complete resection is achievable in 20% to 80% of patients who receive induction chemotherapy.

Occasionally, neoadjuvant chemotherapy plus radiotherapy is performed largely on the basis of institutional preference.[71]

Typically, patients receive 2 to 6 cycles of neoadjuvant therapy before surgery. Patients are then re-evaluated for resection following repeat chest CT.

See local specialist protocol for dosing guidelines.

Primary options

cisplatin

and

doxorubicin

and

cyclophosphamide

OR

cisplatin

and

etoposide

OR

cisplatin

and

vincristine

and

doxorubicin

and

cyclophosphamide

OR

carboplatin

and

paclitaxel

Plus – re-assessment followed by surgery or radiotherapy

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ACUTE
|
locally advanced tumour
Plus – 

re-assessment followed by surgery or radiotherapy

Treatment recommended for ALL patients in selected patient group

After a course of neoadjuvant therapy, patients are re-evaluated for resection following repeat chest CT. Most will be able to undergo resection at this stage.

However, some locally advanced thymic tumours remain unresectable following neoadjuvant chemotherapy. In cases where the disease is confined within a reasonable radiation portal, definitive thoracic radiotherapy can lead to prolonged progression-free survival.[64][72]

Consider – postoperative radiotherapy or chemoradiotherapy

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ACUTE
|
locally advanced tumour
Consider – 

postoperative radiotherapy or chemoradiotherapy

Additional treatment recommended for SOME patients in selected patient group

After resection, the majority of patients will receive adjuvant mediastinal radiotherapy or combination postoperative radiotherapy and chemotherapy, as some unresectable or partially resected tumours may benefit from a combined approach.

Plus – medical optimisation pre-surgery

Back
ACUTE
|
locally advanced tumour
|
with myasthenia gravis
Plus – 

medical optimisation pre-surgery

Treatment recommended for ALL patients in selected patient group

Patients must be medically optimised before undergoing surgery because the stress of surgery can precipitate a myasthenic crisis, leading to respiratory failure.[85]

As thymoma surgery is always elective, there is sufficient time to medically optimise status. See Myasthenia gravis (Treatment approach).

ONGOING

recurrent tumour

1st line – chemotherapy or other systemic therapies

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ONGOING
|
recurrent tumour
1st line – 

chemotherapy or other systemic therapies

The most important factors associated with recurrence include stage, WHO histology, and completeness of resection.

Thymoma tends to relapse in the chest, usually in the pleura (locoregional 87%; distant 13%), whereas thymic carcinoma tends to relapse at distant sites, particularly the lung, bone, brain, and liver (distant 60%; locoregional 40%).[8][12][13][73]

Distant recurrences are usually treated with chemotherapy. Active chemotherapy agents include cisplatin, paclitaxel, etoposide, cyclophosphamide, doxorubicin, vincristine, ifosfamide, pemetrexed, and gemcitabine with or without capecitabine.[75][76]​ Combination chemotherapy may also be utilised, and this may result in higher response rates.

Targeted therapies and anti-angiogenic agents are also options in the recurrent setting, including everolimus, sunitinib, and lenvatinib.[77][78][79] Octreotide, a somatostatin analogue, is an alternative systemic therapy in recurrent thymoma.

Pembrolizumab may be considered in patients with thymic carcinoma, however, it should be used cautiously due to the higher risk of immune-related adverse events.[63][83][84] If used, it is often in the heavily-pretreated setting when other modalities may be limited, and after a thorough risk-benefit discussion is had with the patient. It is avoided in patients with thymoma.

See local specialist protocol for dosing guidelines.

Primary options

cisplatin

OR

paclitaxel

OR

etoposide

OR

cyclophosphamide

OR

doxorubicin

OR

vincristine

OR

ifosfamide

OR

pemetrexed

OR

gemcitabine

OR

gemcitabine

and

capecitabine

OR

everolimus

OR

sunitinib

OR

lenvatinib

OR

octreotide

OR

pembrolizumab

Consider – surgery and/or radiotherapy

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ONGOING
|
recurrent tumour
Consider – 

surgery and/or radiotherapy

Additional treatment recommended for SOME patients in selected patient group

Most locoregional recurrences are treated in a multimodal fashion; resection, chemotherapy, and radiation are each often important components of therapy.[67][80][81][82]​ Surgery is generally appropriate for disease that appears completely resectable.

Plus – medical optimisation pre-surgery

Back
ONGOING
|
recurrent tumour
|
with myasthenia gravis
Plus – 

medical optimisation pre-surgery

Treatment recommended for ALL patients in selected patient group

Patients with myasthenia gravis should have formal evaluation with neurology. Patients must be medically optimised before undergoing surgery because the stress of surgery can precipitate a myasthenic crisis, leading to respiratory failure.[85]

As thymoma surgery is always elective, there is sufficient time to medically optimise status. See Myasthenia gravis (Treatment approach).

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Please note that formulations/routes and doses may differ between drug names and brands, drug formularies, or locations. Treatment recommendations are specific to patient groups. See disclaimer

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