Table 1.

A Summary of 18 Cases Reported Metastatic Inflammatory Myofibroblastic Tumors in Recent 10 Years

Age (Years)/Sex	Presentation	Tumor Primary Site	IHC	Gene Mutation	Recurrence	Tumor Metastasis Site	Treatment	Follow-up and Prognosis	Reference
55/F	A sudden slurred speech	Nasopharynx	Highly expressed PD-L1	Tertiary mutations in NSD1 and SOX9 genes	1 month	Skull base, slope and pterygoid sinus	Surgery, radiotherapy and immunotherapy (PD-L1 inhibitor Sintilimab)	Better than before to date	This case
19/M	Macroscopic hematuria and progressive anemia	Bladder	ALK pos	NA	7 months	Lung and left iliac bone and	Surgery and targeted therapy	Complete remission	Bonvini et al, 20218
16/M	NR	Right chest wall with pleural involvement	ALK neg, highly expressed PD-L1	TFG-ROS1 fusion, an acquired G2032R mutation in the TKD of ROS1 and MAPK1 amplification	Continuing progression	Brain, right triceps	Antiinflammatory therapy, chemotherapy, targeted therapy and immunotherapy (PD-L1 inhibitor Nivolumab)	Died of respiratory complications	Carcamo et al, 20219
40/M	Dyspnoea and productive cough	Upper lobe of right lung	ALK pos	TPM4-ALK fusion	Transient improvement and continuing progression	Hilar lymph nodes, right trapezius muscle, left frontal lobe, left adrenal, left gluteal	Chemotherapy, radiotherapy and targeted therapy	Dead	Wong et al, 202010
57/F	Tightening sensation around the retrosternal region	Anterior mediastinum with left pleural invasion	ALK neg	6 germline mutations (PARP1 p.V69I, ATR p.S1007N, GRM8 p.T97A, MLLT10 p.G409R, TCF7L2 p.N185S, SMARCA4 p.A321T) and 1 somatic mutation (TSHR p.Q720H)	Recurring at the left anterior mediastinum after nine months post first surgery, and at the left anterior mediastinum and right anterior pleural space at the age of 65	Left anterior mediastinum and right anterior pleural space	Surgery and radiotherapy	NR	Hou et al, 202011
59/M	Consistent fatigue	Medium lobe of right lung	ALK neg	NA	Continuing progression	Bone and abdominal cavity	Targeted therapy	Stable condition on follow-up 9 months	Liu et al, 201912
81/M	Anal pain	The posterior rectal wall	ALK neg	NA	2 months after surgery	Liver	Surgery	Dead	Shimodaira et al, 202013
76/M	Upper back pain and motor weakness	Upper lobe of right lung	ALK neg	NA	NA	Right renal hilum	Hormonotherapy and radiotherapy	Improvement in symptoms on follow-up 1 month	Na et al, 201814
55/M	Left pneumonia	Lower lobe of left lung	NA	NA	3 months after first surgery	Lingula, lung and liver	Surgery and radiotherapy	The patient was referred to another oncological center.	Rodrigues et al, 201715
37/F	Cough and stridor	Upper lobe of left lung	ALK pos	NA	1 year after first surgery	Uterine	Surgery	Dead 1 year after second surgery	Zhang et al, 201816
18/F	Headaches	Lung	ALK pos	ALK-1 gene rearranged	Continuing progression	Brain	Targeted therapy	Alive and well on follow-up 2.5 years since primary diagnosis	Yuan et al, 201717
43/F	Heart failure symptoms	Small intestinal	ALK neg	NA	1 year	Left ventricle, stomach, liver, vertebra, and pelvic bones	Surgery and chemotherapy	Dead 9 months after surgery	Zorinas et al, 201718
16/F	A localized left shoulder mass around the subacromial region	Left shoulder	ALK pos	EML4-ALK translocation	45 months	Left clavicle, the arm, and the anterior chest wall soft tissues	Surgery, chemotherapy, radiotherapy and targeted therapy	Remains in complete remission on follow-up 3 years	Gaudichon et al, 201619
49/F	Cough	Lower lobe of left lung	ALK pos	ALK-gene rearrangement	4 months	Right anterior-end of third-rib and right adrenal gland	Surgery and targeted therapy	NR	Sethi et al, 201520
28/F	Post-prandial abdominal pain	Abdominal extensive solid masses involving multiple viscera	ALK neg	NA	Continuing progression	Vertebral body, liver and peritoneum	Surgery and chemotherapy	Dead	Kim et al, 201521
27/F	A painless palpable mass in the upper outer quadrant of the right breast	Right breast	ALK neg	NA	2 years	The upper inner quadrant of the right breast and right cervical area	Surgery	NR	Choi et al, 201522
36/M	Hematochezia, tenesmus, and constipation	Rectum	ALK pos	NA	18 months	The pelvic floor muscles, sacrococcyx, pre-sacral fascia	Surgery and chemotherapy	Follow-up every 6 months and disease-free	Sun et al, 201423
26/M	Chronic nonproductive cough	Mediastinum	ALK neg	NA	Continuing progression	Lymph nodes and the thoracic vertebra	Hormonotherapy and chemotherapy	No radiological evidence of tumor progression or recurrence for 7 months	Kubo et al, 201224
52/M	Dyspnea and cough	Upper lobes of bilateral lung	NA	NA	No	Left adrenal gland	Surgery	Alive and well, without recurrence on follow-up 1 year	Carillo et al, 201125

Abbreviations: ALK, anaplastic lymphoma kinase; F, female; M, male; NA, not available; neg, negative; NR, not reported; pos, positive.