Table 2 Clinical characteristics of mast cell sarcoma.

From: Mast cell sarcoma: clinicopathologic and molecular analysis of 10 new cases and review of literature

 

Our study (n = 10)

Literature (n = 24)

Combined (n = 34)

Age, Median (years)

54.5 (1–63)

39 (4–77), n = 23

39 (1–77; n = 33)

Gender

5 M, 5 F

13 M, 11 F

18 M, 16 F

Locationsa

  Bone

9/10

12/24

21/34 (62%)

  GI

0/10

7/24

7/34 (21%)

  Soft tissue

2/10

3/24

5/34 (15%)

  Skin

0/10

3/24

3/34 (9%)

  Spleen

2/10

1/24

3/34 (9%)

  Other locations

Brain (1), liver (1), lymph node (1), lung (1)

Peritoneum (3), nasal cavity (2), brain (1), liver (1), lung (1), mid ear (1), subglottis and larynx (1), tonsil (1), uterus/ovaries (1)

Peritoneum (3), brain (2), liver (2), lung (2), nasal cavity (2), lymph node (1), mid ear (1), tonsil (1), uterus/ovaries (1), subglottis and larynx (1)

 History of SM

1/9

6/21

7/30 (23%)

 B-symptoms

1/9

1/8

2/17 (12%)

 MCAS

3/9

7/13

10/22 (45%)

 Leukemia transformation

2/9

5/21

7/30 (23%)

 BM involvement by MCS

3/9

5/17

8/26 (31%)

 BM involvement by SM

1/9

3/17

4/26 (15%)

 Anemia

2/7

8/12

10/19 (53%)

WBC count

  Normal

4/6

8/12

12/18 (67%)

  Leukocytosis

2/6

2/12

4/18 (22%)

  Leukopenia

0/6

2/12

2/18 (11%)

 Platelet count

  Normal

5/7

7/10

12/17 (71%)

  Thrombocytosis

0/7

3/10

3/17 (18%)

  Thrombocytopenia

2/7

0/10

2/17 (12%)

 Elevated serum tryptase, median and range (ng/ml)

6/7 132 (34.1–1034)

11/16 200 (18–400)

17/23 (74%) 200 (18–1034)

 Chemotherapy

7/8

16/19

23/27 (85%)

 Radiotherapy

4/8

7/18

11/26 (42%)

Response to therapy

  CR

0/6

3/17

3/23 (13)

  PR

0/6

4/17

4/23 (17%)

  No

6/6

10/17

16/23 (70%)

 Disease progression (yes)

4/6

13/17

17/23 (74%)

 Stem cell transplant

0/7

1/19

1/26 (4%)

 Death

4/8

12/23

16/31 (52%)

 Median follow up (months)

5 (2–35)

13 (2–81)

12 (2–81)

 Median OS (months)

  

24

  1. CR complete remission, GI gastrointestinal tract, MCAS mast cell activation symptoms, OS overall survival, PR partial response, SM systemic mastocytosis.
  2. aSome patients had tumor involvement in multiple locations.