Clear Cell Sarcoma (Malignant Melanoma) of Soft Parts: A Clinicopathologic Study of 52 Cases

Table 1

Clear cell sarcomas: clinical, histological, molecular, therapeutic, and follow-up data.

Case	Age	Sex	Site	Size (cm)	Treatment; (*)	CHT; regimens	Surgical; margin	LRec; (**)	LNMet; (**)	Met; (**)	FU; (**)	Status	EWS- ATF1	Mitoses	Necrose	Grade	BRAF15	NRAS 61
1	36	F	Buttock	6	Surg+R+CT	IVA	pos	no	no	77	80	4	pos	10	0	2	neg	neg
2	23	F	Shoulder	9	CT+Surg+R	Adri	pos	no	no	9	12	4	pos	34	1	3	neg	neg
3	11	M	Heel	4	CT+Surg+R	I	neg	no	no	23	40	4	pos	12	1	3	neg	neg
4	53	F	Foot	6	CT+Surg+R	IVA	neg	14	no	7	16	1	pos	6	1	2	neg	neg
5	57	F	Forearm	5	Surg+R		neg	23	no	no	45	0	pos	5	0	2	neg	neg
6	23	M	Foot	2,5	Surg+R+CT	MAID; MTX, C	neg	no	no	no	11	0	pos	5	0	2	neg	neg
7	34	F	Forearm	4	Surg+CT+R	Adri,I, Cispl, F, Ditic	pos	1	0	0	8	4	pos	18	1	3	ni	ni
8	20	M	Hand	4	CT+surg+R	Adri, DTIC, limb perf	neg	no	no	no	28	0	pos	10	1	2	ni	ni
9	46	F	Hand	1,2	Surg		neg	24	no	no	40	0	pos	5	1	2	ni	ni
10	54	M	Foot	3	Surg+CT	MAID; MTX, C	pos	1	no	5	24	4	pos	18	2	3	ni	ni
11	45	F	Foot	3	Surg+R		pos	no	no	no	12	?	pos	8	1	2	ni	ni
12	48	M	Foot	1,8	Surg+R		pos	no	no	no	60	0	pos	3	2	3	neg	neg
13	37	M	Foot	2	Surg+R+CT	4 E+I, D	pos	no	no	57	120	1	pos	2	0	2	ni	ni
14	33	M	Leg	4	Surg+R+CT	6 I	neg	no	87	87	96	4	pos	10	2	2	ni	ni
15	31	M	Foot	?	Surg+CT+R	INF+Adri+T/EI	neg	no	0	10	16	4	pos	20	2	3	ni	ni
16	37	F	Foot	2	Surg+R+CT	Adri+I	pos	no	no	no	87	0	pos	9	0	2	neg	neg
17	42	F	Leg	6	Surg+CT+R	Adri+I	pos	no	54	no	60	1	neg	15	1	2	neg	neg
18	51	M	Thigh	15	Surg+R+CT	Adri+I+DTIC	pos	24	no	33	60	4	pos	12	2	3	ni	ni
19	9	M	Sacrum	7	Surg+R+CT	IVA	pos	12	no	12	20	4	ni	4	1	2	ni	ni
20	31	M	Knee	10	Surg+CT+R	I+C+V+DITC	neg	no	18	no	20	4	pos	2	1	2	neg	neg
21	45	M	Knee	4	Surg+CT+R	VACA	neg	no	no	no	48	0	ni	3	2	2	ni	ni
22	29	F	Foot	3	Surg+R		neg	12	no	16	40	4	pos	9	1	2	neg	neg
23	26	M	Foot	3	Surg+CT+R	Adri+I	neg	144	no	228	312	1	pos	5	1	2	neg	neg
24	38	F	Thigh	6	Surg+CT+R	VACA	pos	12	36	52	60	4	ni	9	1	2	ni	ni
25	17	M	Behind bladder	4	Surg+CT	Adri+I	neg	no	no	no	65	0	pos	10	1	2	ni	ni
26	6	F	Arm	8	Surg+CT	V+Carbo+ epirubicin	pos	1	0	no	180	0	ni	9	1	3	ni	ni
27	15	F	Scapula	5	Surg+CT	Carbo + Adria, IL2	pos	10	no	12	24	4	ni	2–4	1	2	ni	ni
28	36	M	Tibia	3	Surg+CT	Adri+I	pos	16	20	22	28	4	ni	10	1	2	ni	ni
29	15	F	Scapula	5	Surg+CT	IVA, IL2, DTIC	neg	no	no	no	12	0	ni	4	1	2	ni	ni
30	20	F	Scalp	4	Surg+CT+R	I+Adri	pos	84	72	96	120	4	neg	2	1	2	neg	neg
31	33	F	Hand	3	Surg+CT+R	Adri	pos	48	30	36	52	4	ni	10	1	2	ni	ni
32	32	M	Pelvis	9	Surg+CT+R	I+Adri	neg	6	10	21	32	4	ni	10	1	2	ni	ni
33	12	F	Foot	3	Surg+CT+R	IVA	pos	no	no	no	144	0	ni	2	1	3	ni	ni
34	14	M	Wrist	5 à 6	Surg+CT+R	IVA	neg	no	no	no	120	0	ni	10	1	2	ni	ni
35	29	M	Heel	2	Surg+CT+R	V+DITC+Adri +C	neg	24	no	76	82	4	ni	19	1	2	ni	ni
36	28	M	Wrist	3 à 4	Surg+CT+R	IVA, IL2, DTIC	pos	no	no	24	39	4	ni	11	1	2	ni	ni
37	10	M	Hip	5,5	Surg+CT+R	IVA	pos	68	72	no	120	0	pos	18	1	2	neg	neg
38	24	M	Foot	3	Surg+R		pos	no	no	no	156	0	ni	3	1	2	neg	neg
39	13	M	Leg	3	Surg+CT+R	VACA	neg	30	30	no	348	0	ni	21	1	2	ni	ni
40	47	F	Foot	3	Surg+CT+R	VACA	neg	24	no	no	240	1	ni	12	1	2	ni	ni
41	76	F	foot	4	Surg		pos	12	no	12	42	4	ni	2	1	3	ni	ni
42	34	M	Wrist	1	Surg+CT+R	VACA	neg	56	no	no	264	0	pos	10	1	2	neg	pos
43	40	M	Leg	4	Surg+CT+R	VACA	pos	60	no	84	96	4	neg	7	2	2	neg	neg
44	12	F	Foot	14	Surg+R		neg	36	no	no	84	0	ni	5	2	2	ni	ni
45	73	M	Scapula	10	Surg+CT+R	Adri+V+C+Plat	pos	48	36	24	96	4	ni	29	2	3	ni	ni
46	20	M	Scapula	6	CT+Surg+R	Adri+V+ DTIC+C	pos	18	18	18	24	4	pos	8	1	2	neg	neg
47	33	M	Foot	5	Surg+CT	F	pos	16	4	4	31	4	pos	9	1	2	neg	neg
48	81	F	Foot	3	Surg		pos	240	no	250	254	4	ni	9	2	2	ni	ni
49	46	M	Foot	4	Surg+CT+R	I	neg	60	72	250	260	4	pos	24	2	3	pos	neg
50	47	M	Hand	6	Surg+CT+R	VACA	neg	no	no	96	144	4	pos	15	2	2	neg	neg
51	29	F	Foot	2	Surg		pos	no	60	no	60	1	ni	10	2	2	ni	ni
52	30	M	knee	10	Surg+CT+R	C+V+ Adri+DTIC	pos	no	6	18	36	4	pos	5	2	2	neg	neg

((*) Surg: surgery, R: radiotherapy, CT: chemotherapy. (**) LRec: local recurrence (months), LNMet: locoregional lymph node metastasis (months), Met: distant metastasis (months), and FU: followup (months). Status: 0: alive with no disease, 1: alive with disease, and 4: died of disease. Mitoses: number of mitoses per ten high power fields. neg: negative, pos: positive, ni: not identified. Necrosis: 0: no necrosis, 1: less than 50% of necrosis, 2: more than 50% of necrosis). A: actinomycin D; Adri: Adriblastina, C: cyclophosphamide, Carbo: carboplatin, cisplt: cisplatin; DITIC: dacarbazine, EPI: eoposide, F: fotemustine; I: ifosfamide, IL2: Interleukin 2, IVA: ifosfamide (3 g/m²) + dactinomycin (1,5 mg/m²) + vincristine (1,5 mg/m²); Limb perf: regional isolated limb perfusion with melphalan and tumor necrosis factor alpha, MTX: methotrexate, MAID: doxorubicin (15 mg/m²) + dacarbazine (250 mg/m²/j) + ifosfamide (2–2,85 g/m²/j), V: vincristine; VACA: cyclophosphamide (1,2 g/m²) + doxorubicin (30 mg/m²) + dactinomycin (0,5 mg/m²) + vincristine 1,5 mg/m²; INF: intrferon; T: taxol.