Hodgkin lymphoma involving extranodal sites in head and neck: report of twenty-nine cases and review of three-hundred and fifty-seven cases

Figures & data

Table 1. Patient’s characteristic of our institution.

Characteristic of 29 cases	Total	Percentage
Gender
Male	22	76
Female	7	24
Age	33 (median)	–
Stage I	7	24
Stage II	18	62
Stage III–IV	4	14
Anatomical site
Nasopharynx	10	34.4
Tonsil	9	31
Parotid	5	17.2
Mandible	2	7
Others (occipital, thyroid, oropharynx)	3	10
Pathology type	29	100
Nodular sclerosis	12	41.4
Mixed cellularity	8	27.6
Lymphocyte rich	3	10.3
Nodular lymphocyte predominant	4	13.8
Classical HL	2	7
Treatment
Chemotherapy alone	9	3
Chemotherapy + Radiation	7	24
Surgery + chemotherapy + Radiation	6	21
Radiation alone	6	21
Radiation + chemotherapy	1	3
Treatment outcome
Complete response	27	93
Partial response /progressive disease	2	7
Relapsed after complete remission	4	14
All treatment failures	6	20

Figure 1. A – Overall and progression-free survival of 29 patients. B – Disease-specific survival results from the literature review.

Table 2. Characteristics of 357 patients.

Characteristic of 357 cases	Total	Percentage
Gender
Male	199	55.7
Female	131	36.7
Unknown	27	7.6
Age – available in	247	69
Median	45 years	-
Age – unknown	35	10
Age as – adult/aged/mid-age in	20	5.6
Age as – range / median in	55	15.4
Stage
Stage I	125	35
Stage I–II	47	13
Stage II	114	32
Stage III	21	6
Stage III–IV	11	3
Stage IV	19	5.3
Stage unknown	20	5.6
Pathology type
Mixed cellularity	99	27.7
Nodular sclerosis	88	24.6
HL – Unknown type / not specified	63	17.6
Nodular lymphocyte predominant	56	15.7
Lymphocyte rich	25	7.0
Classical / NOS	16	4.5
Lymphocyte depleted	7	2.0
HL – interfollicular	3	0.8
Anatomical sites
Nasopharynx	109	30.5
Tonsil	67	18.8
Parotid	58	16.2
Thyroid	45	12.6
Oral (mucosa, oropharynx, cavity)	15	4.2
Adenoid	10	2.8
Mandible	10	2.8
Orbit	7	2.0
Skin	6	1.7
Tongue	6	1.7
Pharynx	5	1.4
Waldeyer ring	5	1.4
Palate	4	1.1
Others^a	10	2.8

^a (Others = maxilla (2), sinus (2) and 1 each of cheek, ear-mastoid, maxilla- alveolar, nose, occipital mass, soft tissue-neck).

Table 3. Treatment and outcome of 357 patients.

Treatment
Unknown	124	34.7
Chemotherapy + radiation	56	15.7
Radiation	56	15.7
Chemotherapy	41	11.5
Surgery + radiation	23	6.4
Surgery alone	15	4.2
Surgery + chemotherapy + radiation	15	4.2
Radiation + chemotherapy	11	3.1
Surgery + chemotherapy	10	2.8
Surgery + radiation + chemotherapy	4	1.1
No treatment	2	0.6
Treatment outcome
Complete remission	209	58.5
Unknown	133	37.3
Progressive disease	15	4.2
Survival / follow-up
Survival status unknown	151	42.3
Total alive	176	49.3
Total died	30	8.4
Total died of disease	21	6
Total died of other causes (only 1 had a relapsed disease)	9	2.5
Relapsed or treatment failure (out of 206 patients)	54	26

Table 4. Pathological distribution of HL at various anatomical sites in 304 patients.

		Anatomical site
Pathology		Nasopharynx	Parotid	Tonsil	Thyroid	Oral-oropharynx	Mandible	Adenoid	Others	Total
classical Hodgkin lymphoma	Count	5	1	1	1	3	1	0	4	16
	% within pathology	31.3%	6.3%	6.3%	6.3%	18.8%	6.3%	0	25.0%	100.0%
	% within Site	5.7%	1.8%	1.9%	2.6%	20.0%	10.0%	0	11.1%	5.3%
	Count	15	9	4	9	5	2	1	11	56
Hodgkin lymphoma	% within pathology	26.8%	16.1%	7.1%	16.1%	8.9%	3.6%	1.8%	19.6%	100%
	% within Site	17.0%	16.1%	7.7%	23.1%	33.3%	20.0%	12.5%	30.6%	18.4%
Lymphocyte depleted	Count	1	2	2	0	0	1	0	0	6
	% within pathology	16.7%	33.3%	33.3%	0	0	16.7%	0	0	100%
	% within Site	1.1%	3.6%	3.8%	0	0	10.0%	0	0	2.0%
	Count	9	25	5	0	1	1	1	6	48
Nodular lymphocyte predominant	% within pathology	18.8%	52.1%	10.4%	0	2.1%	2.1%	2.1%	12.5%	100%
	% within Site	10.2%	44.6%	9.6%	0	6.7%	10.0%	12.5%	16.7%	15.8%
Lymphocyte rich	Count	8	1	8	0	1	1	5	1	25
	% within pathology	32.0%	4.0%	32.0%	0	4.0%	4.0%	20.0%	4.0%	100%
	% within Site	9.1%	1.8%	15.4%	0	6.7%	10.0%	62.5%	2.8%	8.2%
	Count	34	7	23	5	4	1	1	7	82
Mixed cellularity	% within pathology	41.5%	8.5%	28.0%	6.1%	4.9%	1.2%	1.2%	8.5%	100%
	% within Site	38.6%	12.5%	44.2%	12.8%	26.7%	10.0%	12.5%	19.4%	27.0%
Nodular sclerosis	Count	16	11	9	24	1	3	0	7	71
	% within pathology	22.5%	15.5%	12.7%	33.8%	1.4%	4.2%	0	9.9%	100%
	% within Site	18.2%	19.6%	17.3%	61.5%	6.7%	30.0%	0	19.4%	23.4%
Total	Count	88	56	52	39	15	10	8	36	304
	% within pathology	28.9%	18.4%	17.1%	12.8%	4.9%	3.3%	2.6%	11.8%	100%
	% within Site	100%	100%	100%	100%	100%	100%	100%	100%	100%

Table 5. Summary of 357 patient’s characteristics and their treatment outcome

	Various sites and their characteristics (percentage)
Characteristics	Nasopharynx	Tonsil	Parotid	Thyroid	Adenoid	Mandible	Others	Total
Total	109 (30.5)	67 (18.8)	58 (16.2)	45 (12.6)	10 (2.8)	10 (2.8)	58 (16.2)	357 (100)
Female	57 (52.3)	17 (25.4)	21 (36.2)	14 (31.1)	2 (20)	1 (10)	19 (32.8)	131 (36.7)
Male	47 (43.1)	44 (65.7)	30 (51.7)	27 (60)	8 (80)	7 (70)	36 (62.1)	199 (55.7)
Gender unknown	5 (4.6)	6 (9)	7 (12.1)	4 (8.9)	–	2 (20)	3 (5.2)	27 (7.6)
Stage I–II	63 (58)	67 (100)	58 (100)	45 (100)	–	–	53 (91.4)	286 (80.1)
Stage III–IV	46 (42)	0	0	–	–	2 (8)	3 (5.2)	51 (14.3)
Stage unknown	–	0	0	–	10 (100)	8 (80)	2 (3.4)	20 (5.6)
Treatment groups
Chemotherapy + Radiation	–	31 (46.3)	6 (10.3)	19 (42.2)	10 (100)	0	20 (34.5)	86 (24.1)
Chemotherapy alone	–	36 (53.7)	9 (20)	9 (20)	0	0	6 (10.3)	51 (14.3)
Radiation alone	42 (38.5)	0	–	–	0	10 (100)	4 (6.9)	79 (22.1)
Surgery alone	–	0	15 (33.3)	15 (33.3)	0	0	–	15 (4.2)
Unknown / others	67 (61.5)	0	2 (4.4)	2 (4.4)	0	0	28 (48.3)	126 (35.3)
Response – CR	57 (52.3)	39 (58.2)	39 (67.2)	35 (77.8)	6 (60)	5 (50)	37 (64)	208 (58.3)
Response – No CR	2 (1.8)	2 (3)	5 (8.6)	–	–	2 (20)	14 (24)	14 (3.9)
Response – Unknown	50 (45.9)	26 (38.8)	(24.1)	10 (22.2)	4 (40)	3 (30)	7 (12)	135 (37.8)
Treatment failure – No	45 (41.3)	29 (43.3)	30 (51.7)	28 (62.2)	3 (30)	–	18 (31)	153 (42.9)
Treatment failure – Yes	7 (6.4)	12 (17.9)	13 (22.4)	7 (15.6)	1 (10)	7 (70)	(12.1)	54 (15.1)
Treatment failure – Unknown	57 (52.3)	26 (38.8)	15 (25.9)	10 (22.2)	6 (60)	3 (30)	33 (56.9)	150 (42)