Risk Factors for Malignancy of Pheochromocytoma and Abdominal Paraganglioma in Children: Clinicopathologic Perspectives

Jihoon Chang; Soo-Hong Kim; Hye Sook Min; Hyun-Young Kim; Sung-Eun Jung; Kwi-Won Park; Seong-Cheol Lee

doi:10.13029/jkaps.2013.19.2.108

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Risk Factors for Malignancy of Pheochromocytoma and Abdominal Paraganglioma in Children: Clinicopathologic Perspectives

Jihoon Chang, M.D., Soo-Hong Kim, M.D., Hye Sook Min, M.D.^∗, Hyun-Young Kim, M.D., Sung-Eun Jung, M.D., Kwi-Won Park, M.D., Seong-Cheol Lee, M.D.

Journal of the Korean Association of Pediatric Surgeons 2013;19(2):108-121.
Published online: December 17, 2013

DOI: https://doi.org/10.13029/jkaps.2013.19.2.108

Department of Pediatric Surgery, Seoul National University Children's Hospital, Seoul, Korea

^∗Department of Pathology, Seoul National University Hospital, Seoul, Korea

Correspondence Seong-Cheol Lee, M.D. Department of Pediatric Surgery, Seoul National University Children's Hospital 101, Daehang-Ro Jongro-Gu, Seoul 110-744, Korea Tel : 02)2072-2338, Fax : 02)747-7730 E-mail: leesc@snu.ac.kr

• Received: September 27, 2013

This is an Open Access article distributed under the terms of the Creative Commons Attribution Non-Commercial License (http://creativecommons.org/licenses/by-nc/3.0/) which permits unrestricted non-commercial use, distribution, and reproduction in any medium, provided the original work is properly cited.

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Figure.

The Kaplan-Meier survival curves between benign and malignant PHEO and aPGL (p = .007)

Table 1.

Immunohistochemical Panel

Table 1.
Antibody	Primary antibody^∗	Company	Dilution	Buffer(pH)^†	Target Stain^‡	Cutoff Values^§
Ki-67	mm	DAKO, Carpinteria, California, USA	1:200	CA (6)	N	> 2 %
p53	mm	DAKO	1:200	CA (6)	N	> 5 %
mdm-2	mm	Leica Biosystems, Heidelberg, Germany	1:100	EDTA (9)	N	> 50 %
mdm-2		Santa Cruz
p21	mm	biotechnology, Dallas,	1:200	CA (6)	N	> 10 %
		Texas, USA
		Spring Bioscience,
p27	rp	Pleasaton, California,	1:300	CA (6)	N	> 30 %
p27		USA
bcl-2	mm	DAKO	1:100	CA (6)	C	> 50 %
cyclin D1	rp	Santa Cruz biotechnology	1:100	CA (6)	N	> 5 %

^∗mm, mouse monoclonal; rp, rabbit polyclonal

^†CA, citric acid; EDTA, Ethylenediaminetetraacetic acid

^‡N, Nuclei stain; C, Cytoplasmic stain

^§indicates high proliferative group in Ki-67; otherwise indicates group showed over-expression

Table 2.

Clinical Information of the Patients with PHEO and aPGL

Table 2.
	Total (n=20)	PHEO (n=14)	aPGL (n=6)	P value
Age (months, mean±SD)	143.90 ±40.87	141.93 ±39.26	148.50 ±19.60	.775^∗
Gender (M : F)	15 : 5	9 : 5	6 : 0	.260^†
Location				.829^†
Right (%)	7 (35)	5 (35.7)	2 (33.3%)
Left (%)	6 (30)	5 (35.7)	1 (16.7%)
Bilateral (%)	7 (35)	4 (28.6)	3 (50.0%)
Greatest dimension (cm, mean±SD)	4.86 ±1.50	4.84 ±1.26	4.88 ±2.08	.966^†
Malignancy (%)	7 (35)	4 (28.6)	3 (50.0%)	.613^†
Hereditary Features (%)	4 (20)	4 (28.6)	0	.267^†
Chemotherapy (%)	2 (10)	0	2 (33.3%)	.079^†
Radiotherapy (%)	6 (30)	3 (21.4)	3 (50.0%)	.303^†
Follow-up(months, mean±SD)	97.25 ±55.67	95.64 ±37.03	101.00 ±90.47	.850^∗
Disease free survival (months, mean±SD)	82.20 ±58.25	82.43 ±43.86	81.67 ±88.82	.985^∗
Median survival (months, range)	86.20 (14-252)	86.50 (50-159)	82.50 (14-252)	NA^§

^∗Student's t-test

^†Fisher's exact test

^§Not applicable

Table 3.

Clinical Information of the Patients with Benign and Malignant Tumors^∗

Table 3.
	Benign (n=13)	Malignant (n=7)	P value
Age (months, mean±SD)	143.77 ± 46.25	144.14 ± 31.78	.983^†
Gender (M : F)	10 : 3	5 : 2	> .999^‡
Hereditary Features (%)	3 (23.1)	1 (14.3)	> .999^‡
Chemotherapy (%)	0	2 (28.6)	.111^‡
Radiotherapy (%)	0	6 (85.7)	< .001^‡
Location			.381^‡
Right (%)	5 (38.5)	2 (28.6)
Left (%)	5 (38.5)	1 (14.3)
Bilateral (%)	3 (23.1)	4 (57.1)
Greatest dimension (cm, mean±SD)	5.01 ± 1.55	4.57 ± 1.44	.547^†
Clinical manifestation
Hypertension (%)	10 (76.9)	4 (57.1)	.613^‡
Palpitation (%)	7 (53.8)	3 (42.9)	> .999^‡
Headache (%)	6 (46.2)	2 (28.6)	.642^‡
Diaphoresis (%)	7 (53.8)	2 (28.6)	.374^‡
Flushing (%)	9 (69.2)	2 (28.6)	.160^‡
Nausea, vomiting (%)	7 (53.8)	3 (42.9)	> .999^‡
Follow-up (months, mean±SD)	90.38 ± 39.48	110.00 ± 80.02	.467^†
Disease free survival (months, mean±SD)	90.38 ± 39.48	67.00 ± 84.92	.690^†
Median survival period (months, range)	93.00 (14-159)	80.00 (22-252)	NA^§

^∗Analysis of all patients with PHEO and aPGL

^†Student's t-test

^‡Fisher's exact test

^§Not applicable

Table 4.

Microscopic Features of Benign and Malignant Tumors^∗

Table 4.
	Benign (n=13)	Malignancy (n=7)	P value^†
Capsular invasion (n, %)	4 (30.8)	3 (42.9)	.651
Vascular invasion (n, %)	0	4 (57.1)	.007
Extension into adipose tissue (n, %)	1 (7.69)	4 (57.1)	.031
Presence of large nests (n, %)	7 (53.8)	1 (14.3)	.158
Central tumor necrosis (n, %)	6 (46.2)	5 (71.4)	>.999
High cellularity (n, %)	3 (23.1)	2 (28.6)	>.999
Tumor cell spindling (n, %)	8 (61.5)	1 (14.3)	.070
Cellular monotony (n, %)	3 (23.1)	1 (14.3)	>.999
Mitosis (n, %)	1 (7.69)	4 (57.1)	.031
Atypical mitosis (n, %)	1 (7.69)	2 (28.6)	.270
Nuclear pleomorphism (n, %)	4 (30.8)	0	.249
Nuclear hyperchromasia (n, %)	9 (69.2)	2 (28.6)	.160

^∗Analysis of all patients with PHEO and aPGL

^†Fisher's exact test

Table 5.

Microscopic Features of Benign and Malignant PHEO^∗

Table 5.
	Benign (n=10)	Malignancy (n=4)	P value^†
Capsular invasion (n, %)	3 (30)	2 (50)	.580
Vascular invasion (n, %)	0	3 (75)	.033
Extension into adipose tissue (n, %)	0	3 (75)	.003
Presence of large nests (n, %)	7 (70)	0	.023
Central tumor necrosis (n, %)	5 (50)	4 (100)	.089
High cellularity (n, %)	3 (30)	1 (25)	.857
Tumor cell spindling (n, %)	7 (70)	1 (25)	.139
Cellular monotony (n, %)	3 (30)	0	.234
Mitosis (n, %)	1 (10)	3 (75)	.019
Atypical mitosis (n, %)	0	1 (25)	.114
Nuclear pleomorphism (n, %)	3 (30)	0	.234
Nuclear hyperchromasia (n, %)	8 (80)	0	.008

^∗Analysis of patients with PHEO only

^†Fisher's exact test

Table 6.

Analysis of PASS Score and Pathologic Score between Benign and Malignant Tumor^∗

Table 6.
	Benign (n=13)	Malignancy (n=7)	P value
PASS Score (mean±SD)	6.00 ± 2.86	7.57 ± 3.36	.317^†
PASS ≥ 4 (n, %)	10 (76.9)	5 (71.4)	>.999^‡
PASS ≥ 6 (n, %)	8 (61.5)	5 (71.4)	>.999^‡
Pathologic Score (mean±SD)	0.31 ± 0.86	3.43 ± 2.57	.001^†
Score < 2 (n, %)	12 (92.3)	2 (28.5)	.007^§
Score ≥ 2 (n, %)	1 (7.7)	5 (71.4)

^∗Analysis of all patients with PHEO and aPGL

^†Student's t-test

^‡Mann-Whitney test

^§Fisher's exact test

Table 7.

Immunohistochemistry^∗

Table 7.
Antibody	Benign (n=13)	Malignancy (n=7)	P value^†
Ki-67 (n, %)	2 (15.4)	4 (57.1)	.122
p53 (n, %)	2 (15.4)	3 (42.9)	.290
bcl-2 (n, %)	8 (61.5)	4 (57.1)	>.999
mdm-2 (n, %)	2 (15.4)	3 (42.9)	.290
cyclin D1 (n, %)	0	0	>.999
p21 (n, %)	3 (23.1)	3 (42.9)	.613
p27 (n, %)	2 (15.4)	3 (42.9)	.290

^∗Number of cases above cutoff values which are listed in Table 1.

^†Fisher's exact test

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12. Nagura S, Katoh R, Kawaoi A, Kobayashi M, Obara T, Omata K. Immunohistochemical estimations of growth activity to predict biological behavior of pheochromocytomas. Modern pathology: an official journal of the United States and Canadian Academy of Pathology, Inc 12(12):1107. 1999.
13. Elder EE, Xu D, Höög A, Enberg U, Hou M, Pisa P, Gruber A, Larsson C, Bäckdahl M. KI-67 and hTERT expression can aid in the distinction between malignant and benign pheochromocytoma and paraganglioma. Modern pathology 16(3):246-255. 2003.
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Risk Factors for Malignancy of Pheochromocytoma and Abdominal Paraganglioma in Children: Clinicopathologic Perspectives

J Korean Assoc Pediatr Surg. 2013;19(2):108-121. Published online December 17, 2013

DOI: https://doi.org/10.13029/jkaps.2013.19.2.108

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Risk Factors for Malignancy of Pheochromocytoma and Abdominal Paraganglioma in Children: Clinicopathologic Perspectives

J Korean Assoc Pediatr Surg. 2013;19(2):108-121. Published online December 17, 2013

DOI: https://doi.org/10.13029/jkaps.2013.19.2.108

Figure

Risk Factors for Malignancy of Pheochromocytoma and Abdominal Paraganglioma in Children: Clinicopathologic Perspectives

Figure. The Kaplan-Meier survival curves between benign and malignant PHEO and aPGL (p = .007)

Figure.

Risk Factors for Malignancy of Pheochromocytoma and Abdominal Paraganglioma in Children: Clinicopathologic Perspectives

Table 1.

Immunohistochemical Panel

Antibody	Primary antibody^∗	Company	Dilution	Buffer(pH)^†	Target Stain^‡	Cutoff Values^§
Ki-67	mm	DAKO, Carpinteria, California, USA	1:200	CA (6)	N	> 2 %
p53	mm	DAKO	1:200	CA (6)	N	> 5 %
mdm-2	mm	Leica Biosystems, Heidelberg, Germany	1:100	EDTA (9)	N	> 50 %
mdm-2		Santa Cruz
p21	mm	biotechnology, Dallas,	1:200	CA (6)	N	> 10 %
		Texas, USA
		Spring Bioscience,
p27	rp	Pleasaton, California,	1:300	CA (6)	N	> 30 %
p27		USA
bcl-2	mm	DAKO	1:100	CA (6)	C	> 50 %
cyclin D1	rp	Santa Cruz biotechnology	1:100	CA (6)	N	> 5 %

Table 2.

Clinical Information of the Patients with PHEO and aPGL

	Total (n=20)	PHEO (n=14)	aPGL (n=6)	P value
Age (months, mean±SD)	143.90 ±40.87	141.93 ±39.26	148.50 ±19.60	.775^∗
Gender (M : F)	15 : 5	9 : 5	6 : 0	.260^†
Location				.829^†
Right (%)	7 (35)	5 (35.7)	2 (33.3%)
Left (%)	6 (30)	5 (35.7)	1 (16.7%)
Bilateral (%)	7 (35)	4 (28.6)	3 (50.0%)
Greatest dimension (cm, mean±SD)	4.86 ±1.50	4.84 ±1.26	4.88 ±2.08	.966^†
Malignancy (%)	7 (35)	4 (28.6)	3 (50.0%)	.613^†
Hereditary Features (%)	4 (20)	4 (28.6)	0	.267^†
Chemotherapy (%)	2 (10)	0	2 (33.3%)	.079^†
Radiotherapy (%)	6 (30)	3 (21.4)	3 (50.0%)	.303^†
Follow-up(months, mean±SD)	97.25 ±55.67	95.64 ±37.03	101.00 ±90.47	.850^∗
Disease free survival (months, mean±SD)	82.20 ±58.25	82.43 ±43.86	81.67 ±88.82	.985^∗
Median survival (months, range)	86.20 (14-252)	86.50 (50-159)	82.50 (14-252)	NA^§

Table 3.

Clinical Information of the Patients with Benign and Malignant Tumors^∗

	Benign (n=13)	Malignant (n=7)	P value
Age (months, mean±SD)	143.77 ± 46.25	144.14 ± 31.78	.983^†
Gender (M : F)	10 : 3	5 : 2	> .999^‡
Hereditary Features (%)	3 (23.1)	1 (14.3)	> .999^‡
Chemotherapy (%)	0	2 (28.6)	.111^‡
Radiotherapy (%)	0	6 (85.7)	< .001^‡
Location			.381^‡
Right (%)	5 (38.5)	2 (28.6)
Left (%)	5 (38.5)	1 (14.3)
Bilateral (%)	3 (23.1)	4 (57.1)
Greatest dimension (cm, mean±SD)	5.01 ± 1.55	4.57 ± 1.44	.547^†
Clinical manifestation
Hypertension (%)	10 (76.9)	4 (57.1)	.613^‡
Palpitation (%)	7 (53.8)	3 (42.9)	> .999^‡
Headache (%)	6 (46.2)	2 (28.6)	.642^‡
Diaphoresis (%)	7 (53.8)	2 (28.6)	.374^‡
Flushing (%)	9 (69.2)	2 (28.6)	.160^‡
Nausea, vomiting (%)	7 (53.8)	3 (42.9)	> .999^‡
Follow-up (months, mean±SD)	90.38 ± 39.48	110.00 ± 80.02	.467^†
Disease free survival (months, mean±SD)	90.38 ± 39.48	67.00 ± 84.92	.690^†
Median survival period (months, range)	93.00 (14-159)	80.00 (22-252)	NA^§

Table 4.

Microscopic Features of Benign and Malignant Tumors^∗

	Benign (n=13)	Malignancy (n=7)	P value^†
Capsular invasion (n, %)	4 (30.8)	3 (42.9)	.651
Vascular invasion (n, %)	0	4 (57.1)	.007
Extension into adipose tissue (n, %)	1 (7.69)	4 (57.1)	.031
Presence of large nests (n, %)	7 (53.8)	1 (14.3)	.158
Central tumor necrosis (n, %)	6 (46.2)	5 (71.4)	>.999
High cellularity (n, %)	3 (23.1)	2 (28.6)	>.999
Tumor cell spindling (n, %)	8 (61.5)	1 (14.3)	.070
Cellular monotony (n, %)	3 (23.1)	1 (14.3)	>.999
Mitosis (n, %)	1 (7.69)	4 (57.1)	.031
Atypical mitosis (n, %)	1 (7.69)	2 (28.6)	.270
Nuclear pleomorphism (n, %)	4 (30.8)	0	.249
Nuclear hyperchromasia (n, %)	9 (69.2)	2 (28.6)	.160

Table 5.

Microscopic Features of Benign and Malignant PHEO^∗

	Benign (n=10)	Malignancy (n=4)	P value^†
Capsular invasion (n, %)	3 (30)	2 (50)	.580
Vascular invasion (n, %)	0	3 (75)	.033
Extension into adipose tissue (n, %)	0	3 (75)	.003
Presence of large nests (n, %)	7 (70)	0	.023
Central tumor necrosis (n, %)	5 (50)	4 (100)	.089
High cellularity (n, %)	3 (30)	1 (25)	.857
Tumor cell spindling (n, %)	7 (70)	1 (25)	.139
Cellular monotony (n, %)	3 (30)	0	.234
Mitosis (n, %)	1 (10)	3 (75)	.019
Atypical mitosis (n, %)	0	1 (25)	.114
Nuclear pleomorphism (n, %)	3 (30)	0	.234
Nuclear hyperchromasia (n, %)	8 (80)	0	.008

Table 6.

Analysis of PASS Score and Pathologic Score between Benign and Malignant Tumor^∗

	Benign (n=13)	Malignancy (n=7)	P value
PASS Score (mean±SD)	6.00 ± 2.86	7.57 ± 3.36	.317^†
PASS ≥ 4 (n, %)	10 (76.9)	5 (71.4)	>.999^‡
PASS ≥ 6 (n, %)	8 (61.5)	5 (71.4)	>.999^‡
Pathologic Score (mean±SD)	0.31 ± 0.86	3.43 ± 2.57	.001^†
Score < 2 (n, %)	12 (92.3)	2 (28.5)	.007^§
Score ≥ 2 (n, %)	1 (7.7)	5 (71.4)

Table 7.

Immunohistochemistry^∗

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