Abstract
Pheochromocytomas are catecholamine-secreting tumors. These tumors are rare in children, and they may be associated with hereditary syndromes such as von Hippel-Lindau (VHL) disease. Most pediatric patients with pheochromocytoma present with sustained hypertension, while 10% to 69% of adult patients are asymptomatic. Herein, we present the case of a 12-yr-old Japanese girl with pheochromocytoma due to a germline mutation in the VHL (Arg161Gln). The only complaint was loss of weight. Pyrexia, anemia, and increases in C-reactive protein (CRP) and ferritin were observed. Abdominal ultrasonography revealed a right adrenal gland tumor. Fractionated catecholamines and metanephrines in plasma and 24-h collected urine revealed elevated levels of norepinephrine and normetanephrine. Although hypertension and tachycardia were inapparent by an ordinary physical examination, paroxysmal mild hypertension and tachycardia were identified by a thorough examination after walking and abdominal compression. Paroxysmal hypertension and tachycardia were profound during operation. In conclusion, pheochromocytoma can be a consideration in the differential diagnosis of weight loss. Hypertension and tachycardia can be inapparent and paroxysmal in pediatric patients as well as in adults; thus, thorough assessment should be repeated.
| Original language | English |
|---|---|
| Pages (from-to) | 87-93 |
| Number of pages | 7 |
| Journal | Clinical Pediatric Endocrinology |
| Volume | 27 |
| Issue number | 2 |
| DOIs | |
| Publication status | Published - 2018 Jan 1 |
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Keywords
- Asymptomatic
- Pheochromocytoma
- VHL
- Weight loss
ASJC Scopus subject areas
- Pediatrics, Perinatology, and Child Health
- Endocrinology, Diabetes and Metabolism
- Endocrinology
Cite this
A pediatric case of pheochromocytoma without apparent hypertension associated with von hippel-lindau disease. / Igaki, Junko; Nishi, Akira; Sato, Takeshi; Hasegawa, Tomonobu.
In: Clinical Pediatric Endocrinology, Vol. 27, No. 2, 01.01.2018, p. 87-93.Research output: Contribution to journal › Article
}
TY - JOUR
T1 - A pediatric case of pheochromocytoma without apparent hypertension associated with von hippel-lindau disease
AU - Igaki, Junko
AU - Nishi, Akira
AU - Sato, Takeshi
AU - Hasegawa, Tomonobu
PY - 2018/1/1
Y1 - 2018/1/1
N2 - Pheochromocytomas are catecholamine-secreting tumors. These tumors are rare in children, and they may be associated with hereditary syndromes such as von Hippel-Lindau (VHL) disease. Most pediatric patients with pheochromocytoma present with sustained hypertension, while 10% to 69% of adult patients are asymptomatic. Herein, we present the case of a 12-yr-old Japanese girl with pheochromocytoma due to a germline mutation in the VHL (Arg161Gln). The only complaint was loss of weight. Pyrexia, anemia, and increases in C-reactive protein (CRP) and ferritin were observed. Abdominal ultrasonography revealed a right adrenal gland tumor. Fractionated catecholamines and metanephrines in plasma and 24-h collected urine revealed elevated levels of norepinephrine and normetanephrine. Although hypertension and tachycardia were inapparent by an ordinary physical examination, paroxysmal mild hypertension and tachycardia were identified by a thorough examination after walking and abdominal compression. Paroxysmal hypertension and tachycardia were profound during operation. In conclusion, pheochromocytoma can be a consideration in the differential diagnosis of weight loss. Hypertension and tachycardia can be inapparent and paroxysmal in pediatric patients as well as in adults; thus, thorough assessment should be repeated.
AB - Pheochromocytomas are catecholamine-secreting tumors. These tumors are rare in children, and they may be associated with hereditary syndromes such as von Hippel-Lindau (VHL) disease. Most pediatric patients with pheochromocytoma present with sustained hypertension, while 10% to 69% of adult patients are asymptomatic. Herein, we present the case of a 12-yr-old Japanese girl with pheochromocytoma due to a germline mutation in the VHL (Arg161Gln). The only complaint was loss of weight. Pyrexia, anemia, and increases in C-reactive protein (CRP) and ferritin were observed. Abdominal ultrasonography revealed a right adrenal gland tumor. Fractionated catecholamines and metanephrines in plasma and 24-h collected urine revealed elevated levels of norepinephrine and normetanephrine. Although hypertension and tachycardia were inapparent by an ordinary physical examination, paroxysmal mild hypertension and tachycardia were identified by a thorough examination after walking and abdominal compression. Paroxysmal hypertension and tachycardia were profound during operation. In conclusion, pheochromocytoma can be a consideration in the differential diagnosis of weight loss. Hypertension and tachycardia can be inapparent and paroxysmal in pediatric patients as well as in adults; thus, thorough assessment should be repeated.
KW - Asymptomatic
KW - Pheochromocytoma
KW - VHL
KW - Weight loss
UR - http://www.scopus.com/inward/record.url?scp=85045415505&partnerID=8YFLogxK
UR - http://www.scopus.com/inward/citedby.url?scp=85045415505&partnerID=8YFLogxK
U2 - 10.1297/cpe.27.87
DO - 10.1297/cpe.27.87
M3 - Article
AN - SCOPUS:85045415505
VL - 27
SP - 87
EP - 93
JO - Clinical Pediatric Endocrinology
JF - Clinical Pediatric Endocrinology
SN - 0918-5739
IS - 2
ER -