Pediatric Radiology
Short reports
9 Springer-Verlag 1992 Pediatr Radiol (1992) 22:377-378
Juvenile xanthogranuloma with extracutaneous lesions P. Garcia-Pefia, A. Mariscal, C. Abellan, A. Zuasnabar, J. Lucaya Department of Radiology, Hospital Materno Infantil Vall d'Hebron, Barcelona, Spain
Abstract. The purpose of this article is to present a case of juvenile xanthogranuloma with pulmonary, retroperitoneal and splenic involvement. The similar echographic pattern of the cutaneous and visceral lesions was very helpful in o r d e r to suggest the correct diagnosis.
Juvenile x a n t h o g r a n u l o m a (JX) is a normolipemic, self-limited histiocytosis occurring in childhood and infancy, and is characterized by cutaneous and ocular xanthomas. Visceral involvement is rare yet can occur in several locations. We r e p o r t a case of a n e w b o r n infant with juvenile x a n t h o g r a n u l o m a involving the lungs, the r e t r o p e r i t o n e u m and the spleen and present the results of a revision of previously r e p o r t e d cases.
Case report
tion, a hypoechoic solid paraduodenal mass, 2.5 x 2 cm in diameter, with a hypoechoic central zone, was detected (Fig. 2 a). The liver was normal in size and echogenicity. The sonographic features of the subcutaneous nodule were similar to those of the retroperitoneal lesion (Fig. 2 b). Thoraco-abdominal CT disclosed three pulmonary nodules on the right side and an abdominal paraduodenal rounded mass. This mass was soft-tissue density with several central hyperdense loci. The spleen was enlarged and showed many scattered small hypodense nodules, which were better seen following the administration of i.v. contrast material (Fig.3). The right axillary nodule was surgically removed. Pathological examination revealed a well-defined yellowish mass, with peripheral fibroblasts surrounding a central zone with multiple xanthomatous cells and Touton-type giant cells. These features confirmed the diagnosis of JX. The patient received no treatment, and in a follow-up exam performed four months later, all his gluteal nodes had considerably decreased in size. On a repeated US the spleen was normal. The retroperitoneal mass, though smaller, persisted.
Fig.1. Chest X-ray showing a pulmonary nodule in the right lower lobe
Fig.2. a Transverse abdominal ultrasound shows a paraduodenal hypoechoic solid mass with a hyperechogenic central area. b Ultra-
sound of soft tissues (right axillary nodule) reveals a similar hypoechoic nodule with an echogenic central zone
Discussion Juvenile x a n t h o g r a n u l o m a is a benign condition included in the b r o a d category of histiocytic diseases. It is characterized by cutaneous and ocular xanthomatous lesions, with the latter being less common. The skin lesions are usually quite obvious and are considered the
A 10-day-old newborn girl was referred to our hospital for evaluation of several subcutaneous nodules and splenomegaly. There were multiple hard right axillary and left gluteal nodules, not adherent to deep planes and which measured from 0.5 to 2 cm. The spleen was moderately enlarged. Routine laboratory data were normal. Skeletal X-ray survey was also normal. Chest X-rayshoweda i cmpulmonary nodule in the right lower lobe (Fig.l). Abdominal US. showed an enlarged spleen with ill-defined hypoechoic parenchymal nodules. In addi-
Correspondence to: Dr. R Garcia-Pefia, Servicio de Radiologia Infantil, Hospital Materno Infantil Vall d'Hebron, Paseo Vall d'Hebron s/n., E-08035 Barcelona, Spain
378
Fig.3a,b. CT of the abdomen, a The noncontrast exam shows splenomegaly and a hypodense retroperitoneal mass with hyperdense central zones, b Multiple hypodense splenic nodules can also be seen following intravenous contrast injection main clinical feature of this condition. They usually appear between the ages of one month and two years although they may also occur at birth or in adoles-
cence. They can be single or multiple and are more frequently located in the head and neck [1, 2]. Size varies from 1 mm to 5 cm; they can be macular or nodular in shape and reddish-brown in color which progresses to yellow. This entity is also characterized by ocular involvement, with the iris being the most frequent location [3]. Visceral involvement is rare but when it occurs, it is always associated with cutaneous lesions. Pericardium, testes, lymph nodes, liver, kidney and lungs are the extracutaneous locations described in this illness. Gilbert and Parker reported the first case with renal involvement shown by US and CT [4]. Sonographically the renal mass had mixed echogenicity. CT showed an isodense renal mass which following the administration of i. v. contrast material became hypodense in relation to the normal renal parenchyma. On chest X-rays, all lesions were nodular and well-defined, and ranged from 1 to 2.5 cm in diameter [1, 5, 6]. Calcifications have never been observed. Diard et al. presented a case with pulmonary, extrapleural and hepatic lesions [6]. A n echogenic nodule in the liver was detected by US and was the first reported case with hepatic involvement studied by US. The diagnosis of JX is established by biopsy of the cutaneous lesion.
Differential diagnosis should include histiocytosis X and systemic dyslipidoses. Bone lesions do not occur in xanthogranulomatosis. On the other hand elevated serum lipids and cholesterollevels are the hallmark of the systemic dyslipidoses. However, biopsy of the cutaneous lesions will be mandatory to establish diagnosis. The prognosis in JX is excellent. Most cutaneous and visceral lesions regress spontaneously without treatment [1, 3, 5, 6].
References 1. Lottsfeld FI, Good RA (1964) Juvenile xanthogranulomatosis with pulmonary lesion: a case report. Pediatrics 33:233 2. Helwing EB, Hachney VC (1954) Juvenile xanthogranuloma. Am J Patho130:625 3. Webster SB, Reister HC, Harman LE (1966) Juvenile xanthogranuloma with extracutaneous lesions. A case report and review of the literature. Arch D ermato193:71 4. Gilbert TJ, Parker BR (1988) Juvenile xanthogranuloma of the kidney. Pediatr Radio118:169 5. Gupta AK, Bhargava S (1988) Juvenile xanthogranuloma with pulmonary lesions. Pediatr Radio118: 70 6. Diard F, Cadier L, Billaud C, Trojani M (1982) Neonatal juvenile xanthogranulomatosis with pulmonary, extrapleural and hepatic involvement. One case report. Ann Radio125:113
Short reports
9 Springer-Verlag 1992 Pediatr Radiol (1992) 22:377-378
Juvenile xanthogranuloma with extracutaneous lesions P. Garcia-Pefia, A. Mariscal, C. Abellan, A. Zuasnabar, J. Lucaya Department of Radiology, Hospital Materno Infantil Vall d'Hebron, Barcelona, Spain
Abstract. The purpose of this article is to present a case of juvenile xanthogranuloma with pulmonary, retroperitoneal and splenic involvement. The similar echographic pattern of the cutaneous and visceral lesions was very helpful in o r d e r to suggest the correct diagnosis.
Juvenile x a n t h o g r a n u l o m a (JX) is a normolipemic, self-limited histiocytosis occurring in childhood and infancy, and is characterized by cutaneous and ocular xanthomas. Visceral involvement is rare yet can occur in several locations. We r e p o r t a case of a n e w b o r n infant with juvenile x a n t h o g r a n u l o m a involving the lungs, the r e t r o p e r i t o n e u m and the spleen and present the results of a revision of previously r e p o r t e d cases.
Case report
tion, a hypoechoic solid paraduodenal mass, 2.5 x 2 cm in diameter, with a hypoechoic central zone, was detected (Fig. 2 a). The liver was normal in size and echogenicity. The sonographic features of the subcutaneous nodule were similar to those of the retroperitoneal lesion (Fig. 2 b). Thoraco-abdominal CT disclosed three pulmonary nodules on the right side and an abdominal paraduodenal rounded mass. This mass was soft-tissue density with several central hyperdense loci. The spleen was enlarged and showed many scattered small hypodense nodules, which were better seen following the administration of i.v. contrast material (Fig.3). The right axillary nodule was surgically removed. Pathological examination revealed a well-defined yellowish mass, with peripheral fibroblasts surrounding a central zone with multiple xanthomatous cells and Touton-type giant cells. These features confirmed the diagnosis of JX. The patient received no treatment, and in a follow-up exam performed four months later, all his gluteal nodes had considerably decreased in size. On a repeated US the spleen was normal. The retroperitoneal mass, though smaller, persisted.
Fig.1. Chest X-ray showing a pulmonary nodule in the right lower lobe
Fig.2. a Transverse abdominal ultrasound shows a paraduodenal hypoechoic solid mass with a hyperechogenic central area. b Ultra-
sound of soft tissues (right axillary nodule) reveals a similar hypoechoic nodule with an echogenic central zone
Discussion Juvenile x a n t h o g r a n u l o m a is a benign condition included in the b r o a d category of histiocytic diseases. It is characterized by cutaneous and ocular xanthomatous lesions, with the latter being less common. The skin lesions are usually quite obvious and are considered the
A 10-day-old newborn girl was referred to our hospital for evaluation of several subcutaneous nodules and splenomegaly. There were multiple hard right axillary and left gluteal nodules, not adherent to deep planes and which measured from 0.5 to 2 cm. The spleen was moderately enlarged. Routine laboratory data were normal. Skeletal X-ray survey was also normal. Chest X-rayshoweda i cmpulmonary nodule in the right lower lobe (Fig.l). Abdominal US. showed an enlarged spleen with ill-defined hypoechoic parenchymal nodules. In addi-
Correspondence to: Dr. R Garcia-Pefia, Servicio de Radiologia Infantil, Hospital Materno Infantil Vall d'Hebron, Paseo Vall d'Hebron s/n., E-08035 Barcelona, Spain
378
Fig.3a,b. CT of the abdomen, a The noncontrast exam shows splenomegaly and a hypodense retroperitoneal mass with hyperdense central zones, b Multiple hypodense splenic nodules can also be seen following intravenous contrast injection main clinical feature of this condition. They usually appear between the ages of one month and two years although they may also occur at birth or in adoles-
cence. They can be single or multiple and are more frequently located in the head and neck [1, 2]. Size varies from 1 mm to 5 cm; they can be macular or nodular in shape and reddish-brown in color which progresses to yellow. This entity is also characterized by ocular involvement, with the iris being the most frequent location [3]. Visceral involvement is rare but when it occurs, it is always associated with cutaneous lesions. Pericardium, testes, lymph nodes, liver, kidney and lungs are the extracutaneous locations described in this illness. Gilbert and Parker reported the first case with renal involvement shown by US and CT [4]. Sonographically the renal mass had mixed echogenicity. CT showed an isodense renal mass which following the administration of i. v. contrast material became hypodense in relation to the normal renal parenchyma. On chest X-rays, all lesions were nodular and well-defined, and ranged from 1 to 2.5 cm in diameter [1, 5, 6]. Calcifications have never been observed. Diard et al. presented a case with pulmonary, extrapleural and hepatic lesions [6]. A n echogenic nodule in the liver was detected by US and was the first reported case with hepatic involvement studied by US. The diagnosis of JX is established by biopsy of the cutaneous lesion.
Differential diagnosis should include histiocytosis X and systemic dyslipidoses. Bone lesions do not occur in xanthogranulomatosis. On the other hand elevated serum lipids and cholesterollevels are the hallmark of the systemic dyslipidoses. However, biopsy of the cutaneous lesions will be mandatory to establish diagnosis. The prognosis in JX is excellent. Most cutaneous and visceral lesions regress spontaneously without treatment [1, 3, 5, 6].
References 1. Lottsfeld FI, Good RA (1964) Juvenile xanthogranulomatosis with pulmonary lesion: a case report. Pediatrics 33:233 2. Helwing EB, Hachney VC (1954) Juvenile xanthogranuloma. Am J Patho130:625 3. Webster SB, Reister HC, Harman LE (1966) Juvenile xanthogranuloma with extracutaneous lesions. A case report and review of the literature. Arch D ermato193:71 4. Gilbert TJ, Parker BR (1988) Juvenile xanthogranuloma of the kidney. Pediatr Radio118:169 5. Gupta AK, Bhargava S (1988) Juvenile xanthogranuloma with pulmonary lesions. Pediatr Radio118: 70 6. Diard F, Cadier L, Billaud C, Trojani M (1982) Neonatal juvenile xanthogranulomatosis with pulmonary, extrapleural and hepatic involvement. One case report. Ann Radio125:113
