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Juvenile Xanthogranuloma of the Testis
0022-534 7/85/1336-1054$02.00/0 Vol. 133, June
THE JOURNAL OF UROLOGY
Copyright© 1985 by The Williams & Wilkins Co.
Printed in U.S.A.
JUVENILE XANTHOGRANULOMA OF THE TESTIS N. H. TOWNELL, A. GLEDHILL, T. ROBINSON
AND
P. HOPEWELL
From the Departments of Urology and Histopathology, Royal Free Hospital and School of Medicine, and Department of Dermatology, University College Hospital, London, England
A 7-month-old boy presented with numerous xanthomatous skin lesions and a hard irregular swelling of the right testis. Clinically, the testicular lesion was impossible to distinguish from a malignant neoplasm. Histological examination of a skin biopsy and of the testis following orchiectomy showed lesions typical of juvenile xanthogranuloma. The term juvenile xanthogranuloma was proposed by Helwig and Hackney in 1954 to describe a disease characterized by the appearance of numerous xanthomatous lesions during the first 6 months of life. 1 The lesions generally are confined to the skin but other organs may be involved, particularly the eye. The dermal and systemic lesions undergo spontaneous involution
CASE REPORT
A 7-month-old white boy presented with a 6-month history of right testicular enlargement. Examination revealed about a dozen pink to yellow papulo-nodules 2 to 10 mm. in diameter on the head, trunk and thighs. The nodules had been present since the patient was 6 weeks old. The right testis was hard, irregular and enlarged (3.0 X 2.0 X 2.5 cm.). Ultrasound examination revealed a homogeneous mass arising in the right testis. Chest x-ray and laboratory investigations, including serum a-fetoprotein and human chorionic gonadotropin, were normal. Orchiectomy was performed and 1 skin lesion was biopsied. Convalescence was uneventful and the patient was well with no evidence of further disease on whole body computerized tomography scan 1 year later. Pathological findings. The skin biopsy contained a tan lesion 0.8 cm. in diameter with an underlying nodule of homogeneous yellow tissue. The testis was replaced by similar yellow tissue but with a central area of hemorrhage and necrosis. Histological examination of the skin revealed interlacing spindle-shaped cells, lipid-laden foam cells and Touton giant cells typical of juvenile xanthogranuloma (fig. 1). Sections of the testis showed spindle-shaped cells similar to those in the skin, displacing and surrounding the seminiferous tubules (fig. 2). Giant cells were sparse and fat was minimal. There were few mitotic figures and no aberrant mitoses. A mild inflammatory infiltrate was present around the necrotic area but was not prominent elsewhere. The tubules showed no evidence of dysplasia or in situ malignant change. The appearances were interpreted as those of the early histiocytic phase of xanthogranuloma, when foam cells and Touton giant cells typically are absent or few. DISCUSSION
Juvenile xanthogranuloma usually presents during the first 6 months of life with the eruption of numerous red papules on
FIG. 1. Section from skin biopsy shows spindle cell background with characteristic Touton giant cells. Reduced from X47. Inset, reduced from X470.
with age. 2 We report on a patient in whom the classical skin eruption was accompanied by a testicular xanthogranuloma. This site is unusual and it is extremely difficult clinically to distinguish the lesion from a malignant growth. Accepted for publication February 8, 1985.
FIG. 2. Section from testis shows interlacing spindle cells surrounding seminiferous tubules. Reduced from Xl88.
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REFERENCES
and of the eye is a recognized cause of intraocular bleeding. 4 There have been 2 reports of testicular involvement but in neither were the appearances described. 1 ' ,s In our the hard, irregular testis with and necrosis resembled a malignant neoplasm on macroscopic examination. In the absence of the characteristic Touton giant cell, histological diagnosis of the spindle cell lesion is difficult. The uniformity of the cell population and low mitotic count in this instance suggested a benign lesion, and the essentially similar appearance of the skin biopsy enabled diagnosis, There-
L Helwig, E. R and Hackney, V. C.: Juvenile xanthogranuloma (nevoxantho-endothelioma). Ame1·. J. Path., 30: 625, 1954. 2. Nomland, R.: Nevoxantho-endothelioma: benign xanthomatous disease of infants and children. J. Invest. Derm., 22: 207, 1954. 3. Webster, S. B., Reister, H. C. and Harman, L. E., Jr.: Juvenile xantlho1;:nm,1ioma with extracutaneous lesions. A case report and review the literature. Arch. Derm., 93: 71, 1966. 4. Linse, R., Thieme, R. and Lukassek, B.: Juveniles xanthogranulom mit olmliir und zentralnervoser Beteiligung. DermatoL I\ilonatsschr., 166: 501, 1980. 5. Nod!, F.: Systematisierte Arch. Klin. Exp. Derm.,
THE JOURNAL OF UROLOGY
Copyright© 1985 by The Williams & Wilkins Co.
Printed in U.S.A.
JUVENILE XANTHOGRANULOMA OF THE TESTIS N. H. TOWNELL, A. GLEDHILL, T. ROBINSON
AND
P. HOPEWELL
From the Departments of Urology and Histopathology, Royal Free Hospital and School of Medicine, and Department of Dermatology, University College Hospital, London, England
A 7-month-old boy presented with numerous xanthomatous skin lesions and a hard irregular swelling of the right testis. Clinically, the testicular lesion was impossible to distinguish from a malignant neoplasm. Histological examination of a skin biopsy and of the testis following orchiectomy showed lesions typical of juvenile xanthogranuloma. The term juvenile xanthogranuloma was proposed by Helwig and Hackney in 1954 to describe a disease characterized by the appearance of numerous xanthomatous lesions during the first 6 months of life. 1 The lesions generally are confined to the skin but other organs may be involved, particularly the eye. The dermal and systemic lesions undergo spontaneous involution
CASE REPORT
A 7-month-old white boy presented with a 6-month history of right testicular enlargement. Examination revealed about a dozen pink to yellow papulo-nodules 2 to 10 mm. in diameter on the head, trunk and thighs. The nodules had been present since the patient was 6 weeks old. The right testis was hard, irregular and enlarged (3.0 X 2.0 X 2.5 cm.). Ultrasound examination revealed a homogeneous mass arising in the right testis. Chest x-ray and laboratory investigations, including serum a-fetoprotein and human chorionic gonadotropin, were normal. Orchiectomy was performed and 1 skin lesion was biopsied. Convalescence was uneventful and the patient was well with no evidence of further disease on whole body computerized tomography scan 1 year later. Pathological findings. The skin biopsy contained a tan lesion 0.8 cm. in diameter with an underlying nodule of homogeneous yellow tissue. The testis was replaced by similar yellow tissue but with a central area of hemorrhage and necrosis. Histological examination of the skin revealed interlacing spindle-shaped cells, lipid-laden foam cells and Touton giant cells typical of juvenile xanthogranuloma (fig. 1). Sections of the testis showed spindle-shaped cells similar to those in the skin, displacing and surrounding the seminiferous tubules (fig. 2). Giant cells were sparse and fat was minimal. There were few mitotic figures and no aberrant mitoses. A mild inflammatory infiltrate was present around the necrotic area but was not prominent elsewhere. The tubules showed no evidence of dysplasia or in situ malignant change. The appearances were interpreted as those of the early histiocytic phase of xanthogranuloma, when foam cells and Touton giant cells typically are absent or few. DISCUSSION
Juvenile xanthogranuloma usually presents during the first 6 months of life with the eruption of numerous red papules on
FIG. 1. Section from skin biopsy shows spindle cell background with characteristic Touton giant cells. Reduced from X47. Inset, reduced from X470.
with age. 2 We report on a patient in whom the classical skin eruption was accompanied by a testicular xanthogranuloma. This site is unusual and it is extremely difficult clinically to distinguish the lesion from a malignant growth. Accepted for publication February 8, 1985.
FIG. 2. Section from testis shows interlacing spindle cells surrounding seminiferous tubules. Reduced from Xl88.
1054
GF TES'f1S
the
lS
to
REFERENCES
and of the eye is a recognized cause of intraocular bleeding. 4 There have been 2 reports of testicular involvement but in neither were the appearances described. 1 ' ,s In our the hard, irregular testis with and necrosis resembled a malignant neoplasm on macroscopic examination. In the absence of the characteristic Touton giant cell, histological diagnosis of the spindle cell lesion is difficult. The uniformity of the cell population and low mitotic count in this instance suggested a benign lesion, and the essentially similar appearance of the skin biopsy enabled diagnosis, There-
L Helwig, E. R and Hackney, V. C.: Juvenile xanthogranuloma (nevoxantho-endothelioma). Ame1·. J. Path., 30: 625, 1954. 2. Nomland, R.: Nevoxantho-endothelioma: benign xanthomatous disease of infants and children. J. Invest. Derm., 22: 207, 1954. 3. Webster, S. B., Reister, H. C. and Harman, L. E., Jr.: Juvenile xantlho1;:nm,1ioma with extracutaneous lesions. A case report and review the literature. Arch. Derm., 93: 71, 1966. 4. Linse, R., Thieme, R. and Lukassek, B.: Juveniles xanthogranulom mit olmliir und zentralnervoser Beteiligung. DermatoL I\ilonatsschr., 166: 501, 1980. 5. Nod!, F.: Systematisierte Arch. Klin. Exp. Derm.,