Use of radiation in treatment of central nervous system juvenile xanthogranulomatosis.

Juvenile xanthogranulomatosis (JXG) represents a subset of non-Langerhan cell histiocytosis that typically manifests in younger children with skin lesions. Unresectable central nervous system (CNS) disease is difficult to treat. We describe the case of a 13-year-old successfully treated with adjuvant radiation therapy for symptomatic intracranial and leptomeningeal JXG. An extensive literature review was performed to identify all previous CNS JXG cases utilizing radiation, of which six of eight total patients demonstrated temporary or long-term improvement of neurologic disease. This suggests that radiation should be considered in cases unresponsive to conventional treatment options.

Juvenile xanthogranuloma in an adult lumbar spine: a case report.

STUDY DESIGN: Case report. OBJECTIVE: To report a very rare case of juvenile xanthogranuloma (JXG) of the spine in an adult. SUMMARY OF BACKGROUND DATA: JXG is very rare in the spine, with only five prior reports in infants and children. To the best of our knowledge, this tumor has never been reported in an adult spine. METHODS: The patient is a 47-year-old woman who presented with bowel and bladder incontinence. Magnetic resonance imaging showed a very large lesion arising from the L2 vertebral body, with massive extension into the retroperitoneum with extensive intradural involvement. She had decreased rectal tone, had 4/5 strength in the right hip flexor, and had diminished sensation in her anterior right thigh and perineal region. She was otherwise neurologically intact. After preoperative embolization, a decompressive laminectomy was performed and the tumor was resected through a posterolateral transpedicular approach, followed by stabilization. Because of extensive involvement of retroperitoneum, complete resection was not possible. RESULTS: After pathologic evaluation of the specimen, a diagnosis of JXG was made. Patient underwent postoperative radiation therapy, and her neurologic examination improved significantly over the next several months. CONCLUSIONS: To the best of our knowledge, this is the first reported case of JXG in an adult spine. Although complete resection of the tumor was not possible, decompression of the dural sac followed by postoperative radiation led to an excellent clinical outcome.

Juvenile xanthogranuloma masquerading as melanoma.

Juvenile xanthogranuloma (JXG) of the uvea is a rare disease that usually responds to systemic steroids or low-dose radiotherapy. We present an atypical case of bilateral JXG involving the entire uveal tract that presented with an aggressive phenotype. The patient was unresponsive to topical and systemic corticosteroids, cyclosporine, and maximal doses of radiation therapy. The disease was ultimately controlled with the alkylating agent chlorambucil.

Juvenile xanthogranuloma of the iris in an adult.

Juvenile xanthogranuloma is a self-limited skin disorder of young children that uncommonly affects the eye. Juvenile xanthogranuloma has been described in adults, but reported intraocular involvement is extremely rare. We report a case of juvenile xanthogranuloma diagnosed in a 25-year-old man who was seen with nontraumatic hyphema and iridocyclitis. Diagnosis was made from a biopsy specimen obtained from a suspicious skin lesion. Topical and systemic steroids, radiation therapy, and finally immunosupression were required to eliminate the iris tumor clinically and resolve the patient's recurrent symptoms.

[Radiotherapy in juvenile xanthogranuloma of the iris]. / Strahlentherapie bei juvenilem Xanthogranulom der Iris.

Nevoxanthoendotheliomas (and juvenile xanthogranulomas) are very rare benign skin diseases which occur in infants. An ocular manifestation, mainly in the iris, has been found in approximately. 10% of the cases, The clinical picture is characterized by a recurrent unilateral hemorrhage of the anterior chamber, which is the reason for seeing an ophthalmologist. The clinical diagnosis of juvenile xanthogranuloma is based on the characteristic iris changes - heterochromia, circumscribed vascularized tumor, or diffuse thickening of the iris. Sometimes elevated intraocular pressure is also found. In combination with a mild local steroid, radiotherapy with 300 to 400 cGy should be started immediately in order to prevent a sometimes uncontrollable secondary glaucoma. The good results achieved in 5 of the authors' own cases underline the value of low-dose radiotherapy in this disease: the affected eye was healed in all 5 cases.

Juvenile xanthogranuloma of the iris managed with superficial radiotherapy.

A case of juvenile xanthogranuloma involving the iris successfully managed with superficial radiotherapy is presented. A review of the literature suggests that this benign condition, if progressive or extensive, is optimally treated with radiotherapy as an alternative to major surgical procedures, including enucleation.

[Radiotherapy of the juvenile xanthogranuloma of the iris (author's transl)]. / Zur Strahlentherapie des juvenilen Xanthogranuloms der Iris.

A report is given on an eight months old child with a juvenile xanthogranuloma of the iris and a secondary glaucoma. After radiotherapy with 6 X 0,5 Gy under conventional deep therapy conditions, a soon regression of the increased intraocular pressure was observed. In case of a juvenile xanthogranuloma of the iris, this secondary glaucoma represents in any case an acute danger to the eye, so it must be treated immediately. Radiotherapy has to be considered as the method of choice; it is also discussed with regard to the risk and the avoidance of a radiation cataract which, however, has to be tolerated in certain cases in order to prevent greater troubles.

[Problems in juvenile xanthogranuloma of the anterior uvea (author's transl)]. / Zum Problem des juvenilen Xanthogranuloms der vorderen Uvea.

With reference to a case of juvenile xanthogranuloma localized in the anterior uvea the authors describe the clinical signs, differential diagnosis, morphology and treatment of this disease. If the juvenile xanthogranuloma is located in the anterior uvea, the eye may be severely affected. Spontaneous hyphema in infants is characteristic. At present radiation is the most successful treatment for preserving the bulbus and visual function. The earlier the disease is discovered the better the chances of successful treatment.