Spontaneous Regression Of Langerhans Cell Histiocytosis: A Case Report
Yamaguchi, S., Oki, S., & Kurisu, K. (2004). spontaneous regression of Langerhans cell histiocytosis: a case report. Surgical neurology, 62(2), 136–141. https://doi.org/10.1016/j.surneu.2003.10.003
View Original Source →Abstract
BACKGROUND: Spontaneous regression of Langerhans cell histiocytosis (LCH) has been reported in skin, bone, and pulmonary lesions. However, such phenomena in the central nervous system (CNS) have not been described previously. CASE DESCRIPTION: A case of LCH in the CNS with spontaneous regression is reported. A 2-year-old boy presented with general convulsion followed by frequent vomiting and diabetes insipidus. Magnetic resonance imaging (MRI) scan revealed numerous multifocal nodules predominantly in the left frontal lobe. The patient underwent surgery to remove part of the frontal lesion. Subsequent to surgery, residual lesions showed regression without any additional treatment, and clinical symptoms also subsided. The multiple lesions disappeared completely and did not relapse during 5-year observation. CONCLUSION: Surgical resection and/or adjuvant therapy such as radiation and chemotherapy has been performed for the treatment of LCH in the CNS. However, there may be a subgroup of patients with multifocal brain LCH that regress spontaneously. Further clinical study is required to establish the natural course and prognostic factors of this disease.
Case Details
Disease Location
Left frontal lobe
Personal Characteristics
2 -year-old male no remarkable illness history
Clinical Characteristics
Presented symptoms of general confusion, then frequent vomiting, polydipsia and polyuria followed after several days MRI revealed numerous nodules involving the cerebral hemispheres, bilateral basal ganglia, hypothalamus, and cerebellum. Lesions were enhanced on t1 and had perifocal edema. A physical exam revealed no cutaneous lesions nor lymphadenopathy systemic CT scans and gallium scintigraphy showed no extra cns lesions intraoperative frozen section revealed the possibility of hemistocytic astrocytoma follow-up mris revealed residual multifocal lesions started to involute spontaneously before definitive histopathological diagnosis several months after the surgery showed that large cells with abundant foamy cytoplasm tended to aggregate around the vessels and form granular lesions. These cells were positive for cd1a and s-100, electromicroscopic failed to document birbeck granules. But the immunohistochemical findings confirmed lch
Remission Characteristics
Neurologic status was unchanged immediately after the op, but polydipsia and polyuria started to subside during the next few days witho the use of vasopressin. Frequent vomiting also stopped 56 months after the onset, MRI showed a complete and continuous remission of the lesions, there are no obvious neurologic deficits complete remission state for 5 years to date
Treatment & Mechanisms
Proposed Remission Mechanisms
Recommendations for surgery for a single lesion radiation/chemo for residual, recurrent lesions, or multifocal disease radiation therapy with a dose of 5-20 gy has been proven to be effective in inducing remission lesions may have "burned out" and turned to fibrosis
Clinical Treatment
Craniotomy frontal partial lobotomy was performed to achieve an internal decompression
Non-Clinical Treatment
None reported