ABSTRACT
Reticulohistiocytoma is a rare non-Langerhans-cell histiocytosis. On exposed skin areas it is a cosmetic problem and can cause mechanical irritation due to its prominent nature. We successfully treated a histologically confirmed reticulohistiocytoma on the back of a 60 year old woman with a pulsed dye-laser (wavelength 585 nm, pulse 0.45 msec). The lesion regressed significantly after the first treatment (spot size: 7 mm, fluence 7.8 J/cm(2)). After another laser treatment, the lesion completely disappeared. At 28 month follow-up, no recurrence was seen. Permanent side effects were not observed. In our case report the use of the pumped pulsed dye-laser has proven to be an elegant and low risk treatment option for reticulohistiocytoma. The mechanism of action remains unknown.
Subject(s)
Histiocytosis, Non-Langerhans-Cell/pathology , Histiocytosis, Non-Langerhans-Cell/radiotherapy , Laser Therapy , Surgery, Plastic/methods , Female , Humans , Middle Aged , Treatment OutcomeABSTRACT
We present the case of a man who died 11 years after the onset of xanthoma disseminatum. His course was marked by numerous, large recurrent intracranial dural-based xanthomatous tumors requiring repeated neurosurgical intervention. Late in his course there was progressive quadriparesis, ataxia, ophthalmoplegia and bulbar palsy as a result of intraparenchymal brainstem involvement. At autopsy, there was extensive meningeal and intraparenchymal CNS disease and widespread systemic infiltrates. The gross and microscopic pathology are presented with immunohistochemical, ultrastructural and biochemical details. The present case is discussed in the context of the current classification scheme and the recent literature, where it is exceptional for the extent of CNS disease. Xanthoma disseminatum is currently classified as a non-X histiocytopathy, the pathogenesis of which remains uncertain. CNS involvement carries a poor prognosis in this otherwise benign condition.
Subject(s)
Brain Diseases/pathology , Histiocytosis, Non-Langerhans-Cell/pathology , Meninges/pathology , Adult , Brain/pathology , Brain Diseases/radiotherapy , Brain Diseases/surgery , Brain Stem/pathology , Combined Modality Therapy , Cranial Irradiation , Fatal Outcome , Follow-Up Studies , Histiocytosis, Non-Langerhans-Cell/radiotherapy , Histiocytosis, Non-Langerhans-Cell/surgery , Humans , Magnetic Resonance Imaging , Male , Meninges/surgery , Microscopy, Electron , Middle Aged , Tomography, X-Ray ComputedABSTRACT
A one year old child diagnosed at two months as having familial hemophagocytic lymphohistiocytosis had a partial response to etoposide. He relapsed and underwent a bone marrow transplant after conditioning with etoposide 65 mg/kg, total body irradiation and cyclophosphamide 60 mg/kg X 2 doses. Post transplant sepsis ensued and the patient expired. At autopsy residual disease was noted. Familial hemophagocytic lymphohistiocytosis continues to be a fatal disorder and etoposide is the only agent known so far with any efficacy. Bone marrow transplantation probably should be attempted early in its course and may have a role in the treatment of this disease.
