Langerhans' cell histiocytosis of the temporal lobe and pons

Abstract

Intracerebral Langerhans' cell histiocytosis (LCH) is rare and tends to involve the hypothalamus. The authors report a rare case of LCH in the temporal lobe that subsequently was followed by a brainstem lesion. This appears to be the first case of temporal lobe and brainstem LCH that has been treated successfully and published. A 24-year-old man complained of cacosmia and nausea with a slight headache. He had a left temporal LCH, which was removed completely, but developed a brainstem lesion a year later. The pontine LCH was treated with radiosurgery. The follow-up period was 4 years without any neurological or radiological symptoms or signs. The 12 cases of solitary intracranial non-hypothalamic LCH reported previously are reviewed. Gamma knife radiosurgery effectively controlled the local growth of the pontine LCH without adverse effect.

Histiocitose X em osso temporal

“…Many authors argue that surgery is an invasive procedure in such cases 3,5 . Most papers, however, recommend surgery associated with some other treatment (corticosteroid therapy, chemotherapy 6 or radiotherapy on focal lesions). Our patient was treated with surgery and corticosteroid therapy, without discarding possible future chemotherapy.…”

Isolated tumorous Langerhans cell histiocytosis of the brainstem: a diagnostic and therapeutic challenge

“…7 Enhancing parenchymal CNS lesions are very rare, and such lesions involving the brainstem have been described in few case reports. 4,11 Such lesions cause considerable diagnostic problems because no morphological patterns characteristic of LCH have been identified to facilitate prompt diagnosis. 9 We present a case of solitary CNS LCH lesion of the brainstem in a child, primarily presenting as right cerebellopontine syndrome.…”

“…5 Intracranial lesions have been observed during the course of disease in patients with proven LCH, and also as the first and presenting manifestations of LCH, albeit rarely. 3,4 Different patterns of CNS involvement are described in the MRI-based classification system of CNS LCH. 7 Enhancing parenchymal CNS lesions are very rare, and such lesions involving the brainstem have been described in few case reports.…”

“…Gamma Knife surgery has been used as a treatment option for a case of brainstem LCH. 4 The response rate to radiation therapy has been estimated at 50%-70%. According to the treatment protocol of the Third International Study for Langerhans Cell Histiocytosis, a 6-month course of chemotherapy is required for CNS LCH lesions.…”

“…The review of literature on intracranial LCH by Bergmann et al and Cagli et al documents that the majority of patients were young males and that they presented with either acute headache or seizures. 3,4 Our patient was a 7-year-old boy presenting with neurological symptoms of right cerebellopontine syndrome. On MRI, tumorous CNS LCH lesions manifest as enhancing masses, which are iso-or hypointense on T1-weighted imaging and hyperintense on T2-weighted imaging.…”