Solitary Langerhans cell histiocytosis lesion of the parieto-occipital lobe: a case report and review of the literature

doi:10.1016/S0303-8467(96)00586-0

Clinical Neurology and Neurosurgery

Volume 99, Issue 1, February 1997, Pages 50-55

https://doi.org/10.1016/S0303-8467(96)00586-0 Get rights and content

Abstract

A 38 year-old woman with a solitary intracerebral Langerhans cell histiocytosis (LCH) lesion is presented, in whom, cerebral magnetic resonance imaging (MRI) revealed a non-enhancing mass in the right parieto-occipital lobe. The surgical specimen consisted of a nodular polymorphic infiltrate of mononuclear histiocytic cells, macrophages, eosinophilic granulocytes, lymphocytes and Langerhans giant cells involving leptomeninges, cerebral cortex and white matter. The histiocytes displayed features of Langerhans cells such as CDla and S-100 immunopositivity, and of reticulum cells such as Ki-M4P and X-12 immunopositivity. This case as well as ten other cases from the literature are reviewed.

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Cited by (22)

Intracranial Langerhans cell Histiocytosis: A review
2020, Interdisciplinary Neurosurgery: Advanced Techniques and Case Management
Citation Excerpt :
Papers were also excluded if there was not a definitive diagnosis of LCH or if the articles focused on non-Langerhan’s cell histiocytosis or malignant histiocytosis. One hundred and forty-six articles with 192 patients were found from this method [1,3,5,6,8–10,12,13,15–31,33,35,37–52,54–56,58–61,63–69,71–76,78–123,125–133,135–138,140–142,144–162,164]. The 146 articles featured 192 patients from a wide range of demographics (Table 2).
Intracranial Langerhans Cell Histiocytosis (LCH) is a rare disease caused by the proliferation and dissemination of antigen presenting cells in the skin. Few cases have been reported on to date and there is a lack of consensus on the most effective means of diagnosis and treatment of these lesions. This paper will serve as a review of this malady.
MeSH database search was performed to include all relevant studies on intracranial LCH.
A total of 146 studies with 192 patients were included in our review. Men were more commonly affected and the average age of diagnosis was 31.6 and 28.0 in men and women respectively. CT and MRI were the most common imaging modalities. The majority of the case reports performed biopsies and the most common lesion location was the hypothalamus-pituitary axis. All studies used surgical resection, radiotherapy, chemotherapy or combination therapy as means of treatment, with resection plus chemotherapy being the most successful. 74 cases were successful in preventing recurrence.
LCH rarely affects the brain as primary or secondary focus. Biopsy is required for precise diagnosis and exclusion of other intracranial lesions. There is no standard treatment for LCH of the central nervous system, but this review may serve as a guide to chronicle previous efficacious therapeutics strategies.
Magnetic Resonance Imaging Features in Solitary Cerebral Langerhans Cell Histiocytosis: Case Report and Review of Literature
2018, World Neurosurgery
Citation Excerpt :
In the former, the patients showed irregular enhancement that could have been multiple sulcal enhancement surrounding the main LCH mass.3 Similarly, in addition to enhancement of the cortical LCH mass, multiple peritumoral sulcal enhancement was also observed in the report by Cai et al.1 Bergmann et al4 identified that Langerhans giant cells involved not only the cerebral cortex and white matter but also leptomeninges on histopathologic examination. Our patient showed high enhancement of the LCH mass and additional sulcal enhancement near the mass.
In this study, we report a case of solitary Langerhans cell histiocytosis (LCH) without skull or systemic lesions including reviewing the initial magnetic resonance (MR) findings of cerebral LCH.
A 36-year-old male patient presented with generalized tonic-clonic type seizure. Brain MR imaging showed a 3-cm mass lesion involving the right frontal lobe. It showed isointensity on T1-weighted images and hyperintensity on T2-weighted images. After gadolinium enhancement, the mass showed heterogeneous enhancement with perilesional edema and additional adjacent leptomeningeal enhancement. Special MR techniques focused on the enhanced lesion showed increased cerebral blood volume on perfusion images, no diffusion restriction on diffusion-weighted images, and a necrotic spectrum on MR spectroscopy. At surgery, we found a yellow mass and yellow tissue debris in the sulci adjacent to the mass and removed them. The final histopathologic diagnosis was LCH in the frontal lobe. The patient underwent scheduled adjuvant chemotherapy with cytarabine for 6 months and has been regularly followed up without any neurologic problem for 3 years.
In conclusion, additional sulcal enhancement around the mass lesion might be a clue MR imaging feature of intracerebral LCH, and special MR imaging techniques such as perfusion imaging, diffusion-weighted imaging, and MR spectroscopy could be helpful in differential diagnosis.
Neuroimaging of Langerhans cell histiocytosis in the central nervous system of children
2015, Radiologia
La histiocitosis de células de Langerhans (HCL) es una enfermedad rara caracterizada por la acumulación en los tejidos de células dendríticas anómalas similares a las células de Langerhans. La presentación clínica varía desde la aparición de una lesión ósea única hasta la afectación multisistémica. La implicación del sistema nervioso central (SNC), manifestada como diabetes insípida secundaria a afectación hipofisaria, es conocida desde la descripción original de la enfermedad. En la actualidad, se diferencian 2 tipos de lesiones del SNC: las lesiones seudotumorales, con infiltración por las células de Langerhans, cuya manifestación más frecuente es la infiltración hipofisaria, y otras, de más reciente descripción, las lesiones neurodegenerativas del SNC, asociadas a deterioro neurológico, que constituyen una complicación de la enfermedad de causa discutida. Nuestro objetivo es describir las manifestaciones radiológicas de la HCL en el SNC en los pacientes pediátricos.
Langerhans cell histiocytosis (LCH) is a rare disease characterized by the accumulation within tissues of anomalous dendritic cells similar to Langerhans cells. The clinical presentation varies, ranging from the appearance of a single bone lesion to multisystemic involvement. Central nervous system (CNS) involvement, manifesting as diabetes insipidus secondary to pituitary involvement, has been known since the original description of the disease. Two types of CNS lesions are currently differentiated. The first, pseudotumoral lesions with infiltration by Langerhans cells, most commonly manifests as pituitary infiltration. The second, described more recently, consists of neurodegenerative lesions of the CNS associated with neurologic deterioration. This second type of lesion constitutes a complication of the disease; however, there is no consensus about the cause of this complication. Our objective was to describe the radiologic manifestations of LCH in the CNS in pediatric patients.
Langerhans cell histiocytosis of the parietal bone with epidural and extracranial expansion - Case report and a review of the literature
2010, Neurologia i Neurochirurgia Polska
Citation Excerpt :
No extracranial component was present. Lee et al. [14] presented a case of an 8-year-old boy who was admitted to hospital with symptoms of increased intracranial pressure after head injury. CT examination revealed epidural haematoma over an occipital lobe overlaid by an osteolytic area with extracranial expansion of abnormal tissue.
Langerhans cell histiocytosis is a rare neoplasm that belongs to the histiocytic and dendritic cell neoplasm group according to the 2008 WHO classification. It has been defined as neoplastic proliferation of Langerhans cells that express CD1a and S-100 proteins and have Birbeck granules on the ultrastructural examination. Clinical presentation and behaviour are heterogeneous and can range from a solitary lytic bone lesion with a favourable course to a fatal disseminated leukaemia-like form, with a wide spectrum of intermediate clinical presentations between these two extremes. Here, we present a case report of a solitary calvarial lesion in an adolescent boy along with a review of the literature. Presenting features, initial diagnostic evaluation and treatment protocol of a unifocal monosystemic calvarial location of LCH are presented.
Histiocytoza komórek Langerhansa to rzadki nowotwór należący wg klasyfikacji WHO z 2008 r. do grupy nowotworów komórek histiocytarnych i dendrytycznych. Jest ona definiowana jako nowotworowy rozrost komórek Langerhansa wykazujących ekspresję białek CD1a i S-100 oraz obecność ziarnistości Birbecka w badaniu ultrastrukturalnym. Klinicznie jest schorzeniem heterogennym obejmującym szerokie spektrum pacjentów, poczynając od chorych z pojedynczym ogniskiem osteolitycznym i korzystnym rokowaniem aż do chorych ze źle rokującym rozsiewem ogólnoustrojowym przypominającym białaczkę. Poniżej zaprezentowano opis pojedynczego ogniska osteolitycznego w kościach sklepistości czaszki obejmujący obraz kliniczny, zakres zalecanych badań diagnostycznych oraz schemat postępowania w przypadkach pojedynczych ognisk osteolitycznych kości czaszki wraz z przeglądem piśmiennictwa.
Solitary intracerebral langerhans cell histiocytosis: report of one case and literature review
2007, Revue de Medecine Interne
L'histiocytose langerhansienne est une pathologie à développement multiviscéral. Une atteinte neurologique est possible mais peu fréquente.
Nous rapportons un cas de tumeur intracérébrale prérolandique droite révélée par une crise épileptique partielle motrice chez un homme âgé de 31 ans. L'analyse anatomopathologique retrouvait une histiocytose langerhansienne (avec expression immunohistochimique par les cellules de Langerhans de la protéine S100 et de CD1a) dont le bilan d'extension fut négatif. L'évolution était favorable après résection chirurgicale complète. Une revue exhaustive de la littérature de ces formes pseudotumorales cérébrales de l'histiocytose ainsi qu'une discussion des manifestations neurologiques de cette maladie a été réalisée.
L'histiocytose langerhansienne peut se révéler par une lésion neurologique cérébrale focale.
Langerhans cell histiocytosis is a multivisceral pathology. Neurological manifestations are rare.
We report the case of a 31 year old man hospitalized for left partial motor seizure revealing a right frontal tumor with criteria for histiocytosis X. The histological and biological examination found criteria for Langerhans cell histiocytosis (CD1a and S100 reactivity). The check-up for extracerebral localisations of the disease was negative. The outcome was favourable after a total surgical resection. The review of the literature and a discussion on neurological manifestations of this disease were carried out.
A neurological manifestation can be the first and only symptom of a Langerhans cell histiocytosis.
Treatment of cerebral Langerhans cell histiocytosis
1999, Journal of the Neurological Sciences
Cerebral Langerhans cell histiocytosis (LCH) is a rare granulomatous disorder which may be primary or secondary or solitary or multiple. Brain structures outside the hypothalamic–pituitary axis are only scarcely involved, even in multisystem varieties. Since there are neither controlled therapeutic trials nor systematic analyses of hitherto reported cases, optimal treatment strategies are not known. To evaluate the effect of different treatment modalities, we analyzed previous reports of extrahypothalamic LCH back to 1980 in which the diagnosis was made on the basis of examination of cerebral tissues. Thirty-five histologically examined cases were identified, including 10 patients presenting with multiple cerebral lesions. Adding one own case followed up for 10 years, 16 patients had cerebral involvement secondary to multisystem LCH, while another 20 patients had primary cerebral LCH. The peak incidence was far beyond the pediatric range for both primary and secondary cerebral LCH. Localized lesions can be treated successfully by surgery or radiation following biopsy. Chemotherapy may be an additional option. Multiple lesions can tentatively be controlled by chemotherapy and, possibly, radiation. The ultimate outcome is determined by whether or not recurrencies or de-novo lesions will appear and the course of the systemic disease. Studies addressing the effects of therapy in cerebral LCH are urgently needed.

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Case reportSolitary Langerhans cell histiocytosis lesion of the parieto-occipital lobe: a case report and review of the literature

Abstract

Immunobiology

Hum. Pathol.

Surg. Neurol.

Lancet

Intracranial histiocytosis X: a case report

J. Comput. Tomogr.

Localized hypothalamic histiocytosis: report of a case

Arch. Neurol.

Su un insolito caso di istio-cytosi con cellule di Langerhans della base cranica: aspetti con tomografica computerizzata e risonanza magnetica

Radiol. Med. Torino.

Cerebral and brainstem Langerhans cell histiocytosis

Neuroradiology

Langerhans cell histiocytosis-clinical and epidemiological aspects

Br. J. Cancer

Primary eosinophilic granuloma of the frontal lobe

Virch. Arch. A. Pathol. Anat. Histiopathol.

The normal Langerhans cell and the LCH cell

Br. J. Cancer

The frequency and natural history of diabetes insipidus in children with Langerhans-cell histiocytosis

N. Engl. J. Med.

Langerhans Cell Histiocytosis-definitive diagnosis with the use of monoclonal antibody 010 on routinely paraffin-embedded samples

Am. J. Surg. Pathol.

Langerhans' cell histiocytosis pathobiology and pathogenesis

Semin. Oncol.

The histopathology of Langerhans cell histiocytosis

Br. J. Cancer

Central nervous system disease associated with Langerhans' cell histiocytosis

Am. J. Pediatr. Hematol. Oncol.

Central nervous system disease in Langerhans' cell histiocytosis

Br. J. Cancer.

Left temporal lobe cerebral cortex mass in a 19-year-old male

Ultrastruct. Pathol.

A clinico-pathological study of adult histiocytosis X involving the brain

J. Neurol. Neurosurg. Psychiatry

Solitary eosinophilic granuloma in the frontal lobe

Neurosurgery

Predominantly cerebral forms of histiocytosis-X

Acta. Neuropathol.

Case report
Solitary Langerhans cell histiocytosis lesion of the parieto-occipital lobe: a case report and review of the literature