Langerhans Cell Histiocytosis Involving Second Cervical Vertebra and the Hypothalamus and Pituitary in an Adult.

BACKGROUND: Adult-onset Langerhans cell histiocytosis (LCH) with simultaneous involvement of the high cervical spine and the hypothalamus is rare. CASE DESCRIPTION: We have reported a case of adult-onset LCH in the second cervical vertebra with bony destruction and subsequent diabetes insipidus due to simultaneous involvement of the hypothalamus and pituitary stalk. Magnetic resonance imaging of the hypothalamus and pituitary lesion and immunohistochemistry of the cervical lesion revealed LCH. Posterior fusion of the cervical spine (first, third, and fourth cervical vertebrae) was performed, followed by systemic chemotherapy. The cervical fusion was well maintained, and the patient achieved clinical remission. No new LCH lesion was found during the follow-up of >2 years. CONCLUSIONS: Patients with known LCH of the spine showing new symptoms of diabetes insipidus should be examined for infiltrating lesions of the pituitary stalk or hypothalamus. In cases of severe instability of the spine, surgical treatment should be performed. If multiple and systemic LCH lesions are found, systemic chemotherapy should be administered.

Narrowband ultraviolet B light in Langerhans cell histiocytosis: a case report.

Langerhans cell histiocytosis (LCH) (previously called eosinophilic granuloma, Hand-Schüller-Christian syndrome, Letterer-Siwe disease, and Hashimoto-Pritzker disease) is a rare, heterogeneous disorder with highly variable presentation. LCH commonly affects the skin, as well as internal organs. Because the skin lesions appear benign, and LCH is unfamiliar to most physicians, diagnosis is often delayed. Treatment is controversial, with further clinical study needed. For persons with extensive, skin-limited disease, the existing topical therapies are impractical. We present a child with cutaneous LCH lesions that responded to ultraviolet light phototherapy with minimal adverse effects or patient discomfort.

Rapidly progressing fatal adult multi-organ Langerhans cell histiocytosis complicated with fatty liver disease.

Langerhans cell histiocytosis (LCH) is a clonal neoplasm that shows diverse clinical manifestations and courses of disease progression. The etiology and pathophysiology of LCH remain uncertain. We describe the clinical course of a 23-year-old Japanese woman with multi-system LCH, who showed rapid progression after steroid reduction and developed multi-organ failure. Liver biopsy showed LCH infiltration with fatty degeneration. She was treated with cytarabine, vincristine, and prednisolone according to the Japan LCH study group 02 protocol, without any clinical improvement. Low expression of Ki67 and bcl-2 failed to explain the rapid clinical course. Panhypopituitarism and hypothalamic dysfunction may have caused nonalcoholic fatty liver disease and liver failure. This case indicates that some multi-system LCH patients with hypopituitarism and hypothalamic dysfunction show very rapid progression and are difficult to treat.

Langerhans cell histiocytosis with central nervous system involvement--complete response to 2-chlorodeoxyadenosine after failure of tyrosine kinase inhibitor therapies with sorafenib and imatinib.

Langerhans cell histiocytosis (LCH) is rare in adults, and only a subset of these patients suffers from central nervous system (CNS) involvement. Hence, evidence-based treatment recommendations are lacking. A case of a 20-year-old student with multisystem LCH and extensive CNS involvement is described, who showed a durable response to 2-chlorodeoxyadenosine after prior therapies with the tyrosine kinase inhibitors sorafenib and imatinib. In accordance to the experiences provided by other case series, which are reviewed herein, 2-chlorodeoxyadenosine can be considered an effective and safe option for adult LCH with CNS involvement.

[Langerhans cell histiocytosis of the dens axis]. / Langerhanscelhistiocytose van de dens axis.

BACKGROUND: Langerhans cell histiocytosis, formerly known as histiocytosis X, is characterised by clonal proliferation of pathologic cells resembling Langerhans cells. Langerhans cell histiocytosis is commonly localised in the bones of the skull or in the skin; however, a great variety of foci has been described. CASE: A general practitioner referred a 25-year-old man to the neurologist because of progressive pain in the neck that had arisen spontaneously. The pain had become so severe over a period of 3 weeks that he had had to support his head with both hands to ease it. Chiropractic treatment had resulted in a worsening of the symptoms. On physical examination, two pathologically enlarged lymph nodes were palpable on the left side of the neck. CT, MRI and PET scans revealed an osteolytic lesion at the site of the dens axis (C2). Immunohistochemistry of the lymph node demonstrated positive staining for CD1a and S100, characteristic of Langerhans cell histiocytosis. The patient was treated with chemotherapy and osteosynthesis of the C1-C2-C3 vertebrae; he was able to return to work after 9 months. CONCLUSION: Langerhans cell histiocytosis is an extremely rare condition; its diagnosis is often missed or made at a later time. The golden diagnostic standard is histopathological analysis of the abnormality. The condition's prognosis is related to its extent. Its localisation in the dens axis has not been previously described.

Histiocitose de células de Langerhans como causa de mielopatia: relato de caso e revisão da literatura / Langerhans-cells histiocytosis as a cause of myelopathy: case report and literature review

Histiocitose de células de Langerhans é uma doença uni ou multifocal que acomete osso, tecido mole ou ambos. Relativamente incomum, abrange entidades clínicas anteriormente conhecidas como doença de Hand-Schüller-Christian, Abt-Letterer-Siwe, Hashimoto-Pritzker, granuloma eosinofílico e histiocitose X. Responsável por menos de 1% dos tumores ósseos, muito raramente leva a acometimento neurológico. Sua etiologia é desconhecida e seu tratamento, controverso. A ocorrência de um caso com comprometimento neurológico nos motivou a este relato. Paciente do sexo feminino, 4 anos deidade, com processo expansivo de C7 a T3 e paraparesia crural; após a cirurgia apresentou melhora importante já no segundo pós-operatório. Encontra-se em terapia complementar com corticoide e quimioterapia estando no nono mês de tratamento. Na revisão da literatura foram encontrados poucos relatos de manejo cirúrgico desse tipo de lesão, em razão de sua característica de doença autolimitada. O tratamento adequado pode variar dependendo das características do paciente e de seu comprometimento neurológico.

Langerhans cell histiocytosis is a uni- or multifocal disease that affects bone, soft tissue, or both. Relatively uncommon, covers clinical entities previously known as Hand-Schüller-Christian disease,Abt-Letterer-Siwe, Hashimoto-Pritzker, eosinophilic granuloma and histiocytosis X. Responsible forless than 1% of bone tumors, rarely causes neurological impairment. Its etiology is unknown and the treatment controversial. We report a rare case with neurological impairment. A 4 year-old female children, presented with paraparesis due to expansive process at C7 to T3; soon after surgery showed significant improvement in the second postoperative day; she is in complementary therapy with corticosteroids and chemotherapy in the ninth month of treatment. In the review of the literature we found few reports ofsurgical management of this type of injury, due to its characteristic of self-limited disease. The appropriate treatment may vary depending on the characteristics of patients and their neurological impairment.

Successful treatment of generalized eruptive histiocytoma with PUVA.

Generalized eruptive histiocytoma (GEH) is a rare benign skin disease mainly affecting adults which belongs to the family of non-Langerhans-cell histiocytoses. A 32-year-old Caucasian woman developed disseminated, monomorphic papules of the trunk after a common cold with sinusitis. Mucous membranes, palms and soles were spared. She also suffered from arthralgia without fever or night sweats. After one month, the patient noticed progression of the reddish papules involving the trunk, extremities and face. Clinical as well as histological examination and immunohistochemistry led to the diagnosis of GEH. The clinical examination and laboratory testing were normal, except for eosinophilia in the peripheral blood and bone marrow. No neoplastic diseases were found during thorough examinations. Systemic PUVA therapy produced rapid regression of the skin lesions. After 10 treatments the lesions began to regress leaving slight papules and multiple brown hyperpigmentations. The lesions resolved completely after 20 PUVA treatments. No relapses occurred. Systemic PUVA therapy represents a promising option for the treatment of GEH.

Response to subcutaneous therapy with mistletoe in recurrent multisystem Langerhans cell histiocytosis.

We report a case of histologically proven multisystem (MS) Langerhans cell histiocytosis (LCH) with recurrent disease reactivations after systemic chemotherapy. Through the use of subcutaneous therapy with mistletoe (MT), an inactive disease state was achieved.

An unusual case of adult disseminated cutaneous Langerhans cell histiocytosis.

Langerhans cell histiocytosis (LCH) represents a group of rare histiocytic syndromes characterized by tissue infiltration with dendritic cells. The management of LCH is difficult because these disorders respond inconsistently to immunosuppressive and chemotherapeutic strategies. We describe a refractory and relapsing case of skin and nail limited LCH in a 27-year-old man. He presented with a 7-year history of an erythematous papular eruption of the scalp, ears, face, trunk, axillae, groins, fingernails, feet, and toenails. Diagnosis of LCH was made based on skin histopathology and immunohistochemical staining. Histological studies of biopsy specimens revealed a dense infiltrate of histiocytic mononuclear cells beneath the epidermis; these cells reacted strongly with anti-S-100 antibodies. In addition, CD1a was positive in most of the infiltrating cells. Extensive investigations failed to detect systemic involvement. The patient's cutaneous eruption did not respond to various therapeutic interventions, including phototherapy with oral psoralen with long-wave UV radiation in the A range (PUVA) and cyclosporine. Marked but temporary clinical improvement was achieved with thalidomide, etoposide with systemic steroid, and total body electron beam radiotherapy. Now the patient is on maintenance therapy with thalidomide and is under acceptable control.

Histiocitoma eruptivo generalizado / Generalized eruptive histiocytoma

Presentamos el caso de un varón de 64 años que desarrolló, a lo largo de un periodo de 4 meses, múltiples pápulas asintomáticas distribuidas simétricamente por el tronco y las regiones proximales de las extremidades, respetando cuero cabelludo, palmas, plantas y mucosas. Las lesiones eran discretas, eritematoparduscas, redondeadas, desde ligeramente elevadas a hemisféricas, de 1 a 3 mm de diámetro, y firmes al tacto. Los estudios clínicos, histopatológicos, inmunohistoquímicos y ultraestructurales fueron de histiocitoma eruptivo generalizado. Las pruebas de laboratorio y los estudios radiológicos no mostraron hallazgos anormales. Tras someterse a fotoquimioterapia, las lesiones remitieron, pero más adelante aparecieron nuevos brotes de pápulas. El histiocitoma eruptivo generalizado pertenece a un espectro de enfermedades que pueden solaparse e incluso progresar las unas a las otras. Puesto que la enfermedad podría progresar hacia una dolencia más grave, habría que considerar seriamente revisiones regulares con exámenes clínicos, histológicos e inmunohistoquímicos

We present the case of a 64-year-old male who developed multiple asymptomatic papules over a four-month period, distributed symmetrically on the trunk and proximal regions of the limbs, while the scalp, palms, soles and mucous membranes were spared. The lesions were discrete papules, brownish-erythematous, rounded, ranging from slightly raised to hemispherical, from 1 to 3 mm in diameter and firm to the touch. Clinical, histopathological, immunohistochemical and ultrastructural studies were consistent with generalized eruptive histiocytoma. Laboratory tests and radiological studies did not show any abnormal findings. After being subjected to photochemotherapy, the lesions regressed, but later on new outbreaks of papules appeared. Generalized eruptive histiocytoma belongs to a spectrum of diseases that may overlap; some may even develop into others. As the disease may progress into a more serious ailment, regular checkups with clinical, histological and immunohistochemical examinations should definitely be considered

Primary cutaneous Langerhans cell histiocytosis treated with photochemotherapy.

A 23-year-old man with Langerhans cell histiocytosis presented with asymptomatic, purplish, slightly scaly, confluent papules of one year's duration. Histological studies of biopsy specimens revealed a dense infiltrate of histiocytic mononuclear cells beneath the epidermis; these cells reacted strongly with anti-S-100 antibodies. Extensive investigations failed to detect systemic involvement. He was treated with repeated oral 8-methoxypsoralen (8-MOP) plus ultraviolet A (PUVA) therapy three times a week for two months and then once or twice with maintenance phototherapy. The lesions did not recur during the four-month follow-up period.

Satisfactory remission achieved by PUVA therapy in Langerhans cell hisiocytosis in an elderly patient.

Langerhans cell histiocytosis is currently regarded as a reactive proliferative process of Langerhans cells rather than a malignancy. The disease is characterized by Langerhans cell infiltration of skin, lung, bone and other organs. We report a 74-year-old man with Langerhans cell histiocytosis who had generalized hemorrhagic and crusted papules. He also had diabetes insipidus. Because he did not have any severe constitutional symptoms or failure of vital organs, we applied topical PUVA treatment to his skin lesions, which responded well to the therapy. Diabetes insipidus, however, remained, in spite of X ray radiotherapy for the pituitary lesion.