Langerhans cell histiocytosis presented as bilateral otitis media and mastoiditis

Langerhans cell histiocytosis (LCH) is a rare disease that may affect multiple organs. The etiology of LCH remains unclear to date. It is currently believed that clonal accumulation and proliferation of CD1a-positive Langerhans cells are causative. The term LCH or histiocytosis X refers to three separate illnesses (listed in order of increasing severity): eosinophilic granuloma, Hand-Schüller-Christian disease and Letterer-Siwe disease. A seven-month-old boy presented with history of recurrent bilateral otitis media and rash and seborrheic areas on his scalp. Two days prior, his mother noticed a small lump over the right mastoid. Lateral skull X-ray (Schüller) was evidence for lytic lesion on right temporal bone. The computerized tomography scan showed inflammatory changes with bone erosion. During surgical exploration, fragile slightly yellowish tissue with necrotic areas was found that was determined as LCH on histology. Chemotherapy was subsequently initiated. The initial presentation of LCH with bilateral ear and skull involvement is a very rare condition. The signs and symptoms of otologic histiocytosis can mimic those of acute and chronic infectious ear disease. Only a surgically obtained biopsy leads to definitive diagnosis and appropriate therapy.     

Unusual manifestations of Langerhans cell histiocytosis of the head and neck

Langerhans cell histiocytosis of the head and neck is an uncommon histiocytic proliferative disorder that often clinically resembles an inflammatory process. A pseudoaneurysm of the external carotid artery was found by contrast-enhanced CT and confirmed by Dopper ultrasonography and angiography in a 9 year old girl with a lytic lesion of the mandible, a prior severe tracheal inflammatory process, and sclerotic lesions of the sphenoid wings. Biopsies of the mandibular lesion, pseudoaneurysm, and trachea demonstrated Langerhans cell histiocytosis. In cases of LCH with head and neck involvement, contrast-enhanced CT allows adequately detailed initial evaluation of the protean manifestations of this disorder.     

Concurrent Langerhans cell histiocytosis and nephroblastoma

Both Langerhans cell histiocytosis (LCH) and nephroblastoma are rare in children. We report herein the first case of a patient with both diseases concurrently. A 2‐year‐old female presented with bone pain and swelling of the right humerus. As a result of the local incision biopsy, she was diagnosed as LCH. A nephroblastoma of the left kidney was discovered during her staging work‐up. After complete resection of the nephroblastoma, she received standard chemoradiotherapy for nephroblastoma. She is alive without relapse 14 months after initial presentation. Pediatr Blood Cancer 2009;52:662–664. © 2009 Wiley‐Liss, Inc.     

Cystic angiomatosis of bone: MR findings

Cystic angiomatosis of bone is a rare disorder characterized by widespread lytic bone lesions with or without associated soft tissue or visceral lymphangiomatosis [1–5]. The disorder is often initially incorrectly diagnosed as Langerhans cell histiocytosis [1, 2]. Unfortunately, biopsy of long bone lesions will often yield only fluid, thereby making a histologic diagnosis difficult. This report describes the MR findings in a patient with cystic angiomatosis of bone. We believe that the MR findings are highly suggestive of the correct diagnosis and that MR should eliminate the need for excisional biopsy in future cases.     

Use of 2-chlorodeoxyadenosine to treat infantile myofibromatosis

A 3-year-old boy had fever and bone pain. Magnetic resonance imaging of his femurs showed marrow replacement; iliac crest marrow biopsy revealed myelofibrosis. Although the pathologic criteria for Langerhans cell histiocytosis were not met, the clinical picture led to treatment with etoposide and methylprednisolone, without clinical improvement. One month after presentation, generalized tonic-clonic seizures occurred, and magnetic resonance imaging revealed parenchymal brain lesions. 2-chlorodeoxyadenosine was used. Because of the unexpected lack of response to etoposide and methylprednisolone, a second bone biopsy was performed. The diagnosis was revised to infantile myofibromatosis. After six courses of 2-chlorodeoxyadenosine, brain and bone lesions regressed, with resolution of the clinical symptoms.     

Langerhans cell histiocytosis case with dense metaphyseal band sign

Eosinophilic granuloma, a type of Langerhans cell histiocytosis, exhibits a classic vertebral collapse, which is called vertebra plana (Calve's disease) and it manifests as a solitary bony lesion. Vertebra plana can cause severe pain in patients. Bisphosphonates (clodronate, pamidronate and zoledronic acid) have been recently used to treat osteolytic bone lesions of LCH. Zoledronic acid has 100 times relative potency that of pamidronate. We report a case of a 10‐year‐old girl who had zoledronic acid treatment for severe back pain due to vertebra plana. X‐ray photographs of the patient's body showed dense metaphyseal band sign, which can be found in lead poisoning, treated leukemia, healing rickets, recovery from scurvy, vitamin D hypervitaminosis, congenital hypothyroidism and hypoparathyroidism. Increased biological potent zoledronic acid deprived her of severe back pain due to vertebra plana and might cause dense metaphyseal band sign of her skeleton. Conclusion; We have cured the severe back pain of a 10‐year‐old girl case of eosinophilic granuloma with zoledronic acid. After that treatment, X‐ray photographs of the patient's body showed dense metaphyseal band sign. There have been few such cases reported until now.     

22月龄女孩外阴反复皮疹半年余

患儿,女,22个月,因发现外阴部反复皮疹半年余就诊。病理组织活检诊断为朗格汉斯组织细胞增生症（LCH）,评估患儿全身情况,无其他系统受累表现,确诊为LCH（皮肤型）。对于仅发生于外阴部的皮疹,临床表现无特异性时,排除传染性软疣、性传播疾病、皮脂腺异位症等疾病后,应考虑LCH可能。     

Eyelid nodule: a rare presentation of Langerhans cell histiocytosis

Langerhans cell histiocytosis occurring as an isolated tumor of eyelid has rarely been reported. We report an unusual case of a 5-year-old boy who presented with a smooth nodular lesion over the right lower eyelid accompanied with hyperemia for a month. The biopsy and CD1a positivity confirmed it to be Langerhans cell histiocytosis. It was localized to the eyelid as no other organ was involved. Although Langerhans cell histiocytosis of the eyelid is exceptional, it must be included in the differential diagnosis of eyelid nodular lesions and the diagnostic and the subsequent management must be multidisciplinary.     

Interleukin-2 therapy of Langerhans cell histiocytosis

A 20-month-old girl was diagnosed with Langerhans cell histiocytosis on the basis of a seborrheic skin rash, multiple punched out bony lesions, and skin biopsy findings. Combination therapy including α-interferon, vincristine, vindesine, cyclophosphamide, etoposide, cisplatin, betamethasone, THP-adria-mycin, cytarabine and methotrexate was ineffective. Because cyclophosphamide enhanced lesion growth within the skull, we administered an intravenous infusion of interleukin-2 with remarkable efficacy. The reduction in lesion size with interleukin-2 treatment paralleled the increase in the percentage of CD16-positive natural killer cells in the peripheral blood. Interleukin-2, Langerhans cell histiocytosis, natural killer cells     

Langerhans and non-Langerhans histiocytosis]

Although Langerhans cell histiocytosis (LCH) was described a century ago, its cause and pathogenesis are still unknown. A wide spectrum of disease and variable clinical behavior are characteristic. The clinical varieties of this enigmatic disease range from a lethal leukemia-like disorder that primarily affects infants to a curable solitary lytic lesion of bone. LCH is a clonal proliferative disorder of histiocytes that resembles in morphology and phenotype the dendritic antigen-presenting Langerhans' cells of the skin and other organs. Despite gaps in understanding, significant improvements in the therapies for this disease have been made. Careful risk stratification is critical for the appropriate administration of therapy. Patients with good prognostic factors may need only observation as their disease spontaneously regresses, or minimal intervention. The active search for more effective treatments for patients with poor prognostic features is a major future challenge for the Histiocyte Society.     

Concomitant nodal involvement by Langerhans cell histiocytosis and Hodgkin's lymphoma

A 10‐year‐old girl with a family history of Hodgkin's lymphoma presented with a 2 month history of cervical lymphadenopathy and weight loss. Biopsy indicated concomitant nodal involvement by Langerhans cell histiocytosis and Hodgkin's lymphoma. Such an association is rare, especially so in children, but is not an isolated phenomenon, thereby prompting the question of whether Langerhans cell histiocytosis is a reactive or a neoplastic process.     

Langerhans Cells Histiocytosis in One Family

Histiocytosis of Langerhans cells (class 1 histiocytosis) includes a range of clinical manifestations that have been described as bone eosinophilic granuloma, Hand–Schüller–Christian syndrome, and Letterer–Siwe diseases. This syndrome represents a spectrum of severity and prognosis of some underlying disorder which is usually sporadic. This report discribes three cases in one family, who developed the disease a few years after their brother was found to be suffering from histiocytosis. All 3 patients had the same clinical manifestations: hyperthermia, eczematic rash, and swelling in skull, hand, and foot. X rays showed lytic areas in the skull and metacarp of fourth finger. Serology for EBV infection was negative. Infiltratration of abnormal Langerhans cells histiocytes were demonstrated in bone biopsies. These patients were given chemotherapy. Case 1 (brother) died 1 year after chemotherapy, case 2 (girl) was given chemotherapy without success. She was given T-cell suppresor (cyclosporine), which induced remission, and case 3 was given chemotherapy, which was successful.     

Langerhans cells histiocytosis in one family

Histiocytosis of Langerhans cells (class 1 histiocytosis) includes a range of clinical manifestations that have been described as bone eosinophilic granuloma, Hand-Schüller-Christian syndrome, and Letterer-Siwe diseases. This syndrome represents a spectrum of severity and prognosis of some underlying disorder which is usually sporadic. This report describes three cases in one family, who developed the disease a few years after their brother was found to be suffering from histiocytosis. All 3 patients had the same clinical manifestations: hyperthermia, eczematic rash, and swelling in skull, hand, and foot. X rays showed lytic areas in the skull and metacarp of fourth finger. Serology for EBV infection was negative. Infiltration of abnormal Langerhans cells histiocytes were demonstrated in bone biopsies. These patients were given chemotherapy. Case 1 (brother) died 1 year after chemotherapy, case 2 (girl) was given chemotherapy without success. She was given T-cell suppressor (cyclosporine), which induced remission, and case 3 was given chemotherapy, which was successful.     

Episodic central neurogenic hyperventilation in an awake child with systemic histiocytosis

Abstract A 3 year old boy with systemic histiocytosis, diabetes insipidus and a lytic parietal bone lesion experienced episodes of central neurogenic hyperventilation 3 weeks after radiation to the head but was conscious and alert at presentation. At admission, the P _ao2 was 133 mmHg, P _aco2 was 8 mmHg and pH 7.65. Magnetic resonance imaging revealed a pontomedullary lesion that resolved during the ensuing year. Central neurogenic hyperventilation has not been described previously as a complication of systemic histiocytosis.     

Vertebral Langerhans-cell histiocytosis in childhood--a differential diagnosis of spinal osteomyelitis

BACKGROUND: The Langerhans cell histiocytosis has lots of different manifestations. Symptoms are not specific enough to identify the disease. In cases of vertebral Langerhans cell histiocytosis it is sometimes difficult to differentiate the lesions from spinal osteomyelitis. PATIENTS: Six children with Langerhans cell histiocytosis who had an operative treatment at our clinic in 1981 to 1995 have been reviewed. All patients had a localised vertebral presentation at the onset of the disease. METHODS: In this study we have been showed the clinical, radiological, histological and laboratory findings of our patients with Langerhans cell histiocytosis for an average of six years. Especially the use of diagnostic tools has been examined. RESULTS: Diagnostic tools such as radiogram, computerised tomography, bone scan and MRI and their value in finding the correct diagnosis have been described. Especially the use of MRI in early diagnosing has been discussed. Early changes in MRI have been presented as well as the useful radiological signs and MRI signs to differentiate Langerhans cell histiocytosis and spinal osteomyelitis. CONCLUSIONS: Vertebral Langerhans cell histiocytosis in childhood is a possible differential diagnosis to spinal osteomyelitis.     

Langerhans cell histiocytosis in a patient with stage 4 neuroblastoma receiving oral fenretinide

Langerhans cell histiocytosis (LCH) has previously been reported in association with other malignancies. The pathogenesis of LCH and its relationship to other malignancies is poorly understood. We present a novel case of a child who developed an LCH bone lesion while receiving a Phase I protocol therapy with oral fenretinide/Lym‐X‐Sorb (4‐HPR/LXS) powder for neuroblastoma. Pediatr Blood Cancer 2009;53:1111–1113. © 2009 Wiley‐Liss, Inc.     

Symptomatic Langerhans-cell-histiocytosis of the cervical spine in a child: case report

We report on a six year old female presented with a painful torticollis and a hemidysaesthesia caused by destruction of the third cervical vertebra and a paravertebral soft-tissue mass. At diagnostic routine finally a biopsy gives the diagnosis of Langerhans cell histiocytosis. In a second open approach the destructed vertebral body was replaced by a precisely adjusted autologous bone interponate and the patient was maintained in halo vest immobilisation. The outcome is described and an overview of the current literature is given.     

Coexistence of neuroblastoma detected on staging of Langerhans cell histiocytosis

Langerhans cell histiocytosis (LCH) is a rare proliferative disease accompanied by the accumulation of pathological Langerhans cells, which often spreads into multi‐site and multi‐organ systems. We here describe a girl with a history of Kawasaki disease and cervical lymphadenopathy who presented with occipital LCH. Adrenal tumor was detected on staging evaluation of LCH and was diagnosed as neuroblastoma on resection using laparoscopic surgery. Neither tumor relapsed following chemotherapy for LCH and resection of neuroblastoma. Although LCH often spreads into multi‐organ lesions, invasive biopsy may be needed for tumors with atypical localization for LCH in consideration of the synchronous occurrence of malignancies.     

Predictors of outcome in children with Langerhans cell histiocytosis

BACKGROUND: Our goal was to examine the clinical course of patients with Langerhans cell histiocytosis (LCH), with a special emphasis on bone disease and to attempt to identify and examine the factors that may predict reactivation and overall prognosis. PROCEDURE: We conducted a retrospective chart review of 132 consecutive pediatric patients treated at Children's Hospital Los Angeles for LCH from 1984 to 2001. RESULTS: The risk for reactivation after initial management is significantly higher for patients with multiple bone and those with multiple organ involvement as compared with patients who had a single bone lesion (hazard ratios are 7.1 and 11.6). Patients younger than 1 year in the multiple organ group have an increased risk of death at 2 years when compared with the older patients in that group (hazard ration = 6.2, P = 0.022). Endocrine abnormalities were seen in 20% and 7.5% of patients with or without skull lesions respectively. CONCLUSIONS: Patients with LCH involving only the bones have a significantly better outcome than those with other organ involvement. Patients with multiple organ involvement who are less than 1 year of age are at high risk of death and should be approached more aggressively upfront.     

Langerhans cell histiocytosis of the sphenoid sinus: a case report

Langerhans cell histiocytosis (LCH), previously known as histiocytosis X, is a rare disorder characterized by clonal proliferation and excess accumulation of pathologic Langerhans cells causing local or systemic effects. Bone is the most common organ involved and a single skull lesion is the most frequent presentation of childhood LCH. However, sphenoid sinus is an uncommon condition of involvement in LCH. Here we report a case of LCH in the sphenoid sinus, which occurred in a seven-year-old girl who presented initially with headache. The girl had suffered from headache for one month before she went to an otorhinolaryngologist one week before. Magnetic resonance imaging (MRI) showed a lesion of inflammatory granuloma. Surgery was performed and the disease was diagnosed pathologically as single-site LCH via hematoxylin-eosin (H&E) and immunohistochemical staining.