Langerhans cell histiocytosis of the clavicle: a case report

A case of Langerhans cell histiocytosis in a 47-year-old male presenting as an aggressive appearing lesion of the clavicle is reported. It illustrates the difficulties of the radiological diagnosis of a solitary bone lesion.     

Association of malignancy and Langerhans' cell histiocytosis: report of three cases

Among 44 children with Langerhans' cell histiocytosis (LCH) seen at the Pediatric Department of the A.C. Camargo Hospital, S?o Paulo, Brazil, three developed malignancy, two before and one after the diagnosis of LCH. Malignancy could be attributed to treatment in one of the three children. Whether the cancer in the other two children represents a chance association of the two processes or is treatment-related, is unknown.     

Langerhans cell histiocytosis of the skin

Cutaneous involvement in Langerhans cell histiocytosis (LCH) occurs in 50% of cases and may be the presenting feature. It is, therefore, important to recognize the wide spectrum of clinical disease that this disorder may adopt in the skin. Cutaneous involvement is not necessarily a benign feature and many patients progress to multi-system disease. There are a number of treatments available for cutaneous LCH. The rationale is to start with the simplest treatment and progress to systemic or interventional therapy as needed.     

Langerhans cell histiocytosis of the orbits

Langerhans cell Histiocytosis is an infrequent disease of the orbit in little children. It is sometimes recognized in a rather late stage. We present three cases and discuss the manifestations and the treatment.     

Langerhans cell histiocytosis of the cervical spine: case report of an unusual location

An unusual location for Langerhans cell histiocytosis of the cervical spine is presented. The osteolytic lesion, instead of being located in the vertebral body, was visualised in the left lateral mass of the fifth cervical vertebra, extending into the vertebral body and through the interapophyseal joint into the lateral mass of the fourth cervical vertebra.     

The epidemiology of Langerhans cell histiocytosis

PURPOSE: During recent years, more intensified systemic and local treatment regimens have increased the 5-year survival figures in localized Ewing's sarcoma to more than 60%. There is, however, concern about the risk of second malignancies (SM) in long-term survivors. We have analyzed the second malignancies in patients treated in the German Ewing's Sarcoma Studies CESS 81 and CESS 86. MATERIALS AND METHODS: From January 1981 through June 1991, 674 patients were registered in the two sequential multicentric Ewing's sarcoma trials CESS 81 (recruitment period 1981-1985) and CESS 86 (1986-1991). The systemic treatment in both studies consisted of a four-drug-regimen (VACA = vincristine, actinomycin D, cyclophosphamide, and adriamycin; or VAIA = vincristine, actinomycin D, ifosfamide, and adriamycin) and a total number of four courses, each lasting nine weeks, was recommended by the protocol. Local therapy in curative patients was either complete surgery (n = 162), surgery plus postoperative radiotherapy with 36-46Gy (n = 274), or definitive radiotherapy with 46-60Gy (n = 212). The median follow-up at the time of this analysis was 5.1 years, the maximum follow-up 16.5 years. RESULTS: The overall survival of all patients including metastatic patients was 55% after 5 years, 48% after 10 years, and 37% after 15 years. Eight out of 674 patients (1.2%) developed a SM. Five of these were acute myelogenic leukemias (n = 4) or MDS (n = 1), and three were sarcomas. The interval between diagnosis of Ewing's sarcoma and the diagnosis of the SM was 17-78 months for the four AMLs, 96 months for the MDS and 82-136 months for the three sarcomas. The cumulative risk of an SM was 0.7% after 5 years, 2.9% after 10 years, and 4.7% after 15 years. Out of five patients with AML/MDS, three died of rapid AML-progression, and two are living with disease. Local therapy (surgery vs. surgery plus postoperative irradiation vs. definitive radiotherapy) had no impact on the frequency of AML/MDS, but local therapy did influence the risk of secondary sarcomas. All three patients with secondary sarcomas had received radiotherapy; however, all three sarcomas were salvaged by subsequent treatment and are in clinical remission with a follow-up of 1 month, 4.3 years, and 7.5 years after the diagnosis of the secondary sarcoma. Thus far, SM contributed to less than 1 % (3/328) of all deaths in the CESS-studies. CONCLUSIONS: The risk of leukemia after treatment for Ewing's sarcoma is probably in the range of 2%. The risk of solid tumors also seems to be low within the first 10 years after treatment and remains in the range of 5 % after 15 years. In the CESS-studies, less than 1% of all deaths within the first 10 years after diagnosis were caused by SM. Effective salvage therapy for secondary sarcomas is feasible.     

Langerhans cell histiocytosis in anogenital region]

We compared thoracoscopic surgery (TS) and open thoracotomy for the diagnosis of interstitial pneumonia. Intraoperative blood loss and duration of postoperative chest drainage were significantly less with TS than with thoracotomy. The length of postoperative hospital stay and social insurance costs with TS was significantly less than with thoracotomy. These results show that TS for the diagnosis of interstitial pneumonia is superior to open thoracotomy in terms of surgical stress and cost.     

Sonographic appearance of hepatic Langerhans cell histiocytosis

Periportal lesions were detected on ultrasound in two cases of Langerhans cell histiocytosis with liver involvement. Hypoechoic or hyperechoic lesions were detected in different stages of evolution. Hyperechoic lesions probably corresponded to periportal inflammation whilst hyperechogenicity suggested xanthomatous change.     

Langerhans cell histiocytosis isolated to the thyroid gland

Isolated Langerhans cell histiocytosis of the thyroid is an extremely rare occurrence, with only one case previously reported. A case of Langerhans cell histiocytosis isolated to the thyroid gland associated with lymphocytic thyroiditis is presented and clinical implications are discussed.     

Central nervous system disease in Langerhans cell histiocytosis

Central nervous system (CNS) disease in Langerhans cell histiocytosis (LCH) is a poorly understood complication of yet unknown frequency. By far the most common manifestation is in the hypothalamic-pituitary system with diabetes insipidus as the leading sign, followed by other endocrinopathies and hypothalamic dysfunction. However, essentially all other parts of the CNS may be involved. On the one hand, space-occupying histiocytic infiltrates may lead to size- and site-depending symptoms, extending from adjacent bone lesions or arising from the meninges or choroid plexus. On the other hand, a progressive neurological deterioration can occur with mainly cerebellar-pontine symptoms. In this article, these clinical patterns are described in correlation with the morphology on MR imaging and histopathology. Further, the therapeutic strategies are reviewed critically, and guidelines for the management of patients with LCH-related CNS disease are presented.     

Cutaneous reactions following herpes zoster infections: report of three cases and a review of the literature

Three patients, one healthy and two immunocompromised, developed cutaneous reactions that histologically mimicked granuloma annulare at sites of resolved varicella-zoster virus (VZV) reactivation infections. Variable latency periods between the infection and the granulomatous reaction were noted. As in other case reports, the presence of VZV DNA in these lesions was inconsistently demonstrated by the polymerase chain reaction (PCR) and appears more common in early, as opposed to late, post-zoster granulomas. In addition to various granulomatous reactions, vasculitic and neoplastic eruptions following resolved VZV infections have been described and are reviewed here. Therapeutically, topical, intralesional and systemic corticosteroids, as well as acyclovir, have been tried with inconsistent results. Although the pathogenesis remains unclear, the presence of VZV DNA in early lesions that histologically do not display viral cytopathic changes, suggests the virus induces an atypical delayed hypersensitivity reaction not affected by antiviral therapy.     

Low-dose oral etoposide monotherapy in adult Langerhans cell histiocytosis

BACKGROUND: The purpose of this study was to test the disease-controlling effect of low-dose oral etoposide monotherapy in adult-onset multisystem Langerhans cell histiocytosis. There are no previous reports of low-dose etoposide monotherapy for this condition. OBSERVATIONS: A 27-year-old man with a 7-year history of multifocal chronic Langerhans cell histiocytosis presented with severe disabling ulcers in intertriginous areas. He had previously been treated with 2 different regimens of antitumoral chemotherapy; one had to be discontinued due to myelosuppression and the other had proved ineffective. We treated with oral etoposide monotherapy at 50 mg/d (22 mg/m2 per day) for 21 days. The treatment was repeated at 28-day intervals for a total of 6 cycles. A rapid initial response with subtotal diminution of the involved skin area was found. No adverse effects were observed. The clinical picture has remained stable during the 7 months following cessation of therapy. CONCLUSION: Low-dose oral etoposide treatment is an adequate therapeutic measure for prolonged disease control in adult-type Langerhans cell histiocytosis.     

CNS sequelae in Langerhans cell histiocytosis: progressive spinocerebellar degeneration as a late manifestation of the disease

Central nervous system involvement in Langerhans cell histiocytosis (LCH), formerly known as histiocytosis X, is manifested mainly by diabetes insipidus reflecting local infiltration of Langerhans cells into the posterior pituitary or hypothalamus. We describe two patients with progressive spinocerebellar degeneration appearing 4 and 6 years after the initial diagnosis of LCH. No correlation was found between the clinical course of the disease or its treatment and the neurological impairment. An extensive search for metabolic, toxic, neoplastic, and hereditary etiologies for progressive cerebellar degeneration was negative.     

Langerhans cell histiocytosis presenting as bilateral eosinophilic granulomata in the molar region of the mandible. A case report

Septic arthritis with Haemophilus influenzae is infrequent in adults and often associated with an extra-articular septic focus. We report the case of a septic arthritis caused by H. influenzae in an elderly (89-year-old) female patient in whom an transoesophageal echocardiogram showed an aortic valve endocarditis.     

Endocrinological aspects of Langerhans cell histiocytosis complicated with diabetes insipidus

Langerhans cell histiocytosis (LCH) is a rare disorder and may be complicated with hypopituitarism and diabetes insipidus (DI) due to invasion of the hypothalamic-pituitary area. In this study, 10 patients with complete (4) and partial (6) type central DI were found among 125 LCH patients in our hospital records. The water deprivation test, followed by the pitressin test, was performed to confirm DI. Hypothalamic-pituitary endocrine function tests were carried out on these 10 patients at the initial diagnosis and during follow-up. All patients revealed growth hormone insufficiency in the insulin hypoglycemic tolerance test. Four patients had impairment of cortisol secretion, demonstrated by insulin hypoglycemic stimulating test results. Two patients had poor response in the thyrotropin releasing hormone stimulating test. Two patients had only partial responses in the luteinizing hormone releasing hormone test. Four patients had hyperprolactinemia. All patients underwent surgical treatment followed by chemotherapy and/or radiotherapy. One patient completely recovered from the endocrine disorder, 3 patients required smaller doses of desmopressin, and one patient had normal adrenal, thyroid, and gonadal function. Hypothalamic-pituitary disorders in LCH should not be neglected. Treatment of LCH can partially or completely reverse associated endocrine disorders. Therefore, endocrine studies and hormone replacement should be mandatory for patients with LCH.     

The role of radiology in the diagnosis and follow-up of Langerhans cell histiocytosis

Diagnostic imaging plays a major role in the management of patients with Langerhans cell histiocytosis. Plain radiography depicts most lesions. Nuclear scintigraphy may detect additional areas of bone involvement, but its routine use is controversial. Ultrasonography may be used to evaluate the abdomen for evidence of solid organ involvement. CT and MR imaging are often of great value in clarifying and delineating findings seen on plain radiographs and other imaging modalities. Ultimately, the choice of imaging study depends on the patient's clinical presentation and the body part affected.     

Controversies and new approaches to treatment of Langerhans cell histiocytosis

There continues to be genuine ambivalence as to whether Langerhans cell histiocytosis (LCH) is a primary neoplastic or immuno-dysregulatory disorder. Treatment strategies have moved from one camp to the other depending upon the most current alleged successes or failures. This has been particularly true for patients who fall outside of the sphere where treatment is minimal or where known treatment approaches are clearly beneficial. However, there is growing evidence that LCH is both the result of clonal proliferation of Langerhans cells and the immunologic consequence of increased cellular activation. This new knowledge should be the basis for the development of new therapeutic approaches for patients with LCH and its complications.     

Bullous sarcoidosis: a report of three cases

Three cases of pulmonary sarcoidosis presented as bullous emphysema with severe airflow obstruction, and the diagnosis of sarcoidosis was unsuspected for at least 2 years. Potential mechanisms of bullous emphysema from sarcoidosis are discussed. The physician should suspect sarcoidosis as the cause of bullous emphysema when young patients who have smoked relatively few pack-years present with emphysema or severe airflow obstruction. Additional clues are the presence of mediastinal adenopathy on a chest radiograph or a CT scan and a history consistent with extrapulmonary sarcoidosis.