Activity and toxicity of 2-CDA in Langerhans cell histiocytosis: a single institutional experience.

BACKGROUND: Langerhans cell histiocytosis (LCH) is a rare disorder characterized by clonal proliferation of immature and abnormal bone marrow derived langerhans cells. Treatment is usually multimodal. Potent anti-monocyte as well as immunomodulatory activity of 2-CDA and its proven efficacy in many lymphoproliferative disorders has made 2-CDA a rational choice in treatment of LCH. AIM: To evaluate the efficacy and toxicity profile of 2-CDA in children with relapsed or refractory LCH. SETTING AND DESIGN: This is a pilot study and we present the initial data of the first seven patients treated at our institution. MATERIALS AND METHODS: Seven patients of relapsed and refractory LCH were enrolled from July 2000 to June 2004. The cohort of seven patients included six males and one female with a median age at initiation of cladribine was 2.25 years (range, 1.67 to 7.0 years). Three patients had received one prior chemotherapy regimen while the rest were heavily pretreated. Cladribine was administered over two hours IV daily for five days and repeated every four weeks. RESULTS: After a median of six courses of cladribine (range, 2 to 9), two (33%) patients achieved PR and two (33%) patients have SD on imaging but are clinically better. None experienced grade 3 or 4 hematologic toxicity. At a median follow-up of 19 months (range, 8 to 52 months), five patients remain alive and one patient has died. CONCLUSION: Our study shows that single agent 2-CDA is active and well-tolerated in children with relapsed or refractory LCH.

Primitive neuroectodermal tumor (PNET) as second malignancy after treatment of Hodgkin's disease.

Hodgkin's disease survivors are at an increased risk of developing second malignant neoplasms including secondary bone tumors. Common secondary bone tumors are osteogenic sarcoma and fibrosarcoma. Secondary primitive neuroectodermal tumor is extremely rare in this group. We present below, a rare case of secondary PNET in an 8-year-old child with Hodgkin's disease which developed unusually early outside the radiation portal and discuss potential factors responsible for its causation.

Metastatic gastric adenocarcinoma.

Gastrointestinal tumors represent less than 5% of all pediatric neoplasms. Within this subgroup carcinomas are rare, especially that of stomach. The authors present this rare entity with an equally rare presentation.

Ewing's sarcoma with cutaneous metastasis--a rare entity: report of three cases.

Ewing's sarcoma (ES) is a small round cell tumor, usually arising from flat bones and diaphyseal region of long bones. It is commonly found in the first two decades of life. It is curable when diagnosed in the localized stage and requires multimodality treatment. ES is a chemosensitive tumor. It metastasizes commonly to lung, pleura and other bones. Less common sites of metastasis are lymph nodes, CNS and liver. Skin metastasis is extremely uncommon. It occurs in up to 9% of all patients with cancer. Growth pattern of cutaneous metastasis is unpredictable and may not reflect that of primary tumor. We hereby report three cases of Ewing's sarcoma that developed skin metastasis.

Calf circumference as a predictor of low birth weight babies.

PIP: To identify an inexpensive, reliable predictor of low birth weight capable of being utilized by untrained personnel, anthropometric measures were performed on 1600 newborns in Kanpur, India. This analysis indicated that, for a birth weight of 2500 g, the critical limit of calf circumference was 10.8 cm or less. Critical limits for crown-heel length, head circumference, chest circumference, midarm circumference, and thigh circumference were equal to or less than 45.2 cm, 32.2 cm, 30.1 cm, 9.0 cm, and 15.4 cm. All these measurements were significantly correlated with birth weight. This correlation was highest for calf circumference (r = 0.98), thigh circumference (r = 0.93), and chest circumference (r = 0.86). The highest sensitivity of detecting low-birth-weight infants was found for calf circumference (98.4%), followed by thigh circumference (91.6%) and head circumference (76.7%); specificity was 98.2% for thigh circumference and 90.0% for calf circumference. Traditional birth attendants can be trained easily to screen out high-risk newborns by measuring calf circumference.^ieng

Modified Levinson's test in rapid diagnosis of tuberculous meningitis.

Modified Levinson's precipitation test was done in 64 cases of TBM, 54 cases of TBM with inconsistent CSF finding, and 32 cases of pyogenic meningitis. The test was positive in 93.7% cases of TBM (sensitivity, 93.7%), 85.5% cases of TBM with doubtful diagnosis and in 9.4% cases of pyogenic meningitis (specificity 90.6%) compared to 79.7%, 72.2% and 18.8% in original Levinson's test, respectively. With CSF examination only 66% cases of TBM could be diagnosed while with modified Levinson's test and CSF analysis 89% cases could be diagnosed (p < 0.001). So modified Levinson's test for diagnosis of TBM is better than Levinson's test (p < 0.05) with an added advantage of time saving.