Biomedical subjects
Publications and source records attributed to A Cangir.
Ten children with lymphoepithelioma of the nasopharynx were reviewed. Six had regional spread at presentation and four developed distant metastases. Seven patients are alive, six free of disease. The 5-year survival rate was 62.5% of this series. Five patients presented later with endocrine abnormalities probably related to the radiotherapy; these endocrine problems were minimal and responded to therapy.
Combination chemotherapy with adriamycin and DTIC was used in 102 evaluable patients under 15 years of age who had previously treated metastatic solid tumors. Responses, defined as 50% or more reduction in all tumor masses, occurred in 10 out of 27 patients with neuroblastoma, 3 out of 8 patients with Wilms tumor, 7 out 15 patients with Ewing sarcoma, 2 out of 6 patients with osteosarcoma, 5 out of 13 patients with rhabdomyosarcoma, and 15 out of 33 patients with miscellaneous tumors which included a patient who had a complete regression of an extensive juvenile angiofibroma. Response rate to combination chemotherapy with adriamycin and DTIC in patients with Ewing sarcoma was significantly superior to the response rate obtained with adriamycin alone in another Southwest Oncology Group Study. Major toxicity included nausea, vomiting, myelosuppression, high incidence of pneumocystis carinii pneumonia (5 patients) and congestive heart failure (4 patients). There was 7 drug-associated deaths due to sepsis (1), pneumocystis carinii pneumonia (4), and congestive heart failure (2).
Trichosporon cutaneum is a fungus known to cause superficial nodules over the distal third of hair shafts, mainly scalp hair, and to produce a clinical entity known as piedra. This superficial mycosis occurs mostly in temperate and tropical regions and is rarely seen in North America. Trichosporon cutaneum spesis is described here in a 12-year-old boy with acute lymphocytic leukemia in relapse. To our knowledge this is the first case reported in the literature. Emphasis is made of the increasing rate of fungal diseases as well as of "opportunistic" infections in this type of immunosuppressed patient.
A four-drug adjuvant chemotherapy regimen (CONPADRI-I) was utilized in the primary treatment of 18 children with osteogenic sarcoma. All patients had surgical amputation for the primary lesion. The children then received cyclophosphamide, vincristine, melphalan, and adriamycin in defined combinations intermittently over a 72-week period. Of the 18 patients, 10 (55%) remain free of disease 24 months or longer from time of amputation.
Pretreatment characteristics of 48 infants (under 2 years of age) with leukemia treated over a period of 18 years at a single institution were studied in relation to response to therapy, extramedullary involvement, and survival. In order to provide a basis of comparison, 45 of the infants were each paired with an older (2-9 years of age) leukemic child, who had the same race, and disease type, who was treated within the same period of time and received similar suportive care and chemotherapy, and when possible, was of the same sex. The overall median survival times of infants and their pairmates were 211 days and 445 days, respectively. Initial status of the spleen was the single significant factor in relation to the length of survival. Median survival time for infants with splenomegaly was 186 days compared to a median survival time of 414 days for infants without splenomegaly. The complete response rate was 75% for infants and 80% for the pairmates. Median duration of remission was 84 days for infants and 280 days for their pairmates. Initial peripheral leucocyte count was significantly related to the duration of remission; patients with very high leucocyte counts had the shortest remissions. The duration of remission increased throughout the period covered by this study, however, prognosis relative to pairmates remained poor. Central nervous system leukemia occurred in 44% of infants and 40% of the pairmates; incidence of CNS leukemia was much higher (70%) in infants under one year of age than in infants one year old (37%). Extramedullary leukemia occurred at other sites in 46% of the infants and 31% of the pairmates. The only pretreatment characteristic of prognostic importance for predicting extramedullary involvement was the patient's age at diagnosis.
Twelve patients with osteosarcoma but without metastases were treated with adjuvant chemotherapy following surgical amputation for the primary lesion. The chemotherapy regimen utilized vincristine, actinomycin D, and cyclophosphamide (in high pulse doses) administered intermittently over a 55-week period. Four of the 12 patients (33%) are surviving with no evidence of disease 54 months or longer from time of surgery. These results suggest that the 3-drug combination used in this study should be considered in the formulation of future multidrug adjuvant chemotherapy programs in the management of patients with osteosarcoma.
Incontinentia pigmenti, a syndrome of developmental defects, was found to be associated with acute granulocytic leukemia in a 4-month-old black girl. She has been responsive to antileukemic drugs, and remission has been induced without difficulty after each relapse. Although survival time was expected to be very short because of her age, race, type of leukemia, and initial peripheral leukocyte count at time of diagnosis, she is still alive 35 months after diagnosis of leukemia. One may speculate that the prolonged survival time might be related to the unknown genetic defect.
Endocrine complications after radiotherapy for tumors of the head and neck are thought to be relatively rare. The availability of synthetic hypothalamic hormones for clinical investigations and the radioimmunoassay of hormones have enabled us to study function of the hypothalamic pituitary axis in 15 patients who had radiotherapy for nasopharyngeal cancer. Fourteen had evidence of endocrine deficiency. Twelve patients had evidence of hypothalamic dysfunction, 7 developed primary pituitary hormone deficiencies, and 3 developed primary hypothyroidism. These results indicate that [1] secondary hypopituitarism due to a hypothalamic lesion after radiotherapy for nasopharyngeal cancer may be more common than suspected in the past; [2] primary hypopituitarism after irradiation of extracranial tumors can occur; and [3[ primary hypothyroidism may result from irradiation of regional neck nodes.
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A recurrent mediastinal mass, believed to be thymus, in an infant with classic idiopathic histiocytosis-X underwent massive cavitation following chemotherapy. Both the mass and cavitation resolved. The exact nature of development of air inside the mass is not known but communication with the airways or airspaces of the lungs is suspected.
Fourteen patients with aggressive juvenile papillomatosis were treated with systemic administration of alpha-type interferon (IFN). This initial dosage of 2 million units alpha-interferon/m2 was modified if a favorable response in the papilloma growth occurred, or if persistent drug-related side effects developed. Half of the patients showed a sustained response while on IFN, and two patients had a complete response. Persistent elevation in SGOT was the main dose-limiting toxicity, especially in infants below age seven years. All side effects subsided after the drug was discontinued. Further studies are recommended.
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