ABSTRACT
Lymphomatoid granulomatosis is a necrotizing angiocentric and angiodestructive infiltrative process involving primarily the lung, skin, central nervous system, and kidney. The incidence is highest in middle-aged men and is rare in children. We report a case of lymphomatoid granulomatosis involving both skin and lung in a 4-year-old boy. The disease progressed to peripheral T-cell lymphoma, which was unusual in light of recent evidence suggesting a B-cell origin in the majority of cases.
Subject(s)
Lung Diseases/diagnosis , Lymphoma, T-Cell, Peripheral/etiology , Lymphomatoid Granulomatosis/complications , Lymphomatoid Granulomatosis/diagnosis , Skin Diseases/diagnosis , Biopsy , Cell Transformation, Neoplastic , Child, Preschool , Diagnosis, Differential , Disease Progression , Hematopoietic Stem Cell Transplantation , Humans , Immunologic Deficiency Syndromes/complications , Lung Diseases/complications , Lymphomatoid Granulomatosis/etiology , Lymphomatoid Granulomatosis/therapy , Male , Skin/pathology , Skin Diseases/complications , Treatment OutcomeABSTRACT
BACKGROUND: The peak incidence of basal cell carcinoma occurs in the seventh decade of life and is rare in children. When found in the pediatric age group, basal cell carcinoma is usually associated with a genetic defect, such as basal cell nevus syndrome, xeroderma pigmentosum, or nevus sebaceus. In areas of intense UV radiation exposure, such as the southwestern United States, children may be at increased risk of developing this malignancy de novo. OBSERVATIONS: Three children (2 boys, aged 8 and 16 years, and an 11-year-old girl) from Tucson, Ariz, with isolated basal cell carcinoma unassociated with any other disease or syndrome are described. CONCLUSIONS: Basal cell carcinoma in children is probably the result of a combination of UV radiation exposure and genetic background. Early recognition in children can prevent extensive tissue destruction and excess scarring after excision. A higher index of suspicion for basal cell carcinoma may also aid in prompt diagnosis of a possible genetic disorder, such as basal cell nevus syndrome.
Subject(s)
Carcinoma, Basal Cell/pathology , Facial Neoplasms/pathology , Skin Neoplasms/pathology , Adolescent , Carcinoma, Basal Cell/surgery , Child , Facial Neoplasms/surgery , Female , Humans , Male , Skin Neoplasms/surgery , Treatment OutcomeABSTRACT
The pyrrolo[1,2-a]benzimidazole (PBI) reductive alkylating agents have been investigated in this laboratory since their discovery in the late 1980s. Of all the structural modifications of the PBIs investigated so far, the variation of the 3-substituent has the greatest influence on cytotoxicity, toxicity, and in vivo antitumor activity. In the present study, we prepared both the R and S enantiomers of nitrogen-containing 3-substituents possessing hydrogen-bonding capability as well as varying basicity. The rationale was to take advantage of stereoselective DT-diaphorase reductive activation as well as hydrogen bonding in the DNA major groove. As a result of these studies, analogues were discovered possessing among the highest hollow fiber tumor assay scores observed in hundreds of natural and synthetic antitumor agents. Our findings indicate that a relatively basic 3-substituent is required for outstanding PBI cytotoxicity but that the importance of using pure enantiomers is still open to study.
