Familial aggregation of nasopharyngeal carcinoma and other malignancies: a clinicopathologic description

Article Abstract:

In recent years, geneticists and cancer researchers have focussed attention on families with increased incidence of various cancers. In many of these families, cancers such as breast cancer and colorectal cancer are common. Nasopharyngeal carcinoma (NPC) is not normally thought of as occurring in family clusters, although several family clusters of NPC have indeed been reported in the medical literature. Sporadic NPC is quite uncommon in the United States, and occurs more frequently in Asia. The authors report the case of an American family of Scandinavian descent in which five cases of nasopharyngeal carcinoma occurred in three generations. NPC was not the only cancer to cluster in this family, however. Six other family members developed cancers: malignant melanoma, squamous cell carcinoma of the tongue, malignant lymphoma, adenocarcinoma of the colon, bilateral ductal carcinoma in situ, and invasive ductal breast cancer. A statistical analysis revealed that the family members who developed cancer were also more likely to have smoked, to have drunk alcohol, to be exposed to dust, to eat salted or spicy foods, and to spend time in poorly ventilated conditions. Examination of blood specimens for indications of Epstein-Barr virus (EBV) infection revealed nothing unusual. EBV is thought to play a significant role in the development of NPC in Asia, but no evidence could be obtained to suggest a similar role in the present family. The family pedigree suggested that the cancer susceptibility in this family was inherited as an autosomal dominant trait. Examination of chromosome preparations from family members did not reveal any consistent abnormalities. Curiously, however, minor chromosome abnormalities appeared to be common in this family. Half the family members tested had some minor abnormality. It is not known whether these abnormalities, different from patient to patient, are related to the increased rate of cancer in this kindred group. (Consumer Summary produced by Reliance Medical Information, Inc.)

Research, Familial diseases

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Significance of blasts in low-cell-count cerebrospinal fluid specimens from children with acute lymphoblastic leukemia

Article Abstract:

The central nervous system (CNS) is relatively protected from many drugs and other substances in the blood. An important advance in the treatment of acute lymphoblastic leukemia (ALL) was the recognition that leukemic cells can hide in this sanctuary and escape destruction by chemotherapeutic agents. Routine presymptomatic treatment of the central nervous system is now given to children with acute lymphoblastic leukemia. However, 5 to 10 percent will still experience a CNS relapse. In the majority of cases, CNS relapse is asymptomatic, although neuropsychologic symptoms are common. One method that may prove useful for the early recognition of CNS relapse is the examination of cells in a specimen of cerebrospinal fluid. However, it is unclear what criterion is most appropriate for predicting a CNS relapse. In a study of 349 children with ALL, a total of 4,543 specimens of cerebrospinal fluid were examined. A criterion was established called the low-count specimen with blasts (LCB); a specimen with fewer than six white blood cells per microliter containing more than five percent blasts was considered to be a LCB. (A blast is a cell engaging in proliferation, in contrast with most leukocytes which are quiescent.) Of the 332 children who could be evaluated, 25 (7.5 percent) suffered CNS relapse. Of these, 22 (88 percent) had at least one LCB specimen. Sixteen children had two or more LCBs during their remission; nine had CNS relapse by standard criteria and a full 15 of 16 had CNS relapse on criteria that included the cerebrospinal fluid findings. The low-cell-count specimen with blasts shows promise for the early detection of CNS relapse in children with acute lymphoblastic leukemia. Whether such early diagnosis can be translated into therapeutic gains remains to be determined. (Consumer Summary produced by Reliance Medical Information, Inc.)

Medical examination, Prognosis, Lymphoblastic leukemia in children, Acute lymphocytic leukemia, Childhood leukemia, Cerebrospinal fluid

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Mucoepidermoid carcinoma of the parotid as a second malignant neoplasm in children

Article Abstract:

Eight to 12 percent of adults who have survived cancer which occurred in childhood develop second malignant neoplasms (SMN) after a latent period of approximately 20 years. Most SMNs are osteosarcomas (cancer of the bones), cancers of the soft tissues and malignancies of the blood cells. Two children developed cancer of the parotid gland, one of the largest pair of salivary glands, six and nine years after multiple drug chemotherapy and irradiation as treatment for acute leukemia. The clinical and pathological findings of these two cases are reported. The children were diagnosed with mucoepidermoid carcinoma, cancer of glandular tissues, which involved the ducts of the salivary glands. Neither SMNs or primary cancer of the salivary glands are commonly seen in children. A survey showed that only 6 percent of 2,410 salivary gland tumors were reported in people under the age of 20. Since tumors of the salivary glands are so rare in children, it is thought that the cancer in these two cases was related to the previous treatment of the patients' leukemia. Both benign and malignant tumors of the head and neck, originating in the salivary glands, thyroid, and brain have been observed in patients who have received irradiation during childhood. The treatment of childhood malignancies with irradiation and chemotherapy can cause mutations which may result in the development of SMNs years after treatment. (Consumer Summary produced by Reliance Medical Information, Inc.)

Care and treatment, Case studies, Causes of, Complications and side effects, Radiation, Antineoplastic agents, Cancer in children, Childhood cancer, Leukemia, Mouth cancer, Head and neck cancer

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