Familial juvenile polyposis coli. A clinical and pathologic study of a large kindred.

A kindred with familial juvenile polyposis coli is described. Of 92 family members, 26 have had symptoms consistent with polyposis, and a definite diagnosis of polyposis has been made in 19. Two family members have developed colorectal carcinoma before age 40 yr. The lesion of juvenile polyposis is a characteristic nonneoplastic polyp that is distinct from the neoplastic lesion of adenomatous polyposis. Patients with this disorder develop symptoms at an earlier age than those with adenomatous polyposis. Both variants of polyposis appear to have a dominant pattern of inheritance. Patients with juvenile polyposis who present in infancy are likely to have severe symptoms and complications. Although the premalignant potential of the juvenile polyp remains in doubt, there may be an increase risk of gastrointestinal cancer in these patients and their families.

Sampling procedures in estrogen receptor determinations.

To determine the significance of proteolysis and delayed freezing of tumor samples on estrogen receptor levels, values from 19 of 31 biopsy specimens were compared with that in remaining tumor at the completion of mastectomy. There was a 15-100% decrease in receptor content. Time-decay studies on selected postmastectomy samples showed a further decrease in estrogen receptor content inversely proportional to the time it was exposed to room temperature. Factors that govern the valid measurement of receptor levels include tumor cell concentration, tumor necrosis, and time between devascularization of the specimen to freezing. A carefully procured histologically confirmed sample of fresh tumor is necessary for reliable estrogen receptor values.

Certain aspects of analysis of complement dependent antibody in breast cancer patients.

Specific anti-breast cancer complement dependent antibody (CDA) was sequentially determined in the sera of patients before and after mastectomy or during progressive recurrent breast cancer. Several observations were made. (1) There appears to be a different pattern of antigenic cross-reactivity detected by cancer patients' anti-tumor CDA than by their anti-tumor cell-mediated immunity; the former being broader than the latter. (2) Patients can be classified into three categories: those with persistent anti-tumor CDA pre- and post-mastectomy; those with detectable CDA post-mastectomy but not pre-mastectomy; and those with only occasionally detectable CDA. (3) Some sera obtained from patients with clinically evident tumor inhibited anti-tumor CDA found in the sera of other cancer patients. The significance of these results are discussed.

Antigenic cross-reactivity between adenocarcinoma of the breast and fibrocystic disease of the breast.

Peripheral lymphocytes obtained from 12 of 13 patients who had fibrocystic disease (FCD) of the breast were specifically cytotoxic to breast adenocarcinoma cells, as measured in vitro by the microcytotoxicity test. Sera from 6 women with active FCD or metastatic breast cancer could specifically block the cytotoxicity of lymphocytes from either population of patients against cancer cells. This implied extensive antigenic cross-reactivity between benign and malignant hyperplastic disease of the breast. Sera from 4 individuals clinically free of FCD or breast fibroadenoma (FAD) neutralized the blocking activity in the sera of patients with metastatic breast cancer. Patients "cured" of FCD or FAD represented a pool of potential plasma donors for immunotherapy of recurrent breast cancer.

Tumors of the small intestine.

An analysis of 80 cases of malignant and benign tumors of the small intestine is presented, and major clinical features of various histologic lesions are discussed. Tumors of the small intestine are rare, and the small bowel may have intrinsic protective systems against the development of neoplasms. Gastrointestinal bleeding and symptoms of intermittent obstruction are prominent findings in patients with symptomatic benign tumors, although many benign tumors are incidental findings at operation for an unrelated disease. Benign lesions should be removed by local excision. Virtually all malignant tumors are symptomatic with abdominal pain, nausea and vomiting, anorexia and weight loss, and gastrointestinal bleeding being common findings. Earlier diagnosis is essential if the prognosis for patients with small intestinal malignancies is to be improved. A high degree of suspicion must be aroused with any vague, nonspecific gastrointestinal complaints in patients over 40 years of age. Exploratory celiotomy should be performed in symptomatic patients even though no abnormality may be detected on roentgenographic examination.

Gastrocutaneous fistula following radiation therapy for seminoma of the testis.

A case presentation of a gastrocutaneous fistula following orthovoltage radiotherapy for prophylactic lymph node sterilization for seminoma of the testis is recorded. Many factors determine the response of an organ to radiation and with certain circumstances irreversible damage ensues. The pathologic effects of radiation on the stomach in the reported patient illustrates two stages of injury, acute and chronic. The insidious course of chronic injury eventually destroyed gastric and abdominal wall tissue to create a gastrocutaneous fistula 45 months after radiotherapy. Radiation therapy is a critical therapeutic modality in the treatment of neoplastic diseases and should be carefully administered with close followup of patients for acute and latent radiation effects.