Gynecological tumors revealing hereditary nonpolyposis colorectal cancer: analysis of a large Lebanese pedigree.

The objective of this study was to evaluate the aggregation of colorectal cancer (CRC) and hereditary nonpolyposis colorectal cancer (HNPCC)-related extracolonic cancers in an extended Lebanese family with HNPCC. This was a pedigree analysis and a prospective follow-up over an 8-year period. The causative germ line mutation was detected using denaturing high-performance liquid chromatography, polymerase chain reaction (PCR) of short fluorescent fragments, and direct DNA sequencing of purified PCR products. The penetrance of CRC is high and accounts for approximately two thirds of risk carriers with an early age of onset (21 years). The extracolonic cancer spectrum includes ovary, endometrium, small bowel, skin, and brain, with an age of onset as early as 30 years. The causative mismatch repair gene mutation is an MSH2 point mutation involving the splice donor site of intron 3 (G-->A). Scrutinized in genomic DNA from 35 consented members, it was found in 18 of them and cosegregates with the cancer phenotype in the family. Early-onset ovarian and endometrial carcinomas may reveal HNPCC families in the Middle Eastern region, with MSH2 germ line mutation. We propose a biannual screening program, starting around the age of 20-25 years, pending additional data on this topic.

Effectiveness of cisplatin in the treatment of anal squamous cell carcinoma.

The therapeutic activity of cisplatin was explored in three consecutive patients with locoregional squamous cell carcinoma of the anus who received no prior therapy. One complete response and two partial responses were achieved. These data, although preliminary, strongly suggest that this disease is sensitive to cisplatin, and further trials are indicated to assess the precise role of this drug in the overall management of anal carcinoma.

Superior vena cava syndrome in childhood: report of ten cases and review of the literature.

Experience with ten children with superior vena cava obstruction is reported. Five patients had non-Hodgkin's lymphoma, two had Hodgkin's disease, two had benign lesions, and one patient was suspected on clinical and radiologic basis to have a lymphoma. The clinical situation at presentation was often critical and required rapid treatment. Radiotherapy and/or chemotherapy were used initially as lymphoma was the most frequent cause of obstruction. Urgent thoracotomy was resorted to when treatment failed. Survival with no evidence of disease for more than 5 years was observed in three children: one had a cystic lymphangioma, another had a mediastinal abscess, and the third had a Hodgkin's lymphoma. Review of the literature from 1951 to 1976 revealed that only 24/150 children reported with superior vena cava obstruction syndrome had mediastinal tumors; the remainder developed the obstruction after surgical procedures on the heart or vena cava.

Histiocytic lymphoma presenting with portal hypertension and bleeding esophageal varices: a case report.

An unusual case of histiocytic lymphoma presenting with portal hypertension and bleeding esophageal varices is reported. Laparotomy revealed bulky nodal disease in the portahepatis compressing the portal vein, common bile duct, hepatic artery, and cystic duct. Liver was free of tumor, and there was no evidence of extra-abdominal disease. Radiation therapy to the right upper quadrant of the abdomen produced a transient remission for one month, followed by relapse in mediastinum and both lung hilae.

Eosinophilic granuloma with involvement of the female genitalia.

Four cases of eosinophilic granuloma involving the female genital tract are reviewed. The disease may remain localized, but more often involves other organs. Of particular interest was the association of the disease with diabetes insipidus in three patients. One of the patients with diabetes insipidus was successfully treated with radiotherapy to the hypothalamic area as soon as the symptoms of the disease appeared. Genital lesions may respond to radiotherapy. Chemotherapy is to be used for disseminated disease. Treatment of this condition requires studying the extent of the disease and establishing early treatment, as with any malignant lesion.

Ten-year experience with childhood rhabdomyosarcoma.

The ten-year (1965-1975) experience of the Pediatric Tumor Clinic with 14 cases of proven CRM was reviewed. Seven cases seen prior to 1970 acted as control. They were treated by surgery and X--ray therapy alone (one received one chemotherapeutic agent). Seven other cases seen after 1970 adhered to a strict protocol of therapy. After surgery (resection or biopsy), roentgenological and hematological survey for diagnosis and staging, they received curative radiotherapy locally in doses of 5,200 r to 6,000 r. Concomitantly a regimen of multiple chemotherapeutic agents (vincristine, dactinomycin and cyclophosphamide) was administered according to the protocol followed at the M.D. Anderson Hospital, Houston. Only one of the seven controls is alive and free of disease for 9 years. Only one of the seven cases who adhered to the protocol died. The other six are alive and free of disease for periods of one to two and a half years (one has a 4 months follow-up so far). One of the six cases presenting with a solitary pulmonary metastasis had a lobectomy 2 and a half years after start of treatment, as no new metastasis developed. Another, presenting with a local recurrence after excision is alive and free of disease 2 years after the star of treatment with the protocol. The results of treatment of CRM with the protocol adopted show a significant improvement in terms of local recurrences, distant metastasis and survival rates.