1/34. Normal cellular radiosensitivity in an adult Fanconi anaemia patient with marked clinical radiosensitivity.BACKGROUND: Fanconi anaemia is a rare disease associated with cellular sensitivity to chemicals (e.g. mitomycin C and diepoxybutane); variable but mild cellular radiosensitivity has also been reported. MATERIALS AND methods: A 32-year-old patient with Fanconi anaemia and tonsillar carcinoma, treated by radiotherapy, was found to exhibit profound clinical radiosensitivity. Confluent, ulcerating oropharyngeal mucositis developed after a conventionally fractionated dose of 34Gy and healing was incomplete by 2 months after cessation of therapy. RESULTS: Cellular radiosensitivity assays and RPLD studies from this patient did not suggest any major detectable radiosensitivity. CONCLUSION: There is a discrepancy between the observed clinical radiosensitivity and the usual "predictive" radiosensitivity assays in this patient with Fanconi anaemia.- - - - - - - - - - ranking = 1keywords = carcinoma (Clic here for more details about this article) |
2/34. Squamous cell vulvar carcinoma associated with Fanconi's anemia: a case report.Fanconi's anemia (FA) is a rare autosomal recessive syndrome associated with a strong predisposition to cancer, particularly squamous cell carcinoma (SCC) of various organs. A few cases of lower genital tract neoplasia have been described. We present a 14-year-old black girl with an advanced squamous cell vulvar carcinoma treated with cisplatin chemotherapy plus radiation therapy. The patient died because of fungal sepsis. polymerase chain reaction (PCR) was positive to human papillomavirus (HPV)-16. Vulvar carcinoma is a very rare condition in teenagers, but the association of Fanconi's anemia and SCC of many sites is common. Vulvar carcinoma when associated with Fanconi's anemia is a great treatment challenge.- - - - - - - - - - ranking = 8keywords = carcinoma (Clic here for more details about this article) |
3/34. adenocarcinoma of the stomach in Fanconi's anemia.A case of Fanconi's anemia presenting for the first time at the age of 23 years is described. He died of adenocarcinoma of the stomach 4 months later. As far as the authors are aware, this is the second case of adenocarcinoma of the stomach occurring in Fanconi's anemia. As Fanconi's anemia is known to predispose to malignancy, all patients need detailed evaluation of the gastrointestinal tract by endoscopy as well as radiology for the early detection of gastrointestinal lesions.- - - - - - - - - - ranking = 6keywords = carcinoma (Clic here for more details about this article) |
4/34. Barrett's esophagus and squamous cell carcinoma in a patient with psychogenic vomiting.We report the association of Barrett's esophagus and invasive squamous cell carcinoma of the distal esophagus in a young 31-yr-old woman with a history of self-induced psychogenic vomiting. The development of intestinalized columnar mucosa and esophageal cancer in this young patient illustrates the complicated associations between human behavior and pathogenetic mechanisms involved in esophageal carcinogenesis.- - - - - - - - - - ranking = 5keywords = carcinoma (Clic here for more details about this article) |
5/34. Cytogenetic characteristics of oral squamous cell carcinomas in fanconi anemia.fanconi anemia (FA) is an autosomal recessive syndrome with a marked predisposition to malignancies, in particular acute myeloid leukemia and squamous cell carcinoma of the oral cavity. We examined oral squamous cell carcinoma tissue from two FA patients (FA-A and FA-C) by comparative genomic hybridization. Both tumors, which were negative for human papilloma as well as Epstein-Barr viral sequences, showed multiple alterations with a high proportion of whole-arm chromosomal gains and losses. This combination of features as well as the sites involved in chromosomal breakage are very similar to what is typically observed in non-FA oral tumors. These results suggest that the process leading to early occurrence of oral cancer in FA patients follows a similar pathway as in non-FA cancer patients, which would support a caretaker function for FA genes in the protection against oral carcinogenesis. Since FA patients are uniquely hypersensitive to DNA cross-linking agents, while oral cancer in the general population is thought to be environmentally induced, these results also suggest that environmental DNA cross-linkers may be causally involved in oral carcinogenesis.- - - - - - - - - - ranking = 6keywords = carcinoma (Clic here for more details about this article) |
6/34. Fanconi's anemia and clinical radiosensitivity report on two adult patients with locally advanced solid tumors treated by radiotherapy.BACKGROUND: patients with Fanconi's anemia (FA) may exhibit an increased clinical radiosensitivity of various degree, although detailed clinical data are scarce. We report on two cases to underline the possible challenges in the radiotherapy of FA patients. CASE REPORT AND RESULTS: Two 24- and 32-year-old male patients with FA were treated by definitive radiotherapy for locally advanced squamous cell head and neck cancers. In the first patient, long-term tumor control could be achieved after delivery of 67 Gy with a-in part-hyperfractionated split-course treatment regimen and, concurrently, one course of carboplatin followed by salvage neck dissection. Acute toxicity was marked, but no severe treatment-related late effects occurred. 5 years later, additional radiotherapy was administered due to a second (squamous cell carcinoma of the anus) and third (squamous cell carcinoma of the head and neck) primary, which the patient succumbed to. By contrast, the second patient experienced fatal acute hematologic toxicity after delivery of only 8 Gy of hyperfractionated radiotherapy. While the diagnosis FA could be based on flow cytometric analysis of a lymphocyte culture in the second patient, the diagnosis in the first patient had to be confirmed by hypersensitivity to mitomycin of a fibroblast cell line due to complete somatic lymphohematopoietic mosaicism. In this patient, phenotype complementation and molecular genetic analysis revealed a pathogenic mutation in the FANCA gene. The first patient has not been considered to have FA until he presented with his second tumor. CONCLUSION: FA has to be considered in patients presenting at young age with squamous cell carcinoma of the head and neck or anus. The diagnosis FA is of immediate importance for guiding the optimal choice of treatment. radiotherapy or even radiochemotherapy seems to be feasible and effective in individual cases.- - - - - - - - - - ranking = 3keywords = carcinoma (Clic here for more details about this article) |
7/34. Vulvar cancer with Fanconi's anemia and neutropenic fever: a case report.BACKGROUND: Fanconi's anemia (FA), an autosomal-recessive aplastic anemia, was first described in 1927. patients usually die from complications of pancytopenia. The longer patients survive the underlying anemia, the higher the risk of other cancers, particularly leukemias, hepatocellular cancer and squamous cell tumors. This report is the sixth reported case of vulvar cancer in a young woman with FA since 1966. CASE: A 25-year-old woman with FA was admitted with neutropenic fever; a rapidly growing, suppurative vulvar mass; and trichomonas vaginalis. The patient had maintained routine, preventive gynecologic care, and 4 months prior to admission she had complete removal of a benign vulvar condyloma and no evidence of genital tract cancer. We removed the vulvar mass to relieve discomfort and eliminate an infectious source. The mass was an invasive squamous cell carcinoma of the vulva arising in a vulvar condyloma. Within 2 months of the diagnosis, the patient developed bulky disease metastatic to the lungs and inguinal lymph nodes. CONCLUSION: FA patients are at high risk of squamous cell tumors, and gynecologic examinations should begin at menarche. However, despite adequate screening, rapidly progressing solid tumors of the genital tract can develop in these immunosuppressed patients, who have a defect in dna repair genes.- - - - - - - - - - ranking = 1keywords = carcinoma (Clic here for more details about this article) |
8/34. Vulvar cancer in a patient with Fanconi's anemia, treated with 3D conformal radiotherapy.Fanconi's anemia (FA) is rare autosomal recessive disorder characterized by aplastic anemia, congenital anomalies, and cancer susceptibility. FA patients have deficiencies in dna repair pathways that cause cellular sensitivity to ionizing radiation and cross-link agents such as mitomycin C and diepoxybutane (DEB). If these patients survive until early adulthood, they are at high risk for developing solid tumors, most commonly squamous cell carcinoma of the oropharynx, esophagus, and vulva. Treatment of these solid tumors with radiotherapy is complicated by the increased risk of normal tissue toxicity. Three-dimensional (3D) conformal radiotherapy is a technique that uses CT images to more accurately target tumors and maximize the dose to the tumor volume while limiting the dose to normal tissue. This report describes application of 3D conformal radiotherapy techniques to the treatment of vulvar cancer in a patient with FA in an attempt to limit the normal tissue volume exposed to radiation.- - - - - - - - - - ranking = 1keywords = carcinoma (Clic here for more details about this article) |
9/34. Fibrolamellar carcinoma of the liver in a patient with fanconi anemia.A 9-year-old boy with fanconi anemia treated with oxymethalone, a synthetic androgen, died of intracerebral hemorrhage. At autopsy, the liver contained several adenomas and a large fibrolamellar hepatocellular carcinoma, as well as phlebectatic peliosis hepatis. The 11 previously reported cases of hepatocellular carcinoma in fanconi anemia were not, apparently, of the fibrolamellar type, which has a better prognosis, occurs in children of both sexes, and generally is not associated with cirrhosis. The malignant potential of primary liver tumors associated with fanconi anemia as well as the nature of their relationship to fanconi anemia and to anabolic steroid therapy is discussed.- - - - - - - - - - ranking = 6keywords = carcinoma (Clic here for more details about this article) |
10/34. Hepatocellular carcinoma and squamous cell carcinoma in a patient with Fanconi's anemia.Acute leukemia, hepatocellular carcinoma, and squamous cell carcinoma have been reported in patients with Fanconi's anemia. We report on a 31-year-old woman who developed squamous cell carcinoma of the esophagus and hepatocellular carcinoma. jaundice and hepatic tumor developed in 1981, after she had received oxymetholone for 10 years. liver biopsy revealed peliosis hepatis. Androgenic therapy was stopped and the jaundice resolved. However, the hepatic tumor was observed to be unchanged. The patient died of disseminated squamous cell carcinoma, but no metastatic lesions from hepatocellular carcinoma were detected in the autopsy. The association of Fanconi's anemia and squamous cell carcinoma is reviewed, and the malignant potential of androgen-related hepatic tumors is discussed.- - - - - - - - - - ranking = 15keywords = carcinoma (Clic here for more details about this article) |
| | Next -> |