The hyperparathyroidism-jaw tumour (HPT-JT) syndrome is an autosomal dominant disorder characterized by parathyroid tumours, which are frequently carcinomas, and ossifying jaw fibromas. In addition, some patients may develop renal tumours and cysts. The gene causing HPT-JT, which is referred to as HRPT2 and is located on chromosome 1q31.2, encodes a 531 amino acid protein called PARAFIBROMIN. To date 42 mutations, of which 22 are germline, have been reported and 97% of these are inactivating and consistent with a tumour suppressor role for HRPT2. We have investigated another four HPT-JT families for germline mutations, searched for additional clinical phenotypes, and examined for a genotype-phenotype correlation. Mutations were found in two families. One family had a novel deletional-insertion at codon 669, and the other had a 2 bp insertion at codon 679, which has been reported in four other unrelated patients. These five unrelated patients and their families with the same mutation were not found to develop the same tumours, thereby indicating an absence of a genotype-phenotype correlation. An analysis of 33 HPT-JT kindreds revealed that affected women in 13 HPT-JT families suffered from menorrhagia in their second to fourth decades. This often required hysterectomy, which revealed the presence of uterine tumours. This resulted in a significantly reduced maternal transmission of the disease. Thus, the results of our analysis expand the spectrum of HPT-JT-associated tumours to include uterine tumours, and these may account for the decreased reproductive fitness in females from HPT-JT families.

Conflicts and natural disasters can cause major disruptions to people's lives. Media and news agencies usually focus on immediate consequences of these events, including loss of life and injuries, environmental and property destruction, and relief efforts. In this article, we will examine the effects of conflicts (focusing on in the Middle East) and global natural disasters on patients already diagnosed with cancer and on those who are diagnosed with cancer during and in the immediate aftermath of these events. We will review the limited literature, provide situational analysis, and discuss medical relief efforts, governmental readiness, and the role of United Nations agencies and international nongovernmental organizations. We will also discuss treatment of patients with cancer in the context of prolonged displacements and limited resources.

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