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Clinical Advisory: Women with Rare Lung_disease Found to Also Have High Prevalence of Meningiomas, First Study to Document Tumor Connection; Possible Hormonal Cause Clinical Advisory: Women with Rare Lung_disease Found to Also Have High Prevalence of Meningiomas, First Study to Document Tumor Connection; Possible Hormonal Cause National Heart, Lung, and Blood Institute (NHLBI) 18 October 2001 Scientists at the National Heart, Lung, and Blood Institute (NHLBI) of the National_institutes_of_health (NIH) report that women with a rare lung_disease known_as LAM (lymphangioleiomyomatosis) have a_high prevalence of meningiomas, a type of brain_tumor. The study, published in the October 17 issue of the Journal of the American Medical Association, is the first to document the extent of the association between LAM and meningiomas.""These findings provide important new information on the nature of this disease and have implications for both the diagnosis and treatment of patients with LAM,"said NHLBI Director Dr. Claude Lenfant. The meningiomas were_detected in an ongoing study of the natural_history of LAM. LAM is_associated with a rare genetic neurological_disorder called tuberous_sclerosis (TS. In an effort to determine whether patients with LAM also showed signs of tuberous_sclerosis, NHLBI scientists performed magnetic_resonance_imaging (MRI) and computed_tomography (CT) brain scans on 250 women with the lung_disease. They were looking for brain abnormalities called"tubers,"one of the criteria for diagnosing TS. Unexpectedly, MRI scans revealed that 8 patients had meningiomas a rate that far exceeds the 1 in 20,000 expected in the general population. According_to the investigators, it is not clear whether the meningiomas are_caused by LAM itself, hormonal treatments for the disease or a combination of the two. They note that the abnormal smooth_muscle_cells found in the lungs and other tissues of LAM patients produce certain growth factors that are_believed to foster meningiomas. However, the possibility that the meningiomas have a hormonal cause has implications for the overall treatment of LAM. Because LAM occurs primarily in women of childbearing age, it has_been_thought to be_affected by hormonal factors. As a result, it has_been_treated commonly with the hormone progesterone. However, studies have found that progesterone can stimulate growth of meningiomas. Anti-progestins (drugs that interfere with this hormone's action) have even been_used to treat meningiomas.""We cannot rule_out the possibility that the high prevalence of meningiomas may_be a result of both LAM and progesterone therapy,"said Joel Moss, M_d.,chief of NHLBI's Pulmonary-Critical Care Medicine Branch and lead investigator of the study, which was_conducted at the NIH Clinical Center in Bethesda, MD. Moss noted that although some of the women in the study had never been_exposed to progesterone, low doses in contraceptives or hormone replacement therapy could have_played a role in initiating the tumor or fueling its growth. Moss and colleagues recommend using MRI to screen for meningiomas in LAM patients and if the tumors are found, they advise against the use of progesterone. The scientists also suggest that patients with meningiomas have yearly MRI scans to evaluate tumor growth. Surgery is_preferred the treatment for these tumors which are slow-growing and rarely spread to other parts of the body. An estimated 8, 000 U_s. women have LAM, which is_characterized by an unusual type of muscle_cell that invades and obstructs the tissue of the lungs, including the airways, and blood and lymph_vessels. Over time, these muscle_cells interfere with the lung's ability to supply oxygen to the rest of the body. The progression of the disease varies among patients as does survival, which may extend more_than 20 years after diagnosis. According_to Moss, patients with LAM are_misdiagnosed often, partly because many of the early symptoms are similar to other lung_diseases.""A common symptom of LAM is shortness of breath, which is_seen in other lung_diseases such_as asthma and emphysema. Other symptoms of LAM include chest_pain and coughing_up bloodstained sputum or blood, "he said. Moss noted that both spontaneous and inherited forms of LAM have_been_reported. LAM's connection to tuberous_sclerosis has_been_documented and there_is evidence that both diseases can have a common genetic basis. To increase understanding about the clinical course of LAM, including its connection to tuberous_sclerosis, Moss and colleagues at NHLBI are conducting research as part_of an Institute-funded study of patients with LAM. Moss is actively seeking patients with LAM including those who may_have been_diagnosed with TS. To interview Dr. Joel Moss, contact the NHLBI Communications Office at (301) 496-4236. For more information on LAM studies conducted on the NIH campus go_to the following NIH website: http://clinicalstudies. info. nih. gov. Information on the disease can be found at www. nhlbi. nih. gov. U_s . National Library of Medicine, 8600 Rockville Pike, Bethesda, MD 20894 National_institutes_of_health, Department_of_health & Human Services Copyright, Privacy, Accessibility Last updated: 16 November 2001