Lymphangioleiomyomatosis (LAM) is a neoplastic disease that generally arises in the lung (pLAM) and may be associated with "Tuberous sclerosis complex" (TSC). Occasionally, LAM can arise at the extrapulmonary sites (eLAM), such as the mediastinum, the retroperitoneum or the lymph nodes. 25-30% of the patients affected by pLAM develop eLAM. In asymptomatic patients, the presence of mediastinal and retroperitoneal eLAM preceded that of pLAM by usually 1-2 years. Nevertheless, some authors reported that the nodal eLAM, detected during pelvic cancer staging, arise in patents without pLAM and/or TSC. In this paper we review the Literature of this rare condition suggesting its diagnostic management.
Diagnostic management of occult nodal lymphangioleiomyomatosis detected during pelvic cancer staging. Localized finding or systemic disease?
Remo, Andrea;Parcesepe, Pietro;Manfrin, Erminia;Micheletto, Claudio
2019-01-01
Abstract
Lymphangioleiomyomatosis (LAM) is a neoplastic disease that generally arises in the lung (pLAM) and may be associated with "Tuberous sclerosis complex" (TSC). Occasionally, LAM can arise at the extrapulmonary sites (eLAM), such as the mediastinum, the retroperitoneum or the lymph nodes. 25-30% of the patients affected by pLAM develop eLAM. In asymptomatic patients, the presence of mediastinal and retroperitoneal eLAM preceded that of pLAM by usually 1-2 years. Nevertheless, some authors reported that the nodal eLAM, detected during pelvic cancer staging, arise in patents without pLAM and/or TSC. In this paper we review the Literature of this rare condition suggesting its diagnostic management.
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