Abstract
Lymphangioleiomyomatosis (LAM) and benign metastasizing leiomyoma (BML) are rare conditions that typically present as multiple lung nodules and more commonly seen in women of reproductive age. Histologically, both entities are composed of a proliferation of cytologically bland spindle cells demonstrating smooth muscle differentiation and nuclear expression of estrogen and/or progesterone receptors.
Differentiation of both entities is important, given that LAM can be a progressively debilitating systemic disease which may ultimately require lung transplantation, while BML is typically indolent. Clinical and radiologic correlation is important to make this distinction: LAM has a typical radiologic appearance of multiple thin-walled cystic lesions, while BML demonstrates solid nodules in patients with prior history of surgically treated uterine leiomyomas.
From an immunophenotypic standpoint, the presence of HMB45 expression, which is seen in the majority of cases of LAM, is useful to separate these entities.
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Mantilla, J.G. (2022). Lymphangioleiomyomatosis Versus Benign Metastasizing Leiomyoma. In: Xu, H., Ricciotti, R.W., Mantilla, J.G. (eds) Practical Lung Pathology. Practical Anatomic Pathology. Springer, Cham. https://doi.org/10.1007/978-3-031-14402-8_58
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DOI: https://doi.org/10.1007/978-3-031-14402-8_58
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