Abstract
Tuberous sclerosis complex (TSC), a rare autosomal dominant neurocutaneous disorder, is characterized by the presence of benign congenital tumors in multiple organs. Neoplasms with perivascular epithelioid cell differentiation (PEComas), including angiomyolipoma (AML) and lymphangioleiomyomatosis (LAM), can occur in association with TSC. This report describes two cases of uterine PEComas presenting characteristic MR imaging features reflecting pathological findings. From MR images, both cases showed single or multiple large, irregularly shaped or lobulated hemorrhagic lesions within the myometrium. They differed from typical adenomyotic cysts in their large size and irregular margins. Histopathologic analysis revealed that the hemorrhage was caused by adenomyosis and tumor cells that proliferated in surrounding stroma of the hemorrhagic lesions, compatible with PEComas. Microscopic observation revealed an infiltrative growth pattern of PEComas, with small nodules formed. The tumor lesions, however, were difficult to detect on MR images. The myometrium showed normal appearance on both T1-weighted and T2-weighted images in both cases. We speculate that PEComas may infiltrate extensively into the myometrium even when the myometrium shows almost normal radiologic appearance.
Similar content being viewed by others
References
Umeoka S, Koyama T, Miki Y, et al.(2008) Pictorial review of tuberous sclerosis in various organs. Radiographics, 28(7):e32.
Hornik J.L, Pan C.-C. (2013) PEComa. In: Fletcher C.D.M., Bridge J.A., Hogendoorn P.C.W., Mertens F. (ed) WHO classification of tumors of soft tissue and bone, 4th edn. IARC, Lyon, pp.230-231.
Hayashi T, Kumasaka T, Mitani K, et al.(2011) Prevalence of uterine and adnexal involvement in pulmonary lymphangioleiomyomatosis: a clinicopathologic study of 10 patients. Am J Surg Pathol, 35(12):1776-1785.
Musella A, De Felice F, Kyriacou AK, et al.(2015) Perivascular epithelioid cell neoplasm (PEComa) of the uterus: A systematic review. International journal of surgery, 19:1-5.
Kwon BS, Suh DS, Lee NK, et al.(2017) Two cases of perivascular epithelioid cell tumor of the uterus: clinical, radiological and pathological diagnostic challenge. European journal of medical research, 22(1):7.
Kim YS, Rha SE, Byun JY, et al.(2011) CT and MR imaging findings of lymphangioleiomyomatosis involving the uterus and pelvic cavity. Korean journal of radiology, 12(2):261-265.
Froio E, Piana S, Cavazza A, et al.(2008) Multifocal PEComa (PEComatosis) of the female genital tract associated with endometriosis, diffuse adenomyosis, and endometrial atypical hyperplasia. International journal of surgical pathology, 16(4):443-446.
Kataoka ML, Togashi K, Konishi I, et al.(1998) MRI of adenomyotic cyst of the uterus. Journal of computer assisted tomography, 22(4):555-559.
Funding
No funding was received for this study.
Author information
Authors and Affiliations
Corresponding author
Ethics declarations
Conflict of interest
The authors declare that they have no conflict of interest.
Ethical approval
All procedures performed in studies involving human participants were conducted in accordance with ethical standards of the institutional and/or national research committee and with the 1964 Declaration of Helsinki and its later amendments or comparable ethical standards. For this type of study, formal consent is not required.
Informed consent
A statement of informed consent was not applicable because the manuscript includes no patient data.
Additional information
Publisher's Note
Springer Nature remains neutral with regard to jurisdictional claims in published maps and institutional affiliations.
Rights and permissions
About this article
Cite this article
Nishio, N., Kido, A., Minamiguchi, S. et al. MR findings of uterine PEComa in patients with tuberous sclerosis: report of two cases. Abdom Radiol 44, 1256–1260 (2019). https://doi.org/10.1007/s00261-019-01918-3
Published:
Issue Date:
DOI: https://doi.org/10.1007/s00261-019-01918-3