Microscopic uterine lymphangioleiomyomatosis perivascular epithelioid cell neoplasm: a case report with the earliest manifestation of this enigmatic neoplasm

Clay MR, Michael R. Clay, Pamela Gibson, Gibson P, Pamela Church Gibson, Lowell J, Jennifer L. Lowell, Jennifer Lowell, Kumarasen Cooper, Cooper K
International journal of gynecological pathology : official journal of the International Society of Gynecological Pathologists · 2010 · vol. 30(1) , pp. 71–75 · doi:10.1097/pgp.0b013e3181efe08d · PMID:21131829 · W2329102939
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Researchers describe a unique microscopic uterine PEComa, potentially an early example of LAM, and compare its features to other intravascular uterine neoplasms.

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This case report presents a unique instance of microscopic uterine lymphangioleiomyomatosis, identified as the earliest manifestation of this enigmatic perivascular epithelioid cell neoplasm within the uterine myometrium. The authors contrast the histological and immunohistochemical features of this lesion with intravascular adenomyosis, intravascular endometrial stromal sarcoma, and intravascular leiomyoma to aid in differential diagnosis. While the existence of these lesions remains controversial among some investigators, the study highlights the distinctive characteristics of perivascular epithelioid cells that define this rare pathology. Relevance to endometriosis: listed as one indication for GnRH antagonists, though the paper's main focus is uterine fibroids.

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Abstract

Lymphangioleiomyomatosis (LAM) is part of a family of lesions collectively known as perivascular epithelioid cell neoplasms (PEComas). Though these lesions are comprised of histologically and immunohistochemically distinctive perivascular epithelioid cells, their diagnosis is controversial and some investigators question their existence entirely. We present a unique case of perhaps the earliest microscopic example of a LAM PEComa in the uterine myometrium, and contrast its features to that of intravascular adenomyosis, intravascular endometrial stromal sarcoma, and intravascular leiomyoma.
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PATHOLOGY OF THE CORPUS: CASE REPORT Microscopic Uterine Lymphangioleiomyomatosis Perivascular Epithelioid Cell Neoplasm A Case Report With the Earliest Manifestation of This Enigmatic Neoplasm - Michael R. Clay - Pamela Gibson - Jennifer Lowell - Kumarasen Cooper International Journal of Gynecological Pathology 30(1):p 71-75, January 2011. | DOI: 10.1097/PGP.0b013e3181efe08d Lymphangioleiomyomatosis (LAM) is part of a family of lesions collectively known as perivascular epithelioid cell neoplasms (PEComas). Though these lesions are comprised of histologically and immunohistochemically distinctive perivascular epithelioid cells, their diagnosis is controversial and some investigators question their existence entirely. We present a unique case of perhaps the earliest microscopic example of a LAM PEComa in the uterine myometrium, and contrast its features to that of intravascular adenomyosis, intravascular endometrial stromal sarcoma, and intravascular leiomyoma. Copyright ©2011International Society of Gynecological Pathologists

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Condition tags

adenomyosis

MeSH descriptors

Lymphangioleiomyomatosis Myometrium Uterine Neoplasms Epithelioid Cells Epithelioid Cells Epithelioid Cells Female Female Humans Humans Lymphangioleiomyomatosis Lymphangioleiomyomatosis Middle Aged Middle Aged Myometrium Myometrium Uterine Neoplasms Uterine Neoplasms

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