Metastatic low‐grade endometrial stromal sarcoma after 24 years: A case report and review of recent molecular genetics

Abstract

Low‐grade endometrial stromal sarcoma (LGESS) is a rare malignant uterine tumor with the potential to metastasize years after the primary resection. There is a broad differential diagnosis for endometrial stromal sarcomas (ESS), including both benign and malignant entities. Herein, we present the case of a 64‐year‐old female with metastatic LGESS to the lung, diagnosed by cytology, 24 years after her initial presentation. This report discusses the cytomorphologic and histopathologic characteristics, and ancillary studies including immunohistochemical stains and recent advances in molecular diagnostics of ESS. Accurate diagnosis of spindle cell lesions in the lung can be challenging. As such, this case highlights the instrumental role of ancillary testing and molecular diagnostics to achieve a more definitive diagnosis.

Pulmonary Metastasizing Low-Grade Endometrial Stromal Sarcoma: Case Report and Review of Diagnostic Pitfalls

“…Our case also showed patchy positivity for desmin, diffuse positivity for h-caldesmon, patchy positivity for CD10, and diffuse positivity for WT-1. Ultimately, the key criterion to differential diagnosis between LM and LG-ESS is morphology [ 24 ]. Typical features, including a rich delicate network of small arterioles and capillaries and tumor cells showing swirling patterns around these vessels, are compatible with LG-ESS [ 25 ].…”

Rapid on-site evaluation of a solitary lung nodule in a patient with remote history of hysterectomy: Cytologic findings and diagnostic challenges

“…The cytologic features of metastatic low-grade ESS have rarely been described in the literature since it is rarely subjected to fine-needle aspiration. [ 4 , 8 - 10 ] In the lung, it has been mistaken for carcinoid tumor, hamartoma, and endometriosis. [ 4 , 5 , 9 ] A predominance of bland spindle cells can also be mistaken for leiomyoma and other benign spindle cell neoplasms.…”

“…[ 4 , 5 , 9 ] A predominance of bland spindle cells can also be mistaken for leiomyoma and other benign spindle cell neoplasms. [ 5 , 7 , 8 ]…”

“…Mindiola-Romero et al . [ 8 ] described a similar clinical scenario of low-grade ESS metastatic to the lung in a patient who presented with multiple lung nodules. A CT-guided core biopsy with rapid on-site assessment demonstrated cells with oval and spindled nuclei with mild-to-moderate atypia and scant matrix component.…”

Metastatic low-grade endometrial stromal sarcoma with variable morphologies in the ovaries and mesentery: A case report

“…Low-grade endometrial stromal sarcoma (LGESS) is rare, accounting for only 1% of all malignant uterine tumors and 10% of uterine sarcomas[ 1 - 3 ]. The tumor is persistent and may metastasize years after initial resection[ 4 , 5 ]. Classically, LGESS exhibits a proliferative morphology.…”