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"Uterus in My Lung?!" A Case of Pulmonary Leiomyomatosis as a Presentation of Uterine Intravenous Leiomyomatosis FREE TO VIEW

Jay Patel, MD; Amy Coan, MD; Alexander Hindenburg, MD; Kent Chan, MD; Girish Nair, MD
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Winthrop-University Hospital, Mineola, NY

Chest. 2014;146(4_MeetingAbstracts):645A. doi:10.1378/chest.1990859
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SESSION TITLE: Cancer Student/Resident Case Report Posters I

SESSION TYPE: Medical Student/Resident Case Report

PRESENTED ON: Tuesday, October 28, 2014 at 01:30 PM - 02:30 PM

INTRODUCTION: Intravenous leiomyomatosis (IVL) is an uncommon intraluminal tumor of benign smooth muscle cells, usually seen in conjunction with uterine fibroids. We report a rare case of uterine IVL presenting as pulmonary leiomyomatosis (PL).

CASE PRESENTATION: A 47 year-old female, active smoker, presented with a chief complaint of increasing abdominal girth, associated with mild dyspnea on exertion. Her medical history included uterine fibroids with subsequent total abdominal hysterectomy (TAH) and bilateral salpingo-oophorectemy (BSO) in March 2012. Physical examination was significant for a distended, firm abdomen, with no tenderness. Chest radiography showed bilateral patchy opacities, and CT imaging of the chest and abdomen revealed a large 25cm pelvic mass, with multiple bilateral pulmonary opacities. A CT-guided tissue biopsy of a pulmonary lesion confirmed spindle cell neoplasm with minimal mitosis and no necrosis. Immunohistochemical staining showed the neoplasm to be TTF1(-), chromogrannin(-), synaptophysin(-), CAM 5.2(-), vimentin(+), desmin(+), and smooth muscle actin(+). Pathology from her prior hysterectomy was reviewed again, and had shown a 9cm mass with wormlike extensions into the large veins of the uterus, thus differentiating IVL from typical leiomyoma. Immunohistochemical staining showed the mass to be morphologically and phenotypically similar to the pulmonary lesion. Surgical report confirmed that due to extensive vascularization, part of the tumor was unable to be resected. She was treated with leuprolide, a GnRH analog, for a total of six months. A follow-up CT scan in January 2014 showed increase in the size of the pelvic mass, with increased pulmonary nodules and masses. A repeat CT guided biopsy of a lung mass ruled out sarcomatous transformation, and confirmed the diagnosis of PL. She is being treated with chemoembolization of the pelvic mass to decrease vascularity, followed by staged surgical resection of the pelvic mass. She has been continued on leuprolide for medical therapy.

DISCUSSION: IVL, despite its benign histology, can behave in a malignant fashion by extensive spread (1). Studies have shown these tumors to be estrogen-dependent (2). The mainstay of therapy is surgical, with TAH and BSO, or medically with progestins, tamoxifen or gonadatropin agonists (3).

CONCLUSIONS: PL is a rare presentation of uterine IVL. Knowledge of these rare entities is important for accurate diagnosis and treatment, and need to be differentiated from malignant lesions.

Reference #1: Bodner-Adler B, et al. Intravenous leiomyomatosis of the uterus with pulmonary metastases or a case with benign metastasizing leiomyoma? Anticancer Res 2009, 29:495-496.

Reference #2: Clement PB, et al. Intravenous leiomyomatosis of the uterus: A clinicopathological analysis of 16 cases with unusual features. Am J Surg Pathol 12: 932-945, 1988.

Reference #3: Banner AS, et al. Efficacy of oophorectomy in lymphangioleiomyomatosis and benign metastasizing leiomyoma. N Engl J Med. 1981 Jul 23;305(4):204-9

DISCLOSURE: The following authors have nothing to disclose: Jay Patel, Amy Coan, Alexander Hindenburg, Kent Chan, Girish Nair

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