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1-30-2020

Metastatic Leiomyosarcoma Presenting as Polycystic Liver Metastatic Leiomyosarcoma Presenting as Polycystic Liver

Disease Disease

Michael Burkholz DO HCA Healthcare, Michael.Burkholz@MedicalCityHealth.com

Apurva Modi MD

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Recommended Citation Recommended Citation Burkholz M, Modi A. Metastatic Leiomyosarcoma Presenting as Polycystic Liver Disease. Poster presented at: Texas Osteopathic Medical Association MidWinter Conference; January 30 - February 2, 2020; Irving TX.

This Poster is brought to you for free and open access by the Research & Publications at Scholarly Commons. It has been accepted for inclusion in Gastroenterology by an authorized administrator of Scholarly Commons.

This is a case of a 39-year-old male developing intra-abdominal leiomyosarcoma (LMS) with hemorrhagic liver metastasis masquerading as Polycystic Liver Disease (ADPLD). He had been mistakenly diagnosed with ADPLD twenty-one months prior to biopsy. This is an atypical presentation in an already rare disease. Patient’s with ADPLD containing complex or hemorrhagic cysts should be considered for dynamic MRI to rule out metastatic disease mimicking ADPLD.

A 39 year-old-male who presented with progressive stabbing and constant abdominal pain. It was associated with jaundice, poor appetite, night sweats and 40 lbs weight loss. A non-contrast CT abdomen/pelvis showed hemorrhagic liver cyst with possible abscess.

Report of Case

Leiomyosarcoma is a malignant smooth muscle tumor that can originate from virtually any part of the body. The most common sites affected are the retroperitoneum and the peritoneal cavity. The peak incidence of leiomyosarcoma is in the sixth and seventh decades

According to the World Health Organization, LMS of the gastrointestinal tract is so rare that there is no significant data on demographic, clinical, or gross features of the tumor. One study found that over twelve-year period 56 cases of leiomyosarcoma were identified in the GI tract. Liver metastases occurred in 27% of the time.

One other case of metastatic LMS masquerading as polycystic liver disease has been published. That patient underwent liver transplant for ADPLD with confirmed LMS in the liver and no ADPLD. This is a second case with a similar presentation.

The atypical presentation of LMS with advanced necrotic liver metastasis can be explained by its propensity to cause necrotic, liquefaction, and cystic change in the liver.

LMS is a fascicular spindle cell neoplasm, and tumor cells have brightly eosinophilic cytoplasm and cigar-shaped nuclei. LMS generally lack KIT and CD34 expression but reliably demonstrate high levels of smooth muscle actin and desmin.

Despite frequent tumor recurrence, the long-term outcome after liver resection for hepatic metastases from LMS is superior to that after chemotherapy and chemoembolization.

We recommend dynamic MRI of the liver with any patient that has suspicious radiographic features when evaluating patients with ADPLD.

Metastatic Leiomyosarcoma Presenting as Polycystic Liver Disease1Michael Burkholz DO; 2Apurva Modi MD

1Medical City Fort Worth Gastroenterology Fellow, 2Baylor All Saints Transplant Hepatology

Case Report

1. Carlo M. Contreras and Martin J. Heslin Sabiston Textbook of Surgery, Chapter 31, 754-772 2. Metastatic Leiomyosarcoma Mimicking Polycystic Liver Disease. Beavers, K., Fried, M., Liver Transplantation. Vol8, No

12 (December),2002:pp 1132-1134. 3. Ann Diagn Pathol. 2012 Dec;16(6):532-40. doi: 10.1016/j.anndiagpath.2012.07.005. Epub 2012 Aug 20. Primary

leiomyosarcomas of the gastrointestinal tract in the post-gastrointestinal stromal tumor era.4. Korean Journal of Clinical Oncology 2017; 13(2): 143-146. Published online: December 31, 2017.

DOI: https://doi.org/10.14216/kjco.17022

Figure 1 Figure 3

Figure 4

Figure 1. Pancreatic uncinate process arterial enhancing mass is concerning for pancreatic neuroendocrine tumor. Figure 2. Some cysts demonstrated soft tissue enhancing component measuring up to 2.9 x 3.2 x 3.9 cm. Figure 3. Multiple scattered nodular arterial enhancing lesions were seen throughout the liver. Figure 4. Liver markedly enlarged by numerous complex hemorrhagic, loculated hepatic cysts but with preserved contour.

Figure 2

Figure 7. H-caldesmon, tumor is strongly positiveFigure 6. Spindle cell tumor

Introduction

He was diagnosed with polycystic liver disease twenty-one months earlier by CT scan. Some nodularity around cysts was noted and dynamic MRI was ordered, but the patient was lost to follow up.

Dynamic MRI demonstrated evidence of a pancreatic arterial enhancing mass concerning for neuroendocrine tumor. Multiple scattered nodular arterial enhancing lesions with numerous complex hemorrhagic, loculatedhepatic cysts were seen consistent with metastatic disease.

Retroperitoneal lymph node was biopsied and demonstrated spindle cell malignancy compatible with leiomyosarcoma.

No palliative chemotherapy was pursued. He was discharged on hospice.

Discussion

Figure 5. MRCP. Multiple hepatic cysts with dilated intra and extrahepatic ducts

References

This research was supported (in whole or in part) by HCA Healthcare and/or an HCA Healthcare affiliated entity. The views expressed in this publication represent those of the author(s) and do not necessarily represent the official views of HCA Healthcare or any of its affiliated entities.