Neil Roy1, Anisha Elizabeth Jacob2, Madhu Mathew Vennikandam3, Anna Lee4, Meaghan Phipps4, Paul Gaglio4. 1. Joslin Diabetes Center, Harvard Medical School, Boston, MA. 2. PSG Institute of Medical Sciences and Research, Coimbatore, India. 3. Gastroenterology, Sparrow Hospital, Michigan State University College of Human Medicine, Lansing, MI. 4. Transplant Hepatology, Columbia University, New York, NY.
A 66-year-old man with a history of alcohol-related liver cirrhosis who underwent liver transplant 7 years earlier with hepatocellular carcinoma discovered on explant presented with fatigue and weight loss. Laboratory tests on admission revealed thrombocytosis to 495 and alkaline phosphatase to 286. α-fetoprotein, cancer antigen (CA) 19-9, and liver function tests (LFTS) apart from alkaline phosphatase was normal. Abdominal magnetic resonance imaging (MRI) showed a large 9.4 × 8.1 × 9.6-cm nonhomogeneous splenic mass, which was not present on his previous MRI. The patient underwent an open splenectomy with biopsy revealing poorly differentiated leiomyosarcoma (LMS) (Figures 1–4). He was disease-free in the following 3 computed tomography (CT) scans. However, surveillance CT scan performed thereafter showed a 6.4 cm mass on the distal pancreatic tail with fluorodeoxyglucose (FDG) avidity on positron emission tomographic (PET) scan for which he was started on liposomal doxorubicin, tacrolimus, and neoadjuvant radiation. He then underwent distal pancreatectomy. He had good liver graft function on 0.5 mg daily of tacrolimus and was switched to an mechanistic target of rapamycin (mTOR) inhibitor after surgery to prevent cancer recurrence. Liposomal doxorubicin was continued for until 3 months after which he returned with worsening frailty and new skin lesions consistent with LMS. He was then transitioned to supportive care in the end because of poor prognosis. LMSs are rare, malignant, mesenchymal tumors of smooth muscle origin.
Vascular LMSs are more common in veins than in arteries.
About half of large-vessel LMSs are located in the inferior vena cava.
Splenic LMSs are rare with only 6 other reported cases. It is an aggressive disease with a poor prognosis. Resection usually provides complete cure for patients without disseminated disease.
Adjuvant radiation, combination radiation, and chemotherapy, in addition to surgical resection, can increase survival but has limited amount of evidence.
To the best of our knowledge, this is the only reported case of splenic LMS in a post–liver transplant patient.
Figure 1.
Abdominal magnetic resonance imaging showing a 9.4 × 8.1 × 9.6-cm nonhomogeneous splenic mass.
Abdominal magnetic resonance imaging showing a 9.4 × 8.1 × 9.6-cm nonhomogeneous splenic mass.Gross specimen after resection.Biopsy showing anaplastic spindle cells (hematoxylin and eosin stain, 20× magnification).Biopsy showing marked nuclear pleomorphism (hematoxylin and eosin stain, 40× magnification).
DISCLOSURES
Author contributions: N. Roy and AE Jacob wrote the manuscript and reviewed the literature. MM Vennikandam and M. Phipps edited the manuscript. A. Lee provided the images. P. Gaglio revised the manuscript for intellectual content and approved the final manuscript. MM Vennikandam is the article guarantor.Financial disclosure: None to report.Informed consent was obtained for this case report.
Figure 1.
Figure 4.