Diagnosis and Treatment of Sacrococcygeal Myeloid Sarcoma Involving Multiple Positions without Leukemia: A Case Report

Case Report

Ann Hematol Oncol. 2016; 3(9): 1110.

Diagnosis and Treatment of Sacrococcygeal Myeloid Sarcoma Involving Multiple Positions without Leukemia: A Case Report

Hou XW¹, Yu HT¹, Zhang XJ¹*, Wang L², Xian CJ²*

1Department of Radiology, Aerospace Central Hospital, China

2Sansom Institute for Health Research, University of South Australia, Australia

*Corresponding author: Xiaojin Zhang, Department of Radiology, Aerospace Central Hospital, 15 Yu’quan Road, Haidian District, Beijing 100049, China

Cory Xian, School of Pharmacy and Medical Sciences, University of South Australia, GPO Box 2471, Adelaide 5001, Australia

Received: August 22, 2016; Accepted: September 24, 2016; Published: September 26, 2016

Abstract

Myeloid sarcoma is a rare malignant haematologic tumour, and cases of sacral and tail presentation involving multiple organs without leukemia are particularly rare. Herein we report a case of a previously healthy man who presented with progressive backache, hip pain, leg numbness, walking difficulty, constipation, dysuria, and urinary incontinence. An ultrasound scan revealed a large sacrococcygeal mass. Spine MRI revealed an epidural irregular mass in the sacral vertebral canal, expansion of the intervertebral foramen, nerve root involvement, infiltrating paraspinal musculature in the lumbosacral area with propagation into pelvis, and formation of a large soft tissue mass in the sacral space. His baseline laboratory data were normal. Two times ultrasound-guided needle biopsies and immunohistochemistry confirmed the myeloid sarcoma diagnosis. A bone marrow biopsy revealed grade-III bone marrow morphology. Immediate induction chemotherapy significantly improved his symptoms. A 6-month following-up did not show leukemia development. Thus, when a large sacrococcygeal mass is seen by imaging, instead of an immediate decision of surgical removal, a needle biopsy should be taken for pathological tests. Immunohistochemistry, in conjunction with the clinical, imaging and laboratory investigations, played an essential role in achieving the correct diagnosis. Immediate and adequate chemotherapy is crucial for achieving the optimal outcomes.

Keywords: Myeloid sarcoma; Ultrasound; MRI; Pathology; Immunohistochemistry; Chemotherapy

Introduction

Myeloid sarcoma (MS), is a focal tumor composed of primitive myeloid cells at an extramedullary site, which has often been described in association with leukemia or myeloid proliferative disorders. It was previously known as granulocytic sarcoma, chloroma, or extramedullary myeloid tumor; and the 2008 World Health Organization classification adopted the term “myeloid sarcomas” as a subgroup of “acute myeloid leukemias, not otherwise categorized” [1]. Myeloid sarcoma most commonly occurs in the soft tissues, bone, and skin, while rarely in the central nervous system (CNS) [2]. This condition can precede, follow, or occur simultaneously with chemotherapy in patients with acute myeloid leukemia (AML), bone marrow hyperplasia syndrome, bone marrow proliferation diseases and other diseases of the blood system. Because of the poor specificity of the morphology and clinical manifestations of MS, this condition (particularly with a single tumour) is easily misdiagnosed as malignant lymphoma or other tumours. Here, we present a case study of an MS located in the sacrum and tail with propagation into the pelvis and adjacent soft tissues but without evidence of systemic myeloid disease.

Case Presentation

Patient presentation

A 45-year-old male farmer, who had been healthy previously, presented with buttock and lower extremity numbness, with mild pain but without an apparent inducing cause. Subsequently, these symptoms progressed to involve severe pain accompanied by difficulty in walking, constipation, dysuria, and occasional urinary incontinence. No fever, cough, sputum, chest tightness, and chest pain were present. The patient had no history of medical or psychological illnesses, including AML and genetic disorders. Because of persistent and worsening symptoms, his physician suggested that he undergo supplementary examinations. The current study adhered to ethical guidelines on informed consent and the authors affirm that written informed consent was obtained from the patient for the study and for publication of this case report and any accompanying images.

Radiological examinations and tumour diagnosis

An ultrasound scan revealed a large tail mass with a large echo and unclear boundaries (not shown). A lumbar spine magnetic resonance image (MRI) revealed an irregular epidural spinal mass. The lesion involved multiple organs including the sacral and tail vertebrae, the intervertebral foramen, the nerve root, the surrounding soft tissues, the left iliac bone, and the rectum. The mass exhibited intermediate signal intensity on the T1-weighted images, was slightly hyper-intense on the T2-weighted images, and was hyper-intense on diffusion-weighted images. Expansion into the intervertebral foramen and the nerve root involvement were observed, and the boundary was unclear (Figure 1). The irregular dorsal soft tissue mass was approximately 11x5 cm, and its boundaries were unclear. A large presacral gap irregular mass of approximately 12x6 cm was present. No visible peripheral capsule, central necrosis or calcification was observed. Bone involvement of the lumbar and sacral vertebrae and the left iliac bone (in which the signal was increased) was observed, but no obvious bone destruction was present. The rectum was pressed forward, and the bladder was slightly compressed. Therefore, the radiologist diagnosed a malignant tumour.

Citation: Hou XW, Yu HT, Zhang XJ, Wang L, Xian CJ. Diagnosis and Treatment of Sacrococcygeal Myeloid Sarcoma Involving Multiple Positions without Leukemia: A Case Report. Ann Hematol Oncol. 2016; 3(9): 1110. ISSN : 2375-7965