Obstructive Jaundice with skin involvement - An unusual presentation of Myeloid Sarcoma.

Biliary obstruction secondary to malignancy is a common clinical problem. Rarely, biliary obstruction is due to leukemia, and obstructive jaundice in these patients usually presents late in the course of the disease. We present a rare case of a patient who presented with fever, jaundice, and pruritus with multiple nodular swellings in the left shoulder, left thigh, and lower back. Magnetic resonance cholangiopancreatography (MRCP) revealed periampullary mass lesion causing dilated common bile duct (CBD) and intrahepatic bile ducts; hence, endoscopic retrograde cholangiography with plastic stenting was done. Biopsy from the shoulder lesion revealed a mesenchymal tumor, and immunohistochemistry (IHC) confirmed the lesion as myeloid sarcoma. Myeloid sarcoma is an extramedullary tumor, a subtype of acute myeloid leukemia, and presentation as biliary lesions with multiple anatomical sites is very rare. The patient was started on chemotherapy after the normalization of bilirubin. The patient showed improvement of skin lesions and normalization of liver function test (LFT) after 3 weeks of chemotherapy.

Myeloid sarcoma incidentally found in lymph nodes dissected for advanced gastric cancer.

Myeloid sarcoma (MS) is a condition characterized by a tumor mass of myeloid blasts in any site of the body other than the bone marrow, with or without acute myeloid leukemia. A 93-year-old man underwent laparoscopy-assisted distal gastrectomy with D1 lymphadenectomy for advanced gastric cancer. Other than metastatic foci of gastric cancer cells, some dissected lymph nodes showed destructive architecture with proliferation of small- to medium-sized atypical hematopoietic cells. Those cells were focally positive for naphthol AS-D chloroacetate esterase. Immunohistochemically, positive results were obtained for CD4, CD33, CD68 (KP1), Iba-1, lysozyme, myeloperoxidase, and PU.1, with focally positive results for CD13, CD14, CD68 (PGM1), CD163, and CD204, and negative results for AE1/AE3, CD1a, CD3, CD20, and S-100 protein. These results suggested MS with phenotypically myelomonocytic differentiation. We report a rare case of MS incidentally found in specimens resected for other purposes. Careful diagnosis and consideration of differential diagnoses including MS using an adequate panel of antibody markers for dissected lymph nodes is warranted.

Myeloid Sarcoma: A Primer for Radiologists.

ABSTRACT: Myeloid sarcoma (MS) is a rare extramedullary neoplasm that can present in association with acute myeloid leukemia, most commonly in children younger than 15 years. This unique extramedullary malignancy may involve a variety of different organ systems and can present following, preceding, simultaneous with, or in insolation to acute myeloid leukemia. Common areas of extramedullary involvement include soft tissues, bones, lymph nodes, and the peritoneum. Imaging plays a critical role in the diagnosis and management of MS, with commonly used modalities including positron emission tomography-computed tomography, magnetic resonance imaging, computerized tomography, and ultrasound. The purpose of this review article is to provide radiologists with a comprehensive guide summarizing the relevant imaging and clinical features of MS, with emphasis on the role of imaging in the diagnosis, treatment, and follow-up of patients with MS. The relevant pathophysiology, epidemiology, clinical presentations, and differential diagnosis of MS will be reviewed. The relevance of different imaging modalities in diagnosis, monitoring of treatment response, and assessment of treatment-related complications will also be outlined. Through summarizing these topics, this review article aims to provide radiologists with a guide for understanding the existing knowledge of MS in the literature and the current role of imaging in the management of this unique malignancy.

Myeloid sarcoma arising at the uterine cervix in a patient with intestinal Behçet's disease and concurrent myelodysplastic syndrome: A case report.

INTRODUCTION: Myeloid sarcoma (MS) is an extramedullary tumor that consists of myeloblasts and rarely involves the female reproductive organs. Intestinal Behçet's disease (BD) is a chronic, inflammatory illness that is often associated with myelodysplastic syndrome (MDS). When MDS is diagnosed, some patients with intestinal BD experience synchronous gastrointestinal flares. CASE PRESENTATION: We report the case of a 49-year-old woman who presented with vaginal bleeding and an incidentally identified MS in the uterine cervix. Subsequent bone marrow biopsy showed simultaneous MDS without chromosomal abnormalities. This is the first reported case of concomitant MS, myelodysplastic disease, and intestinal BD. CONCLUSIONS: The accurate diagnosis of MSs that develop at non-predominant sites is crucial for a positive patient prognosis. MDS should be suspected in patients with a history of intestinal BD diagnosed with MS.

Isolated myeloid sarcoma with pericardial and pleural effusions as first manifestation: A case report.

RATIONALE: Myeloid sarcoma (MS) involves the proliferation of extramedullary blasts from 1 or more myeloid lineages, replacing the original tissue structures, and these neoplasias are called granulocytic sarcoma, chloroma, or extramedullary myeloid neoplasms. These tumors develop in lymphoid organs, bones, skin, soft tissues, various mucous membranes, organs, and the central nervous system. MS is rare in non-leukemic patients, while MS patient with effusion as the first manifestation is even rare. PATIENT CONCERNS: We report the case of 44-year-old woman with abdominal pain, diarrhea, and vomiting. DIAGNOSIS: Ultrasound examination and computed tomography of the chest revealed large pericardial effusions and bilateral pleural effusions. Cytomorphological examination of the pericardial and pleural effusion, flow cytometry, and immunohistochemical markers suggested myeloid tumor cells. However, concurrent peripheral blood and bone marrow examinations showed no evidence of acute myeloid leukemia. The patient was eventually diagnosed with isolated MS. INTERVENTIONS AND OUTCOMES: After chemotherapy with pirarubicin + cytarabine and high-dose cytarabine + etoposide, the pericardial effusion and pleural effusion were absorbed, and the mediastinal mass significantly shrunk. One year after patient gave up treatment, acute myeloid leukemia (AML) was confirmed by bone marrow examinations. CONCLUSION: The early manifestations of the patient lacked specificity and were highly susceptible to misdiagnosis. Cytomorphology and flow cytology indicated important directions for the diagnosis of the disease in the early stage. Administration of chemotherapy regimen containing cytarabine could prolong disease-free survival and time before progress to AML.

A ZBTB16-RARα Variant of Acute Promyelocytic Leukemia with Concurrent Myeloid Sarcoma Presenting as Sudden Onset Paraplegia.

BACKGROUND: The co-occurrence of myeloid sarcoma (MS) and acute promyelocytic leukemia (APL) is a rare clinical event. METHODS: A 56-year-old man presented with lower extremity paralysis that occurred 6 hours before admission. Magnetic resonance imaging revealed an epidural mass. RESULTS: Histopathological examination demonstrated an extramedullary myeloid malignancy. Bone marrow examination showed abnormal promyelocytes and dysplasia, and an immunophenotyping study indicated very strong myeloperoxidase activity, suggesting APL. CONCLUSIONS: The final diagnosis given was spinal MS with a ZBTB16-RARα variant of APL. The possibility that patients with rapidly progressive neurologic symptoms could have MS and concurrent APL should be considered.

Polyserositis as a Unique Presentation of Isolated Myeloid Sarcoma: A Case Report.

BACKGROUND: Myeloid Sarcoma (MS) are tumors containing myeloid blasts occurring in a location other than the bone marrow, including lymph nodes, skin, and soft tissues. MS presenting as polyserositis however is very rare, with only a few cases in the literature. CASE REPORT: A 20-year-old male presented with cough, shortness of breath and was found to have left upper lobe consolidation, left pleural effusion, pericardial effusion, and a large anterior mediastinal mass. A transthoracic echocardiogram showed pericardial effusion with tamponade physiology. He underwent emergent pericardiocentesis and thoracentesis. The fluid studies showed flow cytometry findings consistent with MS/ acute myeloid leukemia (AML) phenotype. A bone marrow aspirate and biopsy were unremarkable and showed no immunophenotypic findings diagnostic of acute leukemia or a lymphoproliferative disorder. Cytogenetics was negative for AML abnormalities per FISH analysis. Videoassisted thoracoscopy surgery (VATS) with biopsy of the mediastinal mass, pericardium, and left upper lobe of the lung was consistent with MS. He was treated with induction cytarabine and idarubicin, and a follow up PET-CT scan showed complete remission. He is currently day 200 + post stem cell transplant with no evidence of disease recurrence. CONCLUSION: To the best of our knowledge, this is the first case of isolated myeloid sarcoma presenting as polyserositis, without prior leukemia/ bone marrow involvement. Hence, fluid studies should involve cytometry analysis and MS should be entertained as a differential for polyserositis, even without a history of prior leukemia. Timely diagnosis can expedite aggressive chemotherapy required for a potentially life-threatening disease.

The transformation of isolated gastric myeloid sarcoma into acute myeloid leukemia presenting with a complex karyotype and TLS-ERG gene fusion: A case report.

RATIONALE: Isolated myeloid sarcoma (MS) is characterized by the rapid proliferation of myeloblasts of acute myeloid leukemia (AML), without any blood or bone marrow involvement. This disease can manifest with extramedullary organ involvement, such as the skin, lymph nodes, bone, brain, breast cervix, and visceral organs, while the occurrence of myeloid sarcomas in the stomach is rare. Isolated MS has been associated with acute myeloid leukemia (AML), but the rapid progression of MS to acute myeloid leukemia with a complex karyotype and TLS-ERG fusion gene is even rarer. PATIENT CONCERNS: A 33-year-old woman suffered from persistent epigastric pain accompanied by two months of anorexia and nausea, as well as 1-week of melena. DIAGNOSIS: This patient was initially diagnosed with gastric MS that eventually transformed into AML with a complex karyotype and TLS-ERG fusion gene, 4 months later. INTERVENTIONS: Only palliative care, including nutrition support, antacids, blood transfusion, anti-infection methods were used on this patient to determine the cachexia status and the family's requirement. OUTCOMES: Routine follow-up results demonstrated this patient had died due to cerebral hemorrhage five months after the diagnosis of MS. LESSONS: Comprehensive integration of patient history, imaging features, mass and bone marrow biopsy, and molecular cytogenetic may provide insights that could help us avoid the misdiagnosis of gastric MS. Isolated gastric MS can rapidly progress to AML with a poor prognosis if the patient does not receive appropriate treatment.

Myeloid Sarcoma of the Temporal Bone: A Unique Cause of Hearing Loss, Otalgia, and Facial Nerve Weakness.

OBJECTIVE: To characterize a clinical triad of symptoms associated with myeloid sarcomas of the temporal bone via a review of all previously reported cases. METHODS: Case report and Ovid MEDLINE database literature review. RESULTS: A literature search revealed that a clinical triad of hearing loss, otalgia, and facial nerve weakness are commonly associated with this rare presentation of myeloid sarcoma in the temporal bone. 44% (18/41) of patients presented with all three symptoms, while 76% (31/41) presented with at least two. The presence of t(8;21) was reported in nine patients with myeloid sarcomas of the temporal bone. CONCLUSIONS: Although myeloid sarcomas are exceedingly rare, it is necessary to consider them as part of the differential diagnosis for patients who might present with middle ear and mastoid opacification on computed tomography (CT) scan, hearing loss, otalgia, and facial nerve palsy. Physicians should maintain a high degree of suspicion in patients with a history of acute myelogenous leukemia (AML), especially if previous cytogenetic analysis revealed a t(8;21).

Isolated Myeloid Sarcoma and Intracardiac Thrombus Resulting in Superior Mediastinal Syndrome.

Superior mediastinal syndrome (SMS) is a relatively common emergency in the practice of Pediatric Oncology. It typically results from the compression of large airways and superior vena cava by a swiftly growing mass. T-lineage acute lymphoblastic leukemia or lymphoma, neuroblastoma, and germ cell tumor are the common etiologies of SMS in children. Occasionally, SMS can be an unexpected presentation of less common childhood cancers and a surprise for the diagnostic and treating teams. The present paper reports the diagnostic and therapeutic challenge of managing a 9-y-old boy with SMS resulting from mediastinal myeloid sarcoma. The presence of a sizeable intracardiac thrombus, in addition, contributed to the SMS. The initial pleural fluid cytology and image-guided fine-needle aspiration cytology of the mediastinal mass were nondiagnostic. A thoracotomy was subsequently performed to debulk the tumor for symptomatic relief and obtain tissue for diagnosis.

Intraoral myeloid sarcoma presenting as toothache and gingival mass.

A female patient in her 70s with a medical history of myelodysplastic neoplasm presented to the outpatient department with a 4-month history of toothache, painful gingival swelling and loose teeth that required extractions. Intraoral examination revealed a swelling in the lower anterior portion of the mandible, which displaced her teeth. Incisional biopsy of the gingival lesion revealed dense aggregates of atypical round cells which stained positive for CD43, CD45, CD33 and myeloperoxidase, consistent with myeloid sarcoma. Subsequent bone marrow biopsy displayed hypercellular marrow with immature myeloid elements and 21% myeloblasts by flow cytometry, compatible with diagnosis of acute myeloid leukaemia (AML). The patient initially went into remission after treatment but later died of AML relapse after 18 months.

Granulocytic sarcoma causing long spinal cord compression: Case report and literature review.

Context: Granulocytic sarcoma (GS) is an extramedullary form of proliferating myeloblasts. It is frequently reported in patients with acute myeloid leukemia (AML) but rarely in patients with chronic myeloid leukemia (CML). Spinal cord compression caused by CML-associated GS is exceedingly rare, with only few cases reported in the literature. To our knowledge, this is the first reported case in which GS caused such extensive compression.Findings: A 37-year-old man with CML suffered from back pain for 2 months. Notably, he had already achieved molecular remission (MR) after receiving imatinib mesylate for CML; bone marrow aspiration results were consistent with CML in chronic phase. Image examination revealed that developed GS occupied nearly the entire thoracic spinal canal, thereby causing extensive spinal cord compression. The tumor completely diminished after his treatment regimen was upgraded. He showed no signs of recurrence after 1-year follow-up.Conclusion: Extramedullary infiltration of CML should be taken into consideration when a mass lesion develops and compresses the spinal cord in a CML patient who has been receiving routine and standard treatment modalities; thus, a sudden and unexpected progression mandates a refinement and upgrade of treatment modality.