Intracranial myeloid sarcoma as the first presentation of acute myeloid leukemia and literature review.

INTRODUCTION: Intracranial myeloid sarcoma is a rare extramedullary presentation of acute myeloid leukemia (AML). It can involve the meninges and ependyma presenting as extra-axial mass lesion. Rarely, it can also invade the brain parenchyma. It is commonly seen in children. It is usually misdiagnosed due to its close resemblance to other intracranial tumors (meningioma, metastasis, Ewing's sarcomas, and lymphoma). These are underdiagnosed if they precede the diagnosis of leukemia. CASE REPORT: A 7-year-old boy with isolated intracranial myeloid sarcoma who presented with raised intracranial pressure (ICP) which was successfully managed by surgical excision. CONCLUSION: Isolated intracranial myeloid sarcoma is a rare presentation of AML. Leukemia can be diagnosed early during the postoperative period and can be started on therapy timely. These patients requires regular follow-ups (clinical, laboratory and radiological) to detect relapses early.

Isolated intracranial myeloid sarcoma: report of a case and review of the literature.

Myeloid sarcoma is a rare malignant tumor of primitive myeloid cell origin often associated with hematologic disorders. The central nervous system is rarely involved and differentiating between myeloid sarcoma and other tumors is not possible on imaging. Here we present the rare case of an isolated intracranial myeloid sarcoma, initially misdiagnosed radiologically as a meningioma, treated with surgical total resection and subsequent chemotherapy, with no signs of any hematological disorder at follow up. Differential diagnosis and management strategies, as well as follow-up implications are discussed along with literature review, which pointed out that only five cases with no further signs of hematological disorders at follow up have been described in the literature so far and this case has the longest follow up of them at 9 years.

[Leukemia Relapse as Granulocytic Sarcoma Beginning with Penile Localization : A Case Report].

A 57-year-old man visited the urology department with a painful mass on the dorsal side of the penis. Magnetic resonance imaging sagittal image showed a small nodule. Leukemia recurrence was suspected due to his history of treatment for acute myeloid leukemia treated with allogeneic hematopoietic stem cell transplantation. No recurrence was identified by bone marrow biopsy ; however, two months later, the recurrence of leukemia was strongly suspected because the tumor grew over time and blasts were found in the peripheral blood. A biopsy of the penile tumor and bone marrow was performed, leading to the diagnosis of granulocytic sarcoma. Patients with a history of leukemia may be preceded by a single recurrence to extramedullary organs, even if blood and bone marrow findings suggest remission.

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.

Diagnosis and surgical treatment of primary isolated aggressive lumbar myeloid sarcoma: a rare case report and review of the literatures.

BACKGROUND: Myeloid sarcoma is a rare, extramedullary, solid tumor derived from immature myeloid cell precursors. It is most frequently accompanied by acute myelogenous leukemia, though infrequently found in non-acute myelogenous leukemia patients. The tumor may involve any part of the body, but the lumbar spine is seldom involved. The present case study aims to understand the diagnosis and surgical treatment of a rare primary isolated myeloid sarcoma of the lumbar spine causing aggressive spinal cord compression in a non-acute myelogenous leukemia patient. CASE PRESENTATION: A 29-year-old man complained of an aggressive radiating pain to the lower extremities and moderate dysuria with a Visual Analogue Scale score that gradually increased from 3 to 8. Lumbar enhanced magnetic resonance imaging and computed tomography revealed a lumbar canal lesion at lumbar spine L2 to L4 with spinal cord compression. A whole body bone scan with fused single photon emission computed tomography/computed tomography demonstrated abnormal 99mTc-methylene diphosphonate accumulation in the L3 lamina and spinous process. No evidence of infection or hematology disease was observed in laboratory tests. Due to rapid progression of the symptoms and lack of a clear diagnosis, decompression surgery was performed immediately. During the operation, an approximately 6.0 × 2.5 × 1.2 cm monolithic, fusiform, soft mass in the epidural space and associated lesion tissues were completely resected. The radiating pain was relieved immediately and the dysuria disappeared within 1 week. Intraoperative pathological frozen section analysis revealed a hematopoietic malignant tumor and postoperative immunohistochemistry examination confirmed the diagnosis of myeloid sarcoma. CONCLUSIONS: The primary isolated aggressive lumbar myeloid sarcoma is rarely seen, the specific symptoms and related medical history are unclear. Surgery and hematological treatment are effective for understanding and recognizing this rare tumor.

[A Case of Myeloid Sarcoma in the Small Intestine Presenting with Intussusception].

A sixty-something man presented with lower abdominal pain in early Y month 20XX, and was examined at the hospital's internal medicine outpatient clinic. An abdominal CT showed a soft tissue mass around the left hip joint, and multiple enlarged lymph nodes from inside the pelvis to the mesentery of the abdomen. We noted a small-intestinal intussusception in the lower right abdomen, and suspected malignant lymphoma. We did a CT-guided biopsy on the left hip joint soft tissue mass, and performed surgery on the small-intestinal intussusception. During surgery, we noted an approximately 30 cm ileal intussusception located about 60 cm from the terminal ileum, and enlarged lymph nodes in the nearby mesentery. We removed the ileal intussusception. The pathological diagnosis was myeloid sarcoma, and the soft tissue mass in the left hip joint was also diagnosed as myeloid sarcoma. We performed a bone-marrow biopsy at the hematology department, and diagnosed acute myeloid leukemia M2. We then started remission-induction therapy and consolidation therapy, and the patient was diagnosed as in remission in Y+5 month 20XX. We also need to keep in mind myeloid sarcoma in the intestine as a subtype of acute myeloid leukemia, as malignant tumor in the small intestine presenting with intussusception.

Sarcoma granulocítico del sistema nervioso central: reporte de 2 casos y revisión de la literatura / Central nervous system granulocytic sarcoma: Report of 2 cases and review of the literatura

Los sarcomas granulocíticos son neoplasias sólidas compuestas de células mieloides inmaduras, de localización extramedular, asociadas a síndromes mieloproliferativos. La afectación del sistema nervioso central es muy infrecuente y puede desarrollarse tras un periodo de remisión completa, coexistir con o preceder a la enfermedad sistémica, siendo esta el elemento fundamental que orienta el diagnóstico radiológico y marca el pronóstico. En este trabajo, presentamos 2 casos de sarcoma granulocítico intracraneal, verificados desde el punto de vista patológico tras ser tratados mediante cirugía, y discutimos sus características clínicas, diagnósticas, terapéuticas y pronósticas tras realizar una revisión bibliográfica de la literatura científica destacada publicada hasta la fecha

Granulocytic sarcomas are solid, extramedullary-located neoplasms composed of immature myeloid cells, associated with myeloproliferative syndromes. Central nervous system involvement is very rare and may develop either after complete remission, coexist with or precede the systemic disease, being the last one that guides the radiological diagnosis and marks the prognosis. In this work, we report 2 pathologically-verified cases of intracranial granulocytic sarcoma treated by surgical means. Their clinical, diagnostic, therapeutic and prognostic features are discussed in the light of the most relevant scientific literature published to date

Myeloid Sarcoma That Infiltrated a Preexisting Sebaceous Lymphadenoma in the Parotid Gland: Diagnostic Challenges and Literature Review.

Myeloid sarcoma (MS) is a rarely encountered extramedullary localized tumor that is composed of immature myeloid cells. We reported an extremely rare case of MS with concurrent bone marrow (BM) involvement that invaded into a preexisting sebaceous lymphadenoma in the parotid gland and neck lymph nodes. Prompted by this case, we also present a literature review of MS invasion into salivary glands. A 62-year-old man was initially diagnosed with carcinoma that arose in a sebaceous lymphadenoma in the parotid gland, through a total parotidectomy with neck dissection. After an extensive histopathological review that included immunohistochemistry, a pathologic diagnosis of MS with infiltration into the sebaceous lymphadenoma with concurrent BM involvement was confirmed. MS is difficult to diagnose accurately; herein, we analyzed the clinical presentations and effectiveness of the various diagnostic methods with a review of the literature. There are 17 cases, including our case, reported in 13 studies. Of the cases in which the salivary glands were affected, 10 involved the parotid gland, six involved the submandibular gland, and one involved both. Isolated invasion of the salivary gland was found in one case of parotid gland invasion and three cases of submandibular gland invasion. In 13 cases, the salivary glands were affected by various other lesions. Although there were no incidences of isolated MS, six patients were diagnosed with secondary MS and eight patients with MS with BM involvement, including this case. The diagnosis of MS is difficult given its rarity, and a high index of suspicion and integrated radiologic and careful histopathologic evaluation are required. Most cases of MS infiltrating the salivary gland might be indicated by the possibility of BM involvement. MS with BM involvement predicts poor prognosis and the need for intensive systemic treatment.

[A Case of Myeloid Sarcoma That Primarily Developed in the Spleen].

We report a case of myeloid sarcoma(MS)that primarily developed in the spleen. The patient was a 60s man with a chief complainant of low-grade fever following a dental implant. Although he underwent intensive antibiotic treatment, including levofloxacin, meropenem, and vancomycin, no significant decline in fever was observed. Abdominal contrast-enhanced CT revealed an LDA occupying the majority of his spleen, which was diagnosed as a splenic abscess. Although a CT-guided biopsy and drainage for the spleen were considered, a puncture of the spleen was not performed due to the substantial concern of bleeding. Subsequently, a splenectomy was performed and HE staining revealed mitosis of tumor cells and massive necrosis. Immunohistochemical analysis revealed that the tumor cells were positive for myeloperoxidase, CD43, CD45, and CD68. Finally, the splenic LDA was diagnosed as MS instead of a splenic abscess. He was treated with systemic chemotherapy. MS primarily develops in the spleen is quite rare: we found only 2 case reports of this disease. The prognosis of MS is poor due to the complications of AML. Accordingly, MS should be considered as a differential diagnosis for accurate diagnosis and treatment of splenic LDA.

Chronic myeloid leukemia manifested as myeloid sarcoma: Review of literature and case report.

In this report, we present the case of a 50-year-old woman presenting with an intraparenchymal myeloid sarcoma manifesting as weakness. She has a history of chronic myeloid leukemia (CML) treated with imatinib not taken consistently with a relapse to blast crisis, and then an isolated relapse as a myeloid sarcoma manifesting as facial and extremity weakness. An MRI of the brain showed an enhancing, well-circumscribed mass within the frontal lobe with edema extending to the motor strip. Based on tumor size, focality, location, growth rate, and patient's symptoms, surgeons determined that the patient should undergo surgical resection. Postoperatively, the patient had full resolution of her acute neurological symptoms without post-operative complications. Post-operative MRI showed minimal enhancement suggesting post-surgical changes vs minimal residual tumor. The patient was scheduled to undergo whole brain radiotherapy with supplemental direct radiation to the site of resection. This is the first report of safe and complete resection of an intraparenchymal myeloid sarcoma. It is meant to inform neurosurgeons that brain tumors can be potentially CML-related; additionally, we review CML's manifestations in the central nervous system and how neurosurgeons can consider optimal management given as there are no guidelines on how to treat CML-related CNS disease.

Rare myeloid sarcoma with KMT2A (MLL)-ELL fusion presenting as a vaginal wall mass.

BACKGROUD: Myeloid sarcoma (MS) is a rare neoplasm of immature myeloid precursors that form tumor mass outside the bone marrow. The diagnosis of de novo MS can be challenging, particularly in patients with no prior history of hematologic malignancies or when MS involves unusual anatomic sites. CASE PRESENTATION: The patient was a 53-year-old woman with a history of uterine fibroids and vaginal bleeding for many years who presented with a vaginal wall mass. The tumor had histologic and phenotypic features of histiocytic sarcoma, however, overlapping with a possible extramedullary MS. Using a comprehensive genomic profiling, we were able to identify recurrent chromosomal aberrations associated with MS including a rare KMT2A-ELL fusion, losses of chromosomes 1p, 9, 10, 15, 18, and gain of chromosome 1q and mutations in FLT3 and PTPN11, and achived the final diagnosis of a de novo MS. The patient received standard treatment for acute myeloid leukemia regimen with stem cell transplantation and achieved complete remission. CONCLUSION: Our case illustrates the clinical utility of comprehensive genomic profiling in assisting the diagnosis or differential diagnosis of challenging MS or histiocytic sarcoma cases, and in providing important information in tumor biology for appropriate clinical management.

Myeloid sarcoma presenting as an unusual limbal mass.

A 19-year-old woman presented to the outpatient department with occasional ocular pain and redness and a perilimbal mass, which she noticed 5 months ago in her left eye. She had no systemic complaints. Ultrasound biomicroscopy of the mass showed a hypoechoic lesion with uniform reflectivity. The patient underwent an excision biopsy and a histopathological analysis revealed features suggestive of a granulocytic sarcoma/myeloid sarcoma. Further haematopathological evaluation confirmed concurrent acute myeloid (myelomonocytic) leukaemia French American British classification M4. There was complete remission of the ocular surface lesion and leukaemia with systemic chemotherapy. At the last follow-up of 18 months post-treatment the patient is free of disease.

Predictors of Discharge Disposition Following Laminectomy for Intradural Extramedullary Spinal Tumors.

OBJECTIVE: To identify independent risk factors for non-home discharge in patients undergoing laminectomy for intradural extramedullary spinal tumors. METHODS: We performed a retrospective cohort analysis of data from the American College of Surgeons National Surgical Quality Improvement Program from 2011 to 2014. Adult patients who underwent laminectomy for the excision of intradural extramedullary spinal tumors were included and divided into 2 groups based on home or non-home discharge disposition. We compared baseline patient characteristics, comorbidities, and operative factors between the 2 groups, and then performed multivariate regression analyses to identify independent risk factors for non-home discharge. RESULTS: A total of 1232 patients were included, of whom 248 (20.1%) were discharged to a non-home facility. Univariate analysis demonstrated that patients discharged to a non-home facility were more frequently aged ≥65 years and American Society of Anesthesiologists classification ≥3 with obesity, diabetes, dyspnea, functional dependence, cardiac comorbidity, renal comorbidity, and anemia. Operative factors correlated with non-home discharge were operative time of ≥4 hours and tumor location in the cervical or thoracic spine. Multivariate regression analysis identified age ≥65 years (odds ratio [OR] 2.73; confidence interval [CI] 1.80-4.13; P < 0.001), American Society of Anesthesiologists classification ≥3 (OR 2.36; CI 1.53-3.65; P < 0.001), dependent functional status (OR 4.30; CI 1.95-9.48; P < 0.001), hospital-acquired conditions (OR 2.32; CI 1.15-4.68; P = 0.019), and prolonged length of stay (OR 4.05; CI 2.72-6.03; P < 0.001) as predictors of non-home discharge. CONCLUSIONS: Early identification of patients at risk for non-home discharge is important in order to implement comprehensive discharge planning protocols that reduce inpatient length of stay, as well as associated complications and costs.