Clinical characteristics, treatment options, and prognosis of myeloid sarcoma: analysis using the SEER database.

BACKGROUND: Myeloid sarcoma (MS) is a very rare hematologic disorder. This study analyzes the early treatment options for patients with different types of MS and explores the prognostic factors of MS. METHODS: Patients aged 15 years and older with MS in the SEER database (diagnosed from 2000 to 2018) were selected, excluding those with an unknown first course of treatment, an unknown location of disease, and less than 1 month of follow-up. Statistical methods used a chi-square test to compare clinical characteristics; Kaplan-Meier analysis to compare survival differences; and Cox proportional risk models to identify prognostic factors affecting overall survival (OS). RESULTS: Data were collected from 472 patients: 244 patients with isolated myeloid sarcoma (IMS) and 228 patients with non-isolated myeloid sarcoma (non-IMS). IMS patients mostly chose local treatment, while non-IMS patients mostly chose chemotherapy. There was a significant difference in OS between IMS patients treated with combined treatment and those without treatment. For non-IMS, treated patients had longer OS than untreated, but the difference was not statistically significant. Among adult patients, those younger than 60 years had a better prognosis. Patients with the urinary system, digestive system, reproductive system and chest and abdomen as the initial site had a better prognosis. CONCLUSIONS: Early combination therapy in IMS patients had a longer OS, and chemotherapy combined with radiotherapy/surgery should be the treatment of choice. For non-IMS patients, early combination therapy did not show a significant advantage. Age and location of first presentation were independent factors affecting MS patients' long-term prognosis.

Haploidentical hematopoietic stem cell transplantation for patients with myeloid sarcoma: a single center retrospective study.

Allogeneic hematopoietic stem cell transplantation (allo-HSCT) has been regarded as a potential strategy for myeloid sarcoma (MS). The previous reports focused mainly on matched sibling donor (MSD) or matched unrelated donor (MUD) transplantation. There are no reports on haploidentical HSCT (haplo-HSCT) in MS. We retrospectively reviewed 14 MS patients who underwent haplo-HSCT. All patients achieved complete donor engraftment. The median time for neutrophil engraftment and platelet engraftment were 10 (12-21) days and 18 (8-31) days. The 100-day cumulative incidence of grade II-IV acute graft-versus-host disease (GVHD) and 3-year cumulative incidence of chronic GVHD were 37.7% (95%CI, 23.2-52.1%) and 35.7% (95%CI, 22.2-49.2%). Cytomegalovirus (CMV) reactivation was documented in 86% patients, and only one patient developed CMV pneumonia. Treatment-related mortality occurred in one (7%) patient. The 1- and 3-year cumulative incidence of relapse was 21.4% (95%CI, 11.8-31.1%) and 35.7% (95%CI, 22.4-49.0%). The probability of overall survival at 1 and 3 years was 71.4% (95%CI, 51.3-99.5%) and 64.3% (95%CI, 43.5-95.0%), respectively. The probability of disease-free survival at 1 and 3 years was 71.4% (95%CI, 51.3-99.5%) and 57.1% (95%CI, 36.3-89.9%), respectively. In conclusion, haplo-HSCT is a feasible method for patients with MS who have no MSD or MUD.

Myeloid sarcoma is associated with poor clinical outcome in pediatric patients with acute myeloid leukemia.

PURPOSE: The impact of myeloid sarcoma (MS) on clinical outcome of pediatric acute myeloid leukemia (AML) patients remains controversial. Moreover, little is known about the role of stem cell transplantation (SCT) in such patients. METHODS: Clinical data of patients with AML under 18 years of age were retrieved from the TARGET dataset. We analyzed the prevalence, clinical profile, molecular characteristics, and prognosis of MS in these patients. RESULTS: Among 884 pediatric patients with AML, the frequency of MS was 12.3%. Pediatric AML with MS was associated with age under 1-year, abnormal cytogenetics, and KMT2A rearrangement. Moreover, MS was associated with a low complete remission rate, high induction death, poor 5-year EFS, and OS. KMT2A rearrangement had a negative impact on clinical outcome in AML patients with MS. In addition, SCT had no significant effect on the survival of AML patients with MS. Multivariate analysis revealed that MS was an unfavorable prognostic factor in pediatric AML in terms of EFS (Hazard ratio 1.670, P < 0.001) and OS (Hazard ratio 1.623, P = 0.004). CONCLUSIONS: The presence of MS at diagnosis of pediatric AML is associated with poor clinical outcomes, particularly when associated with KMT2A rearrangements. Moreover, pediatric patients with AML and MS may not benefit from SCT.

Myeloid Sarcoma Predicts Superior Outcome in Pediatric AML; Can Cytogenetics Solve the Puzzle?

BACKGROUND: The purpose of our study was to evaluate the clinical, cytogenetic, and molecular features, and survival outcomes in patients with acute myeloid leukemia (AML) with myeloid sarcoma (MS) and compare them with patients with AML without MS. PATIENTS AND METHODS: This was a retrospective analysis of de novo pediatric AML patients with or without MS diagnosed at our cancer center between June 2003 and June 2016. RESULTS: MS was present in 121 of 570 (21.2%), the most frequent site being the orbit. Patients with MS had a younger median age (6 years vs. 10 years) and presented with higher hemoglobin and platelet but lower white blood cell count compared with patients without MS. Further, t (8; 21) (P < .01), loss of Y chromosome (P < .01), and deletion 9q (P = .03) were significantly higher in patients with AML with MS. Event-free survival (EFS; P = .003) and overall survival (OS; P = .001) were better among patients with AML with MS (median EFS 21.0 months and median OS 37.1 months) compared with those with AML without MS (median EFS 11.2 months and median OS 16.2 months). The t (8; 21) was significantly associated with MS (odds ratio, 3.92). In a comparison of the 4 groups divided according to the presence or absence of MS and t (8; 21), the subgroup of patients having MS without concomitant t (8; 21) was the only group to have a significantly better OS (hazard ratio, 0.53; 95% confidence interval, 0.34-0.82; P = .005). CONCLUSION: Although t (8; 21) was more frequently associated with MS, it did not appear to be the reason for better outcome.

Nonleukemic granulocytic sarcoma of orbit after blunt trauma: A case report and review of literature.

RATIONALE: Granulocytic sarcoma without invasion of bone marrow or blood is very rare. The diagnosis of it is usually overlooked and the treatment has not reached a consensus. Meanwhile, the onset of this kind of disease is not clear. PATIENT CONCERNS: Diagnose patients in early stage and help choose the right treatment strategies. DIAGNOSES: The ultimate diagnosis was nonleukemic granulocytic sarcoma after blunt trauma. INTERVENTIONS: Surgery was the initial treatment option. Chemotherapy including idarubicin (70 mg, D1-D3) and cytosine arabinoside (100 mg, D1-D7) and radiotherapy of total 3,060 cGy were then administered but failed to control the disease. Hematopoietic stem cell transplantation was finally administered. OUTCOMES: No evidence of disease progression or spread according to the latest follow-up. LESSONS: The etiology of nonleukemic granulocytic still remains unclear, though trauma seems to be a potential predisposing factor and deserves more attention for early diagnosis and timely and proper treatment. Systemic chemotherapy is more effective than radiotherapy or surgery. Hematopoietic stem cell transplantation is an alternative choice after the failure of chemotherapy.

How Rare Is an Oral Presentation of Myeloid Sarcoma in the Infant?

Myeloid sarcomas of the oral cavity are exceedingly rare. This report describes a recent case, and reviews the literature. This case report serves three purposes: 1) to demonstrate that the use of an intraoral biopsy can diagnose severe systemic disease; 2) to remind practitioners to be cognizant of less common diagnoses in the differential diagnosis of facial swelling; and 3) to contribute a case of myeloid sarcoma that was confirmed by flow cytometry to the published data.

Myeloid Sarcoma as the First Sign of Progression of Chronic Myeloid Leukemia in Medullary Chronic Phase: Experience from a Tertiary Cancer Centre in Southern India.

INTRODUCTION: Myeloid sarcoma (MS) in chronic myeloid leukemia (CML) is a rare entity which is suggestive of advanced phase of the disease and poorer outcomes. There is little data in literature available regarding its presentation in medullary chronic phase (CP) as well as outcomes in the era of tyrosine kinase inhibitors (TKI) and needs to be carefully evaluated as it can present the first sign of progressive disease before haematological progression. METHODS: We identified cases of MS presenting with medullary CML-CP from January 2002 to December 2015. We analyzed their clinical profile and outcomes with TKI. RESULTS: Only 8 out of 615 CML-CP cases developed MS. Median age of presentation was 43 years with male: female ratio of 1.7:1. Sites of presentation were soft tissue deposits (7 cases) and lymph nodes (2 cases). All the cases had myeloblastic morphology. With higher dose Imatinib/Nilotinib, median overall survival was 14 months with longest survival of 36 months in a case on Nilotinib while 4 patients progressed to medullary BP at a median duration of 9 months (2-10) and expired. CONCLUSION: MS in medullary CML-CP carries better prognosis than medullary CML-BP. Due to rarity of presentation, MS presenting in soft tissues might be overlooked as an infection/hematoma unless proven otherwise. Our series emphasizes the need of meticulous examination and investigation of such presentations for earlier intervention to improve patient outcomes.

[Primary breast sarcomas: About 30 cases treated at Salah-Azaiez institute in Tunisia]. / Sarcomes mammaires primitifs : à propos de 30 cas traités à l'institut Salah-Azaiez de Tunis.

PURPOSE: To identify retrospectively prognostic factors of primary breast sarcoma and review its treatment modalities. MATERIALS AND METHODS: This is a descriptive study on 30 cases of primary breast sarcoma. We carried out a univariate and multivariate analysis correlating clinical, pathological and therapeutic parameters with disease-free survival and overall survival. RESULTS: The mean age was 46.8 years. The mean tumour size was 10cm. The 30 cases were 18 phyllodes sarcomas, eight angiosarcomas, three liposarcomas and a case of granulocytic sarcoma. Sixteen patients had adjuvant radiotherapy and only seven patients received adjuvant chemotherapy. The median follow-up was 64 months. Overall survival rates at 3 and 5 years were 49.1% and 33.7%. Disease-free survival rates at 3 and 5 years were 22.8% and 15.2% respectively. The analytical study of the following parameters: tumour size and presence or absence of node or distant metastases, showed no correlation with overall survival nor with disease-free survival. Furthermore, adjuvant radiotherapy did not improve overall survival (P=0.298; hazard ratio [HR]=1 [0.982-1.04]) nor disease-free survival (P=0.61; HR=0.942 [0.862-1.029]). By univariate analyses, we identified a correlation between overall survival, surgical margins (>1cm) (P=0005; HR=3.4 [1.217-9.919]) and tumour necrosis (P=0.028; HR=0.099 [0.014-0.682]). We did not find any independent prognostic factor by multivariate analysis. CONCLUSION: The prognosis of primary breast sarcoma seems to depend essentially on optimal surgical excision (margin over 1cm). The only potential histological parameter correlated with the prognosis is the presence of tumour necrosis. The histological subtype should not be considered as a prognostic marker for overall or disease-free survival in patients with primary breast sarcoma.

Anatomic distribution of hematolymphoid malignancies in the head and neck: 7 years of experience with 122 patients in a single institution.

CONCLUSION: Most hematolymphoid malignancies in the head and neck were malignant lymphomas that most often occurred in sexagenarian men. Approximately 80% of them were B-cell lymphomas with a predominance of diffuse large B-cell lymphoma (DLBCL) in both nodal and extranodal sites. Our results were compatible with those in a previous study that was conducted in the central part of Japan. OBJECTIVES: This analysis was performed to describe the anatomic distribution of hematolymphoid malignancies that were diagnosed by biopsy in our department. METHODS: Clinical medical records of 122 patients with hematolymphoid malignancies in the head and neck from January 2004 to December 2010 were retrospectively reviewed. The anatomic site of origin according to the histopathology of each malignancy was analyzed. RESULTS: The incidence ratio of hematolymphoid malignancies was 15.1%. The male:female ratio was 2.3:1. Ages ranged from 17 to 89 years (median, 66). Of the 122 cases, 121 were lymphoid neoplasms (4 cases of Hodgkin lymphoma and 117 cases of non-Hodgkin lymphoma) and the remaining 1 was myeloid. The most common histopathology was DLBCL (54.9%), followed by follicular lymphoma (8.2%), and peripheral T-cell lymphoma (8.2%). Most commonly, the oropharynx (36.1%) and the cervical lymph node (34.4%) were affected.

[Primary granulocytic sarcoma of the peritoneum: a case report and literature review]. / Sarcome granulocytaire à localisation péritonéale primitive : à propos d'un cas et revue de la littérature.

Granulocytic sarcoma is a rare tumor composed of immature granulocytic cells. Prognosis is poor. The periosteum is preferentially involved. A peritoneum localization is unusual. We report the case of a 20 years old man without particular previous pathologies, which brutally presented an ascitic syndrome in a context of health impairment state. The laparoscopy showes many white nodules on all the peritoneum. The histologic examination of one of these nodules showed granulocytic sarcoma. The blood and bone marrow cell count are without any anomaly. The treatment consisted of a standard acute myeloid leukaemia's chemotherapy with very good evolution. The rarity of peritoneal chloroma causes a diagnostic problem, especially in the absence of hematologic abnormalities. It must be mentioned in the presence of peritoneal nodules even if the blood count and bone marrow are normal.

Intracerebral granulocytic sarcoma.

Granulocytic sarcomas, also known as chloromas, are rare extramedullary tumors of myeloid or myelocytic origin. They are usually associated with both acute and chronic myelogenous leukemia and myeloproliferative disorders. Leukemia involvement of the central nervous system most commonly presents as meningeal leukemia; intracerebral granulocytic sarcoma (IGS) is rare. Signs and symptoms depend on the brain structures involved. Magnetic resonance imaging with and without gadolinium is the imaging of choice to evaluate the tumor; however, tissue biopsy is essential for definitive diagnosis. Treatment usually involves radiation followed by chemotherapy, depending on the previous systemic treatment. Because medical literature about IGS is scarce, optimal treatment is unclear. With the number of leukemia patients in remission, the incidence of IGS is expected to rise. This is because most chemotherapeutic agents do not cross the blood-brain barrier, making the brain a target for leukemia recurrence. Nurses play a vital role in helping patients and families understand the disease process, the treatments involved, and the necessary adjustments, such as performing mundane activities of daily living, especially when neurocognitive impairments are present.

Extramedullary infiltration at diagnosis and prognosis in children with acute myelogenous leukemia.

BACKGROUND: Extramedullary infiltration (EMI) is an occasional clinical symptom in childhood acute myelogenous leukemia (AML), but there is considerable controversy regarding the prognostic significance of EMI in AML. PROCEDURE: We evaluated the frequency and prognostic significance of EMI at diagnosis of AML in children. RESULTS: Of 240 cases of de novo AML excluding children with Down syndrome and acute promyelocytic leukemia, 56 (23.3%) showed EMI at diagnosis. Patients with EMI had a higher initial WBC count and a higher proportion of M4/M5 morphological variants. The complete remission rate following induction chemotherapy was lower in patients with EMI. However, the overall survival and event-free survival did not differ between patients with and without EMI. A detailed analysis showed that patients with EMI with a WBC count at diagnosis of over 100 x 10(9)/L or infiltration into the central nervous system are likely to have a poor prognosis. CONCLUSIONS: CNS leukemia and EMI together with a WBC count of >100 x 10(9)/L at diagnosis of AML are high risk factors for relapse, and alternative treatment approaches for patients with these characteristics should be explored.

Solitary bone and extramedullary plasmacytoma.

Less than 5% of patients with a plasma cell dyscrasia present with a single bone (SBP) or extramedullary plasmacytoma (EMP) without evidence of systemic disease (normocalcemia, absence of anemia, preservation of uninvolved immunoglobulins, or renal disease attributable to myeloma). Diagnosis requires biopsy confirmation of a monoclonal plasma cell infiltrate from a single site. The treatment of choice for both entities is radiotherapy given with curative intent (> 4000 cGy) resulting in long term disease-free survival in approximately 30% of patients with SBP and 65% of patients with EMP.

Extramedullary myeloid cell tumours--the NIMS experience.

Extramedullary myeloid cell tumours are rare clinical entities, which often pose diagnostic problems. From the pathology record files of Nizam's Institute of Medical Sciences, Hyderabad, 16 cases of EMCTs were traced, over a period of 14 years. The clinical details, follow-up were noted and morphology re-evaluated, and immunohistochemistry with LCA was performed. Of the 16 cases, the distribution was as follows--skin and subcutaneous nodules, lymph nodes, extradural masses presenting with cord compression and one case each with eyelid, orbital and breast masses. The problems in diagnosis are presented and a panel of immunohistochemical markers suggested for proper diagnosis and treatment.

Treatment of acute promyelocytic leukemia: strategy toward further increase of cure rate.

Acute promyelocytic leukemia (APL) has become a curable disease by all-trans retinoic acid (ATRA)-based induction therapy followed by two or three courses of consolidation chemotherapy. Currently around 90% of newly diagnosed patients with APL achieve complete remission (CR) and over 70% of patients are curable. To further increase the CR and cure rates, detection and diagnosis of this disease at its early stage is very important, hopefully before the appearance of APL-associated coagulopathy. In induction therapy, concomitant chemotherapy is indispensable, except for patients with low initial leukocyte counts. Prophylactic use of intrathecal methotrexate and cytarabine should be done, particularly for patients with hyperleukocytosis. If patients relapse hematologically or even molecularly, arsenic trioxide will be the treatment of choice under careful electrocardiogram monitoring. Am80, liposomal ATRA, gemtuzumab ozogamicin or ATRA in combination with cytotoxic drugs may be used at this stage or later. Allogeneic SCT will be the treatment of choice after patients of age <50 years have relapsed, provided that they have HLA-identical family donors or DNA-identical unrelated donors.