Ewing's sarcoma in adolescents and adults - 10-year experience from a tertiary cancer center in India.

BACKGROUND: Ewing's sarcoma (EWS) is an aggressive small round cell tumor, affecting bone and soft tissues and is mostly seen in childhood and second decade of life. EWS accounts for 10-12% of bone tumors in more than 15 years age group and is even rarer after 40 years of age. MATERIALS AND METHODS: This retrospective analysis was conducted among patients aged more than 15 years with histologically proven EWS. RESULTS: Among 240 cases of EWS treated at our center during 2001-2010, 130 (54%) were more than 15 years of age. The median age was 20 years with a male: female ratio of 2.4:1. Ninety percent had skeletal EWS, 10% had extra skeletal EWS, and 37% patients were metastatic at presentation. Eighty-two received curative treatment with chemotherapy (vincristine, doxorubicin, cyclophosphamide, ifosfamide, etoposide (VAC/IE)) along with local treatment, radiotherapy (RT) in 61, surgery alone in seven, and RT plus surgery in 14. Two- and 5-year overall survival (OS) was 43.3% and 25.5%, respectively, for the entire series. The OS for the non-metastatic group was 63.2% at 2 years and 36.5% at 5 years, and the progression free survival was 53.7% at 2 years and 37.8% at 5 years. High lactate dehydrogenase was found to be a significant poor prognostic factor (P = 0.001). Median OS for localized central EWS was 49.2 months and that for peripheral EWS was 24 months. Patients more than 20 years of age with non-metastatic disease had better OS compared to those with 15-20 years of age. CONCLUSION: Treatment of EWS requires a multidisciplinary approach with radical surgery and/or radiation to control local disease and multiagent chemotherapy to control systemic disease. Long-term follow-up is essential because of disease relapse and treatment-related complications.

Castleman disease: Experience from a single institution.

Castleman disease (CD) describes a group of rare heterogeneous lymphoproliferative disorders characterized by enlarged hyperplastic lymph nodes. It is classified into unicentric CD (UCD) and multicentric CD (MCD). The present retrospective study examined the data of 11 patients with CD diagnosed and treated at a tertiary cancer center from 2017 to 2022. The median age of the study group was 41 years (range, 24 to 68 years). There were 8 males and 3 females. In total, 7 patients were diagnosed with UCD and 4 patients with MCD. The hyaline-vascular variant was the most common histology in both UCD and MCD. Among the 7 patients with UCD, 5 patients underwent excision, 1 patient underwent debulking followed by radiotherapy and 1 patient received single agent rituximab. Of the patients with UCD, 6 had a complete response (CR) and 1 patient had a partial response (PR). All 4 patients with MCD received systemic treatment, which included single agent rituximab (2 patients), rituximab, cyclophosphamide, doxorubicin, vincristine and prednisolone (RCHOP) (1 patient) and CHOP (1 patient). Among the patients with MCD, 1 patient attained a CR, 2 patients had a PR and 1 patient succumbed. The 3-year survival rate for the study population was 91%. In summary, CD is a rare disease occurring in immunodeficient patients. UCD is more common and is associated with better outcomes. Surgery is the mainstay of management in UCD whereas MCD requires combination chemotherapy.

Erratum: Anderson Localization in the Fractional Quantum Hall Effect [Phys. Rev. Lett. 128, 116801 (2022)].

This corrects the article DOI: 10.1103/PhysRevLett.128.116801.

Histiocytic Sarcoma: Clinical Features and Outcomes of Patients Treated at a Tertiary Cancer Care Center.

Histiocytic sarcoma (HS) is an extremely rare histiocytic disorder of unknown etiology. It is not a true sarcoma and is named so, due to the pathological resemblance to mature histiocytes. The clinical presentation of HS is diverse and is related to the involved organs. Due to its aggressive nature, with poor prognosis and lack of a standard treatment regimen of choice, its diagnosis and management pose a challenge to the clinician. Limited literature is available on the management of this entity. Here, we report four patients with HS, diagnosed over 15 years in a tertiary cancer center, with varied clinical presentation, management, and outcomes. The first patient presented with a localized unresectable esophageal mass. He was treated with cyclophosphamide, doxorubicin, vincristine, and prednisone (CHOP) combination chemotherapy and attained complete remission. The second patient had a painless mass of the hand, treated with wide excision and adjuvant Radiotherapy. She is disease-free for the past 12 years. The third patient had presented with an anterior mediastinal mass. He had progressive disease on chemotherapy. The fourth patient had multifocal disease with generalized lymphadenopathy. She was treated with CHOP chemotherapy and is now disease-free at 13 months. To summarize, the patients with the localized resectable disease did well, with surgical excision and adjuvant radiotherapy, while patients with the multifocal disease did well on CHOP chemotherapy. The take-home message from this case series is - CHOP off whenever you can and if not give CHOP to chop off the disease.

Acute myeloid leukemia patients with variant or unusual translocations involving chromosomes 8 and 21 - A comprehensive cytogenetic profiling of three cases with review of literature.

Background: t(8;21)(q22;q22) is the most frequent recurrent translocation in acute myeloid leukemia (AML) resulting in an in-frame fusion of RUNX1/RUNX1T1 that regulates various genes involved in the signaling pathways. This leukemogenic alteration is usually associated with a favorable clinical outcome. Variants of t(8;21) can be formed involving a third or fourth chromosome in ~3-4% of t(8;21)-AML. Due to the rarity of variant t(8;21), its clinicopathological features and prognostic significance are still unclear. Here we present three AML cases with cryptic rearrangements of chromosomes 8 and 21 without standard RUNX1/RUNX1T1. Materials and Methods: Conventional karyotyping and fluorescence in situ hybridization and/or spectral karyotyping of the pretreatment bone marrow aspirate of de novo AML patients were performed to delineate chromosomal abnormalities. Results: We identified three cases with novel variants of t(8;21); der(13)t(8;21;13), isodicentric derivative 8 with chromosome 21[,+idicder(8)(q11.1)t(8;21)(q22;q11.1)] and der(21)t(8;12;21)(q22;q?;q22). Conclusion: AML with t(8;21)(q22;q22);RUNX1-RUNX1T1 forms a distinct WHO subcategory and hence the identification of variants or unusual translocations associated with t(8;21) deserves more attention. Contribution to the variant/ unusual t(8;21) database will further refine the risk stratification and may help to significantly advance the current treatment regimen.

Anderson Localization in the Fractional Quantum Hall Effect.

The interplay between interaction and disorder-induced localization is of fundamental interest. This article addresses localization physics in the fractional quantum Hall state, where both interaction and disorder have nonperturbative consequences. We provide compelling theoretical evidence that the localization of a single quasiparticle of the fractional quantum Hall state at filling factor ν=n/(2n+1) has a striking quantitative correspondence to the localization of a single electron in the (n+1)th Landau level. By analogy to the dramatic experimental manifestations of Anderson localization in integer quantum Hall effect, this leads to predictions in the fractional quantum Hall regime regarding the existence of extended states at a critical energy, and the nature of the divergence of the localization length as this energy is approached. Within a mean field approximation, these results can be extended to situations where a finite density of quasiparticles is present.

Synchronous Primary Multifocal Skeletal Chondrosarcoma of Extremity: A Report of 2 Cases.

CASE: Multifocal synchronous primary skeletal chondrosarcomas of an extremity are rarely reported. In this study, we report 2 such cases. The first case is a 32-year-old woman who presented with extensive right femoral and tibial diaphysis lesions. The second case is a 36-year-old woman with lesions in the left proximal humerus, the coracoid process of scapula and sternum. Both patients underwent limb salvage surgery and were disease-free at the 38- and 20-month follow-up. CONCLUSION: Athough rare, the possibility of multifocal chondrosarcoma should be kept in mind during the workup of a patient with chondrosarcoma.

Serum Free Light Chain Assay as a Prognostic Marker in Patients with Aggressive B-Cell Non-Hodgkin's Lymphoma: Impact on Survival Outcome.

T.M. AnoopBackground The role of serum free light chain (FLC) as a prognostic biomarker in lymphoproliferative diseases is being increasingly studied. In this study we present the 5-year survival outcome for patients with aggressive B-cell non-Hodgkin's lymphoma (NHL) and their relation to FLC and other known prognostic markers. Materials and Methods This is a prospective study conducted in patients diagnosed with aggressive B-cell NHL. Serum FLC level and ratio were estimated prior to initiation of treatment. Results A total of 100 patients were included in the study from December 2013 to December 2015 with a median age of 53 years. Thirty-eight patients (38%) had elevated FLC level of which 26% were polyclonal and 12% were monoclonal elevations. Abnormal FLC ratio was noted in 12% patients. Median follow-up duration of the study was 75 months. Five-year relapse-free survival (RFS) for the study population was 54.4%. Five-year RFS was 64.1% for early stage and 48.2% for advanced stage diseases ( p = 0.05). The RFS was significantly better in age less than 60 years (59.5% vs 43.8%, p < 0.001). Five-year overall survival (OS) was 61.3%. OS was significantly better in younger patients (73.6% vs 33.4%, p < 0.001), with International Prognosis Index score of 0 to 2 (87.4% vs 26.7%, p < 0.001). Patients with elevated FLC had inferior RFS (50% vs 71.4%, p = 0.04). Abnormal FLC ratio also strongly corresponded to inferior RFS (54.5% vs 66.2%, p = 0.001). OS was also significantly inferior in patients with abnormal FLC ratio (72.6% vs 63.6%, p = 0.001). Conclusion In patients with newly diagnosed aggressive B-cell NHL, elevated FLC levels and abnormal FLC ratio were significantly associated with inferior survival.

Star-topology registers: NMR and quantum information perspectives.

Quantum control of large spin registers is crucial for many applications ranging from spectroscopy to quantum information. A key factor that determines the efficiency of a register for implementing a given information processing task is its network topology. One particular type, called star-topology, involves a central qubit uniformly interacting with a set of ancillary qubits. A particular advantage of the star-topology quantum registers is in the efficient preparation of large entangled states, called NOON states, and their generalized variants. Thanks to the robust generation of such correlated states, spectral simplicity, ease of polarization transfer from ancillary qubits to the central qubit, as well as the availability of large spin-clusters, the star-topology registers have been utilized for several interesting applications over the last few years. Here we review some recent progress with the star-topology registers, particularly via nuclear magnetic resonance methods.

Exactly Solvable Model for Strongly Interacting Electrons in a Magnetic Field.

States of strongly interacting particles are of fundamental interest in physics and can produce exotic emergent phenomena and topological structures. We consider here two-dimensional electrons in a magnetic field, and, departing from the standard practice of restricting to the lowest LL, introduce a model short-range interaction that is infinitely strong compared to the cyclotron energy. We demonstrate that this model lends itself to an exact solution for the ground as well as excited states at arbitrary filling factors ν<1/2p and produces a fractional quantum Hall effect at fractions of the form ν=n/(2pn+1), where n and p are integers. The fractional quantum Hall states of our model share many topological properties with the corresponding Coulomb ground states in the lowest Landau level, such as the edge physics and the fractional charge of the excitations.

Magnetic-Field-Dependent Equilibration of Fractional Quantum Hall Edge Modes.

Fractional conductance is measured by partitioning a ν=1 edge state using gate-tunable fractional quantum Hall (FQH) liquids of filling 1/3 or 2/3 for current injection and detection. We observe two sets of FQH plateaus 1/9, 2/9, 4/9 and 1/6, 1/3, 2/3 at low and high magnetic field ends of the ν=1 plateau, respectively. The findings are explained by magnetic field dependent equilibration of three FQH edge modes with conductance e^{2}/3h arising from edge reconstruction. The results reveal a remarkable enhancement of the equilibration lengths of the FQH edge modes with increasing magnetic field.

Early Normalization of Free Light Chains Predicts Better Outcomes in Patients with Multiple Myeloma.

Background: The half-life of free light chain is short and can be used as an early marker for tumor response in patients with multiple myeloma [MM]. This prospective study is aimed at evaluating whether early light chain response can predict response to treatment in patients with MM. Materials and Methods: Thirty six patients with a diagnosis of MM and with an abnormal to normal light chain ratio of > 10 were included in this study. Results: The median age at presentation was 56 years. Fourteen patients had lambda light chain disease, whereas 22 patients had kappa light chain disease. Twenty-four patients [66.6%] had reduction of abnormal to normal light chain ratio to < 10 after 2 cycles, of whom 15 [62.5%] achieved a CR or VGPR after 6 cycles. Among 12 patients who did not have reduction of abnormal to normal light chain ratio to < 10, only 1 patient achieved CR while 11 patients [91.6%] achieved a PR or less[Fishers exact p=0.004]. Median follow-up was 13 months. Median progression-free survival for the entire cohort was 15 months. One-year Progression-Free Survival was 77% vs 57.1%, [p= 0.008], respectively for patients with early normalization and those who did not show early normalization. Conclusion: Early light chain response after 2 cycles of chemotherapy is a good predictor for treatment response in patients with MM treated with bortezomib based chemotherapy. Treatment intensification based on early light chain response merits further evaluation in a prospective trial.

Emergent SO(5) Symmetry at the Columnar Ordering Transition in the Classical Cubic Dimer Model.

The classical cubic-lattice dimer model undergoes an unconventional transition between a columnar crystal and a dimer liquid, in the same universality class as the deconfined quantum critical point in spin-1/2 antiferromagnets but with very different microscopic physics and microscopic symmetries. Using Monte Carlo simulations, we show that this transition has emergent SO(5) symmetry relating quantities characterizing the two phases. While the low-temperature phase has a conventional order parameter, the defining property of the Coulomb liquid on the high-temperature side is deconfinement of monomers, and so SO(5) relates fundamentally different types of objects. Studying linear system sizes up to L=96, we find that this symmetry applies with an excellent precision, consistently improving with system size over this range. It is remarkable that SO(5) emerges in a system as basic as the cubic dimer model, with only simple discrete degrees of freedom. Our results are important evidence for the generality of the SO(5) symmetry that has been proposed for the noncompact CP^{1} field theory. We describe an interpretation for these results in terms of a consistent hypothesis for the renormalization-group flow structure, allowing for the possibility that SO(5) may ultimately be a near-symmetry rather than exact.

Temporal Order in Periodically Driven Spins in Star-Shaped Clusters.

We experimentally study the response of star-shaped clusters of initially unentangled N=4, 10, and 37 nuclear spin-1/2 moments to an inexact π-pulse sequence and show that an Ising coupling between the center and the satellite spins results in robust period-2 magnetization oscillations. The period is stable against bath effects, but the amplitude decays with a timescale that depends on the inexactness of the pulse. Simulations reveal a semiclassical picture in which the rigidity of the period is due to a randomizing effect of the Larmor precession under the magnetization of surrounding spins. The timescales with stable periodicity increase with net initial magnetization, even in the presence of perturbations, indicating a robust temporal ordered phase for large systems with finite magnetization per spin.

Cavernous sinus syndrome.

Cavernous sinus syndrome (CSS) is a condition characterized by multiple cranial nerve palsies manifesting with ophthalmoplegia, ptosis, and facial sensory loss due to involvement of adjacent cranial nerves. Tumors, trauma, and vascular, infectious, and noninfectious inflammatory disorders have all been described as causes. Lymphomas have been reported to involve the cavernous sinus, both as primary cavernous sinus lymphomas or as secondary lesions. Here, we describe the case of a 63-year-old-man with untreated chronic lymphocytic leukemia (CLL), diagnosed 4 years earlier, who presented with CSS. Our patient underwent standard chemotherapy, but he succumbed to infection during the neutropenic period.

Primary spinal epidural B-lymphoblastic lymphoma.

Extranodal lymphomas constitute 20% to 30% of all non-Hodgkin's lymphomas. The common sites involved are skin, stomach, brain, and small intestine. Epidural localization is a rare site for lymphomas, accounting for 10% of spinal epidural tumors. Lymphomas occurring primarily in the epidural space without other previously detected lymphomatous foci (i.e., primary spinal epidural lymphomas) represent an even rarer entity. We report a case of primary spinal epidural B-lymphoblastic lymphoma. The patient presented with paraparesis, and a spinal epidural lesion was diagnosed. Considering the rapidity of symptom onset, the possibility of epidural abscess was considered, and he underwent partial laminectomy with decompression of the lesion. Histopathology and immunohistochemistry were diagnostic of B-lymphoblastic lymphoma. The present case is the first report in the literature of B-lymphoblastic lymphoma presenting as a spinal epidural lesion.

Acute myeloid leukemia presenting as galactorrhea.

Acute myeloid leukemia (AML) presents with symptoms related to pancytopenia (weakness, infections, bleeding diathesis) and organ infiltration with leukemic cells. Galactorrhea is an uncommon manifestation of AML. We report a case of AML presenting with galactorrhea.