-
Cancer Radiotherapie : Journal de La... Feb 2024Haematologic malignancies are particular in that they can generally be cured, even when distant metastases are present at diagnosis, unlike solid malignancies. Systemic... (Review)
Review
Is oligometastatic disease an applicable and useful concept in haematologic malignancies? A narrative review of radiation therapy standards, modern techniques, and innovations.
PURPOSE
Haematologic malignancies are particular in that they can generally be cured, even when distant metastases are present at diagnosis, unlike solid malignancies. Systemic treatments, including chemotherapy, targeted therapies, and immunotherapy, are the standard of care with excellent results. The considerable progress made in the management of these diseases in the last 20years has redefined the role of radiation therapy as minor in many clinical situations. We propose a literature review of data, showing that radiation therapy still has a role in curative, salvage, and palliative therapy situations.
MATERIAL AND METHODS
A document and literature search was carried out in the following databases: Medline and ClinicalTrial.gov, for the terms "radiotherapy", "haematologic malignancies", "Hodgkin lymphoma", "non-Hodgkin lymphoma", "CAR T cells", "multiple myeloma", "solitary plasmocytoma", "intensity-modulated radiotherapy", "extracranial stereotactic body radiation therapy" and "proton therapy references".
RESULTS
Haemopathological malignancies include a wide range of diseases and radiation therapy indications have been assessed over the past 20years. Currently, radiation therapy is indicated for localized disease (solitary plasmocytoma), as an adjuvant (Hodgkin lymphoma), in palliative settings, or after systemic treatment in relapsed patients (chimeric antigen receptor [CAR] T-cells) with a low recurrence burden, which can therefore be considered "oligorecurrence". Radiation therapy, through total body irradiation, has important indications, thanks to its immunomodulatory and/or myeloablative effects. Moreover, recent technological developments have made possible significant improvement in safety, contributing to radiation therapy being positioned in the treatment strategy of several indications.
CONCLUSIONS
Given the effectiveness of systemic treatments in hematologic malignancies, the oligometastasis stage is of little importance. A curative intent after local radiation therapy, even advanced stage, is possible, both with residual disease for advanced Hodgkin lymphoma, aggressive non-Hodgkin lymphoma, or solitary plasmocytoma, and even without evidence of disease after chemotherapy for Hodgkin or non-Hodgkin lymphoma. The role of new treatments, such as CAR T cells, allows us to consider radiation therapy after systemic treatment of relapsed diseases with low volume recurrence, which can be considered oligorecurrence.
Topics: Humans; Hodgkin Disease; Plasmacytoma; Lymphoma, Non-Hodgkin; Hematologic Neoplasms
PubMed: 38143233
DOI: 10.1016/j.canrad.2023.08.008 -
International Journal of Biological... Dec 2023Plasmacytoma Variant Translocation 1 (PVT1) is a long non-coding RNA located at 8q24.21 immediately downstream of MYC. Both the linear and circular PVT1 transcripts...
Plasmacytoma Variant Translocation 1 (PVT1) is a long non-coding RNA located at 8q24.21 immediately downstream of MYC. Both the linear and circular PVT1 transcripts contribute to cancer pathogenesis by binding microRNAs. However, little is known about their roles in B-cell lymphoma. Here we studied their expression patterns, role in growth, and ability to bind miRNAs in B-cell lymphoma. Linear PVT1 transcripts were downregulated in B-cell cell lymphoma lines compared to germinal center B cells, while circPVT1 levels were increased. Two Hodgkin lymphoma cell lines had a homozygous deletion including the 5' region of the PVT1 locus, resulting in a complete lack of circPVT1 and 5' linear PVT1 transcripts. Inhibition of both linear and circular PVT1 decreased growth of Burkitt lymphoma, while the effects on Hodgkin lymphoma and diffuse large B cell lymphoma were less pronounced. Overexpression of circPVT1 promoted growth of B-cell lymphoma lacking or having low endogenous circPVT1 levels. Contrary to other types of cancer, linear and circular PVT1 transcripts did not interact with miRNAs in B-cell lymphoma. Overall, we showed an opposite expression pattern of linear and circular PVT1 in B-cell lymphoma. Their effect on growth was independent of their ability to bind miRNAs.
Topics: Humans; MicroRNAs; Hodgkin Disease; Homozygote; Sequence Deletion; Cell Proliferation; RNA, Long Noncoding; Burkitt Lymphoma; Cell Line, Tumor
PubMed: 37689284
DOI: 10.1016/j.ijbiomac.2023.126744 -
Journal of Microscopy and Ultrastructure 2024One of the most challenging diagnostic categories in the sinonasal tract includes small-blue-round-cell tumors. These are malignant tumors which show many overlapping...
BACKGROUND
One of the most challenging diagnostic categories in the sinonasal tract includes small-blue-round-cell tumors. These are malignant tumors which show many overlapping histomorphology and immunohistochemistry (IHC) findings. Limited, small biopsy of these not completely excisable tumors adds to the diagnostic confusion.
MATERIALS AND METHODS
A cross-sectional study was done for 2 years (January 2018-December 2020) in a tertiary care institute, which included 70 cases of tumors of which 49 cases were malignant. All paraffin-embedded blocks were subjected to hematoxylin and eosin stain and IHC followed by molecular study wherever needed.
RESULTS
Of the total cases, small-blue-round-cell tumor constituted the major category comprising 20 rare and interesting cases which included sinonasal undifferentiated carcinoma (4 cases), malignant lymphoma (2 cases of diffuse large B-cell lymphoma and 2 cases of extranodal natural killer/T-cell lymphoma), rhabdomyosarcoma (2 cases), olfactory neuroblastoma (2 cases), malignant melanoma (2 cases), plasmacytoma (2 cases), atypical Ewing's sarcoma (EWS) (1 case), EWS (1 case), nuclear protein in testis (NUT) carcinoma (1 case), and small-cell neuroendocrine carcinoma (1 case).
CONCLUSION
Tumors of the sinonasal tract are very diverse, more so in small-round-cell tumor which present with a undifferentiated morphology. Thus, accurate diagnosis needs clinicoradiological parameters and special ancillary techniques such as IHC and molecular study in addition to histopathology for early diagnosis and therapy to prevent significant morbidity and mortality caused in these tumors.
PubMed: 38633570
DOI: 10.4103/jmau.jmau_70_21 -
International Journal of Surgery Case... Mar 2024Solitary ovarian plasmacytoma is a rare form of extramedullary plasmacytic tumor that develops outside the bone marrow.
INTRODUCTION
Solitary ovarian plasmacytoma is a rare form of extramedullary plasmacytic tumor that develops outside the bone marrow.
PRESENTATION OF CASE
We report the case of a 52-year-old female patient who consulted for pelvic pain and abdominal distension. Clinical examination revealed an abdominopelvic mass and a pelvic MRI confirmed a right ovarian mass. The diagnosis of ovarian plasmacytoma was made after exploratory laparotomy.
DISCUSSION
Extramedullary plasmacytoma (EMP) is rare and most often found in the upper aerodigestive tract, ovarian localization is exceptional. Histological and immunohistochemical features of EMP highlights similarities with multiple myeloma. EMP treatment options are discussed, including radiotherapy, surgery, and chemotherapy. The prognosis of EMP depends on several factors, including tumor size and timing of treatment.
CONCLUSION
Solitary ovarian plasmacytoma is a rare but important entity to consider in the diagnosis of abdominopelvic masses. Appropriate treatment, such as surgery combined with radiotherapy, can be effective, although regular monitoring is necessary due to the risk of relapse and transformation into multiple myeloma.
PubMed: 38364751
DOI: 10.1016/j.ijscr.2023.109096 -
Internal Medicine (Tokyo, Japan) Apr 2024A 65-year-old woman presented with fever and abnormal behavior. Magnetic resonance imaging showed swelling of the left medial temporal lobe and an intracranial...
A 65-year-old woman presented with fever and abnormal behavior. Magnetic resonance imaging showed swelling of the left medial temporal lobe and an intracranial extra-axial occipital tumor. While her neurological symptoms improved after the administration of corticosteroid therapy under the suspicion of autoimmune encephalitis, the occipital tumor unexpectedly shrank, and the diagnosis of a solitary plasmacytoma was confirmed by biopsy. Additional examinations revealed elevated anti-glutamate receptor antibodies in the cerebrospinal fluid. The patient was diagnosed with autoimmune encephalitis concurrent with an intracranial solitary plasmacytoma. Central nervous system involvement can be considered a neurological complication in patients with a solitary plasmacytoma.
PubMed: 38569910
DOI: 10.2169/internalmedicine.2888-23 -
Cureus Sep 2023Type 2 scleredema on the background of monoclonal gammopathy of undetermined significance (MGUS) is a rare and progressive connective tissue disorder with very few cases...
Type 2 scleredema on the background of monoclonal gammopathy of undetermined significance (MGUS) is a rare and progressive connective tissue disorder with very few cases reported to date. It is characterized by chronic and diffuse induration of the skin that begins in the upper back and neck and progresses proximally to distally, involving the shoulders, trunk, and arms; the hands are usually spared. Here, we present an unusual case of long-standing scleredema that progressed to involve the hands and fingers. This case was further complicated by new-onset Raynaud's phenomenon, splenomegaly, lymphadenopathy, the development of a plasmacytoma, and eventual progression to multiple myeloma. We highlight the differential diagnoses for his complex presentation, the workup that was completed, and current treatment options.
PubMed: 37701163
DOI: 10.7759/cureus.44968 -
The American Journal of Case Reports Aug 2023BACKGROUND The differential diagnosis for a parotid mass is broad, including infectious, autoimmune, and neoplastic etiologies. In people with HIV, regardless of viral...
BACKGROUND The differential diagnosis for a parotid mass is broad, including infectious, autoimmune, and neoplastic etiologies. In people with HIV, regardless of viral suppression or immune status, neoplastic causes are more common. This report describes the evaluation of a woman with a large parotid mass, with an ultimate diagnosis of multiple myeloma with extramedullary plasmacytoma. CASE REPORT A 51-year-old woman with HIV infection presented with headache, weight loss, and right facial mass that was present for 5 years but more rapidly enlarging in the prior year. CD4 count was 234 cells/mL, and HIV RNA was 10 810 copies/mL. Physical examination was significant for a large deforming right-sided facial mass, decreased sensation in the V1 and V2 distributions, and right-sided ophthalmoplegia and ptosis. MRI and PET/CT scan confirmed a metabolically active large parotid mass with extension into the cavernous sinus. An IgG kappa monoclonal spike was present on serum protein electrophoresis. Incisional biopsy of the facial mass showed atypical lymphoid cells with plasmablastic and plasmacytic morphology with a high mitotic rate and proliferation index. She was diagnosed with R-ISS stage II IgG kappa multiple myeloma with extramedullary plasmacytoma, and initiated on chemotherapy, radiation, and antiretroviral therapy. CONCLUSIONS A rapidly enlarging parotid mass should prompt timely evaluation and biopsy for definitive diagnosis, particularly in immunocompromised patients, including people with HIV. Extramedullary plasmacytomas have a more aggressive disease process in people with HIV and are associated with high-risk multiple myeloma and progression, as seen in this patient.
Topics: Female; Humans; Middle Aged; Plasmacytoma; Multiple Myeloma; HIV Infections; Positron Emission Tomography Computed Tomography; Hypertrophy; Immunoglobulin G
PubMed: 37533236
DOI: 10.12659/AJCR.938431 -
Cureus Dec 2023This case report details the case of a 57-year-old male who initially manifested low back pain radiating from the lumbar region to the left leg. Progressive symptoms...
This case report details the case of a 57-year-old male who initially manifested low back pain radiating from the lumbar region to the left leg. Progressive symptoms included paresthesia on the plantar surfaces of both feet and gait instability attributed to weakness in the pelvic limbs. Computed tomography imaging revealed osteolytic lesions in the T9, T10, and T11 vertebral bodies, resulting in compression of the spinal cord. Subsequent contrast-enhanced magnetic resonance imaging validated these findings, confirming the presence of an extradural tumor. In accordance with the Spinal Instability Neoplastic Score (SINS), the case was categorized as indicative of potential spinal instability. Consequently, a surgical intervention was performed to excise the lesion. Thus, the role of SINS played a pivotal role in guiding the decision-making process for the chosen treatment modality.
PubMed: 38226126
DOI: 10.7759/cureus.50627 -
Medicine Apr 2024Megalosplenia in newly diagnosed multiple myeloma (MM) is extremely rare, posing diagnostic and therapeutic challenges due to its unusual location and clinical... (Review)
Review
INTRODUCTION
Megalosplenia in newly diagnosed multiple myeloma (MM) is extremely rare, posing diagnostic and therapeutic challenges due to its unusual location and clinical manifestations and lack of optimal therapeutic strategies.
CASE PRESENTATION
A 65-year-old female who was previously healthy presented with a history of ecchymosis on her right leg accompanied by progressive fatigue for 2 weeks. She was admitted to our center in July 2019 due to thrombocytopenia. The patient presented with megalosplenia, anemia, monoclonal protein (λ-light chain type) in the serum and urine, and 45.6% malignant plasma cells in the bone marrow. Splenectomy was performed due to persistent splenomegaly after 3 cycles of the bortezomib plus dexamethasone regimen, and immunohistochemistry results indicated λ-plasmacytoma of the spleen. The same cytogenetic and molecular abnormalities, including t(14;16), 14q32 amplification, 16q32 amplification, 20q12 amplification, and a novel CYLD gene mutation, were identified using fluorescence in situ hybridization and next-generation sequencing in both bone marrow and spleen samples. Therefore, a diagnosis of MM (λ-light chain type, DS III, ISS III, R-ISS III, high-risk) with spleen infiltration was proposed. The patient did not achieve remission after induction treatment with bortezomib plus lenalidomide and dexamethasone or salvage therapy with daratumumab plus ixazomib and dexamethasone. However, she ultimately did achieve very good partial remission with a regimen of bendamustine plus lenalidomide and dexamethasone. Unfortunately, she died of pneumonia associated with chemotherapy.
CONCLUSION
To our knowledge, only 8 cases of spleen plasmacytoma at MM diagnosis have been described previously. Extramedullary myeloma patients with spleen involvement at diagnosis are younger and that the condition is usually accompanied by splenic rupture with aggressive clinical features and poor prognosis. Further studies are needed to explore pathogenesis and effective therapies to prolong the survival of such patients.
Topics: Humans; Female; Aged; Multiple Myeloma; Lenalidomide; Bortezomib; Plasmacytoma; In Situ Hybridization, Fluorescence; Dexamethasone; Mutation; Antineoplastic Combined Chemotherapy Protocols; Deubiquitinating Enzyme CYLD
PubMed: 38579060
DOI: 10.1097/MD.0000000000037624 -
Cureus Feb 2024Orbital plasmacytomas are exceedingly rare neoplasms characterized by the proliferation of monoclonal plasma cells in the orbital soft tissues. Their presentation and...
Orbital plasmacytomas are exceedingly rare neoplasms characterized by the proliferation of monoclonal plasma cells in the orbital soft tissues. Their presentation and clinical course can be diverse, making early diagnosis and management challenging. This case report sheds light on one such instance, emphasizing the diagnostic and therapeutic aspects of this uncommon condition. We present an 81-year-old patient with a prior diagnosis of multiple myeloma and bilateral orbital plasmacytomas, highlighting the importance of imaging in diagnosis and management.
PubMed: 38465072
DOI: 10.7759/cureus.53823