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1.
Am J Hematol ; 97(9): 1150-1158, 2022 09.
Article in English | MEDLINE | ID: mdl-35713565

ABSTRACT

Intravascular lymphoma (IVL) is a rare extranodal non-Hodgkin lymphoma. We performed a retrospective analysis of 55 IVL patients who were treated at our institution 2003-2018. Median age at diagnosis was 68 years, and 64% were males. The most frequent presenting symptoms were skin rash 43% and weight loss 30%. MRI brain on IVL patients with CNS involvement (CNS-IVL) showed multifocal involvement in 76% (13/17). 89% (17/19) of non-CNS-IVL patients with abnormal FDG-PET had biopsy of an avid lesion resulting in definitive diagnosis. The top diagnostic biopsy site was the bone marrow (45%). 56% had multiorgan involvement. Based on CNS involvement, 36.5% (20/55) had CNS-IVL and 63.5% (35/55) had non-CNS-IVL. CNS-IVL group consists of clinically isolated CNS involvement (CNS-only IVL) (22%;12/55) and mixed clinical CNS and peripheral site involvement (M-IVL) (14.5%; 8/55). Non-CNS-IVL group consists of clinically isolated skin involvement (skin-only IVL) (9%; 5/55) and peripheral IVL with or without skin involvement (P-IVL); (54.5%; 30/55). Skin involvement was predominantly in the lower extremities. Pathologically, 89% (48/54) were B-cell IVL. Rituximab + high-dose methotrexate-based regimen were used in 75% (12/16) of CNS-IVL patients and RCHOP in 60% (17/28) of non-CNS-IVL patients. Estimated 5-year progression free survival (PFS) and overall survival (OS) for the entire cohort were 38.6% and 52%, respectively. Skin-only IVL was associated with excellent survival. Platelet count <150x109 /L, age > 60Y, and treatment without Rituximab were poor prognostic factors. Further research is necessary to identify novel therapies.


Subject(s)
Central Nervous System Neoplasms , Lymphoma, B-Cell , Lymphoma, Large B-Cell, Diffuse , Lymphoma, Non-Hodgkin , Lymphoma , Skin Neoplasms , Central Nervous System Neoplasms/drug therapy , Female , Humans , Lymphoma/pathology , Lymphoma, Large B-Cell, Diffuse/drug therapy , Male , Prognosis , Retrospective Studies , Rituximab/therapeutic use , Skin Neoplasms/pathology
3.
Adv Radiat Oncol ; 6(4): 100714, 2021.
Article in English | MEDLINE | ID: mdl-34409210

ABSTRACT

PURPOSE: This study compares reduced (<27 Gy) to standard dose (≥30 Gy) radiation therapy (RT) in the treatment of gastric extranodal marginal zone lymphoma of mucosa-associated lymphoid tissue (gMALT lymphoma). METHODS AND MATERIALS: Forty-two patients with stage I or II disease were retrospectively reviewed. Response to RT was assessed with endoscopy after RT. Complete response rate (CR), freedom from treatment failure, and overall survival (OS) were calculated. RESULTS: All patients were stage I (n = 40) or II (n = 2). All patients had residual biopsy proven gMALT lymphoma before RT. Twenty-six patients (61.9%) were treated with standard dose RT, 30 to 36 Gy, and 16 (38.1%) with the reduced dose RT, 23.5 to 27 Gy. The median follow-up was 29.5 months (range, 6-85). Thirty-six patients (86%) achieved complete response (CR), and 6 patients (14%) achieved partial response (PR). The complete response rate (CR) at the first endoscopic assessment, median time of 3 months, was 81% (95% confidence interval, 0.61%-0.93%) for standard RT, and 94% (confidence interval, 0.69%-0.99%) for reduced RT. Among CR patients, one patient had locally relapsed disease at 50 months. The 1-year overall survival (OS) was 100% in both groups. The 1-year freedom from treatment failure (FFTF) was 100% in the reduced RT group and 92% in the standard RT group. The 2-year FFTF and OS of the whole cohort were 92% and 96%, respectively. There was no significant difference in the OS, FFTF, and CR between the 2 treatment groups (P = .38, P = .18, and P = .267, respectively). For toxicity, the mean liver dose and the mean V20 heart dose were significantly lower in the reduced RT group (P <.001 and P = .001, respectively). However, incidence and severity of reported toxicities were similar between the 2 groups. CONCLUSIONS: Reduced dose RT (23.5-27 Gy) achieved excellent complete response rates with minimal toxicity, comparable with standard dose RT (30-36 Gy), for gMALT.

4.
Dermatopathology (Basel) ; 8(2): 221-228, 2021 Jun 17.
Article in English | MEDLINE | ID: mdl-34204191

ABSTRACT

Primary cutaneous γδ T-cell lymphoma (PCGD-TCL) is an extremely rare and aggressive T-cell neoplasm with complex heterogeneity. We present a series of two patients who presented with firm, subcutaneous nodules and were diagnosed with PCGD-TCL. In both cases, biopsies demonstrated a both superficial and deep adnexotropic infiltrate comprised of angiocentric, medium- to large-sized atypical lymphocytes. The infiltrate extended into the panniculus. Immuno-histochemical stains highlighted atypical lymphocytes that expressed CD3, CD8 and CD56 but were negative for EBV ISH. A brisk histiocytic response with focal aggregation into granulomas was highlighted with a PG-M1 stain. The atypical lymphocytes were positive for gene rearrangements on a TCR delta stain and negative for ßF-1. CT and PET scan in one of the two patients demonstrated diffuse, subcutaneous, ground-glass foci; hypermetabolic soft tissue nodules; and lymphadenopathy in the lungs, as well as splenomegaly. A diagnosis of histiocyte-rich PCGD-TCL was rendered. A histiocyte-rich, granulomatous variant of γδ T-cell lymphoma is extremely rare. Its potentially misleading resemblance to inflammatory granulomatous conditions could pose a diagnostic pitfall in this already challenging condition. This variant may resemble granulomatous mycosis fungoides and granulomatous slack skin syndrome, but it has a distinct, aggressive clinical outcome.

5.
Clin Case Rep ; 9(5): e03971, 2021 May.
Article in English | MEDLINE | ID: mdl-34094552

ABSTRACT

EBV-positive HHV8-negative EBL is part of the spectrum of EBV-positive diffuse large B-cell lymphoma NOS. This entity can be labeled as primary age-related EBV-associated EBL and appears to respond well to rituximab and thoracentesis.

6.
Leuk Res Rep ; 15: 100238, 2021.
Article in English | MEDLINE | ID: mdl-33816105

ABSTRACT

Myeloid sarcoma, also known as chloroma or granulocytic sarcoma is an extramedullary disease process that typically presents in association with acute myeloid leukemia during initial presentation or at relapse. Often associated with cytogenetic mutations, including t(8;21)(q22;q22); RUNX1/RUNX1T1, and less frequently with inv(16)(p13.1q22) or t(16;16)(p13.1;q22); CBFB/MYH11, myeloid sarcoma is most commonly discovered in skin, soft tissue, bone, and connective tissue. In rare circumstances, myeloid sarcoma can present without any evidence of bone marrow or leukemic involvement. These cases of de novo myeloid sarcoma are rare, and are commonly misdiagnosed due to similarities with other entities. We report an unusual case of a primary de novo peritoneal myeloid sarcoma, in association with inv(16)(p13;q22) and clonal heterogeneity at different sites of involvement, that has responded well to AML induction therapy and consolidation treatment with gemtuzumab ozogamicin and high dose cytarabine. Cytogenetics, immunophenotyping, and chromosomal analysis, were each critical in establishing a proper diagnosis as well as helping to develop appropriate therapeutic strategies for this rare entity.

7.
Clin Lymphoma Myeloma Leuk ; 21(2): 106-112.e1, 2021 02.
Article in English | MEDLINE | ID: mdl-33160933

ABSTRACT

BACKGROUND: Hepatosplenic T-cell lymphoma (HSTCL) is a rare subtype of peripheral T-cell lymphoma accounting for less than 1% of non-Hodgkin lymphomas. It is generally associated with poor prognosis. PATIENTS AND METHODS: We performed a cohort study of patients with HSTCL treated at the Mayo Clinic between 1996 and 2020 exploring the clinical characteristics and therapeutic outcomes. RESULTS: Twenty-two cases of HSTCL were identified with a median (range) age at diagnosis of 45.5 (15.5-80.6) years and a male predominance (15/22, 68.2%). Clinical characteristics include massive splenomegaly in 16 patients (73%), hepatic involvement in 13 (59%), and chronic immunosuppressed state in 8 (36%). Phenotypically, lymphoma cells had gamma/delta T-cell receptor expression in 18 (82%) and alpha/beta in 4 patients. Cytogenetic abnormalities included isochromosome 7q (i7q) in 8 (62%) of 13 and trisomy 8 in 4 (44%) of 9. The median (range) follow-up of surviving patients was 33 (2.5-137) months. The median progression-free and overall survival were 9.5 months (95% CI, 1.8, 16.3) and 12.4 months (95% CI, 4.9, 18.5), respectively. Long-term survival was seen in 4 (18%) of 22 patients, with survival of 55, 74, 95, and 137 months. Moreover, 3 of 4 long-term survivors had splenectomy as part of initial treatment, and 2 of 4 long-term survivors received an allogeneic hematopoietic cell transplant (allo-HCT). CONCLUSION: Liver involvement and chronic immunosuppression were associated with shorter survival. Although splenectomy and allo-HCT have anecdotal benefit in the literature, our data do not show a statistically significant benefit of splenectomy and/or allo-HCT, likely as a result of our small sample size.


Subject(s)
Hematopoietic Stem Cell Transplantation/statistics & numerical data , Liver Neoplasms/mortality , Lymphoma, T-Cell, Peripheral/mortality , Splenectomy/statistics & numerical data , Splenic Neoplasms/mortality , Adolescent , Adult , Aged , Aged, 80 and over , Chromosome Aberrations , Female , Follow-Up Studies , Humans , Liver Neoplasms/diagnosis , Liver Neoplasms/genetics , Liver Neoplasms/therapy , Lymphoma, T-Cell, Peripheral/diagnosis , Lymphoma, T-Cell, Peripheral/genetics , Lymphoma, T-Cell, Peripheral/therapy , Male , Middle Aged , Progression-Free Survival , Retrospective Studies , Splenic Neoplasms/diagnosis , Splenic Neoplasms/genetics , Splenic Neoplasms/therapy , Transplantation, Homologous , Young Adult
8.
Leuk Res Rep ; 14: 100228, 2020.
Article in English | MEDLINE | ID: mdl-33240789

ABSTRACT

Interleukin 6 receptor (IL6R) inhibitor, tocilizumab, has been effectively used in the treatment of cytokine release syndrome in patients receiving chimeric antigen receptor T-cell therapy. Here we present a patient with chronic myelomonocytic leukemia (CMML) who developed a steroid refractory, post-operative myelomonocytic leukemoid reaction (PO-MMLR), effectively treated with tocilizumab. Although, further studies are needed to validate the effectiveness of tocilizumab in management of PO-MMLR, this case serves to provide a new management approach in treatment of this rare but lethal syndrome with no standardized treatment options.

9.
Clin Case Rep ; 8(10): 2003-2006, 2020 Oct.
Article in English | MEDLINE | ID: mdl-33088539

ABSTRACT

This case illustrates an unusual presentation of paraneoplastic myositis in stage IV follicular lymphoma managed with high-dose steroids, a previously unreported entity in the literature.

10.
Hematol Rep ; 10(3): 7658, 2018 Sep 05.
Article in English | MEDLINE | ID: mdl-30283621

ABSTRACT

Concurrent presentation of acute promyelocytic leukemia (APL) with other hematologic diseases in the absence of previous chemotherapy or ionizing radiotherapy treatment is very rare. We present a case of simultaneous occurrence of APL with myelodysplastic syndrome (MDS)-related acute myeloid leukemia (AML). A 43-yearold female presented with 3 month of history fatigue, night sweats, chills and pancytopenia. Bone marrow aspirate and biopsy demonstrated 20% myeloid blasts with dysplastic changes admixed with abnormal promyelocytes. Cytogenetic analysis showed tetraploidy and deletion in chromosomes 5q and 7q and polymerase chain reaction showed presence of PML/RARA mRNA transcripts, confirming the presence of concurrent APL and MDS-related AML. Induction chemotherapy with cytarabine and daunorubicin was initiated along with all-trans retinoic acid. This is the first case to be reported in the literature of concurrent occurrence of APL with MDS-related AML. Treatment with 7 + 3 regimen and ATRA was successful in inducing complete remission.

11.
BMJ Case Rep ; 20182018 Jun 28.
Article in English | MEDLINE | ID: mdl-29954760

ABSTRACT

Given the prevalence of breast cancer and the mortality associated with metastatic disease, it is imperative for physicians to not only be aware of common sites but also of rare metastatic destinations such as the bladder. A postmenopausal woman with a medical history of stage 2 invasive ductal carcinoma, oestrogen receptor/progesterone receptor positive and human epidermal growth factor receptor 2 negative, in remission for 9 years, presented to her primary care physician with concerns of increased urinary urgency, frequency and incontinence. The patient underwent cystoscopy with biopsy of an area of granulation tissue. Biopsy revealed adenocarcinoma consistent with breast primary. The common sites of metastases from breast cancer are lung, bone and liver. This case is unique where breast cancer was found to metastasise to the bladder. It is important for physicians to consider further investigation when a breast cancer survivor develops urinary symptoms even without haematuria.


Subject(s)
Antineoplastic Agents, Hormonal/therapeutic use , Breast Neoplasms/pathology , Cancer Survivors , Carcinoma, Ductal, Breast/pathology , Estradiol/analogs & derivatives , Urinary Bladder Neoplasms/secondary , Urinary Bladder, Overactive/etiology , Breast Neoplasms/drug therapy , Carcinoma, Ductal, Breast/drug therapy , Cystoscopy , Estradiol/therapeutic use , Fatal Outcome , Female , Fulvestrant , Humans , Middle Aged , Receptor, ErbB-2 , Urinary Bladder Neoplasms/complications , Urinary Bladder Neoplasms/diagnosis , Urinary Bladder Neoplasms/drug therapy
12.
Rare Tumors ; 9(2): 6834, 2017 Jul 03.
Article in English | MEDLINE | ID: mdl-28975018

ABSTRACT

We previously reported an extremely rare case of follicular dendritic cell sarcoma (FDCS) presented as a thyroid mass. Given the rarity of this disease, there are no personalized and molecularly targeted treatment options due to the lack of knowledge in the genomic makeup of the tumor. A 44-year-old white woman was diagnosed with an extranodal FDCS in thyroid. The patient underwent a total thyroidectomy, central compartment dissection, parathyroid re-implantation, and adjuvant radiation therapy. Tumor DNA sequencing of 236 genes by FoundationOne panel found truncating mutations in PTEN and missense mutations in RET and TP53. However, patient-matched germline DNA was not sequenced which is critical for identification of true somatic mutations. Furthermore, the FoundationOne panel doesn't measure genomic rearrangements which have been shown to be abundant in sarcomas and are associated with sarcoma tumorigenesis and progression. In the current study, we carried out comprehensive genomic sequencing of the tumor, adjacent normal tissues, and patient-matched blood, in an effort to understand the genomic makeup of this rare extranodal FDCS and to identify potential therapeutic targets. Eighty-one somatic point mutations were identified in tumor but not in adjacent normal tissues or blood. A clonal truncating mutation in the CLTCL1 gene, which stabilizes the mitotic spindle, was likely a driver mutation of tumorigenesis and could explain the extensive copy number aberrations (CNAs) and genomic rearrangements in the tumor including a chr15/chr17 local chromothripsis resulted in 6 expressed fusion genes. The fusion gene HDGFRP3→SHC4 led to a 200-fold increase in the expression of oncogene SHC4 which is a potential target of the commercial drug Dasatinib. Missense mutations in ATM and splice-site mutation in VEGFR1 were also detected in addition to the TP53 missense mutation reported by FoundationOne.

14.
Rare Tumors ; 9(1): 6518, 2017 Mar 24.
Article in English | MEDLINE | ID: mdl-28458788

ABSTRACT

Hairy cell leukemia (HCL) is a low grade B-cell lymphoproliferative disorder that typically presents with splenomegaly, cytopenias, and diffuse bone marrow infiltration. There have been few cases in the literature of HCL presenting as lymphomas in extra-nodal locations, such as soft tissues and bones without circulating leukemic cells, splenomegaly, or iliac crest bone marrow involvement. We present an additional case presenting as a thoracic mass, and discuss potential diagnostic pitfalls and management of these rare cases.

15.
Hum Pathol ; 64: 207-212, 2017 06.
Article in English | MEDLINE | ID: mdl-28132860

ABSTRACT

Mantle cell lymphoma (MCL) is typically characterized by t(11;14), which places the IGH@ enhancer elements upstream of CCND1. This fusion results in up-regulation of CCND1 and consequently its protein product cyclin D1. Recent studies have shown that in MCL, mutations or translocations occurring within the 3' untranslated region (UTR) of the CCND1 gene can result in a truncated mRNA transcript that is more stable and associated with more aggressive disease. We identified a case of MCL showing cyclin D1 overexpression by immunohistochemistry and a t(11;12)(q13;p11.2) by conventional cytogenetic studies. Next-generation genomic sequencing indicated a chromosomal break through the CCND1 3'-UTR and fusion with a non-coding region of chromosome 12. We suggest that, in the absence of the typical CCND1/IGH@ fusion, this rearrangement promotes MCL pathogenesis by eliminating miRNA interaction elements within the 3'-UTR of the CCND1 mRNA transcript consequently resulting in CCND1 overexpression.


Subject(s)
Biomarkers, Tumor/genetics , Chromosomes, Human, Pair 11 , Chromosomes, Human, Pair 12 , Chromosomes, Human, Pair 13 , Cyclin D1/genetics , Lymphoma, Mantle-Cell/genetics , Translocation, Genetic , 3' Untranslated Regions , Aged , Biomarkers, Tumor/analysis , Biopsy , Cyclin D1/analysis , Gene Expression Regulation, Neoplastic , Genetic Predisposition to Disease , High-Throughput Nucleotide Sequencing , Humans , Immunohistochemistry , Lymphoma, Mantle-Cell/chemistry , Lymphoma, Mantle-Cell/pathology , Lymphoma, Mantle-Cell/therapy , Male , MicroRNAs/genetics , Phenotype , RNA, Messenger/genetics , Up-Regulation
16.
Blood ; 129(12): 1646-1657, 2017 Mar 23.
Article in English | MEDLINE | ID: mdl-28087540

ABSTRACT

Human herpesvirus-8 (HHV-8)-negative, idiopathic multicentric Castleman disease (iMCD) is a rare and life-threatening disorder involving systemic inflammatory symptoms, polyclonal lymphoproliferation, cytopenias, and multiple organ system dysfunction caused by a cytokine storm often including interleukin-6. iMCD accounts for one third to one half of all cases of MCD and can occur in individuals of any age. Accurate diagnosis is challenging, because no standard diagnostic criteria or diagnostic biomarkers currently exist, and there is significant overlap with malignant, autoimmune, and infectious disorders. An international working group comprising 34 pediatric and adult pathology and clinical experts in iMCD and related disorders from 8 countries, including 2 physicians that are also iMCD patients, was convened to establish iMCD diagnostic criteria. The working group reviewed data from 244 cases, met twice, and refined criteria over 15 months (June 2015 to September 2016). The proposed consensus criteria require both Major Criteria (characteristic lymph node histopathology and multicentric lymphadenopathy), at least 2 of 11 Minor Criteria with at least 1 laboratory abnormality, and exclusion of infectious, malignant, and autoimmune disorders that can mimic iMCD. Characteristic histopathologic features may include a constellation of regressed or hyperplastic germinal centers, follicular dendritic cell prominence, hypervascularization, and polytypic plasmacytosis. Laboratory and clinical Minor Criteria include elevated C-reactive protein or erythrocyte sedimentation rate, anemia, thrombocytopenia or thrombocytosis, hypoalbuminemia, renal dysfunction or proteinuria, polyclonal hypergammaglobulinemia, constitutional symptoms, hepatosplenomegaly, effusions or edema, eruptive cherry hemangiomatosis or violaceous papules, and lymphocytic interstitial pneumonitis. iMCD consensus diagnostic criteria will facilitate consistent diagnosis, appropriate treatment, and collaborative research.


Subject(s)
Castleman Disease/diagnosis , Castleman Disease/etiology , Herpesvirus 8, Human , Consensus , Diagnosis, Differential , Humans , Internationality , Practice Guidelines as Topic
17.
Rare Tumors ; 8(3): 6220, 2016 Sep 05.
Article in English | MEDLINE | ID: mdl-27746876

ABSTRACT

Post-transplant lymphoproliferative disorders (PTLD) are a serious complication of transplantation with a high mortality. Most PTLD present within the first year of transplantation and are associated with Epstein-Barr virus (EBV) infection. Plasmablastic lymphoma (PBL) is a rare but aggressive disease originally described in patients with HIV, presenting most commonly in the jaw and oral mucosa. To our knowledge, this is the first case of PBL presenting as PTLD of the lung in a HIV and EBV negative patient. Given the increasing number of transplants performed, we would like to share this uncommon presentation of PTLD as PBL.

18.
Case Rep Crit Care ; 2016: 5407597, 2016.
Article in English | MEDLINE | ID: mdl-27648312

ABSTRACT

Giant cell myocarditis (GCM) is a rare and commonly fatal form of fulminant myocarditis. During the acute phase, while immunosuppressive therapy is initiated, venoarterial extracorporeal membrane oxygenation (VA-ECMO) support is commonly used as a bridge to heart transplantation or recovery. Until recently, conventional transesophageal echocardiography and transthoracic echocardiography were the tools available for hemodynamic assessment of patients on this form of mechanical circulatory support. Nevertheless, both techniques have their limitations. We present a case of a 54-year-old man diagnosed with GCM requiring VA-ECMO support that was monitored under a novel miniaturized transesophageal echocardiography (hTEE) probe recently approved for 72 hours of continuous hemodynamic monitoring. Our case highlights the value of this novel, flexible, and disposable device for hemodynamic monitoring, accurate therapy guidance, and potential VA-ECMO weaning process of patients with this form of severe myocarditis.

20.
Ear Nose Throat J ; 94(7): E1-4, 2015 Jul.
Article in English | MEDLINE | ID: mdl-26214671

ABSTRACT

Otolaryngologists are called upon to evaluate and treat sinonasal masses discovered incidentally on imaging studies. Although common conditions such as sinonasal polyps and mucus retention cysts predominate, it is prudent practice to formulate a differential diagnosis to identify unusual conditions. We present a case of a maxillary sinus mass in a 78-year-old man that was discovered incidentally on brain imaging and subsequently identified on biopsy as an angiomyolipoma (AML). AMLs are benign hamartomatous tumors that rarely occur in extrarenal locations. Only a few cases have been reported in the nasal cavity. We believe our case represents the first reported instance of AML arising within a maxillary sinus. Identification of intratumoral fat within the mass on imaging studies may suggest the diagnosis of AML preoperatively. Close interdisciplinary collaboration among the otorhinolaryngology, radiology, and pathology services is beneficial for patient management. We report this case to raise awareness that AML can arise in this previously unreported location. Moreover, we wish to emphasize that AML should be considered in the differential diagnosis when imaging studies demonstrate a well-defined, heterogeneous, fat-containing solitary mass in the nasal cavity or maxillary sinus.


Subject(s)
Angiomyolipoma/diagnostic imaging , Maxillary Sinus Neoplasms/diagnostic imaging , Aged , Angiomyolipoma/pathology , Diagnosis, Differential , Humans , Incidental Findings , Magnetic Resonance Imaging , Male , Maxillary Sinus Neoplasms/pathology , Tomography, X-Ray Computed
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