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JSM Neurosurgery and Spine

*Corresponding author

Case Report

Feyza Ka ra goz G uzey, Topka pi Ma ha llesi, Ka ha lba gi Soka k 46/2 Fa tih Ista nbul, Turkey, 34093, Tel: 905326334032; Ema il:

Primary Spinal Intramedullary Lymphoma: A Case Report and Review of the Literature

Submitted: 28 November 2014 Accepted: 05 Ja nua ry 2015 Published: 08 Ja nua ry 2015 Copyright

© 2015 G uzey et a l.

Feyza Karagoz Guzey1*, Yucel Hıtay1, Cihan Isler1, Abdurrahim

Tas1, Ozgur Aktas1, Azmi Tufan1, Mustafa Vatansever1 and Aslı

Kahraman Akkalp2

OPEN ACCESS

Keywords

• C entra l nervous system lymphoma • Lymphoma • Nonhodgkin lymphoma • Spina l intra medulla ry lymphoma • Spina l intra medulla ry tumor

1Department of Neurosurgery, Bagcilar Research and Training Hospital, Turkey 2Department of Neurosurgery and Pathology, Bagcilar Research and Training Hospital, Turkey

Abstract

Background: Primary spinal intramedullary lymphoma is a very rare lesion. It

has not specific laboratory or radiologic findings. Therefore false and late diagnosis is frequent.

Case Report and Literature Review: A 55 year-old lady with multifocal primary

spinal intramedullary B-cell non-Hodgkin’s lymphoma was reported. Review of

literature yielded 45 cases with primary spinal intramedullary lymphoma and clinical

and radiological characteristics of these 46 cases were evaluated.
Results: Male/female ratio was 24/22. Median age of the patients was 51.5 years for all cases except a series consisting 14 cases to be reported in an article in which detailed data case by case was not given. Median age of those 14 cases was 62.5. Most of the cases were localized in the cervical and then thoracic segments. Multifocal spinal tumors were found in 12 patients. Most of the patients were admitted with rapidly progressing myelopathy symptoms and signs. Most frequently seen magnetic resonance appearance was T2 hyperintensity and dense and homogeneous contrast enhancement with spinal cord enlargement. Misdiagnosis was frequent (23 cases) and certain diagnosis could be provided with autopsy in 5 cases. Tissue sampling was performed mostly with spinal cord biopsy (27) and brain biopsy (10 cases). Most of the cases had B-cell lymphoma (26 cases). Patients were treated with various types of chemotherapy, radiotherapy or combination of them. Twenty-six cases were died during follow-up median 16.5 months (for 10 cases of the largest series consisting 14 cases) and 10 months (for other cases) after onset of symptoms and signs.

Survival time was significantly different between neither cell types (B cell and other lymphomas) nor initial treatment modalities (chemotherapy, radiotherapy or combined of them).

Conclusions: Primary spinal intramedullary lymphoma diagnosis is difficult, and misdiagnosis is frequent. It must be kept in mind for differential diagnosis especially in middle aged patients with rapidly progressing myelopathy findings. Prognosis is poor despite aggressive treatment.

other CNS sites sequentially or concurrently [2]. It is not a rare event in the patients with brain lesions because of spread via direct invasion from the medulla oblongata or dissemination with

cerebrospinal fluid (CSF) [3]. Kawasaki et al [4] reported that there was spinal cord involvement in 4 of 14 cases with primary

brain lymphomas. However, isolated spinal cord involvement is

exceedingly rare and observed in 1-3.3% of all cases of primary

CNS lymphomas [5,6].

INTRODUCTION

Primary central nervous system (CNS) lymphoma sourced from brain, leptomeninges, eyes or spinal cord is an uncommon type of extranodal non-Hodgkin’s lymphoma which constitutes

only 1% of all lymphomas in the body [1]. Patients are classified

as “primary” if there is no evidence of systemic lymphoma by screening at the time of diagnosis [2]. Spinal cord lymphomas may be primary and originate in the spinal cord or accompany

Cite this article: Guzey FK, Hıtay Y, Isler C, Tas A, Aktas O, et al. (2015) Primary Spinal Intramedullary Lymphoma: A Case Report and Review of the Litera - ture. JSM Neurosurg Spine 3(1): 1049.
Guzey et al. (2015)
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Because of rarity of these lesions, primary intramedullary lymphomas (PIML) are usually not suggested in differential diagnosis in the patients with spinal intramedullary masses. Therefore, delayed diagnosis and misdiagnosis is very frequent. However, early diagnosis is important because effective treatment in early period of the disease may provide quite long survival.

We reported a case with primary spinal intramedullary B-cell

lymphoma and also we found 45 cases with detailed data in literature. Characteristics of these 46 cases were discussed.

CASE REPORT

A 55 year-old lady who had not any disease previously was referred to our clinic for spinal intramedullary mass at the lumbar segments of the spinal cord and conus region. She had a monoparesis of her left leg for one month. On her physical examination, there was a left leg paresis with 1-2/5 strength

on proximal and 3/5 strength on distal muscles. There was

not hypoesthesia or sphincteric disturbances. Spinal magnetic resonance imaging (MRI) revealed an intramedullary mass,

41x14 mm in size, locating in the lumbar segments of the spinal

cord and conus medullaris at T12 to L1 levels. It extended into the left neural foramen along with spinal root. The mass was hypointense on T1-weighted, and slightly hyperintense on T2- weighted sections and it was enhanced homogeneously after

intravenous contrast injection (Figure 1 a-d). In addition, there was a small mass with same imaging characteristics in the filum terminale at L2 level (Figure 1 c). Brain, cervical and thoracic

MRIs did not reveal other pathologies.

Figure 1a Lumbar T1 (a) weighted sagittal and T2 weighted axial.

Routinely performed blood biochemical parameters, infection markers and peripheral blood cell counts were at normal levels

and serologic tests including human immunodeficiency virus

(HIV) and hepatitis viruses were negative. Abdominal and

thoracic computerized tomography and breast investigations

performed for primary tumor screening were also normal.

Figure 1b MRI sections showed an intramedullary mass with T1 iso and T2 hyperintensity.

She operated on for especially histological diagnosis. The

intramedullary tumor was infiltrative and it had not distinct

borders intraoperatively. Only biopsy and decompressing duraplasty could be performed. On the day of surgery, prednisolone 250 mg intravenously was performed and it was continued for the day after and then gradually decreased. Just after operation, her leg paresis partly improved. Pathological investigation showed that the tumor was large B-cell lymphoma

(Figure 2). Bone marrow aspiration and thorax and abdomen computerized tomographies revealed no pathology. The tumor was accepted as a PIML. It could not be find any immunological

risk factor.
Her treatment was continued in an oncology center. She was

treated with whole craniospinal radiotherapy (RT) consisting 36

grays in 20 fractions and additional doses to the posterior fossa (9 grays in 5 fractions) and to the operation site (T12-L2) (9 grays in 5 fractions). However she died because of dissemination of the

disease after 3 months from operation (4 months from onset of

the disease).

Figure 1c

systemic disease during first admission were not included into

the study. The cases that had been reported in CNS lymphoma series were introduced into the study only if their detailed

characteristics had been reported in the articles. Forty-five cases found in the literature [2,3,6-34] and total 46 cases with our one

were evaluated for their clinical and radiological characteristics

REVIEW OF LITERATURE

Medline were searched for PIML. All found papers were evaluated. Cases with previously known lymphoma story or

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Table 1: Articles reporting the cases with primary spinal intramedullary lymphoma.

Literature

Herbst et al [7]

Year

1976 1977 1980 1982

1983

1986 1987 1990 1990

1994

1995 1995 1996 1996 1998 1999 2000 2000 2001 2002 2002 2002 2002 2002 2007 2007 2010 2011 2012

2013 2014 2014

n

111111111111111111121111111

14

1111

46

Bruni et al [8] Mitsumoto et al [9] Slager et al [10]

Hautzer et al [11]

Itami et al [12]

Landan et al [13]
Bluemke and Wang [14]
Slowik et al [3]

Figure 1d It had indistinct borders and enhanced homogeneously after intravenous contrast injection (c and d). In addition, there was a

second small mass with similar characteristics in the filum terminale

at L2 level (c).

Nakao et al [15] Schild et al [16] McDonald et al [17] Urasaki et al [18]

Kim et al [19]

Caruso et al [20] Drouet et al [21] Pels et al [22]

Thurnher et al [23] Bekar et al [24] Nakamizo et al [25]

Herrlinger et al [26] Legais et al [27]

Schwarz et al [28]

Lee et al [29]

Figure 2 Diffuse and strong CD20 immunoreactivity in the neoplastic

cells (CD20 X400).

Machiya et al [30] Peltier et al [31] Toshkezi et al [32] Flanagan et al [2] Lin et al [33]

(Table 1). Intravascular lymphoma cases [26,27] were not excluded in spite of some peculiar characteristics of this entity

because there were 3 cases with intravascular B cell lymphoma in the series of Flanagan et al [2]. Also, two cases with primary

spinal intramedullary histiocytic lymphoma without systemic

involvement [9,32] were included into the study. One case [15]

was included into the study despite the paper could not be found. However, characteristics of that case were given in detail in other papers.

Sato et al [6]

Bhushanam et al [34]

Our case

Total

n: number of patients reported in articles

Fourteen of these 46 cases were reported in a series by
Flanagan et al [2]. In these series, characteristics of the patients

were given altogether without detailed in case by case. Therefore,

some data were given separately for Flanagan’s series and other

cases in our review.

RESULTS

Male/female ratio was 24/22. Ages of the patients were between 11-82 years with a 62.5 years median age for Flanagan’s

series and 51.5 years median age for other cases (Table 2).

STATISTICAL EVALUATION

The relationships of metric independent parameters were evaluated with Student-t independent test and nonmetric independent parameters were evaluated with chi-square and

Fisher’s exact tests according to the subject numbers. Survival was demonstrated as Kaplan-Meier curves according to the cell

type and treatment modalities. Survival time of patients with different cell type and treatment modalities was compared by

Wilcoxon test. It was accepted as significant if p value was <0,05.

Clinical picture

All patients had an initial neurologic presentation of progressive myelopathy according to the level of their lesions except one who had conus medullaris symptoms and signs (Table

2). Symptom onset was subacute (>8 weeks) in 21 (46.6%) and insidious (<8 weeks) in 24 cases (53.3%). In one case there was

not adequate data in the article [23].

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Risk factors

Table 2: Demographic data and clinical pictures of the patients and

localizations of the tumors.

There were some immune disorders such as acquired immune

deficiency syndrome (AIDS), or myasthenia gravis in 7 patients [2,3,11,12,18,23]. Pathological types were not mentioned in 2 of

them [2], however, other 5 cases had non-B-cell type lymphomas.

These patients with immunological risk factors was significantly younger than the immunocompetent patients (p=0,023).

Demographic data

Gender#: 24 male/ 22 female Age: Median 51.5 and mean±SD 50.41±16.03 (11-77) years* and median 62.5 (41-82) years&

Clinical pictures*

Paraparesis/tetraparesis: 26 cases Hemiparesis/leg monoparesis: 5 cases Conus syndrome: 1 case

Localization and dissemination of the tumor

Duration of the symptoms from onset to admission:

Median 1, mean±SD 3.27±4.95 month (5 days-23 months)

Localization*α

In the Flanagan’s series detailed tumor localization data were

not given. In 6 of those cases, the lesion was located in the conus

medullaris, however the localization of the 8 intramedullary lesions was not mentioned. In the other 32 cases, tumor localizations were listed in Table 2.

Cervical 13; Thoracic 10; Lumbar 2; Conus 3; Longer segments

7

Dissemination#

Multiple spinal lesions: 12 (9 cases from Flanagan’s series, 3

cases from other reports)

CNS involvement: 21 (9 cases from Flanagan’s series, 12 cases

from other reports)

Lesions were multicentric in the spinal cord/ filum terminale

or spinal leptomeninges in 12 cases [2,20,28,our case]. In 26 cases, the disease disseminated to other sites of CNS (in 21 cases) or outside of the CNS (in 5 cases) (Table 2). In 2 of them, CNS [11] or abdominal lymph node [26] involvement were

diagnosed during postmortem examination. In other 24 cases, dissemination was diagnosed during the first diagnosis (limited to the CNS) in 9 patients, or after median 3 (1 to 18) months from

onset of symptoms in the others.

Systemic dissemination: 5

#All cases; *32 cases other than the cases of Flanagan’s series [2]; α3 cases of 32 had multiple spinal lesions &14 cases reported by Flanagan et al.

Table 3: MRI findings of 34 patients investigated with MRI except one

whose detailed MRI data were not mentioned in the article [19].

Radiological examinations

  • T2 signal characteristics
  • n

27

4*

2

In 38 cases out of 46, diagnosis was made with MRI, and in 7

cases with myelography. In one case, it was not mentioned in the article [19].

Hyperintense: Hypointense Isointense

In most of the cases, there was a typical appearance on MRI:
Iso-hypointensity on T1-weighted sections, hyperintensity on T2-weighted sections, dense and usually homogeneous contrast enhancement with spinal cord enlargement and edema. Masses

usually had indistinct borders. However these findings were not

rule, and this typical appearance had been shown in 19 cases

(Table 3). Tumor might not be enhanced [14,27], or it might have central hypointense area in the contrast enhancement [32], or

spinal cord enlargement might not be seen [21].

  • NM
  • 6

Expansion of spinal cord

  • Positive
  • 27

  • 7
  • Negative

  • NM
  • 2

Contrast enhancement

Positive

35

0

In 24 cases there were some misdiagnoses prior certain diagnosis (Table 4). In 5 cases, certain diagnosis could be made after death [2,8,10,11,13]. Four of them were from pre-MRI era

and were evaluated with myelography.

Negative

  • NM
  • 1

Typical MRI appearance(T2 hyperintensity,

  • cord expansion and contrast enhancement
  • 19

Other investigations

n: number of patients NM: not mentioned in the article

*in 3 of those 4 cases (from Flanagan’s series) there were multiple lesions,

and some of them were hyperintense on T2 weighted MRI.

Blood chemistry and cell counts of all patients with adequate data in the papers were normal on admission except one with myasthenia gravis whose immunoglobulin G and M levels were high [11].

Tissue diagnosis

Cerebrospinal fluid results were reported in 33 cases including 12 cases from Flanagan’s series. In 5 of them, CSF findings had been reported as “normal”. In other cases, protein

level and cell count were high (Table 5). There were malignant

cells in CSF in only 4 patients [2,19].

Tissue diagnosis was provided with spinal cord in 27 cases,

with brain biopsy in 10 cases. In one case [23], type of tissue sampling was not mentioned in the article, in 3 cases diagnosis could be performed with CSF cytology [2,19], and in 5 cases it could be performed with autopsy [2,8,10,11,13].

Tests for screening of systemic malignancy such as thorax or

abdomen computerized tomography and bone marrow biopsy

were negative in all patients.
There were several histological diagnoses from general terms such as non-Hodgkin lymphoma to very detailed ones such as B-cell perivascular low grade lymphoma. In 26 cases, tumor was

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Table 4: Misdiagnoses of the patients.

Treatment

  • Misdiagnoses
  • Number of patients

It is well known that steroid treatment may result marked improvement of clinical picture in cases with CNS lymphoma.

In 16 out of 46 cases, steroid was administered prior to certain diagnosis, and 14 of them were improved dramatically for a while [2,6,9,26,27,32,our case].

Transverse miyelitis Multiple sclerosis Syringomyelia
55

3

Chronic demyelinating disease Disk disease

3

None of the cases whose diagnosis was provided with spinal

cord biopsy deteriorated after surgery. In 4 of these 27 cases

subtotal resection was also added to the biopsy and in our case, a wide duraplasty was also performed.

2
Disseminated encephalomyelitis Infectious myelitis Sarcoidosis
211

In 38 patients whose diagnosis could be performed in vivo

and whose treatment data were adequate in articles, various types of radiotherapy (RT), chemotherapy (CT), or combination of them were performed after staging of the disease (Table 7).

Neuromyelitis optica

Total

1

23

Table 5: CSF findings of the patients.

Outcome

CSF findings

Level (median/interval)

In Flanagan’s series [2] outcome of the patients and results

of treatment modalities were not given case by case. In this series, 10 patients died (median duration symptom onset to

death, 16.5 months; range 2–97), and 4patients remained living at last follow-up (median 59 months from symptom onset; range 3–138). In 3 cases from other reports, there was not follow-up data in the articles [23,30,33]. In other 29 cases, median duration of follow-up from onset of the symptoms was 17 (range 4- 60) months, and 17 cases died median 10 (range 4- 30) months after first symptoms. Kaplan-Meier curves of those 29 cases were shown in Figure 3.

Protein

110&/70.75* (24-466) mg/dl

Cell count (mainly polyclonal lymphocyte)

&12 cases of Flanagan et al; *16 cases from other series except 5 cases
92&/48* (2-369)/mm3 whose CSF findings had been reported as “normal”

from B-cell lineage (5 of them had intravascular lymphomas), and in 7 cases from T-cell lineage consistent to the rate of cell

type reported in literature for CNS lymphomas [35] (Table 6).
Histological types did not show statistically significant

differences according to the gender or age of the patients (p=0,08

and p=0,94, respectively).

Outcome and survival times of the various treatment groups

were shown in Table 7. There was no statistically significant

Table 6: Histological type and their outcome.

Gender*α male/female
Age (year)*α Median (interval) fu (month)
Outcome on last fu*

  • Histological type
  • Number of patients

median (interval)&

  • Alive
  • Dead
  • NM

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  • Intravascular Large B-Cell Lymphoma: a Case Serie

    Intravascular Large B-Cell Lymphoma: a Case Serie

    Case Report | Iran J Pathol. 2020; 15(4): 346-350 Iranian Journal of Pathology | ISSN: 2345-3656 Intravascular Large B-cell Lymphoma: A Case Serie Fereshteh Ameli1*, Fatemeh Nili Ahmad Abadi1, Hana Saffar1 1. Department of Pathology, Cancer Institute, Imam Khomeini Hospital Complex, Tehran University of Medical Sciences, Tehran, Iran KEYWORDS ABSTRACT Extranodal large B-cell lymphoma, One of the rare variants of extranodal large B-cell lymphoma is intravascular large Intravascular lymphoma, B-cell lymphoma (IVLBCL). Characteristics of IVLBCL include intraluminal selective Fever of Unknown Origin (FUO) proliferation of atypical lymphoid cells in small to medium-sized vessels. The etiologic Scan to discover online of IVLBCL is unknown, but due to the growth pattern of this tumor, it is speculated that IVLBCL is caused by a defect in homing receptor of tumor cells. IVLBCL can involve any organ but central nervous system, lungs, and skin are the most involved sites. IVLBCL does not usually involve lymph nodes. IVLBCL mainly occurs in the middle aged to elderly population with a slight male predominance. Generally, IVLBCL is aggressive and rapidly fatal if left untreated. Main Subjects: We here reported two cases of IVLBCL who succumbed to the disease at initial phase Hematopathology of treatment to emphasize the difficulty in diagnosis of IVLBCL due to its exclusive intravascular growth pattern and fulminant clinical course. Received 04 Jan 2020; Accepted 10 April 2020; Published Online 16 July 2020; 10.30699/ijp.2020.119590.2299 Fereshteh Ameli; Department of Pathology, Cancer Institute, Imam Khomeini Hospital Complex, Tehran Corresponding Information: University of Medical Sciences, Tehran, Iran.
  • WHO/EORTC Classification of Cutaneous Lymphomas 2005: Histological and Molecular Aspects

    WHO/EORTC Classification of Cutaneous Lymphomas 2005: Histological and Molecular Aspects

    J Cutan Pathol 2005: 32: 647–674 Copyright # Blackwell Munksgaard 2005 Blackwell Munksgaard. Printed in Singapore Journal of Cutaneous Pathology WHO/EORTC classification of cutaneous lymphomas 2005: histological and molecular aspects 1 1,2 Abstract: The new WHO/EORTC classification for cutaneous Gu¨ nter Burg , Werner Kempf , lymphomas comprises mature T-cell and natural killer (NK)-cell Antonio Cozzio1, Josef Feit1,3, neoplasms, mature B-cell neoplasms, and immature hematopoietic Rein Willemze4, Elaine S. Jaffe5, malignancies. It reflects the unique features of lymphoproliferative Reinhard Dummer1, Emilio diseases of the skin, and at the same time it is as compatible as possible Berti6, Lorenzo Cerroni7, Sergio with the concepts underlying the WHO classification for nodal Chimenti8, Jose´ L. Diaz-Perez9, lymphomas and the EORTC classification of cutaneous lymphomas. Florent Grange10, Nancy L. This article reviews the histological, phenotypical, and molecular Harris11, Dmitry V. Kazakov12, genetic features of the various nosological entities included in this new Helmut Kerl7, Michael Kurrer13, classification. These findings always have to be interpreted in the Robert Knobler14, Chris J.L.M. context of the clinical features and biologic behavior. Meijer15, Nicola Pimpinelli16, Aim: To review the histological, phenotypical and molecular genetic Elisabeth Ralfkiaer17, Robin features of the various nosological entities of the new WHO/EORTC Russell-Jones18, Christian classification for cutaneous lymphomas. Sander19, Marco Santucci20, Methods: Extensive review of the literature cited in Medline and own Wolfram Sterry21, Steven H. data of the authors. Swerdlow22, Maarten H. Results: The WHO/EORTC classification of cutaneous lymphomas Vermeer4, Janine Wechsler23 comprises mature T-cell and NK-cell neoplasms, mature B-cell and Sean Whittaker18 neoplasms and immature hematopoietic malignancies.
  • Primary Pulmonary B-Cell Lymphoma: a Review and Update

    Primary Pulmonary B-Cell Lymphoma: a Review and Update

    cancers Review Primary Pulmonary B-Cell Lymphoma: A Review and Update Francesca Sanguedolce 1,*,†, Magda Zanelli 2,† , Maurizio Zizzo 3,4 , Alessandra Bisagni 2, Alessandra Soriano 5, Giorgia Cocco 6, Andrea Palicelli 2 , Giacomo Santandrea 2 , Cecilia Caprera 7, Matteo Corsi 7, Giulia Cerrone 7 , Raffaele Sciaccotta 7, Giovanni Martino 7, Linda Ricci 7, Francesco Sollitto 8, Domenico Loizzi 8,‡ and Stefano Ascani 7,‡ 1 Pathology Unit, Azienda Ospedaliero-Universitaria, Ospedali Riuniti di Foggia, 71122 Foggia, Italy 2 Pathology Unit, Azienda USL-IRCCS di Reggio Emilia, 42122 Reggio Emilia, Italy; [email protected] (M.Z.); [email protected] (A.B.); [email protected] (A.P.); [email protected] (G.S.) 3 Surgical Oncology Unit, Azienda USL-IRCCS di Reggio Emilia, 42122 Reggio Emilia, Italy; [email protected] 4 Clinical and Experimental Medicine PhD Program, University of Modena and Reggio Emilia, 41121 Modena, Italy 5 Gastroenterology, Division and Inflammatory Bowel Disease Center, Department of Internal Medicine, Azienda USL-IRCCS di Reggio Emilia, 42122 Reggio Emilia, Italy; [email protected] 6 Radiotherapy Unit, Azienda Ospedaliero-Universitaria, Ospedali Riuniti di Foggia, 71122 Foggia, Italy; [email protected] 7 Pathology Unit, Azienda Ospedaliera S. Maria di Terni, University of Perugia, 05100 Terni, Italy; [email protected] (C.C.); [email protected] (M.C.); [email protected] (G.C.); [email protected] (R.S.); [email protected] (G.M.); [email protected] (L.R.); [email protected] (S.A.) 8 Institute of Thoracic Surgery, University of Foggia, 71122 Foggia, Italy; [email protected] (F.S.); [email protected] (D.L.) Citation: Sanguedolce, F.; Zanelli, * Correspondence: [email protected]; Tel.: +39-0881-736315 M.; Zizzo, M.; Bisagni, A.; Soriano, A.; † These authors contributed equally to this work.
  • Autologous Hematopoietic Stem Cell Transplantation for Intravascular Large B-Cell Lymphoma: the European Society for Blood and Marrow Transplantation Experience

    Autologous Hematopoietic Stem Cell Transplantation for Intravascular Large B-Cell Lymphoma: the European Society for Blood and Marrow Transplantation Experience

    Bone Marrow Transplantation (2017) 52, 650–652 © 2017 Macmillan Publishers Limited, part of Springer Nature. All rights reserved 0268-3369/17 www.nature.com/bmt LETTER TO THE EDITOR Autologous hematopoietic stem cell transplantation for intravascular large B-cell lymphoma: the European Society for Blood and Marrow Transplantation experience Bone Marrow Transplantation (2017) 52, 650–652; doi:10.1038/ hematopoietic stem cell transplantation (autoHSCT) has been – bmt.2016.339; published online 19 December 2016 reported to result in a more favorable outcome in single cases7 14 and small case series, almost exclusively in patients of Asian origin (Supplementary Material). In detail, in the pre-rituximab era, two 5 4 Intravascular lymphomas are rare lymphoproliferative disorders out of four and five out of seven autografted patients with IVLBCL were described to survive without relapse. After introduc- characterized by the selective growth of malignant lymphoid cells 6 15 within the lumina of small vessels. On histological and immuno- tion of rituximab, seven out of seven and six out of six patients histochemical examination, around 90% of reported cases meet have been reported to remain alive and relapse-free after 1 autoHSCT. the criteria of large B-cell lymphoma and are categorized into a fi distinct entity in the WHO classification under the designation The objective of the present study was to analyze for the rst time the efficacy of autoHSCT for IVLBCL in a larger cohort of intravascular large B-cell lymphoma (IVLBCL).2 Although IVLBCL Western patients in the rituximab era. can involve extranodal sites in virtually any organ, skin and central This was a registry-based retrospective multicenter study nervous system are predilection sites in Western countries, including patients aged 18 years or over with histologically whereas Asian patients more often present with involvement of fi 1,3,4 veri ed IVLBCL who underwent autoHSCT between 1 January bone marrow, liver and spleen.
  • Review Histopathology, Pathogenesis and Molecular Genetics in Primary

    Review Histopathology, Pathogenesis and Molecular Genetics in Primary

    Histol Histopathol (2004) 19: 211-219 Histology and http://www.hh.um.es Histopathology Cellular and Molecular Biology Review Histopathology, pathogenesis and molecular genetics in primary central nervous system lymphomas M. Nakamura, K. Shimada, E. Ishida and N. Konishi Department of Pathology, Nara Medical University, Nara, Japan Summary. Recent increases in the incidence of primary or lymphatics within the nervous system, however, the central nervous system lymphoma (PCNSL), a rare non- pathogenesis and histogenetic origin of PCNSL in Hodgkin’s lymphoma arising in the brain, have been immunocompetent patients is still poorly understood noted in both immunodeficient and immunocompetent regardless of the phenotypic similarities between CNS patients. Compared with lymphomas originating outside and non-CNS lymphomas. the central nervous system, the biology of PCNSL at the molecular or cytogenetic level has not been well Epidemiology characterized, yet it is important to thoroughly understand the etiology of this rare malignant lymphoma The incidence of PCNSL has been increasing if effective therapies are to be developed. This review steadily since the 1970s. Data from the Surveillance, will focus on the epidemiology, clinical aspects, Epidemiology and End Results (SEER) database of the histopathology, pathogenesis, and molecular genetics of National Cancer Institute show that PCNSL increased this aggressive, extranodal lymphoma in more than 10-fold from 0.025/100000 in 1973 to immunocompetent patients. 0.3/100,000 in 1991 and the forecasted incidence for the year 2000 is 0.5/100,000 (Corn et al., 1997). The Key words: Primary central nervous system PCNSL/glioblastoma rate was 1/250 in 1974 and rose to lymphomas, 6q LOH, R-PTP-κ, p14ARF, p16INK4A 1/36 in 1980, reaching 1/6 in 1991 (Eby et al., 1988).
  • Lymphoproliferative Lung Disorders: Clinicopathological Aspects

    Lymphoproliferative Lung Disorders: Clinicopathological Aspects

    EUROPEAN RESPIRATORY UPDATE LYMPHOPROLIFERATIVE DISORDERS Lymphoproliferative lung disorders: clinicopathological aspects Venerino Poletti1, Claudia Ravaglia1, Sara Tomassetti1, Carlo Gurioli1, Gianluca Casoni1, Silvia Asioli2, Alessandra Dubini2, Sara Piciucchi3 and Marco Chilosi4 Affiliations: 1Dept of Diseases of the Thorax, Ospedale GB Morgagni, Forlı`, 2Dept of Anatomic Pathology, Ospedale GB Morgagni, Forlı`, 3Dept of Radiology, Ospedale GB Morgagni, Forlı`, and 4Dept of Anatomic Pathology, Universita` di Verona, Verona, Italy. Correspondence: V. Poletti, Diseases of the Thorax, Ospedale GB Morgagni, via Forlanini 34, Forlı` 47100, Italy. E-mail: [email protected] @ERSpublications Lymphoproliferative disorders of the lung can be reactive lesions, malignant diseases or post- transplant disorders http://ow.ly/onfxh Lymphoproliferative disorders are rarely observed as primary lesions in the lung, representing only 0.3% of all primary pulmonary malignancies, ,1% of all the cases of non-Hodgkin lymphoma and 3–4% of all the extra nodal manifestations of non-Hodgkin lymphoma. The earliest comprehensive studies of pulmonary lymphomas were those of SALTZSTEIN [1] and PAPIOANNOU and WATSON [2], which were published in the 1960s. These investigators made important observations: low-grade neoplasms were more frequent than higher grade lymphomas (reticulum cell sarcomas) and both tumours had better clinical outcomes than their nodal-based counterparts. The authors also concluded that the majority of neoplasms with a low-grade cytological appearance should be considered as reactive proliferations, introducing the term ‘‘pseudo- lymphoma’’. This hypothesis showed significant weak points and, approximately 20 years later, ADDIS et al. [3] concluded that ‘‘most if not all the cases of pseudo-lymphoma can be classified, when re-evaluated, as malignant lymphoma’’.