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  1. Article ; Online: Anaplasia in Wilms tumor: A critical review.

    Vujanić, Gordan M / Mifsud, William

    Pediatric blood & cancer

    2024  , Page(s) e31000

    Abstract: Anaplasia in Wilms tumor is recognized as the most important prognostically unfavorable histological feature. It is subtyped as focal anaplastic Wilms tumor (FAWT) and diffuse anaplastic Wilms tumor (DAWT). Outcomes of patients with DAWT remain poor in ... ...

    Abstract Anaplasia in Wilms tumor is recognized as the most important prognostically unfavorable histological feature. It is subtyped as focal anaplastic Wilms tumor (FAWT) and diffuse anaplastic Wilms tumor (DAWT). Outcomes of patients with DAWT remain poor in patients with stage III and IV tumors. Important issues relevant to anaplasia in Wilms tumor, including prevalence, treatment, outcomes, biomarkers, anaplasia, and chemotherapy, and the concept of tumor aggressiveness, are reviewed and discussed here. We also consider the differences in clinical approaches to anaplasia in Wilms tumor between the two major renal tumor clinical research groups: the International Society of Paediatric Oncology (SIOP) Renal Tumour Study Group and the Children's Oncology Group (COG) Renal Tumor Group. We emphasize the importance and implications of recognizing FAWT and DAWT as separate clinico-pathological entities.
    Language English
    Publishing date 2024-04-11
    Publishing country United States
    Document type Journal Article ; Review
    ZDB-ID 2131448-2
    ISSN 1545-5017 ; 1545-5009
    ISSN (online) 1545-5017
    ISSN 1545-5009
    DOI 10.1002/pbc.31000
    Database MEDical Literature Analysis and Retrieval System OnLINE

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  2. Article ; Online: Renal tumours of childhood

    Vujanić Gordan M. / Đuričić Slaviša M.

    Scripta Medica, Vol 53, Iss 4, Pp 337-

    A review

    2022  Volume 345

    Abstract: Renal tumours of childhood are rare, although they are one of the most common solid tumours in children. They include numerous entities, which have different clinical, histological, molecular biological and prognostic features, so their precise diagnosis ...

    Abstract Renal tumours of childhood are rare, although they are one of the most common solid tumours in children. They include numerous entities, which have different clinical, histological, molecular biological and prognostic features, so their precise diagnosis and staging are critical for appropriate treatment. The most common is Wilms' tumour (WT) with ~80-85 % of all cases, whereas other entities including mesoblastic nephroma, clear cell sarcoma, rhabdoid tumour, renal cell carcinoma, metanephric tumours and others are very rare (2-4 % each) which explains why they represent a big diagnostic challenge for diagnostic pathologists. They are subclassified into three risk groups - low, intermediate and high - which have different treatments and prognosis. There are two big study groups which have different approaches but remarkable similar outcomes. The International Society of Paediatric Oncology approach (followed in most of the world) is based on preoperative chemotherapy, followed by surgery and further therapy, whereas the Children's Oncology Group approach (followed mainly in the United States and Canada) is based on primary surgery, followed by postoperative treatment.
    Keywords renal tumours ; wilms' tumour ; prognostic groups ; clinico-pathological features ; Medicine ; R
    Subject code 616
    Language English
    Publishing date 2022-01-01T00:00:00Z
    Publisher Medical Society of the Republic of Srpska, Banja Luka, University of Banja Luka. Faculty of Medicine
    Document type Article ; Online
    Database BASE - Bielefeld Academic Search Engine (life sciences selection)

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  3. Article ; Online: Omission of adjuvant chemotherapy in patients with completely necrotic Wilms tumor stage I and radiotherapy in stage III: The 30-year SIOP-RTSG experience.

    Vujanić, Gordan M / Graf, Norbert / D'Hooghe, Ellen / Pritchard-Jones, Kathy / Bergeron, Christophe / Tinteren, Harm van / Furtwängler, Rhoikos

    Pediatric blood & cancer

    2024  Volume 71, Issue 3, Page(s) e30852

    Abstract: Background: Completely necrotic Wilms tumor (CN-WT) following preoperative chemotherapy has been regarded as low-risk WT since the International Society of Paediatric Oncology (SIOP) 93-01 study, and patients have been treated with reduced postoperative ...

    Abstract Background: Completely necrotic Wilms tumor (CN-WT) following preoperative chemotherapy has been regarded as low-risk WT since the International Society of Paediatric Oncology (SIOP) 93-01 study, and patients have been treated with reduced postoperative therapy. The aim of the study was to evaluate whether the omission of adjuvant chemotherapy in patients with localized CN-WT stage I and radiotherapy in stage III was safe.
    Patients and methods: The retrospective observational study of outcomes of patients diagnosed with localized CN-WT on central pathology review and treated according to the SIOP 93-01 and SIOP-WT-2001 protocols (1993-2022).
    Results: There were 125 patients with localized CN-WT: 90 with stage I, 10 with stage II, and 25 with stage III. Sixty-two of 125 (49.6%) patients had a discrepant diagnosis and/or staging between the institutional pathologist and central pathology review. In the group of 90 patients with stage I, postoperative chemotherapy was not given to 41 (46%) patients, whereas 49 patients received postoperative chemotherapy-in the latter group, two patients relapsed, and one of them died. One stage I and one stage II patient developed chemotherapy-induced toxicity and died. Nineteen of 25 patients with stage III received no flank radiotherapy. No stage III patient relapsed or died. The overall 5-year event-free survival (EFS) estimate for the entire cohort (stages I-III) was 96.8% [95% confidence interval, CI: 93.6%-99.6%] and the overall survival (OS) was 97.6% [95% CI: 95.0-100%]. The EFS and OS were 97% and 98%, respectively, for stage I, and 100% for stage III.
    Conclusion: Omission of postoperative chemotherapy for patients with CN-WT stage I, and radiotherapy for stage III is safe. Rapid central pathology review is required to assign appropriate treatment and avoid treatment-related side effects.
    MeSH term(s) Child ; Humans ; Infant ; Antineoplastic Combined Chemotherapy Protocols/therapeutic use ; Chemotherapy, Adjuvant/methods ; Kidney Neoplasms/drug therapy ; Kidney Neoplasms/pathology ; Neoplasm Staging ; Treatment Outcome ; Wilms Tumor/drug therapy ; Wilms Tumor/radiotherapy ; Retrospective Studies
    Language English
    Publishing date 2024-01-07
    Publishing country United States
    Document type Observational Study ; Journal Article
    ZDB-ID 2131448-2
    ISSN 1545-5017 ; 1545-5009
    ISSN (online) 1545-5017
    ISSN 1545-5009
    DOI 10.1002/pbc.30852
    Database MEDical Literature Analysis and Retrieval System OnLINE

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  4. Article ; Online: Dataset for the reporting of nephrectomy specimens for Wilms' tumour treated with preoperative chemotherapy: recommendations from the International Society of Paediatric Oncology Renal Tumour Study Group.

    Vujanić, Gordan M / D'Hooghe, Ellen / Vokuhl, Christian / Collini, Paola

    Histopathology

    2021  Volume 79, Issue 5, Page(s) 678–686

    Abstract: Datasets for histopathological reporting of many cancer types are developed by the International Collaboration on Cancer Reporting (ICCR), and are used in order to ensure standardised and uniformly accepted reporting as one of the essential requirements ... ...

    Abstract Datasets for histopathological reporting of many cancer types are developed by the International Collaboration on Cancer Reporting (ICCR), and are used in order to ensure standardised and uniformly accepted reporting as one of the essential requirements for comparison across patient populations in evaluating and validating pathological prognostic and predictive factors. Wilms' tumours are rare, and international reporting guidelines have not yet been published by the ICCR. The assessment of Wilms' tumours differs according to the treatment approach. The Children's Oncology Group, whose approach is followed mainly in North America, advocates primary surgery, and the International Society of Paediatric Oncology Renal Tumour Study Group (SIOP-RTSG), whose approach is followed in most countries around the world, uses preoperative chemotherapy as a first step, resulting in different subclassifications, staging criteria, and histopathological prognostic factors. This dataset is developed for the countries and institutions following the SIOP-RTSG approach, and it contains core (required) and non-core (recommended) elements, based on the results of the previous SIOP-RTSG studies, which are incorporated in the latest SIOP-RTSG UMBRELLA 2016 Study protocol. The core elements include clinical information, additional specimen submitted, macroscopic tumour site and appearance, tumour focality, tumour dimensions, macroscopic extent of invasion, block identification key, histological tumour type, histological tumour grade and risk group assessment, microscopic extent of invasion, lymphovascular invasion, resection margin status, regional lymph node status, histologically confirmed distant metastases, and pathological staging (SIOP staging system). The dataset should improve communication for patient care and prognostic determination of the old and new histopathological features.
    MeSH term(s) Datasets as Topic ; Drug Therapy ; Humans ; Kidney Neoplasms/pathology ; Medical Oncology/standards ; Neoplasm Grading/methods ; Neoplasm Staging/methods ; Nephrectomy ; North America ; Prognosis ; Risk Factors ; Wilms Tumor/classification ; Wilms Tumor/pathology
    Language English
    Publishing date 2021-07-08
    Publishing country England
    Document type Journal Article ; Review
    ZDB-ID 131914-0
    ISSN 1365-2559 ; 0309-0167
    ISSN (online) 1365-2559
    ISSN 0309-0167
    DOI 10.1111/his.14394
    Database MEDical Literature Analysis and Retrieval System OnLINE

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  5. Article ; Online: Characteristics and outcomes of preoperatively treated patients with anaplastic Wilms tumors registered in the UK SIOP-WT-2001 and IMPORT study cohorts (2002-2020).

    Vujanić, Gordan M / Mifsud, William / Chowdhury, Tanzina / Al-Saadi, Reem / Pritchard-Jones, Kathy

    Cancer

    2022  Volume 128, Issue 8, Page(s) 1666–1675

    Abstract: Background: Since the International Society of Paediatric Oncology Wilms' Tumour 2001 (SIOP-WT-2001) study, focal anaplastic Wilms tumors (FAWTs) have been treated as intermediate-risk Wilms tumors (WTs), and diffuse anaplastic Wilms tumors (DAWTs) have ...

    Abstract Background: Since the International Society of Paediatric Oncology Wilms' Tumour 2001 (SIOP-WT-2001) study, focal anaplastic Wilms tumors (FAWTs) have been treated as intermediate-risk Wilms tumors (WTs), and diffuse anaplastic Wilms tumors (DAWTs) have been treated as high-risk tumors.
    Methods: The authors performed a retrospective analysis of preoperatively treated patients with FAWT or DAWT recruited in 2 consecutive UK Children's Cancer and Leukaemia Group WT studies.
    Results: One hundred twenty-one of 1237 patients (10%) had an anaplastic WT confirmed by central pathology review (CPR): 93 (77%) had DAWT, and 28 (23%) had FAWT. The 4-year event-free survival (EFS) was 51% (95% confidence interval [CI], 41%-63%) for DAWT, 88% (95% CI, 76%-100%) for FAWT, and 84% (95% CI, 82%-87%) for intermediate-risk nonanaplastic Wilms tumor (IR-non-AWT). Overall survival (OS) was 58% (95% CI, 48%-70%) for DAWT, 95% (95% CI, 86%-100%) for FAWT, and 95% (95% CI, 93%-96%) for IR-non-AWT. In a multivariate analysis, the presence of DAWT was a significant prognostic factor for both EFS and OS in stages II, III, and IV. In a multivariate analysis of unilateral DAWT, stages III and IV remained the only significant prognostic factors for both EFS and OS. In 28% of the cases, there were discrepancies affecting the recognition of anaplasia, classification (DAWT vs FAWT), or the local pathologic stage.
    Conclusions: Preoperatively treated patients with FAWT had excellent outcomes in comparison with those with identically treated IR-non-AWT, whereas patients with DAWT showed significantly worse outcomes. All patients with stage I disease had comparable good outcomes, regardless of the presence/absence of anaplasia. In contrast, the presence of DAWT was associated with significantly worse outcomes for patients with stage II to V disease. Finally, significant diagnostic discrepancies emphasize the value of CPR.
    Lay summary: Anaplasia is an unfavorable feature in Wilms tumor (WT), and it is classified as focal (focal anaplastic Wilms tumor [FAWT]) or diffuse (diffuse anaplastic Wilms tumor [DAWT]). This study reports the outcomes of patients with FAWT and DAWT who were, for the first time, treated differently. Patients with FAWT received less intensive treatment, and their outcomes were comparable to the outcomes of patients with identically treated nonanaplastic WT. Patients with stage I DAWT also had good outcomes when they were treated without radiotherapy, whereas patients with stage II to V DAWT had poor outcomes despite more intensive treatment.
    MeSH term(s) Child ; Humans ; Infant ; Kidney Neoplasms/pathology ; Neoplasm Staging ; Prognosis ; Retrospective Studies ; United Kingdom/epidemiology ; Wilms Tumor/pathology
    Language English
    Publishing date 2022-02-04
    Publishing country United States
    Document type Journal Article ; Research Support, Non-U.S. Gov't
    ZDB-ID 1429-1
    ISSN 1097-0142 ; 0008-543X ; 1934-662X
    ISSN (online) 1097-0142
    ISSN 0008-543X ; 1934-662X
    DOI 10.1002/cncr.34107
    Database MEDical Literature Analysis and Retrieval System OnLINE

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  6. Article ; Online: Stage I epithelial or stromal type Wilms tumors are low risk tumors: An analysis of patients treated on the SIOP-WT-2001 protocol in the UK-CCLG and GPOH studies (2001-2020).

    D'Hooghe, Ellen / Furtwängler, Rhoikos / Chowdhury, Tanzina / Vokuhl, Christian / Al-Saadi, Reem / Pritchard-Jones, Kathy / Graf, Norbert / Vujanić, Gordan M

    Cancer

    2023  Volume 129, Issue 12, Page(s) 1930–1938

    Abstract: Background: Patients treated with preoperative chemotherapy with stage I intermediate-risk Wilms tumor (IR-WT) represent the largest group of patients with Wilms tumor (WT), and they have excellent outcomes.: Methods: The authors performed a ... ...

    Abstract Background: Patients treated with preoperative chemotherapy with stage I intermediate-risk Wilms tumor (IR-WT) represent the largest group of patients with Wilms tumor (WT), and they have excellent outcomes.
    Methods: The authors performed a retrospective analysis of patients with stage I epithelial (ET-WT) or stromal type WT (ST-WT) treated pre- and postoperatively according to the International Society of Paediatric Oncology-WT-2001 protocol in the UK Children's Cancer and Leukaemia Group and Gesellschaft für Pädiatrische Onkologie und Hämatologie groups' participation in the relevant WT trials and studies (2001-2020).
    Results: There were 880 patients with stage I IR-WT, including 124 with ET-WT, 156 with ST-WT, and 600 with other IR-WT (oIR-WT). Patients with stage I ET-WT or ST-WT were significantly younger than patients with oIR-WT, represented a large proportion of stage I WTs in their groups, and tumors showed poor histologic response to preoperative chemotherapy. The 5-year event-free survival (EFS) estimates for patients with stage I ET-WT (96.8% ± 1.8 SE) or ST-WT (96.8% ± 1.6 SE) were significantly better than for patients with oIR-WT (90.3% ± 1.3 SE) (p = .014 and p = .009, respectively). A multivariate analysis showed that histologic type (ET-WT or ST-WT) remained a significant factor for EFS when adjusted for age and gender (p = .032 and p = .022, respectively). In both groups, relapses occurred in 3.2% of patients, and the overall survival was 99.2%.
    Conclusions: The results suggest that stage I ET-WT or ST-WT could be regarded as low-risk WT, for which omission of postoperative chemotherapy should be considered.
    Plain language summary: Patients with pretreated intermediate-risk Wilms tumor (WT) represent the largest group of patients with WT. This study reports the outcomes of patients with stage I epithelial type (ET-WT) or stromal type WT (ST-WT). These patients were significantly younger and had a larger proportion of stage I cases than patients with other intermediate-risk WT (oIR-WT). The event-free survival for patients with stage I ET-WT and ST-WT was significantly better than for patients with oIR-WT. Rare relapses were curable resulting in 99.2% overall survival.
    MeSH term(s) Child ; Humans ; Infant ; Kidney Neoplasms/drug therapy ; Kidney Neoplasms/surgery ; Kidney Neoplasms/pathology ; Retrospective Studies ; Neoplasm Staging ; Neoplasm Recurrence, Local/pathology ; Wilms Tumor/drug therapy ; Wilms Tumor/surgery ; Antineoplastic Combined Chemotherapy Protocols/therapeutic use ; United Kingdom/epidemiology
    Language English
    Publishing date 2023-03-17
    Publishing country United States
    Document type Journal Article ; Research Support, Non-U.S. Gov't
    ZDB-ID 1429-1
    ISSN 1097-0142 ; 0008-543X ; 1934-662X
    ISSN (online) 1097-0142
    ISSN 0008-543X ; 1934-662X
    DOI 10.1002/cncr.34734
    Database MEDical Literature Analysis and Retrieval System OnLINE

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  7. Article ; Online: "Teratoid" Wilms Tumor: The Extreme End of Heterologous Element Differentiation, Not a Separate Entity.

    D'Hooghe, Ellen / Mifsud, William / Vujanić, Gordan M

    The American journal of surgical pathology

    2019  Volume 43, Issue 11, Page(s) 1583–1590

    Abstract: Wilms tumor (WT) may show a diverse range of heterologous elements (HEs). Cases with predominant/prominent HEs have been reported as "teratoid" WT, albeit on the basis of poorly defined criteria. It has been suggested that "teratoid" WTs are rare, and ... ...

    Abstract Wilms tumor (WT) may show a diverse range of heterologous elements (HEs). Cases with predominant/prominent HEs have been reported as "teratoid" WT, albeit on the basis of poorly defined criteria. It has been suggested that "teratoid" WTs are rare, and associated with a poor response to chemotherapy, but a good outcome. However, these claims have not been tested previously in any large cohort of cases. Here, we performed a systematic study to determine the incidence, diversity, and clinicopathologic association of HEs in 691 WTs, all of which were treated according to the same protocol, which included preoperative chemotherapy, and all with central pathology review. We found that 4% (28/691) of WTs showed ≥3 HEs ("teratoid" WT in our study), which was comparable to the numbers of completely necrotic, epithelial, focal anaplastic, and blastemal WTs. "Teratoid" WTs were strongly associated with younger age at presentation (21 vs. 39 mo, P=0.0001), bilateral disease (28.6% vs. 7.2%, P=0.001), stromal-type WT (57.1% vs. 11.0%, P<0.00001), and intralobar nephrogenic rests (35.7% vs. 11.9%, P=0.0001), when compared with non-"teratoid" WT. We also found that stromal-type WT, regardless of HE differentiation, was itself associated with younger age, bilateral disease, and intralobar nephrogenic rest. Furthermore, >80% of cases with ≥3 HEs, and also of cases with 2 HEs and 1 HE, showed ≥50% stroma in their viable components. We conclude that a tendency toward stromal differentiation is a strong and unifying factor in HE formation. "Teratoid" WT represents the more extreme end of HE differentiation, rather than a separate entity, and therefore the term should not be used in the final diagnosis. The prognosis of WTs depends only on their overall histologic type and stage, and it is not additionally influenced by the presence of "teratoid" features.
    MeSH term(s) Adolescent ; Adult ; Cell Differentiation ; Chemotherapy, Adjuvant ; Child ; Child, Preschool ; Female ; Follow-Up Studies ; Humans ; Infant ; Kidney Neoplasms/diagnosis ; Kidney Neoplasms/mortality ; Kidney Neoplasms/pathology ; Kidney Neoplasms/therapy ; Male ; Neoadjuvant Therapy ; Neoplasm Staging ; Nephrectomy ; Prognosis ; Retrospective Studies ; Survival Analysis ; Teratoma/diagnosis ; Teratoma/mortality ; Teratoma/pathology ; Teratoma/therapy ; Wilms Tumor/diagnosis ; Wilms Tumor/mortality ; Wilms Tumor/pathology ; Wilms Tumor/therapy ; Young Adult
    Language English
    Publishing date 2019-10-10
    Publishing country United States
    Document type Journal Article
    ZDB-ID 752964-8
    ISSN 1532-0979 ; 0147-5185
    ISSN (online) 1532-0979
    ISSN 0147-5185
    DOI 10.1097/PAS.0000000000001335
    Database MEDical Literature Analysis and Retrieval System OnLINE

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  8. Article: In Memoriam.

    Vujanic, Gordan M / Alaggio, Rita

    Pediatric and developmental pathology : the official journal of the Society for Pediatric Pathology and the Paediatric Pathology Society

    2017  Volume 20, Issue 2, Page(s) 103–104

    Language English
    Publishing date 2017-03
    Publishing country United States
    Document type Journal Article
    ZDB-ID 1463498-3
    ISSN 1615-5742 ; 1093-5266
    ISSN (online) 1615-5742
    ISSN 1093-5266
    DOI 10.1177/1093526617700554
    Database MEDical Literature Analysis and Retrieval System OnLINE

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  9. Article ; Online: Comment on: "Indications and results of diagnostic biopsy in pediatric renal tumors: A retrospective analysis of 317 patients with critical review of SIOP guidelines".

    Jackson, Thomas J / Vujanic, Gordan M / Pritchard-Jones, Kathy

    Pediatric blood & cancer

    2019  Volume 66, Issue 7, Page(s) e27746

    MeSH term(s) Biopsy ; Child ; Humans ; Kidney Neoplasms ; Retrospective Studies ; Wilms Tumor
    Language English
    Publishing date 2019-04-02
    Publishing country United States
    Document type Letter ; Comment
    ZDB-ID 2131448-2
    ISSN 1545-5017 ; 1545-5009
    ISSN (online) 1545-5017
    ISSN 1545-5009
    DOI 10.1002/pbc.27746
    Database MEDical Literature Analysis and Retrieval System OnLINE

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  10. Article ; Online: Pathology of Wilms' tumour in International Society of Paediatric Oncology (SIOP) and Children's oncology group (COG) renal tumour studies: Similarities and differences.

    Vujanić, Gordan M / Parsons, Lauren N / D'Hooghe, Ellen / Treece, Amy L / Collini, Paola / Perlman, Elizabeth J

    Histopathology

    2022  Volume 80, Issue 7, Page(s) 1026–1037

    Abstract: Excellent outcomes for patients with Wilms' tumour (WT), >90% for all stages together, have been achieved through researching WT in multicentre and multinational trials and studies in the last 50 years, led by two major groups-the International Society ... ...

    Abstract Excellent outcomes for patients with Wilms' tumour (WT), >90% for all stages together, have been achieved through researching WT in multicentre and multinational trials and studies in the last 50 years, led by two major groups-the International Society of Paediatric Oncology (SIOP) and the Children's Oncology Group (COG) (previously the National Wilms' Tumour Study Group). Despite the two groups having different approaches, the survival outcomes are remarkably similar. In general, in the SIOP approach, which is followed in Europe and most other countries around the world, patients are first treated with preoperative chemotherapy; this is followed by surgery and, if necessary, postoperative chemotherapy and radiotherapy. In the COG approach, which is mainly followed in North America, patients are treated with upfront surgery, followed, if necessary, by postoperative chemotherapy and radiotherapy. In both groups, postoperative treatment primarily depends on tumour histological classification and stage, although, in recent studies, other prognostic factors have also been included (tumour volume, response to preoperative chemotherapy, and molecular markers). Owing to separate initial treatments, there are differences in histological assessment and subtyping of WT, and, more importantly, in staging criteria. In this review, we discuss the similarities and differences between the two groups in order to help pathologists who are dealing with WT to understand and follow the pathological protocol that is appropriate for a particular case, because, in many centres, both approaches may be followed, depending on individual case/patient circumstances.
    MeSH term(s) Child ; Europe ; Humans ; Kidney Neoplasms/pathology ; Medical Oncology ; Neoplasm Staging ; North America ; Wilms Tumor/therapy
    Language English
    Publishing date 2022-03-24
    Publishing country England
    Document type Journal Article ; Review
    ZDB-ID 131914-0
    ISSN 1365-2559 ; 0309-0167
    ISSN (online) 1365-2559
    ISSN 0309-0167
    DOI 10.1111/his.14632
    Database MEDical Literature Analysis and Retrieval System OnLINE

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