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  1. Article: Proton Therapy in Non-Rhabdomyosarcoma Soft Tissue Sarcomas of Children and Adolescents.

    Vennarini, Sabina / Colombo, Francesca / Mirandola, Alfredo / Orlandi, Ester / Pecori, Emilia / Chiaravalli, Stefano / Massimino, Maura / Casanova, Michela / Ferrari, Andrea

    Cancers

    2024  Volume 16, Issue 9

    Abstract: This paper provides insights into the use of Proton Beam Therapy (PBT) in pediatric patients with non-rhabdomyosarcoma soft tissue sarcomas (NRSTS). NRSTS are a heterogeneous group of rare and aggressive mesenchymal extraskeletal tumors, presenting ... ...

    Abstract This paper provides insights into the use of Proton Beam Therapy (PBT) in pediatric patients with non-rhabdomyosarcoma soft tissue sarcomas (NRSTS). NRSTS are a heterogeneous group of rare and aggressive mesenchymal extraskeletal tumors, presenting complex and challenging clinical management scenarios. The overall survival rate for patients with NRSTS is around 70%, but the outcome is strictly related to the presence of various variables, such as the histological subtype, grade of malignancy and tumor stage at diagnosis. Multimodal therapy is typically considered the preferred treatment for high-grade NRSTS. Radiotherapy plays a key role in the treatment of children and adolescents with NRSTS. However, the potential for radiation-induced side effects partially limits its use. Therefore, PBT represents a very suitable therapeutic option for these patients. The unique depth-dose characteristics of protons can be leveraged to minimize doses to healthy tissue significantly, potentially allowing for increased tumor doses and enhanced preservation of surrounding tissues. These benefits suggest that PBT may improve local control while reducing toxicity and improving quality of life. While clear evidence of therapeutic superiority of PBT over other modern photon techniques in NRSTS is still lacking-partly due to the limited data available-PBT can be an excellent treatment option for young patients with these tumors. A dedicated international comprehensive collaborative approach is essential to better define its role within the multidisciplinary management of NRSTS. Shared guidelines for PBT indications-based on the patient's age, estimated outcome, and tumor location-and centralization in high-level referral centers are needed to optimize the use of resources, since access to PBT remains a challenge due to the limited number of available proton therapy facilities.
    Language English
    Publishing date 2024-04-26
    Publishing country Switzerland
    Document type Journal Article ; Review
    ZDB-ID 2527080-1
    ISSN 2072-6694
    ISSN 2072-6694
    DOI 10.3390/cancers16091694
    Database MEDical Literature Analysis and Retrieval System OnLINE

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  2. Article ; Online: Management and pharmacotherapy of pediatric colorectal carcinoma: a review.

    Bergamaschi, Luca / Chiaravalli, Stefano / Signoroni, Stefano / Di Bartolomeo, Maria / Ferrari, Andrea

    Expert opinion on pharmacotherapy

    2023  Volume 24, Issue 14, Page(s) 1527–1535

    Abstract: Introduction: Colorectal carcinoma (CRC) is one of the most common tumors in adult, but is extremely rare in children. In childhood, CRC often presents unfavorable aggressive histotypes, advanced clinical stage at onset and a worse prognosis. Pediatric ... ...

    Abstract Introduction: Colorectal carcinoma (CRC) is one of the most common tumors in adult, but is extremely rare in children. In childhood, CRC often presents unfavorable aggressive histotypes, advanced clinical stage at onset and a worse prognosis. Pediatric CRC series are limited and include few patients, therefore information about treatment strategy and pharmacotherapy is scarce. For this reason, management of these patients represents a real challenge for pediatric oncologists.
    Areas covered: The authors provide an overview of the general features and management strategies of pediatric CRC with specific attention to systemic treatment. Literature data regarding pharmacotherapy in published pediatric series are summarized and analyzed in detail, according to adult treatment standards.
    Expert opinion: In the absence of specific recommendations for pediatric CRC, the general therapeutic strategy should follow the same principles as for adults and should be the result of a multidisciplinary discussion. Patient access to optimal treatment is difficult due to the lack of new drugs approved for the pediatric age group and non-availability of clinical trials. Collaboration between pediatric and adult oncologists is considered crucial in order to overcome these issues and find solutions to increase knowledge and improve the outcome of such a rare disease in children.
    MeSH term(s) Adult ; Humans ; Child ; Colorectal Neoplasms/drug therapy ; Colorectal Neoplasms/pathology ; Prognosis
    Language English
    Publishing date 2023-06-29
    Publishing country England
    Document type Review ; Journal Article
    ZDB-ID 2001535-5
    ISSN 1744-7666 ; 1465-6566
    ISSN (online) 1744-7666
    ISSN 1465-6566
    DOI 10.1080/14656566.2023.2230123
    Database MEDical Literature Analysis and Retrieval System OnLINE

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  3. Article: Clinical Insight on Proton Therapy for Paediatric Rhabdomyosarcoma.

    Vennarini, Sabina / Colombo, Francesca / Mirandola, Alfredo / Chiaravalli, Stefano / Orlandi, Ester / Massimino, Maura / Casanova, Michela / Ferrari, Andrea

    Cancer management and research

    2023  Volume 15, Page(s) 1125–1139

    Abstract: This paper offers an insight into the use of Proton Beam Therapy (PBT) in paediatric patients with rhabdomyosarcoma (RMS). This paper provides a comprehensive analysis of the literature, investigating comparative photon-proton dosimetry, outcome, and ... ...

    Abstract This paper offers an insight into the use of Proton Beam Therapy (PBT) in paediatric patients with rhabdomyosarcoma (RMS). This paper provides a comprehensive analysis of the literature, investigating comparative photon-proton dosimetry, outcome, and toxicity. In the complex and multimodal scenario of the treatment of RMS, clear evidence of the therapeutic superiority of PBT compared to other modern photon techniques has not yet been demonstrated; however, PBT can be considered an excellent treatment option, in particular for young children and patients with specific primary sites, such as the head and neck area (and especially the parameningeal regions), genito-urinary, pelvic, and paravertebral regions. The unique depth-dose characteristics of protons can be exploited to achieve significant reductions in normal tissue doses and may allow an escalation of tumour doses and greater sparing of normal tissues, thus potentially improving local control while at the same time reducing toxicity and improving quality of life. However, access of children with RMS (and more in general with solid tumors) to PBT remains a challenge, due to the limited number of available proton therapy installations.
    Language English
    Publishing date 2023-10-10
    Publishing country New Zealand
    Document type Journal Article ; Review
    ZDB-ID 2508013-1
    ISSN 1179-1322
    ISSN 1179-1322
    DOI 10.2147/CMAR.S362664
    Database MEDical Literature Analysis and Retrieval System OnLINE

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  4. Article ; Online: Palliative sedation in paediatric solid tumour patients: choosing the best drugs.

    Podda, Marta Giorgia / Schiavello, Elisabetta / Nigro, Olga / Clerici, Carlo Alfredo / Simonetti, Fabio / Luksch, Roberto / Terenziani, Monica / Ferrari, Andrea / Casanova, Michela / Spreafico, Filippo / Meazza, Cristina / Chiaravalli, Stefano / Biassoni, Veronica / Gattuso, Giovanna / Puma, Nadia / Bergamaschi, Luca / Sironi, Givanna / Massimino, Maura

    BMJ supportive & palliative care

    2024  Volume 13, Issue e3, Page(s) e1141–e1148

    Abstract: Objectives: Cancer remains the leading cause of mortality by disease in childhood in high-income countries. For terminally ill children, care focuses on quality of life, and patient management fundamentally affects grieving families. This paper ... ...

    Abstract Objectives: Cancer remains the leading cause of mortality by disease in childhood in high-income countries. For terminally ill children, care focuses on quality of life, and patient management fundamentally affects grieving families. This paper describes our experience of palliative sedation (PS) for children with refractory symptoms caused by solid tumours, focusing on the drugs involved.
    Methods: We retrospectively collected data on all children treated for cancer who died at the pediatric oncology unit of the Fondazione IRCCS Istituto Nazionale dei Tumori between January 2016 and December 2020.
    Results: Of the 29 patients eligible for the study, all but 4 received PS. Midazolam was always used, combined in 16 cases with other drugs (mainly classic neuroleptics, alpha-2 agonists and antihistamines). Throughout the period of PS and on the day of death, patients with sarcoma were given higher doses of midazolam and morphine, and more often received combinations of drugs than patients with brain tumours. Sarcoma causes significant symptoms, while brain tumours require less intensive analgesic-sedative therapies because they already impair a patient's state of consciousness.
    Conclusions: Optimising pharmacological treatments demands a medical team that knows how drugs (often developed for other indications) work. Emotional and relational aspects are important too, and any action to lower a patient's consciousness should be explained to the family and justified. Parents should not feel like helpless witnesses. Guidelines on PS in paediatrics could help, providing they acknowledge that a child's death is always a unique case.
    MeSH term(s) Child ; Humans ; Brain Neoplasms ; Hypnotics and Sedatives/therapeutic use ; Midazolam/therapeutic use ; Neoplasms/drug therapy ; Palliative Care ; Quality of Life ; Retrospective Studies ; Sarcoma ; Terminal Care
    Chemical Substances Hypnotics and Sedatives ; Midazolam (R60L0SM5BC)
    Language English
    Publishing date 2024-01-08
    Publishing country England
    Document type Journal Article
    ISSN 2045-4368
    ISSN (online) 2045-4368
    DOI 10.1136/bmjspcare-2022-003534
    Database MEDical Literature Analysis and Retrieval System OnLINE

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  5. Article ; Online: Local treatment in initially unresected non-rhabdomyosarcoma soft-tissue sarcomas of children and adolescents: A retrospective single-center experience.

    Ferrari, Andrea / Vennarini, Sabina / Fiore, Marco / Bergamaschi, Luca / Chiaravalli, Stefano / Morosi, Carlo / Colombo, Chiara / Pecori, Emilia / Puma, Nadia / Luksch, Roberto / Terenziani, Monica / Spreafico, Filippo / Meazza, Cristina / Podda, Marta / Biassoni, Veronica / Schiavello, Elisabetta / Massimino, Maura / Casanova, Michela

    Pediatric blood & cancer

    2024  Volume 71, Issue 4, Page(s) e30901

    Abstract: Background: Pediatric non-rhabdomyosarcoma soft-tissue sarcomas (NRSTS) are a heterogeneous group of aggressive tumors. Patients with locally advanced/initially unresected disease represent a subset of patients with unsatisfactory outcome: limited data ... ...

    Abstract Background: Pediatric non-rhabdomyosarcoma soft-tissue sarcomas (NRSTS) are a heterogeneous group of aggressive tumors. Patients with locally advanced/initially unresected disease represent a subset of patients with unsatisfactory outcome: limited data are available on the best treatment approach, in particular regarding local therapy.
    Methods: This retrospective analysis concerned 71 patients < 21 years old with nonmetastatic, initially unresected adult-type NRSTS, treated at a referral center for pediatric sarcomas from 1990 to 2021. Patients were treated using a multimodal approach, based on the protocols adopted at the time of their diagnosis.
    Results: The series included a selected group of patients with unfavorable clinical characteristics, i.e., most cases had high-grade and large tumors, arising from axial sites in 61% of cases. All patients received neoadjuvant chemotherapy, 58 (82%) had delayed surgery (R0 in 45 cases), and 50 (70%) had radiotherapy. Partial response to chemotherapy was observed in 46% of cases. With a median follow-up of 152 months (range, 18-233), 5-year event-free survival (EFS) and overall survival (OS) were 39.9% and 56.5%, respectively. Survival was significantly better for patients who responded to chemotherapy, and those who had a delayed R0 resection. Local relapse at 5 years was 7.7% for patients who did not undergo delayed surgery.
    Conclusions: Our series underscores the unsatisfactory outcome of initially unresected NRSTS patients. Improving the outcome of this patient category requires therapeutic strategies able to combine novel effective systemic therapies with a better-defined local treatment approach to offer patients the best chances to have R0 surgery.
    MeSH term(s) Child ; Adult ; Humans ; Adolescent ; Young Adult ; Retrospective Studies ; Antineoplastic Combined Chemotherapy Protocols/therapeutic use ; Neoplasm Recurrence, Local/drug therapy ; Sarcoma/pathology ; Soft Tissue Neoplasms/pathology ; Rhabdomyosarcoma/drug therapy
    Language English
    Publishing date 2024-01-31
    Publishing country United States
    Document type Journal Article
    ZDB-ID 2131448-2
    ISSN 1545-5017 ; 1545-5009
    ISSN (online) 1545-5017
    ISSN 1545-5009
    DOI 10.1002/pbc.30901
    Database MEDical Literature Analysis and Retrieval System OnLINE

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  6. Article ; Online: Treatment at relapse for synovial sarcoma of children and adolescents: A multi-institutional European retrospective analysis.

    Ferrari, Andrea / Orbach, Daniel / Bergamaschi, Luca / Schoot, Reineke A / van Noesel, Max M / Di Carlo, Daniela / Bisogno, Gianni / Alaggio, Rita / Milano, Giuseppe Maria / Chiaravalli, Stefano / Fuccillo, Fernando / Laurence, Valerie / Corradini, Nadege / Gasparini, Patrizia / Vennarini, Sabina / Pasquali, Sandro / Casanova, Michela

    Pediatric blood & cancer

    2024  , Page(s) e31038

    Abstract: Purpose: Though the prognosis for pediatric patients with localised synovial sarcoma (SS) is generally good, the chances of being cured after relapse are limited. This study describes a retrospective multi-institutional series of relapsing SS patients ... ...

    Abstract Purpose: Though the prognosis for pediatric patients with localised synovial sarcoma (SS) is generally good, the chances of being cured after relapse are limited. This study describes a retrospective multi-institutional series of relapsing SS patients treated at six selected European referral centers for pediatric sarcoma.
    Patients and methods: The study included 41 patients <21 years with relapsing SS, treated between 2002 and 2022. The analysis included patient's characteristics at first diagnosis, first-line treatments, clinical findings at relapse, and second-line treatment modalities.
    Results: The first relapse occurred within 3-132 months (median 18 months) after first diagnosis and was local in 34%, metastatic in 54%, and both in 12%. Treatment at first relapse included surgery in 56% of cases, radiotherapy in 34%, and systemic therapy in 88%. In all, 36 patients received second-line medical treatment, that was chemotherapy in 32 cases (with 10 different regimens) and targeted therapy in four. No patient was included in an early-phase clinical trial as second-line therapy-line therapy. Overall response rate was 42%. Median event-free survival (EFS) was 12 months, postrelapse 5-year EFS was 15.8%. Median overall survival (OS) was 30 months, postrelapse 5-year OS was 22.2%. At the Cox's multivariable regression analysis, OS was significantly associated with time and type of relapse.
    Conclusion: Pediatric patients with relapsed SS have a poor prognosis and generally receive an individualized approach, due to the lack of a uniform standardized approach. New comprehensive strategies are needed to improve the knowledge on the biologic landscape of SS and develop tailored prospective clinical trials.
    Language English
    Publishing date 2024-04-28
    Publishing country United States
    Document type Journal Article
    ZDB-ID 2131448-2
    ISSN 1545-5017 ; 1545-5009
    ISSN (online) 1545-5017
    ISSN 1545-5009
    DOI 10.1002/pbc.31038
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  7. Article ; Online: VIVA (vinorelbine, ifosfamide, vincristine, actinomycin-D): A new regimen in the armamentarium of systemic therapy for high-risk rhabdomyosarcoma.

    Ferrari, Andrea / Chiaravalli, Stefano / Zecca, Marco / Recupero, Santina / Pascale, Silvia / Bergamaschi, Luca / Casanova, Michela

    Pediatric blood & cancer

    2020  Volume 67, Issue 11, Page(s) e28649

    Abstract: The study reports the treatment feasibility, and secondly efficacy, of a novel chemotherapy regimen, which adds vinorelbine to the ifosfamide-vincristine-actinomycin-D combination (VIVA regimen), used in four patients with high-risk rhabdomyosarcoma. All ...

    Abstract The study reports the treatment feasibility, and secondly efficacy, of a novel chemotherapy regimen, which adds vinorelbine to the ifosfamide-vincristine-actinomycin-D combination (VIVA regimen), used in four patients with high-risk rhabdomyosarcoma. All patients received nine cycles of the VIVA regimen followed by maintenance chemotherapy with vinorelbine and cyclophosphamide. All patients experienced significant hematological toxicity, but no other major complications (in particular neurotoxicity) or required treatment dose modifications. We observed a major response after three cycles in all patients, and they remained alive after a median follow up of 11 months from diagnosis.
    MeSH term(s) Adolescent ; Antineoplastic Combined Chemotherapy Protocols/therapeutic use ; Child ; Child, Preschool ; Dactinomycin/administration & dosage ; Female ; Follow-Up Studies ; Humans ; Ifosfamide/administration & dosage ; Infant ; Male ; Prognosis ; Rhabdomyosarcoma/drug therapy ; Rhabdomyosarcoma/pathology ; Risk Factors ; Vincristine/administration & dosage ; Vinorelbine/administration & dosage
    Chemical Substances Dactinomycin (1CC1JFE158) ; Vincristine (5J49Q6B70F) ; Vinorelbine (Q6C979R91Y) ; Ifosfamide (UM20QQM95Y)
    Language English
    Publishing date 2020-09-07
    Publishing country United States
    Document type Journal Article
    ZDB-ID 2131448-2
    ISSN 1545-5017 ; 1545-5009
    ISSN (online) 1545-5017
    ISSN 1545-5009
    DOI 10.1002/pbc.28649
    Database MEDical Literature Analysis and Retrieval System OnLINE

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  8. Article ; Online: Desmoplastic small round cell tumor: from state of the art to future clinical prospects.

    Hovsepyan, Shushan / Giani, Claudia / Pasquali, Sandro / Di Giannatale, Angela / Chiaravalli, Stefano / Colombo, Chiara / Orbach, Daniel / Bergamaschi, Luca / Vennarini, Sabina / Gatz, Susanne Andrea / Gasparini, Patrizia / Berlanga, Pablo / Casanova, Michela / Ferrari, Andrea

    Expert review of anticancer therapy

    2023  Volume 23, Issue 5, Page(s) 471–484

    Abstract: Introduction: Desmoplastic small round cell tumor (DSRCT) is an extremely rare and highly aggressive soft tissue sarcoma, presenting mainly in male adolescents and young adults with multiple nodules disseminated within the abdominopelvic cavity. Despite ...

    Abstract Introduction: Desmoplastic small round cell tumor (DSRCT) is an extremely rare and highly aggressive soft tissue sarcoma, presenting mainly in male adolescents and young adults with multiple nodules disseminated within the abdominopelvic cavity. Despite a multimodal approach including aggressive cytoreductive surgery, intensive multi-agent chemotherapy, and postoperative whole abdominopelvic radiotherapy, the prognosis for DSRCT remains dismal. Median progression-free survival ranges between 4 and 21 months, and overall survival between 17 and 60 months, with the 5-year overall survival rate in the range of 10-20%.
    Area covered: This review discusses the treatment strategies used for DSRCT over the years, the state of the art of current treatments, and future clinical prospects.
    Expert opinion: The unsatisfactory outcomes for patients with DSRCT warrant investigations into innovative treatment combinations. An international multidisciplinary and multi-stakeholder collaboration, involving both pediatric and adult sarcoma communities, is needed to propel preclinical model generation and drug development, and innovative clinical trial designs to enable the timely testing of treatments involving novel agents guided by biology to boost the chances of survival for patients with this devastating disease.
    MeSH term(s) Adolescent ; Young Adult ; Humans ; Child ; Male ; Combined Modality Therapy ; Peritoneal Neoplasms/drug therapy ; Desmoplastic Small Round Cell Tumor/drug therapy ; Desmoplastic Small Round Cell Tumor/pathology ; Prognosis ; Antineoplastic Combined Chemotherapy Protocols/therapeutic use ; Sarcoma/drug therapy
    Language English
    Publishing date 2023-04-10
    Publishing country England
    Document type Review ; Journal Article
    ZDB-ID 2112544-2
    ISSN 1744-8328 ; 1473-7140
    ISSN (online) 1744-8328
    ISSN 1473-7140
    DOI 10.1080/14737140.2023.2200171
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  9. Article ; Online: Multiagent chemotherapy including IrIVA regimen and maintenance therapy in the treatment of desmoplastic small round cell tumor.

    Ferrari, Andrea / Bergamaschi, Luca / Chiaravalli, Stefano / Pecori, Emilia / Diletto, Barbara / Alessandro, Ombretta / Giandini, Tommaso / Livellara, Virginia / Sironi, Giovanna / Casanova, Michela

    Tumori

    2021  Volume 108, Issue 1, Page(s) 93–97

    Abstract: This study reports the treatment feasibility and efficacy of a novel multiagent intensive treatment program for young patients with desmoplastic small round cell tumor. This small series includes three patients and should be seen as a first suggestion of ...

    Abstract This study reports the treatment feasibility and efficacy of a novel multiagent intensive treatment program for young patients with desmoplastic small round cell tumor. This small series includes three patients and should be seen as a first suggestion of integration of the dose density and the maintenance chemotherapy concept. The IrIVA regimen (irinotecan, ifosfamide, vincristine, and actinomycin-D) is added-used at a short interval between chemotherapy administrations-at more classic intensive ifosfamide-based regimens. The vinorelbine and low-dose oral cyclophosphamide maintenance therapy is added at the end of conventional chemotherapy to achieve an antiangiogenic effect.
    MeSH term(s) Adolescent ; Adult ; Antineoplastic Combined Chemotherapy Protocols/administration & dosage ; Antineoplastic Combined Chemotherapy Protocols/adverse effects ; Child ; Cyclophosphamide/administration & dosage ; Cyclophosphamide/adverse effects ; Dactinomycin/administration & dosage ; Dactinomycin/adverse effects ; Desmoplastic Small Round Cell Tumor/drug therapy ; Desmoplastic Small Round Cell Tumor/pathology ; Desmoplastic Small Round Cell Tumor/surgery ; Dose-Response Relationship, Drug ; Doxorubicin/administration & dosage ; Doxorubicin/adverse effects ; Humans ; Ifosfamide/administration & dosage ; Ifosfamide/adverse effects ; Irinotecan/administration & dosage ; Irinotecan/adverse effects ; Male ; Treatment Outcome ; Vincristine/administration & dosage ; Vincristine/adverse effects ; Vinorelbine/administration & dosage ; Vinorelbine/adverse effects ; Young Adult
    Chemical Substances Dactinomycin (1CC1JFE158) ; Vincristine (5J49Q6B70F) ; Irinotecan (7673326042) ; Doxorubicin (80168379AG) ; Cyclophosphamide (8N3DW7272P) ; Vinorelbine (Q6C979R91Y) ; Ifosfamide (UM20QQM95Y)
    Language English
    Publishing date 2021-02-17
    Publishing country United States
    Document type Journal Article
    ZDB-ID 280962-x
    ISSN 2038-2529 ; 0300-8916
    ISSN (online) 2038-2529
    ISSN 0300-8916
    DOI 10.1177/0300891621995250
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  10. Article ; Online: Gardner-associated fibroma of the neck: role of a multidisciplinary evaluation for familial adenomatous polyposis diagnosis.

    Signoroni, Stefano / Piozzi, Guglielmo Niccolò / Collini, Paola / Cocco, Ivana Maria Francesca / Biasoni, Davide / Chiaravalli, Stefano / Ricci, Maria Teresa / Vitellaro, Marco

    Tumori

    2021  Volume 107, Issue 6, Page(s) NP73–NP76

    Abstract: Introduction: Familial adenomatous polyposis (FAP) is a hereditary autosomal dominant disorder characterized by the development of multiple adenomas in the colon and rectum with a lifetime risk of 80%-100% to develop colorectal cancer if undetected or ... ...

    Abstract Introduction: Familial adenomatous polyposis (FAP) is a hereditary autosomal dominant disorder characterized by the development of multiple adenomas in the colon and rectum with a lifetime risk of 80%-100% to develop colorectal cancer if undetected or untreated. Gardner-associated fibroma (GAF) is a rare, benign soft tissue lesion with uncertain pathogenesis. GAF is generally associated with FAP in its clinical variant, called Gardner syndrome (GS).
    Case description: A 16-year-old boy with no comorbidities and no significant medical history was referred to the Unit of Hereditary Digestive Tract Tumours, Department of Surgery, Fondazione IRCCS Istituto Nazionale dei Tumori, Milan, Italy, for genetic counselling after surgical removal of a right anterior cervical paramedian fibroma. The histopathology on the specimen led to the diagnosis of GAF. He had no family history for colorectal cancer or gastrointestinal polyposis and denied any gastrointestinal symptoms. Physical examination showed a small frontal osteoma and colonoscopy showed the presence of multiple small sessile polyps (>100 polyps, diameter <5 mm) diffusely present on the large bowel. Genetic testing revealed a pathogenic germline variant in the
    Conclusions: GAF may represent a sentinel sign of FAP, preceding gastrointestinal symptoms and endoscopic findings. A careful multidisciplinary approach is determinant for correct and early diagnosis of FAP.
    MeSH term(s) Adenomatous Polyposis Coli/diagnosis ; Adenomatous Polyposis Coli/genetics ; Adolescent ; Colonoscopy ; Diagnosis, Differential ; Fibroma/diagnosis ; Fibroma/genetics ; Gardner Syndrome/diagnosis ; Gardner Syndrome/genetics ; Genetic Testing ; Germ-Line Mutation ; Humans ; Male
    Language English
    Publishing date 2021-04-13
    Publishing country United States
    Document type Case Reports ; Journal Article
    ZDB-ID 280962-x
    ISSN 2038-2529 ; 0300-8916
    ISSN (online) 2038-2529
    ISSN 0300-8916
    DOI 10.1177/03008916211009316
    Database MEDical Literature Analysis and Retrieval System OnLINE

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