Rare Tumors最新文献

{"title":"Postoperative adjuvant eribulin in high-risk patients with soft tissue sarcoma: A retrospective study of eight cases.","authors":"Yudai Murayama, Hirokazu Ideta, Rumi Nakagawa, Yasutaka Sukawa, Masanobu Takahashi, Hiroyuki Kawashima, Kazutaka Kikuta","doi":"10.1177/20363613261450109","DOIUrl":"10.1177/20363613261450109","url":null,"abstract":"<p><strong>Background: </strong>The role of perioperative chemotherapy in localized soft tissue sarcomas (STS) remains controversial, particularly in older adult patients and those with considerable comorbidities who may not tolerate anthracycline-based regimens. Although eribulin has demonstrated efficacy and safety in advanced STS, its use as a postoperative adjuvant treatment has not been well documented.</p><p><strong>Objective: </strong>To evaluate the feasibility, safety, and clinical outcomes of postoperative adjuvant eribulin administration in patients with localized STS, with a focus on older adults and patients with comorbidities.</p><p><strong>Methods: </strong>We conducted a single-institution retrospective study of a case series of patients with localized STS who had undergone surgical resection with curative-intent, followed by postoperative adjuvant eribulin administration. Data on patient characteristics, treatment administration, adverse events, and oncological outcomes were obtained from the medical records. Adverse events were graded based on the Common Terminology Criteria for Adverse Events.</p><p><strong>Results: </strong>Eight patients, most of whom were older adults or had substantial comorbidities, were included in the study. Postoperative eribulin therapy was initiated in all patients. Hematological toxicities, including neutropenia and leukopenia, were relatively common, but manageable with dose modification and supportive care. Non-hematological toxicities, such as peripheral neuropathy and dysgeusia, were generally mild to moderate. No treatment-related deaths or clinically significant cardiac adverse events were observed. Six patients remained continuously disease-free during follow-up, whereas two died of the disease.</p><p><strong>Conclusions: </strong>Postoperative adjuvant eribulin therapy was feasible and well-tolerated in a high-risk population of patients with localized STS. Although its efficacy cannot be determined from this small case series, its favorable safety profile suggests that eribulin may be a viable treatment option for patients who are poor candidates for anthracycline-based perioperative chemotherapy. Further studies are warranted to clarify its role in perioperative treatment strategies for STS.</p>","PeriodicalId":46078,"journal":{"name":"Rare Tumors","volume":"18 ","pages":"20363613261450109"},"PeriodicalIF":1.2,"publicationDate":"2026-06-02","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC13230673/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148165005","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Palatal malignant peripheral nerve sheath tumor: A case report and review of literature.","authors":"Abbas Karimi, Samira Derakhshan, Monir Moradzadeh Khiavi, Seyed Mohammadmoein Hosseini","doi":"10.1177/20363613261453768","DOIUrl":"10.1177/20363613261453768","url":null,"abstract":"<p><p>Malignant peripheral nerve sheath tumor (MPNST) is a rare, aggressive soft tissue malignancy associated with neurofibromatosis type 1. Although MPNST most commonly occurs in the extremities and trunk, it is rare in the head and neck region and extremely rare in the oral cavity, including the palate. MPNSTs arise from Schwann cells, de novo or from benign neural tumors. MPNSTs often occur between the ages of 30 and 50 years closely associated with neurofibromas. The median survival rate of the patients is 46-58% over ten years. Diagnosis is based on medical history and clinical examination, and treatment includes surgery, radiotherapy, and sometimes chemotherapy. Prognosis varies depending on location, size, and metastasis. We report an elderly female with a MPNST of the palate presenting with an exophytic lesion with details of clinicoradiographic and histopathologic features and long-term follow-up. This report describes a rare case of palatal MPNST and reviews the relevant literature.</p>","PeriodicalId":46078,"journal":{"name":"Rare Tumors","volume":"18 ","pages":"20363613261453768"},"PeriodicalIF":1.2,"publicationDate":"2026-05-19","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC13187396/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"147989465","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Durable remission of metastatic myxoid liposarcoma following the cautious addition of pembrolizumab to ongoing pazopanib treatment: A case report.","authors":"Hussain Abbas, Wolfram Samlowski, Amin Hedayat","doi":"10.1177/20363613261450115","DOIUrl":"10.1177/20363613261450115","url":null,"abstract":"<p><p>Responses to conventional treatments, like chemotherapy and targeted agents, are generally infrequent, and of brief duration in patients with metastatic soft tissue sarcomas. We present a case of metastatic myxoid liposarcoma that achieved biopsy-proven complete remission with minimal toxicity following the addition of pembrolizumab to pazopanib as fourth-line treatment. This regimen resulted in significant tumor regression following just five doses. After 15 doses of pembrolizumab, radiographs demonstrated an ongoing response, with stable residual lesions, and a biopsy of a sacral lesion confirmed a pathologic complete remission. The patient subsequently discontinued all therapies and remains in an ongoing unmaintained remission. The combination of pembrolizumab and pazopanib demonstrated unexpected clinical benefit with minimal toxicity, suggesting that it may be worthy of further investigation in myxoid liposarcoma and possibly other histologic subtypes of STS.</p>","PeriodicalId":46078,"journal":{"name":"Rare Tumors","volume":"18 ","pages":"20363613261450115"},"PeriodicalIF":1.2,"publicationDate":"2026-05-15","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC13180121/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"147976278","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Diagnostic utility of Ki-67 proliferation index, PSA, and other immunohistochemical markers in prostatic basal cell carcinoma: A review of 24 cases in the current literature and discussion on current treatment and diagnostics.","authors":"Lister DeBinya, Vivek Mathesh, Jue Wang","doi":"10.1177/20363613261450124","DOIUrl":"10.1177/20363613261450124","url":null,"abstract":"<p><strong>Background: </strong>Basal cell carcinoma (BCC) of the prostate is a rare and poorly characterized malignancy distinct from conventional prostatic adenocarcinoma. Due to its rarity, diagnostic challenges arise, often leading to misclassification and delayed management. Immunohistochemical markers such as Ki-67 proliferation index and prostate-specific antigen (PSA) levels may aid in distinguishing BCC from typical adenocarcinoma, but data remain limited.</p><p><strong>Methods: </strong>This case series review study retrospectively analyzes published case reports and series spanning from 1988 to 2023 to evaluate Ki-67 staining patterns, PSA levels, and other immunohistochemical features to identify distinguishing characteristics of prostatic BCC. Data extracted included patient demographics, PSA levels at presentation, Ki-67 proliferation index values, and results of other immunohistochemical staining (i.e., PSA, P63).</p><p><strong>Results: </strong>Of the 24 BCC cases included in the final analysis, 20 reported PSA level at first presentation (mean: 2.56 ng/mL, range 0.08-7.3 ng/mL, normal range <4), 15 reported PSA IHC staining, and only 13 reported Ki-67 percentage values obtained through primary tumor biopsy staining (mean: 46.88%, range 0-80%). In comparison, Ki-67 expression numbers from National Comprehensive Cancer Network (NCCN) for prostate adenocarcinoma report varied Ki-67 expressions between risk groups: low-risk patients averaged 5.1% ± 3.8%, intermediate-risk patients averaged 7.4% ± 6.8%, and high-risk patients averaged 12.0% ± 12.4%. Various immunohistochemical staining was also collected yielding positive staining with Ki-67 in 13/13 cases, HMWCK/34βE12 in 14/14 cases, P63 in 14/14 cases, and BCL-2 in 12/13 cases.</p><p><strong>Conclusion: </strong>This retrospective analysis suggests that prostatic BCC may be characterized by higher Ki-67 proliferation indices and lower PSA levels compared to typical prostatic adenocarcinoma. This review further explores and proposes the potential of combining Ki-67 and PSA assessments along with other biomarkers to aid in distinguishing BCC from conventional prostate cancers, facilitating earlier recognition and appropriate management of this rare malignancy.</p>","PeriodicalId":46078,"journal":{"name":"Rare Tumors","volume":"18 ","pages":"20363613261450124"},"PeriodicalIF":1.2,"publicationDate":"2026-05-10","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC13167287/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"147943168","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Chronic myeloid leukemia presenting with isolated central nervous system lymphoid blast crisis: A case report.","authors":"Álvaro Zarama-Valenzuela, Monica Arévalo-Zambrano, Álvaro Cadena-Andrade, Alejandro Zárate-Mesa","doi":"10.1177/20363613261444290","DOIUrl":"10.1177/20363613261444290","url":null,"abstract":"<p><strong>Background: </strong>Chronic myeloid leukemia (CML) is a myeloproliferative neoplasm driven by the Philadelphia chromosome, typically progressing through chronic, accelerated, and blast phases. Blast crisis may be myeloid or lymphoid. Isolated central nervous system (CNS) involvement is rare and represents a diagnostic and therapeutic challenge.</p><p><strong>Case presentation: </strong>We report a 40-year-old man with no prior hematologic history who presented with four months of progressive lower-limb paresthesias, neuropathic pain, and subacute weakness impairing ambulation. Neurologic examination revealed asymmetric paraparesis, hyporeflexia, and distal hypoesthesia in a stocking distribution, consistent with a subacute asymmetric sensorimotor polyradiculoneuropathy. Complete blood count demonstrated marked leukocytosis with neutrophilia, left shift, and circulating blasts. Contrast-enhanced spinal magnetic resonance imaging showed extensive leptomeningeal enhancement. Cerebrospinal fluid (CSF) analysis revealed pleocytosis, hypoglycorrhachia, and elevated protein levels; flow cytometry identified 14% B-lymphoid blasts (CD34+, CD19+, CD20+, CD22+). Bone marrow aspirate and biopsy showed hypercellularity with trilineage hematopoiesis, basophilia, and small hypolobated megakaryocytes, without a significant increase in blasts (<5%). Cytogenetic analysis identified t (9; 22), and molecular testing confirmed the BCR::ABL1 p210 transcript, consistent with chronic-phase CML. An isolated CNS lymphoid blast crisis was diagnosed. Treatment with HyperCVAD (Block B only) plus dasatinib 140 mg daily resulted in neurologic improvement and hematologic normalization. After five cycles, CSF clearance was achieved, followed by consolidation with haploidentical allogeneic hematopoietic stem cell transplantation. The patient remains in complete remission.</p><p><strong>Conclusions: </strong>Isolated CNS blast crisis may represent the initial manifestation of CML. Prompt recognition through integrated neurologic, hematologic, and molecular evaluation is critical to enable timely, targeted therapy.</p>","PeriodicalId":46078,"journal":{"name":"Rare Tumors","volume":"18 ","pages":"20363613261444290"},"PeriodicalIF":1.2,"publicationDate":"2026-04-16","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC13091979/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"147730280","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Bronchoscopy, surgery and radiation therapy for bronchial adenoid cystic carcinoma: A retrospective cohort study.","authors":"Hiroshi Handa, Yu Numata, Kazuhiro Nishiyama, Hajime Tsuruoka, Kei Morikawa, Takeo Inoue, Masamichi Mineshita","doi":"10.1177/20363613261436697","DOIUrl":"10.1177/20363613261436697","url":null,"abstract":"<p><strong>Background: </strong>Adenoid cystic carcinoma (ACC) of the bronchus is a rare malignancy, accounting for 1-2% of all respiratory tumors. Its slow growth often results in delayed diagnosis. Although surgical resection is the preferred treatment, ACC frequently demonstrates local invasiveness, leading to central airway obstruction that worsens respiratory symptoms and adversely affects prognosis. Consequently, bronchoscopic interventions and radiation therapy are often required for disease management.</p><p><strong>Objective: </strong>To evaluate the long-term outcomes of multimodal management, including surgery, radiation therapy, and bronchoscopic interventions, in patients with bronchial ACC treated at our institution.</p><p><strong>Methods: </strong>We conducted a retrospective review of medical records for patients diagnosed with bronchial ACC at our institution between April 1, 2005, and December 31, 2024. Clinical data, including patient demographics, treatment modalities, and overall survival (OS), were collected and analyzed.</p><p><strong>Results: </strong>Eleven patients with central airway lesions were included. The mean age at diagnosis was 61±15 years, and the median OS was 73.4 months (range: 1-158 months). Bronchoscopic interventions under general anesthesia, including argon plasma coagulation, high-frequency snaring, and airway stenting, were performed in nine patients. Seven patients underwent surgical resection, including six tracheal anastomoses and one left total pneumonectomy. Postoperative radiation therapy was administered to seven patients.</p><p><strong>Conclusion: </strong>A multimodal approach incorporating bronchoscopy, surgery, and radiation therapy may improve outcomes in patients with bronchial ACC.</p>","PeriodicalId":46078,"journal":{"name":"Rare Tumors","volume":"18 ","pages":"20363613261436697"},"PeriodicalIF":1.2,"publicationDate":"2026-03-16","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC13009619/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"147515824","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Primary epithelioid angiosarcoma of the vulva: A rare tumor at an unusual site.","authors":"Elizabeta Miovska, Jasmina Dobrevska, Katerina Nikoloska, Bisera Nikolovska","doi":"10.1177/20363613261433351","DOIUrl":"10.1177/20363613261433351","url":null,"abstract":"<p><p>Epithelioid angiosarcoma of the vulva is an exceptionally rare and highly aggressive malignancy originating from endothelial cells. Its etiology remains unclear, though risk factors such as prior radiotherapy and chronic lymphedema have been identified. We report a case of a 44-year-old woman with no significant medical history who presented with a rapidly enlarging, foul-smelling mass on the right labia majora. The tumor measured 30 cm and exhibited central necrosis, ulceration, and bleeding. Radical tumor resection and right inguinal lymphadenectomy were performed, followed by reconstruction using a keystone design perforator island flap and a V-Y advancement flap. Histopathology confirmed a high-grade epithelioid angiosarcoma, positive for ERG, CD31, CD34, podoplanin, EMA, and a Ki-67 proliferation index of 60%. Further treatment consisted of adjuvant radiotherapy (61 Gy) and doxorubicin-based chemotherapy. At one-year follow-up, the patient remained free of recurrence or metastasis. Primary epithelioid angiosarcoma of the vulva is extremely rare, particularly in patients without known risk factors. Management involves radical surgical resection with adjuvant radiotherapy and chemotherapy as indicated. Reporting such cases is essential to enhance understanding and guide evidence-based management of these challenging neoplasms.</p>","PeriodicalId":46078,"journal":{"name":"Rare Tumors","volume":"18 ","pages":"20363613261433351"},"PeriodicalIF":1.2,"publicationDate":"2026-03-06","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12966541/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"147379020","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Burden and unmet needs of liposarcoma in the United States: Patient perspectives on the liposarcoma diagnostic journey.","authors":"Dan Mulligan, Amy Shaberman, Jennifer Han, Jennifer Horonjeff, Brandi Felser","doi":"10.1177/20363613261428805","DOIUrl":"10.1177/20363613261428805","url":null,"abstract":"<p><strong>Purpose: </strong>Studies investigating the impact of liposarcoma from the patient's perspective-including diagnosis, disease presentation, treatment challenges, and quality of life (QOL)-are limited. We report findings from a survey of patients with liposarcoma evaluating the diagnostic/treatment journey, biomarker testing, and QOL.</p><p><strong>Methods: </strong>Patients with liposarcoma in the United States completed an online survey assessing demographics, diagnostic journey, disease burden, treatment, QOL, and sources of support during diagnosis and treatment. Descriptive statistical analyses were performed, and qualitative written responses from participants were assessed.</p><p><strong>Results: </strong>Seventy-seven patients with liposarcoma participated in the survey. The proportion of participants with stage I/II/III/IV/unknown liposarcoma at diagnosis was 22%/16%/26%/13%/23%, respectively. Within 12 months of symptom onset, 44/60 respondents (73%) saw a medical professional, and 39/77 (51%) first consulted an internal medicine specialist. Fifty-one of 77 participants (66%) were aware of biomarker testing, and 32/51 (63%) reported receiving it. The most common initial treatments were surgery (47/77, 61%), radiotherapy (15/77, 19%), and chemotherapy (9/77, 12%). Health insurance provided coverage for biomarker testing for 27/31 respondents (87%). Of 77 participants, 38 (49%) traveled ≥1 hour to reach their treatment site, and 27 (35%) relied on care partners such as family members for transportation. Upon treatment initiation, QOL improved for 30/77 participants (39%), did not improve for 29/77 (38%), and 10/77 (13%) were unsure. Forty participants (52%) used social media to obtain disease-specific information and support.</p><p><strong>Conclusion: </strong>Our survey identified gaps in patients' awareness and understanding of key aspects of liposarcoma diagnosis and treatment, highlighting a need for better patient education, more effective communication between medical professionals and patients, and a deeper understanding of how treatment impacts patients' QOL. A fuller picture of the patient burden due to liposarcoma underscores the need for research aimed at improving symptom and disease management, including the development of more effective therapeutics.</p>","PeriodicalId":46078,"journal":{"name":"Rare Tumors","volume":"18 ","pages":"20363613261428805"},"PeriodicalIF":1.2,"publicationDate":"2026-02-27","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12949793/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"147345221","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"A rare case of bilateral adnexal Müllerian adenosarcoma arising from endometriosis: A case report and literature review.","authors":"Firouze Jafari, Mojgan Akbarzadeh Jahromi, Saeed Askari, Elham EntezarAlmahdi","doi":"10.1177/20363613261426718","DOIUrl":"10.1177/20363613261426718","url":null,"abstract":"<p><strong>Background: </strong>Müllerian adenosarcoma is an uncommon mixed epithelial-mesenchymal neoplasm that predominantly develops in the uterine corpus and rarely occurs in extrauterine sites, including the ovary. Ovarian adenosarcoma is associated with a more aggressive course and is often linked to endometriosis.</p><p><strong>Case presentation: </strong>We present the case of a nulliparous woman in her early 30s who presented with abdominal bloating, pelvic pain, and significant ascites. Imaging studies revealed bilateral adnexal solid-cystic masses. Histopathologic and immunohistochemical (IHC) evaluation of the resected specimens demonstrated Müllerian adenosarcoma involving both adnexa and peritoneal surfaces, likely arising from endometriosis.</p><p><strong>Conclusion: </strong>This case highlights the diagnostic difficulty of extrauterine Müllerian adenosarcoma, particularly when presenting as bilateral adnexal Müllerian adenosarcoma with peritoneal involvement arising in the setting of endometriosis. Detailed histopathologic evaluation is essential, especially when glandular elements show mucinous differentiation, which broadens the differential diagnosis.</p>","PeriodicalId":46078,"journal":{"name":"Rare Tumors","volume":"18 ","pages":"20363613261426718"},"PeriodicalIF":1.2,"publicationDate":"2026-02-21","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12925028/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"147277344","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Subglottic inflammatory myofibroblastic tumor: A rare case report in a pediatric patient.","authors":"Shary Acosta Suárez, Lina Estefanía Barón Méndez, Diana Patricia Romero Lara","doi":"10.1177/20363613251407005","DOIUrl":"10.1177/20363613251407005","url":null,"abstract":"<p><p><b>Background:</b> Inflammatory myofibroblastic tumour (IMT) of the pediatric laryngotracheal complex is exceedingly rare, with fewer than nine subglottic IMT cases reported in the literature up to 2025. We present an additional case and highlight the importance of early recognition. <b>Case presentation:</b> A previously healthy 10-year-old boy presented with a 3-week history of progressive dyspnea on exertion and nocturnal biphasic stridor, initially mislabelled as refractory asthma. Contrast-enhanced CT revealed a left-sided subglottic soft-tissue mass that reduced the airway lumen to 2.7 mm, while rigid bronchoscopy confirmed an 80% occlusion caused by a 10 mm submucosal lesion spanning the cricoid and first tracheal ring. After emergent tracheostomy at the referring institution, he underwent lesion resection and simultaneous decannulation. Histopathology demonstrated spindle-cell proliferation within a myxoid inflammatory stroma; immunohistochemistry was <i>ALK-1</i>-positive, confirming IMT. Post-operative bronchoscopy on day seven showed a widely patent airway. Five years later the child remains asymptomatic and oxygen-independent, with no radiological or endoscopic evidence of recurrence. <b>Conclusion:</b> This case reinforces that persistent pediatric stridor mandates prompt endoscopic evaluation and that complete surgical excision remains the gold-standard therapy for airway IMT. Given the tumour's intermediate malignant potential and a reported 18-40% recurrence rate when margins are positive, meticulous long-term surveillance is indispensable, and <i>ALK</i>-directed therapy should be considered when resection is not feasible. Timely diagnosis and radical local therapy can avert prolonged tracheostomy and enrich the scant literature on pediatric subglottic IMT.</p>","PeriodicalId":46078,"journal":{"name":"Rare Tumors","volume":"18 ","pages":"20363613251407005"},"PeriodicalIF":1.2,"publicationDate":"2026-02-19","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12923926/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"147272386","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}