A Rare Case of Inflammatory Myofibroblastic Tumor Causing Left Pulmonary Artery Stenosis and Successfully Treated with Localized Radiotherapy in a Patient with Perinuclear Antineutrophil Cytoplasmic Antibody Vasculitis

Browne, Claire P.; Zeman-Pocrnich, Cady; Dar, A. Rashid; Wyllie, Blair; Joseph, Mariamma

doi:10.7759/cureus.6709

Cited by 6 publications

(3 citation statements)

References 18 publications

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“…Surgical resection is the preferred mainstay of treatment [ 16 ]. However, there are cases of successful treatment with chemotherapy, NSAIDs, steroids, radiation and targeted therapy in different combinations and as individual therapy for unresectable or recurring disease as well in the literature [ [22] , [23] , [24] , [25] ].…”

Section: Discussionmentioning

confidence: 99%

Case report of an unexpected inflammatory myofibroblastic tumor

Raza

Arshad

Ahmad

et al. 2021

Annals of Medicine &Amp; Surgery

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Introduction Inflammatory myofibroblastic tumor is a benign entity that may present as a locally aggressive malignancy, predominantly in the lung. 500 cases have been reported in the literature, and an estimated prevalence ranges from 0.04% to 0.7%. Case presentation An eighteen-year old male presented to the surgical clinic with abdominal pain. The pain was recurring despite trials of analgesics and remained undiagnosed. Radiological imaging demonstrated a lesion in the spleen. An infectious cause was presumed due to their endemicity in South Asia, however pathology showed an inflammatory myofibroblastic tumor. Discussion Pre-operative imaging is yet to develop a set criterion that may identify this lesion, though clinicians may be clued in by the benign appearance despite the large size. Usual diagnosis is on pathology after complete surgical excision, which is the mainstay treatment advocated. Conclusion Reporting of the tumor in unusual sites such as the spleen is scarce, increase of which may help establish guidelines, understand tumor behavior and guide clinicians that may encounter it in surgical practice.

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Section: Discussionmentioning

confidence: 99%

Case report of an unexpected inflammatory myofibroblastic tumor

Raza

Arshad

Ahmad

et al. 2021

Annals of Medicine &Amp; Surgery

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“…Another case study focuses on a patient with IMT leading to severe stenosis of the left pulmonary artery who was also receiving immunosuppressive treatment for perinuclear antineutrophil cytoplasmic antibody vasculitis. The patient received radiotherapy with a total dose of 45 Gy over five weeks and has been followed for over seven years post-treatment without persistent toxicity [ 170 ].…”

Section: Radiotherapymentioning

confidence: 99%

Inflammatory myofibroblastic tumor from molecular diagnostics to current treatment

CHMIEL,

SłOWIKOWSKA,

BANASZEK

et al. 2024

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Inflammatory myofibroblastic tumor (IMT) is a rare neoplasm with intermediate malignancy characterized by a propensity for recurrence but a low metastatic rate. Diagnostic challenges arise from the diverse pathological presentation, variable symptomatology, and lack of different imaging features. However, IMT is identified by the fusion of the anaplastic lymphoma kinase (ALK) gene, which is present in approximately 70% of cases, with various fusion partners, including ran-binding protein 2 (RANBP2), which allows confirmation of the diagnosis. While surgery is the preferred approach for localized tumors, the optimal long-term treatment for advanced or metastatic disease is difficult to define. Targeted therapies are crucial for achieving sustained response to treatment within the context of genetic alteration in IMT. Crizotinib, an ALK tyrosine kinase inhibitor (TKI), was officially approved by the US Food and Drug Administration (FDA) in 2020 to treat IMT with ALK rearrangement. However, most patients face resistance and disease progression, requiring consideration of sequential treatments. Combining radiotherapy with targeted therapy appears to be beneficial in this indication. Early promising results have also been achieved with immunotherapy, indicating potential for combined therapy approaches. However, defined recommendations are still lacking. This review analyzes the available research on IMT, including genetic disorders and their impact on the course of the disease, data on the latest targeted therapy regimens and the possibility of developing immunotherapy in this indication, as well as summarizing general knowledge about prognostic and predictive factors, also in terms of resistance to systemic therapy.

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“…31 IMT has also responded to systemic therapy including chemotherapy and radiation treatment in select cases. 31,33 The rare ROS1rearranged IMTs have been found to respond to crizotinib, an antibody that has demonstrated significant reduction in tumor size. (Mai) Trabectedin, a marine-derived anticancer agent, is a microenvironment-targeting drug that is currently being investigated.…”

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confidence: 99%

Inflammatory Myofibroblastic Tumor 12 Years After Treatment for Synovial Sarcoma: A Case Report

Shah¹,

Pey²,

Achonu³

et al. 2021

ORR

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Inflammatory myofibroblastic tumors (IMTs) are mesenchymal neoplasms most seen in the abdominopelvic region, lung, and retroperitoneum; and less commonly seen in virtually any other site. We report a case of two lower limb masses consistent with diagnosis of IMTs. This is a 39-year-old woman with a history of right lower extremity popliteal fossa synovial sarcoma diagnosed 12 years prior and treated with chemotherapy, surgery, and radiation. She presented with two new -one anterior and one posterior -right thigh masses. Biopsies of the lesions demonstrated low-grade inflammatory spindle cell lesions at both sites. Wide resection was performed for both masses and further characterization of the surgical specimens was most consistent with IMT. At follow-up, the patient is well with no signs of recurrence 19 and 7 months postoperative to the resection of the anterior and posterior thigh masses, respectively. This case represents the first reported IMTs occurring as late as 12 years after primary cancer treatment, and the first occurring after synovial sarcoma.

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A Rare Case of Inflammatory Myofibroblastic Tumor Causing Left Pulmonary Artery Stenosis and Successfully Treated with Localized Radiotherapy in a Patient with Perinuclear Antineutrophil Cytoplasmic Antibody Vasculitis

Abstract: Browne et al. This is an open access article distributed under the terms of the Creative Commons Attribution License CC-BY 3.0., which permits unrestricted use, distribution, and reproduction in any medium, provided the original author and source are credited.

Cited by 6 publications

References 18 publications

Case report of an unexpected inflammatory myofibroblastic tumor

Case report of an unexpected inflammatory myofibroblastic tumor

Inflammatory myofibroblastic tumor from molecular diagnostics to current treatment

Inflammatory Myofibroblastic Tumor 12 Years After Treatment for Synovial Sarcoma: A Case Report

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