A Case Report of HIV-Related Bilateral Inflammatory Myofibroblastic Tumors of Adrenal Gland

Inflammatory myofibroblastic tumor (IMT) is a rare disease which mostly occurs at younger age and locates in lung in general population. We report a rare case of 44-year-old man diagnosed adrenal IMT with HIV infection, who refused regular highly active antiretroviral therapy (HAART) 13 years ago until in hospital because of findings of adrenal masses. The patient underwent CT-guided needle biopsy successfully, and the pathological analysis documented the diagnosis of IMT by the feature of proliferation of fibroblastic-myofibroblastic with inflammatory infiltration. We failed to perform tumor complete resection due to diffuse invasion of tumor under laparoscope. The patient was complicated with severely multiple pulmonary infection post to surgery because of immunodeficiency, that eventually caused the death of patient 2 months later. This case reminds us that IMTs may be too aggressive and progressed in HIV-positive patients with irregular HAART to perform surgical resections, and severe immunodeficiency can be more fatal. To our knowledge, this case is the second IMT patient with HIV infection worldwide, but the first case occurs at adrenal gland rather than lung in adult.

The evolving role of precision medicine in the management of advanced sarcomas – A mini review

Sarcomas are a heterogenous group of cancers, traditionally with dismal outcomes. They were initially treated with histology agnostic chemotherapy-based regimens usually centered around anthracyclines. With the availability of molecular diagnostics especially next-generation sequencing, the advanced genomics of sarcomas was slowly unveiled. Precision medicine not only enables a better diagnosis in sarcomas but also allows in identifying better targets for treatment of sarcoma subtypes. GENSARC study proved that using correct molecular diagnostics, enabled in a better diagnosis and treatment of soft tissue sarcomas (STSs). Notable examples of targeted therapies with great success in sarcomas include imatinib and other tyrosine kinase inhibitors in gastrointestinal stromal tumors, neurotrophic tyrosine receptor kinase inhibitors infantile fibrosarcoma, and crizotinib in inflammatory myofibroblastic tumors. Thus, treatment of sarcomas has been gradually changing from traditional chemotherapy-based treatments to the modern targeted therapy. In this review, we hope to impress on the evolving role of precision medicine in sarcoma subtypes especially STS.

Inflammatory myofibroblastic tumour of genitourinary system

We describe two cases of inflammatory myofibroblastic tumor (IMT) involving genitourinary system. One patient with mass of urinary bladder presented with lower abdominal pain. Other presented with a mass involving right kidney presented with right flank pain. At time there are no specific guidelines exist for the management of inflammatory myofibroblastic tumors. In this case series we will discuss the work-up and management approaches done for each case. Continuous...

Inflammatory Myofibroblastic Tumors in Children: A Clinical Retrospective Study on 19 Cases

Background: Inflammatory myofibroblastic tumor (IMFT) is a rare neoplasm mainly affecting children and young adults. We conducted a retrospective study to evaluate the clinical features and treatment alternatives of childhood inflammatory myofibroblastic tumors.Methods: A total of 19 patients who were pathologically diagnosed with IMT between December 2008 and October 2018 were included. Collected data were demographic information, main complaints, tumor characteristics, treatment, pathological results, immunohistochemical analysis, and prognosis.Results: The male/female ratio was 13:6. The mean age at disease onset was 44.9 ± 33.9 months (range 4 to 111 months). The mean tumor size was 6.5 ± 4.0 cm (range 1.2 to 17.0 cm). The most common site was the abdomen (13/19). The most commonly used detection tool was CT. Eleven patients (57.9%) had aggressive tumor growth, including eight receiving extensive resection and three receiving palliative resection due to high local invasiveness and postoperative chemotherapy. Eight cases whose tumors were completely enveloped received complete resection. Immunohistochemistry was performed for 17 patients and ALK positivity was found in 11 patients. Despite three children lost to follow-up, sixteen patients were followed up for 6 to 132 months (average 63.9 months, median 66 months). Of which, twelve children survived with no evidence of IMT, and four cases (21%) showed local recurrences (two of them died). No distant metastasis was detected.Conclusions: IMT is rare in children with various locations, mostly appearing in the abdomen. Whether the tumor could be completely removed, the location and the invasiveness of surrounding tissues might be highly prognosis-related.

Intra-Abdominal Multicentric Inflammatory Myofibroblastic Tumors Mimicking Ruptured Appendicitis.

Inflammatory myofibroblastic tumors (IMTs) are a rare soft tissue neoplasm, usually seen in children and adolescents, which are predominantly found in the pulmonary region. The extrapulmonary multicentric lesions are exceedingly rare. We herein report the case of a 19-year-old female who developed acute bowel obstruction which caused by multicentric IMTs. We described her clinical presentations, operative finding, and pathological finding.