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Low-grade malignant myofibroblastic sarcoma of the larynx: a case report.

ABSTRACT: Low-grade myofibroblastic sarcoma (LGMS) is a rare malignant mesenchymal tumor derived from myofibroblasts. It is commonly identified in the head and neck, and particularly in the oral cavity, but rarely in the larynx. In this case report, we describe a patient who presented with hoarseness and underwent electronic fiber laryngoscopy, which revealed a neoplasm on the surface of his left vocal cord. The vocal cord tumor was resected under general anesthesia, and a malignant LGMS was diagnosed on postoperative pathologic examination. The results of immunohistochemical staining of the sections for vimentin (diffuse +), actin (partial +), and desmin (-) were consistent with this diagnosis. The patient recovered well after the surgery, and there was no recurrence of the neoplasm.

SUBMITTER: Feng Y

PROVIDER: S-EPMC10492498 | biostudies-literature | 2023 Sep

REPOSITORIES: biostudies-literature

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Low-grade malignant myofibroblastic sarcoma of the larynx: a case report.

Feng Yu Y Du Yongya Y Li Maocai M Jia Guotao G Gong Lili L Li Lianqing L

The Journal of international medical research 20230901 9

Low-grade myofibroblastic sarcoma (LGMS) is a rare malignant mesenchymal tumor derived from myofibroblasts. It is commonly identified in the head and neck, and particularly in the oral cavity, but rarely in the larynx. In this case report, we describe a patient who presented with hoarseness and underwent electronic fiber laryngoscopy, which revealed a neoplasm on the surface of his left vocal cord. The vocal cord tumor was resected under general anesthesia, and a malignant LGMS was diagnosed on ...[more]

PMID: 37684014

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Project description:BackgroundLow-grade myofibroblastic sarcoma (LGMS) originating from breast is rare. Existing literature comprises clinical and pathological reports, with limited information on imaging characteristics. This study reports a case of LGMS of the breast and presents its imaging characteristics, with an emphasis on those observed using contrast-enhanced magnetic resonance imaging (MRI).Case descriptionA 50-year-old patient presented with a left breast mass for 1 year. One year before the presentation, the patient had palpated a mass of approximately 1.5 cm in size in the upper part of the left breast without any obvious cause. The mass was perceived to be growing slowly. There was no relevant family history of breast conditions. Physical examination revealed a hard, ill-defined, irregularly shaped, non-tender mass of approximately 3.5 cm × 3 cm in size, with poor mobility and a close connection to the deep skin. The mammography showed a high-density mass without microcalcifications and boundary wrapping. Ultrasonography showed an oval, ill-defined hypoechoic mass. The combination of mammography and ultrasound examination results ruled out the possibility of ductal carcinoma and benign fibroepithelial tumor. On contrast-enhanced MRI, the mass exhibited heterogeneous enhancement, high signal intensity on T2-weighted imaging (T2WI), high signal intensity on diffusion-weighted imaging (DWI), and a type I time-intensity curve (TIC). A core needle biopsy (CNB) suggested a spindle cell tumor. To confirm the diagnosis, the patient underwent surgical excision, and postoperative pathology confirmed LGMS of the breast. The patient subsequently received adjuvant radiotherapy. Seven months postoperatively, bone scintigraphy suggested possible metastases.ConclusionsLGMS of the breast exhibited a degree of malignancy on ultrasonography, mammography, and MRI, with the contrast-enhanced MRI showing a persistent enhancement pattern (type I TIC). A preoperative biopsy indicated a spindle cell tumor. Surgical excision remains the best diagnostic method. A thorough understanding of the imaging characteristics and biopsy results of this tumor type provides comprehensive information for formulating corresponding treatment plans.

Project description:BackgroundLow-grade fibromyxoid sarcoma (LGFMS) is a rare soft tissue tumor with a misleadingly bland histological appearance and fully malignant behavior, typically occurring in the deep soft tissues of the proximal extremities or trunk of young adults. However, no cases of primary middle ear LGFMS have been reported previously. LGFMS is characterized by high rates of local recurrence and metastatic spread, which should be attached of great importance to clinical diagnosis and treatment.Case descriptionHerein, we report an unusual case of LGFMS occurring primarily in the middle ear of a 12-year-old boy, who presented with aural fullness and gradually progressive hearing loss in the left ear for 6 months, without other related symptoms and family history. Preoperative imaging examination suggested that the lesions were located in the tympanic cavity, tympanic antrum, and mastoid portion, with equisignal or hypointense on T1 weighted image (T1WI), apparent hyperintense on T2 weighted image (T2WI), and slight enhancement on T1WI following administration of gadolinium. A decision was made to perform mastoidectomy, as the lesion was limited to the middle ear and did not invade the facial nerve canal or the inner ear. During the surgery, the mass exhibited a hard texture and smooth surface that was approximately 1.0 cm × 1.5 cm in size, not easy to bleed, and non-adherent to surrounding tissues. After consultation, a diagnosis of LGFMS was made by postoperative pathology. The patient showed an excellent recovery from surgery without any complications. At present, the patient has been followed up for 24 months, and no local recurrence or distant metastasis has been observed.ConclusionsThe primary LGFMS in the middle ear is very rare, and the clinical manifestations and related examinations lack specificity, so a clinical diagnosis of LGFMS is very difficult, and the final diagnosis is mainly determined by pathological diagnosis. Due to its malignant behavior, clinical diagnosis and treatment should be vigilant against it. Treatment of LGFMS mainly requires extensive resection combined with radiotherapy and chemotherapy if necessary, and long-term follow-up is essential. Reporting and identification of this rare case are imperative to improving our understanding of LGFMS and reducing misdiagnosis.

Dataset Information

Low-grade malignant myofibroblastic sarcoma of the larynx: a case report.

Publications

Low-grade malignant myofibroblastic sarcoma of the larynx: a case report.

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