Figure - available from: Journal of Medical Case Reports
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a Infiltration of tumor within myometrium. b Rhabdoid cells with eosinophilic brightly abundant cytoplasm and numerous mitotic figures (yellow arrow)
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Background
Uterine sarcoma accounts for 3–7% of uterine malignant neoplasms. It is more aggressive than epithelial neoplasms, and patients have a poor prognosis. Rhabdomyosarcoma is classified as a heterologous uterine sarcoma. It is the most common soft tissue malignancy in children while rare in adults. In young patients, the majority of genital...
Citations
... Meanwhile, patients with uterine corpus RMS were found to be older (median age, 65 years; range=21-90 years), with larger tumor sizes (median size, 10.0 cm; range=5.0-20.0 cm) than those with cervical RMS, in a review of 41 cases with uterine corpus RMS (11). The median survival time for patients with uterine corpus RMS was 12.9 months. ...
... Multimodal therapy consisting of surgery, chemotherapy, and radiation, has also been conducted for uterine RMS in adults as well as children (5,9,11). Fertility preservation is a treatment strategy for children with uterine RMS. ...
... Pelvic and/or aortic lymph node dissection has been performed in several cases; however, whether it can improve the outcome of uterine RMS remains unknown. Lymph node excision should be performed with caution because some patients die several days or weeks following surgery (9,11,13). Most patients undergo additional therapies, including chemotherapy and/or radiotherapy, after surgery. ...
Background/Aim: Rhabdomyosarcoma (RMS) is the most common malignant soft tissue tumor in children. Adult primary RMS of gynecological origin is a rare condition and uterine RMS is an aggressive malignancy with a poor prognosis. The genetic variants associated with uterine RMS in adults have yet to be fully elucidated, and there is no established therapeutic strategy for rare tumors. Case Report: A 69-year-old Japanese woman was referred to our hospital with abdominal bloating. Imaging examination revealed a tumor with diameter of 85 mm located in the uterus and multiple regional lymph node metastases. Biopsy of the uterine corpus indicated possible uterine carcinosarcoma or RMS. Following debulking surgery, the patient was diagnosed with stage IVB pleomorphic RMS. The patient was treated with two courses of doxorubicin every three weeks and one course of combination chemotherapy with vincristine, actinomycin, and cyclophosphamide. Because of rapid progression of the disease, we decided to perform multi-gene panel testing to determine the most effective therapeutic strategy. However, no therapeutic plan based on genetic information was identified. The patient with chemotherapy-refractory RMS died 11 weeks after surgery. Conclusion: Our patient had advanced uterine RMS with an unresectable tumor that was resistant to chemotherapy, resulting in poor outcomes. Despite conducting multi-gene panel testing, no tailored therapeutic approach based on genetic information was found. This case highlights the challenges in managing uterine RMS in adults and underscores the urgent need for further research to identify effective treatment modalities.
... It is extremely infrequent in adults and it accounts for less than 1% of all adult cancers [2]. The reported sites for primary RMS are head, neck, upper limbs [7], legs, trunk, pelvis [8], uterus [9][10][11], liver [5,12], ovary [13] and mesentery [14]. The omental tissue is of mesothelial origin, and it possesses stem cells' function, and angiogenic, immune and fibrotic activities. ...
Rhabdomyosarcoma (RMS) is a very rare, highly malignant neoplasm thought to originate from the pluripotent mesenchymal tissue. Predominantly diagnosed among children and teenagers, however they can also be encountered in adults. There are a few risk factors associated with RMS like family history of malignancy and genetic syndromes like neurofibromatosis type 1, Li-Fraumeni syndrome, Noonan syndrome and Beckwith-Wiedemann syndrome; however, most cases of RMS are sporadic. Other factors like radiotherapy for other malignancy and pre-natal radiation exposure also are associated with increased risk of developing RMS. The most common reported sites for RMS are head, neck, trunk, pelvis and lower limbs. Omental involvement of primary RMS has been rarely reported in the literature. Principally, the survival of treated RMS cases has improved, primarily due to multidisciplinary management approaches. In this paper, we report a case of primary pleomorphic RMS in a 50-year-old female who presented with abdominal pain.
