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Sinonasal tumours
Published in Neeraj Sethi, R. James A. England, Neil de Zoysa, Head, Neck and Thyroid Surgery, 2020
Yujay Ramakrishnan, Shahzada Ahmed
These sinonasal tumours often spread to adjacent sites like the skull base and orbit. About 40% metastasise to lymph nodes, bones and lungs. The tumours are staged according to the Intergroup Rhabdomyosarcoma Study. The first treatment strategy is chemotherapy, with or without radiotherapy. Surgery is reserved for non-responders or recurrent disease [38].
Paediatric cancer
Published in Peter Hoskin, Peter Ostler, Clinical Oncology, 2020
In childhood, there are few other tumours that are likely to arise in similar sites to rhabdomyosarcoma. Other causes of presenting symptoms such as epistaxis or haematuria in the absence of an obvious mass should be sought.
The locomotor system
Published in C. Simon Herrington, Muir's Textbook of Pathology, 2020
Embryonal rhabdomyosarcoma occurs mainly in children, in the head and neck, genitourinary tract, and retroperitoneum. Tumours occurring in the vagina or bladder project as grape-like gelatinous masses and are known as ‘botryoid’ (grape-like) sarcomas. They consist of spindle-shaped cells and show varying degrees of skeletal muscle differentiation. An embryonal rhabdomyosarcoma usually responds well to combined chemotherapy, radiotherapy, and surgery.
Recurrent Giant Cell Fibroblastoma in an Infant: A Diagnostic Challenge
Published in Fetal and Pediatric Pathology, 2022
Priyanka Maity, Uttara Chatterjee, Mou Das, Sabita Patra
A 14 months old boy presented with a recurrent mass in the left hemiscrotum. He first presented with a mass at the same site six months earlier. USG and CT suggested a testicular tumor (Fig. 1a). His serum alpha-fetoprotein was normal. FNAC from the mass was a spindle cell tumor, suggestive of embryonal rhabdomyosarcoma (ERMS). The tumor was excised and diagnosed as embryonal rhabdomyosarcoma. The patient received six cycles of vincristine, actinomycin D and cyclophosphamide (VAC). Our review of the FNAC slides showed a moderately cellular lesion composed of bland spindle cells in clusters and singly. The spindle cells had bland nuclei, bipolar cytoplasm and indistinct nucleoli. Occasional giant cells were noted with nuclei gathered at one end. Myxoid material was present focally. There were no mitoses, necrosis or atypia. There were no strap cells to suggest rhabdomyoblastic differentiation (Fig. 1, b–e).
Rhabdomyosarcoma from uterus to heart
Published in Journal of Community Hospital Internal Medicine Perspectives, 2020
Sohaib Roomi, Mahnoor Fatima Sherazi, Waqas Ullah, Omair Ali Khan, Artem Minalyan, Muhammad Atiq, Nishant Thalambedu
Cardiology Department was consulted. Initially, a presumptive diagnosis of atrial myxoma was made. Computed Tomography (CT) scan of the abdomen & pelvis revealed a 3 cm mass in the uterus. Biopsy of the uterine mass revealed it to be a rhabdomyosarcoma resembling an undifferentiated pleomorphic sarcoma (Figure 2). Pleomorphic cells with abundant mitosis and areas of necrosis were seen. At this point, atrial mass was presumed to be a myxoma and the cause of palpitations. As per Cardiology recommendation, It was excised and the mitral valve was repaired. Histological analysis of the atrial mass was consistent with the diagnosis of rhabdomyosarcoma (Figure 3). Histology of both atrial and uterine mass revealed a cellular malignant neoplasm composed of round to elongated markedly atypical cells enlarged with hyperchromatic nuclei and some cells with eosinophilic cytoplasm. Desmin positivity was observed on immunohistochemistry testing of uterine as well as the atrial mass. These findings, along with the patient’s clinical history, were consistent with the diagnosis of the rhabdomyosarcoma. After the surgical excision of the cardiac mass, the patient was scheduled for chemo/radiation therapy for uterine pathology. The patient stayed in the hospital for five days. She was discharged home and regular follow up was advised in the clinic with Gynecologic-Oncology, Cardiac Surgery, and Primary care Physician.
A Review of the Role of Cytogenetics in the Diagnosis of Orbital Rhabdomyosarcoma
Published in Seminars in Ophthalmology, 2019
Paula Cortes Barrantes, Frederick A. Jakobiec, Thaddeus P. Dryja
Rhabdomyosarcoma (RMS) is a malignant soft tissue tumor with skeletal muscle differentiation. It does not develop from preformed or dedifferentiated striated muscle myofibers, but instead arises from multipotential mesenchymal stem cells resident in the fascial tissue planes associated with connective tissues. It is the most common sarcoma of childhood and adolescence1−4, with an estimated incidence rate of 4.3 cases per million children, according to the Cancer Incidence and Survival in Children and Adolescent Rhabdomyosarcoma.5 Approximately 10% of childhood RMS cases arise in the orbit2,4,6, which is considered a favorable anatomic site, since most of the patients present with localized disease.4 (Figure 1)