Spindle cell embryonal rhabdomyosarcoma of the prostate in an adult patient – case report and review of clinicopathological features

Spindle cell/sclerosing rhabdomyosarcoma (RMS) is an uncommon type of RMS and has been classified as a separate entity by the WHO in 2013. It affects both children and adults with a greater incidence in males. These tumors can pose a diagnostic challenge and can be difficult to differentiate from other spindle cell malignant tumors in the head and neck. Here, we report a case of spindle cell/sclerosing RMS in a young woman presenting with a swelling on the left side of the face of 3 months duration. A careful correlation with the radiographic images, histopathological findings and immunohistochemistry helped to arrive at a diagnosis.

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“…Scattered large rhabdomyoblasts with an eccentric nucleus and striated, eosinophilic cytoplasm may be seen. [ 8 ]…”

Section: Discussionmentioning

confidence: 99%

Spindle cell rhabdomyosarcoma in the adult

Jakkampudi

Sajitha

Hegde

et al. 2022

Journal of Oral and Maxillofacial Pathology

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“…It may develop within the parotid glands [91], the thyroid [92], and the oral cavity [93]. RMS within the urogenital tract may occur, among others, in the bladder [94], prostate [95], urethra [96], uterus [97,98], vulva [99], or scrotum [100]. In adult RMS patients it more commonly develops in an unfavorable anatomical localization, i.e.…”

Section: Clinical Picturementioning

confidence: 99%

Diagnosis and treatment of rhabdomyosarcomas

et al. 2023

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Rhabdomyosarcoma (RMS) is a soft tissue sarcoma. The primary tumor is most commonly localized in the head and neck, the urogenital system, or the limbs. Classification by the World Health Organization has distinguished four histopathological RMS subtypes: embryonal, alveolar, pleomorphic, and spindle cell/sclerosing. Differential diagnosis of RMS includes melanoma, malignant neoplasm of peripheral nerve sheaths, liposarcoma, and PE-Coma. Among typical cytogenetic changes in RMS are chromosomal translocations t(2;13)(q35;q14) and t(1;13) (p36;q14). They lead to the formation of fusion genes that have a prognostic value. In the course of RMS, changes may also be present in signaling pathways, including RAS-PI3K, Wnt/b-catenin, receptor tyrosine kinase pathways, and myogenesis regulation. In 30% of patients at the time of diagnosis of RMS, distant metastases are present, most commonly to lungs, lymph nodes, bones, and bone marrow. Treatment of patients with RMS requires a multidisciplinary approach, and steadily perfected diagnostic techniques contribute to the individualization of therapeutic strategies. Optimal treatment of localized RMS is based on surgery combined with radiotherapy and chemotherapy. If distant metastases are present, the basic therapeutic method is multidrug chemotherapy, most frequently based on vincristine, dactinomycin, ifosfamide/cyclophosphamide, and etoposide. Despite intensive treatment, the 5-year survival index for RMS is not greater than 50%. There are still no unequivocal guidelines concerning the treatment in patients with local or distant recurrences.

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“…4 SpRMS that lacks these molecular alterations is classified as spindle cell ERMS. [5][6][7] Childhood SpRMS most commonly occurs in paratesticular sites and has a better prognosis than several other subtypes. 8 Adult SpRMS was originally reported by Rubin et al 9 Unlike SpRMS in children, SpRMS in adults most commonly occurs in the head and neck; additionally, it has a significantly poorer prognosis than in children, with recurrence and metastasis rates as high as 50%.…”

Section: Introductionmentioning

confidence: 99%

Spindle cell embryonal rhabdomyosarcoma of the prostate in an adult patient: a case report and review of the literature

Luo,

Weng,

Zhong

et al. 2024

J Int Med Res

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Rhabdomyosarcoma of the prostate is a rare mesenchymal tumor that originates from undifferentiated mesenchymal cells. Spindle cell rhabdomyosarcoma is a variant of embryonal rhabdomyosarcoma. The vast majority of these two pathological types occur in children, with only a few adult cases reported to date, and both are associated with poor clinical outcomes. We herein report a case involving a man in his early 40s with spindle cell embryonal rhabdomyosarcoma of the prostate. His chief complaint was difficult urination. The diagnosis was confirmed by puncture biopsy of the prostate, and pelvic lymph node metastasis was already present at the time of diagnosis. The patient underwent three courses of chemotherapy. However, his response to the treatment was very poor, and he died of the disease 4 months after diagnosis.

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Spindle cell embryonal rhabdomyosarcoma of the prostate in an adult patient – case report and review of clinicopathological features

Cited by 13 publications

References 23 publications

Spindle cell rhabdomyosarcoma in the adult

Spindle cell rhabdomyosarcoma in the adult

Diagnosis and treatment of rhabdomyosarcomas

Spindle cell embryonal rhabdomyosarcoma of the prostate in an adult patient: a case report and review of the literature

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