We report a unique case of botryoid-type embryonal RMS of the proximal and mid ureter in a pregnant 29-year-old woman. The ureteral polyp consisted of a malignant small blue round cell tumor with a myxoid background and contained evidence of foci of immature cartilage and aggregates of epithelial cells reminiscent of hair follicle. Immunohistochemical stains for myogenin and desmin confirmed skeletal muscle, or rhabdomyoblastic, differentiation. The compact epithelial cell fragments reminiscent of hair follicle differentiation were positive for p40. Treatment included 6 cycles of adjuvant chemotherapy (vincristine, actinomycin and cyclophosphamide (VAC). No recurrent or metastatic disease was identified post-surgery.
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http://dx.doi.org/10.1016/j.eucr.2023.102388 | DOI Listing |
Indian J Otolaryngol Head Neck Surg
December 2024
Dept of ENT and Head & neck surgery, Kasturba Medical College, Mangalore Manipal Academy of Higher Education, Manipal, India.
Embryonal rhabdomyosarcoma is the malignant mesenchymal proliferation of undifferentiated skeletal muscle. It is the most common soft tissue tumour found in children. Head and neck location accounts for 40% of the cases with the most common variants being of embryonal Nasopharyngeal RMS is a type of parameningeal RMS (arising from tissues adjacent to the meninges) having propensity for CNS and known for having a poorer prognosis, early recurrence, rapid growth and invasion to adjacent structures.
View Article and Find Full Text PDFAm J Surg Pathol
August 2024
Department of Pathology & Laboratory Medicine, Memorial Sloan Kettering Cancer Center.
Embryonal rhabdomyosarcoma (ERMS) is the most common subtype of RMS, occurring in soft tissue and visceral sites of young children, and is associated with favorable outcomes. A subset occurs in mucosal-lined luminal structures, displaying a unique grape-like growth termed as "botryoid-type." To further delineate the differences between conventional (cERMS) and botryoid-type (bERMS) RMS, we performed a comparative histologic review and comprehensive molecular profiling of 48 cases (25 bERMS and 23 cERMS).
View Article and Find Full Text PDFJ Oral Maxillofac Pathol
July 2023
Department of Oral Pathology and Microbiology, Sri Ramakrishna Dental College and Hospital, Coimbatore, Tamil Nadu, India.
Rhabdomyosarcoma (RMS) is the most common sarcoma among children and accounts for 20% of soft tissue sarcomas. In children, close to 50% of rhabdomyosarcomas arise in the head and neck. RMS of the oral cavity is rare and is seen in only 10-12% of all head and neck lesions and the involvement of the jaws is extremely rare.
View Article and Find Full Text PDFGenes Chromosomes Cancer
December 2023
Department of Pathology, Institut Bergonie, Bordeaux, France.
DICER1-mutated rhabdomyosarcoma is a rare, emerging entity with a predilection for the gynecologic and genitourinary tracts. We report here a case of DICER1-mutated rhabdomyosarcoma of the ovary in a 14 years old girl which displayed interspersed mature teratoid glands, neuroectodermal rosettes and immature blastematous-like tubes. Morphologically the sarcomatous component predominated, corresponding to a high grade spindle cell rhabdomyosarcoma with botryoid features.
View Article and Find Full Text PDFRom J Morphol Embryol
May 2023
Department of Radiology, Emergency County Hospital, Sibiu, Romania; Department of Surgical Oncology, Institut Jules Bordet, Université Libre de Bruxelles, Brussels, Belgium;
Background: Uterine embryonal rhabdomyosarcoma (uERMS) in adult women is a very rare malignant entity. The study aim was to report a case of adult uERMS and to discuss the implications of histopathological diagnosis on the treatment and prognosis.
Case Presentation: We present here the clinicopathological features of a uERMS case in an adult woman.
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