Embryonal Rhabdomyosarcoma (Botryoid Subtype) Affecting the Buccal Mucosa

Cunha, John Lennon Silva; Lima-Júnior, Francisco De Assis Almeida; Júnior, Wilson Déda Gonçalves; Santos, Thiago de Santana; Silveira, Éricka Janine Dantas da; Sousa, Sílvia Ferreira de; Albuquerque‐Júnior, Ricardo Luiz Cavalcanti de

doi:10.1007/s12105-018-0957-8

Cited by 14 publications

(8 citation statements)

References 21 publications

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“…Because mucoceles are hollow structures, multilocular botryoid proliferation into the luminal space is a logical pattern of fibrovascular proliferation and would result in spherical inclusions within the mucocele wall and lumen. Similar botryoid proliferation has been described in canine and human rhabdomyosarcomas 16,17 . It remains unclear why they were present in such a large number in this case.…”

Section: Discussionsupporting

confidence: 81%

An incidental finding of numerous hypocellular nodules within a mandibular–sublingual gland complex sialocoele in a dog

Davenport

Thompson

Cazzini

et al. 2022

Vet Record Case Reports

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An 11-month-old female Labrador Retriever presented with a 3-month history of a progressively enlarging, unilateral, ventral mandibular swelling. Cytology was consistent with saliva, and the dog was referred for further investigations. Computed tomographic (CT) imaging was performed, and findings were suggestive of a leftsided mandibular sialocoele. The dog underwent sialoadenectomy surgery of the left mandibular-sublingual gland complex. On incising the thick capsule of the sialocoele, the swelling was incidentally found to contain hundreds of 1.5-2 mm firm, spherical structures. Cytological and histological examination of the structures demonstrated that these were non-mineralised, hypocellular, proteinaceous nodules. The nodules were not able to be identified on review of the pre-operative CT scan. To the authors' knowledge, this is the first report of such radiolucent, proteinaceous nodules being present within a canine sialocoele. BACKGROUNDSialocoeles are the most commonly presenting disease of the salivary glands of dogs. 1 This case report describes a case of a canine sublingual-mandibular sialocoele, with concurrent radiolucent, proteinaceous, hypocellular, nodules present within. Computed tomographic (CT) imaging has been considered the most sensitive imaging modality for diagnosing and differentiating salivary gland disease, 2 although sialolithiasis may be diagnosed by plain radiography due to the mineral component found in the sialoliths. [2][3][4][5] In the presented case, the numerous nodules were not visible on the CT scan and found incidentally at surgery. Cytological and histological analyses revealed no mineral component to the nodules. To the authors' knowledge, this is the first case report describing the presence of radiolucent, non-mineralised nodules found within a canine siaocoele. CASE PRESENTATIONAn 11-month-old, female entire, Labrador Retriever presented to the referral hospital with a 3-month history of a progressive, soft, non-painful swelling in the left ventral mandibular region. The patient had no other significant medical history and was noted to be fed a raw diet. No trauma or known inciting cause had occurred prior to the owner noticing the mass, and the patient had no other associated clinical signsThis is an open access article under the terms of the Creative Commons Attribution License, which permits use, distribution and reproduction in any medium, provided the original work is properly cited.

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Section: Discussionsupporting

confidence: 81%

An incidental finding of numerous hypocellular nodules within a mandibular–sublingual gland complex sialocoele in a dog

Davenport

Thompson

Cazzini

et al. 2022

Vet Record Case Reports

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“…Grossly and histopathologically, botryoid fibroepithelial polyps may be indistinguishable from botryoid embryonal rhabdomyosarcoma. Hence, their differential diagnosis includes, amongst other lesions, embryonal rhabdomyosarcoma [ 44 ], as well as other entities, based on the anatomic location of the lesion [ 35 , 45 ].…”

Section: Discussionmentioning

confidence: 99%

DICER1-Mutated Botryoid Fibroepithelial Polyp of the Parotid Duct: Report of the First Case

Erber

Preidl

Stoehr

et al. 2021

Head and Neck Pathol

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DICER1, a member of the ribonuclease III family, is involved in the biogenesis of microRNAs and, hence, it influences gene expression regulation. DICER1 germline (associated with the inherited DICER1 syndrome) or somatic mutations have been linked to tumorigenesis in histogenetically diverse benign and malignant neoplasms in different organs including pleuropulmonary blastoma, cystic nephroma, embryonal rhabdomyosarcoma, nasal chondromesenchymal hamartoma, poorly differentiated thyroid carcinoma, thyroblastoma, intracranial sarcoma and gonadal Sertoli-Leydig cell tumors in addition to others. Moreover, rare botryoid (giant) fibroepithelial polyps may harbor this mutation. Herein, we describe the first reported case of a DICER1-mutated botryoid fibroepithelial polyp occurring within the parotid duct of a 65-year-old female who has no other features or family history of the DICER1 syndrome. Based on its distinctive morphology, we tested this lesion specifically for DICER1 mutations and confirmed the presence of a pathogenic DICER1 variant with a low allele frequency, consistent with a somatic mutation.

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“…The microscopic differential diagnosis of OFM is wide and includes soft tissue myxoma, NSM, myxoid solitary fibrous tumor, fibrous hyperplasia with myxomatous degeneration, plexiform neurofibroma, and odontogenic myxoma 1,3,4 . Fibrous hyperplasia is the most common reactive process in the oral cavity; it is often associated with local irritation 41 and can usually present with myxoid degeneration 1,4 . In general, to distinguish a true OFM from fibrous hyperplasia with myxoid degeneration can be difficult.…”

Section: Discussionmentioning

confidence: 99%

Oral focal mucinosis: A multi‐institutional study and literature review

et al. 2020

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Background: Oral focal mucinosis (OFM) is a rare benign condition of unknown etiology, considered the oral counterpart of cutaneous focal mucinosis. We report the clinicopathologic features of 21 cases of OFM in conjunction with a review of the literature. Methods: Clinical data were collected from the records of five oral and maxillofacial pathology services. All cases were evaluated by hematoxylin and eosin staining, histochemistry, and immunohistochemistry (vimentin, S-100, α-SMA, CD34, and mast cell). Results: The series comprised 14 females (66.7%) and seven males (33.3%), with a mean age of 48.2 ± 20.7 years (range: 8-77 years) and a 2:1 female-to-male ratio. Most of the lesions affected the gingiva (n = 6, 28.6%) and presented clinically as asymptomatic sessile or pedunculated nodules with fibrous or hyperplasic appearance. All cases were negative for S-100 protein, CD34, and α-SMA and positive for Alcian blue staining. Conservative surgical excision was the treatment in all cases, and there was only one recurrence. Conclusion: OFM is a rare benign disorder that is often clinically misdiagnosed as reactive lesions or benign proliferative processes. Dermatologists and pathologists should consider OFM in the differential diagnosis of soft tissue lesions in the oral cavity, mainly located in the gingiva. K E Y W O R D S connective tissue diseases, diagnosis, oral focal mucinosis, oral pathology 1 | INTRODUCTION Oral focal mucinosis (OFM) is considered to be the oral counterpart of cutaneous focal mucinosis (CFM) that likely develops due to an overproduction of hyaluronic acid by fibroblasts. 1-3 As a result, a well-demarcated myxomatous tissue positive for Alcian blue (pH 2.5) is observed microscopically; which may mimic other oral myxoid lesions, such as soft-tissue myxoma, fibrous hyperplasia with myxoid degeneration, nerve sheath myxoma (NSM), myxoid neurofibroma, and mucocele. 1

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Embryonal Rhabdomyosarcoma (Botryoid Subtype) Affecting the Buccal Mucosa

Cited by 14 publications

References 21 publications

An incidental finding of numerous hypocellular nodules within a mandibular–sublingual gland complex sialocoele in a dog

An incidental finding of numerous hypocellular nodules within a mandibular–sublingual gland complex sialocoele in a dog

DICER1-Mutated Botryoid Fibroepithelial Polyp of the Parotid Duct: Report of the First Case

Oral focal mucinosis: A multi‐institutional study and literature review

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