Rhabdomyosarcoma, Spindle Cell/Sclerosing Variant: A Clinical and Histopathological Examination of this Rare Variant with Three New Cases from the Oral Cavity

Smith, Molly Housley; Atherton, Daniel S.; Reith, John D.; Islam, Nadim; Bhattacharyya, Indraneel; Cohen, Donald M.

doi:10.1007/s12105-017-0818-x

Cited by 26 publications

(30 citation statements)

References 18 publications

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“…Other less common variants include the pleomorphic RMS that only rarely occurs in the pediatric group and comprises about 5% of all cases diagnosed ( 11 , 17 ); the spindle cell subtype that has previously been considered a variant of the EMB, but it is now recognized as a separate subtype ( 1 ); and the botryoid variant that represents an EMB subtype with a grapelike macroscopic and histologic appearance caused by sub-epithelial tumor aggregates ( 21 ). More recently, a sclerosing RMS was also recognized ( 27 ). In our series, two cases were diagnosed as pleomorphic RMS, one case showed features consistent with the spindle cell variant and one was classified as ALV.…”

Section: Discussionmentioning

confidence: 99%

Linicopathological analysis of head and neck rhabdomyosarcoma: A series of 10 cases and literature review head and neck rhabdomyosarcoma

Ji²,

Souza³

et al. 2018

Med Oral

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BackgroundTo describe the clinicopathological characteristics of a series of head and neck rhabdomyosarcoma (RMS) and to review the literature.Material and MethodsCases diagnosed as RMS affecting the head and neck region were retrospectively retrieved from the files of two Brazilian institutions from January 2006 to January 2017. Data on clinical features (sex, age and affected site), microscopic subtype, immunohistochemical results, treatment employed and follow-up status were obtained from the patient’s medical charts.ResultsDuring the period considered, 10 cases of RMS were identified. Females predominated (4M:6F), the mean age at diagnosis was 16.5 years-old and the orbit was the most affected site (4 cases). Microscopically, most cases were classified as embryonal RMS (6 cases) and the Desmin/Myogenin/Myo-D1 immunohistochemical positivity was useful to confirm the diagnosis. Chemotherapy and radiotherapy were applied to 9 and 8 patients respectively, whereas 2 patients were treated by surgery. Recurrences occurred in 3 patients and distant metastasis in 2 cases. Nine patients were alive in their last follow-up, 3 of them with disease, whereas 1 patient died due to the disease.ConclusionsHead and neck RMS is an aggressive malignant neoplasm which demands especial concern to achieve early diagnosis and successful treatment. Key words:Rhabdomyosarcoma, soft tissue tumors, head and neck, oral cavity, chemotherapy.

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Section: Discussionmentioning

confidence: 99%

Linicopathological analysis of head and neck rhabdomyosarcoma: A series of 10 cases and literature review head and neck rhabdomyosarcoma

Ji²,

Souza³

et al. 2018

Med Oral

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“…After revised WHO classification, Spindle cell variant of RMS has been reported in oral cavity in approximately 5%-13% of the cases. 10 This rare variant should always be considered in case of an aggressive palatal swelling in adults.…”

Section: Discussionmentioning

confidence: 99%

Rare Variant of Adult Rhabdomyosarcoma Presenting as a Palatal Swelling

2021

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A 26-year-old male was referred to the Department of Oral and Maxillofacial Surgery of a tertiary care hospital in Lahore with chief complaint of painless swelling on the right palate of 40 days duration. Clinical differential diagnosis included squamous cell carcinoma, Ewing sarcoma, fibrosarcoma, neuroblastoma and rhabdomyosarcoma. Computed tomography scan revealed hypodense mass with necrotic changes. Histological examination of the excised tumor revealed malignant neoplasm arranged in fascicles and bundles comprising of spindle cells with pleomorphic, hyperchromatic nuclei and increased atypical mitosis. Immunohistochemical analysis showed negative staining with Cytokeratin, S100, CD34, Stat6, h-Caldesmon and EMA while the tumour cells were positive for desmin, myogenin, smooth muscle actin, CD-99 and MyoD1 thus confirming the diagnosis of spindle cell rhabdomyosarcoma. doi: https://doi.org/10.12669/pjms.37.3.3305 How to cite this:Iqbal HA, Anjum R, Naseem N. Rare Variant of Adult Rhabdomyosarcoma Presenting as a Palatal Swelling. Pak J Med Sci. 2021;37(3):---------. doi: https://doi.org/10.12669/pjms.37.3.3305 This is an Open Access article distributed under the terms of the Creative Commons Attribution License (http://creativecommons.org/licenses/by/3.0), which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.

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“…2 The diffuse and strong positivity for MyoD1 supports our diagnosis, as Myo-D1 has been shown to be a more sensitive marker for sclerosing variant of RMS. 10,13 Patients with S-ScRMS demonstrate heterogenic genetic alterations that may have particular importance on prognosis. 10 Recent studies focusing on SRMS-ScRMS shows a variable prognosis based on their age at diagnosis & genetic abnormalities.…”

Section: Discussionmentioning

confidence: 99%

Adult spindle cell/ sclerosing rhabdomyosarcoma of the buccal maxillary gingiva: Unique entity, a rare case report

Chopra

Jindal²,

Joseph³

et al. 2021

ACHR

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Rhabdomyosarcoma is a malignant neoplasm of mesenchymal cells, which shows varying degrees of striated muscle cell differentiation. It predominantly occurs in children while rarely found in adults. Involvement of the oral cavity accounts for only 10-12% of all head and neck cases. Herewith, we report a rare case of oral spindle cell / sclerosing rhabdomyosarcoma in a 47-year-old male presented with a small mass involving the gingiva of right upper incisor. The mass was excised with a preoperative diagnosis of gingival epulis and subjected to histopathological and immunohistochemical examination which confirmed it to be spindle cell / sclerosing rhabdomyosarcoma. Data regarding its clinical course, genetic abnormalities and prognosis as a combined subtype is scant.

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Rhabdomyosarcoma, Spindle Cell/Sclerosing Variant: A Clinical and Histopathological Examination of this Rare Variant with Three New Cases from the Oral Cavity

Cited by 26 publications

References 18 publications

Linicopathological analysis of head and neck rhabdomyosarcoma: A series of 10 cases and literature review head and neck rhabdomyosarcoma

Linicopathological analysis of head and neck rhabdomyosarcoma: A series of 10 cases and literature review head and neck rhabdomyosarcoma

Rare Variant of Adult Rhabdomyosarcoma Presenting as a Palatal Swelling

Adult spindle cell/ sclerosing rhabdomyosarcoma of the buccal maxillary gingiva: Unique entity, a rare case report

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