Incidence trends and long-term survival analysis of sinonasal rhabdomyosarcoma

Sanghvi, Saurin; Misra, Poonam; Patel, Neal; Kalyoussef, Evelyne; Baredes, Soly; Eloy, Jean Anderson

doi:10.1016/j.amjoto.2013.04.012

Cited by 40 publications

(73 citation statements)

References 28 publications

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“…Embryonal and alveolar patterns are the most common. Histologically, embryonal rhabdomyosarcoma is comprised by primitive round spindle-shaped cells with rhabdomyoblasts, whereas alveolar rhabdomyosarcoma consists of malignant cells grouped in fibrovascular septae that form alveoli-like spacings 10 . Poorly differentiated rhabdomyosarcomas can be difficult to distinguish from poorly differentiated Ewing sarcoma or neuroblastoma.…”

Section: Discussionmentioning

confidence: 99%

“…Immunohistochemically, intermediate filaments specific to muscle cells such as myoglobin, myosin, desmin, and creatine kina- se MM isoenzyme are regarded as rhabdomyosarcoma-specific markers 12 . Microscopically, tumor cells tend to be smaller and rounded, offering a densely cellular appearance, and they are named after the similarity they show with the tiny air sacs within the lungs, and therefore, they are often included in the round, blue cells classification, where malignant lymphoma, neuroblastoma, and synovial sarcoma (biphasic) often stand out as differential diagnoses 10 . In spite of tumor slow growth, the prognosis is generally poor 11 and depends on a combination of patient age, histological nature, clinical stage, and tumor location 11 , with a high tendency toward early metastatic spread 11 .…”

Section: Discussionmentioning

confidence: 99%

“…These tumors usually occur in adolescents or young adults' lower limbs with rhabdomyosarcoma of the nasal or paranasal region even being more unusual 7,8 . Rhabdomyosarcomas are generally divided in meningeal and parameningeal sites 9 with parameningeal sites including the middle ear, nose cavity, paranasal sinuses, nasopharynx, and the infratemporal fossa 10 . There are only 4 cases reported in the literature, with this malignancy being more common in females than in males at a 2:1 ratio 8 .…”

Section: Discussionmentioning

confidence: 99%

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Alveolar rhabdomyosarcoma of nasal presentation: Case report

Rico-Ramírez¹,

Ornelas-Ramos²,

Abbud-Abbud³

et al. 2022

GAMO

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Sarcoma is a malignant tumor originating in mesenchymal primitive cells that in normal circumstances develops in supportive tissues such as muscle and bone. There are three rhabdomyosarcoma variants according to its histological presentation: Embryonal, alveolar, and pleomorphic with alveolar rhabdomyosarcoma being the most aggressive. The case is presented of an 8-year-old male patient who started with a progressively growing left nasal wing 3-4 mm mass with no color change diagnosed as an alveolar rhabdomyosarcoma with pathology documenting a small round blue cell tumor with positive immunohistochemistry to desmin and myogenin. Skull and face magnetic resonance, chest computed tomography, and bone marrow aspirate were negative for disease extension. The lesion was macroscopically resected at 100%, with pathology reporting isolated tumor foci, with microscopic residual tumor on lateral margins, and it is therefore finally classified as E II. This is a very rare neoplasm with an unusual presentation, with only 4 cases reported in the literature, hence, the importance of taking into account this diagnostic possibility as well as the knowledge on how to approach it. (creativecommons.org/licenses/by-nc-nd/4.0/).

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Section: Discussionmentioning

confidence: 99%

Section: Discussionmentioning

confidence: 99%

Section: Discussionmentioning

confidence: 99%

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Alveolar rhabdomyosarcoma of nasal presentation: Case report

Rico-Ramírez¹,

Ornelas-Ramos²,

Abbud-Abbud³

et al. 2022

GAMO

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“…Rhabdomyosarcoma, although uncommon, is still the most common sinonasal sarcoma, [29][30][31] with a slight female to male predilection (1.2:1). 32 In adults, alveolar rhabdomyosarcoma is the most common subtype in the sinonasal tract. Syndrome association (Li-Fraumeni, Costello, and neurofibromatosis type 1) may be seen in children.…”

Section: Mesenchymal Chondrosarcomamentioning

confidence: 99%

“…41 There is an overall poor prognosis of sinonasal tract alveolar rhabdomyosarcoma (5-year survival 30-40%), 29,35,[41][42][43][44] with patients frequently showing regional and/or distant metastases, although young patients (5-year survival 62.5%) tend to have a better prognosis. 28,32,43 Sinonasal Undifferentiated Carcinoma (SNUC) SNUC is a rare tumor, lacking glandular or squamous features, and is not otherwise classifiable. Thus, it is a tumor of exclusion, comprising 3-5% of all sinonasal tract carcinomas.…”

Section: Mesenchymal Chondrosarcomamentioning

confidence: 99%

Small round blue cell tumors of the sinonasal tract: a differential diagnosis approach

Thompson

2017

Modern Pathology

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One of the most challenging diagnostic categories within tumors of the sinonasal tract is the small round blue cell tumors. Biopsies are usually small and limited, resulting in considerable diagnostic difficulty for practicing surgical pathologists. These tumors share several overlapping histologic and immunophenotypic findings while also showing considerable variation within and between cases. Specific tumor site of origin, imaging findings, and clinical findings must be combined with the histology and pertinent ancillary studies if the correct diagnosis is to be reached. Discrimination between neoplasms is critical as there are significant differences in therapy and overall outcome. It is important to have a well developed differential diagnosis for this category of tumors, where each of the diagnoses is considered, evaluated, and either confirmed or excluded from further consideration. In an undifferentiated tumor, showing a small round blue cell morphology, using the mnemonic 'MR SLEEP' helps to highlight tumors to consider: melanoma, mesenchymal chondrosarcoma, rhabdomyosarcoma, sinonasal undifferentiated carcinoma, squamous cell carcinoma (including NUT carcinoma), small cell osteosarcoma, lymphoma, esthesioneuroblastoma (olfactory neuroblastoma), Ewing sarcoma/primitive neuroectodermal tumor, pituitary adenoma, and plasmacytoma. A panel of pertinent immunohistochemistry studies, histochemistries and/or molecular tests should aid in reaching a diagnosis, especially when taking the pattern and intensity of reactions into consideration.

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