Orbito-Ethmoidal Rhabdomyosarcoma in an Adult Patient: A Case Report and Review of the Literature

Sepúlveda, Ilson; Spencer, M. Loreto; Cabezas, Claudia; Platino, Maria Olga; Schorwer, Max; Ortega, Pablo; Ulloa, David

doi:10.1159/000365547

Cited by 7 publications

(13 citation statements)

References 19 publications

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“…When it occurs in the nasal cavity, the nasal obstruction can be the only symptom 11 . Different histological types of rhabdomyosarcoma of head and neck region have been identified, including embryonal, alveolar, and pleomorphic rhabdomiosarcoma [2][3][4][5][6][7][8][9] . Embryonal and alveolar patterns are the most common.…”

Section: Discussionmentioning

confidence: 99%

“…Of the tumors occurring in head and neck structures, almost 40% correspond to rhabdomyosarcomas 3 . Anatomically, they are classified as parameningeal, orbital, nonparameningeal, and nonorbital; parameningeal sites include the nose, paranasal sinuses, nasopharynx, middle ear, mastoid process, infratemporal fossa, and pterygopalatine fossa 4 . In nearly 90% of cases, chromosomal translocations are appreciated in genes PAX3 (2q33) FKHR/ ALV (13q14), and less commonly in PAX7 (1p36) KHR/ALV (13q14) 5 .…”

Section: Introductionmentioning

confidence: 99%

See 1 more Smart Citation

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: Introductionmentioning

confidence: 99%

Alveolar rhabdomyosarcoma of nasal presentation: Case report

Rico-Ramírez¹,

Ornelas-Ramos²,

Abbud-Abbud³

et al. 2022

GAMO

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“…Pediatric HNRMS has an overall survival rate of 28.7% [ 16 ]. 44% percent of all HNRMS occurs in the parameningeal region: paranasal sinuses, nasal cavity, nasopharynx, middle ear, and the skull base [ 8 , 25 - 26 ].…”

Section: Introductionmentioning

confidence: 99%

Surgical Interventions for Advanced Parameningeal Rhabdomyosarcoma of Children and Adolescents

Choi¹,

Iwanaga²,

Tubbs³

et al. 2018

Cureus

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Owing to its rarity, rhabdomyosarcoma of the head and neck (HNRMS) has seldom been discussed in the literature. As most of the data is based only on the retrospective experiences of tertiary healthcare centers, there are difficulties in formulating a standard treatment protocol. Moreover, the disease is poorly understood at its pathological, genetic, and molecular levels. For instance, 20% of all histological assessment is inaccurate; even an experienced pathologist can confuse rhabdomyosarcoma (RMS) with neuroblastoma, Ewing’s sarcoma, and lymphoma. RMS can occur sporadically or in association with genetic syndromes associated with predisposition to other cancers such as Li-Fraumeni syndrome and neurofibromatosis type 1 (von Recklinghausen disease). Such associations have a potential role in future gene therapies but are yet to be fully confirmed. Currently, chemotherapies are ineffective in advanced or metastatic disease and there is lack of targeted chemotherapy or biological therapy against RMS. Also, reported uses of chemotherapy for RMS have not produced reasonable responses in all cases. Despite numerous molecular and biological studies during the past three decades, the chemotherapeutic regimen remains unchanged. This vincristine, actinomycin, cyclophosphamide (VAC) regime, described in Kilman, et al. (1973) and Koop, et al. (1963), has achieved limited success in controlling the progression of RMS. Thus, the pathogenesis of RMS remains poorly understood despite extensive modern trials and more than 30 years of studies exploring the chemotherapeutic options. This suggests a need to explore surgical options for managing the disease. Surgery is the single most critical therapy for pediatric HNRMS. However, very few studies have explored the surgical management of pediatric HNRMS and there is no standard surgical protocol. The aim of this review is to explore and address such issues in the hope of maximizing the number of options available for young patients with HNRMS.

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“…Anatómicamente se clasifican como parameníngeo, orbital, no parameníngeos y no orbitales. Los sitios paramenín-geos incluyen la nariz, los senos paranasales, la nasofaringe, el oído medio, la mastoides, la fosa infratemporal y la fosa pterigopalatina 4 . En cerca del 90% de los casos se aprecian translocaciones cromosómicas, en los genes PAX3 (2q33) y FKHR/ALV (13q14), y con menos frecuencia en el PAX7 (1p36) y el KHR/ALV (13q14)…”

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“…Se han identificado varios diferentes tipos histológicos de RMS de la región de cabeza y cuello, incluyendo embrionario, alveolar, pleomórfico [2][3][4][5][6][7][8][9] . Los patrones embrionarios y alveolares son los más frecuentes.…”

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Rabdomiosarcoma alveolar de presentación nasal: informe de caso

Rico-Ramírez¹,

Ornelas-Ramos²,

Abbud-Abbud³

et al. 2022

GAMO

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Resumen Un sarcoma es un tumor maligno procedente de células mesenquimales primitivas, que en circunstancias normales se desarrolla en los tejidos de apoyo como los músculos y los huesos. Existen tres variables del rabdomiosarcoma de acuerdo a sus presentaciones histológicas: el embrionario, el alveolar y el pleomórfico, siendo el rabdomiosarcoma alveolar el más agresivo. Se presenta en un paciente masculino de ocho años de edad, el cual inicia con elevación sin cambio de color en ala nasal izquierda de 3-4 mm, de crecimiento progresivo, diagnosticada como rabdomiosarcoma alveolar documentado por patología como tumor de células redondas, pequeñas y azules, con inmunohistoquímica positiva a desmina y miogenina. La resonancia de cráneo y cara, la tomografía computarizada de tórax y el aspirado de médula óseafueron negativos para extensión tumoral. Se efectúa resección de su lesión, la cual se resecó macroscópicamente al 100%, reportando patología focos aislados de tumor, con tumor residual microscópico en márgenes laterales, por lo que queda finalmente como E II. Se trata de una neoplasia maligna muy rara con una presentación inusual, con reporte de cuatro casos en la literatura, de ahí surge la importancia de tener en cuenta esta posibilidad diagnóstica, así como los conocimientos para su abordaje. (creativecommons.org/licenses/by-nc-nd/4.0/). Abstract A sarcoma is defined as a malignant tumor from primitive mesenchymal cells that, in normal circumstances, is developed in bone and muscular tissues. In accordance with their histological differences, three different types of rhabdomyosarcoma have been identified: embryonal, alveolar and pleomorphic, being the alveolar rhabdomyosarcoma the most aggressive of them. Male patient age 8, which starts with no color change elevation in left nasal wing of approximately 3-4 mm of progressive growth, diagnosed as alveolar rhabdomyosarcoma tumor documented as a tumor of small round blue cells, with desmin and myogenin positive immunohistochemistry. It was detected in the magnetic resonance of skull and face, in chest computed tomography and bone marrow aspirate, negative for tumor. Resection of the lesion, which is resected 100% macroscopically, reporting disease outbreaks isolated tumor with microscopic residual tumor in side margins, finally occurring as E II. This is a very rare malignancy with an unusual presentation, around 4 cases have been reported in the literature, hence the importance of taking into account this diagnostic possibility and knowledge to address them.

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Orbito-Ethmoidal Rhabdomyosarcoma in an Adult Patient: A Case Report and Review of the Literature

Cited by 7 publications

References 19 publications

Alveolar rhabdomyosarcoma of nasal presentation: Case report

Alveolar rhabdomyosarcoma of nasal presentation: Case report

Surgical Interventions for Advanced Parameningeal Rhabdomyosarcoma of Children and Adolescents

Rabdomiosarcoma alveolar de presentación nasal: informe de caso

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