Sinonasal rhabdomyosarcoma histopathology pdf

Biphenotypic sinonasal sarcoma see comment and synoptic report comment. The scan revealed significant destruction of the medial and lateral walls of the maxillary sinus as well as of the left orbital floor. Introduction rhabdomyosarcoma rms is a highly malignant tumor and most common softtissue sarcoma of. The differential diagnosis of sinonasalnasopharyngeal. Request pdf management of paediatric sinonasal rhabdomyosarcoma rhabdomyosarcoma is the commonest malignant tumour of the nose and paranasal sinuses in. Other sites include the nasal passages, paranasal sinuses, mouth, pharynx. The authors described 28 cases of an infiltrative, cellular spindle cell neoplasm with uniform nuclear features, which were negative for synovial sarcoma fusion transcripts. Rhabdomyosarcoma of the paranasal sinuses initially. Data were analyzed for demographics, treatment type, stage, and histopathologic type. Pathology of sinonasal tumors and tumorlike lesions. Who classification of head and neck tumours, 4th edition, 2017 thought to arise from olfactory membrane or olfactory placode platelike thickening of embryonic ectoderm from which a nerve ganglion or sensory organ will develop which extends from. Biphenotypic sinonasal sarcoma bsns is a rare lowgrade sarcoma first described by lewis et al 1 in 2012 as lowgrade sinonasal sarcoma with neural and myogenic differentiation.

Sinonasal undifferentiated carcino mas are usually large. All submissions of the em system will be redirected to online manuscript submission system. To describe patient demographics, histology, treatment modalities, and survival associated with adult sinonasal rhabdomyosarcoma. Pdf rhabdomyosarcoma is an aggressive malignant soft tissue tumor that arises from primitive striated muscle cells. Biphenotypic sinonasal sarcoma bsns is a rare, slowgrowing soft tissue sarcoma of the sinonasal tract, typically presenting with nonspecific obstructive nasal symptoms. Pathology of the nasal cavity mass was consistent with alveolar rms. Mri with its superior soft tissue contrast and multiplanar capability is superior to ct in pretreatment evaluation of primary malignant tumors of sinonasal cavity.

Biphenotypic sinonasal sarcoma sns is a locally aggressive tumour that occurs in the sinonasal region. Adult onset sinonasal rhabdomyosarcoma malaysian journal of. There was no statistical difference in 5year overall survival between alveolar and embryonal subtypes 30. Alveolar sinonasal rhabdomyosarcoma was most common 66. It furthers our understanding of the epidemiological, clinical, radiological, and molecular features of sinonasal tumors. The tumor is usually considered in pediatric patients in contrast to adults, where the diagnosis is more common 2, 18. Introduction rhabdomyosarcoma rms is a highly malignant tumor and most common softtissue sarcoma of the head and neck in childhood 1. Keywords nasal cavity paranasal sinus neoplasms pathology rhabdomyosarcoma, alveolar adult. Treatment, outcomes, and demographics in sinonasal sarcoma.

Regarding histopathological subtype of rhabdomyosarcoma, eight patients had embryonal 50%, five had alveolar 31%, two had botryoid. Malignant melanoma sinonasal melanoma is an uncommon tumor constituting about 5% of all neoplasms of the sinonasal tract and less than 5% of all melanomas. Undifferentiated malignant neoplasms of the sinonasal. Due to its aggressive growth and its deepseated locations, alveolar rhabdomyosarcomas are often harder to treat effectively in. Malignant neuroectodermal tumor commonly located at superior aspect of nasal cavity showing neuroblastic differentiation elnaggar. Pediatric sinonasal rhabdomyosarcoma psr is a rare condition, with a limited number of previously reported cases. From the department of pathology and laboratory medicine. Nasal rhabdomyosarcoma, pediatric age, case report, oman about 58% of childhood tumours are rhabdomyosarcomas rm. Pdf rhabdomyosarcoma of nose, nasopharynx and paranasal. There are invaginations of benign epithelium with squamous metaplasia. Biopsies are often limited, and tumours can share several overlapping morphological and immunophenotypic features. The patient initially responded well to chemotherapy, but subsequently. The spindle cells are low grade with slender to ovoid nuclei and inconspicuous nucleoli.

So a high suspicion and regular follow up should be done to look for other organ involvement table 1. Sinonasal tract snt alveolar rhabdomyosarcoma arms are frequently misdiagnosed, especially in adults. The alveolar type mainly affects children over 5 years of age, adolescents and young adults. It is generally considered to be a disease of childhood, as the vast majority of cases occur in those below the age of 18. Biphenotypic sinonasal sarcoma radiology reference. It is an aggressive malignancy associated with poor outcomes, and no consensus has been reached on the optimal therapeutic strategy.

Squamous cell carcinoma, keratinizing or nonkeratinizing and, usually, the poorly differentiated type need to be. Metastatic disease occurs to cervical lymph nodes and to distant. Sinonasal rhabdomyosarcoma in children and young adults. Fusion gene profile of biphenotypic sinonasal sarcoma. This report describes two cases of alveolar rhabdomyosarcoma of the nasal cavity with unusual histological appearances mimicking clear cell carcinoma. These differences include the thin, less specialized appearance of the. Prognosis is poor, despite aggressive treatment, with a median survival of less than 18 months and a 5 year survival of less than 20% am j surg pathol 2002. Thus, it is critical in primitive appearing and nonkeratinizing squamous cell carcinomas of the sinonasal tract, that a muscle marker, such as desmin, myogenin or myod1, be performed in order to exclude the possibility of a cytokeratin positive rhabdomyosarcoma, 18. Neoplasms heretofore unclassified andor previously lumped under the rubric of another defined sinonasal tract neoplasm are now. Granulomatosis with polyangiitis of sinonasal tract. Adult onset sinonasal rhabdomyosarcoma a rare case. Sarcomas comprise a diverse group of soft tissue mesenchymal malignancies.

Infants less than 1 year of age comprise 10% to 15% of all cases. Botryoid embryonal rhabdomyosarcoma sarcoma botryoides only occurs in certain locations, specifically beneath a mucosal epithelial lined viscera, such as the bladder, biliary tract, vagina or upper respiratory tract, extrahepatic bile ducts or near a space. Singla a, garg p, gupta n 2016 granulomatosis with polyangiitis of sinonasal tract. The separation and classification of epithelial or neuroepithelial tumours is sometimes challenging, especially when treatment and prognosis are different. Sinonasal malignancies characterised by cells with a round to rhabdoid morphology offer a diagnostic challenge. Authors are requested to submit articles directly to online manuscript submission system of respective journal. Sinonasal undifferentiated carcinoma is a rare tumor, with fewer than 100 reported cases.

Patients with limited or sinonasal gpa, would not fulfill the american college of rheumatology criteria 1990 classification criteria for gpa. An impression of round cell tumour was given and histopathological examination was advised. Fortythree percent of cases occur in children younger than 5 years, 70% before the age of 12 years. It is an aggressive malignancy associated with poor outcomes, and no consensus. Rhabdomyosarcoma is a malignant tumor that originates from striated skeletal muscle, rarely occurring in the head and neck region. Adult onset sinonasal rhabdomyosarcoma a rare case report with cytohistological features neelam sood md pathology and nisha sehrawat md pathology department of pathology and laboratory medicine, deen dayal upadhyay hospital, new delhi abstract rhabdomyosarcoma rms is a fast growing, malignant tumour arising from immature mesenchymal. Rhabdomyosarcomas comprise a relatively common diagnostic entity among. In this study we sought to characterize the molecular profile of a large series of cases. Pathology outlines sinonasal undifferentiated carcinoma. The patient was referred to the head and neck surgery department of a local hospital where contrastenhanced computed tomography was performed. Sinonasal undifferentiated carcinoma of the maxillary sinus. Clinicopathologic traits and prognostic factors associated. Kaplanmeier analysis was used to assess and compare survival.

Clear cell rhabdomyosarcoma of the nasal cavity and. Pax3maml3 has recently been identified as a recurrent fusion gene event in this entity. Rhabdomyosarcoma is the most common sinonasal malignancy seen in children. Alveolar rhabdomyosarcoma is a relatively uncommon malignancy of the sinonasal tract, encountered in the setting of a primitive small round blue cell neoplasm 3, 617. Undifferentiated malignant neoplasms of the sinonasal tract. Sinonasal tract alveolar rhabdomyosarcoma in adults. Small round blue cell tumors of the sinonasal tract. Biphenotypic sinonasal sarcoma, initially named lowgrade sinonasal sarcoma with neural and myogenic features, is a rare and only recently described lowgrade sarcoma of the nasal and paranasal sinuses. Pediatric sinonasal rhabdomyosarcoma rms is an aggressive and rare malignancy.

Head and neck equivalent terms and definitions c000c148, c300c339, c410, c411, c442, c479 excludes lymphoma and leukemia m9590 m9992 and kaposi sarcoma m9140 jump to. In the sinonasal region, rhabdomyosarcoma constitutes a clinically important group because of the difficulty of surgical resection and its generally poor prognosis. The sinonasal tract is host to numerous benign and malignant entities that can pose diagnostic challenges to pathologists as a result of limited exposure in daily practice. The national cancer database was queried for the period from 2004 to 20 for all cases of malignant sinonasal rms in the pediatric population.

The tumor exhibits both neural and myogenic differentiation and thus can be problematic to diagnose histologically as elements can closely resemble other tumors e. It is commonly described as one of the small, round, blue cell tumours of childhood due to its appearance on an. In the sinonasal region, rhabdomyosarcoma constitutes a clinically important group because of. Rhabdomyosarcoma is an aggressive malignant tumor often developing in the head and neck in children. Rhabdomyosarcoma, or rms, is an aggressive and highly malignant form of cancer that develops from skeletal muscle cells that have failed to fully differentiate. Addendum andor comments on tissuepathology report c.

Sinonasal polyps can have four distinct histological subtypes, which include eosinophilic polyp, chronic inflammatory polyp, hyperplastic polyp, and polyp with stromal atypia. A diagnosis of sinonasal undifferentiated carcinoma snuc was reached. Sinonasal tract alveolar rhabdomyosarcoma in adults lester dr. Magnetic resonance imaging versus computed tomography. There was a significant statistical difference in the prevalence of a ccp between ip and malignant sinonasal tumors with the overall sensitivity, specificity, positive predictive value, negative predictive value, and accuracy 100%, 87%, 64%, 100%, and 89%, respectively. The nasal cavity mass shows a cellular spindled proliferation with areas showing a herringbone pattern. Management of paediatric sinonasal rhabdomyosarcoma. The histopathology report was alveolar rhabdomyosarcoma.