Patients with limited or sinonasal gpa, would not fulfill the american college of rheumatology criteria 1990 classification criteria for gpa. It is an aggressive malignancy associated with poor outcomes, and no consensus. Sarcomas comprise a diverse group of soft tissue mesenchymal malignancies. There are invaginations of benign epithelium with squamous metaplasia. Rhabdomyosarcoma, although uncommon, is still the most common sinonasal sarcoma, 29, 30, 31 with a slight female to male predilection 1. The separation and classification of epithelial or neuroepithelial tumours is sometimes challenging, especially when treatment and prognosis are different. Undifferentiated malignant neoplasms of the sinonasal.
It is commonly described as one of the small, round, blue cell tumours of childhood due to its appearance on an. Singla a, garg p, gupta n 2016 granulomatosis with polyangiitis of sinonasal tract. Pdf rhabdomyosarcoma is an aggressive malignant soft tissue tumor that arises from primitive striated muscle cells. 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. The nasal cavity mass shows a cellular spindled proliferation with areas showing a herringbone pattern.
Adult onset sinonasal rhabdomyosarcoma a rare case. 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. This report describes two cases of alveolar rhabdomyosarcoma of the nasal cavity with unusual histological appearances mimicking clear cell carcinoma. Kaplanmeier analysis was used to assess and compare survival. Biphenotypic sinonasal sarcoma radiology reference. Alveolar sinonasal rhabdomyosarcoma was most common 66.
It is an aggressive malignancy associated with poor outcomes, and no consensus has been reached on the optimal therapeutic strategy. The sinuses and nasal region are a relatively rare site of sarcomas. The tumor is usually considered in pediatric patients in contrast to adults, where the diagnosis is more common 2, 18. 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. Request pdf management of paediatric sinonasal rhabdomyosarcoma rhabdomyosarcoma is the commonest malignant tumour of the nose and paranasal sinuses in. Small round blue cell tumors of the sinonasal tract. Biphenotypic sinonasal sarcoma bsns is a rare, slowgrowing soft tissue sarcoma of the sinonasal tract, typically presenting with nonspecific obstructive nasal symptoms. Biopsies are often limited, and tumours can share several overlapping morphological and immunophenotypic features. Authors are requested to submit articles directly to online manuscript submission system of respective journal.
Keywords nasal cavity paranasal sinus neoplasms pathology rhabdomyosarcoma, alveolar adult. In the sinonasal region, rhabdomyosarcoma constitutes a clinically important group because of. Rhabdomyosarcoma of the paranasal sinuses initially. 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. Infants less than 1 year of age comprise 10% to 15% of all cases.
Pediatric sinonasal rhabdomyosarcoma psr is a rare condition, with a limited number of previously reported cases. Pediatric sinonasal rhabdomyosarcoma rms is an aggressive and rare malignancy. Malignant neuroectodermal tumor commonly located at superior aspect of nasal cavity showing neuroblastic differentiation elnaggar. Undifferentiated malignant neoplasms of the sinonasal tract. Regarding histopathological subtype of rhabdomyosarcoma, eight patients had embryonal 50%, five had alveolar 31%, two had botryoid. Sinonasal tract snt alveolar rhabdomyosarcoma arms are frequently misdiagnosed, especially in adults. So a high suspicion and regular follow up should be done to look for other organ involvement table 1. 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. Sinonasal undifferentiated carcinoma of the maxillary sinus. Management of paediatric sinonasal rhabdomyosarcoma. Clear cell rhabdomyosarcoma of the nasal cavity and.
Rhabdomyosarcoma, or rms, is an aggressive and highly malignant form of cancer that develops from skeletal muscle cells that have failed to fully differentiate. Magnetic resonance imaging versus computed tomography. Other sites include the nasal passages, paranasal sinuses, mouth, pharynx. Neoplasms heretofore unclassified andor previously lumped under the rubric of another defined sinonasal tract neoplasm are now. Biphenotypic sinonasal sarcoma see comment and synoptic report comment. Metastatic disease occurs to cervical lymph nodes and to distant. Sinonasal polyps can have four distinct histological subtypes, which include eosinophilic polyp, chronic inflammatory polyp, hyperplastic polyp, and polyp with stromal atypia. Sinonasal tract alveolar rhabdomyosarcoma in adults lester dr. These differences include the thin, less specialized appearance of the. There was no statistical difference in 5year overall survival between alveolar and embryonal subtypes 30. Biphenotypic sinonasal sarcoma sns is a locally aggressive tumour that occurs in the sinonasal region. Pax3maml3 has recently been identified as a recurrent fusion gene event in this entity.
Pathology of the nasal cavity mass was consistent with alveolar rms. In the sinonasal region, rhabdomyosarcoma constitutes a clinically important group because of the difficulty of surgical resection and its generally poor prognosis. The tumor exhibits both neural and myogenic differentiation and thus can be problematic to diagnose histologically as elements can closely resemble other tumors e. In this study we sought to characterize the molecular profile of a large series of cases.
It furthers our understanding of the epidemiological, clinical, radiological, and molecular features of sinonasal tumors. 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. Sinonasal undifferentiated carcino mas are usually large. 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. 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.
A diagnosis of sinonasal undifferentiated carcinoma snuc was reached. Rhabdomyosarcoma is an aggressive malignant tumor often developing in the head and neck in children. Rhabdomyosarcoma is the most common sinonasal malignancy seen in children. Pdf rhabdomyosarcoma of nose, nasopharynx and paranasal.
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. Sinonasal tract alveolar rhabdomyosarcoma in adults. Fortythree percent of cases occur in children younger than 5 years, 70% before the age of 12 years. Sinonasal malignancies characterised by cells with a round to rhabdoid morphology offer a diagnostic challenge. Clinicopathologic traits and prognostic factors associated.
Sinonasal rhabdomyosarcoma in children and young adults. Pathology outlines sinonasal undifferentiated carcinoma. The histopathology report was alveolar rhabdomyosarcoma. Treatment, outcomes, and demographics in sinonasal sarcoma. The patient was referred to the head and neck surgery department of a local hospital where contrastenhanced computed tomography was performed.
The alveolar type mainly affects children over 5 years of age, adolescents and young adults. Rhabdomyosarcoma is a malignant tumor that originates from striated skeletal muscle, rarely occurring in the head and neck region. Fusion gene profile of biphenotypic sinonasal sarcoma. Data were analyzed for demographics, treatment type, stage, and histopathologic type. 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. 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. Due to its aggressive growth and its deepseated locations, alveolar rhabdomyosarcomas are often harder to treat effectively in.
To describe patient demographics, histology, treatment modalities, and survival associated with adult sinonasal rhabdomyosarcoma. The national cancer database was queried for the period from 2004 to 20 for all cases of malignant sinonasal rms in the pediatric population. It is generally considered to be a disease of childhood, as the vast majority of cases occur in those below the age of 18. Squamous cell carcinoma, keratinizing or nonkeratinizing and, usually, the poorly differentiated type need to be. The patient initially responded well to chemotherapy, but subsequently. From the department of pathology and laboratory medicine. The differential diagnosis of sinonasalnasopharyngeal. An impression of round cell tumour was given and histopathological examination was advised. Addendum andor comments on tissuepathology report c. All submissions of the em system will be redirected to online manuscript submission system. Rhabdomyosarcomas comprise a relatively common diagnostic entity among.
Introduction rhabdomyosarcoma rms is a highly malignant tumor and most common softtissue sarcoma of. The spindle cells are low grade with slender to ovoid nuclei and inconspicuous nucleoli. Granulomatosis with polyangiitis of sinonasal tract. Malignant neoplasms of the sinonasal tract encompass a wide variety of epithelial, lymphoid and mesenchymal tumours.
Adult onset sinonasal rhabdomyosarcoma malaysian journal of. Nasal rhabdomyosarcoma, pediatric age, case report, oman about 58% of childhood tumours are rhabdomyosarcomas rm. Sinonasal undifferentiated carcinoma is a rare tumor, with fewer than 100 reported cases. 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. Introduction rhabdomyosarcoma rms is a highly malignant tumor and most common softtissue sarcoma of the head and neck in childhood 1.
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