Alveolar rhabdomyosarcoma of paranasal sinus with cervical metastasis-a case report

Nayak, Dipak Ranjan; Balakrishnan, R.; Shahab, A. S.; Rao, Laxmi

doi:10.1007/bf03000338

Cited by 2 publications

(1 citation statement)

References 4 publications

Supporting

Mentioning

Contrasting

Order By: Relevance

“…It mostly occurs in adolescents aged 10–15 years and is characterized by more aggressive behaviors and a worse prognosis than other sarcomas, prone to lymphatic and hematogenous metastasis in the early stage as well as metastasizing to breast, testis, pancreas, and other parts ( 3 , 4 ). ARMS often occurs in the limbs and genitourinary system, however, head and neck ARMS are relatively rare when involving the nasal cavity or sinuses ( 5 , 6 ).…”

Section: Introductionmentioning

confidence: 99%

Multimodality imaging evaluation of nasal sinus alveolar rhabdomyosarcoma: Two case reports

et al. 2022

View full text Add to dashboard Cite

BackgroundRhabdomyosarcoma (RMS) is a common pleomorphic malignant soft tissue sarcoma in children and adolescents that originates from rhabdomyoblasts or mesenchymal precursor cells. Alveolar rhabdomyosarcoma (ARMS) mostly occurs in adolescents aged 10–15 years and is characterized by more aggressive behaviors and worse prognosis than other sarcomas, prone to lymphatic and hematogenous metastasis in the early stage as well as metastasizing to breast, testis, pancreas, and other parts. ARMS often occurs in the limbs and genitourinary system, however, head and neck ARMS are relatively rare when involving the nasal cavity or sinuses. The role of MRI and 18F-FDG positron emission tomography combined with computed tomography (PET/CT) remains to be established in ARMS.Case reportCase 1: An 18-year-old male was found with a left submandibular mass of approximately 1 cm in diameter 2 months ago, which gradually increased in size. CT showed multiple soft tissue masses in maxillofacial and neck regions and the lesions invaded the frontal lobe and the inner wall of the left orbital lobe. MRI showed the masses with hypointensity on T1WI, hyperintensity on T2WI, and diffusion-weighted imaging (DWI) with significant enhancement. 18F-FDG PET/CT showed multiple hypermetabolic lesions located in the maxillofacial, neck region, 3rd lumbar vertebra, and the right sacrum. A nasal endoscopic tumor biopsy and molecular testing finally helped to diagnose the ARMS. Case 2: A 14-year-old male presented with left maxillary pain with nasal congestion and left ocular swelling 15 days ago. CT demonstrated a soft tissue mass in the nasal cavity and sinuses with local protrusion into the left orbit. MRI showed the masses with a slightly low signal on T1WI, a high signal on T2WI, and DWI with significant heterogenous enhancement. 18F-FDG PET/CT showed hypermetabolic lesions in the left maxillofacial and neck regions. ARMS was finally diagnosed by a nasal endoscopic tumor biopsy and molecular testing. The patient had a recurrence of the lesion after chemotherapy and surgical resection and is currently undergoing radiation therapy.ConclusionNasal sinus ARMS is highly malignant with a poor prognosis. Accurate diagnosis relies not only on histopathology and immunohistochemistry examination but also on genetic detection of characteristic chromosomal translocations and fusion genes. Imaging methods, such as MRI and PET/CT can accurately assess the extent of the lesions and metastases, assist in the diagnosis of the disease and the selection of treatment regimens, provide precise localization for surgery, and help with treatment monitoring and follow-up.

show abstract

Section: Introductionmentioning

confidence: 99%

Multimodality imaging evaluation of nasal sinus alveolar rhabdomyosarcoma: Two case reports

et al. 2022

View full text Add to dashboard Cite

show abstract

Solid Variant of Alveolar Rhabdomyosarcoma of Nasal Cavity

Bhattarai

Shrestha

Dhakal

2020

Kathmandu Univ. Med. J.

View full text Add to dashboard Cite

Rhabdomyosarcoma comprises about half of the soft tissue tumors. Approximately 40% of the alveolar subtype occur in the head and neck region. Patients present with unilateral nasal mass with/without lymphadenopathy and with/without orbital manifestations. Diagnosis is aided radiologically by CT scan, MRI along with biopsy and is confirmed by immunohistochemistry. Treatment involves surgical resection, chemotherapy, radiotherapy or a combination of these. In spite of treatments, however, the outcome is poor. This is a case report of 14-year-old male who presented with unilateral nasal mass with proptosis of right eye who was eventually diagnosed as a case of alveolar rhabdomyosarcoma of solid variant.

show abstract

Alveolar rhabdomyosarcoma of paranasal sinus with cervical metastasis-a case report

Cited by 2 publications

References 4 publications

Multimodality imaging evaluation of nasal sinus alveolar rhabdomyosarcoma: Two case reports

Multimodality imaging evaluation of nasal sinus alveolar rhabdomyosarcoma: Two case reports

Solid Variant of Alveolar Rhabdomyosarcoma of Nasal Cavity

Contact Info

Product

Resources

About