Rhabdomyosarcoma of nose, nasopharynx and paranasal sinuses

Abstract

Rhabdomyosarcoma is an aggressive malignant soft tissue tumor that arises from primitive striated muscle cells called rhabdomyoblasts. It is the most common soft tissue malignant tumor in children and accounts for about 7-8% of childhood cancers. It can arise from almost anywhere in the body but it is most common in the head and neck region. This article presents six cases of rhabdomyosarcoma involving the nose, nasopharynx and the paranasal sinuses four of which were treated with a combination of radiotherapy and chemotherapy and two were treated with medial maxillectomy combined with chemotherapy and radiotherapy.

Linicopathological analysis of head and neck rhabdomyosarcoma: A series of 10 cases and literature review head and neck rhabdomyosarcoma

“…Microscopically, RMS can be classified into different histologic subtypes, and as shown in our study, the most common is the embryonal subtype (EMB), accounting for 60% of all cases, characterized by undifferentiated, small, round and hypercromatic cells with variable number of strap or tadpole-shaped, eosinophilic rhabdomyoblasts ( 11 , 20 ). Alveolar subtype (ALV) represents approximately 30% of the cases, and it is characterized by small round rhabdomyoblasts arranged in nests separated by connective tissue trabeculae and focal areas of alveolar architecture with hypercromatic nuclei and eosinophilic cytoplasm ( 11 , 15 , 16 ).…”

“…Other complains may also be observed, including proptosis, nasal stuffiness and nasal discharge( 15 , 19 - 24 ). In addition, as observed in one case of our series where an infectious lesion was initially clinically considered, misdiagnosis may also occur, potentially leading to an incorrect therapeutic approach and significant delay to achieve the correct diagnosis ( 20 , 25 ).…”

Nasopharyngeal Rhabdomyosarcoma: A Rare Malignancy Incidentally Found in a Middle-Aged Male with a Diagnostic Dilemma

“…13 Microscopically, the embryonal type shows increased cellularity, containing several undifferentiated mesenchymal cells and the presence of myxomatous changes. 4 In contrast, the alveolar type shows small circular rhabdomyoblasts, arranged in nests or cards, separated by connective tissue trabeculae and focal locations of alveolar architecture. 4 Metastasis via intracranial spread or to distant locations is the most common cause of death.…”

“…*Muhammad Sohaib Asghar, 1 Mariam Amir, 1 Hiba Shariq, 1 Narmin Khan, 1 Maira Hassan, 2 Rumael Jawed, 2 Uzma Rasheed, 2 Faran Khalid 1 and paranasal sinuses of the paediatric population. 4 Incidence per annum of RMS in children is reported at 4.3 cases per million. 1 It follows a bimodal distribution in the general population, with peak occurrences between 2 and 4 years and 12 and 16 years, respectively.…”

“…3 Furthermore, its occurrence in adults in the head and neck area is extremely rare. 4 RMS of the head and neck region is divided into three perceptible groups: the orbital group, parameningeal group, and other head and neck location group. 7 Among the parameningeal group, the nasopharynx is the most commonly reported site.…”