Retain
- A rhabdomyoma is a very rare benign tumor of soft fabrics that arises in skeletal muscles.
- These tumors are classified into 2 types according to their location: heart and extracardian. Extracardiac rhabdomyomas are particularly rare and are subdivided into fetal, juvenile and adult subtypes depending on their histological characteristics.
- Adult rhabdomyomas mainly affect people aged 40 to 70 and are more frequent in men. To date, no case of adult type rhabdomyoma in the pharyngeal region in children had been documented worldwide.
- Li Wang and his colleagues from the ENT service of the Popular Liaocheng Hospital, in the Chinese Shandong province, describe for the first time a cas In a 9 year old boy.
The patient and his history
A 9 -year -old boy was sent with a medical history of 6 months of feeling of a foreign body in his throat. This was manifested by night snoring.
Examination results
An optical fiber laryngoscopy revealed a round mass with a pedicle in the throat about 2 cm in diameter. The surface of the mass was smooth, the pedicle being rooted in the upper pole of the amygdal.
Transoral excision of the oropharynx tumor was carried out. On macroscopic examination, the mass measured 2.2 × 2.0 × 1.3 cm, was clearly delimited and presented a dark gray color with a dense and braided texture on the cutting surface.
Under the microscope, the cells had an abundant and grainy eosinophilic cytoplasm with clearly defined edges, large polygonal skeletal muscle cells and often extensive vacuolization.
Immunohistochemical coloring was positive for MSA (specific muscular actin), desmine, myod1 and myogenin, which led to a final pathological diagnosis of adult rhabdomyoma.
Treatment
The postoperative period took place without complications and the boy was able to leave the hospital 5 days after the intervention, with complete relief of the symptoms. He was followed for 18 months without any recurrence.
Discussion
Extracardiac rhabdomyomas are extremely rare tumors which represent less than 2 % of the neoplasms of the striated musculature. The subgroup describes, adult rhabdomyomas has a significantly lower impact than its clever equivalent, rhabdomyosarcoma.
This subtype mainly affects the head and neck region and comes from the muscles of the third and fourth branchial arches. Most cases are lonely (70 %) and generally occur in men over 50.
Adult rhabdomyomas from the pharyngeal region of a child are even rarer. To the knowledge of the treating team, the one that is described is the first reported case of adult rhabdomyoma in the pharyngeal region of a child.
The signs and symptoms at the time of the presentation depend on the location of the tumor. In this case, the boy had a feeling of a foreign body in his throat and snoring at night for 6 months. The symptoms described in the literature include hearing loss, a mass in the undermaxillary triangle, swelling of nasopharynx, hemoptysis and obstructive sleep apnea, pure dysphagia being rare.
Current diagnostic imaging techniques, in particular ultrasound, computed tomography and MRI, have been not very specific for the diagnosis of rhabdomyoma in adults.
In this case, the objective image showed a mass with a pedicle in the throat, which led the doctors to carry out direct excision.
It is important to combine imaging with histopathological confirmation, especially in pediatric patients, in whom unlikely tumors, such as rhabdomyomas, are less often envisaged. If imaging is useful for location and surgical planning, histopathology remains the definitive diagnostic method.
Surgery is the reference treatment. Although rhabdomyisms in adults are mild, up to 42 % of them can reoffend due to incomplete resection. In addition, residual tumors can have a clever potential.
The microscopic appearance of adult rhabdomyomas is relatively uniform and characterized by polygonal cells densely grouped together with prominent nuclei, located in the center or on the periphery. The cytoplasm is eosinophilic, granular and has an extensive vacuolization in certain cells.
These characteristics have also been observed in this case. The diagnostic immunohistochemical characteristics include cytoplasmic positivity for MSA, desmine, myoglobin and myod1.
This case underlines the importance of taking into account adult rhabdomyomas in the differential diagnosis of pharynx tumors in children, despite their rarity in this age group. Surveillance of recurrence in clinical centers is essential to guarantee optimal treatment and optimal results for patients.
This article has been translated fromUnivadis.de. The content was reviewed by the editorial staff before publication.
