|Year : 2018 | Volume
| Issue : 1 | Page : 82-84
Embryonal rhabdomyosarcoma of the nasopharynx
Department of Radiology, St. John's Medical College, Bengaluru, Karnataka, India
|Date of Submission||13-Feb-2017|
|Date of Acceptance||19-Jun-2017|
|Date of Web Publication||23-Apr-2018|
Dr. Reddy Ravikanth
St. John's Medical College, Bengaluru - 560 034, Karnataka
Source of Support: None, Conflict of Interest: None
Embryonal rhabdomyosarcoma has the second highest frequency in the head and neck region. A rare case of embryonal rhabdomyosarcoma in a 9-year-old male child involving nasopharynx with hydrocephalus is reported. The child presented with difficulty in breathing and swallowing with bleeding from nose and mouth. Local examination revealed a granular mass in the left nasal cavity. There was associated proptosis of the left eye. On probing, the mass was friable, nontender and did not bleed on touch. Posterior limit of the mass could not be assessed. Examination of throat showed smooth downward bulge of soft palate on the left side extending into the lateral wall of pharynx behind the posterior pillar of left tonsil. Examination under general anesthesia confirmed the presence and attachment of granular mass to the posterosuperior wall of the nasopharynx and filling the left choana as well. Fibrosarcoma, mucormycosis, nasal polyposis, juvenile nasal angiofibroma, and rhabdomyosarcoma of the nasopharynx were considered in the differential diagnosis. Nasal polyposis was excluded from the differentials after gating computed tomography (CT) scan report was obtained. Histopathology of the excised mass proved the diagnosis of rhabdomyosarcoma and CT scan of the brain showed communicating hydrocephalus. Treatment plan included multi-drug chemotherapy and debulking surgery.
The following core competencies are addressed in this article: Medical knowledge, Patient care.
Keywords: Embryonal rhabdomyosarcoma, epistaxis, hydrocephalus, nasopharynx
|How to cite this article:|
Ravikanth R. Embryonal rhabdomyosarcoma of the nasopharynx. Int J Acad Med 2018;4:82-4
| Introduction|| |
Rhabdomyosarcoma is the most common soft tissue sarcoma in children. The name is derived from the Greek words rhabdo, which means rod shape, and myo, which means muscle. As its name suggests, the tumor is believed to arise from a primitive muscle cell. Rhabdomyoblasts sometimes have discernible muscle striations that are visible on specimens under light microscopy, although electron microscopy may be needed to detect subcellular elements. Cells are usually positive for intermediate filaments and other proteins typical of differentiated muscle cells, such as desmin, vimentin, myoglobin, actin, and transcription factor myoD. In patients with localized disease, overall 5-year survival rates have improved to more than 80% with the combined use of surgery, radiation therapy, and chemotherapy.
| Case Report|| |
A 9-year-old male child presented to the ENT-outpatient department of St. John's Medical College Hospital in January 2016 with a history of nasal obstruction and several episodes of epistaxis for the past 2 months and proptosis of the left eye for the past 15 days [Figure 1]. Imaging revealed a nasopharyngeal mass lesion invading the infratemporal fossa, sinuses [Figure 2]a, and the orbit [Figure 2]b with significant bony destruction [Figure 2]c and hydrocephalus [Figure 2]d. Written consent was taken for the biopsy. Histopathological examination of the biopsy tissue from the tumor revealed a hypercellular malignant neoplasm formed by atypical rhabdomyoblasts cells with necrosis, chronic inflammation, and infiltration by plasma cells and lymphocytes. Small undifferentiated round cells with dense hyperchromatic nuclei and haphazardly arranged primitive mesenchymal cells with stratified squamous epithelium were noted [Figure 3]. Tumor cells showed cytoplasmic immunohistochemical staining with desmin and showed positivity for specific immunohistochemistry (IHC) markers MyoD1, myogenin. Exclusion IHC markers CD31, CD34, leukocyte common antigen, and epithelial membrane antigen and thus, a final histological diagnosis of embryonal rhabdomyosarcoma was made. Extensive debulking surgery of the mass lesion in the nasopharynx, paranasal sinuses, and infratemporal fossa region was done through lateral rhinotomy leaving a small residual of tumor in the orbital apex. Four cycles of chemotherapy including vincristine (1.4 mg/m 2), cyclophosphamide (750 mg/m 2), and actinomycin D (50 mg/m 2). Localized chemotherapy was administered after 4 cycles and 6 months follow-up showed no signs of recurrence or metastasis.
|Figure 1: Clinical photograph of the child presenting with proptosis of the left eye|
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|Figure 2: (a) Contrast enhanced computed tomography image of the brain showing a heterogeneously enhancing soft tissue density lesion involving the nasopharynx, maxillary sinus, and the infratemporal fossa on the left (block area). (b) Contrast-enhanced computed tomography image of the brain showing the extension of the nasopharyngeal lesion into the left orbit causing proptosis (block area). (c) Computed tomography image of the brain in bone window showing bony destruction involving the nasal septum, vomer, clivus, sella and left-sided greater wing of sphenoid, pterygoid process, sphenoid bone, petrous part of the temporal bone, and turbinates (block area). Left-sided mastoiditis also seen. (d) Computed tomography image of the brain demonstrating dilated lateral ventricles (block area). There was also dilatation of the 3rd and 4th ventricles (not shown in the image) suggesting communicating hydrocephalus|
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|Figure 3: Histopathology image demonstrating a cluster of pleomorphic and polygonal tumor cells (block area) with hyperchromatic nuclei and acidophilic cytoplasm (H and E, ×40)|
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| Discussion|| |
There are four variants of rhabdomyosarcoma such as embryonal, alveolar, pleomorphic and botryoid varieties. Botryoid rhabdomyosarcoma is a subtype of embryonal rhabdomyosarcoma, that originates in mucosa lined structures such as the nasopharynx, common bile duct, urinary bladder, and vagina in younger age groups. Embryonal rhabdomyosarcoma has the second highest frequency in the head and neck region, orbit being the most common in children. Rhabdomyosarcoma of the nasopharynx presents as a polypoid growth which can be easily mistaken for nasal polyps. The histopathological diagnosis of rhabdomyosarcoma relies on demonstrating myxoid areas in different mesenchymal blastoma of elongated cells just under the squamous epithelium. Where surgical resection of the tumor is not possible debulking followed by radiotherapy alone or along with chemotherapy should be the ideal treatment. Preoperative assessment of the tumor size, bone erosions, and intracranial invasion using imaging modalities such as computed tomography and magnetic resonance imaging is very important to assess resectability of the tumor.
| Conclusion|| |
The tissue of origin of embryonal rhabdomyosarcoma is derived from totipotent mesenchymal cells or from muscles and in the present case may be palatal muscles around the Eustachian tube More Details. Although the characteristic feature is embryonal myoblasts, the diagnosis of rhabdomyosarcoma depends on showing the myxoid area in different mesenchymal blastoma of elongated cells just under the squamous epithelium. Tumor debulking followed by a combination of chemotherapy and radiotherapy should be the ideal treatment.
Declaration of patient consent
The authors certify that they have obtained appropriate patient consent documentation, wherein the patient(s) has/ have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patient(s) understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.
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Conflicts of interest
There are no conflicts of interest.
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[Figure 1], [Figure 2], [Figure 3]