A Rare Case of Adult Rhabdomyoma in the Parapharyngeal Space: Diagnostic Challenges and Surgical Management

Dear Editor; With curiosity I read the original article by Jeong et al. [1]. They proposed to modify the classification of trigeminal schwannomas (TSs) according to their extension into the adjacent compartment, shape, and location of origin, and concluded that TSs could be removed safely and effectively according to their modified classification. The authors are to be congratulated for conducting such a well-managed study on a topic of schwannomas. Here, I would like to add some additional points about the rare locations origin of schwannomas. Schwannomas may originate from any cranial, peripheral or autonomic nerve of the body and are uncommon nerve sheath tumors. About one-third of schwannomas are identified to arise in the neck and head region. TSs are known to arise from the ganglion, root, or intracranial portion of the peripheral divisions of the nerve-mandibular, maxillary, and ophthalmic. Schwannomas including the mandibular division of trigeminal nerve and localizing particularly in the parapharyngeal space is entirely rare. The most common nerves of origin of TSs in the parapharyngeal space are the sympathetic chain and the vagus nerve [2]. On the other hand, an exceedingly rare schwannomas is olfactory schwannoma as the olfactory nerve is unsheathed by schwann cells, till today, only 48 cases have been reported worldwide [3]. Also, there have been no persuasive theories that are comprehensively accepted to define the origin of olfactory schwannoma. Additionally, a rare bone tumor is intraosseous schwannomas which is accounting for less than 1%. Notably, mandible is the most common site of intraosseous schwannoma, however, intraosseous skull vault schwannomas are very rare [4]. As is obvious from above discussion, parapharyngeal, olfactory, and intraosseous spaces are extremely rare locations origin of schwannomas which the management of them is a clinical challenge. In this regard, more studies may need to focus on this area.

Endoscopic endonasal resection of a parapharyngeal cavernous hemangioma: Technical case report and literature review

Schwannoma of parapharyngeal space is an uncommon benign tumour of head and neck region. These are slow-growing and usually well-encapsulated masses arising from Schwann cells of cranial nerves. The majority...

A prognostic scoring system for locoregional control in nasopharyngeal carcinoma following conformal radiotherapy

Solitary fibrous tumors (SFTs) are rare spindle cell neoplasms that are mostly found arising from the pleura. Although SFTs recently have been reported in other regions, they are rare in the head and neck and have often been misdiagnosed due to their rarity. SFTs are benign in most cases. Clinically, SFTs usually manifest as well-circumscribed, slow-growing, smooth and painless masses. Symptoms are often minimal, although they may include sore throat, difficulty in swallowing, change of voice or trismus. CT-Scan and MRI are the most sensitive imaging procedures used. The treatment of choice is complete surgical excision of the lesion. Because recurrences have been noted up to 30 years after surgery, long-term follow up is mandatory. In this article, we present a case of a Solitary Fibrous Tumor arising in the parapharyngeal space in a 20-year-old man, involving the carotid sheath, treated by surgical excision with no recurrence after 1 year. The clinical presentation, surgical management and pathological findings are described.

A parapharyngeal space schwannoma arising from the vagus nerve: A case report

Ganglioneuroma is a rare benign tumor that originates from neural crest. Tumor tends to be slow growing, asymptomatic but can cause symptoms because of pressure to neighboring structures. In the head and neck region they are relatively rarely seen. We hereby present a rare case of multiple ganglioneuromas that were located in parapharyngeal space, iliac bone and other bones in a 13-year-old girl. Patient underwent surgery for the excision of a large mass, extending from parapharyngeal space to neck, with transparotid and transcervical combined approach. After operation, MIBG (iodine-123-meta-iodobenzylguanidine) scintigraphy was performed and involvement of parietooccipital bone, lumbal vertebra, right iliac wing medial cortex and left humerus were detected. No adjuvant therapy was given to the patient. There is no evidence of recurrence in the head and neck region in the following 12 months. In conclusion, complete surgical excision of the tumor, if possible, is the treatment of choice with high success rate. Close clinical and radiological follow-up for these tumors after surgery should be made.

Chondrosarcoma of the parapharyngeal space: Case report and literature review of an extremely rare location

Desmoid tumours (extraabdominal desmoids, aggressive fibromatosis) are rare connective tissue tumours, that grow infiltratively and destructively. The analysis of the course of disease in three patients suffering from desmoid tumours in the head and neck region (regio submandibularis, regio parotidea, parapharyngeal space) confirmed the data in literature that painless swelling and dysfunction are the first non-specific signs of the disease. Morphologically reactive fibromatosis and fibrosarcoma should be considered (amongst others) in differential diagnosis. CT and MR scans are useful in determining the extent of the tumours and help to distinguish the tumour from nerves, vessels and bone. Surgery is the therapy of choice in the head and neck region. Primary surgery was performed in our patients. Due to recurrences two of our patients have been operated on several times including major plastic reconstructive surgery. One patient died three and a half years after primary treatment due to sarcomatous degeneration. A large parapharyngeal recurrence was successfully reduced by percutaneous radiation therapy in our second patient. Follow-up of our third patient was NAD 15 months after resection of a large parapharyngeal/mediastinal process including blocking of two cervical vertebrae. Summing up, desmoid tumours are rare connective tissue tumours in the head and neck region with a destructive biological behaviour similar to malignant tumours and a high recurrence rate. Surgery is the treatment of choice. In recurrences or if the tumour is not properly resectable, radiation must be considered, whereas the chemotherapeutical approach is not yet well established.

Introduction: Although lymph node metastases are common with papillary thyroid cancer, parapharyngeal and retropharyngeal lymph node metastases are relatively rare. Although small metastatic parapharyngeal lymph nodes (e.g., <1 cm) may be treated with radioactive iodine or observed, larger lymph nodes may require surgical excision. Surgical approaches to the parapharyngeal and retropharyngeal space include transoral and transcervical.Materials and Methods: A 47-year-old female presented with a 2 cm conventional papillary thyroid cancer in the right thyroid lobe with central right lateral neck metastases, as well as a 2.5 cm right parapharyngeal lymph node metastasis extending to the skull base. Surgical technique for transcervical resection of the 2.5 cm parapharyngeal lymph node is illustrated, identifying important anatomical structures.Results: After opening the right neck and removing the right level 2 lymph nodes (not illustrated), the parapharyngeal space is exposed. First, the posterior belly of the digastric and stylohyoid muscles is divided. Next, the hypoglossal nerve is identified and mobilized. Branches of the external carotid artery are then divided and retracted. The sympathetic chain is visualized posterior to the internal carotid artery. The external branch of the superior laryngeal nerve is visualized as it courses posterior to the carotid artery. After gentle retraction of the hypoglossal nerve, superior laryngeal nerve, carotid artery, and sympathetic chain, the parapharyngeal space is exposed with the aforementioned metastatic lymph node. The metastatic lymph node is then freed from the alar fascial and skull base attachments and removed en bloc.Conclusion: To our knowledge, this is the first video demonstration of a transcervical parapharyngeal lymph node resection in the peer-reviewed literature. Transcervical excision of parapharyngeal and retropharyngeal lymph nodes requires a thorough understanding of the anatomy of the neck and parapharyngeal space, along with a stepwise surgical technique to safely expose the parapharyngeal space.No competing financial interests exist.Runtime of video: 8 mins 44 secs

Solitary fibrous tumours (SFTs) are rare, mesenchymal neoplasms. The purpose of this study was to analyse the radiological and clinicopathological features of SFTs in the extracranial head and neck region. We retrospectively reviewed the clinical, computed tomography (CT), magnetic resonance imaging (MRI), and pathological features in 18 patients (12 men and 6 women), aged 18-75years, with histologically proven SFTs in the extracranial head and neck region. Fourteen patients underwent CT scanning and nine underwent MRI. The histological techniques included routine haematoxylin-eosin staining and immunohistochemical analysis. Clinical data were retrieved from the medical records. Most tumours presented as a slow-growing painless mass. Eighteen SFTs arose in the orbit, cheek, masticator space, the parapharyngeal space, infratemporal fossa, maxillary, submandibular space and the parotid gland, respectively. All 18 lesions were found as a solitary mass, ranging in size from 1.2 to 6.8cm (mean 3.57cm). They mostly presented with an ovoid shape, with well-defined margin, and isodensity on plain CT, isointensity on T1-weighted imaging, mild hyperintensity on T2-weighted imaging and diffusion-weighted imaging, and marked heterogeneous enhancement on contrast-enhanced CT and MRI. The time-intensity curves (TICs) exhibited a rapidly enhancing and slow washout pattern on dynamic contrast-enhanced MRI and dual-phase CT. Imaging findings of the SFTs depended on the histopathological components. SFTs most commonly present with an asymptomatic mass in adults. A solitary, ovoid and well-defined mass with strong enhancement after contrast agent injection is suggestive of this diagnosis. Rapidly enhancing and slow washout pattern TICs may be additional valuable features.

Introduction. De novo pleomorphic adenomas in the parapharyngeal space are rare and cause difficulties in its surgical management. We report the largest de novo pleomorphic adenoma arising from the parapharyngeal space and discuss its surgical management. Presentation of Case. A 34-year-old male presented with a giant de novo pleomorphic adenoma arising from the parapharyngeal space, which was initially misdiagnosed as an impacted wisdom tooth. Measuring 8.4 × 6.5 × 3.9 cm in size and weighing 87.3 g, this is the largest primary salivary gland tumour arising de novo from the parapharyngeal space reported in the literature, presenting challenges in its surgical management. Discussion. Parapharyngeal space tumours cause nonspecific symptoms and may be difficult to diagnose, which can allow the tumours to become very large and cause obstructive and compressive symptoms in an anatomically difficult area. A combined trans-cervical and trans-oral approach can be used to safely perform an en bloc resection. Conclusion. We report the diagnosis and surgical management of the largest pleomorphic adenoma arising de novo from the parapharyngeal space reported in the literature.

The parapharyngeal space (PPS) is a potential space that extends from the skull base to the hyoid bone and is bounded medially by the fascia surrounding the pharyngeal constrictor muscle and laterally by the ramus of the mandible and the medial pterygoid muscle. PPS neoplasms comprise only 0.5% of all head and neck tumors, and neurogenic tumors such as neurilemmomas, schwannomas or paragangliomas constitute only 30% of these PPS tumors. We report the case of a 38-year-old woman who on presentation complained of a foreign body sensation when swallowing and was without neurological abnormalities. She was found to have a submucosal growth on the right side of the oropharyngeal wall. A tumor was found in the prestyloid compartment of the PPS with magnetic resonance imaging (MRI). A well-encapsulated tumor was surgically excised via a transcervical approach and proved to be a neurilemmoma. Postoperatively, paralysis of the right side of the tongue developed. Follow-up examinations have showed gradual recovery without disease recurrence over six months of follow-up Based on radiological classification of a PPS tumor as either prestyloid or poststyloid, the clinician can formulate the most appropriate management plan. MRI is valuable as a routine study for the differentiation of PPS tumors.

The objective of this study was to determine the value of barium swallow pharyngoesophagography in the investigation of patients with globus pharyngeus who had no known disease, no operation history in the head and neck region, and no thyroid pathology. We compare the clinical presentation with the radiologic findings to assess the diagnostic accuracy of barium swallow in such patients. This was a retrospective study of the clinical records and radiologic reports of 194 patients with globus pharyngeus, between October 1998 and October 2004, that were reviewed and analyzed for correlations between clinical signs and symptoms and barium swallow findings using the chi2 test. All the patients had normal ear, nose, and throat examination, including indirect laryngoscopy and/or nasoendoscopy. Patients with known pharyngeal and esophageal disease, neurologic disease known to cause pharyngeal or esophageal dysfunction, evidence of autoimmune disease or diabetes mellitus, with a thyroid operation in the past and thyroid nodules detected with radiologic investigation were excluded. There were 116 (59.8%) female and 78 (40.2%) male patients. In 114 patients (59%), globus was the only symptom. Sore throat was the most common associated complaint. Benign barium swallow findings were detected in 63.9% of the patients. Cervical osteophyte indentation was the most common finding. In 36.1% of the patients, the radiologic finding was normal. The statistical analysis using the chi2 test showed a significant relationship (P < .001) between the symptoms of globus pharyngeus and the barium swallow results. Barium swallow pharyngoesophagography is useful to detect underlying benign characteristic pathologies in patients with globus who have no known head and neck illness.

Background and objectivePleomorphic adenoma is the most common major salivary gland neoplasm. Around 80% of them arise in the parotid glands, whereas 10% originate in the minor salivary glands. The pleomorphic adenoma of the extra-parotid site is defined by its location outside the primary salivary gland. The minor salivary gland adenomas occur at the hard and soft palate, lips, tongue, lacrimal glands, pharynx, larynx, paranasal sinus, and nasal cavity. Pleomorphic adenoma in parapharyngeal space may occur de novo or as an extension of the deep lobe of the parotid tumors. Our objective in this study was to assess the location and presentations of extra-parotid pleomorphic adenoma and frequency of low-grade salivary gland malignancy diagnosed as pleomorphic adenoma via fine-needle aspiration cytology (FNAC) in the head and neck region and the treatment outcomes after the resection of the tumors.Materials and methodsThis was a retrospective observational study. All patients with FNAC-diagnosed pleomorphic adenoma of extra-parotid locations of the head and neck region who underwent curative surgery in the Department of Otorhinolaryngology and Head and Neck Surgery at a rural tertiary care center between August 1997 and August 2022 were included in the study. Data on the symptoms, FNAC report, surgical techniques, pathological results, adjuvant therapy, and any recurrence were documented. Data were entered into a Microsoft Excel sheet and analyzed using IBM SPSS Statistics version 22 software (IBM Corp., Armonk, NY).ResultsOur study included 23 patients, of which 14 were females and nine were males. The various sites of involvement were as follows: parapharyngeal space (four), larynx (one), nasal septum (two), hard palate (five), soft palate (four), hard and soft palate (three), and submandibular salivary gland (four). Of note, 17.3% of the patients had local recurrence with an average time frame of three years post-surgery: 20% in patients with low-grade malignancy and 16.6% in patients with pleomorphic adenoma.ConclusionExtra-parotid pleomorphic adenomas are common and have a high malignant potential. The preferred choice of treatment for extra-parotid salivary tumors is complete resection with adequate clearance. Malignant pleomorphic adenomas may require staging neck dissection and adjuvant treatment for a better prognosis.