Ganglioneuroma is a rare benign tumor originating in the sympathetic ganglia, composed of differentiated ganglion cells, nerve sheath cells, and nerve fibers, which tend to occur in the posterior mediastinum, adrenal gland, retroperitoneal, and other locations, occurring in the head and neck is relatively rare, and parapharyngeal space involvement is extremely rare. In our report, we present 2 adult male patients whose preoperative imaging and fine needle cytology did not confirm the diagnosis of a parapharyngeal space mass and who completely resected the tumor through a combined cervical and oral approach. Finally, pathology confirmed ganglioneuroma; we also reviewed the English articles on parapharyngeal ganglioneuroma over the past 40 years, and summarized the diagnostic and treatment characteristics of parapharyngeal ganglioneuroma in combination with our cases to improve understanding of the disease.

Chordomas are rare, locally aggressive bone malignancies with poor prognoses. However, those with minimal or no bone involvement are more easily resectable because of their well-delineated margins and thus have better prognoses. Such extraosseous chordomas of the spine are localized both intradurally and extradurally. Only a few case reports have focused on extraosseous, extradural spinal chordomas. Radiologically, this type of chordoma has a dumbbell shape; however, dumbbell-shaped spinal tumors are traditionally thought to be neurogenic tumors (i.e., schwannomas or neurofibromas). We herein report a unique case involving a woman with a dumbbell-shaped extraosseous chordoma protruding predominantly into the retropharyngeal space. A 44-year-old woman presented for evaluation of a left submandibular mass. A T2-hyperintense, gadolinium-enhancing mass was found in her cervical spinal canal, protruding through the C2/3 neural foramen into the retropharyngeal space with minimal vertebral involvement. The initial diagnosis was a neurogenic tumor, most likely a schwannoma. After subtotal removal, the pathologic diagnosis was a chordoma. Because chordomas and schwannomas have significantly different prognoses, caution is warranted when a dumbbell-shaped tumor is identified in the spine with minimal or no vertebral deterioration on radiology. This report also provides the first thorough review of extraosseous dumbbell-shaped intraspinal-extraspinal chordomas.

Background: MYC associated factor X (MAX) is a tumor suppressor gene and has been identified as one of the pathogenic genes of hereditary pheochromocytoma (PCC). To date, there have been no reports of ganglioneuroma (GN) with MAX variants. Case Presentation: The proband was a 45-years-old Chinese female with paroxysmal hypertension and palpitations who had undergone adrenalectomy for PCC 14 years ago. Her plasma free normetanephrine and 24-h urinary norepinephrine excretion were significantly increased, and abdominal computed tomography (CT) revealed an irregular mass in the left adrenal region, suggesting a recurrence of PCC. The mass was surgically removed and pathologically diagnosed as PCC with lymph node metastasis. The proband\'s son suffered from paroxysmal hypertension and palpitations. His plasma free metanephrine levels were normal. CT revealed a mass in the right adrenal. The tumor was surgically removed, and the pathological diagnosis was GN. Genetic testing of peripheral blood DNA revealed that the proband and her son had germline pathogenic MAX variant c.C97T, p.Arg33Ter, while proband\'s parents did not have MAX variants. Tumor DNA sequencing showed the same MAX variant (c.C97T, p.Arg33Ter) in PCC of the proband and GN of her son, both with retention of heterozygosity. Immunohistochemistry demonstrated loss of MAX protein expression in most tumor cells in PCC of the proband and some Schwannian cells in GN of the proband\'s son. Conclusion: We report a family with a new clinical phenotype of germline pathogenic variants in MAX who developed both PCC and GN. Germline pathogenic variants in MAX may contribute to the development of GN. Our findings suggest that it is not just paternally inherited MAX variants that can cause tumors.

A chest wall schwannoma arises from peripheral nerve sheath Schwann cells of the intercostal nerves. We describe the presentation and imaging findings of a patient who presented with a chest wall swelling. The imaging findings were highly suspicious for a chest wall schwannoma and the histopathology confirmed the diagnosis following surgical excision. Imaging findings are reviewed in detail.

BACKGROUND: For a long time, chordoma has been known as an osseous tumor mainly found at the clivus and sacrococcygeal region. However, spine extra-osseous chordoma (SEC) with a better prognosis than the classic type has been neglected. According to our literature review, only several case reports have been published in English literatures. Here in this article, three cases of SEC, plus a literature review, are presented.
METHODS: Three cases of SEC were presented from our center. Surprisingly, neurologic tumors were considered as the first diagnosis. Thereafter, en bloc resection was performed in all the three cases. Especially, the dumbbell-shaped one in the cervical spine was removed by en bloc through the combined anterior and posterior approach for the first time. Follow-up within 12-58 months after surgeries proved no recurrence or metastasis.
CONCLUSIONS: Spine extra-osseous chordoma, commonly located in the cervical and epidural region, is extremely rarely met. SEC is characterized with less aggressiveness, the lower rate of recurrence and metastasis, and better prognosis than those of the osseous origin. Though complete excision can be achieved generally, differential diagnosis of spine neurogenic tumors and the following en bloc resection should be made as carefully as possible.

Schwannoma of the mediastinum is a rare and typically benign type of tumor. The present study describes the case of a 61-year-old woman who presented with a continuous cough and facial edema. Pre-operative chest radiography, enhanced computed tomography (CT) and three-dimensional CT scans identified a well-circumscribed mass. The large cyst, measuring 6.5×6.1×5.0 cm, was located anterior to the trachea and posterior to the superior vena cava. The mass was observed to be in close proximity to the right pulmonary hilum and the superior vena cava was flattened due to the pressure on the right vagus nerve. Therefore, the encapsulated tumor was completely resected under thoracoscopy and was subsequently diagnosed as a benign schwannoma upon pathological examination. The respiratory tract symptoms and facial edema resolved immediately after the surgery, and no recurrence was observed during the 6-month follow-up period. At the time of writing, the patient remained alive. The present study records the rarely successful resection of a middle mediastinal tumor by video-assisted thoracoscopy.

The main purpose of this pictorial review is to highlight the important MR imaging findings of various conditions involving the cavernous sinus in addition to brief description of normal anatomy. The pathological conditions that can involve the cavernous sinus can be categorized into infective, inflammatory, granulomatous, vascular and neoplastic causes. Imaging, especially with MRI, plays an important role not only in detection but also in definition of disease extent and in characterization of the pathology. Currently, high-resolution MR images clearly show various components of cavernous sinus which help in making a proper diagnosis and thus appropriate further management.

Ganglioneuromas (GNs) arising from neural crest sympathogonia are rare benign neurogenic tumors. The most commonly affected sites are the posterior mediastinum, the retroperitoneum and the adrenal gland. GNs often present as a solitary, painless and slow-growing mass, and multiple occurrences in the cervical region are extremely rare. Here, we report a case of massive multiple cervical GN in a 4-year-old girl, and review cases of cervical GN that have been reported in the past 10 years. The results demonstrated that cervical GN, compared to other sites, is seldom secretory. The signs and symptoms of cervical GN are unspecific; the ultimate diagnosis of GN depends on pathological examination. Fine-needle aspiration biopsy has limited value in diagnosis. Surgical excision is the treatment of choice and the prognosis is excellent even in cases where complete excision cannot be achieved. Furthermore, GNs should be considered in patients with multiple masses in the neck.