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Vagal schwannoma involving esophagus
Vagal Schwannoma Involving Esophagus Reza Vaghei, MD, and Nathan I. Yost, MD Thoracic-Vascular Surgery and Pulmonary Medical Sections, Veterans Affairs Medical Center, Martinsburg, West Virginia
A rare case of vagal schwannoma (neurilemoma) with involvement of the distal esophagus is presented. Chronic progressive dysphagia was the only complaint related to the schwannoma. Barium swallow showed narrowing of the distal esophagus. The tumor was embedded in the wall of the esophagus. After biopsy and
diagnosis of benign schwannoma, it was dissected from the esophageal muscle and mucosa. Diagnosis of benign schwannoma should be made before excision of the lesion to avoid an unnecessary esophagectomy.
S
tion between the mass and intercostal nerves or the paravertebral area. The right vagus nerve along the esophagus entered the tumor mass and could not be traced until 2 cm below the mass. Part of the tumor was embedded in the wall of the esophagus. The medial third of the circumference of the esophagus was free. There was no obvious demarcation between the tumor and the other two thirds of the esophagus for approximately 4 cm. A biopsy and frozen section showed the mass to be benign schwannoma. With meticulous dissection the tumor was dissected from the esophageal muscle and mucosa, then it was excised in conjunction with a segment of involved vagus nerve. In the involved portion of the esophagus in which the esophageal muscle was cut, the mucosa partially bulged out between the edges of the cut muscles. Pathologic analysis showed a 12.5 x 12 x 6.5-cm
chwannomas (neurilemomas) may arise in any nerve where Schwann cells occur. Schwann cell tumors of the peripheral nervous system were given the name neurilemoma by Stout and Carson in 1935 [l]. Such tumors characteristically produce few symptoms, exhibit slow growth, and rarely cause serious neurological disturbances. Schwannomas arising from the vagus nerve are rare; here, we report a vagal schwannoma involving the esophagus. A 67-year-old white man was admitted with a mass in the right parotid gland (for biopsy and possible operation). An admission chest roentgenogram revealed a large mass in the right posterior and lower mediastinum. The patient stated that he had had some difficulty in swallowing which gradually became worse. Before hospital admission the patient had been receiving captopril, 25 mg twice per day, and glipizide, 5 mg twice per day. Barium swallow showed narrowing of the distal esophagus with a large right posterior mediastinal mass, displacing the distal esophagus to the left (Fig 1). A chest computed tomographic scan showed involvement of the distal esophageal wall by tumor. The mucosa of the esophagus was essentially intact (Fig 2). On physical examination there was no cervical lymphadenopathy. The right parotid gland was enlarged (3 x 3-cm), firm, and fixed. No lesion was seen in the oral cavity, pharynx, or larynx. The chest was clear, and the cardiovascular examination results were within normal limits. Laboratory test results, including electrolyte levels, white blood cell count, hematocrit, and urinalysis, were within normal limits. Because of dysphagia and severe narrowing of the distal esophagus, it was decided to treat the mediastinal mass before biopsy and excision of the right parotid tumor. With the presumed diagnosis of a posterior mediastinal mass, most likely neurogenic in nature, the patient was explored through a right sixth intercostal space thoracotomy. At exploration there was a large movable mass located over the distal esophagus. There was no connec-
(Ann Thorac Surg 1991;52:1334-6)
Accepted for publication June 7, 1991. Address reprint requests to Dr Vaghei, Thoracic Vascular Section, VA Medical Center, Martinsburg, WV 25401.
Fix 1 . Lateral chest roentgenogratn with barium swallow.
Ann Thorac Surg 1991;52:1334-6
CASE REPORT VAGHEI AND YOST SCHWANNOMA INVOLVING ESOPHAGUS
1335
Fig 2. Chest computed tomographic scan of distal esophagus showing tumor involving the esophageal wall.
encapsulated mass weighing 580 g (Fig 3). Histologically, the tumor showed densely cellular spindle shaped cells in palisading arrangements (Antoni A pattern) with Verocay body formation, characteristic of benign schwannoma. Postoperative recovery was uneventful. A barium swallow days after 'peration showed minimum postoperative changes and an otherwise normal esophagus (Fig 4).
Fig 4. Barium swallow showing minimum postoperative changes and an ofhenvise nor ma^ esophagus.
Subsequently, after open biopsy of the parotid gland and diagnosis of undifferentiated carcinoma, the patient underwent parotidectomy and radiation therapy. At present he is asymptomatic. Chest roentgenograms remain normal.
Comment
Fig 3. Gross appearance of the tumor mass.
Intrathoracic neural tumors are uncommon, but account for 75%of tumors of the posterior mediastinum [2]. Vagal schwannoma is very rare. In 1990 Dabir and associates [3] reported 2 cases of vagal schwannoma involving the thoracic portion of the vagus nerve adjacent to the aortic arch. In reviewing the literature they found 27 cases of vagus nerve tumors of which 9 were schwannomas. In the same year Heitmiller and co-workers [4] reported another case of intrathoracic vagal schwannoma, for a total of 12 cases. In 1956 Davis and Brown [5] reported an intrathoracic neurofibroma of the left vagus nerve at the level of the gastroesophageal junction associated with diaphragmatic hernia. The patient had no digestive tract symptoms. In our patient, schwannoma arising form the vagus nerve involved the esophagus, causing dysphagia. Peripheral tumors are first identified as a painless mass, whereas intrathoracic tumors are usually identified incidentally on chest roentgenograms. When symptomatic, or if associated with Von Recklinghausen's disease, malignant schwannoma should be suspected. In our patient dysphagia was due to extraluminal pressure by a large
1336
Ann Thorac Surg 1991;5213346
CASE REPORT VAGHEI AND YOST SCHWANNOMA INVOLVING ESOPHAGUS
mass in an otherwise normal esophagus. These tumors are benign, but malignant degeneration has been reported [6]. Furrer and Fox [7]reported a case of fibrosarcoma of the left vagus sheath. In our case, biopsy and frozen section before the excision of the lesion was very helpful. The tumor was large and embedded in the wall of the esophagus. Without a diagnosis of benign schwannoma, one may mistakenly attempt to resect the esophagus. Nasogastric tube within the lumen of the esophagus was used as a guide, and the tumor was dissected from the esophageal muscle and mucosa without injuring the mucosa. Das Gupta and associates [8] reported that 49 or 16.1% of 303 patients with benign solitary schwannomas had a malignant tumor unrelated to peripheral nerves. Twentytwo patients gave a history of being treated for cancer before the development of a schwannoma. Sixteen patients had cancer concomitant with the benign solitary schwannoma, and cancer developed in 11 patients at a later date. Das Gupta and associates found this was a relatively high incidence of malignant tumors in otherwise normal patients. Although no conclusions regarding association of schwannomas with other types of cancers was drawn, they suggested that a careful study be made to ascertain if any such association does in fact exist. In
our patient, carcinoma of the right parotid gland was concomitant with the esophageal schwannoma. Enucleation of the vagal schwannoma from the esophageal wall is the treatment of choice. In the differential diagnosis of esophageal tumors, vagal schwannoma should be included.
References 1. Stout AP, Carson W. The peripheral manifestations of the
2. 3. 4.
5. 6. 7. 8.
specific nerve sheath tumor (neurilemoma). Am J Cancer 1935;24:751-96. Currier AR, Gale JW. Mediastinal Tumors. Arch Surg 1949;58: 797-818. Dabir RR, Piccinoe W Jr, Kittle CF. Intrathoracic tumors of the vagus nerve. Ann Thorac Surg 1990;50:494-7. Heitmiller RF, Labs JD, Lipsett PA. Vagal schwannoma. Ann Thorac Surg 1990;50:811-3. Davis C Jr, Brown G. lntrathoracic neurofibroma of the vagus nerve associated with a diaphragmatic hernia. J Thorac Surg 1957;33:532-6. Cavstens H, Schrodt G. Malignant transformation of a benign encapsulated neurilemoma. Ann J Clin Pathol 1969;51:4&9. Furrer ED, Fox IR. Perineural fibrosarcoma of the vagus sheath. World J Surg 1940;48:584-6. Das Gupta TK, Brasfield RD, Strong EW, Hajdusi SI. Benign solitary schwannomas (neurilemomas). Cancer 1969;24: 355-66.
A rare case of vagal schwannoma (neurilemoma) with involvement of the distal esophagus is presented. Chronic progressive dysphagia was the only complaint related to the schwannoma. Barium swallow showed narrowing of the distal esophagus. The tumor was embedded in the wall of the esophagus. After biopsy and
diagnosis of benign schwannoma, it was dissected from the esophageal muscle and mucosa. Diagnosis of benign schwannoma should be made before excision of the lesion to avoid an unnecessary esophagectomy.
S
tion between the mass and intercostal nerves or the paravertebral area. The right vagus nerve along the esophagus entered the tumor mass and could not be traced until 2 cm below the mass. Part of the tumor was embedded in the wall of the esophagus. The medial third of the circumference of the esophagus was free. There was no obvious demarcation between the tumor and the other two thirds of the esophagus for approximately 4 cm. A biopsy and frozen section showed the mass to be benign schwannoma. With meticulous dissection the tumor was dissected from the esophageal muscle and mucosa, then it was excised in conjunction with a segment of involved vagus nerve. In the involved portion of the esophagus in which the esophageal muscle was cut, the mucosa partially bulged out between the edges of the cut muscles. Pathologic analysis showed a 12.5 x 12 x 6.5-cm
chwannomas (neurilemomas) may arise in any nerve where Schwann cells occur. Schwann cell tumors of the peripheral nervous system were given the name neurilemoma by Stout and Carson in 1935 [l]. Such tumors characteristically produce few symptoms, exhibit slow growth, and rarely cause serious neurological disturbances. Schwannomas arising from the vagus nerve are rare; here, we report a vagal schwannoma involving the esophagus. A 67-year-old white man was admitted with a mass in the right parotid gland (for biopsy and possible operation). An admission chest roentgenogram revealed a large mass in the right posterior and lower mediastinum. The patient stated that he had had some difficulty in swallowing which gradually became worse. Before hospital admission the patient had been receiving captopril, 25 mg twice per day, and glipizide, 5 mg twice per day. Barium swallow showed narrowing of the distal esophagus with a large right posterior mediastinal mass, displacing the distal esophagus to the left (Fig 1). A chest computed tomographic scan showed involvement of the distal esophageal wall by tumor. The mucosa of the esophagus was essentially intact (Fig 2). On physical examination there was no cervical lymphadenopathy. The right parotid gland was enlarged (3 x 3-cm), firm, and fixed. No lesion was seen in the oral cavity, pharynx, or larynx. The chest was clear, and the cardiovascular examination results were within normal limits. Laboratory test results, including electrolyte levels, white blood cell count, hematocrit, and urinalysis, were within normal limits. Because of dysphagia and severe narrowing of the distal esophagus, it was decided to treat the mediastinal mass before biopsy and excision of the right parotid tumor. With the presumed diagnosis of a posterior mediastinal mass, most likely neurogenic in nature, the patient was explored through a right sixth intercostal space thoracotomy. At exploration there was a large movable mass located over the distal esophagus. There was no connec-
(Ann Thorac Surg 1991;52:1334-6)
Accepted for publication June 7, 1991. Address reprint requests to Dr Vaghei, Thoracic Vascular Section, VA Medical Center, Martinsburg, WV 25401.
Fix 1 . Lateral chest roentgenogratn with barium swallow.
Ann Thorac Surg 1991;52:1334-6
CASE REPORT VAGHEI AND YOST SCHWANNOMA INVOLVING ESOPHAGUS
1335
Fig 2. Chest computed tomographic scan of distal esophagus showing tumor involving the esophageal wall.
encapsulated mass weighing 580 g (Fig 3). Histologically, the tumor showed densely cellular spindle shaped cells in palisading arrangements (Antoni A pattern) with Verocay body formation, characteristic of benign schwannoma. Postoperative recovery was uneventful. A barium swallow days after 'peration showed minimum postoperative changes and an otherwise normal esophagus (Fig 4).
Fig 4. Barium swallow showing minimum postoperative changes and an ofhenvise nor ma^ esophagus.
Subsequently, after open biopsy of the parotid gland and diagnosis of undifferentiated carcinoma, the patient underwent parotidectomy and radiation therapy. At present he is asymptomatic. Chest roentgenograms remain normal.
Comment
Fig 3. Gross appearance of the tumor mass.
Intrathoracic neural tumors are uncommon, but account for 75%of tumors of the posterior mediastinum [2]. Vagal schwannoma is very rare. In 1990 Dabir and associates [3] reported 2 cases of vagal schwannoma involving the thoracic portion of the vagus nerve adjacent to the aortic arch. In reviewing the literature they found 27 cases of vagus nerve tumors of which 9 were schwannomas. In the same year Heitmiller and co-workers [4] reported another case of intrathoracic vagal schwannoma, for a total of 12 cases. In 1956 Davis and Brown [5] reported an intrathoracic neurofibroma of the left vagus nerve at the level of the gastroesophageal junction associated with diaphragmatic hernia. The patient had no digestive tract symptoms. In our patient, schwannoma arising form the vagus nerve involved the esophagus, causing dysphagia. Peripheral tumors are first identified as a painless mass, whereas intrathoracic tumors are usually identified incidentally on chest roentgenograms. When symptomatic, or if associated with Von Recklinghausen's disease, malignant schwannoma should be suspected. In our patient dysphagia was due to extraluminal pressure by a large
1336
Ann Thorac Surg 1991;5213346
CASE REPORT VAGHEI AND YOST SCHWANNOMA INVOLVING ESOPHAGUS
mass in an otherwise normal esophagus. These tumors are benign, but malignant degeneration has been reported [6]. Furrer and Fox [7]reported a case of fibrosarcoma of the left vagus sheath. In our case, biopsy and frozen section before the excision of the lesion was very helpful. The tumor was large and embedded in the wall of the esophagus. Without a diagnosis of benign schwannoma, one may mistakenly attempt to resect the esophagus. Nasogastric tube within the lumen of the esophagus was used as a guide, and the tumor was dissected from the esophageal muscle and mucosa without injuring the mucosa. Das Gupta and associates [8] reported that 49 or 16.1% of 303 patients with benign solitary schwannomas had a malignant tumor unrelated to peripheral nerves. Twentytwo patients gave a history of being treated for cancer before the development of a schwannoma. Sixteen patients had cancer concomitant with the benign solitary schwannoma, and cancer developed in 11 patients at a later date. Das Gupta and associates found this was a relatively high incidence of malignant tumors in otherwise normal patients. Although no conclusions regarding association of schwannomas with other types of cancers was drawn, they suggested that a careful study be made to ascertain if any such association does in fact exist. In
our patient, carcinoma of the right parotid gland was concomitant with the esophageal schwannoma. Enucleation of the vagal schwannoma from the esophageal wall is the treatment of choice. In the differential diagnosis of esophageal tumors, vagal schwannoma should be included.
References 1. Stout AP, Carson W. The peripheral manifestations of the
2. 3. 4.
5. 6. 7. 8.
specific nerve sheath tumor (neurilemoma). Am J Cancer 1935;24:751-96. Currier AR, Gale JW. Mediastinal Tumors. Arch Surg 1949;58: 797-818. Dabir RR, Piccinoe W Jr, Kittle CF. Intrathoracic tumors of the vagus nerve. Ann Thorac Surg 1990;50:494-7. Heitmiller RF, Labs JD, Lipsett PA. Vagal schwannoma. Ann Thorac Surg 1990;50:811-3. Davis C Jr, Brown G. lntrathoracic neurofibroma of the vagus nerve associated with a diaphragmatic hernia. J Thorac Surg 1957;33:532-6. Cavstens H, Schrodt G. Malignant transformation of a benign encapsulated neurilemoma. Ann J Clin Pathol 1969;51:4&9. Furrer ED, Fox IR. Perineural fibrosarcoma of the vagus sheath. World J Surg 1940;48:584-6. Das Gupta TK, Brasfield RD, Strong EW, Hajdusi SI. Benign solitary schwannomas (neurilemomas). Cancer 1969;24: 355-66.