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Intrathoracic neurofibroma of the vagus nerve
Brit.
3.
Dis.
Chest
(‘974)
68, 46
INTRATHORACIC THE C. L. SARIN*, Thoracic
Surgical
NEUROFIBROMA VAGUS NERVE M. H. BENNETT
Unit and Pathology
AND
Department,
J. W.
Harefield
OF
JACKSON
Hospital,
Harefield,
Middlesex
commonest neoplasm in the posterior mediastinum is a neurofibroma. The vagus nerve is but rarely involved. We recently came across such a case, and we report it here.
THE
Case Report A 22-year-old female was referred to the thoracic surgical unit of Harefield Hospital for investigation of an abnormal shadow in the mediastinum detected on mass miniature radiography. She was sympton-free. Clinical examination revealed a healthy female of average build. She had cu.&’au Zait spots all over the body and several soft swellings along the tenth to twelfth ribs on the right side. These had not changed appreciably in size over 2 years. The chest radiograph showed an abnormal shadow, convex to the left in the mediastinum, extending from the level of the aortic arch to the ninth thoracic vertebra. A further small shadow was seen behind the heart just above the diaphragm. In the lateral view the major shadow was seen to overlap and extend anterior to the descending aorta. The smaller one was behind the heart (Figs I, 2). An aortogram revealed a mass arising posteriorly and intimately related to the arch and the upper part of the descending aorta, but there was no evidence of aortic abnormality, nor was any vascular supply demonstrated from the tumour to the aorta. An exploratory thoracotomy was undertaken. A long cord-like tumour overlaid the arch of the aorta and descended in front of it. It extended upwards into the posterior triangle of the neck and its lower end passed through the oesophageal hiatus into the abdomen. This occupied the course of the vagus nerve. The phrenic nerve also showed a nodular thickening. The tumour was dissected in both directions and the accessible portion of it was excised. Postoperative recovery was uneventful. The specimen was an irregular cylindrical cord of tissue 20 cm long and 0.7 cm minimum diameter, with a smooth glistening surface and fusiform swellings throughout its length; the largest measured IO x 5 cm and 4 x 2.5 cm (Fig. 3). The cut surface of these swellings was homogeneous and opalescent. Microscopy showed loose spindle-celled tumour tissue of variable cellularity with long wavy cytoplasmic processes, between which there were multiple * Present address: Department Allen Park, Michigan, U.S.A.
of Surgery,
(Received
Wayne
for publication
Mqy
State University, 1973)
and V.A. Hospital,
INTRATHORACIC
FIG.
I.
Radiograph
NEUROFIBROMA
of the chest, Another smaller
showing shadow
OF
VAGUS
a mediastinal is seen lying
just
NERVE
shadow behind
47
with convexity the heart
to the
left.
nerve fibres, mainly non-medullated. There were more cellular areas in the largest swelling, with some pleomorphism, but no real indication of malignancy. Features were typical of the plexiform and nodular neurofibromatosis of von Recklinghausen’s disease. Discussion
Neurofibromas may occur singly, but are usually multiple swellings as part of von Recklinghausen’s disease. In the thoracic cavity they usually involve the spinal nerves or the sympathetic chain. They are usually symptomless and do not normally interfere with nerve conduction or give rise to any spontaneous impulses, though when situated in an enclosed space they may produce pressure symptoms. The vagus nerve, as it traverses the mediastinum, is particularly immune to reported 18 cases of intrathoracic neuroneoplastic change. Kent et al. (1944) fibroma and reviewed another 105 cases from the literature. None of these Between involved the vagus nerve. The incidence of malignancy was 41%. 1935 and 1956 only IO tumours of the vagus nerve were seen in the Nayo Clinic (Penido et al. 1957). Only 4 of these were intrathoracic. One was a neurofibroma associated with von Recklinghausen’s disease and the rest were
SARIN
48
FIG.
2. A I:omogram
FIG.
showing
3. A Specimen
the
tumour in the mediastinum behind the heart
of the excised
neurofibroma
and
of the
another
vagus
small
ET
Imass
AL.
just
nerve
schwannomas. We have been able to find only 12 cases of intrathoracic vagal neurofibroma, which are listed in Table I. There were 2 more cases reported by Oberman and Abel (1g6o), which are not included as their precise details
INTRATHORACIC
NEUROFIBROMA
OF
VAGUS
NERVE
49
were not available. Only one of these tumours (Parella 1950) was considered sarcomatous, but doubt has been cast on its malignant nature as the patient was alive 6 years later. TABLE
I.
Authors
INTRATHORACIC
NEUROFIBROMAS
OF
THE
VAGUS
Von Recklinghausen’s disease
NERVE
Site
Sex
Age
Blades & Dugan (‘943) Parella ( r 950) Gerbode & Marguiles
M
35
Yes
Left,
M M
51 24
No Yes
Right, cervicothoracic. Left, vagus and recurrent
Gi!?%m & Lillihei (‘954) Tuttle et al. (1956) Penido et al. (1957) Davis & Brown (1957) Ecker et al. (1963)
M
40
Yes
Bilateral
M F M F
No Yes No Yes Yes No Yes
Left, Left, Left, Right, Left, Right, Right,
below recurrent nerve multiple, mid-portion multiple, mid-portion above carina above aortic arch below recurrent nerve below recurrent nerve
F
Yes
Left,
cervicothoracic
Gayola et al. Pampari & Larerencza Present report
(1965)
M p
multiple,
below
vagus
and
recurrent
nerve
? Malignant nerve
recurrent
nerve
(I 959) abdominal
In 7 patients the tumour was on the left side, in 4 on the right side and in I bilateral. Eight of these were part of von Recklinghausen’s disease and 4 were isolated tumours. The tumours are usually detected on a routine radiograph taken at work or as part of mass miniature radiographic surveys, as in the present case. A diagnostic thoracotomy is usually necessary and the appearances at surgery are usually typical. The present case is the most extensive of the ones described. It extended from the neck, through the thorax and into the abdomen. Since vagal neurofibromas are seldom malignant only the accessible portion of the tumour should be excised. Vital structures, such as the recurrent laryngeal nerve, if not involved in the neoplastic process but lying adjacent, should be spared. Summary
A case of neurofibroma of the vagus, presenting as a mediastinal shadow on mass minature radiography, is described. It extended from the neck, through the thorax and into the abdomen. The rarity of this tumour has is emphasized and the relevant literature reviewed. REFERENCES BLADES, B. & DUGAN, D. G. (I 943) Resection of the left vagus neurofibromas. J. Amer. med. Ass., 123, pg. DAVIS, C., jun. & BROWN, G. (I 957) Intrathoracic neurofibroma with diaphragmatic hernia. 3. thorac. Surg., 33, 532.
nerve
for
multiple
of the vagus
intrathoracic nerve
associated
SARIN
50
ET
AL.
ECKER, R. R., TIMMES, J. J. & MISCALL, L. (1963) Neurogenic turnouts of the intrathoracic vagus nerve. Arch. Surg. 86, 222. GAYOLA, G., JANIS, M. & WEIL, P. H. (1965) Intrathoracic nerve sheath tumours of the vagus. 3. thorac. cardiovasc. Surg., 49, 4 I 2. GERBODE, F. & PVIARGUILES, G. S. (1952) Neurofibromatosis with intrathoracic neurofibroma of the vagus nerve. 3. thorac. Surg., 25, 429. GIBERTSON, V. A. & LILLIHEI, C. W. (1954) Bilateral intrathoracic neurofibroma of the vagus nerve with a note on the physiologic effects of the cervicothoracic vagotomy in man. A case report. 3. thorac. Surg., 28, 78. KENT, E. M., BLADES, B., VALLE, V. R. & GRAHAM, E. A. (1944) Intrathoracic neurogenic tumours. 3. thorac. Surg., 13, I 16. OBERMAN, H. A. & ABEL, M. R. (1960) Neurogenic neoplasms of the mediastinum. Cancer (Philad.),
13, 882.
PAMPARI, D. & LARERENCZA, C. A. (1959) A case of intrathoracic neurofibroma of the vagus nerve on a woman with von Recklinghausen’s disease. Surgery, 45, 470. PARELLA, G. S. (1950) Neurofibrosarcoma of the vagus nerve. .New Engl. 3, Med., 242, 324. PENIDO, J. R. F., DIDGE, H. W., CLAGGET, 0. H. & STARR, G. F. (1957) Tumours of the vagus nerve. Proc. Mayo Clin., 32, 239. TUTTLE, W. M., SANAI, V. & HARMS, H. P. (1956) Intrathoracic neurofibroma of the vagus nerve. 3. thorac. Surg., 31, 632.
3.
Dis.
Chest
(‘974)
68, 46
INTRATHORACIC THE C. L. SARIN*, Thoracic
Surgical
NEUROFIBROMA VAGUS NERVE M. H. BENNETT
Unit and Pathology
AND
Department,
J. W.
Harefield
OF
JACKSON
Hospital,
Harefield,
Middlesex
commonest neoplasm in the posterior mediastinum is a neurofibroma. The vagus nerve is but rarely involved. We recently came across such a case, and we report it here.
THE
Case Report A 22-year-old female was referred to the thoracic surgical unit of Harefield Hospital for investigation of an abnormal shadow in the mediastinum detected on mass miniature radiography. She was sympton-free. Clinical examination revealed a healthy female of average build. She had cu.&’au Zait spots all over the body and several soft swellings along the tenth to twelfth ribs on the right side. These had not changed appreciably in size over 2 years. The chest radiograph showed an abnormal shadow, convex to the left in the mediastinum, extending from the level of the aortic arch to the ninth thoracic vertebra. A further small shadow was seen behind the heart just above the diaphragm. In the lateral view the major shadow was seen to overlap and extend anterior to the descending aorta. The smaller one was behind the heart (Figs I, 2). An aortogram revealed a mass arising posteriorly and intimately related to the arch and the upper part of the descending aorta, but there was no evidence of aortic abnormality, nor was any vascular supply demonstrated from the tumour to the aorta. An exploratory thoracotomy was undertaken. A long cord-like tumour overlaid the arch of the aorta and descended in front of it. It extended upwards into the posterior triangle of the neck and its lower end passed through the oesophageal hiatus into the abdomen. This occupied the course of the vagus nerve. The phrenic nerve also showed a nodular thickening. The tumour was dissected in both directions and the accessible portion of it was excised. Postoperative recovery was uneventful. The specimen was an irregular cylindrical cord of tissue 20 cm long and 0.7 cm minimum diameter, with a smooth glistening surface and fusiform swellings throughout its length; the largest measured IO x 5 cm and 4 x 2.5 cm (Fig. 3). The cut surface of these swellings was homogeneous and opalescent. Microscopy showed loose spindle-celled tumour tissue of variable cellularity with long wavy cytoplasmic processes, between which there were multiple * Present address: Department Allen Park, Michigan, U.S.A.
of Surgery,
(Received
Wayne
for publication
Mqy
State University, 1973)
and V.A. Hospital,
INTRATHORACIC
FIG.
I.
Radiograph
NEUROFIBROMA
of the chest, Another smaller
showing shadow
OF
VAGUS
a mediastinal is seen lying
just
NERVE
shadow behind
47
with convexity the heart
to the
left.
nerve fibres, mainly non-medullated. There were more cellular areas in the largest swelling, with some pleomorphism, but no real indication of malignancy. Features were typical of the plexiform and nodular neurofibromatosis of von Recklinghausen’s disease. Discussion
Neurofibromas may occur singly, but are usually multiple swellings as part of von Recklinghausen’s disease. In the thoracic cavity they usually involve the spinal nerves or the sympathetic chain. They are usually symptomless and do not normally interfere with nerve conduction or give rise to any spontaneous impulses, though when situated in an enclosed space they may produce pressure symptoms. The vagus nerve, as it traverses the mediastinum, is particularly immune to reported 18 cases of intrathoracic neuroneoplastic change. Kent et al. (1944) fibroma and reviewed another 105 cases from the literature. None of these Between involved the vagus nerve. The incidence of malignancy was 41%. 1935 and 1956 only IO tumours of the vagus nerve were seen in the Nayo Clinic (Penido et al. 1957). Only 4 of these were intrathoracic. One was a neurofibroma associated with von Recklinghausen’s disease and the rest were
SARIN
48
FIG.
2. A I:omogram
FIG.
showing
3. A Specimen
the
tumour in the mediastinum behind the heart
of the excised
neurofibroma
and
of the
another
vagus
small
ET
Imass
AL.
just
nerve
schwannomas. We have been able to find only 12 cases of intrathoracic vagal neurofibroma, which are listed in Table I. There were 2 more cases reported by Oberman and Abel (1g6o), which are not included as their precise details
INTRATHORACIC
NEUROFIBROMA
OF
VAGUS
NERVE
49
were not available. Only one of these tumours (Parella 1950) was considered sarcomatous, but doubt has been cast on its malignant nature as the patient was alive 6 years later. TABLE
I.
Authors
INTRATHORACIC
NEUROFIBROMAS
OF
THE
VAGUS
Von Recklinghausen’s disease
NERVE
Site
Sex
Age
Blades & Dugan (‘943) Parella ( r 950) Gerbode & Marguiles
M
35
Yes
Left,
M M
51 24
No Yes
Right, cervicothoracic. Left, vagus and recurrent
Gi!?%m & Lillihei (‘954) Tuttle et al. (1956) Penido et al. (1957) Davis & Brown (1957) Ecker et al. (1963)
M
40
Yes
Bilateral
M F M F
No Yes No Yes Yes No Yes
Left, Left, Left, Right, Left, Right, Right,
below recurrent nerve multiple, mid-portion multiple, mid-portion above carina above aortic arch below recurrent nerve below recurrent nerve
F
Yes
Left,
cervicothoracic
Gayola et al. Pampari & Larerencza Present report
(1965)
M p
multiple,
below
vagus
and
recurrent
nerve
? Malignant nerve
recurrent
nerve
(I 959) abdominal
In 7 patients the tumour was on the left side, in 4 on the right side and in I bilateral. Eight of these were part of von Recklinghausen’s disease and 4 were isolated tumours. The tumours are usually detected on a routine radiograph taken at work or as part of mass miniature radiographic surveys, as in the present case. A diagnostic thoracotomy is usually necessary and the appearances at surgery are usually typical. The present case is the most extensive of the ones described. It extended from the neck, through the thorax and into the abdomen. Since vagal neurofibromas are seldom malignant only the accessible portion of the tumour should be excised. Vital structures, such as the recurrent laryngeal nerve, if not involved in the neoplastic process but lying adjacent, should be spared. Summary
A case of neurofibroma of the vagus, presenting as a mediastinal shadow on mass minature radiography, is described. It extended from the neck, through the thorax and into the abdomen. The rarity of this tumour has is emphasized and the relevant literature reviewed. REFERENCES BLADES, B. & DUGAN, D. G. (I 943) Resection of the left vagus neurofibromas. J. Amer. med. Ass., 123, pg. DAVIS, C., jun. & BROWN, G. (I 957) Intrathoracic neurofibroma with diaphragmatic hernia. 3. thorac. Surg., 33, 532.
nerve
for
multiple
of the vagus
intrathoracic nerve
associated
SARIN
50
ET
AL.
ECKER, R. R., TIMMES, J. J. & MISCALL, L. (1963) Neurogenic turnouts of the intrathoracic vagus nerve. Arch. Surg. 86, 222. GAYOLA, G., JANIS, M. & WEIL, P. H. (1965) Intrathoracic nerve sheath tumours of the vagus. 3. thorac. cardiovasc. Surg., 49, 4 I 2. GERBODE, F. & PVIARGUILES, G. S. (1952) Neurofibromatosis with intrathoracic neurofibroma of the vagus nerve. 3. thorac. Surg., 25, 429. GIBERTSON, V. A. & LILLIHEI, C. W. (1954) Bilateral intrathoracic neurofibroma of the vagus nerve with a note on the physiologic effects of the cervicothoracic vagotomy in man. A case report. 3. thorac. Surg., 28, 78. KENT, E. M., BLADES, B., VALLE, V. R. & GRAHAM, E. A. (1944) Intrathoracic neurogenic tumours. 3. thorac. Surg., 13, I 16. OBERMAN, H. A. & ABEL, M. R. (1960) Neurogenic neoplasms of the mediastinum. Cancer (Philad.),
13, 882.
PAMPARI, D. & LARERENCZA, C. A. (1959) A case of intrathoracic neurofibroma of the vagus nerve on a woman with von Recklinghausen’s disease. Surgery, 45, 470. PARELLA, G. S. (1950) Neurofibrosarcoma of the vagus nerve. .New Engl. 3, Med., 242, 324. PENIDO, J. R. F., DIDGE, H. W., CLAGGET, 0. H. & STARR, G. F. (1957) Tumours of the vagus nerve. Proc. Mayo Clin., 32, 239. TUTTLE, W. M., SANAI, V. & HARMS, H. P. (1956) Intrathoracic neurofibroma of the vagus nerve. 3. thorac. Surg., 31, 632.