Primary intratracheal neurilemoma

J

THORAC CARDIOVASC SURG

85:313-320, 1983

Brief communications Primary intratracheal neurilemoma Alan G. Horovitz, M.D., Kamal G. Khalil, M.D., F.A.C.S., Regina R. Verani, M.D., Agnes M. Guthrie, M.D., and Daniel F. Cowan, M.D.,

Houston, Texas From The University of Texas Medical School of Houston, The Texas Medical Center, Houston, Texas. We report a case in a 38-year-old white woman of a benign primary intratracheal neurilemoma that recurred 12 years after an initial endoscopic excision. Of the 12 intratracheal neurilemomas that have previously been reported. all occurred in white persons in an age range of6 to 7l years and most were located in the lower trachea and produced symptoms of cough and wheezing.

Benign neurogenous tumors arise from the peripheral nervous system,' are composed of Schwann cells," and appear as neurilemomas or neurofibromas." 4 These tumors are slow growing and may present at any age. Although neurogenous tumors are the most common primary tumors of the posterior mediastinum.v ? they are exceedingly rare in the trachea. 4. 8. 9 The first report of a tracheal neurilemoma was by Straus and Guckien l Oin 1951. A few neurilemomas, previously classified as endobronchial ,10--12 are actually tracheal tumors. Twelve cases of intratracheal neurilemomas have been reported and we now report a thirteenth case. Case report. A 38-year-old white woman presented to The University of Texas Medical School at Houston, Hermann Hospital, with a 3 month history of difficult respirations, progressive shortness of breath, and paroxysms of dry coughjng that had intensified during the 3 days prior to admission. Physical examination revealed persistent post-tussive, coarse rales bilaterally, mild suprasternal retractions, and flaring of the alae nasi. The resting respiratory rate was 26 to 32 breaths/min. The remainder of the physical examination was unremarkable. The patient had a "bite" resection of a single posterior

Fig. 1. Frontal tomogram of the trachea. Note oval intraluminal defect just above the carina, occupying most of the tracheal width. Steep course of left lower main bronchus is due to collapse of the left lower lobe.

Address for reprints: A. G. Horovitz, M.D., Department of Pathology and Laboratory Medicine, The University of Texas Medical School at Houston, P.O. Box 20708, Houston, Texas 77025.

tracheal, supracarinal, obstructing neurilemoma via endoscopy in 1970. Follow-up endoscopy in 1970 confirmed that the tumor had been extirpated. There was no other relevant past history or family history of pulmonary disease, fibromatosis, stigmata of von Recklinghausen's disease, neurilemomas, or other specific medical problems. Arterial blood gas values while the patient breathed room air were pH 7.45, P0 2 54 mm Hg, Pco, 37 rnm Hg, bicar-

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Fig. 2. Gross resection specimen, superior aspect, demonstrating intraluminal and extrinsic lobes of a tracheal mass. Note vascularity of the smooth, intraluminal surface surrounded by the tracheal ring (arrows). bonate 26 mmoles/L, and base excess + 3 mmoles/L. A chest roentgenogram showed atelectasis of the lower lobe of the left lung. Tomography of the tracheobronchial tree revealed an ovoid soft tissue sessile mass, 1.2 by 1.6 em, arising from the posterior wall of the lower trachea, 0.5 em above the carina (Fig. 1). Barium esophagogram showed mild compression of the anterior wall of the esophagus. Computed tomography was not possible because of uncontrollable coughing when the patient assumed a supine position. By endoscopy, a sessile tumor proximal to the carina and arising from the left posterolateral tracheal wall on a narrow pedicle filled approximately 80% of the tracheal lumen. The surface of the tumor was smooth and marked with prominent vessels. A purulent bronchitis was present in the right and left bronchi. A biopsy was not performed. On March 12, 1982, the patient underwent right posterolateral thoracotomy with exposure of the lower half of the trachea and both main-stem bronchi. By means of standard anesthetic and surgical techniques.P the lowest three tracheal rings and the carina were resected, including the pedicle of the tumor and a substantial extratracheal component. The carina was reconstructed by suturing the adjacent surfaces of the left and right main bronchi. The double-barrelled bronchial tube was sutured to the distal end of the transected trachea with interrupted 3-0 polypropylene sutures. The patient was extubated on the second postoperative day. On the seventh postoperative day, repeat bronchoscopy confirmed satisfactory healing of the suture lines and wide patency of both main-stem bronchi. The patient was discharged on the tenth postoperative day and has resumed her managerial position. Pathological anatomy. The resected segment of trachea was 0.8 em long and 1.8 em in diameter. Approximately 80% of the lumen was occluded by the intraluminal portion of a bilobed dumbbell-shaped tumor (Fig. 2). The intraluminal portion of the tumor was 1.6 em in diameter, and the extrinsic portion was 2.0 by 2.0 by 1.8 em. The constricted intramural

segment passed between two tracheal rings. Resection margins were free of tumor. The tumor was encapsulated, rubbery, and pale tan-yellow without foci of necrosis or hemorrhage. Large vessels were evident on the surface. Microscopically, it was composed of spindle cells in compact (Antoni type A) and loose myxoid (Antoni type B) patterns. Palisading cells and nuclei formed typical Verocay bodies in the Antoni type A regions. Capsular invasion, nuclear pleomorphism, and mitoses were absent. Ultramicroscopically, Schwann cells with characteristic cytoplasmic processes invested by basement membrane ramified and interdigitated with each other. The interstitium contained collagen fibers in high concentration. Collagen fibers of long periodicity (long-spacing) and of increased width (Luse bodies) were associated with cytoplasmic processes. The gross, histologic, and electron microscopic findings characterize neurilemomas at any site. 14 - 18 The tissue removed during the initial operation in 1970 had the identical histologic features of a neurilemoma (Fig. 3).

Review of the literature. Fifteen cases of isolated primary tracheal neurogenic tumors have been reported: three neurofibromas (not components of von Recklinghausen's disease )19-21 and 12 neurilernomas'" 10. 12.22-29 (Table I). The age of the patients at presentation varied from 6 to 71 years. Six of 11 cases in which sex was specified occurred in female subjects. Six of 11 whose site was specified occurred in the lower one third of the trachea; others occurred in the middle and upper thirds. All patients whose race was identified have been white; however, the Russian reports do not specify race. Discussion. In the trachea, neurogenous tumors occur most frequently in the distal third, followed in decreasing order by the proximal and then middle

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Brief communications

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Fig. 3. Histologic comparison of specimens resected in 1970 (A) and in 1982 (B); demonstrate Antoni type A tissue regions containing numerous Verocay bodies with palisading nuclei and compact orderly cellular arrangements.

thirds. 26- 3 0 Although they may occur in either sex, there is a predilection for the occurrence of neurilemomas in women; the three reported intratracheal neurofibromas (unassociated with von Recklinghausens disease) occurred in men." 20--22, 28 Benign tracheal lesions are more common in children (93% of reported cases) than in adults (50%).22, 31-33 Dumbbell-shaped lesions of the trachea are usually malignant and the intraluminal and extrinsic extension represents mural invasion by a mucosal lesion. An exception to this tenet are the neurilemomas of the trachea that arise from intramural neurogenous tissue. The expanding lesion is compressed by the tracheal cartilaginous rings, so that the tumor is forced to extrude into the tracheal lumen or out into the extrinsic soft tissue. Neurilemomas and neurofibromas both arise from the Schwann cell of the nerve sheath. These tumors -vary from each other in that neurilemomas are single, encapsulated lesions attached to a nerve but containing no neurites. Neurofibromas are usually multiple, unencapsulated, occur within the nerve sheath, and contain neurites. Neurilemomas are only rarely associated with von Recklinghausen's disease, rarely undergo malignant change, and often contain benign degenerative changes. Neurofibromas are frequently associated with von Recklinghausen's disease, and 12% undergo ma-

lignant change; benign degenerative changes are less frequently seen than in neurilemomas.t" 34 The clinical presentation of symptoms relates to the location of the lesion. With lesions in the trachea, the most common symptoms are cough, wheezing, difficult respirations, hemoptysis, shortness of breath, and occasionally fever. 4. 28. 31 Pain is only rarely associated with benign neurilemomas. The inclusion of intratracheal tumors in a differential diagnosis of wheezing is exceedingly rare, so that the average delay time in diagnosis is 10 to 15 months after the patient first seeks medical attention. 26 Radiography demonstrates the tracheal mass and outlines its intraluminal extent. 35 Chest radiographs show secondary complications such as collapse or pneumonia. However, fluoroscopy, xeroradiographs, and tomograms of the trachea, especially using filtered beam and multiple projections, depict the size, shape, configuration, and attachment site of the tumor. Functional dynamic tracheal abnormalities, such as tracheal collapse in different phases of respiration, can be identified by fluoroscopy and recorded in videotape or cine-mode. Computed tomography may be the optimal medium for determining the extent of the extraluminal mass." The extratracheal component is traditionally studied by tomography, esophagography, or a combi-

The Journal of Thoracic and Cardiovascular Surgery

3 16 Brief communications

Table I. Primary intratracheal neurilemoma Ref. No.

Symptoms

Therapy

Cough, fever, pneumonia Difficulty breathing, cough, bronchitis Cough, difficulty breathing Difficulty breathing, stridor on exertion Difficulty breathing, cough, weakness Difficulty breathing, asthma, hemoptysis Difficulty breathing, cough, pneumonia Cough, difficulty breathing

Two-stage endoscopy, silver nitrate cautery Endoscopic excision, cautery Surgical resection

Well, 6 mo

Endoscopic excision, repeated after 2 wk Surgical transection postop. Tracheal sleeve resection Tracheal fissure resection Surgical resection*

Well, 2'/2 yr

10

1951

28

C

M

L

29

1953

21

C

M

L

25

1953

35

C

F

M

27

1961

23

C

F

L

23

1964

6

C

F

L

12

1970

43

C

F

L

26

1973

21

C

M

U

22

1975

28

C

M

U

22 24

1975 1977

71

C

F

M

Recent onset of asthma, cough

28

1979

36

C

M

L

8

1981

23

C

F

U

1982

38

C

F

L

Difficulty breathing, hemoptysis, wheezing Difficulty breathing, wheezing, shortness of breath Difficulty breathing, coughing, shortness of breath

Current case

Follow-up

No symptoms, 5 yr Well, I mo

Pneumonia, died Well, 2'12 yr Well, I yr Lost to follow-up

Surgical resection* Endoscopy aborted, tracheal sleeve resection Endoscopy with electrocagulation

Lost to follow-up Renal insufficiency, mediastinitis" died postop. Well 4 yr*

Tracheal sleeve resection

No symptoms, I yr

I. Endoscopic removal 2. Tracheal transection

I. Recurrence, 12 yr 2. Asymptomatic

Legend: C, Caucasian. L, M, and U, Lower, middle, and uppertrachea. *Personal communication.

nation of the two. Identifying the configuration of the extratracheal component and differentiating it from a peritracheal vascular ring may require angiography or computed tomography. flexible tube bronchoscopy, the definitive diagnostic procedure for tracheal tumors, should be carried out under local or light general anesthesia with the patient breathing spontaneously to permit clearing of secretions in the event that rapid bleeding occurs. Extreme care should be taken in handling the tumor to avoid swelling and further compromise of the airway. If the local appearance is typical of a benign lesion and especially if the tumor appears highly vascular, a biopsy should be avoided or deferred until the time of definitive surgical resection. Precise localization of the tumor pedicle and its relationship to other landmarks in the trachea and main bronchi are helpful in planning the surgical resection. The technique of carinal reconstruction employed in this patient was feasible because of the limited extent of trachel resection. In more extensive resections, it is

easier to anastomose one main bronchus to the trachea and then implant the other bronchus into the side of the trachea, as described by Grillo. t3 The prognosis for patients with neurilemomas removed endoscopically or surgically is usually excellent. Endoscopy allows direct visualization, biospy, and, if the tumor is amenably located, may allow resection.': 10. 16. 30, 31, 33, 37-41 The value of endoscopic excision, however, is qualified. The current case is an example of the slow-growing nature (12 years) of a neurilemoma and the possibility of recurrence after endoscopic excision. The possibility of late recurrenee": 24, 27, 42 in patients treated by endoscopy must be considered. The neurilemoma in the current case recurred at the same site 12 years after initial "piecemeal" resection through the endoscope and required subsequent surgical excision. Five other reported lesions had been treated by endoscopy: Two required follow-up endoscopy to achieve complete removal'< 27 and two were removed successfully-'" 29; in the fifth case, endoscopy

Volume 85 Number 2 February, 1983

was aborted in favor of thoracotomy and tracheal sleeve resection." No recurrences have been noted in the patients undergoing thoracotomy and surgical resection. Surgical resection is advocated as the procedure of choice for complete excision of extratracheal extension of the tumor. 13. 42 We thank Dr. Harvey S. Rosenberg for reviewing the manuscript. REFERENCES

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4 5 6 7

8 9 10 II 12

13 14

15

16 17

18

19

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