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Case 10-2008: A 10-Year-Old Girl with Dyspnea on Exertion

Kenan E. Haver, M.D., Christopher J. Hartnick, M.D., Daniel P. Ryan, M.D., Randheer Shailam, M.D., and Eugene J. Mark, M.D.

Pr e sen tat ion of C a se

From the Pediatric Pulmonary Unit (K.E.H.) and the Departments of Pediatric Surgery (D.P.R.), Radiology (R.S.), and Pathology (E.J.M.), Massachusetts General Hospital; Pediatric Otolaryngology, Massachusetts Eye and Ear Infirmary (C.J.H.); and the Departments of Pediatrics (K.E.H.), Otology and Laryngology (C.J.H.), Surgery (D.P.R.), Radiology (R.S.), and Pathology (E.J.M.), Harvard Medical School. N Engl J Med 2008;358:1382-90.
Copyright 2008 Massachusetts Medical Society.

A 10-year-old girl was seen in the multidisciplinary Airway, Voice, and Swallowing Center for Children at the Massachusetts Eye and Ear Infirmary, associated with this hospital, because of dyspnea and noisy respirations. Approximately 3 weeks earlier, a sharp pain in her chest had developed, associated with shortness of breath, while she was walking home from school, a distance of a quarter to a half mile. Thereafter, she had dyspnea with exertion. Her respirations became audible on both inspiration and expiration during exercise but were normal at rest and during sleep. Two weeks before this evaluation, her pediatrician found that her lungs were clear on auscultation before and after exercise. Peak expiratory flow rate (the maximum flow rate generated during a forced expiratory maneuver) was measured at 75 liters per minute (1.2 liters per second), or 27% of the predicted value of 273.6 liters per minute (4.6 liters per second). After treatment with a short-acting bronchodilator, wheezing was heard on examination, and the peak flow increased to 100 liters per minute (1.7 liters per second, 37% of predicted value). Albuterol and fluticasone by metered-dose inhaler were prescribed, but after 12 days, the symptoms had worsened. On reexamination by her pediatrician, wheezing was audible during inspiration, and peak flow was unchanged at 100 liters per minute. A chest radiograph taken on the same day was reported to reveal no abnormalities. The in halers were discontinued, and she was referred to the outpatient pediatric pulmonary clinic of this hospital. Her symptoms were not accompanied by coughing, choking, or difficulty eating. The patient had been born at full-term by cesarean section, with a birth weight of 3.2 kg (7 lb). Growth and developmental milestones were normal. A diagnosis of gastroesophageal reflux disease had been made 2 years before the current evaluation, and it was treated with cimetidine as needed, approximately monthly. She had a history of snoring, without apnea. She had not received influenza or meningococcal vaccines; other immunizations were up to date. Cephalexin had caused urticaria. The patient was a student in elementary school. Her maternal grandmother

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had had ulcerative colitis and had died of leukeA mia, her maternal grandfather had bone cancer, 6 maternal aunts had lung cancer and ulcerative 5 Predicted curve colitis, and her paternal grandmother had throat 4 cancer. Her mother and other maternal relatives FEV1=0.60 3 had gastroesophageal reflux disease and ulcer 2 disease. Her mother had two benign colonic FEV3=1.40 1 polyps that were diagnosed by colonoscopy, and Expiratory flow-volume curve 0 5 months earlier she had had a viral illness with dyspnea that resolved; her father had a port-wine 1 Inspiratory flow-volume curve stain. Her siblings were healthy. 2 The patient appeared well. The height was 3 0 1 2 3 4 5 6 140.8 cm (50th percentile), the weight 33.3 kg Volume (liter) (73 lb, 50th percentile), the pulse 109 beats per minute, the respiratory rate 18 breaths per minute, and the oxygen saturation 100% while the B 6 patient was at rest and breathing ambient air. 5 Predicted curve Tympanic membranes revealed diminished light 4 reflex without effusions; the nares were not FEV1=2.35 3 flared, and clear rhinorrhea was present. The 2 tonsils were large but did not meet in the midExpiratory flow-volume curve line. The chest was symmetric, without hyperin1 flation. No stridor was noted on either tidal or 0 accentuated respiration. Mild wheezes, without 1 crackles, were heard bilaterally on tidal and 2 Inspiratory flow-volume curve forced breathing. The second heart sound was 3 accentuated, louder to the right of the upper 0 1 2 3 4 5 6 sternum than to the left, without splitting. There Volume (liter) was no clubbing or cyanosis of the fingers; the Figure 1. Results of Pulmonary-Function Tests. remainder of the examination was normal. RETAKE 1st AUTHOR: Haver Results ICM of tests performed 2 days before the current Results of pulmonary-function tests revealed 2nd FIGURE: 1 of 5 evaluation (Panel A) show that there is a plateau of REG F 3rd a forced vital capacity (FVC) of 111% of predictflow observed on both the inspiratory and expiratory CASE Revised ed value, a forced expiratory volume in 1 second flow-volume curves, indicating limitation of flow; these Line 4-C EMail SIZE ARTIST:the ts presence H/T H/T obstruction in findings suggest of a fixed (FEV1) of 30%, and an FEV1:FVC ratio of 30. Enon Combo the airway. Results of tests performed 3 months later There was a plateau of flow observed on both 16p6 AUTHOR, PLEASE NOTE: (Panel B) show normal inspiratory and expiratory flowthe inspiratory and expiratory flow-volume loops Figure has been redrawn and type has been reset. volume curves. FEV forced expiratory volume 1 denotes Please check carefully. (Fig. 1A). in 1 second, and FEV3 forced expiratory volume in Two days later, the patient was seen by an oto3 seconds. JOB: 35813 ISSUE: 03-27-08 laryngologist. On examination, there was stridor on inspiration. Transnasal fiberoptic laryngoscopy revealed mobile vocal folds. A transthoracic diagnosis. The differential diagnosis includes asthechocardiogram was normal. Three days later, a ma; congenital diseases affecting the lung, such as cystic fibrosis; and a disorder of the trachea, diagnostic procedure was performed. such as tracheomalacia or an intratracheal mass. In this patient, the flow-volume curve reveals Differ en t i a l Di agnosis abnormalities in both the expiratory and inspiDr. Kenan E. Haver: I participated in the care of this ratory limbs of the flow-volume loops (Fig. 1A). patient and am aware of the diagnosis. In this 10- A concave tracing, when increasing expiratory flow year-old child with a history of chest pain, dyspnea is represented on the positive side of zero on the on exertion, and noisy breathing, the results of spi- ordinate, represents airflow obstruction of the rometry testing are important in the differential type most commonly seen in asthma. In children
Flow (liters/sec) Flow (liters/sec)

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with asthma, treatment with a short-acting bronchodilator, such as albuterol, rapidly reverses airflow obstruction and will reverse the concavity of the flow-volume curve, which was not seen in this case. The flow-volume curves for children with cystic fibrosis will have a similar appearance the curve becomes more concave as the disease progresses and generally there is no improvement after treatment with bronchodilators. The expiratory limb of the flow-volume curve of children with tracheomalacia characteristically appears flat because the abnormally pliable trachea collapses with exhalation. In this child, both the expiratory and inspiratory limbs of the flow-volume curve were affected, making all these diagnoses unlikely. When there is a well-defined plateau observed on both the expiratory and inspiratory loops, which indicates limitation of flow in both phases of respiration, the problem is likely to be located in the large airways rather than in the distal airways or pulmonary parenchyma, and one thus needs to consider a fixed-airway obstruction involving the trachea. The obstruction could be either intrinsic (within the airway or airway wall) or extrinsic (from adjacent structures). The differential diagnosis in this case includes subglottic stenosis, hemangioma, complete tracheal ring, and possibly a foreign body. Congenital subglottic stenosis, hemangioma, and complete tracheal ring are likely to present well before 10 years of age. Subglottic stenosis can be acquired, but in this case there was no history of the use of instruments in the airway that might have resulted in scarring and stenosis. An inhaled foreign body could explain the sudden onset of chest pain and shortness of breath in a child who had been completely well; even without a clear history of aspiration of a foreign body, this possibility needed to be considered. I reviewed the spirometry results the day after the patients visit to the pulmonary medicine clinic. The history and the flow-volume curve suggested the need for rapid evaluation of her airway. I referred her to a pediatric otolaryngologist, who agreed to see her the next day. Dr. Christopher J. Hartnick: In view of this patients history and findings on spirometry, bronchoscopy clearly was indicated. A previous chest radiograph had shown no obvious tracheal stenosis below the level of the sternum. If there had

been a suggestion of such a narrowing, then pediatric or thoracic surgeons would have been asked to be present during bronchoscopy, in case either a sternal tracheotomy or extracorporeal membrane oxygenation was required because of complete low-airway obstruction. Two days later, she was taken to the operating room for rigid bronchoscopic evaluation of the airway. After induction of anesthesia without a muscle relaxant, and while the patient was breathing spontaneously, a rigid bronchoscope was introduced into the trachea. In a patient such as this one, with a possible obstructing tracheal lesion, it is important not to give a muscle relaxant during initiation of anesthesia and to allow the child to breathe spontaneously until the airway is safely secured. If difficulties had been encountered, a tracheotomy could have been performed below the site of obstruction while the child was being ventilated by mask. In this case, the bronchoscope was inserted distal to the vocal cords without difficulty. A large, circumferential mass with a smooth surface was noted in the airway in the middle-to-lower trachea (Fig. 2A). The mass obstructed approximately 80% of the tracheal lumen. A 4-mm telescope could be passed distal to the mass to reveal a patent distal airway to the carina. Next we had to decide whether it was safe to perform a biopsy of the mass. Many lesions that can produce a tracheal mass, such as hemangioma, hemangiopericytoma, inflammatory pseudotumor, carcinoid tumor, Langerhans-cell histiocytosis, juvenile xanthogranuloma, and respiratory papilloma, can bleed extensively when a biopsy is performed.1-5 Thus, with the airway secure, an intraoperative computed tomographic (CT) scan with contrast material was performed to assess the vascularity of the mass as well as to assess its size and whether it extended beyond the lumen of the trachea. Dr. Randheer Shailam: CT of the chest after the administration of intravenous contrast material was performed during the rigid bronchoscopy. Sequential axial sections through the middle trachea and coronal reconstructions (Fig. 3A and 3B) demonstrate a solid mass, 18 mm by 13 mm by 18 mm, with both an intraluminal and a transmural component in the middle portion of the trachea. The large luminal component causes 80 to 90% occlusion of the midtracheal airway. The mass is sharply circumscribed and seems to arise

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from the posterior and right lateral walls of the trachea. The transmural component extends close to and may involve the pleura of the right superior mediastinum. The mass is relatively homogeneous in attenuation, with no visible fat or calcification, and no large arterial feeding vessels are seen. Magnified images (Fig. 3C) show the transmural extension of the mass, and a threedimensional rendition of the tracheobronchial tree (Fig. 3D) shows the midtracheal mass, with a defect in the tracheal wall indicating transmural extension. Virtual bronchoscopic images reconstructed from our axial images (Fig. 1 in the Supplementary Appendix, available with the full text of this article at www.nejm.org) depict what one might expect to see on bronchoscopy: a large intraluminal mass arising from the posterior and right lateral walls, with a normal carina distal to the mass. The lungs were clear, without atelectasis, and no mediastinal lymphadenopathy and no skeletal lesions were present. Posteroanterior and lateral chest radiographs obtained at another hospital 1 week before the CT scan were reexamined (Fig. 3E and 3F). The radiographs initially were reported as normal, but in retrospect, a soft-tissue density is seen interrupting the tracheal air column on both the posteroanterior and lateral views, which correlates with the abnormalities seen on the CT scan performed 7 days later. In summary, there is a solid, enhancing mass in the middle portion of the trachea that has both transmural and endoluminal components and nearly occludes the tracheal lumen. There is no evidence of metastasis. Dr. Hartnick: Since the mass appeared neither friable nor hypervascular, a large intratracheal biopsy was performed, both to secure a diagnosis and to debulk the tumor (Fig. 2B). We used the Nd:YAG (neodymium:yttriumaluminum garnet) laser traveling through the side port of the rigid bronchoscope to achieve hemostasis, which allowed the airway to be maintained during coagulation. The patient was awakened from the procedure and taken to the recovery room in stable condition. The final pathology report was awaited. The differential diagnosis of this tracheal tumor includes malignant tumors such as squamous-cell carcinoma, adenoid cystic carcinoma,

Figure 2. Bronchoscopic Evaluation of the Trachea. At the time of the initial evaluation (Panel A), a large, smooth-surfaced intratracheal mass that obstructed 80 RETAKE 1st AUTHOR: Haver toICM 90% of the airway was seen. A bronchoscopic image 2nd FIGURE: 2a-c of 5 REG F taken after the tracheal mass had been debulked and a 3rd biopsy specimen examined (Panel B) shows removal of CASE Revised Line most of the intraluminal portion of4-C the tumor. A bronEMail SIZE ARTIST: mst H/T H/T the tracheal rechoscopic image taken 6 months after Enon 16p6 Combo section and reanastomosis (Panel C) shows a normalAUTHOR, PLEASE NOTE: appearing trachea.
Figure has been redrawn and type has been reset. Please check carefully.

JOB: 35813 ISSUE: 03-27-08and neurogenic tumors such as neurofibroma neurilemmoma, other neoplasms such as carcinoid and mucoepidermoid tumors, and respiratory papilloma. In one series of 189 cases from

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Figure 3. Radiologic Images of the Chest. Axial (Panel A) and coronal (Panel B) CT images of the chest obtained after the administration of contrast medium, performed during rigid bronchoscopy, show a solid, enhancing mass arising from the posterior and right lateral walls RETAKE 1st AUTHOR: Haver of the middle portion of the trachea (arrows) that has endoluminal and transmural components. A magnified axial ICM 2nd CT image (Panel C) shows the margins the mass outlined; the arrows show the adjacent normal tracheal wall. FIGURE: 3a-f of 5 REG F of 3rd A reconstructed three-dimensional rendition of the trachea and bronchial tree (Panel D) shows the filling defect CASE Revised (arrow) in the tracheal lumen. Chest radiographs from 1 week before4-C evaluation show a soft-tissue density interrupting Line EMail SIZE mst the tracheal air column (arrows) on both ARTIST: posteroanterior (Panel and lateral (Panel F) views, corresponding to the H/T E) H/T Enon 33p9 Combo lesion seen in the CT images.
AUTHOR, PLEASE NOTE: Figure has been redrawn and type has been reset. Please check carefully.

this hospital,6 36% of primary tracheal tumors JOB: 35813 were squamous-cell carcinomas, and 40% were adenoid cystic carcinomas. Among 12 patients younger than 19 years of age, the most common neoplasms were carcinoid tumor (33%) and mucoepidermoid carcinoma (17%). Other tumors in children included granular-cell tumor, invasive fibrous tumor, malignant fibrous histiocytoma, and adenocarcinoma.7

Pathol o gic a l Dis cus sion ISSUE: 03-27-08

Dr. Eugene J. Mark: The biopsy specimen showed a tumor with small, round cells. The intraoperative frozen-section diagnosis included carcinoid tumor, rhabdomyosarcoma, and lymphoma. Examination of permanent sections confirmed the epithelioid nature of the tumor cells, which had oval, regular nuclei with delicate nuclear membranes (Fig. 4A). Cytologic features of cancer were not present, and mitoses were sparse. The tumor cells Cl inic a l Di agnosis formed small nests, and arborizing blood vessels Primary tumor of the trachea, possibly carcinoid were present, suggesting the possibility of a glomus tumor (Fig. 4B).8,9 Immunohistochemical and tumor or mucoepidermoid carcinoma.

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V V

D
N

Basal lamina

Figure 4. Pathological Features of the Tumor. A biopsy specimen of the tumor (Panel A) shows a relatively uniform population of small cells with dark and oval nuclei (hematoxylin and eosin); arborizing vessels are present within the tumor (arrows). At higher magnification RETAKE 1st AUTHOR: Haverappear bland, and (Panel B, hematoxylin and eosin), ICM the tumor-cell nuclei the arborizing vessels can be seen (V). 2nd 4a-d positive of 5 Immunohistochemical staining of the cells was for smooth-muscle actin (Panel C, immunoperoxidase). REG tumor F FIGURE: 3rd An electron micrograph (Panel D)CASE shows basal lamina (arrows) surrounding the glomus cells. Revised
EMail Enon

ARTIST: mst

electron microscopical studies were required to numerous mitochondria. Basal lamina (Fig. 4D) AUTHOR, PLEASE NOTE: confirm this diagnosis. and pinocytotic Figure has been redrawn and type has been reset. vesicles were present. These Please check carefully. are not specific, but they are consistent An immunohistochemical evaluation showed changes that the tumor cells stained for smooth-muscle with the presence of a glomus tumor.10,11 JOB: 35813 ISSUE: 03-27-08 actin (Fig. 4C), muscle actin, and vimentin. There Glomus tumors typically occur in the nail beds, was weak staining for synaptophysin and neuron- but they also occur in muscle, tendons, ligaments, specific enolase. The tumor cells did not stain for periosteum, and visceral organs. The glomus cell endothelial markers (CD31 or CD34), lymphocytic is a modified smooth-muscle cell that surrounds or histiocytic markers (leukocyte common anti- an arteriovenous anastomosis, forming a glomus gen, CD3, CD20, CD43, CD1A, or CD68), epithe- body, which functions in temperature regulation. lial markers (cytokeratin AE1/5.2, cytokeratin 903), Glomus tumors are almost always benign, as this melanoma marker (S100 protein), rhabdomyo- lesion appears to be, although cases with atypisarcoma markers (myogenin, desmin, or myoD), cal features have been described.12 or another neuroendocrine marker (chromoGlomus tumors of the trachea are distinctly granin A). Ultrastructurally, the neoplastic cells uncommon; all of the reported cases have been were epithelioid, with cytoplasmic processes and in adults, and the most common presenting symp-

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tom is dyspnea on exertion, as was seen in this case.13-17 The tumor typically occurs in the lower third of the trachea in the posterior wall, where mucus glands and vessels are most numerous; this is the most common site of origin for most tracheal tumors. Glomus tumors in the trachea, bronchi, and lung overall are sufficiently uncommon that they are not tabulated in the World Health Organization classification of lung tumors.

Dis cus sion of M a nage men t

Dr. Hartnick: In considering the management of this patients condition, it is reassuring that none of the primary tracheal glomus tumors that have been reported have been associated with metastases.13-17 Three cases were reported to have extratracheal extension, but these appeared to have pushing margins outside the lumen of the trachea and did not appear to be infiltrative. One case of a glomus tumor in the chest extended into the trachea and required radiation therapy, since it could not be completely excised. In our patient, there was no evidence of metastatic disease, but the tumor was locally extensive, and we consulted Pediatric Surgery to assist in management.
Surgical Management

Dr. Daniel P. Ryan: In considering removal of this lesion, one could ask whether it could be destroyed endoscopically with a laser. However, we saw that the tumor extended outside the wall of the trachea posteriorly, so that any endoluminal treatment would ultimately fail. We believed that removing the segment of the involved trachea was the only option to cure this patient. The next decision was whether to approach the lesion through the neck or through the chest. Considerations included the location of the lesion, how bulky the extratracheal component was, and whether the airway could be mobilized sufficiently both to remove the segment with the tumor and then to create a tension-free anastomosis that would allow primary healing. Approximately 1 month after the initial bronchoscopy, the child was admitted to the hospital for surgery. To plan the best approach to treatment, the first step in the operating room was repeated rigid bronchoscopy with the patient un-

Figure 5. Operative Resection of Tracheal Glomus Tumor. The anterior trachea was exposed (Panel A) and the RETAKE 1st AUTHOR: Haver lateral ICM blood supply left intact (arrow) to facilitate heal2nd of 5 ing after primary 5a-c reanastomosis of the trachea. The REG F FIGURE: 3rd segment of trachea containing the tumor was resected CASE Revised 4-C (Panel segment (Panel C), a large, EMail B). On opening theLine SIZE ARTIST: mst H/T from H/T dark-red tumor is seen arising the posterior wall. Enon
Combo 16p6

der almost completely regrown in the month since the JOB: 35813 ISSUE: 03-27-08 debulking. The tumor was about 2 cm in length; the distal edge was about 2.5 cm above the carina, and the proximal edge was about 5 cm below the cricoid. The overall length of the involved trachea was thus small enough to allow for complete excision with a primary anastomosis. With the patients chin extended, we could see the site of the tumor in her neck externally and we could see the light from the bronchoscope in the su-

AUTHOR, PLEASE NOTE: Figure has been redrawn and type has been reset. Please check carefully. anesthesia. We found that the tumor had

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prasternal notch, so we were confident that removing the tumor through the neck incision would be possible. We made a low-collar incision in the neck, which allowed us to expose the trachea from the larynx to the carina, if necessary (Fig. 5A). We exposed the anterior trachea and left the lateral blood supply intact. We then performed flexible bronchoscopy to identify the endoluminal extent of the lesion and marked it with a needle at the site where we planned to divide the trachea. After the trachea was divided, ventilation was maintained by placing sterile tubing in the distal airway, and the segment containing the tumor was removed (Fig. 5B and 5C). Frozen sections of the tracheal margins were negative for tumor. The trachea was reconstructed18 with multiple interrupted Vicryl sutures for an end-to-end anastomosis. By not mobilizing the trachea and by keeping the lateral blood supply intact, one can expect primary healing. The airway was brought together without tension by flexing the chin toward the chest. This maneuver brings the larynx and upper trachea down to the lower trachea to allow a tension-free anastomosis. We kept the patient in that position for about a week after the surgery in order to allow tension-free healing. Finally, before she was awakened, we performed another flexible bronchoscopy to clean the airway and make sure that no blood or secretions remained that might compromise her breathing. We were able to extubate the trachea in the operating room, and the postoperative course was uneventful. Dr. Mark: Pathological examination of the resected specimen showed that the tumor was completely excised; it invaded the tracheal wall, but the surgical margins were free of tumor.
References
1. Naiman AN, Bouvier R, Colreavy MP,

Dr. Mark S. Pasternack (Medicine and Pediatrics): In retrospect, can you comment on the episode of chest pain? Dr. Haver: I do not have an explanation for the chest pain. It did not recur. Dr. Ben Z. Pilch (Pathology): The more common subungual glomus tumor typically presents with pain; in fact, it presents with excruciating pain. Dr. Nancy Lee Harris (Pathology): Can you tell us how the patient is doing? Dr. Hartnick: Surveillance bronchoscopy at 1, 3, and 6 months postoperatively showed no evidence of recurrence (Fig. 2C). She had transient paresis of the left vocal fold, which manifested as mild dysphonia, but she had no evidence of clinical aspiration, and a modified barium swallow did not result in gross or occult aspiration. It is now approaching 2 years since the operation, and a recent bronchoscopy showed a normal trachea with no evidence of recurrent tumor. Dr. Haver: Three months after the operation, both vocal folds had regained full mobility, and the pulmonary-function studies were normal (Fig. 1B). Nine months later, the patient had started running again; in a 5-km race, not only was she able to keep up with her father, who is a marathon runner, but he had a hard time keeping up with her. We plan to continue annual follow-up examinations in the multidisciplinary clinic with tracheal magnetic resonance imaging and spirometry.

A nat omic a l Di agnosis

Glomus tumor of the trachea.
No potential conflict of interest relevant to this article was reported.

Bellon G, Froehlich P. Tracheal juvenile xanthogranuloma in a child. Int J Pediatr Otorhinolaryngol 2004;68:1469-72. 2. Fridlender ZG, Glazer M, Amir G, Berkman N. Obstructing tracheal pulmonary Langerhans cell histiocytosis. Chest 2005;128:1057-8. 3. Kim TS, Han J, Kim GY, Lee KS, Kim H, Kim J. Pulmonary inflammatory pseudotumor (inflammatory myofibroblastic tumor): CT features with pathologic correlation. J Comput Assist Tomogr 2005; 29:633-9.

4. Gaissert HA, Grillo HC, Shadmehr

MB, et al. Uncommon primary tracheal tumors. Ann Thorac Surg 2006;82:268-72. 5. Kayser K, Zink S, Link B, et al. Endobronchial juvenile hemangioma a case report of a neonate including immunohistochemical monitoring and nuclear, cellular, and vascular morphometry. Virchows Arch 2001;438:192-7. 6. Grillo HC, Mathisen DJ. Primary tracheal tumors: treatment and results. Ann Thorac Surg 1990;49:69-77. 7. Gaissert HA, Mathisen DJ, Grillo HC, Vacanti JP, Wain JC. Tracheobronchial

sleeve resection in children and adolescents. J Pediatr Surg 1994;29:192-7. 8. Fabich DR, Hafez G-R. Glomangioma of the trachea. Cancer 1980;45:2337-41. 9. Shin DH, Park SS, Lee JH, Park MH, Lee JD. Oncocytic glomus tumor of the trachea. Chest 1990;98:1021-3. 10. Heard BE, Dewar A, Firman RK, Lennox SC. One very rare and one new tracheal tumour found by electron microscopy: glomus tumour and acinic cell tumour resembling carcinoid tumours by light microscopy. Thorax 1982;37:97-103. 11. Ito H, Motohiro K, Nomura S, Tahara

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E. Glomus tumor of the trachea: immunohistochemical and electron microscopic studies. Pathol Res Pract 1988;183:778-84. 12. Folpe AL, Fanburg-Smith JC, Miettinen M, Weiss SW. Atypical and malignant glomus tumors: analysis of 52 cases, with a proposal for the reclassification of glomus tumors. Am J Surg Pathol 2001;25:1-12. 13. Menaissy YM, Gall AA, Mansour KA. Glomus tumor of the trachea. Ann Thorac Surg 2000;70:295-7.
14. Garcia-Prats MD, Sotelo-Rodriguez 16. Altinok T, Cakir E, Gulhan E, Tastepe

MT, Ballestin C, et al. Glomus tumour of the trachea: report of a case with microscopic, ultrastructural and immunohistochemical examination and review of the literature. Histopathology 1991;19:459-64. 15. Chan J, Fogarty G, Ball D, Wright G, Slavin J. Tracheo-innominate artery fistula following stenting, surgery and radiotherapy for large glomus tumor of the chest. ANZ J Surg 2005;75:252-3.

I. Tracheal glomus tumor. J Thorac Cardiovasc Surg 2006;132:201-2. 17. Gowan RT, Shamji FM, Perkins DG, Maziak DE. Glomus tumor of the trachea. Ann Thorac Surg 2001;72:598-600. 18. Grillo HC. Surgery of the trachea and bronchi. Hamilton, ON, Canada: B.C. Decker, 2004.
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