Ann Thorac Surg 2005;79:e15-e16
© 2005 The Society of Thoracic Surgeons
Case report
Granular Cell Tumor of the Trachea in a Child
Franziska Stieglitz, MDa,b,
Richard Kitz, MDa,
Hans J. Schäfers, MDc,
Olaf Eickmeier, MDa,
Markus Rose, MD, MPHa,
Christian Fellbaum, MDb,
Stefan Zielen, MD*,a
a Department of Pediatrics and Frankfurt, Germany
b Department of Pathology, Johann Wolfgang Goethe Universität, Frankfurt, Germany
c Department of Cardiovascular Surgery, University of Saarland, Homburg, Saar, Germany
Accepted for publication December 30, 2003.
* Address reprint requests to Prof Zielen, Zentrum für Kinderheilkunde, Theodor Stern Kai 7, Frankfurt 60590, Germany
stefan.zielen{at}kgu.de
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Abstract
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Granular cell tumors are uncommon benign neoplasms. Their location is mostly in the head and neck region; appearance in other parts of the body is rare, but it has been reported. We present the case of a 14-year-old girl with a granular cell tumor of the trachea. The tumor was incidentally found at bronchoscopy performed to exclude suspected foreign body aspiration. It was located in the ventral part of the main carina. Biopsies revealed the histologic pattern of a benign granular cell tumor. The girl underwent resection of the main carina followed by reconstruction of a neo-carina with both main bronchi. She has not had any recurrence of the tumor during 3 years of follow-up.
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Introduction
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Granular cell tumors (GCTs) develop between the third and forth decade of life. They have been described in all parts of the body, predominantly in the head and neck region. The appearance in the trachea of a child is extremely rare.
The 14-year-old girl presented with symptoms of aspiration pneumonia. Previously there had been an aspiration event with severe coughing while eating a fruit salad containing nuts. After a symptom-free interval of 12 hours, she had a fever develop and respiration-dependent chest pain with intermittent dyspnea. At that time she was seen by her pediatrician. He started her on antibiotics, bronchodilator therapy, and arranged for hospitalization to rule out foreign body aspiration. The chest roentgenogram revealed an infiltrate in the right lower lobe. On admission to the hospital, which was 3 days after onset of the symptoms, the patient was in reduced general condition. On admission there were no cough, no fever, and no dyspnea. Auscultation of the lung showed sharp respiratory sounds and wheezing in the right lower lobe. The white blood cell count was 8.95 K/µL with 4.92% lymphocytes, 92.6% neutrophil, and no bands; C-reactive protein was 63 mg/L. Erythrocyte sedimentation rate was 13/30; all other blood measurements were within normal limits. In view of a possible aspiration event and the results of the physical examination, our initial diagnosis was aspiration pneumonia. Surprisingly the bronchoscopy showed no foreign body, but did show a tumor-like mass located in the main carina. The mass had a white colored surface and a vascular pattern that measured approximately 1.5 x 0.5 cm (Fig 1).
Biopsies of the mass were taken and histologic and immunologic investigations verified a GCT of the trachea. Microscopically, the lesion consisted of large, elongated cells with prominent granular cytoplasm (eosinophilic granulations). The immunohistochemically stained tumor cells were strong for S-100 protein and was positive for periodic acid-Schiff stain, which confirmed the diagnosis of a GCT. There was no proliferation activity in Ki-67 staining, which is a marker for cell proliferation. After the histologic result, a computed tomographic scan and a magnetic resonance image of the chest were performed, showing the tumor with extension from the lower part of the trachea to its bifurcation.
In order to achieve long-term airway patency, a decision was made to resect the tumor. Six weeks after the initial presentation, the girl underwent surgery. After induction of anesthesia the trachea was intubated with a single lumen tube. The chest was opened through a right posterolateral thoracotomy and the carina dissected. The carina was found bulging with some tissue growth outside the airway wall. Mechanical ventilation was interrupted briefly, and the trachea was transsected cranial to the palpable tumor. The left main bronchus was transsected in its middle portion, the distal left main bronchus intubated through the surgical field, and ventilation was continued. The right main bronchus was resected en bloc with the carina. Using interrupted sutures (PDS [Ethicon, Hamburg, Germany]) a neo-carina was constructed with both residual main bronchi; this was then anastomosed in end-to-end fashion to the distal trachea. Intermittent apnea was used during this procedure to facilitate suturing. The patient was extubated in the operating room and had an uneventful postoperative course. She was discharged from the hospital on postoperative day 10.
Recently the patient had her third annual follow-up bronchoscopy, which showed no evidence of disease. All prior bronchoscopies were also normal. In addition, she had an allergic rhinoconjunctivitis develop with allergic bronchial asthma and an allergy to pollen for which she has now been treated with specific immunotherapy.
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Comment
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Granular cell tumors are uncommon benign neoplasms. They occur most commonly in the head and neck region, particularly in the tongue, and have been described in all parts of the body [1]. The appearance of GCTs in the trachea is rare. Of 145 cases in the last 30 years, Burton and colleagues' [1] review presented only 6 cases in the trachea. Granular cell tumors predominantly develop between the third and fourth decade of life. Although primary pulmonary tumors in children are a rarity in themselves, reports of this type of tumor and its location in children are extremely rare. Thus far only a few cases have been reported [2]. The first case report was that of a 15-year-old girl in 1939 with a bronchial GCT.
Symptoms of GCTs can vary and are mostly determined by the location of the tumor and its size. Cough, recurrent pneumonia, and atelectasis can be symptoms of tracheobronchial obstruction by the tumor and should raise suspicion [2, 3]. As our case shows, there can be no typical symptoms at all, or according to other authors, only mild ones such as hoarseness [4]. It can be speculated that the tumor itself was the leading cause of pneumonia. However, the tumor mass was rather small and it is well known that an obstruction of up to 50% to 75% of the tracheal lumen does not necessarily produce symptoms. With 73% of tracheal GCTs being located intraluminally, bronchoscopy with diagnostic biopsy appears to be an effective tool for diagnosis.
Abrikossoff [5] first described the case of a GCT in 1926, which was called "granular cell myoblastoma" based on its similarity to myoblasts. Later, there was much discussion about the origin of this tumor. Derivation from fibroblasts or histiocytes was also assumed. Recent studies suggest an origin in Schwann cells with the presence of S-100 protein supporting this hypothesis [6]. The histologic pattern of the tumor consists of polygonal cells with centrally placed nuclei and prominent granular eosinophilic cytoplasm [1]. The periodic acid-Schiff stain of the cytoplasmic granulations was positive both before and after diastase digestion, which is usually evidence for neutral mucopolysaccharides. The granular cells reacted strongly with S-100 protein and neuron-specific enolase and weakly with the Leucine-7 antibody (CD57) [3].
Although malignant variants of GCTs have been reported in a very few cases [6], they have never been documented in infants or children and have not been reported in the trachea [1, 7]. Nonetheless a preoperative radiologic evaluation for localization of the lesion and exclusion of occult metastatic disease should be performed [7]. Because GCTs usually fail to respond to radiotherapy and have been shown to grow progressively, wide local excision of these tumors is the treatment of choice. In those cases where extensive surgical excision would produce unacceptable morbidity, adjuvant radiotherapy may have a role in the treatment of certain GCTs. There is one report in the literature of a patient who was treated with adjuvant radiation therapy and followed up without evidence of recurrence [8]. The tumor should not be treated with bronchoscopic techniques like electrocautery or laser surgery, because of transbronchial extension that prevents complete ablation by these techniques. Endoluminal treatment would thus expose a young patient to a high risk of recurrence. Postoperative follow-up examinations (eg, bronchoscopy or computed tomographic scan) are highly recommended every 12 months for a 5-year period to detect recurrence.
In conclusion, we presented a 14-year-old girl with a GCT of the trachea, which was an extremely rare case. Because of the progressive growth of the tumor with local tissue destruction, wide excision was the therapy of choice. Although the majority of GCTs are benign, a small fraction of GCTs (as many as 2%) become malignant. Therefore, the mainstay treatment is the wide local excision of the lesion and regular annual postoperative follow-ups.
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Acknowledgments
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We thank Prof Dr Dieter Harms, Institute of Paidopathology of the University of Kiel, Germany, for reviewing the case.
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References
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- Abrikossoff A. Ueber Myome, ausgehend von der quergestreiften willkuerlichen Muskulatur. Virchows Arch Pathol Anat. 1926;260:215–233
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- Rosenthal SA, Livolsi VA, Turrisi AT. Adjuvant radiotherapy for recurrent granular cell tumor. Cancer. 1990;65(4):897–900[Medline]
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Abstract
Introduction
